SICKLE CELL ANAEMIA SIGN AND SYMPTOMS

[Mpimba]

Clinical Features of Sickle Cell Disease

•  Symptoms of sickle cell disease vary  and range from  mild to severe, and symptoms  may be less severe or different in  children who have inherited  a sickle cell gene from  one parent and a different abnormal haemoglobin gene from  the other.

•  Most kids with sickle cell disease have some  degree of anaemia  and might develop one or more of the following conditions and symptoms  as  part of the disorder. o  Acute Chest Syndrome Inflammation or trapped red blood cells  in the lungs cause this syndrome.

 Features  include chest pain, coughing,  difficulty breathing and  fever o  Hand-Foot Syndrome (also called Dactylitis) This painful swelling of the hands and feet, plus fever, may be the first sign of sickle cell anaemia in some  infants.

o  Aplastic Crisis

This is when the bone marrow temporarily slows its production of RBCs due to infection or another cause, resulting in  a severe drop in the red cell count and severe anaemia Signs include paleness,  fatigue, and rapid pulse. Haemolytic crises are acute accelerated  drop in haemoglobin level.  The red blood cells break down at a faster rate.

o  Painful Crises

These may occur in any part of the  body and may be brought on by cold or dehydration.  The pain may last a few hours or up to 2 weeks or even longer, and may be  so severe that a child needs to be hospitalized.

o  Splenic Sequestration Crises

The spleen becomes enlarged by trapping (or ‘sequestering’) the abnormal RBCs. This leads to  fewer cells in the general circulation. Early signs include paleness, weakness,  an enlarged spleen, and pain in the abdomen.

o  Stroke

Poor blood flow in the brain can occur when the sickle-shaped cells block small blood vessels.  This may lead to a stroke.  Signs can include headache, seizures, weakness of the arms  and legs, speech problems, a facial droop, and loss of consciousness.