Physical Activity in Adolescents with Haemophilia: Difference between revisions

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<div class="noeditbox">Welcome to [[Contemporary and Emerging Issues in Physiotherapy Practice|Queen Margaret University's Current and Emerging Roles in Physiotherapy Practice project]]. This space was created by and for the students at Queen Margaret University in Edinburgh, UK. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox">
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== '''Introduction'''  ==
== Introduction==


<br>Welcome to this online wiki resource which will focus on ‘The Role of a Physiotherapist in Promotion and Management of Physical Activity in Adolescents with Haemophilia.’ This wiki has been designed as learning resource for final year physiotherapy students and newly qualified physiotherapists. It is an online self-study module and should take approximately 10 hours to complete.<br>  
Physical activity and exercise, are often used synonymously, however, physical activity is defined as&nbsp;“as any bodily movement produced by skeletal muscles that require energy expenditure”&nbsp;<ref name="WHO 2016">World Health Organization. Physical Activity. http://www.who.int/topics/physical_activity/en/ (accessed 25 January 2016).</ref>, and exercise is a subsection of physical activity, involving planned or structured activity which aims to improve or maintain physical fitness <ref name="Caperson et al. 2016">Casperson SJ, Powell KE, Christenson GM. Physical activity, exercise, and physical fitness: definitions and distinctions for health-related research. Public health Rep 1985;100;(2):126–131. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1424733/ (accessed 25 January 2016).</ref>.<br><br>The physiotherapist's role in treating and managing adolescents with haemophilia (AWH) is extremely important, and recent developments within this profession have increased its value <ref name="Buzzard and Beeton 2008">Buzzard B, Beeton K. Physiotherapy Management of Haemophilia. Oxford: Wiley, 2008.</ref>. One of the key interventions involved in its treatment is the promotion of physical activity <ref name="WFH 2015">World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 October 2015).</ref>.


Before exploring this topic it is important to clearly define the difference between physical activity and exercise, as they will be used synonymously throughout the module.<br>
== Definition ==


Physical activity is defined as&nbsp;“as any bodily movement produced by skeletal muscles that requires energy expenditure” (WHO 2016). <br>Exercise is a subsection of physical activity, involving planned or structured activity which aims to improve or maintain physical fitness (Casperson et al. 1985).<br><br>We hope you find this resource useful and informative.<br><br>  
[[Hemophilia|Haemophilia]] is a rare inherited disorder where blood in the body has an inability to produce sufficient clotting factor, a protein which controls bleeding <ref name="NHS choices 2015">NHS Choices. Haemophilia. http://www.nhs.uk/conditions/haemophilia/Pages/Introduction.aspx (accessed 7 Jan 2016).</ref>. This lack of clotting factor can lead to severe and prolonged bleeds which may cause permanent damage to the site of haemorrhage and surrounding tissues<ref name="Severity">World Federation of Haemophilia. Severity of hemophilia. http://www.wfh.org/en/page.aspx?pid=643 (accessed 7 January 2016).</ref>. There are an estimated 6000 people living in the United Kingdom with the condition. It occurs mainly through a gene passed from parent to child on the X chromosome, i.e. it mainly affects males<ref name="Great Ormond Street 2015">Great Ormond Street Hospital. What causes Haemophilia? http://www.gosh.nhs.uk/medical-information/search-medical-conditions/haemophilia (accessed 10 Jan 2016).</ref>.&nbsp;<br>There are four main types of haemophilia. The table below shows the missing clotting factor and commonality for each type:[[Image:Types table.jpg|center|500px]]
* Haemophilia A is the most common type affecting around 80% of the haemophilic population<ref name="Haem Society 2015 A">The Haemophilia Society. Haemophilia A.fckLRhttp://www.haemophilia.org.uk/bleeding_disorders/bleeding_disorders/haemophilia_a  (accessed 9 November 2015).</ref><span style="line-height: 1.5em; font-size: 13.28px;">. </span>This is where the individual will either have low levels of clotting factor eight or it is completely missing<span style="line-height: 1.5em; font-size: 13.28px;">.&nbsp;</span>
* Haemophilia B is less common. This is where clotting factor nine is either very low or is missing from the blood. Around 20% of PWH will have this type<ref name="Haem Society 2015 B">The Haemophilia Society. Haemophilia B. fckLRhttp://www.haemophilia.org.uk/bleeding_disorders/bleeding_disorders/haemophilia_fckLRb (accessed 9 November 2015).</ref>.
* Haemophilia C is ten times rarer than type A and differs from both A and B as it can present in both genders. However, this is extremely rare and can only occur when the mother and father are both carriers of the gene&nbsp;<ref name="Haemophilia Information 2013">Haemophilia Information. Haemophilia Genetics. http://www.hemophilia-information.com/hemophilia-genetics.html (accessed 12 September 2015).</ref>. Individuals with type C will have low levels of clotting factor eleven, or this will be completed absent.&nbsp;
* Acquired Haemophilia is extremely rare. This type is not inherited in the same way, as it is an auto-immune disorder. This is where the body's immune system attacks the clotting factors. This condition can also affect both males and females <ref name="Grethlein 2014">Grethlein SJ. Acquired Haemophilia. http://emedicine.medscape.com/article/211186-overview#a2fckLR (accessed 7 January 2016).</ref>.
=== Classifications and Severities ===


=== Aims and Learning Outcomes ===
The table below categorises&nbsp;haemophilia into mild, moderate and severe, showing the percentage of sufferers in each category <ref name="Preston et al. 2004">Preston FE, Kitchen S, Jennings L, Woods TA, Mackris M. SSC/ISTH classification of hemophilia A: can hemophilia center laboratories achieve the new criteria? J Thromb Haemost 2004;(2):271-4. http://www.ncbi.nlm.nih.gov/pubmed/14995989 (accessed 10 January 2016).</ref>.[[Image:Severityhaem.jpg|center|600px]]<br>Like other disorders, haemophilia can affect individuals to varying degrees. The symptoms can be mild, moderate or severe, depending on the level of clotting factor present<ref name="nhs choices 2016">NHS Choices. Haemophilia symptoms. http://www.nhs.uk/Conditions/Haemophilia/Pages/Symptoms.aspx (accessed 10 Jan 2016).</ref>. 
=== Multidisciplinary Team Involvement&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;  ===
Despite recent development in the management of the condition, patients still require comprehensive multidisciplinary care throughout all stages of their life <ref name="Breakey et al. 2010" />. A multidisciplinary team involves a range of professionals who collaborate together to structure a treatment plan for an individual, aiming to achieve high quality patient-centered care <ref name="Department of Health 2015">Department of Health. Multidisciplinary teams (MDT’s). http://www.health.nt.gov.au/Cancer_Services/CanNET_NT/Multidisciplinary_Teams/index.aspx (accessed 7 Jan 2016).</ref>.


The collaboration of all professionals allows a holistic approach to be considered which takes into account the individual’s physical, social, emotional, cultural and spiritual needs <ref name="CSP 2015">Chartered Society of Physiotherapy. Physiotherapy works for social care – Why Physiotherapy? http://www.csp.org.uk/physiotherapy-works-social-care (accessed 10 Jan 2016).</ref>. This is especially important during the transition from childhood into adulthood which can cause additional stress for those with chronic disorders. Therefore during this time they need for the MDT to work together is essential <ref name="Breakey et al. 2010">Breakey VR, Blanchette VS, Bolton-Maggs PHB. Towards comprehensive care in transition for young people with haemophilia. Haemophilia 2010;16(6):848-857. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02249.x/abstract (accessed 18 Dec 2015).</ref>. This may mean referring the patient to another team member to allow high-quality treatment, specific to the patients needs.&nbsp;[[Image:Mdt altered.jpg|center|725px]]


== The Role of the Physiotherapist ==


The aims of this wiki are as follows:<br>
=== Assessment  ===


*To present a learning resource for final year physiotherapy students and newly qualified graduates which aims to develop their knowledge and understanding of the management of adolescents with haemophilia.
[[Image:Joint swelling.jpg|250px|right]]When assessing a patients condition or evaluating the success of interventions it is often beneficial for the physiotherapist to use a standardised outcome measure. These offer an objective measure which can help to monitor change in a patients symptoms<ref>American Physical Therapy Association. Outcome Measures in Patient Care. http://www.apta.org/OutcomeMeasures/ (accessed 20 Jan 2016).</ref>.<br><br>Symptoms of a joint bleed may include swollen and hot joints, pain in the area and stiffness when mobilising. Many patients have described the overall feeling as an 'aura' surrounding the joint<ref name="Haem care">Haemophilia Care. How to recognise a bleed. http://www.haemophiliacare.co.uk/how-to-recognise-a-bleed.html (accessed 7 Jan 2016)</ref>, for example swelling in the knee during a joint bleed.
*To introduce final year physiotherapy students and newly qualified graduates to the skills, competencies and resources which can be utilised within their clinical practice in order to offer more effective and comprehensive management of adolescents with haemophilia.<br>


Learning Outcomes:<br>The following learning outcomes have been constructed using the levels determined by Bloom’s Taxonomy to facilitate learning development (Bloom et al. 1956).  
=== Outcome Measures: ===
[[Image:Hjhs.jpg|414x414px|right]]'The Haemophilia Joint Health Score'&nbsp;<ref name="International Prophlaxis Study Group 2015">International Prophylaxis Study Group. HJHS. http://ipsg.ca/working-groups/physical-therapy/info/hjhs (accessed 15 Dec 2015).</ref> is designed for physiotherapists and recommended for use by professionals specialised in haemophilia treatment. The outcome measure takes approximately 45 – 60 minutes to complete and is designed specifically for children with haemophilia. It examines the condition of a joint affected by a bleed and can be used to demonstrate degeneration in a joint over time.[[File:Paediatric_Haemophilia_Activities_List_picture.jpg|right|401x401px]]
The 'Paediatric Haemophilia Activities List’ is another outcome measure used for individuals with haemophilia. Adolescents will complete the child/teenager version explaining to what extent their haemophilia impacts upon their daily activities The questionnaire can then be used by healthcare professionals involved in their care<ref name="WFH ACTIVITES">World Federation of Haemophilia, Paediatric Haemophilia Activities List: http://www.wfh.org/en/page.aspx?pid=886 (accessed 20 Oct 2015).</ref>.<br> 


[[Image:Blooms_taxonomy.png|center|300px]]
Although outcome measures exist for the assessment of children with haemophilia many of these have demonstrated poor validity and reliability<ref>Stephensen D, Drechsler WI, Scott OM. Outcome measures monitoring physical function in children with haemophilia: a systematic review. Haemophilia 2014;20:306-321. http://onlinelibrary.wiley.com/doi/10.1111/hae.12299/epdf (accessed 19 Jan 2016).</ref>.


<br>Blooms Taxonomy, shown above, demonstrates a hierarchy of learning. This wiki aims to encompass various levels of this hierarchy throughout in order to optimise the learning of the reader. The identified learning aims have been constructed to suit this learning theory.<br>Through completion of this learning module and related tasks, final year physiotherapy students and newly qualified physiotherapy graduates will be able to:<br>[[Image:Learning_outcomes.jpg|center|500px]]
=== Management  ===


The main role of the physiotherapist when treating an AWH involves the management of impairments from a musculoskeletal perspective, aiming to restore and improve function. Some of these include: haemarthoses, synovitis, joint contractures, haemotomas and haemophilic arthropathys<ref>Buzzard B, Beeton K. Physiotherapy Management of Haemophilia. Wiley-Blackwell, 2010.</ref>.


During the acute phase, the physiotherapist will attempt to minimise bleeding using the PRICE regime (protection, rest, ice, compression elevation)<ref name="HAEM CARE">Haemophilia Care. How is Physiotherapy used in Haemophilia care. http://www.haemophiliacare.co.uk/physiotherapy.html (accessed 10 Oct 2015).</ref>. This will also work in conjunction with factor replacement if necessary.[[Image:PRICE2.png|center|500px]]<br>Following the acute phase, the physiotherapy management will focus on regaining full range of movement, muscle strength and reducing pain<ref>Talking Joints. Let’s talk about physiotherapy for haemophilia patients. https://www.novonordisk.com/content/dam/Denmark/HQ/aboutus/documents/cpih-2015-talkingjoints-brochure-lets-talk-physiotherapy.pdf (accessed 10 Oct 2016).</ref>. As soon as the pain and swelling begin to decrease, the patient should be encouraged to gradually increase the joint range aiming to achieve complete extension. This should be done actively by the patient to encourage muscle contraction, however passive movements may initially be used. It is vital that early active muscle control occurs to prevent the loss of joint movement <ref name="Hermans et al. 2011">Hermans C, De Moerloose P, Fischer K, Holstein K, Klamroth R, Lambert T, Lavigne-Lissalde G, Perez R, Richards M, Dolan G. European Haemophilia Therapy Standardisation Board. Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations. Haemophilia 2011;17(3):383-92. http://www.ncbi.nlm.nih.gov/pubmed/21323794 (accessed 30 Sept 2015).</ref>;<ref name="Gomis et al. 2009">Gomis M, Querol F, Gallach JE, Gonzalez LM, Aznar JA. Exercise and sport in the treatment of haemophilic patients: a systematic review. Haemophilia 2009;15(1):43-54. http://www.ncbi.nlm.nih.gov/pubmed/18721151 (accessed 10 Jan 2016).</ref>;<ref name="Mulder 2006">Mulder K. Exercises for people with haemophilia. Montreal: World Federation of Haemophilia. 2006. http://scholar.google.co.uk/scholar_url?url=http%3A%2F%2Fhaemophilia.ie%2FPDF%2FExercise_Guide_med.pdf(accessed 22 Jan 2016).</ref>. Rehabilitation involving active exercises and proprioceptive training must then be continued until full pre-bleed joint ROM and function is restored <ref name="Heijnen and Buzzard 2005">Heijnen L, Buzzard BB. The role of physical therapy and rehabilitation in the management of hemophilia in developing countries. Semin Thromb Hemost 2005;31(5):513-7. http://www.ncbi.nlm.nih.gov/pubmed/16276459 (accessed 25 Sept 2015).</ref>.


=== Why is there a need for this Physiopedia page? ===
Another key role of the physiotherapist when treating an AWH is promotion of physical activity. Exercise is encouraged as it will improve the child’s fitness, reduce obesity levels, improve muscular strength, and reduce frequency of bleeding episodes and joint deterioration <ref name="Blamey 2010">Blamey G, Forsyth A, Zourikian N, Short L, Jankovic N, Kleijn PDE, Flannery T. Comprehensive elements of a physiotherapy exercise program in haemophilia – a global perspective. World Federation of Haemophilia 2010;16:136-145 http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02312.x/abstract;jsessionid=BB3B137A0C21342D378A949A977850CA.f04t01fckLR(accessed 10 Oct 2015).</ref>. The World Federation of Haemophilia<ref>World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).</ref> advise particular focus towards strengthening, co-ordination, achieving a healthy body weight and improving self-esteem.  As well as the physical aspects concerned, a major aspect of physiotherapy management focuses on education and advice.  


