What is Sickle Cell Disease/Anemia/Syndrome?
     It is an inherited blood disease, which can cause bouts of pain, damage to vital organs, and for some, death in childhood or early adulthood. Sickle cell disease affects a protein inside the red blood cells called hemoglobin. It occurs when a person inherits two sickle cell genes or a combination of one sickle cell gene plus any one of several other abnormal hemoglobin genes that affect the red blood cells. It is neither contagious nor infectious.
     Hemoglobin in the red blood cells caries oxygen from the lungs and takes it to every part of the body. A change in the oxygen carrying hemoglobin inside red blood cells causes the symptoms of sickle cell disease. Red blood cells are normally round and flexible. But when oxygen is released by the red blood cells in people with sickle cell disease, the cells become distorted, forming a rigid banana or sickle shape that can clog blood vessels.
     Sickle cells tend to become trapped and destroyed in the liver and in the spleen. These result in a shortage of red blood cells called anemia, which, when severe, can cause the client to be pale, short of breath, and easily tired. Certain conditions such as infections may worsen a client's anemia by speeding up destruction of red blood cells or reducing red blood cell production.
   
     
 
     
     
 
     
  Medical Problems
     Normal red blood cells live about 120 days in the bloodstream, but sickled red cells die after about 10 to 20 days. Because they cannot be replaced fast enough, the blood is chronically short of red blood cells, a condition called anemia.
 

    The sickled cells can also block the flow of blood through important blood vessels which may result in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest, and abdomen), stroke and priapism (painful prolonged erection).
 

    It can also cause damage to most organs including the spleen, kidneys, and liver. Damage to the spleen makes sickle cell disease clients, especially young children, easily overwhelmed by certain bacterial infections, hence the need for penicillin and special immunization to keep pneumococcal bacteria at bay.
   
     
 
     

     
 
     
  What are some of the hemoglobin variants?
     The more common types of abnormal hemoglobin are those which cause the red blood
cells to develop a sickle shape rather than its normal round shape.

This change from a round to sickle shape causes the pain crisis experienced by many
individuals who have sickle cell syndromes such as:

SS (Sickle Cell Anemia)
SC (Sickle Cell Hemoglobin C Disease)
SD (Sickle Cell Hemoglobin D Disease)
SE (Sickle Cell Hemoglobin E Disease)
   
     
 
 
     
   
     At present, there is no cure for sickle cell disease. Only the clinical problems (symptoms) are treated. The clinical features associated with sickle cell diseases (other than sickle cell anemia) are usually not as severe as the symptoms associated with
sickle cell anemia itself. Some sickle cell clients tire easily, have pain in their joints, stomach cramps, and retarded growth, jaundice (yellow tinge to the white of the eyeballs), leg ulcers, frequent colds and infections, and restriction of physical activities.
   
     
 
 
 

Myth #1
   Only Black people of African/Caribbean decent are affected by sickle cell anaemia.

Reality
   Not true, Sickle cell anaemia is a blood disorder that affects people of different racial and ethnic backgrounds. It is particularly common among people whose ancestors come from Sub-Saharan Africa, South America, Cuba, Central America, Saudi Arabia, India, and Mediterranean countries
such as Turkey, Greece, and Italy. For this reason, all races must be screened at birth for the type of haemoglobin responsible for causing sickle cell anaemia.
 
 
 
 

Myth #2
   Sickle cell anaemia care and management is basically medical and must be administered by doctors, nurses and other healthcare workers. Nothing is under our control as a family.

Reality
   Actually, there's quite a bit a family can do to care for a child with sickle cell: Families need to strike a balance between completely denying the presence of the disease and accepting the situation. They can learn to recognise problems early when medical treatment often is more effective and take precautions to ward off pain crises.

   Flexibility in responding to problems, building up a support system of friends and family or joining a support group can make a big difference in how much a child's life is affected by sickle cell.
 
 
 

Myth #3
   You can contract sickle cell anaemia from another person.

Reality
   NO, Sickle cell is not contagious. It's strictly an inherited genetic disease, and only people who are born with this genetic defect can develop it.
 
      Contrary to the medical/proper method of management of Sickle Cell crisis, those in rural areas of some developing countries like Africa tend to use a crude method of treatment [body mutilation], making sacrifices and appeasing gods which tend to expose the sufferer to secondary infection thus endangering their life...as shown in these pictures.
     
 
   Dr Ihuoma speaking at a seminar in Port Harcourt (Rivers State -- Nigeria) on Better Sickle Cell crisis management.
    
   Dr Ihuoma speaking in London, UK on Better Sickle Cell crisis management.

"Knowledge is Health, educate yourself and let's join hands to walk for the cure of Sickle Cell Anemia!
 
 
 
 
 
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