Sickle Cell Disease

Sickle cell disease (SCD) is a group of inherited red blood cell disorders that normally occurs when someone inherits two abnormal hemoglobin genes from their parents. Hemoglobin is a protein that allows red blood cells to carry oxygen to all parts of the body. People who only inherit one abnormal hemoglobin gene (S) and a normal gene (A) have the trait and not the disease.

Unlike healthy blood cells that are round in shape, sickle cell blood cells are C-shaped. As healthy blood cells are able to move freely to carry oxygen to the human body, the sickled blood cells are sticky and hard and are unable to travel freely through the blood vessels. This results in pain that is normally referred to as the sickle cell crisis.

The pain can be experienced in any part of the body but are very common in the bones, chest, and abdomen. There is mostly a need for blood transfusion due to constant shortage of red blood cells dying early. Life expectancy for sickle cell patients is between the ages 40 to 60.

Causes and Problems

A sickle cell crisis is normally caused by changes in temperature, dehydration, and stress. There can be other serious problems such as bacterial infection, acute chest syndrome, stroke, Kidney dysfunction eye conditions, pulmonary hypertension and swelling in the hands and feet.

Diagnosis

Blood test is used to diagnose sickle cell in people. Babies are tested in some countries at birth. Diagnosis is by a blood test and some countries test all babies at birth for the disease. It is also possible to diagnosis suckle cell during pregnancy is possible. Early diagnosis and treatment are important.

Treatments

Depending on the level of pain during sickle cell crises and how long the may pain last, different types of treatments are available. Mostly over the counter pain medication can to used to treat lower level pain. A severe crises usually requires an IV fluids, morphine which is a stronger pain medication and oxygen.

Prevention

In prevention of sickle cell, it is important to always stay hydrated by drinking a lot of fluids. Drinking a lot of fluids will help make your urine light yellow or clear. Dressing warmly in cold water and also preventing being too hot / warm. Rest is also important with people with sickle cell. It is very important to get a lot of rest and also reduce and manage stress levels very well. it is OK for sickle cell patients to do some exercise but not one that are tirelessly since it can trigger sickle cell crises.

Sickle Cell Types

There is a 50% chance of a child to have sickle cell trait if both parents have the trait. There is a 25% chance that a child will have sickle cell disease if both parents have sickle cell trait and also a 25% chance that the child will not have sickle cell disease.

The most common types of Sickle Cell Disease are:

HbSS

This type of sickle cell occurs when a person inherits two sickle cell genes (S) from both parents. This is commonly called sickle cell anemia and is usually the most severe form of the disease.

HbSC

This type of sickle cell occurs when a person inherits a sickle cell gene (S) from one parent and another abnormal hemoglobin called gene “C” from the other parent. This form is usually a milder form of SCD.

HbS beta thalassemia

This type of sickle cell occurs when a person inherits a sickle cell gene (S) from one parent and People who have this form of SCD inherit one sickle cell gene (“S”) from one parent and one beta thalassemia gene from the other parent. The beta thalassemia gene is also another form of anemia. The beta thalassemia can be either “0” and “+”. HbS beta 0-thalassemia is usually a severe form of SCD than HbS beta +-thalassemia that is a milder form of sickle cell disease.

HbSD, HbSE, and HbSO

This type of sickle cell occurs when a person inherits one sickle cell gene (S) from one parent and another abnormal type of hemoglobin gene that is D, E, or O. These types of sickle cell disease are mostly rare.

Clinical Services

These are some the services that are mostly provided at the clinics.

  • Newborn screening
  • Genetic testing and testing during pregnancy.
  • Pain management and preventive services
  • Periodic medical check ups.
  • Counseling
  • Supportive services