<br>The physiotherapists role in treating and managing adolescents with haemophilia (AWH) is extremely important, and recent developments within the profession have increased its value (Buzzard and Beeton 2008). One of the key interventions involved in its treatment is promotion of physical activity (World Federation of Hemophilia 2015).<br><br>As physiotherapists, we have an obligation and responsibility to actively promote physical activity in this population, as well as educating the individuals correctly in self-management of their life-long condition, the intention being to maximise quality of life (Buzzard and Beeton 2000). It is therefore essential for us to have a deep understanding of this condition and the effects and benefits of physical activity and exercise.<br><br>  
==== Education ====
Below is a mind map which summarises key points regarding the advice that should be offered to patients around physical activity.&nbsp;<br>


=== How to navigate this wiki  ===
[[Image:Education picture.jpg|637x637px]]


[[Image:Posture.jpg|right|400px]]
The physiotherapist plays a crucial role in:


<br>  
# Educating patients regarding physical activity, exercise prescription and selection of appropriate activities<ref name="Heijnen 1993">Heijnen L. Rehabilitation and the role of the physical therapist. Southeast Asian J Trop Med Public Health 1993;24(Suppl. 1):26–9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3570911/ (accessed 25 Sept 2015).</ref>.
# Ensuring patients, relatives and peers are made aware of the positive impact physical activity can have, helping to prevent joint damage and functional impairment<ref name="Buzzard 1997">Buzzard B. Physiotherapy for Prevention and Treatment of Chronic Haemophilic Synovitis. Clinical Orthopaedics and Related Research. Orthopaedics and Related Research 1997;343:42-46. http://www.ncbi.nlm.nih.gov/pubmed/9345204 (accessed 9 Jan 2016).</ref>. Individuals who regularly exercise will also experience fewer bleeds<ref name="Tiktinsky 2002">Tiktinsky R, Falk B, Heim M, Martinovitz U. The effect of resistance training on the frequency of bleeding in haemophilia patients: a pilot study. Haemophilia 2002;8:22–7. http://www.ncbi.nlm.nih.gov/pubmed/11886461 (accessed 15 Dec 2015).</ref>.
# Informing patients that just as with healthy individuals, exercise has various benefits. Additional benefits&nbsp;such as enhanced lean tissue mass, improved fitness and strength and a decrease in body fat percentage are apparent during puberty in adolescents<ref>U.S. Department of Health and Human Services. Physical Activity Guidelines Advisory Committee report. http://health.gov/paguidelines/report/pdf/committeereport.pdf (accessed 17 Jan 2016).</ref><span style="line-height: 1.5em; font-size: 13.28px;">.</span>
# Advising them to attempt non-contact sports such as swimming, walking, cycling, golf, archery, badminton, rowing and sailing in place of contact sports like rugby <ref name="WFH guidelines">World Federation of Hemophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd. 2012;2:6-73. 2012. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).</ref>.
# Working in conjunction with the medical team when advising timing of prophylaxis appropriate to their chosen sport or activity<ref name="McGee">McGee S et al. Organized sports participation and the association with injury in paediatric patients with haemophilia. Haemophilia 2015;21(4):538-542. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2786948/ (accessed 15 Nov 2015).</ref>;<ref name="WFH GUIDELINES">World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).</ref>.
# Educating on and encouraging the use of protective equipment e.g if patient has a target joint, or does not take prophylaxis prior to activity <ref name="Philpott et al. 2010">Philpott J, Houghton K, Luke A. Physical activity recommendations for children with specific chronic health conditions: Juvenile idiopathic arthritis, hemophilia, asthma and cystic fibrosis. Paediatrics Child Health 2010;15(4):213-218. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2866314/ fckLR(accessed 6 Oct 2015).</ref>. Protective equipment is discussed later in the module.
# Educating school personnel regarding suitable activities for the child, discussing immediate care in case of a bleed, and explaining modifications in activities that may be necessary following bleeds<ref name="WFH GUIDELINES" />.&nbsp;<br>Some parents have found a traffic light coding system beneficial to inform teachers at school of the appropriate actions to take in event of specific injuries or bleed. This is also alluded to in the “get fit and active” video later in the module.<br><br>  


The wiki is organised under the following three main sections:
&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;Figure 10. Traffic light system
<div><br>
#Background of Haemophilia
#The Role of the Physiotherapist
#Benefits of Physical Activity<br>


<br>  
[[Image:Traffic lights.jpg|center|600px]]<br> <br>  


<br>
=== Physical Activity Promotion  ===


'''How long you should spend working through each section:'''
It is believed that physical activity in this population is reduced compared to healthy age-matched peers<ref name="Darby et al. 2007">Darby SC, Wan Kan S, Spooner RJ, Giangrande PLF, Hill FGH, Hay CRM, Lee CA, Ludlam CA, Williams M. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 2007;110(3):815-825. :http://www.bloodjournal.org/content/110/3/815?sso-checked=true (accessed 2 Jan 2016).</ref>. The Kings Fund<ref name="The Kings Fund">The Kings Fund. Inequalities in life expectancy Changes over time and implications for policy. http://www.kingsfund.org.uk/sites/files/kf/field/field_publication_file/inequalities-in-life-expectancy-kings-fund-aug15.pdf (accessed 24 Nov 2015).</ref> acknowledge health behaviour patterns to impact an individual's overall health by as much as 40%, when compared to healthcare which contributes only 10%. Therefore, as Physiotherapists, our role in health and activity promotion is crucial in order to influence positive behavioural change to impact upon health<ref>Chartered Society of Physiotherapy. Physio 15: Physios can improve public health by influencing behaviour and promoting physical activity. http://www.csp.org.uk/news/2015/10/21/physio-15-physios-can-improve-public-health-influencing-behaviour-promoting-physical (accessed 27 Nov 2015).</ref>.&nbsp;


<br>  
Health promotion involves supporting people to gain control and responsibility over improving their own health. It extends beyond focusing on individual behaviour and includes a variety of social and environmental interventions<ref>World Health Organization. Health Promotion. http://www.who.int/topics/health_promotion/en/ (accessed 28 Dec 2015).</ref>.


<br>  
The Corporate Policy and Strategy Committee have introduced a Physical Activity for Health Pledge which the NHS is engaging with. This pledge involves ensuring primary care staff have the necessary skills and resources available to assess physical activity levels. It also ensures they offer education detailing recommended minimum requirements for physical activity, brief advice and intervention, along with increasing awareness of available community resources. Unpublished data from NHS Health Scotland reveals only 13% of primary care practitioners are presently aware of the recommended weekly activity levels <ref name="Corporate Policy and Strategy Committee 2015">Corporate Policy and Strategy Committee. Physical Activity for Health Pledge. 2-12 www.edinburgh.gov.uk (accessed 8 Oct 2015).</ref>. It can therefore be assumed this is not currently being promoted effectively.


<br>
The following video [https://www.youtube.com/watch?v=aUaInS6HIGo "23 and a 1/2 hours"] strongly promotes physical activity for at least 30 minutes of your day. Although not specific to the haemophilic population, the take home message can be followed for AWH.&nbsp;


<br>  
Although various studies have described the benefits of engaging in physical activity for people with haemophilia (PWH), results from research conducted in the USA among adolescent haemophilia patients have demonstrated a lack of knowledge concerning the role of physical activity in managing their condition<ref>Nazzaro AM, Owens S, Hoots WK, Larson KL. Knowledge, attitudes, and behaviors of youths in the US hemophilia population: results of a national survey. American Journal of Public Health 2006;96(9):1618-1622. http://www.ncbi.nlm.nih.gov/pubmed/16873741 (accessed 3 Jan 2016).</ref>. Whilst it is recognised this information is important, this study has not been conducted in the UK so results must be generalised with caution. However, this suggests more work must be done to promote physical activity and emphasise its positive role in enhancing the lives of PWH <ref name="Negrier et al. 2013">Negrier C, Seuser A, Forsyth A, Lobet S, Llinas A, Roas M, Heijnen L. The benefits of exercise for patients with haemophilia and recommendations for safe and effective physical activity. Haemophilia 2013;19(4):487-498. http://www.ncbi.nlm.nih.gov/pubmed/23534844 (accessed 7 Jan 2016).</ref>.


<br>  
Physical activity promotion for health exists worldwide <ref name="Heath et al. 2012">Heath GW, Parra DC, Sarmiento OL, Andersen LB, Owen N, Goenka S, Montes F, Brownson RC. Evidence-based intervention in physical activity: lessons from around the world. Lancet 2012; 380(9838):272-81. http://www.ncbi.nlm.nih.gov/pubmed/22818939 (accessed 26 Jan 2016).</ref>. The benefits of such in the general population are widely known, providing health improvements along with the potential for enhancing disease outcomes<ref>World Health Organization. Health Promotion. http://www.who.int/topics/health_promotion/en/ (accessed 28 Dec 2015).</ref>. National and international guideliness set out clear recommendations on the physical activity levels required to promote health<ref name="Department of Health">Department of Health. Multidisciplinary teams (MDT’s). http://www.health.nt.gov.au/Cancer_Services/CanNET_NT/Multidisciplinary_Teams/index.aspx (accessed 7 Jan 2016).</ref>. Although many studies have reported the benefits of participation in physical activity for PWH, researchers in the USA have identified a lack of knowledge among&nbsp;young haemophilia patients (aged 13–18 years) as to the role of exercise in the management of their condition.
=== Physical Activity - Associated Risks and Considerations ===
As physiotherapists it is vital to be aware of risks and considerations when advising patients to participate in physical activity or exercise.&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; <br><br>Knowing whether patients have target joint bleeds allows the physiotherapist to adapt and alter their advice and treatment plan. Discussion can then be made around the use of protective equipment for the target joint, or whether there are any other activities which may reduce the risk of contact involving that specific joint. <br><br>It is important to know when the patient’s last acute bleed was and where. Activity must be gradually re-introduced following a bleed to minimise the chance of it reoccurring<ref>World Federation of Haemophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).</ref>.<br><br>Appropriate timing of prophylaxis treatment surrounding physical activity is critical when striving to prevent bleeds. Prophylaxis should be individualised and taken to coincide with exercise. The aim being the factor will be at its peak during the period of activity<ref>Anderson A, Forsyth A. Playing it safe: Bleeding Disorders, Sport and Exercise New York: National Haemophilia Foundation. 1- 44. https://www.hemophilia.org/…/docume…/files/PlayingItSafe.pdf (accessed 6 Oct 2015).</ref>. This will minimise the risk or potential for bleeds<ref>World Federation of Haemophilia. About bleeding disorders. http://www.wfh.org/en/page.aspx?pid=642 (accessed 7 Jan 2016).</ref>. As discussed previously, physiotherapists must be aware of this and communicate with the patient’s medical team regarding best advice.
==== Exercising with Inhibitors  ====


<br>  
Approximately 30% of patients with haemophilia will develop an immune response to medications used to treat the condition. If this occurs an alternative treatment may be necessary to manage bleeds. Immune tolerance therapy is introduced which aims to help the individual become accustomed to the factor<ref>The Haemophilia Society. Inhibitors. http://www.haemophilia.org.uk/bleeding_disorders/bleeding_disorders/inhibitors (accessed 26 Jan 2016).</ref>.


<br>  
Individuals with inhibitors may initially be apprehensive towards engaging in activity and exercise, causing their physical condition to rapidly decline. A program consisting of active range of motion, isometric and isotonic strengthening along with balance exercises can facilitate function and help to maintain independence<ref>Caviglia H, Narayan P, Forsyth A, Giangrande P, Gilbert M, Goddard N, Mulder K, Rahima S. Musculoskeletal problems in persons with inhibitors: How do we treat?. Haemophilia 2012;18(4):54-60. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2012.02874.x/epdf (accessed 26 Jan 2016).</ref>. 


Throughout this learning resource, various tasks, activites and additional reading have been provided to engage the learner. Examples of how these are laid out are detailed below.
=== Guidelines and Recommendations  ===
 
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These have been developed considering different learning styles (VARK 2016). Learning styles are common ways in which people learn. These can in visual, auditory, reading and writing or kenisthetic learners. If you are unsure of your learning style, please refer to the following link provided&nbsp;[http://vark-learn.com/the-vark-questionnaire/ VARK Learning Questionnaire].&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;&nbsp;
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== '''Background'''  ==
It is important to note that the recommended guidelines for physical activity in children with haemophilia are the same as those advised for healthy individuals. Government-issued guidelines exist for children and young people aged 4 – 18. These guidelines state that this age group should be participating in 60 minutes of moderate to vigorous activity each day. Vigorous activity, which strengthens muscle and bone, should take place at least 3 times a week<ref>Department of Health. Physical Activity Guidelines for Children and Young People. https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/213739/dh_128144.pdf (accessed 10 Oct 2016).</ref>.<br>The World Haemophilia Federation<ref>World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).</ref>&nbsp;has released updated guidelines for the management of people with haemophilia:


This chapter will offer a brief understanding of haemophilia including pathophysiology, incidence and prevalence, classification and severities and medical management. The role of the MDT in treating an adolescent with this condition will also be briefly discussed.&nbsp;
*The primary aim of management is to prevent and treat bleeds


The learning outcomes to be addressed in this section are: <br>1. '''Summarise the characteristics and underlying pathophysiology of haemophilia including explanation of different types.'''
*Comprehensive care aims to promote health physically and psychosocially, as well as improving quality of life and reducing morbidity and mortality. Physiotherapists may play a more significant role in this aspect compared to managing the more acute phase of a bleed. Physiotherapy involvement contributes to both the prevention and treatment of bleeds.


