Saturday, March 29, 2014

Living Life With Congenital Central Hypoventilation Syndrome

An article appeared today in "Things You Don't Know" that states, 

"Congenital central hypoventilation syndrome or CCHS is an extremely rare condition. Some consider it to be one of the most horrific conditions a human being could ever suffer from. The reason is that each and every time you fall asleep could be your last. The condition refers to a sudden stop of all respiratory functions while a person is deep asleep. More simply put the brain just forgets to tell the lungs to breathe."

Because this is the breathing disorder that Caleb is diagnosed with, I would like to respond to the article by
painting a picture of what life really looks like living with an individual with this disorder. 

Within the first 20 minutes of birth, Caleb was not breathing properly and was placed on a ventilator (a machine that provides him with breaths). Two weeks later, once we had the diagnosis of CCHS, a tracheotomy was performed and a trach  was inserted in Caleb's neck. The following two months were spent getting Caleb stabilized and then training Bob and me on all of the equipment so we could bring him home. 

Once home, Caleb was on the ventilator 24 hours a day and that meant that there were no quick trips into the community. Every trip was well planned and would need to include bringing the ventilator, back up battery, emergency bag along with the normal diaper bag. Initially I limited my store trips for when Bob was home, but over time I learned to navigate the store with all of the necessary equipment. Of course there wasn't much room left in the cart for groceries, but I felt a great victory in these outings. This experience was not horrific, but it was cumbersome and tiring.

During the past several years there have been many medical advances. Decades ago, individuals with CCHS would have had to utilize an Iron Lung. To me, that does seem horrific, but nowadays the ventilators are much more portable, the batteries are smaller, and many of our kids are implanted with diaphramatic pacers which stimulate their nerve to breathe. As Caleb grew, we were able to have diaphramatic pacers implanted and they took the place of day time venting.  The pacers gave Caleb great freedom, as he could run around and play like other children, without being attached to a machine. The first time Caleb was able to play in our backyard without the machine was a day I will always remember because or the gratitude I felt. 

The lack of normal response to shallow breathing  requires parents and caregivers to be ever vigilant. For children who do not use the ventilator during the day, the parents must make sure the children do not fall asleep while riding in the car. Children cannot be left alone to play in their rooms and as the children become teenagers, caution must be taken that the teens do not fall asleep while watching television or playing video games. 

Because of the need for a higher level of care and also the need for constant watchfulness, most families have nurses that come on a regular basis. From the time Caleb was discharged from the hospital, we had a nurse that stayed the night with us. The nurse remained awake because if Caleb disconnected from the ventilator, he could die. Caleb continues to have a nurse that accompanies him to school to care for his medical needs and a nurse must be at our house if we are to go out for the evening without Caleb. In Caleb's case and I believe most others, the nurse is paid for through a state waiver program or the insurance company. 

 Apart from the nursing care and the extra equipment, though , kids with CCHS are no different than your children. They laugh and love and enjoy a fulfilling life. Many accomplish much more than the medical profession ever imagined and continue to amaze all of us. So, although the author of this article was not able to share the stories of our amazing children that live with this diagnosis, I thank him for giving us the opportunity to make our story known.

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