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.