What is your experience with cystic fibrosis?

Question

Our daughter who is a month old was diagnosed at 12 days. We are looking for personal stories and experiences from others to help us learn. It doesn't have to be you...maybe a relative, child, friend, or spouse. Thank you!

Answer 1

I am sorry to hear this.
I have no experience in this. Never heard a child with it.

Found this :
Cystic fibrosis (CF), also called mucoviscidosis, is a hereditary disease that mainly affects the lungs and digestive system, causing progressive disability and early death. Formerly known as cystic fibrosis of the pancreas, this entity has increasingly been labeled simply "cystic fibrosis".[1] Life expectancy is on average 37.5 years old.

Difficulty breathing and insufficient enzyme production in the pancreas are the most common symptoms. Thick mucous production as well as a low immune system results in frequent lung infections, which are treated, though not always cured, by oral and intravenous antibiotics and other medications. A multitude of other symptoms, including sinus infections, poor growth, diarrhea, and potential infertility (mostly in males, due to the condition Congenital bilateral absence of the vas Deferens) result from the effects of CF on other parts of the body. Often, symptoms of CF appear in infancy and childhood; these include meconium ileus, failure to thrive, and recurrent lung infections.

Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases. In the United States, 1 in 3900 children is born with CF.[2] It is most common among Europeans and Ashkenazi Jews; one in twenty-two people of European descent carry one gene for CF, making it the most common genetic disease among such people.

Individuals with cystic fibrosis can be diagnosed prior to birth by genetic testing or in early childhood by a sweat test. Newborn screening tests are increasingly common and effective. There is no cure for CF, and most individuals with cystic fibrosis die young—many in their 20s and 30s from lung failure although with many new treatments being introduced the life expectancy of a person with CF is increasing. Ultimately, lung transplantation is often necessary as CF worsens.


Check these sites:

Answer 2

My father is nearly 70 after years of lung and health problems has recently been diagnosed with CF. He has another lung condition caused by complications from having his tonsils out at 4. It took doctors 70 years to diagnose him, (though there weren't test when he was younger) he has been in out of hospital with pneumonia and other complications all his life, and because of his other lung condition has had lung resections on both lungs in childhood and now only has 50% of each lung. He only has a mild case of CF and with medication and keeping active and fit is still going strong and working outside (by choice) at 69. What I'm saying is it really doesn't matter if she has mild CF or not as long as you treat her symptoms and keep her fit and healthy, it shouldn't be an excuse not do something she should have long and for filling life.

Answer 3

I am a nurse who has taken care of cystic fibrosis patients over many years. I know the outlook for them is ever so much brighter than it was. The survival rate has improved so over the years. God Bless you and your beautiful child! How did they diagnose her, by the sweat test? Mothers often notice when they kiss their CF children they taste more salty than their other children.

Answer 4

One of my co-workers has it. She lives a normal life. I say she enjoys most things that we wouldn't though. She loves opera and ballet. She only has one lung, but that doesn't keep her from doing much. Last year she had to be hospitalized for about 3 weeks. But she's better than ever. I think she is a strong woman that tries to enjoy life. I don't ask her much about it, these are just things I have observed.
God bless you and your daughter! Keep a strong outlook on life and encourage your child to develop the same.

Answer 5

Honey, put your question in Health.
Search cystic fibrosis.
I'm a nurse. I know this, but not from personal experience. Find a support group. I'm sure they're online.
Good luck to you all.

Answer 6

my niece has it--e-mail me if you like

Answer 7

Hi, I'm a 40 year old CF'er, having been born June 3, 1967 at 12:37 am.

I had several complications, due to the CF, at birth and had two surgeries within the first few days of my life. Obviously don't remember it, but still have a large scar on my abdomen from it. I continued to have various issues but was fortunate enough to have a pediatrician that knew about CF and he had basically diagnosed me long before the official diagnosis just after I was a year old.

I've known I have CF since I was a toddler. I have a few memories of mom and dad having conversations with me about CF. Of course they didn't tell me everything at that point - no need for me to know it. Aside from the twice daily CPT (chest percussive therapy), nebulizer treatments and taking enzymes when I ate my childhood was pretty normal. My social life wasn't as active as other kids since mom/dad tried to keep me out of situations where I would be more exposed to colds, the flu, etc. (other than school). I continued through school and graduated high school on time and with honors.

