Like Mother Like Daughter
My daughter and I have a unique medical condition currently known as Mandibular Dysplasia. Our condition affects the growth of our lower jaw (or mandible), ears and also makes our airway very small. Furthermore it affects our ability to eat, swallow, and breathe safely. Over the last thirty years there have been several different approaches to caring for Mandibular Dysplasia. Medically, there have been changes in most every aspect of the care we have both received. The changes in hospitalizations, the types of family support services available, as well as surgical procedures that have developed have paved the way for a better life for my daughter. After having three normal healthy pregnancies there appeared to be no reason to subject my mother to genetic testing or the expense of a prenatal ultrasound. On May 4, 1977, I made an early and very unexpected entrance into the world at five weeks early. I spent eight months in Good Samaritan Hospital of Puyallup, WA before I was transferred for another 10 months to Children's Hospital of Seattle. At that time that I was allowed to go home for visits that lasted anywhere from a few hours to a few days. On October 24, 1998, my daughter, Nina made her way into the world about four weeks early. Nina had a much easier beginning thanks to diagnostic testing being available and in depth genetics testing that I underwent. Prior to Nina's birth we were able to be prepare for any complications and plan accordingly for the care she needed because the tests gave us warnings that she too was afflicted by Mandibular Dysplasia. Nina spent only twenty-one days in the hospital before finally coming home. I was finally released to come home long term at two years of age. In 1977 the only people who could change a tracheotomy or feeding tube had to be doctors, so having very little training and no support services my parents took me home knowing they could return at any moment. My tracheotomy tube was hard plastic and had very little flexibility. My feeding tube extended at least twelve inches outside my stomach. If either tubes became dislodged I was immediately rushed back to the closest hospital to have them surgically replaced. At that time a public health nurse would come by twice a month for less than an hour to make sure I was being cared for; however, she was neither trained, nor was she experienced with a tracheotomy or feeding tube. I slept in a mist tent which was a see through tent-like covering for my crib that added moisture with mist into the air I breathed. I was in the mist tent twenty-four hours a day. There was no such thing as respite care for my parents to have a break and it was very financially draining. If families could not pay for nurses out of their own pocket then they survived without the help. While Nina was still in Children's of Seattle I received detailed training in tracheotomy and feeding tube care. I was taught how to place both tubes in addition to how to react in several emergency situations. If either of these tubes were to become dislodged I am trained to replace them quickly and painlessly. Nina did not require to be in a mist tent but she did require the use of a mist collar that attached to her tracheotomy tube at night. In the daytime she was free to play and crawl around like other healthy babies. Nina's tracheotomy tube was made of flexible soft plastic and her feeding tube is a very small white tube that fits flush against her stomach. I also had the support of highly trained nurses who came to my home seven days a week for twelve hours each day once Nina came home. Which allowed me to rest so I could be a better parent. Nina was not readmitted to the hospital for any emergencies or illnesses thanks in part to the help from the nurses and the training I received before bringing her home. When I reached the age of six, doctors decided it was time for my first major surgery to correct the problems in my lower jaw. The only surgical option for my condition at that time was for the doctors to perform a bone graft, which is a procedure to remove a piece of bone from one part of the body and transplant it to another. The bone graft was taken from my skull and put into my jaw. After several complications from the surgery and physical therapy I went home a year later. I was left unable to speak for another six to eight weeks because the doctors had to wire my mouth shut. I was on a diet of Ensure and runny mashed potatoes the entire time. If my parents could not afford the Ensure or if there were no donations available my diet consisted of baby food and runny mashed potatoes. There was very little assistance to be found for my parents. Nina had her first surgery when she was only two years old. Fortunately for her, instead of doing a bone graft, she had a procedure called mandibular distraction. In her procedure Nina wore a halo-like device for a brief 12 weeks. The device was manufactured in a way that she would grow 1mm of new bone each day. She was able to eat a soft diet of macaroni and cheese, hot dogs, and all the cooked vegetables she could handle. Her speech did not suffer from this surgery, but if anything was improved. She spent a mere five days in the hospital for the entire process. We received as much Ensure as she could drink by mail thanks to home delivery and insurance coverage. Nina also receives assistance for her and I with lodging, transportation, and all of her meals are covered when we travel. The changes in how Mandibular Dysplasia is approached and cared for has advanced significantly through the years. The condition is still a very rare, affecting only four people in the world including Nina and me. I know that as the years come there will be even bigger changes in how doctors treat our condition. With ongoing support services for families and new research developing every day I am confident that Nina will have a brighter and healthier future than was available to me.
By Samantha DeBois.
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