A New York family struggles to give their son a ‘normal life’
By Joe Pompeo
The tracheostomy tube is one of the things that really makes Hunter different from the other kids. Inserted through a small hole in his neck, it creates an airway amid the tumors that clog his throat. He can’t breathe without it.
Then there is the gastric feeding tube that attaches to a hole in Hunter’s stomach, providing him with the nutrients he is unable to swallow. Though he’s recently been able to start drinking small bits of water, at age six, Hunter has never eaten a piece of food.
There’s also the hearing implant that snaps into his skull like a button just above his right ear; the 11 large boxes of medical supplies delivered to his home each month––syringes, tubes, feeding bags, nebulizer kits, you name it––and, not least of all, the large, inexplicable tumors that deform his body.
“What happened to me? Why is my face so messed up?” he’s recently begun to ask.
Hunter Cavanaugh suffers from a rare genetic disorder that causes severe benign tumor growth in the face, head and neck. Though his condition closely resembles the more common disease neurofibromatosis, he lacks some of the features typically associated with it and has tested negative for the defective gene scientists have identified as its cause. This leaves Hunter in a state of diagnostic limbo. He doesn’t qualify for the clinical trials and experimental treatments being developed for people with neurofibromatosis, and there are no medicines that can help shrink his tumors, known as neurofibromas, or stop them from growing. For Hunter’s quality of life, this means round-the-clock care and frequent surgeries, like the operation two doctors at St. Luke’s-Roosevelt Hospital Center will perform in a few days to remove the excess tissue between his eyes.
To make matters worse, no private insurance companies will cover all of Hunter’s medical expenses—from the surgeries, to the seemingly endless string of tests and doctors’ visits, to the extensive supplies that facilitate his daily care (these alone cost roughly $9,000 a month). As a result, his parents have had to put him on Medicaid, which means their combined annual income must remain near the poverty level.
But despite all of this, one word best describes this young Manhattan resident: Happy.
“We’ve never had the ‘poor Hunter’ attitude, or seen him as a victim,” explains Bianca Cavanaugh, Hunter’s mother.
She says she and her husband, Eric, go out of their way to give their son as normal a life as possible. “He really is happier than most kids I know.”
It seems true. He almost always has a smile on his face. He enjoys going to school each day. He has an array of hobbies and interests: toys, books, movies and, like most boys his age, superheroes, Hunter’s favorite obsession (Bianca says he draws pictures of them “all day long”). And, he’s booked solid on playdates.
In a park near his home on the Upper West Side, Hunter runs around with his two best friends and the roughly 20 other children who are playing outside on this sunny afternoon in April. He mimics Superman on the swing set, swinging back and forth on his stomach with his arms outstretched and an excited grin on his face. The jungle gym seems to pose no challenge as he successfully traverses the monkey bars, and he laughs energetically while gliding down the spiral slide again and again and again.
For Bianca, who keeps an eye on Hunter while chatting with some mothers from the neighborhood, it’s just another Saturday on the playground. But six and a half years ago, she might not have expected to be here with him.
“They told us he was blind, deaf and retarded, and that he probably wouldn’t live past three months,” she recalls of that November morning in 2000 when Hunter’s birth went terribly awry. “There was an agonizing hour when we didn’t know if he was alive or dead.”
Until that day, Bianca’s pregnancy had no complications, and there were no signs that Hunter was anything but a normally developing infant. But after 21 hours of unsuccessful labor, she could tell something was wrong. The doctors saw that Hunter’s face was severely deformed as soon as they pulled him out during an emergency C-section. His heart temporarily stopped beating, and he needed the trach tube put in right away to help him breathe. He ended up having to spend more than the first year of his life in the hospital under 24-hour care.
Miraculously, after six months it became clear that Hunter wasn’t blind or retarded. On the contrary, he was “so normal, so responsive.” However, it had become obvious that he was almost entirely deaf. For Eric, who is a musician, the news was devastating.
“How is he going to listen to his daddy’s rock ’n’ roll if he can’t hear?” Eric cried, putting his head in his hands.
Still, Hunter was an otherwise healthy baby, and after 14 months, Bianca and Eric were finally able to take him home.
Though he was eligible for live-in nursing, they decided to care for him on their own. Would there even be room for a nurse in their small, rent-stabilized one-bedroom apartment?
Hunter had his first major surgery at 19 months. In a 14-hour procedure called a “debulking,” doctors removed the mass of excess tissue he was born with. To their dismay, it grew back almost immediately.
In 2002, a U.S. Army pathologist identified the tissue as isolated plexiform neurofibromas; rare tumors that are almost always benign, but do in some cases degenerate into cancer. They can also lead to other potentially dangerous health problems, like infringing on vital organs or compressing the spinal cord. The tumors are the cause of Hunter’s deafness and the reason he cannot eat food, and they recently threatened his vision when they began pressing on the optic nerve behind his left eye.
The initial conclusion was that Hunter must have NF1, the more common of two types of neurofibromatosis. But aside from the presence of neurofibromas, he had none of the other symptoms associated with the disease––like small, chocolate-like spots on the skin or freckling in the armpit and groin areas––and a blood sample taken in 2003 did not link him with the NF1 gene. Hunter also did not have any symptoms of NF2, a subset of NF1 which occurs 10 times less frequently, and for which scientists are farther behind in terms of developing experimental treatments and combative drugs.
Last year, the Canavaughs learned of a new, highly specific genetic test for NF1. They hoped it would detect what the initial blood test could not, and give Hunter an official diagnosis so he could qualify for clinical NF trials. So a second test for the NF1 gene was performed, the results came back in January: negative.
