The Day I Relearned the Sickle Cell Crisis

I frantically called the ambulance dispatcher for a third time to ask when the paramedics might arrive. I knew full well that I would receive the same answer: the ambulance service is very busy; we are aware of you; we don’t know when they will arrive. It dawned on me that I had never spent long with the same patient in a sickle cell crisis pain before and never outside of the hospital context. As we waited, it seemed as though time itself slowed down for Mary, the helpful but frightened hotel staff, and me.

Mary had joined me at a national hematology conference to speak on her experiences of pain as a person with sickle cell disease. After months of preparation, she traveled up from London to the conference in Glasgow, only to develop an acute painful crisis within hours of arriving in the city. This pain rapidly worsened and forced Mary to curl up on the floor of an unused reception room at the conference hotel, only minutes away from the stage she was due to speak from the following day.

I had spent several years caring for many patients experiencing painful sickle cell crises. I had taken some steps to try to address the lack of understanding and compassion that patients encountered from healthcare professionals, and this was the motivation for organizing the conference session. But the five or six hours I spent with Mary in Glasgow taught me that my own understanding of a painful sickle cell crisis was a shallow doctor’s-eye view, and a drastic relearning was required.

I had overlooked the “waiting” experience during a painful crisis: waiting for an ambulance, for traffic en route, for a triage nurse, for a doctor, for the analgesia to start, for the next dose, for the suffering to lessen. There is also a profound “impotence.” Here, I did not have my normal roles to hide behind: medicine-tinkerer, note-writer, and plan-maker; the next patient on my list already taking up some of my thought space. Instead, I was there as a fellow human being in a city unfamiliar to us both. Being present as a witness to suffering that I could not even try to prevent was far more draining and difficult than my usual professional role—how much more difficult for patients themselves or the family member who powerlessly witnesses such crises in a loved one regularly.

For much of the time, I sat (or paced) in silence. Sometimes I would ask again about the pain or express some hope that it would lift soon. Occasionally we would quietly pray together—our shared faith a focus during those long hours. I say hours because that was my experience on the first day, with two shorter visits back to the hospital in the following days. “Hours” was not Mary’s experience—she spent a further two weeks up in Glasgow, suffering continuous pain while surrounded by strangers.

Pain creates several obscuring barriers between the sufferer and the observer. One such barrier is language. The writer Elaine Scarry¹ notes that “physical pain does not simply resist language but actively destroys it, bringing about an immediate reversion to a state anterior to language, to the sounds and cries a human being makes before language is learned.” In another very concrete way, Mary’s pain had destroyed language. She had carefully gathered up thoughts and reflections in the months leading up to her talk, but these were now being simultaneously destroyed and corroborated by her crisis.

Another barrier is the perception of time. While, for me, time seemed to have slowed down, I can have no idea how slowly each second of pain was experienced by Mary. John Berger² notes that “the subjective experience of time is liable to be so grossly distorted—above all by suffering—that it becomes extremely difficult to correlate with time proper.” This barrier of time that separates the anguished person from the unafflicted “intimidates the imagination of the latter.” While we may have an increasingly sophisticated understanding of the underlying pathogenesis of a painful sickle cell crisis, the lived experience of it still escapes our understanding.

I sat by Mary’s bed on the high-dependency unit (HDU) as she tried to dictate sentences of her talk to me, fighting the invasiveness of her immediate pain and the drowsiness induced by analgesia. The HDU staff understandably interpreted our perseverance with the dictation as inappropriate and asked me to leave. In one sense, they were right: should a patient in crisis be asked to do anything apart from endure and wait? I wondered if they thought of me as callously focusing on the success of the conference session rather than her pain. But we were motivated by a shared desire “not” to let this crisis destroy Mary’s work and words. These words were precious, a rope to traverse the gulf of experience between the sufferer of pain and the clinician—to allow us to better care for the next person we meet in their own painful crisis.

The following day at the conference, my voice cracked as I read out the short fragments that Mary had part-dictated, part typed from her hospital bed:

“Not only is the physical impact of this pain difficult to bear, but like an octopus the tentacles of sickle cell stretch out into every aspect of life. So, sometimes I make plans and they fall through because of a sickle related issue. When I word it like that, it sounds so simple. But imagine agreeing to speak on something you are passionate about almost a year in advance. Booking time off work, preparing for your speech, booking your flight, hotel, etc, only to get to the destination and fall into crisis twelve hours before you are scheduled to speak. Well, that’s exactly what has just happened to me. Imagine this being something I experience frequently. Then comes the emotional pain of constantly feeling inadequate and constantly letting others down, both in my professional life and private life. Trying to structure a ‘normal’ life around these bouts of pain is quite difficult. And doctors can help reduce the severity of a crisis, and consequently the longevity of it, by listening to the patient.”

FOOTNOTE

Mary did not get to give her talk at this conference, but a few months later, she gave an interview about pain in sickle cell disease. The video recordings of this interview are available at https://b-s-h.org.uk/education/lectures-and-videos/patient-perspective-the-journey-of-pain-in-sickle-cell-disease/.

AUTHOR CONTRIBUTIONS

SH wrote the article. MA shared her direct experiences, wrote the quoted section, and reviewed the article.

DISCLOSURES

The authors have no conflicts of interest to disclose.