Breathing Deeply: My Life With Cystic Fibrosis

Some of the best discoveries happen quite by accident--telephones, silly putty and popsicles, just to name a few. The newest breakthrough in treatments for managing CF was also an incidental discovery. Someone noticed that people with CF who happened to be surfers exhibited fewer lung exacerbations than expected. This led to further investigation, and formal research of the benefits of salt water.

Although prevailing research for the use of hypertonic saline took place approximately 5 or more years ago, the use of hypertonic saline inhalation treatments for mucus clearance is the latest buzz in the CF world. My sincere appreciation goes out to those of you (and you know who you are) who were willing and able to participate in the clinical trials for this latest advance. In my opinion, every little bit helps when it comes to research, especially when the studies result in a worthwhile treatment option.

For those out there who may be starting on a regimen of nebulized hypertonic saline, or those who have been on it for awhile and can't seem to tell if it's making a difference or not, I'd like to explain in plain English the science behind the solution (pun intended).

First, let's get some background on those fancy scientific words.

Hypertonic saline solution: this means that the ratio of salt to water in the solution is greater than the ratio of salt to water in the cells of the body. The body prefers that its cells are in an isotonic state, meaning that the ratios of salt to water inside the cell are equal to the amounts in the rest of the body's cells.

In people with CF, our lungs are pretty dried out, which is related to the fact that we secrete so much salt when we sweat, and that salt pulls with it a lot of the water molecules we need. Without that water in our cells, it is very hard to effectively cough up the thick mucus we produce. There's so much gunk in there that needs to come out, but the body chemistry isn't right to make that happen easily. It's about as effective as trying to go the distance on a slip-n-slide before you turn on the water.

The amount of salt (concentration) in a hypertonic saline solution is different from the amount of salt in the cells lining our lungs. (This is why it is imperative that the proper concentration (i.e. 7%) be used in the nebulizer). This of course, makes things more moist in there, which is good. The increased moisture in the cells lining our lungs makes it much easier to cough productively.

Although the body is designed in such a way that the introduction of the high concentration of salt trigger's the cells to produce moisture to accomodate the chemical imbalance, many CF patients are on medications that interfere with this function of the body. Medications containing anti-histamines to deal with allergies and some antidepressants for example, rob the body of moisture and cause a state of dehydration. Also, diarrhea (not uncommon for CF patients with pancreatic insufficiency) can further deplete the body's water supply.

The greatest benefit from the hypertonic saline treatment is not so much in the chemical processes that occur in the body, but in the physical changes that result. The beneficial physical change that results is that the environment in the lungs is wetter.

On a personal note, I feel like I am doing well with the hypertonic saline treatments. They are making me cough more, which is slightly annoying, but I know that coughing is a good thing. The more gunk I'm able to cough up, the less there is remaining in there to harbor a nasty infection. Hypertonic saline treatment might not make you "feel" better per se, like a bronchodilater will when it opens up the airways. That's because the treatment is more like a chemical form of chest PT, and not like taking a round of antibiotics. Slow and steady wins the race with this one.

Don't expect to feel a dramatic difference after a saline treatment; instead, be diligent with your treatments knowing that it is preventive in nature and will likely reduce the number of infections that you'll get later on. Yes, it can be cumbersome and annoying to have even more treatments to add to the daily regime, but 20-30 minutes spent following your treatment plan have potentially significant payoffs when it comes to quality of life.

I hope this explanation has been helpful. As with anything regarding your own treatments, please check with your doctor if you need more information.

Last night Brad and I had to bid farewell to our trusty little crockpot. The entire thing tumbled out of the cupboard last night, the crock hit the kitchen floor and broke cleanly into four large pieces. This sounds silly, but I almost cried over it. Part of that might be because it hit my foot, but mostly I was sad because that little counter-top appliance was one of the first gifts we had received from our wedding registry. My mother-in-law got it for us while Brad and I were still engaged. In its honor, I have composed the following poem.

I can't believe I've been back at work for an entire week now! Time really does fly when you're having fun. Well, maybe fun isn't the right word, but it's good to feel productive. I feel like I have accomplished a lot not only by being at work, but by keeping up relatively well with health and work simultaneously. The most difficult part is still the matter of getting enough sleep. A couple times this week I went home in the middle of the day to take a nap, then came back to work. I'm glad I live so close to the office.


Maybe I should try to convert the area under my desk into a little lounge/sleep area like George Costanza did on Seinfeld. Then again, maybe not. That didn't work out too well for him.


