Haemoglobin (Hb) is the substance in the blood which is responsible for transporting oxygen around the body.  Each person has 2 “sets of Hb”.  There is Normal Hb [Hb A] and there is Abnormal Hb [Hb S, Hb C …etc].

Most persons have 2 sets of normal Hb, and are therefore designated Hb AA. Some persons have 1 set of Normal Hb, and one set of Abnormal Hb e.g. Hb AS, Hb AC…etc.  More rarely some persons have 2 sets of Abnormal Hb e.g. Hb SS, Hb CC, Hb SC ...etc. The incidence [frequency with which it is found] of Hb S in Jamaica is approximately 1 to every 10 persons therefore, 1:10 persons in Jamaica is Hb AS [ i.e. they have the trait for HB S …The Sickle Cell Trait] Mathematics dictates that if the frequency of the Hb S Trait is 1:10, then the chance of the 2 Abnormal S-Hb’s coming together is 1:10 x 1:10 x 1:4 = 1:400 therefore, approximately 1:400 persons in Jamaica is Hb SS [i.e. they have full blown Sickle Cell Anaemia ].

When the S-Hb is present along with any other Abnormal Hb, the condition is Sickle Cell Disease [SS, SC, or S “Any other Abnormal Hb”]
When the S-Hb is present along with another S-Hb in an individual [Hb SS], the condition is called Sickle Cell Anaemia.  Sickle Cell Anaemia is therefore only one form of Sickle Cell Disease.  

The Presence of the S-Hb in the Red Blood Cell (RBC) confers on it (The RBC) a “Sickled-Shape” as compared with its Normal Spherical Shape. Because the RBC’s are sickled, they tend to block the small blood vessels travelling to the various organs of the body.  When these vessels become blocked, they either stay blocked for a prolonged time or the RBC’s burst to relieve the blockage…and therein lie the reasons for most of the symptoms associated with Sickle Cell Disease.  

  • Blockage 
    • Bone Pain, Abdominal Pain, Lung Problems, Stroke, Visual Problems, Ulcers Leg …….etc.

  • RBC Rupture   
    • Anaemia with resulting weakness and lethargy
    • Jaundice [yellowing of the eyes] …..etc.

  A full discourse on “Sickle Cell” is obviously not possible here, but it is hoped that this brief introduction will assist us in our understanding of the Condition.