Hi! New here!

GG1995

New member
Hello Everybody!

I am new here. My daughter is almost 6 months old and was diagnosed at birth with CF. She had emergency surgery the day after she was born because she had meconium ileus. She was on breastmilk at first that we were having to fortify with formula. Now she is on a highly concentrated recipe of strait formula. We feed her every 3 hours. She is on Creon and Source CF Vitamins. The CF center that we visit did bloodwork last month and her Vitamin D level was low, so we have now added a seperate Vitiamin D drop to her regimine. We also do CPT on her for 20 minutes two different times a day.They also told us that her liver function is abnormal and want us to see a GI specialist because of it. Just looking at her, you would think that there is nothing wrong with her............she looks as healthy as any other beautiful baby girl! :)

I have a question. When she was dianosed, they told us that she had DD508. If have been lurking on this site for a couple of weeks, and I have noticed that some people say that DD508 is one of the worst combinations that a CFer can have. Can someone explain why? We were told that eventually this would affect her lung function..............and we understand about the pancreas and the enzymes and the mucus. I guess I am just wondering why some people this this diagnosis is worse that some.

Thanks for reading! I look forward to getting to know you all. :)
 

Ratatosk

Administrator
Staff member
We have a similar story. DS diagnosed at birth due to MI, surgery. Extra calorie formula, enzymes, adeks vitamins with additional a & e. Also had abnormal liver function test results and was put on Urisidoil (actigall) to keep his liver and gallbladder happy.

Not sure if it's necessarily the worst combination. With this mutation you pretty much know what type of symptoms to expect -- digestive for sure. With DDF508, there is most likely pancreatic insufficiency. The mucus seems to be extra thick, so it's important to do CPT to keep the lungs happy and healthy despite there being no symptoms. DS is 9 and his baseline is still no cough. Mostly digestive issues, though the extra thick mucus does cause some sinus issues -- his nose doesn't run, so we had to do saline drops and the dreaded booger sucker as a baby so he could eat and sleep better.
 

JennyCoulon

New member
It is a very similar story to what we went thru with our son who just turned 13 last week. It is crazy to believe it has been that long but reading your story makes it feel like it was just yesterday. Mason had abnormal liver function also and they put him on Ursodial which helped it b/c it was sluggish. He was on that for maybe a year or two and he hasn't had issues since then. Mason and my 7 year old son Tucker who also has CF are DDF508. My oldest has never cultured pseudomonas and has never grown any other bacteria such as MRSA, etc. My 7 year old cultured Pseudo about 2 years ago and he did 2 weeks IV antibotics at home and hasn't cultured it since. ***Knock on wood***. I am not sure if it is specific to the mutation or not but both of my children are very healthy lung wise and are mostly affected in their GI tract. They both have G-buttons and take enzymes. They do Vest 2 times a day for 30 mins. as well has albuterol nebulized and pulmozyme. I haven't heard that about that mutation but I have heard it is one of the most common ones. Please feel free to let me know if you have any question that I can possibly help you with. Good luck....
 
Hi and welcome. Mutations have very little to do with outcomes. i saw a site that someone posted up here that had stats based off of your mutation combo. It was pretty interesting. I think what is most important is early treatment and a qualified CF center. Sounds like you are two for two there.

Good luck and if i may offer a friendly word of advice, be careful what you read on the internet. Even on this site.
 

GG1995

New member
Thank you Ratatosk for replying and telling me your story.
Is your son still on the liver medicine?
 

GG1995

New member
It is a very similar story to what we went thru with our son who just turned 13 last week. It is crazy to believe it has been that long but reading your story makes it feel like it was just yesterday. Mason had abnormal liver function also and they put him on Ursodial which helped it b/c it was sluggish. He was on that for maybe a year or two and he hasn't had issues since then. Mason and my 7 year old son Tucker who also has CF are DDF508. My oldest has never cultured pseudomonas and has never grown any other bacteria such as MRSA, etc. My 7 year old cultured Pseudo about 2 years ago and he did 2 weeks IV antibotics at home and hasn't cultured it since. ***Knock on wood***. I am not sure if it is specific to the mutation or not but both of my children are very healthy lung wise and are mostly affected in their GI tract. They both have G-buttons and take enzymes. They do Vest 2 times a day for 30 mins. as well has albuterol nebulized and pulmozyme. I haven't heard that about that mutation but I have heard it is one of the most common ones. Please feel free to let me know if you have any question that I can possibly help you with. Good luck....


Hi Jenny!

