Sickle Cell Diseases

Sickle Cell Diseases

            Sickle cell diseases is a group of disorders that affects hemoglobin , the molecule in red blood cells that delivers Oxygen to cells throughout the body  people with this disorder have atypical hemoglobin molecules called hemoglobin S. which can distort red blood cells into a Sickle or crescent shape.

            Normally our red blood cell are flexible and round moving easily through our blood vessels. In sickle cell the red blood cell become redyed and sticky and shaped like sickles or crescent moons. These irregular shape cells can get stuck in small blood vessels , which can slow or block blood flow and oxygen to parts of the body.

                   Symptoms of sickle cell anemia usually show up at young age . They may appear in babies as early as months old , but generally occurs around the 6 months mark.    

             While there are multiple types of SCD(Sickle Cells Diseases) they all have similar symptoms, which vary in severity these include:

- excessive fatigue or irritability , from anemia .

- fussiness , in babies.

- bed-wetting, from associated kidney problems.

- Jaundice , which is yellowing of the eyes and skin.

- frequently infections.

- swelling and pain in hands and feet.

- pain i  chest , back , arms and legs. 

        There are several types of sickle cell diseases. The most common are:

Sickle Cell Anemia(SS):-

   When a child inherits one substitute beta globin genes(the sickle gene) from each parents , the child has Sickle Cell Anemia(SS). Population that have a high frequency of sickle cell anemia are those of African and Indian descents.

Sickle hemoglobin-C Disease(SC)  :-

        This diseases (SC) have a slightly different substitution in their beta globin genes that produces both hemoglobin C and hemoglobin S.Sickle Hemoglobin C disease may cause similar symptoms as sickle cell anemia but less anemia due to a higher blood count level. Population that have a high frequency of Sickle Hemoglobin V disease are those of West-African, Mediterranean and middle Eastern descents.   

Sickle beta-plus Thalassemia :-

   Individuals with sickle beta -plus thalassemia (SB) diseases also contain substitutions in both beta globin genes . The severity of the disease verier according to the amount of normal beta globin is produced , the symptoms are almost identical to sickle cell anemia with severe cases needing chronic blood transfusions . Population that have high frequency of beta Thalassemia are those of Mediterranean and Caribbean descents.  

Sickle Hemoglobin D diseases :-

  Through research Hemoglobin is different substitution of the beta globin gene, has been found to interact with the sickle hemoglobin gene . Individuals with hemoglobin D disease (SD) have moderately severe anemia and occasional pain episodes.