Growing Up with CCHS

Shortly after Jason Sloan was born in Peoria, Illinois, in 1977, he was diagnosed with Congenital Central Hypoventilation Syndrome (CCHS), a disorder of the autonomic nervous system that affects breathing. The most recognized symptom of CCHS is the inability to control breathing that varies in severity, resulting in the need for life-long ventilatory support during sleep in some patients or all the time in others. There are estimated to be 1000–1200 cases of CCHS worldwide.

“When he was born,” recalled his mother, Debra, “I thought that whatever was wrong with him could and would be fixed by all the medical personnel. I was a very young mother and had no clue that my life was about to change drastically forever. I still remember my Jason being born that day. He was so purple and hooked up to tubes and everything. A few days later they called in our pastor to give last rites. Remember, I'm a young mother. What did I think would happen? Again, I thought he would be ‘healed’.”

Debra left the hospital after seven days without her son, traveling nearly 30 miles each way, every day, to sit in the rocking chair next to Jason. “Placing my hand thru that incubator, trying to talking to him, trying to kiss him, trying to comfort him...then I would make the long journey home, try to sleep and get back up the next day and start over.”

In early 1978, Jason was moved to Children's Memorial Hospital in Chicago. His doctor there, Carl E. Hunt, MD, made the suggestion to implant phrenic nerve pacers. This would allow Jason greater mobility and freedom. At the time, they didn’t expect him to develop the ability to speak or even eat independently. Though the surgery was successful, eight months later one of the pacers became infected and required surgery to replace it. As his mother explains, “No one knew what to expect at the time as this was such new technology. But the surgery was successful.”

Jason, with mother, Debra

Jason spent most of his time at Children’s Memorial over the next four years before coming home for good in December 1981. At that time, Jason was using a Puritan Bennett MA-1 ventilator, which Debra called “a blessing” due to its portability. Prior to that he had used an older Emerson ventilator. “It was a dinosaur, even at that time,” she said. “It would break down from time-to-time, and we would have to go to the hardware store to fix what we could.”

Once home, Jason flourished. “He went to school, made friends, participated in the Special Olympics. We were trying to live a normal life, but what is ‘normal,’ right?” said Debra. “When he was little, Jason tried so hard at his tasks and chores and always wanted to please others by doing his best. Teeth brushing? He should get a trophy! He was an expert at that. He was so cute because he always had to show me his results. I started that little scenario (checking out his work), but he really enjoyed getting the praise for a job well done.”

Adulthood brought new challenges. “There were no adult programs in place. We met with a few, and they said they wouldn’t take Jason, as he was on a ventilator,” said Debra. “You can imagine my response after hearing that on more than one occasion over the years.”

Jason required 24-hour monitoring. When he was 23, his phrenic nerve pacers finally stopped working. “They didn’t know if we had burnt out the phrenic nerve or if it was scar tissue. I made a hard decision not to put Jason through another surgery,” said Debra. The decision, though, meant he would need to use his ventilator and supplemental oxygen fulltime.

About eight years ago he was diagnosed with pulmonary fibrosis, which meant the pacers would not have been adequate even if they were still working. In addition, Jason has a seizure disorder, developmental disorder, interstitial lung disease, congestive heart failure and cerebral palsy.

Debra has taken precautions to make sure they are prepared for emergencies. “If the power goes off, so does the oxygen concentrator and the ventilator. The suction machine won’t work, etc.,” said Debra. “We installed a whole-home gas generator so there would be no more worries. I highly recommend that to families. And it seems like the power always goes out at nighttime. Jason has round-the-clock caregivers. They either work an 8-hour or a 12-hour shift, but there is always someone in the home, even if it’s myself or my husband.”

The financial burden can be considerable. His mother worries about the shortage of nursing/caregivers, medications, medical supplies and specialized food. She pays almost $500/month for special OTC supplies, other medical supplies, supplies and gloves for the workers, and special hand soap, among other items. If they need extra help with Jason, that comes out of pocket. “What will happen to Jason, when the money runs out?” wonders Debra. “That is a fear and keeps me up at night.”

Covid has brought additional worries. Both Debra and her husband contracted Covid earlier this year. The residual effects have made it more difficult to attend to Jason’s needs. Two of his caregivers have also tested positive. Luckily, Jason has avoided it thus far, but still they worry.

Yet they persevere and are thankful for the time they have together. “His smile is infectious,” said Debra. “He is a fighter. He is my hero.”