Breathing Deeply: My Life With Cystic Fibrosis

Yesterday was a busy day for CF-related things. I had the altitude study done at the hospital downtown. Parking was a bit of a nightmare, mostly because it was a parking structure. Those things give me the creeps. I was too creeped out to take the elevator so I took the stairs which were nice and in the sunshine. I was proud of myself for taking a more active route.

The altitude study was a lot simpler than I expected it to be. First they drew some arterial blood gas. Thankfully the respiratory therapist used a tiny needle, so I hardly felt it. Next he attached the pulse oximeter to my ear and took an initial reading. 99% on room air. Pretty awesome, eh?

Next they handed me this big hose with a mouthpiece on it. The hose was attached to a giant cylinder of 15% oxygen, which is approximately the concentration that the oxygen is on planes. For 20 minutes I just had to breathe in and out. Every so often they told me to take a big sighing breath. My saturation dropped from 99 to 91, which means I should be fine on the plane. I might get a little bit of a headache, but I'm bringing my vicodin so I shouldn't have a problem.

I think the test must have affected my FEV1 because at my clinic appointment last night I had the lousiest numbers I've ever seen. My first one was a mere 1.3L. Brad's jaw dropped and I got a little teary. How could it be so low after working so hard with the IVs and oxygen therapy and stuff? My next ones weren't much better. I didn't even hit 1.6, let alone the 2.3 that my doctor wants me to have.

I just can't understand how I can feel so good and have such bad numbers. My energy level is fine, I get up on time in the morning, I don't feel like I need naps. The only thing that's bothering me is my sinuses. Brad and I are praying that the sinus surgery happens soon.

Today the company who provided my oxygen concentrator dropped off what was SUPPOSED to be a portable oxygen concentrator for our trip to New York. I was absolutely horrified at what they brought! It was just as big as the concentrator in our bedroom. The only thing that made it portable was that it's on WHEELS! Oh, that and they provided a big stupid cardboard box for us to carry it in to check onto the plane. The thing was the size of my old HillRom vest. 40 lbs of disappointment. Apparently portable does not equal travel-ready.

I promptly opened my wallet, ran to the computer and looked up the Inogen One website and placed my order. It's well worth the out of pocket expense to have my own concentrator that is actually portable and a good travel size. I requested priority shipping, so it will be here on Saturday. I hope the holiday weekend doesn't mess things up.

A Day in the Life of a CF Patient
Aside from all the medications and airway clearance treatments, a day in my life as a cystic fibrosis patient really isn't that different from anyone else's day.
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The first thing I do every morning upon waking is cough. I cough because the thick, sticky mucus so characteristic of cystic fibrosis, has lodged itself in my airways during the night. Some mornings it only takes five minutes to clear my lungs of sputum. Cystic fibrosis patients like me have affectionately dubbed these secretions "lung oysters."

My first round of coughing complete, I move on to a more dramatic approach to airway clearance. I stumble downstairs to the refrigerator where I keep one of my most effective medications. Pulmozyme, or DNase, is a mucolytic developed by Genetech. When inhaled as a mist via nebulizer, it works in my lungs to cut apart the bacteria that thrive there. Pulmozyme is just one of the inhaled medications I need to open up my airways to their fullest. A bronchodilator in the form of a metered dose inhaler (MDI) goes a long way to help with that. Later in the day, I may need that bronchodilator again if I feel my chest becoming tight.

The highlight of my morning routine is my high-frequency chest wall oscillation (HFCWO) vest. It looks like a life vest. On the front of the vest are two hoses that attach to an air compressor. As the vest fills with air and begins to vibrate, the controls on the compressor allow me to set the frequency, pressure and length of time for the vibration therapy. I wear the vest for a minimum of 20 minutes on days when I feel well and up to an hour when I'm struggling with chest congestion.

Read my complete published article: A Day in the Life of a CF Patient

Wednesday night my CF doctor came by and told me that I'd be able to go home on Thursday. He did want to check on my lung function and make sure that it was still making positive progress. Thursday afternoon the pulmonary function lab called and asked me to come down for a 6 minute walking test (6mwt) and spirometry.

