Cystic Fibrosis

[kbochniak]

Any other mom's out there with a CF kid? My son is 8 weeks old, he was diagnosed at 3 weeks of age through the newborn screening. Just looking for support from others who are going through the same thing.
Thanks,
Kristy

[askew]

Welcome to MCD! While I don't know what it is like to have a child with CF, I do know what it is like to be told there is something wrong with your newborn. I know there is at least one mom here with a CF kiddo, I am sure she will be by soon.

[SiobhanAoife]

Hi there - I am in the middle of toddler bed time, I will come back and post more as soon as I can, but I wanted to say hello and let you know that there are a couple other CF moms out here. My daughter was diagnosed at 5 weeks old based on newborn screening. She's a healthy, hearty, smart 16 month old now. Don't despair, this is hard hard hard to handle hearing, but it is not the end of the world. Also check out the message forums at forums.cysticfibrosis.com. Huge hugs to you, I'll come back and post more later.

[Pookietooth]

to you mama my cousin has it.

[SiobhanAoife]

Hello again.

My heart goes out to you, I remember all too well the phone call from the newborn screening results. It's a terrible thing to be told especially just postpartum when you're full of hormones and your baby is so tiny and seems so defenseless. One thing that I have found is that the horribleness of finding out fades over time, so at least you have that to look forward to.

So, tell us more about your baby! Is he sick? It's extremely common that kids who are caught by newborn screening aren't sick at all yet, which can make it even more confusing. Are they putting him on enzymes with every feeding? Are they pressuring you to use formula? Is he gaining weight OK? Have you done the sweat test yet? Fecal elastase test? Did they tell you which mutations he has? Are you doing CPT with him? Are you being seen at a certified CF Center? That's very important, btw.

Your son can grow up strong and happy and healthy and smart, with CF. It takes work, sometimes a lot of work, but it's doable.

Has anyone told you about the two really really exciting new drugs in the pipeline for CF, VX-770 and VX-809? If they prove out in testing, they may make it possible to manage CF with two pills a day instead of the much lengthier and more complex regimens that are the current standard. There is a lot of hope and a lot of positive, constructive things to do to keep your son healthy and thriving. Come back and tell us more about where you're at -- I know that when I had only known for 5 weeks I was deep in grief and fear still.

Huge hugs.

[kbochniak]

Thanks for the replies. It was hard to hear the news especially because I am a nurse and I have seen some really sick kids with CF. My son has the double DF508 mutation - I am excited to see the research that is being done on the new medications! Currently he is only on the enzymes and Zantac. The enzymes seem like a miracle - He was born at 9 pounds even (which is such a blessing considering) and then lost down to 7lbs 14 oz. We tried and tried to put weight on him, they kept telling me that I wasn't making enough milk for him and that I needed to supplement. I eventually did supplement a little before we knew about the CF, he was eating about 30 oz a day and I definitely wasn't making that much milk. Once he got on the enzymes though, he started eating less and gaining weight. At this point I was able to stop supplementing and he has been exclusively breast-feeding for the last 4 weeks. We are seeing a pulmonologist at the CF center (thank goodness we have one here in Charlotte) and he has also been referred to a pediatric gastroenterologist. I was very happy with the GI doc when he said to continue doing what we are doing and that he really believes in breastfeeding as long as possible. Currently my son is 8 weeks old and weighs 10lbs 2oz - he has gained almost 2 pounds in the last four weeks (since starting the enzymes). He is currently very healthy, no respiratory involvement so far. I resigned my nursing position to stay home with him to keep him out of daycare. This was a tough choice but we had to make the decision that was in the best interest of my son - we are fortunate that my husband has a good job and we can afford to live on one income, although it will be a little tighter than we are used to. We have not started the chest PT yet, the doctor wants him to be able to stabilize his body and head a little more before we start but I'm assuming that we will probably start that after our next visit to the CF center. Thanks again for your replies, it's nice to hear from someone who is going through the same things. BTW - is there anyone else out there who is questioning vaccines for a special needs child? If he didn't have any problems we probably would not vax at all but I'm considering doing selective vaccines because of the possible respiratory complications of his condition.
Thanks again,
Kristy

