NURSING GUIDELINES

FOR CHILDREN SUFFERING

FROM BETA THAL ASEMIA

AG U S H E R M AWA N
E R I N A PAT I M A H N .
ROS I M ARTINI K.
TIARA MUTIARA SARI
INTRODUCTION

Thalassemia is an inherited autosomal recessiveblood disorder characterized by

abnormal formation of hemoglobin. Thalassemia is caused by missing genes that affect how the
body makes hemoglobin, the protein in red blood cells that carries oxygen. Thalassemia is a
heterogeneous group of hemoglobin production disorders that is primarily found in the
Mediterranean,Asian, Indian, and Middle Eastern regions.
OVERVIEW OF THALASEMIA

Thalassemia is a group of inherited blood disorders. There are

two kinds of proteins that produce hemoglobin, called alpha protein
and beta protein. The degree of excess nonfunctional alpha chains is
the major predictor of disease severity.
COMMON DEFINITIONS USED IN
THALASEMIA
Thallasemia is a genetic disorder that involves the decreased and defective
production of hemoglobin

Alpha
mayor
THALASSEMIA

Beta minor

intermedia
PATHOPHYSIOLOGY OF BETA
THALASEMIA

Beta thalassemia is due to impaired production of beta globin

chains, which leads to a relative excess of alpha globin chains.
These excess alpha globin chains are unstable, incapable of
forming soluble tetramers on their own, and precipitate within
the cell, leading to a variety of vlinical manifestation.
PATHOPHYSIOLOGY OF BETA
THALASEMIA

Alpha thalassemia in comparasion is due to impaired production of

alpha globin chains, which leads to a relative excess of beta globin
chains. The toxicity of the excess beta globin chains in alpha
thalassemia on the red cell membrane skeleton appears to be less
than that of the excess partially oxidized alpha globin chains in beta
thalassemia.
CLINICAL MANIFESTATION FOR
CHILDREN WITH THALASEMIA
Pallor, Slight sclera
icterius, enlarged Growth retardation
abdomen

Severe hemolytic Metabolic symptoms

process that suggest diabetes,
Sign&Symptoms thyroid disorder
B-Thalasssemia: extreme
pallor, swollen adomen due
to hepato-splenomegaly Neuropathy/ paralysis
in patients with severe
Bone deformities anemia not receiving
transfusion therapy

Hyper-metabolism from
ineffective erythropoiesis

Heart problem
Iron Overload
MANAGEMENT OF THALASEMIA

Children with thalassemia characteristic feature does not require medical

treatment or follow up treatment after the initial diagnosis is made. Children
with savere thalassemia require medical treatment. Blood transfusion is an
effective measure to cure thalassemia. A bone marrow transplant can offer the
possibility of drugs on young people who have a matched transfusion human
leukocyte antigen.
STANDARDIZED NURSING GUIDELINES

Developing guidelines for caring of children suffering from thalassemia

include combining four strategies including education, carrier screening, conseling
and prenatal diagnosis. Nurse can play a critical role in the wellbeingand quality
of life of children . In particular, nurses should be sure that patients are educated
about their disease ad about the treatment options available.
THANKS FOR YOUR ATTENTION
ANY QUESTION?