By Dr John Afam-Osemene
People with Sickle Cell Disease have many health problems — some of the commonest are the ‘Sickle Cell Crises’. The first step to managing sickle cell crises is knowing what to avoid. In this article, you'll learn about sickle cell diseases, managing sickle cell crises, and what to avoid.
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What is Sickle Cell Disease?
Sickle cell disease describes different changes in the body causing serious ill health and oftentimes, hospital admissions. The good news for individuals with Sickle Cell Disease is that they may be able to live a normal, crisis-free life. This depends on being able to successfully avoid all the triggers for a sickle cell crisis. The difficult part is avoiding the crisis, of course, but knowing what to avoid is the first step.
The normal adult haemoglobin is HbA. (Haemoglobin is the part of the red blood cell that carries Oxygen).
Haemoglobin SS is common in Nigeria and is expressed by 1–2 persons in 100 Nigerians.
The SS genotype occurs following the inheritance of the HbS gene from both parents when both parents are AS genotypes.
Hb SS also happens when one parent has the AS genotype, and the other has the SS genotype.
What are Sickle Cell Crises?
Sickle cell crises can be broadly classified into 3 types as follows:
The body has an amazing capability to adjust to unfavourable conditions.
However, due to a β–globin mutation, rather than increase its capacity, the haemoglobin S converts to thin, long microfibres within the red blood cells (RBCs).
These change the shape of the cells from the normal sphere to a sickle; thus the name, “Sickle Cell Disease”.
These now-sickle RBCs do not flow as freely as normal cells in the blood vessels.
Eventually, they get to a point where the abnormally shaped blood cells can no longer flow through.
Here, they accumulate and clump together, and cause blockage in the blood vessel — leading to a vaso-occlusive crisis.
How the Vaso-Occlusive crisis evolves
When vaso-occlusion happens, it cuts off the blood supply to the surrounding tissue.
Since these tissues need blood and oxygen to survive, they die as a result of lack of blood supply.
First, small blood vessels and small areas of tissue are affected.
These tissue deaths (infarction) could worsen to involve large portions of organs causing severe pain in the organs where these tissues are located.
Secondly, sickle cells are fragile and easily broken down within the vessels.
The spleen is the major organ responsible for the destruction of old or abnormal RBCs.
In an attempt to restore things to normal, the spleen works double-time to destroy the sickle cells.
The bone marrow also works at a rapid pace to replace the destroyed cells.
This is how the Haemolytic crisis happens.
The term haemolysis refers to the destruction of blood cells.
Loss of the blood cells reduces the haemoglobin available for carrying oxygen and causes the anaemia common in people with Sickle Cell disease.
This is how haemolytic anaemia happens — making the individual easily tired with reduced stamina.
Haemolytic anaemia is a blood disorder resulting from excessive destruction of red blood cells (RBCs), leading to the reduced oxygen-carrying ability of the blood.
Sickle Cell Anaemia is a type of haemolytic anaemia commonly referred to as “SS”.
Finally, the Aplastic crisis happens specifically because of an infection with a virus called — Parvovirus B19.
Usually in children without Sickle Cell Disease, the Parvovirus causes an infection that is harmless in nature.
It settles itself — usually by the fifth day - this is why it is also known as ‘the 5th disease’.
The symptoms include mild fever, a rash and appearing a little ‘unwell’.
In kids with Sickle Cell Disease, the virus kills red blood cell producers in the bone marrow.
This effect leads to a massive drop in haemoglobin available well before the viral infection ends by itself and new cells start to form again.
How Do The Sickle Cell Crises Start?
Certain triggers lead to sickle cell crises.
When exposed to these triggers that ultimately increase the body’s need for oxygen, the HbS gene is not as efficient as HbA.
The triggers for these crises include:
- Malaria infections, Typhoid infections, Parvovirus B19 infection (as in Aplastic crisis).
- This happens when the body has insufficient fluid to meet its needs.
- It can be caused by: drinking insufficient water, excessive sweating in very hot weather, or losing fluids from an illness like diarrhea and vomiting.
- This occurs when the body finds itself in conditions with low oxygen levels, high altitudes, violent cold, extreme heat, excessive exercise, smoking, obstruction to blood flow (from tight clothing, prolonged posture etc).
- This happens when the body’s fluids contain too much acid and is a sign that the Kidneys or lungs which regulate the body’s acidity are not working properly.
- This could be stress from different causes - emotional, physical etc.
Some Features of Sickle Cell Crises:
- Bone pains.
- Painful swollen fingers and toes in children;
- Leg ulcers due to obstructed blood flow and tissue death.
- Difficulty in breathing
- Chest pain due to lung involvement.
- Enlarged liver and spleen especially in children
- Jaundice (yellowing of the mucous tissues);
- Gallstones due to hemolysis.
Successfully Managing Sickle Cell Anaemia
1. Know your genotype to inform important decisions about your family life, social life and work life.
2. Avoid triggers of sickle cell crises.
3. Good nutrition and hygiene.
5. Stay warm, well-rested and hydrated.
6. In cases of crisis, see a doctor as soon as possible.
Thank you for reading. Have you any thoughts about how to avoid, recognise and deal with Sickle Cell Crises; or has this article been helpful?
You might want to check out our FREE handy guide - AS Genotype Checklist, too.
Let us know in the comments. Stay Well.
Editing by AskAwayHealth Team
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