Although Philip Bach, a”h, struggled with Familial Dysautonomia (FD) from birth and only lived to the age of 27, he managed to touch the hearts and lives of many in the Five Towns and Far Rockaway community.
On Thursday, June 19, FDNOW, the Familial Dysautonomia Now Foundation, a research organization, will hold a dinner at the Sephardic Temple in Cedarhurst to raise funds to continue research to improve the lives of those with FD and honoring Philip’s memory.
FD is a rare, genetic disease that impairs the autonomic nervous system; sufferers have difficulty breathing and eating, their heart rate, blood pressure and temperature are erratic and unpredictable, and their pain sensors are affected. Those with FD can go into “autonomic crisis” due to various environmental triggers, with surges in blood pressure, heart rate and violent retching.
Philip peacefully passed away in his sleep on Dec. 29, 2013. The oldest child of Mindy and David Bach of North Woodmere, he was diagnosed when he was 10 days old by an FD specialist at NYU Medical Center. Philip could not eat and was fitted with a nasogastric tube in his nose and a fundoplication to prevent reflux internally. From birth, he never tasted food.
“The prognosis was not too promising,” recalls Mindy. “I was very afraid and it was overwhelming.” Philip was developmentally delayed but reached walking and talking milestones and had surgery at age 11 for scoliosis caused by FD, she said; he suffered from corneal abrasion, “lacked tears from day one” and was legally blind from infancy.
The family dealt with the many crisis and manifestations of the disease, the erratic blood pressure, nausea, extreme temperature and heart rate fluctuations. “It’s a sensory illness, they don’t know where their body is in space. There was always something to be done,” she said, regarding his care. “With all that, I wish he was here and I was doing all that stuff because he gave back a lot.”
He went to a school in Baldwin that offered medical care, but “he was a time bomb.”
His two siblings, a boy and girl twins, are seven years younger than Philip, now both in nursing school, and interacted very well with him. “They were his friends and pals and were very tolerant and were never ashamed or embarrassed (by him). I always wanted him to be treated with dignity and I instilled that in them.” She said if he drooled they would wipe his mouth with “no fuss.”
But he was much more than his FD. He had a photographic memory and could do complex math in his head—he was able to tell someone the day of the week they were born when given a birthdate. He was inquisitive and thirsted for knowledge in spite of his limited vision. He was a Yankee fan and knew all the statistics, and loved game shows, explaining how much he learned from their question and answer format.
Mindy said that he loved Judaism, going to shul and he was bar mitzvahed. “He would always amaze me with facts he learned and would teach himself. He was fun, funny and had a dry sense of humor.’
“He never complained, he was a trooper,” she said. “He brought an amazing joy to the whole family. Now there is an empty hole.”
FDNOW is a research organization that developed supplements that affected and controlled some of the symptoms, said Mindy. She said that the supplements decreased the intensity and frequency of autonomic crisis and seemed to improve his concentration, awareness and alertness. The organization supports the research of Drs. Berish Rubin and Sylvia Anderson at the Laboratory for Familial Dysautonomia Research at Fordham University.
One in 27 Ashkenazi Jews are carriers of FD; genetic testing to detect the mutation is available. There are estimates of 600 individuals with the disease worldwide.
Philip’s funeral at Boulevard Funeral Home in Hewlett was packed with supporters and friends of the family, with rabbis, politicians, principals and staff of local schools and his siblings’ classmates; people stood in the aisles for lack of space. Speakers recounted his joy and their love for him and his caring for others. “He was very unique,” said Mindy. “I don’t think there is another like him.”
May his memory be a blessing.
FDNOW’s dinner: Sephardic Temple, 775 Branch Blvd., Cedarhurst, Thursday, June 19. For more information go to fdnow.org.