Pain from Sickle Cell Disease Prompts Discussion at U.S. Meeting

Nov 16, 2015

Participants at a November meeting of the American Society for Hematology identified chronic pain management as a significant unmet need among individuals suffering from sickle cell disease. Representatives of health care and research organizations concerned with sickle cell disease discussed ways to enhance cooperation and collaboration among the diverse array of groups, which included IASP.

The meeting focused on a guideline produced by an expert panel convened by the U.S. National Heart, Lung, and Blood Institute. Participants identified two vital areas where huge gaps exist:

  • Obtaining access for all patients to expert specialty care for acute and chronic health issues related to sickle cell disease. This is particularly important for patients in rural areas or locations where most treatment is given by primary care physicians and where local sickle cell disease expertise is lacking.
  • Increased awareness and advocacy in the scientific community of the large gaps in knowledge about sickle cell disease—and thus, the great need for far more research across multiple fields.  

In discussing the need for improved access, the group suggested empowering patients and their families with best-practice medical knowledge of what patients should receive when they suffer acute and chronic complications. This could be in the form of a smartphone app, a family-friendly guidebook, or a card with information about the patient’s condition that a primary care team could review.

In addition, participants suggested improved education for physicians and residents—specifically, online education materials, pocket treatment guides, telephone consultations with expert clinicians, and lunch-and-learn sessions for primary care residents with sickle cell clinical experts.

Participants also agreed that more sickle cell research is essential. Sickle cell disease is an “orphan disease” that affects approximately 300 million individuals worldwide. Yet enormous gaps remain in our scientific knowledge of the disease mechanisms, complications, comorbidities, and treatments. Research efforts should focus on such disciplines as hematology, vascular biology, immunology, genetics, and pain. Indeed, pain is a key component of both the acute and chronic complications of patients with sickle cell disease.

The major acute complications patients are fever, stroke, acute chest syndrome, and vaso-occlusive pain crises. The key chronic complications patients are chronic “all over” pain, avascular necrosis, and leg ulcers. The group repeatedly and unanimously identified chronic pain management as the greatest unmet need. Approximately half of adult patients with sickle cell disease develop chronic pain that often affects them daily.

While hydroxyurea is the frontline treatment for vascular aspects of sickle cell disease, it does little if anything to alleviate the chronic pain. Blood transfusions and bone marrow transplants also apparently do little to reverse the chronic pain, at least for a year or two. Opioid class analgesics are the mainstay pain treatment for both the acute vaso-occlusive pain and the chronic pain in sickle cell disease, but the multiple side effects of opioids present huge problems in this patient population. Therefore, non-opioid treatments for chronic pain are the greatest need in this patient population, and research on the underlying pain mechanisms in sickle cell disease is only currently at the tip of the iceberg.

IASP Press has published the definitive work on the topic, Sickle Cell Disease, Second Edition, by Samir K. Ballas.

— Cheryl L. Stucky, professor of cell biology, neurobiology, and anatomy, Medical College of Wisconsin, Milwaukee, Wisc.