Breathing Deeply: My Life With Cystic Fibrosis

We were invited to San Francisco to be part of a video project for a Cystic Fibrosis community information website, called CForward.net. They are working to develop a better resource page for adults, and to that end they interviewed us about what it's like to be a couple managing CF. We were excited for the opportunity not only to be a positive and encouraging voice to CFers and their spouses, but also for a little weekend getaway. We've been through so much these last few months that it was a great time for us to reconnect.

Bonnie, if you read this, please know how grateful and truly appreciative we are to have been a part of this project.



Our trip started off on a high note! Originally our seats were not together on the flight, but Brad checked with the ticket agent and asked if we could be reassigned.

What a shame that the only seats they had available at the last minute were in first class! Is my husband resourceful or what?



Our hotel was about 20 miles away from the downtown area, so we took the hotel shuttle to the airport, where we were able to catch a ride on BART (Bay Area Rapid Transit.)

I remember hearing somewhere that Fresno wanted to put in a similar public transportation system for the Fresno area, but were unable to come up with a suitable acronym. *rim shot*















We each took a turn posing beside this artsy-fartsy thing in the Justin Herman Plaza. It's called the "Villancourt Fountain" and apparently is one of the city's best known landmarks. It was quite interesting, that's for sure.



These sculptures reminded me of Paul's letter to the church at Colosse, particularly this verse:

He has lost connection with the Head, from whom the whole body, supported and held together by its ligaments and sinews, grows as God causes it to grow.
(Colossians 2:19, NIV)






This is the part I was looking forward to the most...the Aquarium of the Bay! I've become quite familiar with some of the details of the San Francisco Bay and its inhabitants, since there's a group of folks at work who go up there quite frequently for one of their projects.





Moon Jellies! Only bounce on their tops, okay Dory?










Brad and I each got to pet the Leopard sharks. I think we were more surprised by how cold the water was than by how the shark felt. This particular fella was very docile, but his buddy in the pool with him was all riled up after being handled roughly by a toddler.






After the aquarium, we walked around the Pier for awhile, stared across the Bay towards Alcatraz, and watched the sea lions huddle together for protection against the biting wind. The sky was every bit as gray and cold-looking as the water. Even so, there were some beautiful spring bulbs flowering nearby. The tulips were definitely the highlight of my day, since they're my favorite flower. I loved how bright they looked, even on such an overcast day.



Seeing something so lovely and serene in a big, indifferent city reminded me that no matter what is going on in life, there is something beautiful to be seen and appreciated. Sometimes it's where you least expect it, and sometimes you have to hike a few miles to find it, but it's there.






Here's the current front-runner in the race to be the Christmas card photo for 2007! Aren't they a cute couple?

Here are some pictures with captions of my old portacath. I was only permitted to keep the port (the little reservoir with the septum) and not the catheter (the tube that goes up into my vein.) Regulations about contaminated waste or something like that. This souvenir EASILY makes up for the piano incident.

These are the dressings that I need to change daily for a total of 4 days. The lower portion with the larger bandage, is where the "pocket' is for my port was sewn up. The new port reservoir is pretty much in the same place as the old one was, which will make things easier for me. I've already gotten the flushing down to a "system" without the aid of a mirror, so it would have been a shame to relearn it all for the opposite side of my chest.

The smaller bandage covers the place where they connected the catheter to my jugular vein. The catheter runs from the port, under my skin to that point on my neck where the bandage is. No portion of it is outside of my body, but the surgeons needed to tunnel down through my skin with their instruments that they used to sew the catheter securely into the vein. It's that part of my neck that hurts the most right now. The tubing in there is a little stiff, which makes turning my head uncomfortable.



The old port! Pretty incredible invention, don't you think? That gray area in the center is the septum, and if you're able to zoom in on it at all, you can clearly see all of the places where a needle pierced it. It looks like a well used dart board!

The little tube on the right is where the catheter connects. When it was still inside my body, I was able to feel that through my skin. Actually, that 1/2 cm of white tubing you see there is probably a tiny portion of the old catheter. Just for kicks, I loaded up a new needle with some saline, and popped it into the septum for a flush. I was surprised at how difficult it was to flush, even though the thing is out of my body. I suppose I shouldn't have been. There was some dried blood in the catheter connection point, and I can only assume that there's some buildup in that little reservoir itself.


Here's a better picture where you can probably see those needle marks. I have it on good authority that these things can withstand 1,400 sticks. Wow. I didn't count every single one of mine, but I know it's nowhere near that many.









