Craniosynostosis : (also
craneosinostosis) written in OpenOffice 2012Jun09
Robert's head: by
PRMcFF edited and posted 2015Mar01 latest edit 2015May10 - add pics
What is Craniosynostosis:
A baby with an abnormally long narrow skull, with a large bulge at
the back, or pronounced forehead, may be diagnosed as having
craniosynostosis. The skull needs to expand in the early months to
accommodate the growth of the brain. To do this the skull has to add
bone growth at an expansion-joint (suture) along the crown of the
head and others across the front and the back. These sutures must
remain open to allow the skull to expand. In babies with
craniosynostosis one or all of the sutures have closed, so that the
skull cannot expand normally. The brain then pushes out against the
skull, typically bulging out at the weakest points at the back and at
the forehead. The lack of space and pressure on the brain can
restrict normal brain development.
Purpose of Blog
This blog tells the
story of Robert who aged 5-months had the operation to reopen closed
sutures in his skull. It tells of the confusion faced by his parents,
their early wish to simply accept Robert as he was, the stages of
coming to accept that there may be a problem, seeking to define the
problem and to understand it, and then finally trying to make a
decision in the best interests of Robert.
This all happened
within the insurance-based health system in Colombia, South America
which caused additional cultural and language problems. These are
commented on separately. However the aim of this blog is universal:
to serve as a case-study to explore the anxiety of parents in coming
to a decision in the best interests of their baby, in circumstances
where the baby has an unusually shaped head.
Acknowledgement
See also the following links which we found useful .We write this post to add to the work of the authors of these sites. We are : baby Robert, mother Clara Ines (Colombian), father PeterMcFF (GB). The blog was written in English by PeterMcFF, with the intention to rework it and translate it into Colombian Spanish. We are grateful for the medical care Robert received in Colombia, and thank all those concerned. Adverse comments on the working of the Colombian health system, should not be taken as criticism of the individuals working within the system, or detract from our gratitude to the system and the Colombian tax-payer for taking care of Robert.
http://craneosinostosisenpr.blogspot.com/
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002557/figure/A001590.B1127/report=objectonly
Contents:
Introductory pics showing
Robert's head-shape.
What is Craniosynostosis
Purpose of the Blog
Pics of Robert's head
immediately before surgery
Acknowledgements
The
Story:
1. Birth and head-shape.
2. Head shape accepted
3. Continuing Issues
4. Stress generated by the
Health System
5. Second Opinion, relieved
6. Contradicted
7. Parents divided
8. Decision revisited
9. Understanding the choice
10. The operation in Wk22/Wk23
Pics immediately after the
Op
11. Time table in hospital
Day1-Day8
12. Time table of clinic visits
and events Wk1- Wk23
13. List of measurements taken
Wk1-Wk12
14. List of 22no.Doctors seen.
Discussion:
1. Near-miss
2 General Issues raised.
3. Why wasn't the condition
diagnosed
4. Why did the father not
accept there was a problem
5. Why did the father change
his mind
6. Defining the problem
7. Giving it a name
8 Diagnosis points to
solutions
9 Why wasn't it obvious
10. Why was the Wk7 scan report
diagnosis ignored
11, The role of parents in
bringing-in the diagnosis
12. Health-system generated
confusion .
The
Story:
1.0 Birth and head-shape:
1.1 Robert was born 5-weeks prematurely
with something of an egg-shaped head, with a pronounced bulge at the
back, called an occipital bun. A Neanderthal like head-shape, you
might call it. Neanderthals were spiritual and gentle they say. We
liked Robert's head, it was part of him and his gentle elf-like
nature.
Robert at Wk3
2.0 Head-shape accepted:
2.1 The shape of Robert's head was
commented on by most of the confusing array of paediatricians and
other doctors who attended Robert. We had seen 17 doctors in Robert's first 14 weeks and 22 by Wk22. Some said Robert's head-shape was
unusual, but that it was inherited from his father, most gave reassurance, none gave clear evidence-based advice.
2.2 Our concern was to be sure Robert's head-shape was not a symptom of something more serious - water on the brain for example - that was pushing out the skull, and which would continue. So we were pleased when at Wk5 we were offered an MRI brain-scan, which was done in Wk6. We were relieved when in Wk7 we collected the results which showed everything was normal. We were then able to relax, and were happy to accept Robert's distinctive head-shape as an important part of him.
