A day in the life with cystic fibrosis…
It's about 4:40a.m. and I'm doing the first of my nebulizer medications for the day. I've been slightly slack on starting them earlier enough lately, opting for additional sleep in the mornings instead of getting my rear in gear. My medication of choice these days for a bronchodilator is Albuterol - it makes me a little jittery though, which can be a challenge if I have to do any writing at work, thank goodness I do more typing! 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 specifically 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 ourselves) go through in a typical day.
Take a deep breath! Ha Ha! Here we go...
Starting the morning
I try to get in at least 8 hours of sleep each night. This week has been a little on the less than 8 hours a night side though... The first thing I do after rising is take a shower to help wake up. Then I typically begin my inhaled medications along with my ThAirapy Vest. 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. Albuterol is a bronchodilator - it opens up the airways. The ThAirapy Vest is a replacement for Chest Phsiotherapy which is done manually - the vest is electric and looks much like a life jacket hooked to a machine that sends pulsating vibrations into the air filled vest that vibrates the chest walls- stimulating coughing while loosening up the mucus inside, so it is beneficial to use it with the inhaled nebulizer treatments. The vest therapy takes about 30 minutes.
Now it's 5:20am and the Albuterol is finished, so it's time to move on to the TOBI treatment. TOBI is a bit metallic in taste so my hubby usually has a glass of OJ or Cranberry juice ready for me as I do this treatment. I'm starting to cough more now, which is good. When I cough, I bring it up and out of my lungs, and I hope, bringing me out of harms way. I have a pretty speedy nebulizer/compressor system, so the treatments only takes about 15 minutes, depending on how much I have to pause to cough. Today it's looking like a 20 minute event. As the TOBI winds down, I'm getting ready to inhale one powder-form medication: Spiriva. This is a long-acting medication that should help keep my airways from closing up between now and my evening treatments. 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. I also do a Symbicort inhaler which is something that is also used by asthmatics, which is why it has some benefit for me as a CFer too. Its 5:45am and I'm putting on make-up and clothes in effort to try to get downstairs to help my husband make some 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 toasted plain bagels topped with peanut butter and jelly and wash it down with a small glass of orange juice and a tall glass of Silk Vanilla Soy 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 being proactive 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 (ABDEK- specially engineered for people with CF), extra vitamin E and some zinc, - to replace what is lost to malabsorption - and Os-Cal since I have osteopenia (a precursor of osteoporosis.) I also down 500mgs Zithromax every other day which is a preventive antibiotic taken to ward off lung infections caused by Pseudomonas, and 300mgs of Ursodiol for my liver- because my liver enzymes are elevated and the ursodiol helps to digest fats. Top it off with an extra vitamin C tab and we’re done!
While breakfast was in the oven, we made our lunches for the day. My husband is very much a creature of habit, so he doesn’t vary too much in what he eats for lunch; my lunch however looks like I am feeding a small army of men. 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. I love pasta of any sort, and veggies- cooked or raw, throw in some fruit, pretzels and cheeto’s for fun and lunch is almost complete! I usually add Ensure Plus for the extra calories, cheese and crackers and yogurt.
Off to work!
I am blessed to be able to work a full time job. And so I work full-time as a records and imaging technician for the local government. 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. (a favorite is peanut butter filled pretzels!)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. I allow myself a soda in the afternoons- thinking of the sugar as a little bit of a boost in energy. Lately I've also been drinking cranberry juice. It's good for my kidneys (especially with all the drugs I am putting through my system!) and it has more calories than the same amount of soda. It's rare that I find something healthy in a small package that offers decent competition.
Between 9 and 5
While I'm at work, CF doesn't really factor in too much unless I'm working on projects that put in the midst of many people all at once. Since this is not the case right now, the main thing that I do that is CF related is to wipe down my desk and other surfaces in my office with alcohol or Clorox 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 germaphobe, but I like to feel like I have at least SOME degree of control.
Things to consider
The effects of CF can be wildly 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 all the time.
I have to pick and choose. If I want to make sure I have the energy to attend church mid-week after work or something, 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 bed early enough the night before. If we want to visit family or friends in the evening, or 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 then come home for dinner, or eat first and then go out. For me, having CF means planning as much as possible, given my limitations, but still being flexible enough to accommodate those limitations as the need arises. That is the biggest mental challenge of having CF.
Twice a week my husband meets me out at my Mother’s house where we complete a workout that we have tailored to benefit both he and I. We start out with stretching and warm up exercises (a few sit ups, pushups, leg lifts for me etc…) and then I hop on the treadmill for a workout that raises my heart rate and pushes me to open up my lungs and take deep breaths! (All the while I am hooked up to oxygen so that I don’t give out during the workout). After a bit on the treadmill- a few sessions of walking, increasing the speed and the incline then back down again- I hop over onto the recumbent bike for a few minutes to work my legs. After we finish with these get your heart pounding exercises, we take a walk around the block to cool down. A short visit with Mom and then we head home for the evening.
When I get home, I plan to use the energy I have left to get some housework done. I usually have an Ensure Plus just before leaving work around 5pm. This helps when I get home and take on small chores of housework like dishes, laundry, medicating and feeding our cat…I do not do any serious cleaning as we have always allowed in our budget to hire a house cleaner. She does all the “dirty” work like scrubbing the bathrooms, vacuuming and dusting, anything that involves chemicals and dust. She comes when we are not at home so I can avoid any strong cleaners that may aggravate my lungs. Doing the light chores makes me feel productive, and they are relatively low-impact, so I don't get too worn out doing them. It's also methodical enough that it helps me wind down at the end of the long day.
Even something as simple as doing the laundry must involve consideration of CF. We have to be sure to clean 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 low on perfume so that my lungs once again aren't aggravated.
On the days we don’t work-out, I arrive home about 15-20 minutes before my husband does, which gives me a chance to have a quick snack while I sit 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. It also helps me get through the light chores I mentioned before.
Dinner preparations again involve careful consideration for nutritional value. A usual on our menu is stuffed baked potatoes. My potato consists of about 2 tablespoons of butter, cheddar cheese, broccoli, chicken strips and a heap of sour cream on it and I washed down my meal with cranrasberry juice. In comparison my husband has his potato sans the butter and sour cream! If need be we add a serving of apple sauce on the side. 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.
CF doesn't sleep
After taking care of light chores, paying a few bills and cleaning up after dinner we may have some t.v. time or short game time together. Then as the late hour approaches I will head up to the bedroom for a repeat performance of all the same nebulizer treatments and inhalers that I did this morning. All of it should take about an hour, which is time we use to get relaxed, watch a late show and even attempt to add to the Sunday crossword for that week. After the treatments I'll brush my teeth and then it is lights out! 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 6 or 8 hours before I start the whole process over again.