Cystic Fibrosis Association of North DakotaNOT AFFILIATED WITH CYSTIC FIBROSIS FOUNDATION OR THEIR NORTH DAKOTA CHAPTER

VOL. 30 January/February 2014 NO. 4

Logan Hahn is CFA’s Ambassador for 2013-2014. At 8 years old, Logan is an energetic, happy young lady who has learned how important it is that she works together with her parents and her medical team in daily doing her part to fight cystic fibrosis. As a result, Logan is not only maximizing her own health, she is serving as an example for others.

Living with CF is a challenge. CFA exists to help Logan and others with cystic fibrosis in their day-to-day battle to be the best they can be in fighting the disease.

If you could help her and others with cystic fibrosis today, would you do it? Is your heart telling you to help her breathe?

This is CFA’s second year participating in Giving Hearts Day, sponsored by The Dakota Medical Foundation and the Impact Foundation. You have an opportunity to help Logan and others with cystic fibrosis on February 13, 2014 by donating online to the Cystic Fibrosis Association of North Dakota at www.impactgiveback.org.

Your online contribution will be secure and will be credited to CFA at that site.

Your contribution supporting the Cystic Fibrosis Association of North Dakota is multiplied on Giving Hearts Day. Donations up to $4,000 will be matched, and all money donated will be spent helping area young people with cystic fibrosis.

Won’t you join us? Help us to help Logan and others who are doing their best to beat this disease. You will know you’ve done the right thing when you experience that feeling of satisfaction and joy after you’ve contributed. Please try it.

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Here’s how it’s done. To join the fun of this 24-hour ONLINE giving campaign, simply follow these steps on Thursday, February 13:

Go to impactgiveback.org

Click on the “donate what you can” tab

Choose Cystic Fibrosis Association of North Dakota

Click the red “donate” button, and give what you can!

Last year we raised over $ 6,000 on this one day. This year we are aiming for $10,000. Think of how much medication $10,000 could help pay for. Please partner with us on February 13.

TO MAKE A SECURE ONLINE DONATION, GO TO: impactgiveback.org

MemorialsGiving memorials and honorariums are special ways of remembering and honoring someone who has made a difference in your life. The idea of helping someone else by honoring these individuals extends the positive impact of their lives in our world. Our thanks to those who remember…and to those they remembered.

Each Christmas, area Taco John’s stores offer the public an opportunity to contribute to help young people with cystic fibrosis through their “Nachos Navidad” promotion, their “Whiplash” ornament promotion and through donation boxes. Receipts from this year’s promotions are beginning to come into CFA’s office as this is being written.We appreciate their giving spirit which has extended since 1998 and raised more than $160,000 to fight CF and to help area young people with the disease. Although Christmas season has passed, please stop in to your neighborhood Taco John’s and while you are ordering lunch, thank them for their community spirit and their willingness to help young people with cystic fibrosis.The programs supported by money raised in this manner include help with medications, therapeutic equipment, lung transplants, college scholarships and support group meetings, among others. Without their help; without your help we could not offer the assistance we are privileged to provide through these programs. Thank you Taco John’s.

Taco John’s Continues to Support CFA

Our thanks are again extended to area friends for the ongoing giving taking place that help us to provide the programs serving our young members with cystic fibrosis. This is the time of year when CFA dedicates substantial amounts of money to college scholarships for its members with CF who are attending college. CFA tries to provide some financial relief for young people with CF who in addition to attending classes, studying, and taking part in college activities, must find time each day for chest physiotherapy, inhalation therapy and taking meds. CFA hopes to “even the playing field” a little with its scholarships for members with CF. We receive thanks from members for these programs, and it is our turn to pass along that appreciation. Donations to CFA help us to provide this service to our members.

Many of the donations received during the past two months have been as a result of holiday donations received through the solicitation envelope included in the November-December issue of the newsletter. Some of the more notable donations are included below together with the donations of other good people who give for other reasons.

Western Steel and Plumbing gave a $500 holiday donation as did Kirk and Nancy Pandolfo. Richard and Belinda Zink donated a holiday $100 as well.

