Can CF Teens and Parents Find Common Ground??

A good friend of the CF community and puppet master Josh Mogren, brought up a great "debate" question over on CysticLife.org that I wanted to get the RSR reader's take on:

I have spoken with a few teenagers who claim that their parents are too overwhelming of a presence in their lives. While this is common place among most teenagers across the globe, what happens when you add Cystic Fibrosis to the equation? Teens feel trapped by their parents because they don't feel like they are allowed to make their own choices about their lives and learn from their mistakes. Most parents I have talked to say the reason that they are so concerned for their children is because they don't see their children doing the best they can with their healthcare and daily routines that would improve their lives. Some have seen other people's children pass away from CF and fear the possibility that this might happen to their child. So they refocus their energy toward making sure their child thrives. The parents feel (rightfully so in some respects) that they have given their blood, sweat, and tears to their children and that CF lives are too precious to learn from mistakes because the results are far too costly. CFer's and Parents....let's honestly discuss...where can we meet in the middle? How can teens understand their parents point of view and earn their trust? How can parents learn to start letting go and trusting their children? Positivity and respectful responses are a must! This is thin ice territory, but it must be traveled on...

Parents, what do you think? What is your responsibility to your child and at want point does that responsibility shift? Teens, when can you begin to shoulder some of the burden for your "cystic life"? What's the best way for your parents to handle this transition?

If you'd like to view the answers over on CysticLife please click here.

My First Ever Chinese Train Station Experience

I ran across this video and I thought I'd share it with you guys. This is from January 2009 about 24 hours before I came down with a CRAZY chest cold that landed me in the hospital for 50 days and started my journey to creating this blog. I think the commentary throughout the video is pretty entertaining, as are the crazy stares we get from most of the locals. Enjoy!!

Thankful for Fun!

Thought I would dig this video out of the archives for Thankful Thursday. I'm always thankful for events to raise money for CF and I'm even more thankful if I get to do fun "commercial" spots for them!

I'm starting to miss my long hair...wait a second, no I'm not.

Put Your Health at the Top of the List!!

I can't stress this enough, working out consistently is tough. I mean, we all have lives. We all have a million things going on. We all have friends to hang out with. We all want to watch TV. We all want to work...ok, maybe I took it too far, but I do in fact want to work! We all have about hundred things that we'd rather do than workout. Here's the problem though, there is nothing that we can put before working out that is going to make a more positive impact on our health.

So what happens when we put all of those other things in front of working out? We're saying loud and clear that all of those other things are more important than our health. We're saying that we'd rather do those things sick, than spend an hour a day working out to do those things healthy. Don't think that's the truth? Then you're lying to yourself. The bottom line is we put things at the top of our priority list that are most important to us. You may have a million other things going on, but like it or not, you are CHOOSING not to take care of yourself. Problem is, choosing not to take care of yourself when you have CF can cost you more than good health.

I'm a big personal responsibility kind of a guy. You can replace all of the "we" or "you" in the last paragraph with "me" or "us" or "I". I haven't been putting my health as my number one priority for a while now. Sure, I'm still going to the gym once in a while. I'm still getting out for walks and occasionally a run. But that's just not good enough. I'm not just saying that either, I'm feeling it, and when I'm feeling it, is when I've definitely had enough.

So today we decided that we were NO MATTER WHAT going to be working out like we should be. It started with a walk in the morning and we had planned for a run or hike later in the afternoon. Wouldn't you know it? Just as we were getting our shoes on to go for a hike, the skies opened up and it started pouring. Now this is Arizona, it doesn't rain but once or twice a decade! It would have been really easy to just throw in the towel and say "hey, there's nothing I can do about that", which believe me, I thought about.

Instead, we packed up and headed to the gym. It ended up being a great workout and I'm of course glad that we forced ourselves to do something no matter what. It feels like such a bigger accomplishment when you push through or even drag yourself to do something.

I would just encourage all of you not to be like me this last week. Put your health as your number one priority. Cause the fact of the matter is, if you're not healthy, you won't be enjoying all of those other things for long.