=== <br>Definitions, Incidence and Prevelance  ===
*Children and adolescents should be reviewed every 6 months by a physiotherapist for assessment and management planning


<br>Haemophilia is a rare inherited blood disorder where blood in the body has an inability to produce sufficient clotting factor, a protein in the blood which controls bleeding (NHS Choices 2015). This lack of clotting factor can lead to severe and prolonged bleeds within the body which may cause permanent damage to site of haemorrhage and surrounding tissues (World Federation of Haemophilia 2015). The condition mainly affects males and is carried genetically through a gene which is passed to child from parent through the X chromosome (Great Ormond Street Hospital 2015)&nbsp;<br><br>There are four main types of Haemophilia. The table below shows the types of haemophilia and more about each type:<br>
*Physiotherapy has a main role in promoting physical activity: to improve fitness, neuromuscular development, strengthening, coordination, functioning, weight management and self esteem


[[Image:Types table.jpg|center|500px]]<br> Haemophilia A is the most common type of Haemophilia. This is where someone either has low levels of clotting factor eight or it is completely missing. This type of Haemophilia affects around 80% of the Haemophilia population (The Haemophilia Society 2015). <br> <br>Haemophilia B is less common. This is where clotting factor nine is either very low or is missing from the blood. Around 20% off people with haemophilia have Haemophilia B (The Haemophilia Society 2015).<br><br>Haemophilia C is ten times rarer than type A. They will have low levels of clotting factor eleven or this will be completed absent. Haemophilia C deficiency is different to A and B as it can present in both males and females. This is extremely uncommon and can only happen when the mother and father are both carriers of the gene (Haemophilia Information 2013). Haemophilia C does not cause joint bleeds but can lead to nose bleeds, bleeding in the mouth, heavy bleeding during periods for girls and prolonged external bleeding following injury (IHTC 2012).<br> <br>Acquired Haemophilia is extremely rare. This type of Haemophilia is not inherited in the same way as it is an auto immune disorder. This is where the immune system attacks the clotting factors. This condition can also affect males and females. (Grethlein 2014).<br>
*Weight bearing activities should be encouraged to enhance bone mineral accrural


<br>The figure below shows how haemophilia is passed on genetically:
*A physical activity plan should be tailored to individuals preference, abilities and considering the severity of their condition


[[Image:Genetics.jpg|center|500px]]<br> <br>
*Non-contact sports will be encouraged – i.e. swimming, walking, golf, badminton, cycling, table tennis


As shown above, when the female is the carrier of haemophilia and the father is unaffected, the son will possess a 50% chance of inheriting the condition. There is a 50% chance the daughter will be a carrier of the gene but will not inherit the condition (HFA 2015).<br><br>When the male suffers from haemophilia and the mother is unaffected, none of the sons will inherit the condition, all daughters will carry the gene but will not be affected (HFA 2015).<br>
*Contact sports and high velocity sports are strongly discouraged due to the risk of a fatal bleed – i.e. rugby, boxing, wrestling, racing, skiing


If the father has haemophilia and the mother is a carrier, there will be a 25% chance that the daughter and son may have haemophilia. This is the only time where the girl can inherit the condition however this is extremely rare.
*Target joints can be protected using braces or splints during activities


<br>
*Following a bleed the activity should be reintroduced gradually to minimise the risk of re-bleeding


=== Classifications and Severities  ===
*Adjunctive management is also vital to physiotherapy. This means activity should be undertaken in conjunction with factor replacement. Physiotherapy can also help to reduce the amount of factor required.&nbsp;


<br>The table below catagorises the severities of haemophilia into mild, moderate and severe, showing the percentage of sufferers in each category (Preston et al. 2004).<br>
*In the acute phase, PRICE will be used by the physiotherapist


<br>
*Following this, rehabilitation is required to restore function


[[Image:Severityhaem.jpg|center|600px]]
<br> "Playing it Safe"<ref>National Haemophilia Foundation. Play it Safe. https://www.hemophilia.org/sites/default/files/document/files/PlayingItSafe.pdf (accessed 20 Oct 2015).</ref> is an online document which provides recommendations and advice regarding participation in sport and exercise when suffering with a bleeding disorder.  
 
<br><br>Like other disorders, haemophilia can affect people to varying degrees. The symptoms can be mild, moderate or severe, depending on the level of clotting factor present (NHS Choices 2015).<br><br>For mild haemophilia the individual will have between 4% - 49% of the factor level in their blood. Around 43-70% of the haemophilia population are considered mildly affected (Haemophilia Information 2013). Symptoms for mild haemophilia sufferers can be non-existent, although may include prolonged bleeding following serious injury, trauma or surgery. In many cases, haemophilia will not be discovered until there is excessive bleeding following injury or if they are undergoing surgery. In some cases it may not even be discovered until adulthood.<br><br>Moderate haemophilia sufferers have between 1% and 5% of factor level in their blood. Between 15-26% of AWH are considered moderate (Haemophilia Information 2013). Moderate sufferers are likely to suffer prolonged bleeding following injury and may have occasional spontaneous bleeding episodes.<br><br>Severe Haemophilia will affect an individual to a greater extent. Those with severe haemophilia will have a factor level between 0% and 1%. This accounts for the other 15-31% of the haemophilia population (National Haemophilia Foundation 2016).They will experience excessive bleeding after slight injuries and surgery and are also highly likely to have spontaneous bleeding episodes into the joints and surrounding muscles. They may also experience joint bleeding and prolonged bleeding into the muscles (Haemophilia Information 2013). This internal bleeding is particularly dangerous as suffers may be unaware it is occurring. If untreated it may lead to permanent joint deterioration and arthritis over time. <br>
 
=== <br>Medical Management<br><br>  ===
 
The medical management of haemophilia typically involves two treatment methods, ‘on demand’ or prophylactic. Haemophilia Care (2014) explains how treatment involves replacing the deficient clotting factor (VIII for haemophilia A and IX for haemophilia B) through intravenous injection.<br><br>On demand treatment is administered immediately following a bleed and is most commonly used in mild or moderate cases. It provides the advantage of not having to infuse regularly, however if there is a delay in ‘on demand’ treatment following a bleed then there is potential for damage to occur.<br><br>Prophylactic is administered regularly as a method of preventative management and is most commonly used in those who suffer with severe haemophilia. The ultimate goal of prophylaxis treatment is to reduce the deficiency of factor VII or factor IX (Berntorp and Shapario 2012) therefore reducing the chance of spontaneous bleeds. It also aims to reduce the severity of the condition (Nilsson et al. 1976). Khoriaty et al. (2005) explains how clotting factors are infused by the patient themselves on a constant basis around three times a week in the case of severe haemophilia A (factor VIII), and twice a week in the case of severe haemophilia B (factor IX). The World Health Organisation recognises prophylaxis treatment as the optimal treatment for patients with severe haemophilia.<br><br><br>
 
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| For more information on the medical management of haemophilia please visit the following link [http://www1.wfh.org/publication/files/pdf-1472.pdf WFH Guidelines for the management of haemophilia]&nbsp;Pages 12-14. &nbsp;Take 10 minutes to do this.
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<br>
 
=== MDT Involvement<br><br>  ===
 
&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; Before continuing further into this section complete the task below.<br>
 
[[Image:Mdt1.jpg|center|300px]]&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;
 
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Despite recent development in management of the condition, PWH still require comprehensive multidisciplinary care through all stages of their life (Breakey et al. 2010). A multidisciplinary team involves a range of professionals who collaborate together to structure a treatment plan for an individual, aiming to achieve high quality patient-centered care (Department of Health 2015).<br>
 
The collaboration of all professionals allows a holistic approach to be considered that takes into account the individual’s physical, social, emotional, cultural and spiritual needs (CSP 2015). This is especially important during transition from childhood into adulthood which can cause additional stress for those with chronic disorders. Therefore during this time the need for the MDT to work together is essential (Breakey et al. 2010). This may mean referring the patient to another team member to allow high quality treatment, specific to the patients needs.&nbsp;<br>
 
The following mind map provides examples of professionals that may be involved in the treatment of an AWH.<br>
 
<br><br>[[Image:MDT mind map.png|center|500px]]<br><br>
 
=== Quiz  ===
 
Follow this link to a quiz on the [https://www.surveymonkey.co.uk/r/MGP7TKJ background of haemophilia ]as seen above. &nbsp;This should take 5 minutes then check your answers below.<br>
 
[[Image:Qanswers.jpg|center|400px]]
 
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{| width="600" border="1" cellpadding="1" cellspacing="1" align="center"
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| [[Image:Keypointstab.jpg|600px]]
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| • Rare, inherited condition caused by a lack or absence of clotting factors within the blood<br>• 4 types of Haemophilia - A is most common<br>• Can acquire haemophilia – autoimmune system attacks clotting factor<br>• Mostly affects boys<br>• Varying severities<br>• Medical management can be ‘on demand’ and prophylactic<br>• Many members of a team involved in treatment of an AWH
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<br> <br>
 
== The Role of The Physiotherapist  ==
 
In this section of the wiki we will examine the role of the physiotherapist in regards to assessment, management, education, physical activity promotion, considerations, personal protective equipment and how to approach a conversation regarding physical activity. &nbsp;
 
The learning outcomes to be addressed in this section are: <br>'''2. Critically reflect on challenges and considerations associated with treating an adolescent with haemophilia.<br>'''
 
=== Assessment<br>  ===
 
One way for physiotherapists to assess aspects of an adolescent with haemophilia is by using outcome measures. Outcome measures are a published, objective measure which allows you to evaluate change in a patients symptoms (REF).<br><br>Symptoms of a joint bleed may include swollen and hot joints, pain in the area and stiffness when mobilising. Many patients have described the overall feeling as an 'aura' surrounding the joint. (REF) &nbsp;The image below shows severe swelling in the left knee during a joint bleed:<br><br>
 
[[Image:Joint swelling.jpg|center|250px]]<br><br>The Haemophilia Joint Health Score (International Prophylaxis Study Group 2015) is designed for physiotherapists and it is recommended for use by professionals specialised in haemophilia treatment. The outcome measure takes approximately 45 – 60 minutes to complete in total. It is designed specifically for children with haemophilia. It examines the condition of a joint affected by a bleed and can be used to show the degeneration of a joint overtime.<br>
 
<br> [[Image:Hjhs.jpg|left|600px]]<br> <br> <br>
 
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The Paediatric Haemophilia Activities List’ is another outcome measure used for individuals with haemophilia. Adolescents will complete the child/teenager version with regards to how easy or difficult they find some activities of daily living and how much it is affected by their haemophilia. The questionnaire can then be used by healthcare professionals involved in their care (World Federation of Haemophilia 2011).<br>
 
<br>
 
[[Image:Paediatric Haemophilia Activities List picture.jpg|600px]]<br> <br>
 
There are limited outcome measures specific to AWH (REF). Physiotherapists play a key role in assessing the joints affected by haemophilia, however it is also helpful for physiotherapists to be aware of other measures that exist with regards to activities of daily living in order to rehabilitate a patient appropriately.
 
Before continuing on to physiotherapy management take some time to complete the case study below based on what you have read previously and using your own knowledge:<br><br> <br>
 
[[Image:Management case study.jpg|center|500px]]<br><br><br>After completing your own plan see the table below of our plan of assessment.
 
=== Management  ===
 
The main role of the physiotherapist in treating adolescents with haemophilia involves management of the main impairments from a musculoskeletal perspective, aiming to restore and improve function. Some of these include: haemarthoses, synovitis, joint contractures, haemotomas and haemophilic arthropathys (Buzzard and Beeton 2000).
 
A common presentation as a result of injury during sports or equally due to adverse affects associated with inactivity can be joint bleeds. Therefore it is important we are able to manage these appropriately.
 
During the acute phase, the physiotherapist will attempt to minimise bleeding using the PRICE regime (protection, rest, ice, compression elevation) (Haemophilia Care 2014). This will also work in conjunction with factor replacement if necessary.
 
Following the acute phase, the physiotherapy management will focus on regaining full range of movement, muscle strength and reducing pain (REF). As soon as the pain and swelling begin to decrease, the patient should be encouraged to gradually increase the joint range aiming to achieve complete extension. This should be done actively by the patient to encourage muscle contraction, however passive movements may initially be used. It is vital that early active muscle control occurs to prevent the loss of joint movement (Hermans et al. 2011; Gomis et al. 2009; Mulder 2006). Rehabilitation involving active exercises and proprioceptive training must then be continued until full pre-bleed joint ROM and function is restored (Heijnen and Buzzard 2005).
 
As previously discussed, another key role of the physiotherapist when treating an adolescent with haemophilia is promotion of physical activity. Exercise is encouraged as it will improve the child’s fitness, reduce obesity levels, improve muscular strength, and reduce frequency of bleeding episodes and joint deterioration (Blamey 2010). The World Federation of Haemophilia (2012) advise particular focus towards strengthening, co-ordination, achieving a healthy body weight and improving self-esteem.
 
As well as the physical aspects concerned, a major aspect of physiotherapy management focuses on education and advice. This is discussed in detail within the following section.<br>
 
==== Education<br>  ====
 
Below is a mind map which summarises key points regarding the advice that should be given to patients regarding physical activity.&nbsp;<br> <br><br>
 
[[Image:Education picture.jpg]]
 
<br>
 
The physiotherapist has a crucial role in educating patients regarding physical activity, exercise prescription and selecting particular activities (Heijnen 1993).
 
First of all, patients/relatives and peers must be made aware that physical activity can actually help to prevent joint damage and functional impairment (Buzzard 1997). Also, individuals who exercise frequently actually exhibit fewer bleeds (Tinktinsky et al 2002).
 
You should inform patients that just as with healthy individuals, exercise also improves psychosocial factors (Negrier et al. 2008). It is also important to address the added benefits of physical activity during puberty in adolescents. Regular physical activity participation during puberty, enhances lean tissue mass, fitness and strength and decreases fat mass (Ara et al. 2004).
 
Depending on the patients severity of their condition, as the physiotherapist make the patient aware that high contact and collision sports such as football, hockey, rugby, boxing, and wrestling are usually best avoided. You can then try to encourage non-contact sports such as swimming, walking, cycling, golf, archery, badminton, rowing and sailing (The World Federation of Haemophilia 2015).
 
You need to work in conjunction with medical teams concerning advice of timing of prophylaxis administration that is appropriate to their chosen sport or activity (McGee et al 2015; The World Federation of Hemophilia 2015). It may also be necessary where appropriate to educate and encourage the use of protective equipment e.g if patient has a target joint, or no clotting factor given prior to activity (Philpott et al. 2010). Protective equipment is discussed in further detail later in the module.
 
As the physiotherapist you could also be involved in educating school personnel regarding suitable activities for the child, immediate care in case of a bleed, and modifications in activities that may be needed after bleeds (The World Federation of Hemophilia 2015).<br><br>
 
=== Physical Activity Promotion  ===
 
It is already known that physical activity in this population is reduced compared to healthy age-matched peers. The kings fund (xxxx) acknowledge health behaviour patterns to impact an individual's overall health by as much as 40%, when compared to healthcare which contributes only 10%. Therefore as Physiotherapists our role in health and activity promotion is crucial in order to influence positive behavioural change to impact upon health (CSP 2016).&nbsp;
 
Health promotion involves supporting people to gain control and responsibility over improving their own health. It extends beyond focusing on individual behaviour and includes a variety of social and environmental interventions (WHO 2016).
 
<br>The Corporate Policy and Strategy Committee have introduced a Physical Activity for Health Pledge which the NHS is engaging with. This pledge involves ensuring primary care staff have the necessary skills and resources available to assess physical activity levels. It also ensures they offer education detailing recommended minimum requirements for physical activity, brief advice and intervention, along with increasing awareness of available community resources. Unpublished data from NHS Health Scotland reveals only 13% of primary care practitioners are presently aware of the recommended weekly activity levels (Corporate Policy and Strategy Committee 2015). It can therefore be assumed this is not currently being promoted effectively.
 
