A teaching case, told alongside the paintings of a man who spent his life making this pain visible — and who died of it at forty-eight.
Christopher Montgomery, MD, MPH · Department of Emergency Medicine,
University of California Los Angeles
Artwork by Hertz Nazaire (1973–2021)
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Sickle cell disease is a manageable condition that many people live with and survive. What proves most lethal is not the sickling. It is being disbelieved.
Everything below comes from one lecture, and the lecture came from one death. It is here in full, free, and downloadable, because the corrections it contains are not difficult — they are simply not taught often enough.
It's the pain of a broken spirit that hurts more than the physical pain. It's the pain of not being believed at school, at work. It's the pain of not being believed at the hospital. It's the pain of the choice to stay at home and suffer, rather than go to a local hospital and be treated like an addict. That's what breaks you. Hertz Nazaire
Hertz Nazaire was a Haitian-American artist who lived with sickle cell disease his whole life and made it the subject of his work. His paintings are not illustrations of a disease. They are testimony — and they do in one image what a chapter of a textbook cannot.
Every clinician asks a patient to rate their pain from zero to ten. This is what ten looks like from the inside — and it is the number most likely to be disbelieved.
There are no reliable, objective indicators of acute sickle cell pain. No blood pressure, no oxygen saturation, no hemoglobin, no reticulocyte count. The gold standard is the face in this painting telling you.
A parent bends over a child. Behind them, a Punnett square — AS, AS, SS — the arithmetic of inheritance that decides a life before it starts. The outline of Africa. Two candles.
The geography is not decoration. Sickle cell trait gives near-complete protection against severe falciparum malaria, which is why the gene is common across sub-Saharan Africa, the Middle East, India and the Mediterranean — and why the slave trade carried it to the Americas and Western Europe.
The transition from fetal hemoglobin to hemoglobin S is complete by nine months. Sickling — and pain — begins in infants.
Hundreds of marks, counted in fives, in blood red on black. Crises. Admissions. Nights. Doses asked for and refused.
Marcus, the patient in this case, had fifteen emergency department visits in six months. In his record, that number was not read as a tally of suffering. It was read as a behavior pattern, and written down as frequent flyer.
A hand reaches up out of a bed of red cells. The blue crescents drifting around it are the sickled cells — beautiful, and the reason the hand is reaching.
Nazaire gave the two titles that open this case study. Hope, and need not suffer alone. He meant them as instructions.
Images of Hertz Nazaire's work are reproduced here for education and awareness, in the spirit in which he shared them. Rights remain with the artist's estate.
Each of these was posed as a question to a room of emergency physicians. Try them before you read the answer.
Which of these is a reliable, objective indicator of acute sickle cell pain? Elevated blood pressure · oxygen saturation below 92% · hemoglobin below 8 · reticulocyte count above 15% · all of the above · none of the above.
None of the above.
Patient self-report is the gold standard of pain measurement. The most persistent misunderstanding is reading hemoglobin as a guide to pain — but pain comes from hemolysis, hyperviscosity, allodynia and neuro-inflammation, all distinct from the hemoglobin level.
Yang F, Smart Health, 2019
How long does a vaso-occlusive crisis typically last?
It varies — but it has a shape, and most patients arrive days into it.
This matters at the bedside for one reason: a patient who has been in phase 3 for four days has not slept in four days. Immense pain prevents sleep. When the pain is finally controlled, they sleep — and sleeping is then misread as evidence they were never in pain.
Jang T, J Transl Med, 2021
What is the average life expectancy for a person living with sickle cell disease in the United Kingdom, the United States, Nigeria, and California?
Same genotype. Same pathophysiology. A forty-six-year spread. Nothing in the biology explains that gap; everything in the systems around it does.
Gardner K, Blood, 2016 · American Society of Hematology, 2023 · California Institute for Regenerative Medicine · Ibemere S, Blood, 2023
Heuristics in medicine are mental shortcuts. They enable rapid decisions, simplify complexity and reduce cognitive load. They also reinforce bias, oversimplify what is in front of us, and produce cognitive distortion.
"Sickler" has undergone a connotative shift. It now carries stereotypes of health, race and socioeconomic status, in a disease where more than 90% of patients in the United States are Black — attaching the unconscious bias of structural racism to a patient at the exact moment they are at highest risk of a poor outcome.
Among 655 emergency physicians across 49 states, there was a statistically significant relationship between negative attitudes toward people with sickle cell disease and use of the word.
Martin K, JGIM, 2024 · Glassberg J, Am J Hematol, 2013
Clinical pearls from Dr. Elliott Vichinsky, Dr. Robert Ward Hagar and Dr. Alexis Thompson.
Acute severe pain from tissue ischemia, caused by sickled cells obstructing microvascular flow. Any body part, commonly long bones, chest and abdomen.
