The case for dedicated sickle cell centres
BMJ 2007; 334 doi: https://doi.org/10.1136/bmj.39133.665810.94 (Published 01 March 2007) Cite this as: BMJ 2007;334:477
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Graham Serjeant (1) bemoans the need for young people with sickle
cell disease to transfer from the care of a known and trusted
paediatrician to an unknown doctor at critical time of adolescence. He
also asks what is the most relevant clinical training for a disease whose
spectrum of complications include hypersplenism, stroke, enuresis,
gallstones, chronic leg ulcers, pulmonary hypertension, pain management
and psychological and social problems and that should also prepare
healthcare staff for education and counselling?
The answer that came to my mind was General Practice. We General
Practitioners (GPs) often care for our patients throughout life and come
to know them and their families and their illness related behaviour. GPs
are increasingly involved in the care of those with multiple system
problems and we can learn much through the long term care of individuals.
This, of course, is not to deny that patients with sickle cell disease
need specialist care but, in the UK at least, every such patient also has
a GP who can and should contribute to their medical care when appropriate.
It is stated that 60-80% of hospital admissions related to sickle
cell disease are due to bone pain crises but that many of these crises can
be managed in the home with simple, supportive measures such as warmth,
fluids, rest and adequate pain relief. I see an opportunity here for
patients, their specialists and GPs to plan together to reduce hospital
admissions and ensure more patient-centred care of these acute and
distressing episodes.
1.Graham Serjeant
The case for dedicated sickle cell centres
BMJ 2007; 334: 477
Competing interests:
None declared
Competing interests: No competing interests
I am privileged to have worked in the past in a big UK haematology
centre providing care for patients suffering from sickle cell disease
(SCD). I have read with interest an article by Graham Serjeant regarding
dedicated SCD centres (1). While I understand the sentiment expressed and
in principle can only agree with author’s suggestion, one needs to be wary
of centralising services for SCD patients. Day centres are excellent, but
during weekends or nights patients may find access difficult. Also, SCD
patients may come under care of non-haematology specialists in district
general hospitals. The key to care for the patients under those
circumstances is active involvement from haematologists, who are likely to
have greatest understanding of this disorder. Just like intensive care has
adopted “without walls” approach with readily available outreach services
it is only reasonable that SCD patients receive expert haematology input
on daily bases whether they are on general surgical ward, in A&E
department or on intensive care. Support for non-haematology staff
including good communication and 24 hour availability of advice would
foster their greater understanding both of problems and their management,
increase experience and thus confidence and competence in looking after
this challenging group of patients.
1. Serjeant G The case for dedicated sickle cell centres BMJ
2007;334:477
Competing interests:
None declared
Competing interests: No competing interests
Dedicated sickle cell teams
Dedicated sickle cell teams
I give Graham Serjeant ‘Full Marks’ for his Personal View [1], but
Piotr Szawarski appears reluctant to do so [2] because he feels what is
needed is active involvement from “haematologists who are likely to have
greatest understanding of this disorder” [2]. There are 2 types of
haematologists: those who look at the patient before looking at the
patient’s blood, and those who do the reverse. The latter are a disaster.
Serjeant had looked after an “SS” patient from childhood. When she moved
to the USA as a qualified Pharmacist and a mother, haematologists found
her to be very anaemic and gave her 6 units of blood within 24 hours to
augment Hb level. She died. [3]
Serjeant says haematologists are looking after sickle-cell disease
patients “not because the haematology of sickle-cell disease is
challenging, but because few other disciplines seem interested” [1]. Korle
-Bu Teaching Hospital’s Sickle-Cell Disease Centre in Ghana, with more
than 22,000 patients on the register by 13 March 2007 is the largest in
the world, followed by Graham Serjeant’s in Jamaica. Dedicated nurses,
public health workers, General Practitioners, clinic assistants, parents,
and haematologists join hands to make these patients achieve in life. As
with wrong use of blood, so is haematologists’ wrong approach to pain
control.
While some British haematologists accept “Serjeant’s and my combined
experience of 80 years covering thousands of patients in sickle-cell
crisis” [4] in not using “morphia or its derivatives”, others would defend
use of diamorphine for a sickle-cell disease woman with pulmonary embolism
from Depo-Provera complication. Indeed, in England “on March 6, 1997, I
was shown a woman who had been on continuous opiate infusion since
September, 1996” [5]. I pointed out that “the patient could not have been
in sickle-cell crisis for 6 months” [5]. The Consultants agreed. There are
British haematologists who readily acknowledge that they do not know it
all. I knew about the disease before I went to medical school. Three of my
siblings had sickle-cell disease http://www.konotey-
ahulu.com/images.generation.jpg so I knew all about priapism, gnathopathy
(a term I coined), and malaria precipitating sickle crisis, before I read
Medicine. My mother, with 3 sickle-cell disease children, knew more about
the patient than many haematologists do.
Some Consultant Haematologists in the NHS have cooperated with me not
only to take patients off regular transfusions thus eliminating need for
desferrioxamine injections, but also weaning them off opiates. We need
haematologists who will NOT dismiss what Serjeant is saying.
These 2 questions I keep asking have yet to be answered:
(i) “Why should a West Indian or West African sickle-cell disease
patient who had never had opiates in their countries be put on a Morphine
or Diamorphine pump on their first admission to hospital in the UK?” [4]
(ii) “Would a British haematologist put their 18-year old daughter on
Diamorphine injections monthly because her dysmenorrhoea was intolerable?”
[5]
Perhaps the National Institute of Clinical Excellence (NICE) will
help the haematologists give a sensible answer to these two questions. If
Piotr Szawarski visits Ghana and Jamaica he will understand how without
regular transfusions, diamorphine pumps, and hydroxyurea, we are able to
take patients from kindergarten to university. And we have fewer
haematologists than the UK does.
Felix ID Konotey-Ahulu Kwegyir Aggrey Distinguished Professor of
Human Genetics, University of Cape Coast, Ghana and Consultant Physician
and Genetic Counsellor in the Haemoglobinopathies, Ten Harley Street,
London W1N 1AA.
felix@konotey-ahulu.com
Competing interests None declared.
1. Serjeant GR. The case for dedicated sickle cell centres. BMJ 2007;
334: 477 (3 March.)
2. Szawarski P. Haematologists should treat sickle cell disease. BMJ
2007; 334: 549-550 (17 March)
3. Serjeant GR. Blood transfusion in sickle cell disease: a
cautionary tale. Lancet 2003; 361: 1659-60.
4. Konotey-Ahulu FID. Opiates for sickle-cell crisis? Lancet 1998;
351: 1438.
5. Konotey-Ahulu FID. Opiates for sickle-cell crisis. Lancet 1998;
352: 651-52.
Competing interests:
None declared
Competing interests: No competing interests