Desperate Mum to son with UARS seeking advice

[BunnyMummy]

We went to a and e (er) last night. It was such a struggle as he felt so exhausted. Again he had been sweaty during the night. They checked bloods, urine, blood pressure, heart with ecg and listened to his heartbeat which sounded regular, all fine! They 'couldn't interpret' the charts I printed and I explained about heartbeat rising at night.They always seem annoyed we have gone to a and e. I stupidly didn't focus on lungs as I was worried after reading about afib so focussed on his heart. I should have asked if he could see someone for lungs. However I don't think they have specialists there overnight anyway (?) so he wouldn't have seen someone there and then. Going to take the charts to general practitioner and ask for him to be referred to pulmonologist. The problem is they have sleep person writing to them suspecting CFS so don't know if he will listen to me. One thing. A shot in the dark maybe. My Mum had sarcoidosis when I was a child, is there any way that hyperventilation, with the sweating and exhaustion could point to that? Apparently it can be hereditary....

[BunnyMummy]

He was diagnosed with UARS by home oximetry from sleep specialist, which showed huge variarions in heart rate and extremely fragmented sleep. I actually paid to see him private first appt we were so desperate. With his physical appearance of small narrow jaw, overbite etc as explained in original post, and videos I had of him sleeping showing noisy breathing, leg twitching, breathing getting lighter and lighter then awakening....
For the last 1 1/2 months I have been waiting for this magical time cpap makes you better which I read can be a while. Then when I started looking at Oscar I noticed the breathing waveform, how much he is still waking up, and have read the machine he is on is not good at picking up subtle variations in flow, hence meddling with pressures. I have read so many stories from people who are incapacitated by UARS, fatigued so badly they cannot function, and assumed diagnosis was correct but treatment inadequate. I'm not doubting what you are telling me about the hyperventilion at all and am SO grateful for your insight, but can I just clarify... The flow limitation in stats shown on that machine, would that be accurate? I will update more when I see primary care doctor about the hyperventilation. If I can get him to send us to a and e with a letter we could go straight to pulmonologist. Thank you all for your concern, it means a lot!

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He has no chest pain. Just breathlessness on exertion, he says it feels different from athsma. He says he knows he is hyperventilating when he is in bed, but rarely in the day, only when stressed and anxious.

[Sleeprider]

First, I'm glad you got the assessment, and my worst suspicions were incorrect. Symptoms seemed to fall into place, including the sweating and exhaustion at the a and e, and out of caution it's best to get the evaluation.

Let's focus on the possibility of UARS and do the charts with Events, flow Rate, Pressure, Flow Limitation and Leaks. We did not have a flow limitation in the graphs posted yesterday, and we need to see how they relate to the periods of higher and lower breathing volume. The summary statistics suggest a median flow limitation range of 0.07 to 0.13 and 0.23 to 0.26 maximum. These are moderate flow limitation and your son is already using EPR 3, which is the same as pressure support. UARS can have debilitating effects, but is is usually seen as high flow limitation with longer inspiratory times and severe flattening of the respiratory flow rate. None of those was present on the charts yesterday. In addition to putting up a new chart showing the whole night with flow limitation, I want to see some 2-minute segments (zoomed images to approximately 2-minutes) where we can see the shape of the flow rate wave. Looking a low-flow section and a high flow may give us a clue on whether it is flow limits leading to hyperventilation.

[BunnyMummy]

As I have the luxury of being able to see when he wakes, these are two snippets which I was in the room for, observing him sleep. He had been sound asleep, I was very quietly sitting on floor and I had to wait about ten mins for something to happen, so I definitley didn't cause him to wake.
He went from sound asleep to wide awake, and I can tell looking from the flow limitation at the same time, that it is low then.... I will do some more later showing higher flow limitation, just posting these off the top of my head as I know they are definite abrupt awakenings from sleep.

Thank you!

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Last one

[Sleeprider]

The sleeping wave-form at 10:31:40 shows normal sleep pattern breathing with moderate inspiratory flow limitation followed by an abrupt respiratory disturbance or arousal. The breathing then appears to have characteristics of expiratory flow limitation. The sequence starting at 97:42 appears to have normal sleep pattern respiration with a possible small snore at the inspiratory peak with an abrupt disturbance or arousal at 07:43:45 and again what appears to be severe expiratory flow limitation.

