1. From the beginning
I thought I’d start today by explaining to new readers and those who don’t know much about life with intestinal failure and waiting for a transplant exactly what our day to day life is like right now. I’ll start by going back to the beginning and bring you up to date with where we are now before outlining a typical day in our life…
William was born in 2004, eleven weeks prematurely, thanks to me. I have a lung condition and wasn't managing to get in enough oxygen for the two of us. He did pretty well for the first month of his life but then ended up going backwards with his oxygen and ventilation requirements. His chest was the main concern. He was nicknamed ‘captain poo’ on the neonatal ward because his nappies were constantly dirty but this wasn’t seen as a concern at the time. On hindsight, we all realise that his absorption was never that great. Wills came home on oxygen at three months old. Feeding was a huge issue as soon as we got him free of the naso-gastric tube that he received his breast milk from in the neonatal unit. Wills was always hungry. He would suckle on the breast for ages and then cry for more within five minutes of being put down. It was a huge struggle. I had a five year old and an eight year old who both needed me and poor Wills just couldn’t settle. He was always hungry yet not gaining weight well. When we tried to wean him it became clear that he wasn’t absorbing much. We called him a baby food factory as whatever we fed him came out looking the same, just a bit more mushed up. At the same time, the oxygen we hoped he would wean from was being turned up and up. Eventually he was admitted to The Royal Brompton Hospital for treatment and tests. He was diagnosed with ‘probable cystic fibrosis’ due to the state of his lungs and his malabsorption, although gene tests never confirmed it.
We spent many long weeks and months in The Brompton and were eventually sat down and told we had to be ‘realistically optimistic’ about his future because he was struggling so much with constant lung infections and poor weight gain. He needed so many intravenous antibiotics that they decided to put in a port-a-cath, a catheter in a vein in his chest, to prevent constant needles and enable us to learn to do his IV antibiotics at home. During an admission a young registrar noticed he coughed a lot when he was eating. She told me she thought he had reflux. I was distraught! Having settled with an unconfirmed, but working ‘best fit’ diagnosis the last thing I wanted was some ‘upshot reg’ questing things and throwing all my jigsaw pieces up in the air again. Later, I thanked this reg for saving the life of my son! She ordered a series of tests, including a pH study to see how much acid William was refluxing from his stomach and some imaging to look at his bowel function and swallow. The results of these showed that he had severe reflux - even on reflux medications, had a bowel that lacked peristalsis to squeeze food along, and even back flowed when he was lying down and an unsafe swallow. The conclusion was that William’s lung condition and constant infection was being caused by stomach and even bowel contents being refluxed into his lungs. We had an emergency operation called a Nissen Fundoplication to prevent reflux and to insert a feeding tube to feed him until he was old enough to learn to swallow properly. He as fifteen months old at the time.
After surgery on the stomach and bowel, you need to wait for the drainage from the stomach to reduce before you can start feeding again. Well, we waited and waited… and the drainage never slowed…until we all realised that this was not just post operative, it was a symptom of William’s underlying condition. William came off oxygen within days of his surgery but we had uncovered the main problem - he wasn’t absorbing food and fluid in his bowel. William was placed on TPN, total parental nutrition, an intravenous feed containing all the fluid, electrolytes and nutrition he needed, formulated individually, based on his blood tests (I’ll come back to this on my next post). We were transferred to Chelsea and Westminster Hospital to the intestinal failure unit. It was November 2005 by then and I’ll always remember a ward round where I asked if we’d be home for Christmas. The constant sat on my bed and explained that this was intestinal failure, organ failure, and we needed to accept that this was long term. Christmas 2005 was the first of several Christmasses in hospital. It was around this time we were told that the only ‘cure’ would be an intestinal transplant.
The following February, we trained to do William’s TPN and got to take him home. We had a good year but William’s condition deteriorated and we spent more and more time in hospital with infections caused by his leaking bowel and dehydration caused by the fluid he lost. In all, we spent 80% of the four years of his life in hospital, separated from his sisters. By 2008, William was so unwell that a small bowel transplant was the only option. He was listed for transplant in August. We got the call in November. It was a very dramatic evening. William was on the operating table for an emergency procedure to fix a perforated when we received the news that we had a donor. I told the story of William’s early years and recovery from transplant in a previous blog which you can still read if you are interested.
We are forever grateful to William’s wonderful donor and her brave family who saved his life that night on November 20th 2008. There have been some setbacks and some tough times but, on the whole, Wills has had a fantastic six years with his new bowel. On 31st December last year I posted on Facebook that this had been the first year we had not had an emergency hospital admission with William. On January 1st this year, Wills woke up poorly. We spent four months in hospital and ended up coming home on TPN with Wills, once again, in intestinal failure. Once again, we hoped his bowel would start to work again but things have slowly deteriorated. In August, he was diagnosed with chronic rejection and assessed for transplant again, this time for a liver, small bowel, possibly colon (if it fits) and a pancreas that will sit alongside his own for plumbing purposes.