The Top Ten Foods That Make Me (CF) Bloated

**I've posted this before, but a great discussion about this topic on CysticLife.org made me think to post it again**

I wanted to follow up on Mandi's great post yesterday about bubbly guts and digestive enzymes. I've compiled a list of the top ten foods/drinks that make me bloated...and not just bloated, CF BLOATED. These are very specific items from very specific places, but believe me, pizza doesn't have to be from Peter Piper to make me feel pregnant. I created the list in this manner because it also gives you some insight into 10 of my favorite things to gorge on. In fact, the little number to the right of the item would be how I would rank them in terms of my favorite to consume.

10. Frosty from Wendys (1)

9. Brachs Jelly Beans (3)

8. Red Stripe Beer (10)

7. Fried Steak Fingers from Lucky Wishbone (4)

6. Chicken Planks from Long John Silvers (6)

5. Shamrock Chocolate Milk (8)

4. 1/2 Pound Beef Combo Burrito from Taco Bell (7)

3. Sausage, Egg and Cheese Biscuit from McDonalds (9)

2. Pepperoni Pizza from Peter Piper (2)

1. Chicken Fried Rice from Pei Wei (5)

So what foods or drinks make you the most bloated? Please be as specific as possible because I would love to try your suggestions. I know, how stupid of me :)

**I did not receive any monetary considerations nor do I represent the above food products in any official capacity...unless of course they are interested. There, that should cover my butt from this new law**

We Sure Do Have a Great Life Don't We?

I know that Thursdays are the days to be thankful around RSR, but I've felt so blessed this week that Monday is the new Thursday...at least this week. Here's a little look into all the great happenings this last week that I am so excited about.

One HUGE blessing this week was the opportunity to spend some time with my dear friend and maid of honor, Lenore. Lenore and I went to high school together and we have remained great friends through college. We went to school just far enough apart (I went to Syracuse and she went to America) that we never saw each other. I saw her for a few hours last year, but that wasn't nearly enough. As you can tell, this visit was much needed and we had a great time catching up. She is one of the most amazing people and it was so incredible getting to spend time. She also had her boyfriend, Mo, with her, and it was awesome to get to meet him. He might be one of the only people I've ever thought was good enough for my Lenore :)

Another HUGE blessing this week was the launch of CysticLife and the incredible support we've received. We had a great week in terms of numbers, a ton of unique visitors, tons of page views, and over 570 new members signed up. The numbers are great, but I can honestly say there was

something else I noticed and realized that just warmed my heart. I haven't been a part of the CF community for that long, and I knew that it was a great group of people, but this week brought my understanding to a whole new level. The feedback and support everyone offered us was incredible. We didn't have too many glitches, but any issues we had people were willing to work through with us. Some people had spent hours on something, and then lost it due to a glitch, and instead of being frustrated and upset, they just tried again and told us it was ok. I can't tell you how much anxiety we had over releasing the site to the wolves and I can't tell you how incredible it has been thanks to this great community. I know a lot of you were over on the site, and some of those people that just hung in there with us through the kinks, so I wanted to say thank you. I feel so blessed to be a part of this community!

The wedding plans are coming along also, which is obviously exciting and feels like a huge blessing. As you know, I have the dress and we had the venue picked. This week we have met with an invite person and have samples on the way, looked at bridesmaid dresses, set up an appointment with the florist, got the contract signed for the venue, so we can move forward with tastings. It feels like it's all coming together, and the best part is, I've been so wrapped up in launching CL, I haven't even really been stressed about it (which if you knew me, and my affinity for worrying you'd know that was HUGE).

Life is good. Plain and simple. Sometimes I get caught up in all of the "stress" and to dos, but this week I sat thinking about how grateful I was for the last week and just life in general. Apparently Ronnie was feeling the same way, as we sat in my kitchen, he gave me a hug and said, "we sure have a great life don't we?" So, so true...Life is good!!

Cystic Fibrosis History and Timeline

Note from Ronnie: I cannot guarantee the accuracy of this timeline nor the information presented as I did not write it nor do the research for it. I did however find it very interesting and wanted to share it with you guys. If you see any distorted "facts" or major discrepancies please let me know so I can be further educated on the history of CF. Thanks!