I'm hoping that once I'm completely finished with the Levaquin I'll be back on track. Even the Anafranil isn't putting me to sleep like it used to. I wonder if that has anything to do with the recent changes to how many multivitamins I take. I'm not sure exactly what effects my malabsorption issues have on how my body uses each individual medication I take. Like everything else though, I suppose that's a learning process. And probably just like everything else, once I think I've gotten it figured out, it will change on me.


I can tell that something is slightly "off" somewhere. In addition to weird sleep patterns, I've been having some OCD issues. Nothing major where I'm a total nut-ball, but a few of the little things that had dissipated once I was on the medication have started to return a bit. For example, I had been doing really well for several months about keeping my keys and my glasses where I can find them easily. The last three days I have spent nearly half an hour each morning searching for one or the other. It's frustrating and embarrassing.

I almost forgot about the latest development with my meds. I have been doing the saline nebulizer treatments for several days now. I'm not sure that I can tell if it's working or not. The main thing I've noticed is that the treatment makes me cough--a LOT! I suppose that's good for keeping the gunk from collecting in my lungs. It's not a particularly comfortable cough though. Last night's treatment was more irritating to my lungs than anything else. I don't think I was really producing more sputum because of it. Oh well. It's only been a week. I'll keep documenting my progress.

***
Current mood: happy
Current snack: pretzel sticks and raspberry yogurt
Health-o-meter: 92% (slightly fatigued)
Emotional Weather: sunny, clear skies

We start to realize that there are anodynes in life that help us through the day. I don't care if it's a walk in the park, a look out the window, a good bubble bath--whatever. Even a meal you like or a friend you want to call. That helps us solve all this stuff in our head.






I've been resting at home for just over a week now, and I have to say, all this rest has been good for me. I'm so glad that I am able to rest and recover at home instead of the hosptial. Being in my own bed, surrounded by my own stuff, eating my favorite comfort foods and being able to email and chat on the phone with friends is good medicine. Although I can't deny that the Levaquin and TOBI are largely responsible for my progress as of late.

I didn't sleep very well last night and I woke up too early. That's okay though because it meant that I was able to putter around a little bit while Brad got ready for his day. I was glad for the opportunity to see him off to work for a change. I even packed a sack lunch for him, which made me feel like I was being productive at least in some small way.

After making some breakfast for myself, I turned up the dial on the electric blanket on the bed and took a short nap while the raindrops beat lightly against the window. It's nice to be able to hide from the world for a few more hours by being tucked cozily into bed--especially when there's nothing else I have the energy to do anyway.

Fresh from a nap, I called my friend Jen in NY. I think we both benefited a lot from the conversation. We're so similar in a lot of ways. She said that right now her big thing is going to the grocery store--it turns into a social event for her. She loves being able to stay at home with the baby, but I can appreciate her need to get out of the house every now and then too. Baby Josh was making all sorts of adorable squeaking and cooing noises in the background. I can't wait until Brad and I get a chance to go out there and see him in person.

For lunch I tried something new. I used some of those Pillsbury "Perfect Portions" biscuits and put some ham and cheese in them about halfway through baking. Mmmmmm. Those are a good way to put a lot of calories in a relatively small portion. I rounded out the meal with a glass of Instant Breakfast, and a cup of raspberry yogurt.

It's 3 in the afternoon and I'm still in my cozy pajamas and trying to think of what relaxing thing I should do next. Perhaps a bubble bath is in order followed by another nap. I'm not spoiled--I'm resting!

The results of last week's sputum culture have come back from the lab, and it appears that once again, B. cepacia is hosting a microbial gala in my lungs. Several years back when B. cepacia was first becoming such a hot topic in CF circles, I didn't really understand the full implication of how devastating this little bug could be. As research has expanded and more case studies have been examined, I have learned a lot more about B. cepacia.

B. cepacia's first and foremost frightening aspect is that it is exceptionally resistant to all of the first line defense antiobiotics that are usually prescribed for those of us with CF. Rarely will one medication be sufficient to touch it let alone wipe it out. The trickiest part for doctors is in finding the right combination of antiobiotics that will have the greatest effect on the cepacia.

When dealing with B. cepacia, the "cure" is just as terrible as the disease. The medications that best combat cepacia are the heavy-duty ones with the nasty side-effects. In my particular case, the antibiotics that work best are the ones that need to be constantly monitored in order to avoid reaching levels of toxicity. The danger posed to the CNS, kidneys, and other vital organs is very real.

