Malin Lövgren, Thomas Sejersen and Ulrika Kreicbergs. Information and treatment decisions in severe spinal muscular atrophy: A parental follow-up. European Journal of Paediatric Neurology, in press.
The parents of children with severe spinal muscular atrophy (SMA) face difficult ethical decisions regarding their child's treatment. This study explored the experience of parents of children with severe SMA concerning information and treatment decisions.
Material and methods
This nationwide survey, conducted in 2013, is based on parents of children who were born in Sweden between 2000 and 2010 and later diagnosed with SMA type I or II where respiratory support was considered the first year of life (N = 61, participation rate: 87%). The survey involved parents' perception of the child's care and the questions used in this study covered information given and treatment decisions. Descriptive statistics were used.
None of the parents reported that the health care professionals made decisions concerning the child's treatment without informing them first, and 80% reported feeling confident about the decisions made. Of the bereaved parents, 11/48 (23%) reported that they got no information about respiratory support, compared to 2/13 (15%) of non-bereaved. Bereaved parents were more likely to report being satisfied with and understanding the information given about the illness and its treatment than non-bereaved parents.
All parents reported having been informed before treatment decisions were made and a vast majority reported feeling confident about the decisions. However, a quarter of the parents declined to have received information about respiratory support, which indicates that the parents did not sufficiently understand the available respiratory treatment options, and that their children may not receive the kind of care that is recommended in guidelines.
From the article
Life can be considerably prolonged for children with severe SMA by introducing respiratory support. However, the impact of such support on the child's wellbeing is unknown, and there is no evidence or consensus among experts on the use of respiratory support for SMA type I. In the absence of consensus, Wang and co-workers developed care guidelines for SMA based on evidence and a Delphi consensus-building process. Their guidelines concluded that caregivers should explore treatment options together with the family in relation to the child's potential, quality-of-life issues, and family desires. This need is particularly evident and important regarding the use of respiratory support. A key issue is to what extent respiratory support affects the quality of life of the child with severe SMA, and recommendations vary between clinics and clinicians. Some favor the use of respiratory support for SMA type I, while others strongly oppose its use, arguing that the burden for the child and family is greater than the benefit. The international care guidelines from 2007 consider such differences in treatment recommendations, and conclude that clinicians have an obligation to present treatment options to the parents in an open, fair and balanced manner…
Overall, 11/13 non-bereaved (85%) and 11/48 bereaved parents (23%) perceived that information about their child's illness had been given in an inconsiderate way, either often or occasionally. Eight of 13 non-bereaved parents (62%) and 42/48 bereaved parents (88%) reported that they were given information that helped them understand what SMA is …
When special respiratory support was discussed, the option they were most likely to be informed about was ventilation by mask. One bereaved parent was informed about tracheostomy. Fourteen bereaved parents and two non-bereaved parents (26% of the total sample) reported that they were never informed about respiratory support ( Table 2 ). Nine of these bereaved parents reported that their child received no respiratory support, two reported that their child received supplemental oxygen by nostril or mask, one that his/her child had CPAP, and two parents did not respond to this question. The two non-bereaved parents who were not informed about respiratory support reported that their child got extra respiratory support by nostril or mask…
When respiratory support was discussed, ventilation by mask was the option parents were most commonly informed about, but a quarter reported that they got no information about respiratory support at all. A vast majority of those who were given no information about respiratory support reported that their child did not receive respiratory support (all these parents were bereaved). In connection with the diagnosis, 90% of the bereaved parents were informed that their child would have a short life, but only 27% reported that a health care professional told them, in the days preceding the child's passing, that the child would pass away shortly. It is noteworthy that bereaved parents seem to have a more positive perception of the care experience than non-bereaved parents, i.e., that the information given at the time of the diagnosis was satisfactory, that the information about the child's illness had been given in a considerate way, that it has been possible for them to understand what different treatment measures could lead to for their child, and that they were given information about possible medical treatment options for their child.
This study found that 90% of the bereaved parents were informed at the time of diagnosis that their child would have a short life, but less than a third reported that a health care professional told them, in the child's last [sic], that he or she would pass away shortly. Previous research among parents who have lost a child to cancer show that communication about the impending death is of importance for parents to make the best possible decisions about the child's care, but also for the parents' long-term psychological health. It has also been found that parents' awareness about a child's impending death owing to a malignancy was associated with receiving home care. Our observation that home care was more common among those who had received information about the impending death is in harmony with that finding, since parental awareness about impending death seemed to predict home care also for children with SMA…
There were descriptive differences between bereaved and non-bereaved parents in terms of how they experienced the information given to them. Since only 13 non-bereaved parents were included in this study we have not tested these differences statistically, but still want to highlight this trend, as parents' responses to several questions differed. The children of the non-bereaved have lived longer than the deceased children and have therefore had more exposure to healthcare. This may mean that non-bereaved parents are more likely to have been exposed to unsatisfactory care at some time over the years.