cystic fibrosis?

Answer 1

You've already seen the standard medical aspects/statistics of Cystic Fibrosis but you haven't received answers from someone that has firsthand experience of life with CF...untill now.

I'm age 39 and have had a basically normal life. I was diagnosed with CF at about one year old and have been in treatment for it my entire life. At age 37 I received the miracle of a bi-lateral (double) lung transplant because the multitude of lung infections and progression of the disease over the years had destroyed my lungs. With the lung transplant I'm now cured of CF from a respiratory aspect, although I do still have the digestive and diabetic aspects of the disease. The last year prior to the transplant was pretty bad - spent months in the hospital and at home getting constant IV antibiotics, was on oxygen 24/7, coughing constantly, etc. My energy and breathing capabilities were so low that I was unable to express emotions - no laughing, no crying. I had to walk so slowly that we referred to it as "slothing" rather than walking. I'm not asking for sympathy, just providing a picture of how it was. Although it was bleak for a while there I made it through each day because I refused to give in to it and had the hope of the transplant. As soon as I woke up from the surgery I could breathe freely -- for the first time in years! It was a very successful transplant and I was out of the hospital in one week...and working from my recovery location 3 days later.

I've been married for almost nine years. I had no problems graduating from high school on time and with honors as well as working since age 16. I'm currently employed full-time (50+ hours/week) for a Fortune 500 company as a Sr. Project Manager and will celebrate my 17th year with the company in November. I graduated from college with honors, earning a BS college degree while working full time and also working a second part-time job. I moved out from home at age 18. I basically have had as normal a life as anyone else.

Not everyone with CF is infertile, although infertility is very common with us, but CF females are encouraged to not have children due to the complications it can cause for both the mother and the child.

Hospitalizations and doctor's offices are frequent. I've found that most of us (CF'ers) have a pretty wicked sense of humor and it certainly helps deal with it (there's a huge amount of humor in the medical field) - it's one of the best medicines available and doesn't require insurance/co-pay! Physical activity can be a challenge depending upon the progression of the disease so we develop other characteristics/talents. Many of us do well in school, learn quickly and are very creative.

While many CF patients do die at a young age I've also met several people over the years that weren't diagnosed with it until well into adult hood. CF is increasingly well known and recognized in the medical field by family doctors, etc. so diagnosis is generally made much earlier than it was a couple of decades ago. There have been several significant discoveries for treatment and some excellent drugs developed that help reduce damage to the lungs. With the increased commonality of lung transplants (they've been doing them since the mid-80's) there's also greater hope for a longer life span although there is the ever present shortage of donor organs.

The CF gene is recessive with over 600 common mutations of the gene and many more that don't occur as frequently. In order for a child to have CF both parents must be gene carriers - although due to the recessiveness there is no guarantee that two parents/carriers will automatically have children with CF. Some families have multiple children with CF, others have some with and some without, and some have no children with CF (although any of the children that don't have CF can be carriers themselves). CF has different levels of impact to the respiratory and digestive systems depending on which mutation occurs within the patient - it also affects the severity of the disease early on and the expected life span.

One of the biggest parts of having a "normal" life with CF is the attitude of the parents, the patient, and those around the patient. If they play the "pity party" then the disease seems to progress more quickly. If they take the attitude that basically everyone has something to deal with and this is what this person has to deal with then it's a slower progression. I remember my parents telling me I had CF when I was 3. While they obviously didn't reveal the full extent of things, they were very clear that I was a little different than other kids because of the CF so I had to do twice daily chest physiotheraphy ("CPT") and nebulizer treatments (we later named the nebulizer "Darth Vader" due to the mask children wear when doing the treatments). They also indicated that I might not be able to do everything that other kids could do but that I shouldn't feel bad because they loved me regardless. I knew the full extent of CF by age 10, including the life expectancy parameters. In the long run you can't beat CF, but what the CF'er gets out of life with it is really up to themself. The doctors are going to have their say and while the patient certainly needs to heed their advice, in the long run it's up to each of us to decide how much life we're going to have outside of being a medical case.

There are a number of support groups for CF patients and their families, which you can find on the web through a standard search. I've included a couple of them below, as well as the link to the national CF Foundation:

CFF has a lot of helpful information and links: http://www.cff.org/home/.

