Some babies with TEF will also have other congenital problems, such as heart, kidney, vertebral, limb, digestive, and/or airway disorders, which are encompassed in the VACTERL condition. Brielle presented with heart and airway disorders alongside her TEF/EA.

Brielle’s airway condition is called Tracheobronchomalacia (TBM), which is described as compression and/or weakness/floppiness resulting in a narrowing of the upper and lower airways. Due to compression on her airways, Brielle has 90% collapse at rest and 100% collapse during cough or forceful exhalation (laughing, crying, breathing heavy after running/playing). This condition results in Brielle having noisy breathing, an ineffective cough, recurrent upper respiratory infections, and recurrent lower airway pneumonias. We use an aggressive airway clearance routine to help manage and compensate for the anatomical and functional deficits that the malacia airway places upon Brielle. On October 7, 2019, Brielle underwent the Posterior Tracheopexy, Anterior Tracheopexy, and Aortapexy surgeries, completed at Boston Children’s Hospital in an effort to provide her with open airways for easier breathing, improved activity tolerance, and to help prevent a future full of recurrent respiratory illness. Our airway clearance routine is still very important to maintain the health of her airway and lungs, but the surgeries definitely give her a better quality of life.

Brielle’s heart condition is called Hypoplastic Aortic Arch with Coarctation of the Aorta, which results in a narrowing of the main artery that takes blood from the heart to the body. Due to the narrowing, Brielle’s heart needs to work harder to pump blood to other parts of her body. Over time this can result in a thickening of the heart as well as high blood pressure, and so for this reason, a repair early in life is recommended. Brielle was able to remain relatively asymptomatic for 2 years to allow for her body to get bigger and stronger for such an invasive repair. She had her repair three days after her second birthday alongside her airway surgeries. 

Brielle also presents with dysphagia, which is an inability to protect her airway while swallowing. For this reason, Brielle uses a G-tube for primary means of nutrition. She takes nothing by mouth, but that does not mean that she doesn’t get all the nutritional benefits of eating real food. Brielle receives a blenderized diet through her G-tube in place of commercial tube feeding formulas. Brielle is fed whole food, fresh food that is blended to a smooth consistency and then put through her tube in order to promote health, growth, and healing. Though the notion of blenderized feeding is far from a "new" approach, as it has been used by families for many years, the widespread awareness of a blenderized diet in the world of tube feeding has greatly increased as of late due to ease of access to information via social media and internet blogs. The increased awareness has provided more parents and caregivers with options beyond the enteral formula recommendations typically made in the medical setting. Though special cases at times require special dietary considerations, the truth is, there is absolutely no reason real food couldn’t be the primary option and first thought recommended by medical professionals for tube-fed nutrition. Due to more tube-fed individuals and their caregivers switching to real food diets and and sharing their experiences with others around the world via internet, Nestle and Abbott commercial formulas came up with their own versions of “blenderized diet” formulas to try to appeal to this community. Later, Kate Farms released a "plant based" enteral formula. Indeed, they may have added a few real foods, but they first heated the very small amount of food at high enough temperatures that result in the majority of the benefits of the food being destroyed. “Real food” labels in their formulas quickly became merely an advertising scam. The reality is that the majority of the “nutrition” in these “real food formulas” is comprised of copious amounts of sugar (>55%!) and unhealthy oils that no one would ever eat orally all day, every day, for the reason that it would make them feel very, very ill. The purpose of a blenderized diet is to provide tube-fed individuals food that they would be eating if they ate by mouth. These Abbott, Nestle, and Kate Farms formulas are in no way a real food diet, so we steer very clear of any and all recommendations for these products. When we are unable to blend fresh, we opt for Functional Formularies (Nourish or Liquid Hope), Real Food Blends, or Whole Story Meals, as these three companies are currently the only prepackaged blenderized meals using whole, real food.

We do not know what other challenges Brielle will have to face in the future, but we feel very confident in facing them. The combination of having an amazing medical team at Boston Children’s Hospital, pursuing chiropractic care, choosing a healthy lifestyle with a fresh, whole, real food blenderized diet, and maintaining an intensive respiratory maintenance routine, Brielle will thrive in her growth, development, and her ability to overcome any challenge.