Cystic Fibrosis and Me- A CF Short Film

Here is a short-film from my Fibro Casey who lives out in the great state of Texas. This film documents his daily morning routine and does a good job of portraying a typical morning for may of us CFers. Make sure you watch until the very end, my favorite part is one of the last scenes and it had me laughing out loud (when you see his feet on the ceiling you'll know what I'm talking about).

**Sickboy does not endorse anything in this video as medical advice...although I have told many woman in the past that I'm a brain surgeon (on my days off from being a rocket scientist)**

Hypertonic Saline Solution (7%)

I pulled this cool article about Hypertonic Saline (HTS) from Cystic Fibrosis Worldwide. I thought it was an pretty interesting read and I learned some things about 7% that I had never known before. Also, keep in mind that it is an older article so some of this stuff may seem a little dated. Enjoy!!!

About Hypertonic Saline

Hypertonic Saline (HS) is just a strong salt-water solution. The concentration used is typically about 7% salt - that is, about twice as salty as seawater — although sometimes weaker or stronger concentrations are used. Hypertonic saline can be turned into a mist by a simple device called a nebuliser. The mist can then be inhaled into the lungs.

How Does Hypertonic Saline Help in CF?

When Hypertonic Saline is inhaled into the lungs, the body tries to dilute the strong salt concentration. The cells lining the airways are triggered to release water. This restores the layer of moisture lining the airways, which helps the mucus to clear the same way it does in healthy lungs. You could think of it as the lungs “flushing out” the stagnant, infected mucus.

Studies done during the mid 1990s showed that inhaling nebulised Hypertonic Saline temporarily increases the speed at which mucus is cleared from the lungs. Subsequent short-term trials showed that inhaling nebulised Hypertonic Saline on a regular basis (usually twice per day) improves lung function in PWCF.

What was the recent trial about?

Although the short-term trials were favourable, it was not known whether Hypertonic Saline is safe and effective when used for long periods. The National Hypertonic Saline Trial tested 164 stable subjects with CF aged at least 6 years of age. These subjects were randomly allocated to inhale either 4mL of Hypertonic Saline (7% saline) or 4mL of placebo (a very weak salt solution — less than one third as salty as sea water). Both solutions contained an inert substance to disguise the taste, so subjects didn’t know which solution they were inhaling. The subjects inhaled their allocated solution twice daily for 48 weeks. They kept up all their other standard therapies, and received whatever additional treatment they normally would whenever they had a flare-up. Patients were checked regularly for signs of improvement, as well as for any possible adverse effects.

What did the trial show?

The main outcome of the trial was lung function — a breathing test, which measures how well the lungs, can shift air in and out. The subjects who were inhaling the Hypertonic Saline had a modest rise in their lung function at the first measurement point (that is, after 4 weeks). This improvement was maintained until at least the last measurement point (that is, 48 weeks).
A much greater effect was seen on the flare-ups of lung infection. The subjects who were inhaling Hypertonic Saline had far fewer flare-ups. The flare-ups they did have tended to be milder, not lasting as long. Although both groups of subjects used a lot of antibiotics to try to prevent flare-ups, those inhaling Hypertonic Saline needed significantly fewer “extra” antibiotics for flare-ups.

Those subjects who were inhaling Hypertonic Saline also reported improved attendance at school, work or other usual activities. On average, those inhaling the placebo missed 17 more days of school/work than those inhaling Hypertonic Saline. People inhaling Hypertonic Saline also felt like their overall health and quality of life was better.

Were there any adverse effects?

Hypertonic Saline can cause the airways to narrow — just like they can do in asthma. In the National Hypertonic Saline Trial, all subjects took a drug to open their airways, called a bronchodilator, just before each dose. This was very effective in limiting the amount of airway narrowing. For this reason, it is strongly recommended that:

• people with CF take a bronchodilator before each dose of Hypertonic Saline and that
• people with CF are supervised when they inhale their first dose of Hypertonic Saline.

Their lung function should be measured before and after the dose, to check that the bronchodilator is strong enough to prevent significant airway narrowing.

Those subjects inhaling Hypertonic Saline also reported more coughing during and immediately after their inhalations. Thus, coughing should be expected when using Hypertonic Saline. However, the coughing tended to settle within the first few doses, the first few days, or at the most, the first few weeks. The coughing was severe enough to prevent people from using Hypertonic Saline in the longterm in only a very small number of cases. In practice, a lower concentration of saline may be more tolerable for these people.