2.2 Our concern was to be sure Robert's head-shape was not a symptom of something more serious - water on the brain for example - that was pushing out the skull, and which would continue. So we were pleased when at Wk5 we were offered an MRI brain-scan, which was done in Wk6. We were relieved when in Wk7 we collected the results which showed everything was normal. We were then able to relax, and were happy to accept Robert's distinctive head-shape as an important part of him.
3.0 Continuing issue:
3.1 Our relaxation was supported by the
opinion of the Canguro paediatrician Doc(7) who saw him weekly to Wk6
before he was signed-off at the weight of 2.5kg. She measured the
circumference of his head each time and raised no alarm.
3.2 But in Wk12 on a visit to another clinic for vaccination after the MRI scan had allayed any residual fears, a passing doctor, unconnected with the vaccination or with Robert, stopped and did raise the alarm. He put the fear of God into Robert's mother, saying that if Robert did not have an immediate operation his head shape would get worse, he would be the laughing-stock of the school play-ground and he risked being mentally impaired. Robert's mother was confused, angry and emotional, by this uninvited intervention. What right did he have to give his opinion, and to upset her
3.2 But in Wk12 on a visit to another clinic for vaccination after the MRI scan had allayed any residual fears, a passing doctor, unconnected with the vaccination or with Robert, stopped and did raise the alarm. He put the fear of God into Robert's mother, saying that if Robert did not have an immediate operation his head shape would get worse, he would be the laughing-stock of the school play-ground and he risked being mentally impaired. Robert's mother was confused, angry and emotional, by this uninvited intervention. What right did he have to give his opinion, and to upset her
4.0 Stress generated by Health System:
4.1 Coping with the insurance-based Health
System in Colombia can be stressful. It appears
to be the nature of the system that you are subject to the opinions
of many different doctors. Each of them specifies test, but these
tests are often received by another unrelated doctor, who often
dismiss the work of the previous doctor. There appears to be little sense of
bringing things together.
4.2 At each stage of the process, which
appears to be unending, we had to visit several clinics in different
parts of the city, at every one to join several queues. After an
order from the doctor, we often had to travel to another clinic to
queue, first to get authorisation from the insurance company, queue
to pay, queue to get an appointment, before attending the appointment
on another day, often at a different clinic. In the early stages,
because Robert was 5-weeks premature, we had to do this carrying
Robert connected to a heavy oxygen cylinder on wheels. This normally
meant being unable to use public transport, being forced to drive in
heavy traffic, with car restrictions on alternate days, and with the
stress of finding and paying for parking at one of the many different
Clinics we were sent to. At home too we had the additional stress
that Robert was tethered to the oxygen cylinder, which made it
difficult to move him around and to cuddle him. So nothing was
entirely natural. System requirements and equipment intervened at the
most basic level.
4.3 Robert had started life in an
incubator, his little hands bound and tied to
the sides to stop him pulling out all the tubes. He was born 5-weeks
prematurely because of the on-set of pre-eclampsia
in his mother and was in an incubator for his first 12-days. He was on a ventilator and found
difficulty breathing, his heart-rate and blood pressure shooting-up
as he struggled against the feeding tube. This was changed every two
days, forced down into his stomach. We had been prevented from
holding him.
4.4 So the unending intervention of the system was hard to take. As Robert's father, I recognise that all this made me hostile to the demands of the system, and may have clouded my judgement. My instinct was to fight, to protect my boy, to defend him from the indignities of the system. There was a sense that there was no-one in the system looking out for him.
4.4 So the unending intervention of the system was hard to take. As Robert's father, I recognise that all this made me hostile to the demands of the system, and may have clouded my judgement. My instinct was to fight, to protect my boy, to defend him from the indignities of the system. There was a sense that there was no-one in the system looking out for him.
5.0 Second opinion, relax:
5.1 Back to the
events: Robert's mother was confused, angry and emotional, with the
unsolicited opinion of a passing doctor in Wk12, who though unconnected with Robert, chose to give his opinion. He said Robert
needed an immediate operation, his head shape would get worse, he
would be the laughing-stock of the school play-ground and he risked
being mentally impaired.
