Cystic Fibrosis

“Has anyone dealt with this disease at all? My newborn nephew has been diagnosed with it. There is info out there, but I'm trying to find out what the "real life" of this disease is. At this point all I can tell is that it sounds like it will be horrible for a mother to watch her child go through. I'd like to know what day to day life will be like, for my nephew, his mother, and for the rest of us.

For example, will my sister be able to keep her job, or will this require all day care? Are the doctors in the emergency room going to get to know my family really well, etc?

As you can tell, I don't know much about this. I may be freaking out a little right now as well, though I'm trying to keep a level head. I know there are drugs in development, but I don't think there's any kind of cure on the horizon.”

“I'm really sorry to hear that, Ductape... I wish I knew something to tell you --”

“I was searching through a few of the main websites on this and it seems that advances have been made receintly to improve the health of children suffering from this disease. I don't know anyone who has it though.

Very sorry to hear about you nephew!”

“Ductape, I'll be sending you an e-mail. My nephew was diagnosed with it when he was 5 and is now 22.

Expect an e-mail from camk24 at some point this evening.”

“I read a book many years ago, still have it, about a girl with CF. I just checked my bookshelf. It's called "Alex, The Life of a Child." It's written by her father, Frank Deford. Published in 1983 so I'm sure that medicine has advanced since then. But it's still a moving story.”

“Ruby...I remember the tv movie about Alex with the same title. Funny you mention it because it was moving enough that through high school, college and beyond, I was very active with the Cystic Fibrosis Foundation.”

“Thanks for the kind words. Mrs. D, I'll keep my eye open.

It appears that people can live for a long time with this, possibly until 40 years old right now. The way we're looking at it is that is 40 years of research ahead of us to come up with better treatments.”

“aw ductape, how heartwrenching. Your family and you nephew are in my thoughts.”

“Hey Ductape, Sorry to hear about your nephew. It really is a horrible disease. I have taken care of a few kids with it in my so far short nursing career. I am sure you have read a bunch already, but this is a progressive disease - - getting worse over time. Your nephew will unfortunately see many doctors in his lifetime. And it honestly depends how the disease affects him as to how normal his life will be and how many times he will be in and out of hospitals. I recently took care of a 23 year old that was being hospitalized for the first time because he discovered what alcohol was... which could have been avoided on his part. Most patients I have seen live into their 30's. The oldest patient I took care of with this disease was 44 when he passed - but that was after a double lung transplant. He lived for almost another three years after the transplant. So like you said, there is hope, new drugs and treatments all the time - take care...”

“I don't have any experience with it other than my son having to be testef for it, so no advice there, but how is your sister and brother in law doing? Yall are in my prayers.”

“Unfortunately, there is no brother in law. Daddy kind of ran away when he found out about being a daddy. But, as far as my parents go, I've never seen two people more happy to be grandparents so there is plenty of support. I think everyone is about as would be expected, kind of not really knowing what to think right now.

There was a phone call from the dr's office with the news this afternoon. Hopefully we will find out some more in the morning after my sister gets a chance to take the baby into the doctor's office.

It turns out there were signs of this that no one recognized until we were looking for them. The baby's been constipated a lot and eats incessantly. Also, he apparently has salty skin as well. The dog really likes to lick the baby, and that particular dog isn't really a licker. One of the symptoms is lack of weight gain, though, and he's not experiencing that. My mom said he was born bigger and is gaining weight faster than any of us 3 kids.

This was really a shock when I heard it. At first, I thought this meant my nephew would be lucky to see his fifth birthday, but it turns out it's not quite that bad. This is really just a nasty disease, though. I'd guess anything affecting breathing is. My wife's grandmother recently died of cancer. Seeing her in hospice was really tough. It's heartbreaking to see someone expend so much energy just on taking a breath.”

“We had to decide whether to test our yet unborn baby for CF. Its scary to think about, and we decided not to test, but with this thread I'm starting to rethink that decision.

I don't know much about the disease, other than its pretty tough to deal with. I'll send good thoughts to your nephew.”

