When their daughter Tziporah began falling, Esther and Arthur Herzfeld wondered at first if her shoes didn’t fit right or whether she was just clumsy.
Over a decade, however, each of the Teaneck couple’s four children began stumbling so frequently that it was clear that something was frighteningly wrong. The falling was scary enough, but as the children each reached the point where they could no longer pick themselves up, the feeling of utter helplessness was almost too much for them, or their parents, to bear.
The Herzfeld children — first Tziporah, now 22, followed by Tzvi, 18, Racheli, 15, and finally Rivka, the oldest at 23 — have progressively lost muscle tone and function, including the ability to walk properly or take on simple, everyday tasks, from washing their hair to buttoning their shirts. Tzvi relies on a motorized wheelchair to get around, while his sisters walk slowly and carefully, with the terrifying knowledge that they could fall at any moment, unable to get up on their own.
The predicament — which experts say is exceptionally rare — has consumed the family financially and emotionally. It has stumped about a dozen doctors and specialists who, to the Herzfelds’ frustration, haven’t been able to say much definitively other than the children all have the same degenerative muscular condition.
At first, doctors thought it was Charcot-Marie-Tooth disease, an inherited nerve disorder that affects one in 2,500 Americans. Over time, CMT degrades the peripheral nervous system, the network that transmits information from the brain and spinal cord to the rest of the body, weakening the extremities and ultimately leading to disability. But so far, numerous tests have come back negative or inconclusive, the elder Herzfelds said. To their knowledge, no one in either of their extended families has CMT.
After a decade of doing little to draw attention to themselves or their situation, the Herzfelds are now raising money online to get the assistance they need to continue to care for their children. Meanwhile, they are eagerly awaiting the results of more specific and expensive genetic tests that they hope will pinpoint the cause behind the debilitating and evolving symptoms.
“We don’t know what the end game is, and that’s very stressful and upsetting to everybody,” Esther Herzfeld said.
Therapy sessions
Upbeat, driven and intelligent, Rivka Herzfeld can do almost anything, though it may take her longer or she may do it differently. With little muscle definition, she struggles when lifting things or transitioning from sitting to standing. She also walks slowly, on her toes, but takes pride in her improved ability — through regular and often difficult physical and occupational therapy sessions — to more easily climb stairs, do laundry and hang up her clothes. She maintains a stringent no-button-or-snaps policy when it comes to clothing. As with her siblings, her fine motor skills are not strong.“No snaps, because snaps are also hard,” Rivka said. “You have to push in, you have to apply pressure. It’s not easy.”
The progression of the children’s symptoms has upended the family’s lives and routines.
Because of this, family members haven’t traveled anywhere together since a wedding in January 2014. Their last family vacation — to Hershey Park — was about eight years ago, and they aren’t planning another one soon.
“It’s physically draining,” Esther Herzfeld said.
For the family, it is important to receive a definitive diagnosis. That way, the children can benefit should a cure be discovered, said Esther Herzfeld, who scours the Internet for promising clinical trials or breakthrough treatments.
That is particularly true for Rivka. There was a time when her parents wondered whether she, as the oldest and the last to develop symptoms, might have avoided the disease. Now she has thrown herself headlong into a search for a diagnosis, agreeing to undergo expensive tests at a time when she is still adjusting to living with the condition.
“At least for me, I need to know a name because a question mark isn’t really doing it for me,” said
Rivka, who wears a silver pendant around her neck that is engraved with her favorite Hebrew scriptures, reminding her to stay strong and focused.
Signs of weakness
Before her children began falling, Esther Herzfeld recalled, each started walking abnormally — at different ages and in different ways.Tziporah was the first. When she was 11, she began putting her toes down first instead of her heel with every step. Then Tzvi, in middle school, started favoring the sides of his feet when he walked. Racheli began tiptoeing in the first grade. Finally, Rivka developed what her mother described as a “chunky” walk late in high school.
Tzvi’s symptoms progressed the fastest. Within about a year, he went from being an active young boy to needing a wheelchair to get around. Racheli uses a scooter, but only at school.
“Imagine being completely healthy one day, and then one day you go to sleep, you wake up, you start walking funny and within the next year you can’t walk anymore,” Tzvi said.
All of these distinctive gaits, Esther Herzfeld said, were actually early signs of muscle weakness in their feet. What followed was a long and frustrating effort to improve the children’s mobility and pinpoint a diagnosis — a journey that involved consultations with about a dozen doctors, including pediatricians, neurologists and geneticists, across North Jersey and at the best hospitals in New York City.
All of the children, except Rivka, whose symptoms are less pronounced, have had several operations that doctors hoped would improve their mobility.
When they were younger, Racheli and Tzvi had procedures to lengthen their Achilles tendons. In each case, their gaits improved modestly, but the improvements proved short-lived; Tzvi eventually reverted to walking on the sides of his feet, and Racheli still walks very slowly. In 2007, when she was 13, Tziporah underwent a procedure known as a muscle transfer, in which healthy muscle was harvested from her legs and implanted where her leg muscles were at their weakest. The idea was to restore her ability to walk and improve her strength, but her mother said the procedure actually made her weaker.
