Management of sickle cell disease patient in the community

Re: Management of sickle cell disease patient n the community

FELIX ID KONOTEY-AHULU, Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana

Consultant Physiician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd., Phoenix Hospital Group, London W1G 9AL

British Medical Journal  Rapid Response

13 April 2014

Re: Management of sickle cell disease in the community. Valentine Brousse, Julie Makani, David C Rees. 348:doi:10.1136/bmj.g1765

Valentine Brousse and colleagues’ Clinical Review [1], gives doctors little guidance for patient management. “Serjeant’s and my combined experience of 80 years covering thousands of patients in sickle cell crisis” can help. [2].


Graham Serjeant’s Jamaican experience [3] and my Ghanaian [4] differ from NICE’s Guidance [5] in UK where patients died from Morphine/Diamorphine overdose. [6 7] We managed sickle cell disease (scd) patients to university without Morphine/Diamorphine, Hydroxyuurea, or regular transfusions. Our experience (References 1965 to 2014} could save UK patients.


(1) Two brothers and one sister suffered from scd “Hereditary Rheumatism” traced back to 1670 AD [9-11]. Siblings’ commonest cause of sickle cell crises characterized by convulsions and priapism [9 10] was Malaria. One brother had ‘gnathopathy’, my invented word for maxillary marrow hyperactivity [10 12 13 14].
(2) Directing largest Sickle Cell Clinic I catalogued 133 Illustrative Case Histories [10] including alcohol-induced myocardial infarction [10, p 503] and mental nerve neuropathy [15], kanumblll sign. Investigate any numb lower lip for scd. [15]
(3) Haematologist Helen Ranney published “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu” [16].
(4) Receiving with Linus Pauling and others “Martin Luther King Jr Foundation Award for Outstanding Research in Sickle Cell Anaemia” my Keynote Address “Difference between Sickle Cell Disease and Sickle Cell Trait” that exposed fraud of Insurance Companies led to being given 4 bodyguards in Philadelphia. [17] By saying Sickle Cell Trait was “largely asymptomatic” [1] Brousse et al repeated Insurance Companies’ misinformation/disinformation [18-21] used to levy Traits 150% Premium.
(5) On WHO Expert Genetics Advisory Panel with Haemoglobinopathy Greats who valued Ethics in Human Genetics. [22]


“In Jamaican experience morphia or its derivatives are rarely used or necessary” [23] and “the most painful crises may be treated in a day centre, the patient returning home in the evening” [24]. See more of Serjeant’s publications from 1968 [23-38].


(1) KEEP A DIARY! Circumstances [10 39], some patient-specific, precipitate crises eg Ghanaian scd man had sickle crisis on eating oranges [10]. Would Grousse et al start Hydroxyurea [1] after twice eating oranges?
(2) ALWAYS CARRY UMBRELLA. One rain-soaked scd boy got sickle crisis and stroke [44]. Trans-cranial Doppler [1] better prophylactic than raincoat? Hot weather too causes sickle crisis [39].
(3) NOSE PICKING causes Epistaxis [39 45].
(4) VALSALVA MANOEUVRE: In 18 causes of ocular bleeds [10] “six were related to sneezing, blowing nose, shouting, bending down, lifting heavy objects, and vigorous exercise” [46]. Avoid Valsalva in labour; Caesarian Section advised.
(5) DOCTORS BEWARE! Ectopic pregnancy, gall stones [47], appendicitis, splenic infarct [10] misdiagnosed as “Abdominal crisis”. Squatting (Tourniquet effect) can start crisis [10]. Opiates cause “Chest syndrome” [48]. Pulmonary embolism missed [49]. Note Intra-family scd phenotype differences [50 51]
(6) EXAMINE COMMUNITY RUMOUR. Medicated bed nets [1] no better than usual nets and may harm babies [52]. Malaria kills scd patients quicker [10 53-55]. Patients shun Hydroxyurea [56 57] and prenatal diagnosis [58 59] Drinking up to 4 L of water a day has stopped crises for past 25 years, as has “Aerobic Oxygen” 20 drops tds in water [60] Coconut juice clearing jaundice? [60.]
(7) STATIONERY BICYCLE aborts early morning priapism [10, 43]
(10) SICKLE CELL DISEASE PATIENT ACHIEVERS teach doctors 1993-2010 [70]
(11) G6PD Deficiency: 1 in 4 male and 1 in 16 female scd patients have this. [71 72]


NCEPOD discovered “9 out of 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opiods” [6 7]. Yet NICE still recommends “a strong opiod intravenously” [5]. But Why? [2 4 10 48 75-87]. Patients complain to BMJ [88-90].

