A 5-year-old African American boy presents to the emergency department with left leg pain. His leg is exquisitely tender to palpation….
If I read this vignette in the first year of medical school, I would have navigated to the multiple-choice answers to select anything related to sickle cell disease. The question writers are stating that the patient is Black, young and has a painful limb — this is not a difficult diagnosis. Rather than envisioning a little boy enclosed by a privacy curtain, I would try to picture the exact page of my heavily annotated copy of First Aid for the USMLE detailing the genetics, treatments and complications of the disease. However, as I started my third-year clerkships, my eyes found a different focal point of that vignette: exquisitely tender.
I remember meeting my first patient with sickle cell disease on the internal medicine wards. I rode the elevator down to the Emergency Department to get a detailed history, confident that this would be a “slam dunk” presentation on rounds. I knew what to do for sickle cell patients. From a third-year’s perspective, the formula was easy: fluids and pain control.
In the room, I found a pleasant middle-aged Black woman. Within minutes of meeting me, she told me she was proud of me and all I had accomplished. As I continued to speak with her, she reminded me of one of my aunts with a similar ability to be witty and humorous even when in pain. She recounted how her sister died during a vaso-occlusive episode several years earlier and how she did everything in her power to keep her sickle cell disease controlled. As I began to examine her, I understood why a clinician would use the word exquisite to describe a person’s pain. Just as Merriam-Webster defines it, her pain was “acute” and “intense.” Even the slightest palpation of her affected limb brought tears to her eyes.
Over the course of her hospital stay, the intravenous fluids and multimodal pain control regimen began to ease her severe nausea; slowly her pain began to subside. She regained her appetite and felt well enough to leave the hospital. Yet, it was not lost on me that despite regularly following up with her primary care physician and hematologist, despite regularly taking her hydroxyurea, some external stressor still caused her to experience this exquisite pain.
On almost every rotation that followed, I continued to see patients who carried the sickle cell diagnosis. I saw these patients suffering on pediatrics, surgery, and obstetrics and gynecology rotations. At times, I heard the stereotypical judgments made about these patients. Their motives for seeking medical care were often questioned, receiving the label of “opioid seeking.” I realized that those who made these judgments had not seen patients with sickle cell disease outside of the hospital or in a context outside of their crises of exquisite pain. They did not seem to value that pain crises may be unavoidable, as a circumstance of changing weather, infection or socioeconomic barriers that prevent patients from affording preventative medications.
As a Black woman, I felt that I was exempt from the biases that my non-Black counterparts applied to this patient population. What I failed to realize was that I was viewing sickle cell patients through the same limited scope, hyper-focused on their experiences of pain. In this country alone, 90,000 to 100,000 individuals suffer from sickle cell disease. These individuals, most of whom are Black, lead lives that are complex and punctuated by these instances of exquisite pain. As medical students, we learn about the preventative medications that have increased the life expectancy for these populations over the last four decades and develop a foundation to manage acute pain crises. While the progress is encouraging, we allow ourselves to be impressed by our own progress and become complacent. I would like to argue that we cannot be satisfied.
Sickle cell disease can lead to a breadth of medical complications in the domains of multiple specialists. Autoinfarction of the spleen leads to increased susceptibility to Pneumococcal species. These individuals are at susceptible to strokes, pulmonary emboli and higher risk pregnancies. It is clear that they not only deserve but need a comprehensive health care team to provide appropriate longitudinal care. However, when compared to counterparts with genetic diseases such as cystic fibrosis or hemophilia, which primarily affect individuals of European ancestry, sickle cell patients receive both less primary and less specialty care.
These disparities in health care mirror the socioeconomic barriers that patients with sickle cell face in daily life. As a new generation of physicians, I believe we have a duty to consider the whole context of our sickle cell patients. We have to consider their family members who watch their loved ones combat this disease. We have to consider their careers, their education, their hobbies and how this disease can bleed into all areas of their lives. We have a duty to demand more research and funding for better treatment of these patients in any specialty we choose, as this diagnosis carries implications for almost every organ system.
Sickle cell disease is more than a couple of sentences in a clinical vignette, and it is more than an experience of exquisite pain. My patients with sickle cell disease have taught me a great deal over the course of my clerkships, and in return, I owe them activism and advocacy. Now that I have finished the last exams of my medical school career, I look at the vignette above differently. I think beyond the pages of First Aid, beyond his exquisite pain. I think about the boy’s grandmother sitting nervously in the corner of the patient’s room. I think about how scared a five-year-old feels in a busy, loud emergency room. I think about how many days of kindergarten he will miss if his pain is not managed quickly. I try to envision the whole context of this patient, and only then can I begin to address his exquisite pain.