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THE STORY OF ADRIANA

Adriana is a seven-year old girl who lives with


her parents in a suburban community. Her
parents brought Adriana to Calcutta from their
home land in Bihar when she was one year old.
At the age of three Adriana was in 10th
percentile for height and weight, pale and her
haemoglobin was 5.8 g/dl. Following further diagnostic studies, she was diagnosed
with beta thalassemia major. Over the course of next four years Adriana was
hospitalized every 1- 2 months so she could be transfused with packet of red blood
cells.

 Signs and symptoms developed in her body as an indicator of Thalassemia:


chest pain, dizziness, giddiness, palpitation, abdominal pain, nausea, altered
bowel habits, hematemesis/melena, hematochezia.

 Significance of Adriana’s laboratory values : Adriana is experiencing


Anaemia as indicated by her hb level of 10 g/dl ; however for children with
beta thalassemia, the goal of treatment to maintain hb levels between 9 and
10 g/dl. The more disturbing value is hertotal serum iron of 150mg/l .The
normal level of serum iron for children is 50-100mg/l. Adriana‘s value
indicates iron toxicity.

 Complication associated with chronic blood transfusion in Adriana’s


condition : The primary complication for chronic blood transfusion is
elevated iron levels that can lead to tissue and organ failure.

 Adriana is diagnosed with Haemosiderosis. What is Haemosiderosis and how


is it treated ? Haemosiderosis is the term used for elevated serum iron
levels. It is treated with the use of a chelating agent. Deproxamine mesylate
is the reagent of choice to reduce serum iron levels. Adriana was prescribed
THE STORY OF ADRIANA

an initial dose of Deproxamine mesylate 1g IM and 400mg SC each day for


five days.

 Options available for Adriana to prevent the need for life long blood
transfusions: Either bone marrow transplantation(BMT) or cord blood
trans plants is indicated to prevent to prevent the life long need for blood
transfusions. Cord blood transplants are similar to BMTsexcept the stem cells
are harvested from the placenta or umbilical cord of suitable donor.

Now Adriana is 8. One year treatment has improved her health conditions to a lot
of extent. Adriana is undergoing treatment in Calcutta Medical Collage and
hospital. A child care foundation named ‘TERESA TRUST’ has stood by her and
offered a better quality of medication. But as a matter of sorrow, blood
transfusion has become a part of her regular activity which is really painful for a
child like her.

A detailed description of the disease and its subtypes are given on the next page.

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