OC13 The epidemiology and long-term outcomes in gastrointestinal dystonia (GID): longitudinal single centre data from a tertiary gastroenterology unit over 8 years utilising the BSPGHAN criteria for GID

Le résumé fourni par la source

The complex and progressive debilitating symptoms associated with severe neurodisability and the gastrointestinal tract have long been recognised by specialist nutrition support teams, however have only recently been characterised as GID.1 Whether progressive disease represents specific GI dysfunction or heralds global decline in patient health is poorly defined. Insights into longitudinal treatment success, disease progression, morbidity and mortality would help inform complex decision making for professionals and carers looking after patients with life limiting conditions. We describe outcomes for patients meeting agreed criteria of GID in a tertiary specialist complex feeding (CEN) service. Patients prospectively gathered on departmental database were identified from referrals 03/2013–12/2020 (entry criteria being ≥2 yrs follow up) and were assessed for: baseline demographics; medications used; surgical interventions; blenderised diet (BD); jejunal feeding; parenteral nutrition (PN) episodes; mortality. Patient notes were independently reviewed by 2 authors to agree if they fulfilled criteria for GID, with a third author adjudicating if discordant. 163 patients were referred to the CEN team over the timeline with 40 fulfilling GID. Median age 5.3 years. Diagnosis included: 80% cerebral palsy; 12% genetic syndrome associated with neurodisability; 8% epileptic encephalopathy. 53% had a fundoplication, 58% jejunal feeding and 73% BD over timeline. 85% had >3 medication at any time for GID symptoms (figure 1). Patients received 1–3 tone medications, 55% also had botox muscle injections and 18% intrathecal baclofen pump therapy. The majority also received multiple sedating and analgesic medications. There were 3 episodes of PN in 3 patients, with one patient being trained for home PN. One patient received PN in what became evidently a palliative phase. Overall mortality was 6 (15%) over 3 years from entry into CEN service. 3 deaths were from GID and 3 from other causes related to primary condition. Associated therapies included: 25% respiratory home ventilatory support; 35% spinal team assessment; 25% salivary gland botox or surgery. We present the first case series using defined criteria for GID. We describe the complexity, co-morbidity and disease burden of this condition. Polypharmacy is substantial and requires multi-speciality co-ordination of prescribing and monitoring to inform overall strategy. We would advocate symptom management plans led by palliative and supporting care colleagues. We highlight the resource implications and need for a co-ordinating case holder for these patients with complex multisystem needs. It is of note that the minority of patients benefited from PN for symptom control and went onto HPN. Mortality is significant from GID or co-morbidities in this population. Reference Barclay AR, Meade S, et al. Frontline Gastroenterology 2022;13:A9–A10.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.