The 6mwt is a test that measures how much ground I can cover in 6 minutes while walking at a fairly aggressive pace. What the respiratory technician does is hook up a pulse oximeter to my finger, and keeps a constant reading on what my saturation is doing. That way they can tell if my saturations go down when I exercise. I kicked some serious butt on the walking test. I covered more ground than I have in a long time, and my oxygen saturation stayed nice and high the entire time.

After the 6mwt I had to do spirometry to measure my lung function. I wish they had done the tests in the opposite order, because I was a little bit winded from the walk. Thankfully I was still able to perform better on the spirometry than I had the time before. My FEV1 went up just a tiny pinch, but since it was an improvement, the doctors were happy.

The attending doctor came around 2:00 with the resident doctors and they said they were going to go ahead and write up my paperwork so I could go home. I was so happy I immediately started packing up all the stuff I had taken with me to the hospital. By the time Brad showed up I was all ready to go! I really love that he works on the UCSD campus. Having him close by is really good for my state of mind.

By 3:30 we were home. I wasn't quite sure what to do with myself after having been cooped up in that hospital room for a week. I wandered around the house for awhile just enjoying being around my own stuff again. I let Gimli out of his cage so he could have some freedom too. Brad took a quick trip to the grocery store to get me some milk and other high-calorie things for me.

It's so great to be back home. Brad had a get-together with some of his computer buddies last night, and I fell asleep on the couch while watching Jaws III. When Brad came home he started my IV for me and tucked me into bed--our OWN bed! I slept so incredibly well. I woke up a couple of times, but had no trouble falling back asleep. Quite a change from the hospital. I woke up this morning actually feeling refreshed. Ahhhh. It's such a great feeling of peace and calm to be home.

Today I'm very frustrated. I had a lousy nurse last night. Things just didn't go well, so I want to rant about it.

I'm in isolation (thanks cepacia) so nurses who come in contact with me must wear that stupid yellow gown. This nurse I had last night came in my room with the gown still folded, just draped over his arm. Yeah, that'll work. He wore gloves at least. He was a mumbler which, even with my hearing aids made him hard to understand. His accent didn't help either. That's not to say that I dislike nurses with accents, it just made this particular situation difficult.

I explained to him about my night meds and how I have to get them by 8:30 otherwise it throws off my whole schedule. He didn't seem to register the conversation. Even though I called him, I didn't get my meds until more than an hour later.

Here's the real kicker. He hung my Merrem at 12:30 am (which he wrote down as being 24:30--what a dummy. I think he meant 00:30.)Well, he hung it but never ran it. I woke up this morning after having had a really rough night, and saw that the pouch of Merrem was still hanging there, completely FULL. Correct me if I'm wrong but isn't skipping a dose of an antibiotic a bad thing?

I filed an incident report with the main nurse here. I'm not happy about what happened.

So today I'm less than cheery. I want to go home so badly. I still haven't even seen my CF doctor and that's bugging me too. Oh yeah, and the RT forgot about me this morning. I'm seriously feeling frustrated here. I want to go home.

Today my mom came for a visit. She brought a Belgian waffle (would you expect anything less?) and another new lounging outfit complete with cushioned socks. After breakfast I was desperate to go for a walk, so we went downstairs. We stopped in the gift shop for a new magazine and a snack, and then just sat on the couch in the lobby for awhile.

While we were sitting there, two gals with IV poles strolled by. One of them said "hi Lauren!" and for a minute I had to try to jumpstart my brain to remember how I knew her. It was Katrina--one of my CF pals. We usually only talk on the phone because of the cross-infection rules. She and I email every now and then and we've seen each other in passing at clinic. She introduced the other girl to me, who was also a CFer in the room next to Kat's.

For almost 20 minutes we chatted with one another from about 20 feet apart. Thank goodness for my hearing aids or that wouldn't have been possible. We covered our mouths when we coughed (which wasn't that frequently) and compared notes on how the RTs are doing (they agree that they're bad at the schedule this time) and what meds we're on.

Believe it or not, speaking in person with another CF woman, let alone 2 at once, was an entirely new experience for me. I felt so connected. I felt an incredible sense of kinship and even joy to know that the road that CF takes us on is not one we walk alone.

That doesn't mean I don't feel connected to the online community of CFers. I do. It's just very different to be able to look at someone's face while you talk to them. I think that's also why I feel so connected to Marjolein when we're in Second Life together. There's something to be said for having a visual aid to accompany the words that are exchanged.

