Name : Prasadh Lakshita

Age : Thirteen years old

Address : Neluwa

BHT Number :

Presenting complaint :
Right sided elbow and knee
swelling for four days duration.

History of presenting complaint :

Diagnosed
patient with haemophilia A since the age of thirteen month
who is on regular factor viii transfusion was apparently well ,
until four days before the admission. He developed right knee
joint swelling followed by right elbow joint swelling which is
the dominant hand of him. They are painful mildly. Aggravate
with the movements.
They are reddened, restricted the movement of affected
joints. There is few red patches around the knee and elbow
joints.
Joints are not hot to touch. They have no morning stiffness or
aggravate at the end of the day. There is no history of any
other joint involvement or migratory type involvement.
There is no associated fever or history of trauma to the
joints.
He is not complain of gum bleeding , PR bleeding
, malena , haematomesis.
No fits , focal neurological signs, generalized or
partial body weakness.
No dysphagia , pharingitis, or abdominal pain ,
back pain.
No dirrhoeal illness during or before the illness.
In this admission no cough wheezing or shortness
of breath
No vomiting abdominal pain or alternation of bowel habits
No urinary symptoms
No headache or drowsiness

Past medical history :

Baby was apparently well until
age of nine month. He developed hematoma following the
nine moth schedule of vaccination at the injection site. It was
not investigate or got treatment. Then he was asymptomatic
until the age of thirteen month and develop continues
bleeding from a wound in mouth following a trauma.
Therefore they seek medical advice from Teaching hospital ,
karapitiya. He was inwarded for several days and investigate
Certain blood investigations were performed most probably
the full blood count, activated partial thromboplastin time
(APTT), Prothrombin time/ International Normalization ratio,
bleeding time and clotting time.

Initially he transfused with cryo precipitate five packs.

Parents were advised to restrict the child from involving body
contact games. one month after this presentation baby was
diagnosed as having Haemophilia A, probably by Factor
collection test. Factor assay was not said to be done.

Then at the age of one and half years and two and half years
similar episodes of bleeding from trauma sites occur. He
admitted to THK due to that and transfused cryo and
blood .[two pint]
At the age of nine years he developed joint swelling probably
a haemathrosis of right knee, elbow which resolve
spontaneously.
In the September 2006 another episode of
haemathrosis of right and left knee and right elbow which is
more severe than previous and admitted to THK. There he
was introduced factor viii treatment. Other than new
treatment strategy he vaccinated for hepatitis B .
Since then he was regularly on factor viii therapy, once in
two weeks. Instead of regular transfusion he developed
several attacks of haemathrosis not only knee and elbow ,
small joints also involved.
No past history of intracranial haemorrage , focal
neurological sign or body weakness.
no history of intra abdominal bleeding or bleeding in
to upper airways causing airway obstruction or hypovolaemic
shock following an excessive bleeding
Despite several episodes of haemarthrosis no evidence of
crippling arthritis.
No history of fever with jaundice and right hypochondriac pain
after starting Cryoprecipitate transfusion
There is no history of any allergic reaction following cryo
transfusion.

Birth history :
Antenatal history uneventful
Antenatal ultrasound scan not done
Full term normal vaginal delivery
Birth weight 2.5 kg.
Baby cried just after delivery and breast feeding start within
first half an hour.
No P.B.U care

Developmental history :
 Age appropriate
School performances good as he place forth in the forty
student class of national school. He score more than 75 for
mathematics , science and Sinhala.

Immunization history :
Age appropriate according to E.P.I schedule
Hepatitis B 1st dose given 2 years back and next 2 booster
doses completed

Dietetic history :
Breast fed up to the age of four
month and weaning start there after. Now he is use to eat
adult diet as usual three meals.

Family history :

42yrs
37yrs

13yrs
10yrs 1y 9m

No consanguinity
No family history of bleeding disorders.

Social history:
He is a school boy studying at grade
seven. School is one km away from the home which travel by
school van with his sister. It cost 300 rupees for each.
He is the elder son of five membered family. Both parents are
planters of their own tea state. Monthly income varies time to
time which is usually ten thousand rupees per month.
Both of them studied up to grade nine.
Patient’s schooling is severely affected by frequent hospital
admissions and episodes of haemarthrosis which affect his
writing skill. In spite of that his school performance was good
who place 4th in the class.
School staff is well known about the boy and they always
try to keep away him from facing to trauma. eg: principal
never force him to any aerobic sessions or assemblies.
He is not take part in any contact games. Doctors has been
ask them to play games like carom ,chess like games which
are still not available for him either in school or home. He
used to play only with his siblings back at home under the
parental supervision.
His younger brother also a diagnosed patient with
haemophilia as it detected on September 2006. but he is not
on factor viii prophylaxis.
Parents are really keen about their children’s ill-health and
it’s not affected to their family harmony. Always one parent
must stay in hospital with the child, usually father and mother
manage to do all the house hold works of her own including
plantation maintenance.
They are economically strong enough to face the
expenditure of illness as one hospital stay cost them about
1500 rupees. No special expenditure for medication.
Parental knowledge about the illness is not adequate and
they bother
about the life expansion of ill childs.
Some degree of social negligence by some neighbors and
collides.

