new medical equipment on its way. . .

Jack had his Pulmo appt. today to go over his sleep study and to discuss his daytime sleepy concerns. His appt. was in the Bellevue Clinic, this is a new Seattle Children's Urgent Care Clinic and Surgery Center, located on the Eastside. It was a beautiful building with lots of whimsical artwork throughout, looking much like a mini version of Seattle Childrens Hospital. (these were taken with my iPhone, I cant believe I left home with no SD card)

Jack was asleep when we got there of course but we were able to arouse him to his feet to to have him do the PFT. Despite the nurse needing to support him so he wouldnt fall backwards and trying it over and over, his last "blow" was good enough, showing his respiratory strength was (low) normal, but normal. Thankfully this hasnt gotten any worse since his last PFT 6 months ago.

Jacks Pulmo spent over an hour going over the sleep study and new treatment plan, which all came down to...

WELCOME TO BIPAP JACK.

Poor kid. Just how will I get our sensory, claustrophobic and "plastic smell" gagging boy to accept anything strapped to his face?  blah.

His sleep study showed only mild apnea, this included 11 obstructive hypopneas, 3 obstructive apneas and 5 central apneas. An index of 2.7 (last sleep study showed 3.4 index)

Sats only going as low as 92%.

(short version) Reads: Significant for CO2 retention with a mean tidal of 49 and a maximum of end-tidal CO2 value of 57. (normal values are 35-40)

So really a pretty mild sleep study in terms of apnea events. Less arousals and apnea events than in 2009. Its just the CO2 thats more of an issue, as I expected.

WHAT IS CO2 RETENTION?

CO2 retention is a pathophysiological process in which too little carbon dioxide is removed from the blood by the lungs. The end result is hypercapnia, an elevated level of carbon dioxide dissolved in the bloodstream. Various diseases may lead to this state; disturbed gas exchange may lead to impaired excretion of the gas. In addition, breathing air with a high carbon dioxide concentration may also lead to hypercapnia.

The principal result of the increased amount of dissolved CO2 is acidosis (respiratory acidosis) when caused by impaired lung function); other effects include tachycardia  (rapid heart rate) seizures, coma, and death.

Jack had a CBG (capillary blood gas) draw after his appt. I received the email from his Pulmo when I got home, His PCO2 was 50mm (definately too high) and his Bicarb was elevated to 28. These numbers could actually be higher but he didnt have the more accurate ABG (artery blood gas) as they are too painful for kiddos to do awake. But whatever they are, both of these numbers are enough to confirm his high end-tidal during sleep and possibly now during the day. SO he MUST be treated now before they keep rising.

Recommendation: Start Bipap at IPAP 12 and an EPAP of 6, using nasal pillow and/or nasal mask interface.

So we should hear from Home Health care in a couple days. He talked to a drowsy Jack about it (he slept through most of the appt.) and he just curled up his nose and said, no way and fell back to sleep. BUT Im going to try to stay optimistic that he will eventually get used to it, just may take a lot of time and patience.

We also talked about his sinking sats. He wants to see if the Bipap fixes that first before starting him on oxygen right away, which makes sense. He said we could easily add a line if we dont see his sats coming back up after giving the Bipap some time.

He also mentioned that some of his kids do use oxygen only to treat their apnea/Hypoventilation and do just fine with it, BUT they will only prescribe it with careful monitoring. Meaning a sleep study with oxygen to be sure he/she isnt showing an increase of end-tidal, along with blood gas draws to be sure their Bicarb isnt rising. BUT he did say this may be used as last resort if the child isnt tolerating the Bipap mask ect...very last resort, as Bipap IS the treatment for Hypoventilation.

 

I do have to add. He nor Dr. S. believe "all" this sleep is due to his CO2 retention. That he has had kids with much much higher numbers than Jack and they dont sleep like he does. Although he did add that Jack is unique and his body may just not be handling these numbers well.

He and the sleep specialist did discuss Narcolepsy but felt it didnt fit, and nothing showed that from his sleep study. But still theyre suspicious that its something else causing all this daytime sleep. If the Bipap doesnt help him with more alert days then they may want to do more testing or even try meds.

So it seems Jack has all his Drs scratching their heads. Naps and fatigue are one thing for Mito but sleeping hours and hours with no sign of illness isnt the norm.

So as I expected he needs this...and actually was going to fight for this time since he's been Hypoventilating for a couple years now... but Im down...just knowing that our little guy is going to have to get adjusted to yet another annoying machine connected to his body, its heartbreaking.  The next few weeks will be difficult, a lot of tears from both of us I imagine...life goes on.