[pixiekisses]

Our little miss cotton ball button has CF as one of the many diagnosis on her lungs. And she gets a lot of the treatment. We do the chest PT, and we have a vest (he has to older to use that I think, since it shakes you pretty good). Trampolines are really great for pulmonary drainage as well, for when he gets older. (We have a little one inside.)
She's on a lot of meds for breathing, and is o2 dependant, but her lungs are really, really bad, and it's not just CF, so I wont go there.
She started the enzymes pretty late, because we didn't get the diagnosis until she was over a year old. And just like a year after she started getting them she had to have a g-tube placed anyway, but again, not the CF. She has a bunch of other gut problems.
The CF center has been great though, glad you have one where you live.
It's not fun finding out that your baby is sick. s

[2boyzmama]

Originally Posted by kbochniak

BTW - is there anyone else out there who is questioning vaccines for a special needs child? If he didn't have any problems we probably would not vax at all but I'm considering doing selective vaccines because of the possible respiratory complications of his condition.
Thanks again,
Kristy

Yes, and it's hard!!

My middle son has multiple issues, but the most pertinent to vaccines is his immune deficiency (t-cell deficit), an airway malformation (laryngo/tracheo/broncho malacia), and palate and swallowing issues that cause him to aspirate (not often anymore, but he's still high risk).

I WANT to vaccinate him against pertussis, flu, and pneumococcal. I did get him Synagis (RSV immunoglobulin) his first winter.

BUT, there's strong family history of reactions to pertussis vax, so that one's out (I'm not happy with the effectiveness of it anyway). And his immune deficiency means he likely wouldn't mount much of an immune response to a vax anyway. The flu shot is such a guessing game, and has so many funky ingredients, I'm just not ready to give it to him.

SO...he is still not vaccinated. But I am very much more nervous about it with him than my older or younger son. And I hate being nervous, usually I would read that nervousness as my mommy gut telling me to change my decision. I literally talk myself around the same circle over and over every time I re-evaluate vaccines for him.

[SiobhanAoife]

Originally Posted by kbochniak

BTW - is there anyone else out there who is questioning vaccines for a special needs child? If he didn't have any problems we probably would not vax at all but I'm considering doing selective vaccines because of the possible respiratory complications of his condition.

Yes. We did selective vaccinations (with vaxes split to more visits than the standard schedule) specifically because there are some things that, given CF, she really really really needs to not get. Are you doing Synagis this season? If it's still RSV season where you are, you should ask about it, though if he is an only child and you're staying home with him, you can take lots of precautions. We did Synagis last year, but not this year since she's doing fine and has no lung problems. RSV in an infant with CF is to be avoided if at all possible.

I'm really really glad to hear that you've been able to keep breastfeeding; there are a lot of folks who get irretrievably derailed to formula during the diagnosis process, and everything I read, plus all the folks at our CF center, agree that breastfeeding for as long as you can is the best path for kids with CF, especially given both the nutritional needs and vulnerability to infections they have. My daughter's still going strong at 16 months, which is a blessing because she's entered the toddler-picky-about-food era and it's a struggle keeping her weight up.

Since you're a nurse I obviously don't have to tell you about the need for constant handwashing, but I will say that I lament how hard it is to make everyone else around you and the baby follow through with it!

I applaud you for making the decision to stay home with your son. It's extremely difficult with a brand new CF kid to get everything they need taken care of, plus protecting them from kids with respiratory germs. If he's doing great with no lung involvement and strong overall health when he's bigger, then playgroups and daycare sorts of settings may make sense, but not when he's so small and you don't yet know how his health is going to play out in the next year.

