Neurofibromatosis is a genetic disorder that causes tumors to grow on nerve tissue throughout the body. NF can lead not only to disabling pain, but also to disfigurement, cancer and even death.
May has been designated National Neurofibromatosis Month and May 17 is World Neurofibromatosis Awareness Day to promote awareness of this tragic disease and to call attention to how it affects children and their families.
To that end, I’d like to share the inspirational story of Alexander Owens, a three-year old boy afflicted with NF1 — a specific form of NF characterized by tumors under the skin.
Alexander, who is one of the Child Tumor Foundation’s “CTF Heroes,” and his family have captured the hearts and attention of many people who previously may have been unaware of NF and its consequences. To get a better understanding of the struggles of Alexander and his family, I spoke with Alexander’s mother, Diane, as well as with Angela Dumadag, who works with the Owens family at CTF, and John Joseph, lead singer of the hardcore punk band the Cro-Mags, who is raising money for Alexander through running the Ironman World Championship Race in Kona, Hawaii, on Oct. 8, 2016, as well as organizing a series of fundraising shows, including the upcoming May 29 hardcore punk show at Highline Ballroom in New York City, featuring the Cro-Mags.
And in learning about Alexander and his family, we may all begin to better understand the hardship that NF causes and become motivated to join the cause to help raise awareness and find effective treatments for the disease.
Diane Owens described how she and her husband first discovered that Alexander had NF1 after noticing “café au lait” spots on his body. Owens told me, “When Alex was 4 months old, he was diagnosed with NF1. We were very fortunate to have a pediatrician who is familiar with NF. Many are not.”
Owens sought out several specialists to confirm that Alexander had NF and eventually discovered that he had inoperable tumors. “She sent us to the ophthalmologist to look for optic gliomas and a geneticist who gave the formal diagnosis based on symptoms,” she said. “By the time Alex was two and a half years old, he was diagnosed with two inoperable tumors: A plexiform neurofibroma, which occupies about a third of his back, and an optic glioma that sits on the hypothalamus on his brain.”
According to Owens, Alexander is lucky because at this point he does not suffer from significant physical setbacks that can plague children with NF1, including severe pain, physical deformity or learning disabilities. “Day to day, Alex doesn’t face too many physical struggles. He’s able to play and enjoy sports and games as much as the average kid, more or less. Although he can suffer from pediatric migraines and sometimes has back pain,” Owens said.
However, physical issues are not the only risk facing children with NF1. They can face a series of psychological issues, including depression and anxiety, as well as more general emotional and social sensitivity. For example, one meta-analysis of 57 studies suggests that parents tend to find their children with NF as having more emotional and behavioral problems, and rated by teachers and peers as being more socially sensitive and isolated.
Luckily, Alexander is able to preserve a positive mood and is doing well socially. “He is a happy, compassionate and endearing child,” Owens said. “He has no problem sharing and finds a way to get along with everyone. Even his preschool teachers are impressed and enchanted with his quiet and loving ways.”
However, Owens says that Alexander displays the sensitivity often found in children with NF, particularly with noise. “He’s extremely sensitive and easily intimidated. Any kind of loud noise, even if it’s expected or familiar, can send him into a panic,” she explained. “Simple things like hand dryers and hair dryers, crowded restaurants, or even a well-meaning visitor who greets him with an overly aggressive ‘hello’ can be overwhelming for him. His feelings are easily hurt, and his reaction to a simple reprimand or correction can be complete devastation.”
And the stress goes beyond the child struggling with NF. It can affect the whole family. For example, in one study that examined family stress, mothers of 37 children with NF1 were compared with a control group of mothers of “typically developing” children on parenting interactions and stress. While the study found no differences in how mothers of NF children reported interacting with their children, mothers had higher levels of parenting stress and overall stress as compared with mothers of “typically developing” children.
Owens explained the multiple ways that having an NF child can cause stress. First and perhaps foremost, people with NF1 have higher mortality rates. Evidence suggests that people with NF1 on average live 15 years less than those without NF1.
“Long-term is unknown. That’s the curse of the NF mom. No one can predict a prognosis. Most parents worry about getting their kids to become responsible adults, ones who will grow to have a career, maybe a family. When you have a child with NF, you worry about if they will make it to adulthood. There’s nothing you can do for your child but watch and wait,” Owens said. “Will it become severe? Or do I need to prepare myself to bury my 7-year-old?”
Even if Alexander lives as long as he would have without NF, Owens worries about the functional and financial consequences of his struggling with a chronic physical condition. “If he does, how will he support himself? How will he pay the copious amounts of medical bills when he’s no longer covered under our insurance? Will he have a family? Will he lose the love of his life because she can’t bear the idea of her own children having to suffer from this disease? Who will take care of him when we die?” she asked.
Moreover, while Owens explained how Alexander is currently functioning well in school, she worries that this may change over time and that he might be subject to teasing, bullying and social isolation.
People who report teasing as children often also report psychological distress in adulthood. And prospective longitudinal studies show that being bullied predicts negative physical and mental effects lasting potentially for decades. Further, the experience of social rejection and isolation that can result from being bullied has the potential for direct harmful mental and physical health outcomes and even early mortality — “the lethality of loneliness.”
