Five year old Piper Kenney was diagnosed with Cystic Fibrosis, an incurable progressive genetic disease that causes persistent lung infections and limits the ability to breathe over time. She was diagnosed at two weeks old through newborn screening tests. She has a particularly aggressive form, and due to a drop in her pulmonary function tests, was recently hospitalized for a “tune up” which involved around the clock Intravenous antibiotics. According to her mother, Lisa Kenney, the medicine was to “help rejuvenate the lungs and kill whatever was sitting in there.” She had a bronchoscopy, which is a procedure that allows the doctor to look at a patients airways through a thin viewing instrument called a bronchoscope. Test results showed Piper had RSV and Corona Virus which attributed to her decreased lung function.
The bronchoscopy also showed hardening of the upper right and lower left areas of her lungs, “Her mutation doesn’t allow for salt and water to leave the cells in the lung which help clear the mucus so it sits there blocking and hardening the airways. Our primary goal is to stop that progression.” After seven days Piper was discharged from Joe DiMaggio Hospital.
Piper’s daily regimen includes wearing a vibrating vest twice a day for 30 minutes to break up mucus and then several medications and a steroid inhaler. “She is pancreatic insufficient so I have to add a lot of fat to her diet, said Lisa. In the morning I cook eggs in coconut oil, butter, milk, and salt. And since her body doesn’t produce Vitamins A, D, E, or K we have to add additional medications, vitamins and fiber to her daily regimen.” The build up of mucus in the pancreas can also stop the absorption of food resulting in malnutrition and poor growth and according to Lisa, “Piper has no appetite. The only thing she really likes is chicken fingers. We are hoping to hold off on getting a feeding tube for as long as possible but realize the likelihood is higher as she gets older.”
On September 3, 2017 Joey Huempfner with Riverside Realty of Florida, and I surprised Piper Kenney with her very own paddleboard and paddle. When Joey heard about Pipers story and the life extending benefits of salt water on fragile CF lungs, he immediately said he would donate the board and paddle to her. Piper was recently hospitalized for a “tune-up” and then back in the hospital for pneumonia. The family is so excited to be able to get Piper out on the water and breathe in some of that invigorating salt air.
February 24, 2018: From Lisa Kenney (Pipers mom): “For the last month we have been to clinic four times and Pipers lung function has dropped around 30%. We have no reason as to why, but need to get her back on track so the Orkambi can do its job. We will be having a two week hospital stay starting around March 15th. This could be sooner if we see any shortness of breath or her cough increases. We are hoping that this will push her lung function back to where she should be.
With the challenges we have faced over the past year, we have to make a really tough decision if going back to her old clinic would be the best for our family. Being so far away for two weeks makes it hard on Piper and our family. Ryder really misses his sister during the separation. If we moved back to our home clinic Ryder could come and see her every day and Piper would be showered with love close to home, making the stay a little more bearable. I have missed a lot of work as well with my drives for all of her appointments. Being close to home would allow Nick, family, and I to share time at the hospital. Praying we make the right decision for our little fighter.”
Piper update from her mom on August 5, 2018:
This little girl has had a tough year. A year ago around this time we were coming home from our first hospital visit. This was the start of a journey we didn’t know the outcome. About 2 weeks after being discharged, we were back at a hospital with pneumonia. After 5 days on lock down, we were home. However, with that discharge we had to make the decision if a feeding tube would be the best thing for Piper’s health. We tried as long as we could to not do this because we feared the scars she would have. We got over that fear and now faced the challenge of how to keep our baby girl healthy so she could fight this battle! Two days before Thanksgiving we were ready for g-tube surgery. She again showed her strength with a fast healing belly and excitement to GET CHUNKY! About 2 months later we get the bad news that her lung function is not where it should be. Now we had to have clinics once a month to monitor this. January 2018, Piper starts Orakambi. This is our miracle drug or is it? February of 2018 we got the news that her lung function had declined almost half her baseline during her PFT. This means her lungs were only at 63%. This can’t be! We do everything we are supposed to and more to keep her healthy, but here we are off for another hospital stay. During this stay the news gets even worse! Piper has a bacteria in her lungs that is hard to kill and we have to act aggressively to keep her on the right track. What???? Well hell…let’s get this started! Nope we had to wait 8 weeks to ensure we started the right medicine for the right strain. Ughhhh! May 16th with an FV1 of 63%, weighing 37 lbs and 4 months of Orakambi we go to the hospital to start our journey. Piper gets a port inserted so she can enjoy her summer and not have to worry about how long we have to do IV medicine. I learn how to access and remove the access. This allows her schedule to be more hers than a home health nurse. Piper gets weekly blood draws to ensure the medicine she is on is not too much for her tiny body and that she is getting the right dosage. This cannot be pulled from the port due to the testing and she has to be poked the normal way. Monthly hearing test as this aggressive IV medicine can cause hearing loss. But having to do all this does not change our decision as we have to fight! August 1st, 2018 Piper goes to clinic for a check up. She had done a culture back on July 17th. We get the update that as of now there are no signs of this bacteria. We still have to wait 4 more weeks for a definite yes, but excitement is upon us!!! Piper had an FV1 of 106% and weighed 44 lbs. This little girl was so excited as were here doctors and myself!!! She proved to them that she means business! We still have a long way to go to keep this bug away! But we are on our way to that outcome! This little girl has a bright future and a large fight in her! Here is to many years and the happiness of how far the Cystic Fibrosis Foundation and Cystic Fibrosis Foundation Palm Beach pushes the limit to find that cure. We are thankful for everything they have found to fight the challenges we face. Here is to more years for our warriors!!! #piperspreciouspath #shemaybelittlebutsheisfierce