Sickle Cell Crises and Their Treatment

Sickle cell disease is caused by a mutation in red blood cells that causes “sickling” during times of stress in the body. A healthy red blood cell is like a squishy balloon filled with water that can get through small blood vessels in the body. A sickle cell is pointy and stiff, easily getting stuck in small blood vessels and blocking blood flow.

Different sickle cell crises have different treatments:

• Painful crisis and dactylitis –People with sickle cell get a pain crisis when blood is blocked in bones and tissues. Infants and young children experience swelling and pain (dactylitis) when the hands and feet are affected.
  • Mild episodes can be treated at home with fluids and over-the-counter pain medications like ibuprofen (for inflammation in addition to pain).
  • More severe episodes require hospital admission, IV hydration and stronger pain medications.
  • Pain reported by the patient and family is the most important indication of a pain crisis since no blood test can reliably show when a crisis is happening.
• Acute Chest Syndrome (ACS) – This is a white area in the lung on chest x-ray, from a combination of infection and the blockage of blood flow from sickled red cells and/or small pieces of fat released from bones during a pain crisis.
  • ACS can cause chest pain, fever, cough, or trouble breathing and is an emergency that needs to be treated at a hospital immediately.
  • Treatment includes IV hydration, pain medications, oxygen and antibiotics. An important part of treatment is a transfusion of healthy red cells to dilute the sickle cells in the body. Doctors may also order an exchange transfusion, which involves removing sickling red blood cells from the body before a transfusion is given.
• Stroke – Sickling red blood cells that block blood flow in the brain can cause a stroke. Signs of stroke include headache, difficulty moving part of the body or drooping on one side of the face.
  • Stroke is an emergency that needs to be treated at a hospital immediately.
  • Treatment includes a blood transfusion or exchange transfusion. People with sickle cell disease need to have ongoing blood transfusions after one stroke to prevent another.
  • Some patients need a yearly specialized ultrasound test (transcranial doppler) to screen for high stroke risk.
• Splenic sequestration – When sickling red blood cells block blood draining from the spleen, the spleen can grow and act as a “sink” for red blood cells and platelets. Families are taught how to feel for a large spleen on the left side of the abdomen under the rib cage.
  • Signs of splenic sequestration are paleness, bruising or a red rash called petechiae, abdominal pain or swelling.
  • This needs to be treated in a hospital with IV hydration and a blood transfusion.
• Priapism – Blockage of blood flow in the penis causes an erection that lasts too long.
  • This is treated in a hospital with IV hydration and a blood transfusion.
• Fever – In most people with sickle cell disease, blood flow to the spleen is blocked early in life, causing the spleen to quit working.
  • Because an important job of the spleen is to filter certain bacteria from the bloodstream, fever in a person with sickle cell disease is a medical emergency and needs to be treated at a hospital immediately with a blood culture and antibiotics.

The good news is that you and your sickle cell doctor can do a lot to prevent crises! Stay up to date with your sickle cell clinic check ups and talk to your doctor about medications such as hydroxyurea to treat your sickle cell disease. There are also options for gene therapy or bone marrow transplant for patients with more severe sickle cell disease too.