Managing Sickle Cell Crises
Sickle Cell Anaemia is a type of haemolytic anaemia commonly referred to as “SS”.
Haemolytic anaemia is a blood disorder resulting from excessive destruction of red blood cells (RBCs), leading to reduced oxygen carrying capacity of the blood.
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.
It occurs following inheritance of HbS gene from both parents, when both parents are AS or between an AS and SS parent.
It is therefore advised that AS individuals should not have children with SS partners but rather with AA partners because AS individuals rarely develop sickle cell anaemia.
People with Sickle Cell have many health problems — some of the commonest are the ‘Sickle Cell Crises’.
The term describes different changes in the body causing serious ill health and often times, hospital admissions.
The good news for SS individuals is that they may be able to live a normal, crisis–free life if they are able to successfully avoid all the triggers of the sickle cell crisis.
The difficult part is avoiding the crisis, of course, but knowing what to avoid is the first step.
Sickle cell crises can be broadly classified into 3 types as follows:
*Vaso–occlusive crisis
*Aplastic crisis
*Haemolytic crisis
The triggers for these crises include:
*Infections — Malaria infections, Typhoid infections, Parvovirus B19 infection (as in Aplastic crisis).
*Dehydration: 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 illness like diarrhea and vomiting.
*Deoxygenation: 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).
*Acidosis: 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.
*Stress.
When exposed to these triggers that ultimately increase the body’s need for oxygen, the HbS is not as efficient as HbA and tends to falter.
The body has an amazing capability to adjust to unfavourable conditions, but due to a β–globin mutation, rather than increase its capacity, the haemoglobin S converts to thin, long micro fibres within the red blood cells (RBCs) that changes the shape of the cells from the normal sphere to a sickle. Hence the name, “Sickle Cell Anaemia”.
These now-sickle RBCs do not flow as freely as normal cells in the blood vessels, and eventually get to a point where they can no longer flow through.
Here, they accumulate and clump together, and cause blockage in the blood vessel — leading to a Vaso-occlusive crisis.
This process cuts off the blood supply to the surrounding tissue. Since tissues need blood and oxygen to survive, they die as a result of lack of blood supply.
‘Micro’ blockades and ‘micro’ tissue deaths (infarction) could worsen to involve large portions of organs causing severe pain in the organs were these tissues are located.
Secondly, sickle cells are fragile and are 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 normalcy, the spleen works double-time to destroy the sickle cells. The bone marrow also works double-time, 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 anemia common in people with Sickle Cell disease — making them easily tired with reduced stamina.
Finally, the Aplastic crisis happens specifically because of an infection with a virus — Parvovirus B19.
Usually in children without Sickle Cell Disease, the Parvovirus causes an infection that is harmless in nature and 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 leading to a massive drop in hemoglobin available before the viral infection ends by itself and new cells start to form again.
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, Gallstones due to haemolysis.
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.
4. Vaccinations.
5. Stay warm, well rested and hydrated.
6. In cases of crisis, see a doctor as soon as possible.
Thank-you for reading.
If you have any other ways crises can be avoided - please comment below!
Stay Well.
Editing by AskAwayHealth Team Disclaimer All AskAwayHealth articles are edited by practising Medical Practitioners on a wide range of health care conditions to provide evidence based guidance and to help promote quality health care. The advice in our material is not meant to replace management of your specific condition by a qualified health care practitioner. To discuss your condition, please contact a health practitioner or reach us directly through info@askawayhealth.org
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