Facing Challenges
By Jocie Lyon
After attending this year’s PSF conference, Kim asked me if I would share my experience as an adult who has Proteus Syndrome. I jumped at the opportunity because I have been meaning to do this for quite some time. I became truly in- spired after meeting and talking with so many wonderful people in Maryland in October.
My life began 34 years ago at Children’s Hospital in San Francisco. After two days of labor, my poor Mom had a cesarean section and I finally said hello to the world. I made a spectacularly shocking entrance. I guess you could say I’ve always been defiant and full of surprises. I was born with four huge fingers: index, middle and ring on my right hand and thumb on the left and my right hand and arm were also about twice the size of my left. Two specialists were asked to evaluate me immediately: hand specialist Robert Brown, MD and plastic/reconstructive surgeon, Milton Edgerton, MD. They both determined that my symptoms were most consistent with neurofibromatosis (Proteus Syndrome had not yet been identified in 1972). So, they spoke with my parents and formed an action plan to try experimental surgery in order to make my right hand as functional as possible and to decrease the size of my arm and shoulder. This meant that my Mom and I would get to travel to Charlottesville, Virginia once a year to see Dr. Edgerton at the University of Virginia Medical Center. Mom always scheduled extra time either before or after surgery so that we could explore the area. I did a lot of griping at the time but now that I’m older, I’m glad she did that. It distracted me from thinking about surgery. As a bonus, I got to freak out my Dad and Gramma with my newly acquired Virginia style southern accent.
At 13, I had undergone 11 surgeries. At that point, my family and I decided that my hand was as functional as it was going to be and my right arm and shoulder were significantly smaller. The de-bulking, growth plate removals, nerve grafts, etc. had accomplished what needed to be done. I was not interested in making my hand look more esthetically pleasing so, we moved on.
Unfortunately, 4 years later, I ended up having emergency surgery. Small cysts had been growing on my ovaries for quite some time. The only thing that told me something was going on was a trip to the emergency room with pain shooting through my abdomen. It was the worst pain I ever felt. Turned out, my right ovary was so consumed with cysts that it had grown to the size of a grapefruit. The weight of the mass had caused the entire thing to prolapse. In the end, everything turned out just fine, minus the right ovary. The left ovary was not as bad so they were able to remove most of the cysts and keep it intact. I still continue to have regular ultrasounds to make sure nothing else is happening. On a personal note, I would like to take this opportunity to strongly encourage all parents of female Proteus patients or the patients themselves to please speak with their doctors about this part of the disease. If it is at all possible to avoid going through what I did – for heaven’s sake, please do it.
The doctors felt that I should have a genetic evaluation to determine if the ovarian cysts had anything to do with what had happened to my hand. I had the evaluation done and was diagnosed with Proteus Syndrome. At that time, all any- one seemed to know was that it was extremely rare, involved overgrowth of bone and tissue and that the continuous rapid growth ceased in adolescence. That was a big relief because in the beginning we were told that neurofibromatosis symptoms can continue indefinitely in most cases.
It was about 8 years ago that I was told about the Proteus Syndrome Foundation and Kim Hoag. It was a life changing experience. I was so happy to know that there were others out there just like me – and within reach! I immediately wrote to Kim who sent me all kinds of information about Proteus and the research being conducted at NIH. Next thing I knew I was on my way to Bethesda for my evaluation. That’s when I met Dr. Biesecker and his staff and learned what they had discovered so far. Shortly thereafter my Dad and I attended our first PSF conference. Words cannot ex- press the overwhelming feelings I had meeting all of the families involved. There was great strength, happiness, sad- ness and a common bond we all share. I still feel that way whenever I get a newsletter, check in on the PSF website or get an email from someone within the organization.
Having researched everything I possibly can on Proteus Syndrome, I believe that I have weathered the storm. I have done that by staying positive and healthy (well, I still love my chocolate). I see a chiropractor and message therapist regularly. Because my right arm is longer and still a bit heavier than my left, I tend to feel uncomfortable at times. I used to get lots of headaches caused by muscle knots in my neck. So, I have become a strong believer and advocate for chiropractics, acupuncture and message. Traditional medicine is still important but these alternative treatments really work for me.
I am not very religious but I have to say that I am truly blessed with so many wonderful people in my life. I am eternally grateful to my parents, Ray and Sharon Lyon. Coping with the challenges of having a child born with Proteus syndrome is a very difficult thing. My Mom and Dad were always involved, supportive and tough when they needed to be. My “Gramma” was another big part of my life. She passed away several years ago but I still think of her often. She was the glue of our family and the most amazing woman (besides my Mom) that I have ever known. My best friend, Lisa Newby and I met 25 years ago in the 5th grade. You know how girls like to talk on the phone every day and it drives everyone crazy? We still do! I am so fortunate to have her in my life. Then of course there is the love of my life, Jim Lippert. We met on-line 2 1⁄2 years ago. Our relationship blossomed and now we are getting married in February. Jim has two boys, Ben (12) and Patrick (15). I wasn’t sure how they would react to me so I was a bit nervous about meeting them at first. They immediately put my mind at ease and thought I was pretty cool (or maybe it was the brownies I brought). The feeling is mutual. Ben and Patrick are amazing young men and I am very lucky to know them.
In conclusion, I believe the key to overcoming the obstacles that Proteus Syndrome can put in front of us is to stay posi- tive and rely on your families, friends and PSF contacts for support and encouragement. I have always faced challenges head on. Nothing is more important than believing that you can do anything. And if you fail, at least you tried.
Thank you Kim, Barbara, Mary and everyone at PSF for giving me this opportunity.
