Palliative care in 9 children with neurodegeneration with brain iron accumulation.

AIM: Evaluation of pediatric palliative home care of families with children suffering from neurodegeneration with brain iron accumulation (NBIA) and their parents. MATERIAL AND METHODS: The children were treated at home by a multidisciplinary team. Densitometry was used to evaluate the condition of the skeletal system. Botulinum toxin was injected into the muscles in doses between 22 and 50 units/kg. The quality of palliative care was assessed on the basis of a specially designed questionnaire for parents. RESULTS: The observations were performed on a group of 9 patients with NBIA. On admission, the median age of patients was 9 years (7-14). The average time of palliative home care was 1569 days (34 days-17 years). The median age at death (6 patients) was 11 years (7-15). The botulinum toxin injections gave the following results: reduction of spasticity and dystonia, reduction of spine and chest deformation, relief of pain and suffering, facilitation of rehabilitation and nursing, prevention of permanent contractures, and reduction of excessive salivation. Bone mineral density and bone strength index were reduced. Two patients experienced pathological fracture of the femur. The body mass index at admission varied between 9.8 and 14.9. In 7 cases, introduction of a ketogenic diet resulted in the increase of body mass and height. The ketogenic diet did not worsen the neurological symptoms. The parents positively evaluated the quality of care. CONCLUSION: Palliative home care is the optimal form of treatment for children with NBIA.

New catalogue of conditions that may require pediatric palliative care.

BACKGROUND: Pediatric palliative care applies to the conditions specified in the Regulation of the Minister of Health. The previous studies have proved that the conditions' catalogue is incomplete and inadequate. AIM OF STUDY: The aim of the study was to verify completeness and adequacy of the Minister of Health's catalogue and to develop a new catalogue based on available epidemiological data concerning the Polish population. MATERIALS AND METHODS: The data of the Statistics Poland on underlying causes of death of children (0-17 years old) and young adults (18-29 years old) who died in 1999-2018 were analyzed. Besides, the study includes data collected by the Warsaw Hospice for Children Foundation on diagnoses of 739 patients treated in 1994-2019, including 519 deceased. The catalogue developed on that basis was compared with the Minister of Health's catalogue. RESULTS: The new catalogue of conditions that may require pediatric palliative care in Poland and the additional criteria of patient recruitment were developed. The incompleteness and inadequacy of the Minister of Health's catalogue were demonstrated. CONCLUSIONS: The Regulation of the Minister of Health necessitates an amendment regarding the list of medical conditions entitling children to guaranteed palliative care and hospice services. The Minister of Health should introduce standards and medical procedures for pediatric palliative care. Patients should be recruited on the basis of recognized standards by physicians and not officials of the National Health Fund using incomplete and inadequate Minister of Health's catalogue of medical conditions.