Support Emmett’s Rare Congenital Heart Defect Journey to BCH

Emmett is my sweet 9-month-old nephew, who was recently diagnosed with a very rare congenital heart defect. It is called a vascular ring, more specifically, a right sided aortic arch with aberrant left subclavian artery from Kommerell diverticulum. ❤️‍ 

 

Here is his story: 

 At 3 weeks old, Emmett began presenting with worsening symptoms, including: back arching, stridor, reflux, gurgling, gagging, choking (especially during feeding), frequent feeding, gas, constipation, and weight loss. At 9 months old, he is still suffering with these symptoms and is unable to have solid foods. For the past 2 months, Emmett has been struggling during the night, requiring frequent nursing and propping up after being fed. He is unable to be left unattended while at rest for fear of choking in his sleep. Emmett has seen multiple doctors and has undergone numerous tests. Despite it all, he maintains the happiest and sweetest disposition and smiles all the time. Emmett is such a delight to our family, and we all just absolutely adore him! 

 After weeks of referrals, testing and doctor visits, the cause of our sweet Emmett’s persistent symptoms have been identified: a very rare type of vascular ring, specifically a right sided aortic arch with aberrant left subclavian artery from Kommerell diverticulum. Only .04% of the population have this particular anomaly. Currently, Boston Children’s Hospital is the only facility in the country that has a vascular ring program suited to address and permanently fix the issues Emmett is experiencing. Unfortunately, BCH is out of network for our health insurance, MediShare. 60% of the cost of surgery is required upfront, and the estimated cost is between $200-300K. The soonest Emmett can have surgery is April. We would appreciate any and every penny contributed to help our family as we try to give our son the best medical care for a long and healthy life. Even though we are facing this giant mountain, God is going to equip us to climb it! He is giving Emmett a powerful testimony that many generous people will be a part of. We have no choice but to trust in His faithfulness, for we know that He is good, and He has a plan that is far beyond our understanding.

 “Blessed are the pure in heart, for they will see God.” Matthew 5:8 “So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand.” Isaiah 4:10 

 In-depth Plan from BCH: Given Emmett's symptoms and imaging findings, our team recommends further work up and surgical intervention at Boston Children's Hospital. We recommend a vocal cord check and visit with our otorhinolaryngology team to provide a baseline of his vocal cord functional assessment. There is a risk of vocal cord injury with this surgery, so a baseline vocal cord function is standard pre-operatively. Additionally, we recommend a 3D model to visualize his baseline anatomy in preparing for surgical planning. We would also recommend office visits with the Vascular Ring and Airway team providers, including cardiology, general surgery, gastroenterology, pulmonology, otorhinolaryngology, and feeding team. The surgical plan is to essentially rearrange his chest cavity for optimal function. There are many moving parts and will require several different pediatric specialists and/or surgeons working simultaneously. This surgical approach would be confirmed after review of the aforementioned preoperative studies. The expected length of stay is 2-7 days: 1-2 days would be spent in the cardiac IC or PACU and the remainder in the cardiovascular stepdown unit. Postoperatively, we would complete a vocal cord check and echocardiogram before discharge. We will see Emmett virtually for follow up appointments after his discharge from Boston Children's Hospital. These visits would occur at 7-10 days postoperatively, 4 weeks, 6 months and 1 year. If symptoms reoccur, we may recommend Emmett’s return to BCH for additional testing and/or intervention. If needed, we can coordinate post-op testing locally, if it is deemed appropriate. We also recommend follow-up with his local providers post-operatively. Our program and approach to these problems are unique. We focus on the patient as a whole and not just in an isolated problem. We will assess if airway or esophageal intervention is needed in addition to the vascular ring repair both preoperatively and intraoperatively, which is unique to Boston Children's Hospital. We also have a program established to allow for consistent, long-term follow up of these patients after surgery, which is important as we monitor both respiratory and esophageal symptoms post-operatively. * Notes from the attending doctors on this case: Dr. Christopher Baird (Cardiac Surgeon), Dr. Benjamin Zendejas (General Surgeon), and Dr. Ed Walsh (Cardiologist)