Letters

Management of sickle cell disease

BMJ 1998; 316 doi: https://doi.org/10.1136/bmj.316.7135.935 (Published 21 March 1998) Cite this as: BMJ 1998;316:935

Non-addictive analgesics can be as effective as morphine and pethidine

  1. Yolande M Agble, Community nurse
  1. Greenford, Middlesex UB6 0SS
  2. Sickle-Watch (UK), West Indian Cultural Centre, London N8 0DT
  3. Northwick Park Hospital, Harrow, Middlesex HA1 3UJ
  4. Department of Obstetrics and Gynaecology and Reproductive Health Care, University of Manchester, Palatine Centre, Manchester M20 3LJ
  5. Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica
  6. Imperial College School of Medicine, Central Middlesex Hospital, London NW10 7NS

    EDITOR—I am concerned at the increasing use of pethidine and morphine analgesia in young African and Afro-Caribbean children with sickle cell anaemia, as described by Fertleman et al and by Davies and Oni. 1 2 I am astonished that a vulnerable section of the community is being given addictive drugs when non-addictive analgesics would be just as effective, particularly in a society where drug addiction among young people is recognised as a problem.

    I have nursed patients with sickle cell disease in Trinidad and in Ghana, where the numbers of sufferers are much larger than those found in the United Kingdom. Education of parents and children by public health nurses in schools and in the community about their condition, regular follow up by clinicians, and admission when in crisis, with paracetamol being given alone or in combination with codeine when something stronger is required, went a long way to ensuring a normal existence for such children. I remember only one occasion when pethidine was used, and that was given in a small dose to a patient in labour. More recently pethidine has been used in small doses in the Caribbean, but never morphine—for obvious reasons. I am baffled why children here should need it.

    The claim that parents and children are demanding opiates for pain control shows a lack of education and knowledge about the illness and the long term effect of such drugs on their part. I have come across several young addicts in hospitals across London, and I wonder how long it will be before they end up in a criminal situation.

    References

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    Management would improve if doctors listened more to patients

    1. Neville Clare, Director
    1. Greenford, Middlesex UB6 0SS
    2. Sickle-Watch (UK), West Indian Cultural Centre, London N8 0DT
    3. Northwick Park Hospital, Harrow, Middlesex HA1 3UJ
    4. Department of Obstetrics and Gynaecology and Reproductive Health Care, University of Manchester, Palatine Centre, Manchester M20 3LJ
    5. Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica
    6. Imperial College School of Medicine, Central Middlesex Hospital, London NW10 7NS

      EDITOR—As a 51 year old patient with heterozygous sickle cell anaemia and a national campaigner for improved services in hospitals since 1974, I note that pethidine has been discounted for morphine infusions in the review article by Davies and Oni.1 In my experience, however, the reason pethidine has a bad name is that it is wrongly prescribed—that is, at too high a dose (200 mg) and too frequently (every two hours)—as happened in the case of the patient with homozygous sickle cell anaemia who died in a London teaching hospital after two days of “continuous intravenous infusion of pethidine at a dosage of 100-150 mg an hour.”2 From morphine we have progressed to diamorphine—that is, heroin—which is now being increasingly used as the first line of treatment.

      I know that these heavy narcotics are lovely to have when you are in acute pain, but as I grow older I am more and more convinced that their dangers greatly outweigh their benefits. I travel around the country teaching and lecturing to patients and their relatives on how to maintain good health and keep themselves out of hospital. What I miss most when I am unfortunate enough to end up at an accident and emergency department with a sickle cell crisis are doctors who will listen to me, for I know more about my condition than any of them. To have a protocol thrust down my throat by the attending doctors without them listening to what I know about how I should or should not be managed is sad indeed. Every adult patient knows something that the doctors do not know, and they should be listened to more often if the management of sickle cell crisis is to be improved.

      References

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      Hearing loss may occur after sickle cell crises, especially in children

      1. Peter Savundra, Locum consultant audiological physician
      1. Greenford, Middlesex UB6 0SS
      2. Sickle-Watch (UK), West Indian Cultural Centre, London N8 0DT
      3. Northwick Park Hospital, Harrow, Middlesex HA1 3UJ
      4. Department of Obstetrics and Gynaecology and Reproductive Health Care, University of Manchester, Palatine Centre, Manchester M20 3LJ
      5. Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica
      6. Imperial College School of Medicine, Central Middlesex Hospital, London NW10 7NS

        EDITOR—Davies and Oni's clinical review of the management of patients with sickle cell disease omits to mention audiovestibular dysfunction—that is, hearing loss and vestibular disease.1 Large series report a prevalence of hearing loss of 11-41% in sickle cell disease.2 The cochlear microvasculature in young children may be particularly susceptible to occlusion,3 and children should have audiometry after sickle cell crises to exclude a readily manageable cause of educational handicap and preventable disability.

        References

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        Contraception with medroxyprogesterone may be beneficial

        1. Rosemary E J Kirkman, Senior lecturer in family planning and reproductive health,
        2. Max Elstein, Emeritus professor of obstetrics and gynaecology and reproductive health
        1. Greenford, Middlesex UB6 0SS
        2. Sickle-Watch (UK), West Indian Cultural Centre, London N8 0DT
        3. Northwick Park Hospital, Harrow, Middlesex HA1 3UJ
        4. Department of Obstetrics and Gynaecology and Reproductive Health Care, University of Manchester, Palatine Centre, Manchester M20 3LJ
        5. Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica
        6. Imperial College School of Medicine, Central Middlesex Hospital, London NW10 7NS

          EDITOR—The comprehensive and helpful review by Davies and Oni on the management of patients with sickle cell disease did not include an important aspect of care relating to contraceptive requirements.1 Some patients with homozygous sickle cell disease have fewer crises while using three monthly injections of medroxyprogesterone acetate 150 mg (Depo-Provera).2 Work published in 1971 suggests that medroxyprogesterone acetate may exert its beneficial action through stabilising the red cell membrane in sickle cell disease.3 Therefore this method of contraception is of particular value for women with sickle cell disease.

