I have recently updated my list of publications on the website. You can view this on the links below
http://www.konotey-ahulu.com/publications_annual.htm
I have also updated my CV which can be seen here
WARNINGS FROM A QUARTER OF A CENTURY AGO
WARNINGS FROM A QUARTER OF A CENTURY AGO
Ethnic minorities and sickle cell disease
British Medical Journal 20 April 1985 Volume 290, page 1214
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.
First, 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 1977;16:15-8.
Current “hit and miss” care provision for sickle cell disease patients in the UK
Current “hit and miss” care provision for sickle cell disease patients in the UK
Following her article [1] on NCEPOD [2] Susan Mayor has, again, produced a succinct summary [3] of the recent publication of ‘Standards’ on the care of patients with sickle cell disease (scd) in the UK [4], written by a multidisciplinary working group chaired by Consultant Haematologist Dr Ade Olujohungbe. The public launch of the publication was on 9 July in the House of Commons where the Archbishop of York, Dr John Sentamu, said: “These Standards are another step in providing consistent care” [3].
HIT AND MISS CARE PROVISION
Hitherto, consistent care has not been UK’s strong point. The description by the ‘Standards’ Group’s chairman, Dr Olujohungbe who, in my opinion, has proved to be the most experienced Clinical Haematologist in the UK on the scd patient since Hammersmith Hospital’s Professor Lucio Luzzatto with whom Olujohungbe was closely associated, underlines what Graham Serjeant [5] and I have bemoaned for years. Olujohungbe said “The care provision for sickle cell disease is currently hit and miss, depending on the attitudes and experience of health professionals” [3]. This sad diagnosis by the leading UK haematologist on the scd patient was precisely why “NCEPOD found that many patients died of complications caused by excessive doses of opiods” [1, 2].
RESPONSE OF TEN DOWNING STREET
During the week when UK media (radio, television, & newspapers) were full of news that the Prime Minister was inviting personal phone calls and contacts on people’s concerns I took the opportunity to write to The Right Honorable Mr. Gordon Brown drawing his attention not only to the publication of the NCEPOD Report [2], but also to the international responses that were beginning to pour in [6]. The reply I got, dated 25 June 2008, from Ten Downing Street was encouraging: “Dear Dr Konotey-Ahulu – The Prime Minister has asked me to thank you for your recent letter and enclosures. Mr. Brown has asked that your letter be forwarded to the Department of Health so that they may reply to you on his behalf. (Signed) MR R SMITH”.
RESPONSE OF THE DEPARTMENT OF HEALTH
I looked forward eagerly to answers to the 3 questions I posed in my correspondence, namely (i) With respect to scd patients dying with overdose of opiods (heroin & morphine) in their blood “What kind of supervision led to this?” [6a] (ii) Why should West Indians and West Africans who did without morphine in their countries be put on morphine pumps when they were admitted to UK hospitals? [6a] (iii) To those UK haematologists who say “unbearable pain is unbearable pain, which must be treated with the most potent analgesic drugs known” I posed this third question, how many of them would prescribe diamorphine (heroin) monthly for their teenage daughters whose dysmenorrhoea was simply unbearable? [6a]. The Department of Health wrote back to me Ref; TO00000325139 dated 16 July 2008. Signed by “Shelley Wilson, Customer Service Centre”. All the questions were totally ignored. Would the National Institute of Clinical Excellence (NICE) now provide specific answers to these my 3 questions?
OUR GENETIC FUTURE
Africans, African-Caribbeans, and African Americans must wake up and realize that their genetic future depends on themselves, and not on any Department of Health. We must reduce the genetic disease burden, starting now, otherwise their children will continue to be subjected to “hit and miss” health care provision, ending up on heroin and morphine pumps. “Unless we Africans are involved in genetic counseling” and voluntary family size limitation (GCVFSL), I said not long ago “the genetic burden on the National Health Service will go up and up” [7]. What is more to the point, unless we take this very seriously our children and grand children will suffer greatly from scd [ACHEACHE syndrome], for “one in three West Africans in the UK has a beta-globin variant gene (ie NORMACHE) whose unsuspecting owner needs to be identified and helped with genetic counseling and family size limitation” [7]. Those who do not quite believe the enormity of the NCEPOD Report [2] should, please, start by taking a good look at how the rest of the world regards the “hit and miss” approach to the present care of sickle cell disease patients in the United Kingdom [6a-6j].