Middle Ages - In medieval folklore, infants with salty skin, a symptom of cystic fibrosis, are considered "bewitched" because they routinely die an early death. "Woe to that child which kissed on the forehead tastes salty. He is bewitched and will soon die." This bit of folklore, handed down through the ages, is an early description of the disease now known as cystic fibrosis. This is one of the earliest references to cystic fibrosis. Folklore from the Middle Ages, from Germany and Switzerland, made this reference. The earliest documented reference in the literature is from 1606, Alonso y de los Ruyzes de Fonteca, professor of medicine in Spain, wrote that it was known that the fingers taste salty after rubbing the forehead of the bewitched child.' This is a clear reference to the fact that children with cystic fibrosis have very salty sweat, due to the abnormal protein manufactured by the cystic fibrosis gene. This protein regulates the movement of sodium and chloride from the body's cells. Sodium and chloride are two components of salt. The sweat glands of a person with cystic fibrosis secrete too much salt, and the person tastes salty. This is also a reference to the fact that in earlier days, people with cystic fibrosis did not live past infancy.

1936 - Fanconi refers to the previously nameless condition as "cystic fibrosis with bronchiectasis."

1938 - Andersen of Columbia University develops the first comprehensive description of cystic fibrosis symptoms. CF also named Mucovicidosis.

1949 - Lowe establishes that cystic fibrosis is a recessive genetic disorder.

1953 - After observing excessive dehydration of cystic fibrosis patients during a New York City heat wave, di Sant' Agnese of Columbia University formally reports to the American Pediatric Society that CF patients secrete excessive amounts of salt in their sweat. This observation leads to development of the sweat test as a diagnostic standard for cystic fibrosis.

1955 - A group of volunteers, including parents of children with CF and physicians meet to form the CF Foundation, headquartered in Philadelphia; among the group is Milton Graub, M.D., a pediatrician and father of two children with CF, who later becomes the CF Foundation’s third president and who remains active in the CF Foundation today; life expectancy for people with CF is 5 years old.

1961 - The CF Foundation establishes the first CF care centers; CF Foundation hosts first conference of CF care center directors.

1962 - Additional CF centers established, bringing total to 30. Predicted median survival increases to about 10 years.

1965 - CF Foundation begins clinical fellowship program; George Frankel, a CF Foundation founder, establishes Guidance, Action and Projection conferences, a fellowship program to bring basic scientists into the field of CF research.

1966 - CF Foundation establishes Patient Data Registry, which tracks the histories of patients treated at CF care centers.

1968 - CF Foundation hosts first National/International Medical Conference.

1972 - First national seminar on needs of young adults with CF held by CF Foundation.

1978 - Headquarters moves to Washington, D.C. to be close to the federal government and the National Institutes of Health (NIH); first nationwide fund-raising event held, called Bowl for Breath, nearly doubling the fund-raising dollars for CF. The number of Cystic Fibrosis Foundation-accredited care centers now total 125.

Early 1980's - Researchers associate organ damage caused by CF with a malfunction of the epithelial tissue.

1980 - Robert J. Beall, Ph.D. comes to the CF Foundation as medical director from NIH.

1981 - Satellite CF care centers for adults were established for the first time; life expectancy reaches 20 years.

1982 - The CF Foundation creates the Research Development Program (RDP) to encourage leading academic and scientific institutions to establish centers of excellence in CF research; Doris Tulcin, Chairman Emeritus of CF Foundation, launches a capital campaign—the first of its kind by a voluntary health agency—to raise $15 million to support the creation of the RDP; Robert K. Dresing becomes volunteer president of the CF Foundation, later becoming the first president and CEO.

1983 - Scientists show that cells lining the lungs fail to properly move chloride into the airways; the Orphan Drug Act is signed into law and provides companies with financial incentives for developing drugs for rare diseases like CF; Frank Deford, sportswriter and novelist, writes the book, Alex: The Life of a Child about his daughter who died from CF at the age of 8; Deford becomes Chairman of the CF Foundation’s Board of Trustees.

