Month: October 2007

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” [1]. 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” [2], 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 [5]; 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” [6] 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” [7].

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” [7]. 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” [7]. 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” [2].

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” [8] 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” [9] How is this possible if hooked up on morphine or diamorphine pump “as in the recommended UK protocol”? [6]

(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”. [10] 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 [11]. So why do British haematologists prefer to use morphine and diamorphine? Answer: “Ketorolac has no product licence in the UK for this indication” [12]

(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?” [7]

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 [1], 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 [10].

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 [6].

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

Link : http://www.bmj.com/cgi/eletters/335/7622/685#177986

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” [3]. It is a reflection of the 'birth position' of the sufferer, before it is a pathology [4], 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” [3] 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 [9]: I was fed with beans, eggs, milk, minced meat, and Ovaltine” [3]. 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” [3].

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” [3]. 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 [10] 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” [4]. 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.

References

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