Background: Kristen was not diagnosed with CCHS (Congenital Central
Hypoventilation Syndrome) until she was 2 1/2 years old. At that time it
seemed that she only needed ventilatory support during sleep. She was like
a typical pre-schooler in every other way. Kristen also has a very
atypical mix of Congenital Heart Defects, SEVERE allergies to all
antibiotics, and a mix of subtle yet significant speech and fine motor
skill delays. From the literature searches I'd completed I know it was
possible to ventilate young children noninvasively. With noninvasive
ventilatory support Kristen would have minimal infection risk, minimal self
esteem issues and no additional stresses on poor speech (oral) and fine
motor skills. Also considered was the knowledge that we were also
parenting another child with significant care needs and the lack of nursing
support. We opted for negative pressure ventilation as it is the only
system with over 50 years of proven success and minimal morbidity. Kristen
tolerated the system and it proved to effectively ventilate her. We did
trial BiPAP for 9 months, tandem with the Port-a-lung/NEV 100, but she
hated it, it was difficult to effectively ventilate her and she developed
chronic sinus/ear infections that were very difficult to treat. It became
apparent about one year ago that Kristen needed some level of daytime
ventilatory support to meet her needs as she became more active. Since the
Port-a-Lung is so restrictive we explored the other negative pressure
options. LifeCare's cuirass has a track record of complications in
children, so our physician wouldn't even trial it. I had learned of the
Hayek Oscillator prior to it's FDA approval and explored that system as an
option. Kristen trialed it last year and discovered it ventilated very
effectively, has a secretion management mode "built in" and Kristen loves
the way it feels when it ventilated. One clinical advantage to the
oscillator is the positive effect oscillcation has on the aveoli during
ventilation. Since using the combination of systems Kristen has had
basically no lactic acidosis and has experienced marked improvement in her
ability to function during the day. The other positive pressure systems
all require a trach and in the CCHS population have demonstrated
significant infections and soem lung injury. The downside to negative
pressure systems is their size/weight (they are cumbersome). The Hayek
Osciullator is awaiting FDA approval on a compact. lightweight model. That
is what we wanted to use in school. We will be getting another big
hospital based unit (at the request of the FDA) within two weeks for use at
school. This will be swapped with the smaller unit in the Spring. We
could obtain the smaller unit (at our expense) from London for use now, but
we just can't afford it. We have not experienced any problems with radio
frequencies affecting Kristen's vents since adding an UPS system in our
home. Hope we don't see that happen at school.
I have a current, comprehensive literature search and manufacturers'
information on the two diaphragmatic pacing systems. They seem to serve
the patients with spinal cord injuries very well. Children with CCHS,
however, experience a very high mortality and morbidity rate. The
hypothesis is that this is related to their normo-active state. They have
greater infection exposure and a high component breakage rate. After
reviewing it with our physician we decided that venting way that better
option for Kristen. He's hoping that within several years, technology will
catch up to our children's needs and then we'll be able to reconsider
pacing as a safe option for daytime use.
Ed- if you'd like a packet with all of the current information, send me a
snail mail address.
Cody- Negative pressure systems are actually significantly easier to use
than the invasive positive pressure systems. The downside of negative
pressure systems their size/lack of portability. In the past two years six
CCHS children have been transitioned to negative pressure systems,
decannulated and all are demonstrating a positive improvement in their
lungs on chest x-ray.
Like all things in life, everyone has unique needs which must be met in
ways that are best for the individual. For some people trachs and positive
pressure support systems are the best option, for others noninvasive
support serves their needs effectively. I think it is unfortunate that
many physicians neglect or fail to offer alternative ventilatory support
options to all of the individuals that are candidates for those systems.
Joanne Kocourek
The University of Chicago
jskocour(AT)midway.uchicago.edu