Sickle Cell Disease

Sickle cell disease is an inherited disease that causes a structural problem in the hemoglobin contained in a person’s red blood cells. Normally, red blood cells are shaped like concave discs. But patients with sickle cell disease have cells that take on the shape a crescent or "sickle” under low oxygen conditions. This causes a plumbing issue in the blood vessels. The abnormally shaped red blood cells can adhere to the walls of blood vessels, and may build up over time to create blockages. These blockages prevent the red blood cells from doing their primary job: delivering oxygen to body tissues. Without oxygen, the cells in bones and organs begin to die. This process causes pain, which is sometimes very severe.

Symptoms of sickle cell disease usually begin when a child is four months of age or older. Pain episodes caused by oxygen shortages to organs or tissues are among the most common symptoms of sickle cell disease. These episodes are called "pain crises.” Pain severity and location vary; it can occur in the bones, chest, abdomen and other areas. Other symptoms of sickle cell disease include fatigue due to anemia, which occurs when sickle-shaped red blood cells break apart and die before they can deliver oxygen to the body; delayed growth; and frequent infections. Because sickle cell disease is a chronic condition, these symptoms may continue into adulthood. Pregnancy can also be challenging for those diagnosed with sickle cell disease.

Sickle cell disease is a lifelong health condition and thus most treatments are also lifelong. Some therapies actually help prevent associated problems; these include antibiotics or an oral daily medicine, which can reduce the frequency of pain crises. Blood transfusions may be part of regular care for patients with sickle cell disease as well.

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Research is underway at University of Minnesota Health to find better ways to treat and manage sickle cell disease. Current research is focused on the mechanisms of pain in sickle cell disease and pain management medication (like opioids, cannabinoids and other evolving therapies). Kalpna Gupta, PhD at the University of Minnesota is conducting research to improve quality of life by finding new ways to reduce chronic and acute pain related to sickle cell disease. She has earned multiple research grants from the National Institutes of Health and other similar funding agencies, which has helped us become a leader in the development of new treatment strategies for sickle cell disease.

A team of researchers are also testing new therapies to prevent sickle-shaped red blood cells from sticking to the walls of blood vessels. These therapies and new anti-inflammatory drugs may prevent blood vessel blockage, which causes organ damage and pain among sickle cell disease patients.

A blood and marrow transplant is the only option for curing the disease. This procedure replaces the cells that make your blood with healthy cells. The transplant procedure has many side effects, so it is usually only done in severe cases and when people meet certain health criteria. Early diagnosis is crucial for proper management of the disease.