I'm so grateful and thankful for the experience I had today. It was a good for me to see others like me--dressed in casual clothes, walking around like normal except for an IV pole. To be honest, I hardly even noticed the poles or whether the girls had PICC lines or a port. I just wanted to commit the image of the two of them to my memory, and I have.

It has been quite awhile since I provided my readers with a list of articles I've written. Here are some links for you to check out should you be so inclined.

Possible Causes of a Low White Blood Cell Count

Treatment Options for a Low White Blood Cell Count

How Marijuana Affects the Brain

Symptoms of Chronic Fatigue Syndrome

Naturally High Fiber Foods

Why we Need Carbohydrates

The Dangers of Prescription Drug Abuse

How to Reduce and Combat Body Odor

I didn't sleep very well last night, so today I'm a little on the crabby side. I'm trying to focus on the good stuff. For example, being awakened at 5:30 am to have blood drawn meant I got to see the sun rise through my hospital window. Then the crabby part of me kicks in and says "yeah, but it was so bright I couldn't fall back asleep."

Sleep has been difficult this time around. I think a lot of it has to do with how crazy my meds schedule is. One of my IV antibiotics runs for 3 hours, and I get it three times a day. If that one gets delayed (which it often does because nurses don't always come on time to start it) then my other medication gets delayed.

The most frustrating thing is how unreliable the respiratory therapists have been this time. I don't know if it's because there are a lot of CF patients admitted right now or what, but I've been getting my treatments sometimes 2 hours later than I asked to have them. My preferred chest PT schedule is 9am, 3pm and 9pm. Right now as I'm typing this it's 11:15 am and I just finished my first treatment.

When I get overtired and crabby like this my brain does funny things. It latches on to repetitive phrases and won't let them go. Apparently that's part of having OCD, but the medication is supposed to keep that from happening. Right now what I've got running through my head is all the songs from a "Wee Sing" video that my sister had as a kid. It was called "King Cole's Party." Nursery rhyme after nursery rhyme from that stupid video keeps playing in my head. "Old King Cole was a merry old soul; a merry old soul was he; he called for his pipe and he called for his bowl and he called for his fiddlers three." What does he need a bowl for anyway? Does he have to spit out his tobacco from his pipe? Is the bowl wooden or aluminum? Why not have a nice string quartet instead of three fiddlers? These are the questions that plague me in the middle of the night.

I hope that this afternoon I'll be able to take a nap and that will restore some of my sanity.

Today was the best day I've had in a long time; I feel really good today. I haven't needed a nap, my arthritis is finally under control, and I have more energy than I've had in quite awhile. I'm really eager to see what my lung function test shows on Tuesday. I'm praying that it's a dramatic enough improvement to send me home on IVs. I know that's a long shot, but that's what I'm hoping for. The real difference between this hospitalization and the last one is that this time I'm getting tobramycin antibiotic. I wish we had used that one in the first place. We could have avoided so much grief. Oh well. Can't change the past.

My dad came by for a visit in the middle of the day. I always like it when he comes. Our conversations usually cover topics from silly to sacred and back again. Today's topic d'jour was the John McCain interview that was televised last night. I hadn't watched more than 5 or 10 minutes of it, so Dad filled me in and gave his impressions and opinions.

After Dad left Brad arrived with some contraband for me. Banana cream pie and a Frappuccino. Boy oh boy did those taste good. At this rate I'll put the weight back on in no time!

Brad and I played cards *gasp! on a Sunday!* "Thirteen" is one of the games we play together most often. With each hand you're dealt progressively more cards. You start with 3 and the final round is, you guessed it, 13. The object is to be the first one in each hand to combine your cards in runs and straights that use up all the cards. Okay, so perhaps that's not the best description. When we got sick of that game we played "Speed." After Speed we played Egyptian War. Egyptian War is sort of like Slap Jack and War combined. It's relatively brainless and takes awhile to play.

This afternoon I had some visitors from church. Our elder, John and his wife came by. We enjoyed talking with them and helping them understand what exactly goes on when I come to the hospital for a tune-up.

