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Check your symptomsManaging Sickle Cell Crises: Tips for Patients and Caregivers
July 10, 2021
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 disease, managing sickle cell crises, and what to avoid.
By Dr John Afam-Osemene
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.
Sickle cell crises can fall into 3 groups
The body has an amazing capability to adjust to unfavourable conditions.
However, due to a β–globin mutation (genetic change), rather than increasing 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 crises progress
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 a 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 the 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.
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:
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.
4. Vaccinations.
5. Stay warm, well-rested and hydrated.
6. In cases of crisis, see a doctor as soon as possible.
More Reading
Editing by AskAwayHealth Team
Disclaimer
All AskAwayHealth articles are written by practising Medical Practitioners to help promote quality healthcare. The advice in our material is not meant to replace a qualified healthcare practitioner’s management of your specific condition.
Please get in touch with a health practitioner to discuss your condition, or reach us directly here.
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