5.2 So to settle
things, a few days later, in the same week, we paid privately for a second opinion from
another paediatrician. His opinion was strongly that there was
nothing to worry about. The fear that there was something wrong was maternal anxiety, nothing more. He showed that he
himself had a bit of an occipital bun, and he was not brain-damaged
or disfigured. In Robert's case it was clearly a characteristic inherited from the father, who had a similar shaped head.
6.0 Contradicted:
6.1 Meantime the visits to
various clinics for scheduled checks and vaccinations continued. The next week, Wk13
Robert was seen by yet another paediatrician (Doc14) this time for a routine vaccination. This doctor again raised the alarm on
Robert's head and insisted he must be referred to a 'specialist', and wrote out an order. He
said he had many years experience, had seen many thousands of babies,
and we must believe him that there was an urgent problem here.
6.2 I was angry with this unending saga,
but actually we should be grateful to that doctor, Dr C
Gilberto Cordoba, for standing up,
and insisting that the system must identify Robert as at risk, and that something must be done. He made out an immediate order for us to see a specialist. Again we had to take his order to another Clinic, queue for authorisation, queue to pay, and to
queue to get an appointment, which was for 4-weeks later in Wk17.
6.3 As Robert's father I was very reluctantly to go the appointment, and had to be dragged along. What now, what new opinions? The
so-called specialist arrived over an hour and a half late for the
consultation, took one look at Robert and said 'I'll cut here and
here, his narrow head will spring open, you'll see'. He cupped his
hands together and sprang them apart to demonstrate. 'You'll be
surprised what a difference it will make to his looks', he said. He
was a surgeon. We were taken by surprise. We hadn't known we were
seeing a surgeon – who would of course favour surgery. He said the
operation was cosmetic. The boys in the playground would make fun of
Robert, because of his head.
6.4 I was angry and hostile to the idea of
major surgery for something that was cosmetic. I liked the shape of Robert's head. It was the mark of the family. Robert's
Mother was undecided.
7.0 Family divided:
7.1 Over the following two-weeks, Wk18 &
Wk19 we were divided: I wanted to accept Robert's head as it was,
the gentle Neanderthal, and his mother was now in favour of surgery
to give the brain more space - in case it needed it.
7.2 But the surgeon said the operation was
entirely cosmetic. A future risk of brain damage from the skull being
too small - that was not cosmetic. Were we making a logical decision
here? In the end the logic seemed to hinge on the answer to the
question: what were the likely consequences and risks of doing
nothing.
7.3 So in an attempt to inform the decision, we decided to go back to the surgeon and ask the two questions: what are the consequences of doing nothing, and is the operation entirely cosmetic. We also decided that this time we would go ready to accept the surgeon's advice.
7.3 So in an attempt to inform the decision, we decided to go back to the surgeon and ask the two questions: what are the consequences of doing nothing, and is the operation entirely cosmetic. We also decided that this time we would go ready to accept the surgeon's advice.
8.0 Decision revisited .
8.1 We travelled
across town to one or other of the clinics almost everyday now, so we
were beginning to know how to work the system to get an appointment
in a few days. We were running out of time and had to speed things
up.
8.2 We managed to
squeeze in an early appointment with the surgeon a few days later, and were able to ask our
questions. But he seemed to give conflicting answers, made more
difficult by language. The answer was that the surgery was entirely
cosmetic, but there was a possible risk of restricted brain
development if the operation was not done. Most significant, a new
idea , that the head-shape was changing all the time, and would
become increasingly abnormal as it grew, and that the operation
became more complex if delayed. The surgeon had shaken his head in
sorrow at the number of parents who came to him too late with infants
who were now too old for the operation
8.3 As agreed between
us before-hand , this time we accepted the surgeon's advice and
agreed to the operation to follow in 15days, by which time Robert
would be 5-months.
8.4 We were still not
convinced we were doing the right thing, but we were running out of
time, and had to act. We were now at the end of Wk20. In the
surgeon's opinion it is necessary to carry out the operation before
the baby is 6-months (Wk26), while the skull-bones are still soft.
The operation is easier, can be done more quickly and this limits the
loss of blood during the operation, which is a risk factor. Small
babies have little blood in their small system and cannot afford to
lose too much. For these reasons this particular surgeon is unwilling
to operate after 6-months.
8.5 Robert's mother
was alarmed by the surgeon now proposing far more extensive surgery.
