Let’s explore the different types of sickle cell crisis

Life-threatening consequences can come from some of these other forms

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by Mary Shaniqua |

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Living with sickle cell disease is no small feat.

It’s a condition that comes with a host of symptoms, complications, and issues that are unpredictable and span a lifetime. When people think about sickle cell disease, the most commonly known symptom is the vaso-occlusive crisis, also known as a pain crisis.

But did you know patients can experience other types of crises? I’ll take you through them now.

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7 other crises

An aplastic crisis occurs when bone marrow temporarily stops producing red blood cells, possibly leading to a sudden drop in hemoglobin levels. That can cause severe anemia and profound fatigue. Aplastic crises are often triggered by infections, most notably parvovirus B19, which suppresses red blood cell production.

Prompt medical attention is necessary to manage the resulting anemia. I’ve written about my own experience of aplastic crisis.

A splenic sequestration crisis, particularly common in young children, is a potentially life-threatening emergency. In this crisis, sickled cells become trapped in the spleen, leading to a rapid drop in circulating blood volume. This sudden pooling of blood causes severe anemia and can result in shock. The spleen may become enlarged and tender, and without urgent treatment, the condition can be fatal.

When you encounter sickle cell patients who’ve had their spleen removed, chances are a splenic sequestration crisis was the root cause.

A hemolytic crisis is an accelerated breakdown of red blood cells. As they’re destroyed faster than they can be replaced, patients may experience symptoms such as jaundice (yellowing of the skin and eyes), fatigue, and dark-colored urine. That’s because the breakdown of cells releases a substance called bilirubin, which is yellow.

Hemolytic crises may be triggered by infections, medications, or other external stressors that affect red blood cell stability.

Acute chest syndrome is a serious and potentially fatal complication of sickle cell disease. It occurs when sickled cells obstruct blood vessels in the lungs, leading to inflammation and impaired oxygen exchange. Symptoms include chest pain, fever, cough, and shortness of breath. This condition requires immediate hospital treatment, including oxygen therapy, pain control, and sometimes blood transfusions.

Acute chest syndrome hurts like hell and is the reason all sickle cell patients have to take chest pain seriously. Mistaking this syndrome for a pain crisis in the chest can have fatal consequences.

Priapism is a painful, prolonged erection that can be caused by sickled cells blocking blood flow in the penis. If not treated quickly, priapism can lead to permanent tissue damage and erectile dysfunction. This crisis is a urological emergency and requires rapid intervention to restore normal blood circulation.

A hepatic crisis involves the blockage of blood flow to the liver, resulting in liver dysfunction. A hepatic crisis may present with abdominal pain, jaundice, and elevated liver enzymes, and like other crises, it demands urgent medical evaluation and management.

Dactylitis, also known as hand-foot syndrome, is often the first clinical sign of sickle cell disease in infants and young children. It manifests as painful swelling of the hands and feet because of blocked blood flow in the tiny vessels.

Early recognition and treatment are critical, as dactylitis can be a marker for more severe complications later in life. I believe this swelling is distinctly different and far more serious than the day-to-day swelling of hands and feet that sickle cell patients can encounter.

Each type of sickle cell crisis affects the body differently, even if there are overlapping symptoms. Timely diagnosis and medical intervention are essential to manage complications, prevent organ and other permanent damage, and improve quality of life for people living with sickle cell disease.

Understanding the different types of sickle cell crises is vital — not just for patients, but for caregivers, medical professionals, and the wider public. These crises are not just medical terms; they’re lived realities that can be painful, frightening, and, as described above, possibly life-threatening.

By raising awareness of their complexity and urgency, we can foster greater empathy, earlier intervention, and better outcomes.


Note: Sickle Cell Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Sickle Cell Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to sickle cell disease.

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