Last Friday

This time last week we'd just about settled William to sleep after a difficult evening. He'd become increasingly distressed with a cough, temperature and a whole load of gunk over the afternoon and spent all evening crying on our bed. The next morning we couldn't get on top of his fever, he vomited his meds and his respiratory rate was double what it should be. We decided to take him in and within 45 minutes of arriving he was in HDU on 15 litres of oxygen and not maintaining his sats very well. The nearest Paediatric Intensive Care Unit was warned. William's consultant came and chatted with us, sepsis was a real possibility and if his breathing got any worse we would be transferred to PICU. We thought this was our turning point.

We've had a lot of chats recently about what we want for William in terms of intervention should he go into respiratory failure. His lungs are starting to struggle and we need to be prepared. For William we have decided that we will intubate and ventilate on his first collapse. After that we're not so sure. Presuming he recovers our next step will be very much informed by how poorly he has been and how long it is until he becomes very poorly again. A consultant recently told us that we will learn a lot from that first PICU admission, If he's stuck on the vent for a week, suffers a lot and falls poorly again within months we might be choosing a very different path than if he bounces back within a couple of days and goes a year or so before falling unwell again. These are the sorts of choices families like ours make all the time, we all choose different paths, there isn't a right or wrong choice, you just choose what is right for you and your child.

So last Friday we thought we were on the cusp of this first major turning point. But thanks to God, some chest percussion and a bit of front lying the situation turned around. By Saturday morning William was managing well on 2 litres of oxygen, we were getting on top of his fever and his breathing was easier. 

We are now home with a whole lot of stuff to remember. William has oxygen in the home now, which we are weaning him off. He has nebulisers at least twice a day, chest physio at least twice a day, suctioning throughout the day and requires frequent position changes to stop the gunk settling in his lungs. This of course is on top of his usual medicines, managing his pretty violent seizures, calorie counting and blending 4 meals a day and all his moving and handling. It's got tough. 

Despite last Friday turning out a lot better than we were expecting I can't now shake the feeling that this monster is just a round the corner. The 10-15 years I'd always hoped we'd have with William is suddenly feeling ridiculously ambitious. Last week we were looking at getting a bigger car for William and his accessories. I was looking at holiday homes for next year after our incredible holiday two weeks ago. Now all of a sudden I daren't. My head is filled with timescales. Two days ago I'd convinced myself he wouldn't make it past 6. Today I can't shake the feeling we've only got 6 months.

The reality is we don't know. He has deteriorated but it's not dire. When he was 6 months old we thought he wouldn't live to see his first birthday and now we've had 4! Maybe 15 is still possible but our hope is shaken.

I think we're going through a period of grief. We've had a lot of illness this winter, our longest hospital stay in a couple of years, our worst chest infection thus far, a lot of new medical equipment and a lot of "those" chats. Hopefully the summer months will bring less infections and we can all have some time to rest, recuperate and take stock.

To end on a happier note...today Eliza was playing a rattle type toy and I started talking to William asking if he could hear it. Without prompting Eliza walked over to William, uncurled two of his fingers and places the rattle in his hand just as Steve or I would do. It brought happy tears to my eyes. I love that she takes him just as he is and is so thoughtful towards him. Having a child like William in our lives is an incredibly profound and wonderful experience and I love that I'm starting to see glimpses of her loving him.

And breathe...

Today William met with a couple of the respiratory team drs at addenbrookes hospital. William has always had some odd ways with his breathing owing to his brain malformation, but recently we've run into more problems.

William has been struggling with very thick copious secretions for the last few months. We now have a suction machine for him to help him clear it from his mouth which is very useful. William often sounds very bubbly and gurgly and uses up a lot of energy breathing through thick gunk then trying to cough it out. He has also developed more of a stridor where he kind of squeaks sometimes when he's breathing. We've also seen the reappearance of some apnoea episodes. A couple of years ago William started having some apnoea episodes but after a while they subsided. They only occurred when he slept sitting up so we could fairly easily stop them. We've now had a couple when he's been asleep lying down so they are more tricky to stop. A few nights ago I happened to check in on him and noticed he wasn't really breathing and then he did a big gasp and repeated. I checked his oxygen saturations and they were dropping very low during the not breathing times. We ended up having to turn him over to get him out of it. 

Due to these ongoing concerns we were referred to the team at addenbrookes for a review. We met with two brilliant consultants, a physio and a speech and language therapist.

