felix Konotey-Ahulu <email@example.com> wrote:
Dear JudithThanks for writing. I'm off somewhere at the moment so I cannot respond in detail to your letter.Where are you? What do you do?I'm copying your letter to a super lady who can help you. Her name is Mawunu Chapman Nyaho.Meanwhile, go to my website www.konotey-ahulu.comOn the Home Page you will find on the right hand side 'PUBLICATIONS'. Click on it. That brings you to a page with dates from 1965. Scroll down the years to the Year 2001. Read all 4 articles listed in that year before getting back to me.Felix Konotey-Ahulu
G6PD Deficiency in Ghanaians: How to recognise it
In their instructive seminar Professors Capellini and Fiorelli put
I declare that I have no conflict of interest
Felix I D Konotey-Ahulu
1 Capellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet 2008; 371: 64-74.
2 Owusu SK. Glucose-6-phosphate dehydrogenase (G-6PD) deficiency in the causation of disease in
3 Owusu SK, Foli AK, Konotey-Ahulu FID, Janosi M. Frequency of Glucose-6-phosphate dehydrogenase deficiency in typhoid fever in Ghana. Lancet 1972; 1: 320.
4 Adu D, Anim-Addo Y, Foli AK, Yeboah ED, Quartey JKM. Acute renal failure and typhoid fever.
Medical Journal 1975; 14: 172-174.
5 Owusu SK, Addy JH,
associated with glucose-6-phosphate dehydrogenase deficiency. Lancet 1972; 1: 1255-1257
6 Owusu SK. Absence of glucose-6-phosphate dehydrogenase in red cells of an African. BMJ 1972; 4:
7 Owusu SK. Clinical manifestations of glucose-6-phosphate dehydrogenase (G-6PD) deficiency
8 Konotey-Ahulu FID. Glucose-6-phosphate dehydrogenase deficiency and sickle cell anaemia. New Eng J
Med 1972: 287: 887-888.
9 Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6-pogosphate dehydrogenase deficiency
incidence in sickle cell disease patients in
10 Konotey-Ahulu FID. The sickle cell disease patient: natural history from a clinico-epidemiological study
of 1550 patients of Korle Bu Hospital Sickle Cell Clinic.
A’Domeno Co 1996.
11 Konotey-Ahulu FID. Alpha thalassaemia nomenclature and abnormal haemoglobins. Lancet 1984; 1:
12 Bedu-Addo G. Chloroquine induced bilateral ptosis. Trans Roy Soc Trop Med Hyg 2006;
13 Morrow RH, Smetana HF, Sai FT, Edgecomb JH. Unusual features of viral hepatitis in
Ann Intern Med 1968; 68: 1250-1264.
14 Ringelhann B, Dodu SRA, Konotey-Ahulu FID, Lehmann H. A survey for haemoglobin variants,
thalassaemia and Glucose-6-phosphate dehydrogenase deficiency in northern
1968; 7: 120-124.
16 Konotey-Ahulu FID. Probing anecdotes in traditional African therapeutics. African Journal of Health
Sciences 1194; 1: 53-56.
17 Fenwick G,
in Food Science and Nutrition 1985; 23: 1-73.
18 Konotey-Ahulu FID. Missing the wood for one genetic tree? In, The First International Symposium on
the Role of Recombinant DNA in Genetics. Proceedings, Chanai, Crete –
Loukopoulos D, Teplitz R. Athens, P Paschalidis 1986, pages 105-116.
19 Konotey-Ahulu FID. The male procreative superiority index (MPSI): its relevance to genetical
Festschrift and liber amicorum to celebrate the life and work of George Robert Fraser.
Wakefield Press, August 2007 pages 48-50.
20 Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1826-27.
Ethnic minorities and sickle cell disease
SIR,-Dr John Black's article (30 March,p 984) is most helpful.
Many family doctors are quite familiar with much of what he has so lucidly stated. The reason why many children (and adults) with sickle cell disease continue to die “before their time” in the UK and elsewhere is failure to appreciate certain facts relating to presentation, a failure of anticipation, and what I usually call a combination of circumstances.
Firstly, presentation. Epistaxis, priapism, enuresis, numbness of the lower lip, sudden aphasia, hemiplegia, blood in the urine, deep jaundice, large tummy, nails white as a sheet, loud precordial murmurs, bruit de diable at the root of the neck, unexplained fever, cough with tachypnoea, fatigability, swollen hands may each and severally be the first indication that the child has sickle cell disease.'
