by: Ken Cover
Molly is a typical 9-year-old girl. She loves to sing, dance, draw, and paint. She loves the outdoors, playing with her friends, and riding her bike. Her dreams are big, as is her heart. She aspires to be a “rock star/doctor,” performing life-saving surgeries by day and thrilling thousands of adoring fans in sold-out stadiums by night. Oh, and she also wants to be a cheerleader in college, with all the spare time she’s going to have while preparing for medical school. She is kind above all, considerate and caring, and careful not to offend or hurt the feelings of others. She is sweet and sensitive, sometimes overly dramatic, quick to forgive, and wants everybody to like her.
She is also an amputee. This minor, niggling detail does not define her. She refuses to resign herself to being “that girl with the fake leg.” I’ll relate an anecdote here: One Saturday morning, Molly and I were having breakfast, talking about the latest shenanigans of her classmates in school, the new Taylor Swift video, or something equally mundane. Molly looked thoughtful and went silent for a second, almost certainly trying to think of some new direction for our conversation. She asked, “Are you happy I was born?” I answered, “Of course, I’m happy you were born. You are very precious to me. I’m sorry you’ve had to struggle with all these problems, though.” Molly is Molly. She replied, “Problems? What problems?” I said, in turn, “You know. With your leg and all that.” As usual, she enlightened me. She casually said, “I don’t have any problems. I can do whatever I want.” Amazing. Yes, Molly is Molly!
Molly has Neurofibromatosis Type 1, as does her mother, her brother, her aunt, her grandmother, her cousin, and other more distant family members. NF 1 is a quite common, yet much misunderstood, genetic disorder. No, it’s not “Elephant Man’s Disease.” That’s something else entirely. No, it’s not cancer. The fact that so many people, if they happen to have heard of NF 1, believe these misconceptions is indicative of just how much it is misunderstood. I don’t pretend to know everything about NF 1, or its more heinous relative, NF 2. I am no geneticist. Here is what I do know: it’s a very common single-gene disorder that affects a lot of people. The symptoms are many and varied, but it commonly causes fibrous benign tumors, called “fibromas,” usually on nerve cells, and “café-au-lait” spots on the skin. It can cause other growths as well, and is often linked to learning disabilities and developmental delays, as well. These things we knew when Connie became pregnant with Molly.
Molly is Connie’s second child. Her first pregnancy, with Molly’s older brother, Jackson, was fraught with difficulties, including toxemia and preeclampsia, and resulting in an emergency C-section. On the other hand, however, her pregnancy with Molly was uneventful: Connie was healthy throughout, and Molly was a full-term baby. Her birth went according to schedule, and was also a C-section. Now, previously I mentioned what we knew about NF 1 before Molly was born. After her birth, we became aware of another, rarer possible effect of NF 1. It seems that around one in ten children with this disorder is born with a fibroma on bone tissue. This variation of fibroma is a lesion which makes the bone spongy, and prevents it from growing and developing normally; these almost always occur on a large bone, such as the tibia, and almost always on a single location on a single bone. This condition is called “tibial pseudarthrosis.” Molly was unlucky enough to be a member of this ten percent.
The deformity of her leg was subtle, and went unnoticed in ultrasounds. No doctors or nurses noticed it after birth, either. I was giving her a bath when she was a few days old, and noticed that her lower left leg seemed to be curved below the knee. At her next doctor’s appointment, I pointed out the curve to her pediatrician, who at first thought it might be a “positional” deformity that occurred in the womb. To affirm this idea, she consulted with another doctor in the clinic. After examining Molly and consulting her chart, the second doctor said he believed it was almost certainly related to her Neurofibromatosis. We were scheduled to visit a local orthopedic doctor, who told us he thought that amputation was likely the best course of action, but our pediatrician insisted that it was too soon just to “give up” on the leg like that. She scheduled a visit with a well-respected pediatric orthopedic surgeon at Arkansas Children’s Hospital in Little Rock. We went home to wait.
Anyone with children understands the waiting game when your child is referred to a specialist, particularly when her condition is not life-threatening, and will know that weeks and weeks went by between the time I saw the curve in Molly’s leg, and when we were to see the surgeon in Little Rock. Molly was a typical baby. She giggled, she cried, she ate and she spit up. She loved bath time; she loved her big brother, who could always make her laugh out loud; and she loved her “bouncy chair.” One evening, she was strapped into her bouncy chair, wearing her footie jammies, bouncing away. Suddenly, she started crying, and was inconsolable. We held her, we cooed, offered her a bottle—everything we could think of, but the crying wouldn’t stop. She was running a slight fever and pulling at her ear, so we thought maybe she was getting an ear infection.
We decided to take her to the emergency room, since she seemed to be so uncomfortable that we figured it might be something worse. The ER doctor couldn’t find anything wrong with her, but said one of her ears did look a bit red, so proceeded as if it were an ear infection. They gave her a couple of shots and sent us on our way, with instructions to follow up with our pediatrician. There followed a sleepless night, and we called our pediatrician the next morning.
