Dr. Kopits hadn’t always been a specialist in dwarfism. However, because Johns Hopkins had such a large patient population with genetic illnesses, he found himself working with many genetic-based orthopedic problems, and his passion soon became the skeletal deformities of dwarfism. He had told me that it was evident to him that this was a unique patient population with unique issues, which no one had yet taken an interest in caring for.
Dr. Kopits was certifiably a hero, a miracle maker, to each and every Little Person who had the pleasure of being his patient. In fact, to those whom I know who were blessed enough to see him, he was infinitely more than a physician. We all considered him a beloved friend and a true member of our families.
According to my mother, that first trip to Johns Hopkins Hospital and the Moore Clinic was much more traumatic for her than for me. She had absolutely no idea what to expect, and two weeks seemed like forever, so she was in a whirlwind of fear. On the one hand, she was relieved to have gotten a diagnosis for me, but she was equally overwhelmed by that diagnosis. One of the genetic specialists gave her an informational pamphlet about the size of a comic book that was filled with black-and-white photos of different people with my condition. The picture pages showed the severe orthopedic complications and deformities of children and adults. It was enough to scare her to death.
Dr. Kopits, a miracle worker to parents as well as patients, assured my mother that I would be well taken care of and that my prognosis was fairly good, but the list of surgeries he thought I would need throughout my life was something no parent wanted to hear. It was clear it wasn’t going to be easy.
My first surgery was about a year later, when I was two. It was probably the biggest surgery I’d ever have, a cervical spine fusion on account of the instability in my spine. I had to have my top two vertebrae, C1 and C2, fused together. These two are the highest on the spine, right behind the skull, and when they are as unstable as mine were, any significant fall or jolt to one’s head or back could cause paralysis, or worse. The fusion essentially turned those two vertebrae into a solid piece. However, it also meant I would have limited mobility in turning my head from side to side and flexing up and down.
The fusion was difficult, and I was under the knife for at least nine hours while my parents anxiously paced back and forth in the designated waiting area outside the operating room. The surgery involved taking bone from my hip to use as the “glue” for the fusion itself. Then, my spine was carefully manipulated and aligned to Dr. Kopits’s satisfaction. Next, he secured my head and neck by placing me in a halo. A halo is a contraption that holds your neck stable after surgery while you heal. It involved four metal screws bolted into the four corners of my skull affixed with a circular metal ring around my head—where the name halo comes from—with bars that went down from the halo and attached to a belt-like fixture anchored at my waist.
I guess I looked pretty beaten up when I came out of the operating room. I was in the halo and was admitted to the Pediatric Critical Care Unit. Even though all this had been explained to my mother beforehand, and she thought she knew what to expect, she still fainted when she first got sight of me in the recovery room. This would turn out to be a recurrent thing for my mom, so much so that Dr. Kopits started carrying smelling salts every time he met my mom in the recovery room.
My mom had to take me home in the halo to heal for about twelve weeks, which wasn’t easy for her first experience of surgery for me. She made the best of it. She said carrying me around was easy because the halo device functioned like an oversized handle, and I was so light. She could grab me by the bar anchored to the belt at my waist.
After I was discharged from the hospital, my mother and I flew home to Orlando where my aunt Barbara would help me recover. However, I have come to realize that things don’t always go smoothly, or as you hope, in these situations. I had come down with some sort of bad cold, which in itself wasn’t the problem. The crisis began when my mom was at work, and my dad and Papa, who was visiting from St. Petersburg, were at home with me and Dad tried to give me a dose of cold medicine. This was when giving children over-the-counter cold medicines was recommended by pediatricians, and I got so upset about the taste of it that I started thrashing my legs around and kicked so hard I literally forced out the front two screws in my skull that held the halo in place.
My mother got a desperate call from my dad to get home immediately, if not faster. When she busted through the front door and into the living room, she found my dad and Papa standing rock-stiff on either side of me, looking terrified, not daring to move a muscle as they held my head in place.