The following video [https://www.youtube.com/watch?v=aUaInS6HIGo "23 and a 1/2 hours"] strongly promotes physical activity for 30 minutes of your day. Although not specific to the haemophilic population, however the take home message can be followed for AWH as the physical activity benefits are not changed or exhaustive.&nbsp;
 
Although various studies have described the benefits of engaging in physical activity for people with haemophilia (PWH), results from research conducted in the USA among adolescent haemophilia patients have demonstrated a lack of knowledge concerning the role of physical activity in managing their condition (Nazzaro et al. 2006). Whilst it is recognised this information is important, this study has not been conducted in the UK This suggests that more work must be done to promote physical activity and emphasise its positive role in enhancing the lives of PWH (Negrier et al. 2013).
 
Physical activity promotion for health exists worldwide (Heath et al. 2012). The benefits of such in the general population are widely known, providing health improvements along with the potential for enhancing disease outcomes (WHO 2015). National and international guideliness set out clear recommendations on the physical activity levels required to promote health (Department of Health 2011). Although many studies have reported the benefits of participation in physical activity for PWH, researchers in the USA have identified a lack of knowledge among<br>young haemophilia patients (aged 13–18 years) as to the role of exercise in the management of their condition.
 
The promotion of exercise in AWH is of particular importance due to recent advances in care over the past 40 years (Manco-Johnson et al. 2003). This has seen changes in exercise prescription; as is it now believed to be a crucial modality and understood it will not attribute to bleeds, as previously believed (Riske 2007).&nbsp;
 
<br><br><br>
 
=== Considerations<br>  ===
 
As the physiotherapists it is vital to be aware of risks and considerations when advising patients to participate in physical activity.<br> <br>When discussing forms of exercise with the patient, the following information is essential to know from your patient;<br><br><br>
 
[[Image:Sbfc.jpg|center|600px]]<br><br>Knowing whether patients have target joint bleeds allows you as the physiotherapist to adapt and alter your advice and treatment plan. This will allow you to discuss the use of protective equipment for the target joint, or if there are any other activities that may reduce the risk of contact involving that specific joint. <br><br>As discussed previously, it is important to know when the patient’s last acute bleed was and where. Activity must be gradually re-introduced after a bleed to minimise the chance of it reoccurring (The World Federation of Haemophilia 2015).<br><br>It is important to discuss with the patient what exercises they enjoy. This way it is much more likely you will be able to actively promote activity. <br><br>Appropriate timing of prophylaxis treatment surrounding physical activity is critical when striving to prevent bleeds. Prophylaxis should be individualised and taken to coincide with exercise. The aim being the factor will be at its peak during the period of activity (Anderson and Forsyth 2005). This will minimise the risk or potential for bleeds (WFH 2012). As discussed previously, you must be aware of this and communicate with the patient’s medical team regarding best advice.<br>
 
<br>
 
=== Guidelines and Recommendations  ===
 
It is important to note that the recommended guidelines for physical activity in children with haemophilia are the same as those advised for healthy individuals. Government issued guidelines exist for children and young people aged 4 – 18. These guidelines state that this age group should be participating in 60 minutes of moderate to vigorous activity each day. Vigorous activity, which strengthens muscle and bone, should take place at least 3 times a week (Physical Activity Guidelines for children and Young People 2011).
 
<br>The World Haemophilia Federation (2012) has released updated guidelines for the management of people with haemophilia:
 
*The primary aim of management is to prevent and treat bleeds
*Comprehensive Care aims to promote health of the individuals physically and psychosocially, as well as promoting quality of life and reducing morbidity and mortality. Physiotherapists may play a more significant role in this aspect compared to managing the more acute phase of a bleed. Physiotherapy involvement contributes to both the prevention and treatment of bleeds.
*Children and adolescents should be seen every 6 months by a physiotherapist for assessment and management planning
*Physiotherapy has a main role in promoting physical activity: to promote fitness, neuromuscular development, strengthening, coordination, functioning, weight management and self esteem
*Weight bearing activities should be encouraged to promote good bone density
*The development of a physical activity plan should be tailored to individuals preference, abilities and condition
*Non-contact sports will be encouraged – i.e. swimming, walking, golf, badminton, cycling, table tennis
*Contact sports and high velocity sports are strongly discouraged due to the risk of a fatal bleed – i.e. rugby, boxing, wrestling, racing, skiing
*Target joints can be protected using braces or splints during activities
*After a bleed the activity should be reintroduced gradually to minimise the risk of re-bleeding
*Adjunctive management is also vital to physiotherapy management. It can be used in conjunction with factor replacement. Physiotherapy can also help reduce the amount of factor necessary. This can be useful as factor replacement is expensive.
*In the acute phase, PRICE will be used by the physiotherapist
*Following this, rehabilitation is required to restore function<br>
 
 
 
Playing it Safe (Haemophilia, 2005) is a document that exists online which provides recommendations and advice about taking part in sports and exercise if you have a bleeding disorder such as haemophilia.<br>This document gives recommendations on the safest activities to participate in and gives advice on the sports and exercises with the highest risk of injury.<br>For adolescents, when considering which activities to take part in, it is important to consider current body condition, history of bleeding and current joint condition.<br>It is also said that the more physically active you were as a child the easier it will be to remain active in teens and into adulthood. <br>As children become teenagers some sports such as basketball become more contact – therefore although these sports may have been possible when they child was younger it may have more risks in adolescents.<br>
 
[[Image:Pis_qs.jpg|center|500px]]
 
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| [https://www.hemophilia.org/sites/default/files/document/files/PlayingItSafe.pdf Playing it Safe]
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=== Personal Protective Equipment  ===
=== Personal Protective Equipment  ===


<br>Protective equipment can be worn by individuals to minimise risk of bleeding either when at a very young age or when taking part in sports and activities at an older age (WHF 2007). Joints which are considered a high risk of bleeds are the elbows, wrists, knees and ankles. Toddlers are encouraged to be protected with elbow and knee pads when crawling to reduce the risk of bleeding and helmets are also beneficial to protect children from falls, especially when cycling or running. Due to the introduction and advances in prophylaxis, the need for protective equipment in day to day activities is less. It has been suggested that although previously extremely important and beneficial, nowadays the social risks outweigh the potential benefits of protective equipment when partaking in normal activities. However, this is a decision that has to be made based on the type and competitiveness of the activity the individual is participating in.  
<br>Protective equipment can be worn by individuals to minimise the risk of bleeding either at a very young age or when taking part in sports and activities at an older age<ref>Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 10 Jan 2016).</ref>. This is generally used considering personal preference, the child’s compliance and the level of sporting activity undertaken. Splints or braces can be useful in protecting target joints whilst participating in sports<ref>World Federation of Haemophilia. About bleeding disorders. http://www.wfh.org/en/page.aspx?pid=642 (accessed 7 Jan 2016).</ref>.  
 
Protective equipment can be worn whilst partaking in sports. This is generally used considering personal preference, the child’s compliance and level of sporting activity undertaken. Splints or braces can be useful in protecting target joints (those which are repeatedly struck with bleeds) whilst participating in sports (WFH 2012).
 
There are many different types of protective equipment available and there are also multiple ways of modifying home environments to make it safer for toddlers and young children.<br>An example of the various different types of protective equipment are shown in the table below:
 
{| width="700" border="1" cellpadding="1" cellspacing="1"
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| This headgear has been proven to prevent traumatic brain injuries. It adapts to all fits, sizes and head shapes and does not slide. It is extremely sturdy and does not need replaced after a fall or impact. This helmet is highly recommended by neurosurgeons for patients with haemophilia among other conditions.
|-
| [[Image:Cap 2.jpg|300px]]<br>  
| This example was designed by a mother of a child with haemophilia. It is much lighter and more comfortable to use. Its thick cotton design provides protection and it helps eliminate any social risks as it resembles a standard cotton hat.
|-
| [[Image:Cap 3.jpg|300px]]
| This is another protective helmet which provides high protection and safety from skull injuries sustained from a bad fall. It is made of rubber and has a chin strap and adjustable hooks. This helmet provides protection at the sides and back of the head, and is commonly used in sports at all ages.
|}


<br>
Joints which are considered at high risk of bleeds are the elbows, wrists, knees and ankles. Toddlers are encouraged to be protected with elbow and knee pads when crawling and helmets can also be used to protect children from falls, especially when cycling or running. Due to the introduction and advances in prophylaxis, the need for protective equipment in day to day activities has been reduced. It is suggested that although previously considered extremely important and beneficial, the social risks now outweigh the potential benefits of protective equipment. However, this is a decision that must be made based on the type and competitiveness of the activity the individual is participating in.


LACK OF EVIDENCE?<br><br>
There are many different types of protective equipment available and multiple ways of modifying home environments to make it safer for toddlers and young children.


=== How to Approach Conversations Regarding Physical Activity  ===
=== How to Approach Conversations Regarding Physical Activity  ===


It can sometimes be difficult for a physiotherapist to approach a conversation regarding how much physical activity a person undertakes. It can often be challenging to encourage change in a person’s behaviour and can be increasingly difficult in adolescents with haemophilia due to the nature of the condition. This a particularly difficult issue in adolescents with haemophilia as this is normally a stage in their life where physical activity levels reduce (REF). However physiotherapists can offer education and advice about what type of activity to undertake in order to prevent bleeds occurring. More recommendations about this have been discussed previously in the education and recommendations sections.
It can sometimes be difficult for physiotherapists to approach the subject of physical activity with an individual and encouraging behavioural change can be increasingly difficult in AWH. This is due to the nature of the condition as this is normally a stage in their life where physical activity levels reduce<ref>Majumdar S, Morris A, Gordon C, Kermode CJ, Forsythe A, Herrington B, Megason GC, Iyar R. Alarmingly high prevalence of obesity in haemophilia in the state of Mississippi. Haemophilia 2010;16;455-459. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2009.02187.x/epdf (accessed 28 Jan 2016).</ref><span style="line-height: 1.5em; font-size: 13.28px; font-weight: normal;">.</span> Physiotherapists can offer education and advice about what type of activity to undertake in order to prevent bleeds occurring.<br>Appropriate communication is essential when discussing physical activity with patients. The Royal College of General Practitioners<ref name="Royal College of General Practitioners">Royal College of General Practitioners. Health literacy: report from an RCGP-led health literacy workshop. http://www.rcgp.org.uk/clinical-and-research/a-to-z-clinical-resources/~/media/Files/CIRC/Health-Literacy-Report/Health%20Literacy%20Final%20edition%2029%2007%202014.ashx (accessed 20 Nov 2015).</ref>&nbsp;makes recommendations on how to appropriately communicate ideas to a patient.  
 
<br> <br>Appropriate communication is essential when discussing physical activity with patients. The Royal College of General Practitioners (2014) makes recommendations on how to communicate ideas to a patient:
 
<br>• Be aware of the amount of information you provide – careful not to overload them
 
<br>• Avoid the use of technical vocabulary as it may cause confusion
 
<br>• Use different methods other than verbal communication to provide information e.g. leaflets, video, online resources. <br> <br> <br> [[Image:5 A picture.jpg|right|400px]] <br> <br>
 
Brief interventions involve short advice and support sessions providing information for the patient regarding the process of behavioural change. One method of brief intervention is the 5 A Strategy (Sim et al. 2009). The picture on the right shows what this involves:<br>  
 
<br>
 
<br> <br>
 
<br> <br>Finally OARS is a form of motivating the patient through communication. The diagram below explains how to use OARS:<br> [[Image:OARS picture.jpg|center|400px]]
 
== Benefits of Physical Activity  ==
== Benefits of Physical Activity  ==


The learning outcomes to be addressed in this section are:<br> '''3. Using the biopsychosocial model, evaluate the physical, spiritual and emotional needs which must be considered when treating an adolescent with haemophilia. <br>4. Critically appraise the evidence base surrounding physical activity in an adolescent with haemophilia.'''<br>
The video below offers some general advice and recommendations regarding increasing physical activity in PWH. It follows the story of Sean, an individual with haemophilia, as he discusses some of the benefits he believes are associated with being physically active when living with this condition.&nbsp;{{#ev:youtube|7wSlRbwqiE8}}<br>Video used with permission from Clinical Specialist Physiotherapist, Jenna Reid.&nbsp;  
 
=== <br>NHS Lothian - Haemophilia: Get Fit &amp; Active<br>  ===
 
<br>{{#ev:youtube|7wSlRbwqiE8}}<br>  
 
=== <br>Physical Activity Relating to Obesity  ===
 
Recent progress in the management of haemophilia over the past few decades has seen improvements in average lifespan of AWH, now matching that of a healthy individual. That being said these changes have introduced other new issues to tackle in the coming years.
 
Childhood obesity is prevalent in the haemophilia population, as well as the general population. The consequences of such weight gain in sufferers can cause added issues, such as increased risk of joint arthropathy (Young 2012). Excess weight can induce joint bleeds due to added stresses placed on the joints (Biere-Rafi et al. 2011).
 
The benefits of physical activity in the general population are widely known, providing general health improvements along with potential enhancement in disease outcomes. However it is thought children suffering with a chronic disease or disability are less active than their age-matched healthy peers (Philpott et al. 2010). A study conducted by Hofstede et al. (2008) comparing healthy subjects to AWH reiterates; revealing triple the number of obese boys with haemophilia in relation to the comparable healthy subjects. As previously mentioned, exercise participation in this population offers further benefits such as improving muscular strength, balance, coordination and fitness levels (WFH 2012).


