This story is about Lindsay Stern. Her parents have been instrumental in being a voice of RRP, research, support system and lead the RRP Foundation. Long before Facebook or Twitter or really the internet as we know it today, they were leading the way….fighting not just for their own daughter but for each one of us that fights RRP daily. They are truly RRP Heroes.
Lindsay Stern’s RRP Story
[The following is reprinted from an article that appeared in the Spring 1996 issue of the RRP Newsletter plus updates to the present.]
We adopted Lindsay from Korea when she was 13 months old. From the first day that she was with us we noticed that she had a low cry and a faint deep voice. We often mentioned this to our pediatrician, who said that we were “lucky” that we didn’t have a baby with an ear piercing scream. She seemed generally happy and healthy, except for an occasional cough, croup, and constant ear infections. Shortly before her second birthday she seemed to be breathing loudly and then began snoring loudly when sleeping. After more than a month involving many visits to the pediatrician, diagnoses ranging from post-nasal-drip to asthma to croup and 3 weeks of prescribed ventolin and prednisone treatment, we were referred to a local otolaryngologist, Dr. Stephen Farmer, in Princeton. After looking into Lindsay’s larynx with a scope, he sat us down and told us what he knew about this disease that we (and our pediatrician) had never heard of. He referred us to Mount Sinai Medical Center for emergency admission that evening, November 14, 1991. Lindsay was scoped again and the resident otolaryngologist suggested the possibility of a tracheotomy (at this point we barely knew what a trach was, let alone the implications when it is used in conjunction with RRP). The next day Dr. Peter Catalano performed a laser surgery, thankfully, he was able to avoid a tracheotomy, despite the fact that she was nearly 99% obstructed ( We remembered the embattled and exhausted look on the face of the anesthesiologist after coming out of surgery. Lindsay was the most severely obstructed two year old they had ever seen!) She spent 9 days at Mount Sinai with a second laser surgery 6 days after the first.
We had many questions and only received a few answers. No one seemed to know much about RRP, only that it is rare. Were we all alone with this disease? What type of life did our little girl face and how many more surgeries will she have to endure? Will she live to go to college, get married and raise a family? Is there anyone doing any research to try to find a cure? No one knew of any support group. We scoured the Mount Sinai medical library for information. The little bit of literature about RRP that we found, told us that it was virtually certain to reoccur for some time. We very much needed to reach out and talk with others in similar situations. It was this deep need for emotional support that motivated us to seek others and develop a support network of RRP families.
We began the process of reaching out to others by contacting the otolaryngology departments of major medical centers in the Northeast U.S. In our search we found out that Children’s Hospital of Philadelphia (CHOP) had a significant RRP patient population and the otolaryngology department clinical nurse, Linda Miller, provided a wealth of information (and if it wasn’t for Linda’s support and guidance, we might not be coping today). Over the next 6 months Lindsay had 7 more surgeries, all at CHOP.
During this brief but aggressive period we felt our emotions wax and wane as this disease went through its unexplained ups and downs. For example, Lindsay’s 5th surgery since diagnosis occurred on February 13, 1992, it was her best to date. Her surgeon, Dr. William Potsic, told us that he found only a small amount of papilloma on the false and true cords (using the Kashima scoring system, my estimate was a score of 2, where 0 is none and 27 is total coverage and obstruction). He lifted our spirits by predicting that we should be able to extend the surgical interval to at least 5 weeks. Well, this fickle, uncaring disease gave us a significant emotional blow, when less than 3 weeks later Lindsay began snoring loudly and her nightime breathing had become distressed (i.e., early stage stridor). Unfortunately, this was a Friday night, which meant an emergency room admission. In anticipation of some difficulties, we video-taped Lindsay’s breathing while she slept that night. On Saturday morning we went to the CHOP emergency room and, as we feared, were presented with difficulties. The emergency room doctors didn’t really have a clue regarding RRP, saying that she might have a touch of bronchitis and were going to send us home with ventolin. We insisted on having the otolaryngology resident on call examine her. He also was about to release her, not finding any significant respiratory problem in his exam. But she was awake now, and as parents of an RRP patient we knew that respiratory problems from papilloma (in young children) always appear more noticeably at night while asleep. We insisted that he look at this video of her sleeping the night before. Reluctantly, he agreed, and 20 minutes later he came back saying that she was obstructed and would be admitted. Our difficulties in this situation did have some positive impact on emergency admission policy for RRP patients at CHOP. Based on our experience, Dr. Potsic, who very much believes, that parents are the best judge of when their kids with RRP are ready for surgery, instituted a policy that a child with RRP will be admitted and surgery performed on their parent’s say so.
