When You're Done at One...

This post is lovingly dedicated to my long distance friend, Lucy... It was written in November 2005 -  a year before Danielle was born.  You never know how things will work out!

Before we got married we talked about everything – where we’d live, what we’d do, how many kids we wanted.  We had decided to wait for 5 years before starting a family.  That would give us time to get established in our careers, buy a house, and have some fun.  Five years came and went.  Every year after that we told ourselves, next year there will toys under the Christmas tree.  Next summer we’ll have a little one crawling across the sand at the beach. 

After another frustrating five years our daughter was born.  Tiny and beautiful, to us she appeared perfect in every way.  After all we’d waited so long for her – more than ten years – what go wrong now?

Well, anyone reading this knows the myriad of things that can go wrong.   Early intervention, hospital trips, therapists, and specialists you never knew existed all became regular parts of our world. Although there were toys under the Christmas tree now our daughter didn’t seem to notice or care about most of them.  She had so many sensory issues that the beach was a nightmare for her.

 Still, we wanted more children.  The doctors had told us that once I was able to conceive and carry a child full term subsequent pregnancies should be easy.  That wasn’t the case with us.  For years we suffered through miscarriage after miscarriage, always hoping that this would be the one that would last.

My 40^th birthday came and went and still we held out hope.  We loved our little girl, but we had dreamed of so many things we would do with our children and she couldn’t do any of them.  Not that we would trade her for anything!  She is the center of our world.  We’ve learned more about unconditional love than either of us knew was possible.

Slowly, I’ve let go of the dream of a “normal” family.  I was torturing myself around holidays always hoping that this would be the year she understood Santa, wanted to open her birthday presents, would actually eat the cake I’d spent hours baking and decorating.   I’ve settled into holiday traditions that work for all of us.  I still want a Christmas tree even if she never looks at it.  I still bake her big elaborate birthday cakes even if she only tastes the frosting.  However, I don’t make her open her own presents or even play with them.

Amanda, Christmas 2005

I do most of these things for myself.  I want her to have everything any other child would have even if she doesn’t seem to care. I would hate to think that someday she might look back on her life and wonder why she never had a birthday party or an Easter basket.   And I’ve let my dream babies go.  While my husband still won’t let me give away the crib, it’s disassembled in the garage.  As holidays approach I’ve stopped dreaming of Normal Rockwell moments... 

 Thank you, Kevin, for keeping that crib! I love you.

Enough, already!

Today I changed the name of my blog. Twice. I'm not sure if Blogger can keep up with me. The truth is I was trying to be something that I'm not. Well, I am, but not all the time.

Confused? Me, too! So, after trying on several new names, I've settled on "Imperfectly Possible" because it seems to fit.

Danielle has been in kindergarten for less than two weeks and I have already run face first into the new breed of ultra competitive parents. Parents whose five year old children are shuttled from school to ballet to violin lessons and then soccer practice. Kids' whose schedules are too busy for play. When did kids start needing day timers?

A childhood friend and I are both  "older mothers" - meaning we had a child in our 40s. We periodically commiserate on how things have changed since our childhood way back in the black and white 1960s. If you listen to us reminisce we really were a watered down version of Brady Bunch meets Family Affair.

Some things were better back then. As kids we could play outside at night without adult supervision. In the summer we were allowed to ride our bikes miles to the nearby pond to swim . We weren't assigned homework until the fourth grade - and then our parents expected it to be our responsibility, not theirs. On the other hand, my parents took "driving beers" in the car with them on road trips and popped "tranks" (tranquilizers like Valium) before family visits.  I'm sure it was easier to adopt a laissez fair attitude toward parenting while heavily medicated. Maybe the good old days weren't so perfect either.

However, there was less emphasis on children being perfect. There were kids in our neighborhood who got Cs and kids who got As. Kids who took shop and kids who went to college. As a child I didn't spend every minute at school or doing homework. I didn't have to go to Kumon or Mathnasium for extra help. Instead, after school we played outside or (heaven forbid!) watched TV.

I am so tired of the pressure we put on kids to be perfect these days. I was truly P-O'ed the other day when my 5 year old's kindergarten teacher sent home a snarky note about her inability to read. It said "Other children have already mastered this list and have moved on to the..." more advanced reading words. She'd been in kindergarten all of SEVEN DAYS when this nasty little note arrived home in her backpack.

I'm sorry. When I was in kindergarten we played outside and learned the ABCs. I wasn't expected to arrive knowing how to READ. The note then went on to say "It is imperative that...practice learning these words on a daily basis since she will be frequently tested. I suggest making flashcards to help drill the words."

My question is, if I'm responsible for teaching my child to read, what is the teacher doing with her day - filing her nails? If my child is going to be tested "frequently" I expect the teacher to TEACH her what she needs to know. Instead it appears that teachers have now become TESTERS. Their primary purpose is to inform you  and your child of how deficient you are. No wonder kids have to go to these tutoring programs - the teachers have abdicated their responsibility for actually helping children learn.

