Why should he now be told in London that Hydroxyurea would save his life? His eloquence and logic impressed everybody. G6PD Deficiency makes the patients worse.
Screening for sickle cell and beta thalassaemia
On qualification, look for the many excellent UK General Practitioners who agree with Serjeant and myself. Competing Interest: Three of my siblings had sickle cell disease so I am quick to discern misleading information from experts.
Management of sickle cell disease in the community. BMJ ; g doi Feasibility and safety of home exercise training in children with sickle cell anemia.
Pediatric blood and cancer, 64 12 , pp. Epub June 9. Severe nocturnal and post exercise hypoxia in children and adolescents with sickle cell disease. Plos one. The acute phase inflammatory response to maximal exercise testing in children and young adults with sickle cell anaemia. British Journal of Haematology, 5 , pp Daily Pain. Journal of Pediatric Psychology, 42 3 , pp Konotey-Ahulu FID. Blood transfusion in sickle cell disease.
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A cautionary tale. Lancet ; Enquiry shows poor care for patients with sickle cell disease. BMJ ; Management of an acute painful sickle cell episode in hospital. NICE Guidance is frightening! Sally Davies: Silk, noir, and diplomacy. Hydroxyurea therapy for sickle cell disease in Britain. BMJ Editorial ; He discontinued Hydroxyurea due to side-effects. Rapid Response 11 February Predictors of Fetal Haemoglobin response in children with sickle cell anemia receiving Hydroxyurea therapy. Blood Jan 1: 99 1 : Sicklaemic Human Hygrometers. Beware of symptomatic sickle-cell traits.
Lancet ; : Sickle Cell Trait. Olympic Athletes and Flying. The Times Letters to the Editor. Thirteen children from twelve pregnancies in sickle-cell thalassaemia. BMJ ; 3: doi Warned when growing up never to swim in the sea, he disobeyed and was drowned at the age of 10 years swimming off Tema beach. This family is the only example in the world where father with hereditary Hb disease and wife also with hereditary Hb disease produced as many as 13 children all of them with Hb disease.
Sickle-cell disease and the patient. Lancet ; : January February 4.source
Management of sickle cell disease in the community | The BMJ
Management of sickle cell disease versus management of the sickle cell disease patient. Technical Report 83 pages. Ghana Med J ; Competing interests: Three of my siblings had sickle cell disease so I am quick to discern misleading information from experts. The authors stress the importance of children and adults with sickle cell disease SCD being physically active. Yet, during my time undertaking a student selected module in paediatric haematology I was struck by the lack of advice and support patients and their families receive in this area.
The physical and mental health benefits of sport and physical exercise are well established and the NHS has published physical activity guidelines for children and young people. Physical activity and sport participation have been shown to protect against suicide and poor mental health in teenagers and improve their psychological wellbeing 5.
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Since children with SCD have the added psychological, educational and social burden of living with a chronic condition, it is surprising no guidelines exist for them 1. More information and advice about physical activity is needed to provide paediatric SCD patients with true holistic care and improve their quality of life. Epub Jun 9. Severe nocturnal and postexercise hypoxia in children and adolescents with sickle cell disease. PloS one, 9 5 , pp. British journal of haematology, 5 , pp.
Journal of pediatric psychology, 42 3 , pp. High school youth and suicide risk: exploring protection afforded through physical activity and sport participation. The Journal of school health, 78 10 , pp. Author recommends prenatal screening in high risk or willing parents and neonatal screening of high-risk babies1. We should select that embryo which is free of trait and implant it in mother's womb. The article identifies future research areas such as the discovery and development of drugs designed to treat sickle cell disease, including those that promote haemoglobin F synthesis, prevent red cell dehydration, and inhibit HbS polymerization.
However working towards development of new drugs keeping these markers as target may be complete wrong direction. Currently used Hydroxyurea increases fetal hemoglobin HbF and improve the clinical course of sickle cell disease SCD patients. However, several issues of myelo-suppression, low WBC count and reticulocytes, low level of patient compliance due to cost or side effects , including differences in patients' drug clearance with hydroxyurea therapy remain unresolved, predictability of drug response, reversibility of sickle cell disease-related organ damage by hydroxyurea, and the efficacy of elevated HbF has been highlighted from practical observant.
There may be transient action of reducing crisis with hydroxyuea use, That is why it is recommended in this article as emergency measure in crisis only, and in only those who had episodes of crisis. HbF as marker of efficiency has been proven inefficient in field situations; hence antisickling effect should eb the target of new drug development for SCD.
Currently available drugs were developed using HbF as marker, however failure is evident by ground level situation of increasing mortality due to SCD.
In view of efficient role as anti-sickling phyto-medicines as upcoming promising therapies like Niprisan which have shown potent anti- sickeling efficiency and prevention from crisis in RCTs, Cochrane review provide clue that even without affecting or giving rise in HbF or inefficient Hb, these phytomedicines may be boon for prevention and management of SCD. Reference: i A cautionary note regarding hydroxyurea in sickle cell disease. Investigate any numb lower lip for scd. Circumstances [10 39], some patient-specific, precipitate crises eg Ghanaian scd man had sickle crisis on eating oranges .
Would Grousse et al start Hydroxyurea  after twice eating oranges? One rain-soaked scd boy got sickle crisis and stroke . Trans-cranial Doppler  better prophylactic than raincoat? Hot weather too causes sickle crisis .
Management of sickle cell disease in the community
Avoid Valsalva in labour; Caesarian Section advised. Squatting Tourniquet effect can start crisis . Pulmonary embolism missed . Medicated bed nets  no better than usual nets and may harm babies . Malaria kills scd patients quicker [10 ]. But Why? Patients complain to BMJ .
Dedicated sickle cell centres. The Sickle Cell Disease Patient. Clinico-epidemiological study of consecutive patients at Korle Bu Hospital, Accra. Effect of environment on sickle cell disease in West Africa; epidemiologic and clinical considerations. Archives Intern Med ; The liver in sickle cell disease. Clinical aspects. Ghana Med J ; 8: Mental nerve neuropathy: a complication of sickle cell crisis.
Four bodyguards and the perils of unmasking scientific truths www. Beware of symptomatic sickle cell traits. Blaming sudden death on Sickle Cell Trait? Sickle cell Trait Misinformation and Disinformation Nov 30 www. Technical Report ; 83 pages. Relatively benign sickle cell anaemia in 60 patients aged over 30 in the West Indies.
BMJ ; 3: The irreversibly sickle cell: a determinant for haemolysis in sickle cell anaemia. Br J Haematol ; Oral zinc sulphate in sickle cell ulcers.
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