November, 2020 CF Annual Fundraiser – Please Donate by December 1, 2020

Welcome November 2020...the month of gratitude. Many of us don’t have that feeling this year. We are bogged down with dealing with the Covid Pandemic and all the areas of our lives that it effects. Jim and I feel it too...we are learning coaches Wednesday-Friday from 8-3 as we lead our grandsons through their online and zoom school lessons. We know our two sons with cystic fibrosis are at a much higher risk if they contact the virus. Just like you, we have had vacations, trips, and family get togethers canceled. I have cooked more meals in a week than ever before and then turn around and do it again and again.

The “whining” list could go on and on. BUT it stops when I hear the word gratitude. We all have something to be thankful for no matter what our situation finds us in. Family and friends are still there, they just have to be reached out to in different and creative ways. My sisters who live in Iowa and Michigan and I zoom with each other every Sunday. We end our time together sharing what we have been most grateful for during the past week. It is with this feeling of gratitude that we come to you once again (actually it is the 43rd time for some of you) asking you to join us in raising funds for Cystic Fibrosis.

I’m sure many of you have seen at least one article in the newspaper, online, or the news about CF and the virus. Yes, people with CF are in a vulnerable place because this virus attacks the lungs...already a place where there are issues in CF patients. Brent and Derik have taken “some extra precautions”:

• Christine and Brent left Chicago the second week of March and spent four months at her mom’s beach house near Wilmington, NC. They felt that with the outbreak and a crowded city it was safer to not be in such a densely populated place. So Miss Leia (their dog) and laptops made the trip down south. They returned to Chicago when they could visit safely with friends in warmer outdoor weather on their rooftop deck...now that COVID numbers are again on the rise and cold weather is approaching they are going to Charleston. SC (where they met and first lived) for the month of December.

• Derik spent a great deal of time at home those first four months because he was furloughed from his job. Helping with spring online school and walks around the neighborhood were all that was on his radar! Derik lost his job due to the pandemic but it was just a month before he found a new one...He continues to work at a corporate cafeteria and is in charge of the grill. Both boys and their families are models of mask wearing, hand sanitizing, hand washing, and social distancing.

Now for that all important research update...last year as we were composing our 2019 CF letter a drug that had been in stage 3 trials was approved. Brent had been in the drug trial and now a year later both boys have been on the drug – Trikafta - for about a year.

Brent says: “I feel like I am 27 again as far as how my lungs are operating. It is exciting to see my Spirometry numbers go up as opposed to staying flat or going down. It is really nice to not cough nearly as much (if at all!).” Derik says: “ It made me gain more weight. Also my PFTs have gone up a little each time I go to clinic (55 to 61). Hopefully, the increase will continue.”

Research News:

A large part of the news in 2020 is how people with CF are dealing with the realities of COVID-19 since they are already at high risk of any kind of infection. It appears that people with CF have an incidence of COVID-19 roughly one fifth as high as the general world population. And 90% of people with CF have relatively few symptoms and complications. This may be because people with CF already take extra precautions to prevent infection.

The other continuing news is the effect Trikafta is having on the CF population. Since 2007, I have continually mentioned CFTR (“cystic fibrosis transmembrane conductance regulator") drugs and how they work to improve the function of CFTR. The names VX770, Ivacaftor, Tezacaftor, Exacaftor have been thrown around as if they were some kind of magic spell. Looking back, it almost seems as if they really are magic spells, especially with the approval of Trikafta and the effect it has had not only on our sons, but on almost the entire CF population. Trikafta improves lung function by about 15% in about 2 weeks! For some people with CF, the improvement nearly doubled their lung function.

Trikafta is currently approved for use by people with CF who are 12 and older. A just completed clinical trial hopes to have the drug approved soon for children 6 to 12. Another study is looking at the use of the drug for children as young as 5 months old. In addition to the nearly immediate effect Trikafta has on improving the quality of life for people with CF, there is a hope that earlier use of Trikafta can help to prevent the damage to internal organs caused by CF.

An interesting aside involves the use of CFTR modulator therapy for other conditions. For example, the malfunction of CFTR is seen in tobacco smokers. In vitro (test tube) shows the acute reduction of CFTR function in cigarette smoke extract can be reversed by certain CFTR modulators – a possible treatment for non-CF COVID-19 patients.

We hope each year to educate you a little more about Cystic Fibrosis and hope that you also spread the news. Research has opened up many doors to discoveries that have led to better care and longer lives for the 70,000 – 100,000 patients worldwide, but there is still no cure for CF...so join us once again as we raise funds for new possibilities. Brent and Derik are “reaping the benefits” of this new drug, Trikafta...who knows what else is out there for them and all those who have Cystic Fibrosis. Remember it is because of YOU that life expectancy has gone from 16 when the boys were born to an average now of 47. Follow the directions below to join us in giving hope and promise to all families affected with CF and to expand CF research.

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, 2020