This tune-up is actually going really well. My oxygen saturation has stayed at 95 or better the entire time. I did have a small episode of hemoptysis (coughing up blood) this afternoon, but it seemed to resolve itself. I think maybe my afternoon physical therapy shook me just a little bit too hard and that's what caused it. Just to be safe, I took it easy on the PT this evening. The respiratory therapist thought that was wise.

Perhaps the most interesting part of my day was when my door opened and a man who looked a lot like Woody Allen wandered in being led by a dog. It took me a few full seconds to register that 1) this man was blind , and 2) there was a dog in my room. I was dumbstruck. Finally I found my voice and said "You're in the wrong room." It turned out he had the right room number but the wrong floor. Poor guy. I hope he wasn't too embarrassed by the situation. It just goes to show you never really know what to expect when you check into a hospital.

Today was my first full day back in the hospital. So far everything is proceeding as it does every other time I'm in here. The first day is always a lot of different people asking me to describe why it is I'm here and what medications I'm taking. Since I was just here a couple weeks ago, all my information was still in the computer. All they had to do was pull up my giant list of medications and check them all off.

We're trying a different combination of antibiotics this time in hopes that it will be more effective in wiping out this infection. I'm on tobramycin, which is something I was on a lot in the past. The biggest drawback to that medication is that it's ototoxic (damages the ears.) I have substantial hearing loss and wear hearing aids because of the damage I've sustained from this potent drug. Nonetheless, I'd much rather sacrifice my ears than my lungs at this point.

One of the doctors who saw me today was a rheumatologist. He was able to diagnose my severe joint pain as CF-associated arthritis. This type of arthritis isn't that common. It happens to a small percentage of CF patients. Generally a flare up will accompany or precede a pulmonary exacerbation, which is how it's been for me this time. There's nothing we can do to prevent flare-ups. The best we can do is manage the pain when it happens. Right now I'm on a reasonable dose of vicodin. I say reasonable because it takes care of the pain without turning me into a zombie or putting me to sleep.

I had a fun visitor tonight; Kelly! She was kind enough to bring her smiling face and a bag of Cinnabons with extra icing. She said they'd help fatten me up. I sure hope so. I can hardly believe she brought those. I had been craving them the last time I was in here, but there aren't any Cinnabon shops nearby. Kelly happens to live near a mall that has them. Mmmmmmmm.

Brad came by at the same time and we shared a meal together. I've decided that take-out from the Peking Palace is nowhere nearly as good as it is at the restaurant. It's all dry and cold. Yuck. Of course, that didn't stop me from eating it. I ordered a cheeseburger from the cafeteria too. Might as well keep working on my weight. I was 114 when I came in here. I hope to be really close to 120 when I get out. We'll see.

I've barely had the chance to get used to my own bed again and already I'm heading back to the hospital. My lung function test last night at my doctor appointment revealed that my breathing is just as shallow as it was when I was hospitalized the last time. What's weird though is that I don't feel as dragged out as last time. Could it be that I've just gotten so used to feeling lousy that I accept it as the norm?

I expected to have to wait for a bed like usual, but of course the only thing predictable about the hospital is that it's unpredictable. The administration just called and said they can admit me this afternoon. I've already got a bag packed with all the "essentials" (yarn, knitting needles, cute jammies, snacks) so as soon as Brad gets here we'll load up the car and head back up to La Jolla.

Emotionally/mentally, I don't really feel anything right now. I'm sort of flat. I'm disappointed of course that I have to go back, but I guess I've sort of come to terms with it. I suppose that's the best way to be when you don't really have a choice about something.

At least this time I'm going in with a better weight. I'm currently 114, which is quite an improvement from the 103 I was when I left the hospital last time. With the help of some banana bread from Grandma, I'm pretty sure I can keep the weight going in the right direction.

I'll try to update as I'm able.

Someone mentioned in the CF forums awhile back that at the onset of an exacerbation they experienced aches in their joints. Someone had a really good explanation for why that happens, but I can't find it at the moment. All I know is that I'm sore. My legs are the worst though. I've taken 2 oxycodone today and that didn't do anything to help it.

