Living with Cystic Fibrosis

Posted on 01. Feb, 2009 by admin in Personal Stories

Looking at Mackenzie you would probably never know she is living with a disease. Yet her body is in a constant battle. Some nights, when Mackenzie has an increased cough, I lay awake listening to her. The worries that I had when we first learned what cystic fibrosis was and what it meant for Mackenzie floods my thoughts and I wonder what irreversible damage is being caused to her lungs.

When my daughter, Mackenzie, was born she became one of 30,000. There are 30,000 children and young adults living in the United States with cystic fibrosis. Cystic fibrosis is a genetic disease that causes the body to produce thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. It also obstructs the pancreas from helping the body to break down and absorb food.

When Mackenzie was born, she had a bowel obstruction and was flown by Angel One to Arkansas Children’s Hospital. She received her diagnosis at just four days old. We were devastated. We had heard of cystic fibrosis but really didn’t know what it was. After her diagnosis we went through several stages – including our own type of mourning. It wasn’t that we had lost a child, it was that we lost the life that we thought we were going to have.

We’ve spent the past eleven years tweaking her care as needed. Currently Mackenzie spends two hours a day doing chest physical therapy – treatments to keep her lungs as clear and healthy as she can. She does this by wearing a vest that vibrates to loosen the mucus and inhaling different medications using nebulizers. She also takes enzymes to help with her digestion. Four times a year she has an appointment with a fantastic pulmonary team at Arkansas Children’s Hospital. Her wonderful doctor, Dr. Robert Warren, encourages her to participate in physical activity to act as additional chest physical therapy and to not suppress her cough (coughing is very important for CF patients, but they sometimes suppress it to not draw attention to themselves). He also reinforces the importance of her nutrition. CF patients with 50% Body Mass Index have been shown to have better lung function but the problems with digestion mean she needs many more calories per day than most kids to achieve this and to grow. Mackenzie also is on rounds of antibiotics to battle bugs in her lungs. In our case, most of the time we are fighting staph.

Cystic fibrosis is not the same for every patient. Gene mutations, severity, and medications vary from patient to patient. Sixty years ago few children with cystic fibrosis lived to the age of five. When Mackenzie was born the average median age of survival was 31 and today it is 37. It is such a great improvement, but still a heartbreaking number for any parent.

We often joke that doing Mackenzie’s treatments is like brushing teeth, something we’ve been doing for so long we don’t have to think about too much. At four days old we were feeding her enzymes sprinkled on applesauce baby food with a spoon and “bopping” her for her chest physical therapy. I want to shout from the rooftops how proud I am of her for everything she does. I know she doesn’t want to wake up an hour earlier than her friends, spend two hours a day hooked up to tubes and take 30 pills a day – but she does it and almost always without complaining! On the rare occasions she does complain, it’s because treatment time is interrupting play time with her sister or her friends. I am so thankful for Mackenzie’s friends and Peyton – they are all so supportive and helpful. They make her feel good about her vest by thinking it’s so fun to put it on and vibrate, they help her remember her pills, and they don’t mind when she’s coughing.

I often wonder when Mackenzie sees her first star or before she blows out the candles on her cake if she wishes for puppies… a cell phone… clothes… or does she wish to not have cystic fibrosis. If by chance she is wishing to not have CF, there are a lot of people working on making that wish come true. I expect Mackenzie – and all the CF children I’ve met along the way – to live a long full life. Family, friends and strangers help us every year to raise funds that will make sure this happens. Not only does this research help to extend Mackenzie’s life but it also helps improve the quality of her life. Jason and I are fortunate to have very supportive friends – they bring us dinner after a hospital stay, they participate in fundraisers, and they listen to me when I need to vent. I’ve also met other CF parents that I now call my friends. We all work together to raise awareness and funds to keep increasing that average median age of survival and our children’s quality of life. All the while we work to fulfill the hope to grant my wishes — a cure for cystic fibrosis.

Wine Opener to Benefit Cystic Fibrosis Foundation

The Northwest Arkansas Cystic Fibrosis Wine Opener is a relaxed, yet lively wine tasting event that benefits the Cystic Fibrosis (CF) Foundation. Sip great wines from international and domestic wineries. Savor great cuisine at festive tasting stations from the area’s hottest chefs. Along with delicious food and wine pairing, guests will be treated to lively auctions and entertainment. When the Foundation was established in 1955, children with CF were not expected to live long enough to attend elementary school. Due in large part to the Foundation’s aggressive investments in innovative research and comprehensive care, the predicted median survival age is now 37 years. Despite advances in CF research and care, we still lose at least one precious life to CF every day. http://arkansas.cff.org/nwawineopener

Doubletree Guest Suites in Bentonville
February 20th, VIP Reception – 6:00 – 7:00 General Event – 7:00 to midnight
VIP tickets $100 / General Admission: $6

Story by Mackenzie’s Mom Christy Jobe of Bentonville. christyjobe@hotmail.com