HEMOPHILIA

HEMOPHILIA
P R E SEN T E D B Y :

SHANE SALES
MC TRISTAN MACANDOG
 DEFINITION
Hemophilia is an X-linked recessive genetic
disorder caused by a defective or deficient
coagulation factor. The two major types of
hemophilia, which can occur in mild to severe
forms, are hemophilia A (classic hemophilia,
factor VIII deficiency) and hemophilia B
(Christmas disease, factor IX deficiency]
 PREVELANCE and INCIDENCE
In the Philippines, there are
approximately 1604 patients
diagnosed with haemophilia
according to the World Federation
of Hemophilia 2020
 GLOBAL PREVELANCE

The researchers calculated that the life expectancy of those with hemophilia is
significantly less than other people, particularly in lower-income countries where there is
lack of treatment. Lack of treatment leads to chronic and disabling joint disease and even
death specially in countries with limited health resources. Severe hemophilia often results
in a significant economic and psychological burden on patients, caregivers, and the
healthcare system as a whole.
 LABORATORY/DIAGNOSTIC
EXAM
DIAGNOSTIC EXAM LABORATORY EXAM
Prothrombin time - Normal. No involvement of
Factor assay test extrinsic system.
Thrombin time - Normal. No impairment of
thrombin fibrinogen reaction
Platelet count - Normal. Adequate platelet
production
Partial thromboplastin time – Prolonged
because of deficiency in instrinsic clotting
system factor
 PATHOPHYSIOLOGY
NON-MODIFYABLE
• Family history of MODIFYABLE
hemophilia •Lifestyle including
• Sex alcohol, diet
ETIOLOGIC FACTOR
• Auto immune disease •Coagulation factor 8 and • Vitamin K deficiency
9 deficiency

High Partial
thromboplastin Time

Hematuria and
Prolonged bleeding from GI Bleeding from
Hemarthrosis Severe Epistaxis potential renal failure Hematoma formation Hemorrhage after
small cuts ulcers
from GI trauma tooth extraction

Pseudotumor Nerve compression

Crippling
Paralysis
 PHARMACOLOGICAL
TREATMENT
HEMLIBRA STIMATE
It can be used to either prevent via a nasal spray
or reduce the frequency of
bleeding episodes in people with
hemophilia A.

CRYOPRECIPITATE AMICAR
was commonly used to can be given through
control serious bleeding in vein or a mouth
the past.
SURGICAL MANAGEMENT

•Radiosynovectomy
 NURSING MANAGEMENT
Avoid use of Maintain optimal
Maintain a safe
aspirin products or physical mobility
environment.
non steroidal anti-
inflammatory rugs

Prevent bleeding
Relive Pain
 PROGNOSIS
Hemophilia has improved significantly
with advancements in medical care. With
proper treatment and management, many
people with hemophilia can lead relatively
normal lives. However, the prognosis can
vary depending on the severity of the
condition and how well it is managed.
Severe hemophilia may carry a higher risk
of bleeding complications, while mild
cases may have a better outlook.
PROGNOSIS
 ANYMORE
QUESTIONS?

THANKYOU!