Diaphragm pacing for congenital central hypoventilation syndrome: A novel case of pacing implanted into the diaphragm

2021 ◽  
Author(s):  
Yosuke Yamada ◽  
Hisaya Hasegawa ◽  
Takanori Ochi ◽  
Atsuyuki Yamataka ◽  
Naohisa Ueda
Keyword(s):  
Congenital Central Hypoventilation Syndrome ◽  
Diaphragm Pacing ◽  
Central Hypoventilation ◽  
Hypoventilation Syndrome

scholarly journals 0874 Patients with Congenital Central Hypoventilation Syndrome Do Not Wake Up to Ventilator Alarms

SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A333-A333
Author(s):  
S K Mathur ◽  
S Kun ◽  
T G Keens ◽  
I A Perez
Keyword(s):  
Pulse Oximetry ◽  
Sleep Disturbances ◽  
Ventilatory Support ◽  
Tube Feeding ◽  
Low Frequency ◽  
Congenital Central Hypoventilation Syndrome ◽  
Diaphragm Pacing ◽  
Central Hypoventilation ◽  
Hypoventilation Syndrome ◽  
Night Waking

Abstract Introduction Congenital Central Hypoventilation Syndrome (CCHS) requires lifelong ventilatory support during sleep. CCHS patients are vulnerable to sleep disturbances associated with treatments, monitoring alarms, and care they receive. We hypothesized that sleep would be disrupted in CCHS patient’s due to ventilatory support and other treatments at night. Methods An anonymous survey of CCHS patients aged 0-17 years was conducted through REDCAP. Patients were recruited in person, by flyer, email, and social media. Data collected included demographics, PHOX2B genotype, ventilatory support, treatments, nursing, and sleep parameters. Results We received 22 responses (27% Female, 8.1 years ± 5.7). PHOX2B genotypes were 20/27 PARM (8), 20/26 PARM (2), 20/24 PARM (2), 20/25 PARM (4), ≥ 20/28 PARM (2), and 2 NPARM (2). Two respondents did not indicate the PHOX2B genotype. 13/22 were ventilated by PPV via tracheostomy, 7/22 by BPAP, and 2/22 by diaphragm pacing. Additional treatments received at night included suctioning (8), aerosol (1), G-tube feeding (2), and none (11). Only 9 received nursing at night. 13 used pulse oximetry for monitoring, and 9 used both pulse oximetry and end tidal CO2 monitor. 16/22 rarely woke up due to ventilator or monitor alarms. 15/22 slept within 20 minutes after going to bed. Sleep latency was not affected by mode of ventilation. 11/22 reported night waking ≥ 2 nights/week and 10/22 returned to sleep without help after night waking. 6/7 BPAP dependent patients reported low frequency of night waking (0-1 time/week). Of the PPV + trach group, 7/13 reported high frequency of night wakings, mostly 5-7 times/week. Conclusion Most CCHS patients do not awaken in response to ventilator alarms. Sleep is rarely disrupted by nursing or feeding intervention. We speculate that CCHS patients contemplating to live independently should be tested to see if they awaken in response to ventilator alarms. Support None

Thoracoscopic placement of phrenic nerve pacers for diaphragm pacing in congenital central hypoventilation syndrome

2015 ◽  
Vol 50 (1) ◽  
pp. 78-81 ◽  
Author(s):  
Kristina J. Nicholson ◽  
Lauren B. Nosanov ◽  
Kanika A. Bowen ◽  
Sheila S. Kun ◽  
Iris A. Perez ◽  
...  
Keyword(s):  
Phrenic Nerve ◽  
Congenital Central Hypoventilation Syndrome ◽  
Diaphragm Pacing ◽  
Central Hypoventilation ◽  
Hypoventilation Syndrome

scholarly journals Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients

Respiration ◽  
2015 ◽  
Vol 89 (6) ◽  
pp. 534-538 ◽  
Author(s):  
Bonnie Diep ◽  
Annie Wang ◽  
Sheila Kun ◽  
J. Gordon McComb ◽  
Donald B. Shaul ◽  
...  
Keyword(s):  
Congenital Central Hypoventilation Syndrome ◽  
Diaphragm Pacing ◽  
Central Hypoventilation ◽  
Hypoventilation Syndrome
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