Physical Activity in Adolescents with Haemophilia

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Introduction

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].

Definitions, Incidence and Prevalence

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 site of haemorrhage and surrounding tissues[6]. There are an estimated 6000 people living in the United Kingdom with the condition. Due to its genetic carriage, through a gene passed from parent to child on the X chromosome, 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].
The figure below demonstrates how haemophilia is passed on genetically:
Genetics.jpg

As shown above, when the female is the carrier 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[12]. 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[12]. If the father has haemophilia and the mother is a carrier, there is a 25% chance the daughter and son may have haemophilia. This is the only way the female can inherit the condition, however, it is extremely rare.

Classifications and Severities

The table below categorises haemophilia into mild, moderate and severe, showing the percentage of sufferers in each category [13].
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[14].

In mild haemophilia the individual will have between 4% - 49% factor in their blood. Around 43-70% of the haemophilic population are considered mildly affected[15]. Symptoms for mild haemophilia can be non-existent, although may include prolonged bleeding following serious injury, trauma or surgery. In many cases, haemophilia will not be diagnosed until there is excessive bleeding following injury or surgery. In some cases it may not even be discovered until adulthood.

Moderate haemophilic sufferers possess between 1% and 5% of factor level in their blood. 15-26% of AWH are considered moderate[15]. Those with this type are likely to suffer prolonged bleeding following injury and may have occasional spontaneous bleeding episodes.

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[16]. They willl experience excessive bleeding following even slight injuries and are also highly likely to have spontaneous bleeding episodes into joints and surrounding muscles[17]. This internal bleeding is particularly dangerous as suffers may be unaware when it is occurring. If untreated it can lead to permanent joint deterioration and arthritis over time.

Medical Management

The medical management of haemophilia typically involves two treatment methods, ‘on demand’ or prophylactic. Haemophilia Care explains how treatment involves replacing the deficient clotting factor (VIII for haemophilia A and IX for haemophilia B) through intravenous injection[18].

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 delay in ‘on demand’ treatment following a bleed increases potential for damage to occur.

Prophylaxis is administered regularly as a method of preventative management and is most commonly used in those with severe haemophilia. The ultimate goal of prophylaxis treatment is to reduce the deficiency of factor VII or factor IX [19], therefore reducing the risk of spontaneous bleeds. It also aims to reduce the severity of the condition [20]. Khoriaty et al 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 severe haemophilia B (factor IX)[21]. Johannes Oldenburg recognises prophylaxis treatment as the optimal treatment for patients with severe haemophilia [22].

MDT Involvement                                                 

Despite recent development in management of the condition, PWH still require comprehensive multidisciplinary care throughout all stages of their life [23]. 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 [24].

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 [25]. 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 [23]. 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

Assessment

Joint swelling.jpg
When assessing a patients condition or evaluating 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[26].

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[27], for example swelling in the knee during a joint bleed
Hjhs.jpg
'The Haemophilia Joint Health Score' [28] 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[29].

Although outcome measures exist for the assessment of children with haemophilia many of these have demonstrated poor validity and reliability[30]. Physiotherapists play a key role in assessing the joints affected by haemophilia, therefore it is beneficial for physiotherapists to be aware of other existing measures available in order to rehabilitate a patient appropriately.

Management

The main role of the physiotherapist when treating an AWH involves 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[31].

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)[32]. 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[33]. 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 [34];[35];[36]. Rehabilitation involving active exercises and proprioceptive training must then be continued until full pre-bleed joint ROM and function is restored [37].

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 [38]. The World Federation of Haemophilia[39] 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

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 educating patients regarding physical activity, exercise prescription and selection of appropriate activities[40].

It is important to ensure patients, relatives and peers are made aware of the positive impact physical activity can have, helping to prevent joint damage and functional impairment[41]. Individuals who regularly exercise will also experience fewer bleeds[42].

You should inform 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[43]

Depending on the severity of the condition, as the physiotherapist you must then advise the patient that high contact and collision sports such as ice hockey, rugby, boxing, and wrestling are usually not advised. You can then attempt to encourage non-contact sports such as swimming, walking, cycling, golf, archery, badminton, rowing and sailing [44].

You must work in conjunction with the medical team when advising timing of prophylaxis appropriate to their chosen sport or activity[45];[46]. It may also be necessary, where appropriate, to educate on and encourage the use of protective equipment e.g if patient has a target joint, or does not take prophylaxis prior to activity [47]. Protective equipment is discussed later in the module.

As the physiotherapist you may also be involved in 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[46]

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

It is believed that physical activity in this population is reduced compared to healthy age-matched peers[48]. The Kings Fund[49] 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[50]

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[51].

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 [52]. 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[53]. 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 [54].