The lungs were also assessed very closely for signs that the constant inhalation of Hypertonic Saline was causing more inflammation in the lungs. There was no evidence of this at all. Also, the use of Hypertonic Saline did not affect which bacteria were found in the lungs, nor their concentration in the mucus.

http://www.cfww.org/pub/english/cfwnl/7/619/Hypertonic_Saline_Research

Thankful for Kevin Foster

Today, I'm very thankful for the life of a friend of mine, Kevin Foster. Kevin died earlier this week and although I'll miss having him around during my hospital stays, I know that he was ready to go Home. Some of you may be thinking that this is a sad blog, but it is not. It is a blog of joy for what Kevin was and for what he did for me in my CF journey.

I met Kevin many many years ago when I was just a kid. He was probably around 30 or so when I met him and I just remember feeling so inspired by his zest for life. Now, let me also say this, Kevin LIVED and when I say lived, I mean it (I feel comfortable talking about this stuff because Kevin and I had talked to lengths about it). Kevin lived a life that would have killed off most "normal" men in their 20's. Kevin drank. Kevin partied. Kevin went to prison. Shoot, Kevin even smoked. This probably sounds crazy, but Kevin gave me A LOT of hope.

I remember thinking as a teenager, "If Kevin can live into his 30's and NOT take care of himself, imagine what I can do if I commit to treatments and exercise". Then when Kevin got into his 40's, it just gave me more motivation. He used to joke around a lot about "living on borrowed time" and that he "should have died decades ago". I know he just looked at it as playful banter with me, but what he didn't know was the confidence it gave me to take control of my life.

This is also a blog to remind all of you to take your health seriously and make wise choices. For various reasons, Kevin was denied a lung transplant, twice. Transplant boards don't look too highly on those with a reckless past and possible drug and alcohol addictions. Everybody knew that Kevin wasn't a good candidate and although he never said it out loud, so did he.

The last time Kevin and I spoke, no more than a couple of weeks ago, he was ready. He gave it the "old college try" (for 48 1/2 years) as he put it (when he was born they said he'd live till 5). He was ready to move on and was 100% comfortable in doing so. My last moments with Kevin were perfectly tender as we held hands and said a prayer. It wasn't an appropriate time to thank him for showing me that I could take CF head-on and that I learned so much from his accomplishments (and mistakes), but I told him I loved him as I left. I honestly thought that I would get the chance to see him again and tell him "thank you". I never did get that chance.

So here it goes Kevin. Thank you for being you. You were no angel, but you put a smile on my face every time I saw you. You were no saint, but you had one of the most loving hearts that I have ever been around. You were in no way a "model CFer", but in a strange way, you showed me how to be one. Most of all Kevin, you were a true friend to me and although I didn't say it before, I'm saying it now, THANK YOU. I love ya brother.

Stick with Exercise!!

I wanted to highlight a special fibro in the CF community named Mike Price. This dude has incredible determination and is out to do things that have never been done before by members of the CF community. If you ever get a chance to talk with him, you'll be super impressed with his positive attitude and go get em spirit. He's the perfect role model for younger (and older) fibros and cysters out in the community!

He recently responded to a post on CysticLife about exercise and whether to limit it because of CF. This woman's doctor basically said that exercise was different for those of us with CF and that we shouldn't "push it". HOG WASH!!!!

Here's Mike's response:

I'm currently training for an Ironman 70.3 triathlon. A 1.2 mile swim, 56 mile bike ride, then 13.1 mile run. It's the best thing that could have ever happened to me. When I first started swimming, riding, and running it really sucked because I had no endurance and I got winded very quickly. It takes time and it can be painful but you will get stronger you just have to stick with it.

When I was in high school I thought the mile would be the death of me and I'd never run anything further than that in my entire life. Last September I ran a half marathon in 2:31:43, it's not the fastest time but I still beat a lot of people.

Even though we have CF we can still do anything anybody else can do our training is just going to be a little harder and we'll have to train a little longer, but we can do it. The biggest thing you have to learn is patience when it comes to endurance with exercise it takes us a little longer to build it up. Stick with exercise, it will only benefit you.

See what I mean? This guy doesn't take "no" for an answer. He's not going to let some faulty cells in his body determine what he can or cannot do. We need more fibros and cysters like this out in the community! Great thing is, that wouldn't be a far cry if we would just pick ourselves up by the bootstraps and say "enough is enough!!!!".

Top Ten Foods I Order at a Restaurant

I thought it would be fun to construct a list about the top ten food items you will generally find me ordering anytime I go out to eat. One thing you may notice is that some of these items can be found on the same menu at some places. So what do I do when that happens? Well, I tried to put them in order from absolutely most favorite down to least (most) favorite. Luckily, there isn't many times that I am faced with such a large conflict :)

10. Reuben Sandwich- To be honest with you guys, I'm not even sure that I know what meat is actually on a Reuben. Here's what I do know however, I lover sauerkraut. This is generally my fall back order if nothing else on the menu looks good at the time. Oh yeah, I add a lot of yellow mustard too.