Felix I D Konotey-Ahulu MD(Lond) FRCP(Lond) DTMH(L’pool) – Kwegyir
Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Haemoglobinopathies, London W1G 9PF.
felix@konotey-ahulu.com
1 Mayor Susan. Enquiry shows poor care for patients with sickle cell disease. BMJ 2008; 336: 1152
2 NCEPOD (National Confidential Enquiry into Patient Outcome and Death) Sickle: A Sickle Crisis? (2008) [Sebastian Lucas (Clinical Co- ordinator), David Mason (Clinical Co-ordinator), M Mason (Chief Executive), D Weyman (Researcher), Tom Treasurer (Chairman)] info@ncepod.org
3 Mayor Susan. Group publishes standards for adult sickle cell disease to reduce number of unexplained deaths. BMJ 2008;337:a771
4 Sickle Cell Society (London) The Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK. http://www.sicklecellsociety.org
5 Serjeant GR. The case for dedicated sickle cell centres. BMJ 2007; 334: 477 (3 March)
6a http://www.bmj.com/cgi/eletters/336/7654/1152-a#196224 [Felix I D Konotey-Ahulu 29 May 2008] Poor care for sickle cell disease patients: This wake up call is overdue
6b http://www.bmj.com/cgi/eletters/336/7654/1152-a#196359 [Kwabena Frimpong-Boateng 30 May 2008] Re: Poor care for sickle cell disease patients: This wake up call is overdue
6c http://www.bmj.com/cgi/eletters/336/7654/1152-a#196514 [Marianne Janosi 3 June 2008] “Poor care for patients with sickle cell disease” BMJ 24 May 2008 Volume 336.
6d http://www.bmj.com/cgi/eletters/336/7654/1152-a#196520 [Cecilia Shoetan 3 June 2008] I lost my Sickle Cell disease adult daughter minutes after being given Diamorphine intravenously when she could not breathe.
6e http://www.bmj.com/cgi/eletters/336/7654/1152-a#196631 [Frank Edwin 5 June 2008] Re: Poor care for sickle cell disease patients: This wake up call is overdue
6f http://www.bmj.com/cgi/eletters/336/7654/1152-a#196848 [Ivy Ekem 9 June 2008] Care for sickle cell patients
6g http://www.bmj.com/cgi/eletters/336/7654/1152-a#197301 [Mawunu Chapman Nyaho 17 June 2008] Poor care for the sickle cell disease patient: “Pain won't kill him, but Morphine could”.
6h http://www.bmj.com/cgi/eletters/336/7654/1152-a#197350 [Emmanuel Jeurry Blankson 18 June 2008] Sickle Cell Disease is managed, NOT treated.
6i http://www.bmj.com/cgi/eletters/336/7654/1152-a#197377 [Yolande M Agble 18 June 2008] Re: Poor care for sickle cell disease patients: This wake up call is overdue.
6j http://www.bmj.com/cgi/eletters/336/7654/1152-a#198669 [Akosua M Dankwa 11 July 2008] Sickle Cell patients deserve to live.
7 Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1826 (December 1)
Competing interests: I come from a sickle cell disease (scd) family, my parents were traits (NORMACHE), and I am actively involved in genetic counselling to reduce the burden of sickle cell disease (ACHEACHE) in future generations.
NCEPOD REPORT Reveals Sickle Cell Disease Patients in UK Hospitals Dying from Overdose of Morphine and Heroin (Diamorphine)
Sickle: A Sickle Crisis? (2008) info@ncepod.org
The National Confidential Enquiry into Patient Outcome and Death (NCEPOD)
published a report in May 2008 which showed that within the 2 years January
1 2005 to December 31 2006 “Nine out of the 19 patients with sickle cell
disease who had pain on admission and who then died had been given
excessiove doses of opiods”.
