13 Physiotherapy
1 Introduction
The role of the physiotherapist in the treatment of people with hemophilia (PWH) has changed over the years because of the improvement of the medical treatment, from historically focusing on passive rehabilitation after acute events and limiting the damage of bleeds, to active rehabilitation, prevention, education and maximizing movement potential [1–3].
Despite improvements in treatment, there is still a need for physiotherapy for PWH to manage musculoskeletal (MSK) issues and help optimize function and participation. It is essential to monitor the MSK status of PWH, also related to new treatments opportunities [4,5].
Physiotherapists at the hemophilia treatment center (HTC) have an expert role in supporting primary care physiotherapists in treatment regimens for PWH [6], which may be particularly needed in the Nordic countries due to the geography of the Nordic region, with centralized hemophilia care, and many patients living far from treatment centres.
Telehealth and virtual consultation could be a complement to in-person visits to the hemophilia treatment center for PWH living far away. PWH can thus get instructions for exercise, follow-up and support without travelling [7–10].
Physiotherapy for PWH can be divided in to three categories: Prevention, assessment and treatment/rehabilitation [11].
1.1 RECOMMENDATION
- Physiotherapy should be provided to all PWH to improve function and optimize movement potential and should include monitoring of musculoskeletal status.
1.2 RECOMMENDATION
- Physiotherapists at the HTC should support primary care physiotherapists treating PWH.
2 Prevention
People with more severe forms of hemophilia will receive prophylaxis with coagulation factor concentrates or novel treatment products from an early age, and can and should be physically active to the same extent as children without hemophilia, resulting in normal physical strength and mobility [12].
Low physical activity can result in impaired bone mineralization and reduced bone mineral density in children with hemophilia compared to those without the condition [13].
Moderate intensity of aerobic walking exercise improves bone metabolism and hand grip strength in adult persons with moderate hemophilia A [14].
Good function of muscles around the joints has been shown to prevent joint- and muscle bleeds. It is therefore essential to train muscle strength, endurance, and coordination from an early age [15].
Not only PWH, but everybody (both adults and children) should be physically active at moderate to vigorous intensity for 30–60 min every day [16]. The Nordic recommendations for physical activity are the same as those of the World Health Organization (WHO).
PWH experience the same benefits of exercise as the general population, being physically healthier than if sedentary and enjoying a higher quality of life (QoL) through social inclusion and higher self-esteem [17,18].
PWH can also gain physical benefit from increased muscle strength, joint health, balance and flexibility achieved through physiotherapy, physical activity, exercise and sport [18,19].
For PWH, physical activity may also help reduce joint and muscle bleeds via improvements in proprioception, balance, muscle strength, joint mobility, stability, and function [20,21].
Recent summarized research indicates increased physical activity in PWH along with improved medical treatment [22].
The physiotherapist has an important role in informing and supporting PWH and their families about physical activity and sports that are appropriate for PWH [23]. The choice of types of physical activities should reflect the PWH’s preferences/interests, bleeding phenotype, physical condition and ability, local contexts, and available resources (including treatment intensity) [18,24,25].
The physiotherapist can also educate parents how to examine the joint mobility of the youngest children for early detection of joint bleeding.
2.1 RECOMMENDATION
- PWH should be regularly physically active as they can experience the same benefits of physical activity and exercise as the general population.
2.2 RECOMMENDATION
- Physical activity may help to reduce muscle and joint bleeds.
2.3 RECOMMENDATION
- PWH should consult a physiotherapist or other musculoskeletal specialist before engaging in sports and physical activities to discuss their appropriateness specific to the individual’s condition and their requirement for physical skills and/or protective gear.
3 Assessment
Assessment instruments that are disease specific for hemophilia have been developed over the past 10 years [26].
The physiotherapist should assess joint- and muscle function regularly, e.g. during the annual control at the HTC. This includes joint mobility, muscle strength, pain, joint and muscle contractures, axial changes in the joints, balance, and gait function. Ideally, standardized outcome measures should be used.
In the case of a suspected bleed, a physiotherapist can help with differential diagnosis between joint bleeding and synovitis together with the physician.
Ultrasound can aid the assessment for a correct diagnosis [27]. When ultrasound imaging is performed and scored by physiotherapists using Hemophilia Early Arthropathy Detection (HEAD-US) [28] there is a good overall repeatability of the protocol and this complements the physical examination when screening and monitoring joint health of PWH [29]. HEAD-US also correlate well with Hemophilia Joint Health Score (HJHS) in persons with hemophilia [30].
