My 17 month old daughter (with complex medical issues) has had problems falling asleep since Feb 2005 which were only diagnosed as Sleep Apnea after a Sleep Study in December 2005.  She grimaces, huffs, jerks and cries while falling asleep but then sleeps quietly for hours once she has fallen asleep.

Expert Sleep Doc (founder of the sleep clinic) said her tonsils were small but said they were probably blocking a very small airway, given the size of her facial structures.  We saw an ENT (recommended by the Expert Sleep Doc) who did a nasal scope and told us Brooke's tonsils & adenoids were small but she wouldn't recommend surgery because her sleep apnea is mild and surgery likely wouldn't help her sleep apnea because T & A are probably not the cause. Called our attending (taught by the Expert Sleep Doc) told him what the ENT said and checked to see if CPAP titration study was truly the next step.  Attending said to meet with Expert Sleep Doc again with another Surgeon they work with for 2nd opinion because the first ENT may be being cautious due to the fact that my DD is under 2 years old (she did say come back in 6 months) and may be assuming there's something behavioral going on that should be fixed first before the physical (which he thinks is unlikely because she struggles to fall asleep even in our arms).  She's had 3 sucessful surgeries since birth so if there is a good chance it will help we'll put her through another one.  Her Sleep Specialist insists that a tonsillectomy was the best treatment option for her right now because she was likely to fight the CPAP nasal mask and it might not fit well enough to avoid leaks, which would make it less effective overall. He also reiterated that CPAP was only an overnight treatment, whereas surgery might improve her daytime sleep as well. A second ENT listened to these arguments but after hearing her history also voiced his concerns that she was an anesthesia risk. Besides our immediate desire to improve her quality of life, I expressed our concern that other risks, such as an increasing red blood cell count secondary to chronic reduced oxygen, were developing as a result of her Sleep Apnea so ultimately we'd like to do whatever treatment is most effective for her. A recent printout of my DD's lab results since May had indicated a gradual upward trend, the opposite of what would be expected with kidney failure, and additional lab tests conducted a couple weeks ago had ruled out other possible causes. While her history and treatment options for her Sleep Apnea were discussed, DD again tried to nap (3 hours after her last attempt, like clockwork). She jerked, huffed, and cried in my arms giving her Sleep Specialist an excellent opportunity to explain her movements to the ENT and giving the ENT a chance to see her airway occluding as she tried to sleep. Witnessing her difficulties, the second ENT stated that he did think surgery would help her but he was not sure an anesthesiologist would be willing take on the risk. The meeting ended with the ENT's plan to talk to an anesthesiologist about our DD's situation. Meanwhile, we had our CPAP Titration Sleep Study. My DD did not mind the noise or the nasal mask when it was placed gently on her face but complained loudly when it was strapped tightly to her head. She also had some trouble adjusting to the air going through her nose, opening her mouth and letting the air come out her mouth instead of letting it travel down her throat to keep her airway open. The technician figured out that she was having trouble exhaling through her nose against the air pressure and lowered the air pressure until she was able to breathe with her mouth closed. With the CPAP nasal mask in place and the air flow continuing, she continued to complain loudly and jerk but surprised me by falling asleep early at 11:30pm (instead of her usual 1or 2am). Unfortunately, however, she had trouble when the air pressure was increased once she was asleep, requiring a chin strap to help her keep her mouth closed and avoid a leak. Multiple times throughout the night I woke up to her coughing or fussing because the nasal mask had slipped and was blowing air into her eyes.

Other than her earlier bedtime I was not so sure CPAP made a difference, having witnessed firsthand many of the concerns voiced by her Sleep Specialist. However, I received a call from a Physician at the Sleep Clinic saying after reviewing the results it appears that CPAP did improve her sleep efficiency (amount of time she is asleep after lights out) increasing it from 63% to 83%. Therefore, we changed her interface to nasal pillows that would not cover her nose and would not leak into her eyes.

I just received my 17 month old Brooke's first CPAP titration study report and saw why they recommended the CPAP for her.  Her Respiratory Disturbance Index went from 16.6 to 3.2 on CPAP and she went from having 90 obstructive hypopneas to 22 on CPAP.  What is the difference between an apnea and hypopnea?  I also discovered she had 4 central apneas (in both studies) when in REM sleep that I didn't know about before because they said she had OSA.  Should I be concerned about the central apneas?  

