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Role of Physiotherapy in the
management of the child with
Haemophilia
Robyn Smith
Department of
Physiotherapy
UFS
2011
What is Haemophilia?
 Lifelong
 Rare
 Inherited
 Bleeding disorder
 Caused by a defect in the genes that are
required for blood clotting factors VIII and IX
 Characterised by severe, spontaneous bleeding
 Results in chronic and painful joint deformities
What is Haemophilia?
A person with haemophilia has a problem
with certain proteins in the blood called
clotting factors, where the level is to low or
clotting factor is absent
This results in the blood taking a long time
to clot after injury
Genetic basis for haemophilia
 Female carriers
 Only sons are
sufferers
Types of haemophilia
There are 2 main types of haemophilia:
Haemophilia A: Clotting factor VIII is low or
missing. Occurs in about 9/10 cases.
Haemophilia B: Clotting factor IX is low or
missing.
Classes of haemophilia
Haemophilia can be:
Mild
Moderate
Severe (most type A’s are severe)
Prevalence of Haemophilia
Haemophilia A: 1/10 000 males
Haemophilia B: 1/50 000 males
Typical signs and symptoms
Bleeding
Bruising
Internal bleeding is common in cases of
severe haemophilia. If not treated
promptly, internal bleeding can lead to
damaged joints, muscles, or other parts of
the body
Typical signs and symptoms
Bleeding in the joints is the most common
problem (80% of all bleeds are joint bleeds)
in severe haemophilia. Bleeding may often
occur without any known injury. The
bleeding can go on for days if not treated
promptly .Early treatment is imperative in
limiting joint damage.
The most common joint affected:
Knees
Elbows
Ankles
Typical signs and symptoms of joint
bleeds:
 Stiffness joint with no real pain
 Pain
 The joint becomes swollen and warm
 Swelling continues as bleeding continues, and
all movement in the joint is lost. Pain can be
severe.
 The bleeding slows after several days when the
joint is full of blood.
 If not treated, the bleeding can lead to disabling
arthritis in the joints.
Typical signs and symptoms of brain
bleeds:
Bleeding in the brain is a serious complication and requires
immediate medical management. This may be caused by a
simple bump to the head or a more severe injury
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Typical signs and symptoms are:
Long-lasting painful headaches
Vomiting
Changes in behaviour
Drowsiness
Sudden weakness or clumsiness of the arm or leg
Neck pain or stiffness
Double vision
Difficulty walking
Convulsions or seizures
Medical Management
There have been significant advances in the
management of Haemophilia over the last 30
years.
The main treatment for haemophilia is
replacement therapy where the clotting factor
that is too low or missing is replaced. Clotting
factor VIII or IX are infused, or injected, directly
into the bloodstream.
Medical management
The treatment approach is determined by the severity of
the haemophilia:
 Mild haemophilia. Replacement therapy is usually not
indicated
 Moderate haemophilia. Replacement therapy is indicated
when bleeding occurs.
 Severe haemophilia. Prophylactic replacement therapy
over the long term as a preventive measure.
When bleeding occurs, it is important to get treatment as
soon as possible. Delayed treatment can lead to
complications.
Interdisciplinary management
The physiotherapist forms an integral part
of the team managing the child with
Haemophilia
Aids the child in improving musculoskeletal function
Physiotherapy management
Subjective interview
Subjective interview
Attain information about patients
haemophilia status, treatment and
previous bleeds’
History regarding current problem/bleed
and associated problems.
Determine the severity and irritability of
problem
Identify possible precautions or contraindications for physiotherapy
Physical examination
Functional abilities and ADL
Observe how the child performs functional
tasks e.g. walking, stair climbing, standing,
sitting, hand function
Posture
Observe for swelling, temperature
elevation over the affected joint
Joint mobility
Active ROM of the affected joint due to
damage of the synovial membrane and
hyaline cartilage due to the increased
pressure in a confined space
Followed by passive ROM of the affected
joint
Be alert to identify the child’s typical
symptoms (comparable sign) and pain
Muscular strength
Joint bleeds may lead to myofibril damage. increased
pain further impair muscle action.
 Isometric contraction
 Isotonic contraction (in accordance with the Oxford
scale).
Muscle weakness may be caused by pain inhibition or
disuse.
Also observe for any obvious muscle atrophy around the
affected joint.
Muscle lengths
Joint bleeds resulting in pain may result in the
child holding the joint in flexion as a protective
measure. With repeated joint bleeds muscle
may not regain their length. This results in
further problems as the joints are later not
sufficiently supported by the muscles.
 This may result in shortening of the muscles
across the joint.