I've worked full-time since graduating high school, and had worked part-time during school. In November I'll celebrate my 18th anniversary with my current employer. I'm fortunate in that my bosses and jobs have allowed me to work offsite during hospitalizations so I haven't had to take time without pay and my sick leave has been maxed out for several years.

My husband and I just celebrated the 10th anniversary of our first date and will celebrate our 10th wedding anniversary in October. He's always been very supportive, loving and my best friend. We have five feline children and some fish. My CF doctors told me in my mid-teens that I likely couldn't have children and shouldn't have them. By that time I had, of course, already found out the full extent of CF so having children wasn't in my plan anyway. Relationships were somewhat of a challenge until I met my husband. Once you tell people you have CF they tend to want to over protect and shelter you, and if they are strongly family oriented then knowing that kids are not in the future can be difficult for them. Yes, there is the option of adoption and there are some CF'ers that have had kids. Given the general life expectancy, however, and having some friends whose parents had already died, I didn't want to cause a child to experience it.

In my early 20's the doctors found my liver was starting to harden and enlarge, as a result of my CF. I don't drink alcohol so there is not an issue of any damage from that. I get ultrasounds on it every couple of years and take meds for it. At around age 27 I was diagnosed with diabetes, again the result of the CF. The type we get is called CFRD (Cystic Fibrosis Related Diabetes) and it carries characteristics of both type 1 and type 2 diabetes. I've been on insulin since around age 30 and an insulin pump for just over three years now.

Around age 30 I had a couple of spontaneous lung collapses and finally had to have pleurodisis performed to resolve the problem. My CF doctors started talking to me about lung transplant. At that time, and with the information available, it wasn't something I was interested in so I waited another three years before going for transplant evaluation. At that point I still wasn't sure if I wanted the transplant but lung allocations in those days were based on waiting time in and not necesssarily the sickest person needing transplant. I figured better to do it and once I got 'to the top' of the list I could decline if I felt it still wasn't the right option for me.

Am I ever glad that I did proceed with the evalution! I did reach 'the top' of the list just over two years later but I wasn't sick enough yet to need it so I went on an inactive status, which allowed me to retain my waiting time. The fall of through spring of 2004 was the worst time of my life for my health (aside from right after birth). I developed a lung infection in October and in spite of virtually continuous IV and oral antibiotics I my health continued to deteriorate while the damage to my lungs was rapidly mounting. We had moved the previous spring and unfortunately the CF docs that were providing my care during this time frame had basically written off that I was going to be able to further delay my transplant. The transplant program in their area was newer than where I was listed and they were strongly encouraging me to change my listing assignment (basically so they could have me on their stats). I did consider it since my listing center was a few hundred miles away and it would mean relocating for the transplant. I had concerns, of course, due to the care I had already received at that hospital and their stats indicated they were having some challenges with transplant survival rates. They acknowledged the lower survival rates and indicated they had done an overhaul of their transplant program over the prior to two years so there wasn't enough time or transplants performed to have much impact to their stats. The deciding influence was my insurance company calling me and telling me that if I changed my listing they would treat the entire procedure as 'out of network', even though the transplant doctors and that hospital were otherwise 'in network'. That sealed the decision.

We went back to St. Louis the spring of 2004 for re-evaluation since it had been five years prior when I was originally listed. The evaluation wasn't the same tests all over again, but more spot checks to make sure nothing had developed that would prevent or impair transplant. We returned home to make arrangements for relocation, time off work, etc.