9. Turkey Club- This one is always a little touch and go. Sometimes these will come out and be huge disappointments, but other times they will totally hit the spot. I don't do any mayo on it however as I prefer yellow mustard and if I can get extra bacon (for free), I'll take that too.

8. Ham, Tomato, Spinach and Swiss Omelet- No place that I've ever been to actually has this exact omelet, so I often just do the ol' "create your own". I usually go light on the cheese and I can switch to cheddar or provolone on a moments notice :)

7. Shredded Beef Chimichanga (enchilada style)- If I go to a Mexican restaurant, this is what I'll get 9 times out of 10. If it's a restaurant that I've never been to before, it will be 10 times out of 10. If a Mexican place can't make a good shredded beef chimichanga, then I say it's "no bueno".

6. Chicken Tortilla Soup- This is the only thing I have ever gotten at a restaurant around these parts called Islands. They are known for their burgers, but they have all you can eat chicken tortilla soup for like 5 bucks. I've tried many many times not to get it, but it just ain't happening. I'll also order this wherever I am if it happens to be their soup of the day.

5. Fried Zucchini- I actually got hooked on these at University Medical Center in Tucson. The cafeteria used to have these on hand at all times, so I would go down with my food pass and order a bunch with ketchup and cheese (not mixed together) to dip them in. The hospital no longer carries them, so my tune-ups in the Hole just aren't the same anymore!

4. Chicken Caesar Salad- I wouldn't touch salad until about my Sophomore year of college. I thought that a bed of lettuce drenched in anything just wasn't for me. That is, until I gave caesar a chance. Now, I will ONLY get a chicken caesar salad at a handful of restaurants that I go to, namely Cheesecake Factory and California Pizza Kitchen.

3. Chili Cheese Fries- I LOVE chili cheese fries!! It really doesn't matter where they're from either, I'll eat them. This was another food item that the hospital USED TO carry and I fondly remember hanging out with the RT's in the cafeteria at around midnight scarfing down a couple orders of these delightful treats.

2. Chicken Parmesan- This is my go to at any Italian restaurant. It's good pretty much no matter what and I can't think of a time I was disappointed after ordering it. I love chicken. I love marinara sauce. Therefore, I love chicken parmesan.

1. Chicken Fried Steak (breakfast or dinner)- If I'm eating at a breakfast spot and they have chicken fried steak, you can almost bet your bottom dollar that I'll be getting it. If they have it at a dinner spot served with mashed potatoes and corn, you can count me in 99 out of 100 times. I think my obsession with cube steak breaded and fried goes back to my Furr's or Luby's Cafeteria days.

So that's it! How does your list compare? Is it as chicken heavy as mine?? I'd love you to fill me in on your favorites!

Early Thoughts on Marrying a CFer

I was contacted earlier this week by a girl that is dating a CFer. She asked how I deal with everything that comes along with dating someone with CF, namely, the possibility of sickness and death. It was a good opportunity for me to put into words what I've thought for so long regarding that issue. Ronnie and I talked very early on about those possibilities and I had a very set way I required myself to think about it all before I would marry Ronnie. Here is my exact response:

"Let me tell you, first and foremost, I am still learning how to navigate all of this myself. Ronnie and I have been together for a little over 1.5 years, so I am relatively new to the game. But it is something I am getting comfortable with.

Here are my thoughts on all of it. Initially, it was scary. Ronnie and I talked very early on about the possibilities. We talked about the uncertainty that comes with CF, and not only death, but the hospital bouts, the good and bad days, etc. I learned early on that CF was something he felt was a blessing in his life and I would need to adopt the same perspective if this was ever going to work. I then began to think about life as a widow. As in, if Ronnie were to die in the early part of our marriage, how would that be for me. How would life look? How would I choose to move on? Would I be able to pick myself up? Would I be happy? These were all important questions for me to answer. Ronnie and I truly believe he is here for a purpose and that he has CF for a reason, and when he is done carrying out his purpose, he'll die. Ronnie has been comfortable with the thought of death and with God's plan for his life, so I knew that if I were his wife, and lost him, I would NEED to continue to have that attitude. I told him that I wouldn't marry him unless I KNEW that I could stand up the next day, put a smile on my face, and look people in the eye when telling them that Ronnie's life was just as it was supposed to be and that I trusted God's plan. So I quickly worked to get my thinking aligned with that so I knew that I could carry on Ronnie's attitude after he died. I don't know if that makes any sense, but that's where I am with all of it.