“SG - unless the outcome of that test will effect any descions you make then I'd recommend against the test. We were asked for all 3 of ours about taking various test like that. It wouldn't effect our decision on having the child, so it was a moot point. If you get a bad outcome and you'll still have the child all it will do is stress you out during a joyous time. Just my unsolicited opinion.

duct - in your sis's defense, if this is her first child, she had no way of knowing any of those symptoms were abnormal. With that first kid it's all abnormal. The salt thing is interesting since the CF test is called a sweat test. I alwyas wondered why.

Glad she has the support, she'll need it. All 3 of ours have spent time in Children's Hospital. It's a humbling and also enlightening feeling. Whatever you've been there for you come away feeling blessed because there is always a room full of kids with more serious conditions. I hope that came across correctly.”

“Ductape, my sister sent me a huge e-mail. I'm going to add my experience to it. It will be on its way in about a half hour to you.”

“My sister-in-law has so far lived to the age of 38 with CF. Although it has been a difficult life for her, she has brought a lot of joy and happines into the lives of many people. When I first met her, I believe the average life expectancy was early 20's, however it has increased to over the age of 30 by now. She married, and she and her husband have adopted two children. Their medical bills are astronomical between her CF and the diabetes that has recently developed. She has regular breathing treatments that she takes, and she has to be hospitalized a couple of times a year now for a "tune-up". As for her childhood though, it was relatively normal and happy. She knew nothing else. I am amazed at her spirit in view of what she has gone through.

Cystic fibrosis is an inherited, or genetic, condition.

Cystic fibrosis occurs because of a faulty gene called the cystic fibrosis transmembrane conductance regulator - or CFTR gene. Cystic fibrosis is an autosomal recessive disorder. This means that in order to develop the condition you need to inherit two cystic fibrosis genes, one from your mother and one from your father. If you inherit only one cystic fibrosis gene, you are called a carrier and do not have symptoms.

Good luck and prayers for your sister and her child.”

“I just found this data that seems to be great news.

In 1959, the median age of survival of children with cystic fibrosis was 6 months. In the United States, the life expectancy for infants born in 2006 with CF is 36.8 years, based upon data compiled by the Cystic Fibrosis Foundation”

“Again, thanks everyone.

Hobbit, that is great news. Hopefully that life expectancy keeps going up. I know that diabetes will also be something my nephew faces. It already runs in the family, and the CF attacks(if that's the right word) the pancreas, which is the insulin producer/releaser.

DH, no defense needed for my sis. I hope I didn't sound like this is something that should have been recognized. None of those things really seemed like a big deal. Just a little constipation and a strange dog. It was more of a thing where we realized that he was showing the symptoms, which at this point are minor, once we found out what they were.

I actually just got home from visiting. My sister had a conversation with the doctor. She and the baby will be admitted to the hospital for 3 days. During that time, they will do tests to find out what enzymes, etc to start giving the baby, what kind of respiratory therapies are needed, etc. Also, my sis will be trained on how to take care of someone with CF.”

“Hey ductape, you have mail.”

“My childhood best friend has CF so I am very familar with the disease. She is 27 and was just recently admitted in the hospital for the first time. She has always taken very good care of herself and her parents did the same for her when she was a child. I'm sure the disease can be discouraging for alot of people because of the number of medications that they have to take along with breathing treatments, etc. I think my friend takes somewhere close to 20-30 pills a day and she has always been on top of her medication. I can't even imagine what it is like to take that many pills a day. She was hospitalized because she kept getting sick- flu and colds- so the doctor admitted her to do a lung catheter to clean out her lungs. She went back for her check-up and she tested at 106% on her lung function test. The doctor told her it is the best that he's ever seen her. So it is very possible for someone with CF to live a normal life as long as they take good care of themselves. On the other hand I went to high school with a guy that had CF and he smoked. He passed away at the age of 19.

I hope everything goes well with your nephew and I will keep your family in my prayers.”

“Wow Mrs Opie, that's excellent for your childhood friend!

My nephew was in and out of the hospital doing cleanouts from childhood until present. It ranged from 1-2x's a year. I think he may have had a bad year in there that caused him to go 3x's at one point.

Every child progresses differently. It's nice to see when a CF child gets over that teenage hump.”

“Wow, 27 and admitted to the hospital for the first time? That is definitely encouraging. From what I've been reading, taking care of one's self is absolutely critical. No use in making things even harder on the body.”