1 Brousse V, Makali J, Rees DC. Management of sickle cell disease in the community. BMJ 2014; 348:g1765 doi:10.1136/bmj.g1765

2 Konotey-Ahulu FID Opiates for sickle cell crisis? Lancet 1998: 351: 1438 “The question that puzzles me is why do west African and West Indian patients with sickle cell disease who did without morphine in our countries have to be given morphine pumps during sickle cell crisis when they come to the UK?”

3 Serjeant G. The case for dedicated sickle cell centres. BMJ 2007; 334: 477

4 Konotey-Ahulu FID. Dedicated sickle cell centres. BMJ Rapid responses March 20 2007

5 NICE GUIDELINES. Management of acute painful sickle cell episode in hospital; summary of NICE Guidance. BMJ 2012; 344: doi:http://dx.doiorg/10 1136/bmj’e’4063

6 NCEPOD – National Confidential Enquiry into Patient Outcome and Death. SICKLE: A Sickle Crisis? 2008 [Sebastian Lucas (Clinical Coordinator), David Mason (Clinical Coordinator), M Mason (Chief Executive), D Weyman (Researcher)/ Tom Treasurer (Chairman)

7 Mason S. Enquiry shows poor care for patients with sickle cell disease. BMJ 2008; 336: 1152 “In 2 years 9 out of the 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opiods”

8 Konotey-Ahulu FID. International request to manage patients, for example, June 2007

9 Konotey-Ahulu FID. Pattern of Sickle Cell Disease in Ghana (A Study Of 1,550 Consecutive Patients) – A Thesis Presented For The Degree of Doctor of Medicine (M.D.) In The University Of London 1971 Awarded Feb 1972.

10 Konotey-Ahulu FID. The Sickle Cell Disease Patient. Clinico-epidemiological study of 1550 consecutive patients at Korle Bu Hospital, Accra. T-A’D Co, Watford 1996 http://www.sicklecell.mde/aboutscd.asp
(Reprint of Konotey-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. London & Basingstoke, Macmillan Press Ltd 1991/1992. Foreword by Roland B Scott MD, Howard Univ, Washington DC)

11 Konotey-Ahulu FID. Sickle Cell Disease In Successive Ghanaian Generations For Three Centuries (Manya Krobo Tribe) In The Human Genome Diversity Project: Cogitations of An African Native. Politics and The Life Sciences (PLS) 1999; Vol 18: No 2, pp 317-322.

12 Konotey-Ahulu FID. Effect of environment on sickle cell disease in West Africa; epidemiologic and clinical considerations. Chapter 3 in SICKLE CELL DISEASE – diagnosis, management, education and research. Eds Ahramson, Bertles JF, Wethers Doris L; St Louis – CV Mosby Co 1973, pp 20-38.

13 Konotey-Ahulu FID. The Sickle Cell Diseases: Clinical manifestations including the Sickle Crisis. Archives Intern Med 1974; 133: 611-619.

14 Konotey-Ahulu FID. The liver in sickle cell disease. Clinical aspects. Ghana Med J 1969; 8: 104-118. (First ever use of word “Gnathopathy”)

15 Konotey-Ahulu FID. Mental nerve neuropathy: a complication of sickle cell crisis. Lancet 1972; 2: 388 [Constitutes discovery of a new physical sign in Clinical Medicine – The kanumblll sign spells out who discovered it, what it is, and where published: “konotey-ahulu numb lower lip Lancet” sign 1972]

16 Ranney Helen. Summary of Symposium 1972 on Sickle Cell Disease – Diagnosis, Management, Education and Research – In SICKLE CELL DISEASE, Eds H Abramson, John F Bertles, Doris Wethers (C Mosby Co), 1973, page 320: “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu”

17 Konotey-Ahulu FID. Four bodyguards and the perils of unmasking scientific truths BMJ 2007; 335: 210-11 BMJ July28 2007.