I still struggle. I cry at the thought of it. I get choked up when I think about it. I get choked up talking to others that have lost a spouse to CF. But I truly feel any amount of life with Ronnie is worth it to me. There is a huge religious aspect of this for me and Ronnie, and I'm certain that helps with my perspective. I don't know where you stand on religion, but I know that it is what really brings me peace with all of the uncertainty. I know that I'm being looked out for and watched over, so I feel as if everything that comes my way, good or bad, in sickness and in health, it is all part of a plan."

Not So Bright and Shiny

I wanted to spotlight today follows the life of a little boy named Conner with CF. It's written by his mother Sarah and is called Not So Bright and Shiny. Sarah considers herself very blessed to have a boy like Conner and knows that she was chosen as a CF mom for a reason. They recently got back from a trip to Hawaii in which Conner got to experience sights and sounds that his mom could have only dreamed for him. I love happy and positive blogs from the CF community. Here is their journey in her words:

Trip of a LIFEtime

Gosh...I'm not even sure where to start. my heart is swelling with the love that was shown to us this past week, that will live with us our entire lives. There's simply too much to tell. The stories of generosity, support, and love from family and complete strangers alike. This trip far exceeded our greatest hopes and Conner's greatest dreams. Pictures really say it all, and we took, almost 900 of them on our own cameras. But the grandparents took more as well.

He saw whales, whales everywhere...he saw lava...he went in a helicopter, he went swimming with the dolphins and fed them fish, he received a lei greeting, he swam in the ocean and pools, he saw a hula show, he went to an authentic luau and saw the fire dancers, he saw his dad and mom do the hula on stage, he saw his papa attempt to blow a conk shell to start the luau, he saw what humidity does to his baby brothers hair (CRAZY!), he went in the cockpit of the hawaiian airlines plane and learned to fly, he got the pilots REAL pilos wings (not fake ones..his own real one), he saw a mongoose, he saw plenty of beautiful flowers, he saw black sand, he saw spinner dolphins and bottlenosed dolphins, he went on a whale watching cruise, he saw the entire island by helicopter, he joined the thousands of locals and travelers who write their name in the black lava with white coral rocks by writing out his name to be there for years to come, he had a Hawaiian pastor pray a healing blessing over him, he left his footprints in the crystal white hawaiian sand, he buried his dad in the sand at hapuna beach, he picked out gifts for his friends, he had time for lots of hugs and kisses for his family, he saw water as blue as he'd ever seen, he saw Gods beauty and grace everywhere, everyday, and every minute.

I can't even begin to say thank you to those who helped us get there by prayer, support, money donation and grace. Conner received over $530 from people we met on the island, when they met him and heard of his story and struggle. People were just so generous to our family. Their generosity made his wish of swimming with the dolphins come true, another persons generosity allowed him to buy the hawaiian airplane he had his heart set on, another generous gift allowed us to be able to go to the authentic luau, another gift bought us meals for the airplane and tonight for dinner just the 5 of us. he was able to get and do everything he wanted. Yet he shared with his brothers and family. His heart is so generous.

There simply aren't enough words in this world to describe the way I feel about this trip. The way we all feel about this trip. How greatful I am, how I am the luckiest woman in the world that I was chosen to be his mother, how blessed I am to be able to hug him and kiss him whenever I want to...to know he's ours, if even for just a moment longer.
No, I'm not a dancer, but to fulfill his wish of me doing the hula in front of an audience was a no-brainer for me. I got to see him smile because of that. Who cares that I looked ridiculous. To see the smile on his face for getting to bury his daddy in the sand which he's been talking about for weeks was heart warming.
Yes he struggeled a bit to breathe, sure he wasn't feeling very well for most of the trip, and of course he missed out on some amazing island food because he was too ill to eat but oh my gosh...he did it! what an amazing journey. He got to do it! and he LIVED every minute of it to the absolute extent of his abilities. He cherished it. He loved it.

there's simply just too much to say, too many stories to share. and simply not enough time or even the words to speak it aloud...just know that God was ever present on this trip, I felt him from the tips of my fingers to the tippiest of my toenails. He made this possible. He brought these amazing experiences to reality and planned them out so perfectly. because he loves us. because he's there for us. because thru all our struggles these past years he's proven to us without any doubt that he infact, will NEVER leave us. and we, as a family will never be the same again.

simply amazing.

If you get a chance today, stop by Sarah and Conner's blog and say "aloha"!