Copyright 2004 The Buffalo News  
Buffalo News (New York)
July 11, 2004 Sunday, FINAL EDITION
SECTION: NEWS, Pg.A1
LENGTH: 1471 words
HEADLINE: THE COST OF COURAGE/ DENEANE CHIPLOCK IS THE 25TH MEMBER OF HER FAMILY TO DEVELOP ALS. THE OTHERS DIED WITHIN 13 MONTHS AFTER SYMPTOMS BEGAN./
BYLINE: Henry L. Davis; NEWS STAFF REPORTER

Not many people can see into the future, but Deneane Chiplock knows enough to terrify her.

At 39, she is the 25th member in three generations of her Buffalo family to develop Lou Gehrig's disease. It is a death sentence. All others in her family who suffered from this disorder died within 13 months after symptoms began.

"I know what I'm in for. I've been there," said Chiplock, whose mother and two of her mother's sisters died from the disease.

Also known as amyotrophic lateral sclerosis, or ALS, the incurable condition progressively paralyzes its victims until they can't speak, swallow or breathe. All the while, the brain remains alert. To the doctors, researchers and patient advocates who have come to know Chiplock and her family, their extraordinary struggle against the disease sheds light on larger issues.

Chiplock's insurance won't pay $9,300 a month for an unproven therapy that might prolong her life -- or shorten it -- forcing her to become a pauper or a panhandler to get the drug.

And now that a genetic test is available, other family members must decide if they want to learn whether they carry the gene mutations for ALS, even though they can do virtually nothing to slow the little-understood deterioration of their nerve cells.

Chiplock's sister, Anita Crawley, needed to know. Her brother, Dennis Crawley, doesn't want to.

Chiplock's story also is a reality check for people with fanciful notions about how they'd spend time if given a year to live.

Like millions of others with costly illnesses, Chiplock and her husband, Jerry, have bills to pay, children to care for and a house to maintain. Their savings is already exhausted. "I feel as though I've done all the things a responsible person is supposed to do. Now, I learn there is no safety net. It's so discouraging," she said.

The couple moved to Saginaw, Mich., in the 1990s for their careers. He is a hospital executive. She worked as a WorldCom account representative until illness forced her to quit in 1998.

Chiplock now spends her days preparing for her decline and death. She talks about it with an urgency that falters only when the subject comes to the couple's sons Sean, 14, and Evan, 10.

She hates the thought of them living without her, and her heart breaks knowing that she will not see them grow up. In her remaining time, she fills "memory boxes" for each boy with photographs, Christmas ornaments, favorite recipes and diarylike notes about memorable family incidents.

She slipped in a Bible and several books about religion, even though Chiplock fights to hold on to her faith.

"Why is my family still waiting for a miracle? Where's the healing?" Chiplock asked. "I haven't figured things out for myself. I hope the books help them."

As the disease causes nerve cells to waste away and die, the brain loses the ability to initiate and control muscle movements. Chiplock, who was diagnosed almost eight months ago, has no use of her right leg and minimal use of her right arm.

Eventually, patients can't dress or feed themselves, sit up, walk, or even speak. Most patients survive three to five years after diagnosis, often dying when they can no longer breathe. "It's like being buried alive," Chiplock said.

Slow progress

About 30,000 Americans suffer from ALS, and 10 percent have an inherited form of the disease. At least 5,600 new cases are diagnosed each year.

ALS killed Yankee great Lou Gehrig and the main subject of the bestseller "Tuesdays With Morrie." The discovery in the 1990s of the first genetic defects linked to ALS generated excitement. This was the first time any cause had been found, although these mutations account for only 20 percent of inherited cases.

Progress has moved slowly as researchers grapple with the complexity of the nervous system and the interactions that lead to disease. "When we identified the first gene, we were very optimistic about understanding how these mutations made something new and toxic happen in ALS. But, in many ways, we're back to square one," said Nailah Siddique, a neurogenetics expert at Northwestern University.

Chiplock, seven months pregnant with Evan, sent her blood to Northwestern for a genetic test in 1993, soon after the test was developed. A child who has one parent with familial ALS stands a 50 percent chance of inheriting the defective gene. People with the mutations have a 90 percent chance of developing the disease by age 70. "I couldn't make responsible decisions without the knowledge," she said.

Looking back, Chiplock said, she would not have had children had she known for sure of the genetic link that her family always suspected. "As much as I love my kids, I would not want them to go through this illness and death," she said. "I wouldn't want them to carry the burden."

The decision to seek genetic testing before symptoms arise is personal.

Those who carry the gene defect can't predict when or whether they will get ALS. Nor do they have a way to prevent it. Individuals with a positive result also worry that insurers and employers will use the results to discriminate against them.

Chiplock's sister, Anita Crawley, got tested in 1996 after feeling numbness in her hand. The sensation turned out to be from a pinched nerve, but the test results showed she carried the mutations. "I kind of always knew inside that I had it but had this hope that I would somehow slip through," said Crawley, a nurse practitioner at Women and Children's Hospital.

At 38, she is a single mother in Buffalo with 6-year-old twins. Like her sister, she is filling two memory boxes in fearful preparation for the future. "I'm scared. Either I gave this disease to them or I leave them," she said.

There is no regret though.

"My children are the best thing that ever happened to me. I look at myself. I've had a life. I wouldn't be here if my mom didn't have kids," Crawley said.

The sisters' younger brother Dennis would rather not know. To him, it's not worth getting tested as long as there is no adequate treatment or cure.

"If I could pull out a crystal ball and show you when and how you would die, would you want to know? What if there was nothing you could do about it?" he asked.

Where both sisters worry constantly about the disease, the 34-year-old brother tries to keep the uncertainty from intruding on the life he has built with his wife and two children. "Things change immensely after you know, and I just want to be able to run around with my kids without that stress," said Crawley, a laborer at Niagara LaSalle Steel and former semipro baseball player who collects Lou Gehrig memorabilia.

Gaps in system

A devastating illness can be a harsh introduction to an imperfect health system.

Chiplock spends many hours trying to arrange and pay for her care. She's frustrated by insurance policies that will support her last days in an institution but not in her home, where she would prefer to die. She can barely make it upstairs, but an automated chairlift is out of the question. It costs more than $10,000.

Instead, the couple will spend almost as much to put a hospital bed in the dining room and add a shower big enough for a wheelchair to the first-floor bathroom. Chiplock and her husband earn too much to qualify for government assistance but not enough to keep pace with medical expenses.

Money is a constant concern, forcing the family to hold fund-raisers and plead for help. In January, they took an advance payment of $70,000 from her $140,000 life insurance policy.

Chiplock estimates her care will cost more than $218,000. Much of it involves expenses for which government and private insurance offer little or no coverage, such as household modifications and help with dressing, bathing and toileting. She is not alone. With financial help lacking, more than 27 million Americans spend 20 hours or more a week caring for a loved one who is frail, disabled or chronically ill, according to the National Family Caregivers Association.

Once the disease strikes, therapy focuses on symptom relief.

Chiplock takes a "cocktail" of more than a dozen pills that includes nutritional supplements. She also uses riluzole, the only drug approved by the Food and Drug Administration for ALS. But the medication prolongs life by only a few months.

Chiplock, desperate for something better, in May started on a $310-a-day antibiotic called ceftriaxone that insurers and others consider unproven for ALS. The medication is normally prescribed for a few weeks. ALS patients would need it daily for the rest of their lives.

"I'll likely be dead before the human trials begin," she said. "I need this drug now. Let me be part of the answer. It's the best hope out there."