This is what the port reservoir looks like underneath. I'm not exactly sure why it is designed in such a way, but you can be sure that I aim to research that a bit.







Anyone for a little "Shell Game?"
Doesn't this make you think of those flying turtle things in the Super Mario Brothers Nintendo game? No? Just me then? Okay.

It looks tall in this picture, but it's not. The picture is enlarged to show detail, and is approximately 200% the actual size of the thing. The next picture gives a little better size reference.




See? It's really not that much larger in diameter than a quarter. The center septum is slightly smaller than a dime. The whole thing is hardly visible under my skin.











It's only half as tall as a quarter, too. If I had to venture a guess, I'd say that it's about 1/2 inch tall, which explains why a 3/4 inch needle was the best for accessing it. A 1-inch needle always stuck out quite far from my chest.


I'm so impressed that this sort of thing is available as an option for people who need long term infusion therapy. Although it was initially disturbing to think of having a piece of medical equipment implanted under my skin (and on my chest no less!) the trade off for easier and more comfortable tune-ups has been well worth it. Here's to 10 years with the new one! Three cheers for the new port.

Hep-hep-Heparin! Hep-hep-Heparin! Hep-hep-Heparin!

Last week I participated in an online writing contest. My articles managed to work their way up through the ranks, and when the final tallies came in, I was ranked 16th on the official Leader Board. (more than 50 entrants trailed me.) This translated into a nice little chunk of change--every freelance writer's goal!

Here are the articles that I submitted to the contest. There were 488 preassigned topics, but I only tackled the ones I could write about from an informed (or at least creative) perspective.

Evaluating a Job Offer
Finding Happiness at Work
Success is About Attitude, not Aptitude
How to Lose your Job

How the Internal Combustion Engine Works
The Case for Hybrid Cars
The Difference Between Hybrid and Electric Vehicles
Finding Hybrids with the Best MPG

The Four Noble Truths
The Seven Virtues
Puritanism's Legacy for American Government and Law

Seda-give? Seda-GIVE?!

--Gene Wilder, "Young Frankenstein"




Today was the big day! I got my blood transfusion, which was a little more than I expected. I got 2 units of "FFP" (fresh, frozen platelets) mmmm....
Basically, they gave me everything that's in blood except the red blood cells. Boy was it cold going in!

For all my nerves about being "awake" for the process, my fears were unfounded. The scariest part was being draped in that big papery tent thing to keep the area sterile. It made me feel a little claustrophobic, but once they gave me the sedative, I didn't care so much.

The new port is made of titanium (NASA grade, thank you very much!) but doesn't contain enough metal to set off any alarms at the airport or anything. Nonetheless, I now have to carry a card in my wallet that says I have a medical device implanted.

I think the coolest part of the whole thing was that THEY LET ME KEEP THE OLD PORT! Woo hoo! As a naturally inquisitive person, I figured it couldn't hurt to ask, so when I was lucid, I said "hey, can I keep that thing?" They thought I was nuts, but so what.

I'll be putting a little show-and-tell together with a diagram and photos of it for ya'll to see soon. (Christy, this is especially for your girls' benefit.) I figure it's one thing to read all the info about these things, and completely another to have a visual aid to back it up.

Thanks everyone for your well-wishes and prayers about today. I'm quite sore, and will be for the next little while, but I'm glad the whole procedure went without incident.

Beasts of England, beasts of Ireland,
Beasts of every land and clime,
Hearken to my joyful tidings
Of the golden future time.

--George Orwell, "Animal Farm"

For some extremely bizarre reason (which I'm going to go ahead and say is a side effect of NyQuil Sinus) I thought of these words verbatim around 7:00am. The weird thing is, I haven't read "Animal Farm" in nearly 15 years. What's more, is that not only did I recall these words, but I got the distinct impression that they should be sung to the tune of "La Cucaracha."

Just to make sure I was quoting those four lines correctly, I did a quick literature search. To my amusement (or was it chagrin?) this wonderful little ditty is supposed to sound something like La Cucaracha or Clementine. I guess my subconscious had stored that information somewhere in the deep recesses of my brain. I must have sung the full tune to myself in order to study for a test or something back when I was forced to read such leftist nonsense.

Seeing as how I've got the song in my head, I'm going to foist it upon you as well. So, here are the full lyrics. Belt it out loudly and proudly to the tune of your choosing (I prefer Clementine.) Make Snowball and Old Major proud, now!

Beasts of England, beasts of Ireland,
Beasts of every land and clime,
Hearken to my joyful tidings
Of the golden future time.

Soon or late the day is coming,
Tyrant Man shall be o'erthrown,
And the fruitful fields of England
Shall be trod by beasts alone.

