Featured Blog: Wow, That Girl Is Tiny! What Is Russell-Silver Syndrome?

List: Posted: 01/13/11

As an infant, I was diagnosed with Russell-Silver Syndrome, a rare growth disorder most prominently characterized by body asymmetry. Basically, everything on my left side is smaller than everything on my right side. For example, there is about a 1.5 shoe size difference between my feet. Kids with RSS seldom make it over the 5ft. mark, and I am no exception at 4’10”. And, as is typical of those with this syndrome, I experienced a lot of physical and health problems growing up, as well as major self-image issues – things I eventually had to deal with and overcome.


When I was born, I was considered to be what is known as Small for Gestational Age. As a full-term baby weighing only 4lbs 5oz, I went straight into the incubator for three extra weeks. Right away, there seemed to be a few unusual things happening with me. My head seemed too large for my tiny body. My left leg and arm were smaller and shorter than the right ones. I had a big red ‘café-au-lait’ spot across my broad forehead, which seemed a contrast to my very small chin. Other things seemed unusual too. I had curved, short pinkie fingers. Low-slung, prominent ears. Hardly any muscle. All typical of RSS, but the disorder was so little known that nobody knew if – or how – to diagnose me. And they didn’t until I was about 15 months.


At one year of age, I had only doubled my birth weight to 9lbs 10oz, which is not far off from what most children weigh when they’re born. Because I had such poor muscle mass, my tiny legs could not support me in walking until I was 14 months old. When my younger brother was born, I was a year and a half old, and he was comically close to my size. My feet were so small that when I finally did start walking, I could just barely fit the smallest size 1 booties sold in stores – which many children are never small enough for, even at birth.


But still, I showed almost no interest in food and was hardly growing. At my 8-month mark, doctors were ready to put me in the hospital with an IV to force me to take in extra nutrition and calories. My worried parents had tried every type of formula, baby food and healthy ground-up regular food they could think of. Several days before I was to be admitted to the hospital, my exasperated doctor asked if there was anything, anything at all that I would eat – my mom mentioned that I liked ice cream. And as this famous family story goes, she said, “Great! I don’t care if she eats ice-cream or nothing but SpaghettiO’s, give it to her!” and after that, my two favorite foods were ice-cream and SpaghettiO’s for years and years, and not a whole lot else. But it kept me out of the hospital. And at least I was eating and starting to grow.


Because my left leg was an inch shorter, I wore a lift on my left shoe to balance my legs and my back. Even as a small child, I was aware that I was different from the other kids, and it caused me to become extremely shy. I was always the smallest kid in my class by far, and so as it often goes, I was picked on by some of the bigger kids. I also had a high-pitched voice, which is another frequent characteristic of RSS. It all amounted to a brutal lack of self-confidence.


With advice from my doctor (one of the only specialists in the state who was familiar with my disorder), my parents encouraged me to find the muscle-and confidence-building activities I liked. So I tried a great number of sports and hobbies, including: soccer, ballet, ice-skating, swimming, piano, karate, skiing, community theater, Girl Scouts and gymnastics. I wasn’t very good at most of these because of my small size and poor strength, but I always had fun anyway and it did help with my self-confidence and encouraged me emerge from my shell a bit.


At age nine, I started taking horseback riding lessons. I rode horses competitively for more than 10 years after that, which really improved the strength in my legs and I found it didn’t make a difference that my left side was smaller. I also discovered I had a knack for writing and I loved to write stories. In high school, while I was great at English and Art, I had a hard time concentrating on subjects in school that didn’t interest me (again, I didn’t know I had A.D.D.), so I had to study harder than my peers to do well, sometimes even using a tutor to keep my grades up.


By the time I got to college, a lot of the physical traits typical of kids with RSS were mellowed out. My ears didn’t seem to stick out as much, my 5 years’ worth of braces were gone, my small side had gained muscle and didn’t seem quite so small, and my voice wasn’t so high-pitched. However, during high school, I had decided that I didn’t want to wear a lift on my shoes anymore, so I bought boots and padded them. But it wasn’t enough padding and before long, my back became crooked with scoliosis. At a routine check-up when I was 19, my doctor announced that the scoliosis was critically bad and something had to be done immediately – the very next week I had leg-lengthening surgery.


There are several ways to do the surgery, which was developed during WWII to aid people with particularly complicated fractures and breaks. The procedure that seemed best for me went like this: they put me out, and the surgeon broke my left femur about 2/3’s of the way up. He then inserted large pins from the outside of my leg into the top and bottom of my femur, three at each end. On the outside of my leg, he attached what looked a bit like a long pipe which functioned as a very big screw to the pins in my leg, so that it ran the length of the bone.


After two days in the hospital and a month of recovery, it was time to start lengthening my leg – I was shown how to turn the giant screw a quarter turn every day. Let me tell you, it is a very odd sensation (luckily not too painful) to feel your bone separating! Because my left leg was shorter by an inch, that’s how far the bone had to be parted. And then it was up to my bones to fill in the gap.


I had the brace in for a total of 13 months. To be honest, it wasn’t much fun. But after a few weeks I returned to school - with a wheelchair to begin with, then crutches for a while and then eventually I was able to walk on my own while bone gradually filled the gap. I even returned to my part-time job. With some grueling physical therapy, a necessary evil, I made a full recovery – the brace was taken out and my legs are now completely even and my back is straight. I can wear regular shoes, and even high heels, though I have to buy them at a special website because my feet are still very small (I wear a size 2 on the left and a 3.5 on the right).


I finished college with Bachelor’s Degrees in Advertising and English and also earned an Associate’s Degree in Photography. I now work as a marketing writer, living in San Francisco, California. I write plays and stories in my free time, and I practice photography often, for fun. I live a very normal, happy life and no one ever suspects the trials and tribulations I went through when I was growing up. I have gained a belief in myself that there is nothing stopping me from doing the things I want to do.


I’m writing this story because I’d like to think it could provide some hope to kids and families dealing with rare diseases or syndromes like mine – have faith, kids can overcome almost anything. Sometimes it seems like a never-ending process, and it can be very, very difficult for kids – or anyone, for that matter – to feel that they are somehow different and that things might never get easier. But they do. And when they do, people can go on to truly thrive... I am proof.


Kirsten Smith, RARE Blog Contributor

 

-          To read the full blog and for more info on Russell-Silver Syndrome, visit www.crdnetwork.org/blog/

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