The International response to this revelation was huge – see
http://www.bmj.com/cgi/eletters/336/7654/1152-a#196224 and the subsequent 9
other comments.
Later, on 9 July 2008 in the House of Commons, another report was published
called “The Standards for The Clinical Care of Adults with Sickle Cell
Disease in the UK”. The Chairman of the Group that produced the report
described the present care of patients in the UK as a “hit and miss affair
depending on the attitudes and experience of health professionals”. Dr
Konotey-Ahulu's reaction to this in the British Medical Journal on line is
reproduced in full below
http://www.bmj.com/gi/eletters/337/jul11_2/a771
Using Aerobic Oxygen
FURTHER ADVICE FROM ONE PATIENT TO ANOTHER PATIENT
From Mawunu Chapman Nyaho (Europe) to Judi Hamilton (USA)
If I remmeber rightly, the little bottle of Aerobic Oxygen will last up to two months if you take about 20 drops three times daily.
I am grateful for your question because I forgot a very important point about pain killers.
My brother “ended up” having a sickle cell crisis in late spring last year (2007). Too much traveling by air, long working hours and enjoying wine or some other alcohol as an aperitif or with his evening meal were among things that led to the crisis.
He was given morphine to alleviate the excruciating pain. As Professor Konotey-Ahulu put it when they spoke over the phone while my brother was in a hospital in Maine, “morphine will keep you in hospital”. (The School of Medicine in Ghana teaches doctors that morphine should be used only when a terminally ill patient is in great pain.). Fortunately, the medical staff in Maine was willing to learn. As soon as they were told that morphine was supressing the oxygen supply to his body (the crisis meant he already was suffering from lack of oygen), on Prof's advice, he was given shots of “Ketorolac”. Within 48 hours my brother had been discharged from hospital.
Some doctors in hospitals in Europe are very stubborn and patietns and their families have to be very, very firm in refusing the use of morphine to alleviate pain. Last summer, a father flew in from Canada and shared his daugher's hospital room for a week, until she was discharged, so that treatment that was further aggravating her condition would be stopped. She had been given morphine and a full blood transfusion instead of a partial blood transfsion, so her blood became even thicker and normal oxygen supply to her body and brain was furher obstructed.
Please do read the Professor's blog and website regularly and, please do contact me, if necessary, for patient-to-patient exchanges.
There is nothing like walking into a doctor's office with tags on the essential pages of his book on The Sickle Cell Patient.
Rule 001 = live healthily, for living a healthy life is living without sickle cell crises. Dr. Konotey-Ahulu asked about your job – that also falls into the “living healthily” category because if it is tiring and stressful you will need to do something about that.
A few more rules:
1. Never allow morphine or any other opiate to be administered as a pain killer when in crisis.
2. Inform yourself about partial blood transfusion.
3. Know what your hemoglobin level is and never accept a blood transfusion that will raise it far above what your body is used to living relatively comfortably with.
4. Avoid alcohol – make water your favorite drink.
I hope you have had your spleen checked. Please do so as soon as possible.
With best wishes,
Mawunu
Patient-Achievers helping other patients with Advice
Dear Ms. Hamilton,
My apologies for not writing sooner. I have not checked my e-mails in several days. Below are a few points which come to mind immediately. I shall be 60 in June.
As long as you feel unwell and have pain in your spleen or any other part of your body, I would avoid flying until your health problems are dealt with. There is nothing like preventing a sickle cell crisis as a crisis is a miserable and unbelievably painful experience.
Drink two to three liters of water each day, avoid alcohol, do not eat heavy meals (prefeably four or five lighter meals a day), and do not have your last meal too late at night, keep your body weight down, stay well rested, never exhaust yourself, do gentle exercise, walk. Always dress warmly; even in hot weather, carry a light shawl around with you to protect you from sudden exposrue to cool draughts and air conditioning.
Your eyes need to be checked regualrly (i.e. approximately every six months) to make sure that you do not have any bleeding into the retina. Unfortunately, this is a problem we 'SC' patients suffer from. If blood vessels in your eyes rupture during a trip, you will most porbably not be able to fly home for medical care so it is extremely important that you should be in good health before flying.