How are you handling all of this? It's a huge deal. Are you getting enough support? How is your husband taking it?

[pixiekisses]

And we do selective vax. We were supposed to get synagis the year she was 1, but then she got RSV. And boy, she was sick. Had to get help breathing and lots of o2. The next year she was sick from fall to winter, so she never got a chance to get the synagis then either. And she got RSV again (yeah, you aren't supposed to be able to get it more than once), and again she was intubated and was really, really sick, with pneumonia on top.
She has serious immune system failure though. And again, that's not the CF. Of course, their more prone to airway trouble. She actually got RSV her third winter too, and again was really sick, she had pneumonia on top that time too.
So, I'd concider the Synagis for sure.
It's hard about the vaccines with little miss cotton ball button, because of her lousy/non-existent immune system, she's sick a lot from fall to spring.
But I'd concider them. Is her doc at the CF center objective, or very vax happy? 'Coz if he/she can be objective and not push them, I'd discuss it there.

[sbrinton]

Our 15 month old son, Zephan, is being tested for cf. He's not growing well despite a very hearty appetite and he's had cold after cold with thick, goopy mucus. I am hoping the answer is not CF, but I'm learning what I can to prepare. We'll find out on Tuesday.

[SiobhanAoife]

Originally Posted by sbrinton

Our 15 month old son, Zephan, is being tested for cf. He's not growing well despite a very hearty appetite and he's had cold after cold with thick, goopy mucus. I am hoping the answer is not CF, but I'm learning what I can to prepare. We'll find out on Tuesday.

Good luck! Regardless of what the cause is, getting an accurate diagnosis will help you help him be healthy and grow well. What testing are you getting - sweat test? Other tests? Are you going to a certified CF center? I ask because sweat tests are not reliable if they're done in non-specialized settings.

[SiobhanAoife]

Originally Posted by sbrinton

Our 15 month old son, Zephan, is being tested for cf. He's not growing well despite a very hearty appetite and he's had cold after cold with thick, goopy mucus. I am hoping the answer is not CF, but I'm learning what I can to prepare. We'll find out on Tuesday.

oh, also, this sounds strange to ask but, what is his poop like?

[sbrinton]

We are going to a specialized center. The children's hospital in our city is one of the top places to treat CF in the states. If it is CF, I think we're in a good place to deal with it.

His poop is poop? I've been trying to pay more attention to what it is like. It seems to really vary depending on what he eats. I don't really know what to look for. It does seem like he doens't digest certain foods well.

I wouldn't be shocked if it was just a milk allergy causing problems with digestion and overproduction of mucus. I'm hoping we have some answers this week anyway.

The other thing I've noticed is that he does taste salty. I feel strange licking my baby, but I've noticed even just a salty taste after kissing his little head. Different than kissing our other kids.

It's hard to wait on testing like this. We have so many other things going on so I'm trying to just keep going on not dwell on it, but it's hard. I'm worried about our little man.

[SiobhanAoife]

The reason that I asked about poop is that the classic CF malabsorbtion issue is a failure to digest fats, with the result that stool is oily and stenchful. Note that this can be subtler or not present in some CF cases, but it's standard in 'normal' CF, if you will. Doesn't sound like it's an issue for your kiddo, which is good! One test they do at our CF center is the fecal elastase test; this confirms that the child has adequate levels of pancreatic digestive enzymes getting where they need to go. If weight gain is one of the concerns that leads you to test to rule out CF, I'd hope that they'd include this test. It's easy, you just need to give them a stool sample.

The salty skin, well thats what the sweat test will look at.

[Pookietooth]

Check out the Specific Carbohydrate Diet for later when he's on solids, and also the low oxalate diet. Might also look into a prescription probiotic for that time called VSL 3, supposed to be excellent for bowel health. My cousin gets bowel obstructions on a regular basis, partly because he had a severe one early on (maybe as a toddler) that left a lot of scar tissue.