1984 - American Airlines, Outstanding Corporate Partner of the CF Foundation, hosts the first Celebrity Ski event to benefit the CF Foundation.

1985 - Life expectancy reaches 25 years.

1986 - The movie, “Alex: The Life of a Child” appears on ABC putting CF in the national spotlight; CF Foundation tops $20 million in fund-raising.

1988 - CF Foundation establishes Cystic Fibrosis Services—a specialty pharmacy—to provide availability, access and assistance with the complex insurance issues faced in obtaining CF medications.

1989 - Francis Collins, M.D., Ph.D., John Riordian, M.D., and Lap-Chee Tsui, Ph.D. discover the CF gene with support from the CF Foundation’s RDP; CF Foundation hosts first GREAT STRIDES walk-a-thon, now a $20 million+ annual fund-raising event. A Team led by Tsui and Riordan of the Hospital for Sick Children in Toronto discover the gene responsible for CF and name its protein product cystic fibrosis transmembrane conductance regulator (CFTR). The gene is mapped to chromosome 7q.

1990 - CF researchers achieve test tube “proof of concept” for gene therapy.

1993 - FDA approves Pulmozyme, the first biotech drug designed for CF, which breaks down thick CF mucus following studies in CF care center network; first gene therapy tested in person with CF—shows gene therapy corrects CF cells in nasal passages; CF Foundation and NIH establish nine gene therapy centers for CF studies; Beall becomes president and CEO of the CF Foundation; The Boomer Esiason Foundation redirects its mission to support the CF Foundation when Esiason's son, Gunnar, is diagnosed with CF.

1996 - The Seattle Breath of Life Gala, which has raised more than $24 million since its inception in 1984, becomes the CF Foundation’s first “million dollar event,” raising $1,002,500 in one night; CF Foundation forms public policy alliance, a grassroots group of volunteers who inform policy decision makers.

1997 - FDA approves TOBI, an inhaled antibiotic, after CF Foundation-supported studies showed safety and efficacy; CF Foundation establishes the Therapeutics Development Program (TDP) with seven centers in the Therapeutics Development Network (TDN); CF Foundation forms Adult Task Force to address specific issues facing adults with CF.

1999 - Bill and Melinda Gates Foundation gives the CF Foundation a $20 million grant for drug discovery; Cam C. Cooper is named Chairman of the CF Foundation’s Board of Trustees.

2000 - CF Foundation-supported researchers complete map of Pseudomonas aeruginosa genome; CF Foundation establishes CF National Bioinformatics Center to help spur discovery of new treatments; CF Foundation awards contract to Aurora Biosciences for up to $46.9 million for CF drug discovery (the largest grant of its kind by a nonprofit organization); CF Foundation establishes Cystic Fibrosis Foundation Therapeutics, Inc. to govern CF Foundation’s drug discovery and evaluation efforts.

2001 - Tom Marsico, CEO of Marsico Capital Management, LLC, commits $25 million over several years in support of the TDP; CF Foundation makes first multi-million award for drug development with Altus® Pharmaceuticals, Inc. to develop TheraClec TM Total, a potentially more efficient pancreatic enzyme.

2002 - Results of CF Foundation-supported clinical trial on azithromycin show that the drug is effective in improving lung function in people with CF; the TDN expands to 18 centers.

2003 - Targeted Genetics, Inc., with CF Foundation support, begins a Phase II CF gene therapy trial that represents the largest and most advanced gene therapy trial for CF to date; CF Foundation tops $150 million in fund-raising; CF Foundation launches Volunteer Leadership Initiative, to bring 2,003 new volunteers to the CF Foundation in 2003.

2004 - Results from Inspire Pharmaceutical’s CF Foundation-supported clinical trial of INS37217 to restore ion transport demonstrate both safety and improvement in lung function in people with CF; pipeline of CF therapeutics in development includes nearly two-dozen potential therapies; CF Foundation recommends nationwide newborn screening for CF at North American CF Conference, which now includes nearly 3,000 attendees; life expectancy increases to the mid-30s.