Another factor contributing to obesity in this population can be attributed to parental influence. Many parents tend to be very protective over their child, preventing them from engaging in physical activity. It was not until the 1970s that exercise in this population was recognised as effective. Prior to this it was considered to increase the risk of haemarthroses (Broderick et al. 2006). It is now recommended for particular activities, mainly swimming, with aims of improved quality of life. This new treatment approach is largely due to recent advances in safety and availability in the clotting factor used to prevent bleeds (Querol et al. 2010). Activities such as contact sports and martial arts are not recommended, however if the undertaken the individual must be carefully assessed prior to participation (Philpott et al. 2010). It is generally believed by professionals working with this condition that physiotherapy rehabilitation, coupled with regular exercise, is crucial in treating AWH, especially in those who do not receive replacement therapy (Gomis et al. 2008). Buzzard and Beeton (2008) also report evidence to suggest changes in blood clotting factors due to exercise. SPORTS TABLE?
===  Physical Activity Relating to Obesity  ===


Physical activity in AWH is extremely important, even more so of late due to increasing rates of childhood obesity. However, exercise prescription should be carefully considered, ensuring the activities they undertake are appropriate for their stage of development (Buzzard and Beeton 2008). This means balancing the benefits of physical exercise with the risks associated (Broderick et al. 2013).<br>AWH will experience reductions in muscular strength, aerobic and anaerobic resistance along with proprioception and physical condition. These factors along with increased vulnerability to stressful motor demands and greater risk of lesions, create a cycle of inactivity which becomes difficult to change. Pain brings about reductions in mobility attributing to muscle atrophy, joint instability and recurring bleeds (REF). These factors contribute to reduced activity levels in this population consequently resulting in higher obesity levels (REF).<br>  
Childhood obesity is prevalent in AWH, as well as the general population. However, the consequences of weight gain in sufferers can cause additional issues, such as increased risk of joint arthropathy<ref name="Young">Young G. New challenges in hemophilia: long-term outcomes and complications. Hematology 2012;1:362 - 368. http://asheducationbook.hematologylibrary.org/…/362.full.pd… (accessed 26 Oct 2015).</ref>. Excess weight can induce joint bleeds due to added stresses placed on the joints <ref name="Tuinenburg et al. 2013">Tuinenburg A, Biere-rafi S, Peters M, Verhamme P, Peerlinck K, Kruip MJHA, Laros-van Gorkom BAP, Roest M, Meijers JCM, Kamphuisen PW, Schutgens REG. Obesity in haemophilia patients: effect on bleeding frequency, clotting factor concentrate usage, and haemostatic and fibrinolytic parameters. Haemophilia 2013;(19):744-752. http://www.pfizerpro.com.co/sites/g/files/g10017511/f/publicaciones/092013%20V19%20I5%20-Obesity%20in%20haemophilia%20patients%20effect%20on%20bleeding%20frequency,%20clotting%20factor%20concentrate%20usage,%20and%20haemostatic%20and%20fibrinolytic%20parameters.pdf (accessed 2 Dec 2015).</ref>. Effects of obesity and overweight can also exacerbate existing arthropathies and influence development of cardiovascular disease<ref>Douma-van Riet DMC, Engelbert RHH, Van Genderen FR, Horst-De Ronde MTM, Goede-Bolders A, Hartman A. Physical fitness in children with haemophilia and the effect of overweight. Haemophilia 2009;15:519-527. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2008.01928.x/epdf (accessed 22 Jan 2016).</ref>.


=== Childhood Development  ===
Parental influence is also considered to be a contributing factor to obesity and overweight in this population. Many parents tend to be very protective of their child, preventing them from engaging in physical activity. It was not until the 1970s that exercise in this population was recognised as effective. Prior to this it was considered to increase the risk of haemarthroses<ref name="Broderick et al. 2006">Broderick CR, Herbert RD, Latimer J, Curtin JA, Selvadurai HC. The Effect Of An Exercise Intervention On Aerobic Fitness, Strength and Quality of life in children with Haemophilia. BMC Blood Disorders 2006;6:2. http://www.biomedcentral.com/content/pdf/1471-2326-6-2.pdf (accessed 9 Oct 2015).</ref>. A previously discussed, it is now recommended for particular activities, mainly swimming, with aims of improved quality of life. This new treatment approach is largely due to recent advances in safety and availability in the clotting factor used to prevent bleeds<ref name="Querol et al. 2010">Querol F, Perez-alendaa S, Gallachc JE, Devis-devis J, Valencia-peris A, Gonzalez Moreno LM. Haemophilia: exercise and sport. Apunts Med Esport 2010;46(165:29-39. http://www.elsevier.es/…/277/277v46n169a90001817pdf001_2.pdf (accessed 15 Oct 2015)</ref>. Buzzard and Beeton<ref>Buzzard B, Beeton K. Physiotherapy Management of Haemophilia. Oxford: Wiley, 2008.</ref> also report evidence to suggest changes in blood clotting factors due to exercise.


A study conducted in 2008, by Tlacuilo-Parra et al. revealed a significant reduction in lumbar spine bone mineral density in children with haemophilia in comparison to healthy subjects of matched age and sex. The results demonstrated statistically significant differences in activity between the groups, with the haemophilia population at three times the risk of inactivity. These results confirm that long periods of inactivity and immobility, with reduced weight-bearing (such as following joint bleeds) are directly related to low bone mineral density in AWH. Weight-bearing activity is crucial in ensuring adequate generation of bone mass throughout childhood and may be even be considered more important than calcium intake through diet (Barnes et al. 2004).<br>Adolescents undergo more physical changes than throughout any other stage of life after birth. Rate of growth in itself can predispose the child to bleeds. Growth spurts around puberty may decrease the muscle mass surrounding joints and consequently increase joint bleeding during this period (Johnson and Gorlin 2013).<br>  
The benefits of physical activity in the general population are widely known, providing general health improvements along with potential enhancement in disease outcomes<ref>Durstine JL, Gordon B, Wang Z, Luo X. Chronic disease and the link to physical activity. Journal of Sport and Health Science 2013;2(1)3-11. http://www.sciencedirect.com/science/article/pii/S2095254612000701 (accessed 13 Dec 2015).</ref>. However, Philpott et al.<ref name="Philpott et al. 2010" /> believe children suffering with a chronic disease or disability are less active than their age-matched healthy peers. A study conducted by Hofstede et al.<ref name="Hofstede et al. 2008">Hofstede FG, Fijnvandraat K, Plug I, Kamphuisen PW, Rosendaal FR, Peters M. Obesity: a new disaster for haemophiliac patients? A nationwide survey. Haemophilia 2008;14:1035-1038. http://onlinelibrary.wiley.com/…/j.1365-2516.2008.01806.x.p… (accessed 30 Sept 2015).</ref> comparing healthy subjects to AWH reiterates; revealing triple the number of obese boys with haemophilia in relation to the comparable healthy subjects. Contrary to this finding, a 2010 study quantifying physical activity levels through accelerometry, revealed AWH to be more involved in physical activity compared to their healthy peers. However, the study also stated the time dedicated to physical activity was mainly focused towards low-intensity activities and sedentary behaviours<ref name="Buxbaum et al. 2010">Buxbaum NP, Ponce P, Saidi P, Michaels LA. Psychosocial correlates of physical activity in adolescents with haemophilia. Haemophilia. 2010;16:656-61. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02217.x/epdf. (accessed 21 Jan 2016).</ref>. Several studies have reported an association between an increase in obesity levels and sedentary behaviours particularly amoung AWH. This increase is concerning, however does not differ much from the un-affected population<ref>Hofstede FG, Fijnvandraat K, Plug I, Kamphuisen PW, Rosendaal FR, Peters M. Obesity: a new disaster for haemophiliac patients? A nationwide survey. Haemophilia 2008;14:1035-1038. http://onlinelibrary.wiley.com/…/j.1365-2516.2008.01806.x.p… (accessed 30 Sept 2015).</ref>;<ref>Douma-van Riet DMC, Engelbert RHH, Van Genderen FR, Horst-De Ronde MTM, Goede-Bolders A, Hartman A. Physical fitness in children with haemophilia and the effect of overweight. Haemophilia 2009;15:519-527. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2008.01928.x/epdf (accessed 22 Jan 2016).</ref>.


=== Psychosocial factors associated with physical activity<br> ===
The overall consensus, as previous stated, is that an accumulation of 60 minutes or more physical activity of moderate intensity, consisting of both aerobic daily activity and vigorous intensity aerobic activity across a minimum of 3 days per week is advised <ref name="Licence 2004">Licence K. Promoting and protecting the health of children and young people. Child: care, health and development 2004;30(6):623-635. http://onlinelibrary.wiley.com/enhanced/doi/10.1111/j.1365-2214.2004.00473.x (accessed 22 Jan 2016).</ref>. However, the physical activity recommendations remain limited<ref>Beardsley DS. Sport and Exercise for children with chronic health conditions. Champaign IL:Human Kinetics 1995:302-9.</ref>;<ref name="Buzzard 1996">Buzzard BM. Sports and haemophilia: antagonist or protagonist. Clinical Orthopaedics and Related Research 1996;328:25-30. http://www.ncbi.nlm.nih.gov/pubmed/8653965 (accessed 19 Jan 2016).</ref>, with findings from recent studies demonstrating controversial outcomes.


Due to the nature of the condition, AWH are usually diagnosed and aware of their condition from a very young age. The childs parent or guardian will also be affected due to their involvement in the care and management of the individual in the early years. (The World Federation of Haemophilia xxxx) There are psychosocial, as well as physical barriers which can be managed using the ICF model. (REF)<br> <br>  
==== Childhood Development ====
Adolescents undergo more physical changes at this age than throughout any other stage of their life. The rate of growth in itself can predispose the child to bleeds. Growth spurts around puberty may decrease the muscle mass surrounding joints and consequently increase joint bleeding during this period<ref name="Johnson et al. 2013">Johnson MJ, Gorlin JB. Child Development with a Bleeding Disorder and Transition. New York: National Hemophilia Foundation 2013:1-16. https://www.hemophilia.org/…/Nurses-Guide-Chapter-13a-Child… (accessed 10 Oct 2015).</ref>.


&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;&nbsp;[[Image:ICF.jpg]]&nbsp;<br><br><br>
Weight-bearing activities are crucial in ensuring adequate generation of bone mass throughout childhood and may be even be considered more important than calcium intake through diet<ref name="Barnes et al. 2004">Barnes C, Wong P, Egan B, Speller T, Cameron F, Jones G, Ekert H, Monagle P. Reduced Bone Density Among Children With Severe Hemophilia. Pediatrics 2004;114:2:e177-e181. http://www.ncbi.nlm.nih.gov/pubmed/15286254 (accessed 9 Oct 2015).</ref>.&nbsp;A study conducted in 2008, by Tlacuilo-Parra et al.<ref name="Tlacuilo-parra et al. 2008">Tlacuilo-parra A, Morales-zambrano R, Tostadorabago N, Esparza-Flores MA, Lopez-Guido B, Orozcalcala J. Inactivity is a risk factor for low bone mineral density among haemophilic children. British Journal of Haematology 2008;140:562-567 http://onlinelibrary.wiley.com/…/j.1365-2141.2007.06972.x.p… (accessed 8 Nov 2015).</ref> revealed a significant reduction in lumbar spine bone mineral density in children with haemophilia in comparison to healthy subjects of matched age and sex. The results demonstrated statistically significant differences in activity between the groups, with the haemophilia population at three times the risk of inactivity. These results confirm that long periods of inactivity and immobility, with reduced weight-bearing (such as following joint bleeds) are directly related to low bone mineral density in AWH.&nbsp;  


&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; (Von Mackensen, 2007). <br><br><br>Recent evidence discussed throughout this wiki resource supports exercise, allowing AWH to participate in peer activities which they would have previously been excluded from.<br><br>The World Federation of Haemophilia (WHF) separates a number of different psychosocial issues into specific age groups:<br>• Infant to toddler (newborn to age 5)<br>• Early childhood (6-9 years)<br>• Pre-adolescence (10-13 years)<br>• Adolescence (14-17 years)<br>• Adulthood (18 years +)<br><br>It is important that at every stage of the child’s journey there is help and support available to the individual and family to ensure that psychosocial barriers are minimised (Cassis, 2007). Some of the psychosocial issues that are common in adolescence are discussed below.<br>
=== Psychosocial Factors Associated With Physical Activity  ===


Transition phase<br>In the adolescence stage, where there is a transition of care from paediatrics to general practitioners. At this stage it is important to focus on self -management and independence in treatment. From pre-adolescence to late teenage years, individuals will become more independent and involved in managing their condition and self- treating. Although this leads to increased confidence in their own bodies as they are able to confidently treat themselves, a common barrier with this at this age is compliance.  
Due to the nature of the condition, AWH are usually diagnosed and aware of their condition from a very young age. Psychosocial, as well as physical barriers may exist in relation to participation in physical activity<ref name="ICF 2016">ICF. International Classification of Functioning, Disability and Health (ICF). http://www.who.int/classifications/icf/en/ (accessed 5 Jan 2016).</ref><span style="line-height: 1.5em; font-size: 13.28px;">.</span> 


<br>Compliance<br>Cassis (2007) states that at this stage developmentally, teens are focussed on present events and do not consider the future consequences that poor management of their condition could cause. Also as a child starts to mature, other activities may take more of a priority over physiotherapy or exercises and it is important that they are made aware of the harmful consequences that this could lead to.<br>  
&nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp; &nbsp;<br><br>The World Federation of Haemophilia (WFH) separates a number of different psychosocial issues into specific age groups<ref>Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 10 Jan 2016).</ref>:<br>
* Infant to toddler (newborn to age 5)
* Early childhood (6-9 years)
* Pre-adolescence (10-13 years)
* Adolescence (14-17 years)
* Adulthood (18 years +)<br>
It is important that at every stage of the child’s journey there is help and support available to the individual and family to ensure that psychosocial barriers are minimised<ref>Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 10 Jan 2016).</ref>. Some of the psychosocial issues that are common in adolescence are discussed below.<br>


<br>Physical activity can play a large part in a child’s social and emotional well-being. Through team-based sports they learn how to work with other peers. Improved emotional status has also demonstrated marked clinical benefits with reported reductions in spontaneous bleeds (Anderson and Forsyth 2005).&nbsp;
==== Transition Phase ====
In the adolescence stage, there is a transition of care from paediatrics to general practitioners. During this period it is important to focus on self -management and increasing independence in treatment. Although this can increase confidence in self-treatment with greater control over their condition, a common issue at this age is compliance.


Cassis et al 2012 conducted a systematic review which looked at 24 studies relating to the psychosocial aspects of haemophilia. It found that psychosocial factors have a significant impact on quality of life for patients with chronic conditions such as Haemophilia. One of the studies, found that in haemophilia one of the most common stressors associated with psychological factors is limitations to an active lifestyle including sports and other physical activities. This may be due to the fear of causing a bleed or stigma associated with the protective equipment that is sometimes required to safely participate in sports. (REF).  
==== Compliance ====
Cassis<ref>Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 12 Jan 2016).</ref> states that at this stage of development, teenagers are often focussed on present events and do not consider the long term consequences of poorly managing their condition. As a child begins to mature, other activities may take priority over physiotherapy or exercise therefore, it is important that they are aware of the harmful consequences associated with inactivity.


<br>The literature review also found that for PWH, participation in physical activites and sports is associated with positive effects not only for physical well-being but also on self-esteem and social interactions. This is supported further in a study by Sherlock 2010 which found that the non-physical benefits of sporting activities included improved self-esteem and perceived social acceptance. It also supported the above view that quality of life in PWH is impaired by limitations in sporting activity.  
Physical activity can play a large part in a child’s social and emotional well-being. Through team-based sports they learn how to work with other peers. Improved emotional status has also demonstrated marked clinical benefits with reported reductions in spontaneous bleeds<ref>Anderson A, Forsyth A. Playing it safe: Bleeding Disorders, Sport and Exercise New York: National Haemophilia Foundation. 1- 44. https://www.hemophilia.org/…/docume…/files/PlayingItSafe.pdf (accessed 6 Oct 2015).</ref>.&nbsp;


<br>However, Buxaum et al 2010 conducted a study which aimed to ascertain social and cognitive factors associated with exercise in PWH aged between 11 and 18. The Peirs Harris Childrens Concept Scale and the Childrens Manifest Anxiety Scale are self-reported scales that were used to measure self-esteem and anxiety in PWH. This study found no differences in the scores obtained in these outcomes between PWH and healthy controls. Although this conflicts with previous evidence only 17 participants in the Haemophilia group were recruited and therefore results must be taken with caution due to small sample size.  
Cassis et al.<ref>Cassis FR, Querol F, Forsyth A, Iorio A. Psychosocial aspects of haemophilia: a systematic review of methodologies and findings. Haemophilia 2012; 18:3:101-14. http://www.ncbi.nlm.nih.gov/pubmed/22059839. (accessed 22 Jan 2016).</ref> conducted a systematic review comparing 24 studies considering the psychosocial aspects concerned with haemophilia. Results demonstrated psychosocial factors to have a significant impact on quality of life for patients with chronic conditions such as haemophilia. One of the studies, found the most common stressor associated with psychological factors to be reductions in lifestyle activity, including sport. This may be due to fear of inducing a bleed through exercise, or the stigma associated with wearing protective equipment.


