I would like to point out the recommendation in a study published last September (Sleep-related breathing disorders in pre-pubertal children with Prader-Willi Syndrome and effects of growth hormone treatment - http://jcem.endojournals.org/cgi/rapidpdf/jc.2006-0765v1):
"It is important to mention that a relatively normal PSG [polysomnography] does not exclude the possibility of unexpected death during mild URTI. Based on our results cardiorespiratory monitoring during URTI in children with PWS before and during GH-treatment should be considered."
The study authors were obviously shaken by the sudden deterioration and death of a three-year-old boy in the study who had a mild URTI even though he had near-normal PSGs both before (AHI 1.7) and during GH treatment (AHI 1.4), as they mention it several times. The day before he died, the boy had been checked by his pediatrician, was running around, and did not generally act as if he was sick.
Note that although having a home pulse oximetry device is definitely a very good idea, pulse oximetry is not the same as full cardiorespiratory monitoring. Pulse oximetry only monitors blood oxygen saturation (SaO2) levels and pulse rate and as such does not monitor all aspects of respiratory and cardiac sufficiency. For example, during hypoventilation (which is fairly common in PWS, especially during respiratory infections), it is possible for pulse oximetry to register adequate Sa02 levels even though the child is hypercapnic (has elevated carbon dioxide levels) with respiratory acidosis (abnormal acidity of the blood). Also, if the heart isn't pumping enough blood due to cardiac insufficiency, a person can still be hypoxic (that is, not have enough oxygen reaching tissues such as muscles and the brain) even if SaO2 levels are adequate. Full cardiorespiratory monitoring, otoh, monitors all of those things: SaO2, carbon dioxide levels, and cardiac function.
In general, parents should not be afraid to take their child to the emergency room if the child starts to repeatedly desaturate below about 90-92% during an URTI or is lethargic and hard to arouse. In such a situation, it also might be a good idea to print out a copy of the Sept. study and take it with you in case the doctor is reluctant to admit the child for full cardiorespiratory monitoring. Remember, too, that aside from the possibility of sudden respiratory deterioration, hypoxia due to low SaO2 levels can have serious impacts on cognitive and neurological development as well as other physiological processes. (For more about that, see the article on hypoventilation and apnea in PWS if you haven't already done so (http://www.tcmnotes.org/apnea-hypoventilation-in-pws/).)
PWS and Respiratory Tract Infections
The Journal of Clinical Endocrinology & Metabolism Vol. 91, No. 12 4911-4915 Copyright © 2006 by The Endocrine Society
Sleep-Related Breathing Disorders in Prepubertal Children with Prader-Willi Syndrome and Effects of Growth Hormone Treatment D. A. M. Festen, A. W. de Weerd, R. A. S. van den Bossche, K. Joosten, H. Hoeve and A. C. S. Hokken-Koelega Dutch Growth Foundation (D.A.M.F., A.C.S.H.-K.), 3016 AH Rotterdam, The Netherlands; Sophia Children’s Hospital/Erasmus Medical Center Rotterdam (K.J., H.H., A.C.S.H.-K.), 3015 GJ Rotterdam, The Netherlands; and Sleep Center SEIN (A.W.d.W., R.A.S.v.d.B.), 8000 AN Zwolle, The Netherlands
Address all correspondence and requests for reprints to: D. A. M. Festen, Dutch Growth Foundation, Westzeedijk 106, 3016 AH Rotterdam, The Netherlands. E-mail: firstname.lastname@example.org.
Context: Recently, several cases of sudden death in GH-treated and non-GH-treated, mainly young Prader-Willi syndrome (PWS), patients were reported. GH treatment in PWS results in a remarkable growth response and an improvement of body composition and muscle strength. Data concerning effects on respiratory parameters, are however, limited.
Objective: The objective of the study was to evaluate effects of GH on respiratory parameters in prepubertal PWS children.
Design: Polysomnography was performed before GH in 53 children and repeated after 6 months of GH treatment in 35 of them.
Patients: Fifty-three prepubertal PWS children (30 boys), with median (interquartile range) age of 5.4 (2.1–7.2) yr and body mass index of +1.0 SD score (–0.1–1.7).
Intervention: Intervention included treatment with GH 1 mg/m2·d.
Results: Apnea hypopnea index (AHI) was 5.1 per hour (2.8–8.7) (normal 0–1 per hour). Of these, 2.8 per hour (1.5–5.4) were central apneas and the rest mainly hypopneas. Duration of apneas was 15.0 sec (13.0–28.0). AHI did not correlate with age and body mass index, but central apneas decreased with age (r = –0.34, P = 0.01). During 6 months of GH treatment, AHI did not significantly change from 4.8 (2.6–7.9) at baseline to 4.0 (2.7–6.2; P = 0.36). One patient died unexpectedly during a mild upper respiratory tract infection, although he had a nearly normal polysomnography.
Conclusions: PWS children have a high AHI, mainly due to central apneas. Six months of GH treatment does not aggravate the sleep-related breathing disorders in young PWS children. Our study also shows that monitoring during upper respiratory tract infection in PWS children should be considered.
Well, we read the article and forwarded to our pediatrician and are getting a pulse oximeter at home. I will let you know what we find out.
Kian has done well on both of his sleep studies and is a pretty robust two year old. That said, he has two older sisters and he attends a toddler Montessori class. So, he is always snuffly with something or the other and it tends to settle in the chest. He is not good at coughing. He doesn't do like most of us do and have a coughing fit first thing in the morning to clear his lungs. We have the doctor check and it isn't pneumonia or bronchitis, just a run of the mill infection. We are starting to do more cupping (pounding on the back and chest) to release the secretions and promote coughing, but he is an active kid and runs away.
I will let you all know how hard it is to use and what we find out.