Prompt aggressive analgesia, appropriate hydration, identify triggers, assess for complications. Leukocytosis above 20,000 with a left shift may indicate infection.
Duroseau Y, JACEP 2021 · Ojo AS, J Clin Med Res 2023
A new pulmonary infiltrate plus at least one of fever above 38.5 °C, cough, wheezing, tachypnea or chest pain. The leading cause of death in HbSS.
Often develops one to three days after admission for a pain crisis. Up to 30% infectious — Chlamydia pneumoniae in adults, Mycoplasma in children. Early antibiotics, oxygen to keep saturation ≥95%, transfusion with hematology.
Vichinsky EP, NEJM 2000 · Alghamdi FA, Sci Rep 2024
Sudden cessation of red cell production with reticulocytopenia — most often parvovirus B19. Hemoglobin falls to 6–9, reticulocyte count below 100,000/μL.
This is why the reticulocyte count matters. High tells you cells are being destroyed; low tells you they have stopped being made.
Serjeant GR, Lancet 1981 · Rees DC, Br J Haematol 2003
Up to 10% of deaths. Lifetime risk of overt stroke 25–30%. Children with sickle cell disease have 333 times the stroke risk of healthy children.
Transfusion within two hours of symptom onset; tPA can be given safely if the patient qualifies within 4.5 hours. Treatment decisions should not delay transfusion.
DeBaun MR, Blood Adv 2020 · Adams RJ, Stroke 2017
Thirty-five. Sickle cell disease, HbSS. Avascular necrosis of the left hip from recurrent osteomyelitis. Asplenic. Wheelchair-bound since March. Grew up in foster care. Transitional housing that left him three blocks uphill from the bus stop.
Fifteen emergency department visits in six months for pain crises. In the record: drug seeking · frequent flyer · opioid dependent and tolerant.
He arrived with right leg pain — different from his usual pain, which is the left. Ten out of ten, sharp, no relief from his home medications, no sleep for days, new numbness in the left leg. He told the team exactly what works for him, because he had a plan and he knew it.
Two rounds of pain management. His physician gave 1 mg of hydromorphone each time, never the 2 mg he asked for. He was awake and on his phone, then asleep and easily roused.
He reported new chest pain and asked for more medication. He swore at the nurse.
A rising temperature. A falling saturation. A rising respiratory rate. New chest pain, one to three days into an admission for a pain crisis. Every criterion for acute chest syndrome was on the board.
Because of this "behavior," Marcus was asked to leave the emergency department. It was documented that he had oral pain medication at home.
Brought in by EMS. His girlfriend called the ambulance. Unresponsive, bilateral crackles, hypotensive and tachycardic with weak distal pulses. GCS 5. Intubated. Point-of-care hemoglobin 5.8. Intranasal naloxone administered.
Transfused two units, started on ceftriaxone and vancomycin, central and arterial lines, norepinephrine. CT without pulmonary embolism; bilateral perihilar ground glass and consolidation, right greater than left.
Transferred to the medical ICU in "stable" condition. He died two days later of multi-organ failure caused by acute chest syndrome.
Autopsy found bone marrow infarction with possible osteomyelitis in the right femur, and lipid-laden macrophages in the bronchoalveolar lavage fluid — fat embolism. Infection is the most common cause of acute chest syndrome, but 9–11% is caused by bone marrow or fat emboli.
He said the pain was different. He said it was in the wrong leg. He said his chest hurt. Every one of those was clinical information, offered freely, by the only instrument that can measure it.
Sickle cell disease in the United States is more than a genetic condition. It is a construct shaped by systemic racism, social inequities, and structural barriers that magnify its impact on Black lives. It is not the sickling alone that kills, but the compounded effects of neglect, discrimination, and the failure to prioritize equitable health care. From the expert discussions
Thirty-nine slides, with the case, the clinical corrections, the expert pearls and the full reference list. Click any slide to read it full size, or take the file and teach it yourself — no attribution required beyond the artist's.
Co-founder and science administrator of Axis Advocacy. The fourth generation of mothers in her family to have a child born with sickle cell disease — a fact that has shaped a life of commitment to improving care and quality of life for those affected.
An early supporter of transformative therapies including bone marrow transplant and the stem cell research pioneered by Dr. Kohn, she has spent recent years as a stem cell activist, championing trial funding through the California Institute for Regenerative Medicine, speaking nationally and internationally, and lobbying for initiatives like the Sickle Cell Education Act.
October 2, 1973 — October 29, 2021
He passed away from complications of sickle cell disease at the age of forty-eight — the United States average, almost exactly.
He spent his life painting what could not be measured, so that someone would believe it. This page exists because he did.
Hope. Need not suffer alone. The two titles that open this case study