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General Information

chILD Week

Children's interstitial lung disease (chILD) encompasses diverse pathologies that are unique to young children and, in particular, infants under 2 years of age. These can include genetic abnormalities, as well as desquamative interstitial pneumonitis (DIP), chronic pneumonitis of infancy, pulmonary interstitial glycogenosis (PIG) or neuroendocrine cell hyperplasia of infancy (NEHI). chILDs are associated with symptoms of breathlessness, fast breathing, coughing (sometimes producing blood), and general weakness linked to hypoxemia (low blood oxygen levels). There is no cure for any type of chILD. Corticosteroids and hydroxychloroquine are the most common treatments, which suppress inflammation and modify tissue repair with an aim to mollify morbidity and worsening of pathology. Some children will require regular or intermittent oxygen therapy. One form of chILD, hypersensitivity pneumonitis, which can be caused by an exposure to pigeons, other birds or some molds, is controlled by removing the child from these exposures. These interventions have value in tempering symptom worsening and pathophysiology, they are not restorative. For some children with severe disease, a lung transplant is the best treatment.

Pathological changes in chILD directly affect the gas exchange units of the lung (alveoli) and their surrounding tissues (the "interstitium"). Historically, chILDs have been defined based on the appearance of tissues from lung biopsies. More recently noninvasive genetic testing and use of computed tomography (CT) scanning to assess the lung are used for specific forms of chILD. As these changes occur in infants and children, chILDs are associated with growth and developmental abnormalities as well as immunological problems. Greatly compromised is the ability for oxygen to diffuse into the blood stream and for damaging carbon dioxide to diffuse out from the blood stream in the lungs. This reduces the efficiency of the lungs to saturate the bloodstream with oxygen that is vital for performance and survival of all tissues in the body. In addition, the "scarring" and thickening of the lung tissues in chILD can lead to stiffening of the lungs, resulting in restrictive lung pathophysiology in which the work to expand the lungs for breathing, even at rest, becomes increasingly more difficult.

Why Does chILD Develop?

Some types of chILD are unexpected with no known cause. Genetic causes include genes linked to surfactant production. As surfactant is necessary for the air sacs to stay open and allow oxygen diffusion into the blood, disturbed surfactant production greater hampers lung function. Some types of chILD can be traced to the presence of others diseases, and these may include infections, cystic fibrosis, and immunodeficiency disorders. chILD likely evolves from some type of injury to the distal airspaces. Fibroblast are cells of the lungs that can accumulate and be "activated" to generate the deleterious lung tissue remodeling (fibrosis) associated with chILD. Understanding biological mechanisms for this response, and how it may occur in the presence or absence of lung inflammation, is an area of ongoing fundamental research. Inflammation, if present, may be due to infection or hypersensitivity, and includes immune cells such as neutrophils, lymphocytes and macrophages that accumulate in the airspaces.

As was the case for adult ILDs a decade ago, the heterogeneity of the chILD disorders and lack of consensus of what constitutes a chILD disorder is a significant barrier to consolidating results across studies, something needed to make real headway for diagnosis, treatment and understanding disease causes. To overcome this barrier international studies that include active disease surveillance that enables creation of complementary patient registries are required to advance understanding and management of chILD. More research is also needed to understand the cause and pathophysiology of chILD, perhaps identifying diagnostic biological markers that can be used for rapid and definitive diagnoses, and that shed understanding of the disease process.  The Rare Lung Diseases Consortium is a network of clinical and research centers and patient support organizations that accelerate clinical research in rare lung diseases, including chILD, and it is hoped that collaboration with centers worldwide, may facilitate use of standardized diagnostic criteria and develop a network for clinical trials.

Four Facts About chILD

  1. Children’s Interstitial Lung Disease (chILD) is not a single disease. It is a group of several rare disorders that affect infants and children.

  2. 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.

  3. Since these disorders are rare and newly recognized, very little is known about the best treatments or long term prognosis for these children.

  4. The numbers of cases of chILD is not known, but doctors and researchers are working collaboratively worldwide to quantify these syndromes.