CF 2021 Annual Fundraiser - Please donate by December 1, 2021
Greetings to friends and family,
Through the ups and downs of the pandemic, we hope this letter finds you healthy and safe. We have definitely embraced ZOOM in the past 18 months and Linda has pulled out the holiday masks she made last year. We’ll start with our family update over the past year…
Linda and her ROOKS (high school friends) did their 25th trip together exploring Lake Erie in June. She also celebrated her 50 plus 1 college reunion at Otterbein University and her 55th high school reunion (all in one week)...and one more big celebration was our 50th wedding anniversary! We continued from January-May 2021 teaching here at Pace’s Place three days a week. We all celebrated the end of a very challenging school year . This year the grands are in school five days a week. Jim continues his filming duties at church and his men’s chorus (American Harmony) is slowly returning to normal.
Misty continues to teach 3rd grade but is now teaching in Columbus Public Schools. Jeffery (8) and Zach (11) are attending their local public school, Licking Heights. Jeffery was seen last week running for the bus in his socks, carrying his shoes! Zach spent a week at Camp Wyandot (overnight camp) and hopes to return next summer. Both boys just achieved the rank of black belt in karate and both are involved in music (Jeffery - piano and Zach - percussion). Derik continues at Hikma Pharmaceuticals in the corporate cafeteria run by Sodexo.
Brent and Christine are still in Chicago, but working from home has found them this past year in warmer climates during the winter months and on their beautiful Chicago rooftop deck in the summer (Brent has become the resident plant guy and this summer’s tomatoes were his specialty!) The Paces have purchased some property in downtown Charleston where they plan to build a house.
If you read no further in this letter, please take 1 minute (literally) to look at the youtube video, (click here->) “Path to a Cure”. If that doesn’t work, copy the following link and paste it into the URL area of your browser:
https://www.youtube.com/watch?v=WWZAJ7PHW4I
It describes, in broad strokes, the CF Foundation’s multi-leg path to improve treatment for CF
RESEARCH UPDATE
- Trikafta: Trikafta was approved for CF patients 12 and older in 2019. Brent had been in one of the clinical trials. Both Brent and Derik have been on the drug since then, and in our 2020 letter we remarked how it had improved their health (in fact, Derik is now trying to lose weight!). In August of this year, Trikafta was approved by the FDA for children 6 through 11.
- Background - DNA “Programs”: Human DNA has 23 pairs of chromosomes. You’re probably most familiar with the 23rd pair (“X” and “Y” chromosomes) which determine sexuality. The CFTR (Cystic Fibrosis Transmembrane conductance Regulator) gene on chromosome 7 contains 7 sequences of DNA (think computer programs). These 7 “programs” build the sequence of 1480 amino acids which are the CFTR protein. We all have the CFTR protein.
The CFTR has the job of regulating chloride movement through cells in the body. It’s called the “chloride channel”. If the “regulator” doesn’t work right, your sodium (sodium plus chloride = salt, think “sweat”) won’t be right, either, and maybe your potassium and bicarbonate (channels) won’t work right, either. You end up with thick mucous. Breathing and digestion problems often result. Before modern science, the grandmother kiss test (“Hmmm, the baby tastes salty!”) was a first indicator of CF. Today, doctors actually measure the “saltiness” with a clinical “sweat” test.
MOST NEWBORN BABIES get a sweat test (a quick painless test) to rule out (or indicate) CF. If you know a youngster born less than 30 years ago, they probably had a sweat test at birth.
- Correcting the Faulty CFTR gene (right-hand path in the youtube video): Researchers at the Hubrecht Institute (Royal Netherlands Academy of Arts and Sciences) used gene editing in cultured human stem cells to correct mutations that cause cystic fibrosis. This is still an early, yet one additional, step toward the process to correct the faulty gene in humans.
- CFTR modulators: If you watched the youtube video, the left-hand path described CFTR Modulators and indicated we need more. A team of scientists at the UNC School of Medicine is looking at small, nucleic acid molecules called “oligonucleotides” that can correct some gene defects in CF that existing modulators don’t work with.
- Nonsense mutations (middle path in the youtube video): If some CFTR mutations are like a bug in a computer program that cause a different result than what you want, “nonsense” mutations are like a computer program that just crashes and quits working. In the case of DNA, this means the protein (like the CFTR protein) is only partially built.
Researchers at the University of Alabama have found a small molecule (they call it SRI-41315) that allows the DNA “program” to read-through (or read past) the nonsense mutations and keep going. There is hope this may improve treatments for CF as well as some types of muscular dystrophy and cancers that are also caused by nonsense mutations.
In 2021 Linda began a gratitude journal… Here is the CF version:
We are thankful that in 1978 our pediatrician wanted to rule out the worst thing that Derik’s symptoms could be so he ordered a sweat test at Children’s Hospital. This led us to an early diagnosis and putting a treatment plan in place.
Remembering those along our journey who offered to learn hand percussion postural drainage (only thing available for about our first 10 years). A few of those people are Linda’s parents who were there whenever we needed them and Dave Russell who went on Boy Scout trips and did Derik’s treatment.
Researchers can’t be thanked enough… advances have led us from teenage survival to an average today of 46. Just ask the boys about their medication regime and how it has changed over the years...new oral medicines, and others in aerosol form. We have to mention again Trikafta (read more detail about it in our 2020 letter). This drug was approved in 2019 for those with one copy of the F508del mutation (about 90% of CF people). In their own words, here is what the boys say about the drug and its effects on their lives...Brent: I feel like I have the lung capacity I did when I was playing soccer at Ohio Northern University. I do not have the heavy deep cough anymore. My life quality has improved in the sense that I do not cough or have exacerbations, but I can only imagine the quality of life change that others have had, like possibly coming off the transplant list. My lung capacity has increased too. I can only hope that this type of drug can be available to EVERYONE with CF. Derik says:”I now have more energy. This drug has also helped me gain weight. On my one year followup exam on Trikafta, it was noted that previously seen tree-in-bud (airway obstruction) and mucous plugging had decreased.”
Gratitude for emotional and spiritual support...the power of prayer, food on our doorstep during diagnosis, hugs, good listeners. Some of these people are our church family, neighbors, friends (new and old, far and near). We have felt your support and still do…
Our gratitude continues as we begin our 44th year to raise funds for Cystic Fibrosis. Join us as we look to the future and with it the challenges and celebrations we know come on any journey. We need you on our team...below are all the details for you to donate. We are in deep gratitude for your continued support of all those families with Cystic Fibrosis.
Love,
Jim, Linda, Brent, Christine, Derik, Misty, Zach, and Jeffery Pace
The easiest way to donate is to Click here (or type “http://jimpace.eml.cc” 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 1, 2021