Reduced-dose intensity therapy for pediatric lymphoblastic leukemia: long-term results of the Recife RELLA05 pilot study.

Treatment-related mortality is common among children with acute lymphoblastic leukemia (ALL) treated in poor-resource settings. We applied a simplified flow cytometric assay to identify patients with precursor B-cell ALL (B-ALL) at very low risk (VLR) of relapse and treated them with a reduced-intensity treatment plan (RELLA05). VLR criteria include favorable presenting features (age ≥ 1 and < 10 years), white blood cell count of <50 ×109/L, lack of extramedullary leukemia, and minimal residual disease level of <0.01% on remission induction day 19. Except for 2 doses of daunorubicin, treatment of patients with VLR B-ALL consisted of a combination of agents with relatively low myelotoxicity profiles, including corticosteroids, vincristine, L-asparaginase, methotrexate, and 6-mercaptopurine. Cyclophosphamide, systemic cytarabine, and central nervous system radiotherapy were not used. Of 454 patients with ALL treated at the Instituto de Medicina Integral Professor Fernando Figueira in Recife, Brazil, between December 2005 and June 2015, 101 were classified as having VLR B-ALL. There were no cases of death resulting from toxicity or treatment abandonment during remission induction. At a median follow-up of 6.6 years, there were 8 major adverse events: 6 relapses, 1 treatment-related death (from septicemia) during remission, and 1 secondary myeloid leukemia. The estimated 5-year event-free and overall survival rates were 92.0% ± 3.9% and 96.0% ± 2.8%, respectively. The 5-year cumulative risk of relapse was 4.24% ± 2.0%. The treatment was well tolerated. Episodes of neutropenia were of short duration. Patients with B-ALL selected by a combination of presenting features and degree of early response can be successfully treated with a mildly myelosuppressive chemotherapy regimen.

Delayed diagnosis of leukemia and association with morbid-mortality in children in Pernambuco, Brazil.

The objectives of this study were to describe the interval between symptom onset and diagnosis of acute leukemia, to assess risk factors for delayed diagnosis, and its effect on early morbid-mortality and event-free survival (EFS). Records of children aged 1 month to 18 years diagnosed with acute leukemia were reviewed for clinical, demographic, and health care provider factors, and for time to diagnosis. Of 288 patients diagnosed, 55% had a delay in diagnosis. The median time to diagnosis was 31 days. There were significant associations between the diagnostic delay and the distance from the tertiary care hospital (P=0.04), initial consultation in an outpatient clinic (P=0.04), presenting symptoms of bone/joint pain (P=0.04), family with more than 3 children (P=0.02), birth order of third or greater (P=0.009), paternal age <30 years (P=0.03), and paternal education <8 years (P=0.008). There was no association between delayed diagnosis and early morbid-mortality or EFS at 5 years. Initial consultation in an outpatient setting, presenting symptoms of bone/joint pain, and birth order of third or greater remained statistically significant in multivariate analyses, but the delay did not have an impact on early morbid-mortality or EFS. Education of primary care providers in atypical presentations of acute leukemia may decrease the diagnostic delay.

Intellectual impairment after treatment for medulloblastoma and astrocytoma in childhood: the Brazilian experience.

BACKGROUND: This study investigated the impact of posterior fossa tumors and their respective treatments, and the impact of clinical and sociodemographic variables, on the intelligence quotient (IQ) of Brazilian children. METHODS: Twenty patients took part in the study, of which 13 were diagnosed with astrocytoma (average age at evaluation 10.2 y) and 7 with medulloblastoma (average age at evaluation 9.2 y). The first subgroup was submitted exclusively to tumor resection surgery and the second subgroup underwent surgery, chemotherapy (Vincristine, Cisplatine, and Carmustine), and radiotherapy (total dose of 54 Gy). The Wechsler Intelligence Scale for Children (WISCIII) was used. RESULTS: The following statistically significant effects were identified: treatment modality on performance intelligence quotient scores (P=0.02) and processing speed index (PSI) (P=0.01); presence of hydrocephalus at diagnosis on verbal intelligence quotient (P=0.04); tumor localization on perceptual organization index (P=0.03); time interval between diagnosis and neuropsychological evaluation on PSI (P=0.05) and freedom from distraction index (P=0.03); and level of parental formal education on full scale IQ (P=0.02). CONCLUSIONS: Exposure to radiotherapy has a significant effect on processing speed and consequently on global intellectual capacity. The impact on intelligence of clinical and sociodemographic variables such as tumor localization, time interval between diagnosis and cognitive evaluation, and parental level of formal education is confirmed in the specific setting of a developing country.

Establishment of a pediatric oncology program and outcomes of childhood acute lymphoblastic leukemia in a resource-poor area.

CONTEXT: The cure rate for childhood acute lymphoblastic leukemia (ALL) differs markedly between developed and developing countries. OBJECTIVE: To assess the effect of a multidisciplinary team approach and protocol-based therapy on the event-free survival of children with ALL in an area with limited resources. DESIGN, POPULATION, AND SETTING: A retrospective cohort study at a pediatric hospital in the resource-poor city of Recife, Brazil. We reviewed medical records of the outcomes of 375 children with ALL diagnosed between 1980 and 2002. Eighty-three children were diagnosed in the early period (1980-1989), in the absence of a dedicated pediatric oncology unit, protocol-based therapy, specially trained nurses, 24-hour on-site physician coverage, and ready access to intensive care. Seventy-eight children were treated (all according to protocol) during the middle period (July 1994 to March 1997). During the recent period (April 1997 to December 2002), 214 children were treated with protocol in a dedicated pediatric oncology unit staffed 24 hours by pediatric oncologists and oncology nurses. Improvements were implemented gradually during the middle period and were completed during the recent period. MAIN OUTCOME MEASURE: Event-free survival was estimated by the Kaplan-Meier method. Events included death from toxicity, disease progression or relapse, and abandonment of treatment. RESULTS: The 5-year event-free survival improved steadily: 32% (95% CI, 21%-43%) in the early period, 47% (95% CI, 36%-58%) in the middle period, and 63% (95% CI, 55%-71%) in the recent period. The probability of cause-specific treatment failure in the early, middle, and late periods, respectively, within 1 year of diagnosis was 14% vs 3.8% vs 3.3% for relapse; 6.0% vs 12% vs 9.8% for death from infection; 2.4% vs 13% vs 4.2% for death from noninfectious toxicity; and 16% vs 1.3% vs 0.5% for abandonment of therapy. CONCLUSION: Treatment of childhood ALL in a dedicated pediatric oncology unit using a comprehensive multidisciplinary team approach, protocol-based therapy, and local support and funding is associated with improved outcomes in a resource-poor area.

Guidelines for the recurrence of pediatric cancer.

OBJECTIVES: To provide practice guidelines to assist patients and parents in coping with a return of disease. DATA SOURCES: Delphi studies, review, and research articles. CONCLUSIONS: When a child's cancer recurs, the patient and parents are at risk of physical and psychologic difficulties. Guidelines to assist patients and parents to cope have shown positive response. IMPLICATIONS FOR NURSING PRACTICE: Practice guidelines to assist patients and parents cope with a return of cancer can help health care professionals develop interventions for particular families.

Construções de significados acerca do adoecimento e morte nas narrativas de crianças com câncer / Construction of meanings about illness and death in the narratives of children with cancer

As narrativas surgem para ordenar a experiência humana alterada pela ruptura no seu estado canônico. Nesse sentido, uma doença como o câncer pode ser considerada uma experiência excepcional, que requer a narrativa para significá-la. Esse estudo investigou a construção de significados acerca do adoecimento e da morte nas narrativas de crianças com câncer em etapas distintas do tratamento. Para tanto, foram realizadas seis sessões de brincadeira com cada participante. A análise dos dados demonstrou que crianças com mais tempo de tratamento tendem a finalizar suas narrativas com a morte dos personagens e que crianças com menos tempo de tratamento apresentam narrativas relacionadas ao desconforto físico que o tratamento ocasiona e aos impedimentos que a doença acarreta em suas vidas. Esses resultados apontam para a necessidade de uma maior atenção à forma como as crianças falam do seu adoecimento e da subsequente possibilidade de morte no decorrer do tratamento oncológico, a fim de que possamos atentar para a diversidade dos momentos de tratamento, compreendendo que esses configuram diferentes relações subjetivas da criança para com sua doença e a morte. (AU)

Narratives come out to arrange the human experience altered by the disruption in its canonical status. In this sense, a disease like cancer can be considered an exceptional experience that requires the narrative to mean it. This study investigated the construction of meanings about the disease and death in the narratives of children with cancer in different stages of treatment. Thus, six sessions of play were performed with each child. Data analysis showed that children in longer treatment tend to conclude their narratives with the death of the characters and the children under shorter treatment time present narratives related both to physical discomfort to which the treatment leads to and impairments that the disease cause in their lives. The results indicate the need for greater attention on how children talk about the disease and the subsequent possibility of death during the course of cancer treatment, so that we can be aware of the diversity of treatment moments, understanding that they configure different subjective relationships about children's health problem and death. (AU)

A vivência do pai diante do câncer infantil / The fathers' experience with childhood cancer / La experiencia del padre frente al cáncer infantil

No tratamento de câncer infantil, geralmente a mãe assume os cuidados com a criança doente, tendendo os estudos a focar a relação mãe-filho. Este estudo teve como objetivo compreender a vivência do pai diante do câncer de um filho pequeno. Esta pesquisa é de caráter qualitativo e dela participaram onze genitores que tinham filhos atendidos no ambulatório de um hospital de referência da cidade do Recife - PE. A delimitação da amostra baseou-se no critério de saturação. A coleta de dados foi realizada por meio de entrevistas no período de outubro a dezembro de 2011, as quais foram analisadas pelo método de análise do discurso segundo Minayo (2000). Os resultados apontaram que as mães foram as principais cuidadoras no tratamento, mas os pais também desenvolveram atividades junto a seus filhos doentes que provocaram várias mudanças na sua rotina. O acolhimento e a escuta clínica ou a criação de grupos de apoio para este público-alvo são de grande importância...

In the childhood cancer treatment, the mother often takes care of the sick child. Consequently, studies have focused on the mother-child relationship. This study aimed to understand the father's experience with childhood cancer. A qualitative study was performed and involved 11 parents whose children were treated at the clinic of a referral hospital in Recife-PE. The delimitation of the sample was based on the saturation criterion. Data collection was conducted through interviews during the period of October to December 2011 and analyzed by the method of discourse analysis from Minayo (2000). The results showed that the mother was the main caregiver to the treatment, but the father also carries out activities with the sick child causing several changes in his routine. The host and clinic listening or the creation of support groups for this target audience is important...

En el tratamiento del cáncer infantil, generalmente, la madre asume los cuidados con el niño enfermo, haciendo con que los estudios tiendan a centrarse en la relación madre-hijo. Este estudio tuvo como objetivo comprender la experiencia del padre frente al cáncer infantil. Se trató de una investigación cualitativa, en que participaron 11 genitores cuyos hijos eran atendidos en la clínica de un hospital de referencia de la ciudad de Recife-PE. La delimitación de la muestra se basó en el criterio de saturación. La recolección de datos fue realizada por medio de entrevistas en el período de octubre a diciembre de 2011 y analizados por el método de análisis del discurso de Minayo (2000). Los resultados señalaron que la madre fue la principal cuidadora en el tratamiento, pero el padre también desarrolla actividades junto al niño enfermo acarreando varios cambios en su rutina. Es importante que haya la acogida y la escucha clínica o la creación de grupos de apoyo para este público meta...

Pediatric cancer knowledge: assessment of knowledge of warning signs and symptoms for pediatric cancer among Brazilian community health workers.

BACKGROUND: Field workers and lay community health educators are often children's first opportunity for correctly recognizing and responding to early signs and symptoms of pediatric cancers. Inadequate familiarity with the warning signs and symptoms of childhood cancer results in delayed referral to a physician for diagnosis and treatment. METHODS: This pilot study assessed community health workers' baseline level of knowledge about childhood cancers. Community health workers from Brazil completed a pediatric cancer knowledge questionnaire. RESULTS: Although all respondents knew to refer a child suspected to have cancer to a physician, their knowledge of the early warning signs and symptoms of pediatric cancer was very low. CONCLUSIONS: The findings demonstrate a link between training and knowledge and confirm the need for targeted education in the warning signs and symptoms of pediatric cancer for community health workers in developing countries.

Intellectual aspects of cognitive performance in children after treatment for medulloblastoma and astrocytoma

The present study investigated the relationship between posterior fossa tumors (astrocytoma and medulloblastoma) and their respective treatments and cognitive performance in Brazilian children by measuring Intelligence Quotient (IQ). Twenty children were enrolled in the study, of whom 13 were diagnosed with astrocytoma (average age at evaluation, 10.2 years; eight girls and five boys) and seven were diagnosed with medulloblastoma (average age at evaluation, 9.2 years; five girls and two boys). The first subgroup underwent exclusively tumor resection surgery, and the second subgroup underwent surgery, chemotherapy (Vincristine, Cisplatine, and Carmustine), and radiotherapy (total dose, 54 Gy). The inclusion criteria for the clinical group were normal social and emotional behavior before the diagnosis and motor and visual performance not excessively deteriorated after surgery. All participants were submitted to the Wechsler Intelligence Scale for Children - third version, and their scores were related to standard norms established for Brazilian children and compared with regard to the diagnosis subgroups and treatment modalities. Statistically significant differences were identified between the two diagnosis subgroups in Performance IQ and Processing Speed scores. The medulloblastoma subgroup presented poorer performance in all domains compared with the astrocytoma subgroup. Time interval between diagnosis and neuropsychological evaluation also had a significant effect on Processing Speed and Freedom from Distractibility in the medulloblastoma subgroup. These results provide empirical evidence of a possible significant effect of radiotherapy exposure on processing speed and global intellectual capacity

Diversão em movimento: um projeto lúdico para crianças hospitalizadas no Serviço de Oncologia Pediátrica do Instituto Materno Infantil Prof. Fernando Figueira (IMIP) / Ludotherapy for hospitalized children: a ludotherapy program for hospitalized children in the Pediatrics Oncology Unit of the Instituto Materno Infantil Prof. Fernando Figueira (IMIP)

O presente trabalho descreve as atividades lúdicas desenvolvidas na enfermaria de oncologia pediátrica do Instituto Materno Infantil Prof. Fernando Figueira (Imip), com sessenta pacientes portadores de neoplasias malignas, no período de janeiro de 2004 a janeiro de 2005. Descreve as etapas do Projeto e apresenta os resultados obtidos, correlacionando as etapas do desenvolvimento da aprendizagem com os instrumentos lúdicos utilizados como recurso minimizador do processo de hospitalização, através dos itens mais solicitados, considerando, para essa análise, as idéias de Jean Piaget e Bruno Bettelheim.

This paper describes a ludotherapy program implemented in the pediatrics oncology ward of the Instituto Materno Infantil Prof. Fernando Figueira (Imip) with 60 malignant neoplasia patients from January 2004 to January 2005. It describes the phases of the project, demonstrates results obtained, relating learning development phases and ludic tools used as a resource to mitigate hospitalization hardships through the most requested items. Jean Piaget and Bruno Bettelheim concepts were used in this assessment.