Also, the recommended pressure was established when she was fully asleep and most of her hypopneas occur in the first stage of sleep, just as she's starting to fall asleep.  So now I'm concerned that it's not enough to help her.  During her trial of CPAP at home she still fought the nasal pillows, but definitely less than she fought the nasal mask.  When she stopped  fighting she had quiet moments because we have a ResMed machine that lowers the pressure when she exhales so she didn't pool her saliva like she did during the titration study.  It's a good thing I pushed for it because the report showed that the tech had put her on BiPap temporarily (didn't know that when I pushed for the CPAP machine we have) when he told me he had reduced the pressure so she wouldn't fight exhaling.  But she still jerked and cried (her hypopneas make her do that) for a while before finally going to sleep.  Then an hour and a half later she was wide awake smacking her lips so I took the nasal mask off, figuring we may have to ease her into it, and she fell quickly back to sleep without any jerks.  Her recommended pressure was 8. How long would you recommend trying this pressure before contacting her Sleep Doctor?  Does it take some time for the recommended pressure to start working?  Or should I expect that the recommended pressure won't stop all her hypopneas but reduces the number to a better range?  To be honest, it's going to be hard to justify using CPAP if it doesn't stop the jerking/yelling that brought us into the Sleep Doc in the first place and is part of what we are trying to get rid of to improve her quality of life.  As I put her to sleep I'll have to repeat the mantra "it's improving her oxygen sat" over and over again.

This is all so new to me so I appreciate any input you can provide to help me understand this treatment.  

Thanks in advance for your input.


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube

Hi norcalmom:
Well, let me toss out a few thoughts as they relate to your questions.

Quote:

He also reiterated that CPAP was only an overnight treatment, whereas surgery might improve her daytime sleep as well.

She should wear the CPAP during naps, too.  Especially since the sleep quality is better.

Quote:

...she had trouble when the air pressure was increased once she was asleep, requiring a chin strap to help her keep her mouth closed and avoid a leak.

If there is so much trouble with mouth leaks, perhaps trying a full-face apparatus might help.  Respironics Simplicity (a very small adult mask) might be the right fit, there are two sizes.  The trouble with small children, there's not a big selection out there, you kind of have to make do.

Quote:

CPAP did improve her sleep efficiency increasing it from 63% to 83%. Therefore, we changed her interface to nasal pillows that would not cover her nose and would not leak into her eyes.

But if you've got something that is working, on the other hand I wouldn't be in a huge hurry to change things.

Quote:

Her Respiratory Disturbance Index went from 16.6 to 3.2 on CPAP and she went from having 90 obstructive hypopneas to 22 on CPAP.

What you need to get is the titration table.  This will show if there are some pressures that work better than others.  But if 3.2 represents the average for the night, that's pretty good.

Quote:

What is the difference between an apnea and hypopnea?

An apnea is an 80% or more reduction in the airflow.  A hypopnea is a 50-80% in airflow that also creates another issue, like an oxygen drop or a disturbance in sleep continuity.  Small children tend to have mostly apneas, and not many hypopneas, but there's no law that says you can't.  Got the study results for arousals and desaturations?

Quote:

I also discovered she had 4 central apneas (in both studies) when in REM sleep that I didn't know about before because they said she had OSA.  Should I be concerned about the central apneas?

Central apneas in children are frequently a normal phenomenon in REM.  They can be up to 20 seconds long and still be considered normal (although I think that's pushing it).  But if they're not causing oxygen desaturations, they're probably not a worry.

Quote:

Her recommended pressure was 8. How long would you recommend trying this pressure before contacting her Sleep Doctor?  Does it take some time for the recommended pressure to start working?  Or should I expect that the recommended pressure won't stop all her hypopneas but reduces the number to a better range?

The results should be pretty much immediate.  Getting her used to the CPAP is the thing of the greatest importance, so I wouldn't be pushing things too quickly.  You sound like you're doing a fantastic job.

One thing, tho, that you touched on.  If the pressure you're on is still subtherapeutic (and it's not clear if that's the case), then BiPAP may be what you need.  It'll give the little burst of pressure to overcome the remaining hypopneas (if there are any) but allow baseline drop to minimize mouth leaks.

Quote:

To be honest, it's going to be hard to justify using CPAP if it doesn't stop the jerking/yelling that brought us into the Sleep Doc in the first place and is part of what we are trying to get rid of to improve her quality of life. As I put her to sleep I'll have to repeat the mantra "it's improving her oxygen sat" over and over again.

