Saturday, October 24, 2009

New Clinical Guidelines

New clinical guidelines for exacerbations in cystic fibrosis

The American Thoracic Society has released new clinical guidelines, developed by the Cystic Fibrosis Foundation, for the treatment of exacerbations in cystic fibrosis based on a review of the literature on current clinical practices.

“This is the first such comprehensive and evidence-based systematic review of best practices for pulmonary exacerbation of cystic fibrosis,” said Susanna McColley, M.D., head of the division of pulmonary medicine and director of the Cystic Fibrosis Center at Children’s Memorial Hospital and associate professor at Northwestern University's Feinberg School of Medicine. “Until these, guidelines were arrived at by a less rigorous process based on the consensus of a committee of experts.”

When cystic fibrosis patients suffer an acute exacerbation, they undergo an acute worsening of symptoms, which typically require medical intervention. While a prospective definition of an exacerbation has not yet been developed, clinical features are generally well-defined and represent a sharp deterioration in the general condition of the patient, often involving systemic symptoms such as weight loss and lack of appetite, as well as worsening of pulmonary symptoms such as cough, sputum production and shortness of breath. Exacerbations are the most common reason for hospitalization of cystic fibrosis patients.

The guidelines, which were presented at the North American Cystic Fibrosis Conference in October, highlighted a number of common practices in cystic fibrosis exacerbations.

The committee gave guidance on two areas of significant interest to clinicians: synergy testing and the dosing of aminoglycoside antibiotics. In the case of synergy testing—a costly and time-consuming practice of determining what synergistic effects different antibiotics may have when used together against multi-drug resistant infections—was found to have little benefit to the patient and the committee recommended against the routine use of it. In the case of aminoglycoside antibiotics, they found that three-times-daily dosing was no more effective than once-daily dosing and recommended once-daily dosing in most cases.

The committee also affirmatively recommended continuation of two current practices—continuing chronic therapies during exacerbation treatment and airway clearance therapies—both of which were found to have moderate benefits to the patient.

Perhaps most strikingly, the committee found that in six of ten investigated practices, there was simply not enough data to recommend for or against them. “This highlights that there are a lot of unanswered questions,” said Dr. McColley, citing the need for research that would clarify whether there are different outcomes associated with the practices, which included inpatient versus outpatient care; simultaneous use of intravenous and inhaled antibiotics; number of antibiotics used to treat Pseudomonas aeruginosa; continuous infusion of betalactam antibiotics; and duration of antibiotic treatment.

“We have incomplete information, but the guidelines provide important guidance to physicians, patients, third party payers on the treatment of this serious and common complication of CF respiratory disease,” she said. “By reducing variability in practice, implementation of these guidelines may help to improve outcomes of care.”


The guidelines were developed and funded by the Cystic Fibrosis Foundation.

Link to original article:

So what do you guys think? Do you see any problems with what was laid out by this committee?

Friday, October 23, 2009

Pictures from Coronado Island

You guys demanded them (ok, ONE of you guys did) and now you have them! Here are pictures from our trip to Coronado Island yesterday. I tried to post them earlier but blogger was down for some reason. We had such a blast yesterday riding our sweet old school beach cruisers around the island! Enjoy the pics :)

View of Coronado from the bridge coming from San Diego.

First stop beach...

...and apparently I'm a big fan of this.
We were both starving so we parked our bikes and grabbed a bite to eat.

My "bite to eat".

He wanted to pose for a picture (and who am I to argue with a bike?).

I know. I'm like James Bond with a total disregard for the law.

Little boats that people take to their bigger boats.

Sorry if you're currently eating, I had to take off my shirt while on the beach.

That girl can jump can't she?

Each your heart out Hasselhoff.

This was actually just nominated for "Cheesiest Picture of the Year 2009".

Man she's hot!

After riding around for about 2 hours we figured we'd take a break and find a view...

...and something to drink.

Did I mention how blue the skies were?

The landscapers asked me to hold up this tree while they grabbed some tools. No problem landscapers.

We then had to return the bikes back to their family.

If you ever find yourself out in Coronado Island I would HIGHLY recommend renting some beach cruisers and hitting the open I coughed up a BUNCH of crud from my airbags.