Now that my griping is out of the way I'll focus on some other stuff. I have good friends. No, make that great friends. I had sent out a mass email asking for some prayer and even asking people to write to me because I'm bored and lonely here at home. I was so happy to see my email box come to life with letters from far and wide! Whether they wrote me a lengthy note or just shared a word of encouragement, I felt happy. I felt like I have purpose and that I mean something to someone.

I also got out of the house today for awhile with my mom. No, no waffles this time...hehehe. But we did go to my favorite Chinese place where I ate 2 orders of eggrolls, and most of my entree plus mom's. I was so proud of myself for having a decent appetite. The waitress assumed that my mom and I were sisters, so I let my mom enjoy the moment and didn't correct the lady.

After lunch mom ran some errands for me while I took a nap upstairs. It was one of those naps that was just light enough that you don't rumple the sheets, but deep enough that you feel rested when you wake up. Perfect. I love it! Right now it's about 5:15 and my mouth is watering for another helping of "Peek-a-Boo Chicken." My mom is awesome. My grandma's cool too (or so her email address says). She sent 2 loaves of banana bread. I've already had two fat slices of it slathered in butter and washed it down with a nice tall glass of whole milk. Please pray that tomorrow's doctor appointment won't result in me being hospitalized. I've got too much good food here at home!

To top it all off, today was cleaning day. My house looks great and our home is at peace. So am I actually. It's a good feeling.

I've been knitting lately. I learned how by watching a video on youtube. I love life in the technology age. It seems funny to me to be instructed in such a folksy art by means of an Internet connection.

I'm actually pretty good at it. I can't do anything fancy yet, but what I can do I do very evenly and smoothly. I'm not working on anything in particular. Mostly I just like the repetition and how it keeps my mind occupied. Without it I think I'd be going a bit crazy here at home. I don't really have much energy for anything else either, so this is a good fit.

Yesterday I did a bit better with stamina. I didn't feel nearly as wobbly going up and down the stairs. I think I'm more properly hydrated now too, because I don't get as dizzy when I stand up. I'm still not in perfect shape though. I went to the hospital today for some lung function tests. One was to see how well my lungs and heart perform while I'm exercising. The other was just the usual spirometry. I was very disappointed to find out that my spirometry showed that I was breathing about as poorly as when I was admitted to the hospital. On the plus side, I'm not in dire need of oxygen.

Next week (or perhaps later this week) I'm going for another test--and altitude simulation test. That will be useful for determining if I need supplemental oxygen on our trip to New York. Yes, that's right, NEW YORK! Brad and I have booked a very fun vacation that will include time with friends and a Broadway show. We went to NY 2 years ago, and I'm anxious to return.

Not much else going on today.

This morning I was greeted with almost simultaneous news from 2 people close to me. The first was from Jon saying he had gotten engaged last night. The second from the administrator of CF site I visit saying that my friend Roschelle passed away yesterday.

Today I rejoice in the season of Jon's life that is just beginning, and mourning for the seasons Roschelle no longer has. I'm blessed to have just spoken with each of them in the last couple days.

The experience reminds me never to miss a moment. Never wait to tell a friend how much they mean to you, and always leave on good terms because you just don't know what could happen.

I'm putting my thoughts here in my blog rather than in the forums because I'm concerned that the topics I bring up may lead to having the thread locked. I'm not seeking to be contrary, I'm just pointing out the things that stood out in the movie that I didn't like. Some of the things are themes and situations that come out in many other movies these days, not just Jack and Jill.

It's rare to find a movie that doesn't show a couple hopping into bed together or living together without commitment. It's sad that such things are so commonplace and no longer taboo. Remember the days when Ricky and Lucy had twin beds across the room from one another? It appears that modesty and decency are a thing of the past.

1. Woke up in my own bed after a wonderful night of sleep next to my husband

2. Prepared my own breakfast rather than having to dial 77007 to order it.

3. Put on clothes that were acceptable for going out in public. This included shoes, not slippers, and a sweatshirt instead of a robe.

4. Put on makeup and styled my hair.

5. Picked up my prescriptions (some things you just can't get away from no matter what)

6. Took a nap on the couch, not in a hospital bed.

7. Talked to an adult who wasn't wearing scrubs.

8. Walked to the fridge to find a snack. Instant gratification.

9. Emailed people via wireless connection that didn't poop out on me.

10. Walked across a room without dragging an IV pole along with me.