Standards for adult sickle cell disease aim to reduce gaps in careBMJ 2008; 337 doi: https://doi.org/10.1136/bmj.a771 (Published 11 July 2008) Cite this as: BMJ 2008;337:a771
All rapid responses
Current “hit and miss” care provision for sickle cell disease
patients in the UK
Following her article  on NCEPOD  Susan Mayor has, again,
produced a succinct summary  of the recent publication of ‘Standards’
on the care of patients with sickle cell disease (scd) in the UK ,
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
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  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” .
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 , but also to the international
responses that were beginning to pour in . 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” . 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” . Those who do not quite believe
the enormity of the NCEPOD Report  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
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.
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)]
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.
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
[Kwabena Frimpong-Boateng 30 May 2008] Re: Poor care for sickle cell
disease patients: This wake up call is overdue
[Marianne Janosi 3 June 2008] "Poor care for patients with sickle cell
disease" BMJ 24 May 2008 Volume 336.
[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".
[Emmanuel Jeurry Blankson 18 June 2008] Sickle Cell Disease is managed,
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)
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.
Competing interests: No competing interests