8 years old, my son, Josh was happy, healthy and very active. He loved sports and dreamed of being a football player when he grew up. All of a sudden, though, for no apparent reason, he couldn’t stay awake to do homework or even any of his favorite activities. Into the bath to wake him long enough to finish up some homework, then wrapped in a towel, he collapses on the floor right behind me.

That was the beginning of a new life for him and our family. That night he suffered full respiratory arrest in the hospital and was revived 5 times. Numerous tests over the next several months ruled out disease after disease and left one last syndrome to blame for his symptoms. In fact, the syndrome is named after the group of symptoms – ROHHAD, which stands for Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation. For some unknown reason my son could no longer sense the need to breathe adequately. His body stopped producing hormones to make him grow, regulate his body hydration and metabolize food correctly.

Three days before his 9th birthday, my son had a surgery to insert a tracheostomy tube, so he could connect to a ventilator to breathe for him. At first, doctors thought he might only need it when he slept. But more in-depth testing a year later showed he was ventilator dependent 24 hours a day.

Josh still had those dreams of playing football – along with any other sport other kids were playing. But not many sports allow for the use of a ventilator. Everywhere we went, we had to lug along his ventilator. He even had to use that ventilator to breathe for him on his plane ride home from the Chicago hospital where he went for his testing.

We heard of a device called diaphragm pacing systems that doctors hoped would mean Josh might no longer need a ventilator or trach – so he could achieve his dreams. We were directed to a group of doctors in Houston, who first wanted to put Josh through testing to see if there was any other possible procedure that might cure him of his disorder. Months of testing by all their specialists, and they finally gave up and agreed to implant the diaphragm pacing systems – made by Avery Biomedical. That was the summer of 2009, just before my son turned 13 years old.

About a month after his 13th birthday, after he’d healed adequately from pacer surgery, his pulmonologist, Dr. Kelly Smith, turned on the pacers in his office. Josh was his first diaphragm pacing system patient. He decided to have Josh use the diaphragm pacing systems for just a short time each day at first. But built up to hours and hours per day on the pacers and off the ventilator. Now, for several years, Josh has used his diaphragm pacing systems all his waking hours.

Now, we don’t even want to imagine life without the pacers. Josh has so much more freedom with them. He bowls, goes mini golfing, fishes, competes in special Olympic events and much more. He can’t quite play football or other full contact sports – because we don’t want to chance damaging his implanted pacemakers. But he has some new dreams – including becoming a chef.

As a senior in high school, now, as he nears his 19th birthday, he is in culinary arts – and PE. He also enjoys learning to work with computers. He has so many hobbies and interests that are so much easier to enjoy with the help of his diaphragm pacing systems. And we are so very glad to still have him with us. We know each day is a gift.

 

Written by Vanessa J Wooten, RRT (mom) Note: In August 2013, Mrs. Wooten became a Registered Respiratory Therapist working in home health care.

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