Faith was born September 26, 2006 with Goldenhar Syndrome, or bilateral Hemifacial Microsomia (which loosely translates to one side of the face is underdeveloped but in Faith's case and others it's bilateral or on both sides of her face which of course sounds completely contradictory!!). You can read more about Goldenhar or HFM here, http://www.faces-cranio.org/Disord/Golden.htm or http://www.ucdmc.ucdavis.edu/children/services/cleft/health/anomalies/oculo.html, but cases are so varied that some of the symptoms do NOT apply to Faith.
We found out Faith's issues about 6 weeks before she was born, giving us the advantage of being able to prepare mentally (as much as you can) and to have the right people present at her birth. At about 28 weeks pregrant, on a trip to Seattle for Laura's baby shower (one of my VT Hokie friends), I was very uncomfortable and became concerned something was wrong. It took a couple more weeks for the second OB-GYN to finally discover that I was measuring full term at only 32 weeks. I had polyhydramnios or too much amniotic fluid - a condition that happens among other reasons, when a baby is not swallowing the amniotic fluid. After more ultrasounds it became obvious that her jaw and ears were underdeveloped. At that point, the doctors thought she had Treacher Collins Syndrome, although her eyes did not appear to be typically affected. Based on that early diagnosis, I was very fortunate to find an amazing support group on the internet of other families dealing with TCS that I still email with regularly. It wasn't until I pointed out a little cyst on Faith's right eye to her opthamalogist at 3 months old that she was diagnosed with Goldenhar Syndrome.
So, since we knew Faith might have airway issues at birth, we were able to change hospitals (to Presbyterian St. Lukes in Denver) to have the right team present at her birth, a planned C-section. Not only were Fred and Robin's mom, Leah, present, but so were about 20 doctors, nurses and nursing students to assist and witness her miracle of life. Immediately after Faith was born they intubated her (took three tries and an ENT with very fancy tools to succeed - like I said, we were blessed to be prepared) and four hours later Faith was trached because the NICU felt her intubation was too difficult to risk it coming out. I heard Faith cry once while they were trying to intubate her -- I was laying in the other room and someone said "that's your baby crying" -- I misunderstood what was going on and thought she was going to be fine and that she could breathe on her own. In fact, they had simply pulled her tongue forward allowing her to breathe and cry for just a moment before they tried intubating again. Having a baby that can't cry or make any sounds was one of the toughest things we dealt with emotionally, but if only you could hear her now - at 15 months and still trached, she can scream so loud just for the fun of it!! It's wonderful to hear!
At 10 days old, Faith had a G-Tube put in, so we could feed her directly into her stomach instead of through a tube going down her nose (due to the small size of her jaw, her tongue is blocking both her airway and her throat). She aslo had a Nissen ("fundal plication" or a wrap around the top of her stomach to control her severe reflux). On October 20th, Faith came home from the hospital and we started caring for her all by ourselves at home with an occassional visit from a nurse (June). Everything seemed to be going well except that Fred and I were struggling with the fact that one of us always had to be with her and we had no one to care for her so we could get out of the house together.
In January, we experienced one of the worst days of our lives, Faith plugged her trach and turned blue. Fred raced her upstairs to me and called 911 while I was able to change her trach and resuscitate her, but we know this was Faith's second miracle and a close call. The ambulance took her to the hospital where we discovered she had RSV (Respiratory Syncytial Virus - a dangerous respiratory cold for infants). She stayed in the hospital for only 3 days and was fortunate to have had a mild case of it since she was getting a monthly shot to help protect against RSV. After changing pediatricians we were referred to a Pulmonologist who has been an amazing advocate for Faith. Shortly after Faith's blue episode, Dr. Guarin helped us get approved for in home nursing care - 14 hours a day/7 days a week (we actually have it 10 hrs a day/5 days a week since more than that is very hard to find and really more than we need/want).
