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PAPERS: Krista Maxwell, Allison Streetly, and David Bevan Experiences of hospital care and treatment seeking for pain from sickle cell disease: qualitative study BMJ 1999; 318: 1585-1590 [Abstract] [Full text]

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Bi-directional trust is needed in pain management of sickle cell disease

Ade Olujohungbe   (2 July 1999)

SMAC report on sickle cell disease

A G Johnson   (5 July 1999)

An unusual case of sickle cell disease with recurrent acute chest syndrome

Umesh Kumar Dashora   (26 August 1999)

Bi-directional trust is needed in pain management of sickle cell disease 2 July 1999
Ade Olujohungbe, Consultant Haematologist University Hospital, Aintree. Liverpool Send response to journal: Re: Bi-directional trust is needed in pain management of sickle cell disease	Editor, We read the very commendable article written by Maxwell et al in the issue of the BMJ, June 12,318:1585-1590 on pain management in sickle cell disease.1 We highlighted this issue of mistrust between patients and carer in our editorial 2 also published in the BMJ, as explaining in part, the "research fatigue or inertia." Not all centres have experienced similar problems 3 although we remain convinced that this problem is of a greater magnitude than is generally accepted and this report reinforces our observation. It is disheartening that the level of mistrust between patients and their carers could have resulted in physical aggression as cited in Maxwell's report (Interview 12.1). There have been recent news features and reports published in the Lancet and the British Journal of Haematology 4,5 about adopting a community based approach to pain management which may be more cost effective and beneficial to patients. Our treatment culture will need to change before success can be guaranteed in adopting this approach. Patients must be trusted and empowered to actively participate in their pain management to avoid under and overtreatment and they must be satisfied there is a rapid response and sympathetic hospital based team available should their coping mechanisms at home fail. What remains clear is that in order to answer fundamental questions we need to recruit large number of our patients into ethically clinical studies. It is essential that barriers that may exist between patients and carers or indeed between institutions be overcome, so that we can recruit patients (a population of about 12,000 individuals in the UK) into ethical randomised clinical trials with the necessary statistical power to change management issues for our patients. Ade.Olujohungbe Consultant Haematologist University Hospital, Aintree Longmoor Lane. Aintree. Liverpool L9 A7 Kornelia Cinkotai Medical Head Manchester Sickle Cell & Thalassemia Centre Oxford Road Manchester M13 9WL References:- 1. Maxwell, K., Streetly A., Bevan, D. (1999). Experiences of hospital care and treatment seeking for pain from sickle cell disease: qualitative study BMJ; 318:1585-1590. 2. Olujohungbe, A., Cinkotai, K.I., Yardumian, A(1998). Hydroxyurea therapy for sickle cell disease in Britain. BMJ; 316:1689 3. Davies, S.C. and Roberts -Harwood, M European register of patients with sickle cell disease treated with hydroxyurea is being set up (1998). BMJ; 317:541 4. Ware, M.A., Hambleton, I., Ochaya, I., Serjeant G.R. (1999) Day-care management of sickle cell painful crisis in Jamaica: a model applicable elsewhere? British Journal of Haematology January; 104 (1) 93-96. 5. Morris, K Crisis of care in sickle cell disease(1999) Lancet, vol 353; No 9163 page 1504
SMAC report on sickle cell disease 5 July 1999
