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Many ventilator-dependent young adults at a crossroads

Dimitra Cilento visits her 22-year-old disabled daughter, Stephanie,

Dimitra Cilento visits her 22-year-old disabled daughter, Stephanie, at St. Mary's Hospital for Children in Bayside, Queens, on Dec. 10, 2015. Stephanie, who is connected to a feeding tube and sometimes a ventilator, is one of thousands of young adults aging out of pediatric care facilities in the metropolitan area. Photo Credit: Newsday / Alejandra Villa

Stephanie Cilento, a young woman who often struggles to breathe and who needs a feeding tube for nutrition, is a member of a quietly growing population: Young people with medically complex conditions in need of skilled round-the-clock care.

Some of these patients have traumatic brain injuries or were born prematurely and have lifelong disabilities as a result. Others arrived in the world with severe birth defects that caused heart malformations or neurological disorders — medical conditions so problematic that living at home is difficult, if not impossible. And there are others still; no two cases are alike.

All, like Cilento, 22, rely on technological assistance for survival, and range from toddlers to young adults. This population in New York, estimated at 24,000, is part of the 3 million coping with medical complexities nationwide — and many are facing a crossroads.

Severely debilitated young adults, Cilento included, have aged out of institutional pediatric care; many more are on the brink of soon doing so. Yet with only geriatric-oriented nursing homes as an alternative, most have nowhere to go. Their cases are too overwhelming for families to handle at home. Pediatric institutional care ends at 21.

Technology has helped drive the expansion of this population, doctors say, but that growth has outpaced the health care system’s ability to meet the needs of patients with ongoing, age-specific care.

Worse, some similarly affected children have been transferred out of state because there are not enough institutions in New York with space for youngsters requiring ventilator assistance. The demand for specialized respiratory care is so high that some of these New York patients have been transferred to New Jersey, others to Pennsylvania and a few as far north as Massachusetts, experts say.

This displaced, ventilator-dependent population is estimated at more than 50 children and teens who can’t live in New York because ventilator beds are occupied. Experts say efforts are underway to solve the dilemma, but some families contend solutions aren’t arriving fast enough.

Cilento has a severe congenital condition that has impaired her motor function, and because of it, she is part of an even more disabled subset of medically complex patients, those considered medically fragile.

While patients like Cilento are located throughout the state, most medically fragile children and young adults reside in the New York City metropolitan area, with the largest concentration living in the area surrounding the Nassau/Queens border, a study based on state Medicaid data has found.

Some medical experts attribute that concentration to the health care facilities in the area — Northwell Health in Lake Success (previously known as North Shore-LIJ Health System) and St. Mary’s Hospital for Children in Bayside, Queens, which specializes in the care of medically complex children.

‘I can’t do it’

Cilento, whose family lives in Howard Beach, Queens, has been an inpatient at St. Mary’s since her teens. But the hospital is designed to meet the needs of children, which means the young woman’s time at the facility has run out. Her family is frantically searching for somewhere to place her, but nothing appropriate has turned up.

“Stephanie has severe cerebral palsy with a seizure disorder,” said her mother, Dimitra Cilento, who shudders at the thought of institutionalizing her daughter in a geriatric nursing home, the most common alternative for young adults who have grown too old for pediatric care.

“She is non-ambulatory and has been here for six years, right after turning 16,” Cilento said.

The mother of three other children, 13 to 20, wishes Stephanie could stay at St. Mary’s, where she said her daughter receives extraordinary care. Mindful of that impossibility, Cilento at least hopes her daughter can be in an environment centered on people her own age.

Cilento cared for Stephanie at home until she was 16, but a tracheostomy — a windpipe incision to which a ventilator tube is attached — requires fastidious care and was beyond what she could deliver at home.

While still at home, Stephanie frequently developed pneumonia, causing her to be rushed to the hospital, where she was confined to an intensive care unit multiple times.

Home care is not an option. “I can’t do it. This child under my care would die,” Cilento said.

She noted that home care relies on help from home health care agencies, which Cilento sometimes found to be unreliable. “If you don’t have a nurse that day, you can’t go to work, or take care of your other children or even go to the grocery store. That’s it. That’s your life.

“Agency nurses also tend to be older, and that means over 50 and they can’t lift. So there are a lot of challenges.”

Facility planned for LI

At a recent Manhattan conference on New York’s growing population of medically complex and fragile patients, experts outlined the needs of this community. Housing for young adults was underscored as critical.

