In order not to be confused, it is good to differentiate between sickle cell anemia and sickle cell crisis. Sickle cell anemia is the disease itself while a crisis occur because of a trigger that causes the red blood cells change their shapes to that of a sickle, hence the name “Sickle cells”. When these red blood cells change to shapes like sickle, they occlude small blood vessels and the CRISIS is started.
Sickle cell anemia crisis therefore is the worsening of symptoms as a result of the red cells becoming less malleable and bursting or destroyed easily while the sickle cell anemia is the disease that can cause the crisis.
This article briefly describes the different types of sickle cell crisis that can affect individuals diagnosed with sickle cell anemia.
Hand Foot syndrome
This is referred to as Painful dactylitis, which is the painful swelling of the back of the hands and feet. This painful swelling of the hands and foot develop suddenly and may last for about a week or two. It normally starts in children from 6 months of age to 3 years and it is not seen when the child becomes older than age 5.
Acute Splenic sequestration crisis
This is a medical emergency and can lead to death unless prompt treatment is carried out. It is usually due to rapid enlargement of the spleen as a result trapping of circulating blood in the spleen. This causes drastic reduction in the blood that flows to other organs of the body. There will be life threatening anemia and high reticulocyte count. Blood transfusion is necessary followed by removal of the spleen (splenectomy) in children that are older.
This is caused by infection with Parvo virus B-19 and the same virus causes a disease known as the fifth disease characterized by fever, felling of being sick (malaise) and rash. Aplastic crisis causes impairment of the bone marrow to produce red blood cells. As a result of this, there is a rapid drop in Hemoglobin (the protein needed for transport of oxygen in the blood). The bone marrow usually recovers after about 7 to 10 days and normal red blood cell production continues.
This is triggered by infections. Over destruction of the red blood cells by the infection causes anemia (there would be reduced haematocrit) and jaundice (yellowish discoloration of the eyes and skin). The yellow eyes may deepen in colour if there was an existing jaundice prior to onset of the haemolytic crisis. There is usually Reticulocytosis (increased in the number of immature forms of red blood cells in circulation) as the bone marrow tries to produce red cells.
This is the same as the hand foot syndrome that occurs in children less than 3 years but has different manifestations as the child grows. It is due to reduced supply of blood flow (ischemia) to organs and tissues of the body. The shortage in blood supply causes pain. It can involve the long bones of the body, the trunk and can affect the central nervous system and other systems of the body. Depending on the organ affected, it manifest with different symptoms :
- In the lungs, it causes acute chest syndrome
- In the brain, it causes stroke
- In the eyes, it causes retinopathy
- In the bones, it causes bone necrosis
- Nephropathy can occur in the kidneys
- In the penis, it causes priapism. Priapism involves the persistent erection of the penis that is often painful and it is not associated with any sexual excitement.
- Chronic leg ulcers can occur as a result of this.
Acute chest syndrome
This may follow an infection, pulmonary infarction (obstruction of blood flow to an organ or tissue) or fat embolism resulting from bone marrow infarction. It usually starts with infarction of the ribs and atelectasis (lung collapse) may occur. It is an emergency that needs immediate treatment to prevent death.
The symptoms of acute chest syndrome in sickle cell anemia crisis are
- Chest pain
- Respiratory distress (evidenced by flaring of the nose and use of accessory muscles of respiration)
- Leucocytosis (increase in the number of white blood cell)
- Pulmonary infiltrates are seen on chest x-ray
How To Manage And Prevent Sickle Cell Crisis
Sickle cell crisis can be life threatening and immediate medical attention is needed to prevent and help relief the exaggerated symptoms the patient has. Consult your doctor in good time if you or your child has any of the above mentioned crisis. The following can be done to manage the condition.
- Bed rest.
- Fluid resuscitation with intravenous infusion (drip).
- Blood transfusion of packed cells when there is severe anemia.
- Analgesics to relief pain.
- Antibiotics to treat any infection.
- Surgery to remove spleen in order to prevent recurrence.
- Oxygen can be administered when there is respiratory distress.
You can successfully prevent sickle cell crisis and greatly reduce the risk of suffering from the different types of crisis when diagnosed with sickle cell disease by adopting certain lifestyle changes and taking certain medical precautions.
- Daily folic acid and vitamin C supplements.
- Daily Proguanil for malaria prophylaxis
- Use of insecticides treated mosquito nets to prevent malaria infection.
- Prompt treatment of malaria and other infections.
- Vaccination against Hepatitis B, Hepatitis C, Pneumococcal bacteria and Haemophilus influenzae type b.
- Preventing precipitating factors that trigger sickle cell anemia crisis by avoiding exposure to col, avoiding stress avoiding becoming dehydrated (take enough water), avoiding climbing or staying in high altitude areas and ensure you sleep in a room with enough ventilation.