On examination :

Weight 35 kg just above the 10th centile.

Height 140cm just above the 3rd centile.
Weight deficit = (45-27)/45 *100 %
=
=20 %
Therefore he is first degree malnourished according to Gomez
classification.

GENERAL EXAMINATION

not warmth to touch

not icteric
not pale
no lymphadenopathy
mucocutaneous manifestations of bleeding around the right
elbow and knee joint together with right knee joint swelling.
Mild tenderness over right knee joint.
It’s not red. Not warmth.
Girth of left knee 38cm (including femoral tuberosities)
Girth of right knee 42cm.
Flexion is restricted than extension.
No any other joint swelling.
He has no limp.

Respiratory system
no gross chest deformities
chest wall moves symmetrically with respiration
respiratory rate 20 cycles per minute
percussion note resonant
vocal resonance normal
vesicular breathing
no added sounds

Cardiovascular system

pulse rate 80 beats per minute good volume regular rhythm

blood pressure 120/80mm hg in supine position.
all peripheral pulses were felt.
no visible pulsations in the precordium.
apex beat left 5th intercostal space midclavicular line
no thrill
S1 and S2 heard all four auscultatory areas. no murmurs

Abdomen
not distended
no surgical scars
not tender
no hepatospleenomegaly
no free fluids
bowel sounds normal

Central nervous system

conscious
cranial nerves clinically are normal
upper limbs and lower limbs normal

summary
Thirteen years old schooling boy, . born to non
consanguines parents, diagnosed patient with haemophilia A ,
depend on regular factor viii transfusion with past history of
haemoathrosis present with right sided haemoathrosis of both
elbow and knee as most often. His brother also suffer from
same illness. On examination he is not pallor, pulse good
volume with rate of 80 and evidence of right knee
haemathrosis

Problems
1. diagnosed patient with haemophilia A , on regular factor
VIII transfusion.
2. right sided haemathrosis of knee and elbow joint
3. male sibling suffer from same illness.
4. school absentees due to hospital admission and
recurrent haemathrosis which is affect more to the
dominant hand.
5. poor educational state of the parents
6. 1500 rupees expenditure for one hospital stay.
Management
Management of haemoathrosis
Pain ; due to mild pain can use paracetamol according to
weight eight hourly.
(15mg * 35 = 525mg) one tablet of pcm
once.
Bed rest
Keep Ice pack
Compression bandage
Elevate the limb
Factor correction
prophylactic factor viii transfusion should do
.
we expect to achieve haemostatic level(35-40%) of factor viii.
so required international units of factor viii =(desired level\2 )
*body weight

=(40\2)*35

=700 international units

1 bottle contains 264 international units

therefore 3 bottles per dose should be given
in the ward usually it is given as twice daily for 2 days

follow up plan

1.continue factor viii prophylaxis 2 weekly

2.advice to come immediately to hospital if any bleeding
manifestations develop

Advise given to parents to prevent complications

1. avoid even a minor trauma

2. brush teeth properly because extraction of a carious
tooth may lead to life threatening bleeding
3. avoid anti-inflammatory agents such as aspirin
4. avoid constipation
5. avoid IM injections
6. encourage the parents to show diagnosis card when
taking medicine for other illnesses
7. educate about the complications of chronic factor viii or
Cryoprecipitate transfusion
-5-20% of patients can develop antibodies against
factor 8 which reduce or completely inhibit the
effect of treatment
-transfusion transmitted infections such as
hepatitis b and HIV

In this case patient complain about

recurrent episodes of haemathrosis instead of regular factor
VIII propylaxis should suspect about development of factor VIII
inhibitors.
For detect that,

Discussion:
• Hemophilia is an X-linked congenital bleeding
disorder with a frequency of about
one in 10,000 births.
• Hemophilia is caused by a deficiency of coagulation factor
VIII (FVIII) (hemophilia A)
or factor IX (FIX) (hemophilia B) related to mutations of the
clotting factor gene.
• The number of affected persons worldwide is estimated to
be about 400,000.
• Hemophilia A is more common than hemophilia B,
representing 80-85% of the total.
• The life expectancy of persons born with hemophilia, who
have access to adequate
treatment, should approach normal with currently available
treatment.

