About CF

Paige's Story...

THE INTRODUCTION:
Paige Ella Christensen is a special little girl who is six. She loves being outside, swimming and fishing at her Grandma and Grandpa’s cabin, playing Go Fish with her Grandma Theo after school, humming, and bossing her little sister, Brooke, around.


Looking back now, everything seems so obvious. The bloated tummy that started at 2 weeks of age, the "colic" that started at 6 weeks of age, the diagnosis and treatment for acid reflux at 8 weeks of age, the inability to digest supermarket formulas at 6 months of age, the pneumonia that just about killed her at 9 months of age, the complete nasal congestion that started when she was 11 months of age, the pneumonias she had at 12 and 15 months of age, the perpetually "drippy" eyes and nose that started at 18 months of age, the stomach cramps, irritability, fatigue, listlessness, headaches and pallor that started at 24 months of age...


THE DIAGNOSIS:
After visiting 6 different doctors multiple times over the course of a year, Paige was diagnosed with allergies, lactose intolerance, asthma, enlarged adenoid, and chronic sinus infections. After trying over 20 different drugs and having her adenoid removed with no improvement, I started researching Paige's health on my own.


On June 6th, 2005, after insisting doctors perform a sweat test on my little girl, my hunch was confirmed; my daughter has cystic fibrosis (CF). The doctor that broke the news to me kindly informed me that my daughter "wouldn't live long" and told me someone from a CF Center would be in touch with me. All that kept running through my mind was the number 30. Everything I had read about CF in the preceding months had listed 30 as the average life expectancy. That ugly statistic doubled me over - there was a strong chance I was going to watch my daughter die. My whole world and the course of my life changed in an instant.


THE DISEASE:
The first thing everybody asks me is "what exactly is CF?" CF is a genetic disorder that Paige inherited from my husband and me. Turns out both of us are unknowingly carriers of a genetic mutation. Neither of us has CF since we each only have one copy of this mutation (1 in 25 Caucasians are unknowingly carriers of CF), however Paige has CF because she inherited 2 copies.
This genetic mutation makes certain cells lining Paige's tear ducts, sweat glands, nasal passages, lungs, stomach, pancreas, liver, intestines and reproductive organs not function properly. Instead of these cells producing a thin, protective mucous like they do in you and I, Paige's cells produce a thick, oppressive mucous. Due to this overly sticky mucous, Paige gets blocked tear ducts, has constant nasal congestion, sweats a lot (increasing her risk for dehydration and overheating), has mucous sitting in her lungs collecting various bacteria, has acid reflux, and has a blocked pancreas that is unable to break down the food she eats (leading to malnourishment, a swollen tummy, and frequently diabetes).


Of all of the organs affected by CF, the lungs are the most vulnerable. As Paige gets older, the bacteria that take up residence in her lungs will get more aggressive. The more aggressive the bacteria, the more inflammation and scarring Paige will have in her lungs. Eventually, the inflammation and scarring will lead to lung failure. If lung transplantation is not successfully performed; lung failure will result in death.


THE INITIATION:
Paige was hospitalized within 3 days of her diagnosis and initiation into life with CF began. Invasive procedures, CT scans, IV drugs, blood draws, pills (and more pills), ultrasounds, bronchial drainage treatments...the list goes on and on.
On top of the medical intervention, my husband and I were bombarded with crash courses on the disease, CF nutrition, respiratory care, genetic counseling, obtaining insurance coverage for persons with chronic diseases...the list goes on and on. For the 8 days that Paige was hospitalized, we got out of our room IV-free for a total of 6 hours. Paige handled everything miraculously well, but this hospitalization changed her. I saw it in her eyes; they looked older, weary and more mature. At almost 3, Paige left that hospital knowing her life would never be the same again. Carefree childhood was but a memory.


THE DUMPS:
Paige now takes 11 different medications for a total of 53 pills/doses throughout each day. Paige has 2 thirty-minute sessions of bronchial drainage each day in which she wears a vest that squeezes her chest and shakes her to loosen up mucous in her lungs. Paige needs to consume an enormous amount of calories each day to get the nutrients she needs so she can grow properly. Paige needs to avoid exposure to viruses. Paige will have good days with this disease, and Paige will have bad days with this disease. By teenage years, the typical child with CF is hospitalized once a year for up to 21 days.


THE HOPE:
In 2003, statistics said Paige would likely die by the age of 31. Statistics now say Paige will likely live to be 38. It is primarily research being funded by the Cystic Fibrosis Foundation (http://www.cff.org) that has given my daughter 7 precious more years to go to college, get married and have children. The Cystic Fibrosis Foundation's mission is to cure CF in the next 10-20 years. Please help make this possible. Please help me achieve my new goal in life: to love my daughter until my dying day. Thank you for your support.