http://danvillesanramon.com/print/story/print/2009/09/18/chelseas-hope


Danville Express

Cover Story - September 18, 2009

Chelsea's Hope

A lifeline for families struck by a rare adolescent disease

by Susan Astbury

Chelsea Gerber was a vivacious adolescent who loved soccer, dance, baseball, band and hanging out with her friends.

"She just wanted to do it all," recalls her mother, Linda Gerber.

When Chelsea, now 18, was in middle school, Linda noticed that she was having small cognitive issues but thought that she just needed a little help.

"Then at the end of eighth grade, when Chelsea was 14-1/2 she had a grand mal seizure in front of school," said Gerber. "The first four months after Chelsea had her first seizure were relatively OK. She started high school at Monte Vista in September - and then a month later, she didn't go back."

Her health deteriorated rapidly, Linda explained. "The seizures became out of control and she lost her cognitive ability very, very quickly."

Linda and Howard, Chelsea's dad, took her to Children's Hospital Oakland to see a pediatric neurologist. After several tests, the doctor suggested checking for Lafora. The test for this rare disease had only been around for five or six years.

"Our doctor said this is the name of the disease but don't look it up," Linda recalled. "On the Internet, there were about three sentences and it was just all devastating."

The test came back positive for Lafora, a rare, progressive and degenerative form of epilepsy that strikes children in early adolescence. Inherited through recessive genes, Lafora causes seizures, muscle spasms, difficulty in walking, dementia and is eventually fatal. Most therapy is primarily aimed at reducing seizures.

"When the progression starts, it starts quickly," said Linda. "So Chelsea's day-to-day activity after four-and-a-half years since her first seizure is quite different."

Linda's day typically starts at 7:30 a.m. when she begins giving Chelsea her medications and nutrition through a gastrostomy tube. "While she can still swallow, she doesn't take in enough," Linda said. "The thought process is difficult to do all that."

Most of the time, Chelsea is still sleeping. "She's lying in bed, very dream-like and kind of checking out her room, very toddler like," said Linda. "If you can imagine a baby in a crib, that's how she is."

"Chelsea was always very, very healthy, walking before she was 1, a chatterbox, reading at 4, just your normal average young girl," recalled Linda. "Before, Chelsea was very independent and now she's 100 percent dependent."

She added, "The only thing that she can do on her own is enjoy music and only because she can still hear it."

Chelsea's body clock is much like that of other teenagers and she can't fall asleep until about 11:30 p.m. "Even if we get her upstairs and in bed, there's still a 45-minute to one-hour process of getting her ready to go to sleep."

Often, Chelsea will wake up in the middle of the night if she's uncomfortable, has seizure activity or has to go to the bathroom. "Sometimes, it's four to five times between midnight and 6:30 in the morning," said Linda. "It's like going back to having a baby that wakes up and needs you in the middle of the night."

Chelsea wakes up at around 11 a.m., and Linda washes her face and brushes her teeth while she's in her hospital bed.

"I try to be as upbeat as I can when I'm doing all this. We turn on music, dance, sing and carry on. She's a good little team player," said Linda. "I tell her, 'Chelsea and mommy are a team.' She's generally very good spirited, but sometimes when she wakes up, she's blue and gets weepy and sad."

While some families tell their children that they have Lafora, the Gerbers' doctor advised against it.

"Our doctor said that it's much more difficult on those around the child affected than it is actually on the child," Linda explained. "There is obviously sadness and frustration on her part but because the brain is involved, it now affects her differently. She does say things like, 'I'm dying or am I dead?'"

If Linda can get Chelsea up and walking, she takes her into the bathroom to put on her clothes. "It's easier to do it when she's sitting up than dressing her in bed." Afterward they do a range of motion exercises.

Through funding from the state, nurse's assistants come to the Gerber house for 40 hours a week to help with Chelsea's care.

"It gives me a break and allows me to get out of the house and run my errands," said Linda. Otherwise she pretty much stays with Chelsea all day. Dad Howard is a mortgage broker and works at home so he can help, too. Brother Colin, 22, attends the University of Oregon.

"I don't like to leave her alone in her bedroom if she's awake," said Linda, "even though she's extremely limited in anything she can do."

In the afternoon, Linda tries to do an activity that will entertain them. "Basically we stick around the house and try to find some things to do. We finger paint, play with shaving cream and play ball. It's all very simple stuff. Everything takes a long time and before we know it, it's 6 p.m. The day goes by kind of quickly - or painstakingly slow."

Linda said that one of her challenges is finding simple things that might be fun for Chelsea even if she really can't participate.