18 Konotey-Ahulu FID. Beware of symptomatic sickle cell traits. Lancet 1992; 339: 555. Doi:10.1016/0140-6736(92)90377-F (Pointing out that the Sickle Cell Trait ‘AS’ may not be true Sickle Trait, and that the Haemoglobin C Trait ‘AC’, may be something else, as the “A” has been shown could stand for Hb Quebec-Chori, by Witkowska et al – New Eng J Med 1991; 325: 1150-54

19 Konotey-Ahulu FID. Blaming sudden death on Sickle Cell Trait? Flaws in article of Charis Kepron, Gino Sobers, Michael Pollanen Exposed Sept 14 2011 or

20 Konotey-Ahulu FID. Sickle cell Trait Misinformation and Disinformation Nov 30 2011 [Comprehensive Review]

21 Konotey-Ahulu FID. Further Communication on “Sickle Cell Trait Misinformation and Disinformation” and Sickle Cell Terminology: Disease or Disorder? April 16 2012

22 Boyo Alex E, Cabannes R, Conley CR, Lehmann H, Luzzatto L, Milner PF, Ringelhann B, Weatherall DJ, Barrai I, Konotey-Ahulu FID, Motulsky AG. WHO (Geneva) Scientific Group on Treatment of Haemoglobinopathies and Allied Disorders. (Technical Report) 1972; 509: 83 pages.

23 Serjeant GR. Sickle Cell Disease. Oxford, Oxford University Press, 1985.

24 Serjeant GR. Sickle cell disease. Lancet 1997; 35: 725-730.

25 Serjeant GR, Richards R., Barbor PRH, Milner PF. Relatively benign sickle cell anaemia in 60 patients aged over 30 in the West Indies. BMJ 1968; 3: 86

26 Serjeant GR, Serjeant BE, Milner PF. The irreversibly sickle cell: a determinant for haemolysis in sickle cell anaemia. Br J Haematol 1969; 17: 527-533.

27 Serjeant GR, Galloway RE, Gueri MC. Oral zinc sulphate in sickle cell ulcers. Lancet 1970; 2: 891

28 Serjeant GR. The clinical picture of sickle cell anaemia in Jamaica. MD Thesis, University of Cambridge, 1971.

29 Serjeant GR, Ashcroft. Shortening of the digits in sickle cell anaemia. A sequel of the hand-foot syndrome. Trop Gegraph Med 1971; 23: 341-346.

30 Serjeant GR, et al 1972. The conjunctival sign in sickle cell anaemia. JAMA 1972; 219: 1428-31

31 Serjeant GR, et al. The clinical features of sickle cell beta-thalassaemia in Jamaica. Brit J Haematolol 1973; 24: 19-30

32 Serjeant GR, et al. The clinical features of haemoglobin SC disease in Jamaica. Brit J Haematolol 1973; 24: 491-500.

33 Serjeant GR et al. Screening of cord blood for the detection of sickle cell disease in Jamaica. Clim Chem 1974; 20: 666-69.

34 Serjeant GR et al. The internal auditory canal and sensori-neural hearing loss in homozygous sickle cell disease. J Laryngol Otol 1975: 89: 453-455

35 Serjeant GR. Sickle Cell Disease, 2nd Ed. Oxford, Oxf Univ Press, 1992.

36 Serjeant GR, De Ceulaer C, Lethbridge R, et al. The painful crisis of homozygous sickle cell disease. Br J Haematol 1994; 87: 586-591.

