We are driving to school. The radio is tuned, as usual, to Radio 2, the station that serves as the compromise between what I want to listen to (BBC Radios 4 and 6) and what F and EB want to listen to (Capital FM). A female singer from the early noughties is singing live. The moment that she is announced, I know that R is going to be unimpressed. It would be fair to say that women of the over-emoting sub-Whitney big-voiced school of singing are not really her cup of tea. I wait for the teeth grinding to begin. Instead, I hear that too familiar syncopated breathing that indicates that R is having a seizure. Sure enough, as I look in the rear view mirror, I can see R convulsing. I switch off the radio. The seizure stops. Immediately.
R’s response to music seems to us to be more than just the expression of taste. Music distresses her as predictably as it soothes her. While we were on holiday, we visited a restaurant with the children. The restaurant, although casual, was trying to project a cool vibe. The music was “ambient”, heavy on woozy synthesisers and lacking in structure. R hated it. As we waited for our food to arrive, she became more and more distressed; colour drained from her face and her pupils dilated; she became floppy. Not knowing what else to do, I brought her downstairs to the toilets. Unusually, the music was not piped into the ladies. Relief. R perked up straight away. Not just a bit. She was back to her usual giggly, squealy self. Immediately.
I have no evidence for this, only a hunch, but I wonder whether part of this may be due to synesthesia. Synesthesia is the rare perceptual phenomenon where there is a cross-talk between the senses. Someone with synesthesia may taste colours or see numbers as textures. Some synesthetes experience music in colours or textures. What if R experiences music like this? Dark, scary film music results in hyperventilation and real distress. What if this were because she experiences dark, scary film music as something quite literally dark and scary? Some of the theories behind synesthesia are based around the idea of an abnormal maturation of the nerve pathways in the brain. Normally developing infants have many more connections – synapses – between the nerve cells in different brain areas than adults. The “correct” pathways are reinforced by experience in early childhood leading to a pruning of the redundant pathways. It has been suggested that some of these pathways, for example linking brain areas responsible for processing music with those for processing colour may, in synesthetes, survive. Rett syndrome is characterised by a disruption to development that begins right around the time that this so-called synaptic pruning should be taking place. Perhaps synesthesia is the result. Perhaps, indeed, this could be of a more intense form than someone who has otherwise had a normal neural development. I asked R whether she sees colours when she hears music; she indicated that she does.
Regardless, the emotional and physical response that R has to music, has intensified of late. R doesn’t see why she shouldn’t dictate the music played outside of our home as well as within it. This really isn’t realistic. So for the foreseeable future, it looks like dining out will be at Frankie and Benny’s. The food may not be great but at least there classic 50’s music of the sort that R loves is always on the menu.
I am all wrung out. R had her first appointment with the wheelchair service this week. It was arranged by the new council OT. She is astonishingly pro-active. I had requested a referral to discuss whether R might qualify for funding to help us pay for the house adaptations that she requires. The OT was surprised by how little equipment R has. The complete inventory comprises a stair climber – obtained optimistically four years ago for the used of care workers providing the home-based respite that we still await – and a Maclaren Major Buggy. By the time she left, the discussion had covered every aspect of her care; she even provided advice around toilet training.
On Friday, our first pieces of adaptive equipment arrived: a bath lift and a Heathfield chair. We have reservations about both. But the OT’s goal with these is to increase not limit R’s independence. We also discussed R’s mobility. R is pretty lucky. She can walk for a start. And her back is straight. But she can’t walk far and, at 9, she is starting to get too heavy for me to carry. R got her buggy just before she started school. The OT felt that a review was long overdue.
R was nervous as we entered the room. The clinician walked ahead pushing R’s buggy. R was gripping tight onto me with both hands and dragging her feet. Clinical consulting rooms all look the same and, for R, are associated with discomfort, pain and disappointment. The clinician, an OT, was very nice and R soon relaxed. She explained that, in her view, it was probably time to think about a wheelchair for R. She said that wheelchairs are more age appropriate than buggies, that a wheelchair raises a child up to a more normal height, that people in the street are more accepting of older children in wheelchairs than they are of children in buggies and are less likely to stare. I think that it was at around this point that I started to cry.
