TrachKids.org

So, it's September. Last week I got word that the Americans can come over between the 5-13th December this year. It's much further away than I'd hoped but it's the closest we've come to getting a date for surgery since the start of all this. Why the big delay since funding was approved?? Well, the Prof in GOSH was stone-walled by the head of ICU who thought that treating kids like Angus wasn't really GOSH's remit/cost effective/etc. The Prof then had to present his case to the Rare and something Diseases group within GOSH. Then we had to wait for their approval, and eventually this all cam eto pass, but the most frustrating thing was that for months we didn't have a clue what the delay was and even whether anyone was doing anything. It was very disheartening after thinking things were more or less done once the funding was approved.
It looks like it's really going to happen now, but we've been at this too long to really think it's going to be straightforward. People could get ill, Angus could get ill, swine flu could strike with a vengeance etc. The possibilities for disappointment are endless. I try not to be too negative, but it seems prudent when there have been so many unseen obstacles along the way.
Angus started school 2 weeks ago. He's on half days until the end of this month. He's enjoying it, but finds it very tiring as he can have fair distances to walk within the school. He's still on the vent and O2 full time and is becoming increasingly unhappy about it. He has a power chair for use during break times so he can move quicker without getting tired, but it does rather separate him from the other kids. He and I talk occasionally about the benefits of being small - we both agree that the best is him being easy to pick up and cuddle. We're fortunate in having a number of good friends with other forms of dwarfism who are very positive role models. I don't see Angus having major issues with his size for a number of years.
One thing he is starting to have an issue with is his extra fingers. He says he wants to get rid of them. We really don't want him to because they're part of him (and we think they're cute), but if he still feels that way in a few years, we will have to discuss this more practically with him. He doesn't like the way they stick out when he makes a fist (no-one in the family has pointed that out to him). It's going to be a tough call. I'm so tempted to be a coward and leave it until he's legally in charge of his own body and then he can go ahead and do what he wants, but I don't want him to get to a stage where he's miserable about it as a kid.

Angus had a cardiology and dietetics appointment today. Neither Gav nor I were worried about them at all. Perhaps we should have been. Angus' pulmonary hypertension has got to its highest ever since his 2nd open heart surgery and is now a cause for concern. He said that it's at a level where it needs treating, but he wants to review him in 2 weeks because Angus has shown a trend of spikes before followed by lower readings, though his spikes have not been as high as this ever. His sats were 86% on the vent, but off O2 - not great.
 
The cardio said that this makes it even more important for him to get his thoracic expansion operation done soon. He's going to contact Angus' respiratory consultant and discuss his concerns. Depending on what happens at the review appointment, I think they'll be aiming to get Angus in for a sleep study ASAP to see if his O2 and vent settings need adjusting. It might also be that he'd benefit from a cuffed trachy at night as he does have a significant leak. I don't know how I feel about this. I suspect I'll get upset, but I have a real delay switch with emotions these days.
 
On the up side, he now weighs 12.86kg, and is 84cm tall so the dietician was very pleased with him. I even got round to phoning the Endocrine nurse today to tell her of our difficulties getting the growth hormone. She got back to me to say that the delivery problems have been sorted now so the pharmacy should try again. Watch this space.

Finally it happened!! We have a decision on Angus' thoracic expansion surgery. The funding has been approved, and it CAN take place in Great Ormond Street. Now we are waiting for a date. This has been such a long journey, and it's not over yet, but this is the first proper, definite decision there has been. It's terrific!!
Later this month Angus will be starting growth hormone treatment. Our only problem is getting hold of the growth hormone. The pharmacy can't get hold of the stuff the GP prescribed, so we'll need to get another script for a different brand. We've got all the stuff to do it except that. Then we have to make an appointment with the Endo nurse to administer his first dose.
Angus is eating pretty well now. He eats

• chocolate
• butter
• cheese
• Marmite
• and his new food - tinned pasta in tomato sauce

He still needs a fair bit of coaxing and reminding to eat, but he's making pretty steady progress.
Since attending his present nursery full time (he was part time last year) his speech and attention span have noticeably improved. They weren't bad anyway, but they've still got better. He has been singing more as well and remembering the words to lots of rhymes and songs.
He's got a cold and runny eyes just now, but his general health has been pretty good this year. He had one course of oral antibiotics near the start of the year, but that's all. Hopefully next year is going to be even better. If the results of surgery as as good as they can be, it's not beyond the realms of possibility that he could wean off the vent. Even if it were only during the day, it would mean a huge increase in freedom and normality for our family. Roll on 2009!!!

