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Ladies and gentlemen, we seem to be hitting some turbulence . . .

Man, I don’t seem to be able to post on here regularly. Other things in my life just seem to take over. Or I just haven’t been very disciplined. Take your pick.

How have we been doing in the last eight months? Well, things have been motoring along. Moo’s neurologist describes her medical future as “stormy”. She put that in writing. Basically, we are no longer looking for cures, we are “managing her symptoms”. She is still having TIAs and headaches but they are in clusters and there can be months in between with only low level activity, which makes life increasingly unpredictable and unplannable. Mr G and I have decided not to travel abroad with her for the time being. Walking into a hospital in England, demanding a drip and morphine, is hard enough, never mind with a language barrier and different ways of doing things. It sucks because it means I can’t visit my family in Italy but there we are.

Moo’s last scan showed no changes in her brain structure which is great. However, she is being referred for an Autism Spectrum Disorder assessment as she has some “red flag” behaviours. She was diagnosed with ADHD in June, finally. I’ve been saying that I thought she had it since she was three, before the Moyamoya, but no one took me seriously until the neuropsychologist said that she thought Moo should be assessed. Cue assessment at the age of 8 and diagnosis. Better late than never. Only issue now is finding someone to prescribe her Ritalin to see if it will work. Neuro team have given the ok but community paediatrician is not comfortable. Her paediatrician at the local hospital is happy to do it, love her, but she’s never prescribed it before so needs guidance. It’s going to take ages to get this sorted. Moo on Class A drugs will be an interesting concept. Only reason I am keen to try is that one of the potential side effects is raised blood pressure, which coincidentally could help her Moyamoya.

Reading about Asperger’s as recommended only did two things. Firstly, it confirmed a long held suspicion that I have had that my father has Asperger’s. My father’s life and behaviour is essentially a checklist of the typical behaviour of a man with undiagnosed Asperger’s. Secondly, I have a lot of the traits. I might even be classified as a sub-clinical Aspie. That is to say, I have the traits but not so severely as to be a hindrance to my functioning in life. It was quite the revelation to realise this but also a complete relief. It explained so much about my behaviour and my feelings when I was growing up. I have already been diagnosed with OCD, my father is severely dyslexic while very intelligent. Both of these conditions is co-morbid with ASD, as is ADHD.

In the last week, I’ve also had conversations with Moo’s SENco (Special Educational Needs Co-ordinator) at school. Depending on how she does this year, there is a significant chance that we will have to move her to a special needs school. This has been very hard to come to terms with. You always hope that things will start to resolve themselves but it is becoming increasingly obvious that while the Moyamoya may not have altered her brain structure, it has completely addled her pathways. The gap between her and her peers is widening, socially and emotionally as well as educationally, and it may become too wide by the end of this year. It’s a watch and wait situation. In the meantime, I need to do some research as there isn’t a special needs school suitable for Moo in our area.

Then I’ve become a joint chair of the school’s PTA. It might seem a tad mad when I am already dealing with all of the above, driving a business that I really love forward and trying to find time to write children’s books, but no other bugger was going to stick their hand up and our school is so amazing, in part due to the PTA. No chair of a PTA, no PTA and I just couldn’t let that happen. I did manage to coerce two other people to do it with me and I think we will make an excellent team.

As a result, I was at the Year 3 welcome meeting this morning. A mother comes up to me and says “I’ve seen photos of your daughter at Shooting Star” and my heart just sinks. There’s only one reason a mother would be at Shooting Star House and she goes on to tell me about her beautiful little 18 month old boy who has SMA Type 1. I had to hug her. I know what that means. I love Shooting Star, I love my friends from Shooting Star but I hate it when I meet people out in the real world and they say they saw pictures at Shooting Star. It chips away at my heart that someone else is going through something so awful too. I know we wouldn’t change the children we have and we are truly blessed to have them but it’s an intense bittersweet love that I wouldn’t wish on anyone.

Tomorrow I am taking Moo to GOSH for a video fluoroscopy where they are going to test her swallow. It’s been doing funky things of late. I’m praying it’s nothing and that I am being over cautious but you just never know. I’ve learnt not to have any expectations at all.