I have found it extremely helpful to own a copy of “The Sickle Cell Disease Patient” by Dr Konotey-Ahulu, and I encourage doctors to have a copy on their bookshelves. I also refer them to his website www.konotey-ahulu.com We need to know our bodies and about our ailment so we can educate doctors as only too few know about it.
I have learned from Dr. Konotey-Ahulu that there is a big difference between managing a patient and treating a disease.
You can help any open-minded, receptive general practitioner who does not know much about sickele cell disease to manage you correctly.
Please ask Dr. Konotey-Ahulu what blood tests you should have. My spleen has disappeared – if I remmeber rightly, it was overworked fighting infections. Every three years or so I have a “pneumovax” shot and, at the beginning of winter, take a course of medication to make my immune system react quickly whenever I have an upper respiratory tract infection.
A few drops of Aerobic Oxygen (stabilised oxygen) in a glass of water about three times a day is very helpful. It is not sold in the US but can be ordred from Canada. As I do not have the address within reach at the moment, I shall send it to you tomorrow.
All that I have learned about managing my problems with sickle cell disease has been thanks to Dr. Konotey-Ahulu. My using Aerobic Oxygen is also thanks to him.
China and Australia will be well within your reach if you are in good health.
Please spend a couple of years looking after your health then plan your trips with the necessary days of rest well below an altitude of 2000m. The correct and constant air pressure on board an aircraft is also important, particularly on long-haul flights.
I live in Geneva, Switzerland. A small group of us is reaching out to other sicke cell patients in Geneva and forming a suport group as there are misconceptions and errors in treatment that are repeated. We patinets need to know our bodies and be firm with doctors. In the long run, they respect us for our knowledge and many of them are grateful to us for helping them improve our health.
If you know of any other sicke cell patints in Fort Lauderdale, you may wish to form a support group.
With best wishes,
Mawunu
felix Konotey-Ahulu <admin@sicklecell.md> 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
G6PD Deficiency in Ghanaians: How to recognise it
In their instructive seminar Professors Capellini and Fiorelli put Africa first in the list of areas with the “highest frequencies of G6PD deficiency” (Jan 5, p 64) [1], so what are the Ghanaian associations?
(i) “Dorkita, I’m passing coca cola urine”. Mist alba, septrin, fansidar, chloramphenicol, APC, are the greatest offenders [2].
(ii) Typhoid disease [3 4]
(iii) lobar pneumonia with jaundice [2 4]
(iv) Renal failure [5](v) No enzyme-at-all in 5% G-6-P-D deficient males [6]
(vi) Greater delay in recovery from coma [7]
(vii) Greater representation in Cirrhosis of the liver [7]
(viii) Greater proportion of diabetics [7]
(ix) Sickle cell disease patients fare worse [8 9 10]
(x) Female homozygotes (X-X-) have more severe disease than hemizygotes (X-Y), making me wonder “how that can be reconciled with the Lyon hypothesis of inactivation of one X chromosome” [10, page 105]
(xi) Periodic intravascular haemolysis from interaction between “alpha thalassaemia type 1 equivalent to African homozygous alpha thalassaemia type 2, with G-6PD Total deficiency” [11]. Exercise acts as a trigger. The combination per se does not appear to account for the hyperbilirubinaemia in babies [All cases of ‘march haemoglobinuria’ must be screened for both alpha thalassaemia and G-6PD deficiency].
(xii) The enzyme is found in liver, brain, kidney, adrenals, skin, pancreas, nerve and muscle, hence the extra-erythrocytic manifestations of G6PD deficiency. Bedu-Addo’s description of chloroquine induced bilateral ptosis [12] could have been in one with no enzyme.
(xiii) Hepatomegaly with quick progression to cirrhosis [7]. (xiv) Viral Hepatitis is commoner in G6PD deficient patients, and characterised by intrahepatic cholestasis [13]. (xv) Terminology of A-minus (attributed to Africans) and B-minus (Mediterranean)is meaningless, as in A-minus Ghanaians “absence of enzyme in new red cells” produces cases “similar to the Mediterranean type of total deficiency” [6]
(xvi) There is an inexplicable north-south divide of incidence: 11% of males are deficient in the north [14] while 23% have G6PD deficiency in the south [15].