Rings shall vanish from our noses,
And the harness from our back,
Bit and spur shall rust forever,
Cruel whips no more shall crack.

Riches more than mind can picture,
Wheat and barley, oats and hay,
Clover, beans, and mangel-wurzels
Shall be ours upon that day.

Bright will shine the fields of England,
Purer shall its waters be,
Sweeter yet shall blow its breezes
On the day that sets us free.

For that day we all must labour,
Though we die before it break;
Cows and horses, geese and turkeys,
All must toil for freedom's sake.

Beasts of England, beasts of Ireland,
Beasts of every land and clime,
Hearken well and spread my tidings
Of the golden future time.

Here are some of my recently added articles to Helium's website. They're currently having a writers contest, and a portion of my score will be based on how many "hits" my articles receive. So please, come check them out!

The Seven Virtues

A Christian's View of Suffering

How the Internal Combustion Engine Works

Exposing Fitness Myths

Freelance Writing: Advice and Encouragement

A Day in the Life of an Intern

How to Be a Team Player

Anemia: Causes and Symptoms

White Noise

Exercise versus Genetics

Eliminating Sugar from the Diet?

Medullary Sponge Kidney Disease

Finding True and Honest Friends

Preventing Indigestion

Are Dandelions Safe to Eat?

How to Protect Yourself from Identity Theft

ANA Test Results and Lupus

I don't like surgery. I don't like elective surgery,
I don't like surgery that you have to have.

As promised, here is the written version of my speech that I gave for the CF Foundation's Spring Gala. If you have CF, I hope it encourages you to know you aren't fighting alone. If you are the loved one of a CFer, I hope my words assure you that even with CF, life is worth living. If you know very little about CF, I hope I motivate you to learn more about the disease, and maybe even donate to the Cystic Fibrosis Foundation to help fund research for a cure.

And most importantly, I hope that through my clumsy words, the beauty and perfection of the one in whom I put my faith, shines brightly.

Since tonight is Spring Solstice, I’d like to share with you a poignant quote by C.S. Lewis, one of my favorite authors and theologians:

He wrote: “The future is something which everyone reaches at the rate of 60 minutes an hour, whatever he does, whoever he is.”

In other words, our lives are bounded and measured by time; none of us knows how much time he or she has. After all, any of us could be hit by a bus at any time, right? Wrong! I looked up those statistics once. Did you know that the odds of being struck and killed by a bus are only 1 in 10 million?

By stark contrast, the odds of a person with Cystic Fibrosis dying before reaching age 37 are 1 in 2.

I have cystic fibrosis. When I was first diagnosed, the doctors told my parents that I may not live to celebrate my 10th birthday. As you can clearly see, I’ve beaten that statistic, but I’m still in a fight for my life, and it’s a race against time. In order to give you a better picture of what this fight looks like, I’d like to describe some of the ways that cystic fibrosis not only steals my breath, but literally takes away the precious hours of my life.

My fight begins each morning before I even get out of bed. As I lie there beside my husband, my breathing sounds like crackling cellophane because of all the mucus that has collected in my lungs during the night. I spend the first 5 to 10 minutes of my day just coughing—coughing so that I can breathe well enough to get dressed and get moving.

Then of course there’s all the medication I need just to function as well as possible. Medications like:

I take no fewer than 11 separate medications to manage my CF. I wish this meant that I only had to take 11 pills, but that’s not the case. I take approximately 50 pills each day.

Each enzyme, each multivitamin, and especially each inhaled medication, is a brazen reminder that my body is held hostage by a disease that has no cure. And even with all this, there’s more. Inhaled medications can take up to 3 hours total during my day.

In addition to the medications, a day in my life with cystic fibrosis is hardly complete without a big dose of lung clearance exercises. These are designed to loosen up the mucus so that I can cough it out easily. One thing that I do is play French horn. That was actually my parents’ idea back when I was in elementary school. I wanted to join the school band and play the xylophone. My parents said “No way, kid. You’re going to play something big that takes lung exercise—because you’re not doing too great in P.E.!”

Ever since, the horn has not only provided method of helping me with my lung capacity, but it serves as an enjoyable creative outlet. Music is a very special bond that my husband and I share. Aside from our common foundation of faith, it’s probably the only thing that CF will never diminish in our relationship.

All of the elements of my life with cystic fibrosis take up a lot of time. Every minute I spend doing my inhaled treatments is a potential trade-off for what I hope to be infection free weeks or even months.