“Other kids can be cruel. What kind of teasing or harassment are we going to have to face when other kids see the tumor on his back or the 25 café au lait spots all over his body?” Owens asked. “How can we empower him and how will we spare him from feeling belittled or, worse yet, being ostracized from his peers?”
Owens also worries about how the extra attention that Alexander receives because of NF will eventually wear on his brother, Justy. “With Alex seeing 11 different specialists for his NF, and the numerous activities we attend or run to raise NF awareness, his condition requires a lot of attention and trips. We worry that Justy will start to feel slighted after a while,” Owens explained.
“Right now, Justy is very supportive and driven to find a cure for NF. He made a video — the story came completely from him on his own without provocation — explaining how he’s going to shrink down to “microscopic size” so he can go into Alex’s body and beat up the tumors so they leave Alex’s body and Alex can feel good again. He also runs in our town’s annual Turkey Trot to raise money for [the Children’s Tumor Foundation]. After hearing about Ironman, he now wants to do a triathlon in Alex’s name.”
“Nonetheless, we worry that the momentum will fade, and he will come to resent his brother’s condition,” Owens said.
For Owens and her family, coping is not easy. But they have simplified their approach.
“Prayer and humor,” she said.
Alexander’s family has also been able to find support. This includes support from the Children’s Tumor Foundation, which boasts a four-star rating on Charity Navigator for its work raising money for research, education and support for families of children with NF and other tumor-related conditions.
“Our relationship with CTF has been beyond measure from Day One,” Owens said. “When we first learned of Alex’s diagnosis, we knew nothing about the disease, what it meant, what to expect, what to do or where to go. CTF’s website grounded us and provided real, correct and knowledgeable information.”
“They provided hope,” said Owens.
But for Owens and her family, the help went beyond hope, to more practical one-on-one help. “When Alex was diagnosed with his first tumor, I had no idea what to do. My first call was to one of the NF Endurance managers. We texted for 2 hours,” she explained. “By 6 a.m. the next morning, I had recommendations from other NF moms who live or lived on the East Coast and have children older than Alex with the same type of tumor. CTF took us from a place of isolation, confusion and fear and brought us to a community of knowledge, empowerment and understanding.”
Angela Dumadag of CTF described how Owens took action by joining the NF Endurance Team, a fundraising program for CTF that helps raise money for the foundation by sponsoring races.
“Many parents are told that NF is a “watch & wait” situation. Diane, however, felt that doing nothing is not an option,” Dumadag explained. “So she came off the couch and decided to begin running marathons through the NF Endurance Team. In 3-1/2 years, she and her husband have already raised over $75,000 to help us fund research for effective treatments for NF.”
With their positive and proactive approach to dealing with NF, Alexander and his family have become an inspiration to many. John Joseph of the Cro-Mags described the affect that Alexander has had on him.
“The Children’s Tumor Foundation offered me a slot for Kona in 2016. So they said when you race this Kona Iron Man, you’ll be assigned a CTF Hero. So they sent me the bios of four or five children, and this kid just stood out,” Joseph explained. “If you look at his pictures, he’s always smiling. He’s always happy. He’s always positive. And when I think of what I’ve been through in my life, the only thing that got me through was PMA — positive mental attitude. And when you see the kid in his photos…”
For Joseph, Alexander’s attitude is infectious. “If we get a little freaking pinched nerve on our spine, we’re in tears. Imagine having tumors on your spine,” Joseph said. “He’s inspiring me. That’s what it’s all about. When I’m out there training and racing, I’m doing it for him. He’s inspiring me. It’s not just what we’re giving. He’s giving back. It goes both ways.”
And so Joseph has dedicated himself to raising money for Alexander. “I want to bring awareness. If 1,000 people donate $5, that’s $5,000. It doesn’t hurt anyone financially. Whatever the hell you can afford. It’s a great cause. It’s helping this kid’s family,” Joseph said.
Owens also finds herself inspired by her son. “To meet Alex is to be inspired by him. He’s only 3 years old. He knows he has NF, but it does not define him,” she explained. “It’s just one of many descriptions that make him up. He doesn’t let his disease or its symptoms stop him from achieving what he sets his mind to. He is an example of how we all should live — with warmth, compassion and acceptance.”
So for those who are also inspired by Alexander’s story, consider getting involved, because there is much work to be done with individual children and families, and on a broader policy level to raise awareness to find effective treatments and get people with NF the care that they need.
“We must provide information about NF within schools, to educators and school administrators, so that they are equipped to accommodate the needs of students with NF. We need to provide info, training and education to the general medical practitioners, so that they can better diagnose and refer patients to NF Clinics,” Dumadag explained. “And we must educate the general public; namely, awareness of neurofibromatosis, so that NF patients will find sympathy and acceptance in their communities. Finally, we need to inform and educate locally and federally elected legislators in order for us to maximize our advocacy/lobbying efforts to secure congressional research funding.”
Getting involved with Alexander and the Owens family might be a good first step. “I love awareness. Ask me about NF. Ask my kids about NF. We’re happy to talk about and share what we know,” she said. “The more people who educate themselves and others about this disease, the easier Alex’s life, and the life of 2 million others affected with NF, becomes.”
“And the more likely we will find more effective treatments and cures,” Owens said.
Photo source: Diane Owens
An earlier version of this article originally appeared in Huffington Post on May 20, 2016.