 Relevant muscle lengths needs to be assessed
Neurological system
 Peripheral neuropathies can be associated with
joint bleeds.
 Brain bleeds may also result in neurological
dysfunction
 In cases where neurological involvement is
suspected a full neurological involvement is
required including
 Sensation
 Muscle strength
 Reflexes
 Neural tension tests
 Balance, proprioception and co-ordination
Other tests
May be necessary with knee bleeds to test
for:
Laxity of the knee ligaments secondary to
the bleed
Leg length discrepancy caused by
hyperaemia at the epiphysial plates with
resultant bone overgrowth
Observation & Palpation
Swelling
Increased temperature
Tenderness
Physiotherapy treatment
Management of an acute
bleed
Joint damage
Muscle inhibition
Muscle wasting
Muscle damage
Pain
 Immobilisation of the joint by means of splint. Movement
of the joint shortly after a bleed will further force blood into
the smooth cartilage, damage muscle
 Initially the splint is only to be removed for icing of the
joint.
 Icing is very important for its analgesic effects and to
maximise vasoconstriction, reducing the flow of blood to
the effected area.
 Measure circumference of joint prior to and after icing to
gauge effect of icing.
 Ice joint 5 min on and then 10 minutes off until ice melts.
Repeat every 2 hours in case of severe bleed.
Rehabilitation during the sub-acute and
chronic stages following a bleed:
After controlling an acute bleed rehabilitation
can be commenced
The rate at which rehabilitation can be
progressed depends on:
 Severity of bleed
 Duration of the immobilisation
 Previously damaged to the joint will make it less
likely that function will return to the pre-b;eed
state
Rehabilitation during the sub-acute and
chronic stages following a bleed:
 Rest the joint/muscle as straight or lengthened
as possible. A resting splint is often beneficial
here.
 Gentle stretching of the muscles functioning
across the joint. 5 -10 repetitions of 10s
 Static muscle contractions as soon as pain
allows:
 5-10 contractions 2x/day
 As soon as pain allows and swelling goes down
increase static contraction to 15 repetitions 3x/day
Rehabilitation during the sub-acute and
chronic stages following a bleed:
 As symptoms improve once can start leaving the splint
off for longer periods of time.
 Progress to free active exercises
 Exercises against resistance with low weights and
high repetitions. 15 repetitions 3x/day
 Once pre-bleed state is achieved, continue with
resistance training to improve strength
 Ensure that in case of a muscle bleed, that one regains
the full muscle length
 Retrain balance and proprioception in the case of
lower limb bleeds.
 Improve endurance and cardiovascular fitness
 Re –educate normal gait in the case of lower limb
bleeds.
Physical activity
Physical activity helps keep muscles
flexible, strengthens joints, and helps in
maintaining a healthy weight.
Children with haemophilia should get
regular physical activity, but they may have
restrictions on what they can do safely.
General activity and exercise programme
In general, some safe physical activities are:
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Swimming
Biking (wearing a helmet)
Walking
Golf
Activities that are not usually considered safe for those
with bleeding problems include most contact sports,
such as:
 Football
 Hockey
 Wrestling
NB !!!!! Advice to parents with children
with haemophilia
Your child will require protection from things in the home
environment and elsewhere that could cause possible injuries
and lead to bleeding.
 Protect your toddler with kneepads, elbow pads, and protective
helmets. All children should wear safety helmets when riding
tricycles or bicycles and should use the proper car seats or seat
belts when riding in a car.
 Be sure to use the safety belts and straps in strollers to protect
your child from a falling out.
 Remove furniture with sharp corners or pad them.
 Keep out of reach or locked away small and sharp objects that
could cause bleeding or harm your child.
 Use baby security gates to keep your child away from stairs
and other areas where he or she could fall or be injured.
Advice to parents with children with
haemophilia
 Check play equipment and outdoor play areas for
possible hazards.
 Keep a cold pack in the freezer ready to use to treat
bumps and bruises. Popsicles work fine when there is
minor bleeding in the mouth.
 Be sure that anyone who is responsible for your child
knows that the child has haemophilia.
 Consider having your child wear a medical ID bracelet or
necklace. If your child is injured, the ID will alert anyone
caring for your child about the condition.
 Learn how to examine your child for and recognise signs
of bleeding.
References
 Haemophilia. 2009. retrieved from
http://www.medic8.com/healthguide/articles/hae
mophilia.html
 Beeton, B & K. Beeton. 2009. Physiotherapy in
Haemophilia. Avaiable online at
http://www.scribd.com/doc/12272721/physiother
apy-in-haemophilia
 Remmers, E. South African Practical guidelines
for Physiotherapy in Haemophilia