Children with mucopolysaccharidosis and nursing: an experience in assistance for dehospitalization

Abstract

This article reports the experience which purpose is to divulge and discuss the process of dehospitalization for children with mucopolysaccharidosis (MPS) at the Pediatric Hospital Professor Heriberto Ferreira Bezerra (HOSPED) in the city of Natal / RN. The MPS are a genetic disease that results from deficiency of lysosomal enzymes. The drug therapy is the enzyme-replacement therapy (TRE). It has a high cost for the dose is administered weekly as a four hour infusion. The problem is that these patients were hospitalized for twenty-four hours to receive the treatment, being discharged after that. At this point the necessity for hospitalization was questioned. The dehospitalization is the new trend in hospital care, as it reduces the risk for infections and trauma, the emotional and social deprivation as well as it lower costs as more hospital beds are made available for other treatments. The non-hospitalization process allows the child to spend a few hours in the clinic, being released after the therapy administration. The nursing staff from the ambulatory is now completely responsible for the TRE, establishes bonds with the children and their families, and is prepared to assist with creative strategies for assistance and social commitment, thus, achieving improvements in physical structure, and creating a comfortable environment. Currently there is concern about the TRE financing, because the hospital is not accredited as a day care hospital and depends on court injunctions to purchase the enzymes and to ensure the continuity of the treatment