The pain from a heart attack is so bad that – if you can imagine – it can feel like an elephant sitting on you. Patients with sickle cell disease, a genetic condition affecting the red blood cells, report that this kind of pain begins before their first birthday and continues intermittently for a lifetime.

Too often these people receive inadequate help managing their pain, a critical health disparity that persists 113 years after sickle cell disease was identified.

I am a professor of nursing, and my research focuses on managing pain in sickle cell and cancer patients. I also am a member of the Lancet Haematology Commission, which recently published a report showing paths forward toward worldwide health equity for patients with sickle cell disease.

Here are some key challenges and opportunities I have seen for helping sickle cell patients cope with this disease.

Sickle cell disease results from genetics – more specifically, the inheritance of faulty hemoglobin genes, one from each parent.

Hemoglobin is the iron-rich part of the red blood cell that allows it to carry oxygen from the lungs to all cells in the body.

But sickle hemoglobin – known as hemoglobin “S,” which is the faulty hemoglobin gene – causes the typically round red blood cell to be crescent-shaped, like a banana or a sickle. These sickle cells, which are sticky and very stiff, get trapped in tiny blood vessels and block blood flow and oxygen delivery to the body’s organs and tissues.

Not only does this cause pain, it also affects those organs and tissues near the blockages, including the eyes, kidneys, bones, lungs, heart and the brain, where damage can be so severe it causes a stroke. Patients may also have infections, particularly pneumonia and blood infections. Some men with the disease may have painful erections.

For patients with the disease, half of their red blood cells can be sickle cells. Typical red blood cells live for about four months, while sickle cells live for three weeks or less.

This means that patients with sickle cell disease do not have enough cells able to carry oxygen to meet their body’s needs. In the meantime, the slow replacement of cells causes anemia, which is characterized by a reduced number of red blood cells and extreme fatigue.

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