"The other day I got in bed with Chelsea with all the markers and put a big white board across our laps and gave her markers and we just played," noted Linda. "But it's getting more and more difficult. While she can still have fun with that, she also gets bored."

"Even though it's tragic what is happening to Chelsea, we still have to work every day to bring joy to her life because it's still her life," she explained. "We just couldn't shut our doors and windows and stay home. We had to eventually share and invite people to be part of our life because this is our life."

As Chelsea became more infirm, the Gerbers lost contact with many friends but they have formed new lifelong bonds with others. One day, Barb Goldsmith re-entered their lives.

Barb had known Linda and Howard since they all belong to Beth Chaim synagogue and had heard that Chelsea was ill.

"My memory of her was this darling little sprite who was 3 years old running around my house at a party," recalled Barb. "I wanted to do something but didn't know what."

She e-mailed Howard, who invited her to come over. "I decided to make a big tray of cookies and left that day and never asked the name of the disease," Barb said. "When I got home we made the decision as a family that we were in this for the long haul. This is not something that anybody should have to go through and certainly not alone."

After a couple of months, Barb arranged to meet researchers at UCLA to find out about starting a foundation and raising funds. She learned that there were others in the country and in the world coping with Lafora, and that she needed to form a nonprofit organization plus start a Web site to educate others and share information. Almost two years ago, Chelsea's Hope was officially launched, and quickly other families became involved.

"We wanted to do bios on other kids as well as Chelsea," Barb said. "It was called Chelsea's Hope but it was really hope for all these kids."

Donations to Chelsea's Hope benefit the Lafora's Children's Research Fund at UCLA to further research and improve treatments and find a cure. To date it has raised more than $150,000.

"I can honestly say that I wouldn't be as good as I am today without Chelsea's Hope," said Michele Ambrose from Pennsylvania, whose 15-year-old daughter Jessica was diagnosed in 2008. "It's not like the American Cancer Society where there is already a network. People who understand can offer advice. Even friends and family can't understand. I don't know what I would have done without Linda and Barb and to be able to talk to other people."

Dr. Berge Minassian, staff neurologist at the Hospital for Sick Children in Toronto, has been working on Lafora research since 1993 when he was doing his residency at UCLA.

"There are only about 12 cases in the United States and 200 documented cases worldwide, and the chances of contracting the disease are about one in a million," he said. Referred to as an "orphan disease," Lafora currently receives little or no government funding.

"Chelsea's Hope has been fantastic and special," said Minassian. "Lafora is so rare, it's very hard to form a cohesive group to raise money. While their children are alive, parents don't have the energy to devote to fundraising."

Dr. Antonio Delgado-Escueta, professor of Neurology at UCLA, and his team are developing two treatment approaches, gene therapy and getamicin treatment.

"The research that is being conducted at the Hospital for Sick Children is focusing on stem cell replacement and gene therapy," Minassian explained.

"It's taken a couple of years for me to develop the strength to be able to talk about it without just crying," said Linda. "The relationships that we have created in joining people on our Web site and personally the level of intimacy that gets crossed over is mind boggling."

"This whole thing is much bigger than me, it's bigger than us," she added. "I just feel that we're at the start and the ball is rolling."

Linda advises others to reach out like her family, not to worry about infringing on their privacy.

"The most important thing is to just show up and say, 'I don't know what to say.' I would like people to go out of their comfort zone and extend a hand and be there for anybody who has a tragedy. But be there in person," she said.

"People will call and say, 'What can I do?'" said Barb. "That puts the onus on Linda and Howard. Call them and say, 'I'm doing my marketing tomorrow and I'm going to pick up stuff and make dinner for you.'"

The Gerbers need to move Chelsea's bedroom downstairs and modify the bathroom to make it handicapped accessible. "Going up and down the winding stairs is treacherous at best," said Linda. "Howard carries her a lot of the times. I just think we're all going to end up at the bottom of the stairs one day."

Linda Stolow, owner of Small-Fry Shoppe, a children's consignment store in Danville, stepped in to lend a helping hand to raise funds for the bathroom renovation. "Catastrophic illnesses can play havoc with one's finances," Stolow said.

After meeting Linda Gerber, Stolow decided to set up an account for Chelsea's Hope and has raised more than $500 from donations of children's clothing, toys and other items. She's also elicited participation from other downtown stores, including Something Old, Something New.

"My theory is that this is Chelsea's life and we have to help live it whatever way that is," said Linda Gerber. "And it's our life and we can't just always stay secluded. That's another reason why we need to move Chelsea downstairs so that when we have friends come over, she is part of it."

"You never know in life," she added. "You think that you have two beautiful healthy children on a Monday, and on Tuesday your life changes. So anybody who thinks that couldn't be them better take a second and realize that 'but for the grace of God' it certainly could be. That's my take from this whole thing."

