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. 2015 Mar;33(1):1–2. doi: 10.1016/j.rpped.2014.12.001
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Nutrition in Cystic Fibrosis: as important as the management of pulmonary disease

Fabíola Villac Adde 1
PMCID: PMC4436949  PMID: 25623727

In recent decades, the survival of patients with cystic fibrosis (CF) has increased significantly, with a current median of approximately 38 years in European and North American countries.1 , 2 Currently, almost 50% of CF patients in the United States are adults.2 A better approach in the treatment of pulmonary disease and improvement of patients' nutritional status were responsible for these changes.

In the 80s, Corey et al, comparing two similar groups of patients with CF followed in Boston and Toronto, observed increased survival in the group from Canada (30 versus 21 years).3 The better nutritional status of these patients was the determining factor for this difference, as the degree of pulmonary involvement was comparable in both groups. While patients from Toronto received a diet without restriction of fat, the ones from Boston received a low-fat diet and, therefore, one with lower caloric intake. It was concluded that although the progressive pulmonary disease is the leading cause of mortality in CF, concerns with nutritional guidance and intervention in these patients was essential.

Many studies have suggested that severe pulmonary disease is consistently correlated with nutritional status deterioration and, on the other hand, prevention of malnutrition is related to a better course of pulmonary disease and longer patient survival.4 , 5 Several authors have shown that patients undergoing aggressive nutritional rehabilitation through high-calorie diets by gastrostomy or nasogastric tube, improved their nutritional status, while pulmonary disease was stabilized.6 , 7

A national study, evaluating 87 patients with CF aged 6 months to 18 years and grouping them according to overall disease severity measured by the Shwachman score (CF severity score) also showed that patients with less pulmonary involvement had better nutritional status and those with increased pulmonary involvement showed poor nutritional status, with significant differences between the groups regarding the different anthropometric variables assessed. The use of simple measures of nutritional advice in the long term allowed attaining greater adherence to enzyme supplementation and high-calorie dietary supplements, contributing to nutritional status improvement observed in some of these patients.8

Hortencio et al, in an article published in this issue of the journal and that evaluated 52 patients with a mean age of 6 years, whose CF diagnosis was made at a median close to two years, found 40.4% of patients with Z BMI / A scores <-2 and 38% with H/A score <-2 at the first visit to the referral center, which reflected severe nutritional impairment. With the implementation of therapeutic measures - enzyme supplementation, hypercaloric diet and pulmonary disease management - most patients showed nutritional recovery, with a decrease in BMI/A and H/A Z scores <-2 to 7.7% (only 4 patients) in both indices. However, as the authors emphasize, patients with BMI/A and H/A Z scores between -1 and -2 must be considered at nutritional risk and this was verified in 23 and 30.8% of patients in this study, respectively.

This sentence group of patients must undergo stringent monitoring of nutritional status. Some associations between better anthropometric indices and lower severity of respiratory disease were observed as fewer hospitalizations, later respiratory symptom onset and better results on spirometry. However, the finding of a better H/A index with longer time between birth and diagnosis and between the first visit and diagnosis was paradoxical, as 90.8% of patients were pancreatic insufficient and in this situation, one would expect a worsening in this anthropometric index with diagnostic delay and thus, at the start of pancreatic enzyme supplementation.9

With the implementation of newborn screening for CF in the state of São Paulo in February 2010 10 we expect to attain an early diagnosis of CF, allowing timely therapeutic interventions to be made and attaining benefits such as improved growth, nutrition, pulmonary function and prognosis, as observed in countries where newborn screening for CF is already a routine.11 , 12

References

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Rev Paul Pediatr. 2015 Mar;33(1):1–2. [Article in Portuguese]

Nutrição em fibrose cística: tão importante quanto o manejo da doença pulmonar

Fabíola Villac Adde 1

Nas últimas décadas, a sobrevida dos pacientes com fibrose cística (FC) aumentou expressivamente, com mediana atual de 38 anos em países europeus e americanos.1 and 2 Atualmente, quase 50% dos pacientes com FC nos Estados Unidos são adultos.2 A melhor abordagem no tratamento da doença pulmonar e a melhoria do estado nutricional desses pacientes foram os grandes responsáveis por essas mudanças.