2005 - Predicted median age of survival increases to nearly 37 years.

2006 - Cystic Fibrosis Foundation Therapeutics-supported studies in Australia and UNC show that hypertonic saline helps clear CF mucus and becomes a therapeutic option.

2006 - The first compound for CF, discovered through high-throughput screening at Vertex Pharmaceuticals, begins clinical trials.

2006 - Inspire Pharmaceuticals begins Phase 3 clinical trials of its innovative denufosol drug to hydrate and clear CF mucus.

2007 - VX-770, a potentiator candidate developed by Vertex Pharmaceuticals, entered Phase 2 testing. This drug may restore the function of the CFTR protein. Vertex also is working to develop corrector candidates, which aim to improve the function of the CFTR.

**The original post was taken from http://www.healingwell.com/community/default.aspx?f=18&m=1247043**

Breastfeeding and Cystic Fibrosis

I can't claim to have a lot of experience on the topic of breastfeeding in relation to Cystic Fibrosis, but recently, a super mom out there, Kacie, gave a great interview that at least partly spoke on the subject. The complete interview is certainly worth the read and I highly encourage you guys to check out the full transcript by clicking here.

Wendy: Since you were exclusively breastfeeding Lucy, did the diagnosis affect this? What did your doctors recommend and what did you do?

Kacie: I had always known that I was going to exclusively breastfeed my children so the decision was made before Lucy was even born. When Lucy arrived, and because she had the blockages, I was not able to nurse her for 2 whole weeks. Imagine my frustration and heart ache to not be able to nurse my child when she cried for me! She never ingested a single thing until 2 weeks old, waiting for her insides to heal up. She was given fluids and nutrients through an IV while I pumped, pumped and pumped, and saved every last drop of milk I could get. I made the NICU nurses write in huge, red lettering, "MOTHER'S BREAST MILK ONLY!" in her file so she would never be given formula. The very first thing that went into Lucy's repaired intestines was exactly 5ml of my milk. She was not to be given more than 5-10ml at a time to insure that the surgery went well. After 2 days of "testing" her new tummy out, we were finally, at last, able to nurse. I will never forget the first time either! She latched on like she had done it before. We were off and running. The doctors were very pleased that I was so adamant about breastfeeding. The recommendations about switching to formula came later. Lucy was not gaining as much as she should have been gaining according to the AAP weight charts. When she dipped below 50th percentile, it was mentioned that we may have to supplement with formula for added calories. I told them that giving her formula would never happen in a million years! I went home that day and started nursing her around the clock like normal, but I added in feedings as well, even when she really didn't even have hunger cues. I became a human pacifier because I refused to put her on formula. Extra nursing worked. She gained enough weight to get above the 50th percentile so Lucy's nutritionist never mentioned formula to me again.


Wendy: How has breastfeeding proved to be a great help in relation to Lucy's diagnosis and her illness in general?

Kacie: I make jokes that my boob has been my best friend and still is! Lucy has been in and out of doctors offices her whole life. Her life is very chaotic and stressful because of CF. More stress than a child should have to go through: blood tests, needles, throat cultures, wearing a vest that shakes her chest and taking up to 9 different medications a day. By nursing Lucy on demand for the last 2 years, we have created a safe place for her to go where she knows everything will be okay. Nursing is her neutral place, a safe place, the quiet place among the storm. I am more in tune with her and her physical and emotional needs because of our nursing relationship. Nursing her for this long has helped her immune system. She is sick less often and when she does get sick it is very short-lived. If I ever get a cold or illness, she has never gotten it from me. She is receiving the antibodies from me that are needed in fighting the infection. I really cannot think of one negative thing to say about breastfeeding Lucy.

Read the full interview at Aruban Breastfeeding Mamas

So what do you guys think? Does/did breastfeeding create a "safe place" for your CF child? Would you recommend breastfeeding to the mothers that are out there reading this?

First Day Out of the Hospital...