And that’s William’s story…in a very very small nutshell! I may well unpack bits of it in future posts but at least this gives the overview for anyone who has been wondering.
2. A day in the life of us
A lot of this blog has been about my thoughts and feelings and not so much on the practical side of life waiting for William’s re-transplant so I thought I’d share a typical ‘day in the life of us;’
William wakes - since he has been unwell at the start of the year, William’s sleep patterns have gone somewhat to pot. He wakes very early but at least now he is 10 he can occupy himself with his iPad. He is really into action films at the moment and has generally watched at least one before I come down to get him ready for school.
I get up and start getting Wills sorted for school - the usual dressed, washed, teeth etc as well as medicines, packing his TPN and pumps into a rucksack, making sure he has a Hickman line emergency kit, gastric drainage bag and gastrostomy extension tube, spare stoma bags, stoma drain bags and everything else he needs for school in his medical ruck sack and checking the nurse/home communication folder.
William’s nurse arrives to take him to school. We handover and the taxi arrives at 8.00.
These days, I mediate for half an hour after William has gone to school. Although I am still a novice and struggle to completely empty my mind, I am finding this to be invaluable in keeping my mind relaxed and focused on the things that really matter as much as possible for the day ahead.
8.30am - 4.00pm:
Wills is at school with his nurse (apart from Tuesday when he finishes at 12 so his TPN can come down early for his weekly blood tests).
I try and get some writing and work done but there is so often management to do like ordering TPN supplies, drugs, stoma supplies, liaising with the agency about his nurse package and other similar things. I play the role of a nurse, physio and ward manager for William at home.
At school William will need his TPN pumps and line monitoring, his stoma bag emptying and putting on the drain in lessons, his gastrostomy putting on drain when he is feeling sick, bloated or in pain and medications given for pain and nausea as required. He has a one-to-one nurse to attend to all of this while he learns. I’ve been told he is getting increasingly tired in school and isn’t playing with his friends much these days, preferring to sit quietly at lunch and break times.
William gets home. He is generally shattered these days. We flush his TPN off and do as much together as we can, although he is usually ready to just flop on the sofa with his iPad and sometimes just goes to bed.
Tea time. Wills is allowed 4 small chips and 1 fish finger which I will watch pouring out, unabsorbed, into his stoma bag a few hours later. He enjoys it though and is longing to be able to eat properly again.
I also cook the family meal at this time and catch up with Ellie.
7.00pm: Physio exercies, bath on bath nights and Hickman Line dressing change once a week
TPN goes up. This is a complex process of connecting him to his intravenous bags of feed, TPN and giving his intravenous anti-reflux medications. Everything has to be done in sterile conditions and it needs a lot of concentration. Mistakes can be fatal. I need to make sure I don’t get any air into him and, most importantly, that I don’t touch anything I am connecting to him to avoid infections. Infections in the Hickman Line he is fed through causes blood poising and septic shock and can kill. This is a very scary thing to do for your child and you never get 100% used to it. I wouldn’t want to get 100% used to it as I need to be aware of the dangers and give it my full attention. I will post a video of the process at some point.
William always asks me when I think the transplant will happen while we do this, if he is awake. More often that not these days he has fallen asleep before I do this.
William’s bed time, again the usual undressed, teeth etc and also more medicines, attaching his overnight stoma drain bag, checking the TPN lines, making sure his monitors are on (I now have special monitors that transmit sounds of him calling or his pumps alarming to a vibrating pager and pad under my pillow)
Time with Ellie, down time and of course more writing
In the night:
Even though he has a two litre overnight bag on his stoma bag, because William’s bowel is secreting so much fluid and gas at the moment we will usually get a bag leak or a bag blown up with gas that needs attention at some point in the night.
3. The greatest gift
I’ll finish today with something very quick and short but so true for me.
I read a quote today;
“About the time one learns to make the most of life, most of it is gone.” (Anon)
I often think that the biggest gift William has given to me is to show me this before most of my life was gone. When William was born, I had a great job, we had more money but my hours were long and I was working at home when I was here. I was working to live and there wasn’t much time to live when I wasn't working! I had to give all of that up and, sure we struggle financially, but the life we have is so so much more than it was. Then, when William had his first transplant I decided that I would spend my life doing what I was supposed to be doing. I would write and I would make it work. I realised life is far to short to go and do anything else. William taught me how to make the most of life and I know he teaches others the same thing too. We talk all the time about how organ donors give the gift of life, and they do but do you know what - all life is a gift and we need to learn to treasure every moment of it.