The most frustrating and exhausting part of having CF is how much time everything takes to stay healthy and how much time I lose whenever I get sick. Cystic fibrosis stands in the way of forming certain friendships. Because of the risk of cross infection, people with cystic fibrosis are not permitted to physically be around each other. Being denied the chance to support and be supported by someone who knows exactly how much this disease gets in the way of being “normal” is one of the unkindest cuts of all.

Even though much of my management of cystic fibrosis requires adherence to routine, and a great deal of self-discipline, each day can still be wildly variable. No matter how well I try to take care of myself, eat right, etc., sometimes I will fall prey to a lung infection anyway. That's just the reality. Someone once described it as "CF didn't read the book." CF doesn't know that I'm doing everything I'm supposed to in order to stay healthy.

Even when I'm feeling "well", I cannot forget that I have CF. I have to pace myself so that I don't deplete my energy. There are times (quite often, in fact) when I would love to do many activities, take on multiple projects at work, and try to be Super-wife at home, but the physical reality is that I cannot do those things.

Having CF demands that I evaluate my priorities, and make wise decisions about how I spent my days. If a project at work will require an early morning meeting, I have to be sure to go to bed on time the night before. If my adoring husband (whom, by the way my respiratory therapists have dubbed "Mr. Phenomenal") wants to take me on a date, we need to plan ahead so to work it around my medication schedule and my available energy.

Given my limitations, we try to remain flexible enough to accommodate those limitations as the need arises. Even with flexibility and an upbeat attitude, there are disappointments.

For two and a half decades, this disease has forced me to be absent from major events and occasions. My mom had to celebrate her first mother’s day without me in her arms, because I was recovering from major surgery.

Twice, I missed friends’ weddings because I was too sick to attend, let alone walk down the aisle as a bridesmaid, like I had been invited to be.

For the last 13 years I’ve been hospitalized at least once a year because of various lung infections or pneumonia. Each of those hospitalizations has been for a minimum of a week, followed by 2-3 weeks of home infusion therapy. Five of the last eight months I’ve been hospitalized; in and out, in and out. I spent my wedding anniversary in the hospital. I ate Thanksgiving dinner there. Even as recently as Valentine’s Day, I was an inpatient over at UCSD Thornton hospital.

I’m telling you all of this because this is the reality of the disease that I fight not just on a weekly, daily, hourly basis--sometimes it’s even a minute to minute fight. Such was the case in December when I lost consciousness due to oxygen deprivation, and had to be revived by a team of doctors while my husband could only watch and pray.

I would give anything if it meant I didn’t have to fight like this anymore. But I find comfort in knowing that I do not fight alone. I believe in a God who is big enough to number the stars in the sky, yet personal enough to breathe in me each morning, and sustain me for every day that is yet to come.

The Cystic Fibrosis Foundation uses the phrase "adding tomorrows every day" as their tagline. I am deeply appreciative of all the work that the CFF does and for the ways that their fundraising efforts are enabling researchers to make "GREAT STRIDES" toward finding a cure for CF. The Foundation has lived up to its motto, and has done much to add many tomorrows for those of us who have CF. Thanks to the advancements in modern medicine and a broader knowledge base about the disease, doctors and healthcare providers are seeing a new generation of CF patients—adults like me.

I consider myself extremely blessed. I have gotten to do things that were once considered an impossibility for people with CF. I am married to a wonderful man, I work a full-time job, I have enough lung power to play the French horn, and best of all, I'm living in an age where others with CF have reached adulthood.

Unfortunately, adult patients like myself, and our doctors alike are stymied and frustrated by the lack of information and understanding about living with CF beyond the pediatric and adolescent years. There is an unmistakable gap that has yet to be filled for us. Tomorrows have been added, but now what?

There is a need for increased awareness of the issues that concern adults with cystic fibrosis. Our quality of life can grow more difficult as each today becomes tomorrow. Today there is a need that must be filled. Today we need the funding for research that will not only add tomorrows every day, but research that will ultimately result in a cure.

Next month I celebrate my 27th birthday. As I said before, the odds that I won’t get to celebrate my 37th birthday are 1 in 2.

I don’t want to die waiting for a cure—I’ve waited long enough.

So at the end of the evening, when you set your clocks forward and think about the hour of sleep that you’re missing, please think of me and remember how much of my time has already been unwillingly relinquished to cystic fibrosis.

You might not agree with me, but I always offer a lot of support.

I believe in a God who is big enough to number the stars, yet personal enough to breathe in me each morning to give me what I need for just one day.









