Sickle Cell: Watch for Disease Chameleons

September 14, 2016
Jeannette Y. Wick, RPh, MBA, FASCP

When SCD patients' presentation accelerates, clinicians need to look further, and consider endocrine problems as possible causes.

Sickle cell disease (SCD) affects roughly 100,000 Americans, and causes production of an abnormal hemoglobin molecule that polymerizes into long chains when deoxygenated. The resultant red blood cells have a characteristic sickle shape, and occlude the vasculature—causing acute and chronic pain—and burst, causing anemia.

When SCD patients present with pain, most clinicians don't look for zebras; they see horses and assume the SCD is causing pain secondary to vasoocclusion. A manuscript published in Case Reports in Medicine reminds clinicians that from time to time, it's critical to ask, "Is something else going on here?" when SCD patients experience chronic pain and acute exacerbations.

The authors call attention to systemic disorders, including some endocrine disorders, which are associated with systemic pain and may occur as comorbid conditions with SCD.

They describe 2 SCD patients with pain that seemed to differ from their usual baseline pain. The first patient experienced a hospitalization that was longer than usual after a period of severe acute pain and revolving-door ER use. The second, a man with comorbid end stage renal disease, experienced a period of multiple ER visits and admissions over a year.

In both patients, a more focused work-up led to a diagnosis of parathyroid lesion. And, in both patients, parathyroidectomy resulted in significant changes in pain levels, and a reduction in ER visits and hospitalizations; both patients used the ER only rarely after surgery and have had very few admissions.

SCD's vasoocclusive episodes involve multiple organs, and the musculoskeletal system suffers most. Over time, constant insult to the bone can create chronic pain requiring analgesics, intravenous fluid, and transfusion.

The authors note that primary hyperparathyroidism has a propensity to mimic vasoocclusive crises in SCD. Primary hyperparathyroidism is usually asymptomatic initially, but progresses to weakness and fatigue, nephrolithiasis, bone disease, constipation, polyuria, and polydipsia if undiagnosed and untreated. It attacks the skeletal system, causing generalized bone pain, compression fracture, and high bone turnover with demineralization. Depending on the genetic variant of the SCD, patients present differently when they develop comorbid hyperparathyroidism.

When SCD patients' presentation accelerates, clinicians need to look further, and consider endocrine problems as possible causes.

Related Articles:

Sickle Cell Disease: Potential Biomarkers for Chronic Pain

Sleep Disorders Common in Sickle Cell Disease