Emma was born July 11, 2020 at 36.5 weeks gestation, weighing 7lbs 6oz, and 20”. At first, she seemed perfectly healthy, happy, and like a normal baby. A couple of weeks in, we noticed noisy breathing, and it looked like she was working harder to breathe. After experiencing laryngomalacia and all of its struggles with her older sister Kaylee (now 4 years old), we had an idea what we were up against, but in reality had no idea what was in store for us.
At her 4 week appointment with our family doctor, it was confirmed that she did, indeed, have laryngomalacia, and it was much more severe than Kaylee. The opening to her airway was severely restricted, and causing her to struggle breathing. Within days of that confirmation, we had appointments with multiple specialists, 2 sleep studies, and she was put on 24 hour oxygen.
6 weeks later, at exactly 10 weeks old and only 8lbs (severe failure to thrive), Emma had her first surgery with the amazing Dr. Naidoo at JPCH. A Supraglottoplasty is an airway correction surgery, where using either laser or cold steel, they cut a small piece of the epiglottis to stimulate proper growth, to hopefully prevent the “floppy airway” strider noises. Only 20% of effected children will need this surgery - both of Kaylee and Emma were part of that 20% and had the surgery performed within 4 months of each other. After a quick stay in the PICU, we were sent to the acute care pediatric care team. To say what followed was eye opening and stressful, is an understatement.
What followed is multiple rounds of steroids as her airway continued to swell post-op, multiple feeding issues, severe reflux symptoms like projectile vomiting, gagging and inconsolable screaming, 5 different specialty teams, multiple rounds of tests, an NG feeding tube, an NJ feeding tube, more blood work, X-rays, and physical stress than should ever be necessary at that age, and a 3 hour, multidisciplinary team meeting to try and find some answers.
On the positive side, her airway had healed, the oxygen tank was no longer needed, and her laryngomalacia was just a mere stepping stone in Emma’s very young life.
But, that was only the beginning.
At the end of our stay, Emma was diagnosed with FPIES - food-protein induced enterocolitis syndrome. A rare type of allergy that effects the intestines causing severe reflux symptoms, projectile vomiting, inflammation and bleeding in the intestines, cramping, diarrhea, diaper rash, and inconsolable screaming. The issue with this condition, is that it doesn’t present like a typical allergy. There are no hives, no anaphylaxis, no itching - so it goes undiagnosed in most kids, sometimes even labeled as colic, or reflux.
In Emma’s case, after trying many, many foods, she has multiple foods that trigger a response - the most severe being dairy, soy, corn, sweet potatoes, and apple. Finding foods now that she can eat and remain our happy, bubbly little girl is hard. We can’t just purchase foods at the store without scouring ingredient labels. In the 2 years since her diagnosis, she has a very restricted diet, but is a very happy little girl.
Since that stay in September and October of 2020, Emma has also been followed by genetics. Recently, Emma has also been diagnosed with clinical Beckwith Weidemann Syndrome - which you can read a lot about in Anastasia’s story from October, 2022.
Her markers include multiple stork bite birth marks (one on her forehead, and 2 on the back of her neck), an umbilical hernia, growth differences between her left and right sides, and an enlarged tongue - which mainly effects her eating, swallowing (caused aspiration of thin liquids until only recently), and her speech. Due to this condition, she also has blood work and ultrasound screenings every 3 months to watch for tumor growth and abnormalities.
This fall, Emma has seen Dr. Naidoo again for a follow up due to her continued obstructional sleep apnea, and has been booked for tonsil and adenoid removal, another airway scope, and bilateral ear tubes. She also has spent multiple days in and out of the emergency room at JPCH with a virus similar to RSV that has developed into a viral pneumonia, severe dehydration, and ear infections.
Our family would like to give the biggest thank you’s, hugs, and so much love to all of the medical team in the pediatric emergency room at JPCH - Emma has received amazing care - despite how busy they are. To all of her specialists - you are our family now, and we thank you so much from the bottom of our hearts for everything you do.
Some further resources the Family has recommended are the Facebook pages: Sask Parents for Children’s Health Coping With Laryngomalacia, Inc. & Beckwith Wiedemann Children's Foundation International