Dr. Helio Pedro, the section chief of genetics at Hackensack University Medical Center, has been working with Rivka since August to find the faulty gene, or genes, causing her muscles — and, by extension, those of her siblings — to degenerate. Rivka recently submitted to an exome sequencing test, a complete review of the critical parts of all of her genes, that came back negative for abnormalities, Pedro said. Now she is undergoing more specific tests for neuromuscular disorders.
The question, Pedro said, is what caused the Herzfeld children to develop symptoms similar to CMT. “Could there be some genetic cause for it that has not been established as of now?” he asked.
Pedro has ruled out an environmental cause because some family members do not show symptoms. He said he has reason to take a closer look at the genetics on the paternal side because it’s possible for a neurological condition to present itself differently — or even unnoticeably — within the same family. It also is possible that the disease could lie in a recessive gene that is dormant in both parents, but when combined, it was passed to their children, Pedro said.
Whatever the cause, the fact all the Herzfeld children are affected makes the family’s situation appear exceptionally uncommon, said Dr. Jan Wollack, director of the Division of Child Neurology and Neurodevelopmental Disabilities at the Rutgers Robert Wood Johnson Medical School.
“It would be a lot of bad luck to get four out of four” children with the same genetic disorder, said Wollack, who has not treated the family.
Seeking a diagnosis in such cases can be both frustrating and punishingly expensive, Wollack said. Genetic tests to pinpoint the cause of a rare disorder can cost up to $10,000 in some cases — a price that, he said, is likely to remain high as more is learned about the variability of genetic defects.
“What we’re learning is defects in a given gene can give several different clinical pictures,” Wollack said. Or, he added, “a clinical picture can be attributed to a number of different genes.”
Dignity, grace, faith
Rabbi Laurence Rothwachs of Congregation Beth Aaron, the Herzfelds’ Teaneck synagogue, said it is not in the family’s nature to ask for help. Esther and Arthur Herzfeld, the children of Holocaust survivors, are hardworking and do not complain, playing the hand they have been dealt with dignity, grace and abiding faith, Rothwachs said.“It’s literally in their blood to fight and move on,” he said.
Eight years ago, the family moved a few blocks within Teaneck to be closer to the synagogue. The move made it easier for the Herzfelds, who observe the Sabbath, to attend services on Saturdays. They have since made modifications to their modest, gray house on Schley Place that range from buying firm couch cushions so the children can sit down and get up on their own, to installing a ramp, a stair lift and, soon, another lift to help Tzvi transfer from his bed to his wheelchair. Their wish list of home improvements includes remodeling their cramped bathroom to make it more handicapped accessible.
But the cost of these renovations is compounded by the children’s required, extensive medical care. Esther Herzfeld teaches English at Ma’ayanot Yeshiva High School in Teaneck; Arthur, who used to be in the jewelry business, has been sidelined by a chronic illness that he declined to discuss in detail. The Herzfelds receive some financial support from various organizations and their synagogue, but it’s not enough. The family is at least $100,000 in debt, Esther Herzfeld said.
“We’re up to our eyeballs in debt just trying to keep things going the way they are going,” she said.
Recognizing the Herzfelds’ growing needs, a small group from the synagogue has helped the family, said Rothwachs, their rabbi, most recently by creating a fund with the Bergen County United Way to collect donations. One of Esther Herzfeld’s former students created a separate online fundraising page, and Rothwachs said both funds, together, have raised more than $50,000, all of which will go to the family.
Despite the financial hardships, Esther Herzfeld said the family does its best to take each day as it comes, but it’s often difficult. The house sometimes feels like a “pressure cooker,” she said, because of the unknown and the seemingly never-ending stress.
She said her faith and the support from the synagogue and friends have helped keep her centered through the chaos. Using an old Hebrew saying, she said one can never know what the next day will bring — both good and bad — which gives her hope that a cure will be found for her children.
Esther Herzfeld described her children as “fiercely independent,” determined, and unwilling to accept help unless it’s absolutely necessary. This summer they will celebrate three graduations: Rivka’s from Yeshiva University’s Bernard Revel Graduate School of Jewish Studies with a master’s degree in Bible studies; Tziporah’s from Yeshiva University’s Stern College for Women with a sociology degree; and Tzvi’s from Teaneck High School, with aspirations to become an attorney. Racheli, meanwhile, will be a sophomore at Ma’ayanot in the fall and dreams of becoming a writer.
“We’re down,” Rivka said with a smile. “But we’re not out.”
Want to help?
Checks may be mailed to:
- Bergen United Way, 6 Forest Ave., Paramus, NJ 07652. “The Herzfeld Family Fund” should be in the memo line or on the face of the check. Tax ID No. 22-6028959
- Congregation Beth Aaron, 950 Queen Anne Road, Teaneck, NJ 07666
