Different types of crises in sickle cell

BY: Mirror Doctor
Sickle-shaped red blood cells are sticky and prone to forming clumps and blocking blood vessels anywhere in the body.

DEAR Mirror doctor, please what are the various crises seen in sickle cell and what can be done to prevent them?

Sickle cell crises are sudden emergency situations or complications that are seen in children with sickle cell disease attributable to the sickling phenomenon. There are several situations that can trigger crisis sickle cell. The basic underlying problem in most crises is attributable to changes that make the red blood cells prone to forming a sickle shape, and crystallising in the small blood vessels making it harder for blood to flow smoothly.

Sickle-shaped red blood cells are sticky and prone to forming clumps and blocking blood vessels anywhere in the body.

There are well known events that can induce crises usually referred to as triggers. These include:
Infections – this could be from viruses or bacteria
Dehydration (loss of body fluids without adequate replacement)
Cold temperature
Stress from various sources
Surgery or medical illness
Low oxygen such as experienced at high altitudes
The major and common types of sickle cell crisis are:

Vaso occlusive pain: This type is considered the most common. It occurs when the red blood cells form a sickle shape and block small blood vessels. This deprives the cells and structures distal to the blocked vessels of much needed oxygen for normal function. As a result, the structures affected “cry out” resulting in extreme pain in the affected areas of the body.

Acute chest syndrome: This occurs when the small blood vessels of the lungs become blocked by sickle-shaped red blood cells. As a result, they present with difficulty in breathing, fast breathing, intense chest pain and low oxygen levels. It is one of the most feared complications that can cause death if not actively diagnosed and treated.

Aplastic crisis: This condition occurs when the bone marrow slows or stops making red blood cells, leading to severe anemia.

Splenic sequestration: This occurs when the red blood cells accumulate in the spleen. This leads to enlargement of the spleen and severe anemia. This can reverse with the pooled blood getting back into the circulation. Thus, the anemia has to be managed carefully so we don’t overload the person with blood transfusion.

Hyperhemolytic crisis: This occurs when a trigger causes accelerated breakdown of the red blood cells leading to rapid and severe anemia. The urine becomes very dark like coca-cola and the eyes become yellow or deepening of the yellow eyes occur. Triggers include malaria and severe infections. Sickle cell disease has other health effects that are not considered sickle cell.

Apart from these well-known crises, people with sickle cell disease crisis can have other complications such as painful penile erections called priapism, strokes, leg ulcers and end-organ damage such as kidney and heart failure.

How Can I Prevent a Crisis?
The following can reduce the number of crisis episodes:
Dress in warm clothes when it’s cold out or when in an air-conditioned building.
Drinking plenty of water to avoid dehydration, a known trigger.
Limit alcohol consumption.
Manage your stress.
Avoid being around people who are sick.
Don’t smoke.
Early care if pregnant or planning to get pregnant.
Manage any other health conditions such as diabetes.
Stay up to date with vaccines.
Pay attention to personal hygiene
All these notwithstanding, the most important measure is to seek early treatment when unwell.

The Mirror Doctor is
a member of Paediatric
Society of Ghana. This email address is being protected from spambots. You need JavaScript enabled to view it.