Later this afternoon, I’m taking Moo to get her beautiful long brown hair cut into a bob. I feel awful about it. She loves her long hair but she hates it being brushed or washed or tied up. She flips out and won’t stay still. She has to have her hair tied up, otherwise it wraps around her trache and goes down her stoma. It has become such a pressure on our relationship that I managed to convince her to cut it into a bob. If she didn’t have the trache, this wouldn’t be so much of an issue. But then again, she plays with one side so much, it has started to thin so it’s not just the trache. In the great scheme of things, hair isn’t that important but to a small eight year old who wants to have hair like Rapunzel, this is huge. It’s another instance where her medical condition is preventing her from having what she wants. It’s another small bruise to the heart.

So today, I am finding it hard to concentrate on work for all the above reasons. I have a meeting later about the PTA but, in the meantime, I think I’m just going to write about boys who live on the moon and escape from my life, just for a while.

 

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I am seriously nuts . . .

. . . on so many levels. Firstly, who goes ahead with a kitchen extension when they know that their child with a tracheostomy is going to have serious neuro surgery? Secondly, Moo is going to be seven. She’s had a tracheostomy since day one. I am on Facebook so much that friends refer to it as Brittiebook. Yet, I have never once thought to join a support group for parents of children with tracheostomies. And I do love a support group. As a rather extreme extrovert, the need to connect with other people is very, very strong. Annoyingly so, some might say. Thirdly, this level of stubbornness and bloody mindedness just can not be normal. Fourthly, at some point in the near future, I have got to deal with my issues with vulnerability. I mean, how can I write about my innermost thoughts and send it out into the ether on this blog but, yet, put me in front of a real life human being and I clam up like a, well, clam?

One thing at a time. First, the kitchen. So, when we moved into our house last year, both Mr G and I said from the beginning we were going to have to extend the kitchen. The house is beautiful but it still has its original floor plan which means that it’s not brilliant for entertaining a group of people. As a passionate cook, I have always dreamt of designing my own kitchen but never thought it was a possibility until this house. We discussed having it done in the new year and, as I am such a proactive planner, I started getting quotes as I knew that the lead time may be long. Then, fatefully, someone mentioned that I had enough time to get it done before Christmas. Well, that was it. The idea of Christmas in a new kitchen, having struggled to manage it in the old one, was too tempting and I went hell for leather to make sure it could happen. I still didn’t have to go ahead with it but I did, even though by this point we knew that Moo’s EC-IC last year hadn’t worked and that the Moyamoya had spread to the right hemisphere which meant another op before Christmas. My problem is that once on a roll, it’s really hard to stop me. I think I can achieve anything. That’s how I ended up having a baby in the first place, against all the odds. This attitude has helped me so much in the past and it means that things get done, even in the face of insurmountable odds. It does also mean that I can push myself to my limits and be blind to how much I’ve taken on. There is also the very real fact that had I not had the stress of organising a build, I would have spent all my time thinking about Moo’s impending operation and that, quite frankly, could have sent me into a severe depression. As it was, the anxiety didn’t hit until the Wednesday before. So day one of the build comes along and I have explained to the head guy, who is brilliant, that Moo has a trache and dust is a real issue for her, like a life threatening issue. I’m all relaxed . . .  until I walk out of the living room to find dust billowing down the corridor as they are smashing concrete with both the back and kitchen doors open. The head guy wasn’t there, to be fair to him, and he gets on the case pretty pronto. Panic stations! I freak, they apologise and I take Moo to her music therapy session at Shooting Star House, where the therapist takes one look at my face and asks what’s wrong. Cue Shooting Star morphing into the big brave knight that they are and coming to my rescue  . . . again. We have essentially decamped here until the dust settles, literally, which I hope will be the end of this week. There’s still at least another three to four weeks of the build to go. That in itself has been a bit of a blessing as Moo’s recovery has been somewhat slower than anticipated. I keep having to focus on the fact that at the end of this, we will have a beautiful kitchen and we can start next year with a clean slate. No builds, no major ops, all we’ll have to deal with is the MRI and angio to tell us whether the op has worked, which, quite frankly, is shit a brick terrifying in itself, simply because the last one, with its 95% success rate, did not do anything. We won’t even go into the fact that next year is an odd year and, as yet, I haven’t had a good odd year in ten years. (2005, lose Ziggy; 2007, nearly lose Moo in pregnancy, 9 weeks labour only to be told she was going to die, tracheostomy and seriously ill; 2009, MLB confirms that trache likely to be more long term thing; 2011 beloved mother in law dies from pancreatic cancer; 2013 Moo diagnosed with Moyamoya . . . ) So, yeah, I was a bit nuts to start a build now but there are positives to be had. I may, however, agree with Mr G now and hold off on the dog.

