My Extensive Medication List

Being asked to list my medication at the doctors is more than just a small list. But trying to explain my medications to an everyday person is way more difficult. Which is why I feel it's important to explain cystic fibrosis medications in layman's terms for everyone to understand. Here is a list and explanation of the medications that I take for my CF on a daily basis. 

Albuterol: helps to expand airways, so it is easier to breathe. It can be taken through a nebulizer or inhaler. 

Pulmozyme: thins mucus so it is easier to cough up. This is taken through a nebulizer. 

Tobramycin (Tobi): a 28 days on, 28 days off antibiotic inhaled nebulizer to help fight bacteria. 

Cayston: also a 28 day on, 28 day off antibiotic inhaled nebulizer to help fight bacteria. 

* the two antibiotics are rotated so that an immunity is not built against either one. 

Multi-Vitamins, Vitamin E & Vitamin D3: these are all because CF'ers don't absorb enough from what we eat that we need to take the extra doseage. 

Antacid: to help with acid reflux with is a symptom of CF. 

Zenpep: enzymes that digest food for the body because a CF'ers pancreas doesn't work properly. 

Vest: a machine that looks a lot like a life jacket. It fills with air then shakes, to shake the mucus loose so it is easier to cough up and clear out of the lungs. 

A Day in the Life with Cystic Fibrosis

Most people have a daily routine that they follow. But with cystic fibrosis the daily routine is a little more complicated.I can't just wake up brush my teeth, put on make up and head out the door. Instead I have to do treatments every morning, afternoon and night. 

A day in my life looks a little something like this. I wake up and start my nebulizers. A nebulizer is a machine that connects to a mouthpiece, the mouthpiece is filled with a liquid medication, the nebulizer machine pump air through the mouthpiece turning it into a vapor which I inhale. In the morning I only do one of these, followed by my vest. The vest is a machine the shakes my lungs so the mucus in them can come loose and I can cough it up. After the vest comes the antibiotic nebulizer.  This round treatments last from 45 minutes to an hour.  Once I am done my treatments I eat breakfast. My breakfast consists of high calorie and high-fat. My go to for breakfast is whole milk with heavy cream and some type of flavoring. In one glass of that milkshake there's about 500 calories. Whenever I eat I have to be sure to take my enzymes. Enzymes are what digest my food for me. Once I am done my breakfast I wait a little while then I exercise. 

After all that is done I can get on with the rest of my day like normal. That is until around three o'clock, when I need my afternoon nebulizer. This only takes about 15-20 minutes. So it doesn't take up too much of my normal life, but it can be a pain to stop what I am doing to get a neb done. After the neb comes a high calorie and high fat snack. The snack is important to give me a boost of energy and extra calories during the day. 

After afternoon treatments my day continues until about seven o'clock when it is treatment time again. This round is about the same as the mornings. Starting with two nebs fallowed by the vest than another neb. This round last an hour. 

Most of my day is normal and I am just like everyone else. It's just that I have moments where my life pauses, and the only thing that matters is breathing in a nebulizer nice and deep or coughing up the mucus as I shake. It's a strange thing to get used to and not everyone can handle it. I have lost friends because I couldn't have a normal life without treatment interruption. And at the same time I have gained great friends. My treatments are a true test, to see if you can handle my lifestyle. Not many make the cut, but I am blessed and thankful for the ones that do.  

Symptoms of my Life

In my first post I briefly explained what Cystic Fibrosis is, well here is a better scientific explanation. 

Cystic Fibrosis is an inherited life-threatening disease that affects the cells that produce mucus, sweat and digestive fluids. This causes severe damage to the respiratory and digestive systems. In a normal person those fluids are thin and slippery, but in someone with CF the defective gene makes the fluids thick and sticky. This causes the fluids to plug passageways in the lungs and pancreas, as well as other parts of the body. To have CF a person must inherit two copies of the defective gene. One from each parent, both parents must have at least one copy of the gene. If someone has just one copy of the CF gene they are called carriers, but they do not have the disease themselves.

Most all people with cystic fibrosis will have the following symptoms; 

Cough: a constant daily cough which can sound similar to a smokers cough, with phlegm, and sometimes blood. 

Gastrointestinal: bulky stools, diarrhea, severe constipation, fat in stool, or heartburn. 

Developmental: slow growth, delayed development, or delayed puberty.

Respiratory: wheezing or shortness of breath.

Whole body: inability to exercise or fatigue. 

Digestive: pancreas does not work properly making the body unable to digest food on its own.

Also common: male infertility, salty sweat, pneumonia, infection, nasal polyps, pulmonary hypertension, club finger, weight loss, trouble gaining weight, and acute bronchitis. 

Cystic Fibrosis - Me & My Shadow

My name is Ashley and I am 21 years young and this is my story. 

I was born to a young couple in 1994. Right from the start I was a sick baby. I cried a lot and went through diapers like it was my job. I also was "failure to thrive" which means I wasn't gaining weight. I was so sick that by the time I was six months old an in home nurse was coming to the house to help my parents learn how to take care of me. One day one of the nurses gave me a kiss, when she did she said I tasted salty. She had my mother "taste" me. After that she asked if I had been tested for Cystic Fibrosis and suggested my parents find out. Low and behold I had CF. 

Cystic Fibrosis is a genetic life threatening disease. It mainly effects the respiratory and digestive systems. It causes the mucus in the body to become very thick. In the respiratory system the mucus coats the lungs. Making it harder to breathe and harder to do daily activities. In the digestive system the mucus is mainly in the pancreas. It blocks the enzymes inside the pancreas from getting out to digest food. 

I could only imagine how overwhelming that could have been for my parents to learn. Not only were they faced with raising a child young, but now a life threateningly ill child. 

When I was first diagnosed CF was still a fairly new disease. Which made it hard for my parents to take care of me. But with the help of the whole family they pulled together to give me as normal a life as someone with CF could have. 

I went to school, had friends, played sports, went on field trips and did my treatments. Looking at me you wouldn't be able to tell I am sick. Cystic Fibrosis is not something you can see in/on a person. But you can see it in all the medications and treatments we do/take. 

In my lifetime I have probably been in the hospital 15 times. Each ranging from a week to three week long stays. I've had around 100 IVs, two PICC lines, and one port-a-cath placed. I take seven pills a day, not including the enzymes I take every time I eat something. I do three types of nebulizers a day along with my Vest. I have to eat a high calorie, high fat diet. If I don't do these things everyday the mucus in my body will thicken, causing bacteria to grow which will lead to infection. I have seen what happens to people who don't do what they are supposed too. They loose their fight with CF and die. It's a little harsh I know, but it's a reality every CF'er lives with. 

The life expectancy of someone with CF is in the early 40's. I'm half way there... But that won't stop me from fighting the fight for life. With new medications an treatments beig developed every day, my strength to fight for life grows. 

There are 30,000 of us with  Cystic Fibrosis in the USA. And without daily medications and treatments someone with CF cannot survive. However these things coast anywhere from hundreds to thousands of dollars. Not all of us can afford them, and the US government gives us no funding. Which is why it is so important to spread awareness for this disease. To get the funding needed to help CF'ers afford medications and treatments. So that they can keep up the fight for life without worry. 

Please share this with everyone you can, help spread the word. 

Please take a moment to donate to the fallowing Cystic Fibrosis foundations as well: 

Www.cff.org

Www.christinadavidsonfoundation.org