Being a stay-home parent can be tedious. Most of my days are spent cooking, cleaning and in car pool lines. Not a great deal of social interaction or intellectual stimulation. And, then, bam! something happens to Amanda and my world is rocked off its axis.
I can look at these events in two ways. First I feel badly for my daughter. I hate that she seems to have an endless series of medical problems. I wish that she had a typical life. I mourn for the 14 year old who should be telling me off and talking on the phone all day - not spending 6-8 days a month at the doctor's office or hospital. A month in our lives is different than most folks, that's for sure!However, when my little pity party is over I take a deep breath and embrace the challenge.
This is one of these weird examples where being the mother to a child with special needs suits me. Sure, I get tired and frustrated with everything Amanda has to go through. But to be completely honest, I get a sense of satisfaction from the research and planning involved in coordinating her medical care.
My informal medical training began before she was born. At my second ultrasound the doctor said grimly, "it looks like Tetralogy of Fallot" with no other explanation. As soon as I got home from the appointment I grabbed the Merck Manual and looked it up. Ever since then I've made an effort to learn as much as I can about what is being discussed in regard to Amanda's disabilities and proposed treatment. I don't want to subject her to any procedure unnecessarily. My first question is typically,"how will this improve the quality of her life?" If it won't make a significant difference to her health or happiness, I won't agree to the procedure.
Over the years I've mastered a great deal of medical terminology. I've had to - at this point Amanda has twenty-two separate diagnoses. We started with cardiac terms and moved into neurological disorders. By now I've got a lot of orthopedic lingo down.... Amanda has had scoliosis, pronated feet and ankles and (my favorite new word) brachymetatarsia. Brachymetatarsia means that one of the five long bones of the foot (the metatarsals) is abnormally short, resulting in a shortened toe. Last year I had to learn all about hip surgery and liability law when she broke her hip on school property.
This has been our craniofacial year. Craniofacial issues concern "the skull, face and jaws". Amanda has what her physicians have always referred to as "facial anomalies" due to her genetic syndrome. Her ears are low on her head, her upper jaw is narrow, her lower jaw is small and recessed and she has a "beak-like" nose. She tends to stick her tongue out. We never worried about these things because we considered them superficial. Now, however, complications are arising from her facial structure.
The roof of her mouth (palate) is high and narrow. It makes it hard for her to chew and eat. The small palate that worked fine for an infant also now obstructs her breathing. It prevents her from being able to retract her tongue into her mouth, which creates another set of issues. Naturally there is an incomprehensible medical term for everything except a high palate - for instance, a large tongue is called macroglossia. First she will have a surgically assisted rapid palate expansion and an uvulopalatopharyngoplasty (no wonder they shorten that to "UPPP"!)
A plastic surgeon will cut the roof of her mouth and an orthodontist will insert a palate expander opened to its maximum width. At the same time the surgeon will remove her tonsils and some of the back of her palate that is blocking her airway. I think sometimes doctors and surgeons forget that patients and/or parents aren't familiar with the procedures they describe. I read online that UPPP typically requires three to five procedures. That is something I wasn't told and need to ask about at our next visit. Another reason it's important for me to do my homework.
And, if that isn't enough she has micrognathia (small chin) which caused endless feeding problems as an infant. She aspirated (breathed in) food and had an abnormal, delayed swallow pattern. She narrowly avoided having a feeding tube. Mainly because I objected. The doctors were surprised. They said that most people were relieved to be offered a G-tube and be done with the feeding issues. Not me. I wanted her to eat real food. To me eating is a big part of socialization. It's important to be able to eat a holiday meal with your family, have pizza with friends, go through a drive-thru on the way home. I had what I called "the Happy Meal goal." I simply wanted to be able to take her to McDonald's. A G-tube would not have (in my opinion) improved her quality of life. At this point the structure of her chin is also contributing to sleep apnea and other breathing problems. Correcting her lower jaw is now important to her health.
Her lower jaw will have to be expanded so that her teeth meet once her upper jaw is expanded. I need to ask how the surgeon plans to extend her jaw - with a bone graft from somewhere else on her body or with an appliance. Hopefully after all of that her tongue will fit into her mouth without flopping back and blocking her airway. If not, then she will have to have tongue reduction surgery (ouch!) called a partial glossectomy.
She'll also have to have several ear surgeries as her hearing is deteriorating. Her low-set ears have narrow, downward sloping canals. This structure has resulted in chronic ear infections. Doctors have tried to insert ear tubes twice, but the extremely narrow canals made it impossible. Her ear drums have spontaneously ruptured several times. She has a very high pain tolerance - sometimes we didn't know she had an ear infection until we saw blood in her ears. As a result her ear drums have retracted causing "tumors" (called cholesteatomas) to develop. Looking that up was fun --- I hate when articles end with statements like "if untreated, deafness, brain abscess, meningitis, and, rarely, death can occur." I guess I have to agree to that surgery!
Unfortunately her narrow (stenotic) ear canals prevent easy removal of these cholesteatomas and her surgeon casually informed me that they would have to be "drilled" before the tumors could be removed. Well, that sounded painful and disgusting. I would have preferred the technical term, but I can't find one. I did discover that the surgery to removed the cholesteatomas is called either a intact canal wall down or canal wall up mastoidectomy, depending on the approach.
All of my new knowledge is the result of a meeting with the doctors at the Craniofacial Clinic at Children's Healthcare in Atlanta. One visit and they had pulled together an impressive treatment plan, involving three surgeons and a dentist. Those facial anomalies that I considered superficial are impacting her health and need to be surgically corrected. The current plan is for her to undergo anywhere from four to eight surgeries over the next few years.