A “homelike” facility — the first on Long Island and in the country — is planned and will likely be in Nassau County, possibly in New Hyde Park, within a year, planners close to the project say. Cilento said her daughter is already on the list. But competition is keen. Even when the facility is ready, it will house only six to eight patients, and state health officials estimate that 9,100 young adults are aging out of pediatric care.

The facility will be outfitted with hospital beds and feature 24-hour nursing care. It is supported through funding from the state Office for People with Developmental Disabilities.

“We have an idea of what the structure would be like and what the rooms would be like, but we are anticipating a lot of fundraising to supplement this project,” said Anita Dowd-Neufeld, a senior vice president at Family Residences and Essential Enterprises in Old Bethpage, a company that develops housing for special-needs populations. A residence for medically complex and fragile patients is a first-of-its-kind undertaking, she said.

Dr. Edwin Simpser, president and chief executive of St. Mary’s, said New York State recently approved accommodations for medically fragile children, but there is still a need for more.

“They have approved additional pediatric long-term care beds in Westchester County,” Simpser said. “But we want to have more space for kids in Nassau, Queens and Suffolk and to try to bring those children back from out of state.”

Technology as savior

Simpser said the population of young, medically complex and fragile patients of all ages is growing, not because more youngsters are being born with severe disabilities or because injuries are occurring at increasing rates. Instead, the population is rising because medical advances are lengthening the lives of youngsters who otherwise would have died.

“All of this is happening because of technology. It is allowing children to survive illness and injury,” said Simpser, who along with other medical experts at St. Mary’s is advocating smoother transitions from pediatric to young adult care.

Simpser defined the conditions that lead to medical complexity as traumatic brain injuries that occur as a result of car accidents and gunshot wounds; cardiac malformations and hereditary errors of metabolism. One such metabolic disorder is known as adrenoleukodystrophy, the condition featured 23 years ago in the movie “Lorenzo’s Oil.”

Lorenzo Odone wasn’t expected to live beyond childhood, but a medication his parents developed lengthened his life. Odone died in 2008, a day after his 30th birthday. Like 2,000 medically fragile young people now in New York, his care was home-based.

Multiple congenital conditions — those occurring after conception or immediately after birth — can underlie medical fragility, explained Simpser, a medical doctor. Cerebral palsy, the disability that afflicts Stephanie Cilento, is a congenital disorder.

Medical experts at the National Institute of Neurological Disorders and Stroke further define cerebral palsy as any one of numerous neurological conditions that appear in infancy or early childhood that permanently affect body movement and muscle coordination. These doctors note that inborn abnormalities in parts of the brain governing muscle movements underlie the condition.

But serious infections are another path to neurological damage that also can lead to medical fragility and the need for lifelong care. Encephalitis, for example, an inflammatory condition occurring when an infectious agent attacks the brain, is one such reason, Simpser said.

Many of these patients cannot stay at home with their families because the level of required care may be too complicated for parents and other family members. And because it’s technology-driven and requires access to trained nurses, the expense may prove too burdensome for some families, he said.

Moreover, as children enter adulthood, Simpser added, aging parents may not have the physical or mental wherewithal to effectively deliver the care.

Nearly forced out of state

Nancy Speller, a Plainview resident and registered nurse with St. Mary’s division in New Hyde Park, is on the committee searching for the Nassau site to develop a homelike setting for medically complex and fragile young people.

Speller knows their needs more intimately than most medical professionals because her son, J.J., suffered from a rare debilitating condition. He died a decade ago at age 18.

A ventilator shortage even then forced the Speller family to consider moving out of state.

“I had a mini-ICU in my home,” Speller said, noting that despite her training as a registered nurse, her son’s needs grew more critical over time. “I actually feared for his safety at home.”

That was when she and her family started searching for alternative care situations, a hunt that led her to geriatric nursing homes and ventilator-equipped facilities in New Jersey. She had planned to move to New Jersey when, fortuitously, her area school district decided to step in and pay for round-the-clock nursing care.

There have been no such strokes of luck yet for Stephanie Cilento, whose family is hoping to find appropriate long-term care soon.

“She needs continuous care. She doesn’t need end-of-life care,” Dimitra Cilento said. “This is what the country has to look at for young adults. These people need real professional, caring care — and you really can’t find it.”

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