So instead of my child going to school to learn she is going to school to be "frequently tested." Teachers have lost track of their true purpose. Instead they have become drill sergeants and disciplinarians, focused on tests results rather than real learning. I am sad that my child won't be allowed to make mistakes and learn from them in an uncritical environment. I am sad that she is discouraged about school after less than two weeks because her teachers have already written her off. I wonder about how this pressure and need for perfection is going to impact this generation.

I am not perfect. I don't expect my five year old to be perfect either.
 

My Informal Medical Training

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.

You can tell from these photos that the thing that most improved Amanda's quality of life was her IPad!

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.

We Survived Scoliosis and Spinal Fusion Surgery!

I didn't notice that Amanda had developed scoliosis - her teacher did. The first time I was aware of it was when her teacher sent home a seemingly innocent note,"Has Amanda's scoliosis gotten worse?" She was six years old.

I don't know why I didn't see it myself. After all, I bathed her and dressed her every day. I guess that it happened so gradually that I couldn't see it. At first I was irritated. I didn't want something so serious to happen to her. However, I did the right thing and got a referral to an orthopedic doctor.

Look how high her right shoulder is. I don't see how I didn't notice at the time.

Sitting in that appointment I felt like a bucket of cold water was dumped over my head. I was almost in shock. Not only did Amanda have scoliosis, she had severe scoliosis - her back curved more than 50 degrees. She has hypotonia (low muscle tone) and tends to favor her right side. That meant that she developed stronger muscles on the right side of her body, from head to toe. Her dominant right side was actually twisting her body. She had to be molded for a body cast immediately to prevent her spine from crushing her heart and left lung.

Being molded for the brace was tough. She didn't want to lay still. I tried to hold my crying, screaming child down while a technician wrapped her body in casting material. Finally we had to have two more men come in to help hold her down. I heard the orthotist mutter,"low tone my ass," under his breath. Oddly, instead of offending me it added a moment of levity to a very stressful situation.

The completed back brace extended from above her collar bone to just below her hip bones. It opened like a clam shell to fit her body then strapped shut with a series of velcro closures. The goal was for Amanda to wear the back brace 16-20 hours a day.  That meant sleeping it in - which turned out to be impossible. She has never been a good sleeper and expecting her to sleep when she couldn't move or roll over was unrealistic. The low point came in the summer of 2005 when she was in the back brace and had both legs in casts following leg surgery.

Unfortunately, after more than a year of wearing the brace 10-12 hours a day her curvature worsened. It had increased to 68 degrees and there was an added torque as she apparently twisted somehow inside the brace while it was on. So in 2006 --when I was 6 months pregnant with Danielle-- Amanda had her first spinal surgery.

It was her third surgery that year and the most serious.The plan was to insert "growing rods" along her spine.  When bracing doesn't work, very young children with progressive scoliosis can be helped by inserting metal rods to support the spine. The rods are surgically elongated about every six months to allow them to continue to grow. The plan was to keep the rods in until she reached puberty and then remove them and do a complete spinal fusion.

The initial procedure took more than 5 hours.  She remained at the Children's Health Care of Atlanta Scottish Rite facility for 5 days.  One of us stayed at the hospital with her every minute of the day. Since Amanda can't talk she wouldn't have been able to tell the nurses if she was in pain or needed something. She couldn't even push the call button on her own.

After that there was an 6 week recovery where she needed to be relatively still, although fortunately not bedridden.  We bought her a recliner and let her watch a lot of TV.

hospital 2006

Six months later, when she was 8, Amanda went in for the first lengthening of the growing rods. The procedure was much quicker (only a few hours) and the surgeon was pleased that he had been able to extend the rods almost a full inch.

Tragically, there was never a second lengthening procedure. Later that Spring Amanda came home from "water day" at school. Her teacher had written a short note that she had fallen, but wasn't hurt. I looked at Amanda and immediately saw that one of her front teeth was broken. I called her dentist and got an emergency appointment to have it repaired that afternoon. When I was changing her into a clean shirt for the dentist I noticed a bump on the back right side of her shoulder. A few days later that bump had swollen up several inches.

We took her to the orthopedic surgeon for x-rays.  It turned out that the fall had broken one end of the growing rods right out of her bone. Extending the growing rods was no longer an option. At only 8-1/2 years old Amanda had to undergo a complete spinal fusion.

In spinal fusion for scoliosis, rods and screws are attached to the curved part of the backbone and the spine is straightened. Small pieces of bone are then put over the spine. The bone pieces grow together with the spinal bone, fusing it into the proper position.

at home post fusion 2007

Amanda's spinal fusion took seven hours to complete. When I first saw her after the surgery she looked like a different child. Her face was horribly swollen from laying on her stomach for so many hours during the surgery. Even her eyes were bulging. She was on oxygen and hooked up to an IV. She remained in the Intensive Care Unit for several days before being released from the hospital.

