Photofinish
BMJ 2001; 323 doi: https://doi.org/10.1136/bmj.323.7327.1498 (Published 22 December 2001) Cite this as: BMJ 2001;323:1498All rapid responses
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I was most interested by the photograph of a married couple who had
both suffered from acromegaly. I would suggest that this is not so
unlikely as it first appears. On the assumption that most of the patients
with acromegaly are followed up in the specialist clinic and most are
married, there are probably 50 spouses of acromegalic patients coming to
the clinic. Each of these has the same chance as any other person of
getting acromegaly- given in the article as 4/million/year. Thus each year
the chance of one of the spouses of a patient also becoming acromegalic is
200/million, or 1 in 5000. Most consultants probably direct an outpatients
clinic for 20 years so the chance of seeing this in their career is about
1 in 250.
Competing interests: No competing interests
In my December 31 response I neglected to make plain the statistical
basis for Mrs Maxine Robinson's conviction for supposedly killing two of
her children.
The jury and appeal judges were told repeatedly that the chances of
two infants of dissimilar ages dying almost simultaneously were "highly
unlikely," therefore they must have been suffocated, although defence
witness Professor
Bernard Knight has said "nothing in the pathological findings in these
cases is indicative of suffocation." There was no more than passing
reference, in court, to the paddling pond, and prosecuting counsel
described the
earlier [identical] death of Victoria as being "a terrible tragedy for the
defendant. It was a death in entirely innocent circumstances, for which
she need feel no responsibility at all."
And yet the actual odds against all three children, without medical
intervention, may have approached 100%.
Competing interests: No competing interests
The odds against husband and wife both developing acromegaly are
certainly not one in about 39 million. But R Adam Brooks'assumption of
1/6250 is also faulty.
These were the odds for the wife, but she and her husband shared
environmental and mode-of-living factors, which could have unknown
relevance. Therefore odds for the husband could be well below 1/6250.
The same arguments apply to unexpected cot deaths. Mrs Sally Clark was
convicted of killing two of her children, although the first death had
been recorded as natural. The jury were told that the odds in an affluent
family were one in 8,500 therefore one in 73 million for two (and
presumably one in billions for three). Professor Emeritus of Medical
Genetics at the University of Calgary, has assessed the odds against Mrs
Clark having a second affected child as perhaps 25%, or even higher
in the case of a mitochondrial mutation. But even this is wrong, in
that it concentrates solely on genetics, and not on shared environment and
mode-of-living. Draughts, room temperature, type of bedding, type of
heating, food preparation, all could have had effect. The one child died
23 days after polio vaccination, the other four hours after a vaccination
with adsorbed diphtheria, tetanus and perrtussia plus three drops of
poliomyelitis vaccine on the tongue. Could there have been some
combination of factors that made these vaccinations dangerous?
Mrs Maxine Robinson is in her ninth year of two life sentences for
supposedly killing her children.
Maxine lost three, in identical circumstances. In 1989 Victoria, aged
nine months, was given a plastic paddling pond. In warm sunshine it was
taken from its wrappings, and filled with tap water. An oily scum rose to
the surface and was dispersed with washing-up liquid. Eight hours later
she was found to have died in bed. This was "natural death."
The tragedy broke up the marriage.
With a second husband, four years
later, an identical paddling pond was bought, filled with tap water in
warm sunshine; an oily scum on the surface was dispersed with washing-up
liquid, and Christine (19 months) and Anthony (five months) splashed
happily in it, blowing bubbles. Eight hours later both children were
found dead in their beds, and were presumed to have been killed by Maxine.
The washing-up liquid squirted into the water contained surfactants, which
emulsify oils and encourage absorption into the skin by up to 50 times
(over the whole bodies of children who may have inherited mitochondrial
allergies). The plastic ponds were Chinese-made and the plasticisers used
in them contained vegetable oils, not the usual mineral ones.
A Newcastle-upon-Tyne mother has written to me saying her child suffered a
similar attack after playing in a plastic paddling pond; a doctor lived
nearby so the child's life was saved.
There are many other instances where child deaths with high likelihood of
being natural or accidental are regarded as murder. Some are on the
Portia Campaign website, www.portia.org or discussed in my book, "The
Lynch-Mob Syndrome" (www.infinityjunction.com, £4.49).
The fact is that statistics for acromegaly, cot deaths and many other
medical problems CANNOT be quantified and guesses can be highly
misleading.