Alternatively, a child's condition may have nothing to do with sickle cell disease but may later be worsened by it-for example, within seven days lobar pneumonia can become pneumonia plus infarction plus pulmonary abscess.' Presentations may also differ from those described in the textbooks.
(1) Teenagers of 12, 13, 14, or 15 years with SS disease may have spleens which, far from vanishing, are enlarged.
(2) The child with genuine SS disease (parents AS, AS) with haemoglobin of 12-3 g/dl should not be treated as having sickle cell trait, because the potential for devastating in vivo sickling is there.
(3) Extreme fatigability with “nails as white as a sheet” during an attack of measles indicates an aplastic crisis from the measles virus.
Haemoglobin drops to 2-5 g/dl within 48 hours. As little as 100-150 ml of packed cells for a 5 year old child and not more than one unit for a 15 year old given over eight hours is lifesaving, even when the haemoglobin was less than 3 g/dl to start with.
Secondly, clinicians should anticipate disasters in patients with sickle cell disease. For example, if perioperative fluid therapy is not planned for a child about to undergo surgery the child will end up with a stroke. Anaesthetists are now more careful than ever so their success is virtually 100″,,, but fluid management before, during, and after operation often leaves a lot to be desired. The clinician should also be aware that the patient who has been admitted and discharged twice within the past week has come in again to die unless the whole management is overhauled.
Thirdly, there are many dangerous combinations of circumstances that may lead to disaster for the unwary.
(1) Personnel: the houseman or GP is away for the weekend; the locum has little clue about what is happening, and goes by the packed cell volume or haemoglobin concentration rather than the patient's condition; the casualty department may refuse to admit the patient, quibbling over whether the patient should go under a physician or a haematologist.
(2) Clinical: abdominal pain might be due to sickle cell crisis,2 3 or the crisis could also have been precipitated by acute appendicitis.'
(3) Genetic: hardly any west African with sickle cell anaemia is without one or more of the other hereditary erythrocytopathies: 86'U/ have concomitant lac or 2ac thalassaemia, and, although this is supposed to ameliorate the SS phenotype, 2OO, of the men and 16″(, of the women also have glucose-6-phosphate dehydrogenase (G6PD) deficiency.4 Contrary to what is claimed from the USA,G6PD deficiency on top of sickle cell disease is an added liability4 (Luzzato L, paper at International Symposium on Sickle Cell Anaemia, Abidjan, Ivory Coast, 1975).
Workers in the UK should always identify which of their patients have G6PD deficiency, because several antimicrobial agents and analgesics which are given to help may in fact harm the patient.
FELIX KONOTEY-AHULU London WIN IAA
1 Konotey-Ahulu FID. The sickle cell diseases. Clinical manifestations including the “sickle crisis.” Arch Intern Med 1974;133:611-19.
2 Hendrickse RG. Sickle cell anaemia in Nigerian children. Cent AfrJa Med 1960;6:45-57.
3 Valman HB. ABC of I to 7. London: British Medical journal, 1982:21.
4 Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6 phosphate dehydrogenase deficiency in sickle cell disease patients in Accra. Ghana Med 7
Source : British Medical Journal 1985 (20 April), Volume 290, page 1214.
Thanks to Dr Geraint James who introduced me to his wife Professor Sheila Sherlock in the early 1960's, I became one of her research fellows on Stanley Shaldon's famous Kidney Unit at the Royal Free Hospital School of Medicine . Working around the clock sometimes, Stanley Shaldon's unit was the first in Europe to introduce home haemodialysis. If, as Professor Christopher Blagg states (5 Jan) “By 1971 58.8% of patients on dialysis in the UK received dialysis at home”  this was mainly due to Stanley Shaldon's extraordinary drive .
As Ken Farrington pointed out “self supervised haemodialysis performed in the patient's home was pioneered ..largely to cope with increasing numbers” . In the tropics, especially west Africa, many deaths from renal failure are an every day occurrence. Only the very wealthy can extend their lives with renal failure, for only they can afford to run generators with uninterrupted electricity in their homes. I would have liked to see some costing in Professor Blagg's editorial .
When Ghanaians woke to the fact that Kwame Nkrumah's “Free Health Care for everybody” was no longer an option, and the government haemodialysis centres collapsed, it became obvious that only the very wealthy could survive end stage renal failure. Not so in the UK and USA, where the poor appear to be able to benefit from expensive health care delivery programmes.
