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• W2012176486 abstract "Marie Stuart and Ronald Nagel's Seminar (Oct 9, p 1343)1Stuart MJ Nagel RL Sickle-cell disease.Lancet. 2004; 364: 1343-1360Summary Full Text Full Text PDF PubMed Scopus (626) Google Scholar and Michaela Buckner's Personal Account (p 1361)2Buckner M Sickle-cell disease: from Sierra Leone to southeast London.Lancet. 2004; 364: 1361Summary Full Text Full Text PDF PubMed Scopus (3) Google Scholar highlight the differences between sickle-cell disease and the patient. Michaela need not worry about the disease being “a systemic disorder of monumental complexity”,1Stuart MJ Nagel RL Sickle-cell disease.Lancet. 2004; 364: 1343-1360Summary Full Text Full Text PDF PubMed Scopus (626) Google Scholar because the patient is far from complex. Three of my siblings had sickle-cell disease (chwechweechwe),3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar which can be traced back in my family to 1670 (family tree available at http://www.sicklecell.md/images/generation.jpg). Stuart and Nagel missed something out of their Seminar, which Michaela emphasises—circumstances. “The dreaded malaria” and “wearing […] traditional African clothes in December in the UK”2Buckner M Sickle-cell disease: from Sierra Leone to southeast London.Lancet. 2004; 364: 1361Summary Full Text Full Text PDF PubMed Scopus (3) Google Scholar that Michaela describes are known causes of sickle-cell crises.3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar, 4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar, 5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar A child I treated who was homozygous for the disease had a stroke because his grandmother took him out without an overcoat; his identical twin, who stayed at home, was not saved by cation homoeostasis1Stuart MJ Nagel RL Sickle-cell disease.Lancet. 2004; 364: 1343-1360Summary Full Text Full Text PDF PubMed Scopus (626) Google Scholar or variations in vascular cell adhesion molecule-1 (VCAM-1) bonding to the endothelium via α4β1. My ancestors3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar knew the difference between “gbagblaa” (SS-phenotype) and “pi-gbagblaa”, SC-phenotype,4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar and made sure that survival was optimised through the rigourous observation of specific circumstances.3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar, 4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar, 5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar One in 16 women with an increased rate of urinary tract infections in pregnancy and one in four men, is homozygous and hemizygous, respectively, for glucouse 6-phosphate dehydrogenase (G6PD) deficiency,3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar, 4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar, 5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar jeopardising antimicrobial therapy.5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar If Michaela Buckner is homozygous for G6PD deficiency, then both her children will be hemizygous; if heterozygous, the son with the more severe disease could well be the one carrying her G6PD-deficient gene.5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar By keeping a detailed diary, she should soon find what tips them into crisis. She should try to meet patients with sickle-cell disease who manage without hydroxyurea and diamorphine or morphine for crisis pain and ask them how. Clinicians should assess new presenting symptoms, such as gnathopathy,3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar, 4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar, 5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar the numb lower lip,3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar, 4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar and accelerated finger clubbing,3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar and seek information about how patients are treating themselves. When a patient who is able to manage their disease without drugs claims that a nutrient like aerobic oxygen has kept them symptom-free for decades, doctors should pay attention. Antibiotics, folic acid, pneumovaccine, plenty of water, a good community nurse, family doctors that listen, together with strict attention to circumstances, will help keep Michaela's boys out of hospital. She should teach them that half their children will have sickle-cell disease if they marry someone with the trait, which one in three west Africans carries.3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar, 4Konotey-Ahulu FID The sickle cell diseases: clinical manifestations including the “Sickle Crisis”.Arch Intern Med. 1974; 133: 611-619Crossref PubMed Scopus (137) Google Scholar, 5Konotey-Ahulu FID Effect of environment on sickle cell disease in West Africa: epidemiologic and clinical considerations.in: Abramson H Bertles JF Wethers DL Sickle cell disease, diagnosis, management, education, and research. The C V Mosby Company, St Louis1973: 20-38Google Scholar Work on transgenic mice might answer some of the questions about sickle-cell disease, but will probably never be able to explain why one of my patients went into severe crisis on hearing that his mother had died.3Konotey-Ahulu FID The sickle cell disease patient: natural history from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh-A'Domeno, Watford1996Google Scholar I declare that I have no conflict of interest." @default.
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