Breathing Deeply: My Life With Cystic Fibrosis

A new day...I knew I'd make it through.

--Celine Dion





It's a few minutes after 6 a.m. and I'm doing the first of my nebulized medications for the day. I've been slightly slack on starting them earlier enough lately, opting for additional sleep in the mornings instead of just getting my rear in gear. My medication of choice these days for a bronchodilator is Xopenex--it doesn't make me nearly as jittery as the albuterol does, and since I do a lot of note taking at work lately, I want to make sure I can read my own handwriting later on!

If you've arrived at this page because you are looking for information about what a "day in the life" of someone with cystic fibrosis looks like--perhaps even specificially an adult with CF--then allow me to share a little corner of my world with you. My healthcare maintenance schedule is fairly rigorous even when I'm not fighting an infection, so you may find yourself experiencing a bit of information overload. Nonetheless, I hope you are able to develop a greater understanding of what people like me (CFers as we call ourselvs) go through in a typical day.

Take a deep breath! Here we go...

Starting the morning

I try to get in at least 8 hours of sleep each night. Last night I was fortunate to have gotten in nearly 10, which means today promises to to be a good day. The first thing I do after waking is take a Prilosec OTC pill. This helps protect my stomach in case my other medications aggravate it or in case my digestive problems cause some acid reflux. After the Prilosec I begin my inhaled medications. This helps open up my airways and make it easy for me to cough out the thick mucus that would otherwise obstruct my breathing and invite nasty lung infections. Xopenex, like albuterol is a bronchodilator--it opens up the airways.

Now it's 6:15 and the Xopenex is finished, so it's time to move on to the hypertonic saline treatment. I mix two concentrations of saline(10% and 3%) and put 4 mL of the resulting 7% solution in the nebulizer cup. I actually like this part of the routine the best. I'm a scientist by profession, so playing with syringes and turning my bathroom into something of a mini-lab is fun. It helps break up the monotony in the morning.

I'm starting to cough more now, which is good. The hypertonic saline (HS) is stimulating my lungs to produce moisture, which makes it easier for the mucus to slip and slide around. When I cough, I bring it up and out of my lungs and, I hope, bringing me out of harm's way. I have a pretty speedy nebulizer/compressor system, so the HS only takes about 10 minutes, depending on how much I have to pause to cough. Today it's looking like a 15 minute event.

As the HS winds down, I'm getting ready to inhale two powder-form medications: Advair and Spiriva. They are both long-acting medications that should help keep my airways from closing up between now and my evening treatments. Advair is something that is also used by asthmatics, which is why it has some benefit for me as a CFer too. Spiriva was originally intended for patients with chronic obstructive pulmonary disease (COPD) and although that's not the same as CF, it's similar enough to warrant the use of the med.

It's 6:30 and I'm ready to head downstairs to make breakfast for the two of us.

Breakfast

Even my selection of food preparation has to be done with CF in mind. I need close to 5,000 calories in a day for the level of energy I exert and in order to gain some weight. A high calorie, high protein diet is what I follow. Unfortunately, part of having CF means that my body does not absorb much of what I eat--a lot of it goes sliding right through, especially if it's got a lot of fat content. To combat this I take enzyme supplements to substitute for the enzymes my pancrease refuses to excrete because it is blocked by mucus. (And you thought CF just meant I had lousy lungs...ha!)

Today I plan to make cinnamon rolls and wash it down with a small glass of orange juice and a tall glass of whole milk mixed with Carnation instant breakfast. This is a whopping calorie meal and slightly higher in fat than what I usually do, but I'm feeling lucky today. Just to make sure I don't overdo it, I'll take 6 enzymes with the meal instead of 4 like I do with something less fatty. Additionally, I will take vitamin supplements--a multivitamin, vitamin E and vitamin A&D softgels,--to replace what is lost to malabsorption--and Os-Cal since I have osteopenia (a precursor of osteoporosis.)


While breakfast was in the oven, I made our lunches for the day. I am very much a creature of habit, so I don't vary too much in what I eat for my noon meal. Although I enjoy food, I have come to think of it merely as fuel for my body. I have learned what foods work well to give me the energy and calories I need, and can be digested relatively easily with the enzyme supplements.

After breakfast I take one more medication--sporanox. This is a liquid and although I've gotten used to its taste, I still hate it. I do it after breakfast because this way it doesn't upset my stomach, and also, because I know that I'll soon be able to brush my teeth to get the sporanox taste out of my mouth! Blech!

