Brianna was a beautiful baby who brought joy to everyone who had the pleasure of knowing her. Unfortunately, Brianna passed away when she was just 14 months old. Brianna still accomplished much in her short life. She was on her way to sitting unassisted, could do ten signs and knew lots of other words, played with her toys, turned pages in books, rolled over from her back to her tummy, played with her toes, and was very smart. She learned something new every day and made everyone around her very happy. Her life was full of love and she is greatly missed every day. We hope you’ll take a moment to read about her life and learn a little more about Moebius Syndrome.
Brianna was born on September 18, 2009, at 39 weeks gestation. We knew prior to her birth that she would have bilateral club feet and I was monitored for polyhydroamnios (excess amniotic fluid) but we were assured the baby was healthy. When Brianna was born, she could not breathe due to a restricted airway caused by her extremely small lower jaw (Pierre Robin Sequence). The neonatologist at our local hospital had to keep his finger in her mouth to keep her airway open. She was rushed to a NICU in Syracuse, 1 hour away, where she spent the first 6 months of her life.
Brianna was intubated and breathed on a ventilator for several weeks. When she was 19 days old, she had tracheostomy surgery to give her a secured airway. When she was just 31 days old, she had a gastro-intestinal tube (G-Tube) surgery to allow her to eat without tubes going down her throat. Prior to the G-Tube surgery, there was a swallowing study done to see if she could swallow…the study came back negative so the surgery was done. However, after the G-Tube surgery (and all tubes removed from her throat), she began swallowing. Probably not enough to sustain her due to the small jaw but she didn’t drool much anymore and we could see her swallows under her chin.
Brianna was still not officially diagnosed with anything but we know that she has Moebius Syndrome. The question on her diagnosis was whether or not she has Carey Fineman Ziter Syndrome (CFZS), which is a combination of Moebius, Pierre Robin, and hypotonia (lack of muscle development). CFZS is extremely rare and there is not much literature available on that syndrome.
Early on, she had difficulty weaning from her ventilator. She was not good at the transitions from ventilator to trach collar directly. We had to first wean her from the ventilator to Continuous Positive Airway Pressure (CPAP) with added pressure at each breath. Then from CPAP + added pressure she was weaned to just regular CPAP. She then worked on transitioning from CPAP to Trach Collar and did a great job for several months. At 4 months old (January 2010), she got a cold and was put back on CPAP full time.
We were told that at-home nursing support in New York State did not provide support for CPAP or ventilator…at least not in our area and not with sufficient hours. The hospital could not discharge a baby on CPAP or a ventilator without a safe healthcare plan. Since there was no nursing support in our area, she wasn’t able to be discharged from Crouse hospital in Syracuse, NY…until she comes off of CPAP. Since it might be a considerable amount of time, we decided to move somewhere that has better at-home nursing support.
Our family moved to Indianapolis, IN on 3/11/10. Brianna was transferred to Riley Hospital for Children in Indianapolis via a medical Learjet on 3/12/10. Immediately after her transfer to Riley Hospital for Children, Brianna showed marked improvements. Her sister, Hailey, was able to visit her regularly in the new hospital – which was something we couldn’t do at the NICU in Syracuse – and that was been great for both of our girls.
Brianna was put on SIMV (Synchronized Intermittent Mandatory Ventilation) in late March 2010. This is partial ventilation that only kicks in if she doesn’t take a breath every 8 seconds. Sometimes when she is in a deep sleep, she has periods of apnea and this will help her through those periods. It is a little more support than CPAP but that was what she needed. Brianna has been seen by an orthopedic surgeon for consultation at Riley and was casted in April 2010. The casts were changed weekly until through July 2010, with surgery as well.
Brianna really became more active after move to Indianapolis and began playing a lot more. She was able to move her head more and had several successful tummy-time sessions where she picked her head up. This was a big step!
On April 13, 2010, we brought Brianna home! She was almost 7 months old and we were so excited to have our daughter home with us finally. Brianna continued to thrive in her home environment. We had in-home therapies (physical, occupational and speech) coming several times a week and several checkups at Riley.
Brianna enjoyed several new toys at home, including a new rocker chair with some hanging mobile toys that she liked to bat around. Brianna loved to be read to and be held…and Mommy spent a lot of time cuddling her every day. She was extremely animated, despite not being able to move her face. She would kick her legs and move her arms quickly when she got excited and you could see her mood in her face and actions.
Brianna loved to use the minty sponges in her mouth for teeth brushing and mouth cleaning. She’d grab it and do it herself and would not like you to take it back from her! She loved music and lights and had several favorite toys that would light up and make sounds when she touched them.
With speech therapy, Brianna became very proficient in signing some key words and was able to indicate some wants/needs. She continually signed “kisses” and wanted hugs and kisses all the time! Brianna also began trying to eat food by mouth and enjoyed baby foods at the dinner table with her family. She loved tasting the foods all by herself.
Brianna loved spending time with her sister, Hailey, at home. They played together all the time. Hailey was very patient and helpful with Brianna. Brianna and Hailey would snuggle while watching TV under blankies on the floor, play with pots and pans like they were chefs, pretend to have a picnic or just dance! The girls enjoyed going to the Children’s Museum and the Indianapolis Zoo as well! Brianna even went trick-or-treating on Halloween with Hailey…although she didn’t actually go door-to-door like her sister!
Unfortunately, on November 4, 2010, we went to work and there was an incident in the home where Brianna went without oxygen for an extended period of time. Due to this, she was officially declared brain dead on November 6, 2010 and we lost our sweet baby girl.
Brianna was an amazing baby and we miss her every single day.