Symptom management and psychological support for families are the cornerstones of end‐of‐life care for children with spinal muscular atrophy type 1

Abstract

Our study found that symptom management and psychological support for families were the cornerstones of end-of-life care for children with SMA1.

The support needs of parent caregivers of children with a life-limiting illness and approaches used to meet their needs: A scoping review

“…37 Further, information delivered to parents using medical jargon left parents distressed and requiring further clarification but often felt apprehensive to do so. 23,38,39…”

Palliative Care in SMA Type 1: A Prospective Multicenter French Study Based on Parents' Reports

“…Morphine and benzodiazepines were used more (77%) over the last period than during the two previous ones studied in France (18 and 60%, respectively), but less than that in other studies when reported (8,22,23), and use of analgesics and/or sedative treatments has not been ruled yet in the last standards of care (5,10). However, pain and dyspnea were the main symptoms reported during the last 48 h in a recent retrospective study (22), underscoring the need to prioritize comfort for those infants. Of note, amitriptyline has also been used to ensure well-being and diminish anxiety due to chronic respiratory insufficiency but needs further evaluation.…”

“…2005-370, promulgated on April 22, 2005, and Law No. 2016-87 promulgated on February 2, 2016, regarding end of life and patients' right) has led to the implication of more home-hospital settings (26-62%) and involvement of dedicated pediatric palliative care teams (11-74%) in the management of those severe patients. If there was no evolution in NIV use over the period studied, oxygenotherapy, suction aspiration system, and enteral feeding through an NGT were used in most patients, as reported in other studies (8,15,22,23) and recommended in the recently revised consensus statement on standards of care in SMA (5,10).…”

“…Our study also collected information about medical conditions at the time of death, which occurred at home for 38% of patients vs. 17 and 23% in the previous periods reported in France (7). While there are little data among studies to enable robust comparison across countries (8,(22)(23)(24), evidence as is collectively suggests that there is an increasing consideration for parents' wishes about their child's death conditions. Morphine and benzodiazepines were used more (77%) over the last period than during the two previous ones studied in France (18 and 60%, respectively), but less than that in other studies when reported (8,22,23), and use of analgesics and/or sedative treatments has not been ruled yet in the last standards of care (5,10).…”

“…The implementation of specific pediatric palliative care in the context of SMA-1 patients needs active collaboration and coordination between the different actors involved to ensure the child's and family's best quality of life. This need for coordination has recently been supported in qualitative studies (25,26), as well as the importance of parents' input about their wishes for their child's treatments and end-of-life conditions (22,24,25,(27)(28)(29).…”