Rather than sink into despair, Avichai Kremer decided to fight back against his rare disease.
By JUDY SIEGEL-ITZKOVICHAvichai Kremer 298 88(photo credit: Courtesy)
One might be flattered to be told one has something in common with brilliant theoretical physicist Prof. Stephen Hawking, Russian composer Dimitri Shostakovich, US baseball hero Lou Gehrig or even Chinese Communist leader Mao Zedong. But it's a tragedy when the connection is amyotrophic lateral sclerosis (ALS) - a progressive and incurable neurological disease with an average life expectancy of three years after diagnosis.
A less-known victim is Avichai Kremer, a 32-yearold Israeli pursuing his master's degree at Harvard Business School. He was engaged to a lovely young woman. He planned to start his own company within a decade, and anticipated a rosy future years and decades ahead - that is, until he felt pain and weakness in his right arm one day, underwent a battery of neurological tests and was diagnosed with ALS (also known as motor neurone disease). He was so shocked by this seeming death sentence that his 60-year-old doctor had to help him from his chair, and Kremer spent the next few days in bed.
But although 18 months have passed since that terrible day, he no longer wants to jump off a tall building. Instead, this impressive young man is fighting not only for his own life but for the 500 ALS victims in Israel and the one in 10,000 around the world.
Kremer became active in the Israel ALS Society (www.israls.org), established two years ago by Haifa businessman and ALS patient David Cohen to boost awareness of the disorder. Kremer has already raised $1.5 million for ALS research, thanks to help from friends here and in Harvard. After taking seven months off from his studies to promote ALS awareness and raise funds in Israel, he has now returned to Massachusetts to complete his degree and raise more research money. In the meantime, the organization's new director-general is former teacher Nir Tzoran - an ALS patient from Moshav Avigdor who went around the country to promote awareness of the disorder.
Born in Haifa, Kremer has a father (a doctor), a mother (a nurse) and a sister and brother. All are free of the disease, as only in about 10% of the cases is there a genetic link; in the rest, it is apparently spontaneous. His mother, he said, tries to pretend his condition doesn't exist, while his father is "super-optimistic and sure that one day it will just go away."
Kremer is more realistic.
About half of those diagnosed die within three years - some even in the first year - usually of asphyxiation while in full possession of their mental faculties. Others live and suffer much longer, their healthy minds held prisoner in helpless bodies. The struggle for the recently passed End of Life Law legalizing passive euthanasia was initiated as a result of the struggle about a decade ago of a former Israel Air Force officer with ALS who demanded to die in dignity and not be kept alive artificially with a respirator.
THE DISEASE was first described 140 years ago by French neurologist Jean-Martin Charcot, and very little has been learned since then. The causes and mechanism of ALS are unknown, yet neurologists do know that mis-folded and damaged proteins clump together in cells to form aggregates. This triggers the death of motor neurons, causing a loss of muscle control. Only cognition and digestion continue to function normally.
At present, ALS is totally untreatable; there is only one drug - known commercially as Rilutek and generically as riluzole - that has been approved by the US Food and Drug Administration to slow its progress, and it has been shown to extend lives for only about three months.
Kremer, who spent two years in South America and the Far East after his army service as an artillery officer, studied computer sciences at the Technion and worked for Elbit Systems. He was overjoyed to be among the few Israelis accepted to Harvard Business School.
"I used to think years ahead; now I think one day at a time. I can walk, but my hands are weak," he says. He has lost 12 kilos of muscle mass. "It's hard to do simple things. I have a problem with balance and, as you can hear, my speaking is a bit slow. Every week there is another thing I can no longer do. I can't turn a key in a lock or hold a cup in my right hand. I don't know what will come tomorrow."
His fiancee, sadly, decided to leave him. "It's hard, the hardest thing to lose, but I understand her." But he has a support system, with family in Haifa as well friends at Harvard. Although huge amounts of money have been invested in AIDS research, almost none has been put into the study of ALS. It's a vicious circle because it's an "orphan disease" - contracted by a relatively small number of people, Kremer says. With ALS, the problem is also the fact that the average victim lives for only three years after diagnosis.