Thursday, October 22, 2009

Connecting to the CF Community

I think I’ve said this before, but let me say it again. Technology is amazing. I’ve met SO MANY CFers and their families since starting this blog and I feel so fortunate to have met each and everyone of them. I recently obtained a Facebook account after many years of stating that I “don’t have time for that”. Well now, a majority of my day is spent online so I was out of excuses. In the short time I have been in Facebook I have had a ton of conversations with others in the CF community via chat, email or wall-to-wall that I would have never of met otherwise. So thankful for that.

I’m also thankful for the time I was able to spend with Eric (Mandi’s dad). Fortunately we’ve gotten along right from the get go. We share many of the same personality traits and we seem to “bond” by ripping each other. I think he like me (at least I hope he likes me) because not only can I take it, but I can dish it too. He’s just a fun guy to be around and although he’s now 50 plus (just kidding Eric, I know you're only 49), I have a hard time keeping up with him. Now that he’s gone, maybe I can get some rest J

One more thing I technology, have you guys ever heard of a Slingbox? If not, seriously, it’s crazy, now let me explain. As I mentioned earlier, Mandi’s parents live in China. One thing that they miss is American TV shows, you know, the greats like “The Bachelor” and “Dancing with the Stars”. Well yesterday we took care of that little problem by purchasing this Sling box device. Now, I took us about 12 hours to figure out how to set it up (I’m serious), but once we had it cranking, we soon realized how worth it that was. Now, from anywhere in the world, you can tap into their TV and watch it live over the computer. You can also control the DVR to record new shows or watch the ones that are already recorded. As we speak, Mandi’s mom is catching up on “Modern Family”. Which, if you haven’t already seen it, I HIGHLY recommend it!

Wednesday, October 21, 2009

I've Got To Get This Mucus Out!!!

I hate to admit it (this is all part of being accountable), but this last week was a TERRIBLE week for working out. I managed to get some really good and long walks in, but I haven’t set foot in a gym. Mandi’s dad flew in from China (he works over there) and we’ve all been hanging out non-stop since he’s been here. So, if you’d like to send some hate mail, please send it to Mandi’s Dad 14151 E Hanging Out St. Excuses, AZ. Luckily I had some really good runs before he got into town so the transition back into it shouldn’t be that painful.

I’ve also been battling something that’s been going on since this weekend. We headed to the Breath of Life Gala in Tucson on Saturday and I was feeling a “little off”. It has since come to my attention that my little brother (whom I hung out with) is now having flu like symptoms. Now some of it may be mental, but ever since I knew that he was sick I seem to be getting worse J Yesterday I felt like I was hot all day and my lungs felt really junky. I ended up doing a couple of extra vest treatments to try a shake all of the junk loose.

So, I must say that something must be going on cause today I’ve been coughing up a bunch of dark or light brownish/orangish colored mucus. I’m pretty sure how I’m feeling isn’t mental cause mucus doesn’t lie. Now all I want to do is get this crud up and out. I can’t wait to go for a run later and cough my brains out. In fact, I’ll finish this blog when I get back from my run and tell you how it went. Stay tuned….

Ok, I'm back from my run....just a second, have to catch my breath and try not to throw up on this computer screen.--(Panting proceeds for approximately 3 minutes)--Alright. Wow, I can't believe how much stamina I can lose in five days. That run was 10 minutes of torture. If you're wondering why I only did ten minutes, and I don't blame you, it was because that was my goal for today. I wanted to "ease" back into it and try to fully recover from whatever has been going on in my lungs before I pushed it too much. The last couple of days my mucus has gotten really dark and even a little rusty/orangish color. It could be some old blood or just something that stained my mouth, but either way, I'm not worried. I've been coughing a ton of it up today and feel better than I did most of the weekend.

I'm sure glad I pushed through tonight. I seriously felt like I was going to puke the whole time we were running and I still had a bloated belly from our late lunch. Worse yet, my burps smelled like the sushi we had. YUCK! We managed (or should I say I managed, Mandi was fine) to push it and even picked up the speed towards the end. All I know is that I can't make any excuses this next week and I have to get back in the groove!

What do you guys find is the most affective way for YOU to get the mucus up and out?

Tuesday, October 20, 2009

The Top Ten Things I Say to Myself While Running

I seem to be constantly talking to myself while I'm running. There are however ten phrases that seem to be said more than others and I put them in order of their frequency. Am I the only one who talks to themselves during exercise or running? What types of things do you find yourself saying to, well, yourself?

10. "Dangit! Blinded by salt filled sweat again. Where's my rag?" - There are often times that I run with my eyes closed because of the acid that drips down from my forehead. People who pass by must think I'm nuts!