Before he had described the operation as making two little cuts at the
sides, when the skull would spring open. Now, three-weeks from the
first consultation in Wk17, he said Robert's head had changed and he
would now needed to make an array of 6 cuts. Robert's mother pleaded
for fewer cuts, to keep to the original two and no more. The
surgeon drew himself up and replied that doña should know he was the expert
and would make as many cuts as he saw fit. We were being told we had to
trust to the surgeon's expertise and experience, which of course is
right. Between the two of us as parents the position had now
reversed: I reluctantly accepted the professional advice, whereas
Robert's mother was in greater doubt, fearful of the increased scale
of intervention, the dangers it held for Robert, and was particularly
upset at the pain Robert would suffer, following on from all the
indignities he had already suffered .
9.0 Understanding the choice:
9.1 The picture
presented now was one of a race against time, to stop further
distortion of Robert's head. His skull was accommodating brain growth
by pushing out at the back and was it perhaps beginning to push-out
just a little at the forehead, which would spoil his good looks. His mother
certainly thought so. If true, then we were harming Robert in
delaying the operation.
9.2 We were now beginning to understand the mechanism. For the first time I started looking on the internet. I looked up 'skull distortion', since I had no other name to give it. I came to the medical name: craniosynostosis. I now had a name to go by. I looked it up on the NHS website and with better information was convinced. This was a great help, and a turning point at the end of Wk20. Robert's mother was helped by the other website listed, which was the experience of another mother.
9.2 We were now beginning to understand the mechanism. For the first time I started looking on the internet. I looked up 'skull distortion', since I had no other name to give it. I came to the medical name: craniosynostosis. I now had a name to go by. I looked it up on the NHS website and with better information was convinced. This was a great help, and a turning point at the end of Wk20. Robert's mother was helped by the other website listed, which was the experience of another mother.
9.3 Robert's mother had earlier
asked for copies of medical records and test results. We looked
through these again carefully – and discovered that at birth he had
been described as dolicocefalia and the MRI
result had used the word scaphocephaly, which is the
term used in Spanish for craniosynostosis. The opinion had been
there all the time, but had been overlooked.
9.4 We were now
convinced that if Robert's sutures were closed – the expansion
joints in the skull which allow it to expand to accommodate the
expanding brain – if these were closed it was obvious and urgent
that they must be re-opened by surgery -whatever it took, as many
cuts as needed.
9.5 Why had no-one explained the mechanism in these simple terms. Our decision had been made difficult because we had been given unsupported opinions, rather than explanations and evidence .
9.5 Why had no-one explained the mechanism in these simple terms. Our decision had been made difficult because we had been given unsupported opinions, rather than explanations and evidence .
9.6 But, were the
sutures closed? Was the diagnosis correct? We owed it to Robert to be
doubly sure, before putting him through the ordeal of the operation.
. So 3-days later we again paid privately for a hurried
second-opinion this time with a surgeon, a touchy-feely ladies man
who put Mum's at their ease. He confirmed the diagnosis, gave explanations and showed
that you could feel the ridges where the two parts of the skull had
grown together. In his opinion all of Robert's expansion joints,
sutures, had been bridged, had grown together.
9.7 We now accepted Robert must have the operation and as soon as possible, though we still felt very emotional at having to hand him over. Robert had the operation 9-days later.
9.7 We now accepted Robert must have the operation and as soon as possible, though we still felt very emotional at having to hand him over. Robert had the operation 9-days later.
10.0 The operation:
10.1 The
operation cuts and pulls back
the scalp to expose the bones of the skull. A 2mm wide channel is cut
through the skull along the line of the sutures: one along the top of
the skull from front to back, others across the front and across the
back – forming the shape of an H as you look down on the skull.
These ensure that the
different plates of the skull are quite separate, and will not rejoin
prematurely. The surgeon may
make other cuts, and move bone to reshape the skull.
The scalp is then pulled back into place and stitched together where
it has been cut. After the operation it is this stitching of the
scalp that you see, rather than the channels cut through the bone of
the skull, which are concealed under the scalp.
10.2 The operation
itself took something over an hour. It was 3hrs from handing him
over, smiling innocently from the arms of the nurse who took him
away, to then seeing him in intensive care.