"Why should international drug companies invest in improving the life of a patient who will live only three years, when they can sell drugs for combating baldness, impotence or acne?"
Harvard Medical School has its own non-profit Project ALS, established in 1999 and dedicated to funding research toward a deeper understanding, treatments and a cure. Research programs include genetics and gene therapy, stem cells, drug discovery and identification of disease pathways.
But Kremer thought that his native country, with its many creative minds, should have its own ALS Project. He and his friends recruited Israeli scientists on the cutting edge of neurology and immunology research and put them in contact with leading Israeli investors, with the goal of finding a treatment and a cure.
A NUMBER of prominent businessmen, including Uzia Galil (Elron), Dov Lautman (Delta Galil), Dr. Ed Mlavsky (Gemini Capital) and Technion Medical Faculty Prof. Peretz Lavie have already started to promote the project. A review committee to decide which research will receive grants includes Weizmann Institute of Science Prof. Michal Schwartz, Hadassah University Medical Center immunobiology researcher and bone marrow transplantation expert Prof. Shimon Slavin, Hadassah embryonic stem cell expert Prof. Benjamin Reubinoff, and Technion Prof. Moussa Youdim, who developed Rasagiline, a new Parkinson's disease drug.
"The Technion, Weizmann Institute and Tel Aviv University are already doing research, and it's very promising. Some are already doing clinical research. If things advance, I will come back before I finish my degree," Kremer says.
Sara Shnider, a Kfar Saba resident who has started studying for her doctorate in neurobiology at Harvard, has become active in ISRALS. She heard about the disease and Kremer's predicament, viewing an interview TV host Yair Lapid did with him (it can be viewed on the Internet at www.israls.org/movie4.htm).
"I took it personally. It touched my heart," she says. Taking a semester off from her studies to help, Shnider volunteers by maintaining contacts between the association and the research teams in biotech startups or academic settings. "I learn a lot, because it melds with my background in science."
IN THE past few years, basic research into ALS has increased understanding of the disorder and offered some hope. Dutch researchers working on a rat model of ALS found the rodents lived longer after getting a protein called VEGF - a signaling substance that controls the growth of blood vessels. They also found that people who produce too little VEGF - due to certain variations in the gene that codes for it - have a greater chance of developing ALS than those who produce normal amounts. The Dutch team also proved that therapy with the VEGF gene increased the life expectancy of ALS mice by 30%. But gene therapy is still a controversial treatment, whose path to the clinic is likely to be long and winding.
Other possible avenues of research include implanting glia cells to support the motor neurons that die off, silencing mutant genes, and using a neuron support factor to treat ALS. University of California at San Diego scientists created a mouse model of ALS with a mutation in the enzyme copper-zinc-superoxide dismutase (SOD1), which causes about 20% of the inherited form of ALS and about 2% of total cases.
They found that when support cells called glia don't perform properly, the neurons suffer. The researchers found that when neurons with the SOD1 mutation were surrounded by glia with normal genes, these support cells were able to stave off the disease. But when mutant glia cells encircled genetically normal motor neurons, the neurons were attacked by the glia. This finding could someday lead to a new ALS therapy.
Contrary to conventional wisdom, most ALS patients are not depressed or more likely to become so as the end nears, according to a University of California at San Francisco study published a few months ago in Neurology. "The resiliency of people with ALS is inspiring for all working in the field, and helps remind us daily of our own mortality," the authors wrote.
That is apparently true for Kremer, who continues to promote research but knows it takes a long time to bring it to the clinical level. He realizes that he may not benefit personally but, as he says: "you have to start somewhere."
(Donations for The ALS Project can be sent to POB 55075, Haifa 34980, by calling tel. (04) 825-2233 or through the Web site at www.israls.org.)
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