Surgery

The day we left for Boston had been an emotional roller coaster.  We were so happy to see both of our families to celebrate Easter and have a nice send-off for Molly, but our sweet cat, Bo, unfortunately and somewhat unexpectedly passed away two days prior which made me that much more emotional.  Then after about 5 hours of driving, Molly became car sick.  There we were on the side of the highway - Kevin changing Molly on the front seat and me scrubbing her car seat and trying not to lose it. We finally arrived in Boston late that night after a very long ride! 

Molly's mom-mom and cousins snuggling her before we left. 

We had our pre-op appointment Monday morning and I was very nervous going to the hospital.  It all started sinking in at that moment.  All of the research we had done, all of the doctors we had seen, the insurance battle, all of the questions and doubts and uncertainties lead us to this point.  As confident as we finally were in the decision we had made, it was still a life-changing decision we were making on behalf of our daughter that will affect the rest of her life. Even though we were in Boston, two days away from surgery, I still asked Kevin if we were doing the right thing.  He reassured me that we were. 

We met with Dr. Kasser to discuss details of surgery.  It was determined that he would remove her second toe, including the metatarsal bone that runs through her foot (ray resection). He would debulk as much excess tissue as he safely could from the bottom of her foot, without compromising blood supply. He also mentioned that the toe flexor in her big toe seemed weak which was causing the toe to point upward.  He suggested a possible partial amputation of her big toe as well.  We both immediately said no - we had come to Boston with a plan - a plan that we had finally accepted as the best course of action for Molly.  We were not prepared in any way to make such a significant decision right before surgery, especially since there is a chance it can get stronger, in which case a partial amputation would not be necessary.  We agreed it was best to watch and see how it develops.  

He then informed us that he happened to have another macrodactyly patient that had an appointment with him that day as well.  We were so excited!  Although we have been fortunate enough to have connected with about 15 families online throughout the world with a child with macrodactyly, we would get to meet a girl, in person, who has it!  How lucky were we!  (Side note: I heard that there are estimated to be approximately only 500-1000 cases of macrodactyly WORLDWIDE). That just illustrates what the odds are that we had an appointment on the same day as another patient!  I cannot say enough about how lucky we were to be able to meet her, but first we had to finish all of our pre-op appointments which included blood work, X-rays and meeting with the anesthesia team.  While we were waiting for the anesthesia appointment, we were approached about donating any extra samples from the surgery to Boston Children's Hospital's Bio bank.  They would use it for various research studies as needed.  During the anesthesia appointment, we had to go over every piece of medical history starting from my pregnancy.  This was definitely a time-consuming task as a lot has happened since then.  One thing that the anesthesiologist questioned was when I informed her that Molly's genetic's doctor from CHOP felt that it was necessary to put a malignant hyperthermia precaution in place, something typically reserved for people with neuromuscular disorders. Even though Molly did not have an official diagnosis, as we were (and still are) awaiting test results, he wanted to err on the side of caution.  The anesthesiologist we met with said she was not necessarily going to be our anesthesiologist on the day of surgery, but she would relay the information to the others; however, she said not to be surprised if the anesthesiologist that day disagrees - apparently the malignant hyperthermia precaution is not something that is commonly done, especially without a formal diagnosis. 

After all of our appointments, we finally went back to the orthopedic department to meet the macrodactyly patient.  We walked in the room and were greeted by a 17 year old girl and her mom.  Her mom started crying as soon as she saw Molly's foot because she said it brought back so many memories of her daughter at that age.  We just hugged and cried and she told me she knew exactly what we were going through.  Our connection was immediate and we had so much to talk about.  They had never talked to or met anyone with macrodactyly before.  Her daughter was the most mature 17 year old I've ever talked to and she had the most positive outlook.  She was friendly, outgoing, had participated in dance and other physical activities - something we worried Molly wouldn't get to do.  She had 7 surgeries up to that point and her foot looked amazing.  If you were not counting toes, you would have no idea there were only 4.  We talked for a very long time and it was the best thing that could have happened to us just two days prior to surgery.  They brought a sense of calmness to me that everything would be fine.  The visit was comforting and made me finally feel with 100% confidence that we were doing the right thing. They both helped us more than we could have ever imagined - it was fate that we met that day! 

On Tuesday, we had a great day in Boston.  We wanted to enjoy the day as a family and tried not to think about surgery.  We went to the New England Aquarium and went out for pizza.  

Surgery day had finally arrived.  I hardly slept the night before. I was up every hour and had a knot in my stomach.  We had to be at the hospital by 7am to get checked-in. 

Daddy walking Molly to the car to head to the hospital. 

