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??

My Equipment

A lot of you have been asking me lately about what equipment I use in terms of a compressor, Vest and neb cleaner*. I thought I would re-post this just in case some of you were wondering but didn't want to bother asking.

My Vest:

The inCourage™ system offers state-of-the-art airway clearance therapy incorporating the most recent advances in high - frequency chest compression (HFCC) science. Demand is growing rapidly as physicians and patients alike express their preference for the advantages of theinCourage™ system. (http://www.respirtech.com/patients/respiratory-products/ICS1-overview.php)

My Compressor:

The durable MOBILAIRE 50 psi Compressor is ideal for use with medications that require more flow and pressure for proper nebulization. (http://www.invacare.com/cgi-bin/imhqprd/inv_catalog/prod_cat_detail.jsp?s=0&prodID=IRC607&catOID=-536885346)

My Portable Compressor:

The Invacare Stratos Portable Plus is a sleek, new portable compressor that will offer speed, ease-of-use and stylish design in one compact package. Small enough to fit in the palm of your hand, it will be ideal for those who desire mobility, expediency and efficiency in an aerosol delivery device. (http://www.invacare.com/cgi-bin/imhqprd/inv_catalog/prod_cat_detail.jsp?s=0&prodID=IRC1730&catOID=-536885346)

My Nebulizer Cleaner:

The Philips AVENT iQ24 Electronic Steam Sterilizer is the most sophisticated steam sterilizer you can buy and the easiest to use. It works quickly and continuously, keeping contents sterile until you need them, giving you more time to get on with your day. http://www.consumer.philips.com/consumer/en/us/consumer/cc/_categoryid_MCC_PREPARING_FOR_FEEDING_CA_US_CONSUMER/

*Oh, yeah, and I don't represent any of these companies nor do I benefit from them financially...my lungs do reap some benefits however!

Other links:

My Medications

My Supplements

My Pulmonary Function Test History

Light Poles, House Hunting and Sushi

On Thursdays I like to reflect on a few things that I'm thankful for just so I'm forced to sit here and think about how good I have it. Don't get me wrong, I generally think about how good I have it every day, but it's nice to get it down on virtual paper once a week :)

Also, HAPPY BIRTHDAY ERIC!!!!!!!! You're the best looking elder statesman that I know! (It's Mandi's dad's 50th birthday today)

Here we go....

I'm so thankful that I've successfully made it through two runs over 1 mile in the last 3 days! It may not sound like much, but after my last hospital stay, it took me a while longer to work back up to any sort of a distance. When working out, I'm always setting markers for myself and trying to approve upon them. For example, on yesterday's run I made it about 3 light poles further than I made it on Monday's run. On Friday, my goal is to run a handful of light poles further. It's all about going forward more than going backwards!

Mandi and I have stepped up our housing search for when we tie the knot! I feel so blessed to even be in the position to buy a home. There are so many people in this world that go their whole lives without ever owning a home. The fact that Mandi and I will be able to (hopefully, fingers crossed) start our lives in a home we chose and purchased, in a neighborhood that we picked out, is just mind blowing to me. Seriously, we are so so so so blessed.

I'm very thankful for being able to spend time with friends yesterday. It's been quite some time since we've hung with friends between being out of town so much and being so busy. We were able to get a small group together and catch up with the going-ons in their lives which was very nice. I'm also very thankful that things seem to be going very well for my friends also! We all caught up over some Happy Hour sushi at Ra which always makes me smile (I'll smile anytime I get something for half price)! Mandi and I are very blessed to have the group of friends that we do :)

Let me hear it guys, what are you thankful for today??

Who's Your Mandi???

Alright, alright, alright!!! I'm into the full swing of things with my workouts and running and thus far we haven't skipped a beat. That's the good news. And the bad news is....NOTHING!! But seriously, I did want to share with you guys my feelings and emotions during my first week of getting back onto the saddle.

The hardest part is obviously just making things a routine again. Going from laying in a hospital bed for a month and then getting right into the holidays doesn't do much for the ol'routine. I had to force myself to get up early this past week and start off every morning with a brisk walk. If any of you are out there and thinking about starting somewhere, I would start here. It is absolutely AMAZING how much mucus I cough up on my morning walk. We're talking gobs upon gobs of the green/yellow/clear super glue. The best part is, it really sets up my lungs for feeling nice and clear for the rest of the day.

We've also been hitting the gym up again. So far we've been circuit training each time cause I haven't written down an actual workout program to follow yet. Circuit training basically means working out each muscle group with lower weight and higher reps in one gym session. We've been switching up the lifts, but hitting all of the main muscle groups, except legs. Why not legs you ask???

Cause they're KILLING me from running. I started out slow last week and didn't push it too hard during my runs. I did some interval running which gave me a chance to catch my breath in-between each "sprint". Even with the ability to catch my breath though it was still incredibly tough. Well this week, Mandi really took no prisoners with me and bumped it up to the next level. We're really pushing for me to do a 5k sooner rather than later, so she challenged me on Monday to run 1.5 miles. HOLY COW! I think the actual distance that we covered was closer to 1.3 miles, but I can tell you without a shadow of a doubt that I couldn't have physically gone any further. I gave it absolutely every ounce of my being. When we stopped, I actually had to grab onto Mandi for fear of passing out. Luckily, I had 1.3 miles on the way back home to get my breath (and consciousness) back :)

So why am I saying all this? Well, I know I sound like a broken record to many of you, but working out and running is HARD. Especially if you've never done it or are just getting back into it. I can tell you that I want to increase my lung function more than almost anybody you know and I have the drive to do it and it's still tough. It certainly helps to have Mandi pushing me and for that I am very grateful. Honestly, I'm not sure that I could do it without her...well, maybe I could, but part of my motivation for taking my health back would be gone.