OSA in children is rated severe if the AHI is over 10.  So you're making great headway here.
sleepydave

Thanks so much sleepdave!  I really appreciate your efforts to answer my questions.  Let's see if I can clarify some more by responding to your responses.

sleepydave wrote:

If there is so much trouble with mouth leaks, perhaps trying a full-face apparatus might help.  Respironics Simplicity (a very small adult mask) might be the right fit, there are two sizes....But if you've got something that is working, on the other hand I wouldn't be in a huge hurry to change things.

When she's awake she almost seems to forget that the SNAPP small nasal pillows are strapped on and smiles at us with it on.  She fought the nasal mask the minute it was strapped to her head.  So I'm assuming she felt too claustrophobic with the nasal mask and would feel the same way with a full mask.  She has a chubby little face so the petite Respironics nasal mask didn't get a good fit and leaked in the eye area any time she shifted in her sleep.  Needless to say we both had a rougher night's sleep during her CPAP titration study because I kept having to adjust it.  Last night I was able to tighten the straps of the nasal pillows enough (we actually had to have a friend re-sew the headgear to make it fit) while she was awake and happy so that it stayed on all night even when she shifted in her sleep.  Last night's problem was leaking through the mouth though but I did figure out a way when she was on her side to tilt her head forward and put a weighted lap pad behind her head and that kept her mouth closed.

sleepydave wrote:

What you need to get is the titration table.  This will show if there are some pressures that work better than others.  But if 3.2 represents the average for the night, that's pretty good....The results should be pretty much immediate.  Getting her used to the CPAP is the thing of the greatest importance, so I wouldn't be pushing things too quickly.  You sound like you're doing a fantastic job. One thing, tho, that you touched on.  If the pressure you're on is still subtherapeutic (and it's not clear if that's the case), then BiPAP may be what you need.  It'll give the little burst of pressure to overcome the remaining hypopneas (if there are any) but allow baseline drop to minimize mouth leaks.

Looking at the titration table and the CPAP and BILEVEL pressure graphs were what made me think 8 cm isn't enough.  

Her BILEVEL pressure as she was falling asleep and crying/jerking between 10:10pm and 11:15pm (when the tech told me he lowered the pressure so she could exhale easier) was 5/4 and she had 10 hypopneas Sa02 high = 97.0, SaO2 low = 80.0, SaO2 mean = 93.6.  

The CPAP pressure resumed at about 11:15 at 4.5cm then worked it's way up to 7.5cm at 2am: 1 Central Apnea, 2 hypopneas, Sa02 high = 100.0, SaO2 low = 90.0, SaO2 mean = 97.5;
Then up to 9cm around 5am: 2 Central Apneas, 1 hypopnea,  Sa02 high = 100.0, SaO2 low = 97.6 SaO2 mean = 97.4.  

To someone who doesn't know her regular sleep pattern, it does look like she is much improved at 9cm.  But she rarely wakes up once she's finally asleep and can sleep quietly without snoring for 6-8 hours.  Her crying/jerking (which I've been told are her crying to breathe) only happens in the first few hours as she's falling asleep.  If she wakes up at all later she easily falls back asleep without any crying/jerking.  Her typical pattern is crying/jerking from 10pm - 1am then she'll sleep quietly from 1-9am, unless she's sick anyway.  That's why it took so long to diagnose her sleep apnea...Everyone (including our Neuro at the time) assumed she'd have trouble all night and wouldn't sleep so quietly most of the night if she had sleep apnea.  

So I think the pressure was set when she typically does better even without the CPAP on.  Last night, for example, she started falling asleep at 11pm and cried/jerked until 1am then slept with only a couple brief yells during that period until 9am (CPAP was on from 10:30 to 7:45am, when she started her usual waking up coughing/vomiting and I was concerned she'd vomit into the nasal pillows (sometimes liquid will come out her nose).  She fell asleep again with just a brief patting of her leg and a few times I just heard a change in the sound of the machine which made me realize her mouth was open again.  

sleepydave wrote:

 Got the study results for arousals and desaturations?

I've got a page of graphs that it's hard to interpret verbally or otherwise.  She had 13 awakenings and from the graph, the majority were before 12am and the rest (4) were between 12am and 7am.  

AWAKE her SaO2 mean = 95.1, SaO2 min = 88.0, Sa02 max= 100.0,  
NREM  her SaO2 mean = 96.5, SaO2 min = 80.0, Sa02 max= 100.0,
REM her SaO2 mean = 97.7, SaO2 min = 93.0, Sa02 max= 100.0  

sleepydave wrote:

OSA in children is rated severe if the AHI is over 10.  So you're making great headway here.