Physical activity promotion for health exists worldwide [55]. The benefits of such in the general population are widely known, providing health improvements along with the potential for enhancing disease outcomes[56]. National and international guideliness set out clear recommendations on the physical activity levels required to promote health[57]. 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.

The promotion of exercise in AWH is of particular importance due to recent advances in care over the past 40 years [58]. 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[59]

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.

When discussing forms of exercise with the patient, the following information is essential to obtain from your patient;

                                                                  Figure 11. Questions to ask the patient before treatment:

Sbfc.jpg


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[60].

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[61]. This will minimise the risk or potential for bleeds[62]. As discussed previously, physiotherapists must be aware of this and communicate with the patient’s medical team regarding best advice.

Exercising with Inhibitors

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[63].

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[64].

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[65].


The World Haemophilia Federation[66] 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"[67] is an online document which provides recommendations and advice regarding participation in sport and exercise when suffering with a bleeding disorder.
This document offers recommendations on the safest activities to participate in and offers advice on specific sports and exercises with the highest risk of injury.
For adolescents, when deciding on which activities to take part in, it is important to consider current body condition, history of bleeding and joint condition.
It is also proven the more physically active you were through childhood the easier it will be to remain active through teenage years and into adulthood.
As children become teenagers some sports such as basketball involve more contact – therefore although these sports may have been possible when the child was younger they may now present with more risks.

Personal Protective Equipment


Protective equipment can be worn by individuals to minimise risk of bleeding either at a very young age or when taking part in sports and activities at an older age[68]. This is generally used considering personal preference, the child’s compliance and the level of sporting activity undertaken. Splints or braces can be serve useful in protecting target joints whilst participating in sports[69].

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.

An example of the various different types of protective equipment are shown in the table below:

Table 5. Examples of protective headgear[70]

Cap 1.jpg This headgear has been proven to prevent traumatic brain injuries. It adapts to all sizes and head shapes. It is extremely secure and can protect against a hard fall or high impact. This helmet is highly recommended by neurosurgeons for patients with haemophilia among other conditions.
Cap 2.jpg
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 reduce social risks as it resembles a standard cotton hat.
Cap 3.jpg This is another protective helmet which provides high protection and safety from skull injuries sustained from a bad fall. It is made from 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 by all ages in sport.

How to Approach Conversations Regarding Physical Activity

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[71]. 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[72] makes recommendations on how to appropriately communicate ideas to a patient.

Here are some tips and approaches which can facilitate behavioural change in an AWH:

  • Be aware of the amount of information you provide – be careful not to overload them.
  • Avoid the use of technical vocabulary as it may cause confusion.
  • Use different methods other than verbal communication to provide information e.g. leaflets, video, online resources.

                                                                                                                     Figure 12. 5 A strategy of discussion
    5 A picture.jpg

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[73]. The picture on the right explains what this involves:

Finally OARS is a form of motivating the patient through communication. The diagram below explains how to use OARS[74]:

                                                         Figure 13. OARS strategy of communication
OARS picture.jpg

Benefits of Physical Activity

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

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[75]. These changes however, have introduced other new issues to tackle in the coming years.

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[76]. Excess weight can induce joint bleeds due to added stresses placed on the joints [77]. Effects of obesity and overweight can also exacerbate existing arthropathies and influence development of cardiovascular disease[78].

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[79]. 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[80]. Buzzard and Beeton[81] 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[82]. However, Philpott et al.[47] 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.[83] 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[84]. 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[85];[86].

There is lacking evidence available to claim that there is a true correlation between reduced exercise levels in AWH and increased weight or obesity and further study must be carried to clarify these findings. However, this information confirms the need for lifestyle modifications in this population, including dietary changes and promotion of physical activity. 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 [87]. However, the physical activity recommendations remain limited[88];[89], with findings from recent studies demonstrating controversial outcomes.

Evidence Table

The table below describes some articles surrounding the evidence base for obesity in PWH.