August 1, 2004 we relocated to St. Louis. For a few days before going to St. Louis and while we were traveling there I had a 'premonition' that my transplant was going to be on a Thursday. I finally told my husband, figuring it was something we could be amused about later one way or the other. I started going to the hospital daily for pulmonary rehab (exercise) and monitoring. I needed to complete a couple more tests and work with doctors on how to address a certain medication allergy. My relisting came of Thursday, August 19th. My premonition went away for a couple of days and then was back. I also had a strong sense that I needed to finish a major project for work. I worked all day/night the following Tuesday and Wednesday - only taking breaks for rehab, showering and eating - I seemed to have an abundance of energy. I continued working on my project until finishing it about 12:15 AM Thursday morning. I did talk to my dad for a few minutes on Wednesday evening and told him about my 'Thursday premonition', including jokingingly telling him that if my transplant call came on a Thursday I was going to call him and simply say '...it's Thursday." My husband finished up a couple of things for his job and the phone rang...at 12:37 AM (the same time as my birth) Thursday, August 26, 2004. It was the transplant center indicating they had a donor and I was to report to the hospital immediately. We called our various parents, grabbed a few things and headed for my new lungs.

The actual surgery started at 5:15 AM and they finished approximately 2:15 that afternoon. I woke up at some point later that day and I could immediately tell that I could breath...so that's what all the fuss about breathing is all about! :) Friday morning they removed the ventilator and I took my first post transplant walk - with four chest tubes, umpteen IV's and other tubes attached to me, but I walked around the ICU going just over 500 ft. Later that day they moved me to a regular room - bypassing the usual step down unit. I walked more Saturday and Sunday - not allowed to go more than 1500 feet at a time. Monday I was back on the treadmill - still with a batch of IV's and chest tubes attached - but they let me walk for 22 minutes at a low speed. I continued to rapidly improve and was released from the hospital that Friday. We returned to the hotel for my 3 month transplant follow-up and daily visits to the hospital for monitoring and pulmonary rehab.

After the three months, during which I had no problems, we moved to Pennsylvania, where our company's world HQ is located. We're both doing well and will celebrate my 'third birthday' in a couple of weeks.

I have written to my donor's family but haven't heard back from them. That's not entirely unexpected since some families are happy just knowing their loved one's death came to some good and that their gift is appreciated. The only thing I know of my donor is that he or she died in Tennessee.

Aside from being able to breath - WOW! - a fantastic side benefit is that I'll never have to clean the litter boxes again. There is a certain bacteria that can reside in the boxes that is dangerous for immunosuppressed people. Can't say I'm real disappointed with that! :)

I'll be on immunosuppression medications for the rest of my life and have daily medical monitoring that I do at home. There are also monthly labs, PFT's (pulmonary functions tests) and x-rays to continue monitoring my health. It's all worth it and I would do it again in a heartbeat. The 'advantage' of CF when it comes to transplant is that we are used to taking large numbers of meds and a medical routine long before we reach the stage of transplant.

My advice would be to treat your daughter as a 'normal' child as much as possible. If you would expect her to have good grades then you should hold that expectation. Make her aware of her CF, but not as a 'handicap' - as just part of her. We all have some challenge whether it's visible or inside so CF happens to be hers. Find humor in the CF too - believe me there's plenty of it. If she can laugh about it and have fun with it she'll get through rough times more easily, as will those around her. Tell her the truth about her CF - in stages. You can be assured that she will know the full truth by about age 10 - particularly given access to information on the internet. If you don't tell her then someone else will - and likely someone that doesn't know enough to provide good information. Talk to your CF center about support groups for the kids - she'll want that sense of belonging and understanding. You may want some support as well - I know it can't be easy raising a 'special needs' child. One of the hardest things for my parents in raising me was whether to let me do anything and everything that I wanted to do because I might have a short life span or whether they should treat me as a 'normal' child that should have a full life - school, career, marriage, etc. In some cases they did better than others, but it was always done with love.

You should recommend to other family members that they be tested for CF - or their kids should be tested. It's important for them to know if they are gene carriers as they are doing their own family planning. I'm the only one on my mom's side of the family with the actual disease although a number of my cousins are carriers. On my dad's side there is one other confirmed diagnosis and strong suspicion that a great uncle had it, although his was never diagnosed. Again, there are a number of carriers.

I'm sure you'll manage just fine. It may be a bit overwhelming at times but don't blame yourself or your husband. There's no good to come from it and it's simply not true. CF just is. I don't know why we have it, but many of us have some pretty remarkable experiences.

Best wishes.