You all know that almost every year since Ava was born I've written a letter asking for donations to the Cystic Fibrosis Foundation. It is a worthy cause for many reasons, especially because it is for a cause that is so personal to us. I usually don't have my Great Strides letter done this early in the year and this year is no different. However, our circumstances with this disease have recently changed.

Ava was able to start the life-changing, closest-thing-we-have-to-a-cure medicine by the name of Trikafta in 2023. For the first few months, she benefited in so many ways, gaining weight faster than ever before, she had quicker recovery times with colds, less salt loss, and more "normal" bowel movements to name a few. By October of that same year in which she started kindergarten, Ava experienced her first ever bowel obstruction. We spent one Friday force-feeding her 30 ounces of liquids mixed with 5 capfuls of dissolved Miralax to clear the impacted stool under the direction of her pediatric GI doctor. It ended up being a success, and then she fell ill to the stomach bug within the next week or two. Between the blockage and stomach virus, her liver levels skyrocketed to their highest ever and it was no longer safe for her to continue taking Trikafta. It took quite 9 months for her liver levels to normalize again. During that time, Ava underwent an endoscopy to rule out celiac disease. She also ensured many colds, which only prolonged the elevated liver enzymes.

In August of 2024, Ava's liver levels were finally back to normal and she restarted Trikafta. A few weeks later, we learned that her liver levels had once again reached an unacceptable high and she would need to stop Trikafta. However, her GI doctor had mentioned that if she were ever to get on Trikafta again he wanted her to have a liver biopsy. So Ava and I headed up to the Golisano Children's Hospital in Syracuse for the procedure. The next day she waited 9 hours without food before being called down for the procedure. It went very smoothly and she was discharged the following day. From the results of that biopsy, we learned that Ava has cystic fibrosis related liver disease and she is a carrier for the a1at deficiency, another genetic-inherited condition in which the risk of lung and liver disease is increased. We learned that it would be very unlikely that she would be able to take Trikafta ever again, medicines are likely causing damage to her liver and she cannot take NSAIDs, and she would need a liver transplant sometime in the future (think teenage years). Since then we've begun working with a liver specialist at Columbia University in NYC in addition to her regular team at Upstate.

Over February break, Ava had a routine follow-up appointment with her cf team at Upstate. She did an amazing job, weighs just over 53 pounds, is almost 4 feet tall, and blew 101% on her PFTs. She keeps rocking her throat swabs and her blood draws are still a work in progress, but overall it was a great appointment. Yesterday, her nurse called with some positive news, but also more devastating news. On a positive note, Ava's liver levels have dramatically decreased since her last labs in January and although she cannot go back on Trikafta, she is able to restart Orkambi, the modulator she was on prior to Trikafta. Her body and especially her liver seemed to tolerate this more easily.

The bad news is that Ava has contracted pseudomonas aeruginosa, one of the deadly bacteria that is difficult to eliminate and highly resistant to antibiotics, especially over time. Pseudomonas A. thrives in moist conditions and is found in soil and water, which is why we have to be extremely careful about sterilizing her neb cups and avoiding things like hot tubs. Eventually, people with cf may pass away from respiratory failure due to this or other deadly bacteria in the lungs. Hopefully, the inhaled antibiotic heading our way this week will eradicate the bacteria, but we will have to treat her twice a day for 28 days on top of her other nebulizer treatments and airway clearance. And with recent cuts to the NIH, who knows what the future will hold for those with cf.

This year, I'd like to ask that you consider donating to the CF Foundation for my birthday in honor of Ava. Thank you to all who have already helped make a difference by donating on our behalf in the past. We've walked at Great Strides for the last 5 years and raised over $10,000. This year, we will still be walking at Great Strides, but Kirby and I will be taking on a different challenge, too. We will be participating in the Xtreme Hike in the Adirondacks near the end of August. We each need to raise a minimum of $2500 in order to participate, so we need your help. Any donation, no matter how small, is appreciated! Help us hike the mountain and make CF stand for "Cure Found." Thank you in advance!