The wearable haemodialysis device recently described by Andrew Davenport and colleagues  would be ideal for the African situation, where ambulant patients can just switch on the system when ominous symptoms after straying from prescribed dietary requirements begin to appear . But how much would the wealthy African be expected to set aside for this?
Ghana has succeeded in introducing a National Health Insurance Policy, but because of the sheer numbers involved, it is incapable of covering haemodialysis whether in hospital, at home, or on foot.
Competing interests: None
1 Konotey-Ahulu FID, Baillod RA, Comty CM, Heron JR, Shaldon S, Thomas PK. Effect of periodic dialysis on the peripheral neuropathy of end -stage renal failure. BMJ 1965; 2: 1212-1215.
2 Blagg CR. Haemodialysis. Wide variations in availability exist, and the UK lags behind some other countries. BMJ 2008; 336: 3-4 (Editorial 5 January)
3 Baillod RA, Comty CM, Ilahi M, Konotey-Ahulu FID, Sevitt L, Shaldon S. Overnight haemodialysis in the home. Proc Eur Dial Transplant Assoc 1966; 2: 99
4 Farrington K. Modality selection and patient outcome. In Akoh JA, Hakim N S (Eds) Dialysis Access – Current Practice, London: Imperial College Press 2001, pp 23-47.
5 Davenport A, Gura V, Ronco C, Beizai M, Ezon C, Rambad E. A wearable haemodialysis device for patients with end-stage renal failure: a pilot study. Lancet 2007; 370: 2005-2010 (Dec 15).
6 Konotey-Ahulu FID, Anderson G. Treatment of hyperkalaemic cardiac arrest by timely haemodialysis. Ghana Medical Journal 1965; 4: 158-163.
Published in the British Medical Journal on line 10 Jan 2008 http://www.bmj.com/chi/eletters/336/7634/3
Competing interests: None declared
Some thirty features of AIDS in
In 1987 I visited sixteen African countries to acquaint myself with the AIDS situation on the continent. I obtained information from doctors and health workers about many of the countries I could not visit. 1 was refused a visa to go to
A synoptic overview of clinical and other features of AIDS in
Aids is not uniform over the 50 countries in
Age block gap. No patients were found between infancy and teens except the blood trans-fused, thus excluding insect vectors in transmission (Dr. Miriam Duggan and Dr. Sewankambo of
Repatriation AIDS. In my Krobo tribe in
100 % Female preponderance. In certain tribes in
Perineal devastation easily visible from the foot of the bed with undressed patient lying prone (Dr. Mate-Kole, Korle Bu Teaching Hospital,
Virgins and the nulliparous can get AIDS from the first intercourse due to tears (Dr. Mate-Kole, Korle Bu Teaching Hospital,
Pervisemos i.e. ‘persistent virus secreting mothers’ who are asymptomatic but continue to bring forth sick children (Dr. Duggan and Dr. Hanny Friesen, Kampala ; Dr. Chintu, Lusaka).
AIDS Precipitators. Caesarian section and minor procedures like salpingohistograms can turn the asymptomatic into full blown AIDS (Dr. Duggan,
Former Director Ghana Institute of Clinical Genetics, and Consultant Physician, Korle Bu Teaching Hospital,
*Published in: Annales Universitaires des Sciences de la Santé 1987; 4 (4): 541-544.
Symptomatology of Slim : 20-40 % weight loss, persistent diarrhoea, fever, lymphadenopathy, respiratory symptoms, oral candidiasis and amenorrhoea in child bearing women, with frequent previous history of sexual exposure, of blood transfusion, and/or unsupervised injections (Dr. Sezi, Serwadda & colleagues in Kampala, physicians in Dar es Salaam, and in Lusaka and Ndola, Zambia, Dr. Neequaye et al, Ghana) (6, 7, 8).
Intractable Pruritus in adults, and in infants : this could be the commonest cause of
insomnia (Dr. Chintu and Dr Subhash Hira,
Generalised hyperpigmentation with crazy-pavement dermatopathy (Professor. Bodo,
Dupuytren's Contracture (Professors Badoe, Archampong and Jaja's new book
“Surgery in the Tropics” p.210 shows this physical sign as a complication of plaque Kaposi's sarcoma) (9). Professor Anne Bayley (
) showed me two cases of aggresive atypical Kaposi’s sarcoma (AAKS) with this sign. Lusaka
Elephantiasis of limbs (upper and/or lower) and genitals from AAKS (Professors
Multidermatomal Herpes Zoster heralds full blown AIDS (Dr. Subhash Hira,
Adult Kwashiorkor. I saw this syndrome in my Krobo tribe where girls with Repatriation AIDS whose diarrhoea must have included creatorhoea with consequent protein calorie malnutrition.
