Bright brown eyes and a big, engaging smile belie the fact that Cameron Brown, 13, struggles daily with the pain and suffering of Juvenile Huntington’s Disease.
“Cameron is an amazing child,” says his mother, Tina LaVornia-Ross. “He is funny, sweet, and loving. He has always been easy to please, and he appreciates everything. He has an amazing soul, and he has unknowingly taught people so much in the short time he has been on this earth.”
On June 13, hundreds of people will get to see that big, happy smile as they meet Cam (as his mom calls him), the official honoree for the Juvenile Huntington’s Disease Walk in Sacramento, the first JHD event ever held in the United States. Every penny of the net proceeds will go to the Institute for Regenerative Cures (IRC) at UC Davis, California, to support Dr. Jan Nolta’s JHD Research Initiative.
Tina said Cameron doesn’t and has never realized truly what is going on and doesn’t grasp that there is anything wrong with him. When he has asked why he has to go to doctors and get tests, she has tried to explain, but he just doesn’t understand. “It has been a blessing in this,” said Tina.
“Cami loves cartoons,” Tina said. “‘SpongeBob’ is his favorite. He has yet to meet a cookie he hasn’t liked. When he was still able to walk, he loved to run and race his brothers. He was always happy. Even now, no matter what he is going through, if you ask him how he is, he will say, ‘Good.’ He has a beautiful soul.”
Cameron’s father, William, was diagnosed with Huntington’s disease in 2000, at the age of 28, when his chorea became too difficult to control, and he was no longer able to work. Tina’s husband knew that his dad had it, but didn’t know a lot about it and wasn’t even sure of his chances of getting it. Tina researched HD at the library, and after seeing eight doctors, one finally gave them a referral to a neurologist for William to be evaluated and possibly tested. The youngest of four children, William began showing symptoms in 1994-95, and went to live with his parents in 2001, due to issues with anger, after Cam and his twin sister were born. He went to a nursing home in 2006, and lived there until he passed away in January of this year. Cameron’s grandfather, who also had HD, passed away in 2005, and one aunt passed away from HD in 2013. After William was diagnosed, and Tina was pregnant with Cameron and his twin sister, the doctor told them not to worry, that there would be a cure by the time the kids were old enough to be affected by the disease.
Tina first noticed something was wrong with Cameron in 2006, the summer before he turned 5. She said the symptoms were slight: he fell a few times over nothing, was dropping items more than normal, and would struggle with the doorknob. Knowing the family history, she took him to her family doctor, a doctor she trusted and whose input she wanted, in August of 2006. When she first suggested that Cam might have Huntington’s, the doctor was dismissive, but Tina persisted, explaining to the doctor the family history and that Cam’s symptoms, however slight, were there. After CT scans and blood work, the doctor decided to test him for Huntington’s. When the test came back, there was only one CAG (cytosine-adenine-guanine) number (which was in the normal range) and a notation from the lab that possibly his second number was too high, which is why it did not appear on the results. (Normally, in the genetic test for HD, the CAG number for both alleles, one inherited from the mother and the other from the father, is given.) The doctor dismissed this and said that Cameron was negative for Huntington’s disease, adding that Tina should not pursue this diagnosis because there was nothing anyone could do to help Cameron if he had JHD. He suggested that she wait and see.
Unable to do that, Tina sought out another pediatrician who said that he honestly knew nothing about HD and that he didn’t see what she was talking about, but, given the family history, he would refer Cameron to a neurologist. Cam saw the neurologist in December of 2006, in Las Vegas. The neurologist walked into the room and silently watched Cameron play with his toys on the floor. After examining him, she said that she saw the chorea Tina had spoken of and that she would like to do further testing. She explained that with children, a different test had to be done to measure the longer CAG repeats. (Most adults with HD have CAG repeats of 40-50, while children with JHD usually, though not always, have CAG repeats over 50. A different, special test must be done to detect the very high numbers.) On Feb. 13, 2007, Cameron was officially diagnosed with Juvenile Huntington’s Disease with a CAG of 95.
Tina said one of the biggest challenges she faces as his mom is making sure she has the best people caring for him; she still struggles with “doctors who give up too easily.” Although Cam was diagnosed in Las Vegas, the lack of pediatric care was hard, so she made the decision to move to Rhode Island. Tina had family there, and Boston was close by, where Cam was cared for by Dr. H. Diana Rosas at Mass General from 2007-2013. The cold weather worsened Cameron’s dystonia and made him very uncomfortable. It was also difficult to make the hour-long trip with Cam to Mass General, so Tina moved to California, where she has family. She says Dr. Rosas was an amazing doctor and one of the most genuine people she’s ever met. Currently, Tina is struggling to find a pediatric neurologist who understands JHD. She and her partner, Carrie Ross, work opposite shifts so that one of them is always there to care for Cameron.
