how long do cystic fibrosis sufferers live to?

Question

one of my friends suffers with it and i just want to know abit more about it.

Answer 1

Cystic fibrosis, or 'mucoviscidosis', is a hereditary disease. A defective gene results in an inability to transport salt in certain kinds of cells in the lungs and in the pancreas. It is the most common hereditary disease in Western Europe.

Why does a person get cystic fibrosis?

Cystic fibrosis is an 'autosomal recessive' disease, which means that both parents have a gene defect that they pass on to their offspring. But the child will have cystic fibosis only if it inherits both of the defective copies of the gene. There is a 1:4 chance of this happening.

In the other three out of four chances the child will inherit a normal copy of the gene and be unaffected by cystic fibrosis. The exact mechanism is not known, but the altered salt transport severely thickens the mucus in the respiratory passages. This makes the respiratory passages more susceptible to bacterial infection, most frequently Staphylococcus and Pseudomonas.

How can I tell if I have cystic fibrosis?

The symptoms are usually noticeable in the first year of life. If cystic fibrosis is suspected, the diagnosis can be confirmed through a special sweat test. The GP can then make a referral to a cystic fibrosis centre at a hospital.

In the lungs, the disease results in repeated cases of pneumonia and breathing difficulties. The pancreas becomes less able to produce digestive juices, which may result in weight loss and greasy diarrhoea.

The disease increases the salt content in the patient's sweat. At high temperatures this may result in the patient losing too much salt and suffering heatstroke.

What can I do?

If somebody is expecting to be a parent, they should check if there have been any cases of cystic fibrosis in their family. If there is, they can talk to their doctor about being referred to a genetic counsellor. The counsellor will be able to assess the chances of the baby having the disease, at which point a decision can be made about continuing the pregnancy.

Someone with cystic fibrosis must avoid unnecessarily straining their lungs, for instance by smoking. Lung exercises with a special mask (PEP) are often useful.

Future prospects

Previously, the prognosis for cystic fibrosis sufferers was very gloomy. But the treatment is getting better and now there is an 80 per cent chance that a child with cystic fibrosis will live for over 40 years.

Genetic treatment holds considerable promise over the next decade but it will be in the future before this becomes any kind of mainstream treatment.

How is cystic fibrosis treated?

The treatment aims to maximise the lung function for as along as possible.


Common treatments include lung physiotherapy as well as antibiotics to minimise the risk of lung infections. Complications within the pancreas can be managed by controlling the patient's diet, and providing supplements of pancreatic enzymes (eg Creon) to add to food.


As a result of cystic fibrosis, some patients may also suffer asthma, which can be alleviated by asthma inhalers.


A lung or a lung and heart transplant may be necessary if the lung function deteriorates very significantly.

Answer 2

Managing CF
At present there's no known cure for CF. Symptoms are treated and eased with drugs, physiotherapy, enzymes and a special diet. People with CF need a daily regime of medication and physiotherapy, as well as regular hospital check-ups.

In 1964 (when the Cystic Fibrosis Trust was formed), the life expectancy of children born with the condition was less than ten years. Today, thanks to worldwide advances in medical research and better understanding of the disease, the average lifespan of a person with CF is around 30 years. A cure for CF is now within sight. The Cystic Fibrosis Trust says: "We are now talking about when rather than if."

Answer 3

Many people with cystic fibrosis die young--in their 20s or 30s--from lung failure. The median life expectancy is 37 years. But with all the new treatments coming out nowadays, life expectancy has increased to as high as 40 or 50.

Answer 4

I'm a CF patient that was officially diagnosed about about age 1 - 1968. I'm now 39 and just over 2 years post bi-lateral lung transplant.

There are varying mutations of the gene that causes CF so some patients have a more progressive form of it than others for lack of better terms. Some of us have more respiratory issues than digestive and vice versa. Early diagnosis and commitment to the prescribed medical routine is also important.

My second cousin also had CF and was many years younger than me but he died when he was about 12 if I remember correctly. I've had a lot of CF friends die over the years, ranging from about age 10 to their mid to late 20's. However, I've also met several CF'ers that weren't diagnosed until adulthood and they were still doing very well. One of the other CF lung transplant recipients that I know was 44 when he received his transplant.

Right now transplant is the only thing that saves us once the damage to our lungs is to the point of killing us. Since we are (typically) used to a medical routine we generally make very good candidates for transplant but, at the same time, there are inherent risk factors for infection afterward because CF affects our entire bodies so sinus cavities, etc. harbor the Pseudomonas bacteria which is very aggressive and difficult to keep under control. However, having a transplant cures the CF in our lungs and essentially gives us a second life.

Current lung transplant survival rates are about 50% at 5 years but I've also met many recipients that are 8-10 years out and still doing very well.

