Sickle Cell Crisis And How To Prevent Them.
The term “crisis” became linked with sickle cell disease in the mid-1920s, over ten years after the initial mention of the disease in literature.
The term was applied to this new condition purely based on physicians’ observations of their patients’ urgent situations.
Although widely used by healthcare providers and patients today, “crisis” may not be the most suitable term for sickle cell patients experiencing severe pain, as individuals endure varying degrees of pain before describing it as a “crisis.”
Consequently, this can lead to inadequate pain treatment or create mistrust between physicians and patients regarding the use of potent pain-relievers.
Some patients find the term useful in conveying the seriousness of their pain and the urgency of relief, especially when seeking care at hospital emergency departments.
However, others believe “crisis” does not accurately reflect the severity of their situation.
Given the ongoing debate among experts, for now, I will continue to use the term in this write-up.
Sickle cell crises are sudden emergency situations or complications that occur in individuals with sickle cell disease due to the sickling phenomenon.
Factors that triggers crisis
Various triggers can lead to crises in sickle cell patients.
The underlying issue in most crises is related to changes that cause red blood cells to take on a sickle shape, leading to blockages in small blood vessels, thereby impeding smooth blood flow.
Sickle-shaped red blood cells are adhesive and prone to forming clots that can obstruct blood vessels anywhere in the body.
Common events known as triggers can induce crises, including infections (viral or bacterial), dehydration, exposure to cold temperatures, stress, medical procedures, medical illnesses, and low oxygen levels, as experienced at high altitudes.
The primary types of sickle cell crisis are as follows:
- Sequestration crisis: The accumulation of red blood cells in the spleen or liver causes enlargement and severe anemia.
Care must be taken to manage the anemia cautiously to avoid overloading the individual with blood transfusions.
- Vaso-occlusive pain: This is the most frequent type, occurring when sickle-shaped red blood cells block small blood vessels, causing severe pain in the affected areas due to oxygen deprivation.
- Hyperhemolytic crisis: This crisis occurs when a trigger accelerates the breakdown of red blood cells, leading to rapid and severe anemia. Triggers may include malaria and severe infections.
- Acute chest syndrome: This condition arises when sickle-shaped red blood cells obstruct the small blood vessels in the lungs, leading to breathing difficulties, intense chest pain, and low oxygen levels, posing a life-threatening risk.
- Aplastic crisis: In this situation, the bone marrow slows or stops producing red blood cells, resulting in severe anemia.
Apart from sickle cell crises, sickle cell disease can also cause other health effects, such as priapism (painful penile erections), strokes, leg ulcers, and end-organ damage, including kidney and heart failure.
How to prevent sickle cell crisis
Preventive measures to reduce the frequency of crisis episodes include:
- Staying well-hydrated to avoid dehydration, a known trigger.
- Limiting alcohol consumption.
- Wearing warm clothing in cold weather or air-conditioned environments.
- Practicing stress management techniques.
- Seeking early care during pregnancy or when planning to conceive.
- Avoiding close contact with sick individuals.
- Not smoking.
- Staying up to date with vaccinations.
- Managing other health conditions, such as diabetes.
- Paying attention to personal hygiene.
Despite these preventive measures, the most crucial action is seeking early treatment when feeling unwell.