Another challenge is being a mom to her other children and not letting them suffer because her attention is always on Cameron. She says it’s a balance she has yet to master, but one she works on daily. Cameron has six siblings. He has a brother from his dad’s previous relationship, a 19-year-old sister, an 18-year-old brother, a 16-year-old sister, his twin sister, and a little brother who is 12. His 16-year-old sister is not symptomatic, but plans to be tested when she turns 18. Tina will also have Cameron’s twseiUewain, who was born with developmental delays but is not symptomatic, tested when she turns 18. The others are not at risk.
She says that the biggest challenge at first was learning not to grieve over Cameron right now. Tina remembers being told by another JHD parent who had just lost his son to enjoy the time with her son now because there will be plenty of time for grieving later. She said that once she internalized that advice, the journey became easier; she’s been able to make the most of the time Cameron has here and to push him to do the most and the best that he can do.
Tina made the difficult decision to pull Cameron out of school in September of 2014. She said he wasn’t happy there and was no longer benefitting from going to school. Even so, it was a decision she and her partner struggled with for a long time.
“Cameron’s biggest challenge right now,” said Tina, “is his ability to eat and communicate. He loves food, especially sweets, and he has had trouble with opening his mouth when he wants to, so some days, all his nutrition comes from his g-tube. Communication has always been a challenge, and some days, it’s hard to understand anything. Without proper use of his hands, due to dystonia, he is not able to use an iPad or any other device to communicate his needs. It’s a guessing game most days, and that is frustrating on both ends.” The medication Cameron takes for seizures has the undesirable side effect of making it difficult for him to open his mouth.
Besides dystonia and difficulty communicating, Cameron’s symptoms over the past nine years have included falling, fine motor deficits, seizures, and swallowing issues. He has lost the ability to walk, talk, and hold himself up. There have been several things that Tina and her family have found to make Cameron more comfortable and happy over the years, and she says that tips from other moms have helped so much on their journey. An egg crate mattress cover keeps Cameron more comfortable, and weighted blankets (used for children with autism) help to soothe him. She’s learned that too much excitement, being upset, or just being off schedule will often trigger seizures. Much of what she’s learned has been from careful observation of Cameron and conversations with other JHD moms on Facebook. She added, “The JHD group on Facebook is my lifeline, and I am thankful for all the moms every day.”
Because Cam spends about 50 percent of the day in bed, Tina has moved his bed into the living room so that he can be a part of everything that goes on in the family. They keep two chairs by his bed so that family members can sit close to him and interact with him. While Cam seems unable to fix his eyes on anything, Tina said she can tell from his laughter at funny things that are said that he understands what is going on around him, and she can tell that his mood has improved.
Huntington’s disease has impacted Tina’s life and the life of her family greatly. She notes that her marriage was destroyed and that her son will never live to do the things others take for granted.
“I am living a life that many parents fear,” Tina said. “People dream of healthy, happy kids.”
Many of her family relationships are strained; some don’t know what to do or say, so they do or say nothing. Others judge her decisions or the way she takes care of Cameron. She believes that grief and fear come in many forms, so she has learned to accept that people handle things in different ways.
The most important piece of advice Tina would give to someone who suspects JHD is, “Don’t give up. Moms have an amazing intuition, and we know when there is something that isn’t right. Fight for your child so that if it turns out they do have JHD, the services and care can begin early, and your child will have what they need.”
Tina is proud that Cameron has been chosen as the Poster Child because the JHD Research Initiative from Jan Nolta’s lab means hope. She is excited about the initiative and wants Cameron to be part of it. She’s also excited at the prospect of meeting other moms that she’s talked to on Facebook for years but has never met.
“Even though Cameron will most likely not benefit from this research,” Tina said, “the fact that this may give other children hope to not have to suffer with this disease is amazing. The scariest thing about this disease is the lack of treatments. There is no hope right now for these kids, just short lifetimes of pain and suffering. Hope that research will eventually bring a cure for these kids means a lot to me.”
Update: Sadly, Cameron Brown lost his battle with JHD in November 2015, as he traveled across country from California back to the East Coast to be closer to family. Heaven has gained another JHD Warrior Angel. Rest in peace, Cami, until we meet again. Cami’s memorial page may be seen here.