I have no expertise with this condition, but since you said your son has used an inhaler, this appears like a sudden onset of asthma or perhaps a physical positional issue. There is no way we can know the cause or mechanisms with just CPAP data. It's obviously a huge problem for sleep, and can induce a sense of being unable to catch a breath, because the lungs are expanded and he cannot exhale freely.

Hopefully his doctor can help you to understand, however I would not go in with the assertion that he has flow limitation or needs a machine other than CPAP. Carefully explain the symptoms and difficulties your son is having, and show him examples like this of the onset of these respiratory anomalies. The doctor is more likely to analyze this and suggest possibilities if you don't tell him what you think the problem is. I hope the doctor will be willing to take him into a sleep study and try to determine what is going on, and what might be the cause. Our previous recommendation to try to obtain an appointment with a pulmonary specialist or possibly an otolaryngologist (ENT) to evaluate any pulmonary condition or airway obstruction that might explain these problems. You can ask the sleep consultant if these referrals are appropriate.

This issue is outside the usual scope of CPAP or BiPAP therapy, and you are going to need medical expertise to diagnose and treat it. I wish I could help, but this is truly a complex problem we can't evaluate on the forum. The problems are real, and perhaps our best role is to help you to prepare a presentation and questions for your physicians. I think we can continue to support you and help put any findings the doctor makes into context, but I have no solutions with regard to CPAP or BiPAP to suggest; this is just too complex and unsual. Will be glad to look at additional examples and see if it provides more insights.

[Geer1]

Thank you. They will test him with his machine on and I will advise technicians. I just hope they are more understanding and open to variables than the consultant! He says he has never had a patient on bi level and scoffed at the idea... So I doubt they will do that. He is highly recognised in this field and has thousands of patients, so I can't believe my son is the only one shoved on a straight apap to be suffering...

I don't get how someone can be highly recognized in this field without patients on bilevel. It sounds like this guy is living in the past. Resmed titration protocol(and other titration protocols) focuses on EPAP to stop obstructive apneas and IPAP to deal with hypopneas, flow limitations and RERA's. Patients that feel they are having breathing issues on CPAP/APAP (trouble exhaling etc) are recommended to try bilevel because it allows for larger IPAP while maintaining low EPAP. Pressure support, PS, is the difference between EPAP and IPAP (IPAP - EPAP) and ia larger pressure support increases flow which is what helps deal with the flow limitations (it is the PS, not IPAP that helps). 

The fluctuations in tidal flow in your son look like hyperventilation and having just gone through something similar (although only one episode while asleep) a couple nights ago I can tell you it is no fun and I was very tired yesterday. My issue was due to a semi plugged CPAP mask vent which would be worth checking for. As sleeprider has stated this kind of hyperventilation is not common and it should be checked out, as mentioned a pulmonologist with knowledge of sleep disorders would probably be best but that will likely take some time.

Chronic fatigue syndrome is something I have been looking into quite a bit. It is diagnosis by exclusion and in order to eliminate other potential causes you need to treat them or prove they are not problems (although too many doctors are quick to jump the gun...). Your son hyperventilates numerous times every night and this is not currently being diagnosed or treated. He cannot have a diagnosis of CFS until the cause of this hyperventilation has been determined and dealt with and I would make this very clear with any doctor/consultant you are dealing with. 

While we are on that topic you said your son had a home sleep study with oximeter and it was determined that he has UARS. Did the home sleep test equipment also have a nasal cannula, chest belt or any other equipment? Do you have the sleep study results and would you be willing to post it (black out any patient or doctor information)? I ask because UARS cannot be determined by oximeter alone. UARS is imo somewhat improperly defined as being a disorder of sleep disruption(arousals) due to disordered breathing without apnea or hypopnea being present, in other words it is commonly accepted as a disorder where AHI is less than 5 but the patient is having numerous arousals due to trouble breathing. The theory behind UARS is that upper airway restrictions cause flow limited breaths which result in a buildup of CO2 levels which eventually causes an arousal. Obviously you need flow rate data in order to confirm there are no apneas causing the problem. In fact you really need a esophageal pressure sensor to truly confirm UARS although this is very rarely done.