Accelerated orphan Kwashiorkor. 1 saw this at
Tuberculous pericarditis as a common complication (Dr. Mboussa,
Non-AIDS Diseases producing HIVseropositivity. (Dr. Fleming and Rosemary Mwendapole,
) (10). Liver pathology can confuse results and Tanzanian physician Professor Aaron Massawe postulates “immunoligical turbulence” with Ndola, Zambia
Anti-TB treatment to fake seropositivity.
Radiological “bat's wing” lung in AAKS (Professors Bugingo
Sworl Facies: a characteristic “Strikingly worried look”, on the faces of the more discerning patients I visited on ward rounds in
Relative Paucity of full blown AIDS. It came as a surprise to find a Zairean man and wife, and a Kenyan itinerant salesman as the only AIDS patients in the 2100-bed
Patients are not dying “like flies” as world media report (13). When
Seropositive twin baby lives while seronegative twin dies. Born to a pervisemo (ie persistent virus secreting mother) the infected twin lived while the seronegative twin died from AIDS, in
AIDS has not changed health priorities in Africa. I cannot speak for
Disagreement about seriousness of the problem. Some expatriate workers in
prophesy doom, but most indigenous doctors while not underestimating the gravity in some countries, consider forecasts exaggerated (
Grade I, not much of a problem; Grade II, a problem exists; Grade III, a great problem;
Grade IV, an extremely great problem, and Grade V, a catastrophe (13). I recommend this approach to health workers and urge them to have their own grading criteria. Clinicoepidemiology rather than seroepidemiology will best bring out the truth about the real state of affairs of each country (1).
The Juliana Phenomenon. AIDS in the lake region of Tanzania, bordering Zaire, is known as “Juliana” because, as one prostitute told me, “A few years ago when the Navy visited Mombasa with 9, 000 troops, some of our girls who travelled there for business were given T shirts with Juliana marked on them. Many of those who wore the Juliana shirts have since had Slim and died”.
Non-Africans with AIDS. The 6 patients seen in Mombasa with AIDS (1983-1987) by a specialist, were a Zairean, and 5 non-Africans from Europe and the USA; in South Africa all the AIDS has so far occured in non-blacks (Dec 1987), and in Zaire at least 21 Europeans and Americans were known to have had AIDS (Source : Resident Greek Businessman). HIV-2 in
Complete Cure Anecdotes were heard in
It is important that doctors living and working in
And she replied, “Oui Monsieur, mais je leur demande une grande somme d'argent”
(17). So, one should now use “peno-vaginal sex” for so called heterosexual sex, and “anal sex”or “sodomy” for what is called “homosexual relationship”. Anal sex has been demanded sometimes for money in several countries in
Finally the kind of research that will help Africans curtail AIDS does not have to be the vaccine orientated research of the developed countries. Public Health methods and clinical epidemiology are
I thank the clinicians who took me on ward rounds during my recent
1. KONOTEY-AHULU F I D, (1987) Clinical epidemiology, not seroepidemiology, is the answer to
2. NEEQUAYE A.R., NEEQUAYE J,., MINGLE J.A., OFORI-ADJEl D. (1986). Propondernace of females with AIDS in
3. NEEQUAYE A. R., ANKRA-BADU G.A., AFRAM R.A. (1987). Clinical features of human immunodeficiency virus (HIV) infection in
4. KONOTEY-AHULU FID (1987) AIDS: origin, transmission and moral dilemmas. Journal of the Royal Society of Medicine 80: 720.
5 KONOTEY-AHULU FID (1987). Surgery and risk of AIDS in HIV positive patients. Lancet ii: 1146
6. SERWADDA D., MUGERWA R.D., SEWANKAMBO N.K, LWEGABA A.,
CARSWELL J. W, KIRYA G.B., BAYLEY A.C., DOWNING R.G., TEDDER R.S., CLAYDEN
7. SEWANKAMBO N., MUGERWA R.D., GOODGANER R.,CARSWELL JW, MOODY A., LLOYD G., LUCAS S.(1987). Enteropathic AIDS in
An endoscopic, histological and microbiological study. AIDS 1:9
8. SEWANKAMBO N., CARSWELL J. W,. MUGERWA R.D., LLOYD G., KATAAHA P.,
DOWNING R.G., LUCAS S.(1987) HIV Infection through normal heterosexual contact in
9. BADOE E.A., ARCHAMPONG E.Q., JAJA M., Eds, Principles and Practice of Surgery in the Tropics.
10. FLEMING A.F., KAZI A.R., SCHEINEDER J., GUILLOT F., MWENDAPOLE R., WENDLER I., HUNTSMANN G. (1986). Comparison of HTLV-111 in some Zambian patients. AIDS Forschung (AIFO) 8: 434.
