Posted on December 31, 2013
Everyone with CF has a different experience. It is a very individualized and isolated disease. Most people are diagnosed at birth or in their first year of life. When I was born it wasn’t always something they checked for, so I was diagnosed at four years old.
CF is very individualized. An example of that is someone with CF who is twenty years old and still is pretty healthy, but then there could be a nine year old who is near death. There are multiple mutations, and they all affect each patient differently. Every CF case is like a snowflake, they are all different.
The way we measure our lung function is through a test called Pulmonary Function Tests (PFTs). These tests are done with a spirometer we breathe into. When I was little, I remember the respiratory therapist telling me to take a deep breath and blow out really hard and fast like blowing out candles on a birthday cake. Sometimes, they would hold a windmill out in front of the spirometer and have me try to make it spin. The goal of the test is to measure the size of the breath taken, the amount of air blown out in the first second compared to the initial breath, and the time it takes to expel all of the air from your lungs. The most important number is the FEV1, which is the amount of air blown out in the first second.
Let me help you understand. Take a deep breath, right now. As deep as you can, and then blow it out really fast and hard, while counting how long it takes you. It was pretty fast right? Well for Cystic Fibrosis patients is takes longer. Another example would be a “Nitrogen washout” which is where you are in an airtight space and then you blow out your air and breathe in pure Oxygen. It is suppose to measure how long it takes you to washout all of the Nitrogen from your lungs. A normal person would take about 3-7 minutes. Mine took over 15 minutes to wash out.
The reason it is so hard to get air out is the same reason it is so hard to get air into our lungs. All of the mucus is blocking air, either trapping it in, or trapping it out.
Well, anyway, I am getting off topic. The point is, my FEV1 is around 25-35%. That basically means 25-35 percent lung function. When they start to refer a patient for transplant is when your baseline FEV1 is around 33. Basically that means I am pretty sick.
I remember one time, my dad took a PFT and he turned purple. He got really good numbers. I think he finally realized how hard PFTs are. If they are hard for someone with perfect numbers like that, imagine how hard it is for me.