Sickle cell crisis treatment

Psychostimulant medication can be useful in the treatment of severe, acute pain, such as that seen during a sickle cell crisis (Yaster et al., 2000). Dextroamphetamine and methylphenidate are also effective as adjuvants, as they have independent analgesic effects, and potentiate the effects of opioid analgesics. The increase in alertness afforded by the use of psychostimulants can also allow the use of larger doses of opioids (Yaster et al., 2000). Methylphenidate and dextroamphetamine have been used to diminish the sedative effects of opioid analgesic medication (Yee and Berde, 1991) in adolescent patients with malignancies or sickle crisis, and may also potentiate the effects of analgesics. Doses used by Yee and Berde (1991) were 2.5 to 10 mg bid of methylphenidate, or 2.5 to 5 mg bid of dextroamphetamine. 

Treatment of SCD is aimed at the preventing and/or minimizing acute and chronic complications, including infection, acute chest syndrome, neurologic damage, and the various forms of sickle cell crises, including vaso-occlusive pain, splenic sequestration, and aplastic crisis. The acute and chronic complications are summarized in Tables 65-2,65-3, and 65-4. 

Otringer EP, Casella JR Ataga Kl, et al. Purified poloxamer 188 for treatment of acute vaso-occlusive crisis of sickle cell disease, a randomized controlled trial. JAMA 2001 286 2099-2106. 

A purified version of pluronic F68 is being developed by SynthRx under the brand name Flocor as a potential treatment of vaso-occlusive crisis associated with sickle cell disease [172]. After establishing the tolerability of this polymer in clinical studies, further studies of sickle cell patients treated with Flocor showed a reduction in the length of painful episodes and an increase in the number of patients who achieved resolution of the symptoms [173]. The effect observed was significant but small, and the effects seen were more pronounced for children imder the age of 15. Further development of Flocor has been reportedly planned, especially for pediatric sickle cell patients. 

Desferrioxamine (Df) has a dissociation constant for binding to Fe at IO M which provides the very high specificity for the chelation of iron required for the treatment of patients with transfusion iron overload [114-116]. As such Df has been very closely monitored as clinical agent for 20 years. The main clinical drawback of desferrioxamine is that the chelator can only be administered by intramuscular injection (usually to Sickle Cell Disease patients in crisis). There has been an active program to introduce new orally active iron chelators [114-116]. 

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