10.3 After surgery the
head and face swell-up like a soft football, and a little later the
eyes swell and turn black. The baby is connected up to various tubes,
is fed by tube, is sedated and will have his hands padded and tied to
the bed to stop him pulling the tubes out. It's pretty grim. It was
difficult to recognise this was our baby Robert. This was made worse
by being prevented from touching him or communicating, speaking or
singing to him, so he might recognise us. Any of these we were told
raises his blood pressure, heart rate and breathing rate, and these
must be kept as low as possible. Later tea-bags were placed over his
eyes to help reduce the swelling.
Robert
in intensive care Day2 & Day3, face and head
swollen, black-eyes, sedated, tea-bags to reduce swelling
Day5 in the ward, head still swollen
Day5 in the ward, head still swollen
11.0 Time-table in
Hospital :
Day1 Operation
mid-day, passes into intensive care
Day1&2 Pretty
grim, upsetting to see, heavily sedated, fed by tube. No
communication or contact
Day3&4 Swelling
beginning to reduce, still sedated, no communication or contact
permitted.
Day5 Swelling much
reduced, clearly improving moves from intensive care to a ward.
Day5&6 In ward,
first contact allowed, becomes Robert again, still unable to feed
normally.
Day7 Can feed,
course of antibiotics against infection completed, all remaining
tubes and
cannula removed, leaves hospital,
cannula removed, leaves hospital,
Day8:
First Day at home, head still very swollen
Some days later when the bandages were removed, showing the stitches in the scalp, swelling reducing
11.1 By Day 8, (pic) the
first full day at home, he still had a dressing on his head wounds,
but he was already behaving and acting normally, and we had our
Robert back, and hopefully free of future risks. We were relieved
that we had followed through, had set-aside conflicting medical
opinion, had challenged our own instincts, had taken evidence and had
eventually made a rational decision and had gone through with the
operation.
11.2 This was the end of
what had seemed a long road, starting when Robert was born with a
long narrow skull, and ending here at Week23, Robert looking relaxed
and smiling again.
12.0 Timetable
of Clinic visits and events
Wk1 Robert delivered on 04Jan2012 by caesarian on Wk35
of pregnancy following pre-eclampsia
Admitted to San Jose Hospital, Bogota, Colombia
intensive care for 12-days. 04-16Jan
Hospital notes: Craneo Normal, dolicocefalia,
fontanela, normotensa, suturas simetricas
Seen by paediatricians Docs (1-6)
Wk2 Delivery of oxygen cylinders to home in preparation
for Robert's release 16Jan
Robert leaves intensive care with cannula and oxygen
cylinder set at 1/8 16Jan
Weight 1.9kg, feeding 2hrly with syringe, unable to
suck, later 3hrly 16-26Jan
Wk3 Weekly Canguro clinic checks: Dr (7): oxygen reduced
to 1/32 18Jan
Robert birth registration, and health insurance
registration Famisanar
Wk4 Weekly Canguro clinic checks: Dr(7) : oxygen
continued at 1/32 24Jan
Famisanar visit office for administration on insurance
cover 24Jan
Wk5 Weekly Canguro clinic checks: Dr (7) 31Jan
MRI brain-scan order issued 31Jan
Visit Colsubsidio for aut horisation of MRI
Scan 04Feb
Famisanar visit office for administration on insurance
cover 06Feb
Wk6 Weekly Canguro clinic checks: Dr (7) 08Feb
Prepare Robert for Scan, to keep him awake all night to
following mid-morning 09Feb
MRI scan: Country SA, Cr16-82 many attempts, very
difficult to scan, stressful 10Feb
Wk7 Weekly Canguro clinic checks: Dr (7) 15Feb
Country SA : to collect resonance results cerebral
simple: inside normal limits,
additional written comment Doc(8): head-shape
consistent with scaphocephaly 15Feb
Wk8 Weekly Canguro clinic Clle 67: Dr(9) 22Feb
Wk10 Admitted to Infantil Hospital for 5-days for
urinary infection 09-13Mar
Order given for Cistografia miccional,Doctors (10,
11) 13Mar
Queue for authorisation for cistografia
miccional 13Mar
Wk12 Canguro clinic check Clle67 23Mar
Visit for vaccination: Cuidadela Colsubsidio,
doc(12) 23Mar
Visit to book for Cistografia, but system down and
unsuccessful 23Mar
Paediatric consultation: Doc(13): Head-shape presents
no problem 23Mar
Wk13 Visit Vaccination: Doc(14): Head-shape problem
order for specialist 28Mar
Renal Scan: Doc(15) Centro Medico Calle63 : 02Apl
Wk14 Renal Scan consultation on results, Doc(16)
correction issued, to 'normal ' 09Apl
Vaccination: Centro Oncologio Clle97. Doc(17) 10Apl
WK17 Consultation: Doc(18): Head-shape needs cosmetic
surgery 30Apl
Wk18 Cistografia miccional: Fundacion Cardio infantil:
Doc(19), normal 02May
Vaccination visit, Centro Oncologio Clle97 not done,
new appointment 04May
Canguro clinic: Doc(9) final check Clle 67 08May
Wk19 Vaccination Centro Oncologio Clle97, Doc(17)second
visit 09May
Wk20 Infantil Hospital to pay for consultation, prior
to appointment 18May
Consultation: Doc(18): Confirm surgery needed, 30May
provisional date
Orders for anaesthetist consultation, blood test, units
of blood for surgery.
