Cystic Fibrosis [CF] is a genetic disease that can be life threatening. It primarily affects the digestive system and the lungs, but can be systemic in nature as well. It develops because of a defective gene that forces mucus to build up in the lungs and other organs in a very thick layer. This mucus is difficult to process and it can begin to clog up airways so that it becomes difficult to breathe.
Statistics on Cystic Fibrosis Life Expectancy
1. The median life expectancy of an individual who is diagnosed with cystic fibrosis is 37.5 years.
2. About 30,000 children and adults in the United States have been diagnosed with this genetic disorder. There are another 40,000 people in the rest of the world who also have a CF diagnosis.
3. The number of Americans who are believed to be carriers of the CF gene: 10 million. That’s 1 in every 31 Americans. Some studies put the number closer to 1 in 20 Americans.
4. CF is most common in Caucasian people, but is found in people of all races and many ethnicities.
5. 75%. That’s the number of cystic fibrosis cases that are diagnosed by the age of 2.
6. CF is usually diagnosed by conducting a sweat test, which measures the amount of salt in a person’s sweat.
7. Statistics from the CF Foundation registry show that patients born in the 1990s will have a longer lifespan then patients born in the 1980s.
8. 1,000. That’s the number of new cystic fibrosis cases that are expected to be diagnosed in the next 12 months.
9. The life expectancy of a child with CF has doubled in the last 30 years.
10. The name, cystic fibrosis of the pancreas, was first applied to the disease in 1938.
11. The birth rate amongst Caucasian population groups of babies with cystic fibrosis: 1 in 3200.
12. Each child born to parents who are both CF carriers has a 1 in 4 chance of having the disease.
13. People who have a close relative with CF are also more likely to carry the CF gene.
14. The ends of the toes and fingers may become rounded and enlarged because of CF, a condition that’s called clubbing.
15. Statistics now show that nearly 40% of the people living with CF in the United States are 18 years or older.
16. CF usually manifests within the first year of life, although it may develop later and not be detected until the teen years.
17. When adequate care levels are provided to patients with CF, their median life span can reach into the early 40s.
Causes and Symptoms
The thicker mucus is also a better home for bacterial that like to invade the human body. These bacteria can help to cause airway infections that promote swelling and inflammation, which makes it even harder to breathe. These infections can also lead to scarring within the lungs and other organs, which means the end result typically is a failure of the affected organ.
When the pancreas is affected, then the mucus blocks the release of many digestive enzymes that are required for proper digestion. The body then struggles to digest the food and absorb the needed nutrients, which means that CF can cause malnutrition to occur and even poor growth outcomes. To get cystic fibrosis, a child must receive a copy of the defective gene from both parents. There are also 1,800 known mutations of the CF gene, so it is impossible to screen out for all mutations.
The one issue that there is with cystic fibrosis and life expectancy is that there really isn’t any way to gauge this information in a general way. CF can come in mild forms to the extent that people with it lead rather productive, fulfilling, and long lives. It can also be so severe that every breath is difficult and the prognosis is not good. Here are the statistics that have been confirmed, however, for your consideration.
Prevention and Treatment
Cystic fibrosis used to be a death sentence. When it was first discovered and classified, it was very common for children to die in the first year of life. Now average life spans are nearing 40 years of age. Milder forms of the disease are seeing people hit 50 and even 60. That’s great news for the children who are born with CF today. The aging process that is being seen means CF patients can pursue a career, get married, or have their own families if they want.
As with many other genetic disorders, there is a good chance that there will not be a cure coming for it any time soon. Treatment options for CF generally include mucus thinners and digestive aids to make sure there is enough airflow and nutrition entering the body. Beyond that, preventing any long-term damage becomes the #1 priority of a treatment team. For the average person with CF, however, the presence of this genetic disorder significantly shortens the lifespan of those who have it.
What does the future of CF look like? In 2012, a drug named ivacaftor was approved to treat people with a very specific mutation of the CF gene. It actually works to fix the problem of the mutation’s underlying causes. The successes that were initially seen helped to have this drug approved for 8 more variations of cystic fibrosis in 2014. Will it be a cure for CF? That is not known as of yet, but researchers have never been as excited about a treatment option as they have been for ivacaftor.
Fighting cystic fibrosis is a team effort. Patients, families, caregivers, and treatment teams are working with researchers every day in the quest to find a meaningful treatment for this disease, but the journey hasn’t been completed yet. Share statistics like these, support your local CF foundation, and even consider joining clinical trials if appropriate to increase the chances of a cure for this genetic disorder being found in our lifetime.