The in hospital sleep study that is lined up(assuming it is a full sleep study with ECG etc) is exactly what your son needs and I would push for it to be a mixed test where they do the first half of the night without CPAP and then assuming they confirm sleep disordered breathing as the cause perform a titration study with CPAP the second half of the night. Just to clarify is the date for this test Feb 9th or Sept 2nd? Hopefully Feb 9th otherwise I would be pushing for greater urgency. 

Now back to the problem at hand, I believe (not 100% sure but I think that is what was happening with me) a buildup of CO2 levels can cause hyperventilation as your body will hyperventilate in order to try and flush out the CO2. I think this is what was happening to me when my mask vent was partially plugged. When this was happening to me while awake I could barely stand it and had to open my mouth and mouth breath and then I eventually stopped using my CPAP mask/machine all together. Your son can't just open his mouth etc though because he is wearing a full face mask so the only way to try and get rid of that CO2 is by hyperventilating which may slowly force the CO2 out of the mask. My guess is that is why these events seem to be occurring in semi regular intervals as CO2 levels slowly build up and then he hyperventilates to try and reduce them. First things first I would try to ensure that the mask is venting properly (and ensure that nothing is happening to block the vents as he sleeps). Trying another mask might be worthwhile especially if there is any question as to whether this one is not working properly. Perhaps if it is possible try using the mask yourself to confirm that it seems like it is not difficult to breath (if it is difficult for you then might indicate there is something wrong with the mask or machine). You mentioned that there seemed to be some improvement the first few days on APAP and then he sunk lower, this could be because this treatment is actually hurting him. Perhaps due to some strange UARS type issue or maybe because he has some sort of lung/breathing related issue which is why you should set up an appointment with a pulmonologist. If you want to try and get some quick tests you could try visiting the ER again with breathing/lung function being your main focus. You could show them the OSCAR data and they might be able to do some tests or be willing to do some sort of sleep study themselves. Maybe they can hook him up to a CPAP machine and monitor what happens (maybe take yours with you). 

You mentioned having an oximeter and using it. What has it shown? Is it is a model that records (in other words can he wear it through the night and then you share the results here or if you already have some results can you share them)? 

Assuming the mask is working properly this APAP machine is already doing as much as it can at the current settings. The key setting for your son being EPR of 3, minimum pressure should be set at 7 so that he gets full benefit of EPR (will only ever drop pressure as low as 4). That said I would be considering stopping APAP use to try and determine if it is even helping or if it is just making things worse. This is where the oximeter data really comes in useful if you have it and can compare oxygen levels with/without APAP. 

If you want to stick with CPAP treatment what I would probably do if I was in your situation is as follows. Print off OSCAR data for numerous days (beginning of APAP use, recent use, provide lots of days maybe even one for every day if you really want to be thorough) showing events, flow rate, pressure, flow limitation graph and minute vent. You can also add info from this study, (https://bmjopenrespres.bmj.com/content/6/1/e000380), resmed titration protocol (https://www.resmed.com/us/dam/documents/...er_eng.pdf) and any other information you can find that supports the use for bilevel in non compliant sleep disordered breathing. Take this data in and show it to the consultant/technologists or whoever you need to and you explain to them your son is hyperventilating numerous times every night and that you believe it is because he is not in compliance with APAP because of the exhalation pressure being too high and that you think this is causing his CO2 levels to increase which then causes hyperventilation. The flow rate chart and minute ventilation chart are the two that will really help show that your son is undergoing numerous periods of stressful breathing so if they say they don't understand the data say it isn't rocket science and that it clearly shows his flow rate and minute vent skyrocketing in these moments of what appears to be hyperventilation. State you are going to stop using the APAP machine as you are afraid it may be doing more harm than good and unless they have some other good ideas ask to use a Vauto trial unit to see if increased pressure support from a bilevel machine will help. If you do not get a satisfactory response walk out of there and go find another sleep clinic to deal with, provide them with the details (sleep study report, OSCAR graphs etc) and ask for the same. 