My husband and I also have a son that will be 3 in December. He does not have CF. Thank you for sharing your story with me. It really helps now that I have found a support group full of people that know what our life is now like. I hope my daughter will stay healthy like your boys have. We do everything humanly possible to keep her surroundings and her toys clean and to keep her well. She is teething so everything goes strait to her mouth right now. LOL
 

GG1995

New member
Hi and welcome. Mutations have very little to do with outcomes. i saw a site that someone posted up here that had stats based off of your mutation combo. It was pretty interesting. I think what is most important is early treatment and a qualified CF center. Sounds like you are two for two there.

Good luck and if i may offer a friendly word of advice, be careful what you read on the internet. Even on this site.


Thank you!
It is funny that you said "be careful what you read." When she was diagnosed, the CF Center that we go to told that first thing. They said that alot of the information out there is out dated, incorrect and it would scare the crap out of you! LOL
 

Ratatosk

Administrator
Staff member
Thank you Ratatosk for replying and telling me your story.
Is your son still on the liver medicine?

Yes he is. Originally it was a suspension -- I referred to it as cherry flavored vomit. Now it's pills 3 times a day. Liver levels have been fine. I think they thought his issues were caused by TPN -- nutritional feeding he received in the NICU. Each year he gets bloodwork and they check liver levels among other things. And it supposedly helps with the gallbladder. Which can also become an issue when they're older.
 

GG1995

New member
Yes he is. Originally it was a suspension -- I referred to it as cherry flavored vomit. Now it's pills 3 times a day. Liver levels have been fine. I think they thought his issues were caused by TPN -- nutritional feeding he received in the NICU. Each year he gets bloodwork and they check liver levels among other things. And it supposedly helps with the gallbladder. Which can also become an issue when they're older.

I wonder if that could be her problem too. She was on TPN and Lipids while she was in the NICU recovering from her surgery due to the MI. How long was he on the TPN when he was in the NICU? She was on it for about 2 1/2 to 3 weeks. She was born on May 10, she had surgery on May 11 and we were released from the hospital to come home on June 5.
 

JennyCoulon

New member
One thing to keep in mind is to still let them be a normal child. My kids still went/go outside to build snowmen, go sledding, etc in the Winter and they also go out in the Summer when it is hot. I use Clorox wipes and Swifter Wet Jet to mop our floors but don't go crazy trying to make everything sterile. It is important to remember to go to their appointments on a regular basis, do treatments, take meds, etc. but also let them be a kid.
 

Ratatosk

Administrator
Staff member
DS was on TPN for a couple weeks when he was in the NICU. Hopefully you'll get in to the GI specialist soon and get it figured out.
 
my daughter was diagnosed with cf at 3 months and she had a failure to thrive. she was born april 14 2012 she is gaining alot of weight. she has the deltaf508 and rh117
 

GG1995

New member
One thing to keep in mind is to still let them be a normal child. My kids still went/go outside to build snowmen, go sledding, etc in the Winter and they also go out in the Summer when it is hot. I use Clorox wipes and Swifter Wet Jet to mop our floors but don't go crazy trying to make everything sterile. It is important to remember to go to their appointments on a regular basis, do treatments, take meds, etc. but also let them be a kid.

We do.We live in Florida, and even though she is only 5 months old, she loves to go outside. We have about 20 acres of family land and we have a golf cart that seats 4...........we take her out riding on the golf cart about twice a week and she loves it! I have one of those back-pack carriers that you wear on the front of you, and I put her in that and she loves it!
I use Colorox wipes a lot too. I wash all of her toys that I know she'll stick in her mouth because she is teething in hot soapy water, and I still sterilize her bottles and nipples.
We have an indoor cat and we have a 3 year old son, and I know I can't disinfect those two! LOL She loves playing with her older brother. He mankes her laugh.......I LOVE watching that!
 

GG1995

New member
DS was on TPN for a couple weeks when he was in the NICU. Hopefully you'll get in to the GI specialist soon and get it figured out.

The CF clinic ordered an ultrasound of her liver yesterday. We will hopefully get in with the GI specialist around the 2nd week of December.
 

GG1995

New member
my daughter was diagnosed with cf at 3 months and she had a failure to thrive. she was born april 14 2012 she is gaining alot of weight. she has the deltaf508 and rh117

What is your secret to getting her to gain weight? We went to the CF clinic yesterday and she had only gained 1 pound in a month.........she had grown 1 1/4 inches though. Her weight is just not keeping up with her length.
How much did she weigh at birth?
 
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