chILD

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General Information

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Childhood interstitial and diffuse lung disease (chILD) is a broad term for a heterogeneous group of rare lung diseases that affect infants and children.  Some children can present acutely with increased work of breathing and respiratory distress, but for others, diagnosis is often delayed due to a more insidious, chronic presentation that results in hypoxia or failure to thrive. ChILD occurs in a variety of clinical contexts, including with respiratory failure in term neonates, in association with systemic disorders such as connective tissue diseases, or after lung injuries. Genetic etiologies are found in an increasing number of cases, yet the underlying causes remain elusive for many children.  Some forms of chILD overlap with ILD that occurs in adults, but there are some entities unique to young children, and the prognosis varies widely. Specifically, Idiopathic Pulmonary Fibrosis, a common and progressive form of ILD in older adults, does not occur in children.  

A 2013 ATS Clinical Practice Guideline focused on the Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy. The approach to evaluation of children with suspected chILD first entails testing to exclude more common causes of respiratory symptoms, such as infection, airway structural abnormalities, or congenital heart disease. The approach to diagnosis then often utilizes laboratory studies, chest computerized tomography (CT) imaging, bronchoscopy, genetic testing, and lung biopsy if clinical severity warrants and less invasive testing fails to identify a cause for symptoms.

Treatment for chILD disorders largely includes nonspecific empiric medications such glucocorticoids and hydroxychloroquine. These therapies have not been rigorously evaluated in clinical trials and available results from clinical experience and uncontrolled studies do not demonstrate clear benefit relative to adverse effects.  Supportive care includes use of supplemental oxygen, focus on nutrition, reduction of infectious risks, coordination with other disciplines, and additional resources for families. 

Current and future priorities in chILD include improved case recognition and diagnosis, infrastructure for multicenter studies, and discovery of the pathogenesis of many forms of chILD including the underlying genetic mechanisms.  There is a critical unmet need to develop and test therapies for chILD.

The Children’s Interstitial and Diffuse Lung Disease Research Network (chILDRN) is a multicenter, interdisciplinary group of providers and investigators which has advanced this field including through improved classification, phenotyping, and a longitudinal Registry in the United States. The American Thoracic Society and the chILD Foundation continue to advocate to increase awareness, facilitate education of families and providers, and promote research needed to accelerate progress to improve the lives of children and families affected by chILD. 

 


Four Facts About chILD

  1. Children's Interstitial Lung Disease (chILD) is not a single disease. There are over 30+ variations of this set of diseases that affect infants and children.
  2. The exact number of chILD cases is not known but it is estimated that there are around 5,000 cases in the United States, making it an "ultra-rare" disease.
  3. The severity of chILD varies with the diagnosis. Most patients will require oxygen therapy, but the amount needed and duration of treatment also varies.  Some will outgrow their symptoms while others will unfortunately require lung transplants to survive. Since these disorders are rare and newly recognized, very little is known about the best treatments or long term prognosis for these children.
  4. Because of the rarity and differences within chILD, there may be 10+ steps to diagnosis children that may have chILD,
Last Reviewed: February 2019