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NIPF HOLDS SECOND FUNDRAISER Coincidence may have given birth to a tradition at NIPF For its second fundraising event, on November 14, the Foundation invited supporters to tour the pre-auction exhibit of contemporary art at Sotheby's New York gallery. Many of the nearly 100 visitors remembered NlPF's first event, a luncheon and tour of Top Gallant, the outdoor sculpture park in Dutchess County. On both occasions, those attending were guided through the exhibition by André Emmerich, member of the board of directors of NIPF and founder of Top Gallant, whose well known art gallery in Manhattan is now affiliated with Sotheby's. It may well be that future NIPF events will be art related, a departure which seemed to please a large group of donors who may have tired of the conventional dinner-dance format followed by many organizations. The response to invitations for the event was highly gratifying, according to Susanne Bross Emmerich, executive director of NIPF. In addition to the enthusiastic group that gathered for the event on a blustery late-fall evening, a substantial number of supporters sent donations, many in excess of the amount requested as admission to the tour. In addition, many of those at the gallery showed interest in receiving more information about IP after a brief introduction by Ms. Emmerich. The art display was in itself remarkable, with more than a dozen of the outstanding artists of our time represented by paintings, sculptures and constructions. In his walk through discussion/lecture of the show, Mr. Emmerich recalled personal anecdotes of his lifelong associations with the artists and, more importantly, placed their work in perspective within that broad category regarded as contemporary art. For many of those present, by their own comments, the lecture was an eye-opener. For NIPF, the event solidified a core of support. There were adults with IP who met other adults with IP, there were parents of children with IP who met other parents of children with IP, and for many it was the first meeting of its kind. There were also many guests whose only connection with IP is a friend whose family is affected and to whom they wish to show support. Among those present was Dr. Judith P. Willner, who chairs NIPF's Scientific Advisory Council. She is director of Clinical Genetics and Associate Professor of Human Genetics at Mount Sinai School of Medicine. Dr. Robert J. Desnick, chairman of the Department of Human Genetics at Sinai, said "awareness that so-called orphan diseases, such as IP, even though they afflict a small segment of the population, require an aggressive quest for answers. This needs public attention and support to help fund the International IP Research Consortium that is already well under way." Because a portion of the planned exhibit was not yet installed on the evening of the event, Sotheby's graciously invited those attending to a Sunday brunch two days later in order to afford them a view of the entire collection. Walter H Stern

SIBLINGS The affect of a child's disorder is not limited to the child but to all family members. A child with a disorder will have a special impact on the siblings, and, in this article that is what I would like to concentrate on. How does it feel to be the sibling with the disorder, and how does it feel to be the healthy sibling? Siblings have a unique, intimate and intense relationship with each other. They have a common environment and share parental time and love. Not surprisingly loyalty, companionship, rivalry, love and hate, jealousy and envy may all be present in varying degrees at different times. Through the forced interaction over the long years of childhood, siblings exert a powerful influence on shaping each other's identity. Siblings take on many roles with each other: mentor, supporter, comforter, protector, socializer. Roles may change with lightning speed, or a number of them may be played out simultaneously over a lifetime. As much depends upon the age, sex, religion, socio-cultural background, type and severity of the siblings disorder, the ideas presented are of necessity very general. Sibling with disorder: o Parents often will overprotect and overindulge the child with the disorder, which intensifies sibling rivalry. o The child with a disorder may become intensely envious of the normality of her sibling. o The child wonders "Why did it happen to me?" o A teenager's most common mechanism to a disorder is denial. This is usually a temporary phenomenon. Most adjust to their disorder after a while. Sibling without disorder: o Their problems may seem less weighty in comparison with the difficulties facing their siblings. Recognition of their problems may be overshadowed by the need of the parents to mask their own conflicts in relation to the child with a disorder. o The existence of two completely different sets of rules within the same family. The healthy child may become bitter over the extra favors and attention given to the afflicted child. This frequently results in feeling guilty for not having the disorder. o A number of studies of healthy siblings show the stress and resentment of the parental attention given to the sick child. They point to the healthy sibling's guilt, which arises from having escaped the disorder, guilt reinforced by the belief of somehow having brought it about. o A healthy sibling may wonder "Why did it not happen to me?" or "When will it happen to me?" As a means of dealing with these anxieties, defenses such as denial, repression may occur to alleviate the stress of the constant awareness of the sibling's disorder. Yet it would appear that the physically healthy children in these families are rarely referred for treatment.

