Interstitial Lung Disease in Children

One of the most frightening times for any parent is when they have a sick child. It's even more frightening when doctors cannot diagnose the problem and are at a loss at how to treat it. Interstitial lung diseases in children are rare. And while they are difficult to diagnose, they can be successfully treated.

Significance

• Interstitial lung diseases (ILD) are rare lung disorders, caused by inflammation, that results in scarring and thickening of interstitial tissues, causing chronic chest problems or breathlessness. While there is some overlap with adult ILD, ILD in children (called chILD) is medically very different. In children, ILD can begin immediately after birth and is usually diagnosed within the first year of life. However, in some cases ILD develops and is diagnosed within a wide range of ages throughout childhood. While the specific causes of these diseases in children are unknown, most are thought to be inherited or the result of environmental conditions. It is also important to note that interstitial lung diseases are not contagious.

Symptoms

• The lungs are responsible for carrying oxygen into the bloodstream and removing carbon dioxide. Chronic inflammation of the lung tissue leads to scarring (fibrosis) of the air sacs. The scarring interferes with the lungs' ability to deliver oxygen to the blood. When oxygen is unable to enter the blood, the lungs become stiff and begin to shrink, resulting in increased breathlessness. While symptoms usually depend on the exact cause of the condition, dry cough and shortness of breath are very common symptoms. In most cases, these symptoms are not initially present, but develop gradually. Some signs of prolonged low levels of oxygen in the blood include an enlarged heart and clubbing of the fingertips.

Identification

• Identifying interstitial lung diseases, as well as what causes them, can be extremely challenging, especially in children. After a series of physical exams, a thorough study of the family's medical history, and ruling out other possible conditions, doctors will usually run a gamut of tests before making a diagnosis of interstitial lung disease. Some imaging tests that have been used to identify ILD in child can include chest radiology, high-resolution CT scanning and barium swallow studies.

  Chest radiology (X-ray) is often the first test given in cases of suspected lung problems, especially in children. However chest X-rays are not as effective as CT scans in diagnosing ILD. Barium swallow study tests ("milk" tests) are also used to find evidence of esophageal reflux, which may signal a chronic lung disorder.

Considerations

• An extraordinarily large number of disorders can be mistaken for interstitial lung diseases, sometimes resulting in an improper diagnosis. While some interstitial diseases present specific symptoms and have identifiable causes, others have no distinctive characteristics and the cause is unknown. Doctors must rule out other medical conditions that can mimic interstitial lung disease--for example, asthma--before making a diagnosis.

  There is now new evidence that some of interstitial lung disorders are seen more frequently in infants, while others are more specific to older children, making the initial diagnosis more accurate than ever before.

Prevention/Solution

• Since there is no exact known cause of interstitial lung diseases in children, it is nearly impossible to take measures against prevention. However, doctors advise women to not smoke and avoid second-hand smoke while pregnant. They also recommend that pregnant woman do not expose themselves to certain chemicals that could be toxic and potentially fatal to the fetus.

  There is no cure for interstitial lung diseases in children or adults, but health care providers commonly recommend oxygen therapy, treatment with inhaled steroids and a number of anti-inflammatory medications (usually steroids). While surgery (lung transplants) is a possibility, it is more of an option for adults, as less than five percent of patients under the age of 18 have had lung transplants.