Sickle cell disease crisis

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

Sickle-cell disease. Hypoxia and dehydration can precipitate a crisis. 

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. 

Women with sickle cell disease are predisposed to occlusion of the microvasculature (because of abnormal, inflexible red blood cells). Studies of women with sickle cell disease have shown no significant differences between OC users and non-users with regard to coagulation studies, blood viscosity measurements, or incidence or severity of painful sickle cell crisis. 

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. 

Most likely diagnosis Sickle cell disease (pain crisis). 

Clients diagnosed with sickle cell disease may go into a crisis situation for several reasons, including dehydration and infection. The nurse would not question an antibiotic. 

A 20-year-old African-American man with sickle cell disease developed a fatal hyper-hemolytic delayed transfusion reaetion 5 days after having received multiple erythrocyte transfusions for sickle cell crisis [18 ]. 

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. 

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. 

Individuals with sickle cell disease vaccinated with the TIIV between 1991 and 2006 were evaluated. No significant association between vaccination and hospitalisation for sickle cell crisis was foxmd (IRR ratio 0.92,95% Cl 0.66-1.28). This large study does not support the suggested association between influenza vaccination and sickle cell crises [67 ]. 

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... 

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