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His whole life, David Vannausdle, 32, of Granger, Iowa, has been tethered to some type of breathing device for at least some part of the day or night.
Without external assistance, his body “forgets” to breathe. He was born with congenital central hypoventilation syndrome, also referred to as Ondine’s curse. Essentially, the brain fails to signal the lungs to breathe.
Thanks to a pacing device implanted at Mayo Clinic last fall, Vannausdle is breathing well on his own.
“When David returned for follow-up after his pacer implantation, he was bright and conversational,” says Ronald Reeves, M.D., a physical medicine and rehabilitation specialist at Mayo Clinic in Rochester, Minn. That contrasts dramatically with Dr. Reeves’ first meeting with Vannausdle. “Due to the high level of carbon dioxide built up in his system, he was so sedated he could barely talk with me,” he says.
Robin Vannausdle, David’s mom, says the device has been a life changer. “He can go places without having to take so much equipment,” she says. “David is more energetic and his personality has changed. He’s much happier.”
As a child, Vannausdle was tethered to a ventilator’s 10-foot hose all the time. As he got older, he used an oxygen tank during parts of the day and the ventilator at night. About 10 years ago, Vannausdle transitioned to using a BPAP (bilevel positive airway pressure) machine, which pushes air to open the airway. Last year, his hometown physician mentioned the diaphragmatic pacing system available at Mayo Clinic in Rochester.
The system is implanted in a minimally invasive procedure. Tiny electrodes are placed on the diaphragm, the main muscle used in breathing. Wires connect the electrodes to an external batterypowered device about the size of a large remote control. The implanted electrodes stimulate the diaphragm to contract and expand, allowing air to fill the lungs. Patients who use the system can reduce or end their need for a ventilator over time.
“All these years, I’ve thought this is just the way it has to be, with David hooked up to a machine at night. We never thought it could be this good,” says his mother.
The article comes from our Sharing Mayo Clinic print publication.
“He is the fourth person in the world with his condition to use this technology.” Not true. In 2008, I attended the CCHS Family Network Conference held in Orlando, Florida. At the conference, there were several people both children and adults, CCHS patients, using diaphragmatic pacemakers. My son has a condition similar to CCHS, called ROHHAD. He also has and uses diaphragmatic pacemakers.
These breathing pacemakers are remarkable. I definitely agree with that. My son’s life, too has changed for the positive since being able to use diaphragm pacemakers.
Thanks for your note. We have removed that statement and are checking into it.
This is a new device, to the knowledge of myself and the company David was the forth person to receive this new device for this problem. There is another device that requires a much more complicated surgery that has been around for over 30 years. David had the device placed as an infant, but it never worked well for him. I suspect the folks commenting are thinking of that older device. Dr. Reeves
There are many more children with CCHS that have the breathing pacer then just 4. My daughter had hers when she was 3
Thanks for the note. We have removed that statement and are checking into it.
Out of curiosity, who is the manufacturer of the pacing system?
The device is manufactured by Synapse Biomedical in Cleveland Ohio (http://www.synapsebiomedical.com/). It’s approved for use in patients with spinal cord injury and amyotrophic lateral sclerosis.
Amazing every breathe we take leads to Talk and intern progress in Technology…which leads to action! Thanks Staff of Mayo for keeping us all on the right track!
This is very interesting. Having been very involved with chairing the 2011 CCHS Conference I was under the impression that Avery Biomedical was the only phrenic nerve pacer manufacturer that was FDA approved. Is this new product FDA approved and if so, how long? There are many CCHS patients that the Avery pacers have not worked for and for various reasons. This could be a very important new option for them!
We are passing your question on. Thanks for the interest.
Dr. Reeves responds: “This is a very sophisticated question. The Avery system you mention has been around for many years. As you also mentioned the Avery System isn’t a perfect solution, and some patients aren’t successful with it. The system implanted for David to use is new, and not approved for use for central hypoventilation. The device is approved for use in Spinal Cord Injury and Amyotrophic Lateral Sclerosis. Because it works so well in those patients we thought it could help David as well. To our knowledge he’s only the fourth person in the world to receive this system for congenital central hypoventilation. We’ve been very pleased with its results thus far. It’s possible that other patients with central hypoventilation might be able to benefit; however, there haven’t been any research studies to prove that. Hopefully, we will be able to do that in the future.”
Hi,
My Name is Jeffrey and I’m 43yrs old.
I’m staying in Singapore..
I’m curious as the i’ve just popped over to this website a while ago.. 28th Oct 2012 4pm (Singapore time)..
Would appreciate if I could get some statistical data of how many CCHS cases has been success and been able to use your product as of now Oct 2012..
This is because I’ve a daughter who was diagnosed with CCHS or ROHHAD syndrome in year 2005 and is currently still on a ventilator support for 24hrs a day since year 2003.
Hopefully, she can have your company endorsement to try it and help to establish your product in Singaporeor south East Asia..
kindly advice..Thanks!
Hope to hear from you soon!
Regards.
Jeff
Jeffrey, Thanks for your note. We’ll forward it to the appropriate team here.