Michelle Ijams and other volunteers with the Cystic Fibrosis Foundation are certain that Tucsonans won’t want to be late for a very important date:
On Nov. 22, they are bringing Wonderland to the Old Pueblo in pursuit of a cure for cystic fibrosis.
“We liked the Alice quote, ‘Sometimes I have believed as many as six impossible things before breakfast.’
“With the gala, it is all about fun and hope and possibilities and dreams — we can dream there will be a cure for cystic fibrosis and eventually there will,” said Ijams, the decor and logistics chairwoman for the 2014 Breath of Life Gala: Top Hats & Cheshire Cats, An Evening in Wonderland.
Ijams is determined to make a cure a reality: The mother of three has two daughters — Isabelle, 9, and Aria, 2 — with cystic fibrosis.
Ijams and her husband, Matt, both Tucson natives, received the devastating news in January 2012, as a result of newborn screening after Aria’s birth. All three children subsequently underwent tests, resulting in Aria’s confirmation and Isabelle’s diagnosis in March 2012.Their son, Sawyer, does not have the life-threatening genetic disease that affects primarily the lungs and digestive system.
More than 2,000 gene mutations can cause CF, which impacts about 30,000 people nationwide. The defective genes result in a build-up of thick mucus in the lungs, pancreas and other organs; common symptoms include persistent coughing, wheezing, shortness of breath, frequent lung infections such as pneumonia or bronchitis, poor weight gain and digestive problems. The life expectancy for people with CF is about 40 years of age.
Ijams said her children’s diagnoses came out of the blue and left her feeling “broken.”
“Cystic fibrosis was not on our radar. No one in our families had ever had it and the diagnosis was beyond devastating. As a mom, you want your kids to be healthy and when something like this happens it is the worst feeling ever,” she said.
Ijams said the girls’ lives — and those of the family — have changed dramatically over the past two years. Aria has monthly visits to the Tucson Cystic Fibrosis Care Center, one of more than 110 care centers and 55 affiliate programs nationwide that receive funding from the nonprofit Cystic Fibrosis Foundation. Isabelle visits the clinic bi-monthly.
Isabelle receives treatments with a nebulizer and inhaler as well as two 20-minute treatments daily with an airway clearance vest — a device similar to a life vest that uses gentle mechanical compression to prevent mucus from settling in the lungs of CF patients.
Aria is too young for a vest and receives 45-minute manual percussion treatments twice daily. The girls adhere to high-calorie diets to facilitate weight gain and exercise regularly to strengthen their lungs. They try to avoid everyday illnesses that can result in months of medication and possibly hospitalization.
“This is our new norm in life and our girls are so amazing. Isabelle’s life changed overnight, but she is so flexible and strong and just goes with the flow and that has made me stronger. And we appreciate every second with them. We just try to have a very positive outlook and live life and have fun,” Ijams said.
Ijams’ optimism was boosted recently with news that the drug Kalydeco, which received approval from the FDA to treat CF patients with the G551D gene mutation, may soon be approved to treat the gene mutation that affects her girls.
“This is huge ... we are crossing our finger and toes,” she said.
The CF Foundation has served as a leader in research for other foundations supporting rare diseases with its drug development model known as venture philanthropy, according to Michele Murphy, executive director of the Cystic Fibrosis Foundation Southern Arizona Chapter.
“Cystic fibrosis is an orphan disease that receives no federal funding for research so the foundation leadership decided to partner directly with pharmaceutical companies and fund research themselves with no vested interest in receiving money. All they want is a cure; they just want to save lives and that approach has advanced research by decades,” Murphy said.
In 1989, foundation-sponsored scientists identified the gene with the defect that causes CF, facilitating a basic understanding of the disease; other milestones have included gene therapy trials; mapping of the genetic structure of the most common bacterium that causes CF lung infections; and FDA approval of various drugs to treat the disease and improve lung function. Research advancements in the past several years have yielded exciting results with Kalydeco alone and in combination with other medication.
“This is a control, not a cure, but it is an amazing advance in improving and lengthening people’s lives. Hopefully by this time next year about half of the people with CF will have a control in place and it will have a big impact on stopping advancement of the disease,” Murphy said.
In the meantime, Murphy said that volunteers will continue to raise funds to support foundation services and programs dedicated to optimizing quality of life for CF patients and promoting CF awareness in the community through events such as the fun, memorable annual gala.
“We try to tap into themes of hope and tap into the fact that this is a community that is hopeful for a cure,” she said.
Ijams reiterated that a cure is attainable and that the stakes are high for CF patients and their families.
“I want my girls to grow up, go to college, fall in love, get married, become grandparents. I want them to grow old, happy and healthy. The only way for them do that is to find a cure for CF,” she said.