This Is Our Normal Now..

People keep asking me if Dylan will be home soon, and that now he is on treatment they hope things will get back to normal soon. It's a lovely sentiment and it makes me smile to know people are thinking of us. I find it difficult to respond at times or say the right thing, I think unless you've ever been in the situation it's hard to understand the implications a long term and life threatening disease has upon a family and what they consider to be 'normal' or for how long that will last...

Chemotharapy for example can last anything from 6 months to 6 years plus. In Langerhans Cell Histiocytosis it is not uncommon for treatment to be up to 5 years! This means more than just 'hair loss' it means stomach problems, bowel problems and soreness, muscle aches, sickness, infection risks, decreased or increased appetite and general fatigue amongst other symptoms depending on which type of drug you are on.

Dylan's constant collapsed lungs, these are happening daily now and whilst eventually it should slow down now new cysts cannot form, this process can take MONTHS. Whilst this is happening he needs constant tubes to drain the air from his chest or it can compress his lungs causing respiratory failure or even compress his heart and cause cardiac arrest or worse. These tubes have to stay below heart level as they are water sealed to prevent air going back in. This means he cannot leave the bed properly, run around, or even go and play in the playrooms.

This is but a glimpse into our life at the moment, and we are only 2 months into a very long journey...

I have only slept in the same bed as my husband for a maximum of 14 nights since Christmas Day 2015.. or even under the same roof for that matter!

We spend one night at the hospital with Dylan each, then swap and spend one night at home with Bryce..

The house feels so empty!! Bryce goes to sleep in a bedroom with an empty bed in it, a bed that his brother only moved in to on Christmas Eve whilst poor Dylan sleeps in a hospital bed away from all his family, as there is no bedside space in HDU only parent rooms down the corridor.

Bryce and Dylan don't see each other 5 days a week, there just isn't time to sit in the rush hour traffic back to the QMC after school and then be back in time for dinner, homework and bedtime.

Dylan is scared, confused, bored and in a strange environment being poked and prodded and he only gets the comfort of both his parents in the same room for 4/5 hours a day at a time before one has to head back to fetch Bryce from school. Bryce also only has one parent at home with him each night / morning.

Mike isn't able to work at the moment..

We have another baby due in June and haven't brought a single item yet. All the boy's old clothes are still in the loft, the nursery needs re-painting, we don't have a pushchair, bottles, steriliser, baby monitor or even a crib anymore and we just haven't had the time to get anything yet! To be honest it has been the last thing on our minds.. it felt like all the excitement of a new baby was ripped away from us at only 3 days after our 12 week scan when Dylan first became ill..

Dylan's birthday is in 2 months.. He missed Bryce's and will no doubt be spending his special day in his hospital bed..

THIS is our 'normal' now and could be for quite some time.. it is exhausting, emotional, stressful and downright lonely at points BUT I would rather have it this way than not have Dylan still with us at all..

And that's what it is all about I guess, putting life into perspective despite the struggles we all face daily <3

Pleurodesis Take 2..

Yesterday the decision was made to fetch Dylan off his oxygen, it was clearly the right decision as it appears his sats are actually BETTER now than they were last admission which is baffling! I guess what this could mean is with all the cysts bursting and treatment going on that his lungs are working slightly better now and less 'stiff'?! Here's hoping!! Oh but doesn't he look beautiful without his nasal cannula in...

They also decided to do a double sided pleurodisis this morning at 8.30am.. this was a massive shock to us as we thought it'd be a long time before that was even discussed let alone booked in! The surgeon that did his previous one weighed in on it all and decided he would do it in the ICU department under ketamine again so not even in theatre! This again surprised us, but it is a fairly simple procedure that uses the chest drains already in place to put the solution inside the cavity, and it minimises the risks associated with his lungs and general anesthetic.

He had the procedure done and has been fantastic ever since! Minimal discomfort and happily playing away despite the chest drains. It'll take some time to see how effective it has been, but it's not like we are going anywhere any time soon! Truth be told it probably HASN'T worked as he should technically be feeling at least some discomfort and it looks like all of the solution has come back out of the lower right side drain which is frustrating. There is always the option to try again though whilst we are still here..