Your friend basically will have the ability to do whatever he/she wants to do within the limits of what their body/breathing can take. They'll have good and bad days and, as the disease progresses, there will be more hospitalizations for "tune-ups" (IV antibiotics and treatment to battle the lung infections). Most of us are rather creative and have a pretty wicked (and sometimes extremely gross) sense of humor. We generally do well in school, assuming regular attendance, other than the phys ed classes and even that isn't beyond us depending upon the stage of the disease.

I graduated college with honors (went to night school full time - 12 to 18 hours a semester - while working both a full time and part time job), I'm a Sr. Mgr. at the world headquarters of a Fortune 500 company where I will celebrate my 17th anniversary on Monday, I've been married for 9 years and generally have a pretty normal life.

I wish the best for your friend and commend you for trying to understand more about the disease. Don't be shy about asking your friend questions - most of us are pretty willing to share.

Answer 5

hi i am glad you are looking for infomation.

my friend died of cystic fibrosis at the age of 13 this was some seventeen years ago and alot has changed since then i have aqantences that are in their late 20s and one who is 47. you have sceen some usefull info on hear. but you might like to read brething for a living by laura rothenberg a diary of cf and the desition to have a lung transplant. a tretment that is somtimes offerd if the lungs become very degraded after years of infection the invention of evermore effective tretments has ment that people with cf can live longer and healther lives. also as you have sceen alot depends on the aderence to care and tretment regimins but not entierley alot can be determined by the particula set of cf genetic mutation a person has some exibit a milder desese due to their mutations others become ill more often. if you want to look at the biology of cf orensteind guide cysitic fibrosis a guide for patient and family is a good one and i think relitavly user frendley. also if you are in the USA you could contact the cystic fibrosis foundation or in uk the cf trust google them and i am sure you will find somthing.

please do not be scared by the stuff you may read i still miss my friend very much even after all this time but i would not be with out the profound influence she continues to have on my life to this day. enjoy time with you frend try not to think about the death to much you may fiend if the subject comes up ask her about it she may walcom someone to talk to about it with. i will say this one last thinng people with cf have to devote much of there free time to treetments. try to allow for this within your friendship visit them in hospital and dont leave them out socially for example when they have to do cpt just orgnise around it be happy enjoy your friend. also part of tne story of laura rothenberg was made into a radio program on nprradiodiaries this can be down loded from there site as an mp3

Answer 6

it varies. The life expectancy is longer than it used to be. It can depend on how well they comply with care and treatment, how well they eat, how well they respond to antibiotics{they often develop lung infections}. They will spend lots of time generally in and out of hospital. They are given treatment to help them breathe easier - nebulisers, physiotherapy, antibiotics and steroids. They are also given enzymes to help digestion of food. If they can't eat at times, they will need tube feeding. They must try to avoid respiratory infections and colds. If their lungs become very damaged, they may need a heart and lung transplant. They can live almost into the 30s now I think but it is improving all the time. Why don't you ask the parents of your friend to explain it a little better or get you a leaflet on it.

Answer 7

You know, there simply is no answer to this question. The median age of survival is now 36.5 years. This is such a hard statistic to interpret. Is just means that half of the people who died from CF died younger than 36.5 and the other half died above age 36.5. It doesn't really tell you what age you can expect to attain.

Also, this applies only to patients who are treated in registered CF centers who report to the CF Foundation. If someone is "healthy" enough to use traditional medical care, they may not only live longer, but their age at death will not be included in the CFF statistics.

I know several people with CF who are in their 50's and even 60's. I know one fellow who is 75!

One thing I DO know is that CF is merciless. Your friend may seem fine on the outside, but this is just because he/she has learned how to deal with it and hide any problems. When it is time to go, there is little warning. A CF patient can decline and die from an apparently healthy state within a matter of just a couple of weeks. Our daughter was "looking good" and ready to be discharged from the hospital when she had a sudden pulmonary bleed that killed her. Live for today.

Join http://www.cystic-l.com to meet MANY adults who are above the median age.

Check out http://www.cff.org/AboutCF/Faqs/ for the answer to this and many other questions.

Answer 8

Because of advances in technology, sufferers are now living a lot longer than they used to. It used to be unusual to live past 20's, 30's max but now people can live into their 50's. It's a nasty disease affecting the lungs and digestive system. Very thick mucous is produced which clogs the lungs and leads to life-threatening lung infections. It also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

Answer 9

usually late 20s... so lets daty 28 - 30....
wikipedia has sme good info on cystic fibrosis...
have a look..

Answer 10

I have had patients die as young as 10yrs and I have also had patients live up to 40 something. A lot of it depends on how well these patients either are cared for or how well they take care of themselves. My thoughts and best wished towards your friend.