Happy Birthday

DSC_0210The girls both reached milestones yesterday: EB bought her first lipstick (a milestone in any girl’s life. The colour is a good deal more flattering than anything in my teenage makeup bag – it was the Eighties). And R turned 10.

R was excited about her birthday but the planned party has been postponed for the time being because she is still so weak. So we celebrated quietly. We had bunting and paper chains (I love bunting and paper chains).  EB made and distributed cinema tickets for a movie premier in our living room. R slept through most of it.

I’ve been neglecting the blog lately.  I’ve been waiting for things to improve (or even just stabilise).  They haven’t but I don’t think that I can let R’s birthday pass without posting an update .

It started with tonsillitis. The tonsillitis triggered seizures.  These seizures required repeated doses of emergency medication.  The medication put her repeatedly into respiratory distress: this is life threatening. The risks were made only too clear to us by the anxiety shown by the paramedics, nurses and medical staff who treated her.

The doctors tried a different tack.  A feeding tube was inserted via her nose into her stomach. The ketogenic diet was now to be given solely as a milk feed via this tube.  This would allow greater control.  The seizures stopped and it was decided that R should be scheduled urgently for surgery for a permanent tube which would deliver the feed (and medicines) directly to her stomach (a PEG).

Then the diarrhoea started.  Terrible diarrhoea. I have never seen so much poo.  Oceans of the stuff.  The doctors suspected the milk feed.  The feed was modified: dairy-free, less fat, less MCT oil. The diarrhoea continued.  Finally, on the Friday evening, after more than a week of diarrhoea and with her surgery scheduled for Monday morning, she was diagnosed with Colostrom Difficile (C Diff ).

I’d like to be able to say that the C Diff was treated successfully and it was happy ever after.  But that’s not how it has turned out.  Thankfully, R’s surgery went ahead regardless (and went well).  Getting R well again has proven more difficult.  At one point that week, with R lying unrousable, and weak from vomitting and diarrhoea, I genuinely thought that we might lose her.  She pulled through – thank God – but after two courses of antibiotics she’s still not free from the C Diff.  She is worn out.

So the birthday party has had to be postponed but we are hoping that she might be well enough to celebrate properly soon.

Happy Birthday, my lovely R. Here’s to a better year.




We are lucky in the UK. Healthcare is free at the point of use and by and large access to it is very good.  Some provision, however, is uneven, with a clear weighting away from paediatric and towards adult (and old age) services.  If I were cynical, I might suggest that this is because paediatric patients, especially those with complex needs, do not vote and may never be in a position to exercise their vote.

Take Speech and Language therapy for example.  R has an obvious need – she is non verbal.  Her need for Speech and Language input is explicitly stated in her Coordinated Support Plan, a document which outlines the services that R should be provided with in order for her to access education.

Speech and Language therapy (or SALT) for children falls within the shadowlands of the NHS, part funded by the NHS and part funded by the council education department.  The service is delivered at school. (When R was very little, she couldn’t access it at all as she wasn’t granted a specialist nursery placement. Without the nursery placement, the waiting list was more than 18 months for a triage appointment.)  I understand the benefits of providing this service at school – reduced travel time for staff and/or parents, fewer missed appointments and the ability to support the teaching staff to apply appropriate strategies in the classroom.  All of this I understand.  What I don’t understand is the reluctance to fund this service at a level that is in any way useful.

At well over 100 pupils, R’s school is large for a special school.  Virtually all of the pupils at the school require SALT at some level.  A rough calculation would suggest that across the school day, allowing for snack time and lunch, a SALT could comfortably see 9 or 10 pupils – lets call it 10.  This allows for a bit of report writing and interaction with the class teachers.  So, if 100 pupils receive input one day a week that would require 100 sessions over 5 days.  So, R’s school needs at least two full time members of staff.  Ideally, the children would be seen more than once a week.  So, not less than four full time members of staff then.  The sums aren’t hard.  Why then, when the allocation is made, does the school only have the equivalent of a single SALT three days a week?  The consequences of this miserable allocation are that no one gets a satisfactory service.  The caseload is quite simply too much.  R has yet to receive a single set of targets after nearly four years at the school.  Apparently targets have been set.  They have never been communicated to us or to her teachers, presumably because there is no time available to do this.  Reports are never written. And despite a formal complaint – which was upheld – the service has not improved. It’s clear that it won’t improve until it is adequately resourced.

