Tropina Elena PavlovnaThe Children’s Palliative Care Center, Russia
Title: Modern pharmacotherapy of the patients with short bowel syndrome. Regional experience in patients management
Patients with short bowel syndrome (SBS) are rare in clinician practice and tend to need a multidisciplinary approach. The most common manifestations of the disease include symptoms of malabsorption: diarrhea, weight loss, macro and micronutrient deficiency, flatulence, abdominal pain. Nevertheless, the number and intensity of symptoms are very individual in nature and, in many respects, depend on the underlying disease and the general condition of patients . Treatment of children is done with life-long parenteral nutrition (PN) and/or intestinal transplantation. Rehabilitation activities in children with short bowel syndrome should include correction of impaired absorption and restoration of normal trophic status; diarrhea management, prevention and treatment of dysbiosis; monitoring and correction of general digestive disorders . We analyzed the medical histories of five children diagnosed with SBS under the supervision of specialists from the Nadezhda Children's Palliative Care Center in Tyumen (Russian Federation). After the diagnosis of SBS, during hospitalization to the Russian Children's Clinical Hospital, children were provided with permanent central venous access, and individual parenteral and enteral nutrition regimens were selected for them. Reconstructive surgical treatment was applied (sequential transverse enteroplasty - STEP). Mothers during hospitalization were trained in the technology of long-term PN, the basis of which is strict compliance with aseptics. A distinctive feature of the integrated approach to the provision of palliative care for children with SBS in the Tyumen region is the provision of specialized products of medical nutrition, enteral and parenteral, as well as consumables intended for the functioning of the Broviak tunneled catheter, in full, according to individual recommendations and needs, through annual regional subsidies. According to clinical guidelines, a child with SBS older than 1 year of age, after reconstructive surgical treatment, who is on PN, is a candidate for treatment with an analogue of glucagon-like peptide 2 (GLP-2). Therefore, we started therapy with teduglutide in 5 children meeting these selection criteria . The results of dynamic observation demonstrated the efficacy of teduglutide, which was expressed in the change in a number of assessed somatic parameters of children taking the drug for one and a half years (from August 2021), as well as in the year (from January 2022). The experience of our center indicates that, when creating certain working conditions in the form of an organization of a multidisciplinary team of specialists, providing dynamic follow-up as well as meeting the personalized need of SBS patients for the necessary medications, consumables, specialized medical food products, including training of parents/caregivers in the conditions of compliance with the principles of aseptics and antiseptics, as well as strict accounting of new and monitoring of the condition of existing patients in the regional register, patients with such a diagnosis can live an ordinary full life and be fully socialized in human society.
Tropina Elena Pavlovna is a head of the Children’s Palliative Care Center, State Autonomous Health Care Institution with Children’s rehabilitation center "Nadezhda", 625043, Tyumen, Russian Federation, she is a chief freelance specialist in palliative care for children of the Tyumen Region Health Department.