          However, women who are considering medroxyprogesterone acetate as a method of contraception must be informed that the vaginal bleeding pattern after the injections will be completely unpredictable and that they must be willing to accept this. During 1996-7 this method of contraception was used by over 1000 women attending our centre, mainly those aged 20-24 years. We believe that its advantages of high efficacy, ease of compliance, and lack of serious adverse cardiovascular effects despite extensive use indicate that it probably should be more widely used for contraception as a positive choice. It has additional benefits for women who have sickle cell disease.

          References

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          Acute chest syndrome is common in children before puberty

          1. Jennifer M Knight, Paediatric pulmonologist
          1. Greenford, Middlesex UB6 0SS
          2. Sickle-Watch (UK), West Indian Cultural Centre, London N8 0DT
          3. Northwick Park Hospital, Harrow, Middlesex HA1 3UJ
          4. Department of Obstetrics and Gynaecology and Reproductive Health Care, University of Manchester, Palatine Centre, Manchester M20 3LJ
          5. Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica
          6. Imperial College School of Medicine, Central Middlesex Hospital, London NW10 7NS

            EDITOR—In their comprehensive review of sickle cell disease Davies and Oni report that the acute chest syndrome is uncommon before puberty,1 but it is in fact one of the commonest causes of admission in children with sickle cell disease. In the cooperative study of sickle cell disease in the United States the incidence of the acute chest syndrome was greatest before the age of 10 years and declined thereafter.2 Over half of children with homozygous sickle cell anaemia had at least one episode of the syndrome in the first 10 years of life in the United States3 and Jamaica (unpublished data). The syndrome is an important cause of immediate death at all ages after 2 years, 4 5 and those who have an episode before 2 years of age are less likely to survive to the age of 40 years.2

            The acute chest syndrome is defined by the appearance of a new infiltrate on chest radiography. Features commonly include fever; respiratory symptoms such as cough, dyspnoea, and chest pain; and respiratory signs of crepitations and tachypnoea. The underlying mechanisms include infection, pulmonary fat embolism, and pulmonary sequestration, and clinical distinction is usually impossible. Infections may be more important in young children, and acute chest syndrome of infectious aetiology may be more severe than pneumonia caused by the same organisms in children without sickle cell disease.4 Caregivers must be aware of the frequency and potentially serious nature of this syndrome in children.

            References

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            Authors' reply

            1. Sally C Davies, Consultant haematologist,
            2. Lola Oni, Nurse director, Brent Sickle Cell and Thalassaemia Centre
            1. Greenford, Middlesex UB6 0SS
            2. Sickle-Watch (UK), West Indian Cultural Centre, London N8 0DT
            3. Northwick Park Hospital, Harrow, Middlesex HA1 3UJ
            4. Department of Obstetrics and Gynaecology and Reproductive Health Care, University of Manchester, Palatine Centre, Manchester M20 3LJ
            5. Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica
            6. Imperial College School of Medicine, Central Middlesex Hospital, London NW10 7NS

              EDITOR—These letters are a microcosm of the debate between professionals and patients about the management of painful sickle crises, reflecting in part the paucity of evidence.

              Pain is subjective, and a person's response to it is defined by cultural, demographic, and socioeconomic factors, as well as their anticipation of a response. Patients may underplay their pain as a coping strategy if good analgesia is unavailable, for whatever reason. So whereas physicians in the United Kingdom, the United States, and France generally recognise the necessity for opiates to be available for patients presenting to hospital, physicians in the Caribbean may not because Caribbean patients suffer less pain. Alternatively, the early death rate related to sickle cell disease is far higher in underdeveloped countries1 than in developed countries,2 so those patients who die early might have become the patients whom we in developed countries recognise as severely affected by pain. We have seen no patients develop opiate addiction after appropriate inpatient treatment, but we advise against opiate use in the community because of these risks.

              Debate ranges over the use of pethidine, yet Pryle et al have shown that many patients have toxic concentrations of norpethadinic acid while receiving optimal analgesia (which may result from the abnormal absorption of pethidine in sickle cell disease).3 This is supported by frequent anecdotes (from at least six of the major London sickle units) of patients having fits while taking pethidine, although the risks may be lowest in childhood as the youngest patient of whom we are aware was aged 12 years. Our local policies have been informed by advice from our patients, pain and palliative care specialists, and clinical pharmacologists, but they do not obviate the central role of the doctor to listen to and communicate with the patient.

              Our review was limited for reasons of space, but we agree with Savundra that audiovestibular dysfunction occurs as a consequence of sickling. In our experience, however, almost all cases are reversible, so we arrange audiometry only when an apparently severe or chronic problem is reported by the family or school.

              We agree with Kirkman and Elstein, but as the frequency of the V Leiden gene is so low in the population affected by sickle cell disease4 we also include consideration of low dose combined contraceptives when counselling patients.

              As for the acute chest syndrome, unlike some practitioners we distinguish and exclude infective pneumonias, which are common in childhood, from other sickle related pulmonary complications.

              We recognise the benefits of a comprehensive care programme but any community services must be linked, with easy access, to specialist hospital services.

              References

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              View Abstract

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