Hello everyone. Im Harriet, and I was diagnosed with CF when I was 11 months old. Of course I have spent lots of time in hospital throughout the years since then, but early in the summer of 2013, I began a hospital admission which kicked off a steady, but drastic decline in my health and condition, which resulted in me being placed on a ventilator and spending 80 days in intensive care. This was as a result of a severe fungal sepsis which caused respiratory failure. I had never struggled to gain or maintain weight, and early in 2013 I was on the 90th centile for my weight, but then I started suffering the effects of CF Related Diabetes. I began to lose weight, and unfortunately the issues with wildly fluctuating blood glucose levels werent addressed and treated properly. Consistent high blood glucose probably helped the fungal bug (Exophiala dermititidis), which had caused me a lot of problems for a few years, to thrive. It was also probably encouraged by the fact that I was treated excessively with bacterial IV antibiotics, speculatively, despite nothing other than the fungal bug ever being cultured (I was on 17 weeks of bacterial IV antibiotics, on this basis, from January to August 2013). So, on September 4th 2013 I was rushed to intensive care and ventilated. I was transferred to a paediatric intensive care unit at a hospital over a hundred miles from home. I was at this hospital for five months. I lost more weight in intensive care and the effects of being so ill and muscle wastage left me unable to even sit up, let alone walk. During my time in ICU I suffered two major setbacks in the form of two pneumothoraces (holes in my lungs), one on each lung, the first caused a total collapse of my right lung...it was the worst pain Ive ever experienced! Eventually I built enough strength back up to be able to walk again, and over time I weaned of the support of the ventilator. After this I was transferred up to the adolescent ward, where I spent another 70 days. The week after leaving intensive care, my lung function was 19%, and the highest its been since is 30%. When I got home in February I continued to try to push forward with my rehab, and slowly built my strength and cardio fitness up. This helped me to get to the point where I started spending periods of time off my oxygen each day. Three and a half months from leaving hospital went by before I needed another course of IV antibiotics, due to my CRPs (infection markers) being only slightly raised. However, one element that continued to hold back my progress was my nutrition. No matter how hard I tried I couldnt put any additional weight on, but only managed to maintain a steady weight. I spent a lot of time and emotion on trying to up my nutritional intake, but experienced a lot of nausea and vomiting. A few months ago, the pain that I had been experiencing in my abdomen and back got much worse and more frequent. My Mom and Dad kept asking for me to be referred to a gastric specialist, and eventually this referral was made. Still, months went by, and my pain and nutritional status got worse whilst waiting for a gastric review. I started having persistent issues with low potassium levels and started losing weight, which in combination, made it impossible for me to continue with my rehab exercise regime, so I started losing fitness and my lung function started dropping (it dropped to 18%), and I started getting out of breath with the smallest amounts of exertion. Because of my dropping weight and lung function I was admitted into hospital on the 12th of June. I was started on IV antibiotics. The day after admission, I started getting symptoms of gastroenteritis, and it was found a few days later that I had roto virus. The symptoms continued for a couple of weeks and the abdominal and back pain Id had for months got more and more severe. My nausea and vomiting got worse, too. My blood albumin levels started falling and I was retaining a lot of fluid. The worry was that fluid might begin to accumulate around my lungs (pulmonary odema). My albumin reached as low as 20, and the doctors said that if dropped any lower they would use a human albumin infusion to bring the levels up for the short term. I was put on Optiflow (like vapourtherm) to ease my breathlessness and in an attempt to wash out some of the excess carbon dioxide in my bloodstream. Over the course of two and a half weeks the pain became more and more severe...it was agony, and the worst day for this was my birthday :( I was being given a cocktail of more and more strong pain killers. I was on IV paracetamol and oral tramadol and morphine, and lidocaine patches on my back, and it was still hardly scratching the surface of the pain. Throughout this time my local consultant was trying every day to get an urgent inpatient transfer to the regional tertiary centre where the specialist gastric team are based. While I was waiting I had xrays ultrasound scans and an MRI scan, and had daily visits from the pain nurse and anaesthetists in an attempt to find something that would reduce the pain. I also saw the Orthopaedic consultant, who confirmed that pain was nothing to do with muscle or bone and was therefore likely to be solely a gut issue. It had previously been asserted by both local respiratory consultants and a transplant consultant from London that my abdominal and back pain was largely contributed to by postural issues, which I always felt was wrong, so it was good to get this opinion from a specialist orthopaedic consultant. On Sunday 29th of June I finally got a bed at, and transfer to, the tertiary hospital. The two hour ambulance transfer was horrible, and I didnt have the best of starts on the ward, but one thing that gave me some instant relief from some of the pain was that they switched the tramadol from oral to intravenous. I was suddenly needing more oxygen and my breathlessness was worse. The following evening we found out that my potassium level was 2.7 and my albumin level had dropped suddenly and drastically to 15. I continued to feel worse with my chest over the next few days, and on the 3rd of July , in the evening, I started coughing up substantial amounts of fresh blood. The respiratory consultant on call came to see me and outreach nurses from HDU and ITU also visited. The ITU consultant in charge was also alerted. Thank God, after a couple of hours the blood had reduced a lot and eventually stopped coming up completely. The following day, Mom and Dad suggested using a few different antibiotics that Mom had been reading some research papers on, and that they thought were worth trying. One of the antibiotics is an antifungal, which has been shown in research studies to work synergistically with an oral antifungal that Ive been on long term. Mom and Dad had been asking our local respiratory consultants for some time to try this combination, to try to eradicate the Exophiala. Thankfully, the consultant at the tertiary centre went for it, because I have felt some big improvements. My breathlessness has got much better, and my oxygen requirement is back down. The results from my blood tests have started showing some good trends too. The most exciting one is the beta d-glucan, which is a marker of the presence of fungal pathogens in the body. In me this has been extremely high, at over 500 for more than a year, the last time it was checked (on Monday) it was 66. This is amazing news for me because anything below 80 is considered normal levels! My CRPs have also come right down to 4. We are praying that the . Exophiala d. wont grow at all on sputum samples, too. Exophiala has caused me so much illness over the last 5 or 6 years, and some have assumed that it wouldnt be possible to eradicate it now. Back to my gut issues. Over the past two weeks Ive had loads more tests and scans and last week I had a capsule endoscopy. I had to swallow a capsule containing a camera, which then transmitted images to a recorder which was attached to a belt my waist. This gave detailed visual information on every part of my digestive tract...nice! ;) I have been on anexclusive diet for the past week, which basically means that I have to exclude all food other than the broken down liquid feed which goes through my NG tube...I cant even drink orange juice, and Ive drunk tonnes of orange juice every day for longer than I can remember! This diet isnt fun, but I have started to see some small steady improvements each day; my potassium levels seem to be stabilising and my albumin and total protein levels are rising (albumin was 20 today), some of my other nutritional markers have been creeping up too. The really frustrating and worrying thing is that I am still losing weight each day, though. I was 34.9 kg yesterday. Previously, the lowest Ive been in the past year, is 36.75kg. We pushed up the amount of continuous feed I had yesterday, which seemed to go well until late last night, when I vomited a lot and my NG tube came back up. So, I had to have another NG tube passed down at 2am (two nurses attempted it but couldnt pass it down, for some reason, a third nurse managed it first time), then I had to have physio, insulin and Itraconazole (antifungal) which has to been timed on an empty stomache. My continuous feed couldnt go back on until 3:45. So, we are playing catch up today, and hoping for a lazier (I mean restful) day.
Posted on: Sat, 19 Jul 2014 12:19:01 +0000
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