In this MD Newsline exclusive interview with hematologist/oncologist Dr. Cheryl Mensah, we discuss the four major crises of sickle cell anemia and how to prevent and manage these crises.
What are the four major crises of sickle cell anemia?
Dr. Cheryl Mensah:
“The four major crises of sickle cell anemia are vaso-occlusive crisis, aplastic crisis, acute splenic sequestration crisis, and hyperhemolytic crisis. The most common is vaso-occlusive crisis, blocking of the blood vessels by red blood cells, which reduces the oxygen supply to the body’s organs and causes pain. Over time, repeated vaso-occlusive episodes can be life-threatening.
Aplastic crisis is a little less common. It’s usually triggered by viral infections and affects the bone marrow’s ability to produce red blood cells. It can be life-threatening if the patient’s red blood cell count gets too low.
Acute splenic sequestration crisis is more common in the pediatric population and is when red blood cells get trapped in the spleen, and the spleen enlarges. This splenic enlargement is painful and can cause a life-threatening drop in the patient’s circulating blood volume.
Finally, hyperhemolytic crisis is also more common in the pediatric population and is very rare. A trigger, such as infections, certain drugs, and toxins, causes the red blood cells to break down, and the patient’s hemoglobin level rapidly drops. It can quickly lead to organ failure and death.”
How do you prevent and manage these crises?
Dr. Cheryl Mensah:
“Currently, the best medication to prevent vaso-occlusive crises is hydroxyurea. It has been shown to prevent vaso-occlusive crises and other complications of sickle cell disease.
Other medications like L-glutamine and crizanlizumab can also prevent vaso-occlusive crises, but they haven’t been shown to prevent other complications of sickle cell disease.
The main way to prevent an aplastic crisis is to prevent viral infections with measures such as proper hand hygiene and social distancing. The treatment for aplastic crisis is supportive care and sometimes blood transfusions when the hemoglobin goes down.
Sometimes splenic sequestration is triggered by bacterial infections but not always. There is some evidence that hydroxyurea may be able to help delay splenic sequestration. Treatment for splenic sequestration consists of simple transfusions or even exchange transfusions.
Finally, hyperhemolytic crisis is also hard to prevent. So, we just have to manage this crisis when it occurs. Treatment for hyperhemolytic crisis is also transfusions.”
Responses have been condensed and lightly edited.