<br>Although results are supported well by similar studies, it is evident that research into the psychosocial aspects of haemophilia is limited (cassis 2012) and actual participation in physical activity among PWH has not been well studied (Buxaum 2010)<br>  
The review also found that for PWH, participation in physical activites and sports will positively effect not only physical well-being but also self-esteem and social interactions. This is further supported by Sherlock<ref>Sherlock E, O’Donnell JS, White B, Blake C.  Physical activity levels and participation in sports in Irish people with haemophilia. Haemophilia 2010;16:202-9.  http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2009.02111.x/epdf.  (accessed 21 Jan 2016).</ref> who found that non-physical benefits of sporting activities included improved self-esteem and perceived social acceptance. It also supported the above view that quality of life in PWH is impaired by limitations in sporting activity. 


<br>  
However, Buxaum et al.<ref>Buxbaum NP, Ponce P, Saidi P, Michaels LA.  Psychocial correlates of physical activity in adolescents with haemophilia. Haemophilia 2010;16:656-61. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02217.x/epdf. (accessed 21 Jan 2016).</ref> conducted a study which aimed to establish social and cognitive factors associated with exercise in PWH. All particpants were aged between 11 and 18. The 'Peirs Harris Childrens Concept Scale' and the 'Childrens Manifest Anxiety Scale' were used to measure self-esteem and anxiety, as these are self-reported scales results must be interpreted with caution. The study found no differences in outcomes when comparing PWH to healthy controls. This conflicts with previous evidence, however, due to its small sample size with only 17 recruits in the haemophilia group, this reduces power to detect significant differences.&nbsp;
 
=== <br> Supporting Evidence ===
 
= Conclusion =
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed]) ==
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== References  ==


References will automatically be added here, see [[Adding References|adding references tutorial]].
Although results are well supported by similar small studies, it is evident that research into the psychosocial aspects of haemophilia is limited<ref>Cassis FR, Querol F, Forsyth A, Iorio A. Psychosocial aspects of haemophilia: a systematic review of methodologies and findings. Haemophilia 2012; 18:3:101-14. http://www.ncbi.nlm.nih.gov/pubmed/22059839. (accessed 22 Jan 2016).</ref> and participation in physical activity among PWH has not been well studied<ref>Buxbaum NP, Ponce P, Saidi P, Michaels LA.  Psychocial correlates of physical activity in adolescents with haemophilia. Haemophilia 2010;16:656-61. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02217.x/epdf. (accessed 21 Jan 2016).</ref>.
== References   ==


<references />
<references />
[[Category:Queen_Margaret_University_Project]]
[[Category:Physical_Activity]]
[[Category:Blood Disorders]]
[[Category:Paediatrics]]
[[Category:Paediatrics - Conditions]]

Latest revision as of 18:05, 3 January 2023

Original Editor - Caitlin MacRae, Hazel Burt, Steven Flett, Alex Leishman and Chloe Allan as part of the Queen Margaret University's Current and Emerging Roles in Physiotherapy Practice Project

Top Contributors - Caitlin MacRae, Hazel Burt, Alex Leishman, Chloe Allan, Kim Jackson, Steven flett, Rucha Gadgil, 127.0.0.1, Admin, Jane Hislop, Michelle Lee and Tarina van der Stockt  

Introduction[edit | edit source]

Physical activity and exercise, are often used synonymously, however, physical activity is defined as “as any bodily movement produced by skeletal muscles that require energy expenditure” [1], and exercise is a subsection of physical activity, involving planned or structured activity which aims to improve or maintain physical fitness [2].

The physiotherapist's role in treating and managing adolescents with haemophilia (AWH) is extremely important, and recent developments within this profession have increased its value [3]. One of the key interventions involved in its treatment is the promotion of physical activity [4].

Definition[edit | edit source]

Haemophilia is a rare inherited disorder where blood in the body has an inability to produce sufficient clotting factor, a protein which controls bleeding [5]. This lack of clotting factor can lead to severe and prolonged bleeds which may cause permanent damage to the site of haemorrhage and surrounding tissues[6]. There are an estimated 6000 people living in the United Kingdom with the condition. It occurs mainly through a gene passed from parent to child on the X chromosome, i.e. it mainly affects males[7]. 
There are four main types of haemophilia. The table below shows the missing clotting factor and commonality for each type:

Types table.jpg
  • Haemophilia A is the most common type affecting around 80% of the haemophilic population[8]. This is where the individual will either have low levels of clotting factor eight or it is completely missing. 
  • Haemophilia B is less common. This is where clotting factor nine is either very low or is missing from the blood. Around 20% of PWH will have this type[9].
  • Haemophilia C is ten times rarer than type A and differs from both A and B as it can present in both genders. However, this is extremely rare and can only occur when the mother and father are both carriers of the gene [10]. Individuals with type C will have low levels of clotting factor eleven, or this will be completed absent. 
  • Acquired Haemophilia is extremely rare. This type is not inherited in the same way, as it is an auto-immune disorder. This is where the body's immune system attacks the clotting factors. This condition can also affect both males and females [11].

Classifications and Severities[edit | edit source]

The table below categorises haemophilia into mild, moderate and severe, showing the percentage of sufferers in each category [12].

Severityhaem.jpg


Like other disorders, haemophilia can affect individuals to varying degrees. The symptoms can be mild, moderate or severe, depending on the level of clotting factor present[13].

Multidisciplinary Team Involvement                                                 [edit | edit source]

Despite recent development in the management of the condition, patients still require comprehensive multidisciplinary care throughout all stages of their life [14]. A multidisciplinary team involves a range of professionals who collaborate together to structure a treatment plan for an individual, aiming to achieve high quality patient-centered care [15].

The collaboration of all professionals allows a holistic approach to be considered which takes into account the individual’s physical, social, emotional, cultural and spiritual needs [16]. This is especially important during the transition from childhood into adulthood which can cause additional stress for those with chronic disorders. Therefore during this time they need for the MDT to work together is essential [14]. This may mean referring the patient to another team member to allow high-quality treatment, specific to the patients needs. 

Mdt altered.jpg

The Role of the Physiotherapist[edit | edit source]

Assessment[edit | edit source]

Joint swelling.jpg

When assessing a patients condition or evaluating the success of interventions it is often beneficial for the physiotherapist to use a standardised outcome measure. These offer an objective measure which can help to monitor change in a patients symptoms[17].

Symptoms of a joint bleed may include swollen and hot joints, pain in the area and stiffness when mobilising. Many patients have described the overall feeling as an 'aura' surrounding the joint[18], for example swelling in the knee during a joint bleed.

Outcome Measures:[edit | edit source]

Hjhs.jpg

'The Haemophilia Joint Health Score' [19] is designed for physiotherapists and recommended for use by professionals specialised in haemophilia treatment. The outcome measure takes approximately 45 – 60 minutes to complete and is designed specifically for children with haemophilia. It examines the condition of a joint affected by a bleed and can be used to demonstrate degeneration in a joint over time.

Paediatric Haemophilia Activities List picture.jpg

The 'Paediatric Haemophilia Activities List’ is another outcome measure used for individuals with haemophilia. Adolescents will complete the child/teenager version explaining to what extent their haemophilia impacts upon their daily activities The questionnaire can then be used by healthcare professionals involved in their care[20].

Although outcome measures exist for the assessment of children with haemophilia many of these have demonstrated poor validity and reliability[21].

Management[edit | edit source]

The main role of the physiotherapist when treating an AWH involves the management of impairments from a musculoskeletal perspective, aiming to restore and improve function. Some of these include: haemarthoses, synovitis, joint contractures, haemotomas and haemophilic arthropathys[22].

During the acute phase, the physiotherapist will attempt to minimise bleeding using the PRICE regime (protection, rest, ice, compression elevation)[23]. This will also work in conjunction with factor replacement if necessary.

PRICE2.png


Following the acute phase, the physiotherapy management will focus on regaining full range of movement, muscle strength and reducing pain[24]. As soon as the pain and swelling begin to decrease, the patient should be encouraged to gradually increase the joint range aiming to achieve complete extension. This should be done actively by the patient to encourage muscle contraction, however passive movements may initially be used. It is vital that early active muscle control occurs to prevent the loss of joint movement [25];[26];[27]. Rehabilitation involving active exercises and proprioceptive training must then be continued until full pre-bleed joint ROM and function is restored [28].

Another key role of the physiotherapist when treating an AWH is promotion of physical activity. Exercise is encouraged as it will improve the child’s fitness, reduce obesity levels, improve muscular strength, and reduce frequency of bleeding episodes and joint deterioration [29]. The World Federation of Haemophilia[30] advise particular focus towards strengthening, co-ordination, achieving a healthy body weight and improving self-esteem. As well as the physical aspects concerned, a major aspect of physiotherapy management focuses on education and advice.

Education[edit | edit source]

Below is a mind map which summarises key points regarding the advice that should be offered to patients around physical activity. 

Education picture.jpg

The physiotherapist plays a crucial role in:

  1. Educating patients regarding physical activity, exercise prescription and selection of appropriate activities[31].
  2. Ensuring patients, relatives and peers are made aware of the positive impact physical activity can have, helping to prevent joint damage and functional impairment[32]. Individuals who regularly exercise will also experience fewer bleeds[33].
  3. Informing patients that just as with healthy individuals, exercise has various benefits. Additional benefits such as enhanced lean tissue mass, improved fitness and strength and a decrease in body fat percentage are apparent during puberty in adolescents[34].
  4. Advising them to attempt non-contact sports such as swimming, walking, cycling, golf, archery, badminton, rowing and sailing in place of contact sports like rugby [35].
  5. Working in conjunction with the medical team when advising timing of prophylaxis appropriate to their chosen sport or activity[36];[37].
  6. Educating on and encouraging the use of protective equipment e.g if patient has a target joint, or does not take prophylaxis prior to activity [38]. Protective equipment is discussed later in the module.
  7. Educating school personnel regarding suitable activities for the child, discussing immediate care in case of a bleed, and explaining modifications in activities that may be necessary following bleeds[37]. 
    Some parents have found a traffic light coding system beneficial to inform teachers at school of the appropriate actions to take in event of specific injuries or bleed. This is also alluded to in the “get fit and active” video later in the module.

                                 Figure 10. Traffic light system

Traffic lights.jpg



Physical Activity Promotion[edit | edit source]

It is believed that physical activity in this population is reduced compared to healthy age-matched peers[39]. The Kings Fund[40] acknowledge health behaviour patterns to impact an individual's overall health by as much as 40%, when compared to healthcare which contributes only 10%. Therefore, as Physiotherapists, our role in health and activity promotion is crucial in order to influence positive behavioural change to impact upon health[41]. 

Health promotion involves supporting people to gain control and responsibility over improving their own health. It extends beyond focusing on individual behaviour and includes a variety of social and environmental interventions[42].

The Corporate Policy and Strategy Committee have introduced a Physical Activity for Health Pledge which the NHS is engaging with. This pledge involves ensuring primary care staff have the necessary skills and resources available to assess physical activity levels. It also ensures they offer education detailing recommended minimum requirements for physical activity, brief advice and intervention, along with increasing awareness of available community resources. Unpublished data from NHS Health Scotland reveals only 13% of primary care practitioners are presently aware of the recommended weekly activity levels [43]. It can therefore be assumed this is not currently being promoted effectively.

The following video "23 and a 1/2 hours" strongly promotes physical activity for at least 30 minutes of your day. Although not specific to the haemophilic population, the take home message can be followed for AWH. 

Although various studies have described the benefits of engaging in physical activity for people with haemophilia (PWH), results from research conducted in the USA among adolescent haemophilia patients have demonstrated a lack of knowledge concerning the role of physical activity in managing their condition[44]. Whilst it is recognised this information is important, this study has not been conducted in the UK so results must be generalised with caution. However, this suggests more work must be done to promote physical activity and emphasise its positive role in enhancing the lives of PWH [45].

Physical activity promotion for health exists worldwide [46]. The benefits of such in the general population are widely known, providing health improvements along with the potential for enhancing disease outcomes[47]. National and international guideliness set out clear recommendations on the physical activity levels required to promote health[48]. Although many studies have reported the benefits of participation in physical activity for PWH, researchers in the USA have identified a lack of knowledge among young haemophilia patients (aged 13–18 years) as to the role of exercise in the management of their condition.

Physical Activity - Associated Risks and Considerations[edit | edit source]

As physiotherapists it is vital to be aware of risks and considerations when advising patients to participate in physical activity or exercise.                   

Knowing whether patients have target joint bleeds allows the physiotherapist to adapt and alter their advice and treatment plan. Discussion can then be made around the use of protective equipment for the target joint, or whether there are any other activities which may reduce the risk of contact involving that specific joint.

It is important to know when the patient’s last acute bleed was and where. Activity must be gradually re-introduced following a bleed to minimise the chance of it reoccurring[49].

Appropriate timing of prophylaxis treatment surrounding physical activity is critical when striving to prevent bleeds. Prophylaxis should be individualised and taken to coincide with exercise. The aim being the factor will be at its peak during the period of activity[50]. This will minimise the risk or potential for bleeds[51]. As discussed previously, physiotherapists must be aware of this and communicate with the patient’s medical team regarding best advice.

Exercising with Inhibitors[edit | edit source]

Approximately 30% of patients with haemophilia will develop an immune response to medications used to treat the condition. If this occurs an alternative treatment may be necessary to manage bleeds. Immune tolerance therapy is introduced which aims to help the individual become accustomed to the factor[52].

Individuals with inhibitors may initially be apprehensive towards engaging in activity and exercise, causing their physical condition to rapidly decline. A program consisting of active range of motion, isometric and isotonic strengthening along with balance exercises can facilitate function and help to maintain independence[53].