...the LAST time I got out of the hospital. I found this video buried in my YouTube account and thought I would throw it up. I often hear other CFers say that, "I'm not going to let CF control me", when I ask them why they won't do their treatments. Funny thing is, that's the BEST way to get controlled by CF, stop or don't do your treatments. Want to really have control over your life? Invest 10% of it really taking care of your lungs and the other 90% will be all yours.

So what do you guys think? Does that make any sense?

Haiti, Rain and Community

What a great week so far!! There is SO MUCH to be thankful for that it's a little tough to narrow it down to just three things...

First, I'm so thankful that Mandi's best friend (and maid of honor) Lenore could be in town with us this week with her boyfriend Mo. They actually came here to Phoenix from Haiti where most of Mo's family lives. I'm very thankful that they were not in Haiti at the time of that devastating earth quake, but I would ask for your continued prayers for Mo's family and friends who lost someone or had their property badly damaged. It's been so nice hanging out with them as they are both very enjoyable people to be around.

I'm so thankful for the nice rain we had yesterday. Now, let me clarify, if we had rain all of the time, I wouldn't be thankful for it. But this is the desert, so it's a pretty rare occasion that we can actually get some rain. Tonight it was coming down that kitties and puppies! There were what seemed like sheets of rain pouring down from the sky. It's definitely nice to have a change from clear blue sunny skies once in a while...although I hope it goes back to being that way today!!

Finally, I'm so thankful for the overwhelming response to CysticLife.org! Many of you have joined us over there and for that I am so thankful. I never in my wildest dreams thought we would have nearly 500 members in just 3 days! The great part is, this is just the tip of the iceberg for where we plan on taking that site and this community. It's so nice to see others buying into our dream and it really validates the last 9 months of hard work :) If you haven't yet joined us, please go to CysticLife.org and sign up for a profile today. See you over there!

Started a New Running Plan!

**I've been getting a lot of questions lately about how I started running and if there was any particular plan that I followed. Well, here it is, and I must say, it REALLY works! Just stick with it and I promise that you'll see great results :)**

So I was back to some running today and I must say, it felt better than I thought it would :) I was fortunate enough to be following the blog of my cysta Sara today after she posted a good running routine which should get my butt back into shape. I'm prone to listen to her since she ran in a half marathon only 35 weeks after her lung transplant! Plus, she is an amazing and awesome person with a super cool husband to match, so why not take her advice right? I started it tonight, but I plan on being on the 2nd week by Monday. And in the effort of full disclosure I did cough up a little bit of blood tonight on the run, but it was very little and mostly streaked, so I'm not worried. I did two sets of 7% pretty close together today so my lungs may be a little irritated, who knows. As long as there's not an increase in blood over the next couple of days, I should be fine.

So anyway, here is the running plan that she posted (courtesy of fitsugar.com):

	Mon	Tues	Wed	Thurs	Friday	Sat. or Sun.
Week 1	Walk 6 min., run 1 min. Repeat 3 times (total 21 min.)		Repeat Monday's workout		Repeat Monday's workout
Week 2	Walk 5 min., run 2 min. Repeat 4 times (total 28 min.)		Repeat Monday's workout		Repeat Monday's workout
Week 3	Walk 3 min., run 4 min. Repeat 4 times (total 28 min.)		Repeat Monday's workout		Repeat Monday's workout	Repeat Monday's workout
Week 4	Walk 2 min., run 5 min. Repeat 4 times (total 28 min.)		Repeat Monday's workout		Repeat Monday's workout	Repeat Monday's workout
Week 5	Walk 2 min., run 8 min. Repeat 3 times (total 30 min.)		Repeat Monday's workout		Repeat Monday's workout	Repeat Monday's workout
Week 6	Walk 2 min., run 9 min. Repeat 3 times (total 33 min.)		Repeat Monday's workout		Repeat Monday's workout	Repeat Monday's workout
Week 7	Walk 1 min., run 11 min. Repeat 3 times (total 36 min.)		Repeat Monday's workout		Repeat Monday's workout	Repeat Monday's workout
Week 8	Walk 5 min., run 20 min., walk 5 min. (total 30 min.)		Walk 5 min., run 23 min., walk 5 min. (total 33 min.)		Walk 5 min., run 26 min., walk 5 min. (total 36 min.)	Walk 5 min., run 30 min., walk 5 min. (total 40 min.)

