Youngster doing well living
with genetic disease HLH
By jeanette M. eng
Staff Writer
MARLBORO — As rare as hemo-phagocytic lymphohistiocytosis (HLH) is, Shirley Zhang, 6, of Marlboro, who died in January, was not alone in her fate, even in the township. In 2002, the News Transcript profiled the story of Marc Weinstein, 4, and Ross Weinstein, 2, of Marlboro, who were diagnosed with familial [genetic] HLH.
After receiving the diagnosis on their sons, Barry and Cindy Weinstein’s lives were put on hold as they dealt with the task of traveling back and forth between their home and a hospital in Cincinnati, Ohio, large medical bills and the news that even following successful treatment, Marc and Ross would have to be separated for up to three years to avoid any chance of infecting each other.
Ross received a bone marrow transplant on March 15, 2002, but suffered complications which required another operation on May 7. On the morning of May 30, at Cincinnati Children’s Hospital, Ross’s battle ended as his little body could fight no more.
"What we wouldn’t give to have another day with Ross," Barry Weinstein said this week, adding that he and Cindy think of their little boy every day. "But we can’t focus on Ross right now. We have to focus on Marc."
Marc has not exhibited symptoms as serious as his brother and is currently living a normal and healthy life, according to Weinstein.
"He’s a normal 5-year-old who just celebrated his birthday," Weinstein said.
Although Marc is fine now, he is far from being in the clear. He is still carrying HLH, although it has not been triggered. The two major triggers are the chicken pox and the Epstein-Barr virus. If Marc is ever exposed to either virus, the HLH would be triggered.
"We constantly have to be wary of who’s around him, and we have to shield him a little bit," Weinstein said. "It’s something we have to live with every day."
The couple, however, remain optimistic and believe there are positive messages in the events which have occurred.
"We thoroughly enjoy Marc, and there’s not a moment we’re with him that we don’t smile inside," Weinstein said. "The fact that we don’t have a crystal ball and we don’t know what’s going to happen makes us live in the present even more."
The Weinsteins also cannot express enough gratitude for the community that supported them in their time of need. Because of that support, they have been able to stay in the community, and they were afforded their time with Ross and time to grieve for Ross, Weinstein said.
Unfortunately, the story of the Weinsteins and the Zhangs is neither well-known or common. HLH is an orphan disease, or a very rare disease which doesn’t get much attention, Weinstein said. Thus, the treatment is not perfected and the knowledge of the disease is limited.
"I feel like I’m alone on an island with this," Cindy Weinstein said.
Ever since HLH came into their lives, Cindy Weinstein has become as active as time has afforded her. She reads every HLH-related article she can find. She meets with specialists everywhere. She attends meetings of the Histiocyte Society and she meets with other families affected by HLH. The fact remains, however, that this disease is very much a mystery.
"There are times when I meet with specialists, and they don’t know much more than me," she said. "That’s really frightening."
According to Weinstein, constant visits to a Histiocyte Society Internet chat room have connected her to many other families who are also dealing with this disease. With new families coming up every day, Weinstein does not believe that HLH is as rare as "one in a million," as it is referred to. Recent research has also revealed evidence of a third form of HLH, she said. Weinstein believes that more research would uncover additional forms of HLH.
"Unfortunately, the money goes to the diseases that affect the most people," Weinstein said, noting that the little research that is done on HLH is mostly funded through the Histiocyte Society or private funding rather than government grants.
Weinstein hopes to witness increased awareness, activism, funding and thus research for HLH. She believes that more research will reveal how comprehensive this disease is, provoking more research. More awareness will lend itself in detection of HLH, too, as doctors and parents will be more inclined to recognize the symptoms.
"I hope that this disease will attract people who are genuinely interested in research for the sake of research," Weinstein said. "There are so many young hematologists and oncologists and immunologists out there now in school who could devote themselves to HLH research."
The various levels of research that remain untouched are overwhelming, according to Weinstein. As of now, there exists no genetic research, which would lead to knowledge of prenatal testing for HLH, knowledge on the origin of HLH and a specific protocol to follow once HLH is diagnosed.
Weinstein now works full time and cares for Marc with her husband. They struggle for control of the disease which has changed their lives.
"We continue to pray on a daily basis," Weinstein said.
For more information visit the Histiocyte Society Web site http://www.histio.org/society. Based out of Pitman, Gloucester County, this nonprofit organization is comprised of an international group of more than 200 physicians and scientists.
Since its inception in 1985, the society has committed itself to improving the lives of patients with histiocytosis by conducting clinical and laboratory research into the causes and treatment of this disease. Member-ship is open to all those wishing to pursue these goals.
The Histiocyte Society will hold its 19th annual conference in Philadelphia Sept. 18-20. The conference will feature specialists on all histiocyte disorders from around the world.