Satisfaction and effectiveness of opioid pain management among adults with cystic fibrosis: A mixed methods study.

BACKGROUND: Chronic pain is common among people living with cystic fibrosis (CF) and associated with worse clinical outcomes. Despite this, little is known about how pain is managed and how opioids are used to treat pain. The purpose of this convergent mixed methods study was to examine self-reported satisfaction and effectiveness of pain management strategies among a sample of adults with CF who are prescribed opioids. METHODS: We developed an online survey querying 4 domains - demographics, pain characteristics, pain communication, and management strategies. This was distributed nationally to adults with CF (n=48) via various online platforms. We obtained quantitative and qualitative responses regarding satisfaction and effectiveness of pain management. Emerged themes from qualitative data were compared with responses from quantitative survey domains. RESULTS: Participants reported high levels of satisfaction and effectiveness with their opioid pain management plans. However, qualitative themes emerged regarding fears of addiction, experiences of feeling stigmatized by the healthcare system and ineffectiveness and inefficiency of alternative therapies for adequate pain relief. CONCLUSIONS: Adults with CF reported opioids as an important component of their current pain management plans despite risks associated with opioid use. CF-specific pain management guideline development is warranted as is further research exploring pain development.

Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia.

BACKGROUND: Central hypoventilation syndrome ("Ondine's Curse") is an infrequent disorder that can lead to serious acute or chronic health consequences. This syndrome, especially in adults, is rare, and even less frequent in the absence of clear pathogenic lesions on MRI. In addition, we are not aware of any previously reported cases with associated cranial nerve neuralgias. METHODS: We describe a patient with baseline trigeminal and glossopharyngeal neuralgia, admitted with episodes of severe hypoventilatory failure of central origin, consistent with "Ondine's Curse". After evaluation, she was found to have a medullary capillary telangiectasia, thought to be the causative lesion, and which could explain her complete neurologic and hypoventilatory syndrome. The patient was treated with placement of a diaphragmatic pacing system, which has been effective thus far. RESULTS: This case illustrates the need for investigation of centrally mediated apnea, especially when co-occurring cranial nerve neuralgia is present and cardiopulmonary evaluation is negative. It provides an example of capillary telangiectasia as the causative lesion, one that to our knowledge has not been reported before. CONCLUSIONS: Placement of a diaphragmatic pacing system was warranted and became lifesaving as the patient was deemed to be severely incapacitated by chronic ventilatory insufficiency.