9. "Please wind, don't blow directly in my face at 126 mph." - Sometimes it seems like the wind blows directly in my face no matter which direction I go. Can the wind purposely be picking on me?

8. "Man it's hot out." - Luckily it's getting to the point now that it feels pretty good if I run early in the morning or early in the evening. But for the past 4 months there was NO WAY I was going to run after 7am or before 7 pm.

7. "This song stinks. Next!" - I generally listen to the same couple of playlists depending on my mood. It's either a hip-hop playlist or a mellow folk playlist. Somehow though a song always sneaks in there that I'd rather not listen to.

6. "There's no way it's only been 10 minutes!" - This is definitely one of the worst feelings. Sometimes I'll run for what feels like at least half an hour only to look down and see that it's been a mere ten minutes. Keep in mind though, 10 weeks ago I was praying for just 2 minutes to pass...

5. "Dear Lord, please help me up this hill." - This is another one of those things where it seems no matter which direction I'm going I'm always running uphill. The worst is when I try to pick up my pace a little bit and then all of the sudden I get to Mt. Kilimanjaro with dead legs. It's a good thing that God is willing to drag me up most of those hills.

4. "You really need to take a deep breath." - If I don't pay attention to my breathing I'll start taking really short and shallow breaths. If I do that for too long then I start to get the old cO2 headaches. I often have to remind myself to take some good deep breaths. It also seems to restore some energy in my legs.

3. "How in the world do my legs hurt already?" - There are some days that I am just sure that I should not be running. These days usually happen when I immediately start to hobble or limp at the beginning of my run due to some leg pain anywhere from my toes to my hips. Don't get me wrong, I always run through it, but that doesn't mean it doesn't stink.

2. "Seriously, it would probably be better if you just stopped." - I probably say this to myself at least five times during my thirty minute runs. When I tell people that I am literally trying to talk myself out of running the ENTIRE time I'm running, I mean it. There are times when I swear to myself that I will pass out, but then a little voice always comes into my head when that happens. The voice says, "Well then I guess you're just going to have to pass out cause you're not stopping". Splendid.

1. "There's no way I'm stopping. I've put in too much work up to this point." - For every time that I repeat number 2 in my head, number one comes quickly afterward. I just know that I have gone through to much pain, sweat and tears in order to sell myself short now and just throw in the towel. For me, failure is not an option. Failure will literally make me sick. To avoid failure, I run. I run until I reach my goal, no matter how high it's set.

I'd love to hear the things that run through your mind during exercise or running. Just try to keep them clean :)

Monday, October 19, 2009

Do We "Domesticate" Death in the CF Community???

Last week I read an article that got me really thinking. It was splattered with ethical dilemmas and question arousing issues. Read THIS article first, read the blog, and then share your thoughts...

The CF community is faced with questions and situations that could never be explained or understood by others. For me, one of the most interesting is the concept of expecting and accepting death from a young age. This article mentioning trying to "domesticate" death and talking about it to the extent that you're almost too comfortable with it. If you're anything like Ronnie and I, it's not discussed very often, but it's understood and becomes a factor in many decisions. Ronnie has never pictured himself being an old man, but expects to live a long life...a result of years of knowing that CF shortens your life. While I teeter between expecting and hoping for the ideal, long life together, and envisioning losing him at a young age. I think for me, it's a coping method. I tend to get my head wrapped around something so much so that I am disappointed when it goes any other way, and want to make sure that that is not the case in this situation. And while Ronnie is healthy, we both fully grasp how fleeting that health could potentially be. We discussed early on in our relationship the possibly of death at an early age...a conversation not had by many couples in their 20s. A conversation we hope is completely hypothetical. A concept we understand could be a reality. What are your thoughts? Is it unhealthy to talk too much about death. Does talking about it enough make you too comfortable with it?

This article also uncovers the issue of remarrying. If your spouse dies, at any age, at what point, if any, is it ok to move on; to allow yourself to love again? I think the answer is different for everyone, but what is my answer? I'm not sure. I wouldn't say to Ronnie that I'd never remarry. That's a promise I don't know if I could or should make. How do you navigate a second relationship when you haven't let go of a first? Is there a way to remain in love with one person while loving another? All questions I don't think anyone has the "right" answer to, but I'd love to hear your thoughts.

The last interesting theme of the article is the moral dilemma of using frozen sperm from a deceased husband, after remarrying with two children. This is something I've never thought about. Interesting predicament. Just thought I'd open up conversation about it...but I have no thoughts of my own to add!

