Monday, January 27, 2014

The Footsteps we Follow

A very important part of getting ready for such a big surgery is hearing the stories of those whose footsteps we follow.  I have a hundred questions, and a bit of skepticism.  Given that the research I originally had done on rotationplasty indicated that many children experienced de-rotation, I knew I needed to hear those stories and the success stories.  Maybe I needed to hear the success stories more.
  I am grateful to be part of a wonderful FB community for PFFD that has offered me great support, advice, and amazing friendship.  In there I can participate in discussions and debates about surgical options, doctors and hospitals, first steps, prosthetics, and all the fears and joys that come with being a parent of a child with this disability.  Not all PFFD patients are the same and in so many ways it is like many other physical disabilities: PT, braces, crutches, walkers, answering questions from strangers, the tears and worries...  Yet, there are many elements of PFFD that are unique.  I mean, how often do you get a phone call that a concerned bus driver saw your son's leg move in a way it shouldn't but he was completely unhurt?
   It is through this wonderful group that I have gotten to meet a handful of other PFFD families at an annual picnic.  There are older children and adults with PFFD who have shared advice and confidence and inspiration.  One such woman had rotationplasty as a child without success.  Why would I then even consider it for my son?  She graciously shared her story and some details of the different surgeries she has had.
  It is my understanding that the specifics of the rotationplasty that my son will undergo is different and improved from the procedure performed in decades past.  So, I needed to talk to a parent whose child has had the "new and improved" rotationplasty.  Along comes Jen.
  Last night, I was blessed to be able to talk to Jen about her daughter's surgery 7 years ago.  Jen is a kindred spirit, from her feelings of being blessed to not know about PFFD prior to birth to the fact that she felt God guided her to rotationplasty though it wasn't in their original plan.  Jen said if she had known then just how well her daughter would be doing now she would never have doubted anything.  These were just the words this nervous mother needed to hear.
   I got to ask about recovering from the surgery, how long her daughter needed PT, any complications, car seats with the spica cast, and playing soccer.  We discussed why we loved Dr. Standard, our journeys to rotationplasty, and how cute 5 year olds are.  We plan to meet this spring, and they will even be in Baltimore while we are there.
  What a blessing Jen and her family are.  I am so grateful that Henry will get to meet someone who has already done what he is about to begin, who can show him that a backward foot is a good thing. I am grateful for Jen, who so graciously shared her wisdom from experience and who, more than anyone else I know, can relate to me in all this right now.

Wednesday, January 15, 2014

Here begins Henry's story

I wrote the following when Henry was 10 months old, just 3 days after we took home Henry's first lucky leg.  I wanted to make sure I remembered Henry's birth, and I wanted to be able to share it with Henry one day.  So, here begins Henry's story.

We’ve all heard it before.  Maybe it was in preschool or your lab partner muttered it in high school Anatomy class.  We’ve all heard it before.
The toe bone connected to the heel bone,
The heel bone connected to the foot bone,
The foot bone connected to the leg bone,
The leg bone connected to the knee bone,
The knee bone connected to the thigh bone,
The thigh bone connected to the back bone,
The back bone connected to the neck bone,
The neck bone connected to the head bone.
   Yes, I knew how it worked.  Or so I thought.
   At 20 weeks pregnant for the second time, I went in for a normal ultrasound.  This would be the day we would find out if we were having another boy or a daughter.  We were amazed to see the active little person inside and were thrilled to hear it was another boy.  I just knew it.  This pregnancy I was not afraid to ask all sorts of questions.  Can you see anything at the back of the neck at this point that might indicate Downs Syndrome?  Can you tell how big he is already?  The little guy kept squirming around as she answered my questions and checked for some of the other more common signs of Downs Syndrome.  We spent 45 minutes, and we were amazed by how adorable his little feet were, and how he seemed to be giving us a thumbs up in the 4-D shot.  The technician even measured his femur to see how big the little boy we would shortly name Henry was.  He measured 10 days bigger than his gestational age, and I remember thinking I was glad I would have a c-section so his size would not matter.
   We left the ultrasound room and met with one of the doctors in my OB’s practice, as was the norm.  She told us that the technician had found an echogenic foci in his heart.  This was a mild marker of Downs Syndrome, and no other markers were found.  My husband and I realized then why the tech had spent so much time, and why she had shown us all these other signs were not evident.  We were given a referral to see a specialist to find out more or get more specific odds that our son would actually have Downs Syndrome. 
   That was a Friday afternoon, and I spent the weekend agonizing over what this meant.  I was less worried about Downs Syndrome.  I had once had a student in my class with Downs, and though I knew it was not always easy, I knew I had the strength to handle it.  No, I was worried about his heart.  Could this mean he would have heart problems?  How would we know?  On Monday I called and talked to my regular OB, and she assured me it most likely meant nothing, possibly a heart murmur.
   Reassured I decided not to proceed with meeting with a specialist.  My understanding is that the doctor could not say for sure if my son had Downs Syndrome without risky tests I was not willing to take.  Though I would wonder for the next 19 weeks, I knew I would love my little guy no matter what.

