Kathleen is a freelance writer who loves researching common diseases and sharing critical information. She has a loved one with CF.
What Is Cystic Fibrosis (CF)?
Cystic fibrosis (CF) is a genetic, chronic disease that impacts the lungs, pancreas, kidneys, and intestines. Although CF is hereditary, about one in every 31 Americans are symptomless carriers of the defective CF gene, which increases the chances of the disease going undiagnosed.
Fibrosis is a term that refers to scarring, and in patients with cystic fibrosis, mucus causes scarring of the lungs. Cystic fibrosis can be easily confused with other fibrotic illnesses such as pulmonary fibrosis and idiopathic pulmonary fibrosis. That is why testing and proper diagnosis are of the utmost importance.
Signs and Symptoms of Cystic Fibrosis
The signs and symptoms of cystic fibrosis vary greatly. Some people with CF present symptoms as a child, others don't notice anything until adulthood. Some people with cystic fibrosis suffer a mild case throughout their life, while others with CF have symptoms that continually worsen.
Although cystic fibrosis does manifest in many ways, it is important to know what to look for if you are at risk, or think you may have CF.
Here is a list of signs and symptoms associated with cystic fibrosis:
- Salty skin, due to high salt content in the sweat
- Producing excessive phlegm, which can irritate the lungs
- Chronic bronchitis, sinusitis, pneumonia, and other lung infections
- Difficulty breathing
- Frequent nasal polyps
- Greasy, dark stool
- Sleep apnea due to congestion and blocked airways
If you suspect that you may have cystic fibrosis, seek medical attention from a doctor in order to avoid further damage to the lungs.
How Cystic Fibrosis Affects the Body
Cystic fibrosis is a chronic disease that is typically progressive (meaning it continually gets more severe).
Mucus and Cystic Fibrosis
When someone has cystic fibrosis, they produce excess mucus that is thick and stickier than the mucus of a healthy person, causing the mucus to build up over time and block airways. Normal mucus is a slippery, watery texture, but if you have cystic fibrosis, your mucus is sticky instead of slippery, and thick instead of watery.
Not only can this mucus build-up cause breathing difficulties, but it can also encourage bacteria to spread throughout the lungs and the rest of the body. This bacteria can cause infections like bronchitis, pneumonia, and more. It can even damage the lungs themselves.
As the mucus thickens and the bacteria spreads, your pancreas becomes overloaded with the mucus, causing disturbances in both the pancreas and the intestines.
The mucus buildup in the pancreas acts as a repellent for the digestive enzymes that allow the body to break down food. If these enzymes cannot do their job, the body cannot receive the nutrients in the food needed for basic survival.
Genetics and Cystic Fibrosis
Cystic fibrosis is a genetic illness, but many times the disease goes undiagnosed because the person doesn't know that one of the parents is a carrier. In many of those cases even, the parent who is the carrier didn't even know about the gene.
Be Vigilant About Symptoms
If you have been suffering from several symptoms that match the criteria for cystic fibrosis, if you have had a lung problem or cough that has stumped doctors, or if you suspect that someone you know has cystic fibrosis, ensure that medical help is sought.
Cystic fibrosis can be lethal if left untreated.
Diagnosing and Treating Cystic Fibrosis
Diagnosis of cystic fibrosis is typically done at birth now, due to the severity of the disease and the need for immediate action if the disease is present.
Tests to check for cystic fibrosis include a sweat test (used to test the salt content in a person's sweat) as well as blood work. Sometimes CAT scans are used to check for abnormalities in the lungs, however, a CAT scan that shows no abnormality doesn't rule out CF.
Solutions for Cystic Fibrosis Patients
Currently, there is no cure for cystic fibrosis. It is a chronic condition that researchers are still trying to understand. Several drugs are available on the market to help reduce the symptoms of those with cystic fibrosis, and breathing equipment like nebulizers and inhalers can help improve the quality of life for those with CF.
If a case is severe, a lung biopsy may be done to test for and diagnose cystic fibrosis. This is typically only done when the patient needs a quick diagnosis in an effort to receive a lung transplant.
60 years ago, the lifespan of people with cystic fibrosis wasn't even 20, and now people are living full lives with the disease. However, treatment is needed, as is guidance by a physician familiar with cystic fibrosis and the lungs.
Tools for Understanding Cystic Fibrosis
As someone who has a loved one suffering from cystic fibrosis, I know that I had many questions. This book was instrumental in my ability to understand this confusing disease and be the best support system I could.
Resources for Cystic Fibrosis
Cystic fibrosis can be a scary, overwhelming condition. Luckily, today there is a wealth of resources available for those struggling as well as their loved ones.
Here are a few:
- The Cystic Fibrosis Foundation: Offering a plethora of information on CF, access to clinical trials, breaking news related to cystic fibrosis and treatment, as well as a patient assistance resource center, CFF is the best place for people who have questions about cystic fibrosis.
- CF Living: Cystic Fibrosis (CF) Living is an online resource for patients with cystic fibrosis and their loved ones. The site provides tips on living with CF, stories from people living with the disease, recipes designed for people with CF, and more.
- Cystic Fibrosis Research Inc.: CFRI is a company working to cure cystic fibrosis. They have a website full of fascinating research pertaining to the condition, as well as many different treatment options for CF.
- Boomer Esiason Foundation: This group is a non-profit created to raise awareness about cystic fibrosis. Their website provides a number of tips for living with CF, ways you can get involved, and information on events being held to help raise cystic fibrosis awareness.
This content is accurate and true to the best of the author’s knowledge and does not substitute for diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed health professional. Drugs, supplements, and natural remedies may have dangerous side effects. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.
© 2014 Kathleen Odenthal
glassvisage from Northern California on June 16, 2014:
Thank you for this Hub. I only know one person with this, a friend from school. In 6th grade she was told that she would live to age 30. It's good to see that there are some treatments available. I didn't know a lot about this before reading this.
Eric Dierker from Spring Valley, CA. U.S.A. on June 14, 2014:
Well done here. Good to bring light to this horrible disease.
Dora Weithers from The Caribbean on June 13, 2014:
Thank you for sharing this important information. The idea of "symptomless carriers" sounds dangerous, but perhaps knowing these facts might help the carrier notice some-thing. Voted Up!
Bill Holland from Olympia, WA on June 13, 2014:
Nice summary of this rather nasty disease. I do have a friend who has this....not good at all. :) Have a great weekend Kathleen.