Thursday, February 4, 2010

Journeying with Quinn and Cystic Fibrosis

Tuesday we went to Doernbecher for Quinn's three month check up. We head to OHSU every three months to visit the Cystic Fibrosis Clinic and meet with the pediatric pulmonologists, dietician, nurse, and support staff that help us on this journey with Quinn. We first met this crew after Quinn's diagnosis almost four years ago--they are a great team to work with and we really appreciate each one of them.

Each person with cystic fibrosis has his or her own story. Just like with any chronic disease, there are individuals with severe issues and those with more mild experiences. In a nutshell, Quinn's body makes more mucus than you or me which typically causes issues related to lung function and digestion. If you'd like to know more about CF you can check out the Cystic Fibrosis Foundation website. You never know what you'll stumble on if you just google CF so we always point people to this website because it can be trusted.

For Quinn, his pancreas does not produce enzymes--enzymes break down fats and proteins and without them a person would have significant digestive issues (gas, foul/loose/oily bowel movements) and lack of weight gain . Since he was two weeks old, he has been taking enzymes mixed into applesauce to help with those issues. He also needs about 1800 calories a day in order to gain weight--because of Quinn's various issues with food (he has had food therapy and was diagnosed with reflux) he isn't a big eater. We've seen great improvement with his interest in food and strong decrease in the vomiting associated with reflux but he can't eat enough to meet that 1800 calorie goal. Two years ago he got a feeding tube which has made a huge difference for him. Folks with CF need additional vitamin supplements of A, D, E, and K so Quinn takes that each day.

To address potential lung issues we do two things. First, Chest Percussion Therapy (CPT) requires us to pound on Quinn each day with some little rubber mallets. Sounds crazy but this has been an effective method for many years to help loosen up the blockage that can occur in the airways of the lungs. We also use a medication called pulmozyme which is inhaled into the lungs with a nebulizer. The pulmozyme is breathed into the lungs and it acts like a little pair of scissors, snipping through the blockage. About once a year we do a full panel of blood work and take x-rays to see what is happening with Quinn's lungs.

At our appointment today we had some concerns because of a cough that has been hanging on for a bit. Often, people with CF develop a chronic cough and we sometimes get nervous about things...when Quinn gets sick we go into overdrive to help him get better. I'm kind of crazy about Quinn getting enough sleep--I think rest helps health and that is something I've always been vigilant about (OK, I admit there is some paranoia there too!) So, we had this concern about the cough and Dr. Powers went ahead and ordered an x-ray for Quinn just to check. Quinn always does a great job. The thing that makes him the most anxious is getting a throat culture. We got the x-ray and the tech asked Quinn if he wanted to see his insides--Quinn wanted to! The tech brought out an x-ray and put it up for Quinn to see...except it was an x-ray of Buzz Lightyear! Quinn LOVED it! We loved it too, especially since he was wearing his Toy Story shirt!

When we met with Dr. Powers, he was super pleased because the x-ray showed that the blockage in Quinn's lung has actually decreased since his x-ray from a year ago! HOW AWESOME IS THAT!!! Seriously! We were thrilled! We know that every visit may not be amazing so we take our victories with great enthusiasm!

Last appointment Quinn was 37 inches and 32 pounds. Tuesday he was 38 inches and 32 pounds. We have to work on getting some more calories into him each day but overall he is doing so well.

The routine for Quinn looks something like this...

At every meal, Quinn takes four capsules of enzymes. We try to make meals high in fat, protein, and calories.

Once a day we do a breathing treatment and chest percussion therapy--it takes about 45 minutes and I usually do it after Quinn's rest time in the afternoon.

At night, Quinn takes enzymes before bed and we get his pump set up for his feeding tube. Quinn is so good about this process and such a trooper. A high calorie formula runs for about 7 hours so Ryan and I usually take turns getting up to stop the pump around 1:30 or 2:30 am.

Quinn really is amazing when it comes to cooperation and patience in this process. He does get frustrated at times but those times are rare and far between. Our prayer is that he will understand the reasons for his care routines and that he will be an advocate for himself too. We also pray that his lungs will be protected. It is our job to pray so we ask for what we really desire for Quinn. We pray that he will have good friends who see him for who he is and not as "the boy with CF". We thank God for our families and for friends who are such an awesome community willing to support us and support Quinn.


Denee Longan said...

Thanks for posting about this! I love hearing anything you have to say about Quinn's CF! That's awesome that his lungs are doing well!!

Amy Wolff said...

Thanks for sharing so much info. A glimpse into your lives- and your precious boy. Your love, his courage, and the care of great doctors is inspiring.

Blessings and health to your family, Meredith!

Aj Schwanz said...

You are an amazing, persistent, faithful mother, and it is so evident that Quinn is such a blessing to you all! Thank you for giving us a glimpse into your life.