Hereditary Spherocytosis

Hereditary Spherocytosis (HS) is a type of anaemia caused by a defective gene. Red blood cells are affected and have a smaller surface area than normal red blood cells. They are shaped like spheres and break open easily, decreasing the life span of the cells.  

Symptoms usually include fatigue, irritability, shortness of breath and in most cases the spleen is enlarged.  Patients may also develop gall stones which can result in the development of pain and jaundice.

Most children with HS do not require any specific treatment.  Sometimes a baby with HS may need blood transfusions for the first few weeks of life, but then may not have any further problems as they grow older.  In older children who are severely anaemic, surgery may be required to remove the spleen. This will cure the anaemia but doesn’t correct the abnormal cell shape. With surgery the life span of the red blood cell returns to normal.  After a child’s spleen has been removed they have an increased risk of severe infections.  Many children will require antibiotics every day to prevent infections, and instructions are given to ensure that strong antibiotics are taken promptly as soon as a child has a fever.  Surgery to remove the gallstones and gallbladder may also be required in children with HS.