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Lessen the Stigma Around Sickle Cell Disease

In the world of health care, it is crucial for professionals to understand the unique challenges faced by patients with sickle cell disease. Beyond the physical symptoms and complications, these individuals often endure a silent battle against the stigma surrounding their condition. With a higher pain threshold and the ability to carry out daily tasks despite chronic pain, they are often met with skepticism from health care workers who struggle to comprehend their level of suffering. Moreover, the emotional toll of witnessing their loved one’s struggles and grappling with their own mental health issues further exacerbates their already complex situation. Dr. Sunny Singh, hematologist and oncologist at the University of Arkansas for Medical Sciences in Little Rock, helps shed light on the stigma and discusses the importance of empathy and support from health care workers.

Sickle Cell Disease Starts with a Faulty Gene

People who have sickle cell disease inherit two faulty hemoglobin genes, one from each parent. The faulty hemoglobin gene creates red blood cells that are sickle-shaped instead of disc-shaped. These sickle-shaped cells are sticky and attach to the walls of blood vessels more easily, causing blood clots in multiple areas of the body. Some people only have a sickle cell trait, which occurs when they inherit one normal hemoglobin gene from one parent and one faulty hemoglobin gene from the other. While people who have sickle cell trait are generally healthy, they can pass on their one faulty gene to their children.

Many of these patients have suffered in silence at their homes for two or three days, have tried taking medications, tried relaxation and other non-pharmacological means. Those have all failed.

On average, around 100,000 people in the United States have sickle cell disease. It is estimated that, in the U.S., most people who have sickle cell disease identify themselves as Black. Around 1 in 13 Black or African-American babies are born with sickle cell trait. 1 in 365 Black or African-American babies are born with sickle cell disease. People who come from Hispanic, Southern European, Middle Eastern, and Asian Indian backgrounds also have an enhanced risk of sickle cell disease.

Chronic Pain is the Norm

“Patients with sickle cell disease have been in pain since their childhood,” Dr. Singh says. “They may also have one or both parents who also have sickle cell disease and have watched them suffer through the pain as well. Perhaps one of their parents or other relatives has died from sickle cell disease,” he says. This means that they may have grown up in a family dynamic full of chronic health issues, pain, socio-economic struggles, and psychological stress that stems from this condition.

A recent study revealed that 35.2% of adult sickle cell disease patients reported that they had been diagnosed with depression. Depression was found to be associated with more frequent hospital visits, more emergency room visits, and more frequent blood transfusions. More frequent visits mean a higher overall cost of care for these patients. In fact, sickle cell disease patients who have depression pay twice the amount of health care costs than those with sickle cell disease without depression. Recurrent pain, repeated hospitalization, and organ damage related to disease complications can all negatively impact health-related quality-of-life outcomes in patients with sickle cell disease.

“When you think about the anxiety and depression, multiple family members suffering from chronic pain, and then the actual physical symptoms the patient experiences all lumped together, it’s easy to see how life can become miserable,” Dr. Singh says. “There are also a lot of presumptions and biases associated with sickle cell disease that make things even worse for patients seeking care.”

Practitioner Bias

Sometimes, providers are not able to build a rapport with patients who have sickle cell disease, often due to a belief that the patient is not actually in pain. “A patient may be on their phone while reporting a 9 out of 10 pain level, and the provider may think, ‘How can you be at 9 out of 10 pain and be able to use the phone?’” These patients have a much higher threshold when it comes to reporting pain and their ability to go about their lives with severe pain, Dr. Singh adds.

Additionally, there are many providers in emergency departments who may think that patients who are willing to wait eight hours to be seen at the emergency department are only there to seek drugs. Dr. Singh says that is far from the truth: “Many of these patients have suffered in silence at their homes for two or three days, have tried taking medications, tried relaxation and other non-pharmacological means. Those have all failed. Now, they’re coming into the hospital because it’s the only option they have left. It’s a cry for help.”

There are many presumptions and biases surrounding sickle cell disease. Patients often face accusations that they are liars and that their pain is not as severe as they say it is. When patients see a provider or visit the emergency department, some may assume that they are doctor-shopping and trying to get more drugs. “Do we have patients who end up not following our recommendations in terms of using medications appropriately in accordance with the agreed-upon plan? Yes, we do, but that is not the majority, and this happens with any disease condition,” Dr. Singh says. “Five to 10 percent of any patient population will be challenging due to various reasons. That is applicable to patients with cancer, heart problems, strokes, and sickle cell anemia. Out of 100 patients, on average, 10 will be challenging.”

When you think about the anxiety and depression, multiple family members suffering from chronic pain, and then the actual physical symptoms the patient experiences all lumped together, it’s easy to see how life can become miserable.

It is also important when treating a patient with sickle cell disease to keep in mind that there are social factors that also affect their health. Many come from a socio-economically deprived background and suffer from anxiety, depression, and other psychiatric issues. Poor mental health results in poor self-care, which results in more frequent visits to the doctor, which results in greater costs of care. All of this repeats in a destructive cycle that neither improves outcomes nor improves the quality of care in the community.

The Fine Line for Treatment

Sickle cell experts like Dr. Singh often refer patients to psychiatrists and behavioral health specialists who can prescribe the medications so that the patient feels better. Dr. Singh adds, however, that treating patients with sickle cell disease is not easy. “When you have opioids involved, it can have a negative impact on the neurocognitive status of the patient.” Over time, opioids heighten sensitivity to pain and can affect cognition. Because patients with sickle cell disease often rely on opioids to alleviate their pain, providers must be careful when prescribing them and do it under a pain contract.

When it comes to treatment, Dr. Singh recommends that primary care providers who see patients with sickle cell disease refer patients to a more comprehensive sickle cell center, such as UAMS sickle cell clinic, for a comprehensive analysis. “The patient may need to be seen at the center once a year so that we can do a detailed assessment while continuing to see their regular primary care provider in the community,” he says. Sickle cell experts will ensure that the care provided by the patient’s provider doesn’t conflict with the care needed to treat sickle cell disease. “We foster a collaborative spirit with the patient and their provider to ensure that we are on the same page when it comes to managing their sickle cell disease.”

With proper treatment, most patients’ pain will be well controlled. “Very soon, we will have more advanced therapies, such as gene therapy, approved, and that should further improve outcomes for sickle cell disease. We already have three new drugs that were recently approved by the FDA, which do improve symptoms for our patients, but unless a primary care provider refers the patient to a specialist, they may never be accessible to the patient.”

In order to provide the best care possible, it is essential to understand the stigma surrounding sickle cell disease and the challenges patients face on a daily basis. It is important for health care providers to recognize and validate patients’ chronic pain and mental health struggles. By acknowledging social factors that also affect a patient’s health outcomes, providers can create a more supportive and empathetic environment for patients with sickle cell disease. A collaborative effort to break the stigma, improve access to care, and enhance the overall well-being of those living with sickle cell disease will prove effective in creating a better quality of life for patients that live their whole lives in pain.

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