37 Serjeant GR, Serjeant B. Sickle Cell Disease, 3rd Ed, Oxford, OUP, 2001.

38 Serjeant GR. Blood transfusion in sickle cell disease. A cautionary tale. Lancet 2003; 361: 1659-60 [Graham Serjeant’s adult not-transfused patient in Jamaica went on holiday in USA, was transfused and died!].

39 Konotey-Ahulu FID Konotey-Ahulu FID. Sicklaemic human hygrometers. Lancet 1965; 1:1003 1004 [Listing precipitating causes of crises eg hot weather etc] [

40 Konotey-Ahulu FID. Torrential epistaxis associated with symmetrical facial skin ulceration in sickle cell anaemia. BMJ 1965; 2:859-860 doi:10.1136/bmj2.5466.859

41 Konotey-Ahulu FID, Kuma Eunice. Skeletal crumbling in sickle cell anaemia complicated by Salmonella typhi infection. Brit J Clin Practice 1965; 19: 575-578.

42 Konotey-Ahulu FID. Acute osteomyelitis in African children unmasks un-masks sickle cell disease with salmonellosis BMJ Rapid Response February 1 2014

43 Konotey-Ahulu FID. Erectile dysfunction: Test, please, for Sickle Cell Disease

44 Konotey-Ahulu FID. Sickle cell disease and the patient Lancet 2005; 382-383 Jan 29-Feb 4 [Importance of Circumstances in initiating crises]

45 Konotey-Ahulu FID. Epistaxis from sickle cell disease must not be forgotten 28 March 2012

46 Konotey-Ahulu FID. Valsalva vitreous haemorrhage and retinopathy in sickle cell haemoglobin C disease. Lancet 1997; 349: 1774

47 Archampong EQ, Konotey-Ahulu FID. Biliary tract disease and sickle cell anaemia in Korle Bu Hospital, Accra. Ghana Med J 1975; 14: 176-180

48 Ringelhann Bela, Konotey-Ahulu FID. Haemoglobinopathies and Thalassaemias in Mediterranean areas and in West Africa: Historical and other perspectives 1910 to 1997. Accademia del Scienze Ferrara Atti, volume 74, Anno Accademico 174 1996-97, pages 297-307 [A Century Review]

49 Shoetan Cecilia. I lost my Sickle Cell Disease adult daughter minutes after being given Diamorphine intravenously when she could not breathe. June 3 2008 BMJ

50 Konotey-Ahulu FID, Ringelhann B. Sickle cell anaemia, sickle cell thalassaemia, sickle cell haemoglobin C disease, and asymptomatic haemoglobin C thalassaemia in one Ghanaian family. BMJ 1969; 1: 607-612. [4 Hbnopathy phenotypes]

51 Konotey-Ahulu FID. Patterns of clinical haemoglobinopathy. East African Med J 1969; 46: 149-156 (With tables that distinguish phenotypes clinically)

52 The Globe and Mail, Toronto. Tough flights for mosquito nets. “If they are safe for babies and mothers in Africa, why are they not safe enough in Canada for a week?” 18 August 2006.

53 Konotey-Ahulu FID. Malaria and sickle cell disease. BMJ 1971; 2: 710-11.

54 Djabanor FFT, Reindorf CA, Konotey-Ahulu FID. The effect of sickle cell disease on Ghanaian children. In First International Publication No (HSM) 1974; 73-9141: 70-87

55 Konotey-Ahulu FID. Malaria and sickle cell: “Protection?” Or “No Protection?” – Confusion reigns. BMJ Rapid Response October 13 2008.

56 Olujohungbe A, Cinkotal I, Yardumian A. Hydroxyurea therapy for sickle cell disease in Britain. BMJ Editorial 1998; 316: 1689. “Many patients are unwilling to take the drug.”

57 Olujoungbe Ade. Bi-directional trust is needed in pain management in sickle cell disease. BMJ Rapid Response 2 July 1999 to Maxwell K, Streetly A, Bevan D BMJ 1999; 318: 1585-1590.