Once you're up to running 30 minutes straight, try to stick with this four times a week.

My Top Ten Most Frequented Websites

So I spend a lot of time on-line. Generally I will be working, but during my night treatment, I'm usually able to stop by some of my favorite sites to see what's going on in the real world! Here are my top ten most frequented site during the past six months:

1saleaday.com- I go to this site every night before I go to bed mostly just to check out the sweet deals on there. I have actually even broke down and bought a few things as well! I've gotten some Wii controllers for my little brother for free (just had to pay shipping) plus I got a Margarita machine for the Melin's that was 150 and I picked it up for 25. Check it out, you may just walk away for something.

Yahoo.com- For whatever reason, I've always like the way Yahoo presented there news, sports, finance, etc..I generally try to stop by at least once a day to check on the national/world news and the stock market.

Espn.com- I love me some sports. I generally read through as many box scores as I can during my last treatment (except for hockey) just so I can stay on top of team records and who's doing what.

WildAboutAZCats.com- I may love sports, but I love Wildcat sports even more! My beloved University of Arizona will always have my heart, especially the basketball team. Basketball isn't even my favorite sport (football is), but I've always felt emotionally invested in our Basket Cats. This site talks all things Arizona sports and is a great resource for what is happening with our teams.

Twitter.com- I'm not on my Twitter account as much as I used to be, but I still find it a very valuable resource for keeping up with what is going on in certain communities. I stay up with many in the CF and running communities through Twitter and I also tweet many of my blogs and CF news.

Facebook.com- I've met many of you through my Facebook page and I'm very thankful for this wonderful site. It's a great way to keep up with some in the CF community as well as flip through photos until your heart is content. There are also a bunch of good groups out there that serve the CF community that have pages on Facebook. One of my favorites is CysticLife's fan page :)

Movoto.com- I've been looking for a house lately and this site is simply one of the best for how much information it has on it. You can find out just about anything and everything about a house that peaks your interest and they generally have good pictures of the property as well.

TinyURL.com- I post a lot of links to interesting articles I find onto Facebook and Twitter. Since I usually don't want to take up a ton of space with a long URL, I'll shorten it with this free service. One thing I like about them is there is no account and no signing in necessary.

RunSickboyRun.com- Yeah, guilty. I spend a lot of time on this site. I probably should be spending more, but I have a little project going on right now that is taking quite a bit of my time, focus and energy. I really enjoy writing for this blog though and I really hope that it can serve as a source of education, inspiration and hope to many of you!

CysticLife.org- Believe it or not, this site has been taking up most of my time the last six months. We launched yesterday, so it feels real good to "release the site to the wolves" (that would be you guys)! If you haven't signed up for a profile yet, please do, over 300 people in the CF community did yesterday!!!

What are your favorite sites? Any that I just need to add to my daily list?

Who Wants to Dream With Us???

Today is the day that Ronnie and I have dreamed about for what feels like forever. We have dreamed big and it feels so incredible to see our dream coming true. I could not be happier or more excited than I am today, to see where else our dreams will lead us. What am I talking about you ask? Let me back up and give you the full story so you can join in on our dream.

As most of you know, Ronnie and I have worked together for the last 10 months. In April of last year, Ronnie was asked by an incredible couple, the Gettels, to create a web site for the CF community. The charge was undefined, but the possibilities lit a fire under Ronnie unlike anything I have ever seen. His love for the CF community and his desire to support and encourage them fueled his imagination. Our life became one huge brainstorming session. This dream became a reality with the formation of an organization called CysticLife. And we were well on our way after assembling a dream team that consisted of Ronnie, two brilliant web developers, and his beloved (this would be me, Jezzabel prefers unemployment) to write, design and spread the word.