females are carriers and males are more commonly

affected
they can present with intracranial haemorrhage in
neonates, prolonged bleeding on circumcision easy bruising
and intramuscular haemotomas, prolonged bleeding minor
traumatic lacerations haemarthrosis

haemophilia patients can be classified in to 3 groups

according to severity

Bleeding Manifestations in Hemophilia

Sites of bleeding
Incidence of bleeding into different joints
• Knee: 45%
• Elbow: 30%
• Ankle: 15%
• Shoulder: 3%
• Wrist: 3%
• Hip: 2%
• Other: 2%

Incidence of different sites of bleeding

• Hemarthrosis: 70%-80%
• Muscle/soft tissue: 10%-20%
• Other major bleeds: 5%-10%
• Central nervous system (CNS) bleeds: < 5%
Serious
• Joints (hemarthrosis)
• Muscle/soft tissue
• Mouth/gums/nose
• Hematuria
Life-threatening
• Central nervous system (CNS)
• Gastrointestinal (GI)
• Neck/throat
• Severe trauma

Chronic Complications of Hemophilia

• Musculoskeletal complications:
- Chronic hemophilic arthropathy;
m Chronic synovitis;
m Deforming arthropathy;
- Contractures;
- Pseudotumour formation (soft tissue and bone);
- Fracture;
• Inhibitors against FVIII/FIX;
• Transfusion-related infections of concern in people with
hemophilia:
-Human immunodeficiency virus (HIV);
- Hepatitis B virus (HBV);
- Hepatitis C virus (HCV);
- Hepatitis A virus (HAV);
- Parvovirus B19;

Psychological aspect of the management

Daily living
• Persons with hemophilia can perform usual tasks and,
therefore, should be encouraged
to engage in productive and leisure activities at home, in the
workplace, and in
recreation areas.

• Persons with hemophilia must be reassured that they have

the care and moral
support of others so they do not become isolated and
experience depression.
For the person with hemophilia
The person with hemophilia should be encouraged to:
• Accept himself as a person with hemophilia who can
successfully function in society
despite this chronic condition.
• Accept his own strengths and limitations and not blame
himself or others for having
hemophilia.
• Think and act positively. Continue with his usual tasks,
choosing activities that have
lower risk of injury.
• Feel confident sharing with a family member or a friend his
feelings and experiences
of his health condition.
• Always have contact numbers or addresses of people,
clinics, and health centres that
can provide immediate information and necessary medical
attention when needed.

For the family

• The patient and all family members must recognize and
acknowledge the presence of
hemophilia within the family.
• Every member of the family must be provided with at least
basic information on the
physical, psychological, and economic dimensions of
hemophilia.
• Family members with no hemophilia must be available to
provide emotional,
physical, and spiritual support, if needed, to the member with
hemophilia.
• Family members must become aware of emotional or
attitudinal changes of the person
with hemophilia as this may indicate stress that may be
related to bleeding occurrences,
physical pain, or emotional difficulty that may need immediate
intervention.
• Family members, as caregivers, must try to be calm when
bleeding, pain, and other
hemophilia signs and symptoms occur in the patient in order
to demonstrate that
this condition can be calmly managed at home or anywhere.
• When the need for medical attention or hospitalization is
necessary, family members
must be able to recognize this need and be available to
assist immediately and in
whatever way possible to avoid further complication.
• The person with hemophilia should always be encouraged to
socialize with other
members of the family and within the community.
• Indoor as well as outdoor activities with lesser risks of
injury or harm to the person
with hemophilia must be encouraged.
• A young, active child with hemophilia commonly has
numerous bruises. Parents
may be wrongfully accused of child abuse.

For the community

• Basic information and education should be given to the
community where there is
an identified member with hemophilia, provided the
patient/family consents. By
doing so community members will be more willing and
available to respond to the

Sports and Hemophilia

• Sports activities should be encouraged to promote muscle
strengthening and
increased self-esteem. The choice of sports should reflect an
individual’s preference,
ability, physical condition, local customs, and resources.
• Low impact activities such as swimming and golf should be
encouraged. High contact
sports such as football, rugby, boxing, and wrestling are not
advised. The patient
should consult with a physician before engaging in sports
activities to discuss
appropriateness, protective gear, and prophylaxis prior to the
activity.
• Organized sports programs should be encouraged as
opposed to unstructured activities,
where protective equipment and supervision may be lacking.
Reference:
GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA
- WORLD FEDERATION OF HEMOPHILIA