58 Konotey-Ahulu FID. Ethical issues in pre-natal diagnosis. BMJ 1984; 289: 185

59 Konotey-Ahulu FID. Refusing to provide a pre-natal test for refusing later termination of pregnancy; can it ever be ethical? BMJ Rapid Response. November 20 2006

60 Konotey-Ahulu FID. The Sickle Achievers (1). Ghanaian Times, July 23 2005 “I cannot think of a single Ghanaian family that did not have or know of someone with sickle cell disease – known by Tribal names”

61 Konotey-Ahulu FID. The Sickle Achievers (2) Ghanaian Times. August 13 2005 “Some time ago I coined the term for cold season Rheumatism which Europeans call Sickle Cell Disease … The ACHEACHE Syndrome (1 ACHE from each parent”. Makes it easy for Genetic Counselling.

62 Konotey-Ahulu FID. Genetic Counselling in sickle cell disease. BMJ 1969; 3: 235 (Put ‘ACHE’ for ‘BAD’) PERSONAL VIEW doi:10.1136/bmj.2.5648.48

63 Konotey-Ahulu FID. Sickle cell disease. The Case for Family Planning ASTAB Books Ltd, Accra, 1973

64 Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1836 [doi:10.1016/50140-6736(07)61771-1]

65 Ringelhann B, Konotey-Ahulu FID, Yawson G, Bruce-Tagoe AA, Miller A, Huisman THJ. Alpha Thalassaemia in West Africa. Symposium in Medical Genetics, Debrecen-Hajduszoboslo, Hungary (April 26-29 1976), pp 614-616 in Szabo G and Papp Z, Eds Medical Genetics, Excerpta Medica 1977

66 Konotey-Ahulu FID. Maintenance of high sickling rate in Africa: Role of polygamy. J Trop Med Hyg 1970 Jan; 73(1): 19-21 (38 References)

67 Bonney GE, Konotey-Ahulu FID. Polygamy and genetic equilibrium. Nature 1977; 265: 46-47 doi:10.1038/265046a0..n5589/pdf/265046a0.pdf

68 Konotey-Ahulu FID. Male procreative superiority index (MPSI): The missing co-efficient in African anthropogenetics. BMJ 1980; 291: 1700-02.

69 Konotey-Ahulu FID. The Male Procreative Superiority Index (MPSI): It’s relevance to genetical counselling. In FIFTY YEARS OF HUMAN GENETICS – A Festschrift and liber amicorium to celebrate the life and work of GEORGE ROBERT FRASER. Ed: Oliver Mayo & Carolyn Leach. Wakefield Press 2007, 1 The Parade West, Kent Town, Sth Australia

70 Omaboe Letitia, Konotey-Ahulu FID. The Second International Conference on The Achievements of Sickle Cell Disease Patients. Accra 19th July 1995 Conference Brochure

71 Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6-phosphate Dehydrogenase Deficiency Incidence in Sickle Cell Disease patients in Accra. Ghana Med J 1977; 16: 4-9

72 Konotey-Ahulu FID. G6PD Deficiency in Ghanaians. How to recognise it.
Click ‘BLOG’ on and click on January 2008 for 20 answers.

73 Konotey-Ahulu FID. WORLD SICKLE CELL DAY 19th June 2012 Featuring (i) The Inheritance of Sickle Cell Disease (ii) The Person with Sickle Cell Disease (iii) The Teenager with Sickle Cell Disease (iv) The Adult with Sickle Cell Disease.

74 WORLD SICKLE CELL DAY 19th June 1013. Broadcast Interview by Tunu Louise Roberts emphasising Public Health Approach to management, and Genetic Counselling with NORMACHE as Trait and ACHEACHE as Disease or (Home Page) Also accessed as [Suitable for patients and parents

75 Konotey-Ahulu FID. Morphine for painful crises in sickle cell disease. BMJ 1991; 302: 1604 (Commenting on Professor Chamberlain’s recommendation of morphine in pregnancy in sickle cell disease – BMJ 1991; 302: 1327-30). “In obstetrics what happens too foetal respiration when morphine is used?”