Best Buy, Inc. gave CFA $1,000 through its Tag Team award for their employees who volunteered at the Bismarck/Mandan CF Golf Classic in June. Fargo’s Amvets Post #7 donated $200. The Knights of Columbus from Drake, ND donated $270.27 to CFA and the Hawley (MN) Area United Fund donated $300. Tesoro Refinery of Mandan, ND donated $1,500.

The Garrison (ND) Community Chest contributed $200 in a holiday donation. Megan Gundtstrom made a $100 holiday contribution as did Dan and Phyllis Lien, Andrew and Ingrid Schneider, Wesley and Judy Belter and Verne Goeppinger. Kevin and Kimberly Karls made a $2,000 holiday donation and Norman and Deborah Wentz contributed $100 through the same holiday opportunity.

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Foundations, service clubs, corporations, cooperatives, local businesses, public employees, schools, families and individuals recognize the importance of helping young people with cystic fibrosis. Your creative methods of remembering young people with cystic fibrosis are much appreciated. We will continue to help them - with your assistance. Thank you to all!

Don’t forget that donations may also be made at www.cfanorthdakota.com.

Dean & Sylvia Fatland in memory of their daughter, Jan

Larry Leslie in memory of Albert Sandberg

Larry Leslie in memory of Betty Leslie

Dean & Sandra Swenson in memory of Lil Sven Carlson

Rhonda Haugen in memory of those special people who’ve lost their fight with CF

Harold Blume in memory of Clarice Rodacker

Mona & Michael Johnson in memory of Ryan Thorson

In Honor Of

Lon & Roxanne Smedsrud in honor of Chelsey Smedsrud Haukos & Sawyer Ann Haukos

Donations Directed Toward Ongoing Need

New Drug Approach Could Lead to Cures for Wide Range of Diseases

December 9, 2013 – A team led by a longtime Oregon Health & Science University researcher has demonstrated in mice what could be a revolutionary new technique to cure a wide range of human diseases – from cystic fibrosis to cataracts to Alzheimer’s disease – that are caused by “misfolded”

protein molecules.

Misfolded protein molecules, caused by gene mutation are capable of maintaining their function but are misrouted within the cell and can’t work normally, thus causing disease. The OHSU team discovered a way to use small molecules that enter cells, fix the misfolded proteins and allow the proteins to move to the correct place and function normally again.

The researchers were led by P. Michael Conn, Ph.D., who was a senior scientist in reproductive sciences and neuroscience at OHSU’s Oregon National Primate Research Center and professor of physiology and pharmacology, cell biology and development and obstetrics and gynecology at OHSU for the past 19 years. This month, Conn joined Texas Tech University Health Sciences Center as senior vice president for research and associate provost.

The team’s work will be published this week in the early online edition of the Proceedings of the National Academy of Sciences. The work was the culmination of 13 years of work on the process by Conn and Jo Ann Janovick, former senior research associate at the ONPRC who is now also at TTUHSC. Richard R. Behringer, PH.D., from the University of Texas MD Anderson Cancer Center, M. David Stewart, Ph.D., from the University of Houston, and Douglas Stocco, Ph.D., and Pulak Manna, Ph.D., from the department of biochemistry/microbiology at TTUJSC, also contributed to the work.

Conn and his team perfected the process in mice, curing them of a form of disease that causes males to be unable to father offspring. The identical disease occurs in humans and Conn believes the same concept can work to cure human disease as well.

“The opportunity here is going to be enormous,’ said Conn, “because so many human diseases are caused by misfolded proteins. The ability of these drugs – called ‘pharmacoperones’ – to rescue misfolded proteins and return them to normalcy could someday be an underlying cure to a number of diseases. Drugs that act by regulating the trafficking of molecules within cells are a whole new way of thinking about treating disease.”

Proteins must fold into three-dimensional shapes in precise ways to do their work within human cells. Before recent discoveries about misfolded proteins, scientists believed that proteins that were inactive were intrinsically non-functional. But work by Conn and others revealed that, when the proteins are misfolded, the cell’s “quality control system” misroutes them within the cell and they cease to function only because of that misrouting. Pharmacoperones can fix misfolded proteins and thus make them functional again.