o They may wonder, "Why do my parents let my sibling "get away" with so much? Why do I have such mixed feelings about my sibling? Am I jealous of the attention the my sibling is receiving?" o They may be concerned whether they will "catch" the disorder. o The healthy sibling may not risk overt expressions of hostility toward the ill sibling but may either internalize the distress or engage in negative attention-seeking behavior in an attempt to have some of his own needs for nurturance noticed. Parents Some suggested strategies for helping siblings are: o Be open and honest; provide straight answers to their questions. o Be fair; the child with a disorder should be treated as normally as possible, don't always take the side of the child with the disorder which will be sure to cause problems between the children. o Accept the disorder; if the parents do not accept it, it is doubtful other family members will. Value each child individually. o Let siblings settle their own differences. o Praise all siblings alike. Don't expect the sibling to be a saint. Like all people, siblings will occasionally lose their patience and understanding. Understand the boundaries of normal sibling feelings and do not expect them to behave in extraordinary ways. o Recognize that you are a powerful role model. o Recognize each child's unique qualities and family contribution. o Parents should not have unrealistic expectations of the healthy child to compensate for the parents' disappointment, guilt, or feelings of failure in relation to the child who has a disorder.   BIBLIOGRAPHY UPDATE Updated copies of the bibliography of articles, written about IP in medical journals, is available at no charge. Please mail your request to the NIPF offices.

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A LETTER TO NIPF Dear Susanne, Our trip to New York City started (by fate) on 20 Sept 1996, the day our daughter Samantha (Sam) was born. She arrived in the usual manner and all were present at her birth, a true gift form GOD. Sam appeared normal at birth. On the second day, Kristian (Kris) my wife noticed a small blister on Sam's pinky finger. She pointed it out to me and at the time I felt that it was nothing to be concerned about. On the third day this rash appeared on Sam's arms, legs, trunk and head. This rash turned into blisters and the pediatrician sought to find this rash in the dusty medical books and wasn't sure just what it was. He then sent us to a dermatologist and a biopsy was sent off. The answer appeared in three days. "Incontinentia Pigmenti", I could barely say it and I had no knowledge of what it was. Out came the dusty medical books and all the information presented just made my heart break. The books painted a scary and bleak picture. Mental retardation, baldness, blindness, missing teeth, poor growth and on it went. Inside I cried and asked GOD what I had done to him to place this on my child. I wanted to stand and shake my fist at him and fight. I have always wondered how I would or if I could handle having a "Special Child", Kris always said that GOD would provide us with all the LOVE to handle it. Me, I wasn't that positive. I work at the Fire Dept. and I have treated and cared for sick, injured and dying children and I felt that it always happened to the other family. But now, it was my child and my family. REALITY had arrived. I looked at Sam and at times I forgot that she had any medical problem (potential), but then I would recall the lab findings and what the books painted. ANGER! As time passed and Sam appeared to be doing fine, Kris had gotten on the internet seeking information about I.P. Then about 6-8 months after Sam's birth, Kris told me about the IP foundation and how she had gotten in touch with a "Susanne Emmerich" (an answer to prayers). Kris was put in touch with Mt. Sinai Medical Center's Clinical Research Center. It was then arranged for Samantha to enter the Center and be evaluated. This would include a trip to New York City and a 3-4 day stay for the tests. To get answers for our questions, I would do whatever it took (All parents know and feel this, normal response). Sam had been evaluated by a genetic Center in Florida and still they could not answer the questions. I felt that I was sitting on a time bomb and the fuse was burning and I could do nothing to help. I asked all the doctors that I had contact with but they had no answers. As Kris talked more and more with Susanne Emmerich, I felt that somebody in New York had the answers and would help us to understand IP and let us know just where Sam was in the ball game. So we started making arrangements for the trip. Susanne helped arrange for our travel and stay at the Ronald McDonald House. Now being from the South and only "Hearing " about New York City, I was not looking forward to this trip, but for SAM, nothing would stop me. I recall talking to Kris as we planned for the trip, I stated that I wonder which gun I should pack and how much ammo I needed (Sorry, but I had never been in New York and I believe in protecting family). Time passed and finally the day arrived. I told Kris that we would stay in the Ronald McDonald House and only travel to the hospital by cab and hopefully not get "Taken" by the cab driver. The flight up was fairly short and uneventful. (I left the gun and ammo at home). We arrived in New York and took a cab to the Ronald McDonald House. As we traveled into town I was thinking how many times the cab would circle the wrong block and run up a bill. The driver took us straight to the address and in a fairly short time. (My picture of New York was getting a different light) The staff at Ronald McDonald House was friendly and helped us get settled in. I asked about a dining place and the staff stated that there were a few places within a couple of blocks of RMH. So at 8:00 pm Kris, Sam and I ventured into the streets of NY. I was expecting the worst, but as we walked around, there were no muggers and bums as I expected. (I looked around as Kris was looking up at the buildings. (Tourist Syndrome) and enjoyed a great meal, conversation with the owner and a couple of other dinner guests. They made a fuss over Samantha and delighted us about NY. (Boy this wasn't what I expected).

