Pulminary Training
WHAT IS ILD?
Interstitial Lung Disease (ILD) is a term that includes a variety of lung diseases. When a patient has ILD, the lung is impacted in three ways.
1. First, the lung tissue is compromised in some known or unknown way.
2. Second, the walls of the air sacs in the lung become inflamed.
3. Scarring begins in the interstitium, and the lung becomes hard.
Shortness of breath during exercise is one of the first symptoms of the disease. A dry cough is usually present. Most patients ignore these systems until they need attention.
Symptoms may vary in severity depending on the type of lung disease diagnosed. X-rays may look alike. Extensive testing is required to establish a positive diagnosis. Some ILD have known causes and some (idiopathic) have unknown causes.
WHY IS IT CALLED INTERSTITIAL LUNG DISEASE?
The tissue between the air sacs of the lungs is called the interstitium. Interstitial lung disease is named after this tissue because this is the tissue impacted by fibrosis. ILD is sometimes also known as IPF (interstitial pulmonary fibrosis). The terms interstitial lung disease (ILD), pulmonary fibrosis (PF) and interstitial pulmonary fibrosis (IPF) may be used for the same diagnosis.
KNOWN CAUSES OF PULMONARY FIBROSIS
Several known causes of pulmonary fibrosis (PF). They include:
1. Occupational and environmental exposures. Many careers - particularly those that involve mining or cause workers exposure to asbestos or metal dusts -- can cause PF. Workers doing these kinds of jobs may inhale small particles, silica dusts, asbestos fibers, fumes that can damage the lungs, especially the small airways, and cause scarring (fibrosis).
Farmers can be affected. Organic substances, such as moldy hay, cause an allergic reaction in the lung. This reaction is Farmer's Lung and can cause PF. Farms are also a source for a number of fumes.
2. Sarcoidosis. A disease characterized by the formation of granulomas (areas of inflammatory cells), which can attack any area of the body but most frequently affects the lungs.
3. Drugs. Certain medications have the undesirable side effect of causing PF. Check with your physician about the medications you are prescribed and ask about side effects.
4. Radiation. (treatment for cancer)
5. Connective tissue or collagen diseases such as rheumatoid arthritis and systemic sclerosis.
6. Genetic/familial. This is not as common as the other causes listed.
IDIOPATHIC PULMONARY FIBROSIS (IPF)
When all known causes of interstitial lung disease (ILD) are ruled out, the condition is called "idiopathic" (of unknown origin) pulmonary fibrosis (IPF).
There are theories as to what causes IPF including viruses and allergic or environmental exposure tobacco and smoking. These theories are being researched. Bacteria and other microorganisms are not thought to be the cause of IPF.
There is also a form of the disease, known as familial idiopathic pulmonary fibrosis (FIPF). Research is being funded to determine whether a genetic tendency to develop the disease, as well as to determine other causes of IPF.
WHAT ARE THE SYMPTOMS OF IPF?
Difficulty breathing is the main symptom of idiopathic pulmonary fibrosis (IPF). Many of the lung diseases and many other illnesses have this symptom making diagnosing IPF on this symptom alone difficult. The shortness of breath usually appears first during exercise. The condition can deteriorate to the point that any exertion will cause difficulty breathing, eventually even at rest the patient will be short of breath. The disease is severe and prolonged, heart failure with swelling of the legs may occur.
WHAT IS THE TREATMENT FOR IPF?
Corticosteroids may be prescribed to treat inflammation present in some people with IPF. Research is continuing on the effectivness of this treatment. Other drugs are added when it is clear that the steroids are not reversing the disease.
Some physicians may use corticosteroids in combination with other medications when the diagnosis is first established. Which treatment plans works, and how long to use the medications is the focus of current research.
100 % oxygen therapy may be prescribed for some patients with IPF. The need for oxygen will be evaluated by your doctor and may depend on the severity of disease, as well as your activity level. Talk with you doctor if you think oxygen is in order, or if you skeptic about oxygen.
Influenza vaccine and pneumococcal pneumonia vaccine are both recommended for people with IPF or any lung disease. These two shots will help prevent infection and keep you healthy.
Rehabilitation and education programs are very beneficial to some patients diagnosed with IPF. Support groups and additional education is important for continuing education.
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