What's AHI?  We have been told she has a mild case of sleep apnea but besides how awful it is to witness her crying/jerking while trying to fall asleep, because of how fragile she is (due to her kidney failure) chronic mild desaturations are causing her kidneys to excrete more epogen which in turn creates too much hemoglobin, which I think puts her at risk for other medical issues.  

Thanks again for your time.  Your post has really helped me understand a little more about my daughter's sleep study results   .


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube

Quote:

(Edited, somewhat)
BILEVEL pressure as she was falling asleep and crying/jerking between 10:10pm and 11:15pm was 5/4 and she had 10 hypopneas SaO2 low = 80.0

The CPAP pressure ...11:15 at 4.5cm ...up to 7.5cm at 2am: 1 Central Apnea, 2 hypopneas
Then up to 9cm around 5am: 2 Central Apneas, 1 hypopnea  

To someone who doesn't know her regular sleep pattern, it does look like she is much improved at 9cm.  But she rarely wakes up once she's finally asleep and can sleep quietly without snoring for 6-8 hours.

She had 13 awakenings and from the graph, the majority were before 12am and the rest (4) were between 12am and 7am.  

NREM  her SaO2 min = 80.0

What's AHI?  We have been told she has a mild case of sleep apnea

Well, got a scanner?  Scan both reports and send 'em in.

BTW, did they monitor End Tidal CO2 level?  This is quite important in youngsters, and may give us a clue about more than just a few things.

If what you're kinda asking is could this be a sleep hygiene issue, sure.  Is she on medications that might affect sleep onset?

When we look at the first study, if she can put together solid sleep w/o respiratory events in the latter part of the study, then that also may say something.

Only hypopneas, and no obstructive apneas, in a child remains somewhat curious to me, but we'll leave that aside for now.

AHI is apnea-hypopnea index, which considers only those two entities.  RDI, respiratory disturbance index includes arousals causes by subtle respiratory events, or RERAs.  Children can often sleep through their events, so I'm not sure if people really rely heavily on RERAs or just look simply at primary snoring to make their decisions in those cases.

Arousals are different from awakenings, that's under a different category.

If the centrals are normal REM events, or simply follow an arousal or an awakening and are a response to a brief period of hyperventilation, then they are not an issue at all.  Subtract them from the calculations entirely.

Within the titration table there should be an RDI, or AHI, for each pressure as well.  That's how you find the ideal pressure.  And again, reduce that number by the harmless central events.  So if what you're asking is, "There is still 1 hypopnea at the therapeutic pressure", that may not be a lot statistically, and still, it's not an apnea.

And oh yeah, the reflux, G-tube, etc.  You must have a pretty good GI guy, you're not feeding too much, too late, I would assume?

For some considerations in what central and obstructive apneas look like, if you haven't looked over here yet;

A Few Examples of Pediatric Sleep Apnea

Desaturations to <90% will perk up everyone's ears in children as well.  But it would be good to see when they occurred.  During respiratory events or periods of wake fussiness.

The somewaht subjective classification of OSA in children varies somewhat from place to place.  Let me c&p:

Quote:

Although there is no consensus on what constitutes abnormal values in childhood OSA, the values are considerably lower than that associated with adults.
Generally, anything over AHI 1.0 is considered abnormal.
Our categories are mild AHI 1.0-5.0, moderate at 5.0-10.0, and severe >10.0.
Desaturations in children are uncommon.  Or put differently, the presence of desaturations raises concern.

When you ask about the ETCO2, ask what they use for scoring criteria.

Quote:

You should be using missing 2 breaths, and not, for instance, the 10 second minimum that you use in adults.

Again, just the curious me.
sleepydave

I'll try and get that data to you (for your curiosity   ) but in the meantime here's more answers to your questions.

sleepydave wrote:

BTW, did they monitor End Tidal CO2 level?  This is quite important in youngsters, and may give us a clue about more than just a few things.

They measured her CO2 for the diagnostic study.  Summary page says her PCO2 Max was 34. They did not measure it in the titration study.

sleepydave wrote:

If what you're kinda asking is could this be a sleep hygiene issue, sure.  Is she on medications that might affect sleep onset?

