I started my career studying in Granada, while my studies I used to spend holidays as an observer in my town small hospital, as my mother was an emergency doctor there. While my studies I spend a year At Centre Hospitalizer Vaudois (Lausanne Switzerland) in the context of Erasmus Grant. Once I finished medicine I move to Murcia to do my residency program on Paediatrics. I was interested in Paediatric Intensive Care and I move to Boston Children's Hospital In Boston for a 3 months observership. As an Intensive Care Professional I was mainly focus in Critical oncologic Children and I think that is the reason why I started to be interested y paediatric palliative Care.
I started the paediatric palliative care program at Murcia (where I actually worked) in 2013 and since then I’ve been working as the leader of this Unit. Recently, the main professionals in the field of paediatric palliative care in Spain have joint to create the Spanish association for paediatric palliative care. This is the first communication in a international meeting.
(1)Hospital Clinico Universitario Virgen de la Arrixaca, Pediatria, Murcia, Spain, (2)Hospital Carlos Haya, Malaga, Spain, (3)Hospital Nino Jesus, Pediatria- Cuidados Paliativos Pediatricos, Madrid, Spain, (4)Hospital Nino Jesus, Madrid, Spain, (5)Hospital Clinico Universitario Virgen de la Arrixaca, Almeria, Spain, (6)Hospital Sant Joan de Deu, Barcelona, Spain, (7)Hospital de Toledo, Toledo, Spain
Background: SMA is a life threatening condition with complex palliative care needs.
Context: In Spain Pediatric Palliative Care Departments (PPCD) are being developed. Also palliative care for SMA disease is growing for children and their families.
Aim: The aim of this study is to review the palliative approach to SMA in Spain during the last 8 years.
Methods: A multicentric retrospective study was performed reviewing clinical reports of children followed and treated in pediatric palliative care resources in Spain.
Results: 41 children and families were reviewed. Clinical classification found was 2 SMA with respiratory distress (SMARD), 37 patients SMA type 1, 1 SMA type 2 and 1 SMA type 3. 36 patients have died. Median age at diagnose was 3 months (1 d-8m). Median delay between diagnose and first PPCD was 6,2 days (1-258d). Median follow up to date PPCU last for 53 days (1 d-1153 d). Most frequent onset symptom was hypotonia. One patient (2,5%) presented at first symptom severe respiratory failure. Median age at onset was 2,5 months. 37 patients (92,5%) received palliative care not only at the end of life. Advanced enteral nutrition was used in 33 patients. 76% received respiratory physiotherapy. 4 patients got invasive ventilation (2 endotracheal tube, 2 traqueostdmy). Night non invasive ventilation (NIV) was used in 8 patients (76%).100% suffer dyspnea.80°/0 got opioids, 15 needed benzodiazepines. At end of life 13 received benzodiazepines and opioids (85% subcutaneous). Median age at death was 6,6 months (22 days-172,5 months). Place of death was home in 67%. Bereavement follow up was performed in 28 patients with no pathologic bereavement process.
Conclusion: Managing SMA is still a challenge. Pediatricians send children to PPCD at early stages of illness. Dyspnea was present in all patients in our study and needs an appropriate treatment. Most of the patients need opioids and benzodiazepines to reach comfort at end of life.