Despite frequent episodes of severe recurrent pain in sickle cell disease (SCD), sensory pain in outpatient adults with SCD lacks sufficient characterization. Furthermore, pivotal barriers may interfere with these patients’ adherence to prescribed analgesic therapies, but have not been studied systematically. We describe sensory pain characteristics, barriers, and analgesic use reported by adults with SCD during routine clinic visits. Patients (N=145, 67% female, 94% African American) completed measures on a pen-tablet computer. Patients reported an average of 3.6±2.3 pain sites; mean current pain intensity (3.3±3.2), least (3.0±2.7) and worst (4.9±3.5) pain intensity in 24 hr on a 0-10 scale; multiple neuropathic (4.5±3.4, 8.3% selected none) and nociceptive (6.8±4.0) pain descriptors; and continuous pain pattern (59%). Their mean pain barriers score was 2.2±0.9, and 33% were dissatisfied with their pain levels. Only 14% reported taking at least one adjuvant drug, 82% were taking nonopioids, 85% step 2 opioids, and 65% step 3 opioids. Patients reported using, on average, 4.9±2.7 analgesics. Their pain barriers scores are similar to or greater than people with cancer. Importantly, their pain may be both nociceptive and neuropathic, contrary to common expectations that SCD pain is only nociceptive. Few patients, however, took drugs effective for neuropathic pain.
Allogeneic hematopoietic stem cell transplantation (HSCT) is rarely performed in adult patients with sickle cell disease (SCD). We utilized the chemotherapy-free, alemtuzumab/total body irradiation 300 cGy regimen with sirolimus as post-transplantation immunosuppression in 13 high-risk SCD adult patients between November 2011 and June 2014. Patients received matched related donor (MRD) granulocyte colony-stimulating factor-mobilized peripheral blood stem cells, including 2 cases that were ABO incompatible. Quality-of-life (QoL) measurements were performed at different time points after HSCT. All 13 patients initially engrafted. A stable mixed donor/recipient chimerism was maintained in 12 patients (92%), whereas 1 patient not compliant with sirolimus experienced secondary graft failure. With a median follow-up of 22 months (range, 12 to 44 months) there was no mortality, no acute or chronic graft-versus-host disease (GVHD), and no grades 3 or 4 extramedullary toxicities. At 1 year after transplantation, patients with stable donor chimerism have normalized hemoglobin concentrations and improved cardiopulmonary and QoL parameters including bodily pain, general health, and vitality. In 4 patients, sirolimus was stopped without rejection or SCD-related complications. These results underscore the successful use of a chemotherapy-free regimen in MRD HSCT for high-risk adult SCD patients and demonstrates a high cure rate, absence of GVHD or mortality, and improvement in QoL including the applicability of this regimen in ABO mismatched cases (NCT number 01499888).
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