Johnny Youngwirth

Johnny Youngwirth - Embarking on a Journey of Care

There was nothing about Robin Youngwirth’s pregnancy to indicate that something would be wrong with her third child, Johnny. But each time the newborn grew tired and began to doze off, he stopped breathing and began turning blue.

Johnny has a rare disorder in how his brain controls his breathing, congenital central hypoventilation syndrome (CCHS). There is no cure for CCHS, and it lasts for life.

Because Johnny does not breathe on his own during sleep, he requires the use of a ventilator when he sleeps. To facilitate the breathing at night, Johnny was fitted as an infant with a tracheostomy so that he could live at home and be attached, each evening, to mechanical ventilation equipment to help him breathe.

Johnny lives a fairly normal life during the day, but the Youngwirths hope to provide a life for him where he is not connected to mechanical ventilators at night. That hope, and their extensive research brought them to our hospital where we have one of the world’s rare and unique diaphragm pacing programs.

Diaphragm pacing is a technique which uses the chiild’s own diaphragm as the “ventilator." It makes the lives of families of children who need mechanical ventilation at night much more convenient and streamlined, and, in some cases, permits  children to remove their tracheostomy.  

Now at age 5, Johnny is old enough to begin the process of using pacing to breathe at night. In October 2008, Johnny underwent surgery, the first step in changing the way he breathes during his sleep. 

Since then, Johnny has successfully begun implementing pacing and has been using pacing to sleep through a portion of each night. Over time, his caregivers will work with him to extend the portion of the night that he is using pacing until finally, he is able to use pacing for an entire night of sleep.  

More about Johnny's Story

Johnny Youngwirth Pacing and Johnny	Robin Youngwirth   Johnny's Mom
Thomas Keens, M.D.  Johnny's Pulmonologist

What Pacing Could Mean for Johnny

The first thing that diaphragmatic pacing represents for the Youngwirth family is the opportunity for Johnny to be free of his tracheostomy as he gets older.

Currently, he is able to participate in virtually every activity that his brother and sister do, but his family realizes that, as he becomes a teenager, the tracheostomy will be something that he is no longer happy about.

In addition, the Youngwirths are an active family. They like to boat and swim, camp and travel. Their ability to do this now is very limited. Being able to use diaphragmatic pacing offers them the possibility of being able to go on family trips without needing to lug an extra 50 pounds of bulky machinery, tubing, and equipment with them for Johnny to breathe during the night.

With the surgery successfully concluded, and pacing implementation initiated, they have a few more months to go before they will know for certain if Johnny’s pacing will “take.” However, if all goes well, they will be able to go anywhere that they want, travel, camp, and participate in outdoor activities. All that Johnny will need to bring with him on these family outings is his diaphragm pacing transmitter unit which (instead of being the size of two laptop cases)  is the size of two DVD cases and a few lightweight accessories.  

Johnny's Mom Shares Her Perspective

"When Johnny was very young, his diagnosis was very scary for us. When babies are small, they doze in and out of sleep all the time and you have to watch them vigilantly and make sure that their breathing doesn’t stop.

"Now that he is older, he requires less vigilant care because his sleep is more predictable, but he still needs assistance with breathing each night and during each and every naptime. 

"Once we got past the rigors of his youngest years with the ventilation requirements that he has, our family became involved with the CCHS Family Network, and we began looking into what his long-term quality of life might be. Children with congenital central hypoventilation syndrome (CCHS) often grow up to be adults that fit into society in every way with the exception of needing to have assistance with ventilation during sleep and when they are sick.

"We also found in our research that, for teens with CCHS, their quality of life is lower because they feel that the tracheostomy makes them stand out in a very obvious physical way from their peers.

"Just as any parents would, we want Johnny to have as normal a childhood as possible in a way that is safe for him. After educating ourselves by talking with other families, we began researching diaphragm pacing.

"I spoke to a few families with children who had undergone the pacing procedure. Each of the families we spoke with who had experienced successful pacing implementations expressed how much better their quality of life was, both for their child and for the rest of their family. After a lot of research, combined with a lot of prayer, we decided to try this option of ventilating for our son.

"When I informed Johnny’s pulmonologist that we were seriously considering exploring diaphragm pacing for him, he immediately recommended that we contact Dr. Keens at Children's Hospital Los Angeles. He shared with us that Dr. Keens is considered one of the world’s leading experts on this particular condition and on diaphragmatic pacing, having published articles on CCHS and its treatment. 

"We met with Dr. Keens and he graciously helped us review and evaluate our options. Not all children are good candidates for the pacing procedure so we worked with Dr. Keens to verify that Johnny was likley to be a good candidate for the procedure, educated ourselves further, and made an appointment for the first step, surgery to implant the phrenic nerve electrodes that stimulate the diaphragm to initiate breathing.

"Although we know that initiating the pacing will require a multi-step process, we are very thankful for the care we received at Children's Hospital. Dr. Keens and his staff were amazing about working with us and answering the multitude of questions that we had. Between the surgeons and Dr. Keens' staff, we were able to get every single one of our questions answered and carefully coordinate all of the details of initiating Johnny’s pacing process.

"The experience of the staff at Children's Hospital Los Angeles shows in the fact that Johnny was able to return home earlier than we anticipated. Over the years, Johnny has needed as many as ten surgeries for his care. We have come to expect longer recovery times than on average for Johnny because he tends to contract infections in the hospital, and for one reason or another he often needs more downtime than the average person undergoing a similar procedure.

"But, for his pacing surgery at Children's Hospital, we were impressed at how quickly Johnny was well enough to leave the hospital. Instead of a two-week stay, as we had originally prepared ourselves for, we were headed home after only four days."

Johnny's Pulmonologist Shares His Perspective

Dr. Keens' Medical Bio

• Anesthesiology
• Critical Care Medicine (PICU)
• General Pediatric Surgery
• Neurosurgery
• Pediatric Pulmonology
• Surgical Nursing

"The collaboration between the different disciplines is important and each member of the various teams is relied upon to carefully communicate with the others in order to provide the highest quality care.

"In Johnny's case, after surgery, he was moved to the Pediatric Intensive Care Unit (also called the PICU).  Once in the PICU, the Pulmonary Team coordinated the post-operative care with the intensive care team and the surgeons. 

"Johnny’s condition steadily improved and, within the week, even though he had undergone a major surgery, he was able to be discharged home from our hospital.

"Now that his tissues have healed after his surgery, we have been able to begin implementing diaphragm pacing. Currently, he is pacing several hours per night, and he is gradually increasing his pacing time.

"The goal is for Johnny to be able to use pacing all night to breathe. Once he has successfully concluded the process of comfortably sleeping an entire night using the pacers, we can plan to remove his tracheostomy. Our team is enjoying helping the Youngwirth family make progress towards their ultimate goal of having Johnny completely free of his tracheostomy and able to use pacing to sleep at night.

Dr. Keens' Medical Bio
More about our Diaphragm Pacing Program
More about our Division of Pediatric Pulmonology

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