EPA-0744 – Concerns of a Teenager with Homozygous Hereditary Spherocytosis: Case Report

Abstract

Introduction:

Spherocytosis is an inherited hemolytic anemia, most common on northern Europe, that causes variable degrees of anemia, jaundice, splenomegaly and in severe cases adolescents are transfusion-dependent prior to esplenectomy.

Chronic illnesses are known to provoke psychosocial dysfunction in affected children and their families. The risk of psychiatric disorder increases with the number of such family's adverse factors as marital discord, low social status and parental psychiatric disorders. It has been reported that children with two family risk factors have a fourfold increase in rate of disorder.

Objectives:

This paper aims to describe the features of psychiatric and psychosocial maladjustment in a patient firstly diagnosed as having sickle cell disease.

Methods:

We describe a 16-year-old girl with homozygous hereditary spherocytosis admitted to the outpatient Child and Adolescent Psychiatry Department who developed progressive social isolation.

Results:

Her psychiatric condition had been evolving since fifth grade, when she started blood transfusions and stricter clinical surveillance. A favorable trend was observed after strategic family therapy and individual cognitive-behavioural therapy focusing on anxiety and shyness desensitization intending to overcome friendship difficulties and improve social assertiveness.

Conclusions:

In psychosocial management of physical illnesses, assessment and care should include a focus on families besides the affected individual only. This study suggests that the bio psychosocial approach to health care and consultation-liaison psychiatry should be emphasized.