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One of the most common of all sickle cell anemia symptoms is intractable pain, but in the United States there just aren't enough doctors who know how to treat it.

When Bernard Fields was young, he thought doctor visits were fun. There were coloring books, and toys, and TV. He had to visit the doctor nearly weekly because of sickle cell anemia, but because of his state's children's Medicaid program he never had a problem getting in to see the doctor.

Then Bernard turned 21, and he was no longer covered by the Children's Health Insurance Program. That didn't make his sickle cell anemia symptoms magically go away. Bernard was too sick really to get a job, but he wasn't on welfare, either. Because there were no Medicaid or Obamacare options for him in his state, Bernard had to start relying on referrals from a charity clinic. Weekly visits to the doctor became visits every six months. Bernard became sicker and even less able to work and less able to get needed treatment. Bernard's story is far from unique.

What Is Sickle Cell Anemia?

Sickle cell anemia, also known as sickle cell anemia, is a hereditary disease that causes distortions in the hemoglobin molecule, which carries oxygen through the bloodstream. The condition is most common among people of African or Mediterranean ancestry. When someone inherits just one of the genes that causes the condition, known as sickle cell trait, there is actually a health advantage, or at least there was before modern times. Relatively small changes in the hemoglobin molecule protect against malaria, which perpetuates itself inside the body inside red blood cells. When someone inherits two copies of the sickle cell gene, there are distortions of the hemoglobin molecule that make it "sticky." Blood flows poorly throughout the body. When red blood cells are deprived of oxygen due to poor circulation, they take on a sickle shape. These elongated sticky blood cells can slow down circulation even more.

What Are Some Sickle Cell Anemia Symptoms?

Sickle cell symptoms are numerous. Just a few of the most common problems include:

  • Anemia. Everyone who has sickle cell disease has anemia. Their bodies can compensate for lack of functioning red blood cells by accelerated heart rate, but people with the condition tire easily.
  • Sudden, severe pain. When red blood cells accumulate in the bones and joints, they can cause intense pain. It's more common for babies with sickle cell disease to have pain in fingers and toes, but excruciating pain crises result from involvement of the long bones.
  • Stroke. Over 30 percent of people with two copies of the sickle cell gene have strokes before the age of 20.
  • Parvovirus. Humans, like their pets, can be infected with parvovirus. In most people, it causes at worst a mild fever, maybe a rash, and a few days of fatigue as the virus attacks the cells in bones that make red blood cells. When people who have sickle cell disease are infected with parvovirus, their already limited capacity for red blood cell production can lead to an aplastic crisis.
  • Other infections. Strep bacteria use the breakdown products of red blood cells to build protein shells that enable them to escape the immune system. This causes strep infections to be especially severe.
  • Leg ulcers. Minor injuries can leave skin that doesn't heal because of poor circulation.
  • Gallstones. Sickle cell disease causes the destruction of red blood cells. The red pigment can accumulate in the gallbladder and cause stones.
  • Developmental deficits. Constant loss of red blood cells interferes with the ability to grow taller during childhood and adolescence, although it does not interfere with weight gain.

These are just some of the symptoms of sickle cell disease. Treatment of sickle symptoms with blood transfusions can cause even more problems with iron overload disease and enhanced risk of stroke and "silent" cerebral infarcts.

Continue reading after recommendations

  • Crosby LE, Quinn CT, Kalinyak KA. A biopsychosocial model for the management of patients with sickle-cell disease transitioning to adult medical care. Adv Ther. 2015 Apr
  • 32(4):293-305. doi: 10.1007/s12325-015-0197-1. Epub 2015 Apr 2. Review. PMID: 25832469.
  • Quinn CT. Clinical severity in sickle cell disease: the challenges of definition and prognostication. Exp Biol Med (Maywood). 2016 Mar 23. pii: 1535370216640385. [Epub ahead of print] PMID: 27013545.
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