Essay: In A Lifetime Of Sickle Cell, The Evolution Of A Disease

Most sickle cell anemia patients do not live long enough to span generations of doctors. But Gladys Jacobs was around when I was a medical resident in the 1980s, and she is around now.

Her career — as a patient and an activist — demonstrates how the understanding of sickle cell disease has changed.

Gladyss condition was initially misdiagnosed, as was all too common for sickle cell cases in the early 60s. It was not until then, as the historian Keith Wailoo writes in Dying in the City of the Blues (University of North Carolina Press, 2001), that the disease found its way into the public consciousness.

When Gladys went to doctors complaining of joint aches, which were the common painful crises characteristic of sickle cell disease, she was met with skepticism. Doctors, she recalls, called her a faker.

Finally, at age 16, she went to a hematologist who diagnosed sickle cell anemia and a second blood disorder, beta-thalassemia. This combined condition may be less severe than pure sickle cell disease and probably helps explain Gladyss longevity. (She will turn 60 in two weeks.)

But one should not minimize her list of complications. They include hundreds of admissions for painful sickle cell crises, spleen removal, shoulder surgery followed by an abscess and degeneration of the hips that has required multiple operations and forced her to use a wheelchair.

On top of all this, Gladys suffered disrespect. Too many physicians and nurses assumed she was faking symptoms to get pain medication. The impression, she said, is that we are all addicts.

Doctors fear of promoting drug addiction led them to underprescribe drugs to sickle cell patients — for example, insisting on checking blood tests before giving pain medications. Gladys never really cared about the test results. I know how I feel, she said.

Race, as Mr. Wailoo shows in his book, was central to the conundrum of treating sickle cell patients, a vast majority of whom are African-American. As one hematologist conceded, delaying the administration of pain medications has a racial undertone.

The frustrations led Gladys and a dozen other sickle cell patients at what is now Columbia University Medical Center to form a self-help group there in the early 80s. Embodying the patient activism of the era, they sought to change health professionals perceptions about the disease. They were given a gratifying invitation from a Columbia internist and professor, Dr. Constance M. Park, to tell their histories to small groups of students.

Yet real change did not occur until medical centers like Columbia introduced pain medication services specializing in sickle cell. Enormous strides have been made in pain management, but work remains to be done. A study in the May 2007 issue of Academic Emergency Medicine reported that sickle cell patients seen in three emergency departments received pain medications 70 to 75 minutes later than recommended by the American Pain Society.

Eventually, the Columbia self-help group fizzled out. So Gladys was especially pleased last fall when her current Columbia physician, Dr. Delphine M. Taylor, asked her to speak to the entire class of 150 first-year medical students in the clinical practice class.

Gladys was daunted at first. Despite years of outspokenness, she is quiet and shy. In the past, she had met with just 15 students.

But she warmed to the task. In the question period, when a student asked what it was like to live with sickle cell anemia for almost 50 years, she answered with one word: Hell.

The students were moved. When I e-mailed them several months later to learn their impressions of the lecture, one student told me that she and others had just been discussing Gladys.

Another student, Solomon Woldu, wrote that Gladys was an incredibly resilient and strong-willed woman whose sense of dignity and positive outlook left many of us students with a sense of awe.

When Gladys finished speaking, she received a standing ovation.

Barron H. Lerner teaches medicine and public health at the Columbia University Medical Center.

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