We, my mother and I, went to see Mamma Mia tonight. I was anticipating a feel good film that would leave me feeling happy and light by the end. Now, I just feel like there's something wrong with me. For those of you who have still to see it, please do. It is a happy-go-lucky film, it's just that as I watched scene after scene of sunshine, dancing and people having a carefree happy time, I just felt so sad and lost. I yearned to be able to go somewhere hot and sunny (I haven't left the country for 5 years). During the scenes with all the women dancing along the paths and pier my eyes started pricking as I felt acutely that I had lost the ability to let my hair down and be carefree. I don't know if I can remember how to have a really good time anymore - one where I haven't got a medication and tube feeding schedule in my head; one where I haven't had to plan it over a week in advance, write lists of care plans for people, feel like I have to check in and have my phone within easy reach blah blah. I just can't let go. I can't remember the last time I let go fully. On the rare occasions I get the chance of a night out, I'm too tired, and too nervous/unsure to go out there and do things. I'm so out of practice. So I sat there holding back tears through most of it, feeling ridiculous, frumpy, boring and fat. I miss the person I used to be. I now feel really ennervated, deflated - surely not the emotional state the film-makers were hoping to evoke!
 
Could I tell my mother? NOOOoooo. I tried to hide how I felt during the film and after as well as I could. She already feels guilty enough for living and working outside the UK. She feels guilty for not being able to help me as much as she would like. She's got some health problems of her own and is rather depressed about them too so I know she would take it really badly if she knew what I'd written above. She'd be so desperate to help, but she's leaving tomorrow to go back to work. It wasn't the right time. Besides, the film helped lift her mood. It really would have been crappy of my to burst that bubble. I don't know if I can ever lay this on her.
 
When I have stopped wallowing, I will pull myself up again (don't we always?) and work out ways of having a bit more fun. I know I really need to push my DP to take more responsibility. It's not that he doesn't take care of the kids, it's just that if I am around he simply doesn't take charge. There have been a few events we've been to where it's been my turn to supposedly let go and have a bit of a drink, but even so I end up doing the lion's share of feeds, med administration etc. This must make him sound like he's useless and thoughtless, but he isn't, I just can't let go of that internal timetable. It doesn't help that this year he's already had 2 trips to Hong Kong on business for a fortnight, and is going away for 3 weeks next month. I'm so used to taking charge of everything that I don't even really miss him. What I really need is to get away from him, and both the kids and have a weekend away with some girlfriends - it doesn't even have to be anywhere exotic, just away! I think I'll leave my mobile phone off or at home too. I need to prove to myself that I AM dispensible in the short term.
 
BTW, still no word from any of the relevant parties re Angus and the LTE. They now have the authorised letter from Prof Elliott. I am going to start phoning and writing letter now instead of doing emails. Letters have to be read and filed. I'm not sure that emails are.

I suppose it was too much to hope that the letter from the Professor at GOSH would get done and sent the next day as promised. :sigh: I emailed Dr. P at the end of last week to see what he thought and to give him my version of the discussion at the appointment. I got an email back on Tuesday - a week after the appointment - to say that he couldn't move things further on until he received the letter. So, the rest of this week I've been phoning his PA to see if they've got it yet, and phoning GOSH to see if they've sent it yet. It turns out that the letter was dictated over a week ago, but it has not been signed/authorised because one of the surgeons is off sick and the Prof is filling in for him. I feel a bit guilty about chasing him up to sign this letter when there are emergency and planned surgeries which are much more important in the grand scheme of things, but my priority is Angus and getting what he needs. I'm fed up of things not being straight forward when they should be. I'm more than fed up of having to check up on progress and chase things up all the time, every step of the way. I have learnt not to trust what busy people say they will do - and I hate having to give up my naturally trusting nature.
 
I had a chat with one of my patients at the start of the week. I've seen him for a number of things over the years and know that he is involved in management within the NHS. So I asked him if he knew anyone on the board to have a chat to them and make our case a little more personal. He said to email him with the details, which I did, but I haven't heard back from him either. I know it's only a fortnight, but all these delays add up - the fact that we've only got this far since Feb 2007 shows that!
 
Angus is doing OK at the moment. He has a major snot nose going on, but it's not making him ill. He's reached his target weight of 12kg so we've reduced his tube feeds to 300ml/300kCal per day again to encourage oral feeding. Since then he's been making bit more progress with eating. He ate 2 tablespoons of puree yesterday which is a first for him - at two separate sittings. He's been eating thin sticks of cheddar, tomato ketchup (I made my own so a bit heavier on the tomatoes and lighter on the salt and sugar), butter and Marmite. He's also been drinking more of his chocolate milk, but the Feeding Clinic want me to start buying low cal chocolate milk so he doesn't fill himself up with drinks and starts getting to grips with solids more.
 
He's heavily into Power Rangers Mystic Force and has been watching Disc 6 of the box set endlessly. I've kind of fanned the flames by buying him 3 different types of "Morpher" from eBay. He loves the Mystic one even though it doesn't work electronically anymore. He's been "rangering up" at every opportunity which is cute and funny.