Palliative care services for children with life-limiting conditions.

BACKGROUND: Palliative care (PC) for children with life-limiting conditions (LLC) is a holistic approach to achieve the best quality of life. AIMS: Highlighting collaboration between pediatric and PC services is essential in providing seamless care. METHODS: Retrospective data obtained including patient cohort, disease profile, place of death, and the resource requirements for children with a LLC in the Mid-West region of Ireland over a 7-year period. RESULTS: Seventy-seven patients were identified (n = 77); five still receiving care, four discharged, and 68 have died. The cohort ranged in age from 1 day to 17 years with a mean of approximately 6 years. Thirty-five percent of patients were less than 1 year old. Fifty-one percent were male. Seventy-one percent were referred initially to PC for end-of-life care and 29% for symptom management. Forty-four percent had home support services in place prior to referral to PC. An advanced care plan was created for 65%. Of those that died (n = 68), 70.5% died at home, 25% in hospital, and 4.4% in residential care or a children's hospice. The clinical nurse coordinator for children with life-limiting conditions (CNCCLLC) was involved with 88% of patients. The specialist PC teams were involved with 87%; 65 patients had community support, 31 patients had in-hospital support, and 29 patients were seen by both hospital and community services. CONCLUSIONS: Our study highlights the growing number of LLCs and current pediatric and PC services require further resources and development.

Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome.

Landau-Kleffner syndrome (LKS) is an acquired childhood aphasia associated with paroxysmal bitemporal electroencephalogram (EEG) abnormalities and, sometimes, clinical seizures. We report the case of a female aged 5 years 6 months who presented clinically with apparent hearing loss, deterioration in speech, and seizure activity over 12 days. The female had previous detailed speech/language assessments at 3 to 4 years of age due to articulation delay. LKS was diagnosed on EEG with bitemporal spike and wave activity during sleep. The patient was treated with high dose prednisolone 3mg/kg/day, intensive speech/language therapy, and followed a modified educational program. We recorded a marked regression in receptive and expressive language skills, as well as her speech, language, and cognitive profiles before and during treatment with prednisolone, during an 18-month follow-up period. The patient demonstrated an excellent clinical response highlighting the importance of a multidisciplinary approach to management of LKS.