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Title: case study haemophilia
Description: case study regarding child whos suffered from haemophilia A and bnen warded in hospital for quite a while.

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Prepared By:
Razaidah
...
P
...

Specialist Practice in Children’s Nursing Case Study
Universiti Brunei Darussalam
Care of a child with Severe Haemophilia A and Intracranial Haemorrhage

INTRODUCTION
During my clinical placement in Paediatric Intensive Care Unit (PICU), I have
chosen one interesting issue on a child whose been diagnosed with Intracranial
Haemorhage with known case of Heamophilia A for my case study
...

Before I'm doing my case study, I introduce myself to the Child’s parent that I'm a
Paediatric student nurse and asking a permission from the child’s parent whether they
are agree if i take the child case for my case study or not
...


The aim for this case study is to advance clinical and caring skills and to provide
child with haemophilia with a comprehensive and holistic nurse-led service
...
And secondly, is to improve the quality of care to a child
1

with hemophilia by enhancing nursing practices and to provide an informative education
to parent
...
1) Child’s background information:
During my clinical attachment in Paediatric Intensive Care Unit (PICU), I receive
one interesting case, a child with intracranial haemorrhage and he has a past medical
history of Severe Haemophilia A
...
Azim, a 6
years twin boy suffers from intracranial haemorrhage of 1
...
According to parents he has a multiple
admissions history for haemarthrosis since May 2013
...

Nausea and vomiting on and off
...
According to parents,
the child is not orientated like yesterday and said some inappropriate words
...
Urgent CT scan
done, from the result it shows bleeding is almost the same like the previous CT except
slightly increase in midline shif
...
Had 3-4 times seizure episode since yesterday
...
After long discussion involving paediatricians,
neurologists and haematologist, they decided to take Azim for urgent Craniotomy /
Evacuation of Subdural haematoma surgery because of Azim’s deteriorating and critical
condition
...


Back form operating theatre, Azim clinically stable on ventilator SIMV with
pressure support and well sedated
...
Informed the
paediatrician on call, to increase the sedative amount and to repeat hemoglobin level
...
Inform the paediatrician on call
...

During my clinical attachment in P
...
C
...
The investigations
done for Azim are Several time Ct Scan, blood investigation such as blood
haemoglobin, platelet, white blood cells level, factor Vll level, arterial blood gases,
coagulation profile, urea and creatinine
...
45%

sodium Chloride), injection Ondansetron, injection Tranexamic acid Infusion,

injection

Midazolam

infusion,

intravenous

Phenytoin

Infusion,

Cryoprecipitate

transfusion, Packed cell blood transfusion, intravenous Atracium infusion, nasogastric
for feeding
...


2
...
Intracranial hemorrhage occurs within the cerebral parenchyma
secondary to lacerations or to contusion of the brain, with injury to larger, deeper
cerebral vessels occurring with extensive cortical contusion (Dawodu, 2013)
...

(Cynthia, 2013)
...


4

The signs and symptoms in Azim relating to ICH are sudden and severe
headache, confusion, drowsiness, vomiting more than twice in 24 hours and unequal
pupil size
...
3) Issue of care:
Azim is a child whose been diagnosed with a severe Haemophilia A since he
was 1 year old and this is the most well-known issue for all child whose suffer from this
disease as it preventing Azim to have faster healing process from the intracranial
hemorrhage and the surgery
...
But the nurses in the pediatric ward should be able to trace back the entire child
with defaulted treatment in factor Vlll, and they should contact Azim’s parent and advice
to bring their child as soon as possible for the factor Vlll treatment as it may cause
serious bleeding or life threatening if the child missed the factor Vlll treatment
...
This treatment means
that the child or young person now has moderate rather than severe haemophilia and,
therefore, is unlikely to experience disabling bleeding into his or her joints (Middleton,
2003)
...
When a
person with hemophilia is injured, he does not bleed harder or faster than a person
without hemophilia, he bleeds longer
...