11. BAYLEY C A. (1983). Aggressive Kaposi’s sarcoma in
12. MONEKOSO G. In Second International Conference on AIDS in
13.KONOTEY-AHULU F I D. (1987). AIDS in
14. BRUCKER G, BRUN-VEZINET F,
15. KINGMAN SHARON. (1987). The Portuguese connection. New Scientist, 15th October, p 27
16. QUARTEY J K M, MATE-KOLE M O, OKAI GLORIA, BENTSI CECILIA, DJABANOR F F T, KONOTEY-AHULU F I D. (1988). Domicilliary management and prognosis of AIDS in the Krobo region of South east
17. KONOTEY-AHULU F I D. Extensive palatal echymosis from felllatio – a note of caution with AIDS at large. (1987). British Journal of Sexual Medicine, 14: 286-287
18. PALLANGYO K J, MBAGA I M, MUGUSI F, MBENA E, MHALU F S, BREDBERG U, BIBERFIELD G. (1987). Clinical case definition of AIDS in African adults. Lancet, ii: 972.
First published in Annales Universitaires des Sciences de la Santé 1987; 4: 541-544
Postscript January 2008: What has happened in
(i) Guisselquist D, Rothenberg R, Potterat J, Drucker E. HIV infections in sub-Saharan Africa not explained by sexual or vertical transmission. Internatrional Journal of HIV & AIDS Oct 2002: Vol 13: pages 657-666.
(ii) Fassin D, Schneider H. The politics of AIDS in
(iii) Financial Times. AIDS in
(iv) Konotey-Ahulu FID. AIDS in
Last week’s Scienceonline (December 6 2007) had a most instructive report by American scientists, Timothy Townes, Rudolph Jaenisch, Jacob Hanna, and others from the University of Alabama and Massachusetts Institute of Technology’s Molecular Biology Department. They proved that mice that had been genetically modified in the laboratory to develop sickle cell anaemia can be made to produce embryonic stem cells from cells collected in the skin of their tails. Embryonic stem cells are known to be capable in later life of developing into any of different tissues. These so-called induced pluripotent stem cells (IPS cells) were then made to produce healthy haemoglobin by replacing the defective beta-globin sickle cell gene (S) with normal adult gene producing haemoglobin-A (nothing to with Blood Group A!). These were then injected into similar mice with sickle cell anaemia and, lo and behold, normal adult haemoglobin was produced and the mice improved clinically.
So far so good news, but the cautionary tale is this: it needs the help of retroviruses to shift the genes around to give the required result, and where did you last hear of retroviral activity? AIDS, of course and as one of the scientists put it: “We need a delivery system that does not integrate itself inside the genome. Retroviruses can disrupt genes that should not be disrupted or activate genes that should not be activated”.
If skin cells of mice can be made to behave like embryonic cells which obey orders given them to produce this or that, the next question is this: “Can human skin cells from sickle cell anaemia patients be similarly programmed to this end?” We can’t wait, though we know that transplanting induced pluripotent cells carry a high cancer risk, not to mention the effect of the high dose of X-rays required to destroy the faulty blood cells in the body before the new ones are planted.
Two more comments:
(i) How much would a New Yorker pay for the procedure, if it proved successful in humans? Cost itself should not disqualify a process that would abolish pain, but one needs ask the question. Until persons producing inherited ‘ACHEACHE’ haemoglobin begin to be able to produce acquired ‘NORMNORM’ or ‘NORMACHE’ haemoglobin through IPS cell manipulation, we need to pursue the public health measures that I have been stressing for preventing crises, and we must continue urging on our kith and kin genetic counselling with voluntary family size limitation [Konotey-Ahulu FID, The Lancet, 1 December 2007, volume 370, pages 1826-27: “Need for ethnic experts to tackle genetic public health”]
(ii) A report by one Eric Bender in
Opiates for pain in dying patients and in those with sickle cell disease
Dorothy Logie and Mhoira Leng’s report of 6 October describes a conference in Nairobi which highlighted opiophobia – the fear of using morphine therapeutically – “as a big obstacle facing palliative care services in the (African) continent” . The conference was concerned with dying patients. In his rapid response, however, Jecko Thachil concentrated on the use of opiates in sickle cell disease patients. He states that while “very high amounts of opiates (often in hundreds of milligrams) are often required for the patients who suffer from recurrent sickle crises” , the expected analgesic effect leaves much to be desired, not to mention side-effects. Dealing with terminally ill patients is one thing; opiate administration to sickle cell disease patients is something else entirely.