This recovery was much harder than the previous back surgeries. She was clearly in pain. She needed to remain in the back brace for the first few weeks while the bones knit. We had to be extremely careful moving her. When she was sitting up it was in her recliner or in a reclining wheelchair.

However, Amanda is a trooper. She has a high tolerance for pain and is amazingly resiliant. Within weeks she was out of the brace and more or less back to normal. She returned to school in the fall of 2007 with explicit written instructions that she be guarded at all times to prevent future falls.

Amanda's First Surgery - Bilateral Heel Cord Lengthening

Bilateral heel cord lengthening was the first surgery Amanda had. Its sometimes called heel cord release or tenotomy. We live 1,000 miles from any family members so there was no one to help us through this emotionally difficult experience.  I was scared and felt very alone. I wasn't sure what to expect during the surgery or the recovery period afterward. The surgeons, who do this operation dozens of times each year, gave us only limited information about the process and recovery.

I tried to have a positive outlook on things that left me trembling. This is an excerpt from a  letter I wrote to our families in 2005 explaining the situation,

"Amanda is facing a tough summer.  In mid-April we brought her in for a routine check up and discovered that she has quite severe scoliosis (57 degree curvature). We decided to put her into a full body brace for the next several years. She doesn’t like the brace and we’re all having a hard time adjusting to the new routine... 

...During the same appointment we discovered that she needs to have both of her heel cords released surgically.  They are contributing to the deformity in her feet and reducing her ability to walk independently... 

Amanda’s leg surgery is scheduled for July 1.  It is a relatively uncomplicated procedure that should be completed in 1-2 hours.  It does require general anesthesia and due to some of her other issues she will need to be on oxygen afterward.  For the next 4-6 weeks she will have both legs in solid casts, unable to walk.  She will also be wearing her new glasses, her back brace and her eye patch.  Not a normal summer by any stretch of the imagination!"

2005 purple casts

Although this is a common procedure for children with special needs we had never considered surgery. Amanda had been in orthotics since she was a year old. She never owned a pair of sandals or dress shoes because they simply didn't fit over the big clunky braces. We had thought the braces would prevent her feet and legs from developing any serious problems. However, despite years in braces her Achilles tendons had tightened to the point where they were impacting her ability to balance and needed to be corrected.

The bilateral heel cord lengthening was a fairly quick procedure. In our case it wasn't combined with any other surgeries. We went into the hospital in the morning and were home by late afternoon. It took just over an hour for the surgeon to make the small incisions and cast her legs. 

She was in the recovery room in less than 2 hours. All we had to do was wait for her to wake up enough to have a few sips of clear liquid and we were on our way home. I was sort of panicked about taking her home - what if something went wrong?

To our surprise, Amanda was given "walking boots". She was up and walking the next day. We found that water shoes (the type you wear at the beach) worked better than the boots supplied by the hospital. She used a walker for support, but she wasn't a very good walker before the surgery, so that was no surprise.

Amanda is nonverbal and seems to have a high tolerance for pain so I can't comment on how painful the surgery was. We kept her on the prescribed muscle relaxer and pain medication for the first few days and then tapered her off. I do know that after a few days she gave no signs of being uncomfortable.

The casts were heavy and awkward so she couldn't walk far in them. We already owned an adult stroller and used it to get her around. I know of parents who have had to rent wheelchairs for their children while they were in casts.

When the casts came off six weeks later, she had a tiny horizontal scar on the back of each calf. It was strange to see such a small incision when she'd had casts covering her entire lower leg, from just below her knee to her toes.

2009 blue casts

At the time I was terrified. I think any time your child is going to have surgery it's frightening. Amanda has had multiple surgeries and I know now that she will come through them fine. Still, every time they wheel her away from me on a gurney I get tears in my eyes. I hate thinking about my baby, unconscious and being operated on.

Four years later Amanda had a repeat heel cord lengthening, again despite wearing ankle-foot-orthotics (AFOs) for the years between the surgeries. Amanda's feet had become deformed over the years, from walking on her navicular bones. She was supposed to have a surgery called calcaneus extension. However, the surgeons determined that her bone quality wasn't good enough for that. They estimated that the donor bone would be approximately 10X stonger than her own bone. Instead they did  a second bilateral heel cord lengthening and casting. The second time they cut through more levels of the tendon with the hope of improving her gait.

First day of school 2009 -
 if you look you can just see the cast on her left leg.

The second time around was easier than the first. Even with the modified surgery it was day surgery. We knew what to expect this time and there were no surprises. Amanda came home in casts. We were all happy to sleep in our own beds that night, knowing that in  a few days Amanda would be feeling better and walking again. In fact, later that summer we took her on a family vacation to New England. She started back to school on time that fall, despite the double casts.

If your child or family member is facing this surgery I want to put your fears to rest. Aa Amanda has had this surgery twice I can honestly say that it was not a big deal.