Cystic Fibrosis 2017 Letter

We welcome Fall 2017 with a few reminders about our annual Cystic Fibrosis Fundraiser. As of 2016, the bowling event is no longer on the fundraising list of the CF Foundation, but that has not stopped the Paces from raising funds for research and patient support. We are fundraising for CF...thought you might like to read the mission statement of the CF Foundation:

“The mission of the Cystic Fibrosis Foundation is to cure cystic fibrosis and to provide all people with the disease the opportunity to lead full, productive lives by funding research and drug development, promoting individualized treatment, and ensuring access to high-quality, specialized care.”

All of your donations go to the CF Foundation...just like it did with the bowling event we participated in for 38 years. Everything that you give is 100% tax deductible. Our goal this year is $5000...join our team of supporters and help us make this happen. Many of you have been with us on our fundraising journey for many years. If you are a “newbie,” welcome!

We’ll start with a history lesson – Cystic Fibrosis was identified as a disease in 1938. At that time, little was known about the cause and most babies with CF died before their first birthday. The Foundation was formed in 1955 and with that came the start of trying to answer many questions. In the 1950s, children rarely lived beyond childhood. A big breakthrough came in 1989 when the gene that carries CF was identified. The Paces were already involved by this time. Brent and Derik were diagnosed in 1978. At that time, life spans were in the teens. As more researchers joined the team and more funds were raised each year, there were promising medications and even some hopes for a cure for a small segment of CF patients with a certain mutation. Today the life span is 37.5 years and many CF patients live well beyond that number. Also, today nearly half of the CF population is over the age of 18. If you have read our letters in the past, you know about the research we highlighted each fall. (You can go back and read about that research and/or make a donation at http://jimpace.eml.cc).

Just what is this thing called CF? CF has been called the invisible illness because when you look at a person with this disease you can’t tell they have it. Cystic Fibrosis is a life-threatening disease that causes thick mucus to build up and clog some of the organs in the body, particularly the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation and infections that leads to lung damage.

Mucus can also block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food. People with CF often need to replace these enzymes with capsules they take with their meals and snacks to help digest their food.

To have CF, a person must inherit two copies of the defective CF gene – one from each parent. About 30,000 people in the US have CF. About one in every 31 Americans are carriers. CF varies widely by country. In the US, CF occurs in 1 in 3500 births. In India, the rate is 1 in 40,000-100,000 births. But in Northeastern Quebec, the occurrence is 1 in 900 births! So now you know the basics…

What is breathing like when you have CF? Try this experiment. Pinch your nose and put a straw in your mouth. Breathe through the straw for one minute….

Family updates:

Jim and Linda are well settled in their condo community. Jim is now an official member of the condo board of directors and he and Linda put out the community newsletter and manage the website. We have great neighbors and even have welcomed to Glenabby two couples we have known for over 40 years! Jim and Linda took their second Viking River Cruise this summer on the Seine River. What an adventure we had exploring Paris, Monet’s Garden, Rouen, and much more. Jim and Brent took an eclipse trip to Castalian Springs, TN (pop. – under 600) to view the total solar eclipse. Jim proudly wears his TN eclipse shirt! The Whitehouse Family Reunion was resurrected in the summer of 2017 at Lake Hope State Park. Many family members gathered to share a great weekend together. Jim is still very involved with singing (church solos and his men’s chorus) and reading to kids at schools. Church finance secretary and the worship committee keep him busy, too. The upstairs man cave has a train table that is under construction...no end in sight! Linda continues her love of music with the Westerville Community Band and flute trio. She tries to stay in contact with friends and enjoys going out to breakfast and lunch. And of course, she also has church commitments. Linda still loves “to help kids be the best they can be” in her tutoring of first and second graders. What’s that old saying… you are busier retired than when you worked!

We are on our last school year of not having both boys in school all day...The boys arrive before 7 am and after breakfast and a little play time, Zach heads off to second grade. He and Linda study spelling and vocabulary words in the car on the way to school. Karate is the sport of choice for Zach and he just tested out of the 6 year old program and has started Beginners with longer classes and lots of new things to learn. He is also playing flag football this fall and Derik is coaching his team. Zach is also in his second year of Cub Scouts with his den leader (guess who), Derik. He had a great time last summer going to a basketball camp, a cooking camp, and scout day camp. Jeffery is going to preschool five mornings a week. He spends the afternoons with Grammy and Papa. Jeffery is currently taking swimming lessons at the Y and has begun Suzuki method piano lessons. Jeffery taught himself to read and loves to read jokes to Linda when they are in the car. Jeffery enjoys playing soccer and went to a camp last summer and a soccer program at his church. We know all this about our grandchildren because we are the transportation for many of their events. We are so blessed to be involved in their lives.

