Cystic Fibrosis 2018 Letter

In 2018 we celebrated Derik’s 40^th birthday with a Transformer party. We also remembered 1978 as the year that our sons were diagnosed with cystic fibrosis. In 1978, we had never even heard of the disease. Within a 2 week period, around Christmas, both boys were diagnosed with CF. We began a learning journey that continues today. Many of you reading this letter have been on this journey with us for many years...others are just joining us. Jim and I cannot express in words what your support, emotionally and financially, has meant to us over the years. Family and friends - where would we be without you? And so we come to you with our 2018 campaign to raise $6000! We have stepped up our goal from last year and hope you will join us as we raise money to support research and improve quality of life for those living with this disease.

The Family Update

Brent (43) continues to be in good health. Living in Chicago poses many challenges of germ exposure in large crowds. If you were on the Chicago “L” you would probably see Brent wearing a mask. He is diligent with his postural drainage treatments, exercise, and medications. His CF doctors are at Northwestern University and later in this letter you will read about the drug trial he is involved in now. He finds his work as an environmental engineer with BP Chemical challenging (in a good way). Travel in his job has become easier with a new battery powered vest for postural drainage (more on that later in the letter). Brent and Christine have now been married two years and are still learning a lot about their relatively new city. Both of them continue to deepen old friendships and make new ones. Christine has received a certificate in Museum Studies from the Northwestern School of Professional Studies. She is using this new knowledge in her volunteer work at the Field Museum.

Derik (40) has also had a healthy year. There have been no hospitalizations and he has even gained a little weight. Derik has CF induced diabetics so adds this component of the disease to his diet/medication management. The battery powered vest has also given Derik new freedom at home - you might see him cooking or doing the dishes while doing his postural drainage regimen. Derik uses a cane because of leg weakness and neuropathy. There are still unanswered questions about this condition that is not directly related to his Cystic Fibrosis. Derik is changing companies, still in the culinary field. His employerr for the last 5 years (Sodexo) lost the contract at the location where Derik has worked. Fortunately, another company (Compass Group) acquired the contract and hired Derik as sous chef. Misty (Derik’s wife) is in her third year teaching third grade. It is a challenging grade since there is a big state test given called the “Third Grade Reading Guarantee.”

Grandchildren update: Derik’s and Misty’s boys are now almost 6 and 9. Jeffery is in kindergarten and Zach in third grade (He is no longer “Zachary”). Both boys are in Cub Scouts. Zach is playing basketball and Jeffery soccer this fall. Zach continues with karate and has moved up to the advanced classes for his age. He also is in a run club at his school. Grammy and Papa field trips and overnights happen on school off days.

Jim and Linda: We continue to watch grandchildren daily but not as many hours since both boys are in school all day. (but morning time begins at 6:30 am). We both also still read to kids and tutor...and are also involved in our music groups. Linda had a heart attack on March 21 and had a stent placed in her Lateral Anterior Descending (LAD) artery. After 4 months of cardio rehab she is doing terrific. Jim does “off and on” PT for his legs. We both are trying to walk 5 days a week to keep these 70 year old bodies healthy!

And now back to Cystic Fibrosis: Jim will fill you in on the new vest and the research study that Brent is involved in.

A Brief History of CF (with pictures of Postural Drainage and equipment)

As we look back on the past 40 years and more, we want to highlight some milestones in history and research…

• Middle Ages: European folklore from the Middle Ages warned “woe is the child who tastes salty from a kiss on the brow, for he is cursed, and soon must die”. Sometimes the child was even thought to be bewitched.

• 1595: The first description of the pancreas in a child who almost certainly died with cystic fibrosis (CF) is usually attributed to Professor Pieter Pauw (1564 -1617), Professor of Botany and Anatomy at the University of Leiden.

• 1948: During a heat wave, Dr. Paul di Sant’Agnese observed infants presenting with dehydration to a New York City emergency room. This lead to his discovery that the sweat of children with CF had abnormally high concentrations of salt, and validated the ancient folklore of the disease.

• 1962: The median predicted age of survival is 10.

• 1978: Our boys were diagnosed with CF. We were told they would be fortunate to live to be teenagers.

  
  Manual Percussion/ Parent Body Builder

• 1980: The median predicted age of survival is 18.

• 1989: A team of Foundation-supported scientists discovers the defective cystic fibrosis transmembrane conductance regulator (CFTR) gene and its protein product years before the human genome is mapped. This discovery opens the door to understanding the disease at its most basic level.

• 1993: The Food and Drug Administration (FDA) approves Pulmozyme®, which is proven to thin the tenacious, sticky mucus in the lungs and is the first drug developed specifically for CF.

  
  Mechanical Percussor - Boys start doing their own PD

• 2000: The median predicted age of survival is 32.

• 2008: VX-770 (later known as ivacaftor) trial participants show an increase in lung function and improvements in overall health and well being, proving that it is possible to treat the root cause of CF.

• 2017: Clinical trials of tezacaftor (VX-661) combined with ivacaftor demonstrate positive results not only for people with two copies of the F508del mutation, but also for those who have one F508del mutation and a second mutation that results in residual function.

  
  The Vest - hands free

• 2018: Most patients with CF should live into their 30’s. Average life expectancy rates vary from about 37 to as high as 47. However that is probably little consolation if you are waiting for a lung transplant.

• 2018: Brent is in a clinical trial for a 3 drug combination (VX-445, tezacaftor, and ivacaftor) for people who have one copy of the F508del mutation and one copy of a minimal function mutation (gene changes that leave the CFTR protein minimally functional).

• In the last few years a new improvement to “the vest” has become available. The new vest (designed by a CF patient) is completely self-contained. It contains 8 motorized oscillating discs to “percuss” and vibrate the chest walls and is battery-powered. A patient using this vest can move around the house, or go outside, or go camping. They are not tethered to a heavy compressor and don’t have to worry if the power goes out. Better have good insurance, though. They cost around $15,000.

  
  CF patient designed battery operated vest

The Silver Lining of CF – A CF carrier (Like Jim or Linda) may have some resistance to both typhoid fever and tuberculosis. This is similar to the protection a carrier of sickle cell anemia has against malaria.

This list of milestones will only continue to grow with added research dollars. We are no longer involved in the day to day routine of medications, postural drainage, doctor appointments, hospital stays, etc., that each CF patient and his/her family is involved in. But we support all those families, caregivers, children and adults with CF. It does “take a village” to keep the research going towards a cure and to improve the quality of life for those with this disease. Join “our village” as we look to a bright future. YOU make a difference. Please make your tax-deductible donation by December 6. We will keep you updated on our progress as we journey together in the cystic fibrosis journey!

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 6, 2018

Many thanks from our hearts,

Jim and Linda Pace

and the rest of the Pace family (Brent, Christine,Derik, Misty, Zach, and Jeffery)