In the first moments after Jack Muñoz was born, he did not cry out loud. The doctors at the hospital where she gave birth reassured his mother, Nakisa Muñoz, that Jack was healthy, but she had a strong feeling there was something wrong. Within five minutes, Jack was taken to the NICU for a serious problem in his digestive tract. During an emergency colostomy the next day, Jack received a breathing tube, which was placed with difficulty, and a feeding tube, which was placed so that Jack could receive nutrition.
Soon after the procedure, Jack was transferred to Lucile Packard Children’s Hospital Stanford. He was diagnosed with 22q11.2 deletion syndrome, a serious genetic disorder that can lead to a litany of health problems involving the airway, facial structure, digestive tract and immune system. Jack suffered from issues in all of these areas. As a result, he required several major operations during the first year of his life. He underwent surgery on his gastrointestinal tract and the aortic arch, one of the largest arteries in the body. Very early in life, Jack was also diagnosed with a web in his airway, making it impossible for him to breathe on his own. Jack’s parents were told that he would likely need a tracheostomy, a procedure during which a surgical hole is placed in the windpipe, in order to have his breathing tube removed. He stayed in the NICU for some time in the hope that his windpipe would grow on its own, but Jack ultimately underwent the procedure.
Though they finally had answers, Nakisa and her husband Michael were growing increasingly overwhelmed by Jack’s numerous doctor visits and the amount of time he had to spend in the hospital. They very much wanted to bring him home.
In the summer of 2014, three months after Jack was born, Douglas Sidell, MD, arrived at Stanford Medicine Children’s Health, where he connected with the Muñoz family. “When I met Jack and his parents, Jack was about a month and a half younger than my first son,” says Dr. Sidell. “I treat all of my patients like I would want my own children to be treated, but I probably had a heightened sense of the Muñoz’s fears and frustrations. I could tell that they were overwhelmed, and they were scared. It was very easy for me to tell them exactly what I would have needed to hear myself and to give them the time and space to process this information.”
Fortunately, Dr. Sidell was in an excellent position to help Jack, Nakisa and Michael. He had recently moved to Stanford from Cincinnati Children’s Hospital to create the Aerodigestive and Airway Reconstruction Center at Lucile Packard Children’s Hospital Stanford. Aerodigestive centers, which were first developed at Cincinnati Children’s Hospital, specialize in complex disorders of the airway and digestive tract. They are designed to coordinate care among many specialists in order to better manage difficult diagnostic problems for children with these disorders while simultaneously minimizing the confusion and complexity of the process for the patient and family.
“A patient like Jack,” says Dr. Sidell, “has a plethora of intertwined diagnoses and has taken countless trips to the operating room for various reasons. It’s sometimes hard for one person to figure out where to start. Our center allows multiple practitioners to focus on their own areas of expertise, but we are always aware of what the other care providers are doing. We are all together, in the clinic and the operating room, and we turn around and talk to each other. This isn’t just convenient care—it is better medicine, plain and simple.”
What most attracted Nakisa and Michael to Dr. Sidell was his approach to patient communication. For example, soon after she met him, Nakisa told him she wanted to try to feed Jack by mouth, a difficult request given his condition. “The day after I told him that,” she says, “I got a call from his office telling me that these are the things I needed to do and how to do them.” The aerodigestive and airway reconstruction team, including speech pathologist April Johnson, CCC-SLP, worked very hard with Nakisa to make sure she could feed him safely in a way that would improve Jack’s ability to swallow.
Feeding Jack by mouth was not without its risks. If not done carefully, it could lead to aspiration or infection. Because of this, and because of other problems with his lungs, they also met with MyMy Buu, MD, a pulmonologist associated with the Aerodigestive and Airway Reconstruction Center. “Dr. Buu met with me, gave me the equipment she thought I would need and trained me for any possible scenario. She did all the things she needed to do for Jack but without overburdening me as a mom.”
Eventually, through constant monitoring from the aerodigestive and airway reconstruction team and persistent work on Nakisa’s end, they were able to do more than just feed him by mouth. They were able to remove his feeding tube. For her part, Dr. Buu says it was a pleasure to work with Jack and the Muñoz family. “I often start patient visits with the question, ‘What matters most to you?’ Nakisa clearly identified her concerns, fears and wishes, so I was able to work with the team to provide her with the tools she needed to address her concerns and monitor Jack’s health.”
Nakisa also applauded the way in which this approach extended to the Stanford Children’s Health system as a whole. Because of the complexity of his genetic condition, Jack sometimes saw specialists outside of the Aerodigestive and Airway Reconstruction Center. During one surgery, Nakisa thought about calling Dr. Sidell to see if he would come. She decided not to bother him, but when they arrived, Dr. Sidell was already suiting up in the operating room and getting ready to insert Jack’s breathing tube.
Soon after the Muñoz family met Dr. Sidell, he told them that he could do the reconstructive surgery Jack needed so they could remove the tracheostomy much sooner than other doctors had initially told them was possible. But it would not be an easy process. Once, there was a question as to whether to give Jack a course of antibiotics to prevent infection. Nakisa was wary of the side effects. Dr. Sidell met with her, explained the risks and benefits, and allowed her to make the final decision. “He gives you tons and tons of information,” says Nakisa. “He puts you in a place where you feel comfortable making the decision despite how much you don’t want to make it.” Ultimately, she chose the antibiotics.
“My job,” says Dr. Sidell, “isn’t to tell parents what to do. It’s to provide them with the most up-to-date, accurate and realistic information about the child’s disease process and prognosis and to explain to them what we have to offer. We take everything into account, including their social situation, their child’s health status and any potential operations that the child may need in the future.” This way, he says, we can work with the family and the patient to find the treatment plan that will actually work well for them.
After Dr. Sidell performed the reconstructive surgery, it looked like a success. They waited to be sure, and sure enough, Dr. Sidell soon announced that the tracheostomy could likely come out within a year. This was more than Nakisa had ever hoped for. “There’s no way,” Nakisa remembers thinking. “This guy’s exaggerating so that I don’t get stressed out. But I was shocked. It really was about a year.”
The tracheostomy came out without a hitch, and Jack’s prognosis is excellent. “Jack still has his own set of obstacles, as many children do,” says Dr. Sidell, “but he also has great potential to live a long and fruitful life, and we will always be around for him if he needs us.” He now has his own voice, can eat by mouth and breathe without limitation, and his airway is expected to grow along with the rest of his body. According to his mother, he recently graduated from San Andreas Regional Services, where he underwent therapies that are helping him succeed, and he is now a thriving student at Mandala Preschool.
Learn more at http://aerodigestive.stanfordchildrens.org >
