Living a good life in the face of certain death Young couple vows to give their son as much of a childhood as they can BY PATRICIA A. MILLER Staff Writer CHRIS KELLY staff Tyler Fox, 4, and his two-year-old sister, Ava, share some toys in their Wood Avenue home Friday. Tyler suffers from a form of Batten disease, an incurable illness that took the life of his five-year-old sister Kaliann last fall. Like most parents, Michelle and Michael Fox wanted only the best for their three children. And like all good parents, they would do anything to spare a child of theirs from suffering. So there will be no feeding tube for their young son, Tyler, when his time comes. Tyler, who turned 4 in February, will die from the same disease that took his 5-year-old sister, Kaliann, last fall. The two children were both diagnosed with the late infantile form of neuronal ceroid lipofuscinosis, or Batten disease, a rare inherited genetic disorder that occurs in only 2 to 4 of every 100,000 births in the United States. It is a killer. It gradually robs children of muscle coordination. They suffer worsening seizures and progressive mental deterioration. Eventually they become blind, bedridden and unable to communicate. There is no cure. The Fox family in happier times. Above, Kaliann gazes at her newborn sister, Ava, in the hospital two years ago. At left. the family gathers for a shot on the lawn of their Wood Avenue home in Brick. "I'm prepared for it," Michelle said, as she sat in her sun-filled living room on Wood Avenue last week while Tyler and his 2-year-old sister, Ava, played. "It doesn't make it any easier. I've accepted it is going to happen." It all began with a fever two months before Kaliann turned 3. It wasn't a high fever, but her parents noticed that the little girl's body twitched as they put her down to sleep. When it happened again, they took her to the emergency room. Kaliann had a partial seizure in the emergency room, then a grand mal seizure the following morning. The doctors told the Foxes she had febrile seizure disorder. Michelle didn't believe the seizures were merely fever-related. The couple made arrangements to take Kaliann to the A.I. DuPont Hospital for Children in Wilmington, Del. A few weeks before the appointment, Kaliann had a seizure without a fever. COURTESY OF MICHELLE AND MICHAEL FOX The little girl was put on a variety of anti-seizure medications, some that left her with tremors. The Foxes put her on a rigorous diet designed to control seizures. But Kaliann began falling. Her speech deteriorated. The seizures increased, sometimes to as many as 50 a day. "She was going backward," her mother said. "She was falling a lot." After six months of MRIs, EEGs and blood work, the Foxes finally had an answer. It was a chilling one. Kaliann had one of the worst forms of Batten disease - the late infantile stage. Neither Michelle nor Michael, both 27, knew that they each carried a recessive gene for the disorder when they married in November 2000. Childhood NCL occurs when a child inherits two copies of the recessive gene, one from each parent. Only Ava has been spared. Unfortunately, Tyler's disease is progressing a lot more quickly than Kaliann's did. Photos of Tyler from a year ago show an alert, smiling little boy. These days, he appears sluggish. He rarely smiles. And today is all that matters for Michelle and Michael Fox. "Right now, we just try to keep Tyler happy," his mother said. "Tyler gets to do what Tyler wants to do most of the time." Kaliann was "a tough cookie," Michelle said. "Tyler is not the same way," she said. "He'll fall and be upset. She didn't care. When she started falling, she would get right back up. She was amazing." The disease affects the swallowing reflex, which can lead to aspiration. Tyler has already had some choking episodes. The Foxes made the decision to have surgery for a feeding tube to be put into Kaliann's abdomen when the choking increased and the little girl could not eat normally. "She went into the hospital walking and talking," her mother said. "She came out of the hospital not walking and talking." Photos of Kaliann after the surgery show a little girl sitting in a padded wheelchair tethered to a machine. As time went on, Kaliann's bowels became severely impacted. She was in severe pain, her mother said. Michelle and Michael do not want their son to go through what his sister did. "We just want to let it happen the way it's supposed to happen," Michelle said. Michelle and Michael are not traditional churchgoers, but they have a strong faith in God. "We have our own little church at 53 Wood Avenue," she said. "I don't think we could go through all of this and not believe in something." And they are allies in this battle to give their children the best while they are here. "We just have a really great support system," she said. "We've been on the same page with everything. A lot of the decisions we had to make, we just felt the same way. We weren't meant to intervene. We just wanted to make them happy while they are here." They took Kaliann out whenever they could, to try and give the little girl some sense of normalcy. There are photos of Kaliann at the Camden Aquarium. There are shots of her at the Manasquan Inlet, one of her favorite places. When she was too ill to go out, Michelle arranged play dates with other children at their home. They are doing the same for Tyler. Michelle can calmly discuss the care both her son and daughter required. She can rattle off the dates of medical emergencies and test results. But her armor cracks when she thinks about what she will tell Ava about her sister and brother. "She is going to be too young to remember Kaliann," she said tearfully. There is a small plaque on the Foxes' front door. "Everything happens for a reason," it reads. "Just believe." "I really believe that," she said, wiping away tears. "But I don't know what the reason is." Underneath the plaque is a birthday cake made out of colored construction paper. There are four candles. "Happy Birthday Tyler!" is written in the middle of the cake. How you can help The Brick Township Policemen's Benevolent Association Local 230 will hold its 18th annual Helping Hands Basketball Game on March 16 at Brick Township High School on Chambers Bridge Road. Members of the police department will play against members of the Brick Township High School Dragons football team. All proceeds from the game will go to the Fox family, who live on Wood Drive in Brick. Michelle and Michael Fox are facing financial hardship from the illnesses of their daughter Kaliann, who died Sept. 30 at age 5, and their 4-year-old son, Tyler. Both children were diagnosed with the infantile form of Batten disease, also known as neuronal ceroid lipofuscinosis, a progressive, inherited neurological disease characterized by seizures, loss of cognitive and motor skills, loss of vision and early death. Tip-off is at 7:30 p.m. in the Brick Township High School East gym. Seating is limited, so plan to arrive early. Tickets can be purchased from any PBA 230 member. Donations can also be mailed to the Brick Township Police Department, 401 Chambers Bridge Road, Brick 08723, c/o Patrolman Richard Nelson Jr. Checks should be made payable to the "Tyler Fox Fund." For more information, contact Officers Nelson, Greg Mullarkey or Robert Scelfo at (732) 262-1152. Donations for the Fox family can also be sent to: Sunshine Fund, P.O. Box 913, Point Pleasant 08742. To see more of Kaliann's and Tyler's stories, go to www.caringbridge.org/visit/kaliannfox or www.caringbridge.org/visit/tylerfox.