Guidelines and Recommendations[edit | edit source]

It is important to note that the recommended guidelines for physical activity in children with haemophilia are the same as those advised for healthy individuals. Government-issued guidelines exist for children and young people aged 4 – 18. These guidelines state that this age group should be participating in 60 minutes of moderate to vigorous activity each day. Vigorous activity, which strengthens muscle and bone, should take place at least 3 times a week[54].
The World Haemophilia Federation[55] has released updated guidelines for the management of people with haemophilia:

  • The primary aim of management is to prevent and treat bleeds
  • Comprehensive care aims to promote health physically and psychosocially, as well as improving quality of life and reducing morbidity and mortality. Physiotherapists may play a more significant role in this aspect compared to managing the more acute phase of a bleed. Physiotherapy involvement contributes to both the prevention and treatment of bleeds.
  • Children and adolescents should be reviewed every 6 months by a physiotherapist for assessment and management planning
  • Physiotherapy has a main role in promoting physical activity: to improve fitness, neuromuscular development, strengthening, coordination, functioning, weight management and self esteem
  • Weight bearing activities should be encouraged to enhance bone mineral accrural
  • A physical activity plan should be tailored to individuals preference, abilities and considering the severity of their condition
  • Non-contact sports will be encouraged – i.e. swimming, walking, golf, badminton, cycling, table tennis
  • Contact sports and high velocity sports are strongly discouraged due to the risk of a fatal bleed – i.e. rugby, boxing, wrestling, racing, skiing
  • Target joints can be protected using braces or splints during activities
  • Following a bleed the activity should be reintroduced gradually to minimise the risk of re-bleeding
  • Adjunctive management is also vital to physiotherapy. This means activity should be undertaken in conjunction with factor replacement. Physiotherapy can also help to reduce the amount of factor required. 
  • In the acute phase, PRICE will be used by the physiotherapist
  • Following this, rehabilitation is required to restore function


"Playing it Safe"[56] is an online document which provides recommendations and advice regarding participation in sport and exercise when suffering with a bleeding disorder.

Personal Protective Equipment[edit | edit source]


Protective equipment can be worn by individuals to minimise the risk of bleeding either at a very young age or when taking part in sports and activities at an older age[57]. This is generally used considering personal preference, the child’s compliance and the level of sporting activity undertaken. Splints or braces can be useful in protecting target joints whilst participating in sports[58].

Joints which are considered at high risk of bleeds are the elbows, wrists, knees and ankles. Toddlers are encouraged to be protected with elbow and knee pads when crawling and helmets can also be used to protect children from falls, especially when cycling or running. Due to the introduction and advances in prophylaxis, the need for protective equipment in day to day activities has been reduced. It is suggested that although previously considered extremely important and beneficial, the social risks now outweigh the potential benefits of protective equipment. However, this is a decision that must be made based on the type and competitiveness of the activity the individual is participating in.

There are many different types of protective equipment available and multiple ways of modifying home environments to make it safer for toddlers and young children.

How to Approach Conversations Regarding Physical Activity[edit | edit source]

It can sometimes be difficult for physiotherapists to approach the subject of physical activity with an individual and encouraging behavioural change can be increasingly difficult in AWH. This is due to the nature of the condition as this is normally a stage in their life where physical activity levels reduce[59]. Physiotherapists can offer education and advice about what type of activity to undertake in order to prevent bleeds occurring.
Appropriate communication is essential when discussing physical activity with patients. The Royal College of General Practitioners[60] makes recommendations on how to appropriately communicate ideas to a patient.

Benefits of Physical Activity[edit | edit source]

The video below offers some general advice and recommendations regarding increasing physical activity in PWH. It follows the story of Sean, an individual with haemophilia, as he discusses some of the benefits he believes are associated with being physically active when living with this condition. 


Video used with permission from Clinical Specialist Physiotherapist, Jenna Reid. 

Physical Activity Relating to Obesity[edit | edit source]

Childhood obesity is prevalent in AWH, as well as the general population. However, the consequences of weight gain in sufferers can cause additional issues, such as increased risk of joint arthropathy[61]. Excess weight can induce joint bleeds due to added stresses placed on the joints [62]. Effects of obesity and overweight can also exacerbate existing arthropathies and influence development of cardiovascular disease[63].

Parental influence is also considered to be a contributing factor to obesity and overweight in this population. Many parents tend to be very protective of their child, preventing them from engaging in physical activity. It was not until the 1970s that exercise in this population was recognised as effective. Prior to this it was considered to increase the risk of haemarthroses[64]. A previously discussed, it is now recommended for particular activities, mainly swimming, with aims of improved quality of life. This new treatment approach is largely due to recent advances in safety and availability in the clotting factor used to prevent bleeds[65]. Buzzard and Beeton[66] also report evidence to suggest changes in blood clotting factors due to exercise.

The benefits of physical activity in the general population are widely known, providing general health improvements along with potential enhancement in disease outcomes[67]. However, Philpott et al.[38] believe children suffering with a chronic disease or disability are less active than their age-matched healthy peers. A study conducted by Hofstede et al.[68] comparing healthy subjects to AWH reiterates; revealing triple the number of obese boys with haemophilia in relation to the comparable healthy subjects. Contrary to this finding, a 2010 study quantifying physical activity levels through accelerometry, revealed AWH to be more involved in physical activity compared to their healthy peers. However, the study also stated the time dedicated to physical activity was mainly focused towards low-intensity activities and sedentary behaviours[69]. Several studies have reported an association between an increase in obesity levels and sedentary behaviours particularly amoung AWH. This increase is concerning, however does not differ much from the un-affected population[70];[71].

The overall consensus, as previous stated, is that an accumulation of 60 minutes or more physical activity of moderate intensity, consisting of both aerobic daily activity and vigorous intensity aerobic activity across a minimum of 3 days per week is advised [72]. However, the physical activity recommendations remain limited[73];[74], with findings from recent studies demonstrating controversial outcomes.

Childhood Development[edit | edit source]

Adolescents undergo more physical changes at this age than throughout any other stage of their life. The rate of growth in itself can predispose the child to bleeds. Growth spurts around puberty may decrease the muscle mass surrounding joints and consequently increase joint bleeding during this period[75].

Weight-bearing activities are crucial in ensuring adequate generation of bone mass throughout childhood and may be even be considered more important than calcium intake through diet[76]. A study conducted in 2008, by Tlacuilo-Parra et al.[77] revealed a significant reduction in lumbar spine bone mineral density in children with haemophilia in comparison to healthy subjects of matched age and sex. The results demonstrated statistically significant differences in activity between the groups, with the haemophilia population at three times the risk of inactivity. These results confirm that long periods of inactivity and immobility, with reduced weight-bearing (such as following joint bleeds) are directly related to low bone mineral density in AWH. 

Psychosocial Factors Associated With Physical Activity[edit | edit source]

Due to the nature of the condition, AWH are usually diagnosed and aware of their condition from a very young age. Psychosocial, as well as physical barriers may exist in relation to participation in physical activity[78].

                                                                                       

The World Federation of Haemophilia (WFH) separates a number of different psychosocial issues into specific age groups[79]:

  • Infant to toddler (newborn to age 5)
  • Early childhood (6-9 years)
  • Pre-adolescence (10-13 years)
  • Adolescence (14-17 years)
  • Adulthood (18 years +)

It is important that at every stage of the child’s journey there is help and support available to the individual and family to ensure that psychosocial barriers are minimised[80]. Some of the psychosocial issues that are common in adolescence are discussed below.

Transition Phase[edit | edit source]

In the adolescence stage, there is a transition of care from paediatrics to general practitioners. During this period it is important to focus on self -management and increasing independence in treatment. Although this can increase confidence in self-treatment with greater control over their condition, a common issue at this age is compliance.

Compliance[edit | edit source]

Cassis[81] states that at this stage of development, teenagers are often focussed on present events and do not consider the long term consequences of poorly managing their condition. As a child begins to mature, other activities may take priority over physiotherapy or exercise therefore, it is important that they are aware of the harmful consequences associated with inactivity.

Physical activity can play a large part in a child’s social and emotional well-being. Through team-based sports they learn how to work with other peers. Improved emotional status has also demonstrated marked clinical benefits with reported reductions in spontaneous bleeds[82]. 

Cassis et al.[83] conducted a systematic review comparing 24 studies considering the psychosocial aspects concerned with haemophilia. Results demonstrated psychosocial factors to have a significant impact on quality of life for patients with chronic conditions such as haemophilia. One of the studies, found the most common stressor associated with psychological factors to be reductions in lifestyle activity, including sport. This may be due to fear of inducing a bleed through exercise, or the stigma associated with wearing protective equipment.

The review also found that for PWH, participation in physical activites and sports will positively effect not only physical well-being but also self-esteem and social interactions. This is further supported by Sherlock[84] who found that non-physical benefits of sporting activities included improved self-esteem and perceived social acceptance. It also supported the above view that quality of life in PWH is impaired by limitations in sporting activity.

However, Buxaum et al.[85] conducted a study which aimed to establish social and cognitive factors associated with exercise in PWH. All particpants were aged between 11 and 18. The 'Peirs Harris Childrens Concept Scale' and the 'Childrens Manifest Anxiety Scale' were used to measure self-esteem and anxiety, as these are self-reported scales results must be interpreted with caution. The study found no differences in outcomes when comparing PWH to healthy controls. This conflicts with previous evidence, however, due to its small sample size with only 17 recruits in the haemophilia group, this reduces power to detect significant differences. 

Although results are well supported by similar small studies, it is evident that research into the psychosocial aspects of haemophilia is limited[86] and participation in physical activity among PWH has not been well studied[87].

References[edit | edit source]

