Children’s Interstitial and Diffuse Lung Disease

  • What is intersitial lung disease?

    Children’s interstitial and diffuse lung disease (ChILD) is not one disease or disorder, but a number of them. These diseases cause a thickening of the interstitium (the tissue that extends throughout the lungs) due to scarring, inflammation or fluid buildup. This thickening affects carbon dioxide and oxygen exchange between the alveoli (microscopic air sacs throughout the lungs) and the tissue surrounding them, which eventually affects a patient’s ability to breathe and distribute enough oxygen to the blood.

    There are several different types of interstitial and diffuse lung diseases, including:

    • Those appearing in infancy, mainly with genetic and development causes
    • Those that occur despite a normal immune system, mainly from infections and environmental agents
    • Those that affect the lungs, along with many other organs and systems
    • Those enabled by a weak immune system
    • Those affecting the interstitium (the lung tissue between cells) or platform for the gas-exchanging alveoli
  • Signs and symptoms

    The most common symptoms of chILD include attempts to compensate for lack of oxygen by breathing faster or by over-working muscles in the neck and chest.

  • Evaluation and diagnosis

    In order to diagnose a form of interstitial lung disease, it is crucial to rule out all other possible causes of the contributing signs and symptoms.

    There are a wide variety of tests that can help to confirm your child’s diagnosis, including but not limited to:

    • Chest X-rays
    • Pulse measurement to estimate how well the hemoglobin is carrying oxygen
    • Pulmonary function test (PFT)
    • Blood tests to measure the levels of oxygen and carbon dioxide in the blood
    • Bronchoscopy with bronchoalveolar lavage (BAL)
    • Lung biopsy to diagnose the type of lung disease present
    • Echocardiography to assess heart function
    • Barium swallow to assess for signs of pulmonary aspiration
    • pH test to look for gastroesophageal reflux
    • Sweat test, a common test for cystic fibrosis
    • Skin test for tuberculosis
    • Electrocardiogram to check for heart defects or pulmonary hypertension
    • Exercise tests
    • Genetic testing to check for several types of chILD diseases with known genetic links
  • Treatments

    There is no single treatment for children's interstitial and diffuse lung disease. Each child treated by CHOP’s ChILD Center will receive an individualized medical care plan based on the underlying cause of the disease.

    All treatment regimens will be focused on preventing disease progression and active illnesses, relieving symptoms, and maximizing growth and development. The following are the most common treatment methods available:

    • Oxygen supplementation
    • Bronchodilators
    • Airway clearance to assist with clearing mucus from lungs:
      • Chest physical therapy
      • Positive expiratory pressure therapy (PEP)
      • Intrapulmonary percussive ventilation (IPV)
      • High frequency chest wall oscillation
    • Intravenous immunoglobulin (IVIG): Some forms of chILD are involved with immune responses and will require regular IVIG treatments to stop the progression of fibrosis in the lungs.
    • Anti-inflammatory drugs (steroids, cytotoxic drugs, immunosuppressive drugs): Some forms of chILD respond very well to steroids while others do not. Steroids and immunosuppressive drugs can have many side effects. Be sure to consult an experienced physician about the use of these types of medications in your child’s treatment.
    • Injection of the prescription medication Synagis: Respiratory synctytial virus (RSV) is a common virus that can be especially damaging to the lungs of patients with interstitial and diffuse lung disease. For chILD patients considered particularly high risk, an injection of Synagis (also known as Palivizumab) may be given to reduce the chances of severe infection. Your child could still catch RSV, but the medication may lessen the severity of the infection and protect the lungs from further damage. It is an expensive therapy and only approved by insurance under special circumstances.
    • Lung or heart-lung transplants: Transplantation is considered an option when life expectancy from end-stage lung disease is limited and there are no other available medical or surgical therapies available. Learn more about CHOP’s Lung Transplant Program.