Dr Thachil feels an “increased need for additional education regarding sickle cell disease…” Has he examined the experiences of two physicians who personally supervised thousands of people with sickle cell disease on both sides of the Atlantic continuously for years? [3, 4] Treating sickle cell disease is not the same as managing the sickle cell disease patient ; the difference in the two approaches tending to separate haematologists on the one hand, from physicians and family practitioners on the other. When a white British consultant physician in a London teaching hospital complained that “the haematologists here have created a cohort of addicts”  a white consultant haematologist in another London teaching hospital tore strips off him, accusing him of being a racist for depriving suffering black patients of pain relief, an attack which made me respond thus: “White physicians who, at the risk of being misunderstood by (that haematologist), voice their displeasure at what they see happening on their wards deserve commendation, not condemnation” .
Not far from where Dr Jacko Thachil works, also on Merseyside, “during a ward round in a provincial teaching hospital with consultant haematologists on March 6, 1997, I was shown a woman who had been on continuous opiate infusion since September, 1996” . I went on to say in my Lancet communication: “Far from the consultants taking umbrage because I pointed out that the patient could not have been in sickle cell crisis for 6 months, they were happy to discuss with me the way forward” . Dr Thachil feels “an increased need for additional education regarding …addiction to pain medication…and treatment of pain” and he concludes “but who and where these should be focussed on is a matter for debate” .
Not a matter for debate at all in my opinion: May I suggest to him certain facts he might wish to probe in his quest for education?
(a) “In Jamaican experience ..morphia or its derivatives are rarely used or necessary”  How did Graham Serjeant achieve this?
(b) “Most painful crises may be treated in a day-care centre, the patient returning home in the evening”  How is this possible if hooked up on morphine or diamorphine pump “as in the recommended UK protocol”? 
(c) “We are convinced that the chest syndrome in the UK and the USA is not entirely unrelated to the routine use of opiates in those countries for sickle cell crises”.  Some nurses I am in touch with can write an MSc thesis on this.
(d) Goodman from the USA (where diamorphine is banned for patients) found the use of ketorolac in painful sickle cell crises as efficient as morphine but without the latter’s respiratory depression . So why do British haematologists prefer to use morphine and diamorphine? Answer: “Ketorolac has no product licence in the UK for this indication” 
(e) Two questions that I have asked British Haematologists several times but which have never been answered, and which Dr Thachil may now ask the National Institute of Clinical Excellence (NICE) for help in answering:
(i) “Why do West African and West Indian patients with sickle cell disease who did without morphine in their countries have to be given morphine pumps during sickle cell crises when they come to the United Kingdom?” [6, 13]
(ii) If pain from whatever cause deserved the most potent analgesic, and dysmenorrhoea has been known to be intolerably painful, would a British haematologist “not consider it unwise for a hospital to administer diamorphine as routine management of young women?” 
Four encouraging signs have emerged in the UK since I have been voicing my displeasure at the use of diamorphine and morphine pumps for patients with sickle cell crises: (1) Some haematologists in the UK and the European continent have abandoned the practice in spite of what the ‘approved protocol’ displays in the emergency rooms. (2) Some sickle cell disease patients have become more vocal in their displeasure of the practice. To them ‘opiophobia’ is not to be condemned , but commended. (3) Some family practitioners are looking after their patients at home, using intravenous fluids and other than powerfully addictive opiates to help these patients instead of submitting them to hospital care. (4) A clearer grasp of the causes of crisis has put more emphasis on public health measures (fluids, warmth, treatment of infections, dressing properly, anticipating hazards, immunisations, avoiding tobacco and alcohol), enabling patients prevent crises and helping them use the excellent non-sickling genes they have inherited from their parents to achieve as much of their full potential intellectually as possible .
It is therefore not surprising to find that the sickle cell disease patients who have become lawyers, teachers, businessmen and women, nurses & midwives, pharmacists, and even doctors are those whose haematologists have abandoned the opiate culture. Occasionally, however, one found even professors of haematology who would defend the prescribing of diamorphine for a sickle cell disease patient with severe difficulty in breathing. “Chest syndrome” was always there to blame, if the patient died .
