Hello, I'm Dr. Sarah Chalmers, a pulmonologist at Mayo Clinic.
In this video we'll cover the basics of cystic fibrosis, what is it, who gets it, the symptoms,
diagnosis, and treatment. And whether you're looking for answers for yourself
or someone you love, we're here to give you the best information available.
Cystic fibrosis is a disorder that damages your lungs, digestive tract,
and other organs. It's an inherited disease caused by a defective gene that could be passed
from generation to generation. Cystic fibrosis affects the cells that produce mucus, sweat,
and digestive juices. These secreted fluids are normally thin and slippery, but in people with CF
they're thick and sticky. Instead of acting as lubricants, these secretions plug up the tubes,
ducts, and airways in your body. Although there is no cure for cystic fibrosis, people with this
condition are generally able to live normal lives. There are many tools and techniques doctors use
to help manage this complicated condition, and with improvement in screening and treatments,
life expectancy for those with cystic fibrosis is better than ever before.
Simply put, cystic fibrosis is a gene defect. A defect to this gene changes how salt moves in
and out of cells resulting in thick, sticky mucus in the respiratory, digestive, and reproductive
systems. It's an inherited condition. A child needs to inherit one copy of the mutated gene
from each parent to develop cystic fibrosis. If they only inherit one copy from one parent,
they won't develop it. However, they will be a carrier of that mutated gene
so they could pass it along to their own children in the future.
Because CF is an inherited disorder, family history determines your risk. Although it
can occur in all races, cystic fibrosis is most common in white people of north European ancestry.
There are two kinds of symptoms associated with cystic fibrosis. The first are respiratory
symptoms. Thick, sticky mucus can clog the tubes that carry air in and out of your lungs. This
can trigger a persistent cough that produces thick mucus, wheezing, exercise intolerance,
repeated lung infections and inflamed nasal passages or a stuffy nose or recurrent sinusitis.
The second type of symptoms are digestive. That same thick mucus that can clog your airways can
also block tubes that carry enzymes from your pancreas to your small intestines.
This can result in foul smelling or greasy stools, poor weight gain and growth, intestinal blockage,
or chronic and severe constipation, which may include frequent straining
while trying to pass stool. If you or your child show symptoms of cystic fibrosis,
or if someone in your family has CF talk with your doctor about testing for the disease.
Since this disease is an inherited condition
reviewing your family history is important genetic testing may be
done to see if you carry the mutated gene that triggers cystic fibrosis
a sweat test may also be conducted cf causes higher than normal levels of salt
in your sweat doctors will examine the levels of salt in your sweat to confirm a diagnosis
because this condition is passed from parent to children newborn screening is routinely done in
every state in the u.s early diagnosis of cf means that treatments can begin immediately
unfortunately there is no cure for cystic fibrosis but proper treatment can ease your symptoms
reduce complications and improve your quality of life doctors may decide that certain
medications are necessary these could include antibiotics to treat and prevent lung infections
anti-inflammatory to lessen the swelling in your airways or mucous thinning drugs to help expel
mucus and improve lung function medications can also help improve digestive function
from stool softeners to enzymes to acid-reducing drugs some medications can even target the gene
defect that causes cystic fibrosis aiding the faulty proteins to improve lung function and
reduce salt in your sweat outside of medications airway clearance techniques also called chest
physical therapy can relieve mucous obstruction and help to reduce infection and inflammation
in the airways these techniques loosen the thick mucus in the lungs making it easier to cough up
in some cases doctors turn to surgery to help alleviate conditions that can arise from cystic
fibrosis for instance nasal and sinus surgeries to help you breathe or bottle surgery to help
improve digestive function in life-threatening instances lung transplant and liver transplant
have been performed managing cystic fibrosis can be very complex so consider getting treatment at
a center with medical professionals trained in the disorder to evaluate and treat your
condition you can even ask your physician about clinical trials new treatments interventions and
tests are constantly under development to help prevent detect and treat this disease
learning you or someone you know has cystic fibrosis can be
incredibly challenging it's okay to feel depressed anxious angry or afraid in time
you'll find ways to cope find support and talk to others who are going through it too
look to your friends and family to help manage stress and reduce anxiety seek professional help
remember physical conditions come with an emotional and mental burden and take the time
to learn about cystic fibrosis it's a complicated severe disorder so don't hesitate to talk to your
medical team about your questions or concerns with the knowledge and treatment available to
doctors today life with cystic fibrosis is better than ever before if you'd like to learn even more
about cystic fibrosis watch our other related videos or visit mayoclinic.org we wish you well