Ray was a man of African descent in his late 30s. He was a married electrical engineer with three children. As a child in Africa, he’d been diagnosed with sickle cell trait. Sickle cell trait is a condition where the person carries one sickle hemoglobin gene. It shouldn’t cause severe pain or big problems.
People with sickle cell disease, on the other hand, inherit sickle hemoglobin gene from both parents. It’s a serious condition where hemoglobin and the red blood cells that carry it are sickle shaped. When the sickle shape cells block small blood vessels in the spleen, lungs, and elsewhere, it causes an extremely painful sickle cell pain crisis. A sickle cell pain crisis requires urgent medical attention because it deprives the person of oxygen to vital organs and tissues, makes it difficult to breathe, and can cause permanent damage. Read more about sickle cell disease: diagnosis, treatment, and medical malpractice.
Strangely, for a person who was told he only had sickle cell trait, Ray had two or three sickle cell crisis episodes every year. Over-the-counter Tylenol always alleviated the pain, but on one occasion it didn’t.
That’s why Ray, and his wife, Christy, went to a suburban Houston, Texas emergency room (ER) for help. They explained how Ray had severe pain in his chest, lower back, and left shoulder. They also shared his previous diagnosis of sickle cell trait in his experience of having occasional sickle cell pain crisis episodes.
Unfortunately, throughout his life, Ray’s doctors only treated his symptoms and never explored further. This same pattern of inattention continued in this ER encounter, with devastating results. According to many hematologists, who are medical experts on sickle cell and other blood diseases, this substandard, poor care happens to black patients all the time.
The ER doctor ordered two different narcotic pain medications (Morphine and Dilaudid) to dampen Ray’s pain. He ordered a chest x-ray that showed abnormal consolidation in the lungs. The nurses noted that Ray had a fast respiratory rate, and that his oxygen saturation dropped to 88% (normal is 95%+). Blood work done by the hospital laboratory showed Ray’s hemoglobin, hematocrit, and platelet levels were low.
The severe pain and all of these additional telltale signs pointed to the fact that Ray didn’t have sickle cell trait, he had sickle cell disease. And he was in a serious crisis that needed immediate medical attention to save his life.
Instead of admitting Ray to the hospital and consulting a hematology specialist, the ER doctor decided to discharge Ray to home with a prescription for another pain medication. Sadly, the same biased and bad decision to treat the symptom, not the condition, continued in Ray’s medical care.
Overnight at home, Christy saw Ray’s oxygen saturation plummeted to 46%. When a person’s oxygen saturation level drops below 90%, doctors and nurses usually pay attention. Below 80%? They get alarmed. Christy took Ray back to the same hospital ER, where they quickly recognized that his oxygen saturation was critically low.
The new ER doctor concluded that Ray probably had life-threatening acute chest syndrome, which is caused sickle cell disease. He ordered intravenous (IV) antibiotics, aspirin, and supplemental oxygen and then arranged transfer to a larger hospital for a higher level of care.
At the new hospital, they tended to Ray’s emergency conditions and placed him on a ventilator to help him breathe. A hematologist ordered a red blood cell exchange transfusion. This is the standard and definitive treatment for a patient with acute chest syndrome caused by sickle cell crisis.
As the name of the treatment suggests, a red blood cell exchange transfusion involves removing a patient’s sickle red blood cells and replacing them with regular red blood cells. This immediately increases the patient’s oxygen-carrying capacity and produces fast results.
Unfortunately though, the treatment was too late. By the time Ray got to the new hospital, permanent ischemic damage (from a lack of oxygen) to his vital organs had occurred. He went into cardiopulmonary arrest and died. We work with many patients whose care has been compromised due to racial bias. See our article about the potential role of race in stroke care for black patients.
How could this sickle cell related death have been avoided?
Ray’s death could have been easily avoided if the medical team properly communicated and adhered to the standard of care. The attending nurse neglected to inform the doctor of Ray’s low oxygen saturation levels and didn’t inform the doctor before putting Ray on an undocumented amount of supplemental oxygen.
Even though Ray’s labored breathing, critical vital signs, and chest pain were all indicative of acute chest syndrome, the doctor never explored acute chest syndrome as a potential diagnosis. The doctor’s premature discharge of Ray without any real treatment ultimately led to his death. Racial bias in healthcare also exists in pregnancy-related cases. See our article about how black women make up a large percentage of the cases when the Texas maternal/mother pregnancy-related death rate jumped 87%.
What is the difference between sickle cell trait and sickle cell disease?
Sickle cell trait is an inherited, recessive disorder that causes a small ratio of blood cells to have an abnormal sickle or “C” shape because one hemoglobin gene is affected. Sickle cell trait does not typically have a severe negative effect on people because they still have enough normal blood cells and sufficient hemoglobin levels to function.
In contrast, sickle cell disease is a life-threatening condition that affects both hemoglobin genes and therefore compromises most of the body’s hemoglobin. People with sickle cell disease will have mostly stiff, sticky sickle-shaped blood cells that can block blood flow and lead to serious complications like anemia, eye damage, kidney damage, sickle cell pain crisis, organ failure, and acute chest syndrome. One of the telling signs of sickle cell disease is if a person has ever experienced a sickle cell pain crisis, which is characterized by the sudden onset of sharp pain throughout the body that lasts several hours or even days.
What is acute chest syndrome and why is it linked to sickle cell disease?
Another common complication of sickle cell disease that is fatal if it’s not treated promptly is acute chest syndrome. This complication occurs in people with sickle cell disease because their defective sickle-shaped red blood cells will block blood vessels in the lungs, which blocks the flow of oxygen through the blood. This will lead to further pulmonary issues, like pneumonia. Unfortunately, in Ray’s case, his neglected acute chest syndrome was a major contributor to his death.
Why are people of African descent statistically more inclined to have sickle cell disease?
Sickle cell disease disproportionately affects individuals of African descent when compared to other ethnicities because the disease developed as a response to recurring outbreaks and mutations of malaria. Sickle cell disease is not exclusive to the African continent, though. Sickle cell disease is now found globally because of mass population migrations.
If you or your loved one have been impacted by medical malpractice or wrongful death because of medical mistakes of a hospital or doctor in Texas, contact a top-rated, experienced Texas medical malpractice attorney for a free strategy session and consultation about your potential claim.
* James Loo, a Painter Law Firm summer 2023 intern, helped in the research and preparation of this article.