Beth Sercus, a 53-year-old accounting consultant from Wharton, N.J., remembers the day her 3-year-old Brian was diagnosed with cystic fibrosis, a genetic disease that affects the lungs and digestive system.
A resident she had never met walked into the hospital room and said, "We think your son might have cystic fibrosis. He may live to be 20 years old. You know, if he had cancer, he would have a better chance of survival."
"Then he walked out of the room," said Sercus. "I was 25, been divorced for one month, had no idea where his dad was, and had never heard of CF. I called my mom right away in tears."
Brian Sercus surpassed those expectations and lived to be 30. He died of a virulent infection last summer, just hours away from a double lung transplant surgery that might have given him decades more of life.
Now, in his memory, his mother has been an outspoken voice for medical programs that are tailored to the social and intellectual needs of adult CF patients.
With medical advances – new medications and more sophisticated transplantation techniques -- patients are now living well into their 30s and 40s, and for the first time this year, doctors say the number of adults with cystic fibrosis will exceed the number of children with the disease.
But the hospitals and rehab facilities where adult patients spend weeks and sometimes months of their lives have not yet caught up.
Sercus said that as her son grew into adulthood -- getting a college degree and later working for a health insurance company -- the medical community still treated him like a child.
When he was 20, Brian was still going to the Babies Hospital at Columbia Presbyterian.
Later, at an adult hospital, the medical staff didn't know what to do with him. "He was put in a room with a 90-year-old man and the doctors and nurses had no clue how to treat CF," said Sercus. "There was nothing for Brian to do, except just sit in bed and watch TV."
Cystic fibrosis, or CF, is an inherited disease that causes the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening infections.
About 30,000 Americans have the disease and 1,000 new cases are diagnosed each year, according to the Cystic Fibrosis Foundation.
Nearly 1,600 people with the disease have received lung transplants since 1991, according to the CF Patient Registry. As many as 90 percent are alive one year after transplantation and half are still living after five years.
Justine Komin, a 26-year-old from Wichita, Kan., was one of the lucky ones. She had a life-saving double lung transplant at the University of Minnesota in 2010. Her 45-year old boyfriend is living with the disease, and so is her 55-year-old aunt.
For Komin, like Brian Sercus, her survival depends on frequent and sometimes lengthy hospitalizations to clear her lungs of bacteria-prone secretions.
"Before my transplant, I was in the children's ward and I was 23 at the time," said Komin. "The nurses didn't know how to accommodate me. I am so knowledgeable about my disease and what worked and what didn't ... They just weren't mentally prepared to deal with a patient who wanted a say."
Komin was often in the hospital for two weeks at a time; her therapy included 40-minute sessions five times a day to clean out her lungs. But dividing their time between adults and children, the respiratory therapists often had to cut those sessions short.
And patients like Komin and Sercus are left to languish with few programs geared to their age group, or facilities that might allow them to continue working.
Cystic Fibrosis Is Now An Adult Disease
"Now we are entering the days when it is an adult disease," said Dr. Ahmet Uluer, director of the adult cystic fibrosis program at Brigham and Women's Hospital and Children's Hospital in Boston where Brian Sercus was treated.
"The burden is that it's a chronic illness and adult hospitals are just not geared like the pediatric hospitals, to taking care of these patients," he said. "But they are changing."
Of the 600 CF patients at Brigham and Women's, 300 are older than 18. "It's definitely a population that needs a lot of services," said Uluer. "It's a daily challenge."
Patients with multi-organ problems "can't be dealt with in 10 minutes," according to Uluer. Their medical visits can last up to three hours and hospitalizations can last days to weeks and months.
Beth Sercus praised the medical care at Brigham and Women's as "top notch" and said her son had become close to his medical team when he was treated in 2009.
But, she said, long hospital stays can "wreak havoc" on both patients and their families.
"Everyone still has to pay bills, and work," she said. "If your family wants to stay close, many times the expense of hotels, even the ones that provide discounts to families, can make it difficult."
When Brian's health began to decline, his employer offered to let him work remotely, but that was impossible, according to Sercus.
"The problem was that when he was hospitalized, the wireless network was not private, so he was not able to work," she said. "Same for me. I would bring my laptop, but was unable to work."
Parking, too, was an issue, and Sercus said a hospital-provided service might have helped. "How many times did I have to get his car out of impound because of the crappy parking situations? ... Or how much money did he spend to get his car out of the hospital parking lots when he did drive himself?"
Boredom was also a problem. "TV just doesn't cut it," said Sercus. "You are left there to sit and vegetate. This does not help to maintain a positive attitude."
In 2009, Brian had a particularly virulent bacterial infection that is contagious, so he had to be isolated from other patients in a private room for many weeks. Such infections are typical among CF patients.
"We tried and failed to hook up his PS3 and XBox, but the portable ones would have been a lifesaver," she said.
When the patients is not in isolation, Sercus suggested that larger rooms for long-term CF patients would make it easier for family and friends to visit. Or hospitals might provide bedding for visitors to spend the night.
Last April, in honor of what would have been her son's 31st birthday, Sercus raised $2,500 for the adult CF program. The hospital has earmarked it for a daily "special meal" of a patient's choosing. One of Brian's biggest complaints was hospital food.
Uluer admits, "CF is very isolating." But he said Beth and Brian Sercus did leave an impact.
Now, three years later, patients have unlimited supplies of food -- so important for weight gain, which is a challenge for adults with CF. The hospital reimburses families for some of their parking costs.
Patients are now supplied with computers if they don't arrive with laptops. Those who have Play Stations and X Boxes can hook them up in their rooms.
"We definitely want them to play games when they get out of bed and be more physical," said Uluer.
Brigham and Women's program is also hosting an educational event June 17 and those patients who are confined to their rooms can watch it and participate in the discussion via Ustream on iTunes.
But often it is the psychological, not the physical things, that matter most.
"Brian's friends are what kept him alive," said Uluer. "They would send him things like a birthday singing gorilla to his room."
Brian had a strong and vibrant personality – and faced his disease head-on. When he got angry, he would chastise his bacteria out loud, said Uluer. "He had a spirit about him that made it that made it very easy to care for him, he wasn't defined by his CF."
As for Komin, she wants to use her second shot at life to help others.
Komin will get a master's degree this fall in public health, hoping to serve as a liaison to help doctors better understand patients' needs.
Her lung transplant has left her with stenosis, which makes her respiration "like breathing through a soda straw."
"I can't run a marathon, but I don't like to do that anyway," she said. "But I can plan my life now. I couldn't before."
To learn more about cystic fibrosis and opportunities to donate, go to Children's Hospital Trust at Children's/Brigham in Boston or call Tara Hardimon at 857-218-5082. Specify "Brian's Circus," benefiting the adult cystic fibrosis program.