I photographed Brielle the day before her heart surgery and two days after her second birthday in the Boston Public Gardens. Her mom Trisha had gotten in touch with me that week before requesting a lifestyle shoot of her family. In the initial email, she shared that her daughter was having heart surgery at Boston's Children's Hosptial and that is why they were traveling to Boston from Pittsburgh.
Even though this was a lifestyle shoot, there was no style as you put on style, our time together was very much real life and a window into their daily interactions, which includes parenting a child that wants to keep moving, explore her world and takes frequent meals throughout the day through a tube. Because we didn't get into the details of Brielle's condition until after the shoot, I will save Trisha's part of the blog and Brielle's story for after the pictures, so you can experience Brielle as I did when photographing her, simply as she is.
The one thing I had wanted from Brielle was a hug. After meeting her I began to learn that probably wasn't going to happen. I struggled a little bit putting aside my wants and accepting reality. At the end of the shoot, Trisha and I talked and she shared Brielle's medical condition and we also discussed Brielle's choice approaches to communicating and socially/emotionally connecting with others. Brielle was within listening distance most of the time. When it was time to go I told Trisha what I wanted was a hug and say goodbye to Brielle. Trisha asked Brielle if she would give me a high five. Brielle was about 10 feet away and turned to me and did something she had not done the entire shoot. She locked eyes with me and when she got close she raised her hand but it wasn't my hand she wanted to connect with it was my lips. She pressed her little hand into my lips and looked right away and said everything she could say with that. I understood it all.
by Trisha Paronish
On the weekend of October 4th, we gallivanted around Boston in celebration of Brielle’s 2nd birthday. Her Dad and I took in the beautiful views of the cityscape while Brielle made sure to collect as many falling leaves as she could fit into her tiny toddler hands. While we were happy to spend her birthday in one of our favorite cities, the reason we had made the trek from Pittsburgh that weekend was in preparation for Brielle’s heart and airway surgeries scheduled for that upcoming Monday. We laughed, we played, we sang, we wrestled a toddler who was dead set on joining the ducks for a brisk swim at Boston Common. We set up the weekend to divulge in as much “normalcy” as we could muster up, but like most of Brielle’s experiences in her short 2 years of life, something medical is usually intertwined.
Brielle was born with two primary medical conditions affecting her esophagus and trachea, known as Tracheoesophageal Fistula (TEF) with Esophageal Atresia (EA). TEF/EA is a condition where the esophagus, the tube that normally carries food from the mouth to the stomach, does not develop properly. The upper portion of the esophagus ended in a pouch. The lower portion of the esophagus came up from the stomach and abnormally connected to her airway. She had her primary repair surgery on day 2 of life to correct the condition which involved disconnecting the lower portion of the esophagus from the trachea/airway and attaching it to the pouched upper esophagus. With surgical correction, her esophagus is now wholly intact; however, the birth defect leaves her predispositioned to ongoing complications that will require a lifetime of medical monitoring, medication, and medical procedures.
Child without EA/TEFChild with EA/TEF
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. The blenderized diet community has been making waves in the world of tube feeding in the last 5+ years (Your child can eat real food through a tube?...what?). But the truth is, there is absolutely no reason it shouldn’t be the primary option and first thought recommended by medical professionals. Due to the increased awareness of tube-fed individuals and their caregivers switching to real food diets, Nestle and Abbott commercial formulas came up with their own versions of “blenderized diet” formulas. 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 and Nestle formulas are in no way a real food diet, so we steer very clear of any and all recommendations for these products.
We do not know what other challenges Brielle will have to face in the future, but we feel very confident in facing these challenges because we have an amazing medical team at Boston Children’s Hospital, we choose a healthy lifestyle with a fresh, whole, real food blenderized diet, and we maintain an intensive respiratory maintenance routine that, combined, will make a big difference in her growth, development, and ability to overcome any challenge.
If you have a child born with TEF/EA, you will learn how to stay strong and positive. Your inspiration will come from the strength and fight that your child is innately born with. It is not easy. Brielle has undergone 5 major surgeries, 15+ procedures, countless testing, and imaging, and participates in multiple therapies to support her development; but, she continues to grow and grace us with a smile and laughter each and every day.
After her posterior, anterior, and aortapexy, as well as her open heart hypoplastic aortic arch reconstruction, Brielle continues to get stronger every day. We were discharged 7 days after surgery, which really shows her strength and resiliency. We went to Fresh Pond Lake Park 2 days before her surgeries and she needed to be carried 90% of the length of the walking path and needed a total of 3 naps that day. Twelve days after surgery we went to the same park again and she walked the entire path from the time we left the parking lot until we returned. Also, she fought her SINGLE nap of the day when we got back to the hospitality home. Like many others on here and around the world, BCH changed our lives and gave Brielle a better quality of life. We have some challenges post-operatively that we will navigate, but like she has proven before and will prove again, she will overcome it all.