Some of the support group links are:
http://www.cysticfibrosis.com/
http://www.reachingoutfoundation.org/...

Best wishes.

Answer 2

As with many of these diseases, there is a Cystic Fibrosis Society which you can find via a search engine.It affects, as the second respondent said, the lungs. However, there has been a huge amount of study into the disease and I helped sponsor a young lady golfer who raised a large amount of money(her name was Katy and she was awarded an M.B.E or O.B.E-can't remember which-for her efforts) and she, despite having to undergo heavy massage to clear the mucus, lived a pretty normal life, complete with boyfriend etc, so whilst not good news, it's not all bad either.

Answer 3

cystic fibrosis(CF) is a genetic disease it is often considered to be a respiratory disorder but it also involves the digestive tract and pancreas The disease is characterised by anomaly thick sticky mucus that clogs the lungs and digestive tracked. It is autonomic recessive disease meaning that four an individual to exhibit the condition they need two copies of the cystic fibrosis gean one from each of their parents if both have the gean then there is a 1 in four chance that the child will inherit the two copies and have CF there are several different mutations I think the current figure is in its hundreds of the gean that course the disease meaning the symptoms can range from mild to severe. It is detected by the use of a swet test as people with cf have extremely salty swet it is characterised by coughing recreant respiratory infection large frequent bowel movements that are loose and sometimes intestinal obstruction and poor weight and growth

The defective gene means that in the person with cf salt and water move from the individuals body cells incorrectly this means that mucus that is present in all individuals for lubricating and cleaning different organs of the body becomes thicker. This means that in the case of the longs it is much harder to cough up this means that it lays in the lungs and attracts bacteria a person with CF is much more prone to long infections as a result, meaning the longs become increasingly damaged over time. It is treated by a mixture of physiotherapy which includes laying them on a special bed or over a pillow and loosening the mucus using cpt ‘clapping’ on the chest and back this is normally carried out 2 to 3 times daily depending on the requirements of the individual at the time parents are given advice and training by a physiotherapist. In resent years there have also been devices invented to help in the elimination of secretions from the lungs one is called the Vest it looks something like a life vest and vibrates to remove secretions the other is called a flutter looking something like a kazoo it is blown into and helps loosen the mucus. the condition is treated by the use of antibiotics (iv),oral and nebulised either given at home or in the hospital as a planned addition to treat infection in the lungs preventing damage for as long as possible and the incaution of pancreatic enzymes in the diet taken before food in order to aid its digestion as most people with the condition are pancreatic insufficient that is they do not produce enzymes of there own or if they do they are not adiquate for the digestive process to be effective. there are however, a small number of people who have CF and who are pancreatic sufficient and do not require enzyme treatments this is unusual. a person with cf must pay particular attention to there nutrition as their ability to absorb is impaired (malabsorbtion) taking in enough nutrients and calories to cope with the demands of the condition allowing them to grow and remain ralitavly well if this can not be achieved by the eating alone high calorie supplement drinks and even tube feeding in addition to normal intake is an option.

treatment for people with cystic fibrosis is improving all the time it is still a life limiting condation but people with CF are now living longer and longer with the development of incresingly effective tretment and and the option of long transplantation when other tretments are exorsted. years ago the desese was one assocated with childhood (and a shortend one at that) now many go on to live into there 30 and even 40s i know somone in their mid 40s they can live productive and forfilling lives
transplantation.

david oresteins book cystic fibrosis a guid for patient and family is exalent and it walks you through the condation and will aid your understanding of the desese

also the cystic fibrosis foundation if you are in the USA or the cystic fibrosis trust if you are in uk are good sorces of help

better info than i have provided hear

you may also like to read breathing for a living by laura rothenberg or CF rules ok by pat chutney ot inner strenth by cloyce jones for personal acounts

Hope this helps if it is about a friend or family member a health professional would give you good advice and would not mind talking to you

Answer 4

is a lung disease which produces very thick debilitating mucous. Both parents must carry the gene..now the only cure is a lung transplant.

Answer 5

its a bone disease that makes bones brittle and crumble and wears away some of the muscle

Answer 6

No

Answer 7

No thanks, I'll pass.