Haemophilia is an inherited bleeding disorder that results from a low level of
proteins needed for normal blood clotting that mostly affects men
...
Haemophilia, in contrast, lead to prolonged bleeding due to inability to
form a strong clot
...
Hemophilia A, a deficiency
of coagulation factor VIII, occurs in approximately 1 in 10,000 males (Baker, Riske &
Drake, 2013)
...
The risk of
ICH varies with the severity of hemophilia, and children with severe hemophilia (factor
level <1%) are at highest risk, from spontaneous and traumatic ICH (Witmer, Raffini &
Manno, 2007)
...
2010)
...
2012)
...


A comprehensive care should be involving a paediatrician, haematologist,
nurses, physiotherapist, occupational therapist, physiatrist, orthopedic, Rheumatologist,
a laboratory specialist, a social worker, a psychologist
...
Acts as the first contact for child with an acute
problem, give an immediate action such as informing the pediatrician for any changes in
child condition and deliver a good nursing care to child and parents
...
All the studies were from the United

7

States
...


Comprehensive care improved child’s and parent knowledge and skills to help
them manage themselves, reduced days of hospitalization and days lost from school
...
They are able to provide diagnostic
services; 24 hour access for the assessment and treatment of bleeds; genetic
counseling; specialized podiatry and physiotherapy services; home treatment training
programmes; home delivery services and, counseling and support for child and their
families
...
A
range of specialists (including Orthopaedics, Infectious Diseases, Obstetrics and
Dental) work closely with the Haemophilia Centre staff
...


3) RECOMMENDATIONS

8

To provide and coordinate inpatient (during hospital stays) care and services to
child and their families
...


To initiate, provide training for, and supervise home-therapy with clotting factor
concentrates where available
...
To collect data in the
Bruhim system on sites of bleeds, types and doses of treatment given, assessment of
long-term

outcomes

(particularly

with

reference

to

musculoskeletal

function),

complications from treatment, and surgical procedures
...
Nurses must be alert on the
importance to give early treatment without any delay in patients presenting with
spontaneous intracranial hemorrhage as it help to decrease hematoma enlargement
and lead to better neurologic outcome such as informing the pediatrician immediately if
the nurses noted any changes in patients conscious level, give immediate treatment
such as blood transfusion as ordered by the pediatrician
...
Correct any identifiable coagulopathy with fresh frozen plasma,
vitamin K, protamine, or platelet transfusions
...
If the nurses able to trace early on Azim defaulted treatment and consult
with his parents earlier maybe the complication, multiples admission because of the
bleeding and several invasive investigation and surgeries can be prevent
...
Learning about bleeding
disorders can vastly improve the care you provide and increase the child’s and parents
confidence in your care
...
Listen
closely as the child describes symptoms
...
Nurses need to
educate parents regarding the importance of the compliance to the factor Vlll treatment
in order to prevent any bleeding happen to their child
...
, Franchini, M
...
, Faienza, M
...
, Valente, R & Molinari, A
...

(2013)
...
Italian journal of Pediatrics
...
ijponline
...
S
...
L
...
Medscape: Intracranial Haemorhage
...
medscape
...
(2003)
...
Nursing Times
...
nursingtimes
...
article
Hitch, D
...
Continuing Education: What every nurse should know about
hemophilia
...
americannursetoday
...
aspx?
id=10064&fid=10018
Lee, L
...
, Dayan, P
...
, Gerardi, M, J
...
A
...
K
...
M
...
(2011)
...
The Journal of Pediatrics
...
pecarn
...
pdf
National Hemophilia Foundation
...
Hemophilia A (Factor VIII Deficiency)
...
hemophilia
...
htm
Mudla, I
...
A
...
(2012)
...
Ministry of Health Malaysia
...
moh
...
my/hta/HTA-Haemophilia
...
, Mrazik, M
...
Incidence of Delayed Intracranial
Hemorrhage in Children after Uncomplicated Minor Head Injuries
...
Retrieved from,
pediatrics
...
org/content/126/1/e33
...
L
...
E
...
, Perkins, E
...
S
...
Intracranial
Hemorrhage in Children: An Evolving Spectrum free
...
Retrieved from, archneur
...
com/article
Title: case study haemophilia
Description: case study regarding child whos suffered from haemophilia A and bnen warded in hospital for quite a while.