Pediatric Nursing Demystified

Beta-Thalassemia

Thalassemia is an inherited disorder involving deficiency in production of

globin chains in hemoglobin. The beta form of the disorder is the most com-

mon and is found most often in persons of Greek, Italian, and Syrian descent.

An alpha form of thalassemia is found in people of Chinese, Thai, African, and

Mediterranean descent possibly due to genetic mutation because of intermarriages

or spontaneous mutation.

What Went Wrong?

Thalassemia is an autosomal-recessive disorder in which the alpha or beta

polypeptide chains in hemoglobin A are impacted. In beta-thalassemia there

is a decreased synthesis of the beta chains with an increased synthesis of alpha

chains resulting in defective hemoglobin and damaged red blood cells (hemolysis)

and resulting anemia.

An overproduction of RBCs (immature cells) may result in compensation

for the hemolysis. Folic acid deficiency may result from increased demand

on bone marrow.

(^140) Pediatric Nursing Demystified

1. Common symptoms of hemophilia in children include (a) ––––––––––,
   (b) ––––––––––, (c) ––––––––––, (d) ––––––––––.
   Answers:


2. What data in a 6-year-old client’s history would alert the nurse most to watch the
   client closely for signs of thrombocytopenia?
   a. The client has reported frequent backache and weight loss.
   b. The client reports urinating three times a day.
   c. The mother mentioned blood on the pillow some mornings.
   d. The school history indicates hyperactivity when doing class work.
   Answer:


3. Idiopathic thrombocytopenic purpura (ITP) commonly results in what finding?
   a. Blood clots
   b. Pallor
   c. Dry membranes
   d. Bruising
   Answer:

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