Now onto the support group. There is a very real, clear reason why I have not joined a support group or a charity supporting children with traches. OS-CS is so rare and Moo’s need for a trache is so unclear that it felt like I was on this journey by myself, really. Yes, she has a trache and that is common denominator but the reason why is a mystery and, invariably, that is what I would have been looking for; someone to tell me why she had one, when it would come out and how we would manage. As it was, my support found me. I have a lovely friend, now, in the US with OS-CS who has also passed it onto her daughter and they both have traches. She tracked me down because I once did an interview on behalf of St George’s when the gene was found. Her support has been invaluable and so cherished that she negated the need for anyone else. Of course, now, six years on, I realise that my experience with Moo, of her trache, her gastrostomy, getting her off of her gastro, her speech disorder, inability to drink sufficiently; all these things are useful experience that I can share with others. I’ve been a little bit self absorbed. I met a set of parents at Shooting Star with a little boy with a trache and no leak, who can’t speak, and saw in them the feelings I once had of needing to see a child beyond their little boy’s stage. I had a need to see that it was all doable, that life would be manageable and that my child would be able to have a life full of joy, wonder and adventure, even with a trache, that we could be normal. So they asked for me to be added to the group, as it is a closed, highly supervised group, and now I get the opportunity to be my friend, to be able to share my experiences and, hopefully, help others the way that I was helped. I get to pay it forward and that feels rather fantastic. Just like how I believe that we had to endure and go through all that we have been through so that we could make our contribution to the human race by being instrumental in finding the OS-CS gene. I feel rather grateful for my life again.

As a result of that group, I’ve “met” a couple of parents with children with OS-CS. This has just spurred me on to make sure that I write the page about OS-CS. I’ve got the info in genetics speak, just need to translate it and run it by my geneticist. That may take a little time but it is now on my urgent list.

As for the third and fourth issues, I think only a qualified therapist can help me there. We all have our foibles. No one is perfect. We are all, actually, quite perfect in our imperfections. Some of us just have a bit more work to do to be comfortable with that.

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Obvious solutions, people!

My husband seems to have bad karma when it comes to toasters. He destroyed our very expensive 11 year old Dualit toaster, that we were given as an engagement present, with a potato farl. Instead of using the toast tongs that were attached to the side of the toaster, he kept pushing on the lever handle more and more aggressively until said potato farl got mashed to a pulp and spread all over the toasting filaments. A classic example of when an obvious solution completely evades a rational, intelligent human being. I mention this because I’ve just toasted a crumpet and the toaster smoked like it was sending signals across the prairie. I looked inside the toaster to see there were cinders of various sizes inside the toaster causing said smoking. I then spent five minutes removing all crumbs and cinders from inside the toaster so that it will now not smoke. I have to add that that was the first time I had used the toaster this week. My husband uses it all the time.

I can’t blame my husband. It seems that there are solution evaders in all walks of life. The one that has really got my goat at the moment is doctors. Moo has had a tracheostomy since the day she was born. At first, it was clear that she needed one as she had absolutely no airway and, without it, she would die. Obvious solution. Over the years, however, she has improved to the point that she now wears a Passy Muir speaking and swallowing valve for up to 12 hours a day. In tracheostomy terms, this shows that she has a very good airway around her tracheostomy because those things are permanently closed and only open when she breathes in. They are very hard to tolerate if your airway is occluded. Each MLB (micro laryngo broncoscopy, the op she has every year to check her airway and see whether her trache is ready to come out) she has had over the last three years has shown no change whatsoever, despite her growth and the fact that her Pierre Robin (cleft, abnormally small jaw) sequence has self corrected and her jaw is now in the normal position. Each time, her consultant mentions that Moo’s mouth doesn’t open that wide and it is a hindrance.