Although Chelsea has been stricken by a devastating disease requiring 24-hour care, her glowing eyes reflect the love and devotion of her parents. They are committed to making the best out of each day of Chelsea's life. And improving the lives of others.

The Lafora children

Professional photographer Cheryl Bigman joined Chelsea's Hope with a vision to bring awareness and assist with fundraising through creating a photo exhibit to display all the images of "the Lafora children" that they have connected with in the United States.

"Sometimes I went alone and sometimes Linda went with me to take the photos," said Cheryl, who donated her time and services. "It was an incredible experience to meet children at different stages and see the progression of the disease."

"The one thing that the families had in common is that they felt completely alone in the world until they found Chelsea's Hope. Now they had a place to talk to other people," she said.

Visit the Web site at www.chelseashope.org. For donations for the Gerber family, contact Linda Stolow at 838-8885.

Bigman's work can be seen at www.cherylbigmanphoto.com.

Comments

Posted by Cookie, a resident of another community
on Sep 18, 2009 at 7:16 pm

I just wanted to say that my heart breaks for Chelsea's family or any family that has to watch their child endure such a disease as Lafora. Everytime you complain about the hardships that you have as a parent, take a step back and think just how lucky you really are.
I am a very close friend to Barb and I know that she and her two daughters have made a difference in Chelsea's life. It just takes one person to step up and do something. Barb has the intelligence, the warmth, the empathy and the love to make a difference! Hooray for Barb!


Posted by Evelyn Gross, a resident of another community
on Sep 20, 2009 at 8:53 am

I am Barb's mom. Every summer I go out to visit Barb and I think it was about 3 or 4 years ago when I got to meet Chelsea, and I am in love with her. She is precious, and my 2 granddaughters love her completly and Chelsea's eyes light up the minute she sees them because they try to do what she wants them to do. They are away now attending college but they are close to home and they try to see her when they are home. My heart goes out to Linda and Howard, they are so loving and adoring to Chelsea. This is the first summer that I did not come to California and I miss the visit with Chelsea. Just tell Chelsea that Alyssa and Danielle's nana says hi and I love her.


Posted by Julie Strong, a resident of another community
on Sep 20, 2009 at 3:47 pm

Linda and I were best friends throughout our carefree high school years. Linda always had great strength of character, but little did we know how that strength would be tested. She is such a wonderful, courageous mother. Since the Gerber's moved fro LA to Danville, I've only been able to visit one time, but meeting Chelsea only once is enough for her to steal your heart. Even through all of her suffering she has a spirit that is infectious and a smile that brightens your life. Linda, Howard, Chelsea and Collin are continually in my thoughts and prayers~keep them in yours as well.


Posted by Linda Stolow, a resident of Danville
on Sep 21, 2009 at 10:22 am

The story of Chelsea is heartwrenching. To see a teenager go from healthy and vivacious to sick and silent is a tragedy. Our community needs to reach out to the Gerbers and help them get through this. We are looking for volunteers to help rebuild a downstairs bathroom so Chelsea does not have to be carried up and down stairs daily, something dangerous both for her and her parents. Diseases like this take both a physical, financial and emotional toll on the entire family. Please contact me at 838-8885 if you are local and able to help.


Posted by Dr. Lisbeth Roy, a resident of another community
on Oct 21, 2009 at 11:37 pm

Thank you for writing this article. I am happy to find that there is a community for support. I just lost my son Liam, age 23 on September 23rd, 2009. He was a beautiful boy who endured 11 years of this progressive illness. His father and I have felt alone for all these years. This is a very difficult disease process requiring constant adjustment to changing levels of cognition and physical function. Thank you to all those taking an interest in helping families live day to day. We all know too well that this disease is fatal... but our children die in pieces over years of declining health, new challenges and what feels like no hope at all. This process is a lesson in being. Liam understood what it is to simply be alive... in the last few years, he wasn't able to speak or do ANYTHING for himself, yet he still managed to laugh and smile whenever possible. I am still learning such grace. I can tell you that this lesson is painfully difficult. Help is what families need but they don't often even know how to ask or what to ask for. Basics such as food, home and yard assistance, and respite are all helpful but what we found gave us the most relief is when people would visit Liam. Liam felt isolated and deserved interaction and attention. These kids are still "in there" even though they have lost much of themselves. They NEED and desire social interaction with peers. They need to feel included and special... like they matter. As parent, we are most light hearted when our children feel loved and valued... visiting them just because always made Liam feel more special. Thanks again for raising awareness. Helping is VERY simple and rewarding beyond belief. These kids have so much to teach us.