After attending this year’s PSF conference, Kim asked me if I would share my experience as an adult who has Proteus Syndrome. I jumped at the opportunity because I have been meaning to do this for quite some time. I became truly in- spired after meeting and talking with so many wonderful people in Maryland in October.
My life began 34 years ago at Children’s Hospital in San Francisco. After two days of labor, my poor Mom had a cesarean section and I finally said hello to the world. I made a spectacularly shocking entrance. I guess you could say I’ve always been defiant and full of surprises. I was born with four huge fingers: index, middle and ring on my right hand and thumb on the left and my right hand and arm were also about twice the size of my left. Two specialists were asked to evaluate me immediately: hand specialist Robert Brown, MD and plastic/reconstructive surgeon, Milton Edgerton, MD. They both determined that my symptoms were most consistent with neurofibromatosis (Proteus Syndrome had not yet been identified in 1972). So, they spoke with my parents and formed an action plan to try experimental surgery in order to make my right hand as functional as possible and to decrease the size of my arm and shoulder. This meant that my Mom and I would get to travel to Charlottesville, Virginia once a year to see Dr. Edgerton at the University of Virginia Medical Center. Mom always scheduled extra time either before or after surgery so that we could explore the area. I did a lot of griping at the time but now that I’m older, I’m glad she did that. It distracted me from thinking about surgery. As a bonus, I got to freak out my Dad and Gramma with my newly acquired Virginia style southern accent.
At 13, I had undergone 11 surgeries. At that point, my family and I decided that my hand was as functional as it was going to be and my right arm and shoulder were significantly smaller. The de-bulking, growth plate removals, nerve grafts, etc. had accomplished what needed to be done. I was not interested in making my hand look more esthetically pleasing so, we moved on.
Unfortunately, 4 years later, I ended up having emergency surgery. Small cysts had been growing on my ovaries for quite some time. The only thing that told me something was going on was a trip to the emergency room with pain shooting through my abdomen. It was the worst pain I ever felt. Turned out, my right ovary was so consumed with cysts that it had grown to the size of a grapefruit. The weight of the mass had caused the entire thing to prolapse. In the end, everything turned out just fine, minus the right ovary. The left ovary was not as bad so they were able to remove most of the cysts and keep it intact. I still continue to have regular ultrasounds to make sure nothing else is happening. On a personal note, I would like to take this opportunity to strongly encourage all parents of female Proteus patients or the patients themselves to please speak with their doctors about this part of the disease. If it is at all possible to avoid going through what I did – for heaven’s sake, please do it.
The doctors felt that I should have a genetic evaluation to determine if the ovarian cysts had anything to do with what had happened to my hand. I had the evaluation done and was diagnosed with Proteus Syndrome. At that time, all any- one seemed to know was that it was extremely rare, involved overgrowth of bone and tissue and that the continuous rapid growth ceased in adolescence. That was a big relief because in the beginning we were told that neurofibromatosis symptoms can continue indefinitely in most cases.
It was about 8 years ago that I was told about the Proteus Syndrome Foundation and Kim Hoag. It was a life changing experience. I was so happy to know that there were others out there just like me – and within reach! I immediately wrote to Kim who sent me all kinds of information about Proteus and the research being conducted at NIH. Next thing I knew I was on my way to Bethesda for my evaluation. That’s when I met Dr. Biesecker and his staff and learned what they had discovered so far. Shortly thereafter my Dad and I attended our first PSF conference. Words cannot ex- press the overwhelming feelings I had meeting all of the families involved. There was great strength, happiness, sad- ness and a common bond we all share. I still feel that way whenever I get a newsletter, check in on the PSF website or get an email from someone within the organization.
Having researched everything I possibly can on Proteus Syndrome, I believe that I have weathered the storm. I have done that by staying positive and healthy (well, I still love my chocolate). I see a chiropractor and message therapist regularly. Because my right arm is longer and still a bit heavier than my left, I tend to feel uncomfortable at times. I used to get lots of headaches caused by muscle knots in my neck. So, I have become a strong believer and advocate for chiropractics, acupuncture and message. Traditional medicine is still important but these alternative treatments really work for me.
I am not very religious but I have to say that I am truly blessed with so many wonderful people in my life. I am eternally grateful to my parents, Ray and Sharon Lyon. Coping with the challenges of having a child born with Proteus syndrome is a very difficult thing. My Mom and Dad were always involved, supportive and tough when they needed to be. My “Gramma” was another big part of my life. She passed away several years ago but I still think of her often. She was the glue of our family and the most amazing woman (besides my Mom) that I have ever known. My best friend, Lisa Newby and I met 25 years ago in the 5th grade. You know how girls like to talk on the phone every day and it drives everyone crazy? We still do! I am so fortunate to have her in my life. Then of course there is the love of my life, Jim Lippert. We met on-line 2 1⁄2 years ago. Our relationship blossomed and now we are getting married in February. Jim has two boys, Ben (12) and Patrick (15). I wasn’t sure how they would react to me so I was a bit nervous about meeting them at first. They immediately put my mind at ease and thought I was pretty cool (or maybe it was the brownies I brought). The feeling is mutual. Ben and Patrick are amazing young men and I am very lucky to know them.
In conclusion, I believe the key to overcoming the obstacles that Proteus Syndrome can put in front of us is to stay posi- tive and rely on your families, friends and PSF contacts for support and encouragement. I have always faced challenges head on. Nothing is more important than believing that you can do anything. And if you fail, at least you tried.
Thank you Kim, Barbara, Mary and everyone at PSF for giving me this opportunity.