Research on internet finally understood: Scaphocephaly
= cranio-synostosis, 21May
Wk21 Change flight to UK to stay for
operation 23May
Visit for vaccination Colsubsidio Clle 67, not 23May
San Jose Hospital to pay for consultation prior to
appointment 24May
Second opinion: Doc(20) cranio-synostosis confirmed
Second visit for Vaccination to different clinic:
Cuidadela Colsubsidio,doc(21) 25May
Infantil Hospital 06.00-09.00am for Robert blood test
preparation for surgery 29May
Order 2-units of blood in preparation for
surgery 29May
Wk22 Consultation with anaesthetist doc(22) for
clearance for operation 30May
Collect blood results, given as A+, contradicting
hospital record as O+ 30May
Visit to Espanola Clinic for private retest of blood,
A+ confirmed 01Jun
Prepare Robert for operation, nothing to eat or drink
after midnight 01Jun
Previous operation cancelled called in early, operation
time advanced 02Jun
Operation, then intensive care for 4.5-days, no contact
with Robert permitted 02-06Jun
Wk23 Recovery continues in ward for 2.5days, stay in
hospital to care for Robert 06-08Jun
Robert goes home on Day7 08Jun
Wk24 First surgery check-up, queue to pay, 2hr queuing
to book next appointment 15Jun
13.0 Measurements
14.0 List of doctors' names
Wk1 Birth weight, incubator peso 2420gr talla
46cm pc 24.5cm 04Jan
Wk2 Leaves Hospital peso 1968gr 16Jan
Wk3 Canguro clinic: peso 2000gr talla
46 cm pc 34.5cm 18Jan
Wk4 Canguro clinic: peso 2200gr talla
46.5cm pc 35 cm 24Jan
Wk5 Canguro clinic: peso 2350gr talla 47
cm pc 35.5cm 31Jan
Wk6 Canguro clinic: peso 2650gr talla
48 cm pc 36.5cm 08Feb
Wk7 Canguro clinic: peso 3050gr talla
49.5cm pc 37.5cm 15Feb
Wk8 Canguro clinic: peso 3350gr talla
49.5cm pc 38 cm 22Feb
Wk12 Canguro clinic: peso 4580gr talla
55 cm pc 42 cm 23Mar
Wk18 Canguro clinic: peso 5750gr talla
58.5cm pc 44 cm 08May
Wk27 peso 7000gr
Omitted to preserve anonymity
Robert Today at 3yrs
Discussion:
1.0 Near Miss
1.1 Robert came near to not having the operation. The opinion and evidence in favour of the operation was not clear. If it was important for him to have the operation, then this lack of clarity came near to causing him harm.
1.1 Robert came near to not having the operation. The opinion and evidence in favour of the operation was not clear. If it was important for him to have the operation, then this lack of clarity came near to causing him harm.
2.00 General Issues Raised
2.1 Can others in a similar position learn from our experience. Can they be helped to come to a timely decision by asking the right questions. Is there something in the operation of the health system* that works against clarity, and which can be changed. Can paediatricians be better trained to spot the symptoms
2.1 Can others in a similar position learn from our experience. Can they be helped to come to a timely decision by asking the right questions. Is there something in the operation of the health system* that works against clarity, and which can be changed. Can paediatricians be better trained to spot the symptoms
3.00
Why wasn't the condition diagnosed?