Sorry, lots of questions and information but I think your son is going through something that needs to be dealt with properly and promptly so hopefully some of this information will help.

[BunnyMummy]

Thank you so much Sleeprider. I have so many questions in my head about what is causing this. He had an asthma check around the time he started to feel tired in the mornings and struggle to get up, no change to treatment, asthma under control. I just don't understand what is causing the flow limitation on expiration. Sadly we have no data to compare before the use on cpap as he had only finger oximetry, it would have been much better had he had a full overnight....
I'm starting to consider COPD but he doesn't really have the other symptoms, and never smoked. He is rarely breathless in the day, has to take his reliever inhaler when around hay and our pets but not on a daily basis. He can get anxious and panicky and hyperventilate in the day occasionally. Panic attacks at night? Again surely not repeatedly and from what appears to be sound asleep to wide awake...
I am reading about expiratory intolerance, would that fit?
Here are more charts, from last night, and his first day on apap, 20/11, where he slept quite well so he thought and woke the most refreshed he has in months, we thought that was the answer. When he first had fatigue he didn't have the sweating, that only arose in the last few months, so something progressive? Hmm this is so hard. Hopefully the psg will bring some more ideas.

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[BunnyMummy]

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I don't understand what the single spikes are like at 03.08....

[DeepBreathing]

I'm coming in to this thread late, and I'm sorry to come up with yet another different suggestion. However I've noticed in the graphs that your son is experiencing sleep breathing disruptions which aren't being flagged as apneas. My guess is that these are in fact apneas but don't quite make the arbitrary 10 second duration.

Oscar has a facility to set "user flags" for events which aren't automatically flagged by the machine. Go to File / Preferences / CPAP and turn on Custom CPAP User Event Flagging. I'd suggest you set UF1 to 20% for 7 seconds and UF2 to 50% for 8 seconds. Let Oscar do its thing then go back to the Daily page. Turn on the event flags for UF1 and UF2 and see what you get. You might like to play around with other UF settings until you get a reasonable idea of what's happening.

I'm guessing that there will be a lot of almost-apneas which could be disrupting his sleep.

This doesn't take away from the advice you've been given above but does give you another data point to understand what is going on.

I don't understand what the single spikes are like at 03.08....

This spike occurs immediately following the end of a small leak. My guess is that the leak caused some minor discomfort, that he has re-settled the mask on his face and taken a deep breath in & out. Generally one-off spikes like this don't mean much, but if they come in clusters then some further investigation may be needed.

[Geer1]

Those single spikes could be as simple as a yawn. I and many others have them.

The data in these machines is helpful but it is too easy to over analyze hoping you might find an answer/solution, I have been doing that for the last 2.5 months... There is one very obvious problem with your son's data and it is the primary cause for concern. That is the periods of high flow rate/minute volume due to what appears to be hyperventilation. This should be your focus right now, if it is due to flow limitations/UARS then it is not the normal response. Expiratory intolerance is perhaps a possibility and that is in a way what I was discussing in my earlier response. If he is not being able to clear CO2 with this mask/machine then it may be building up and causing him to hyperventilate. Cover your mouth/nose partially until you have trouble breathing, you will notice you have the same response (panic and hyperventilation). As I mentioned before if that is the case this intolerance to APAP may be doing more harm then good as the machine/mask for some reason is effectively suffocating your son. That is not normal and would indicate an issue with the mask (not venting) or potentially some sort of lung/breathing issue hence the recommendation to see a pulmonologist. 

Try to find an answer about the hyperventilation(from a pulmonologist, sleep doctors/consultants/technicians, ER whatever it takes) and we will work from there. Consider a night or two off of APAP to see if he seems to sleep a bit better and not have these periods of hyperventilation(would help pinpoint if APAP is the source of the issue). If nothing else push for a bilevel (Resmed Vauto) trial to see if the increased pressure support helps overcome these issues(it would if it is being caused by expiratory intolerance). I have other ideas but none are worth mentioning until you can get some answers about this obvious issue.

In case this point of issue that I am referring to isn't obvious to you (this is what Sleeprider and others has been referring to as well) I have circled the instances in one of your examples. These are periods of distress and they are occurring half of the night.