When in my genetic counselling and family size limitation (GCFSL) drive in Ghana and in the Ghanaian community here in the UK I sense that the urgency of my message is being glossed over I tell my fellow countrymen and women in plain language that if they continue to procreate at the rate they are doing, and more sickle cell disease (ACHE/ACHE) patients are born, the chances are that in the UK they may end up on a heroin drip. This concentrates the mind, and they listen to me. “One in three of you is a NORM/ACHE. Do you want to end up with ACHE/ACHE children who will be given heroin for pain?” [http://www.konotey- ahulu.com/diagram.asp]
Felix I D Konotey-Ahulu MD(Lond) DSc (UCC) FRCP DTMH FGA FGCPS FAAS FTWAS
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor, Ten Harley Street, London W1N 1AA, England.
Conflict of interest: None declared
1 Logie D, Leng M. Africans die in pain because of fears of opiate addiction. BMJ 2007; 335: 685
2 Thachil J. The fear of opiate addiction – not unique to Africa. Rapid response BMJ 2007, 8 October.
3 Serjeant GR. Sickle cell disease. Oxford: Oxford university Press, 1992 (Second Edition)
4 Konotey-Ahulu FID. The sickle cell disease patient. London: Macmillan 1991; Waftord: Tetteh-A’Domeno Co, 1996.
5 Konotey-Ahulu FID. Sickle cell disease and the patient. Lancet 2005; 365: 382-383.
6 Konotey-Ahulu FID. Opiates for sickle cell crisis? Lancet 1998; 351: 1438.
7 Konotey-Ahulu FID. Opiates for sickle cell crisis. Lancet 1998; 352: 651-652.
8 Serjeant GR. Sickle cell disease. Oxford. Oxford University Press, 1985, page 204.
9 Serjeant GR. Sickle cell disease. Lancet 1997; 350: 725-730.
10 Ringelhann B, Konotey-Ahulu FID. Hemoglobinopathies and thalassaemias in Mediterranean areas and West Africa: historical and other perspectives 1910 to 1997. Atti dell’Accademia delle Scienze di Ferrara 1998; 74: 267-307.
11 Goodman E. Use of ketorolac in sickle cell disease and vaso- occlusive crisis. Lancet 1991; 338: 641-642.
12 Liesner RJ, Vandenberghe EA, Davies SC. Analgesics in sickle cell disease. Lancet 1993; 341: 188.
13 Konotey-Ahulu FID. Morphine for painful crises in sickle cell disease. BMJ 1991; 302: 1604.
Competing interests: None declared
There is no mystery [1, 2] about “kwashiorkor”, which is a word from a Ghanaian tribe, my very own Krobo/Dangme/Ga megatribe [3, 4]. Indeed, it was when I was a toddler that the remarkable white woman based at the Princess Marie Louise Hospital in Accra (before the Gold Coast became Ghana) first described the condition which was brought to her attention by my fellow tribes folk [5,6].
Imagine my utter astonishment when, coming over to England to read Medicine in London University, I heard Lecturer after Lecturer state with unwarranted confidence that “kwashiorkor means red hair in an African language”. The word means no such thing in my tribe. Kwashiorkor, as I once pointed out to a British Professor of Paediatrics in this very medical journal “is, primarily, a sibling positional word, which requires careful explanation to the non-native” . It is a reflection of the 'birth position' of the sufferer, before it is a pathology , a fact emphasized by Dr Cicely Willams whose descriptions of the condition have not been bettered by anyone [5, 6, 7, 8].
Apart from being a Krobo tribesman, I have a further reason for claiming that I know more about Kwashiorkor than all the non-Ghanaian experts that have written volumes about the syndrome (Cicely Williams excepted). And I say that for this reason: In my tribe, it was said of me (the second child) the day my younger brother was born while I was not yet completely weaned – “afor ese kwasiorkor”  which, literally means “it has been born after him kwasiorkor”, stressing my positional risk of being sandwiched between my elder brother, Agbetey, who was just 13 months older than me, and the just arrived sibling who was also just 17 months my junior. See http://www.konotey-ahulu.com/images/generation.jpg for the first 3 children of my parents Rev & Mrs Konotey-Ahulu in Generation VII, and note how closely spaced we were.
