Can You Live a Long Life With Cystic Fibrosis?

Persons with cystic fibrosis can experience lung problems, digestive issues, infertility, fever, night sweats, jaundice, and sinus infections.

Cystic fibrosis (CF) is agenetic diseasein which themucusin thelungsanddigestive systembecomes thicker and stickier than usual. This leads to chronic lung and digestive issues.

People with cystic fibrosis are living longer than ever before. According to the Cystic Fibrosis Foundation Patient Registry, half of people with cystic fibrosis who were born between 2015 and 2019 have a predicted life expectancy of at least 46 years. Some people with cystic fibrosis live into their 60s and 70s. New medications and treatments for cystic fibrosis have increased life expectancy.

As people are living longer with cystic fibrosis, they may experience other conditions, or comorbidities, along with the condition. People who have cystic fibrosis may also experience endocrine disorders such asdiabetes, poor growth and development duringpuberty, bone disease,vitamin D deficiency, and thyroid and adrenal disorders. Newer treatments for cystic fibrosis seek to reverse or slow down some of these cystic fibrosis comorbidities.

Lung problems

People with CF often have a frequentcoughwithphlegm. They get frequent lung infections likepneumoniabecause the sticky mucus traps germs. They may feel short of breath or wheeze on a regular basis.

The lack of oxygen can causeclubbingof the fingers and toes. With clubbing, the ends of the fingers appearbloatedand the fingernails could be deformed. Luckily, once the lack of oxygen is addressed, clubbing often goes away on its own.

Digestive issues

People with CF may have greasy, large bowel movements more frequently than normal. Conversely, they may be prone to intestinal blockages orconstipation. Due to thick mucus in the digestive system, they may not gain weight or have adequatenutritiondespite eating a normal amount of food. This can cause delayed growth and development in children.

Other signs of cystic fibrosis

• Salty tasting skin
• Maleinfertility: up to 98% of men with CF areinfertile
• Fever
• Night sweats
• Jaundice
• Frequentsinus infections

Cystic fibrosis is caused by a genetic mutation that parents pass to their children. There are over 1700 variations of the mutation, which may make it hard to diagnose adults as carriers.

囊性纤维化常染色体隐性遗传, meaning a person has to have two copies of this gene get cystic fibrosis. People who have only one copy of this gene -- carriers -- do not have cystic fibrosis but could pass it on to their children if they have a child with another carrier.

Most people get a diagnosis by age two, due to the progressive nature of the disease. However, some people with a less severe version of cystic fibrosis may not get a diagnosis until their teenage years.

To diagnose CF, doctors will perform the following tests:

Sweat test

People with cystic fibrosis have higher levels of salt in their sweat. So, doctors test sweat to see how much salt is in it. If your sweat has over 60 millimoles per liter of chloride, then you may have cystic fibrosis. However, this alone is not enough, so doctors will perform these other tests listed to make a clear diagnosis.

Genetic testing

Doctors may test both a child and their parents for cystic fibrosis genes. Testing the child can confirm the presence of two CF genes. Testing the parents can confirm if both are a carrier, and may help them with futurefamily planningdecisions.

However, the genetic carrier tests available only test for the most common mutations. So, it's possible for someone to be a carrier but still test negative for the gene.

Doctors usually offer carrier testing topregnantpeople and couples who are thinking of having children. If someone in your family has CF, you are at higher risk of having a child with cystic fibrosis and should get tested.

Sometimes, doctors perform genetic testing for cystic fibrosis on infants while they are still in utero.

Pancreatic testing

积累啊f mucus in the digestive system often prevents the pancreas from releasing importantdigestive enzymesin people with CF. Doctors may test the凳子for the enzyme chymotrypsin to see if a cystic fibrosis diagnosis is likely.

They may also test your凳子for elastase, another digestive enzyme.

Another pancreatic test is a blood test for trypsinogen -- a precursor to a digestive enzyme created by the pancreas.

Cystic fibrosis is a complex disease and each case is different. So, treatment may be different for each person.

There is currently no cure for CF. However, people with it are living longer than previously with advances in treatment. In the 1950s, many children with the disease didn't live to school age. Now, the current average lifespan for people with cystic fibrosis is 44.

Treatments to manage the condition include:

Airway clearance

Keeping the airways clear is an important aspect of CF management. This prevents infections and helps people breathe. People with CF work with specialists to learn particularbreathingandcoughingtechniques.

There are also assistive devices like vibrating vests to help clear mucus. In some cases, parents bang on a child's chest and back to break up mucus.

Infection management

People with CF get frequent lung infections. Doctors may use antibiotics to proactively prevent infections, or use them to treat current infections.

They may also take a sputum culture -- a test in which a personcoughsto produce mucus to test -- to detect exactly which bacteria is causing the most current infection.

Medications

In addition to antibiotics, other medications may help people with CF, including:

• Anti-inflammatory medications
• CTFR modulators (medicines that work on the protein affected by the genetic mutation that causes CF)
• Medicines to thin mucus

Lung transplant

People with severe cases of cystic fibrosis may benefit from a lung transplant.