What is Cystic Fibrosis anyway?

Since sharing the news that Kelsie has Cystic Fibrosis (CF), we have had an outpouring of support from friends and families. But also realize that many people don't even know what CF is. So I just wanted to share what the disease is, how a person gets it, and what it means to have it.  But first and foremost, I want people to understand that a person with CF looks and acts just like anyone else! This disease would not cause Kelsie to be mentally or physically impaired...in fact I fully expect her to be as smart as her father and as independent as her mother! She can do all the things other kids do and will have the same opportunities she would have if she didn't have the disease. She is still my beautiful and precious daughter and I can't wait to discover her unique personality, her talents, her intelligence, her strengths, her beauty, her sense of humor, her dreams, her aspirations, and everything else that makes her KELSIE. Yes this diagnosis is a medical condition so she will have more treatments and symptoms than most kids but I have a feeling it will not slow her down or keep her from enjoying the best life can offer. And if anything, the trials she will face will make her an even stronger and independent person, qualities that will make her stand out among her peers.

So here is a bit of education for those interested:
Cystic Fibrosis is a genetic disease that is inherited. In CF, both parents have to be carriers of the disease to pass it to the baby. As a carrier, you do not have the disease and you do not have any symptoms so in most cases, you have no idea.  In our case, neither Nathan nor I knew we carriers of the CF gene and no one in our family knew of any family history.

In CF, there is a problem with the exchange of salt and water between the cells. This causes the body to make unusually thick and sticky mucous.  This predominantly affects the body is 2 ways:
  1) LUNGS: The thick mucous clogs the ariways in the lungs making it hard to clear. When mucous sits in the lungs, it provides a good environment for bacteria to grow, increasing the risk of lung infection and inflammation.   Over time, multiple lung infections can lead to chronic lung damage. 
 2) PANCREAS: The thick secretions block the ducts in the pancreas so digestive enzymes are unable to get into the intesttines to break down fats, proteins and carbohydrates. This malabsorption (or pancreatic insufficiency) leads to problems digesting food and gaining weight.  

CF is a chronic condition so Kelsie will have it her entire life and there is currently no cure. But there are treatements and preventative measures. So knowing that this is what is going on in Kelsie's body, we have to do a few things everyday to keep her healthy! These are the basics but they will be modified as she grows and more will be added if/when she gets sick.

 1) For her lungs :
       - Preventing infection - wash our hands like crazy, make anyone who enters our home or wants to touch her wash their hands, use hand sanitizers when we are out in public, and keep her away from sick people!
       - Chest PT - pat on her back and sides over her lung fields to help loosen and drain the mucous in her lungs - the purpose of this is to clear the airway and prevent infections from growing in stagnant mucous.  
        - Keep the air in our home as clean as possible - this was not ordered as absolutely necessary but we figured it could only help - so we have purchased air purification systems for upstairs and downstairs
         - Avoid tobacco smoke and other air pollution

2) For her digestive problems :
         - Breastfeed as usual - this is all the calories she needs right now. But in the future she will be on a high calorie/high fat diet including salty foods - very atypical of what considered a healthy diet. But with Kelsie, she can eat all the fat and calories she wants and will never be overweight!! (The challenge will be for Nathan and I to provide the calories and fat she needs without gaining weight ourselves!!)
         - Give enzymes with every feeding - this replaces the enzymes that are unable to make their way into her intestines to break down the food. These are in the form of capsules so I have to break open the capsule and sprinkle the beads in applesauce. Enzymes are a very important part of her daily routine and something she will have to take with every meal and snack for the rest of her life.  If not, not only will she not absorb the nutrients she needs, but she will also likely have tummy pain, gas, bloating, and large, greasy stools. 
        - Give Vitamins A, B, D, E, K

3) General:
        - Give salt - due to the problem with salt/water exchange, her body loses salt more easily so we have to give her a little everyday to help replace what she is losing
        - Keep her from overheating and/or getting dehydrated       

Hopefully that was somewhat helpful for those interested!

Chest PT