The Hemophilia Joint Health Score (HJHS) was initially developed for children from 4 to 16 years of age [31]. It is currently validated and reliability tested for both children and adults (ages 4-18 [31,32], ages 14-30 [33], ages 18-82 years [34]).
It is now frequently used for the evaluation of joints in children and young adults, as well as for older populations [31,33]. For adults and elderly patients, the HJHS needs to be complemented with assessment of possible age-related conditions for example problems with the hip and shoulder joints.
Several other evaluation instruments may also be relevant to use is the functional assessment of PWH.
The visual analog scale (VAS) can be used to rate the pain experience in daily activities or at acute trauma/bleeding [35].
The Hemophilia Activities List (HAL) can be used to get the patient’s own perception of their ability in terms of activity (a person carrying out a task or action) and participation (a person’s involvement in a life situation) [36].
Furthermore, the Functional Independence Score in Haemophilia (FISH), which is a performance‐based tool, can be used to assess an individual’s functional ability. Eight activities of daily living are assessed: eating, grooming, dressing, chair transfer, squatting, walking, step climbing, and running [37].
Finally, the generic self-administered questionnaire, Health Assessment Questionnaire Disability Index (HAQ-DI) could be used as a self-reported functional status when FISH or HAL is not useful [38].
Based on clinical examination and other assessments, the physiotherapist can recommend relevant steps that can benefit PWH, such as rehabilitation, contact to an occupational therapist, and assistive devices for activities of daily living (ADL).
3.1 RECOMMENDATION
- Musculoskeletal status in PWH should be monitored on a regular basis, and at least annually.
3.2 RECOMMENDATION
- MSK monitoring should be carried out by a physiotherapist with knowledge and experience in hemophilia.
3.3 RECOMMENDATION
- MSK assessment should include standardized outcome measures/protocols/tools.
4 Intervention (treatment/rehabilitation)
Muscle- and joint bleeds cause pain and swelling, and usually reduced range of movement and function.
Repeated joint bleeds cause synovitis, cartilage damage and skeletal bone irregularities and osteophytes, also known as hemophilic arthropathy.
The purpose of rehabilitation after an acute bleeding in the joint or muscle, or in hemophilic arthropathy, is to limit damage, reduce pain and restore or improve function.
Active exercise under the guidance of a physiotherapist in combination with intensive treatment with factor concentrate/novel treatment products can improve function. The results are better the sooner physiotherapy begins [39].
In the acute phase the early management can be summarized as “PRICE”, meaning Protection and joint Rest, relive acute pain with Ice and prevent and treat swelling with Compression and Elevation [40].
After the acute phase, the physiotherapist can plan and help execute an exercise program to speed recovery and restore lost function. Several studies show that mobility and strength exercise leads to faster normalization of the function and also significantly reduces the risk of permanent disability [15,41].
Treatment may include different types of mobility exercises (active, active unloaded, passive), posture instructions, careful manual therapy modalities for increased mobility and pain relief purposes, strength and endurance exercise, coordination training, etc.
Aquatic exercise can be used for different types of intervention such as mobility, aerobic capacity and strength [42].
Exercise in warm basin can also be useful as pain relief, as well as the use of TENS, heat and cool pack [23,40,43].
There is, however, limited evidence for the use of physiotherapy intervention for pain relief in PWH [44]. There is limited high-quality evidence for exercise in persons with hemophilia due to a small numbers of randomized controlled trials, but no adverse effects are reported in the different exercise intervention studies published [45–49].
Patients undergoing orthopedic surgery, for example synovectomy or different types of joint replacement should receive physiotherapy exercise both before and after surgery [2,50].
Before surgery it is important to train muscle strength around the joints and maintain the mobility that exists. After surgery the patient trains their mobility and strength according to the relevant protocol at the orthopedic clinic for the current operation. If the hemophilic arthropathy has caused malalignment, stiffness and pain, the physiotherapist may prescribe or recommend orthotics and orthopedic shoes together with the attending orthopedic surgeon depending on the rules in different countries [51]. The physiotherapist also tests out walking aids and recommend other appliance needed in daily life.
4.1 RECOMMENDATION
- A suspected bleed should immediately be treated according to PRICE.
4.2 RECOMMENDATION
- Mobility and strength exercise should be provided after muscle- and joint bleeds, as this leads to faster normalization of the function and significantly reduces the risk of permanent disability.
4.3 RECOMMENDATION
- PWH undergoing orthopedic surgery, for example synovectomy or different types of joint replacement, should receive physiotherapy, including adapted exercise both before and after surgery.