By contrast, adults with acquired speech and language impairments are well served. The service which they receive is differently organised and differently funded but the nagging suspicion remains that the primary difference is that children are lower priority.  I am genuinely baffled by this.  Politicians should surely be able to recognise that it is potentially going to cost them far more to support a young person for the length of their life than an older person.  The more that the young person can be supported towards independence, then, the greater the saving to the public purse in the longer term. But perhaps, politicians are only thinking as far as the next election, and R and her school mates won’t be voting any time soon.

On an encounter in a hospital waiting room

The young woman was sitting in a wheelchair in the neurology waiting room today when we returned there after R had been weighed (she’s losing weight now; one more thing to worry about). The young woman started to grind her teeth and her mother moved away from the seating area to the corridor where R was plotting her escape. Her mother apologised for the teeth grinding.  I genuinely hadn’t noticed.  She started to chat.  Her daughter was 40 (she looked 20 years younger) and was “severely mentally handicapped”.  It was their first visit to the hospital for many years. Apparently nothing very much had changed.  The mother told me that her little grandson had just been diagnosed with epilepsy and because her daughter had epilepsy too, she was now to have genetic tests to establish whether they shared a diagnosis.  The daughter had never been given a diagnosis and now finally it looked as though she might be.  She watched R wander about, always trying to make another bid for freedom.  Her daughter had been the same when she was small, she said. Kept her on her toes.

The daughter was sitting quietly in her wheelchair – save for the teeth grinding – wringing her hands.  I asked her if she was feeling nervous, if she ground her teeth when she was anxious.  I told her that R did that and that she was always nervous when she visited the neurologist.  She turned and looked straight at me with clear eyes.

Of course, I’m not a neurologist, and maybe this woman doesn’t have Rett Syndrome but our encounter today gave me pause. We’ve never been in any doubt that R is cognitively far better than she presents, despite being assured by specialists from health and education that she was profoundly cognitively impaired. There now seems to be an increasing consensus that professionals should, at the very least, retain an open mind about the abilities of women and girls with Rett.  But what if we had listened? What if we had been persuaded that R understood very little?

We were lucky enough to be able to visit Great Ormond Street Hospital for a consultation with the excellent Dr C a few years ago.  This was a specialist Rett clinic and we waited with another couple whose little daughter had just been diagnosed.  The waiting area was a narrow corridor.  Two women arrived wheeling in a young women with Rett Syndrome. The two new women found themselves a seat, turned the wheelchair to face the wall, out of the way, and proceeded to completely ignore the woman that they had brought.  Its easy to be judgemental about this but just think: if you have been told that it makes no difference to someone whether or not you interact with them, that that person understands nothing of what is going on in the world around them, perhaps you might park the wheelchair facing the wall too. The thought brings me out in goosebumps.

On hospital admin and IT

R is back in hospital. I am writing this, as R sleeps, to a non-stop soundtrack of “The Big Bang Theory”, courtesy of the teenage boy in the opposite bed (please let it stop soon).

It was a planned admission this time, which is something of a novelty. But it’s been far from straightforward getting this far. Clinical care, as always, has been good. When your child has a complex health history, it seems that you are spared, by and large, being treated by junior doctors: for as long as I can remember R’s care has been delivered by consultants, regardless of the specialty (the unfortunate exception to this is A and E where there is a tradition of using junior doctors as canon fodder).

This high quality of care contrasts sharply with the administration procedures in place to support it. In particular, it seems that the (new and hugely expensive) computer system at R’s hospital is seriously flawed. R’s current admission required coordination between a number of specialties. This proved almost impossible to achieve. Letters from one specialty to another do not appear to be flagged up by the system for action by the consultants involved. It was me that had to coordinate things in the end. One specialist was informed this morning that R was a no-show by the day surgery unit. That she had been admitted to a ward several hours earlier surely ought to appear on the hospital computer system but apparently it doesn’t.

From where I’m sitting, it would appear that the problem lies in procedures which assume a linear – and predictable – patient journey. Patients with complex needs may be a minority group but they account for a disproportionate number of hospital appointments and admissions. A system optimised for this group would work for all patients. The fault lies not with programmers, I suspect, but with the administrators and managers who commissioned the software. Who did they consult? Not me. Had they asked, I would have also suggested that every patient record have a front page listing all the current diagnoses and medications and involved specialities; that it would be impossible to close a record without this being updated; that all new prescriptions be automatically shared with the GP. But they didn’t and it doesn’t.

Things went ok today in the end and R is recovering well. But it could all have been so much better.