Já na década de 1980, Corey et al., comparando dois grupos semelhantes de pacientes com FC acompanhados em Boston e Toronto, observaram maior sobrevida no grupo do Canadá (30 versus 21 anos). 3 O melhor estado nutricional desses pacientes foi o fator determinante dessa diferença, pois o grau de acometimento pulmonar era comparável nos dois grupos. Enquanto os pacientes de Toronto recebiam dieta sem restrição de gordura, os de Boston recebiam dieta hipogordurosa e, portanto, com menor aporte calórico. Concluiu-se, que apesar de a doença pulmonar progressiva ser a maior causa de mortalidade na FC, a preocupação na orientação e na intervenção nutricional desses pacientes era fundamental.

Muitos estudos sugerem que a doença pulmonar grave se correlaciona consistentemente com a pioria do estado nutricional e que, por outro lado, a prevenção da desnutrição está relacionada com melhor curso da doença pulmonar e maior sobrevida do paciente.4 and 5

Diversos autores mostraram que pacientes submetidos a reabilitação nutricional agressiva, por meio de dietas hipercalóricas por gastrostomia ou sonda nasogástrica, melhoraram o estado nutricional e ao mesmo tempo estabilizaram a doença pulmonar.6 and 7

Estudo nacional, que avaliou 87 pacientes com FC de seis meses a 18 anos e os agrupou de acordo com a gravidade geral da doença, medida pelo escore de Shwachman (escore de gravidade da FC), também mostrou que pacientes com menor acometimento pulmonar apresentavam melhor estado nutricional e aqueles com maior acometimento pulmonar encontravam-se muito comprometidos do ponto de vista nutricional. As diferenças entre os grupos foram significativas nas diversas variáveis antropométricas avaliadas. Com medidas simples de aconselhamento nutricional em longo prazo, foi possível obter-se maior adesão à suplementação enzimática e ao uso de suplementos dietéticos hipercalóricos, o que contribuiu para a melhoria do estado nutricional observada em uma parcela desses pacientes.8

Hortencio et al., em artigo publicado nesta edição da revista e que avaliou 52 pacientes com média de seis anos cujo diagnóstico de FC ocorreu em mediana próximo aos dois anos, encontraram 40,4% dos pacientes com escore Z IMC/I <-2 e 38% com escore Z A/I <-2 na primeira consulta no centro de referência, o que refletiu grave acometimento nutricional. Com a implementação das medidas terapêuticas - suplementação enzimática, dieta hipercalórica e manejo da doença pulmonar - houve recuperação nutricional na maioria dos pacientes e caíram os valores dos escores Z IMC/I e Z A/I abaixo de -2 para 7,7% (apenas quatro pacientes) em ambos os índices. Porém, como os próprios autores frisam, pacientes com escores Z de IMC/I e de A/I entre -1 e -2 devem ser considerados em risco nutricional e isso ocorreu em 23% e 30,8% dos pacientes desse estudo, respectivamente. Esse grupo de pacientes merece vigilância extrema do estado nutricional. Algumas relações entre melhores índices antropométricos e menor gravidade da doença respiratória foram constatadas, como menos internações, manifestações respiratórias mais tardias e melhores índices na espirometria. Entretanto, foi paradoxal o encontro de relação de melhor índice de A/I com maior tempo tanto entre o nascimento e o diagnóstico quanto entre a primeira consulta e o diagnóstico, uma vez que 90,8% dos pacientes eram pancreato-insuficientes e, nessa situação, esperar-se-ia uma pioria nesse índice antropométrico com o retardo no diagnóstico e, portanto, no início da suplementação das enzimas pancreáticas.9

Com a implantação da triagem neonatal para FC no Estado de São Paulo em fevereiro de 201010 espera-se que tenhamos o diagnóstico precoce da FC, o que permitirá intervenções terapêuticas em tempo oportuno e se obterem benefícios como melhor crescimento, nutrição, função pulmonar e prognóstico, como observado nos países onde a triagem neonatal para FC já é rotineira.11 and 12

Articles from Revista Paulista de Pediatria are provided here courtesy of Sociedade De Pediatria De Sao Paulo

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