Competing interests: No competing interests
Dear Sir,
We agree with Dr. Rebecca Torrens (1) that the Ottawa Ankle Rules are
valuable prediction criteria which aid the clinician in making a decision
regarding the need for radiography after acute ankle injuries.
Fortunately, her paediatric case had a demonstrable bony injury on
radiography, which must have facilitated subsequent decision-making.
We would like to emphasise that in children and young adults,
radiographs of the ankle may frequently appear normal despite strongly
positive Ottawa Rules (2). Indeed, significant growth plate injuries may
be present, and only detectable by more detailed imaging, such as magnetic
resonance studies (3).
The dilemma for junior Accident and Emergency staff remains
therefore, what to do when faced with an unremarkable ankle radiograph,
but positive Ottawa Rules in a young patient.
We recommend that the clinician should retain a high index of suspicion
for growth plate injury, and seek experienced orthopaedic advice.
Competing Interests: None declared.
References
1). Torrens R. BMJ 2001. 323: 1498.
2). Singh-Ranger G, Marathias A. Comparison of current local practice
and the Ottawa Ankle Rules to determeine the need for radiography in acute
ankle injury. Accident and Emergency Nursing. 1999. 7: 201-206
3). Stuart J, Boyd R, Derbyshire S, Wilson B, Phillips B. Magnetic
resonance assessment of inversion ankle injuries in children. Injury 29
(1): 29-30.
Competing interests: No competing interests
As a humble orthopaedic surgeon, I am puzzled by the learned
professors statistics regarding the probability of both husband and wife
developing acromegaly.
If the lifetime frequency of developing the disease is 1/6250, then
the probability of both members of a couple pulled at random off the
street developing the disease is indeed 1 in approx 39 million. However,
in an endocrine clinic where we already know that the wife is acromegalic,
the probability of the husband developing the disease is the same as that
for any other member of the public - ie 1/6250. I would therefore expect
that 1/6250 patients with acromegaly will also have a spouse who at some
point develops the disease. The prevalence of couples who both show signs
of acromegaly will of course seem less frequent because of asynchronous
presentation of the disease.
Yours sincerely,
Adam Brooks
Competing interests: No competing interests
PHOTOFINISH
BMJ 2001; 323: 1498 [Full text]
Look-Alike Significant Others
for Profs. A B Atkinson, D R Hadden
Dear Both,
Enjoyed your article.
Naturally, article assumes that probability of spouses developing disease
is independent for each spouse. Anecdotally (at least) spice choose other
spice who are similar. Hence, is it also possible that this choice was a
factor in the similarity, which would--alas--bring down the statistics?
Presumably there are other similar diseases, so I look forwards to your
future letter in next Winter Solstice’ BMJ.
Regards
Len Finegold
PS
“The probability of a person developing the disease in their lifetime is 1
in 6 250. The probability of a husband and wife developing the disease (1
in 39 070 000)” . Pedantically, my ancient calculator (ca. 1974) gives a
slightly different number for 6 250 x 6 250 =39 063 000. (Your spacing of
the number is in the best physics tradition ... eg., Physics Today,
August 2001 p. BG16, right column, near top.)
Competing interests?: Maybe I’ll think of some.
Competing interests: No competing interests
The innocence of Mrs Sally Clark
Professor Meadow leads a small but influential group of
paediatricians who proclaim their belief in “thinking dirty” and assert
that 40% of unexplained sudden deaths of infants are caused by murder,
The report of the confidential enquiry into stillbirths and deaths in
infancy (CESDI) published on February 1, 2000, after a thorough
examination over three years, assessed maltreatment, including deliberate
harm, as well as neglect or extremely poor care, as probably in the region
of 6%, which confirms other assessments of murder as perhaps 3%.
Naturally enough, “dirty thinkers” are called in by police and
prosecutors, they are extremely persuasive (with judges and jurors wholly
reliant upon expert opinion), and the consequence is that 37 out of every
40 convictions may well be false.
“Neither the defence nor any of the expert witnesses advanced the
claim that the deaths of Mrs Sally Clark’s two children were examples of
sudden infant death syndrome,” says Professor Meadow.
Christopher’s death was originally certified as natural due to
respiratory infection and this was only overturned when Harry also died.
Sally’s father a retired chief inspector of police, has written that
over a period of 14 months the Prosecution culled support from Drs Smith
and Keeling and the Defence assembled eight eminent paediatricians and
pathologists, each expert in a particular specialisation.
