Bravery in BiPAP: How Angel Built Confidence Through the PAP Desensitization Clinic

Haga clic aquí para ver esta información en español.

Note: The interview with Angel’s family for this story was done with the help of a certified medical Spanish interpreter on Lurie Children’s staff. Quotes and information from the family were shared in Spanish and translated. 

Medical appointments can come with a lot of fears. Doctors, tubes and machines can feel intimidating — even more so when a child is told one of those machines needs to come home with them.

BiPAP (bilevel positive airway pressure) machines are essential for many children with muscular dystrophy. The disorder causes progressive muscle degeneration, and even respiratory muscles often weaken over time. PAP machines can help support breathing by providing gentle pressure to help the lungs expand during sleep.

For Angel, a 9-year-old boy with a rare type of muscular dystrophy called COL6A2 collagen myopathy, wearing a mask overnight was intimidating at first.

In the morning, Angel would wake up with debilitating headaches. His body was not breathing deeply enough during sleep, allowing carbon dioxide to build up overnight. This is a common symptom of collagen myopathy. When he was six years old, physicians at Ann & Robert H. Lurie Children's Hospital of Chicago Muscular Dystrophy Association (MDA) Clinic recognized this problem and prescribed a BiPAP machine to help him breathe more effectively while sleeping.

“Angel felt very scared at first,” said his mother, Angelita. “He didn't want to use it throughout the whole night because he felt like he was going to suffocate.”

About Lurie Children’s PAP Desensitization Clinic That’s where Lurie Children’s PAP Desensitization Clinic came in. Maria Gonzalez, Family Nurse Practitioner at Lurie Children’s, helped develop the clinic alongside pediatric psychologist Dr. Allison Clarke in 2023 after identifying a gap in care for children needing PAP machines. Often, once children were sent home with these machines, fear overcame need.

“There are so many kids struggling,” Maria said. “We began to research what we could do to help. What are the barriers to adherence?”

[Lurie Children’s Webinar - Understanding BiPAP: Why It’s Needed, What Gets in the Way, and How to Increase Compliance]

The team’s research revealed a list of both physical and emotional barriers, including anxiety or fear of wearing the mask, difficulty incorporating into routine, lack of resources for parents, mask discomfort and more.

After identifying these obstacles, they next researched ways to both mitigate challenges and monitor patient success. Each patient receives individualized treatment plans based on their specific barriers.

“The PAP clinic provides ongoing monitoring, adherence review, equipment assessment, pressure evaluation and early identification of therapy gaps,” she said.

Attainable Goals to Adherence

Angel’s treatment plan started with education on the device itself. After Angel and his mom reviewed the benefits of the device, Maria worked with them to create attainable milestones.

“We start with achievable goals,” Maria said. “We don’t want to overwhelm or discourage patients.”

At first, Angel began using the mask for short intervals. As he became more comfortable, they began working toward nighttime usage. If falling asleep with the mask was too difficult, Angelita would put the mask on Angel after falling asleep. Adjustments were consistently made to ensure proper fit.

“Maria made a lot of changes in regard to the masks until we found the proper one, the one that Angel felt safe with,” Angelita said.

Since they began working together three years ago, Maria has maintained regular check-ins to ensure Angel stayed on track toward his goals. Over time, and largely because of the trust he built with her, he steadily progressed toward consistent full-night use of the BiPAP.

“Maria always kept an eye on us,” Angelita said. “She would even give Angel acknowledgments whenever she would see that he was putting a little bit more of an effort.”

Courage in Recovery

In May 2025, Angel’s desensitization training became vital to his survival. After undergoing extensive orthopedic surgery for his collagen myopathy, complications during recovery led to water accumulation in his lungs. Angel had to wear his BiPAP 24/7.

“If he ever took it off, it would have been impossible for him to breathe on his own,” Angelita said.

During this time, Angel’s desensitization training was put to use. Even though the mask had been difficult for him in the past, Angel met the moment with courage. Maria said that Angel’s continual hard work with the clinic is exemplary.

“This case illustrates the importance of the PAP clinic as a continuity-of-care bridge between inpatient management and long-term outpatient success,” Maria said.

Now, Angel is recovering at home and back to only needing his BiPAP at night. His fears have dissipated, and his confidence has grown. Angel hopes that his story might help other kids feel brave enough to use their devices overnight, too.

“It's like his best friend,” Angelita said. “Whereas before he didn't want to put it on, now when we have to go to bed, he reminds me on his own.”