“What the heck is holding her head going to do? We need to secure her head!” my mom yelled as she ran to grab towels to stabilize my head within the halo and then to the phone to call 911.
My first memory ever is of riding in the back of the ambulance that came for me. The lights were flashing, and my parents were tucking towels around my head, trying to make sure I was secured for the trip to the hospital. Only one of them could go with me, so my dad offered to go, and my mom followed in her car. Once the back door closed and the ambulance started to move, I remember my dad singing me rhyming songs he made up on the spot to distract me while holding a tiny stuffed lion he brought with us. I still remember the words—“the flying lion stops the crying.” He sang it all the way to the hospital in an effort to calm me down.
My dad was always the creative one, with a great imagination, whether or not there was an emergency. He loved creating stories and tales to make me feel better, and his plan usually worked. The “flying lion,” made famous in the ambulance, became so special that my dad would later use him as a character in stories that became a full series. To this day, I still remember the plot lines.
My mom was always the tough one, always getting the job done. Their roles were complementary and equally important. My mom was already anxiously pacing the emergency room of Orlando Regional Medical Center when the paramedics raced me in on the stretcher. An adult orthopedic surgeon, Dr. Johnson, met us there. At that time, he had never seen this type of halo before, let alone a head halo that was displaced and semidangling. He and his staff began trying to communicate with Dr. Kopits’s office at Johns Hopkins Hospital. When they were told he was in South America and not reachable (this was the era before cell phones), the situation became increasingly intense. I was admitted to the hospital while my entire family, the office in Baltimore, and Dr. Johnson tried to reach Dr. Kopits to get instructions on how to replace the halo. Dr. Johnson didn’t want to just react without speaking to Dr. Kopits. However, because of the time passing and concern for injury to my spinal cord if I moved, he offered to attempt to replace the halo.
However, my parents really wanted to wait for Dr. Kopits, confident somebody would reach him. The last thing they wanted was for someone not familiar with the halo to mess things up and paralyze me forever. They had been warned that this was a possibility. They figured as long as I was in the hospital, secured, and monitored closely, I was safe.
Four days after I was admitted, there was still no word from Dr. Kopits. Dr. Johnson announced he and his team were going to have to take me into surgery to try to reset the halo. As terrified as my parents were, they agreed to go ahead.
Just as the surgical team was prepping me for the OR, into the waiting area walked Dr. Kopits! My mother and father practically fainted at the sight of him, breaking down in tears and thanking God for a miracle.
According to Dr. Kopits, intuition told him he needed to call his office, and when he followed his instinct and checked in with the office in Baltimore, he heard what was going on with me in Florida. He went straight to the airport, caught the next flight to Miami, and then drove the three hours to Orlando at ninety miles an hour in a rental car. What made the story even more exceptional was that he was in South America to receive an honor for his orthopedic work back where he had trained, and he left right in the middle of the dinner to take care of me before he could eve
n receive his award.
After he arrived at the hospital, Dr. Kopits spoke briefly with Dr. Johnson, and soon he was scrubbed and ready to accompany him into the OR. The whole thing made my mom a firm believer in Dr. Kopits’s dedication to his patients. With Dr. Kopits’s direction, the halo was resecured. As he left, Dr. Kopits hugged both my parents and said to my mom, “Little mother, I am going to teach you how to care for the screws so this doesn’t happen again.” Dr. Kopits taught my mom how to tighten the screws in my halo, which had to be done every couple of days, and we never had another “halo emergency” again. Soon, I was back at home for the rest of my recovery.
The most unbelievable part of that whole story is that Dr. Kopits never charged my parents for any of it. I later heard of other stories about Dr. Kopits traveling across the country to care for one of his patients, and always without charge.
