12 Physiotherapy

Revision by: Elisabeth Brodin (Göteborg) and Ruth Elise Dybvik Matlary (Oslo)

Reviewed by: Karin Juel Hansen (Copenhagen)

Recommendations

Summary of physiotherapy work at treatment centers in the Nordic countries:

  • Informs about the joints and muscles function to parents, teenagers and adults.

  • Assess physical activity, joint mobility, muscle strength and function.

  • Proposes appropriate recreational and sporting activities.

  • Tests out and supervises practice of utilizing assistive devices.

  • Designs exercise programs after a bleeding event.

  • Prescribes and supervises exercise to increase mobility and muscle strength.

  • Prescribes and supervises exercise before and after orthopedic surgery.

  • Provides treatments for pain relief.

  • Is a resource for colleagues outside the hemophilia treatment center/for physiotherapists in primary care.

  • Provides telehealth/virtual consultations for PWH as a complement to visits at the hemophilia treatment center.

12.1 Introduction

The role of the physiotherapist in the treatment of the hemophilia patients has changed over the years because of the improvement of the medical treatment [6]. Despite improvements in treatment, there is still a need for physiotherapy for PWH to manage musculoskeletal issues and help optimize function and participation. It is also essential to monitor the musculoskeletal status of PWH related to new treatments opportunities [146,147].

The physiotherapist at the hemophilia treatment center has an expert role in supporting primary care physiotherapists in treatment regimes for PWH [148].

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 ups and support without travelling [149152].

Physiotherapy for PWH is divided in to three categories: Prevention, assessment and treatment/rehabilitation [153].

12.2 Prevention

Patients will at an early age receive prophylaxis with coagulation factor concentrates and can be physically active to the same extent as non-hemophiliac children resulting in normal physical strength and mobility [154]. Low physical activity can result in impaired bone mineralization and reduced bone mineral density in children with hemophilia compared with healthy [155]. Moderate intensity of aerobic walking exercise improves bone metabolism and hand grip strength in adult persons with moderate hemophilia A [156]. 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 at an early age [157]. Not only patients with hemophilia, but everybody (both adults and children) should be physically active for 30–60 min every day. The physiotherapist has an important role in informing and supporting PWH and their families about physical activity and sports that are appropriate for PWH [158].

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 [159,160]. PWH can also gain physical benefit from increased muscle strength, joint health, balance and flexibility achieved through physiotherapy, physical activity, exercise and sport [160,161]. Recent summarized research indicates increased physical activity in PWH along with improved medical treatment [162]. The physiotherapist can also educate parents how to examine the joint mobility of the youngest children for early detection of joint bleeding.

12.3 Assessment

Assessment instruments that are disease specific for PWHs have been developed over the past 10 years [163]. The physiotherapist will assess the joint and muscle function during the annual control at the treatment center. This includes joint mobility, muscle strength, pain, joint and muscle contractures, axial changes in the joints, balance and gait functions.

In acute bleeding a physiotherapist can help with differential diagnosis between joint and muscle bleeding and synovitis together with the physician. Ultrasound can complete the assessment for a correct diagnose [164]. When US imaging performed and scoring by physiotherapists using Hemophilia Early Arthropathy Detection (HEAD-US) [165] there is a good overall repeatability of the protocol and this complements the physical examination when screening and monitoring joint health of people with hemophilia [166]. HEAD-US also correlate well with Hemophilia Joint Health Score (HJHS) in persons with moderate hemophilia [167].

The Hemophilia Joint Health Score (HJHS) has been developed for children from 4 to 16 years of age [168]. It is validated and reliability tested up to the age of 30 ([45,168] (ages 4-18), [169] (ages 14-30)). It is used for the evaluation of joints in children and young adults, as well as for older populations [168,169]. 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.

Other evaluation instruments that may be present are visual analog scale (VAS) to rate the pain experience in daily activities or at acute trauma/bleeding [170]. 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) [171].

Based on our examination we can recommend relevant steps that can benefit PWH such as contact to occupational therapist, when the patients need assisted devices at home for the ADL (activities of daily living). A disease-specific ADL status is developed in India [172], but is not used in the Nordic Countries at the moment due to cultural differences between the countries that means the manual is not suitable for the Nordic conditions. Functional Independence Score in Haemophilia (FISH), is a performance‐based tool 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 [172]. 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 [173].

12.4 Intervention (treatment/rehabilitation)

The purpose of rehabilitation of hemophilia arthropathy and after an acute bleeding in the joint or muscle is to reduce pain, restore joint mobility and muscle strength. Repeated bleedings in a joint leads to cartilage damage and give a hemophilia-related joint disease (hemophilic arthropathy). Active exercise under the guidance of a physiotherapist in combination with intensive treatment with factor concentrate can break the vicious circle. The results are better the sooner physiotherapy begins [174].

In the acute phase the early management can summarize as PRICE meaning Protection and joint Rest, relive acute pain with Ice and prevent and treat swelling with Compression and Elevation [175]. The physiotherapist plans an exercise program to 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 [157,176].

Treatment may include different types of mobility exercises (active, active unloaded, passive), posture instructions, careful manual extractions 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 [177]. Exercise in warm basin can also be useful as pain relief, as well as the use of TENS, heat and cool pack [158,175,178]. There is however limited evidence for the use of physiotherapy intervention for pain relief in PWH [179]. Physiotherapy treatment techniques used in routine clinical practice can be used if the person with hemophilia has appropriate treatment with clotting factor [175]. There is a lack of confidence in the evidence for exercise in persons with hemophilia due to small numbers of randomized controlled trials, but no adverse effects are reported in the different exercise intervention studies published [180182]. Recommended frequency is 3 times per week to reach desired results [158].

Patients undergoing orthopedic surgery, for example synovectomy or different types of joint replacement receive physiotherapy exercise both before and after surgery [183]. 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 actual programs/protocol at the orthopedic clinic for the current operation. If the hemophilia-related arthritis 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 [184]. The physiotherapist also tests out walking aids and recommend other appliance needed in daily life.