According to a new prospective study of pain in adult patients with sickle cell disease, the prevalence and severity of pain is appreciably higher than previous studies had suggested.

In this latest study, researchers looked at the diaries of 232 patients; they all had sickle cell disease. The diaries recorded each patient’s pain – data on how they responded to pain by either using hospital emergency or unscheduled ambulatory care was also included.

According to previous pain studies, it was assumed that if a patient did not request medical attention or go to hospital he/she was not in pain. Those pain studies used the number of hospital visits to gauge frequency and severity of pain.

Study leader, Wally R. Smith, MD, wrote “The major finding of our study. was that pain in sickle cell disease is a daily phenomenon and that patients are at home mostly struggling with their pain rather than coming into the hospital or emergency department.”

You can read about this in Annals of Internal Medicine, January 15th issue.

Sickle cell disease, which affects approximately 1 in every 400 African Americans, is a group of hereditary red blood cell disorders. Sickle cell disease is also more prevalent in parts of the Mediterranean, the Middle East and India.

In patients with sickle cell disease, a mutation in the red blood cell gene changes smooth, round blood cells into C-shaped (or sickle-shaped) cells – these cells lose their suppleness and become stiff and sticky and have a tendency to clot within the blood vessels. When these get trapped in the smaller blood vessels, blood flow to the arms/legs and other organs in the body becomes blocked, causing pain, organ damage, and serious infection. The lungs, kidneys, spleen and the brain are particularly vulnerable to damage.

A patient with sickle cell disease generally has a shorter life-span than someone who does not have the disease, mainly due to the potential damage to organs.


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The only treatment that is FDA approved for sickle cell disease is the drug Hydroxyurea. There are other medications that can help with symptoms and complications. Also, bone marrow transplantations can help the patient.

Pain for a sickle cell patient can be rapid, severe, and short term (acute), or long-lasting (chronic). The acute episodes of pain are called ‘crises’. Previous sickle cell disease pain studies have generally focused on crises.

In this study, more than 50% of all the participants who completed their pain dairies for up to six months reported experiencing pain on most days – nearly one third of them said they experienced pain almost daily.

Dr. Smith said “I believe that this study could change the way people view the pain of the disease. It is a chronic pain syndrome. And the study results have implications for medical care, and research. We need more drugs to prevent the underlying processes that cause pain in this disease. And we need better treatments to reduce the chronic pain and suffering that these patients go through.”

“Daily Assessment of Pain in Adults with Sickle Cell Disease”
Wally R. Smith, MD; Lynne T. Penberthy, MD, MPH; Viktor E. Bovbjerg, PhD, MPH; Donna K. McClish, PhD; John D. Roberts, MD; Bassam Dahman, MS; Imoigele P. Aisiku, MD, MSCR; James L. Levenson, MD; and Susan D. Roseff, MD
Annals of Internal Medicine 15 January 2008 | Volume 148 Issue 2 | Pages 94-101
Click here to view abstract online

Written by – Christian Nordqvist