0

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.

 

Advertisements
0

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.

2

And the hits just keep on coming . . .

Two weeks ago, Moo and I spent three nights on Koala Ward at Great Ormond Street. It was a significant stay. She had an angiogram one day and an MRI the next to see if the EC-IC (extra cranial to intracranial, sometimes called an STA-MCA) bypass she had done in August had been successful. Moo seems to sail through surgeries but give her an angio and it’s hell. Last year, she had a 5% chance of reacting to it and proceeded to be completely paralysed down the right side of her body for four hours. This year, she woke up ok but was struck by crippling headaches from 7.20pm until 6.20am. Broken sleep, shrieking in pain, vomiting, it was all going on. So we stayed an extra night after the MRI to make sure two GAs back to back didn’t muller her.

The surgery has a 90% success rate. I went to Great Ormond Street on Thursday to get the results. When doctors won’t give you the results over the phone but ask you to come in to look at imagery, you know you are in for a rough ride. All they had told me in advance was that there was nothing untoward. I knew they were lying. I decided that meant that they hadn’t found a tumour.

The best news they could give me was that she has not had a stroke and the left side of her brain “seems to have stabilised”. The operation itself failed. It did not work at all. She still has a compromised left hemisphere with very little blood flow. On top of that, she has developed Moyamoya in her right hemisphere. In a year, it’s gone from healthy to compromised as the blood is not getting everywhere it did. She also has three little areas in her blood vessels where it looks like little balloons but they don’t know what is causing that. They may or may not be aneurysms or it could be that the vessel feeding into that bit is so narrow that it makes the “normal” part look like a balloon. The right side is providing some blood flow to the left so we need to stem the Moyamoya on that side before we do anything to the left. Her arteries in her brain are odd too. Apparently, they are not as wiggly as they should be and are a bit too straight. The EC-IC didn’t work so there is no point doing it on the right side. They think the best way forward is to do burr hole surgery to increase the blood flow. They drill holes all around the skull to encourage blood flow but the fact that she has a thick skull, thanks to the OS-CS, makes it more tricky. They could make things worse rather than better but if we leave it, she’ll have an oxygen starved brain, suffer a brain injury or worse and that would be insane. It explains why she has suddenly been having a lot of headaches. I reckon we’ve got about four months until she starts having serious TIAs on the left regularly and we want to avoid that. When Moo first had TIAs on the right after her left side of her brain was affected, they were severe. She ended up in ICU at one point because she developed waking apnoea where we had to remind her to breathe. Not only that but the TIAs addle her brain and she lost a lot of skills that she had worked so hard to achieve. Now it’s about to happen all over again.

Going home and telling my husband all of this was heartbreaking. He had no idea. As usual, I knew all along that the surgery hadn’t worked. My understanding of Moo is on an almost psychic level. There had been too much Moyamoya activity for it to have worked. However, those around me, including Mr G, assumed that because her TIAs were less severe and not as frequent, it would be good news. They didn’t take into account how often she blanched, word searched, slurred, how tired she could get. Those were the signs I was looking at. The news about the right hemisphere completely blind sided me. I knew she would develop it at some point but I thought we were years away from that.

They will do the surgery on the right first in September, hopefully, and then look to do it on the left. The left is more difficult because she has already had neuro surgery there. But they can’t tell us what the chances of success are or the prognosis because they said they are in uncharted territory. They’ve never seen a patient like Moo. It’s all an educated guess.

My greatest fear in all this is that Moo suffers a brain injury and starts to lose some of her ‘Mooness’, if that makes sense. Since the day she was born, our focus has been on quality of life. We want her to have the fullest, funnest life possible. She is such a character, so funny, loving, bright in her own way, infuriating, down right rude sometimes. She is Moo.

What do I wish for now? That they save her, no matter what? After the shock wore off and I was in bits, I sat and talked it out with our neuro nurse. If we are in uncharted territory, and things are not looking great, as a her Mummy, what do I hope? I don’t want her to suffer a severe brain injury and lose who she is, just so that I don’t have to say goodbye to her. I don’t want her to die either, obviously. It’s like being stuck in the most hellish no man’s land where no one can tell you what the future holds. For a control freak planner like me, it’s unbearably terrifying.

So I’ve decided not to think about it. At all. We are having an extension built as planned. I’ll focus on my dream kitchen. I’ve contacted the breeder that I want to buy a puppy from and found that she hopes to have puppies at Christmas time, which is perfect timing for us. We are going live life full blast and enjoy every moment.

People naturally want to reassure me, tell me to think positively, that everything will work out for the best. I would say to those people, please don’t. That’s not empathy. That doesn’t help me. You don’t know what the future holds. I don’t. I don’t want platitudes. Platitudes will wind me up. What I need is what my best mate gave me, a text back that said “Fuckety, fuckety fuck. Gutted. xxx” Then let’s plough on.

We have never focused our life around Moo’s health issues. We focus it around who she is and what she needs. She’s six. She needs love, boundaries, guidance and a lot of fun. So that is what she is going to get.