3.1 Robert
was in intensive care for 12 days after birth. In this time between
different shifts he
was
seen by at least 5 paediatricians, and then weekly by the
paediatrician in the canguro** clinic in his first 5-weeks. The
question we asked them all was: 'Is there any reason to be concerned
about his head-shape'. None of them said yes. The usual answer was
that his head-shape was unusual, but nothing to worry about. It was
just within acceptable limits, and the circumference was increasing
with age. If there were no increase in circumference, than that would
be a worry, but the shape itself did not seem to matter.
3.2 The
canguro paediatrician did agree to send him for an MRI scan at
5-weeks, since we were concerned. However the scan only looked at
brain development, which was found to be 'within normal limits',
thankfully, but it did not scan the skull, for closed sutures,
something at the time we did not know about. It was the wrong sort
of test for that – BUT in their comments the scan team gave the
opinion that his head shape was consistent with ' Una configuracion
escafocefalica' .cranio-synastosis:
3.3 This
was read by the commissioning paediatrician and all subsequent
paediatricians and doctors, yet none of them took any action, or
explained to us the term and what it meant. It was seven weeks later
and after many more visits to many different clinics and doctors for
other things, that by chance one doctor giving a vaccination in Wk13
insisted there was a problem with the shape of Robert's head – and
he had not seen the report. He said he had many years experience, had
seen many thousands of babies, and we must believe him that there was
an urgent problem here. So it was a chance encounter with the a
doctor (Doc14) who had experience and the courage to act, that
finally raised the alarm. He wrote an instruction and said we must
take Robert to see a 'specialist' immediately. What sort of
specialist he didn't say.
4.0 Why
did the father not accept there was a problem?
4.1 After
13 weeks of asking 'Is there any reason to be concerned about his
head-shape' and receiving reassurance from 13 doctors that there was
no problem, it was a shock for the 14th doctor to say it
was indeed an urgent and serious problem.. We had been reassured by
the inaction of all the others. Still no name was given to 'the
problem', and we were given no explanation of it, or told what sort
of 'specialist' we were going to see.
4.2 The
general 'problem' was posed in terms of Robert's head-shape being
unusual enough to be labelled abnormal, and that in the future it
would cause him social embarrassment. I was hostile to this sort of
argument. To me Robert's head-shape was noble, distinctive. All of us
in the family had occipital buns. It was a characteristic of the
family. True Robert's bun was more pronounced, but to me it was not
unsightly, it was lovable. There needed to be more than this to
justify Robert having an operation.
4.3 Of
course this view was mine, and I was alive to the idea that Robert
may not hold the same view when older, and by then it would be too
late to make any correction.
5.0
Why did the father change his
mind?
5.1 As
time went on I gradually realised that the 'problem' as it was
labelled at the time, was not fixed. I had been accepting the shape
of Robert's head as it was at Wk5, assuming it was fixed. It was not
a problem. It had not occur to me at the time that the back of the
head and the forehead may continue to push-out. Infact it was not
until later-on when there was a suggestion that his forehead would
become progressively more pronounced that I realised there were
limits even to my acceptance of his head-shape. So I too shared some
of society's attitudes. I wanted his otherwise good looks to be
preserved and not to be spoiled, and for there to be no further
change.
6.0 Defining
the problem
6.1 Labelling
something as a problem is imprecise. It is not until 'a problem' is
defined, analysed named and understood that a solution can be
devised. When we were asking 'Is there any reason to be concerned
about his head-shape' , we were asking is there anything going on
that we should be worried about. We weren't asking for opinions on
his looks and the social acceptability of his head shape. Infact
there was something going on, and it was not until it was given a
name, defined, and finally understood by us, that the solution was
obvious.
7.0 Giving
it a name.
7.1 If
I had understood from the outset that the process was dynamic, then I
would have taken a different view. Indeed if at any stage the
mechanism of cranio-synostosis had been explained – that the skull
needs to expand to accommodate the rapidly growing brain, that it
does this by adding bone at expansion joints, but that these joints
called sutures can close and so not allow expansion which inevitably
leads to distortion of the skull – then I would immediately have
understood and would have wanted Robert to have the operation at the
earliest possible date. So why hadn't I understood that?
8.0 Diagnosis
provides solutions.
