Wednesday, September 5, 2012

What is it like to have cystic fibrosis? A message on my 39th birthday

Many of you ask me about my health and what it's like to have CF. I wanted to give it a shot and explain it. Unfortunately, there is no simple answer.

Before I begin, there are worse things in this world than having cystic fibrosis. I also want to be clear that I am lucky compared to most people with CF. There are many people with far worse symptoms and who deal with quarterly hospital visits. I've been more fortunate. This is not a pity party. I have been truly fortunate in my life and I want you, the reader, to be aware of that before you go any further.

Also, I just had my most recent appointment (Friday August 31st). Some of my numbers were down and others were up. My doctor said she could basically tell that as a whole I am quite a big stronger. I also had my annual blood tests (picture enclosed). I had to give 12 vials of blood at 9am and then another vial of blood at 11am. I could not eat from midnight the night before until I had my last vial taken at 11am. I also had to drink an orange drink that tasted like a very sugary Sunkist. It was not pleasant.


Still I was satisfied with the results but not ecstatic. I always expect better. Like most people, I'm my worst critic.

Having cystic fibrosis (CF) is in a word, different. Every morning I wake up and have to do 40 minutes of treatments which include my vest and two aerosol treatments. I do them anytime between 5:30am and 6:30am. Following my treatments, I usually work out for 30 to 40 minutes and then I either do my TOBI treatment which lasts 40 minutes or I get ready for work and do my TOBI treatment in the car with my portable machine (I call myself the superhero known as "THE NEBULIZER DRIVER"). Prior to my TOBI treatment, I go through my pills and pick out 12 pills for the day (13 on Mondays, Wednesdays and Fridays when I take Zithromax which is an antibiotic) plus my 20 to 25 pills for pancreatic digestion. I have to make sure I bring my pill bottle with me to work or anywhere I go every day or I have to go back home and get it. It's not like CVS or Walgreens carries the pills I take. I get them from an on-line pharmacy. Every day before I leave for work, I make sure I grab my wallet, my cell phone, my keys and most importantly, my pill bottle.

At night, I have to do the same treatments except I don't have to do my Pulmozyme aerosol unless I'm sick. During the day, my mind can be consumed with when I'll find the time to do my night time treatments yet still get time with my kids and wife and yet still not go to bed near midnight. It's tough to balance sometimes. When my kids go to bed, I want that time to spend with my wife. The latest I usually do my treatments is around 9:30pm. I find myself watching TV, answering e-mails, writing my newest blog or playing X-Box during my treatments.

Traveling with CF is in a word, time-consuming. I have to pack about 40 pills for each day that I'm gone plus I bring an extra day of pills in case there is bad weather or something comes up where I cannot make it home at the expected date. I also bring 3 to 5 nebulizers per day depending on if I'm on TOBI that month or not. I also have to bring my vest therapy machine, my aerosol machine, my inhaler and all of my sinus meds. So for a 7-day trip when I'm on TOBI, that's 40 nebulizers (including the extra day just in case), 320 pills and all of my nasals meds, aerosol meds and machinery. Yes, I still have to bring clothes though I would probably be have the most luggage at a nudist colony.

The emotional difficulties of having CF are many. I worry about each doctor's appointment. What am I going to tell my family if the results are poor? How am I going to cope? Will I have to spend more time doing IV's or extensive treatments? What's the newest antibiotic and where will it fit in my 40-pill rotation?

The emotional difficulties with traveling are there too. What if I forget a certain medication? What if my therapy machine stops working (it's happened before)? What if I get sick? Where is the nearest hospital?

It's also difficult knowing that since the day I was born that I have had a very low median life expectancy compared to the people around me. When I was born, my median life expectancy was 12 years. So that means my mid-life crisis would be around the age I was learning to ride a bike. That means I was considered elderly in grammar school. Sadly, it signifies that I would die just as I was entering high school. That just seems crazy to me.

When I was dating, CF was tough to hide. Every night before a big date, I used to do my therapy early and hide my machine in case the girl came home with me. Every night I had to take my pills by hiding them in my hands and swallowing them when she would go to the bathroom or order her food. With every relationship came the big CF talk which I never looked forward to bringing up. Fortunately, I ended up with my soulmate, Andrea, who accepted me for me and not the guy with a lung disease and poor sense of humor. I added the poor sense of humor part but I don't think Andrea would disagree nor would any of my other friends or family for that matter.

Now as a parent, CF provides a whole new worry for me. What happens if my kids get into my pills? Andrea was smart enough to create a lock box that they can't get in. Still sometimes I leave my bottle out unintentionally yet Avery and Ethan understand everything about my meds. In fact, when Ethan sees my pill bottle out, he grabs it and gives it to me. He's only four.

What will my kids do if I don't make it to see them graduate, go to college or get married? I worry about this but it doesn't usually hit me unless I'm at a doctor's appointment or I'm feeling really under the weather.

How much time do I have to make a difference in their lives? I am 39 so I have passed the current median life expectancy of 37 so I like to look at life as if every day I have is icing on the cake. I was not supposed to be here. Still every day is an opportunity to make a difference in my kids' lives.

