Baby Stoma & Cystic Fibrosis

[TheUmpire]

My DD is 6 weeks old today. She has been diagnosed with CF, and had an operation to remove a blockage and now has a (temporary) stoma.

Questions....
(1) We were given some stoma bags from the hospital that were quite large, so asked the local stoma nurse to find us some more appropriate-sized ones, which she has. We have trouble keeping them stuck down, though. This is possibly because we are not adequately drying the area. I know some use a hair drier. I mentioned this to the stoma nurse, who said she wouldn't use it on a 6-week-old due to their skin. I'd nonetheless be interested to know exactly how do use use the drier? What setting do you have it on? How far away do you hold it? How long do you use it for? etc

(2) A lot of people are bigging up drugs. Fair play, I'm glad they are around, but I'm also keen to know what else will help natural food-wise - i.e. liver oil or broccoli or something along those (non-drug) lines?

(3) They say CF kids shouldn't mix. But if a kid has a bug of some sort which my DD could catch, what difference would it make if it comes from a kid with or without CF?

Re: 2 I would talk to the dietician about this when you see them. Cf. is quite an innovative field and there is a lot of interest in genuine discoveries so between your team there should be knowledge of whether specific things in diet night be helpful. Is there a particular reason you are interested in the things you name?

Re: 3, people with CF tend to develop unusual bacteria in their lungs, that are suppressed in non-CF people naturally but have potential to colonise and affect other CF people.

1. Stoma care - it’s a pain in the arse and nothing we did got things perfect. We did use a hair dryer on the cool setting and light blast for our DS. He seemed to like it. In fact as a boy we could er… tell he liked the sensory experience! We’d then put it onto a warm setting and use that to warm the stoma bag and soften it up. You can stick it in your armpit or I used to put in my bra but this was faster. Just make sure it isn’t too hot before you apply it. We used cavilon sticks to create a barrier around the stomas and then used brava elastic tape around the bag for extra stick. Have attached photos of these products but everyone is different and it is essentially trial and error. Also when you are trying different bags think about the direction it will fill in and how it sits with the nappy. NICU will often stick them on sideways which works well for them with their incubators but can mean you’re not able to use much of the bag capacity. Not sure if you have other lines but that can be worth just thinking about too, I was so paranoid about the Hickman line getting contaminated. I don’t know if you are recycling any of the stoma output but that might be a factor as well. If you’re making your own holes though you can try a few different variations even with the same bag is my point really. Also if the bag came off and we weren’t busy sometimes we just decided that was bath time, rather than bath time being at 6:30 or whatever. Let him have a bit of bag off time and a splash. No products needed.

1. You’re right, diet is really important and you should have a dietician as part of the team. Kids with CF lose salt though sweat and also with the stoma she will lose salt though there too and salt is needed to grow. They are possibly monitoring this with urine tests. Most babies are told not to have salt but she might need some extra - your team should advise. Calories are important. I don’t know if your DD is pancreatic sufficient or not. As she has had meconium ileus I’d imagine it’s quite likely she’s pancreatic insufficient. Many people with CF aren’t able to produce the normal pancreatic enzymes you would make to digest fats so they have to be given them when they eat any fat containing foods (Creon). Even with this absorption of fat isn’t always perfect. Many people with CF require a lot more calories than those without. They’ll be keeping a beady eye on her growth and advising you. Previously the advice was always eat whatever junk you like, now it’s much more targeted. People are living longer and so the long terms effects of this are actually becoming important. Also the new drugs are meaning some patients get some pancreatic function back and having better absorption than ever before. In older patients obesity has started to be a problem when previously it was always weight gain struggles. So for this one - speak to your own team but essentially healthy balanced diet, eating the rainbow etc is still important.

The other non drug thing which is super important (although not at 5 weeks old) is exercise. It’s so important for the lungs. Google Josh Llewelyn-Jones, Sophie Grace Holmes and Ben Mudge. All people with CF now in their 30s who have massively embraced exercise. Also for interest the guy who invented those Trunki suitcases you always see in the airports has CF.

1. The type of bugs you are trying to avoid when not mixing with people with CF are those that “colonise”. They like to live in warm moist wet spaces. In people without CF they get cleared away easily as their mucus isn’t so sticky and they don’t really cause problems for people without CF. They can set up a bit of a cosy home in the mucus of people with CF and then flare up from time to time and they are hard to clear if you have CF because of the sticky mucus problem. Years ago CF charities used to arrange camps/weeks away and kids used to come back with all new bugs they’d caught from each other.

She may still catch regular bugs from regular kids. You won’t be able to completely avoid this as she gets older. People with CF aren’t usually immunocompromised, their immune systems generally work fine, the concern is that the irritation from infections might cause scarring, and because of the sticky mucus when they get a viral cold they’re then more likely to get a bacterial infection afterwards. Our team always do a cough swab when DS gets any infection, and if he has a wet cough for more than a couple of days they just give him a 2 week course of antibiotics to try and prevent secondary infection and inflammation and we double up on the physio. I found it so hard when he first got any colds but now he’s in nursery we’re a lot more used to it.

Many thanks for your input, Iesugrist. "Is there a particular reason you are interested in the things you name?" No, just trying to soak up some knowledge.

PeigiSu: Thanks again. "I don’t know if your DD is pancreatic sufficient or not" Me neither! Scientific stuff was never my strong point - it will take me several readings and a dictionary to fully digest your comments lol. I will look into it. I can tell you that we have yet to be told what type of (the 100's of types of) CF she has. Regarding exercise: We had hoped she would grow up to be some form of professional sportsperson, so in some ways we are lucky that exercise is a must. I will research those people you suggest, but also note multiple ironman winner and World Championship medallist Lisa Bentley, who is also a CF sufferer. She has a memoir on the market (which I have just finished). www.lisabentley.com/

Google cepecia in cf. Drug resistant and it means they are permanently banned from the luxury cf rooms and have to bunk on general resp med ward if they catch it

Also ask the stoma nurse about the donut things that can he attached to stoma to get it to stick.