   So, fast forward to birth day, May 15, 2009.  We left early in the morning, after kissing our older son, to be at the hospital at 8.  I admit, a tiny piece of me was apprehensive about finding out that Henry had Downs Syndrome, mostly because I wasn’t sure how you shared that kind of news.  What would I say?  Mostly, though, I couldn’t wait to meet and see the baby who had been hiccupping inside of me for months.
   Around 10 the c-section began.  The first surprise was to discover that Henry was breech.  Due to the scheduled c-section, my doctor had not done the typical exams to see if I was dilating or what position the baby was in, so we had not known.  Oh, my, I had been patting his head up by my ribs thinking it was his butt!  Shortly after that I heard the most incredible sound – my son’s cry.  My husband assured me that he looked as perfect as he sounded.
   The nurse who was in charge of checking him out and weighing him, soon became nervous, according to my husband.  He remembers that whispering soon began.  He could tell something must not be right, but could not see what it was.  The nurse carried my 6 pound, 14 ounce baby over to me and held him up.  She said that his left leg was not right.  She wondered aloud if the skin was somehow stuck in a way that did not allow him to stretch out his left leg.  The nurse also pointed out that his head tilted to the left.  I did not see anything wrong.  His right leg was also bent, and no new born can hold their head up when upright.  No, I could only see the miracle who had just joined our family.  The nurse said that they would have to take him to find out what was wrong.  Okay was all I could say.
   With my belly still open, I lay there looking at my husband wondering what was going on.  Henry stayed in the OR until after the second Apgar score was taken at five minutes.  I would later learn his Apgar was 9, a perfectly healthy little guy.  Then I got one last look at my now bundled baby, a chance to admire how adorable his nose was, and then he was whisked away to the nursery, my husband at his side.  Left alone in a room full of doctors and nurses, my OB talked about what she was doing, and how she was glad my ovaries looked good.  I don’t recall any comments about what might be wrong with my newborn.
   Soon, I was returned to my room, where my father and sister awaited.  After greeting them, it became apparent that they had no idea where Henry was.  I told them what I knew, and they both went down to the nursery to be with my husband and newborn.  My sister returned to quickly give an update and to be with me.  The pediatric nurse came in shortly thereafter and told me what they knew so far.  They had done some x-rays and think he has no femur in his left leg.  Later x-rays would show he also had no fibula in that same leg.  Wait a minute, the song says that the foot bone is connected to the leg bone, the leg bone is connected to the knee bone, and the knee bone is connected to the thigh bone.  How does he have a beautiful foot without a thigh bone?  I had never heard of such a thing.
   After 2 hours of x-rays and testing (they could find nothing else to worry about), Henry finally returned to my room.  We were quickly moved into our official hospital stay room, but the adventure was just beginning.  Henry had been gone long enough that his blood sugar had dropped, and my first attempt at nursing did not alleviate this.  I had to give him a supplemental bottle for him to drink one ounce.  For the next nearly 24 hours, nurses had to prick his heel and take his blood sugar before and/or after every feeding to make sure he was okay.  Luckily, his blood sugar went to normal and stayed there.
   Our next issue was getting Henry all cleaned up.  Normally, the newborn is bathed in the LDR room I had previously occupied.  However, Henry was not there long enough before they moved us to prepare that room for another mother.  So the nurse had to be convinced that she needed to do it, and she had to find all the equipment from the other wing.  The room we would spend the next two days was newly renovated, so it was beautiful and comfortable but did not have everything stocked yet.
   Over the next few hours, we would be visited by the pediatrician, the pediatric nurse, two physical therapists, and even my OB returned to check on us herself.  She came to see what we knew, how we were handling it, and to apologize for not having discovered it before Henry was born.  I simply responded, how could she have known.  We had seen two beautiful feet, so who would have thought two of the bones in between would be missing?  Besides, he had been moving so much, we had just assumed he was kicking his leg up out of the way in the one ultrasound picture we had of the butt and right leg.  It had never crossed my mind that it could be anything different.
   In between all these visits, I sent a quick text message to a co-worker to spread the news that Henry had arrived.  I gave no details.  If I had been worried about how to tell people my son had had Downs Syndrome (the doctors assured us he did not) which is so much more common, how did I tell them about such a rare physical birth defect?  My father had stepped out of the room between visitors and called family and told them the news.  The family who did come to visit that day were not surprised and responded wonderfully to the miracle that Henry still was.
   Back to all the visitors.  The pediatrician of course came in to tell me in person what she found and to reassure me all else was well.  She scheduled appointments for Henry with a genetics specialist and orthopedic specialist at Cincinnati Children’s Hospital to help us get more answers.  You see, she could not give a name to his birth defect, for it was a first for her as well.  The pediatric nurse came in frequently that day to answer questions.  She even spent time discussing the x-rays with the technician so she could better explain what they showed to us.  She made sure we had multiple copies to give to doctors and to keep.  She even discussed with us whether or not we thought it was causing him any pain.
   The two physical therapists came in together and were there to discuss his leaning neck.  They were not there to address his leg at all.  We learned the neck issue is called torticollis, and it is relatively common among newborns, especially those that are breech.  We would learn months later that Henry’s torticollis was more likely related to his leg birth defect.  We were given some advice and some exercises to try.  Unfortunately, they gave me a list of what to do if his head was tilted in the other direction.  Lucky for them, it was the least of my worries at the time.
   One of the special visitors was my mother-in-law with my older son.  We, as a family, got to experience that wonderful moment when brothers meet.  It was all that I had hoped, and Henry’s leg didn’t play a part.  It was quite possibly the most “normal” moment we had all day. 
   Sometime around midnight, when all my family had gone (except my husband who rarely left mine or Henry’s side), the specialists had stopped coming in, and I was peacefully holding my new miracle (he had the longest fingers I had ever seen!), I finally had to figure out how to tell the rest of the world (or at least the part of it that was waiting to hear about my new baby) about the surprise birth defect.  So how do you share news like this with friends and co-workers?  We were so blessed that Henry’s birth defect was life affecting and not life threatening, but most of the time you just heard how big the baby was and that mother and baby were doing fine.  I personally had not experienced a precedent on how to spread the news.
   After some thought, I attached a picture of a typically swaddled sleeping newborn (though Henry was cuter than most!), titled the message “Isn’t he adorable!”, and typed the following:

Henry Joseph Horton made his great arrival Friday, May 15 at 10:36 a.m., weighing in at 6 lbs and 14 oz (not quite as big as I thought!) and 20 in.

 He has a sweet personality! Though he isn't afraid to let us know when he isn't so happy.

 Henry is perfectly healthy, but he was born with a serious birth defect. He is missing the femur bone and fibula bone in his left leg. We just ask for a special prayer as we await meetings with specialists to help us better understand this unexpected reality.
   It wasn’t more than a minute after I sent the message that I wished I had changed the word “reality” to “blessing”.  “Reality” gave it a more negative spin, and though I was like every mother who just wanted her new baby to be perfect, I did not feel negatively about it.  Just overwhelmed.  Surprised.  Amazed.  It was early on a Saturday morning, so most would not get the message until Monday, which was fine.  A few people did read it on Saturday and sent messages of congratulations and prayers.  Our blessings continued.

  Day 2 of Henry’s life was a little calmer, but was still full of visitors.  I chalk this up to being a Saturday when not as many doctors are around.  Another pediatrician who was on call at the hospital checked him out, saying that he looked healthy.  I had a long list of questions to get answers to, and she didn’t help either.
   Henry also suffered the agony of a circumcision, which left a bruise that had me scared a few days later.  I hadn’t really been a nervous mommy with my first son, but to some level, that had completely changed.  Especially in those first few days, I kept waiting for the other shoe to drop.  What else was wrong?  What had we not yet discovered?  Could there be something more serious, something life threatening, we had yet to detect?  I even began to picture Henry years out as a 20-something looking for love.  I prayed that he would be able to find someone to love him, someone who could disregard the leg as just some bones and see how truly wonderful he was. 

   More family came and properly oohed and aahed.  We came home the next day.

Sunday, January 12, 2014

2014 - The Beginning, Sort of

For the last 9 months we have been anticipating Henry's big trip to Baltimore this coming summer.  I guess now that the year on the calendar matches, it is starting to feel like it is just around the corner, that 5 months will pass in no time.  This has my stomach in knots.  Ugh. Yeah!  Ugh.
   We currently have Henry's rotationplasty with Dr. Standard scheduled for June 18, 2014.  It will be his first surgery, and a mighty big one at that.  My baby will be under for 6-8 hours.  They will for all intents and purposes remove his left leg and reattach it, backwards.  This is no small thing.  This is scary, and yes, it is also exciting.  Right now, I feel much more scared.  Did you read the part about that this is my baby we are talking about?  Wait, scratch that, terrified.
   No, Henry is not a baby anymore.  A month prior to the day in discussion Henry will turn 5.  In fact, trying to explain all of this to him and how he will react when he sees his leg afterwards is part of what scares me.  Will he take it with his usual positive attitude and smile?  Or will this change take away his comfort in his self-identity with a leg that is different already?  I can only pray that my confidence that my husband and I are making the right decision to give Henry the best quality of life long term is not misplaced.
  We did not make this decision lightly.  What is exciting about this procedure is the benefits we believe it will offer Henry both short term and long term.  We have carefully chosen a doctor whom is a specialist in PFFD (or CFD) and who has successfully performed this surgery.  Dr. Standard took the time to give us options for helping Henry, and he helped us understand how rotationplasty is the answer to our biggest question.  It is not a surgery intended to make our prosthesisists' job easier in helping Henry walk, but rather will give him improved gait and long term hip stability.  Long term I know is no guarantee, but this is the best bet and our doctor has confidence Henry will have it.
   Now to keep my focus on that. Ha!  Did I mention my stomach is in knots?
   With only 5 months left, it is time to iron out the details.  As we do that, I will tell you the real beginning of this journey, introduce you to Henry, and tell you all I know about PFFD and FH and rotationplasty.  OK, 2014, let's get this journey started.