  1. ↑ World Health Organization. Physical Activity. http://www.who.int/topics/physical_activity/en/ (accessed 25 January 2016).
  2. ↑ Casperson SJ, Powell KE, Christenson GM. Physical activity, exercise, and physical fitness: definitions and distinctions for health-related research. Public health Rep 1985;100;(2):126–131. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1424733/ (accessed 25 January 2016).
  3. ↑ Buzzard B, Beeton K. Physiotherapy Management of Haemophilia. Oxford: Wiley, 2008.
  4. ↑ World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 October 2015).
  5. ↑ NHS Choices. Haemophilia. http://www.nhs.uk/conditions/haemophilia/Pages/Introduction.aspx (accessed 7 Jan 2016).
  6. ↑ World Federation of Haemophilia. Severity of hemophilia. http://www.wfh.org/en/page.aspx?pid=643 (accessed 7 January 2016).
  7. ↑ Great Ormond Street Hospital. What causes Haemophilia? http://www.gosh.nhs.uk/medical-information/search-medical-conditions/haemophilia (accessed 10 Jan 2016).
  8. ↑ The Haemophilia Society. Haemophilia A.fckLRhttp://www.haemophilia.org.uk/bleeding_disorders/bleeding_disorders/haemophilia_a (accessed 9 November 2015).
  9. ↑ The Haemophilia Society. Haemophilia B. fckLRhttp://www.haemophilia.org.uk/bleeding_disorders/bleeding_disorders/haemophilia_fckLRb (accessed 9 November 2015).
  10. ↑ Haemophilia Information. Haemophilia Genetics. http://www.hemophilia-information.com/hemophilia-genetics.html (accessed 12 September 2015).
  11. ↑ Grethlein SJ. Acquired Haemophilia. http://emedicine.medscape.com/article/211186-overview#a2fckLR (accessed 7 January 2016).
  12. ↑ Preston FE, Kitchen S, Jennings L, Woods TA, Mackris M. SSC/ISTH classification of hemophilia A: can hemophilia center laboratories achieve the new criteria? J Thromb Haemost 2004;(2):271-4. http://www.ncbi.nlm.nih.gov/pubmed/14995989 (accessed 10 January 2016).
  13. ↑ NHS Choices. Haemophilia symptoms. http://www.nhs.uk/Conditions/Haemophilia/Pages/Symptoms.aspx (accessed 10 Jan 2016).
  14. ↑ 14.0 14.1 Breakey VR, Blanchette VS, Bolton-Maggs PHB. Towards comprehensive care in transition for young people with haemophilia. Haemophilia 2010;16(6):848-857. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02249.x/abstract (accessed 18 Dec 2015).
  15. ↑ Department of Health. Multidisciplinary teams (MDT’s). http://www.health.nt.gov.au/Cancer_Services/CanNET_NT/Multidisciplinary_Teams/index.aspx (accessed 7 Jan 2016).
  16. ↑ Chartered Society of Physiotherapy. Physiotherapy works for social care – Why Physiotherapy? http://www.csp.org.uk/physiotherapy-works-social-care (accessed 10 Jan 2016).
  17. ↑ American Physical Therapy Association. Outcome Measures in Patient Care. http://www.apta.org/OutcomeMeasures/ (accessed 20 Jan 2016).
  18. ↑ Haemophilia Care. How to recognise a bleed. http://www.haemophiliacare.co.uk/how-to-recognise-a-bleed.html (accessed 7 Jan 2016)
  19. ↑ International Prophylaxis Study Group. HJHS. http://ipsg.ca/working-groups/physical-therapy/info/hjhs (accessed 15 Dec 2015).
  20. ↑ World Federation of Haemophilia, Paediatric Haemophilia Activities List: http://www.wfh.org/en/page.aspx?pid=886 (accessed 20 Oct 2015).
  21. ↑ Stephensen D, Drechsler WI, Scott OM. Outcome measures monitoring physical function in children with haemophilia: a systematic review. Haemophilia 2014;20:306-321. http://onlinelibrary.wiley.com/doi/10.1111/hae.12299/epdf (accessed 19 Jan 2016).
  22. ↑ Buzzard B, Beeton K. Physiotherapy Management of Haemophilia. Wiley-Blackwell, 2010.
  23. ↑ Haemophilia Care. How is Physiotherapy used in Haemophilia care. http://www.haemophiliacare.co.uk/physiotherapy.html (accessed 10 Oct 2015).
  24. ↑ Talking Joints. Let’s talk about physiotherapy for haemophilia patients. https://www.novonordisk.com/content/dam/Denmark/HQ/aboutus/documents/cpih-2015-talkingjoints-brochure-lets-talk-physiotherapy.pdf (accessed 10 Oct 2016).
  25. ↑ Hermans C, De Moerloose P, Fischer K, Holstein K, Klamroth R, Lambert T, Lavigne-Lissalde G, Perez R, Richards M, Dolan G. European Haemophilia Therapy Standardisation Board. Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations. Haemophilia 2011;17(3):383-92. http://www.ncbi.nlm.nih.gov/pubmed/21323794 (accessed 30 Sept 2015).
  26. ↑ Gomis M, Querol F, Gallach JE, Gonzalez LM, Aznar JA. Exercise and sport in the treatment of haemophilic patients: a systematic review. Haemophilia 2009;15(1):43-54. http://www.ncbi.nlm.nih.gov/pubmed/18721151 (accessed 10 Jan 2016).
  27. ↑ Mulder K. Exercises for people with haemophilia. Montreal: World Federation of Haemophilia. 2006. http://scholar.google.co.uk/scholar_url?url=http%3A%2F%2Fhaemophilia.ie%2FPDF%2FExercise_Guide_med.pdf(accessed 22 Jan 2016).
  28. ↑ Heijnen L, Buzzard BB. The role of physical therapy and rehabilitation in the management of hemophilia in developing countries. Semin Thromb Hemost 2005;31(5):513-7. http://www.ncbi.nlm.nih.gov/pubmed/16276459 (accessed 25 Sept 2015).
  29. ↑ Blamey G, Forsyth A, Zourikian N, Short L, Jankovic N, Kleijn PDE, Flannery T. Comprehensive elements of a physiotherapy exercise program in haemophilia – a global perspective. World Federation of Haemophilia 2010;16:136-145 http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02312.x/abstract;jsessionid=BB3B137A0C21342D378A949A977850CA.f04t01fckLR(accessed 10 Oct 2015).
  30. ↑ World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).
  31. ↑ Heijnen L. Rehabilitation and the role of the physical therapist. Southeast Asian J Trop Med Public Health 1993;24(Suppl. 1):26–9. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3570911/ (accessed 25 Sept 2015).
  32. ↑ Buzzard B. Physiotherapy for Prevention and Treatment of Chronic Haemophilic Synovitis. Clinical Orthopaedics and Related Research. Orthopaedics and Related Research 1997;343:42-46. http://www.ncbi.nlm.nih.gov/pubmed/9345204 (accessed 9 Jan 2016).
  33. ↑ Tiktinsky R, Falk B, Heim M, Martinovitz U. The effect of resistance training on the frequency of bleeding in haemophilia patients: a pilot study. Haemophilia 2002;8:22–7. http://www.ncbi.nlm.nih.gov/pubmed/11886461 (accessed 15 Dec 2015).
  34. ↑ U.S. Department of Health and Human Services. Physical Activity Guidelines Advisory Committee report. http://health.gov/paguidelines/report/pdf/committeereport.pdf (accessed 17 Jan 2016).
  35. ↑ World Federation of Hemophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd. 2012;2:6-73. 2012. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).
  36. ↑ McGee S et al. Organized sports participation and the association with injury in paediatric patients with haemophilia. Haemophilia 2015;21(4):538-542. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2786948/ (accessed 15 Nov 2015).
  37. ↑ 37.0 37.1 World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).
  38. ↑ 38.0 38.1 Philpott J, Houghton K, Luke A. Physical activity recommendations for children with specific chronic health conditions: Juvenile idiopathic arthritis, hemophilia, asthma and cystic fibrosis. Paediatrics Child Health 2010;15(4):213-218. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2866314/ fckLR(accessed 6 Oct 2015).
  39. ↑ Darby SC, Wan Kan S, Spooner RJ, Giangrande PLF, Hill FGH, Hay CRM, Lee CA, Ludlam CA, Williams M. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 2007;110(3):815-825. :http://www.bloodjournal.org/content/110/3/815?sso-checked=true (accessed 2 Jan 2016).
  40. ↑ The Kings Fund. Inequalities in life expectancy Changes over time and implications for policy. http://www.kingsfund.org.uk/sites/files/kf/field/field_publication_file/inequalities-in-life-expectancy-kings-fund-aug15.pdf (accessed 24 Nov 2015).
  41. ↑ Chartered Society of Physiotherapy. Physio 15: Physios can improve public health by influencing behaviour and promoting physical activity. http://www.csp.org.uk/news/2015/10/21/physio-15-physios-can-improve-public-health-influencing-behaviour-promoting-physical (accessed 27 Nov 2015).
  42. ↑ World Health Organization. Health Promotion. http://www.who.int/topics/health_promotion/en/ (accessed 28 Dec 2015).
  43. ↑ Corporate Policy and Strategy Committee. Physical Activity for Health Pledge. 2-12 www.edinburgh.gov.uk (accessed 8 Oct 2015).
  44. ↑ Nazzaro AM, Owens S, Hoots WK, Larson KL. Knowledge, attitudes, and behaviors of youths in the US hemophilia population: results of a national survey. American Journal of Public Health 2006;96(9):1618-1622. http://www.ncbi.nlm.nih.gov/pubmed/16873741 (accessed 3 Jan 2016).
  45. ↑ Negrier C, Seuser A, Forsyth A, Lobet S, Llinas A, Roas M, Heijnen L. The benefits of exercise for patients with haemophilia and recommendations for safe and effective physical activity. Haemophilia 2013;19(4):487-498. http://www.ncbi.nlm.nih.gov/pubmed/23534844 (accessed 7 Jan 2016).
  46. ↑ Heath GW, Parra DC, Sarmiento OL, Andersen LB, Owen N, Goenka S, Montes F, Brownson RC. Evidence-based intervention in physical activity: lessons from around the world. Lancet 2012; 380(9838):272-81. http://www.ncbi.nlm.nih.gov/pubmed/22818939 (accessed 26 Jan 2016).
  47. ↑ World Health Organization. Health Promotion. http://www.who.int/topics/health_promotion/en/ (accessed 28 Dec 2015).
  48. ↑ Department of Health. Multidisciplinary teams (MDT’s). http://www.health.nt.gov.au/Cancer_Services/CanNET_NT/Multidisciplinary_Teams/index.aspx (accessed 7 Jan 2016).
  49. ↑ World Federation of Haemophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).
  50. ↑ Anderson A, Forsyth A. Playing it safe: Bleeding Disorders, Sport and Exercise New York: National Haemophilia Foundation. 1- 44. https://www.hemophilia.org/…/docume…/files/PlayingItSafe.pdf (accessed 6 Oct 2015).
  51. ↑ World Federation of Haemophilia. About bleeding disorders. http://www.wfh.org/en/page.aspx?pid=642 (accessed 7 Jan 2016).
  52. ↑ The Haemophilia Society. Inhibitors. http://www.haemophilia.org.uk/bleeding_disorders/bleeding_disorders/inhibitors (accessed 26 Jan 2016).
  53. ↑ Caviglia H, Narayan P, Forsyth A, Giangrande P, Gilbert M, Goddard N, Mulder K, Rahima S. Musculoskeletal problems in persons with inhibitors: How do we treat?. Haemophilia 2012;18(4):54-60. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2012.02874.x/epdf (accessed 26 Jan 2016).
  54. ↑ Department of Health. Physical Activity Guidelines for Children and Young People. https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/213739/dh_128144.pdf (accessed 10 Oct 2016).
  55. ↑ World Federation of Heamophilia. Guidelines for the Management of Haemophilia. Canada: Blackwell Publishing Ltd 2012;2:6-73. http://www1.wfh.org/publication/files/pdf-1472.pdf (accessed 2 Oct 2015).
  56. ↑ National Haemophilia Foundation. Play it Safe. https://www.hemophilia.org/sites/default/files/document/files/PlayingItSafe.pdf (accessed 20 Oct 2015).
  57. ↑ Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 10 Jan 2016).
  58. ↑ World Federation of Haemophilia. About bleeding disorders. http://www.wfh.org/en/page.aspx?pid=642 (accessed 7 Jan 2016).
  59. ↑ Majumdar S, Morris A, Gordon C, Kermode CJ, Forsythe A, Herrington B, Megason GC, Iyar R. Alarmingly high prevalence of obesity in haemophilia in the state of Mississippi. Haemophilia 2010;16;455-459. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2009.02187.x/epdf (accessed 28 Jan 2016).
  60. ↑ Royal College of General Practitioners. Health literacy: report from an RCGP-led health literacy workshop. http://www.rcgp.org.uk/clinical-and-research/a-to-z-clinical-resources/~/media/Files/CIRC/Health-Literacy-Report/Health%20Literacy%20Final%20edition%2029%2007%202014.ashx (accessed 20 Nov 2015).
  61. ↑ Young G. New challenges in hemophilia: long-term outcomes and complications. Hematology 2012;1:362 - 368. http://asheducationbook.hematologylibrary.org/…/362.full.pd… (accessed 26 Oct 2015).
  62. ↑ Tuinenburg A, Biere-rafi S, Peters M, Verhamme P, Peerlinck K, Kruip MJHA, Laros-van Gorkom BAP, Roest M, Meijers JCM, Kamphuisen PW, Schutgens REG. Obesity in haemophilia patients: effect on bleeding frequency, clotting factor concentrate usage, and haemostatic and fibrinolytic parameters. Haemophilia 2013;(19):744-752. http://www.pfizerpro.com.co/sites/g/files/g10017511/f/publicaciones/092013%20V19%20I5%20-Obesity%20in%20haemophilia%20patients%20effect%20on%20bleeding%20frequency,%20clotting%20factor%20concentrate%20usage,%20and%20haemostatic%20and%20fibrinolytic%20parameters.pdf (accessed 2 Dec 2015).
  63. ↑ Douma-van Riet DMC, Engelbert RHH, Van Genderen FR, Horst-De Ronde MTM, Goede-Bolders A, Hartman A. Physical fitness in children with haemophilia and the effect of overweight. Haemophilia 2009;15:519-527. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2008.01928.x/epdf (accessed 22 Jan 2016).
  64. ↑ Broderick CR, Herbert RD, Latimer J, Curtin JA, Selvadurai HC. The Effect Of An Exercise Intervention On Aerobic Fitness, Strength and Quality of life in children with Haemophilia. BMC Blood Disorders 2006;6:2. http://www.biomedcentral.com/content/pdf/1471-2326-6-2.pdf (accessed 9 Oct 2015).
  65. ↑ Querol F, Perez-alendaa S, Gallachc JE, Devis-devis J, Valencia-peris A, Gonzalez Moreno LM. Haemophilia: exercise and sport. Apunts Med Esport 2010;46(165:29-39. http://www.elsevier.es/…/277/277v46n169a90001817pdf001_2.pdf (accessed 15 Oct 2015)
  66. ↑ Buzzard B, Beeton K. Physiotherapy Management of Haemophilia. Oxford: Wiley, 2008.
  67. ↑ Durstine JL, Gordon B, Wang Z, Luo X. Chronic disease and the link to physical activity. Journal of Sport and Health Science 2013;2(1)3-11. http://www.sciencedirect.com/science/article/pii/S2095254612000701 (accessed 13 Dec 2015).
  68. ↑ Hofstede FG, Fijnvandraat K, Plug I, Kamphuisen PW, Rosendaal FR, Peters M. Obesity: a new disaster for haemophiliac patients? A nationwide survey. Haemophilia 2008;14:1035-1038. http://onlinelibrary.wiley.com/…/j.1365-2516.2008.01806.x.p… (accessed 30 Sept 2015).
  69. ↑ Buxbaum NP, Ponce P, Saidi P, Michaels LA. Psychosocial correlates of physical activity in adolescents with haemophilia. Haemophilia. 2010;16:656-61. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02217.x/epdf. (accessed 21 Jan 2016).
  70. ↑ Hofstede FG, Fijnvandraat K, Plug I, Kamphuisen PW, Rosendaal FR, Peters M. Obesity: a new disaster for haemophiliac patients? A nationwide survey. Haemophilia 2008;14:1035-1038. http://onlinelibrary.wiley.com/…/j.1365-2516.2008.01806.x.p… (accessed 30 Sept 2015).
  71. ↑ Douma-van Riet DMC, Engelbert RHH, Van Genderen FR, Horst-De Ronde MTM, Goede-Bolders A, Hartman A. Physical fitness in children with haemophilia and the effect of overweight. Haemophilia 2009;15:519-527. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2008.01928.x/epdf (accessed 22 Jan 2016).
  72. ↑ Licence K. Promoting and protecting the health of children and young people. Child: care, health and development 2004;30(6):623-635. http://onlinelibrary.wiley.com/enhanced/doi/10.1111/j.1365-2214.2004.00473.x (accessed 22 Jan 2016).
  73. ↑ Beardsley DS. Sport and Exercise for children with chronic health conditions. Champaign IL:Human Kinetics 1995:302-9.
  74. ↑ Buzzard BM. Sports and haemophilia: antagonist or protagonist. Clinical Orthopaedics and Related Research 1996;328:25-30. http://www.ncbi.nlm.nih.gov/pubmed/8653965 (accessed 19 Jan 2016).
  75. ↑ Johnson MJ, Gorlin JB. Child Development with a Bleeding Disorder and Transition. New York: National Hemophilia Foundation 2013:1-16. https://www.hemophilia.org/…/Nurses-Guide-Chapter-13a-Child… (accessed 10 Oct 2015).
  76. ↑ Barnes C, Wong P, Egan B, Speller T, Cameron F, Jones G, Ekert H, Monagle P. Reduced Bone Density Among Children With Severe Hemophilia. Pediatrics 2004;114:2:e177-e181. http://www.ncbi.nlm.nih.gov/pubmed/15286254 (accessed 9 Oct 2015).
  77. ↑ Tlacuilo-parra A, Morales-zambrano R, Tostadorabago N, Esparza-Flores MA, Lopez-Guido B, Orozcalcala J. Inactivity is a risk factor for low bone mineral density among haemophilic children. British Journal of Haematology 2008;140:562-567 http://onlinelibrary.wiley.com/…/j.1365-2141.2007.06972.x.p… (accessed 8 Nov 2015).
  78. ↑ ICF. International Classification of Functioning, Disability and Health (ICF). http://www.who.int/classifications/icf/en/ (accessed 5 Jan 2016).
  79. ↑ Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 10 Jan 2016).
  80. ↑ Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 10 Jan 2016).
  81. ↑ Cassis FRMY. Treatment of Haemophilia. Psychosocial Care for people with Haemophilia 2007;1-12. https://haemophilia.ie/PDF/Psychosocial-Care.pdf (accessed 12 Jan 2016).
  82. ↑ Anderson A, Forsyth A. Playing it safe: Bleeding Disorders, Sport and Exercise New York: National Haemophilia Foundation. 1- 44. https://www.hemophilia.org/…/docume…/files/PlayingItSafe.pdf (accessed 6 Oct 2015).
  83. ↑ Cassis FR, Querol F, Forsyth A, Iorio A. Psychosocial aspects of haemophilia: a systematic review of methodologies and findings. Haemophilia 2012; 18:3:101-14. http://www.ncbi.nlm.nih.gov/pubmed/22059839. (accessed 22 Jan 2016).
  84. ↑ Sherlock E, O’Donnell JS, White B, Blake C. Physical activity levels and participation in sports in Irish people with haemophilia. Haemophilia 2010;16:202-9. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2009.02111.x/epdf. (accessed 21 Jan 2016).
  85. ↑ Buxbaum NP, Ponce P, Saidi P, Michaels LA. Psychocial correlates of physical activity in adolescents with haemophilia. Haemophilia 2010;16:656-61. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02217.x/epdf. (accessed 21 Jan 2016).
  86. ↑ Cassis FR, Querol F, Forsyth A, Iorio A. Psychosocial aspects of haemophilia: a systematic review of methodologies and findings. Haemophilia 2012; 18:3:101-14. http://www.ncbi.nlm.nih.gov/pubmed/22059839. (accessed 22 Jan 2016).
  87. ↑ Buxbaum NP, Ponce P, Saidi P, Michaels LA. Psychocial correlates of physical activity in adolescents with haemophilia. Haemophilia 2010;16:656-61. http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2010.02217.x/epdf. (accessed 21 Jan 2016).
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