76 Konotey-Ahulu FID. Management of patients with sickle cell disease. African Journal of Health Sciences 1998; 5: 47 [On Sally Davies and Lola Oni in BMJ 315: 656-60 “The Central Middlesex management protocol uses morphine infusions”] Response: “I fear Davies and Oni’s statement will make morphine the accepted drug for sickle crisis management. The consequences for such an approach are dire, especially when some UK hospitals are already making diamorphine their first choice”. And what did NCEPOD find in 2008?

77 Konotey-Ahulu FID. Opiates for sickle cell crisis. Lancet 1998; 352: 651-2

78 Konotey-Ahulu FID. Opiates for pain in dying patients and in those with sickle cell disease BMJ Response 11 Oct 2007

79 Konotey-Ahulu FID. Current “hit and miss” care provision for sickle cell disease patients in the UK. BMJ Rapid Response 16 July 2008.

80 Konotey-Ahulu FID. Management of sickle cell disease versus management of the sickle cell disease patient. BMJ Rapid Response 17 Sept 2008

81 Konotey-Ahulu FID. Clinicians facing conflicting recommendations. Use common-sense? December 2008.

82 Konotey-Ahulu FID. Poor care for sickle cell disease patients: This wake-up call is overdue. BMJ Rapid Response (May 28) BMJ 2008; 336: 1152 to Susan Mayor’s “Enquiry shows poor care for patients with sickle cell disease” on National Confidential Enquiry into Patient Outcome and Death (NCEPOD) REPORT “SICKLE: A Sickle Crisis? (2008)”

83 Konotey-Ahulu FID. Inquest into diamorphine deaths: Does NCEPOD sickle cell patients report warrant a similar inquest? BMJ Rapid Response March 7 2009

84 Konotey-Ahulu FID. UK Drug related deaths are still rising: So where is NICE? BMJ Rapid Response to S Mayor: “UK drug related deaths are still rising.” Sept 6 2009. .

85 Konotey-Ahulu FID. The politics and economics of pain relief in the West and Third World. Response Aug 24 to T Anderson doi:10.1136/bmj.c3800 Aug 11 2010

86 Konotey-Ahulu FID. Management of an acute painful sickle cell episode in hospital. NICE Guidance is frightening! BMJ Rapid Response Sept 7 2012 (42 References)

87 Konotey-Ahulu FID. Opiods for chronic non-cancer pain – Chemotherapy – Clinical Guidelines: Where does ultimate responsibility lie? BMJ Rapid Response June 25 2013

88 Dankwa Akosua M. Sickle Cell patients deserve to live. BMJ Rapid Response to NCEPOD Report 11 July 2008 [Adult “SS” wrote to the BMJ: “I know 60 and 70 year olds (yes, sickle cell patients) who have got to that age without ever receiving this as treatment”.

89 Chapman Nyaho Mawunu. Poor care for the sickle cell disease patient: “Pain won’t kill him, but Morphine could”. BMJ Rapid Response to NCEPOD by adult Sickle cell haemoglobin C disease grand-mother 17 June 2008

90 Amanor-Boadu Dorothy, Bruce-Tagoe Alexander, Konotey-Ahulu Felix. The Third International Conference On The Achievements Of Sickle Cell Disease (ACHEACHE) Patients, Accra – 19th July 2010. Adeko Ltd, Accra ISBN: 978-9988-1-3927-8 “One known ‘SS’ man with a PhD, who had never in his 63 years been transfused though Hb level was never above 8.8 g/dL and who had never been prescribed Hydroxyurea, astonished delegates when he announced ‘I do not remember when I last took a pain killer for my sickle cell anaemia. Drink plenty of water, avoid malaria, and have a positive attitude to life’” [page 15] Quoted in full in link of Reference 85 above. [Dorothy Amanor-Boadu herself is a 59-year old “SS” Nurse Oncologist in Accra].

Competing interests: Coming from a family with 3 Sickle Cell Disease (scd) siblings I find it very difficult to remain detached when the received wisdom in the management of scd patients that was proven by NCEPOD to be harmful is still endorsed by NICE.

FELIX ID KONOTEY-AHULU, Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana

Consultant Physiician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd., Phoenix Hospital Group, London W1G 9AL

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