The two big seizure meds she's on are Klonopin and Lamictal.  She was on Phenobarbital from birth until December 2005 because of a history of seizures at birth.  Her last "official seizure" was in Sept 2005 but her EEG remains abnormal so she is at risk for seizures.  The Klonopin and Lamictal were added in May and June of 2005, respectively, because at that time it was thought that her jerking/crying might be seizures or hypnic jerks that would respond to these meds.  After 3 EEGs (including a 48 hour EEG in October) indicated that these events were not seizures we weaned the Phenobarbital and are now in the process of very slowly trying to wean her Klonopin (nasty addictive drug, starting with 1/3 of her dose over 13 weeks), because of it's tendency to cause breathing issues.  She will stay on Lamictal as a preventative measure.

sleepydave wrote:

And oh yeah, the reflux, G-tube, etc.  You must have a pretty good GI guy, you're not feeding too much, too late, I would assume?

We do four small meals (around 5 oz) every three hours between 9 and 6:30pm...although sometimes if we're running late it can as late as 8:30pm.  She starts getting tired around 10pm and sometimes as late as 11ish and then she is put on an overnight continuous pump feeding at a very slow rate (1.5 oz/hr) when she's finally calming down and closer to being sleep usually around 12:30am which runs until about 7:30am.  Whether we finish feeding her at 6:30pm or 8:30pm it doesn't seem to have an effect on her sleep problems.  However, she has real trouble with delayed gastric emptying when she is sick so we are in the process of changing her Nutramigen (hypallergenic infant formula) to an Elemental child formula that should be even easier to digest (because it's mostly amino acids). So I'm hoping if there is any connection that should help.

Thanks again for your patience.  She is a complicated baby...so I greatly appreciate your efforts to consider the relationship between her sleep problems and her other issues.


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube

norcalmom wrote:

The Klonopin and Lamictal were added in May and June of 2005, respectively, because at that time it was thought that her jerking/crying might be seizures or hypnic jerks that would respond to these meds.

I guess if the sleep onset issues were there prior to the start of the medications, that kinda lets them off the hook, at least for now anyway.  That woulda been too easy.

norcalmom wrote:

They measured her CO2 for the diagnostic study.  Summary page says her PCO2 Max was 34. They did not measure it in the titration study.

The kids with significant OSA tend to run higher pCO2's, in fact, exceeding certain limits will send you to surgery, like if your pCO2 is >50 mmHg for >8% of sleep.  So that maximum of 34 mmHg is extraordinary...

...except if you had a metabolic acidosis, which is common in such things as, say, chronic renal failure.  Then you hyperventilate to try to correct the acidosis.  So, dead end again.  You'd think the acidosis and/or electrolyte imbalance could be considered as a contibutor to the jerks, but if that were the case, the twitches would occur all the time, not just for a couple hours at night.

If she were 2 years older, boy you'd think Restless Leg Syndrome, especially given the other things that may accompany RLS, like renal failure.

How's the iron level, BTW, in the youngest of RLS cases the iron was was low.  Or anemia.  But an increasing red blood count?  The oxygen sats don't seem severe enough to generate polycythemia.  Any PLMS on the study?  And in a child you don't need many.  On the other hand, if it were RLS/PLMs, the Klonopin should've put a dent in it.

But this is somewhat of a sidebar, what we're really trying to find out if the respiratory events are a result of severe sleep disturbance, rather than the cause.  Maybe the graphs and other info can shed more light.

sleepydave

Sleepydave,

Sorry for the delay in responding...I've had two sick kids at home this past week...by the way...does CPAP help nasal congestion/runny nose?  

Anyway, to answer more of your questions...

sleepydave wrote:

...except if you had a metabolic acidosis, which is common in such things as, say, chronic renal failure...You'd think the acidosis and/or electrolyte imbalance could be considered as a contibutor to the jerks, but if that were the case, the twitches would occur all the time, not just for a couple hours at night...If she were 2 years older, boy you'd think Restless Leg Syndrome, especially given the other things that may accompany RLS, like renal failure.

She is on a Sodium Bicarb Supplement because her blood is too acidic as a result of the kidney damage at birth.  I asked her kidney docs about her jerking because I randomly came across the link between RLS and kidney failure.  However, they said her kidney function (about 28%) and electrolytes do not suggest RLS.

sleepydave wrote:

How's the iron level, BTW, in the youngest of RLS cases the iron was was low.    Or anemia.  But an increasing red blood count?  The oxygen sats don't seem severe enough to generate polycythemia.  