There sure is a lot of room for discussion with many of the issues this article unfolds. I'm sure the topics are ones you've thought about at one point or another. I'd love to hear your thoughts.

Sunday, October 18, 2009

Dancing & Biking My Way To Healthy Lungs

Guest Post by Emma D'Agostino

I’ve always been pretty active. My parents have always wanted my siblings and I to participate in some form of exercise and the doctors encouraged it too. So we tried tee ball (a failure), dancing (still dancing today) and soccer (I danced on the field. I consider it to be another failure). Dancing was and is fantastic because of its fast nature-I’m an Irish dancer (think Riverdance). But dancing has one flaw that we really only recently discovered, which is why I’m writing about exercise. It’s stop and go. While you’re going, it’s great, but you’re only going for about a minute and a half, tops. Still, it helped keep me healthy for about 10 years with no other form of exercise.

About a year ago, we started thinking that maybe some type of other exercise would help as well. I believe that in the beginning we wanted to help out my stamina for dancing-it is hard to build stamina without CF getting in the way, let alone when your lungs fail long before your legs. I get shin splints really easily, so running and jump roping were out. So we bought a stationary bike.

At first, I really didn’t do much with it. I did bike some over Christmas break, right after we got the bike, when I get about a three week break from dancing. Generally, I do absolutely nothing because I’m exhausted from going four days a week to dance class in October and November. I let my body recover. This, however, presents a problem CF wise because my lungs don’t appreciate my sedentary-ness. I did see that biking helped a lot-my stamina was actually ok when I went back to class in January. But I really didn’t enjoy biking all that much and so over the next few months I mostly just let the bike sit.

Then, May came around. Because of the way my high school is set up, I don’t have a free period to take PE and health. So they offer PE online as an alternative. My parents and I figured ok, it’ll help prepare me for (dancing) nationals in July and I’ll get it out of the way. I also made a deal with my dance teacher. She was determined that all the dancers would build stamina and was going to make everybody jump rope every day, but she knew how badly I get shin splints and we agreed that biking was a suitable alternative.

Every day for the entire month of June, between the PE class and my teacher nagging, I biked for around 30 minutes. I was completely shocked at how effective it was. In no time-a week, maybe-I saw a gigantic difference in my dancing. My legs and lungs could go further than they had ever gone before. And I finally realized that biking was great and that it would be an extremely bad idea to stop.

So, of course, my knees got mad at me what with all the demand being placed on them. Three hours of strenuous work a day, I will admit, is a little much. I would start, and I wouldn’t get up the street on my regular bike. I couldn’t even do a quarter mile on the stationary bike. And I’m not stupid, so I stopped biking and since it was summer, I swam every day until I thought my knees had been given a reasonable amount of time off. By then it was August and I knew my lungs were missing biking, so I started back slowly. Sometime in August, I noticed something. Maybe it snuck up on me slowly or maybe it was as sudden a change as I thought. But I could suddenly breathe better than I can remember breathing in…well , a really, really long time. Years, maybe? And my cough, which we can never get rid of no matter what, all but disappeared. I completely attribute this to biking.

I had my CF appointment and my PFTs were 97, which aren’t really super duper high for me since my numbers are usually good, but recently I had been going to clinic and seeing my numbers drop. So it was nice to see them go up for a change!!

The very next day after that wonderful clinic appointment, my lungs of course went on a breathing hiatus and stopped being so wonderful. But, after a two week long temper tantrum, they stopped, and now I’m breathing better than ever. It is amazing not to have to struggle to breathe. I cannot tell you how much I enjoy the act of breathing now. And I completely attribute this to biking. So go out and find some kind of exercise that you enjoy. Or maybe you don’t enjoy it, but you can at least stand it. I feel better than I have in such a long time and I want everybody to have that feeling, so go! Don’t wait until it’s too late.

Note from Ronnie: I just wanted to give a big thank you to Emma for writing an outstanding guest post for RSR. Although she knows that dancing is good for her lungs, she also knew that she needed to do more for optimal lung health. It just goes to show you how in tune us CFers are with our bodies. We always need to be working towards putting ourselves in the best position for clear lungs; No matter what it takes! I encourage you to leave Emma your comments as well as any questions you may have! Emma can also be found on Facebook by clicking here.

If you are interested in writing a guest post for RSR simply send me an email with a little bit about yourself and what you'd like to write about.