I wanted to share – and dispel – six misconceptions, or “myths”, as often believed by the general public – those without intimate knowledge and experience with Cystic Fibrosis. There are many possible items to cover, but these are the big ones.
1. Cystic Fibrosis is a childhood disease.
Until the 1970s, people with CF couldn’t expect to live into adulthood, but today, nearly half of all CF patients are over the age of 18!
Thanks to newborn screening in the US, more patients are diagnosed at birth, and the earlier patients begin preventive treatment and their caregivers learn about what to expect, plus the newer maintenance medications and brand-new medications designed to work at the source – the genetic defect – there is so much more hope for living longer, fuller lives. Patients are graduating high school and pursuing even further education, perhaps getting married and/or starting a family, building a career, and being able to achieve dreams that others said would never happen.
2. Cystic Fibrosis a “lung disease”.
Courtesy of the Cystic Fibrosis Foundation cff.org
The fact is that the faulty gene exists every cell in the body. The thick, sticky mucus clogs organs, impeding natural functions, causing a chain reaction of issues.
Therefore, CF can affect most major organ systems. In addition to the lungs, the most effected is the GI system – including the pancreas (which also causes Diabetes), stomach, intestines, and also may affect the Liver, Kidneys, Heart, and Reproductive system.
3. You “get” or develop CF.
Cystic Fibrosis is genetic – it’s in every cell in your body.
Most of us are diagnosed by age 2, but for 25% of patients, diagnoses is delayed by a few years, a decade, or even half a century. Late diagnoses happen because the symptoms weren’t recognized as possible symptoms of CF, or that symptoms were mild enough to dismiss them, or because the patient received a wrong, but plausible diagnosis. I know of someone recently diagnosed in her 60s. Imagine that – going your whole life knowing that “something” was going on, but never having an answer, never finding out why. For some patients, it’s validating to finally have a name put to their symptoms, but they have a lot of education to suddenly wrap their minds around.
CF does not “develop”, and is not “triggered” by occurrence of another illness. However, a doctor may accidentally uncover issues while testing or treating a patient for their existing problem, or it might take the collection of symptoms that sparks a theory in someone’s head to test for CF. And finally, it could take the worsening of previously mild symptoms to consider further testing.
4. After a tune-up, we are “all better”.
Many CF patients refer to a two-week hospitalization and/or course of IV antibiotics as a “tune-up”. When done in the hospital, we do 4-6 Airway Clearance sessions a day, extra breathing treatments, eat a high calorie diet, do Physical Therapy, etc. Some patients elect to do tune-ups at home – using a temporary PICC line (long, threaded IV that goes into the upper arm, through a vein that reaches the heart), or wearing a permanent PORT – both which allow the patient (or a family member) to give themselves the IV medications at home.
A tune-up basically “resets” us to baseline – or as close to it as possible. However, it is not a cure. It’s basically a reset button. I often joke that when normal people go to the hospital, they rest. When CF patients go into the hospital, they work – hours of airway clearance (4-6 hours a day), daily walks and/or sessions on the exercise bike, breathing treatments, eating as much as possible…it really is a lot of work. Once I return home, I usually need several weeks to regain my strength from being so sick and doing so much work.
My biggest hope each time is to gain back the lung function I had lost since the most recent exacerbation (or string of exacerbations). However, with CF, each year usually means additional permanent damage.
5. Kids with CF should live in a bubble, stay away from other kids our age, and never tire ourselves out.
This is a huge myth. When I was a child, my parents were very overprotective. I was discouraged from playing outside in any temperature extreme. This meant a lot of missed recesses, field trips, and gym classes.
My parents also discouraged me from playing sports – I remember wanting to play youth soccer when we moved to a new town when I was in 6th grade, and they said the only way I could join is if my brother played, too. Well, that didn’t happen.
It wasn’t until the 8th grade when I had another chance to DO something. I begged my parents to let me try out for the cheer squad. To everyone’s amazement, I made it. The following year, in high school, I auditioned for, and was selected to appear in the school musical (long was my dream of acting, singing and dancing) – which required a lot of singing and dancing at the same time. To keep up, I begged my mom to let me take some dance and voice lessons, and I also participated in every choir/chorus/play/musical that gave me a role.
It was so amazing to exert my body to its fullest potential, sweating while cheering and dancing and finding my breaths while cheering and singing. I never felt more alive, and never knew that my body COULD do things. I surprised everyone, and I credit my constant rehearsing and performing with maintaining excellent lung function during those years (despite getting sick).
KIDS SHOULD MOVE!!! We all should move!!!
6. Cystic Fibrosis is contagious.
First, CF itself is not contagious. It is genetic. You cannot “catch it”. Are are either born with 2 copies of the CF gene (and have CF), carry one copy of the gene (and are a carrier), or born with no mutated genes.
In addition the germs that we carry enjoy living in our lungs because they like the thick, sticky mucus that coats our airways, giving them a moist, warm place to grow. They really wouldn’t like to live in the lungs of otherwise healthy lungs.
Think of a lizard relocating from a tropical rainforest where it rains every day and temperatures soar over 100 in the hottest times of the year and that has thousands of species of bugs to eat, to a dry, snowy tundra where it’s too cold for insects to live and the sunshine isn’t abundant or strong enough to warm up the cold-blooded reptile.
Our colonized bacterial infections are the same way – they need a certain type of environment in which to grow and thrive. They don’t want to set up shop in clear lungs.
However, we are very contagious to one another – so there are now contact protocols for staff taking care of patients in care settings – they have to wear disposable gloves, masks and paper gowns, and dispose of them before leaving the room, so that if they see another patient, microscopic bugs aren’t attaching themselves to doctors, nurses, etc., and latching onto and into the next (CF) patient they see.
Between patients ourselves, it’s suggested that CF patients remain at least 6 feet away from one another when outside, and not even be in the same room inside a building.
Have any questions or myths about CF that you’d like dispelled? Share below!