Crisis in sickle cell disease

Placebo-controlled studies In a randomised, double-blind trial including 38 children, the efficacy of five days arginine therapy lOOmg/kg thrice daily for treating vaso-occlusive crisis in sickle cell disease was evaluated. There... 

E. P. Orringer, E. A. Binder, R. P. Binder, R. P. Thomas, D. S. Blythe, J. A. Bustrack, D. H. Schroeder, and M. L. Hinton, Abstracts of 14th Annual Sickle Cell Centers Conference, April 16-18,1989, Duke University, Durham, North Carolina. Phase I Study of 12C79 in Sickle Cell Disease (SCD) Patients Not in Crisis. 

Yale SH. Approach to the vaso-occlusive crisis in adults with sickle cell disease. Am Fam Phys 2000 61 1349-1356,1363-1364. 

Gonzalez, P. Hackney, A.C. Jones, S. Strayhorn, D. Hoffman, E.B. Hughes, G. Jacobs, E.E. Orringer, E.P. A phase I/II clinical study of polymerized bovine hemoglobin in adult patients with sickle cell disease not in crisis at the time of study. J. Invest. Med. 1997, 45 (5), 258-264. 

A typical vaso-occlusive crisis with increased hemolysis was noted within minutes after intracutaneous injection of GM-CSF in a patient with stable sickle cell disease (SEDA-19, 342). Topical GM-CSF was later uneventful. 

Homozygous Hemoglobin S (HbSS). In homozygous Hb S, a valine for glutamic acid substitution occurs on both p-globin chains because of the inheritance of mutated P-globin chain genes from both parents. The condition is described as sickle cell anemia or sickle cell disease because of the sickle shaped RBCs that occur when there is a sickle cell crisis and is sometimes written as p p. ... 

Some of the clinical consequences in SS disease include megaloblastic erythropoiesis, aplastic crisis, stroke, bone pain crisis, proneness to infection particularly by Pneumococcus, Salmonella, and Haemophilus due to hypos-plenism and acute chest syndrome. Prophylactic use of penicillin and antipneumococcal and Haemophilus vaccines has aided in the management of life-threatening infectious complications of SS disease. Neonatal screening has been used in the identification of infants with sickle cell disease so that risk of infection can be modulated by appropriate immunizations and penicillin prophylaxis. The acute chest syndrome characterized by chest pain is due to clogged pulmonary capillaries in a small number of studies, patients have been treated with inhaled nitric oxide, which dilates blood vessels with clinical improvement. 

Medical pain morphine is the drug of choice for the relief of pain due to myocardial infarction. Relief of ischemic pain decreases sympathetic nervous system activity thus reducing myocardial oxygen demand. Morphine is used in patients with acute pulmonary edema for its cardiovascular effects and to decrease air hunger. Morphine can be used to treat the pain of sickle cell disease with crisis. Morphine can be used to treat the pain of Guillain-Barre syndrome, osteoarthritis and obstetric pain. Morphine s sedative effects can be utilized in the intubated and ventilated patient. 

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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