Do you have a Mandi in your life that can push you? If not, maybe it's because they don't know you want to be pushed. I encourage you to reach out to a family member or friend and let them know how important increased lung function is to you. I guarantee that if you take the first step in approaching your Mandi, they'll help get you across the finish line.

Top Ten Gifts I Wanted for Christmas

I actually wrote down the list of stuff that I could use or would want a couple days (December 21st in fact) before Christmas cause I thought it would be fun to see what I did or didn't get. Keep in mind, I actually didn't ask for any of this stuff. The only way anybody would have known would be through casual conversation or because they saw holes in my underwear :)

This list is in no particular order (gifts I received are highlighted):

Underwear- What do you know, I got underwear for Christmas! It's actually pretty funny why I received new chonies (can guys use that word?) as a gift. Mandi and her mom (Nancy) were folding laundry one day and Nancy held up a pair of my boxer briefs that had a baseball sized hole in the back and said, "Well, I know what I'm getting him for Christmas!" As they made it through the rest of the laundry it became pretty apparent that I needed some as I don't think I owned a pair without holes. Ventilation, right guys??

Germ Guardian- I've been using a baby bottle sterilizer to clean my nebs, but I was getting annoyed with how long they would take to dry. More than a couple of CFers have suggested the Germ Guardian (uses dry heat to clean baby toys) to me as a way to speed up the drying process. Guess what? I got one for Christmas!!!

Fizzy NAC- I've heard too many good things about Fizzy NAC not to give it a shot. I did not get any for Christmas, which would have been a complete shock if I had, so I will be ordering some sooner rather than later.

Hair clippers- Having a shaved head is one the the easiest hair styles around, but it still requires some up keep. My hair grows super fast so I really should be shaving it once a month. Problem is, I'm too cheap to pay 15 bucks a month to get my head shaved. We tried using a shaver that Mandi has, but I ended up having long hair, short hair and everything in between :)

Brita Water thingamajig- This is one of those things that every time I pass one in the store, I think to myself, boy, that would be nice to have. But again, cheap ol' me doesn't want to give up the 30 bucks to get one. I actually did end up getting one for my brother though as a present :)

Pots and Pans Set- Mandi and I like to cook (although we need to start doing it more) so we know that we're going to need some good pots and pans for down the road. Nancy and Eric (Mandi's parents) got her a nice Iron Clad set for Christmas!!! Nancy uses a website that carries imperfect sets for a fraction of the cost. We sent a couple back because the coating looked a little off, but they'll send us replacements shortly!!

Beach Cruisers- Mandi and I had such a great time riding beach cruisers in California that I figured it would be nice to have a set here. Plus, I'll be able to sneak in some exercise without really thinking about it. At some point this year I'll have to spring for some.

New shower head- My shower head has been duct taped on for quite some time now :) It actually doesn't bother me at all so I don't plan on replacing it. That's the joys of not having a wife around, just duct tape it and it's fixed! Won't be able to do that much longer!!!

Video camera- I didn't expect to get this for Christmas, but it's definitely something I want. I love to put videos on the blog and we'll need a nice video camera for CysticLife stuff. Mandi and I have talked about getting one for each other (we didn't exchange gifts so we could save money) so we may be doing that early on this year.

Cold weather running shirt- It's getting a little chilly outside (Arizona standards) during my runs, so I figured that a cold weather running shirt would be nice. I prefer to run with no shirt at all, but I tried that the other day and the cold wind stung a bit :) Time for a shirt!

I would be remiss if I didn't mention one other gift that I got for Christmas. My mom and step-dad got me a single serve coffee maker. The type that you put little capsules in for a single cup of coffee. They both know how fanatical I am about coffee, so it was definitely the perfect gift!!!

Holiday Highlights

HAPPY NEW YEAR!! I hope you all had a great holiday season. My mom left Saturday, my dad left Sunday, and today it's back to the grindstone for me. Before saying goodbye to my favorite time of year, I thought I'd relive some of my favorite moments with you all, in a photo montage I like to refer to as my holiday highlights...ENJOY!

A tradition started last year, Ronnie gives "suggestive" funny photos to my parents. This year, he was gifting himself to them as the newest addition to the family...ahh nothing says Merry Christmas like your future son-in-law pretending to be naked in a box!

I like to call this "scrapping paper". I "scrapped" most of my gifts this year. What is scrapping you ask? Take all the little random scraps left from cutting real sheets for other gifts and put them all together...Taaadaaa!

We realized on Christmas eve that we were short one stocking. Ronnie cheerfully said, "just give me a sock!" "NO WAY JOSE," I thought to myself, "EVERYONE NEEDS A STOCKING!" So 30 minutes later, he had one. Made for him by his very own little seamstress. **Please note the Christmas tree detailing with the fancy button on top!

Christmas morning! We have a photo of us sitting on a staircase EVERY year since we were born.

My parents gave me a BEAUTIFUL pearl ring to balance out my ring fingers...I think it was my dad's secret ploy to remain just as much the man in my life as Ronnie :)

Ronnie got a TON of games from his family for Christmas...but this one was my personal favorite!!! **Please note the cover of the box.

Not too long before 2010!! Ronnie was really excited about the new year ;-)