Wow! What a night it turned out to be at the Spring Gala. When I showed up for sound check my hands were shaking so badly that I was afraid I'd never be able to hold onto my horn. Thankfully, there was plenty of time between then and the actual speech for me to calm down and get settled.

During the silent auction portion of the evening, my husband and I milled about, meeting and greeting some of the local influences in the world of cystic fibrosis. I was truly an honor to meet people whose names I had only heard or read about prior to that evening. It was humbling to realize that I was in the midst of people who cared so much about patients with CF that they willingly gave of their financial resources to help find a cure. In some cases, the sacrifices were more than just monetary. There were two men in attendance, each of whom had donated a lobe of his lungs to a CF patient.

I can't believe I was calm enough to enjoy my dinner. Of course, that's usually the only thing a CFer can focus on...food! Surf and turf (filet mignon and salmon). Chet Magnifique!


Then, it was time. The emcee invited me to the front to share my words and my music with the guests in attendance. I had rehearsed both so much that I hardly faltered. I was told to prepare approximately 10-15 minutes of a speech. I had it so well times that it took 13 minutes--even with a few ad libs. As I closed upon the final sentences of my presentation, my voice began to break...

"So tonight, when you set your clocks forward and think about the hour of sleep you'll lose due to time change...please think of me, and all the time that I have already unwillingly had to relinquish to cystic fibrosis..."

Thunderous applause and a standing ovation. I couldn't believe it. Many in the room were wiping their eyes; my parents, sister, and husband beamed at me with expressions of utmost love and appreciation. It was a heady thing. I couldn't even begin to soak it all in. My mind was already preparing for what was next--a solo performance on my French horn, accompanied by a CD track that my husband had prepared on the computer.

I hit some wrong notes. Somewhere in the middle, I got a little out of tune, but incredibly, it didn't throw me completely off. Within moments I was back in the right place, hardly having appeared to miss a note. I summoned up every ounce of strength I had and breathed in one final breath, for that lingering, last note.

I held that G as long as I dared. When at last I lowered my mouthpiece from my lips, the room once again burst into wild applause. I bowed somewhat sheepishly and stood there, sweeping the entire room in my glance. How many were there? 200 some odd people? All had just witnessed one of my personal triumphs.

What sounded to them like a speech and a song by someone who just happens to have CF, was in fact, a culmination of months of hard work and fighting against the disease. A month ago I couldn't sit still long enough without coughing to play the piano, let alone my horn.

It may have been just 15 minutes of fame for me, but it was a tribute to the lifetime of faithfulness that God has been in my life. He has strengthened and sustained me. Without him, I can do nothing. Without his constant presence and comfort in my life, this disease would consume me from the inside out, and would certainly leave me an embittered, worthless pile of ashes.

Labels: french horn, Spring Gala, testimony

Laughing helps. It's like jogging on the inside. --anon



You know those "ups and downs" that are part of life? It seems I've finally come to an "up"! I can't remember the last time I felt like this. I'm not perfect, I realize that. I'm still underweight and I still cough a bit first thing in the morning, but wonder of wonder, I've gotten a lot of my energy, enthusiasm and stamina back.

I've been doing a lot lately to prepare for the CF Spring Gala, which is coming up on Saturday. I've rehearsed my speech, practiced my horn, and have provided the CFF with a handful of photos of me to use as a slide show while I'm speaking. I'm excited about the whole thing.

Something else fun on the horizon is that Brad and I get to be part of a video about what it's like to be married to someone with CF. The video is part of a larger project that's going to be part of CForward.net, an internet resource for people whose lives are touched by CF in some way. I've heard that Ana and Isa Stenzel (CF adult sisters, each of whom are about 10 years my senior, and wonderful athletes!) are part of the same project. I've been reading about this impressive duo for years now, and I'm honored to be part of something with the likes of them. In my mind, I pretty much regard them as celebrities.

In other news, I may be having my portacath removed and/or replaced next week. The poor thing just celebrated its 10th birthday, and I think it's finally ready to retire. It has been acting up lately, and within the last week has been giving me more problems than usual. Last Thursday I ended up taking myself to the emergency room to have the darn thing accessed because I couldn't do it, and it was hurting--which is atypical. In all honesty, the whole thing was really a predicament more than a true emergency, but hospitals don't have "predicament rooms," only ERs.

All in all, life is good. God is always a part of it, which always makes things bearable. He has been teaching me a lot lately about how to align my heart to be more rightly aligned to worship and adore him at every turn. As a result, I'm experiencing a great deal of peace and calm, both of which have been a LONG time in coming. I don't know what tomorrow will bring, but I know that no matter what, God's in charge and fully capable of doing more than I could even imagine.