8.1 It
was not until Wk20, though still not having a name for the problem,
that I looked up 'skull distortion' and found craniosynostosis. For
the first time I understood the mechanism that was at play. Of course
a rigid structure must have expansion-joints to allow for movement
and change. It was then that I became determined that if the
diagnosis was correct, Robert must have the operation as soon as
possible. That was when we went for a hurried second opinion to
confirm the diagnosis, and then pressed for an early date for the
operation.
9.0 Why
wasn't it obvious?
9.1 Both
surgeons who saw him, took one brief look and without any close
examination declared he had craniosynostosis. Apparently also he had
raised ridges where the sutures were fused. If it was that obvious to
them, why did the many paediatricians who saw him in Wk1-Wk17 not
recognise it, especially when they had the MRI report issued in Wk7
suggesting the diagnosis.
9.2 Perhaps
paediatricians need to be trained to recognise the condition.
10.0 Why
was the Report in Wk7 giving the diagnosis ignored.?
10.1 It
appears to be the nature of the insurance-based Colombian health
system that tests are carried out, but there is no adequate mechanism
for analysing and weighing the significance of the results. The
results do not necessarily go back to the commissioning doctor. The
imperative of the system is financial control, not medical need. The
insurance company exercises financial control by requiring
commissioning of test and procedures to be passed through them for
authorisation. There is a sense in which doctors will accede to
patient demands for test, and transfer the responsibility to the
insurance overseers. A doctor's decision to ask for a test is
therefore in some respects speculative – why not, let's see where
we get to on this one – and therefore perhaps it is not specified
with sufficient care. After all he may not be the one who will
receive the results, and is unlikely to be responsible for carrying
things forward with the patient. The casualness with which test are
commissioned seems to mean the results are ignored. Once ignored by
the first doctor, they are ignored by later doctors who assume
earlier doctors will have already responded to anything significant.
11.0 The
role of the parents in bringing a diagnosis.
11.1 As
parents we have learnt that to protect the interests of our son, we
need to be his advocate – to chase and make things happen –
but then just as importantly to stand back and sift the evidence. So
why did we not do that more effectively?
11.2 I
came near to shying away from the solution- an operation - when it
was offered, because I had not understood the nature of the problem,
so had not recognised the solution when it was presented to us. There
was still no clear diagnosis, and therefore no clear need for action.
11.3 My
resistance to the offer of an operation, was because it was not a
solution to a clearly defined problem. It appeared to be a case of
being referred to a surgeon, who would naturally propose an operation.
My attitude came partly from hostility to the system. I had got
caught-up in a fight against the system, believing perhaps I was in a
heroic struggle to protect Robert. I felt very emotional about it,
how the system was serving its own ends, going through the motions,
commissioning test, more doctors, more consultations, but with no-one
cutting through all this and saying Robert needs this for this
reason, and here is the evidence. - except for Doc14.
11.4 At
the time I was only dimly conscious of my attitude. I was acting
instinctively and emotionally, but at the same time believing I was
trying to strip away emotion,and get to the facts to make a logical
decision. I was aware that Robert's mother was feeling very
emotional, and felt I had to set aside my emotion and be the one who
acted logically. This naturally caused tension between us. I was
accused of being a 'very stressful person', always challenging and
wanting answers, insensitive , treating this as some decision-making
exercise. My hostility to the system, also meant that I was sceptical
that the individual doctors were concerned for Robert's welfare, so I
wanted less involvement with the system.
.
11.5 Robert's
mother on the other-hand wanted more involvement with the system. She
was happy to exploit the formless nature of the system to pressed for
more tests, more doctors, more consultations, believing this must be
in Robert's interest. Her view was we were lucky we had a system that
allowed so much medical intervention, at little cost, and we should
use it to the full. She was willing to put up with any amount of
queuing for authorisation, queuing to pay, queuing to make
appointments, travelling across town each time, more consultations,
more tests, more reports, more system-generated stress. A scatter-pattern hoping something sticks. And infact
she was right. Excluding the two surgeons, we saw 18no.doctors
between wk1 and Wk20, and it just happened that one of them, Doc14
raised the alarm in an effective way and diagnosed the problem – though he didn't give
us the diagnosis, or give us any explanation.
Re-edited 2015Mar03 by PMcFF. Problems still with alignment.More pics added 2015May10
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ReplyDeletethanks a lot for the time and effort writing down this experience. My baby is 4 weeks and I think he has a long head, will see a neuro surgeon next week, now with more information because of this blog. I really hope that Robert is doing fine today.
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