To us, CysticLife (CL) has become a dream not only for an organization, but for a community. CysticLife isn't just a new organization; to us, it's a new movement. We want CysticLife to be a place where people can come when they're down to be picked up, and a place to share your accomplishments when you're riding high. It will be a place to gain knowledge from your peers who have lived it and get it. CL is about becoming comfortable with who you are and what CF means in your life. It is about transforming the CF community into being their own cure. We want to give people the skills to manage their CF and take care of themselves. We want people to go through life with a smile on their face, a can-do attitude and the confidence to take on whatever challenges might come their way.

We have taken our first steps to making our dreams come true. Today, we launched CysticLife.org, a social network (like facebook) made specifically for the CF community. On CysticLife.org people can post blogs, ask questions, maintain a profile, contact others, and search members by age, location and relation to CF. This is going to be an incredible resource, and the best thing is...this is only the beginning.

We are super excited about this site and would love for you to check it out, and hopefully share in our excitement. We truly love and value each of you and your opinions. You never hold back on RSR when it comes to sharing what you think, so we're hoping you do the same for CysticLife.org. We know if you think it's terrible, you'll tell us like it is, and that if you think it's great, you'll join in our on dream. We're really shooting for the latter ;-) !!!

Oh yeah...in my excitement I forgot to give you the link. Here you go: www.CysticLife.org

Welcome to Joshland!!

Are you looking for a new CF/Life related blog to follow? Then look no further than Welcome to Joshland! Josh has got to be one of the most involved and inspiring fibros out there and I thoroughly enjoy the friendship we have built the last handful of months. Recently, Josh has been working on his YouTube page to be a kid friendly CF show of sorts. He has only filmed the intro "episode" thus far, but I'm sure with a little bit of nudging from you (and your kids) he'll be eager to do more. Josh has a BIG heart for kids and it really shines through with his warm and inviting personality. I encourage all of you to check out the "pilot episode" below and stop by Welcome to Joshland after you leave here!

If you didn't think that was enough, check out the talented fibro Josh and a couple fellow cysters contribute to The Breathe Song Event. This song has been a real source of inspiration to many of us in the community and, in my opinion, one of the best CF related materials ever put out to mainstream. Enjoy!!

The Cells of CF

I thought this article did a good job explaining what is going on in the cells of a CFer, cause remember, it's not a lung disease. Cystic Fibrosis is a CELL disease.

Inhibition of Proteostasis Restores Ion Channels in CF Cells

Tuesday January 5, 2010

Our cells are full of enzymes that mediate activities like growth, metabolism, replication,transcription and cell signalling. They are all unique in their structure, and have evolved over time to perform the specific functions for which they are made. Some enzymes are hydrophillic(water-loving, or water soluble) and remain in the cytoplasm of the cell, while others arehydrophobic, thus more lipophillic. The lipophillic enzymes are generally found embedded in cell membranes and tend to have roles such as mediating the transport of small molecules and ions across the membrane. Cystic fibrosis transmembrane conductance regulator (CFTR) is one such membrane protein.

In order to study its structure and function, it is necessary to first purify a protein. Like all hydrophobic transmembrane proteins, study of CFTR is hindered because it is difficult to purify and study in a stable form. However, it has been determined that mutations in the gene encoding CFTR result in an improper protein structure, that folds incorrectly, is recognized as defective by cellular machinery, and is destroyed.

Without CFTR, chloride ion transport in the lungs is hindered, resulting in a mucus buildup characteristic of the disease. Efforts to treat CF by altering the gene or protein structures in patients have not been terribly effective, but last month a study was published that showedrestoration of ion transport could be achieved by controlling the actions of a class of enzymes responsible for proteostasis (destruction of proteins) called histone deacetylases (HDACs). A compound called suberoylanilide hydroxamic acid (SAHA), already approved by the FDA for treatment of lymphoma, was able to restore up to 28% of channel activity in isolated cell cultures from CF patients. The authors, Hutt et al., postulated that by preventing destruction of imperfect proteins, those polymorphisms that are not completely disfunctional can restore some ion exchange capacity in cell membranes.

Full article at http://biotech.about.com/b/2010/01/05/inhibition-of-proteostasis-restores-ion-channels-in-cf-cells.htm

Make sense??