Dear Friends and Family,

Can you believe we have been involved with CF for 46 years and have been fundraising for that long, too! Many of you have been with us from the beginning and we always welcome newbies to our team. Without your support we would not have raised money for research and patient support. To borrow a quote from another organization Linda is involved in (My Very Own Blanket) “If we all do a little, together we can accomplish a lot.” It definitely fits here, also. The Pace family is blessed to have your support.

Speaking of support...thoughts down memory lane bring up supporters that are no longer with us. One of those is Linda’s parents who from day one were there to help emotionally and to help in the daily routine that each boy had to go through twice a day. Katie and Harry learned how to do hand percussion for treatments and how to prepare meds and neubulizers.. and remember they didn’t live in Columbus so travel was involved. Linda’s parents also did fundraising...getting the North College Hill Community and beyond to support CF.

Our fundraising efforts this year are dedicated two friends from our condo community who recently passed away. Don was an avid supporter even when he left our community and moved to PA to be nearer his family. Stu was a neighbor who moved into an assisted living facility and we helped care for him for 2 ½ years.

Family Update

Jim and Linda: We are still very involved in many activities: Community Band (both), 2 singing groups (Jim), reading to two school classes (Jim) tutoring at 2 schools (Linda), flute trio (Linda), My Very Own Blanket (blankets for foster children) (Linda), church activities (both), and we both try to stay connected to friends and family. We traveled to Iowa City in July where Linda’s sister, Carol, remarried. We welcome Andy to the family.

Jim’s scheduled September prostate biopsy had to be postponed when he contacted COVID (Linda followed several days later with COVID). The biopsy he finally had in October was negative for cancer! Jim is now scheduled for minimally invasive surgery (PAE – click here for more info) on Dec. 3. Linda loves tripping (literally) at The Farmers’ Market, but luckily nothing serious! She strengthened and stretched her muscles in PT. We are both blessed with our health. Linda does jazzercise and walks. Jim joins in walking “sometimes.”

Brent (49) and Christine: Hard to believe that Brent has outlived the predicted age of 15 (predicted 1978 life expectancy for CF) by more than 3 times. Today a child born with CF has a predicted age of 61. Charleston SC is still home and luckily they only had rain events from the hurricanes. Brent had his 3^rd bout of COVID and then a lingering virus brought back a cough similar to his CF cough before beginning Trikafta. All is well with him now. He and Christine continue to work from home. They are avid golfers and have played in several fundraisers, including one for CF. Their home is cozy and their gardens amazing. They even have several banana trees!

Derik (46) and Misty: Derik has had some recent concerns over the past 6 months with his liver enzyme counts. Possible causes could be side effects from his CF drug, Trikafta, or his seizure meds (last seizure was 10 years ago). Before doing any changes on his CF meds, it was decided to try a different seizure medicine. A “break through seizure” occurred as he was reducing the old med and increasing the new. This happened at work and he fell on the floor hitting his head and shoulder. Luckily he had no serious injuries. After 8 hours at OSU ER, Derik was released. After 2 weeks of not driving (waiting to see if new meds settled in) the Senior Pace (Jim & Linda) Uber ran at 3 AM and 3 PM. All are happy he is driving again. More blood work will show if liver numbers come down or if CF Med might have to be adjusted. Derik also has difficulty walking and is in the process of getting some braces to wear (not related to CF). Overall, Derik’s CF health is good, but CF induced diabetes needs constant monitoring. Misty is in her 10^th year of public school teaching, 9 of those years teaching 3^rd grade. Jim and Linda continue to be involved with her class. Misty and Derik will celebrate their 25^th wedding anniversary in March 2025. Zach (14) is a freshman in high school. He still has a strong interest in art and enjoys a “pick up game” of basketball. Jeffery (11) is in 6^th grade and has begun to play the trumpet. Jeff enjoyed a trip to Niagara Falls with his grandparents in August. After a trip to the Wilds Conservation Center (more here) Jim and the grandboys have a new passion – ziplining.

