Posted 3:24 pm Tuesday, February 07, 2012
Child Battles Very Rare, Incurable, Fatal Disease
Editor's note: This is the first in a series of occasional stories on Casen Cameron, who suffers from Batten Disease.
By MELISSA CROWE
Staff Writer
By MELISSA CROWE
Staff Writer
HOLLY LAKE RANCH — In the video, baby Casen Cameron is sitting on his grandmother's kitchen floor, pulling a cabinet door open and squealing and grunting with delight as it slams shut.
Casen appears happy, alert and healthy, but it didn't stay that way with the onslaught of a rare neurological disease that has robbed him of his health and his childhood. Ultimately, it will take his life.
Casen has Batten disease, an illness shared by only 1,200 children worldwide, with 20 of those in Texas.
Casen has Batten disease, an illness shared by only 1,200 children worldwide, with 20 of those in Texas.
For 19 months after his first sign of the disease, doctors treated it as epilepsy, a common misdiagnosis. Casen, now 5, lost his speech, his ability to walk, his eyesight and his ability to swallow.
There is no treatment. There is no cure.
There is no treatment. There is no cure.
Casen's father, Corey Cameron, a Smith County sheriff's deputy, big brother Colby and grandmother Pamela Cameron are spearheading a campaign to increase awareness of the disease and the need for research and a cure. They call it “Casen's Crusade.”
“They may not save Casen, but they can save another child,” his grandmother said.
Casen's first seizure was in November 2009, just before his third birthday.
His eyes rolled back. His body fell completely limp.
“You almost think that he's died in your arms,” his grandmother said. “The first time he did that, I was holding him. I thought he died.”
Corey is not sure of an exact number of seizures. He only knows there has been “a bunch,” probably more than 10 a year.
When Casen comes out of it, he is disoriented and cries for hours.
“We have to rock him and assure him that we're there,” his grandmother said. “It's hard. It's hard to watch a baby go through that.”
Casen had a few more seizures through the holiday season, and a series of them on Christmas day resulted in a wide-range quest for answers.
“We had just opened presents,” Casen's father said, and then it happened.
Casen had another seizure on the way to a Tyler emergency room from their home at Holly Lake Ranch. They stopped at the Hawkins Fire Department and made the rest of the trip by ambulance.
Corey remembers exactly what he was thinking as well as the anger and confusion.
“Why? What's wrong? How can I help?” he said. “I felt helpless.”
Casen had a third seizure in the emergency room before doctors referred him to a children's neurologist in Dallas.
“They did everything they could,” Corey said. “They thought maybe he'd outgrow it.”
After brain scans, X-rays, heart scans and blood tests, doctors continued diagnosing him with epilepsy.
The Dallas specialist recommended Casen attend a special education program to help him develop.
He did not develop. In fact, he regressed, his grandmother said.
Casen was never a big talker. His vocabulary included typical baby words: Nana, Poppa, Mama, Dadda, truck, drink and eat.
“He wouldn't say his ABCs,” Casen's father said. “He'd get to the first six or seven letters and stop.”
Then one day, nothing.
His grandmother remembers the grunts and squeals Casen made as a toddler.
“He'd make these sounds that would just make you laugh all day,” she said. “He hasn't said ‘Nana' in a long time.”
The family watched as Casen began falling for seemingly no reason. They thought he was having leg trouble, but after multiple doctor's visits, everything seemed fine.
“They couldn't find anything,” Casen's grandmother said. “Now we're thinking it was the Batten disease.”
The family was desperate for help and answers.
They took Casen to more doctors, more specialists, more hospital stays, more tests. They even tried a special high-fat diet that has been known to reduce seizures in epileptics.
“It can't hurt to try,” his grandmother said.
Meanwhile, they were referred to another doctor in Dallas.
“While we're waiting for that appointment, Casen quit walking,” Corey said.
Doctors thought it was a side effect of the stiff epilepsy medication.
“It's not the medication,” Corey told them. “You know your own children. It's something else.”
In June 2011, 19 months after his first seizure, doctors performed a muscle biopsy on Casen.
The results were devastating.
“I've cried more in the last year than I have in my whole life,” his father said.
Batten Disease is a neurodegenerative disease found in children. It is rarely diagnosed immediately and is often mistaken for epilepsy, mental retardation, bipolar disorder, schizophrenia or an eye condition called retinitis pigmentosa.
