Cystic Fibrosis 2016 Letter

We welcome Fall 2016 with a few changes to our annual Cystic Fibrosis Fundraiser. The bowling event is no longer on the fundraising list of the CF Foundation, but that does not stop 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 the money we collect in donation goes to the CF Foundation - just like it did with the bowling event we participated in for 38 years. Every thing that you give is 100% tax deductible. We set our goal this year to raise $4000...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 and 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 break through came in 1989 when the gene that carries CF was identified. The Paces were already involved by this time, having had Brent and Derik 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 years and many CF patients live well beyond that number. 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 by going to http://www.jimpace.eml.cc/).

Family updates:

We (Jim and Linda) can’t believe it has been almost two years since we moved into our condo in Westerville, OH. We still love the place and we welcome you all to visit us. Each day begins early when our two grandsons arrive. Our schedules of church activities, lunches with friends, volunteering at schools, and OSU classes are mixed in with taking Zachary (7) to school and picking him up (Monday–Friday), and preschool for Jeffery (4) three mornings a week. There isn’t much down time during the week here with Papa and Grammy reading, playing games, going to the park, and having picnics inside and outside! Jim continues to sing as much as he can, take classes at OSU, and serve on the Worship Planning committee and Finance committee at our church. He was excited to show Zachary Saturn and Mars through the kid telescope we have. As Derik was loading the boys in the car one morning, Jeffery looked up and pointed out the constellation Orion and his belt. Jim also did some solar observing at a park. Linda has her retired teacher luncheons, Jazzercise, her Wesley Glen Girls get-togethers, and many friends she tries to stay in touch with. Music is still a big part of her life with her involvement with the Westerville Community Band and a flute trio.

Brent’s big news is that he and Christine were married in Las Vegas on July 1, 2016. The surprise came to their 20 guests who thought they were there for a Bachelor/Bachelorette weekend and not a wedding! We are excited to welcome another daughter into our family. Brent just celebrated his 20th year at BP. He still plays in bands and loves the weather in Charleston, but that is soon to change. The newlyweds are moving to Chicago in December! Brent will still be working for BP but in a new position. He will be working downtown and Children's Hospital (where the adult CF clinic is) is a short walk from his office. Christine works for The Make a Wish Foundation. Her base is their home, but she does a lot of traveling to chapters all over the US and the Make A Wish headquarters in Phoenix, AZ. They are very involved in the Charleston Chapter of the CF Foundation and host a party called “The Champagne Campaign” each spring. Brent’s health has been “status quo” this past year. He experienced a drop in his pulmonary function numbers and worked very hard to discover why. These physical changes also require some mental adjustment, too.

Derik and Misty are busy working parents. Derik continues at the corporate cafe for Express Scripts and Misty is in her second year teaching second grade. They are active in their church and can be found on Sunday afternoons watching their boys play flag football. All the Paces just celebrated the two year anniversary of the adoption of Zachary and Jeffery. Derik’s health concerns with CF have been his weight loss and some minor flareups with infections. At the beginning of October, Derik spent 6 days in Children's Hospital for a condition called DIOS (distal intestinal obstruction syndrome). The previous name was meconium ileus equivalent. (Meconium ileus is the intestinal blockage in newborn infants with CF). About 20% of CF adult patients have this problem and are at risk for repeated episodes (This is the 3rd time he has been hospitalized for this condition). The treatment is a combination of pancreatic enzyme replacement, hydration, and laxatives. Another concern (unrelated to his CF health) - weakness and pain in his legs have him using a cane. His neurologist is sending him to The Ohio State University for further testing.

CF News and Research highlights:

Orkambi: Last year we mentioned the approval by the FDA for the use of Orkambi in CF patients over 12 who have two copies of the most common CF defect (F508del). On September 28, 2016, the FDA approved the use of Orkambi in children as young as 6 years old with two copies of F508del. Almost 40% of CF patients have this mutation combination! Orkambi improves lung function and significantly reduces the rate of pulmonary exacerbations, which can lead to frequent hospitalizations and accelerated lung disease.

Gary Shandling: On October 6, 2016, actor-comedian Gary Shandling gave a legacy gift to the Foundation in honor of his late brother, who had cystic fibrosis. Barry Shandling was 13 when he passed away. Shandling began his work with the CF Foundation in the 1980s and championed the battle against CF until the end of his life in March, 2016.

Another celebrity who works hard raising funds is Boomer Esiason. Boomer is a retired NFL quarterback who played for the Bengals and the Jets. His son, Gunner, was diagnosed with CF when he was two. Gunner is now 25. He writes a blog and podcast on life with CF. The Boomer Esiason Foundation was formed when Gunner was diagnosed and has raised over $130 million dollars.

CFF Opens One-of-a-Kind Research Lab: On September 16, 2016, the Cystic Fibrosis Foundation opened a one-of-a kind cystic fibrosis research facility, based in Lexington, Mass. The lab is designed to identify and test potential therapies for CF, readying them for further development with the ultimate goal of bringing new drugs to people living with the disease.

Operated by Cystic Fibrosis Foundation Therapeutics Inc. (CFFT), an affiliate of the CF Foundation, the lab has a team of 25 employees who focus on finding promising drugs for both common and rare mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. To accomplish this, the lab capitalizes on emerging scientific discoveries related to CFTR modulators, nonsense mutations, gene editing and stem cell research. (Click here to see a short video describing the work at the CFFT)

Drug-Resistant Bacteria and ALX-009: We’ve all heard about the dangers from drug-resistant bacteria. What if there were a way to fight off drug-resistant bacteria, naturally? Guess what? Our lungs already have some of that capability. Well, they do if you don’t have CF. Most individual immune systems include two antimicrobial molecules (hypothiocyanite anion and the protein lactoferrin). These two molecules are absent or deficient in lungs with cystic fibrosis. Initial testing shows a drug containing these molecules (ALX-009) is effective against a number of bacterial species including some especially nasty drug-resistant bacteria that colonize in the lungs! A Phase 1 clinical trial is underway in France to test the safety of this drug.

Wow...so much information to absorb…. There are so many people out there working hard to find a cure and help CF patients live a healthy life. Join us, Boomer Esiason, and others who long for a day when there will be a cure for this disease.

We need your donations to help end CF. Here are the ways you can join our team and help “Set the Pace.”:

Please send your donation by December 1, 2016

From all of the Paces, THANK YOU.

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







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