What should you know about sequestration crisis in sickle cell disease?

In sickle cell disease, the spleen may suddenly enlarge as red blood cells collect there. In some people, the liver can also become enlarged. The body then has less blood moving through the circulation, so the heart works harder and tissues receive less oxygen.

Possible clues include unusual tiredness, weakness, dizziness, pale skin, a fast heartbeat, breathlessness, or a swollen or painful abdomen. A child may become unusually sleepy, irritable, or floppy. A sequestration crisis is a presentation of sickle cell disease, but symptoms can overlap with other urgent problems.

Treat suspected sequestration as an emergency. Contact your sickle cell team or emergency service immediately, especially if the abdomen is enlarging or the person looks pale, weak, faint, confused, or short of breath. Keep the person resting and warm, and take their medicines and medical information with them.

Clinicians may check the blood count, reticulocyte count, vital signs and spleen or liver size. A blood test helps assess anaemia and guide treatment. Hospital care may include fluids, close monitoring and a blood transfusion when clinically needed. Do not wait for home treatment to correct a suspected crisis.

Seek emergency care at once for fainting, severe weakness, difficulty breathing, chest pain, confusion, reduced alertness, a rapidly swollen abdomen, or inability to drink. These signs can indicate that circulation or oxygen delivery is deteriorating. Even milder new symptoms deserve same-day advice from the sickle cell team because the change can progress quickly.

Ask how to measure abdominal enlargement, which symptoms should trigger an ambulance, whether you need a personal emergency plan, and how your family should respond if a crisis is suspected.