Physiotherapy and Hemophilia

What Is Hemophilia?

Hemophilia is a rare blood disorder. It is a hereditary condition that impacts the body's capacity to form blood clots, which is required to stop bleeding. It is an inherited bleeding illness caused by a deficiency or absence of blood clotting factor VIII or IX. Hemophilia is divided into two types: hemophilia A, which is caused by a lack of clotting factor VIII, and hemophilia B, which is caused by a lack of clotting factor IX. Hemophilia patients frequently experience joint bleeding, followed by muscle bleeding and brain hemorrhage. Hemophilia is estimated to affect roughly 1 in every 10,000 births, with the severe form of the condition accounting for about 6 % of the population.

What Are the Complications of Hemophilia?

The complications of hemophilia include:

• Joint contracture and deformities, especially at the hip, knee, elbow, and ankle joints.

• Hemophiliac arthropathy is a condition where the articular cartilage softens, turns brown due to hemosiderin, and becomes pitted and fragmented.

• Muscle weakness and atrophy around affected joints.

• Peripheral nerve compression by hematoma.

• Postural scoliosis.

• Decreased aerobic fitness.

What Is the Role of Physiotherapy in Managing Patients With Hemophilia?

The ability to move is critical to one's health and well-being, and it is at the heart of what it means to be healthy. Individuals' movement needs and potential are addressed by physiotherapy, which provides rehabilitation and treatments to build, maintain, and restore maximum movement and functional capacity while also preventing musculoskeletal disability. Within the domains of promotion, prevention, treatment and intervention, and rehabilitation, physiotherapists attempt to identify and maximize the quality of life and movement potential. The availability of coagulation factors with a longer plasma half-life that allows for longer intervals between treatments, therapeutic products based on processes other than the replacement of the defective factor, and gene therapy have all improved hemophilia treatment choices in the previous decade. The shifting role of physiotherapy in the treatment of hemophilia in both children and adults has been widely documented. To prevent musculoskeletal bleeding, physiotherapy intervention should be considerably more proactive in terms of optimizing biomechanics.

Physiotherapists are likely to focus on the following as part of a multidisciplinary team:

• Differential diagnosis of musculoskeletal bleeding.

• Supporting and encouraging higher levels of physical activity.

• Rehabilitation to maximize physical potential and capabilities with increased expectations of what is achievable.

• Assessment and treatment of non-bleed-related musculoskeletal issues.

• Managing comorbidities and falls risk.

• Improving the longitudinal surveillance of musculoskeletal health.

What Are the Benefits of Doing Physical Activity for Patients With Hemophilia?

Physical activity improves the efficiency of therapy and prevents bleeding episodes in hemophilia patients in a number of trials. A lack of physical activity in older adults can raise the risk of diabetes, high blood pressure, high blood fat levels, obesity, osteoporosis, and related fractures, as well as hemophilia-related problems.

Patients with hemophilia have higher health concerns connected with physical inactivity than the general population. Obesity, for example, has been related to an increased risk of cardiovascular disease and chronic joint inflammation, both of which exacerbate joint bleeding and fracture risk. Falling injuries can also be caused by muscle and bone diseases induced by hemophilic joint inflammation and age. In general, a strong physique aids in the protection of a person against bleeding. Furthermore, physical activity has mental and spiritual benefits in addition to physical ones.

The main aims of exercise that are considered to be important for people with hemophilia are:

• Flexible and strong muscles support the joints, which help and eventually prevent bleeds and joint damage.

• Improved balance and coordination help joints and muscles work better together, which again helps protect against bleeds.

• Sweating is an important phenomenon, and it is a great outlet to help relieve stress and relax the mind.

• Being active boosts self-esteem and confidence and is fun for children and adults, as long as you do something you like.

• Having high energy and feeling fit helps to reduce fatigue.

• Maintaining a healthy weight reduces the stress on the joints, which is particularly important in aged people.

• Exercising regularly can promote social acceptance and helps people to socially interact.

• Promote normal neuromuscular development.

• Preserve or restore joint range of motion (ROM) and muscular flexibility.

• Increase muscular strength and endurance.

• Maintain healthy body weight and decrease if needed as it decreases stress on joints.

• Promote the maintenance and development of correct bone density.

• Improves and develops aerobic conditioning.

• Improve participation and function, which eventually improves the quality of life.

• Decreases health risks associated with a sedentary and improper lifestyle.

What Activities Are Recommended for Patients With Hemophilia?

The following activities are recommended for patients with hemophilia:

• Swimming.

• Fishing.

• Dancing.

• Badminton.

• Sailing.

• Golf.

• Bowling.

• Table Tennis.

• Walking.

• Cycling.

What Activities Should Be Avoided by Patients With Hemophilia?

The following activities should be avoided by patients with hemophilia:

• Boxing.

• Karate.

• Wrestling.

• Motorcycling.

• Judo.

• Hang-gliding.

• Hockey.

• Rugby.

• Soccer.

• Football.

• Skateboarding.

What Are the Goals of Physiotherapy in Hemophilic Patients?

Physical therapy for a child with hemophilia is aimed at maintaining ROM (range of movement) and strength in all joints and at preventing or diminishing disability.

Specific goals include:

1) PRICE Protocol in Case of Acute Bleeding:

• PRICE (Protection, Rest, Ice, Compression, Elevation) is a procedure many health care professionals and doctors recommend for joint bleeds. It reduces swelling and tissue damage when used together with clotting factor concentrates.

2) Prevention of Contractures:

• Manual traction and mobilization.

• Active ROM exercises and passive ROM are generally contraindicated.

• Progressive or dynamic splinting.

• Serial casting or drop-out casts.

3) Maintaining Strength:

• Initially, isometric strengthening exercises then graded progressive exercises.

• Exercise in a pool, hot soak in a tub. It is easier and more comfortable to move the body in water and is also good for aerobic conditioning.

4) Preventing or Diminishing Disability:

• Gait training.

• Proprioceptive training.

• Bracing or splints provides stabilization and protection.

Thermotherapy is contraindicated in hemophilic patients as heat causes dilatation of blood vessels and increases blood flow.

Conclusion:

Hemophilia is treated in a holistic manner. Educating and creating awareness among the patient and his family about the condition, clotting factor replacement, comprehensive care, controlling bleeding, and maintaining general health can be made easier with evidence-based therapeutic exercise and good nutrition. Otherwise, life will be at risk. The focus should be on primary bleeding prevention as well as acute bleeding therapy. Physiotherapy is regarded as a way to improve quality of life, speed up recovery, reduce the frequency of bleeding, and avoid joint degeneration from multiple bleeds.