I don't know her iron level but she's still on an infant formula that has iron in it.  No anemia...her hemoglobin is borderline high along with RBC, MCV, and hematocrit (which was also high at birth due to the hypoxic intrapartum event).  Her Sleep Specialist said even if her O2 Sats are only 90% but a chronic 90% might cause those levels to increase...especially when you consider the fact that she tries to sleep every 3 hours during the day.  

sleepydave wrote:

Any PLMS on the study?  And in a child you don't need many.  On the other hand, if it were RLS/PLMs, the Klonopin should've put a dent in it.

The Klonopin only helped reduce myoclonus (a movement disorder that looked like startles but they are not triggered by anything...nothing like her movements in response to her apneas). No PLMS on either study. I PMed you the raw data to review.  Let me know if it provides any new insights.

Thanks again!


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube

OK, erin sent in a ton of info, there's a few excerpts. The top graph is from the diagnostic study, the lower 3 are from the CPAP titration.

Just to mention, the sleep needs of children are probably 25% more than what people think they are, not that anything can be done here.

Total sleep time is reduced, a 17 month old needs about 13.5 hours of sleep total, counting nap time.  And for this little gal, trying to keep up with a significant acidosis all day long (shoot, all night long, too) she needs every minute.

As erin had noted before, the sleep architecture in the CPAP is considerably better than the diagnostic.  Oddly, the slow wave sleep (SWS)(stage 3 & 4) is extremely reduced in the CPAP titration, darn near non-existent.  I don't know why that is, the diagnostic study had a good quantity.  So perhaps that's not a concern and should normalize.

You can see in diagnostic study that most of the events occur in REM, so that's where you have to make sure the CPAP works.
There's no arousal table, you have that?

The Mean SaO2 in the diagnostic study was 96.4%.  There's a few desats, but frankly I don't think that's enough to explain polycythemia.

Awright, so now we have to look in the CPAP titration table to find a pressure that takes care of all the events in REM.  There are two, 7.5 and 9.0.  I wouldn't even count those centrals.

Quote:

they said her kidney function (about 28%) and electrolytes do not suggest RLS.

I saw the electrolytes that you sent, (-HCO2 18, BUN 24, Creatinine 1.35).  Does RLS start at the moment of abnormal kidney function?  No, just that there is an increased incidence in CRF.  The problem, of course, is that RLS is a symptom, a complaint offered by the patient.  And unfortunately, your DD can't tell us that.  Or maybe she can.  Yet, the pharmacological treatment of RLS in a 17 month old, let alone with CRF, is something that you really have to think carefully about.

But the whole thing might be academic, if it was RLS, again, you woulda thought that Klonopin would've done something.
sleepydave

I'm back...and sadly my absence did not mean we were getting sleep...still no help from the CPAP...lower pressure of 6.6 cm reduced the crying/jerking time but she was still having 24 hypopneas an hour so we had to increase it closer to the recommended pressure (8cm) to 7.6cm.  The minute it was increased she started yelling for 2-3 hours again.  Add to it the fact that our slow Klonopin wean has given her more tone so now every morning at around 5-6am (about 4 hours after she's finally quietly asleep) she rustles and rubs her face, pulling her nasal pillows off and down around her neck.

Kidney docs did an iron study...her levels came back fine.  However, her RBC, MCV, Hematocrit, and Hemoglobin are still on the rise.  Right now her major kidney issues are high magnesium and low vitamin D production (causes Parathyroid Gland to secrete its hormone and remove Calcium from the bones because it thinks that there isn't enough Calcium being produced).  They're not sure why her Magnesium is high but we're getting her Parathyroid Hormone under control with Vitamin D supplements.  She finally grew again so we're closer to controlling it.

Really starting to wonder if this is just sleep apnea or if something like "hypnogenic paroxysmal dystonia" either alone or with her sleep apnea is what's making it hard to treat.  Her Neuro said given her brain damage some sort of parasomnia might be a possibility and I have an appt next month with her Sleep Docs to discuss this possibility and her treatment...because I'm soooo ready to give up on the CPAP.  Surgery is definitely not an option...learned from the second ENT that in addition to her anesthesia risk there is concern that her airway is so small that the swelling post tonsillectomy will cause additional breathing problems.  

If there is any chance that she's just fighting it and CPAP will work for her...how long does it take a child to get used to CPAP?


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube

Hi erin:
I'm sure everyone is sorry to hear that you're still having so much trouble.  Dealing with all these issues, while both of you must be sleep-deprived, must be a real mountain to climb.

CPAP tolerance varies with everybody, and trying to predict compliance with an 18 month old with those additional issues, especially the inability to take sleep, would be pretty difficult.