The next day we went to Mt. Sinai and met the staff. They made us feel welcome and were really concerned about our welfare and Sam's. They got Kris and Samantha settled into their room and all the time, the staff fussed over Sam. Kim, one of the nurses, really took special care and concern about Sam. The staff as best as I can recall was as follows, Kim, Gayle, Florence, Mydia, Paulett, Dr. Willner, Dr. Banikazemi and Connie (EEG Dept). Please forgive me if I over-looked anyone. These people are pure gold and they all made our stay great. As Sam went into the evaluation phase, the nursing staff told us that they would monitor the evaluation and that they were there mainly on her behalf to make sure she was not stressed. I can tell you from my working at the Trauma Center, that a caring staff as this is worth its weight in gold and this meant a lot to us. Each department was caring and kind in it's duties and I have never met a person like Connie in EEG. She is a champ. Our stay in NY was getting better by the minute. We went into Central Park across from the hospital and it was beautiful. There we talked to a few people walking their dogs and Sam had a blast playing with the dogs after being inside for the first two days. Each night I went back to RMH and stayed while Kris stayed at Mt. Sinai. The hospital security guards came to know me (the accent). They were friendly and asked about Sam when I showed up in the morning at 6:30. The concept about just staying in RMH and only going out to the hospital, went up in flames after the first day. I felt like a total fool about the preconceived notions that I had about NY. People you have a great city (I know that it's not perfect, but where is'?). Sam was released on Thursday morning and we only had half a day to see NY. We visited the Zoo, Central Park, FAO Schwartz Toy store, walked down 5th Avenue to the museum and visited it for a couple of hours. You cannot see NYC in half a day. We found a small diner a couple of blocks from RMH, (Blue star or Silver Spoon, I think was the name) and found some good down home cooking there. During dinner we met the couple across from us and started talking about NYC. As we talked, they told us about Broadway shows and they gave us a brief history lesson about the islands that make up NYC. They made us feel welcome in their city. We left Friday morning and saw the beautiful Statue Of Liberty as we flew over. It felt good to be an American! Now I have told you about our trip, but I would like to talk to the next family, that has a child with IP and is planning to come to New York. Please let any preconceived thoughts about NYC flee from you. For I can tell you that all you will find in NY is kind, caring, loving, professional and concerned people. These people will put all concerns to ease and provide you with current and accurate answers to your questions. Susanne Emmerich is a person that one stands back and admires for all she has done in the area of IP. She is a true Blessing and answer to prayers. We left NY blessed, informed and at ease about our daughter and IP. Information is the best defense and weapon that one can have. So pack your bags, get on the aircraft and relax. New York is a great place and the folks are real friendly. We plan to take 7 -8 days in April next year when we bring Sam (and her 6 year old brother) back for follow-up evaluation and see the NEW YORK CITY that is REAL. I know now why New York is "My kind of Town". Sam is 19 months old and she is a beautiful growing girl in love with "BARNEY and I thank GOD for his gifts and the people at Mt. Sinai. I apologize for the misconception about New York. I tell everyone that they need to visit NYC and explore the gifts of the city. If a Southern Boy as I can walk about the city and feel safe, then anyone else can and I mean it from my heart. Susanne and the staff at Mt. Sinai, THANK YOU and may GOD keep each one of you close and protected in his loving arms. Sincerely; Robert, Kristian, Seth & Samantha   NEED FOR CONTRIBUTIONS AND FUNDING NIPF is grateful to its supporters for their ongoing generosity. Several people have taken the opportunity to make a gift in honor of a deceased friend or loved one, or sent in a contribution to celebrate a special occasion such as a birthday, anniversary, graduation, etc. When such a contribution is made a letter is sent to the family being so honored, acknowledging the contribution. These donations help NIPF continue its valuable work the services it provides, as well as funding the expenses of the International IP Research Consortium. Please keep in mind that whatever the reason for the contribution, it is always fully tax deductible.

