... this is an excerpt from my website
She is not your average High School cheerleader, this patient of ours. Sure, she's blond, tall, beautiful. One of the more popular girls in her school in Indiana. One of the brightest, too.
But this cheerleader can't whistle. She can barely raise her arms over her head, which limits the number of cheers she can perform. She cannot help lift the other girls. She isn't strong enough for some of the pyramids.
Mary Jo* has a genetic disorder called Facioscapulohumeral Muscular Dystrophy. It is a rare disorder, with prevalence of 1 in 20,000 people. It is also autosomal dominant, meaning that it will be passed along to one half of the children of affected persons. Unlike other forms of Muscular Dystrophy, people with this disorder have a normal life expectancy.
The muscles in the face and the shoulder girdle do not function well. Affected persons have hollow cheeks. They also have drooped shoulders and are unable to raise their arms with any strength. They also have scapular winging, which happens since the shoulder blade is untethered to the underlying ribcage. Until gene therapy provides a more definitive solution, many patients opt for surgery to create a stable shoulder girdle.
Six years ago, Mary Jo arrives at the Mayo Clinic with her large family. I am a first year resident on service with the Chairman of my department, a pioneer in shoulder surgery. It is my second month working as a doctor.
Mary will have a seemingly simple yet alarmingly complex surgery called a scapulo-thoracic arthrodesis. We will use metal cables and metal plates and screws to fix the shoulder blade to the ribcage in hopes that the bones will heal together as a fracture does. This will create stability in the shoulder girdle, improving her strength as well as her appearance.
The dangers here are quite real. When the cables are passed, they must skim underneath the ribs, over the surface of the lung. A millimeter or two too deep, and the lung is punctured. The plates and screws must traverse past major blood vessels. If one of these vessels is nicked, our patient could lose a large percentage of her blood volume in a matter of seconds.
After surgery, Mary Jo will be in a body cast with her arm held away from her body. She will be held like this for six weeks, after which she will use a brace for another six weeks. She will not be able to use that arm. For this reason, we can only operate on one side at a time.
Mary Jo is anxious, but brave. She looks forward to looking more "normal." She can't wait to be able raise her arm above her head. I wheel her on a cart into our operating room, making silly jokes. She squeezes my hand when the anesthesiologist starts her IV. I continue to hold her hand as the medicines are delivered through that IV and she is made unconscious.
Incision. Our dissection to the bone is immaculate. The cables are passed; the plate is fixed. We close. We mold the body cast before she awakens.
She is in the hospital for a week, during which time we discuss her dreams and goals. She tells me about her family, her church. She teases me about my ending up marrying a dairy farmer from the Midwest. Her casted arm akimbo, we enjoy an awkward hug on the day she leaves the hospital. She is exhausted and happy.
The next time I see Mary Jo, it is a year later. I am no longer on service with my Chairman. It is the nature of a residency program that we move around to experience all aspects of our specialty, and I was with another mentor. But she asks the nurses to call me. She will undergo surgery on the other side and she wants for me to be there.
Over the course of my residency, we see one another two more times. Recovery from her second surgery was rocky and the fixation needed to be redone. Fortunately, the second attempt is successful.
My final personal encounter with Mary Jo is when she returns to have all of the cables and plates removed from her back. This time, five years after her first surgery, I am met by a confident young woman. She now holds a Bachelor's Degree. She is married to a wonderful man from her church community. They have a son. And she can lift him above her head.
**Addendum** I received email from Mary Jo and her family last week. They have another son, who is now three months old. In her email, she wishes me luck in my private practice in New York City (my hometown), and asks if I have met any nice dairy farmers.