After her last MLB, I started thinking. Could the fact that her jaw does not open that wide be the reason that they can’t see her trachea safely? If they can’t see her trachea safely, they can’t remove the trache because she is classified as unintubatable. If she was knocked unconscious without a trache, they could not guarantee they could intubate her and save her life so the tracheostomy has to stay put.

So I go to an appointment at the cleft clinic at Great Ormond Street, where all of Moo’s complicated care is, and mention this to the doctor. The doctor talks to the ENT registrar. The ENT registrar talks to me. It is possible to make a jaw open wider. It’s not easy and it doesn’t always work but it is possible. There is a potential problem with the larynx that would mean it would be pointless and, if Moo’s consultant thinks she has this problem, there is no point trying. He’ll talk to the consultant and get back to me. I get a call a couple of days ago and the consultant thinks that, actually, it’s worth trying.

Basically, after three years of no change, there is the chance that widening her mouth may result in her being able to be decannulated and get rid of the life threatening tracheostomy. Now, seriously, why has no one thought of that before? Doctors are so stretched that they see patients, deal with what they see and can treat but all thinking seems to stop there. Could there be a way around this problem, could there be something we could try to make things better?

When Moo was a baby, she could projectile vomit about 30 feet. It was impressive. She managed to vomit across a brand new Starbucks; the carpet, the coffee table and the brand new leather sofas. My NCT group were shocked. The Exorcist had nothing on this baby. It was evident from birth that something was going on when she ate. It started with her wiggling and looking uncomfortable. She had terrible wind. At three months, the vomiting started and just got worse and worse. At first, it was only a couple of times a day, then it was after every single feed. We would keep her quiet and still for an hour and a half and hope it would stay down. Sometimes, it did. St George’s diagnosed reflux, our local hospital agreed with them. She was on Domperidone, Ranitidine, Gaviscon; the maximum doses of all three. The vomiting continued. She had two 24 hour pH studies done, numerous barium swallows; all of which showed minimal reflux or none at all. The hospital insisted it must be reflux. She vomited on doctors and nurses and, even though the milk would look like it had come from straight out of the bottle an hour after being ingested, they still said it was reflux. My mother said “maybe she has an allergy”. I told her the doctors would have thought of that. I was recovering from my trauma and only after six months on medication did I feel like my normal self and started thinking about it. At which point, Moo had been on medication for 10 months, to no avail. Finally, I put my foot down and demanded that they try changing her milk. So she went on Nutramigen which improved things slightly. Then they changed her to Neocate which your body has to learn to digest because it is milk broken down to its amino acids. Ten days later, the exact time it takes for the body to learn to digest it, and Moo stopped vomiting, completely. The irony is that she grew out of her allergy eight months later.

The doctor said, “so Moo has a milk protein allergy”. Really?! No shit, Sherlock. You let her suffer for ten months without thinking about it. She was on seriously strong medication for no reason. The only reason they found out about her allergy is because I put my foot down.

My cousin has just had a baby. Said little dude is suffering with all manners of trapped wind. The story sounds familiar. Has he been tested for intolerance or allergy? Apparently not, as the doctors will only do that as a last resort. Is it just me or is that ten tonnes of stupid? A child may or may not have an allergy to milk but they’re going to make the child ingest it until they have tested every other avenue. Here’s an idea. Why not test for milk allergy or intolerance first, rule that out, then move onto reflux? To treat an allergy you change the milk, to treat reflux you have to dose the poor child up with meds and get the balance right. However, if a child has an allergy, no amount of medication (which costs the NHS thousands) is going to help.

The moral of this rant is really this: if you think you have an idea of how to solve a problem with your child and you are dealing with the establishment, speak up because, if it’s obvious, they won’t have thought of it. They simply don’t have the time to analyse and think about each and every case. And sometimes, the most obvious solutions are the ones that are missed completely.