Recent news headlines described a novel gene therapy and potential “cure” for sickle cell disease (SCD). These findings are truly groundbreaking and demonstrate the optimistic future for SCD therapeutics. In contrast, the current health care experiences of SCD patients are heartbreaking.
Many disparities exist in the way doctors manage SCD patients and their pain symptoms. Several factors have played a role, including physician comfort, knowledge, experience, and bias. Most recently, the misguided application of controlled substance policies that are intended for the general population to stem the opioid crisis, have created limits on SCD patients’ access to medications that are the mainstay of therapy in treating their painful episodes.
SCD is a genetic disorder that is defined by misshapen red blood cells. These misshapen red blood cells cannot perform their proper function of delivering oxygen to the body and vital organs. Patients not only experience debilitating pain but also fatigue and a cascade of complications. A compromised immune system causes susceptibility to infections like pneumonia. Multi-organ damage and strokes starting in childhood can also occur. Although a relatively infrequent occurrence for most of the general population, visits to a doctor’s office or emergency room, are common occurrences for SCD patients. Some adult SCD patients have been interacting with the healthcare system longer then some of their healthcare providers have been in clinical practice.
Individuals of Mediterranean, Middle Eastern, Indian and Latin descent are affected by SCD; yet, the disease largely affects those of African ancestry. SCD patients receive a lower standard of care and race may play a role in these disparities. Studies have shown that SCD patients have longer wait times in hospitals, receive lower doses of pain medication and medical professionals question or doubt reported pain levels in emergency rooms.
Patient care should be standard throughout the medical system, and many in the general population, and medical community assume that it is. But large discrepancies exist in all avenues of the medical system ranging from emergency room care, to outpatient care, to pharmacies. For example, one SCD patient working with one pharmacist for over six years never encountered an issue receiving her standard opiate regimen. After moving to another town, she was unable to receive her usual opiate regimen; the new pharmacist expressed a lack of comfort dispensing that same opiate regimen. As a result, she suffered a pain crisis and was hospitalized while her prescribing physician worked to resolve the issue with the new pharmacist. Sadly, her story is typical of the care SCD patients receive in the face of the government’s attempt to stem the tide of the opioid epidemic.
According to New York State Public Health Law, providers can only provide a seven-day supply of opiates for those in acute pain, with exclusions for patients with chronic pain, cancer, and palliative care regimens. A SCD patient’s acute pain flare-up does not fall neatly into these categories. Providers must exercise sound judgment to assess whether the acute pain crisis is due to a larger chronic disease and therefore requires more than a seven-day supply, as it frequently will with SCD. Providers also need additional training in SCD treatment, as many do not realize that it currently falls under a palliative care regime. Thus the seven-day supply limit should not apply to patients with sickle cell disease.
The efforts to limit the supply of narcotics have had unintended consequences. In order to avoid opiate overprescribing, medical prescribers are encouraged to limit the distribution of narcotic medications. Providers should not adopt policies that ultimately punish vulnerable populations such as those born with SCD. It is a condition that is prone to lifelong pain with acute episodes overlying the chronic state. As an analogy, the medical system would not limit the pain medication provided to a third-degree burn victim. The provider’s analysis of the injury and necessary care would have her conclude that the burn will not heal in seven days; thus, the patient would require an additional supply of pain medication. Some healthcare professionals need prescribing limits imposed on their practice. But, prescribers must also use clinical judgment and common sense. They must recognize that prescribing opiates to those with acute pain is not as simple as applying a rule in black-or-white, especially when these rules create unintended consequences. Policies and guidelines are made “for all” but can harm those patients who meet exceptions to the rule and who are sometimes the most defenseless and least able to fight for themselves.
The healthcare delivery model for SCD patients must be comprehensive, adjustable to the needs of the patients, and most importantly, empathic. Providers must understand that SCD patients ultimately suffer in three ways — the pain and complications of the condition, the maltreatment from healthcare providers, and the unintended consequences of reduced opioid prescribing policies. It is important to realize that NY State recommendations do not apply to those who fall under palliative care such as those with SCD. Healthcare professionals can work closely with sickle cell community-based organizations (CBO) to improve the experience for SCD patients, advocate for increased government funding for the disease but also to lobby NYS legislature to list SCD as an excluded condition under the law.
The promise of a cure for sickle cell is exciting. Cautious optimism is warranted as the benefits may be years down the line. In the meantime, providers should consider how current pain policies affect this vulnerable population. Providers must use their judgment and not their biases when treating SCD patients to live up to the physician’s creed of “Do No Harm.”
Dr. Tartania Brown is a palliative care specialist who has sickle cell disease. She works for Metropolitan Jewish Healthcare System. Dr. Lyndonna Marrast is a physician-researcher and an advocate for sickle cell patients. She works for Northwell Health.