This is why those of us who culture B. cepacia must be sequestered from others with CF. My body already has adapted somewhat to this microorganism's presence; however, if I were to inadvertently pass along cepacia to someone else, the likelihood of passing along a swift and terrible infection is high.

I hate that having B. cepacia keeps me from being able to interact with others who have CF on an "in-person" level. On the other hand, I'm glad that such safeguards exist that keep me from harming other people. I can't even begin to imagine how awful I would feel if I knew that I was responsible for harming someone else--particularly someone else like me who already has enough of a struggle with health-issues.

Well, today was not my day to get a bed at the hospital. But that's okay. I don't mind waiting for a bit. It's nice to be able to prepare myself mentally for what lies ahead. My mom came to spend the day with me just so I wouldn't be by myself, and so that if I did happen to get a bed then Brad wouldn't have to drive home to get me--he'd just be able leave work and meet us at the hospital.

But, like I already said, that wasn't the case. Mom and I did a bit of pre-hospital shopping (jammies, slipper-socks and a sooooo cozy robe). It's nice to have some designated duds for hospital cooties. I think the walking around and trying clothes on helped keep my blood sugar under control.


That's been the major concern the last couple days. On Friday night I was feeling particularly icky. I was shaky and feverish and PARCHED. I should have guessed from my extreme thirst what was going on, but for someone who eats as many pretzels as I do, being thirsty is sort of a constant thing. My blood sugar was well over 400. For those of you who don't know what that means, it means that I was in trouble. Since my last oral glucose tolerance test showed that I wasn't diabetic, I was quite shocked to see such a terrible "score." I had some old insulin from the last time I was on prednisone and having freakishly high blood glucose levels, so I began self medicating with that just to keep things under control until I could get in touch with the doctor.

Please note that this is not an endorsement of self-medicating for any purpose.

I was unable to get my numbers under control. However, I anticipate that will be a relatively easy fix once I'm under a doctor's very watchful eye in the hospital. In fact, I think this hospital visit will be a piece of cake compared to last spring. The last time I needed a tune up I was back and forth to the hospital 2 times in a very short period of time (3 if you count outpatient surgery and a visit to the ER. ) I think I only was able to work 10 full days in a 4 month period.

This time I'm anticipating a 10-14 day tune-up with IV meds Tobramycin and Fortaz. Brad and I call Fortaz "KTP" because we think it smells like cat urine. Kitty pee--get it? Trust me, you'll laugh to yourself about that one later. With a little luck I'll be back to work within 3 weeks and I'll be rip-roaring to go for a trip to Oklahoma.

Well, for all my big talk and my seemingly positive attitude, I realize that I'm up far too late and that's probably because deep down I am experiencing some anxiety about going to the hospital. It's a routine thing for me, but that doesn't mean it's enjoyable. The fact that it's routine does not make it any less of an upset or an inconvenience to my regularly scheduled life. On the other hand, knowing what to expect of the whole deal is comforting.

Plus sides of being in the hospital:

• I get my own room thanks to B. cepacia
• There's a grand piano in the lobby that's fun to listen to
• I get to sleep in because my doctor has a "do not disturb" policy for his CF patients
• The food actually isn't too bad
• I get to wear new jammies
• and best of all...I don't have to stay very long!

No clever quote today and no cute photo, but I wanted to at least post something so that my praying friends can have some info.

I had a doctor appointment on Wednesday, and it didn't go well. My lung function was only 66% predicted. My doctor has put me on levaquin, but is doing so with the condition that if my PFTs don't improve within two weeks, he's going to admit me.

Admittedly, I don't want to be admitted :) I bit the bullet yesterday at work and let my supervisor and project colleagues know that I'm going to be out of the office for awhile. I'm hoping that now that I'm back on TOBI, I'll be able to get in shape soon. I left the office at noon yesterday and plan to stay put for the next several days. I'm going to do nothing but take my meds, eat, and rest.

This is hard for me. I hate slowing down this much; I hate missing work, especially when I'm working on exciting and challenging projects. However, I also know that by taking it easy now, I'll be able to have a longer run of good health later. I am so incredibly blessed to be at a job that is relatively flexible, and that I have a supervisor who understands (or at least is sympathetic to) my situation.

Please keep me in your prayers over the next several days. Pray that my body will begin to heal itself with the antibiotics and that my lung function will improve. Pray also for my husband that he won't be too stressed out--we've got a lot on our plates right now as far as stuff to deal with, and me ending up in the hospital would only make things tougher. I am glad to know that no matter what is going on in my life, God is in absolute control. He has been keeping us safely in the hollow of His hand for a long time now, and I know that won't ever change.