“We were scared to death knowing what was coming,” Pamela said. “The doctor told us exactly how bad it was going to be.”
Batten disease's onset can include progressive vision loss, seizures, clumsiness, memory loss and personality and behavior changes. It causes continuing physical and mental deterioration and leads to early death.
The younger the person is when the disease appears, the greater the risk for disability and early death, according to an article in the U.S. National Library of Medicine. Casen is not expected to live past 12 years old.
At this time, there is no treatment or cure.
Since being diagnosed with Batten disease, Casen quit walking and talking. He is losing his eye sight. He had a feeding tube put in.
“A lot of people tell us there is still hope; there's not,” his grandmother said.
According to the national medical library, the disorder involves the buildup of an abnormal material called lipofuscin in the brain. Evidence suggests that it is caused by problems with the brain's ability to remove and recycle proteins.
Casen's grandmother explained it in laymen's terms.
“The junk just keeps collecting until it takes over the brain,” she said, resulting in loss of speech, eyesight, motor skills, “and then pretty soon, you go.”
Those who develop the disease early can have vision problems that progress to blindness, and problems with mental function get worse. If the disease emerges in the first year of life, death by age 10 is likely.
If the disease occurs in adulthood, symptoms will be milder with no vision loss and a normal life expectancy. The family opted out of gene therapy, which could slow the progression.
“I don't want to subject him to any more pain,” his father said.
Through Casen's journey with the disease, the family has found consolation and support from another “Batten family” in Longview.
Paul and Leann Spencer lost their son, Joshua, in June 2007 to Batten Disease.
He was 7.
Their daughter's drill team instructor, who lives in Holly Lake Ranch, put the two families in touch with each other.
Mrs. Spencer said she believes they were destined to meet. “There was little divine intervention happening,” she said. “We're supposed to help them, to be a shoulder. We can't offer much, but we're trying.”
The Spencer family gave Casen Josh's custom-made wheelchair. It was a perfect fit.
“Casen looks just like our son did. … They both have those great blue eyes, we all started bawling,” Mrs. Spencer said. “It was really hard because he was so much like our son.”
Josh was the second child in Texas to be diagnosed with Batten disease.
The family opted to include him in a gene-therapy clinical study through New York-Presbyterian Hospital/Weill Cornell Medical Center in New York City.
Since he passed away, Mrs. Spencer said she is noticing more children being diagnosed with the disease. Before, she said she believes children would die before the parents or doctors reached the diagnosis.
“We survived it. We didn't feel like we ever would, but we did,” Mrs. Spencer said. “There is life after this, although you don't feel like there will be. It just takes time. If nothing else, they can see that.”
Meanwhile the Cameron family is taking life one day at a time. “We're going to do whatever we can to raise awareness,” Casen's father said. “What else can you do?”
Casen's first seizure was in November 2009, just before his third birthday.
His eyes rolled back. His body fell completely limp.
“You almost think that he's died in your arms,” his grandmother said. “The first time he did that, I was holding him. I thought he died.”
Corey is not sure of an exact number of seizures. He only knows there has been “a bunch,” probably more than 10 a year.
When Casen comes out of it, he is disoriented and cries for hours.
“We have to rock him and assure him that we're there,” his grandmother said. “It's hard. It's hard to watch a baby go through that.”
Casen had a few more seizures through the holiday season, and a series of them on Christmas day resulted in a wide-range quest for answers.
“We had just opened presents,” Casen's father said, and then it happened.
Casen had another seizure on the way to a Tyler emergency room from their home at Holly Lake Ranch. They stopped at the Hawkins Fire Department and made the rest of the trip by ambulance.
Corey remembers exactly what he was thinking as well as the anger and confusion.
“Why? What's wrong? How can I help?” he said. “I felt helpless.”
Casen had a third seizure in the emergency room before doctors referred him to a children's neurologist in Dallas.
“They did everything they could,” Corey said. “They thought maybe he'd outgrow it.”
After brain scans, X-rays, heart scans and blood tests, doctors continued diagnosing him with epilepsy.
The Dallas specialist recommended Casen attend a special education program to help him develop.
He did not develop. In fact, he regressed, his grandmother said.