MORTON F. GOLDBERG, M.D. (Director, Department of Ophthalmology, Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine; Member of the NIPF Scientific Advisory Council) While the search for the gene that causes IP must go on, the fight against its most serious symptoms may have arrived at a crossroads in the opinion of Dr. Morton Falk Goldberg. Though the name Incontinentia Pigmenti implies irregularities of skin coloration, the dermatological focus may need to be de-emphasized in favor of concentration on the more serious effects on vision and brain function, he says. Dr. Goldberg, one of the nation's leading ophthalmologists, is Ophthalmologist-in-Chief at the Johns Hopkins Hospital in Baltimore, MD and, over the years has held an impressive number of chairs at various universities and hospitals. He also serves on the Scientific Advisory Council of the IP Foundation. He is the author or co-author of seven books and more than 300 articles in professional and scientific publications, including writings about IP. His interest in IP was piqued some years ago when, in collaboration with Dr. David Valle, of the Pediatrics Department of Johns Hopkins, he made the discovery that there are ophthalmological similarities between IP and sickle-cell anemia, a malfunction that afflicts solely patients of African descent. Specifically, Dr. Goldberg discovered a pattern of blood vessel abnormalities in both disorders by the use of fluorescein angiography, a photographic test of the retinas. This finding was significant, he says because this closure of the blood vessels leading to the retina can affect the vision and brain function of both IP and sickle-cell patients. Unless this malfunction is addressed within days of birth, the defect might never be corrected in later life, he warns. Though not apparent to most laymen, the retina is embryologically a part of the brain. Similarly, teeth are closely related to the skin embryologically. This, Dr. Goldberg believes, may be the reason why IP frequently manifests itself in one or all of these four areas -- brain function and eyesight, as well as skin pigmentation and dental deformities. In his practice, Dr. Goldberg sees a good many IP patients, some of them referred to him by NIPF. And while the genealogical search for the gene holds the ultimate key to eliminating or understanding the disorder, he feels it is essential to pursue an urgent program for helping those already born with IP. He acknowledges that the earliest diagnosis is usually made by pediatric dermatologists when confronted with an infant who develops persistent skin rashes. It is at this point, however, that the intervention of an ophthalmologist becomes critical because both vision and brain function must be protected before damage is irreversible. "Without making light of it, one can go through life more or less normally by dealing with skin discoloration through cosmetics and malformed teeth with dental appliances," he says, "so long as normal vision and unimpaired brain function are assured by counteracting the closure of the blood vessels that threaten the retina and the brain." Dr. Goldberg obtained his A.B. degree magna cum laude in biology at Harvard College in 1958 and his medical degree, cum laude, at Harvard Medical School in 1962. He served his internship at Peter Bent Brigham Hospital in Boston and his residency at the Wilmer Ophthalmological Institute of Johns Hopkins Hospital. From these beginnings, his fame in the field has spread consistently and he is a sought-after lecturer and author. Of his two sons, the younger, now 21, will be attending medical school while the 25-year-old practices environmental law. When out of harness, Dr. Goldberg and his wife, Myrna, enjoy travel, contemporary art and especially scuba-diving in the Caribbean.   The information provided in our newsletter should not be substituted for personal, professional advice. It is our intention to keep you informed and ask you to always check any treatment with your physician.

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