River was born 5 weeks early whist we were on holiday camping in Cornwall with our son who was 11, my dad and our two dogs. I wasn’t concerned about going 5 weeks before my due date as it had been the perfect text book pregnancy with no issues. We didn’t quite make it to the hospital, I gave birth at the side of the road in my husband’s company car. From there River and I were taken to Truro Hospital.
She was suffering from jaundice so was kept in the SCBU. She wasn’t keeping down any of her milk and so far, hadn’t pooed. By the second day the doctors where concerned about her lack of bowel movement so River was x-rayed. It showed that she had an obstruction in her bowel. That night she was transferred from Cornwall to Bristol Children’s hospital as they were the closest hospital who were equipped to carry out surgery if need be.
Following further scans and x-rays the consultant told me that the type of blockage was very consistent with Cystic Fibrosis sufferers.
Cystic Fibrosis is a genetic condition which primarily effects the lungs and bowel. Patients create excess mucus which can clog the lungs and cause permeant damage. Because of this they are prone to chest infections. The build-up of mucus in the lungs causes chronic infections, meaning that people with cystic fibrosis struggle with reduced lung function and have to spend hours doing physiotherapy and taking nebulised treatments each day. Exacerbations (a sudden worsening of health, often owing to infection) can lead to frequent hospitalisation for weeks at a time.
My husband had gone back to our home in Northamptonshire to arrange care for our son so was told the news over the telephone. He left home to drive back to Bristol immediately.
On the Friday evening the surgeons decided to operate to remove the obstruction. They said the intestine was quite damaged and inflamed so they removed 20cms of the bowel and gave River a stoma to give it time to recover. We named her stoma Phyliss.
Over the next week she made amazing progress and recovered well, tolerating her milk and putting on weight. She had a sweat test to confirm the Cystic Fibrosis diagnosis which was positive. All babies in the UK are screened for Cystic Fibrosis via the heel prick test, so has she not of had a blockage the condition would still have been picked up.
Because we were still so far from home Bristol arranged for River to be closer to home so she was transfer to John Radcliffe Children’s hospital in Oxford.
When we arrived at Oxford we were met by the surgeon and the Cystic Fibrosis nurse. River was also pancreatic insufficient and therefore cannot absorb the fat from her food, this means that she does not gain weight easily. They started her on Creon Micro with her milk which replaces the enzymes she’s missing and aids in the fat absorption. Because of this River’s bowel movements can be very loose and fatty, in the early days her nappies were very prone to a poosplotion, which would then result in a full change clothes and a bath! The CF team also started her on vitamins and showed us how to do River’s percussion physio to assist her in clearing her lungs.
After 5 weeks in Oxford the surgeons reversed River’s stoma and two weeks later we could take her home.
Since leaving hospital we have been very fortunate that River has been quite well and has not needed intravenous antibiotics, but the process of keeping her well is quite vigorous.
River does her chest physio therapy twice a day for 30 minutes when she is well and 3 times is she is unwell. This involves a lot of physical exercise, breathing exercises and percussion (patting her lung area to loosen the mucus).
She takes her Creon tablets every time she eats or drinks something containing fat. At the moment, she takes around 20 a day, but as she gets older and consumes more this will increase. With her Creon she takes vitamin A&D capsules and vitamin E solution as these are fat soluble vitamins so she doesn’t absorb them naturally from her food. She has Dnase (helps loosen the mucus) once daily via a nebulizer and antibiotics to help prevent infections.
As she doesn’t effectively absorb fat River is on a high fat, high calorie diet to which we also add salt as her condition causes her to sweat more than other children, her skin is very salty.
We attend hospital visits every 8 weeks to monitor her health and once a year we go back to Oxford for her annual review where they x-ray her lungs, ultrasound her organs and take bloods.
River started school this September and despite everything she goes through and scars on her body she is an amazingly happy little girl who embraces all the opportunities given to her!