Lindsay continued in an aggressive mode, requiring surgeries 7 and 8 over the next 5 weeks – we were starting to consider adjunct treatments, such as acyclovir or interferon. About a week after her eigth surgery, we received a call from Linda Miller. She had just spoken to some one from Long Island Jewish Medical Center (LIJ) and during the conversation a study involving indole-3-carbinol (I3C) and RRP was mentioned in passing. We quickly pursued this by contacting the LIJ department of Otolaryngology and were put in touch with the researcher, Dr. Karen Auborn. Dr. Auborn told us of encouraging results with a mouse study and suggested cruciferous vegetables as a source of I3C. She estimated that about 8 ounces of cabbage (or about 4 ounces of cabbage juice) might provide a sufficient amount of I3C to induce a shift in estrogen metabolism so as to suppress papilloma growth (see the RRP Newsletters Fall 93 through Fall 94, http://www.rrpf.org/newsletter, as well as Newfield et al., 1993, Anticanc Res 13:337-342). Lindsay’s 9th surgery occurred several weeks after initiation of a daily regimen of between 4 and 6 ounces of cabbage juice (The juice was made palatable by mixing approximately 2 oz. of cabbage juice with 5 oz. of apple juice.) In contrast to the 8th surgery, which (like most of the prior operations) involved laser excision of a large amount of papillomas from multiple sites, the ninth surgery revealed a very small amount of papilloma which could easily be removed with a microlaryngeal forceps.
Lindsay continued the cabbage juice regimen and had two more visual examinations of her respiratory tract under general anesthesia at which no papilloma were found. The first was at the end of June 1992, 6 weeks after her ninth surgery and then again at the end of September 1992, shortly before her third birthday. At the beginning of 1994, when it was learned that we could obtain I3C in powdered form we gradually modified her sources of I3C to include approximately 190 mg of powdered I3C daily and reduced her cabbage juice intake to about 2 ounces per day. As of the Spring of 1996, when Lindsay was 6 1/2 years of age, she continued with this therapy and remained free of symptoms since her last RRP surgery in September 1993.
We are most thankful for Lindsay’s apparent remission and wish the same for all RRP patients. It is our firm belief that I3C played a key role and this appears to born out by the accounts of some others in the support group as well as the clinical work of Dr. Clark Rosen and Dr. Karen Auborn.
A few years later a better form of I3C became available, so we switched Lindsay’s adjunctive therapy to BioResponse DIM (http://bioresponse.com/Home.asp). Now in the fall of 2014, about 18 ½ years since our original Newsletter article about Lindsay, we are happy to report that she remains in symptomatic remission. We are extremely fortunate that Lindsay has been able to lead a very normal and active life. She was a competitive swimmer into college. These years have not been without episodes of hoarseness causing us concern that active RRP might be returning. However, scopings have not revealed any recurrence of papillomas. Lindsay does seem more sensitive to voice abuse and did have a number of tonsillar infections including one incidence of a tonsillar abscess that led to a tonsillectomy at the age of 19.
We know that I3C / DIM does not work for everybody, but during the more than two decades that we have been involved with RRP we have seen significant progress in other treatment approaches. In our opinion a remaining major challenge is to produce an effective treatment for pulmonary RRP, which affects a small percentage of RRP patients, but transforms an annoying disease into a life threatening one. One of the RRP Foundation’s main initiatives is to motivate the RRP research community to develop new approaches to produce effective treatments for aggressive, deeper, generally inoperative RRP. Data suggests that lung involvement impacts about 5% – 7% of RRP patients, but it does represent at least 95% of the mortality associated with this disease. In addition, treatments that prove to be effective in these aggressive RRP cases should also be effective at other sites in the respiratory tract.
This article would not be complete without noting that without the inspiration of a number of RRP practitioners and researchers, i.e., Tom Broker, PhD, Bettie Steinberg, PhD and Haskins Kashima, MD, we would not have been motivated to actively seek information, network and organize. In particular, we are indebted to an article in the New York Times early in 1992 which put us in contact with Dr. Tom Broker . Tom literally spent hours on the phone with us. His enthusiastic support for developing a close working relationship between RRP researchers and patients was critical in directing our efforts and creating the RRP Foundation (www.rrpf.org).