I've searched the net and this is the Best Site for any info on sleep apnea!!  Thanks in advance for any insite you can give us!!!!

So to start, heres a little background.  Alex, 9, was diagnonsed with Neurofibromatosis type 1 back when he was 3, he's low tone, ADHD and has a few LD's.  My hubby went camping with our son in June and said he didn't get any sleep because of his snoring.  So we started this little investigation by going to an allergist, as Alex is a big mouth breather and always seems stuffy.  CT revealed enlarge tonsils and adnoids, so that doc refered us to an ENT.  The ENT said he'd benefit from a T&A but ordered a polysomnography first.  We were half expecting an OSA report but what really suprised us was that he had more Central apnea episodes than OSA.  

Here are the findings (which I'll just type out like they have it written):

Stages 1, 2, 3, 4 and REM were achieved.  REM latency was 173 minutes.  REM comprised 16.5 % of total sleep time.  Sleep efficiency was 94.2% with a total sleep time of 449 minutes.

A total of 77 apneas and hyponeas were seen.  There were 40 Central apneas, 9 obstructive and 28 obstructive hypopneas.  Patient's total AHI was 10.3 respiratory events per hour.  REM AHI was 14 respriratory events per hour.  Oxygen saturation averaged 97% with oxygen saturation nadir at 88%.  Central apneas occured both during nonREM and REM sleep and were associated with clinically significant oxygen desatruartions followed by EEG arousals.  Light intermittent snoring was seen.

End tidal CO2 was consistantly elevated about 45mmHg with the maximum recoreded value of 50 mmHg, indicating obstructive hypoventilation and sleep.

Periodic limb movemnets were not a feature of this study.  Occasional spontaneous arousals were seen at the rate of 6 per hour.  Electrocardiogram showed sinus rhythm with an average heart rate of 85 bpm.

Now here are my questions...remember now that I'm trying not to freak out about all this!  I guess my first question is, is this really serious or can this wait  before treatment?  Am I going to walk in one morning and find my son stiff and blue?  We have an appt with a new ENT (another long story...I didn't click with the first) on November 7th.   I know that the obstructive apneas can be taken care of with a T&A, but what do they do for the central apneas?  Is it the biPAP or CPAP therapy?  Do kids really get used to sleeping with those?   Should we even be seeing an ENT for central apnea issues...or should it be a sleep doc, neurologist, pulmonologist???   Does his low tone have anything to do with this?   Is he at higher risk under anethesia with a central apnea problem?

I probably wouldn't have given all this much thought until the doctor's appt. but I spoke to his NF doc at a conference this past weekend and asked him about the central apnea issue and HE seemed very concerned about it!  Akkk.  Said we should call him after the ENT appt. and tell him what they said, mentioned MRIing Alex too.  Guess he's thinking tumor (which is what NF is all about).

Again, thanks in advance for any insights!

Sheri, mom2alex

So the child is not established with a neurologist?


_________________
RPSGT88

I will try to answer your questions based on my limited knowledge. I am a social worker recently diagnosed with obstructive sleep apnea and on CPAP for about 600 hours. I am the father of a son diagnosed with a developmental disability that he appears to have outgrown. I have spent most of my professional life serving individuals with mental retardation and their families. I have recently had cause to learn more about central apnea in adults. I am not qualified to interpret your son’s sleep study results. However, I hope the following might help address your questions.

Let me start with the question about who your best resource will be for solid information. Sleep medicine lies rather at the intersection of neurology, pulmonary medicine, and ENT medicine. Sleep medicine has its own accreditation procedures and what you want is a certified sleep specialist with experience with the sleep disorders of children (not all sleep specialists are up on pediatric sleep and standards for diagnosing and treating apnea in children is somewhat different than the standards for diagnosing and treating apnea in adults). Hopefully, the sleep specialist in charge of the lab that did your son’s sleep study is accredited and has experience dealing with pediatric sleep disorders. At some point you will want to talk with the sleep specialist and/or have your neurofibromatosis doctor (who I presume is likely to be a pediatric neurologist) consult with him about treatment issues. You will also want to keep your ENT in the loop as well. In the long run, you, your husband, your son, your son’s pediatrician, the NF doctor, the ENT and the sleep specialist are likely to all play important parts in addressing your son’s apnea.