The whole tribe expected me to develop kwashiorkor because of the birth of my brother. “The reason I escaped the syndrome first described by Dr Cicely Williams [3, 4] was simply because my educated parents took the same steps that Professor Marsden described from Brazil : I was fed with beans, eggs, milk, minced meat, and Ovaltine” . Less fortunate relatives of my age in my tribe, whose parents also had 3 children in less than 3 years, “and of whom it was also said 'afor ese kwasiorkor' went on to develop kwashiorkor because they could only afford to be fed with maize products such as akasa and k enky” .
I went on to make the point that: “The sibling positional word became almost invariably associated with the syndrome Cicely Williams was investigating, and although the term kwashiorkor was occasionally attached to a child who never went on to get the disease ” (just as in my own case), whenever the the syndrome kwashiorkor was seen in a child there was, more likely than not, very close proximity to a younger sibling” . Exceptions, of course, occurred, “when children were orphaned” (as would be the case in Malawi ravaged with AIDS), regardless of sibling positioning, or multiple births. Indeed, I described such children (with photographs) on my AIDS tour of African countries [Ref 10 – See Figure 6.14, page 124]. I also described in a man who was mistaken for suffering from HIV-AIDS [Ref 10, photographs on pages 80-82] what has been called the Adult Kwashiorkor syndrome “in which diarrhoea, pitting oedema, hepatomegaly, dermatopathy, mental apathy, and hair changes result from protein energy malnutrition as, for example, in the creatorrhoea and steatorrhoea of severe alcoholic chronic pancreatitis”. This syndrome was completely reversible in the man described  when his alcoholism [he was on one litre and half of gin a day] was treated, diabetes reversed, pancreatin supplied, and protein supplements given [See remarkable change in just 6 months – Ref 10]. Should we have spent research funds finding out how much antioxidants this man's diet contained?
I had the enormous privilege of meeting up with 93-year old Dame Cicely Williams in Oxford in 1986 when we were photographed together[See The Lancet, Ref 4 for what she looked like at 93!]. I thanked her profusely for all that she did in my tribe in those colonial days wihout electron microscopes and sophisticated analysers. We both were greatly baffled why many of today's experts (especially those who have little experience of a tropical sojourn)find it difficult to accept that “Kwashiorkor is the result of a social pathology before it is the outcome of a biochemical pathology” . Clinical epidemiology ie answering the questions Who? Which? Where? When? Why? What? and How? is far and away the best tool to investigate a tropical phenomenon such as kwashiorkor, and Cicely Williams gets my Full Marks for employing that tool with hardly any funds for medical research.
I repeat what I said a decade or so ago in The Lancet: “Those of us who grew up in the kwashiorkor belt and who have also had the benefit of an excellent medical education cannot but caution our ministries of health and social welfare about the danger of missing the social pathology wood for the trees of free radicals and leukotrienes”.
Nothing to declare, except that Kwashiorkor is my tribal language.
1 Fuchs GJ. Antioxidants for children with kwashiorkor. Brit Med J 2005; 330: 1095-1096.
2 Ciliberto H, Ciliberto M, Briend A, Ashorn P, Bier D, Manary M. Antioxidant supplementation for the prevention of kwashiorkor in Malawian children: randomised, double blind, placebo controlled trial. Brit Med J 2005; 330: 1109-1111.
3 Konotey-Ahulu FID. Kwashiorkor. Brit Med J 1991; 302: 180-181.
4 Konotey-Ahulu FID. Issues in kwashiorkor. Lancet 1994; 343: 548.
5 Williams CD. A nutritional disease of childhood associated with a maize diet. Arch Dis Child 1933; 8: 423-8.
6 Williams CD. Kwashiorkor – a nutritional disease of children associated with a maize diet. Lancet 1935; 2: 1151-52.
7 Williams CD. Kwashiorkor. JAMA 1953; 1280-85.
8 Williams CD. Social Medicine in Developing Countries (Millroy Lecture, Royal College of Physicians). Lancet 1958; 863-66.
9 Marsden PD. Kwashiorkor. Brit Med J 1990; 301: 1036-37.
10 Konotey-Ahulu FID. What Is AIDS? T-A'D Co. Watford 1996.
Competing interests: None declared
Source Link as http://www.bmj.com/cgi/eletters/330/7500/1095#106854 14 May 2005
BMJ 2007;335:210-211 (28 July), doi:10.1136/bmj.39268.553021.47
Views & reviews
The perils of unmasking scientific truths
Felix I D Konotey-Ahulu, consultant physician
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Published : BMJ [www.bmj.com]