“Three days before the trial, Professor Green was brought to London
to meet defence expert Professor Luthert, the leading retinal surgeon in
Europe, at Moorfields Hospital, following which Professor Green admitted
that his original diagnosis of baby-shaking was wrong. He made a further
statement that his original statement and evidence at the committal should
be totally disregarded. But he then put forward the possibility that
Harry had been smothered, which had cautious support from Smith and
Keeling though Dr Williams (in the prosecution’s team) not only maintained
his original diagnosis of baby-shaking but also disagreed that there was
any evidence of smothering.
“In the case of Christopher the Prosecution’s case was based on blood
traces in the lungs and a slight cut inside the lip as evidence of
smothering. Despite that, 14 months earlier Dr Williams had found nothing
suspicious and had certified ‘Natural causes.’ At the trial Professor
Green claimed there were blood traces in the lungs whilst at the committal
he had said there was NO blood in the lungs. He explained the discrepancy
by saying he had been busy at the time and had not had time to look
properly!!!
“The Defence attributed the blood staining as being from the nose-
bleed suffered a week before and the cut lip to resuscitation in the
ambulance, a not unusual mishap. Professor David, the independent
consultant, thought the symptoms consistent with a pulmonary ailment and
remains convinced that ‘natural causes’ cannot be ruled out. Bruising on
the leg was post-mortem and attributable to hand-held resuscitation.
“This was the medical evidence on which Sally was convicted of abuse
of Christopher.
“In the case of Harry, as explained, the trauma/retinal injuries at
first thought to be shaking were conceded by both sides (except Williams
himself) to have been caused during the post-mortem. There was an old
fracture of the rib suffered at 1-4 weeks of age, that had healed
naturally which, though unexplained, is not unknown in young babies and
had caused no discomfort and certainly had nothing to do with death. The
Prosecution relied on slight hypoxia as evidence of possible smothering
which was not only dismissed by Dr Williams himself but also by the
Defence medical team as present in all cot deaths to some degree and which
is part of the dying process.
“That was the medical evidence on which Sally was convicted of abuse
of Harry.
“It was agreed by ambulancemen, nurses and hospital doctors that
there was not a mark on either baby on arrival at the hospital. It was
agreed by all the Defence medical team that the abuse alleged by Williams
could not be occasioned without leaving marks. It was agreed by the
clinic staff that both babies were bonny and thriving when regularly
examined, including a few hours before death. It was agreed by midwives,
health visitors, neighbours, the daily nanny, and husband Steve that there
was at all times a strong bond of love between mother and child. It was
agreed by the medical witnesses on both sides (again except Williams) that
the cause of both deaths was ‘unascertained.’ The public cannot have
known this as the newspapers reported only the Prosecution evidence.
Neither could they know that Williams’ pathology was described variously
as ‘a blood bath’; as ‘cavalier, proved wrong in every area’; ‘never
seen so many inconsistencies in a pathology report’; ‘no weight can be
attached to any of the findings in a lot of contradictions.’
This was summarised by counsel as: ‘That catalogue of errors of
findings and interpretations fills me with horror that this is the
foundation of this woman being charged with murder.’
“The only common factor, which may or may not be relevant, is that
both deaths followed shortly after vaccination.” [Harry died only four
hours later.] Mindful of a possible genetic problem Sally and Steve asked
for specialist pathological examination - as did the hospital
paediatrician. Unfortunately this was ignored and the post-mortem was
performed by the local Home Office pathologist, Dr Williams.
“The consensus is that, backed by five eminent specialist professors,
the Defence won the medical arguments and the jury’s verdict astonished
everyone present. The speculation is that the jury did not understand the
medical evidence and took soundbites, reaching a majority decision on the
disbelief that ‘lightning could strike twice’ - plus the damning
statistics from Professor Meadow, a paediatrician not an epidermologist,
which are universally refuted even by the authors of the report from which
the purports were made.
“Contrary to reports Sally and Steve have never claimed two cot
deaths - indeed Christopher was certified as ‘respiratory infection’. The
figures from the Care of Next Infant charity (CONI) are one cot death in
every 8,500, but after one cot death the risk of a second actually
increases to one in 200. The formula 1:73 million (There is five times
more chance of winning the lottery) is dangerous nonsense. At least, it
was dangerous to Sally and will be for every grieving mother hereafter
when, following a double death, it is transposed as the chance of a mother
telling the truth being 1:73 million.”
Please visit the www.portia.org website and read my book, “The Lynch-
Mob Syndrome” (www.infinityjunction.com (£4.49).
Competing interests: No competing interests