How do I explain to them about the disease that daddy fights everyday? That's a tough one but I have explained a little to my kids. I think they get it to an extent. My daughter is 6 going on 35 so she may get it better than many people my own age.


CF is also difficult financially. I am fortunate to have great insurance but the price of my machine is the equivalent of buying a car. My meds cost thousands of dollars a year. Between sputum cultures, pulmonary function tests and bone density tests, I spend several thousand dollars annually. Andrea and I spent several thousand dollars on each of our In Vitro Fertilization attempts in order to have our children. I am also fortunate to have a full-time job and make money that way. Most people with CF do not have the strength to have full-time jobs which makes the financial constraints an even greater burden.

I go to a psychologist and a psychiatrist for my anxiety and depression. I believe that I experience these two components because as a child I had to grow up very fast. I didn't go to the doctor and think about getting a lollipop or a sticker. I had to worry about sputum cultures and chest X-rays.

What's it like to have CF? Challenging. First off, CF affects my lungs. Some days it's like breathing through a coffee straw. Many CF patients end up getting lung transplants. I'm hoping to avoid it by working out every day but I know that doesn't mean my lungs won't get worse. CF also affects my digestion. I'm constantly taking pills to aid with this issue. CF affects my reproductive system. My wife and I had to go through In Vitro fertilization because of me (98% of CF males are infertile including myself). Having CF means I also have very smelly stools. I sweat excessively though my dog Magic loves to lick the salt off of my arms and legs. I am more prone to getting colds and having them last longer. I have a better chance of having colon cancer. I can get CFRD (Cystic fibrosis related diabetes). I have a better chance at getting osteoporosis which requires annual bone density tests. I already have osteopenia which is the step before it. I have a hiatal hernia and acid reflux. Also, having CF, means it is very difficult to gain weight. I take supplements to gain weight though I don't take them as much anymore as I am at a healthy weight right now. Most CF patients have extreme difficulty keeping their weight up. Thanks to all of these problems, I also deal with the emotional issues I mentioned above - anxiety and depression.

Having cystic fibrosis is not a walk in the park but I repeat that there are worse things in this world. I am fortunate for many reasons. I have a family that can financially support my issues and who loves me very much. I live in a country that I have the ability to get many of my meds through an on-line pharmacy through my wife's amazing insurance. I have very supportive friends who look out for me.

I wonder quite often how I would adjust if CF is one day cured. These are some of the questions I'd have.

What would it be like not to have to take a pill bottle everywhere I went? For as long as I remember, I've carried a pill bottle with me.

What is it like not having to worry about getting germs from other people at all times? I feel sometimes like the boy in the bubble. When someone sneezes, it's like I'm in the movie "Outbreak" and have to run away.

What is it like not having to plan my days around my treatments? I'd be more than willing to do more household chores in exchange. Andrea, stop smiling.

What is it like during a storm not having to worry if the power goes out that I will have to stay up until the power is back on or go to someone else's house so I can finally do my treatments? I'd rather worry about finding a flashlight or having to deal with the A/C going out like most people who lose their electricity.

What is it like to have every doctor's appointment not be a big deal? I feel like each quarterly appointment is make or break. It's stressful. I've heard that most people have annual appointments only. That would be so nice.

Thanks for taking the time to listen to this. If you have cystic fibrosis, I hope it made you feel better that someone else empathizes with you and what you go through. If you don't, please know that I'm not saying that what you deal with on a daily basis is any easier; it's just different.

One funny CF-related joke I was just thinking about. One time my buddies and I were talking back in the day when we were single and we asked each other what 3 things we would wish for if we were stuck on a deserted island. I don't remember the specifics exactly but it went sort of like this.

One guy said "Britney Spears, a 6-pack of beer and a big screen TV."

Another guy said "Jessica Alba, a keg of Miller Lite and a lifetime subscription to Playboy."

My answer was simple.

"My therapy machine, a lifetime supply of meds, a working generator so that my machine would always work...and Britney Spears."

They were generous enough to give me the option for a fourth thing!

What's it like having cystic fibrosis?

DIFFERENT!

I hope all of you are living your dreams and loving your life.

Andy

2 comments:

  1. ANDY!!!! You are one awesome individual!!! I really loved reading this post it meant so much to me to see and read how you feel about our disease at the age of 39. I am living my dreams everyday thats why I titled my blog living my dreams with CF.

    So one question if you have yet to post about this on a prior blog. How was it like telling your kids about our disease? I wonder how I will approach this once our daughter is born and old enough to understand what a disease is.

    Thanks for doing this Andy and I am really glad I found you on the blogger community. I look forward to your blogs and you are a rockstar!!

    Also, great pictures of the family everyone looks great!!!

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  2. John, thanks for the response! I tell my children that daddy has something that is different than a lot of people. My daughter has a peanut allergy so I explain to her that daddy is allergic to smoke. Daddy does not have strong lungs so he has to exercise them. Daddy does therapy every day twice a day like most people brush their teeth. Daddy can't skip though. I just try to compare it to things in their own lives and that seems to work. Thanks for asking. Good luck with your daughter.

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