I was on duty for my clinical practice yesterday and I met a student colleague of mine who was on admission for Vaso-Occlusive Crises coupled with anaemia. He was in intense/severe pain and I just don't want anybody to make that mistake of giving birth to children with SCD and hence this writing to create awareness on it.
What is SCD?
Sickle Cell Disease (SCD) is a group of inherited red blood cell disorders.
‘‘Inherited’’ means that the disease is passed by genes from parents to their children.
‘‘Red blood cells’’ contains haemoglobin, a protein which carries oxygen throughout the body. The defect in this haemoglobin results in SCD.
Causes
•Abnormal haemoglobin called haemoglobin S causes Sickle Cell Disease.
How can one get SCD?
•NB. SCD is not contagious.
•When an abnormal haemoglobin, haemoglobin S gene, is inherited from only one parent and a normal haemoglobin gene, haemoglobin A, is inherited from the other parent, a person will have sickle cell trait or a carrier state, that is haemoglobin AS.
•A child has a chance of having SCD if the child’s parent has sickle cell trait and the other parent has another abnormal haemoglobin gene like haemoglobin S, C, D, E, etc.
•When a person inherits two haemoglobin S genes, Haemoglobin SS, one from each parent, the disease is called sickle cell anaemia which is the most common and often most severe kind of SCD and hence the focus of the presentation.
•When both parents have sickle cell trait, there is 25% chance the give birth to a child with normal haemoglobin AA, 50% chance of a child with a sickle cell trait (haemoglobin AS) or another 25% chance of giving birth to a child with abnormal haemoglobin SS.
Diagnosis
•People who do not know whether they have sickle haemoglobin (haemoglobin S) or other abnormal haemoglobin such as C,D,E.
•NB. Knowing your sickle cell status enables one to be informed about the chances of having a child with some type of SCD.
Signs and symptoms
•Painful swelling of the hands and feet (dactylitis)
•Fatigue from anaemia
•A yellowish colour of the skin/eye known as jaundice as a result of haemolysis.
Major Complications of SCD
Acute pain/Vaso-occlusive crises (VOC)
Pain episodes can occur when sickle cells block blood flow and decrease oxygen delivery. The pain often occurs in the;
•lower back
•legs
•arms
•abdomen and chest.
A pain crises can be brought on by
•Illness
•temperature changes
•dehydration
•being at high altitudes and etc.
NB. The pain is often described as sharp, intense, stabbing and even more than post-surgical pain or childbirth.
Severe anaemia
People with SCD usually have mild to moderate anaemia. However they can have severe anaemia that can be life threatening. The severe anaemia may be caused by
a)Splenic sequestration crises and
b)Aplastic crises
Acute chest syndrome (ACS)
This occurs when sickle cells block vessels of the lungs thereby depriving the lungs of oxygen. Tissues affected in the lung are damaged and cannot exchange oxygen properly. This condition is known as ACS, and very serious and should be treated right away at a hospital.
Prevention
•Screening to know your Sickle cell trait status.
•So that if the test result is positive, couples who are planning to have children need to meet with a genetic counsellor.
You can read more from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease