Caracterização e circunstâncias da ocorrência de óbitos em crianças com doença Falciforme triadas pelo Programa Estadual de Triagem Neonatal de Minas Gerais, no período de Março de 1998 a Fevereiro de 2005

Abstract

Objective: To characterize and describe the circumstances that surrounded the deaths of children with sickle cell disease who were screened by the State Program of Newborn Screening in Minas Gerais (PETN-MG) from March, 1998 to February, 2005. Methods: All children who died during the afore mentioned period after having the diagnosis of sickle cell disease and follow up by the PETN-MG were included. Deaths were identified through active search for children who did not show up for their regular scheduled visits to Hemominas Foundation clinics. Data were collected from the PETN-MG data bank, Hemominas clinical charts, death certificates, IBGE and DATASUS data banks, and personal interviews with a member of the localizedfamilies, after her or his signing the informed consent form.Results: During that period 1,833,030 newborns were screened by the PETN-MG; 1,396 cases of sickle cell disease were detected and 78 deaths were recorded (5.6%): 63 were SS (80.8%), 12 SC (15.4%), and 3 S+thal (3.8%). The median age at the screening was 9 days; at first clinical visit, 2.1 months, and at death, 13.7 months. Age distribution at deaths was as follows: 33 children (42.3%) died before 1 year of life; 23(29.5%) between 1 and 2 years; 10 (12.8%) 2-3 y; 5 (6.4%) 3-4 y; and 7 (9%) between 4 and 7 y; 52% were males. Death occurred in hospitals for 59 children (75.6%) and at home or in transit for 18 (23.1%). 78% of children lived in urban areas and 22% in rural areas. The causes of death as recorded on the death certificate were: infection for 30 children (38.5%), acute splenic sequestration for 13 (16.6%) and other causesfor 7 (9%). In 16 cases (20.5%) death certificate was not conclusive and 12 children (15.4%) died without medical care. 62.5 of children who died lived in small towns (less than 50,000 inhabitants) and 83.6% in cities with the Human Development Index between 0.5 and 0.8 (medium index). The total number of scheduled clinical visits at Hemominas (580) was the expected one as prescribed by the protocol but no showreached 22.7% of those visits. Personal interviews were done with 52 families. The median ages of dead childrens mother and father were 25.5 and 29 years, respectively. 13.5% of mothers were illiterate and 46% had reached the 4th series of fundamental school, at maximum. The numbers for the fathers were 10% and 57.8%, respectively. 71.2% of fathers were manual workers; 78.8% of mothers were housewives without any income. 19% of families had other children with sickle celldisease. The mean number of members in the family was 5.6. Per capita monthly income was less than 200 reais in 96% of the families. 44% of the families lived in their own properties, 42% in handed-over houses, and 10% in rented ones. 52% of houses had sanitary sewage, 94% electrical supply, and 71% water filtration devices. Only 58% of dead children had regular medical follow-up at the Basic Health Unity; theothers only attended emergency rooms or Hemominas outpatient clinics for the periodic visit. 65% of the children had been admitted to hospitals at least once before the death event; 19% had been hospitalized for more than 3 times. Previous episodes of acute splenic sequestration was reported by 27% of the families; previous episodes of pain in 67.3%. Fever, pain, vomit, prostration, and increasing pallor were the mostfrequent signs or symptoms heralding death event. Medical care in the first 6 hours from the beginning of symptoms was possible for 57.7% of cases and in the first 24 hours for 71%; 19% died without any medical care. In 25 cases (48%) hospitals were the first health unit to be looked for; in 23%, Basic Health Units. Infection (19 cases) and acute splenic sequestration (17 cases) were the leading causes of death. Whencompared to official death certificate, sequestration was reported as the cause of death in only 7 out of those 17 cases. Inadequate infrastructure for health care in emergency situations, distant health care units, unavailable speedy ground transportation and low understanding of the disease by health staff and families were reported as contributing factors for death in personal interviews. The estimated probabilities (S.E,, mean standard error) of surviving 5 years for SS, SC, and S+thal children were 89.4% (1.4%), 97.7% (0.7%), and 94.7% (3.0%), respectively (SSversus SC, P < 0.0001).Conclusions: The majority of deaths occurred in infancy and in SS children. The main causes of death were infection and acute splenic sequestration. The number of reasons for death labeled as indeterminate may indicate insufficient knowledge of sickle cell disease itself and of recognizing intervening acute crises by health staffmembers. Taking into consideration the social and economic background of the majority of small towns in Minas Gerais, future programs for sickle cell patients should be strategically directed to Basic Health Units. Educational aspects, both for health caregivers and for parents and children, should be an integral part of such a program.Even after a carefully controlled newborn screening program the probability of SS children dying was found to be still high. Most causes of deaths would be preventable by education and improvement in the quality of health care and general social conditions