(xvii) Protection against malaria has not been proved in Ghana for hemizygotes and female homozygotes [7]. Indeed, blackwater fever is often related to G6PDdeficiency
(xviii) “Sabolaa kε Emanbii yi” is a truism in my Krobo tribe: “Onions and M&B disagree” [16]. In the Colonial days some who took sulphonamides (‘M&B’) for infections fell gravely ill on eating onions. Dipropyl disulphide in onions “alters G6PD in the metabolic chain within the erythrocytes, which causes denaturing and precipitation of haemoglobin” [17].
(xix) Genetic Counselling goes beyond haemoglobinopathy to erythrocytopathy. A G6PD deficient sickle cell trait mother (AS) has two healthy daughters with her sickle cell trait (AS) husband, and they seek advice for a third pregnancy hoping to get a boy. She is told that although neither daughter has inherited her deficiency, and both avoided sickle cell anaemia (SS), her next child could be SS with G6PD deficiency (severe if a boy) [18]
(xx) Voluntary family size limitation (VFSL) [19] is the advice I give hemizygotes who (in Africa) have a high male procreative superiority index (MPSI) [20] in that males have more children than females, with consequent greater donation of abnormal genes to the next generation.
I declare that I have no conflict of interest Felix I D Konotey-Ahulu
felix@konotey-ahulu.com
10 Harley Street, London W1G 9PF, UK
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 Ghana. Ghana Med J 1974; 13: 168-170.
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. Ghana Medical Journal 1975; 14: 172-174.
5 Owusu SK, Addy JH, Foli AK, Janosi M, Konotey-Ahulu FID, Larbi EB. Acute reversible renal failure 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: 25-26
7 Owusu SK. Clinical manifestations of glucose-6-phosphate dehydrogenase (G-6PD) deficiency in Ghana. Ghana Med J 1978; 17: 235-39.
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 Accra. Ghana Med J 1977; 16: 4-7
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. London: Macmillan 1992; Watford: Tetteh- A’Domeno Co 1996.
11 Konotey-Ahulu FID. Alpha thalassaemia nomenclature and abnormal haemoglobins. Lancet 1984; 1: 1024-1025
12 Bedu-Addo G. Chloroquine induced bilateral ptosis. Trans Roy Soc Trop Med Hyg 2006; 100: 696-697.
13 Morrow RH, Smetana HF, Sai FT, Edgecomb JH. Unusual features of viral hepatitis in Accra, Ghana. 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 ehydrogenase deficiency in northern Ghana. Ghana Med J 1968; 7: 120-124.
15 Owusu SK, Opare-Mante A. Electrophoretic characterization of glucose-6-phosphate dehydrogenase (G6PD) enzyme in Ghana. Ghana Medical Journal 1972; 11: 304.
16 Konotey-Ahulu FID. Probing anecdotes in traditional African therapeutics. African Journal of Health Sciences 1194; 1: 53-56.
17 Fenwick G, Hanley AB. The genus Allium – Part 3 Section X Medicinal Effects. CRC Critical Reviews 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 – Greece, May 13 -16, 1985. Eds 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 counselling in Africa. In: Eds, Oliver Mayo, Carolyn Leach. Fifty Years of Human Genetics. A Festschrift and liber amicorum to celebrate the life and work of George Robert Fraser. South Australia, 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
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
1977;16:15-8.
Source : British Medical Journal 1985 (20 April), Volume 290, page 1214.
Home haemodialysis just the thing for Africa’s wealthy patients
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 [1]. 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” [2] this was mainly due to Stanley Shaldon's extraordinary drive [3].
As Ken Farrington pointed out “self supervised haemodialysis performed in the patient's home was pioneered ..largely to cope with increasing numbers” [4]. 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 [2].
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 [5] 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 [6]. 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
References
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 Africa
Some thirty features of AIDS in Africa
KONOTEY-AHULU F.I.D.*
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 (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, 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
Comment
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
Acknowledgements
I thank the clinicians who took me on ward rounds during my recent
REFERENCES
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
References
(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