Cautious optimism

Just over 3 years ago I took a small unusual looking, floppy, oxygen dependent boy to a sleep study. A sleep study is a "routine" investigation for children with neurological problems. It involves stretchy bands around the chest, oxygen and carbon dioxide monitoring, and sometimes nasal flow monitoring. Nate's was performed at 11weeks because of the severity of his difficulties. We had been told some heartbreaking things about Nate already but after that sleep study night, instead of getting to go home we embarked on a very long hospital stay. The sleep study results explained that Nate didn't breathe properly when asleep- he had dangerous central and obstructive sleep apnoea. This led to dramatic drops in oxygen levels which did not self resolve quickly , and extremely high carbon dioxide levels.  I'm a science teacher and I had no idea of the effect of high levels of co2. Our little boy just lay there- the carbon dioxide made him extremely lethargic. A respiratory team doctor talked us through their plan- they would try administering CPAP using a ventilator and a mask. I was in bits. I'm not ashamed to say I sobbed my heart out. The lovely doctor was very supportive explaining everything but I simply couldn't take it all in. I couldn't grasp the idea of needing a machine to breathe. I'd only seen machines like that on tv with people intubated in intensive care. But that night Nate tried a few hrs of CPAP 

the following day our son smiled for the first time. 

That was the clincher. The "thing" that not only made Nate's need real but made  the treatment worthwhile. Not only could it "fix" nate's co2 and o2 levels but it gave us a result we could see and appreciate. It turned out that CPAP was insufficient and BIPAP was needed instead. 

However ventilation did not prove straightforward. 

Nate would thrash as much as his little body could to fight the mask. He would then stop breathing and turn blue. It's an odd colour to describe. More purple really, and etched in my brain forever. The plan was that we needed a week without him needing bagging to get him home. Many weeks passed with these "events" still happening and we had to discuss getting a tracheostomy for ease of ventilation. We put this off and asked for more time. Not for fear of the trache, but because Michael had faith that Nate could do it. Eventually we came home. Night one was fine, night two involved resuscitation and an ambulance.  Eventually a new mask appeared and from that point on ventilation became safe!  Yes he's had blueys since, but not due to the machine. We think the old style mask shifted as he thrashed and fought it, blocked his tiny nostrils, and for some reason Nate's brain panicked and shut down. For a long time after he came home with the new mask I was scared to be alone with him at night incase it happened again. I made Michael put the mask on him and then I would check his work ( this drove him mad). Nate had rocky times with illness where the ventilator proved no use even on 12l of oxygen, and humicare was required. But it has made a huge difference to his life; helping him develop and keeping his lungs clear. 

We became accustomed to the ventilator and it's soothing sounds. (When Nate started having respite away from the home none of us could sleep as it was so quiet) Gradually we became confident in his mask and using the machine. It became part of the bedtime routine. Yes there have been times I've wanted to put my fist through the bloody machine for its alarms but it has changed our lives for the better. Apart from being extra baggage to lug about, and the fact not all hospitals have staff trained in its use, it didn't turn out to be scarey at all. 

Last night we went in for another sleep study. We have them on a regular basis and usually they show that Nate's pressures need upping. This time, however, his consultant was very happy with him in clinic and requested a sleep study without use of his machine. I was nervous and excited. Nate went to sleep on oxygen administered by nasal cannula ( he was thoroughly unimpressed by this) . As the night went on nurses changed his oxygen and adjusted his probes, and I watched those important numbers. The sats alarm went off frequently but they had the levels set high for oxygen fiddling. The lowest he dipped to was low 80s and then came straight back up. His carbon dioxide was normal. Like what normal people have. People who breathe properly. Ace. 

So the verdict then- after 3 yrs of being ventilated at night Nate gets a month off ( for good behaviour). We have an appointment to go back in at the end of November for another sleep study (to check his levels are still acceptable). 

We've been told not to get our hopes up. That he may only manage a few days and need to go back on,  may need it when unwell, but you know what? 

That's fine by me. 

I haven't lost my blogging mojo... Honest

I havent truly, but it feels like it's been a while. 

Poor Leeds suffered the indignity of having 30-odd swan parents descend on it a few weeks ago. That's not that we are odd. You know what I mean. Cocktails, dancing, plasters, PJs, a dodgy nights sleep followed by the worst breakfast ever sum it up really. Lovely to see so many fabulous people :) all in the same boat so to speak. We do this every 6 months or so- meet up and have some fun. A break from our "undiagnosed" lives. 

Me and little mama. 

Then back to our "normal"

The mixed messages I mentioned last post have continued and tbh my head is battered by it all. "Yes Nate is having gelastic seizures, yes you've told me they can't damage him, but no I don't think its acceptable for him to have to just live with them when they take such a chunk out of his life" All very ARGHH really. Have to take him in when it happens next for another EEG. 

This weekend we have a sleep study sans ventilator. I think it's brilliant that they want to see how he is doing, and what his sats and co2 are like without it, but I can see it all going horribly wrong. A night of BEEEEEEEEEEEPs probably but you never know it might be extremely positive ( I'd settle for slightly positive to be fair). 

We have a run of appointments coming up - genetics, ENT, feeding clinic, and another MRI. The MRI is primarily to look at nates myelination, but also to see if there is an incredibly rare brain tumour in there. There will also be a skin biopsy for a mosaic genetic syndrome done at the same time. Oh and endocrinology! I nearly forgot about that one! 

With all of these appointments approaching I'm bound to need a moan so I will be undoubtably back blogging in my regular pattern! Yeah consider that your warning! 

On a positive note Nate has settled well at school and I actually feel happy leaving him there. He becomes animated and noisy when we get there, and greets staff with a huge grin. Nate had a fabulous day today but can be very tired much of the time. Add that to colds and now a urine infection and this can make it difficult for staff to get him to engage with activities ( always tricky which you have a reluctant often sleeping student) and each day can be totally different. When Nate is in the right mood he does well. Hopefully as time goes on he's in " the right mood" more often at school and less tired. I am happy so far though. He is getting all the therapies and attention he needs. The staff care about him and it's a nurturing environment. 

After a good run of form I'll have a think about what progress he has made and update you. 

Speaking of which we are thrilled to hear about the progress some of Nate's pals are making! 

http://littlemammasaid.blogspot.co.uk/2013/10/celebrating-progress.html?m=1

http://babyavasmiraclejourney.blogspot.co.uk/2013/10/proudest-moments.html?m=1

 It's up to Nate and his pesky genes now what he can achieve.