We recently started implementing CPAP therapy with Zella, our four year old with ADNP syndrome. Although the following probably only appeals to an incredibly tiny segment of the population, I thought I would share some of the information we have gleaned in going down this road less traveled.
Zella’s History with Obstructive Sleep Apnea
Zella has mild to moderate obstructive sleep apnea. She was first diagnosed as a two year old after a particularly interesting (read: HEINOUS) sleep study. (Shoutout to Jake for suffering through that one.)
At this age Zella was averaging about 2 apnea events per hour of nighttime sleep. A sleep study one year later at age 3 further confirmed this diagnosis with similar results. But it wasn’t until age 4 that we decided to embark on our CPAP therapy journey.
Step one involved a consultation with one of the area’s premiere sleep medicine physicians, Dr. Dominic Gault of Greenville, South Carolina. (Dr. Gault has seen a handful of ADNP patients and thus we consider him to be a resident ADNP specialist.) A few weeks after the consult, Zella headed to Greenville’s pediatric sleep lab and had her third sleep study, giving us surprising results. Her formerly mild sleep apnea had worsened to become moderate. Zella was now averaging 5.5 apnea events per hour.
Dr. Gault and his nurse walked us through what should have been an incredibly stressful phase of life: beginning CPAP therapy with a nonverbal, intellectually disabled toddler. I can think of roughly 3,784 things I would rather do including, but not limited to, cleaning my bathroom floors with a toothbrush.
Lessons Learned Starting CPAP Therapy
- Equipment matters, especially for pediatric CPAP patients. Double and triple check your durable medical equipment provider to be sure they provide exactly what your doctor ordered. The wrong tubing, mask, or headgear could render CPAP therapy useless for your child.
- Don’t rush desensitization. We desperately wanted to start Zella wearing her mask all night long from day one, but I’m glad we followed our doctor’s advice and introduced the mask, tubing, and machine gradually. Good things really do take time.
- Reach out to the experts. Dr. Gault’s RN, Anne, was an amazing, seasoned resource for us. She provided tips and tricks that helped alleviate Zella’s non-compliance issues.
- Always go to your child’s scheduled appointments with their sleep doctor. I know I know. Common sense, right? But this is worth mentioning as it is an integral part in insurance continuing to cover your child’s machine and supplies. If you don’t show up and check in when scheduled, insurance will refuse to pay for CPAP therapy, stating there is no proof the child is consistently using the machine.
- Plan to spend plenty of quality time in your kid’s room. Stay until they fall asleep. Trust me. We are nearly 8 weeks in and still doing this ritual. Once we place the mask on Zella and zip her into her safety sleeper, Jake or I sit quietly next to her without making eye contact. Thanks to half a clonidine at 6pm every evening, it takes Zella anywhere from 20 minutes to an hour to fall asleep. Needless to say, we do quite a bit of web browsing and game playing. Eventually we hope the bedside sitting routine will grow to be unnecessary. For now, though, Zella will remove and disassemble the mask if we leave before she is asleep. Oh, the joys of this process.
- Research your CPAP machine and download any available apps. Most modern equipment will communicate with your doctor via WiFi. Zella’s machine also has a convenient patient app where we have access to many of her statistics. I can tell everything from the number of hours she successfully wore her mask to the level of air leakage to the number of apnea episodes she had on a particular evening.
- Deviate from the plan for special occasions with no guilt. We have a couple excellent regular babysitters who know the drill when it comes to Zella’s CPAP therapy. Sometimes, however, we will have a new sitter. In these instances we have no problem letting Zella go to sleep without her mask as it makes life far less complicated. When Jake and I get home, one of us will covertly put the mask on her. She does wake up, but generally goes right back to sleep within about 10 minutes. Your nightly routine should make life easier – don’t let yourself become a slave to it.
The Best Advice
DO NOT BEAT YOURSELF UP OVER THE BAD NIGHTS. Because yes, there will be bad nights.
So many of them.
Some evenings your child may keep their mask on all night and have almost no leaks. You will cheer and yell and stop people on the street to tell them about this somewhat bizarre and yet incredibly important milestone.
And then there will be those nights where somehow, despite near constant monitoring, the kid randomly removes their mask a mere 2 hours after going to sleep. Wailing and gnashing of teeth will ensue. And you will question your approach. Example: Zella has weird nights where she insists on sleeping on her face. It is awesome. And her statistics look *great* on those days. Even still, we have been told that Zella has better compliance than many adults on CPAP therapy.
And so we keep on keeping on, as should you.
Above all else, know you are doing your absolute best. At the end of the day, that is all any of us can do.