A G Johnson, Professor of Surgery & Chairman SMAC University of Sheffield Send response to journal: Re: SMAC report on sickle cell disease	I am surprised that the authors make only one reference to the SMAC report on Sickle Cell, Thalassaemia and other Haemoglobinopathies, and that about education, referring to it as "unpublished data". We had patients on the Working Party and made over 60 recommendations covering all those that are referred to in the BMJ paper, especially the diversity and the empowerment of patients. E.g. "Whenever possible, patients' wishes should be taken into account when deciding where treatment should take place". "Each patient should have an individual plan for the management of pain", etc. The SMAC report was widely distributed and gave the NHS the information it needs to enable achievement of the best possible care and provide a focus for further debate, development and collaboration. The Department of Health has funded a number of projects, and videos and booklets have been produced to improve information to ethnic minority communities and health care professionals. It is sad to read that there are, apparently, still so many of the problems that we were told about in 1993 (although we have no quantitative study before and after). However, the revised national haemoglobinopathy card carrying important patient details, which we recommended, will soon be available.
An unusual case of sickle cell disease with recurrent acute chest syndrome 26 August 1999
Umesh Kumar Dashora, Specialist Physician and Diabetologist Ibri Regional Referral Hospital,Sultanate of Oman Send response to journal: Re: An unusual case of sickle cell disease with recurrent acute chest syndrome	An unusual case of sickle cell disease with recurrent acute chest syndrome. Dr Murad Meena Faragala, MD, Jr specialist Dr Umesh Dashora, MRCP, Specialist Dr Kamiran Dabbagh MRCP, Consultant Dr R .Siddique, ABIM , Sr Specialist Department of Medicine,Ibri Regional Referral Hospital, POB46, PC 516,Sultanate of Oman.Ph 968 491905 To 8 Ext 265 Fax: 968 491915 E mail: dashora@hotmail.com Editor-Sickle cell disease (SCD) causes repeated painful crises (1).But recently we came across a 20 year old chronic smoker with sickle cell disease ( Hb S: 89.4%) who suffered recurrent acute chest syndrome(ACS) of varying intensity, thrice in one year, last time requiring ventilation and exchange transfusion. First time he had hemolytic crisis and rt. sided pneumonia. After one month there was pleuritic chest pain without any clinical or radiological abnormality. On his third admission he was febrile and jaundiced. Chest was clear. Relevant abnormal tests were: Hb: 8.9 gm/dl. Ret. % :7.8(The last Hb in 20.9.98 was 9.2 gm/dl.) T. Bilirubin: 206.14 umol/L. D. Bilirubin:42.70 umol/L. Chest X-ray: Normal. Abdominal Ultrasonography: Atrophied spleen. Urine for hemoglobinuria: Positive. Left sided pleuritis with SCD was diagnosed. Two days later he deteriorated with clinical and radiological signs of left lower consolidation.A diagnosis of pneumonia vs. ACS was made. Despite antibiotics his PO2 dipped to 57.5 mmHg. Assisted ventilation improved PO2 to 93.4 mmHg. However fever and Xray shadow showed no response for ten days but exchange transfusion with 6 Units of blood led to clinical and radiological recovery. Off-ventilator and antibiotics, he was discharged in good clinical condition. He was happy when last seen and was considering marrying. Acute cardiopulmonary problems in SCD include acute ACS, pneumonia, and left ventricular failure. (2) Our patient developed acute chest syndrome on multiple occasions and in varying severity. ACS is a phenomenon of pulmonary sequestration in SCD patients. It may carry upto 100 percent mortality. (3) Our patient responded to simple measures first two times but the third time we almost lost him. ACS may present as new pulmonary infiltrate with fever or respiratory symptoms in a patient with sickle cell disease. Transfusion is indicated in hypoxic patients and can be used to prevent recurrent episodes. ( 4,5) Exchange transfusion dramatically changed the outcome in our patient 1Experiences of hospital care and treatment seeking for pain from sickle cell disease:qualitative study.Maxwell K,Streetly A, BevanD.BMJ1999;318:1585-90 (12th June) 2 Leong CS, Stark P.J Thoracic manifestations of sickle cell disease. Thorac Imaging 98 ;13(2):128-34 3Delatte SJ, Hebra A, Tagge EP, Jackson S, Jacques K, Othersen HB Jr.Acute chest syndrome in the postoperative sickle cell patient. J Pediatr Surg 99 ;34(1):188-92 4Golden C, Styles L, Vichinsky E Acute chest syndrome and sickle cell disease. Curr Opin Hematol 98 ;5(2):89-92 5Samal GS Acute chest syndrome in sickle cell disease.Indian Pediatr 97 ;34(9):817-9 Editorial note The patient gave written consent to the publication of the above mentioned material