We have often commented to each other how lucky we were to have the doctor we did for Molly. She insisted on our bringing Molly in right away and working us in between appointments. She looked little Molly over, who was now about eight or ten weeks old, and said, “She doesn’t have an ear infection. I’m going to send her to radiology. I’m pretty sure her leg is broken.” Sure enough, that’s what it was. Apparently, while she was kicking and bouncing happily in her bouncy chair, her toe caught on the elastic band in the ankle of her footie jammies, and the abnormal bone in her tibia simply tore apart. That was when we learned that when Neurofibromatosis causes such a lesion on a bone, that bone will almost always spontaneously break during the first few weeks of life. This was another surprise to us; we thought we had researched and become knowledgeable about NF 1. How little we did know, after all! I don’t really remember who it was, but there was a doctor somewhere along the line that told us that many doctors don’t know much about it. This doctor said that he or she had spent maybe a half-hour on NF during medical school.
This day, which had started off as just a normal day with a trip to the doctor for a supposed ear infection, rapidly devolved into a nightmare. Molly and Connie left by ambulance for Little Rock that afternoon, while I collected Molly’s older brother from school and followed, about an hour or so behind, in our car. The ER at Children’s Hospital was chaotic, as should be expected. There was some confusion and we had to explain more than once that we had not, in fact, broken Molly’s leg. A colleague of the surgeon we were meant to see was on call for the ER, and once Molly’s leg was put in a cast, he was going to release her and have us come back at some future date to see the surgeon. I told him that it was my understanding that due to the tibial psuedarthrosis, and the break being related to that, the whole reason for the frantic run to Little Rock had been specifically to see this guy, presumably for emergency surgery. Thankfully, he called the surgeon at home after re-reading Molly’s chart and notes from her pediatrician. We were then given instructions to find a place to spend the night and come back first thing in the morning.
The following morning, we met with the surgeon. We were prepared for a longer stay, assuming there would be a surgery, if not that day, then within a day or so. The surgeon told us her bones weren’t big enough to operate on. She would have to do some growing before the surgery. And, the broken tibia would almost certainly not knit together and heal properly since the tissues at the break were abnormal. As it turned out, she was almost two years old before having her first surgery. In the meantime, she was fitted with a clamshell brace.
Her surgery consisted of having approximately an inch of “bad bone” removed, with a pin, a plate, and some screws joining the 2 sections of remaining tibia. Then, Molly wore a succession of braces as she grew, and she learned to walk with a walker, and subsequently learned to walk without it. About a year after that first surgery, her tibia separated again at the same location. The bone just never knit together properly. For her next surgery, they took a graft from her hip bone and used this, along with the plate and screws, to help encourage growth. This worked with moderate success; the bone fused, thanks to the graft, but growth was slow. With her left leg already shorter after having bone removed, her right leg outpaced the growth of her left. The length difference was increasing, and we knew that somewhere in her future lay the painful and protracted lengthening process, which would involve actual stretching of the tibia.
Molly, still in a brace, continued with life as usual. School, playing with neighborhood kids, reading, TV, and video games occupied her time, like any other kid. She learned to ride a bike, and was always on the go. Visits with her orthotist for new braces or adjustments to an existing brace were commonplace, and exposed her to photos and stories involving amputees achieving great things with their prosthetics. Molly started asking why she couldn’t just get a new leg. Wouldn’t that be better than this one? Wouldn’t she be able to run? Dance better? Maybe even skip? So, we started talking about amputation, first with Molly, making sure she understood that it mean another surgery, some hard work, and probably a good deal of pain… but it might mean fewer surgeries in the long run. And, yes, it might make it easier for her to do some things that she couldn’t do with her weaker, shorter leg and brace. Molly decided quickly that she wanted an amputation and a new leg. She announced to her teacher and classmates that she was “getting her leg chopped off and getting a new one.” One of the teachers at her school arranged a meeting with a Razorback cheerleader who also happened to be an amputee, and who happened to show up at Molly’s school with a TV news crew. I contacted her surgeon with questions and asked his opinion concerning Molly’s wishes, but he already knew of her decision, since she had been on television as “the brave little Rogers girl facing amputation.” This was attention we had not been seeking, but there it was, and Molly really enjoyed her fame. Soon thereafter, we scheduled the surgery. Her leg was amputated below the knee in July of 2015, when Molly was seven years old. She was supposed to be in a wheelchair until September, when she took delivery of her first prosthetic, but it was awfully hard to keep her in it. She preferred hopping on one leg to riding in the chair, and as always, she was fiercely independent, whether in the chair or not.
She adjusted to her prosthetic with that easy grace that only children seem to possess; her prosthetist said it’s always easier for kids than for adults. Of course, kids keep growing, so they also need new prosthetics as time goes on. She is now on her third prosthetic. Molly continues being the strong, capable person she is. She recently achieved a long-sought-after goal, and learned to run properly, with both feet off the ground in mid-stride, something she probably never would have been able to do in a brace. Molly has taught me more about strength and perseverance than I ever thought possible. Molly was right. She doesn’t have any problems. She can do anything she wants.
Molly… is Molly.