Not Enough Doctors, Limited Pain Treatment For People With Sickle Cell Trait

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.

READ Hope for a Cure: Sickle Cell Disease

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.

Why Sickle Cell Disease Treatment Is Largely Unavailable For Adults

To all the other challenges of living with sickle cell disease, add the problem of not being able to find medical care. Until recently, it was relatively unusual for someone who has sickle cell disease to live to adulthood. In 2015, statistics showed that over 93 percent of children who had the disease lived past the age of 20. Even now, the average life expectancy for a man who has sickle cell is 53, and for a woman, it's 58. There are a few people with the disease now living into the 70's and 80's.

Because of the extremely short life expectancy of people who had the disease, sickle cell disease was largely handled by pediatricians. As a result, other doctors tend not to be familiar with it. This leads to an unfortunate cycle of events:

• Sickle cell patients lose their insurance at the age of 21, so they tend to rely on emergency room care.
• Emergency rooms triage their patients on the basis of the severity of their symptoms, and the primary presenting symptom in a sickle cell crisis is pain. Unfortunately, pain is not considered to be an "important" symptom, so sickle cell patients go to the end of the line.
• A sickle cell crisis does not cause changes in the lab values that are most commonly tested in the ER. The doctor may tell the patient "There's nothing wrong with you."

Emergency rooms simply don't run some of the tests that are important for diagnosing sickle cell crises. Patients can get sent home without any treatment at all. Not infrequently, the result is death. The highest rates of death from sickle cell disease in the United States occur during the first year people age out of children's healthcare programs. There are activist physicians who are lobbying for better care of sickle cell patients. But if you are the patient who has sickle cell disease, what can you do?

READ Sickle Cell Anemia: Symptoms, Diagnosis And Treatment

• If at all possible, get health insurance coverage so you can get the follow up care you need.
• File with Social Security Disability not just for the income, but also for (after two years) Medicare coverage.
• If you have to use emergency rooms for your care, find an emergency room that treats you with respect and gives you care and then treat them with respect and consideration. They are your lifeline.
• Make sure people who would take you to the ER are familiar with symptoms of "silent stroke." Symptoms may include personality changes, memory problems, changes in the way you walk, changes in the way you talk, and issues with sight and hearing. This problem is more common in children receiving blood transfusions but it can affect people with sickle cell disease at any time. Then make sure they know to speak up if they see those symptoms in you.
• You almost certainly need a relatively long list of medications. Almost no one who has sickle cell disease can afford their medications. See a medical social worker to get on drug company medical assistance programs. Most public hospitals will have one or more people who do this full time.