SWAYAM A Physio Volunteers Group

IMPORTANCE OF PHYSIOTHERAPY IN HAEMOPHILIA Dr. Maulik Patel (B.P.T.,MIAP)

What is Haemophilia ? An incurable hereditary bleeding disorder due to absence or deficiency of clotting factors in the blood. Repeated spontaneous bleeds inside the joints and muscles of the patient, if not treated promptly lead to permanent disability.

How do clotting factors work ? Clotting factors in blood - Work one after the other At the end of the chain, bleeding stops. If one clotting factor is missing or does not work, clots will not form properly and bleeding will continue.

Types Haemophilia A most common type caused by lack of clotting factor VIII. Haemophilia B caused by lack of clotting factor IX. Haemophilia C caused by lack of clotting factor XI.

Severity Mild :- Level is 5-50%. Only occasional bleeding usually related to significant trauma or surgery. Moderate :- Level is 1-5%. Spontaneous bleeding is uncommon but occurs after minor trauma or surgery. Severe :- Level is < 1%. Spontaneous joint and soft tissue bleeding several time several month.

Shoulder Elbow Hip Knee Ankle Arm Thigh Calf Neck Head Thigh Bleeding Sites

Common Bleeding Sites Joints Knee Ankle Elbow Muscles Iliopsoas Fore-arm muscles Calf Thigh muscles

Other sites of bleeding Haematuria Gastro-intestinal bleeding - rare Intracranial bleeding - death

Which bleeds are serious or life-threatening?

Is haemophilia lifelong? Yes The level of clotting factor in his blood usually stays the same throughout his life.

How can bleeds be treated with first aid? REST ICE COMPRESSION ELEVATION

How is Haemophilia treated? Replacing the missing clotting factor in the blood Whole blood Plasma Cryoprecipitate Factor concentrates

Why physiotherapy is important?

Maintenance of healthy joints and muscles crucial to the quality of life of a PWH. Clotting factor replacement may stop or even prevent bleeds but does not restore joint or muscle function. Only regular movements and exercise can do that

Aims of Physiotherapy Diagnosis and treatment of the musculoskeletal system; Advise and education regarding physical activities; Collaboration with other team members;

Common Conditions in Relation to Physiotherapy Muscle bleed Ilio-psoas bleed Joint bleed Synovitis Arthropathy

MUSCLE BLEEDS

Muscle Bleed Can occur in the groups of thigh, shoulder, upper arm, forearm, and calf. Swelling or pain usually accompanies bleeding.

Signs and Symptoms Early Tingling sensation Feeling of warmth Late Difficulty in movement Pain with movement Tightness or swelling, possibly even when muscle is at rest Numbness or tingling feeling (may be described as feeling "asleep") Area is warm to touch

What are the long-term effects of muscle bleeds? After repeated bleeds, muscles can become weak, scarred, and shorter than normal (sometimes permanently). Joints above and below the muscle cannot move properly.

If nerves are damaged during muscle bleeds, the muscle may become weak or even paralysed. Permanent damage to joints, muscles, and nerves Affects the way a person sits, stands, and walks.

Ilio-Psoas Bleed Patient adopts a poor posture Hip & knee flexed Parasthesia – front of the thigh

In acute stage Rest Ice Elevation Factor replacement

In subacute stage antagonist muscles, i.e. abdominals and greater gluteal muscles Gradually extend the affected hip as pain and sensory changes allow

Once the patient can lie supine with the affected leg resting almost flat on the bed, or prone with assistance of only one pillow, it is time to stretch the Iliopsoas muscle further into its length.

As pain subsides Active assisted exercises of the iliopsoas, antagonist muscles (gluteals and hamstrings) In non-weight bearing position

In the recovery stage start exercising in the standing position weight transfers in various stance positions, strengthening the lower limbs

JOINT BLEEDS

What causes a joint bleed? If the capillaries in the synovium are injured, they bleed. Often there is no clear reason for the bleed, especially in severe hemophilia. In a person who does not have hemophilia, the clotting system stops the bleeding quickly. In hemophilia, the bleeding continues causing the joint to swell and become painful.

Which joint bleeds are most common? Common joints involved Knees Ankles Elbows Joints of the hands are not usually affected except after injury.

Signs and Symptoms Early An "aura" or feeling that something is wrong with the joint A feeling of bubbling, tingling or warmth in the joint Late Stiffness of joint Pain Swelling Inability or refusal of patient to straighten joint or to bear weight Limited movement

What are the long-term effects of joint bleeds? Repeated bleeding into a joint causes the synovium (lining) to swell and bleed very easily. Some blood remains in the joint after each bleed. The synovium stops producing synovial fluid.

Accumulation of blood Irritates synovial membrane Synovial lining swells Develop more blood vessels More Bleeding Synovitis

Hemophilic Arthritis This damages the smooth cartilage that covers the ends of the bones. Joint becomes stiff, painful to move, and unstable. muscles around the joint weaken. With time, most of the cartilage breaks down and some bone wears away. Sometimes the joint cannot move at all. The whole process is called hemophilic arthritis.

Symptoms of arthritis Pain Stiffness Decreased flexibility Feeling that the limb is "frozen" in place Gradual muscle weakening

Arthropathic knee joint Flexion contracture Dorsal subluxation of tibia Valgus deformity Outward rotation of tibia Patella subluxated laterally

Physiotherapy Traction Intermittently – manually Continue – through weight Mobilization Ventral sifting of tibia Patella mobilization

Muscle strengthening Weakness in extensor muscles Quadriceps, hamstrings, hip abductors

Stretching Only active stretching Hamstrings, femoral head of quadriceps calf

Electrotherapy To relieve pain Promote tissue repair Assist in the restoration of function Modalities used Ultrasound TENS Pulse SWD

Ultrasound therapy Useful at a later stage in the rehabilitation programme for muscle haematomas When there is clear evidence that acute bleeding has stopped.

Pulsed short-wave diathermy (PSWD) This is often the treatment of choice for early bleeding episodes. PSWD machines do not emit a continuous beam of energy and therefore no heat is transmitted to the affected area.