We started off discussing the secretions. The dr felt there was a chance the excessive secretions were being worsened by an ongoing infection so we have a two week course of a particular type of antibiotic and William is being started on longer term prophylactic antibiotics. The physio wants us to start giving daily chest physio and has talked us through what she wants us to do. We also discussed maybe trying a special vibrating vest like this http://www2.hill-rom.com/medicraft/TheVest.htm to help keep everything moving in his lungs. We may also try some stronger saline nebulisers if the antibiotics don't help. We have already tried 0.9% and 3% saline nebs and will try 7% next to see if they help loosen everything up. 

William has previously tried two different medicines to treat excess secretions but they haven't really helped him. We could also look at having surgery to remove part of his saliva glands or having botox infections into his saliva glands to help control secretions. For this reason we are being referred to Addenbrookes' ENT team to discuss this further. 

It was also thought there was the possibility the secretions were linked to some kind of sinus infection. They are going to try some nasal flushes to see if this helps clear anything out stuck at the back of his nose. The antibiotics we have will also help treat this if this is contributing. 

We have recently started William on a blended diet. Instead of giving him medical formula we blend normal food and give it through his tube. One of the reasons we made the switch was to see if it might help reduce his secretions as it has done with other children. We are yet to see any improvements but will continue with the diet to see what happens. The drs thought it was good that we were trying this so we felt encouraged to continue.

We then moved on to discuss William's generally noisy breathing (see video below). There is still a possibility that he has large adenoids so the ENT team will review these to see if they need removing. William has very low tone because of his condition so probably has floppy muscles in his airways. The dr described his breathing as uncoordinated which all contributes to the noise. 

We then talked about William's apnoea episodes. I was able to show the dr a video (see below) of one of these episodes from a couple of years ago. In it you can see William making very little effort to breathe for a while before taking a big gasp. There are two types of apnoea, central and obstructive and the dr thought William's looked more likely to be central as he makes very little effort to breathe before the gasp. He explained it is difficult to treat a central apnoea as it's a problem with the brain sending out the signals to breath. We have discussed the possibility of William having some breathing support for the apnoea but if it is central they feel he would probably need a ventilator. If we are still having problems in 6 months they might look to do a proper sleep study to get more information. We discussed in some depth having CPAP but don't feel it will really benefit William or add to his quality of life. It won't help to extend his life and would just be more equipment to worry about. I asked whether there was the possibility of one of these episodes just causing William to stop breathing and not start again but they said that was very unlikely and you would wake up. We saw this the other night when William desatted to 57 then woke himself up to breathe. 

We will now be getting some oxygen for home for palliative use. At times William seems to work so hard just to breathe and it exhausts him. We are hoping have oxygen for occasional use will help to keep him more comfortable and provide us with a bit of reassurance when we are on long car journeys with him and can't reposition him if he starts tiring. 

On top of all this we had a lot of discussion today regarding William's ongoing lung health. Both the drs we saw today work a lot in the paediatric intensive care unit and see a lot of children like William when they are very poorly. We know that William is most likely to die from lung problems caused by increasing chest infections. We talked a lot about how this is most likely to unfold and what sort of treatments we would want for William. This is not new to us but it was useful to revisit these topics and get a clearer understanding of what to expect. Obviously things can always take a different turn and we can't know what will happen but it's good to be as prepared as possible. We felt quite concerned after our initial appointment at how concerned the dr was with the current state of William's chest, particularly with all his secretions and the amount he is likely to be aspirating. He was surprised that given William's current state he had not already had a PICU stay for a chest infection. In the second appointment I asked the dr outright what her thoughts were on William's life expectancy given our current situation. I was concerned they might say a couple of years max but thankfully she was more positive. She explained that most children like William make it to their mid teens before they start running into serious difficulties. While she can't obviously guarantee what's going to happen for William she also didn't rule out the possibility that William could still make it into his mid to late teens. Whereas we might consider a healthy person to live into their 80s, we have always known that surviving past his teenage years is very unlikely for William. We don't know what's around the corner, we don't know when William's first major chest infection will hit and how bad it will be but so long as we can keep hoping for more years then we're ok. 

Below are two videos, one showing William's apnoea and one showing William's stridor noises. I thought these might be helpful in explaining what I'm talking about.

 

Just to end on a lighter note...these are the smiles that keep us going. The smiles that let us peek at William's incredible personality and that show us we have to keep doing everything we can to keep him healthy and happy.