How sickle cell affects children

Sickle cell is one of the inherited childhood illnesses, in an autosomal recessive manner. In sickle cell anaemia, red blood cells assume an abnormal, rigid, sickle shape, which become stiff and block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain and organ damage.

The term ‘sickle cell crisis’ describes different conditions, including blockage of blood vessels, causing severe pain, swollen painful fingers and toes, acute chest syndrome, massive destruction of red cells and aplastic crisis. The complications of sickle cell disease include frequent overwhelming infections due to a non-functional spleen, stroke, gallstones, and destruction of the hip bone, bone infections, leg ulcers, eye problems, chronic pain, and kidney failure.

Your child’s doctor may give treatments that prevent complications from the disease, including folic acid, penicillin and prophylaxis against malaria. Pain medications are prescribed so that the child can take them whenever it strikes.

Sometimes crises are so severe that the child needs to be admitted in hospital for intravenous fluids and stronger pain medications. Many children with sickle cell require frequent blood transfusions when the haemoglobin levels falls very low.

The use of a drug called hydroxyurea has helped reduce painful crises and episodes of acute chest syndrome for adults and children with sickle cell. Children on hydroxyurea have less frequent hospitalisations and crises.

The only cure for sickle cell disease is bone marrow transplant.

Though an expensive, complex and risky procedure, it is increasingly offering a solution to a very troublesome problem. Research is focussing on gene therapy as a treatment for sickle cell.

If your child has sickle cell, provide balanced diet, folic acid supplements, malaria prophylaxis, plenty of fluids, avoid heavy physical work or exercises and get adequate rest.

Alcohol, drugs and smoking must be avoided.

Give pneumococcal vaccine to the child to reduce possibility of serious infections.

Photo: themastercopy.com