When cystic fibrosis patient Amy Long woke up one night eight years ago coughing up blood, she was afraid for her life.
Her doctor told her to rush to the emergency room at his Sacramento hospital, which was three hours away from Long’s Coalinga home. So, she packed her daughter into the backseat of the family car, had her husband drive, and for three hours sat in the passenger seat, praying she wouldn’t drown in her own blood.
Now, thanks to a new clinic in Fresno, Long doesn’t have to wait anymore to receive lifesaving treatments when she needs them.
A new UCSF Fresno clinic at Community Regional Medical Center is prolonging the lives of those affected with cystic fibrosis. The only one of its kind in the central San Joaquin Valley, the clinic currently treats more than 40 patients with the disease.
Dr. David Lee came to Fresno nearly two years ago from Brown University to open the first clinic to treat young patients with cystic fibrosis, a disease that affects nearly 28,676 people across America, according to a 2014 Cystic Fibrosis Foundation report.
Cystic fibrosis is a genetic disease that affects the cells that produce mucus, sweat and digestive juices. The disease makes such fluids become thick and sticky. They then plug tubes, ducts and passageways in the body. It can impact every organ, from reproductive tissue to bone.
I do nebulizer treatments and chest therapies every day … so it’s definitely like having another full-time job.
KAIL’s Austin Reed, who credits the Fresno cystic fibrosis clinic for helping make his life more manageable
“Unfortunately it can affect almost every part of your body, so it becomes an extremely complicated disease,” Lee said. “Every patient has a different severity of the disease, depending on the genetic defect.”
Lee and his seven-member team – including a pediatric pulmonologist, pediatric gastroenterologist, social worker, nutritionist and respiratory therapist – treat both child and adult patients. Before the clinic opened, Lee said, patients had to travel to San Francisco, Los Angeles or Sacramento for care. Although Valley Children’s Hospital treats child cystic fibrosis patients, they must leave Valley’s care by age 21, Lee said.
Average patients can see up to seven specialists in one visit, with the usual appointment taking two to three hours, Lee said. Add in hours of driving, and frequent appointments depending on the severity of the disease and medications required, and many patients weren’t able to have access to proper care they needed before the clinic opened.
Coalinga woman finds hope
Long is one of those patients. The mother of three and college counselor stopped getting proper treatment entirely for almost three years, battling health insurance companies and having to be prescribed asthma-related medications – lower versions of what she needed for her treatments.
As a full-time student at Fresno State, she often had to take treatments in her car and on her way to school just to save time.
When she got the swine flu during an outbreak in 2009, her lungs began to deteriorate for the first time and she battled the flu for more than a year. By the time she was able to see Lee, her lungs had deteriorated to 50 percent capacity, and Long was scared.
“I was pretty emotional, really upset when I met Dr. Lee,” Long said. “When I first met him, he told me ‘We’re going to become like family, and I’m going to get to know your family, I’m going to get to know you and we’re going to be a team.’ ”
Lee held her accountable, Long said, and helped her overcome an eating disorder she had developed over the years along with knocking out the infections she already had. Long was finally healthy, but even a typical day for a cystic fibrosis patient isn’t easy.
KAIL reporter has a mission
Austin Reed, a broadcast reporter for KAIL’s MyNetworkTV who has cystic fibrosis, started a GoFundMe account to raise money for the Cystic Fibrosis Foundation, the leading-research organization for the disease.
“Every time I eat I have to take six to eight pills to just digest my food,” said Reed, who lives in Madera. “I do nebulizer treatments and chest therapies every day … so it’s definitely like having another full-time job.”
Although the severity of the disease varies from patient to patient, most suffer from some form of lung disease that causes scarring and infections. Lung problems are the most common cause of death, Lee said. Even though lung transplants are available, Lee said survival rates aren’t as high as other forms of transplants like heart or liver.
“In the 1980s, the average life expectancy of a patient with cystic fibrosis was early 20s,” Lee said. Thanks to advances in medical research and treatment, that number has increased to the early 40s, Lee said. Lee’s oldest patient was 67, and he said with the right care and motivation, patients can have fulfilling lives.
Many of the achievements in cystic fibrosis, Lee said, have emerged from the foundation.
Among the key discoveries: a pill that treats a specific form of the genetic disease actually corrects the disease at the cellular level, Lee said. In some cases, he said, cystic fibrosis is close to being cured.
“Unfortunately, this only represents about 4 percent of patients with CF, but we are working very hard with all the care centers nationally to expand research for other patients,” Lee said. “You can imagine how often do you find a genetic disease that can be pretty much cured?”
Both Long and Reed have lived those full lives Lee encourages all his patients to aspire for. Both have experienced milestones this year. Reed is expecting twins in July and Long just turned 38, an age she was told from birth not to expect to reach.
“My whole life I’ve been chasing the life expectancy,” Long said. “When I was born my mom was told basically that I wouldn’t live to be a teenager. Then it moved up to 21 and it moved up to 30, and then now I’m here at 38. I hit the life expectancy impact for the first time, and I’m still doing well. I’m able to work and maintain my children and my household.”
Lee said he has learned from his patients about life, and what is important. They don’t care about how many books he publishes or papers he writes, but how deeply he is involved in every aspect of their lives, and that he has a sense of gratitude. He says he’s not just a doctor, but tries to be a friend and a life coach.
Lee looks to the future now for his patients, focusing on transitional care at an early age. He is partnering with other cystic fibrosis clinics at UCSF Benioff Children’s Hospital and Valley Children’s Hospital to provide better transitional care for cystic fibrosis patients starting at age 12. He wants to prepare children to deal with their disease, their medications and the consequences of not taking them.
With every moment important for patients with cystic fibrosis, having a clinic in Fresno has saved time. Long says that she now gets to spend more time with her family, which helps balance her life. She feels more connected to the Valley now, and wants to give back.
“I want to be able to make a difference somehow and utilize my life experience and my gifts and abilities to impact my community,” Long said. “I want be a role model for my family and others who have been in worse pain than myself. I’ve been really blessed.”
Megan Ginise: 559-441-6614
How to help
To donate to Austin Reed’s “Cystic Fibrosis Benefit” account, go to https://www.gofundme.com/3z48ns28