A blog about being too bendy. To raise awareness of Ehlers Danlos Syndrome.
Being fed through tubes.
(2006 to present).
Since the start of my illness I’ve struggled with tachycardia after eating certain foods. Anything high in sugar or fat was a problem. In 2006 I started getting tachycardia after every meal, whatever it contained. Digestive problems are common with EDS (ref) (ref), but also common with POTS (ref) (ref). Even after tests doctors were unsure what the cause was. To manage the problem I started eating in small portions rather than whole meals at a time. It was frustrating, but it was the only tolerable way I could eat.
The problem persisted, and in 2010 my symptoms became so bad I could barely eat or drink at all. I ended up in hospital with dehydration. I was transferred to The Royal London Hospital under Professor Qasim Aziz, a gastroenterologist and specialist in the digestive problems associated with connective tissue disease. I was assessed, then had a PEG tube inserted. A PEG tube (Percutaneous Endoscopic Gastrostomy tube) is a device fitted to patients who are unable to eat normally. A tube is inserted into the stomach through the abdominal wall, enabling liquid food to be pumped in. The surgeon reported that my stomach was very large, tortuous, and made of soft tissue. This was thought to be a complication of Ehlers Danlos Syndrome and may explain some of my symptoms.
I found PEG feeding very difficult to tolerate. Various treatments were tried including injections of Octreotide, which prevents vasodilation in the gut reducing the drop in blood pressure after eating. I improved, but found I could only manage about 1400 calories a day. To keep myself hydrated I had a Hickman line inserted so I could receive daily IV fluids. A Hickman line is a central venous catheter which provides long term intra-venous access.
When I was finally discharged I’d spent nearly twelve months in hospital. My health was precarious on several occasions and my weight had dropped to eight stone (51 kgs). It was great to be home, but it was a constant struggle to maintain my weight. Blood tests also showed persistent problems with anemia, low platelet count and lack of vitamins, all due to poor nutrition. I managed to stay at home for two years with that treatment regime. My quality of life was very low but it was better than being in hospital.
A 2 litre bag of parenteral nutrition.
During the summer of 2013 my weight fell to 7 stone and 1 pound (45.1 kgs). My body mass index was 13.2, categorized as ‘Very Severely Underweight’. I was admitted to The Royal London Hospital under Dr Michael Glynn, a gastroenterologist and specialist in clinical nutrition, to be transferred from daily IV fluids to parenteral nutrition.
Parenteral Nutrition is the process of feeding a person intravenously. It bypasses the digestive system putting nutrients straight into the bloodstream. It provides the body with everything it needs, but risks infection, blood clots and liver damage. It’s generally a treatment of last resort, but I was so thin that a change had to be made.
One of the first things Dr Glynn’s team said to me was ‘We have a lot of EDS patients’, so I knew I was in the right place. I was in hospital just over two weeks and the transition went very smoothly. I receive 1300 calories per day into my bloodstream and 700 calories through PEG feeding, mostly just to stop me feeling hungry. I’ve been receiving parenteral nutrition for over a year now and gained over three stone (20 kgs). My blood tests are almost normal after several years of problems. I also look much better and feel better too. A number of doctors advised me against parenteral nutrition, but so far its effect on my body has been fantastic and it’s clearly been the right decision (so far!).
In contrast to my experiences with POTS and EDS, I think the healthcare I’ve received for my nutritional problems has been extremely good. Parenteral nutrition emerged as the long term solution and its effect on my body has been profound. I miss food everyday, but I’m aware I’m living past the point where my body can sustain itself by normal means. A PEG tube and a Hickman line are relatively small surgical interventions, but if they hadn’t been made, if nature had been left to run its course, I’d have died in 2010. Things aren’t easy for me but I’m very grateful to still be around.
Currently I’m very limited by POTS symptoms and old joint injuries which haven’t healed. My POTS symptoms would improve with exercise, but I can barely exercise due to the joint problems. It’s frustrating, however I’m getting very good nutrition now and I’m stronger than I was a year ago. Progress has been slow, but hopefully I’ll keep improving.
Thank you for reading my blog. – I’d like to finish by saying a very big thank you to Professor Christopher Mathias and his team, Professor Qasim Aziz, Professor Rodney Grahame, Dr Alan Hakim, Dr Simon Moodie, Dr Michael Glynn and his nutrition team, the numerous GPs, nurses, carers and dietitians that have looked after me, and my friend and physiotherapist Mr Andrew Trott. Thank you all so much for somehow managing to keep my body going!
