Valerie and Bo Foley were just beginning to share the exciting news of her pregnancy when results from an ultrasound revealed that their baby had a rare birth defect.
Valerie was referred to the maternal fetal medicine program at EVMS to coordinate with CHKD for immediate access to our neonatal intensive care unit and pediatric surgeons. As the reality of their overwhelming diagnosis began to set in, Valerie chronicled her young family’s experiences in a blog she called, “The Amazing Adventures of Claire Bear.”
The following excerpts from her blog offer a glimpse into the Norfolk family’s medical journey as it unfolded.
My First Blog Post
February 28, 2013
Meet Claire, a.k.a. Claire Bear, who is set to join our family in May of 2013. Most of our friends and family know about Claire’s impending arrival, but until now, we have only told a few about her condition. On November 6, 2012, we learned that Claire would be born with a condition known as an omphalocele. The word that took me a while to be able to pronounce, and even longer to spell. An omphalocele is a rare birth defect that arises when the abdominal wall does not close properly during fetal development. As a result, some organs develop outside the abdomen within the umbilical cord.
Claire’s omphalocele most likely contains her bowel and possibly some of her liver. She will need surgery after she’s born, so we were referred to the pediatric surgeons at CHKD. We’ll do our best to keep loved ones up-to-date.
Meeting Claire’s Surgeon
March 25
We’ve had MANY appointments since we learned of Claire’s diagnosis, including a recent consultation with the neonatologist, Dr. Kenneth Tiffany, at CHKD. I had no idea what a neonatologist was before our appointment, but essentially, Dr. Tiffany will be in charge of Claire’s overall care while she’s in the NICU. Both Bo and I really like Dr. Tiffany.
Today was the all-important consultation with CHKD pediatric surgeon, Dr. Frazier Frantz. Bo and I were extremely comforted by him – from his demeanor to his extensive knowledge and everything in between. There have been four other babies with omphaloceles born in Hampton Roads in the last two years, and Dr. Frantz was the surgeon for three of them. So we know Claire will be in extremely experienced hands. We learned that there are two ways to repair an omphalocele: through surgery to put the organs into the abdomen soon after birth, or a process called “paint and wait” where you keep the omphalocele medicated and wrapped to give the baby’s abdomen a chance to grow before closure a few months later. The general thought is that Claire will not have surgery right away. This could all change but Dr. Frantz has given us tremendous confidence going forward, despite the fact that there are still a lot of unknowns.
Claire’s Arrival
May 16
Claire was born at 9:18 a.m. via C-section. She weighed 8 lbs., 4 oz., and was 21.25 inches long. She cried within seconds of birth, and it was the best sound EVER. Seriously, I know that sounds trite, but a lot of omphalocele babies are born with underdeveloped lungs ... so Claire’s wail meant that she was already ahead of the game.
Week One
May 22
Claire is pretty much taking care of things on her own - all of her nurses and doctors, including the amazing Dr. Frantz, have been impressed with her progress. Claire’s omphalocele contains a part of her liver and a part of her bowel. Although it is considered “giant” due to the presence of the liver, it is actually quite small in comparison to the rest of her abdomen. I think Bo put it best when he compared it to a cue ball. About three-quarters of Claire’s omphalocele was covered with skin at birth, which is GREAT considering that most omphalocele babies just have a membrane covering it -- which is subject to rupture.
Home Sweet Home
May 23
Claire was discharged today, exactly one week after she was born. Every day has been an unparalleled joy as she continues to amaze us at each turn. Dr. Frantz taught us how to apply ointment and gauze to Claire’s omphalocele and an ACE bandage for compression. This will help gently keep her organs in place until surgery.
Month Two
July 3
Dr. Frantz is the epitome of calm, therefore we were happily surprised when, upon seeing Claire’s abdomen, Dr. Frantz pulled out his cell phone and asked if he could take a picture. He was extremely happy with both the amount of skin coverage and how much the omphalocele has reduced in size.
Month Three
August 7
Claire had her third appointment with Dr. Frantz since her release from the NICU. As always, it was very positive. Bo and I walked away feeling extremely optimistic for the coming months. The omphalocele is now 100 percent covered in skin, and we are looking at closure surgery when Claire is around 6 months old.
Month Four
September 9
Our little Bear is getting bigger and bigger every day! She enjoys playing on her playmat and sitting in her bouncy chair.
Our next appointment with Dr. Frantz is October 9th when we will be scheduling the date for her surgery. While I am excited that Claire will be having her closure surgery, I am nervous all the same. I know Claire will be in the wonderful care of Dr. Frantz and the rest of the amazing CHKD staff.
I’m probably not doing a very good job of explaining how I feel, but suffice to say that all my fears are pushed aside whenever I look at her precious face.
Month Five
October 9
Claire’s surgery is scheduled for next week. During the surgery, Dr. Frantz will be making an incision right in the middle of Claire’s omphalocele, cutting out existing scar tissue. After pushing her liver and bowels back into her abdominal cavity, he will stretch her rectus abdominus muscle (her “abs”) and close her abdomen.
We should expect Claire to be in the hospital for a couple of days, mainly for pain management. At that point, she should have some good scar tissue forming that will assist with the recovery.
Surgery Day
October 16
The surgery lasted almost exactly 90 minutes, but it felt like forever. Dr. Frantz once again proved how fantastic he is when, instead of sending out a nurse or resident, he came to the waiting room himself. Not surprisingly, things went better than expected. You’d think we would know by now how much of a fighter our daughter is! Dr. Frantz discovered that Claire actually had extra fascia (connective tissue) tucked under her sternum, which he was able to use to close the hernia, along with her “ab” muscles. Dr. Frantz could not stress enough how beneficial our compression wrapping had been – allowing Claire’s liver and bowels to easily slide into place.
Post-Surgery
November 6
Exactly one year ago today, Bo and I were sitting in the doctor’s office hearing the word “omphalocele” for the very first time and realizing that our world was about to be turned upside down. If you told me then that we would be where we are now, I would have laughed at you. I am at a loss for words thinking of all the miracles we have experienced over the past 12 months.
Month Six
November 19
Our Claire Bear turned 6 months old on Saturday. She has continued to have a fantastic recovery since we have been home.Except for the scar, you can’t even tell that Claire had major surgery less than a month ago.
I’m going to end this post with a picture of Claire’s “new” stomach for those who would like to see it. She does have a little extra skin around her incision but we expect that to start to disappear as she grows. Dr. Frantz did say that it could be fixed later, but we think she is beautiful just the way she is.
A New Year
January 2, 2014
I hope everyone’s year is off to a wonderful start. I know that, for us, 2013 started as a year of unknowns and uncertainties, but it ended as a year full of amazement and miracles. When we first learned about Claire’s omphalocele, I never once imagined that she would already be where she is today. Thank you again to all of you for being on this journey with us, for all your well wishes, prayers, and support. We couldn’t have come this far without you.
Dr. Frazier Frantzpractices with CHKD Surgical Group's pediatric surgery practice. Dr. Kenneth Tiffany is a neonatologist with Children's Specialty Group, PLLC, at CHKD.