The spinal fusion was just the first of many surgeries I would have. It would be followed by numerous osteotomies on my hips, knees, and ankles. These osteotomies were corrective surgical procedures in which bones were broken in order to realign my deformities. Without these surgeries, my bones would likely become so deformed that I would be wheelchair-bound. My first osteotomy was on my hip when I was three years old. My primary nurse at this surgery was named Donna, and she became one of our closest friends. At each yearly checkup in Baltimore with Dr. Kopits, he would tell me if this would be a year for one surgery or two, as he would often do two at once. Of most concern to him were my knock knees and the fact that my femurs were growing at different rates, causing severe deformities that, if not corrected, might not be fixable down the line and would make me unable to walk. My knock-knee legs made it extremely difficult for me to keep up. Walking long distances was difficult, so I’d have to use a stroller, wheelchair, or when I was old enough, a scooter for distance. At school I had the aid of some device for distance for as long as I can remember. At Disney World, I may have gotten to jump the line in a wheelchair, but of course would have preferred to walk with everybody else.
CHAPTER TWO
Bill
It’s a Boy!
MY MOTHER AND FATHER, William Walter Klein, Sr., and Barbara Jane Diecidue, were both from Long Island, New York. They grew up only a few miles from each other, as my father was from Levittown, a five-minute drive from Mom’s hometown of North Massapequa. They met for the first time when my mother was in her senior year of high school, and my father, three years older, was home on leave from the army. He had been stationed at a base in Alaska.
They started their relationship as pen pals. A friend of my mother’s was “pen palling” with my father, which was a common thing to do with soldiers back then, and she thought my mother would enjoy it, too. Mom didn’t know it, but this friend, a former girlfriend of Dad’s, suspected the two might be a good match.
My parents’ first in-person date was actually when my father was home on Long Island for a twenty-one-day leave before being shipped to Southeast Asia for a tour of duty in Vietnam. By then, he and my mother had already been “dating by mail” for one year. The story goes that when Dad came to Mom’s house to take her out, Mom’s younger sister, Lori, answered the door. According to Mom, Dad probably thought that Lori was his date and was surprised when his real date, the little preppy schoolgirl named Barbara (my mother), popped up behind her.
Dad volunteered to serve in Vietnam, and in 1970 he was deployed to serve one tour of duty. Shortly after arriving, he volunteered to serve as a door gunner on a UH-1H helicopter and was immediately assigned to an aviation detachment stationed in Phu Bai. Afterward, his unit was relocated to an airbase in Da Nang. When not flying, Dad and other members of his unit visited orphanages in hopes of helping some of the kids displaced by the war. I think that is what my father is most proud of. It speaks to both his character and the character of the other servicemen who served with him in Vietnam. Like most veterans, my dad never really speaks about Vietnam or the war. I know he was (and still is) proud to have served our country.
I was born on October 13, 1974, at Mid Island Hospital (now St. Joseph’s) in Bethpage, Long Island. The doctors knew immediately that something was wrong.
To begin with, I was cyanotic, a blue baby, which meant that I had some sort of heart malformation that prevented my blood from being fully oxygenated. I was in respiratory distress and had to be provided with oxygen in order to breathe. But there was also something really wrong with my proportions. I was seventeen inches long, fairly normal, but the concern was I was all head and torso, with very short appendages, so there was definitely something not right.
My parents were actually taller than average. My father was exactly six feet, when the average height for men at that time was five-eight. In fact, my mother was five-eight, four inches taller than the average woman. There was tall stature in the genetic pools of both their families as well. My mother’s brother is six-four, and an uncle on Dad’s side is six-two.
It has been speculated that my father’s exposure to Agent Orange while serving in Vietnam was a contributing factor to my short stature. The exposure, which can result in flu-like symptoms among other things, is now known to denature the sperm’s DNA, causing random mutations. But they don’t know for sure if what happened was a mutation associated with exposure to Agent Orange or if it was a true random genetic mutation.
