Parents' Experiences and Wishes at End of Life in Children with Spinal Muscular Atrophy Types I and II

Abstract

Parents' communication with the physician about their wishes and concerns regarding their child's end-of-life care and preferred LoD contributed to their wishes being fulfilled. The wish of hospital death was fulfilled more often than the wish of home deaths. A vast majority of siblings did not receive psychological support after death of their brother or sister.

Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics

“…One such concern is the challenge of managing the burden of care when the 'therapeutic ratio' between side-effects and benefits must be balanced. Second is managing the phases of transition across the disease trajectory points at which advancing disease signals a transition in favor of palliative care and the cessation of life-extending treatments [69]. The challenge of managing expectations in this fluid context, especially where expectations are shaded by many conflicting opinions, adds further complexity to the task of establishing a standard of care.…”

A Prospective, Crossover Survey Study of Child- and Proxy-Reported Quality of Life According to Spinal Muscular Atrophy Type and Medical Interventions

“…As the length of lifetime may be extended through progressive medical interventions, understanding and enhancing the child's quality of life remains an understudied and yet essential neurologic care team priority. 8,9 Although the current pediatric neurology literature reports extensively on the technologies and novel pharmaceuticals available for children with spinal muscular atrophy, [10][11][12][13] the impact of these medical interventions on patients' selfperceived quality of life, parental perception on the patient's quality of life, and family experience is notably underexplored. 14 Little is known about the impact of spinal muscular atrophy or interventions on quality of life and family function.…”

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

“…As many professionals are involved in the management of those children, parents as caregivers play a major role in transmitting information, ensuring a continuum of care as no home hospitalization settings enables constant nurse or medical staff presence. This empowerment of parents in their child's care has been claimed in recent studies (24,25,28,29) and seems a key point to ensure the best care for the child in real life. In our study, not only did parents report as "Clinical Research Assistant" their child's symptoms and treatments in the HB, but they also spontaneously evaluated the treatments and recommendations made by care providers, and most of all they made propositions on everyday management of a child with SMA-1 (plays, installation, and feeding), enlightening that in addition to being a caregiver, they take care of their child as every parent does.…”

“…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).…”