Although many people have very low infrequent episodes of central apnea and/or obstructive apnea without any further problems, both obstructive sleep apnea and central sleep apnea can be serious problems. When both occur together, I believe this is sometimes referred to as mixed apnea. There does appear to sometimes be an inter-relationship between obstructive apnea and central apnea but I have no understanding of the nature of this relationship (this would be a good question to discuss with the sleep specialist). From what I have read, your son’s apnea certainly appears to be a significant problem. I suspect that both the central apnea and obstructive apnea/hypopnea need to be addressed and I would suspect that you might want to get input from your sleep specialist (as well as your NF doctor and your ENT) as to how to develop a coordinated treatment strategy for addressing them.

Although central apnea is sometimes described as being due to the brain’s failure to signal the body to breath, this is a very unsatisfactory definition. Central apnea is defined in practice as apnea (cessation of breathing-for 10 seconds or longer in adults, I believe the standard is shorter in children) that occurs without an apparent effort to breath (as opposed to obstructive sleep apnea which is cessation of breathing despite physical efforts to breath). Central apnea may be the result of many different problems and it can take many forms. Many people with central apneas have relatively low incidence of events and they occur for only a fairly short duration before the person resumes breathing. The primary risks of apnea in children appear to lie in the body’s response to lack of sleep (fatigue and lethargy, attentional deficits and problems with cognitive functioning) and the response of the body to the lack of oxygen (which might include heart problems and cognitive damage). This being said, some people with apnea do die suddenly in their sleep. This appears to be a very rare occurrence however. In other cases, an apnea might go away either with treatment of an underlying problem or (especially in the case of apnea occurring in premature infants) it might go away as the child’s body continues to mature.

Central apnea in adults is often associated with heart problems. In children, this is less likely to be the case. Other known causes of central apnea include gastroesophageal reflux (this can cause a spasm of the larynx to protect the airway from aspirating stomach contents), certain diseases, spinal cord injury, certain substances (such as carbon monoxide), and stroke, tumor or other structural injuries or problems with the brain. It seems possible that your son's low muscle tone might be contributing to his central apnea and you may want to discuss this with both the sleep specialist and the NF doctor. There are some known neurodegenerative diseases that can cause or contribute to central apnea. Given the variable nature of NF, it seems possible that NF could contribute to apnea in some cases but none the websites on NF and none of the apnea websites I have visited mentioned any association between NF and apnea. You may well want to discuss this further with your NF specialist and maybe with your sleep doctor (assuming he is a pediatric sleep specialist). I would note that MRI of the brain appears to be a standard study for people found to have central apnea.

If an underlying problem can be identified as contributing to a patient's central apnea, and if it can be treated, this would be the first line of treatment. CPAP, Bi-level CPAP, and a special device known as Adaptive Servo Ventilation may be used to help support breathing. This last device is especially designed for patients with central apnea. Supplemental oxygen may be used to help keep the body's oxygen level up. Supplemental oxygen may be used by itself or in conjunction with CPAP or related devices. In some cases, medications may used to try to stimulate breathing.

Both obstructive apnea and central apnea can present some complications to the use of sedation and anesthesia during and following surgical procedures. The primary problems are that either sedation or anesthesia may trigger an apnea and that the patient could suffer significant loss of oxygen as a result. The increased risk of apnea may persist beyond the surgery itself and into the recovery period. These are best addressed by making sure you discuss these conditions with doctors and anesthesiologists before surgery. These problems are best handled through preparation.

I hope that this helps you prepare for your next steps and I would welcome any corrections, clarifications or additions to what I have stated above from those with knowledge or experience in these areas. If you have further questions, please feel free to follow up. Please keep in mind that this information represents only my best understanding of these issues at this time and that it is not a substitute for professional medical advice.

Best wishes to you and your family,
Bill