Craniosynostosis: Robert's Head

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.

Robert 22weeks on the day before surgery: disfiguring enough for cosmetic surgery?

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

http://www.nhs.uk/conditions/Craniosynostosis/Pages/Introduction.aspx

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.

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

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.

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.

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.

9.3 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 .

9.6 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.

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

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,

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

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

14.0 List of doctors' names
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.

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

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 14^th 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

Craniosynostosis: Robert's Head

Sunday 1 March 2015

Unusual head-shape in babies