Country Bumpkin: 65 Roses

This is unprecedented. Three posts in a row!! I'm usually a once-a-week girl (if that) but I've just been so excited about stuff lately that I must share it! Today's topic is one that I am very passionate about and the more people who know about it the better it will be. I'm talking about 65 Roses. What's that you say? Let me explain.

In 1965, a woman named Mary G. Weiss joined an organization called the Cystic Fibrosis Foundation after learning all three of her sons had Cystic Fibrosis (CF). Her role for the foundation was to call every civic club, social, and service organization for financial support for CF research. Her middle son, Richard age 4, listened closely to every call she made. After several calls he told her, "I know what you're working for." How could he know? He's only four years old! Shocked, Mary asked, "What am I working for, Richard?" He answered, "You're working for 65 roses." Since then, 65 Roses is what young children with CF call their disease.

So what is Cystic Fibrosis? It is a genetic disease affecting the lungs, pancreas, and digestive system. Mutated genes cause disturbances in the salt and water distribution creating thick, sticky mucus. This mucus causes two sever problems:

First in the lungs. In a healthy person the CF gene (a.k.a. CFTR [Cystic Fibrosis conductance Transmembrane Regulator]) is found in cells that line the lungs and pancreas. It allows particles found in salt (such as chloride and sodium) to pass in and out of the cell. With a defective gene occurring in CF patients, the salt balance is disturbed. The faulty genes let salt and water escape causing the mucus that naturally lines our lungs to protect them from germs become thick and sticky. The mucus is so hard to dislodge and cough out that it often clogs the airways and leads to infections which damage the lungs.

The second problem is in the pancreas. This organ produces enzymes that break down food so that their nutrients can be absorbed. The thick mucus prevents the pancreas from secreting these enzymes to the intestines. If our body cannot break down food it passes through and it's as if we ate nothing at all. Children with CF are often smaller than others even though they have healthy appetites. Since they cannot get the nutrients they need for a strong body, they are even more at risk for infection.

The predicated median age of survival for a CF patient is in the late 30's. However, this number is growing all the time thanks to all the hard work of the folks at the Cystic Fibrosis Foundation. They work endlessly on finding a cure and treatments to better patient's quality of life. A new drug called Kalydeco was approved by the FDA this past January 31st. It is the first drug that targets the underlying cause of CF (the faulty gene and its protein product). With medications like this and diligent treatment, some patients are living up to their 60's!

This is Emily. She was 6 months old when she was diagnosed.

Why am I so passionate about this disease? Almost seven years ago, we found out that my little niece, Emily, has CF. It was devastating. I had never even heard of this before. For a patient to develop this disease both parents have to carry the gene. One our of four children will have it. That was exactly my sister's conditions. She has four children and only Emily was CF. This was a complete shock since we didn't know of anyone in our family who had this strange disease. It was a very scary time at first. Everything was new and we all had to learn her treatment schedule. My sister is the strongest person in the world. She has accepted this as part of their lives and incorporated it into everyone's daily routine.

Wonder what it's like for Emily everyday?

She has to wake up at least 30 minutes before everyone else in the morning before school to take her breathing treatment and wear her vest. The vest fits snugly around her chest and vibrates increasing how hard it beats every few minutes. This helps loosen the mucus from her lungs so she can cough it up. She has to do the same routine before bed.
She must take several medications for the other symptoms caused by CF in the morning and before bedtime.
Before every meal she must take enzymes to help her digest the food.
If she has a particular bacteria growing in her lungs she has to take her breathing treatment and vest compressions after school.

She has to do this every single day. If the family goes on a trip, all her meds and treatments go with them. If she comes to stay with Mamaw and Papaw for the weekend, all her meds and treatments come with her then too. Most CF patients will have to have several bronchoscopys (lung wash) in their life and most will have a lung transplant. Emily had her very first lung wash this past winter. What a trooper! It sure didn't slow her down any. She had to stay home for three weeks after that for intense IV therapy but kept on playing as if nothing had happened.

She also has limitations to what she can do or be around. She will never be able to go to an indoor pool, cave, or get in a hot tub. These are damp places where bacteria thrive. She cannot be around other children with CF because they can catch bacteria from one another. She cannot take certain medications such as decongestants. These meds dry up the mucus. She has a hard enough time as it is coughing it up when its moist.

But life is not all bad for her. She is spunky, energetic, and full of life. Nothing will slow her down. Emily acts as if nothing were wrong with her. If you just met her you would never know. She lives happy everyday and we all thank God for every second he grants us with her.

Me and Emily planting peppers for Mamaw

May is National Cystic Fibrosis Month. Spreading awareness is the key to a cure. Currently there is no cure for CF. Only the symptoms can be treated. I believe that one day they find one. Until then we will continue to raise awareness and keep funding research. There are all kinds of different ways to help CF patients. Get involved by:

Making a donation to the Cystic Fibrosis Foundation for research
Volunteer (Great Strides walks are happening all over!)
Get your company involved to make donations
Fund-raising
Write to your congressmen to support CF research
Shop!! Certain shops offer discounts and some are giving big percentages to the foundation for every item sold.

Together we can make a difference. We can help give these kids and adults the chance to live better, healthier lives. I'll leave you with this video from the CFF. It really shows you what fighters these people are and how much we love them.