The thing about parasomnias, including nocturnal dystonia, is that they are generated out of sleep.  And except for maybe the Rem Behavior Disorders, they come out of NREM sleep.  So if the issue is, "I put my child down, and they start fussing and crying for 3 hours", parasomnia doesn't jump to the top of the list.

And all the events pretty much occur in REM (AHI 53.6 vs 4.3 in NREM), sleep is generally maintained, so the issues of OSA and sleep initiation and maintenance appear to be quite distinct.

Ever get those arousal numbers?  It would be nice to get a closer look at those REM periods.  But since you have a ton of events in the diagnostic study, and they're gone on CPAP, it's gonna be tough to argue that the CPAP has no benefit.

Ok, so how about looking at two things.

Based on those sleep studies, and perhaps by your history, insufficient sleep is a major issue.  You've got to get to 13.5 hours.  How much total sleep do you think she's getting, including naps?

Put both sleep studies up on the computer screen.  That's Sandman-Melville software so I know it can be done.  Look at those REM periods and see exactly what the CPAP is doing.  Look for arousals and desats.  By those graphics, there doesn't appear to be a significant change in oxygenation, but that doesn't necessarily mean we're free and clear in a small child, all I'm saying is that if you had significant desats, there'd be no question, you need the CPAP, surgery, something.

We're also keeping in mind that these are hypopneas, not apneas, and she has a somewhat abnormal breathing requirement to begin with (that underlying metabolic acidosis) but to repeat, the before and after is distinct and dramatic.

But anyway, when you're looking at the waveforms side-by-side, one of you (DD or physician) is going to say, "Aha, now I see what CPAP is/isn't doing" and you can plan your next step from there.
sleepydave

BTW, how did you get this number?

Quote:

she was still having 24 hypopneas an hour so we had to increase it closer to the recommended pressure (8cm) to 7.6cm.

Are you reading off the machine?  That's a huge change from where you were at.
s.d.

SleepyDave,

All the numbers I gave you are all the numbers I have...so I guess no arousal numbers.  The new 24 hypopneas per hour was data from the machine...told to me by our Respiratory Therapist who provided the equipment and manages compliance issues related to the use of the CPAP.  To be honest, that number seemed unusually high...given that her sleep studies said she had 90 hypopneas total but when I pressed it he said she averaged 24 per hour for the use of the machine (?).  So I'm guessing maybe in the studies she was on the higher pressure when she was already asleep so had less hypopneas than she did on the higher pressure while falling asleep (because she's fighting the air pressure?)?  

We've also noticed she's very gassy so we think she's actually swallowing large amounts of air.  She will wake after she's finally quiet with a loud cry then I'll start moving her legs around and she will let out lengthy gas sounds (sorry TMI) which is out of character for her.

She doesn't nap at all anymore.  She tries every three hours during the day but after being asleep for no more than 10 minutes she starts crying and jerking (because of her hypopneas I'm guessing) and then she ends up waking herself up.  She isn't getting more than 7-8 hours sleep a day total.

I don't know much about parasomnias but I was thinking maybe they started after she is woken by her hypopneas as she's falling asleep...and that's what causes all the yelling...because she seems to be asleep for a couple minutes before the whole mess starts...but I know I'm grasping at straws here...


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube

Hi erin:

Quote:

So I'm guessing maybe in the studies she was on the higher pressure when she was already asleep so had less hypopneas than she did on the higher pressure while falling asleep (because she's fighting the air pressure?)?

Had to think about that for a sec, but if what you're saying is that much of that 24 hypopneas per hour is artifact, that would be my first guess, too.

And hypopneas again, never apneas.  In a youngster, especially with an airway so small that she's considered a surgical risk, that's odd.  But I mumbled that before.

Based on the info that you have, tho, there doesn't seem to be a lot of support for sleep onset hypopneas which in turn create a myriad of other problems.  As a matter of fact, there's almost no hypopneas in NREM, period.  You could even argue that the couple in NREM in the first study might be artifact as well, cause there's prolonged NREM period with no respiratory events.

Are you using the CPAP during naps as well?  That should definitively answer the hypopnea issue during naps.

If one were to ignore all the other medical issues for a moment, and you were to walk in and say

Quote:

My baby has trouble falling asleep, gets fussy and cranky and BTW sleeps 7 hours a day when she really needs 14

There'd be no question what everybody's response would be.  Gad, that's gotta be creating some problems. Even it's not the primary cause, it's gotta be contributing.

