Análise comparativa da ocorrência de óbitos em crianças com doença falciforme triadas pelo Programa Estadual de Triagem Neonatal de Minas Gerais nos períodos de 1998-2004 e 2006-2012

Abstract

Introduction: The term "sickle cell disease" (SCD) encompasses genetic blood disorders in which hemoglobin S predominates. When homozygous (Hb SS), it is called sickle cell anemia. The hemoglobinopathies are responsible, globally, for 3.4% of deaths in children under 5 years of age. Objectives: To characterize the deaths occurred in children with SCD screened by the State Newborn Screening Program of Minas Gerais (PTN-MG) from 01/03/2005 to 29/02/2012 and to compare survival of children born between 1998 and 2004 versus those born between 2006 and 2012, with the aim of describing possible changes regarding the quality of care provided to these children. Methods: Deaths were identified by active surveillance of children who missed their scheduled appointments in Fundação Hemominas (FH) blood bank centers. Data were retrieved from the databases of the PTN-MG, DATASUS and IBGE, death notification documents, FH medical records, and personal interview with the families who were located, after they had signed an informed consent form. Results: From March 2005 to February 2012, 1,784,889 children were screened by PTN-MG. Of these, 1,195 children had abnormal hemoglobin profile compatible with SCD and 117 deaths were identified. For comparative analysis, 76 deaths occurred between March 1998 and February 2005 (Fernandes et al, 2010) were added, totaling 193 deaths (7.4%). The genotypes were SS/S0-talassemia 153 (79.3%), 34 SC (17.6%), and 6 S+-thalassemia (3.1%). The median age of children was 6 days at newborn screening (n = 117), 1.4 months at first clinical appointment, and 1.7 years at death. 76.7% of deaths occurred in children under 5 years and 50.3 % were boys. Regarding the place of death, 78% died in hospital and 21% at home or during transportation. 78% of children lived in urban areas. Comparing the period 2006-2012 versus 1998-2004, there was an increase, not significant, of 11% in the number of hospital deaths. The causes of deaths were: 45% infection, 28% indeterminate, 14% acute splenic sequestration (ASS), 5.7% stroke, 1.6% multiple organ failure and 5.7% other causes. "Sickle cell disease" or "sickle cell anemia" were recorded in death certificates as associated to or the cause of death in 53% of cases, but in an impressive number of documents (46%) no mention to the terms was found. The proportion of positive mentions to sickle disorders as associated with death significantly increased in the last six-year period, indicating advancement in awareness of the disease by physicians. Most children received medical care before death, but 24% did not. Comparing the periods 2006-2012 versus 1998-2004, there was a 13% increase in the frequency of medical care provided before death in recent years, but the difference was not statistically significant. Estimates of specific mortality for SS/S0 children at one and five years were, respectively, 6 and 12 times higher than the general rates for the state of MG. For SC children, these estimates were 2.5 and 3.5 times higher, respectively. The mortality rate of children with sickle cell disease was significantly higher in small municipalities. During interviews, three-quarters of 84 relatives of children who died between 2005 and 2012 declared themselves as pardo or black. Nonattendance rate to 1,733 scheduled medical appointments in blood bank centers was about 20%. Illiteracy was around 5% of households. Two-thirds of mothers had no paid work and 30% of fathers were farm workers. About 90% of the families had income below the minimum wage and 50% received federal benefits. Only 55% of families were visited by Family Health Program (PSF) providers when the child was alive, 79% of children had medical files in in Basic Health Units (UBS), and special immune products were delivered to only 74% of children. Almost 60% had been admitted in hospitals at least once before death and 24%, more than three times. Before death, ASS had been recorded in more than a quarter of children, painful crises in nearly 60%, and blood transfusions in half of them. In almost half the cases, medical care occurred within 6 hours of symptom onset and in two-thirds, in the period up to 24 hours. Death occurred within 12 hours of onset of symptoms in about 30% of cases and in 40% within 24 hours. In 60% of cases, the first health service sought was the hospital. The estimated 5-year survival rate of children with SCD (all subtypes) was slightly higher in the last six years of the PTN-MG (94.2% versus 93.4% in the first six years, p=0.47). Conclusions: Screening for sickle cell disease, even performed in a comprehensive and effective way, was not sufficient by itself to significantly reduce mortality in PTN-MG when the first six years of the program were compared to the last six. It is necessary to foster social and economic development of the state to change this picture. Children with acute events should have precedence in health facilities. Specialist consultations, adequate transportation of patients and continuing education for families and health care teams should be recognized as a priority in the service line care of patients with SCD.