Again, I can look at this in two ways. I feel terrible that Amanda has to go through all of this. I detest the idea of surgery. I hate the chaos it causes for the entire family. On a positive note, I'll have a lot more things to learn as we progress.
I can look at these events in two ways. First I feel badly for my daughter. I hate that she seems to have an endless series of medical problems. I wish that she had a typical life. I mourn for the 14 year old who should be telling me off and talking on the phone all day - not spending 6-8 days a month at the doctor's office or hospital. A month in our lives is different than most folks, that's for sure!However, when my little pity party is over I take a deep breath and embrace the challenge.
This is one of these weird examples where being the mother to a child with special needs suits me. Sure, I get tired and frustrated with everything Amanda has to go through. But to be completely honest, I get a sense of satisfaction from the research and planning involved in coordinating her medical care.
My informal medical training began before she was born. At my second ultrasound the doctor said grimly, "it looks like Tetralogy of Fallot" with no other explanation. As soon as I got home from the appointment I grabbed the Merck Manual and looked it up. Ever since then I've made an effort to learn as much as I can about what is being discussed in regard to Amanda's disabilities and proposed treatment. I don't want to subject her to any procedure unnecessarily. My first question is typically,"how will this improve the quality of her life?" If it won't make a significant difference to her health or happiness, I won't agree to the procedure.
Over the years I've mastered a great deal of medical terminology. I've had to - at this point Amanda has twenty-two separate diagnoses. We started with cardiac terms and moved into neurological disorders. By now I've got a lot of orthopedic lingo down.... Amanda has had scoliosis, pronated feet and ankles and (my favorite new word) brachymetatarsia. Brachymetatarsia means that one of the five long bones of the foot (the metatarsals) is abnormally short, resulting in a shortened toe. Last year I had to learn all about hip surgery and liability law when she broke her hip on school property.
This has been our craniofacial year. Craniofacial issues concern "the skull, face and jaws". Amanda has what her physicians have always referred to as "facial anomalies" due to her genetic syndrome. Her ears are low on her head, her upper jaw is narrow, her lower jaw is small and recessed and she has a "beak-like" nose. She tends to stick her tongue out. We never worried about these things because we considered them superficial. Now, however, complications are arising from her facial structure.
The roof of her mouth (palate) is high and narrow. It makes it hard for her to chew and eat. The small palate that worked fine for an infant also now obstructs her breathing. It prevents her from being able to retract her tongue into her mouth, which creates another set of issues. Naturally there is an incomprehensible medical term for everything except a high palate - for instance, a large tongue is called macroglossia. First she will have a surgically assisted rapid palate expansion and an uvulopalatopharyngoplasty (no wonder they shorten that to "UPPP"!)
A plastic surgeon will cut the roof of her mouth and an orthodontist will insert a palate expander opened to its maximum width. At the same time the surgeon will remove her tonsils and some of the back of her palate that is blocking her airway. I think sometimes doctors and surgeons forget that patients and/or parents aren't familiar with the procedures they describe. I read online that UPPP typically requires three to five procedures. That is something I wasn't told and need to ask about at our next visit. Another reason it's important for me to do my homework.
And, if that isn't enough she has micrognathia (small chin) which caused endless feeding problems as an infant. She aspirated (breathed in) food and had an abnormal, delayed swallow pattern. She narrowly avoided having a feeding tube. Mainly because I objected. The doctors were surprised. They said that most people were relieved to be offered a G-tube and be done with the feeding issues. Not me. I wanted her to eat real food. To me eating is a big part of socialization. It's important to be able to eat a holiday meal with your family, have pizza with friends, go through a drive-thru on the way home. I had what I called "the Happy Meal goal." I simply wanted to be able to take her to McDonald's. A G-tube would not have (in my opinion) improved her quality of life. At this point the structure of her chin is also contributing to sleep apnea and other breathing problems. Correcting her lower jaw is now important to her health.
Her lower jaw will have to be expanded so that her teeth meet once her upper jaw is expanded. I need to ask how the surgeon plans to extend her jaw - with a bone graft from somewhere else on her body or with an appliance. Hopefully after all of that her tongue will fit into her mouth without flopping back and blocking her airway. If not, then she will have to have tongue reduction surgery (ouch!) called a partial glossectomy.
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| You can tell from these photos that the thing that most improved Amanda's quality of life was her IPad! |
Unfortunately her narrow (stenotic) ear canals prevent easy removal of these cholesteatomas and her surgeon casually informed me that they would have to be "drilled" before the tumors could be removed. Well, that sounded painful and disgusting. I would have preferred the technical term, but I can't find one. I did discover that the surgery to removed the cholesteatomas is called either a intact canal wall down or canal wall up mastoidectomy, depending on the approach.
All of my new knowledge is the result of a meeting with the doctors at the Craniofacial Clinic at Children's Healthcare in Atlanta. One visit and they had pulled together an impressive treatment plan, involving three surgeons and a dentist. Those facial anomalies that I considered superficial are impacting her health and need to be surgically corrected. The current plan is for her to undergo anywhere from four to eight surgeries over the next few years.
Again, I can look at this in two ways. I feel terrible that Amanda has to go through all of this. I detest the idea of surgery. I hate the chaos it causes for the entire family. On a positive note, I'll have a lot more things to learn as we progress.
it is a funny, interesting column- it's also shocking and heart wrenching. how do you all do it?
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