What the drug list now?  I wonder if that Lamictal is giving her a headache.

That back of the book stuff can be intriguing, but...

...oh what the hey, here's my back of the book idea.  At the beginning of both studies, there are moderate to severe oxygen desaturations.  But if someone is jerking, jumping, etc, then this is usually dismissed as artifact.  Especially, since once she settles down, the oxygen saturation becomes quite normal.  But now you're saying that the polycythemia continues.  If it's due to hypoxia, it can't occur during sleep, she's fine there, even without CPAP.  But if she desats with the least amount of exertion (IOW, those oxygen desaturations, or at least some of them, are NOT artifact) then perhaps adding supplemental oxygen during the day can stop the shortness of breath associated with the drops in oxygen level.  Might even promote sleep.  Severe shortness of breath is a great way to generate anxiety.  Measuring daytime oximetry is easy enough, and most oximeters have a signal quality indicator to rule out poor signal quality.
sleepydave

SleepyDave,

I really appreciate you trying to sift through the details with me...it's all so confusing...as a stranger I'd think the lack of sleep is the main issue and that she's just fighting sleep but then she doesn't even sleep well in your arms...she has the same issues no matter how she is held...the huffing, grimacing, jerking and crying starts no matter what you try to do...so I don't think it's behavioral but it is something physical.

Because she tolerates the air pressure fine with the nasal pillows on when she's awake I'm having a hard time understanding why all hell (excuse the expletive) breaks loose when she starts to actually fall asleep.  The first sleep study confirmed our hunch that it started during a phase of sleep because the time when she started to fuss was very close to the sleep onset time.  The second sleep study was a different story because she fussed the minute the nasal mask was put to her face.  She doesn't use the CPAP while napping because she doesn't have a regular naptime (seems to be trying to catch up on her sleep all the time) and by the time she starts to show signs of being tired we can't get her CPAP machine warmed up etc. in time before she has already woken herself up from whatever it is that is disrupting her ability to fall asleep.  I think she must be getting something restorative from her fussing while falling asleep because she should be a lot more irritable given what little total sleep she gets.  She only fusses when she's trying to fall asleep.

I don't think the average 24 hypopneas/hr are artifacts because it looks like a breathing issue from the outside...at least it made sense when the movements were explained as a breathing issue.  When she's fussing while falling asleep she will snort/huff and then push her tongue out really far and it looks as though she's trying to get it out of the way.  If she's on her side, she will start twisting her chest and start throwing an arm away from her chest (Sleep Specialist said she's trying to open an airway) and then the jerking and crying starts.  But the hypopnea only issue is still strange.  It seems to me like something is causing her to hyperventilate/panic while she is in the early phases of sleep and that's what causes the fussing...and that's why I thought maybe a parasomnia.  It really seems like the higher air pressure makes things worse while falling asleep not better.  I have no idea if it helps overall though and if the average 24 hypopneas/hr are gone...I just sent the data to the Respiratory Therapist last week and he has yet to get back to me.  

Right now she weighs 20 lbs 10 oz and she's on 10 mg 2x/day of Lamictal along with 2ml 2x/day + .5ml nightly of Klonopin (1mg per 10ml suspension).  We increased her Lamictal in October/November/December when we began weaning her Pheno and it actually stopped some seizure-like movements (that weren't seizures on an EEG) she was doing when she was getting tired and after she awoke that involved her bringing both her legs and arms simultaneously toward the center of her body and which caused a crunching that used to make her vomit whatever was in her stomach.  We started weaning her Klonopin in mid-January (used to be on 2.5ml nightly) but it is really slow so it will be more than 9 months total before she is fully weaned.  She has become more emotional since we started weaning the Klonopin...makes happy sounds more often and seems to work herself up more intensely too. But I'd expect that as she becomes less sedated in general stuff would bother her more.

Everytime anyone has ever placed an oximeter on her during the day (and it's usually when she's not fussing) her levels are fine 99 etc. so no one has ever mentioned her daytime oxygen levels being an issue.  

By now you may be experiencing why I really wish she could tell us what disturbing her so I must thank you again for your patience and support.  Our attending is not in the office this week or he'd be getting an earful too.


_________________
Erin
Mom to 3 children 4 and under!! including a sweet 2 year old who has chronic kidney failure with severe cystic encephalomalacia, cortically blind, mixed cerebral palsy, severe global delays, Sleep Apnea, possible RLS, reflux, G tube