Research notes:

Some developments indirectly involving CF have occurred this year with our family: Linda’s sister Carol has bronchiectasis, a lung disease that may benefit from CF research. Most often caused by repeated lung infections that cause inflammation of the airways. Several other family members also have this disease. Carol was alerted to a study found by Linda’s other sister, Jan, that involved being a CF carrier and having bronchiectasis. Carol is a CF carrier. She traveled to Atlanta for tests and then was put on Trikafta (the same drug that Brent and Derik take for CF) for a month to see if it improved lung function and overall health. Carol found the drug to dramatically reduce her mucus production. She then had to go off the drug for a month and see how she fared. After the study concluded and powered with a strong will and a willing doctor in Iowa, Carol tried to get on Trikafta. She was rejected twice. Then for some (unknown to her) reason, she was cleared to take the drug. It has continued to reduce mucus production and her doctor will monitor her through the winter to see how she does.

Definitions

CFTR – Cystic Fibrosis Transmembrane conductance Regulator gene - the gene (present in all humans) that causes cystic fibrosis when the gene has certain mutations.

CFTR modulator – a drug, or combination of drugs, designed to correct the malfunctioning protein made by the CFTR gene. You’ve heard us talk about one in the last several years, Trikafta, taken by both Derik and Brent (and now, also, Linda’s sister Carol). This is because all three have one copy of the mutation F508del as do 90% of people with CF. That’s good, but work needs to be done to help the other 10%. You can read our letters from 2017 and 2019 for more information about modulators and Trikafta.

CFTR Modulator News:

• Vertex Pharmaceuticals (makers of Trikafta) is seeking FDA approval for a new once-a-day modulator therapy. Known as the vanzacaftor (vanza) triple, the modulator would be approved for people with CF ages 6 and older who have at least one F508del mutation or one other mutation that responds to the vanza triple (which include Trikafta®-responsive mutations). Clinical trial results of the drug showed improvements in lung function similar to those seen with Trikafta and a reduction in sweat chloride levels that exceeded treatment with Trikafta.

• Also, Vertex reported that it submitted an application to the FDA to approve Trikafta for additional rare mutations that have been shown to be responsive in lab tests.

• Sionna Therapeutics has acquired the rights to develop three potential CFTR modulator therapies from AbbVie — two of which have been shown to be safe and well tolerated in Phase 2 studies. The company plans to combine one of these potential modulator therapies with one of several of its own compounds to target different places in the CFTR protein than existing modulators. The objective is to give people with CF additional modulator treatment options.

Screening for CF in Newborns:

A study in Georgia showed “cases missed by newborn screening occurred disproportionally among babies of color.” A Georgia Newborn Screening Committee “recommended implementing an expanded DNA panel in the state to achieve equitable diagnoses for all infants.” Providers are being reminded “that CF can occur in infants of all races and ethnicities, with infants of color being at higher risk of delayed diagnosis.” 
( https://www.cff.org/news/2024-09/cystic-fibrosis-foundation-improving-cf-newborn-screening )

Mental Health Challenges Faced by Children With CF:

Led by the University at Buffalo and launched this summer, the new study is an outgrowth of The International Depression Epidemiological Study (TIDES), which began in 2014 and was the largest study of mental health in adolescents and adults with CF. As a result of TIDES, annual screening for depression and anxiety is now part of routine CF care for nearly 90% of adults and adolescents with CF in the U.S.

“That’s the goal of this new study, which we are calling TIDES 2.0,” says Beth A. Smith, MD, principal investigator. “It will allow us to take what we have done nationally and internationally for adolescents and adults with CF and do it for children from 18 months up to 11 years old.”
( https://medicine.buffalo.edu/news_and_events/news/2024/09/smith-cf-mental-health-20762.html )

How to Donate:

The easiest way to donate from your web browser is to click here (or type “https://cf.glenabby.org/” into your web browser) to go to the “CF Letters from the Pace’s” web page and click on one of the pictures at the bottom of the page!

You can also mail your contribution to us at:

CFF Annual Fund
c/o James M. Pace
6251 Abby Gate Ct
Westerville, OH 43081

Checks should be payable to “Cystic Fibrosis Foundation”

Please donate by December 7, 2024

With Love from all of us,

Jeffery, Zach, Misty, Derik, Christine, Brent, Linda, and Jim