In February, Faith was sick again and we discovered she had a hiatal hernia and needed to have it repaired. She spent 10 days in the hospital, mostly getting better before they could do surgery and then recovering. After that things started to get easier for us and we started getting the nursing care on March 5th. Having nurses in your home caring for your children full-time is both wonderful and very challenging. You lose your privacy and you lose a lot of freedom in how you care for your child. Simple decisions like changing the quantity of food you give your child has to be approved by a doctor first and everything is tracked and written down including whether or not your child had a poopie diaper overnight! We've had both good and bad experiences, and we've had to let two nurses go. This was really hard to do, but in the end it is worth it as we now have two nurses who fit with our family very well. Lisa has been with us since March and is like family to us now, in fact her youngest daughter, Chelsea, is the same age and goes to the same school as Dana and they have become good friends!
In June, we were able to take Faith and Anthony to Virginia for almost 6 weeks to be with my family, staying in my parents available rental house that all of my sisters helped them furnish! This was a really special treat for both me and the kids and Fred was very accomodating in making it happen and taking care of Faith without nursing help again (but some help from my sister and mother) while I made a couple road trips to PA for work. We also drove up to NY for a long weekend with Fred's best friends, Michael and Linda, and for Faith to be evaluated at NYU. She was also evaluated by a craniofacial team in Virginia, at Fairfax INOVA hospital. Both teams confirmed what we had already been told by our team here in Denver, that Faith is missing most of the right side of her lower jaw and TMJ and the joint is significantly underdeveloped on the left side. She will need a bone graft to rebuild the joints around age 3 and then likely go through a jaw distraction about a year later to lengthen the jaw. So right now our best guess is that she will not have her tracheostomy closed before she is 4-5 years old. There is at least one more team I would like to see, Childrens Hospital of Philadelphia (CHOP) before we decide where to have these surgeries. In addition to her jaw surgeries, when she is older, we will start looking into doing ear reconstruction.
In August, after we returned home from VA, we had another frightening incident with Faith turning blue again, this time when only her nurse Lisa was home with her. Faith accidentally caught her thumb under her trach tabs and pulled the tube out, after which Lisa could not immediately get it back in because of too much blood coming from the stoma (the trach hole). She did succeed but Faith then passed out and Lisa had to revive her and call 911. Faith was taken once again to the hospital and this time airlifted from the first hospital to her primary hospital in downtown Denver where she was born (PSL). Fred, Anthony and I made it to the first hospital in time to see her before she was loaded on the helicopter. She spent the night under observation and came home the next day.
On Wednesday, September 26, 2007, Faith turned one year old!! It was a very special day and we took her to have her pictures taken (see album). On Saturday we had a big party for her at our house with a handful of close friends and some of her support system - nurse Lisa, her speech pathologist, Kim, our sign language teacher, Teresa, plus a couple out of town guests, including my sister, Sonya, and surprise visitors Grandma and Grandpa!! It was a wonderful party even though the wind forced us mostly indoors!
Now 2007 is behind us and we look forward to the new year and hope to have a much smoother year without any unplanned surgeries or hospital stays!! Faith is just about to start walking - standing all by herself for many seconds and taking an occassional step! But the most amazing part of her growth is her ability to sign - she is easily over 20 words that she can sign already including "Daddy, Cat, More, Dog, Nap, Hi/Bye, Mommy (sort of!), Bath, Light, Tickle Me, Where, Come/Give Me" and more that I can't think of right now!! She can also do sign names for Dana and is just learning Anthony's and Ryan's in the past couple of days! Her pediatrician said kids around her age usually know "Mommy, Daddy and maybe 4 more words" so we are really thrilled with how quickly she has learned to sign! The other joy is seeing her laugh (kind of a breathy sound right now but it's wonderful!).
I look forward to posting regular journal entries for anyone who is interested in reading them and for my own way to keep a record of our busy life!!
Much love to you all,
Robin and Fred and Lauren, Ryan, Dana, Anthony and FAITH!!
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