We got Molly changed into her hospital gown and we were seen by all of the specialists that would be involved with the surgery.  It was all very overwhelming.  Each team of doctors had a specific plan for her.  The pain management team talked about the nerve block that would hopefully eliminate her pain while she was in the hospital.  Then we saw Dr. Kasser and his team, who again went over the exact plan and marked her foot.  They were followed by a woman who talked about macrodactyly research that was being done.  She had asked permission to collect samples from the surgery to be used specifically in macrodactyly research (separate from the Bio bank research).  We were very excited to hear that research was being done!  The anesthesiologist then came in and had many questions for us regarding the request for a malignant hyperthermia precaution.  Like the previous anesthesiologist mentioned during pre-op, she too questioned the necessity of it since there was no formal diagnosis, but said she wasn't going to go against CHOP's request.  The amount of medical information I gave her lead her to ask if I was in the medical field.  I laughed at that, but it made me realize the amount I had learned over the last year and a half.  Another member of the pain management team then came in to administer a liquid medication to Molly to make her sleepy since she would not be getting a mask.  Our final visit was from a woman who asked what color cast we wanted.  I think I started crying and laughing at that point because I had just been bombarded by so much information and asked so many questions, I didn't even care what color her cast would be.  Molly took the edge off of the situation as she just smiled the whole time and started getting giggly after the medication kicked in.  Just as things were getting hard, she knew we needed a laugh.

The next thing we knew they were carrying her off to the OR.  Kevin and I hugged and cried and were eventually escorted to the waiting area.  

It was unbelievable to see the amount of families in the waiting area.  We had a nurse that would update us every hour or so and we were also able to request updates.  There was so much anxiety as you saw various nurses and doctors entering the waiting area and wondering if they would stop at you.  Boston Children's Hospital really did a great job at trying to make a tough situation more relaxing.  They had a harpist playing and also had a therapy dog walking around.  Around 10:30am, the nurse came over and said they had just started surgery!  Molly was taken to the OR at 8:30am.  We were hoping they would be farther along at that point.  

Surgery ended around 12:30pm and Dr. Kasser came out to update us.  He said everything went as planned and thought we would be very happy with the results.  We couldn't wait to see her, but it took us a while before we could.  When we finally made our way to the PACU, Molly was screaming and seemed very agitated (I don't blame her)!  That was really the hardest part (besides saying goodbye before surgery).  There were two nurses by her side - one was carefully monitoring her vitals while the other was holding oxygen up to her.  The first question they had was, "Is she normally a breath-holder?"  Apparently Molly had a very tough time after waking up and that's what took so long to see her.  She was crying so hard and holding her breath until she turned blue.  No one was very happy about that.  We had to get her detailed cardiology results sent over ASAP.  I don't even remember at this point the name of the exact thing they wanted to check for, but there is some heart condition that can cause you to turn blue and they wanted to rule that out.  Everything ended up being fine and she stayed in the PACU for at least 2-3 hours until she calmed down.

First time holding her after surgery. 

Once she was stable, they moved her into a room.  It was actually right off of the PACU and not in the main hospital.  It was fairly quiet and she had her own room.  She ate immediately and the nurses were quite impressed with her appetite!

She had a nerve block in her leg until about 6am the following morning along with pain medicine and Tylenol every 4 hours.  

After only one night in the hospital, we were told that we had the option to stay another night or be discharged; however, Dr. Kasser wanted to see us Friday morning regardless.  We decided to go back to the hotel for the night so we could all try and get a good night's sleep.  Molly again amazed us by crawling as soon as we set her down!  She just had major surgery the day before and was crawling like it was no big deal, even with a full, bent-knee leg cast!  We had a quick visit with Dr. Kasser Friday morning and then we were on our way home!  

About 2 1/2 weeks later, Molly had her first plane ride up to Boston for cast change #1. This would be the first time we would get to see her foot.  Kevin and I were very eager to see what it would look like.  We were amazed at how different it looked and couldn't believe how much tissue he was able to remove from the bottom of her foot.  We were thrilled at how it looked so far.  After a quick cast change, we were on our way home.  Molly did great on the plane!

Before surgery and after first cast change!

Cast #2 - pink!

Four weeks later, we flew up to Boston again.  This time, Molly would get a weight bearing cast!  So after almost 7 weeks of not being able to stand, we were so excited for her to get this new cast. 

Waiting for x-rays to check the alignment. 

Cast #3 - weight bearing!

Finally, after another 3 weeks, the final cast was removed!  Molly did so great with this whole process and she made it much easier than Kevin and I initially anticipated.  Everyone told us that kids are resilient, but Molly really proved this to be true.  She is my inspiration and I am a much stronger person because of her. 

Final cast is coming off!

Before surgery and after cast removal

Our next follow-up appointment in Boston is in December.  The next surgery will likely be in the Spring and will focus on her big toe.