#thisisKelly

I’m 40 and have just started on parenteral nutrition (PN) – I am writing this in hospital on my second bag. I’m a wife, a mum to a 9-year-old daughter, and a retired GP. That last one isn’t something I admit to very much, but it’s a huge part of who I am, something I am very proud of and something that I miss hugely.

I have Hypermobile Ehlers Danlos Syndrome and, due to severe hip instability and failed surgery, have been a wheelchair user for over 12 years. My gastrointestinal symptoms started when I was 9 and was admitted to hospital with tummy pain which turned out to be severe constipation. We didn’t realise at that point that it was the start of something that would turn into this.

When I started using a wheelchair, I found it very difficult to eat. If I did eat when in my chair at work all day, my tummy would blow up and eventually I would get horrid tummy pains and sometimes vomit. So, I started drinking shakes during the day to replace meals, and would then eat an evening meal at home while lying down on the sofa, which was the only way I could avoid the symptoms. This was how I went on for a good few years and I didn’t really think much more of it. My weight was stable and, with my doctor’s hat on, I just put it down to dysmotility due to the EDS. I managed my constipation as best I could with laxatives, and got on with it, like we all do.

Then, in September 2020, things changed. I started vomiting regularly, every time I tried to eat anything more than a few mouthfuls. Then I started vomiting even after a large drink. My weight started to drop and I lost 1.5 stone. I was already Coeliac at this point, but my Gastroenterologist didn’t want to look into these new symptoms as he didn’t have the appropriate specialist experience. So, out of desperation, I found a local private Gastroenterologist who did specialise in gastroparesis, which is what I suspected may be going on. He saw me and picked up the phone to my local Intestinal Failure Unit, who saw me in December 2020. At this clinic review, I met my fantastic current consultant, who diagnosed me with Pan GI Dysmotility due to EDS and laid out the expected course of the illness in her experience, plus her concerns about needing to avoid tubes, and certainly PN, for as long as possible.

This was a difficult time for us as a family. It was a heck of a lot to process. My consultant was extremely honest and open with me, which is what I wanted. I think, as a doctor myself, I understood enough to know what this diagnosis meant for the future, and with a child at home who also has EDS and GI issues already, it was a lot to take on board.

Fast forward through 2020 to now. Drugs worked for a while, nasojejunal feeding worked until they could no longer get it through the exit of the stomach into the jejunum due to reverse peristalsis. A transgastric jejunostomy worked for a while, but then became displaced very regularly due to the same reason. During this time, we also tried many, many feeds, but all eventually caused catastrophic diarrhoea, causing dehydration that I could not take enough fluid in to treat. Even on daily St Mark’s solution, and being connected 24 hours, I was getting very dry and losing weight. 

We then tried a surgical jejunostomy as a last-ditch attempt at gaining access, although we knew we were running out of enteral feed options anyway, but I really wanted to avoid PN for as long as possible due to the risks.  

I had the jejunostomy done some weeks ago, and sadly this was the last straw for my bowel. It very quickly became unusable due to severe pain and reverse peristalsis forcing faeces out through the tube. I have spent 7 weeks fighting against PN and have tried eating as much calorie-dense food as I possibly could. But my BMI dropped to 15, and my consultant told me it was time for PN, so here we are.

My Hickman line was put in a few weeks ago now, at the time of writing this I was having my second bag of PN. I can eat and drink for comfort as my transgastric jejunostomy tract was converted to a venting percutaneous endoscopic gastrostomy PEG, for which I am eternally grateful as I really love food!!

I am very aware of the sepsis risks now and have made very proactive decisions about when I want PN to be withdrawn in the event of repeated sepsis episodes. I appreciate this is not for everyone, but as a medic I have seen too many people with repeated episodes of sepsis in very difficult positions, and so I have always been very clear about my wishes regarding this.

Don’t get me wrong though, although I am realistic about the risks, and clear about my wishes regarding ceilings of care, I am feeling extremely positive about starting PN. I already feel much better now that I am appropriately hydrated and have my electrolytes within the normal ranges. I am looking forward to gaining weight and energy and being able to do things with my lovely family that I’ve not been well enough to do for some time now. We already have a holiday to Shetland planned soon which my team here are very kindly working towards making possible for me.

I am also feeling extremely grateful for living in a country where I can access PN and rejoice in how lucky I am that my life can be saved by this treatment. I am being given time to live, that I otherwise wouldn’t have. Without PN, my life expectancy would now be months. But it isn’t. And although, because of my advanced care decisions, I know that my life expectancy may well be limited, I feel grateful for every single day that this treatment will give me to be with my wonderful family and live life.

I find joy in each and every day, despite all of the difficulties that we each face. I appreciate that I am very fortunate in many ways, but we each have a choice in how we deal with our situations, no matter how difficult or dire they may be. We can either feel negative about things, or we can choose to feel positive. We can cry, or we can smile – even if we don’t feel we have anything to smile about. Sometimes forcing ourselves to smile anyway can make us feel much better in that moment.

I don’t mean to oversimplify things, or offend anyone at all. I just want to share how I approach things, because it seems to work well for me. I send love and strength to each and every one of you reading this. Yes, life is difficult, but it is also wonderful in so many ways.