Two Weeks

So, it's been two weeks since Dylan's episode..

That's two weeks since I thought my baby boy was going to die in my arms..

Two weeks since I was repeatedly told he wouldn't make it..

Two weeks since Mike had to fly back from Barcelona early so he could say goodbye..

Two weeks since we were told his only option was Ecmo (temporary lung bypass) or transplant which he was too sick for anyway and wouldn't have survived..

We have had our ups and downs along the way..  He currently has three chest drains in after needing an extra one on his right hand side yesterday. He is still on 2ltrs of oxygen but that is mainly for helping healing not through necessity. He is physically and mentally back to where he was prior to his episode despite the constant bursting cysts and the original worry of brain damage from lack of oxygen..

Today Dr. Helen Spencer from Great Ormond Street Hospital came to visit us here at the QMC. She is the transplant consultant for GOSH and was here to do his transplant assesment that prior to his episode was booked for Monday the 22nd. A lot has changed since that appointment was made, we now have a diagnosis and we are on treatment to stop the disease but unfortunately that will not reverse the damage already done. She told us a lot of information we had already researched ourselves.. lung transplants are not a cure, the life expectancy after one is not guaranteed but their median expectancy is now ten years with 60% surviving that long. If he gets listed there is no going back, we cannot change our mind if a set of lungs become available and risk wasting them as they are so valuable. Lungs can become available within hours or years and there is no way of predicting that so we cannot leave the decision until the last minute or it may be too late. There are many many risks with the surgery and afterwards as it is a very complex and serious operation taking up to 12 hours or even more. It is not a decision to be taken lightly and we need to seriously think about whether we want to go down this route or not..

If it was clear that it was the only option we would go ahead, myself and Mike had discussed this previously at great length before his diagnosis when we thought he was terminal. It sounds selfish in some aspects putting him through such a serious procedure if it might only give him a few more years with us but we only want what is best for him..

However, it is not a decision we need to make right now! As it stands, in her opinion, he is NOT a candidate for transplant at the moment and it is important that we give the treatment chance to work and see how well it works first.. This was actually the first thing she said to us before the assessment began and it was a breath of fresh air! We were worried we would be pushed in to making that decision today based on how bad he was on the 10th, but the fact he has 'recovered' back to where he was prior to that is very promising.

This means we can consider more effective methods of pleurodisis to stop his lungs collapsing and that as long as his lungs eventually stop collapsing and the cysts stop bursting, he should cope for now with the amount of healthy tissue he has available. Whether that will be enough in the long run is a question nobody can answer, it is just a wait and see, but for now he is doing ok!

Expect The Unexpected

So apparently having two 'working' chest drains in isn't enough peace of mind.. especially if the left one isn't working again at the point it is needed!.

Dylan burst another left bullae last night and the chest drain wasn't working again.. it was a slow leak luckily and got worse gradually resulting in a new drain at 3am ish. Dylan really likes keeping us on our toes and giving us sleepless nights, so the title of this blog is apt really!

They wheeled him back around to ICU to do the drain in the end as things just weren't getting done on the ward and people kept getting crossed wires or not reading all the information. We are staying on ICU/HDU until all the departments can have a meeting and sort out some strict protocols and procedures for dealing with him when he has a pneumothorax.

He is fine in himself and both drains are working nicely for the moment. Dr Spencer from GOSH is still coming Wednesday morning so tomorrow he will have all his tests in preparation for that which he won't enjoy but it has to be done!

Despite having a rough night last night he has refused to nap today and has instead spent most of the day eating! I think the steroids have well and truely given him the munchies that's for sure!

He is asleep now, hopefully he will stay settled and we will have a peaceful night fingers crossed!

Downgraded Again!

Yesterday was Bryce's birthday and in the morning we went to visit Dylan and Daddy at the hospital with his presents (and a present for Dylan) just as he had requested. It was lovely watching them together, Bryce kept letting Dylan open his presents and had taken him a party bag and a cupcake.

Despite having two 'working' chest drains in, Dylan's right lung was still deflated so shortly after myself and Bryce left at 12.30 they resited it and it started working much better!