Scientists had in recent years observed this process in cells under a microscope. The work of Conn’s team is the first time the process has worked in a living laboratory animal.

“These findings show how valuable laboratory animals are in identifying new treatments for human disease,” said Conn. “We expect that these studies will change the way drug companies look for drugs, since current screening procedures would have missed many useful pharmacoperone drugs.”

A wide range of diseases are cause by an accumulation of misfolded proteins. Among the diseases are neurodegenerative diseases like Alzheimer’s disease, Parkinson’s disease and Huntington’s disease. Other diseases include certain types of diabetes, inherited cataracts and cystic fibrosis.

Conn said the next steps will be clinical trials to see whether the same technique can work in humans.

The research was funded by the National Institutes of Health (grants OD012220 and DK85040), the Ben F. Love Endowment, the American Heart Association, the Texas Heart Institute and the Robert A. Welch Foundation.

Source: Oregon Health & Science University. “New drug approach could lead to cures for wide range of diseases.” ScienceDaily, 9 December 2013

December 9, 2013 – An international team of researchers has zeroed in on a protein that plays a key role in many lung-related ailments, from seasonal coughing and hacking to more serious diseases such as MRSA infections and cystic fibrosis.

The finding advances knowledge about this range of illnesses and may point the way to eventually being able to prevent infections such as MRSA.

The key protein is called MUC5B. It’s one of two sugar-rich proteins, with similar molecular structure, that are found in the mucus that normally and helpfully coats airway surfaces in the nose and lung. The other is MUC5AC.

“We knew these two proteins are associated with diseases in which the body produces too much mucus, such as cystic fibrosis, asthma, pulmonary fibrosis and COPD,” said researcher Chris Evans, PhD, an associate professor in the University of Colorado School of Medicine. “We also know that many patients with asthma or COPD have as much

as 95% less MUC5B in their lungs than healthy individuals, so we wanted to see if one of these is the bad player in chronic lung diseases.”

The researchers compared mice that lacked one or the other of the proteins. The animals without MUC5B got sick. Those that lacked MUC5AC were fine. The findings, in a paper co-authored by Evans, other CU faculty members and researchers from several other states as well as Mexico and England, were reported today in the journal Nature.

The paper also noted that the immune systems of the mice without MUC5B failed over time. That made the mice more vulnerable to infections including the MRSA “superbug,” a major source of infections in hospitals and in the community, especially in people whose immune systems are compromised, such as cancer patients.

That has interesting implications for anyone with a runny nose. Getting rid of your mucus may make you more comfortable and may help patients with chronic lung diseases,” Evans said. “But if you block it too effectively, this actually could be harmful in the long run. If a

From Common Colds to Deadly Lung Diseases, One Protein Plays Key Role

treatment gets rid of MUC5B, it may make people more vulnerable to additional infections.”

An oddity of the proteins being examined is that they are encoded in a part of the human genome that is highly variable. Twenty percent of the population carries a DNA mutation that makes them produce about 30 times more MUC5B than normal.

More research is needed to learn whether people with that mutation are more or less susceptible to infections, including MRSA, said Evans, who is in the CU medical school’s Division of Pulmonary Sciences and Critical Care medicine. It’s also unclear what’s happening at a molecular level that allows MUC5B to help control certain infections.

“Knowing the key role of MUC5B allows us now to focus on how the protein works and, we hope, to find ways to help patients with these diseases,” Evans said.

Source: University of Colorado Denver. “From common colds to deadly lung diseases, one protein plays key role.” ScienceDaily, 9 December 2013

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Birthdays

February

Sophia Backlund 2/2Ryan Stroup 2/4Marcia Julson 2/9Riley Evitt 2/17Travis Jahner 2/17Tarah Gackle 2/23

March

Adalyn Zimmerman 3/3Abby Helbling 3/5Mary Dockter 3/11Shawna Gackle 3/12Travis Skuza 3/18Nicole Moore 3/27

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