August 2002 Actuarial Review - Combating a Crippling Disease</I>

Nonactuarial Pursuits of Casualty Actuaries
Combating a Crippling Disease
by Marty Adler
Heretofore my columns have focused on interesting avocations of our fellow professionals, but you can also get enormous satisfaction and a sense of accomplishment by using your free time in working for a charity. Sometimes the incentive comes when a debilitating disease strikes a family member. Peter Licht was impelled to such activity when his son, Daniel, was afflicted with Fibrodysplasia Ossificans Progressiva (FOP). FOP is a rare genetic disorder. Bone forms in muscles, tendons, ligaments, and other connective tissues. Extra bone develops across joints, progressively restricting movement. Overall, the body not only produces too much bone, but an extra skeleton that immobilizes the joints of the body.
Except for congenital malformations of the great toe, children with FOP appear normal at birth. But soon, they form painful fibrous nodules over the neck, back, and shoulders, which then develop into bone. After progressing along the trunk and limbs, these lesions slowly replace the body's muscles with normal appearing bone. Any attempt to remove the extra bone produces even more robust bone formation because trauma accelerates the disease process. People who have FOP experience different and unpredictable rates of new bone formation. It appears, however, that the progression has a characteristic pattern, with impact on the back and shoulder areas almost always preceding the development of FOP in lower areas, such as the hips and knees. Most adults are wheelchair-bound by their 30's.
Daniel showed symptoms since age one but was misdiagnosed. As a result he underwent unnecessary surgery and chemotherapy. Eventually a pediatric oncologist at the Sloan-Kettering Institute told Peter and his wife, Jeri, that Daniel did not have what they had thought. She directed them to the University of Pennsylvania Hospital where Daniel was diagnosed with FOP. That was five years ago on Daniel's third birthday. On that fateful day they also met another family with a child with FOP. The families would become a great comfort and support to each other.
The FOP diagnosis changed life for Peter and Jeri, who abandoned her career as a personal injury attorney to dedicate herself to Daniel's special needs and daily happiness. Peter immediately devoted his time to looking for information about FOP on the Web and discovered the International FOP Association (IFOPA), a support organization of families affected by FOP.
IFOPA supports education, communication and a search for a cure. There are fewer than 300 members from 26 countries with FOP, and fewer than 200 in the U.S. However, with an incidence of one in two million, it is estimated that about 2,500 people worldwide actually have the condition. Once the shock of the diagnosis wore off a little, Peter and Jeri started a letter-writing campaign, letting their families know about the diagnosis and asking for funds for research. Peter then expanded this to their friends, to business associates, and ultimately to their entire community.
Jeri then began volunteering her time to the IFOPA, which as a charitable nonprofit, depends on volunteers. Initially, she chaired the Membership Committee, where she contacted newly diagnosed families, fielded many questions on member problems, directed people to sources of information, and more. She has now joined the Fund-Raising Committee.
After first becoming active on the IFOPA Finance Committee making recommendations to the board of directors, Peter became treasurer in January 2001. It requires about two hours each week, plus 10 more at the end of each quarter, including a report to the board of directors, and 20 hours at year-end. The IFOPA must make conservative investments, as it needs a steady cash flow. It has about a six-month planning horizon because the funds are not guaranteed. Almost all funds come from family fund-raising. In addition to the IFOPA grants for research, the FOP Laboratory at the University of Pennsylvania, where most FOP research is done, receives support from the National Institutes of Health and the University of Pennsylvania. The scientists in the lab are dedicated to finding a genetic switch that will turn off the bone-making gene. This research can benefit more than just those with FOP by providing insights into such prevalent bone-related disorders as osteoporosis, arthritis, certain heart valve disorders, and some spinal cord injuries. For that reason, in November 2000, there was a research symposium on FOP in Philadelphia attended by scientists from around the world.
In addition to working for the IFOPA, Peter stays in contact with its members. Since FOP is such a rare disease, there are few parents to contact for helpful advice on handling various aspects of living with it. The IFOPA has an Internet mail group, which enables members to tell one another how they handled difficult situations. Normal health problems may have threatening implications for those afflicted with FOP. For instance, if the bones of the rib cage are affected, the lungs cannot expand. This can make an ordinary upper respiratory infection very serious. When his son required orthodontia, Peter and Jeri knew that great care was needed because of the risk of unintentionally accelerating bone formation in the mouth. They found an orthodontist in New Jersey who had worked on another IFOPA members' child, then reached a member in Australia whose son had had a similar procedure, and connected the two specialists by e-mail to develop a plan of treatment for Daniel.
FOP has been called a "terrorist disease." One does not know when it will strike and whether a flare-up will be temporary or cause permanent loss of movement of a limb. Peter finds that his son's affliction has put his job stress in perspective. He and Jeri remain upbeat and optimistic. They believe that the funds that they and others are raising to support the research at Penn will lead to alleviation of the effects of the disease and perhaps some day find a cure.
Editor's Note: For more information about FOP or the IFOPA, or to make a donation, please visit www.IFOPA.org or write to International FOP Association, PO Box 196217, Winter Spring, Florida 32719-6217.