Bryce's party was fab! He had a few no shows but there were plenty there and everybody seemed to have a good time. I had lots of help from family and I couldn't have stayed sane without them! Bryce got lots of lovely gifts and is very grateful to everybody.

Once arriving home and after Bryce had opened all his gifts from his friends, I was informed by Mike that Dylan had been moved onto the Oncology ward! This was the good news I had been waiting for all day as it officially meant he was over the worst of our ordeal and stable enough to begin a long term care routine.. Obviously he will have more pneumothorax and need chest drains putting in for a long time to come but they have a plan in place now that seems to be working so hopefully the risk of him crashing like he did before is minimal now.

Myself and Bryce went back to visit him today and take him some gifts people had dropped off for him, including some 'wiggly bags' for his Hickman line. The ward is absolutely lovely.. it's like a private hospital! Dylan's tv isn't working but they should be fixing it tomorrow so that's no major issue. He is getting a lot more care and attention now he is the correct place which is great. His right hand chest drain is still bubbling away a lot which shows there is clearly an ongoing leak, we know these can take weeks to heal, hopefully it will slow down soon.

Having two chest drains in really limits his mobility and he is pretty much confined to his bed which I think he found difficult at points watching the other children playing and racing up and down the corridor on ride-on cars. As much as I like the peace of mind I get knowing he has a working drain in each side, I wish he could get up and play and try to live as normal a life as possible... hopefully the pneumothorax will go soon and occur less frequently eventually...

Sleep Deprived!!

So after my last post yesterday things just got worse..

Dylan slowly deteriorated as the night went on.. his breathing got worse, he got very distressed and his CO2 level crept up. We all assumed from the right hand side leak! But nope! An xray at 1am showed the left side chest drain had actually become dislodged and air had reaccumulated quite a lot.. Now whether this could have been discovered earlier rather than allowing him to struggle and suffer all night is another matter, but they soon wheeled him back around to ICU and by 2am he was having a new chest drain put in on that side..

He came round fairly quickly after the drain.. in fact whilst they were cleaning him up he was babbling away like a drunk which was pretty hilarious! His breathing improved straight away as did his CO2 so no need for ventilation.

They did an xray to check placement after the new drain was fitted and the right hand side then had started to reaccumulate. Luckily it was only doing so slowly as air could still escape and he was breathing fine so they left him overnight and I finally went to bed at 3.45am!!

We got moved back to HDU this morning and then they put a right sided chest drain back in this afternoon, and after a bit of suction it seems to be working well so that's is positive. We've just got to accept that Dylan might need constant chest drains for the next few months but as long as he is happy and stable then it really doesn't matter as they don't seem to phase him now.

Tonight is my night at home, busy baking and prepping for the biggest boy's birthday and party tomorrow! Fingers crossed Daddy has a smoother night than I did yesterday!!

Emotional Rollercoaster..

That's the best way to describe today!!

When I got to the hospital this morning, I could hear Dylan giggling and playing from down the hallway! He was so full of energy and it was lovely to see. The right hand chest drain had been clamped for well over 16 hours with no more air leakage so at 11am they removed it...

Then air started leaking causing the dressing to fill up like a balloon! Dylan got very distressed by it all of course. Eventually the air found it's way out of the dressing and has continually leaked since. At the moment whilst it is leaking and can escape they aren't rushing to put a new drain back in, the problem when it's only a small amount of air is that there isn't a lot of space to put the tube into without catching more cysts.

The left hand side isn't doing anything so they will be clamping that drain at 2am tomorrow all being well and doing an xray at 9am. Fingers crossed the right side won't accumulate / collapse overnight if the air can escape out at the moment. That way at least we will be able to see what is happening in the morning.

I can see myself being on edge all night worrying about that darn right hand side!!

On another note.. I have had contact, through one of the LCH groups, with a lady in the US whose daughter had the exact same rare presentation as Dylan and her outcome was very positive! I have passed her doctor's details on to the team here so hopefully they can discuss Dylan..

Lots of ups and downs, but that is to be expected. The next few months are not going to be smooth sailing at all and we may be in for a LONG stay here but fingers crossed there is a light at the end of the tunnel, no matter how long it is...