Off to Work!

I work full-time as an environmental scientist. Most of my job is indoors working at a desk. While I'm at work I continually strive to meet my caloric need by snacking on pretzels or other small foods. I find that pretzels are particularly easy for me to digest with a minimal number of enzymes. They are non-fat, and since they are made up of complex carbohydrates I know that they are a beneficial resource for my body. Another good snack for me is nuts, but since they are higher in fat, that doesn't always work out too well, so I have to be careful. Most of the day I sip water so I stay hydrated. Lately I've also been drinking cranberry juice. It's good for my kidneys and it has more calories than the same amount of cola. It's rare that I find something healthy in a small package that offers decent competition for the junk food!

Between 9 and 5

While I'm at work, CF doesn't really factor in too much unless I'm working on an outdoor project. Since this is not the case right now, the main thing that I do that is CF related is to wiple down my desk and other surfaces in my office with alcohol wipes. I do this every morning to make sure that my "CF cooties" aren't sticking around and making me sick. I wipe down the desk once a day, but the phone gets a going-over two times, since that comes in contact with my face. Maybe I'm a bit of a germa-phobe, but I like to feel like I have at least SOME degree of control.

Things to Consider

The effects of CF can be widlly variable, and 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. 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.

I have to pick and choose. If I want to make sure I have the energy to drive out to spend time with my prayer group friends, then I have to plan ahead. If a project at work will require an early morning meeting, I have to be sure to go to be early enough the night before. If my husband wants to take me on a date, we need to decide in advance if it would be better to go out to an early movie then come home for dinner, or eat first and then see a movie. For me, having CF means planning as much as possible, given my limitations, but still being flexible enough to accomodate those limitations as the need arises. That is the biggest mental challenge of having CF.

After work

I arrived home about 15 minutes before my husband did, which gave me a chance to have a quick snack (I don't like to tempt him with my high calorie goodies!) while I sat down to unwind from my day at work. It seems like I'm always eating, which I suppose is true. Like I already said, food is fuel to me, so I'm not much good without it. If I get hungry I get crabby, and that's no fun for anyone.

Dinner preparations again involve careful consideration for nutritional value. Tonight we had marinated tri-tip steaks, baked potatoes, and California mixed vegetables. My potato, however, had about 2 tablespoons each of butter, cheddar cheese, and sour cream on it. I washed down my meal with 2 cups of whole milk. And let's not forget the enzymes...dinner is typically my largest meal of the day, and the one with the most fat content. In order to process the evening meal, I took 7 enzymes to handle it all. In addition to the enzymes, I took another multivitamin and another Os-Cal tablet.

After Dinner

As my body digests my dinner, I plan to use the energy I have left to get some housework done. We did all of our heavy-duty chores on Saturday, so tonight's "to-do" list really only involves a bit of laundry. I like doing laundry. It makes me feel productive, and it's relatively low-impact, so I don't get too worn out doing it. It's also methodical enough that it helps calm me down at the end of the long day and put me in a state of calm that is suitable for sleeping. To aid in that process, I'll also be taking 75mg of a medication called Anafranil. It helps keep my brain from running continuously all night. It's an anti-anxiety med, so it's not exactly unique to CF, but it's not uncommon for people with CF to take anti-anxiety medications or anti-depressants.

Even something as simple as doing the laundry must involve consideration of CF. We have to be sure to turn on the vent so that fiber particles don't blow around everywhere when the dryer is running. We only use detergents and dryer sheets that are "free-style" (aka no perfumes or dyes) so that my allergies and asthma aren't aggravated.

After taking care of some laundry, or perhaps even while I fold some of it, I will do a repeat performance of all the same nebulizer treatments and inhalers that I did this morning. All of it should take about an hour, and then it's off to bed, but not without one last snack to tide me over until morning. After the snack, taken with the appropriate number of enzymes, I'll take the last of my evening meds. Singulair will help keep my asthma in check throughout the night. Zithromax is a preventive antibiotic taken every other day to ward off lung infections caused by Pseudomonas. Last but not least, it's time for sporanox. I'll brush my teeth, take a quick soak in the tub, and then it's off to bed.

If my medications do what they are supposed to and I don't cough much during the night, I'll be able to get in a good 8 or 9 hours before I start the whole process over again.

Thanks for sharing my day with me. I hope you've learned a lot. Feel free to ask any questions or leave your comments by clicking on the "comments" link below!