The delivery took a long time. At one point the doctor sent my father home and promised to call when I was closer to making my debut. Just as my father was arriving at my maternal grandparents’ house, the phone rang. A nurse from the hospital said the doctor wanted my father to know I had been born, but that something was terribly wrong. He added to “not rush.” Not rush?! My father drove at breakneck speed from North Massapequa back to Bethpage in less than five minutes, a ride that normally took double that. The doctor who had delivered me had already disappeared. According to my father, he was so seemingly nervous or unable to negotiate the terms of my novel condition that he never returned, ever. None of us ever saw him again.
Dad demanded to see me as soon as he got to the hospital. The baby nurse in the nursery held me up so he could see me through the glass window. He was in shock when he realized the severity of my condition. As the day progressed, the doctors and nurses were at a loss for words. But wanting to help in some way, they moved my mom to a private room, something that was unheard of then. With Mom not yet knowing exactly what was going on with me, Dad made the decision to decline the private room, fearing it would cause her undue stress. He also knew that this decision would dictate how my life would be, and he didn’t want me to be segregated because of other people’s prejudices, even if their intentions were good.
Nobody knew how my mother would handle the news, so for a couple of days, the medical team taking care of me avoided bringing me to her. Because of the difficult delivery, she was heavily medicated, and they told her I was still getting treatment. Finally, three days after I was born, my mother absolutely demanded to see me, so a nurse on the unit got up enough nerve to carry me in, hand me over, and run out of the room.
My mother’s sister was a nurse in the hospital, but even she didn’t know exactly what was going on. She knew the buzz that it was bad and everybody was upset, but other than that, my condition was still a mystery. I was baptized many times over just in case I didn’t make it.
I can’t fathom the fear my mother must have had. Her first baby, her little bundle of pride and joy, had arrived, and everyone on the medical staff was scratching his or her head, bewildered. You can almost imagine my mom, in her room agonizing, scared, wondering, and waiting, the door closed to the conversation of the doctors going on in the hall. The secrecy made it even more difficult, as the doctors seemed to be mumbling things to each other, but not to her. My parents only had each other.
At one point, the pediatrician came in to talk to my mother. She told Mom she didn’t know what was wrong with me; as far as she could tell, I only presented as “short.”
“He’s a dwarf, isn’t he?” my mother blurted out, which caused the pediatrician to almost fall over in disbelief. Mom knew of somebody whose child had growth issues, and doctors were considering dwarfism. Otherwise, she probably never would have come up with this idea, but the pediatrician was in no way ready to agree.
My breathing issues continued for at least four days, so finally the decision was made to send me to the NICU at Nassau County Medical Center in East Meadow, now called Nassau University Medical Center. The neonatologist from that unit who had been assigned to my case came with the NICU transport to collect me. I was lying in the isolette that I would be transferred in, and he wheeled me over to my mother and told her she could say good-bye to me now, if she wanted.
“No, I am not saying good-bye to him,” she responded with defiance. “This is ‘hello,’ and I will see him later.” She said that after she gave the specialist this correction, she and I made complete eye contact, which is very unusual for a newborn. With my big blue eyes, I told her, “I am not going anywhere, Mom. I’ll see you soon.”
Not long after my departure in the ambulance, Mom was also discharged and my parents were by the side of my isolette within an hour. They kept vigil in the NICU for hours every single day for the next three weeks. There was one doctor on the unit who said he thought I had a form of dwarfism called achondroplasia, because of my very large head and very short arms and legs. This was the most common form of dwarfism, characterized by short limbs and a large head with a prominent forehead. Apparently, if you stretched out my arms and lifted them up, they didn’t reach the middle of my head.
Having a baby in critical care without a diagnosis was extremely hard on my parents. They had two or three relatives and five or six friends who were having babies at the same time they were, and those babies were all healthy and thriving. In time, they learned there was a better shot at getting hit by lightning three times over than being born with my condition, but at the moment, they only had questions without answers. It was almost a miracle that I made it.
Life Is Short (No Pun Intended) Page 2