I hadn’t expected to react like this. It’s not like there is big difference between pushing a 9 year-old in a buggy and pushing the same child in a wheelchair. And yet there is.
The clinician went to get a chair while I composed myself. R was impressed. A little chair with a metallic red frame, it apparently also came in pink. R chose pink with large orange wheels (decorated with daisies). She was very excited to convey this news to her friends at school yesterday. I am just barely holding it together.
Well, we made it to the coast in the end, to the great relief of everyone. F and EB as well as R had been looking forward to this holiday all year. This year, as for the last 5 or 6, we were to spend the holiday together with a school friend of G’s and his family. He has 4 children who are all steps and stairs with ours: his older son is a year older than F; EB comes right between his two girls; then comes R and then his younger son, P. P is a very special little boy. P is R’s best friend.
One of R’s great strengths is her open, friendly nature. She loves nothing more than being right in the centre of things as part of a crowd of children. This rarely ever happens. Mostly children respond in one of three ways to R: they ignore her, they stare at her from a “safe distance” or they treat her like a doll. R just wants to play but it is both difficult for her to articulate this and difficult for her to do.
P has known R his whole life. Being younger than R, he cannot remember a time when she could talk or run. Being the youngest, he understands what it feels like not to be able to keep up with the older ones. And like R, he enjoys sitting down with a book and being read to. They clearly enjoy each other’s company and always have.
Both families had rented a house this summer. The children always beg to sleep over with one another. R wants to do this too but for obvious reasons, she needs to stay with us. On the first night of our holiday, all four girls stayed with us. R was desperately jealous of the fun that the older girls were quite obviously having in their room. The second night, it was the turn of the boys. P gallantly agreed to start the night with R (if he could then go through to the other boys – fair enough). R was just a little bit thrilled.
We are not where we planned to be. Right now we should be enjoying a seaside holiday on the north coast of Germany. Where we are, is back on the neurology ward of the hospital near to G’s parents, 7 hours further south. R has had a horrible 36 hours of seizures.
R really doesn’t travel well. Invariably, she arrives at her grandparents in Germany very much below par: pale, listless and lacking her usual sparkle. So when I start on my usual speech about how smart, alert and curious she is – in imperfect German – it’s perhaps unsurprising that family and friends here are hard to persuade.
A few days before R’s seizures started we were at dinner with an old friend of G and his family. J is a Professor at the same hospital that R is currently being treated. A man of little vanity (to the exasperation of his wife I suspect), he has decided that a monocle is the most practical form of reading correction for him in his clinic. Mostly he wears more conventional reading spectacles outside work but on this evening he had apparently forgotten to bring them. In response to his enquiry about R’s schooling, I started ranting on about how we consider her to be cognitively far better than most professionals and how she has demonstrated her literacy using her Tobii Eye Gaze. I recognised the signs. He didn’t really believe me. Undeterred, I borrowed my brother-in-law’s phone and showed him the pictures of R from this blog. He took one look and his monocle dropped out onto the table. R roared with laughter.
I have been known to grumble about the NHS. It was my impression until now that German healthcare was better funded and possibly better organised. We arrived at the hospital on Friday night to find a single junior doctor was manning the entire children’s accident and emergency department. There are no doctors on the ward and no neurologists on call. I phoned home yesterday (Saturday) and was able to speak to R’s consultant, who fortuitously was on call. He immediately sent an email for the attention of the doctor in Germany regarding R and how he would wish her to be managed. It’s not ok that there are insufficient nurses on UK wards to provide the basic care (feeding and hygiene) that a child like R needs. But on balance, I would prefer to provide that care myself if it means that specialists are available on site when they are needed.