Brent and wife, Christine, have lived in Chicago for almost one year. Their first Winter in the Windy City did not have lots of snow but the gray days were a big change from sunny Charleston, SC. Linda and her high school friends visited Chicago and enjoyed visiting with the Paces. Brent is doing quite a bit of traveling in his new job...takes the “L” to work and has become a real city boy! He recently has been playing some music around town… live Karaoke… Can you picture that? Christine will soon be able to give the architecture boat tour as she has entertains out of town guests (I think it is 6 or 7 times and counting!). Her best friend from Charleston just moved to Chicago… she can get her ready for the cold weather and a new wardrobe of boots, hats, and scarves! Christine is also a docent at the Field Museum; she practiced on Linda’s friends last June. Jim and Linda are flying to Chicago for a weekend in November… going to celebrate an early Thanksgiving! Brent visits the adult CF clinic downtown, not far from his office. He has been renting a new treatment vest that runs on batteries. It is quiet enough that he can wear it at work and get in a treatment during working hours, even in an open concept work area. Derik tried the vest out when Brent was in Columbus in August and he is now in the process of trying to get one ( it comes with a hefty price of over $10,000 so you can see how important insurance is).

Derik continues to try to hold on to his weight and do his vest treatments to keep him healthy. He spent a week in Children's Hospital last May having IV meds and adjusting some medications. He still doesn’t have an explanation for the weakness in his legs. He is seeing a new doctor at OSU and after some new tests, he will see him in November to see where to go from here. As you can see from reading the above, he doesn’t let CF or his leg weakness stop him from being involved in his kids’ lives. Misty is busy teaching third grade and also early childhood classes on Saturday mornings. She is the adult involved with the Suzuki piano teaching method. Her niece has moved in with them and is working as a preschool aide at the school Misty teaches at along with doing online classes for college.

CF News and Research highlights:

How do I love thee, CF? (NOT) Let me count the ways!

A little corny, perhaps. But it points to something that may be kind of mysterious. Is there any rhyme or reason to the way CF is investigated or is it, “just find some way to stop this … thing”? Over the years I’ve babbled on and on about new drugs, different DNA tests, salty ocean air, etc.. It turns out there is some method – they are called “Intervention Types”. Here’s a list of (at least) some of them and the number of current and upcoming trials (in 2017) for each “Type”:

2184insA? F508del?

Geneticist speak describing where the defect occurs and what form it takes. Also, the mutations that Brent and Derik have. Linda has one, Jim has the other.

I’ve mentioned before that every CF patient receives one copy of the mutated CF gene from each parent. There are around 2000 known mutations possible in the CFTR protein, but almost 75% of individuals with CF (almost 65,000) have at least one copy of the CF gene with the F508del mutation (including our boys). Both of our boys also have the 2184insA mutation. There are only 219 individuals with CF registered with this gene combination, worldwide. So Brent and Derik represent about 1% of this population or about .2% of the worldwide CF population (est. 70,000-100,0000).

Why am I rambling on about this? I saw a study which show that in Western Ukraine (formerly Galicia), the 2184insA mutation occurs in 7% of the CF population – much higher than the rest of the world. So this mutation probably originated in this area before spreading to Europe and beyond.

So maybe somewhere in the Pace’s, Overholt's, Riggle’s, Whitehouse’s ancestry is some Western Ukraine blood. Just a thought...

We are honored to be a part of the fundraising team for CF. We do this for all the CF kids and adults, and their families. We have seen much progress in research in the past 39 years and we are confident that the funds we help raise will continue to help improve the lives of those with CF and also search for a cure.

Our boys both have their own families now. Please help us keep their husband/daddy around for as long as possible.

We need your donations to help make an end to CF. Here is how you can join our team and help “Set the Pace.” The easiest way 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 made payable to Cystic Fibrosis Foundation

Please donate by December 1, 2017

The Pace Family