Table 6. Evidence surrounding obesity in AWH and limitations of each

Author/ Title Purpose/Aims Details/Protocol Measures Conclusions Limitations
Hofstede et al.[90]
Obesity: a new disaster for haemophiliac patients? A national survey
To determine the prevalence of overweight and obesity in haemophilia patients in the Netherlands, assessed over a decade. A nationwide postal survey among all haemophilia patients in the Netherlands was conducted in 2001, following four previous surveys in 1972, 1978, 1985 and 1992. Data on length, weight, age and severity of haemophilia were obtained. The haemophilic boys where restricted to those aged 4–16 years. None. Prevalence of obesity tripled between 1992 and 2001. The aetiology is similar to that in the general population as can be attributed to a more sedentary lifestyle and poor diet habits. Boys with haemophilia are particularly at risk of becoming overweight as parents may be protective over their child and limit physical activities aiming to prevent bleeds. Physical activity should be promoted in all these patients. Study was conducted in the Netherlands reduces transferability.
Majumdar et al.[91]
Alarmingly high prevalence of obesity in haemophilia in the state of Mississippi
To estimate the prevalence of overweight and obesity in children and adults diagnosed with haemophilia in Mississippi and to assess whether race/ethnicity and the severity of haemophilia are important risk factors. A chart review was performed for all haemophilic patients seen within the last 2 years at the Mississippi Haemophilia Treatment Centre. 132 haemophilic patients. Overall, 51% of the haemophilic patients were considered either obese or overweight. Height and weight records were obtained and the body mass index (BMI) was calculated. None. There is a significantly higher prevalence of overweight or obese in those age >20 years. However, examination of subgroups within the 2–19.9 age range demonstrated a higher prevalence of obesity in haemophiliacs aged 11–19.9 years compared to younger patients. This suggests a trend; haemophilic patients will gain weight during the adolescent period. Race/ethnicity and severity of haemophilia were not significant risk factors for overweight and obesity. Cross-sectional observational study, therefore only offers a snapshot of results. The study was conducted in Mississippi in America therefore reducing transferability to UK populations.
Gonzales et al.[92]
Comparison of physical activity and sedentary behaviours between young haemophilia A patients and healthy adolescents.
To determine the amount and intensity of habitual PA among haemophilia A and healthy adolescents, and in haemophilia A patients with and without bleeding episodes in the previous year.
To identify the type and determine the time spent in sedentary activities in which both groups participate to obtain a broadened view of their daily activities.
A cross-sectional study of 41 adolescent patients with haemophilia A and 25 healthy adolescents, aged 8–18 years. Participants with haemophilia A met the following clinical criteria: attended the hospital during the previous year, had all of the habitual clinical assessments made and had not experienced any bleeding during the 2 months prior to the assessments. Healthy participants were selected from secondary schools in the same region. The PA of participants was measured using an accelerometer. Adolescent Sedentary Activity Questionnaire Adolescent haemophilia A patients deomonstrated a higher daily mean time participating in PA compared to healthy participants. Findings suggest that increased participation in moderate intensity PA along with reduced sedentary time should be recommended among adolescents with haemophilia A. Current findings show that the PA in which haemophilic youth were engaged, were nit sufficinet to benefit physiological from. An increase in time spent on moderate physical activities should be encouraged. Additonally, patients should be cautioned to limit the amount of time spent engaging in sedentary behaviours. One of the main limitations of the study is that the accelerometer cannot be worn in water. Also, the characteristics of the healthy counterpart group could have been more carefully selected, as they belonged to the same school. This may be the reason why possible differences between the two groups regarding the use of transportation could not be addressed.
Douma-van Riet et al.[93]
Physical fitness in children with haemophilia and the effect of overweight.
To assess physical fitness (aerobic capacity), muscle strength, join t status.
To determine the prevalence of overweight.
To study whether overweight is associated with physical fitness, motor competence and quality of life (QoL).
Patients treated at five Centres in the Netherlands were invited to participate in this study. All boys aged 8–18 diagnosed with haemophilia A. BMI was calculated. The children performed a maximal aerobic exercise test and were encouraged to cycle to exhaustion. Other areas used 6-min walk test (6MWT). Quality of life was measured using Haemo-QoL Index. Muscle strength, motor competence and joint mobility were assessed. 6MWT The results from this multi-centre study demonstrated Dutch children with haemophilia to be as physically fit as their healthy peers; overall aerobic capacity and muscle strength are comparable to healthy children. The prevalence of overweight is only slightly increased compared to the general population. Although the 6MWT is a standardised test, the three centres which used this measure walking courses used were not identical. Also in one of the centres, where the highest z-scores of walking distance were found, children received extra encouragement.
Therefore, some of the results on the 6MWT should be interpreted with caution.

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[94].

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[95]. A study conducted in 2008, by Tlacuilo-Parra et al.[96] 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

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[97]. In the early years the childs parent or guardian will be heavily involved in managing their care, therefore they may also be affected[98]. The International Classification of Functioning, Disability and Health (ICF) is a framework which considers the impact of a health condition on all aspects of life. This can be useful in identifying pshycosocial or physical barriers to physical activity.  

                                                                                        Figure 14. ICF model

Icf1.jpg


Recent evidence discussed throughout this wiki resource supports exercise, allowing AWH to participate in peer activities which they would have previously been excluded from[99].

The World Federation of Haemophilia (WFH) separates a number of different psychosocial issues into specific age groups[100]:

  • 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[101]. Some of the psychosocial issues that are common in adolescence are discussed below.

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.

Compliance

Cassis[102] 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[103]

Cassis et al.[104] 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[105] 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.[106] 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[107] and participation in physical activity among PWH has not been well studied[108].

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