Casen was never a big talker. His vocabulary included typical baby words: Nana, Poppa, Mama, Dadda, truck, drink and eat.
“He wouldn't say his ABCs,” Casen's father said. “He'd get to the first six or seven letters and stop.”
Then one day, nothing.
His grandmother remembers the grunts and squeals Casen made as a toddler.
“He'd make these sounds that would just make you laugh all day,” she said. “He hasn't said ‘Nana' in a long time.”
The family watched as Casen began falling for seemingly no reason. They thought he was having leg trouble, but after multiple doctor's visits, everything seemed fine.
“They couldn't find anything,” Casen's grandmother said. “Now we're thinking it was the Batten disease.”
The family was desperate for help and answers.
They took Casen to more doctors, more specialists, more hospital stays, more tests. They even tried a special high-fat diet that has been known to reduce seizures in epileptics.
“It can't hurt to try,” his grandmother said.
Meanwhile, they were referred to another doctor in Dallas.
“While we're waiting for that appointment, Casen quit walking,” Corey said.
Doctors thought it was a side effect of the stiff epilepsy medication.
“It's not the medication,” Corey told them. “You know your own children. It's something else.”
In June 2011, 19 months after his first seizure, doctors performed a muscle biopsy on Casen.
The results were devastating.
“I've cried more in the last year than I have in my whole life,” his father said.
Batten Disease is a neurodegenerative disease found in children. It is rarely diagnosed immediately and is often mistaken for epilepsy, mental retardation, bipolar disorder, schizophrenia or an eye condition called retinitis pigmentosa.
“We were scared to death knowing what was coming,” Pamela said. “The doctor told us exactly how bad it was going to be.”
Batten disease's onset can include progressive vision loss, seizures, clumsiness, memory loss and personality and behavior changes. It causes continuing physical and mental deterioration and leads to early death.
The younger the person is when the disease appears, the greater the risk for disability and early death, according to an article in the U.S. National Library of Medicine. Casen is not expected to live past 12 years old.
At this time, there is no treatment or cure.
Since being diagnosed with Batten disease, Casen quit walking and talking. He is losing his eye sight. He had a feeding tube put in.
“A lot of people tell us there is still hope; there's not,” his grandmother said.
According to the national medical library, the disorder involves the buildup of an abnormal material called lipofuscin in the brain. Evidence suggests that it is caused by problems with the brain's ability to remove and recycle proteins.
Casen's grandmother explained it in laymen's terms.
“The junk just keeps collecting until it takes over the brain,” she said, resulting in loss of speech, eyesight, motor skills, “and then pretty soon, you go.”
Those who develop the disease early can have vision problems that progress to blindness, and problems with mental function get worse. If the disease emerges in the first year of life, death by age 10 is likely.
If the disease occurs in adulthood, symptoms will be milder with no vision loss and a normal life expectancy. The family opted out of gene therapy, which could slow the progression.
“I don't want to subject him to any more pain,” his father said.
Through Casen's journey with the disease, the family has found consolation and support from another “Batten family” in Longview.
Paul and Leann Spencer lost their son, Joshua, in June 2007 to Batten Disease.
He was 7.
Their daughter's drill team instructor, who lives in Holly Lake Ranch, put the two families in touch with each other.
Mrs. Spencer said she believes they were destined to meet. “There was little divine intervention happening,” she said. “We're supposed to help them, to be a shoulder. We can't offer much, but we're trying.”
The Spencer family gave Casen Josh's custom-made wheelchair. It was a perfect fit.
“Casen looks just like our son did. … They both have those great blue eyes, we all started bawling,” Mrs. Spencer said. “It was really hard because he was so much like our son.”
Josh was the second child in Texas to be diagnosed with Batten disease.
The family opted to include him in a gene-therapy clinical study through New York-Presbyterian Hospital/Weill Cornell Medical Center in New York City.
Since he passed away, Mrs. Spencer said she is noticing more children being diagnosed with the disease. Before, she said she believes children would die before the parents or doctors reached the diagnosis.
“We survived it. We didn't feel like we ever would, but we did,” Mrs. Spencer said. “There is life after this, although you don't feel like there will be. It just takes time. If nothing else, they can see that.”
Meanwhile the Cameron family is taking life one day at a time. “We're going to do whatever we can to raise awareness,” Casen's father said. “What else can you do?”
