acute and chronic cough

What is childhood interstitial lung disease?

Childhood interstitial lung disease (chILD)

Childhood interstitial lung disease (chILD) describes a group of rare lung diseases that can affect babies, children, and teens. It is also called diffuse lung disease.

These diseases have some similar symptoms, such as shortness of breath, rapid breathing, and coughing. They may also damage the lungs in similar ways, making it harder for oxygen to pass through the lungs and into the body.

Some children are born with an interstitial lung disease (ILD) while others develop an ILD later during childhood. ChILD can be mild, serious, or life-threatening. Some types of chILD may get worse over time. Other types may improve or stay the same. There is currently no cure for chILD, but early diagnosis and treatment can help improve symptoms. Your child’s healthcare providers can help you learn how to manage the condition to improve your child’s quality of life.

Types of chILD that are more common in children younger than 2 include:

  • Developmental disorders, such as alveolar capillary dysplasia: These are problems with how your child’s lungs develop before birth. They are often serious, and you may notice symptoms within days after your baby is born.
  • Genetic surfactant disorders: Surfactant is a substance that coats the inside of your lungs and helps them work better. Mutations, or changes, in the genes that control how your child’s body makes surfactant can lead to lung damage.
  • Neuroendocrine cell hyperplasia of infancy (NEHI):
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     This is a rare lung disease that causes rapid, noisy breathing and low oxygen levels. NEHI has no known cause. It is also called persistent tachypnea of infancy.
  • Pulmonary interstitial glycogenosis: This is a rare lung disease in which a sugar called glycogen builds up in some cells in the lungs. It usually occurs in children younger than 1.

Types of chILD that happen in children of any age may be caused by:

  • Another medical condition: Infections, other lung diseases, and problems with your immune system can cause chILD.
  • Dust or mold in the environment: These materials may cause hypersensitivity pneumonitis and bronchiolitis obliterans.

Symptoms and diagnosis

Symptoms of chILD may include:

  • Shortness of breath, or trouble breathing: Your child may have rapid breathing, flaring nostrils, a grunting sound when breathing out, or a high-pitched sound when breathing (wheezing). Breathing problems are more commonly seen in babies while eating and in children during physical activity.
  • Pulling inward of the muscles between the ribs when breathing (chest retractions)
  • A chronic (long-term) cough
  • Problems with growth and development
  • Bluish skin
  • Clubbing, a widening and rounding at the ends of the fingers or toes, along with a downward sloping of the nails

Your child’s healthcare provider will diagnose chILD based on your child’s family and medical histories, physical exam, and results from lung tests, blood tests, and genetic testing.

Causes and risk factors

Genetics, infections, or repeated exposure to things in the environment that damage the lungs or make it easier for your child’s lungs to get damaged may cause chILD. This lung damage can lead to inflammation and scarring in your child’s lungs. Problems with how the lungs develop before birth can also cause chILD. Many times, your child’s doctor may not know what caused this condition.

Childhood ILD is rare, so most children are not at risk. However, your child’s environment, family history, medicines, and other medical conditions can raise the risk of chILD:

  • Environment: Repeated or long-term exposure to substances in the environment can raise the risk of chILD. Such substances include bacteria, fungi, chemicals, tobacco smoke, and air pollution.
  • Family history: Mutations, or changes, in the genes that control lung development can cause chILD. Other changes in your genes can affect how your body makes surfactant. Your child may have a higher risk of some types of chILD if other people in your family have had ILDs or medical conditions that can cause chILD.
  • Lifestyle habits: Illegal drugs, such as cocaine, can hurt the lungs and cause chILD. This is more common in teens.
  • Medicines: These include radiation, chemotherapy, medicines that weaken your immune system, antibiotics, heart medicines, and medicines to treat inflammation.
  • Other medical conditions: Certain health problems that affect the lungs can raise the risk of chILD. These conditions include the following:
    • Aspiration, which is when substances such as food, liquid, or vomit get into your lungs
    • Autoimmune diseases, such as lupus, rheumatoid arthritis, scleroderma, inflammatory bowel disease, eosinophilic lung diseases, and vasculitis
    • Infections from bacteria or viruses
    • Lung cancer
    • Problems with your child’s metabolic system
    • Complications after a lung transplant

Treatment

There is currently no cure for chILD. Your child’s doctor may prescribe medicine to help slow down or stop lung damage and help your child breathe easier. Treatment for chILD depends on the type and the cause. If medicine does not work or your child’s symptoms get worse, they may need oxygen therapy or ventilator support.

Your child will need ongoing care from a healthcare team. This team may include doctors, nurses, dietitians, social workers, physical therapists, and home health aides. Your child may need medicines, pulmonary rehabilitation, oxygen therapy, or procedures.

Managing chILD

The following steps can help manage your child’s condition:

  • Receive routine follow-up care. It is important to follow your child’s treatment plan. For some types of chILD, the condition may get worse over time. For other types of chILD, the condition may improve.
  • Make sure that your child receives recommended vaccines, including the flu, pneumonia, and COVID-19 vaccines if eligible. This can help prevent infections that make your child’s symptoms worse.
  • Know the warning signs of worsening lung disease and when to seek emergency medical care. Call 9-1-1 if your child has trouble breathing or a bluish color on the skin, lips, and fingernails; or if your child becomes confused, very sleepy, or unconscious.
  • Ask your child’s doctor about whether your child needs nutritional supplements.
  • Help your child avoid secondhand smoke, which is smoke in the air from other people smoking. Also, keep your child indoors when the air quality is poor.
  • Talk to other people in your child’s life to discuss the special needs related to chILD. For example, you may need to work with your child’s teachers to decide how to meet school-related needs.
  • Take care of your mental health. Living with or caring for a child who has an ILD may cause fear, anxiety, depression, and stress. Get support to help you, your child, and your other family members cope with the effects of chILD on daily life.

Source: https://www.nhlbi.nih.gov/health/childhood-interstitial-lung-diseases

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