Saving Hunter With Surgery
Without a diagnosis, the only option for improving Hunter’s quality of life is surgical management, says pharmaceutical scientist Dr. Bill Knowles of the NF Cure Project, a nonprofit organization that works with pharmaceutical companies in applying cancer drugs to neurofibromatosis research. Even if some of the experimental treatments being developed might help Hunter, it is highly unlikely he would be approved for clinical trials since it cannot be proven that he has NF. It is even less likely that scientists any time soon will identify the defective gene responsible for Hunter’s condition, according to Knowles.
“The NF1 gene was discovered in 1990, but scientists were looking for it for years before that, and they’ve been studying NF1 for the entire century. So the money spent and the man-hours put in have been very large,” he explains. “How do you do that for Hunter? It would be mind-boggling.”
Dr. Bruce Korf, chair of the genetics department at the University of Alabama at Birmingham, is an expert on neurofibromatosis and a leading clinical researcher in the field. Over the 25 years he’s been treating NF patients, he’s seen “a couple dozen” who, like Hunter, had large, isolated plexiform neurofibromas, but otherwise did not fulfill the diagnostic criteria for NF1. He says if any of the drugs currently being tested on NF1 patients show promising results, he hopes that these “trials can be broadened reasonably quickly to accommodate others who have NF-like problems, yet don’t have definite NF1.” But he confirms that in the meantime, “there is currently no established mode of effective therapy other than surgery, and surgery can be very difficult and may not offer complete relief from symptoms.”
Nevertheless, the two surgeons currently working with Hunter are optimistic. During a phone interview, one of them
outlines the goals of the three to four surgeries Hunter will have undergone by year’s end. The first of these, preserving Hunter’s vision by removing a tumor pushing on the optic nerve in his left eye, was achieved with the first of the operations in March. They also plan to alter Hunter’s nasal and oral cavities in such a way that his trach tube will no longer be necessary, position his eyes to become symmetrical and remove the substantial amount of sock tissue that so amorphously shapes his lower face.
“He’ll never be the way God should have made him, but we hope we can provide him with a life where he can interact with people in a more reasonable way,” says the surgeon, Dr. Peter Costantino, an internationally recognized expert in craniofacial tumor surgery who will operate on Hunter June 11 with Dr. Milton Waner, a “pioneer” in the treatment of vascular birthmarks and other head and neck malformations.
During this next operation, excess tissue from between Hunter’s eyes will be turned down into the bridge and tip of his nose, giving it bulk and shape (currently, his nose barely projects from his face). To complete the roughly four-hour surgery, an incision will be made literally down the center of Hunter’s face, as well as in his lips and gum line. During the first surgery, an incision was made from ear-to-ear across the top of his head, and the tissue of his entire forehead was peeled down exposing his temples and eye sockets.
How must Hunter feel in the hours leading up to such invasive procedures? “Fearless,” says Bianca. “Pictures from the day of the last surgery show us clowning around, putting on funny hospital outfits and pretending they were astronaut suits, just goofing off. We try to present the surgery to him as an exciting thing.”
Kids Can Be Cruel
Perhaps for Hunter, there are scarier things than surgery, like the kids who point, laugh and tease as he passes them by, or the ones who cry because his appearance frightens them; the people who stare when he’s out in public, or on the other hand, the people who can’t bring themselves to look at him. Which is worse?
Hunter had a hard time adjusting when he entered New York City’s American Sign Language and English Lower School, says Rose Werner, who was his preschool teacher for two years. When he came into the classroom, the other children asked questions like “Why is Hunter’s face fat?” or “What happened to his head?” But it wasn’t long before Hunter’s classmates grew quite fond of him, and he in turn “became comfortable with the school environment and was able to enter the classroom eagerly each morning…He was, and is well loved by his classmates and teachers alike.”
However, when Hunter began first grade this past fall, he started becoming furious when kids from other classes would stare at him in the hallways. He would confront them out of anger and sign the phrase, “I am ignoring you,” telling his parents that kids he didn’t know were “always looking at my face. Not nice!”
Dr. Laurence Pick is a Manhattan-based clinical neuropsychologist whose patients include children and other individuals with craniofacial malformations. He says these children run a greater risk of developing emotional issues based on their appearance, issues that sometimes manifest themselves in the form of anger, sadness, depression, irritability or oppositional behavior. And as these children enter adolescence and adulthood, they will more frequently experience life events that could exacerbate the already stressful experience of looking different.
“Just dealing with the social issues all teenagers deal with can lead to greater stress,” he says.
But Pick also points out that for many disfigured children, looking different never interferes with the ability to function and socialize. And with the support of their families, schools and communities, they often are able to deal with the psychological impact of disfigurement without needing therapy.
With the help of both therapy and support from his friends, teachers and family, Hunter was able to overcome his anger issues, and after his surgery in March, he couldn’t have been more excited to get back to school. His classmates couldn’t wait to see him either, Bianca says, and upon his return they asked about how he was feeling and what the surgery was like.
But more obstacles may lie ahead. Will Hunter’s friends stick by him as they get older and it’s not “cool” to be seen with the kid who has the weird-looking face? Will he find a girlfriend? Get married? Have children of his own? Will he ever face discrimination when applying for a job or be told he can’t do something because of the way he looks? Will people ever stop staring?
The Cavanaughs do sometimes wonder what the future holds for Hunter. But for the most part, they say, they try to stay “in the here and now,” smiling as their son hangs from the monkey bars on the playground, draws endless pictures of superheroes that adorn his bedroom walls, battles an imaginary swarm of villains with his plastic toy sword, plays with his new remote-controlled truck, which he says is “cool, but too big,” finishes his homework so he can watch some of Batman Returns 10 minutes before bedtime, and, above all, continues to be what he always has been: a very happy, very well-loved and very lucky little boy.
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