Background and Methods The management of episodes of severepain in patients with sickle cell disease is a difficult clinicalproblem. We studied 36 children and adolescents with sicklecell disease who had 56 acute episodes of severe pain (44 in27 patients with sickle cell anemia, 8 in 7 patients with sicklecell-hemoglobin C disease, and 4 in 2 patients with sickle cell-+-thalassemia).The patients were randomly assigned in double-blind fashionto receive an intravenous infusion of either saline placeboor high-dose methylprednisolone (15 mg per kilogram of bodyweight, to a maximum of 1000 mg) on their admission to the hospitaland again 24 hours later. All the patients received intravenousmorphine sulfate until severe pain abated and were then givenacetaminophen with codeine.
Results For all episodes of pain, the duration of inpatientanalgesic therapy (intravenous and oral) was significantly shorterfor the patients who received methylprednisolone than for thosegiven placebo (mean, 41.3 vs. 71.3 hours; P = 0.030). The differencewas still significant (31.0 vs. 62.5 hours; P = 0.010) whenwe excluded seven episodes that were complicated by the chestsyndrome (three in the methylprednisolone group and four inthe placebo group). The patients who received methylprednisolonehad recurrent episodes of pain shortly after the discontinuationof therapy more often than did the patients receiving placebo.No adverse effects of methylprednisolone were observed.
Conclusions A short course of high-dose methylprednisolone decreasedthe duration of severe pain in children and adolescents withsickle cell disease, but patients who received methylprednisolonehad more rebound attacks after therapy was discontinued. Onbalance, corticosteroids are promising as an adjunct to supportivetherapy for painful episodes in children and adolescents withsickle cell disease.
Source Information
From the Division of Hematology-Oncology, Department of Pediatrics (T.C.G., G.R.B.), and the Academic Computing Center (D.M.), University of Texas Southwestern Medical Center at Dallas, and the Center for Cancer and Blood Disorders, Children's Medical Center, Dallas (T.C.G., G.R.B.).
Address reprint requests to Dr. Buchanan at the Department of Pediatrics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX 75235-9063.
Bartolucci, P., El Murr, T., Roudot-Thoraval, F., Habibi, A., Santin, A., Renaud, B., Noel, V., Michel, M., Bachir, D., Galacteros, F., Godeau, B.
(2009). A randomized, controlled clinical trial of ketoprofen for sickle-cell disease vaso-occlusive crises in adults. Blood
114: 3742-3747
[Abstract][Full Text]
Field, J. J., DeBaun, M. R.
(2009). Asthma and sickle cell disease: two distinct diseases or part of the same process?. ASH Education Book
2009: 45-53
[Abstract][Full Text]
Ataga, K. I.
(2009). Novel therapies in sickle cell disease. ASH Education Book
2009: 54-61
[Abstract][Full Text]
Couillard, S., Benkerrou, M., Girot, R., Brousse, V., Ferster, A., Bader-Meunier, B.
(2007). Steroid treatment in children with sickle-cell disease. haematol
92: 425-426
[Abstract][Full Text]
Firth, P. G.
(2005). Anaesthesia for peculiar cells--a century of sickle cell disease. Br J Anaesth
95: 287-299
[Abstract][Full Text]
Belcher, J. D., Mahaseth, H., Welch, T. E., Vilback, A. E., Sonbol, K. M., Kalambur, V. S., Bowlin, P. R., Bischof, J. C., Hebbel, R. P., Vercellotti, G. M.
(2005). Critical role of endothelial cell activation in hypoxia-induced vasoocclusion in transgenic sickle mice. Am. J. Physiol. Heart Circ. Physiol.
288: H2715-H2725
[Abstract][Full Text]
Lottenberg, R., Hassell, K. L.
(2005). An Evidence-Based Approach to the Treatment of Adults with Sickle Cell Disease. ASH Education Book
2005: 58-65
[Abstract][Full Text]
Siddiqui, A K, Ahmed, S
(2003). Pulmonary manifestations of sickle cell disease. Postgrad. Med. J.
79: 384-390
[Abstract][Full Text]
Orringer, E. P., Casella, J. F., Ataga, K. I., Koshy, M., Adams-Graves, P., Luchtman-Jones, L., Wun, T., Watanabe, M., Shafer, F., Kutlar, A., Abboud, M., Steinberg, M., Adler, B., Swerdlow, P., Terregino, C., Saccente, S., Files, B., Ballas, S., Brown, R., Wojtowicz-Praga, S., Grindel, J. M.
(2001). Purified Poloxamer 188 for Treatment of Acute Vaso-occlusive Crisis of Sickle Cell Disease: A Randomized Controlled Trial. JAMA
286: 2099-2106
[Abstract][Full Text]
Nagel, R. L.
(2001). The Challenge of Painful Crisis in Sickle Cell Disease. JAMA
286: 2152-2153
[Full Text]
Solovey, A. A., Solovey, A. N., Harkness, J., Hebbel, R. P.
(2001). Modulation of endothelial cell activation in sickle cell disease: a pilot study. Blood
97: 1937-1941
[Abstract][Full Text]
Belcher, J. D., Marker, P. H., Weber, J. P., Hebbel, R. P., Vercellotti, G. M.
(2000). Activated monocytes in sickle cell disease: potential role in the activation of vascular endothelium and vaso-occlusion. Blood
96: 2451-2459
[Abstract][Full Text]
Bernini, J. C., Rogers, Z. R., Sandler, E. S., Reisch, J. S., Quinn, C. T., Buchanan, G. R.
(1998). Beneficial Effect of Intravenous Dexamethasone in Children With Mild to Moderately Severe Acute Chest Syndrome Complicating Sickle Cell Disease. Blood
92: 3082-3089
[Abstract][Full Text]
Michaels, L. A., Ohene-Frempong, K., Zhao, H., Douglas, S. D.
(1998). Serum Levels of Substance P Are Elevated in Patients With Sickle Cell Disease and Increase Further During Vaso-Occlusive Crisis. Blood
92: 3148-3151
[Abstract][Full Text]
Davies, S. C, Oni, L.
(1997). Fortnightly review: Management of patients with sickle cell disease. BMJ
315: 656-660
[Full Text]
Adams-Graves, P., Kedar, A., Koshy, M., Steinberg, M., Veith, R., Ward, D., Crawford, R., Edwards, S., Bustrack, J., Emanuele, M.
(1997). RheothRx (Poloxamer 188) Injection for the Acute Painful Episode of Sickle Cell Disease: A Pilot Study. Blood
90: 2041-2046
[Abstract][Full Text]
Foley, K. M., Portenoy, R. K., Rosenbloom, B. E., Tanaka, K. R., Buchanan, G. R., Griffin, T. C.
(1994). Treatment of Pain in Sickle-Cell Crisis. NEJM
331: 334-335
[Full Text]
(1994). IV STEROIDS MAY EASE SICKLE CELL PAIN. JWatch General
1994: 3-3
[Full Text]
Platt, O. S.
(1994). Easing the Suffering Caused by Sickle Cell Disease. NEJM
330: 783-784
[Full Text]
+-thalassemia). The patients were randomly assigned in double-blind fashion to receive an intravenous infusion of either saline placebo or high-dose methylprednisolone (15 mg per kilogram of body weight, to a maximum of 1000 mg) on their admission to the hospital and again 24 hours later. All the patients received intravenous morphine sulfate until severe pain abated and were then given acetaminophen with codeine. 
