Pulmonary Fibrosis

WHAT YOU SHOULD KNOW:

Pulmonary fibrosis is also called interstitial pulmonary fibrosis, interstitial lung disease, or ILD. Pulmonary fibrosis occurs when the lungs have been damaged and become swollen and fibrotic (scarred). Some common and some rare diseases can cause pulmonary fibrosis, but in most cases, the cause is not known. Shortness of breath is the main symptom of pulmonary fibrosis. It occurs during exercise or even when resting when the disease gets worse.

Pulmonary fibrosis is diagnosed by blood tests, chest x-ray, bronchoscopy, CT scan, or lung biopsy. Treatment may include oxygen, medicine and surgery and may help you continue with your normal daily activities. Ask your caregiver for more information about these tests and treatment.

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

RISKS:

Treatment for pulmonary fibrosis may cause unpleasant effects. Steroids may cause weight gain, high blood pressure, increased chance of infections, or osteoporosis (weak or brittle bones). Drugs that weaken your immune system may cause problems with your blood, liver, or digestive system. Your immune system is the part of your body that fights infection.

Too much pressure on your lungs may cause air leaks or holes in your lungs. You could get an infection or bleed too much if you have lung surgery. Sometimes even with treatment, your disease may still get worse. Ask your caregiver if you are worried or have questions about your disease, care, or treatment.

WHILE YOU ARE HERE:

Informed consent

is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

Medicines:

You may be given the following medicines:

• Antibiotics: This medicine is given to help treat or prevent an infection caused by bacteria.
  
  
• Antiviral medicine: This is given to prevent or treat an infection caused by a germ called a virus. Antiviral medicine may also be given to control symptoms of a viral infection that cannot be cured.
  
  
• Heart medicine: This medicine is given to strengthen or regulate your heartbeat. It also may help your heart in other ways. Talk with your caregiver to find out what your heart medicine is and why you are taking it.
  
  
• Immunosuppressants: These medicines are often given with steroids to weaken the activity of your immune system. The immune system is the part of your body that fights infection.
  
  
• Steroids: Steroid medicine may help to open your air passages so you can breathe easier.

Oxygen:

You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your caregiver before you take off the mask or oxygen tubing.

Pulse oximeter:

A pulse oximeter is a device that measures the amount of oxygen in your blood. A cord with a clip or sticky strip is placed on your finger, ear, or toe. The other end of the cord is hooked to a machine. Never turn the pulse oximeter or alarm off. An alarm will sound if your oxygen level is low or cannot be read.

Tests:

You may need one or more of the following tests:

• Blood gases: This is also called an arterial blood gas, or ABG. Blood is taken from an artery (blood vessel) in your wrist, arm, or groin. Your blood is tested for the amount of oxygen and carbon dioxide in it. The results can tell caregivers how well your lungs are working.
  
  
• Blood tests: You may need blood taken to give caregivers information about how your body is working. The blood may be taken from your hand, arm, or IV.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
  
  
• Pulmonary function tests: Pulmonary function tests (PFTs) help caregivers learn how well your body uses oxygen. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time. PFTs help your caregivers decide the best treatment for you.

Treatment options:

You may need any of the following:

• Surgery: You may have surgery to remove part of your lung. You may also have a lung transplant if your lung has very serious disease. The diseased lung is removed and replaced with a healthy and donated lung.
  
  
• Ventilator: This is a machine that gives you oxygen and breathes for you when you cannot breathe well on your own. An endotracheal (ET) tube is put into your airway through your mouth or nose. You may need a trach if an ET tube cannot be placed. A trach is an airway tube put into an incision (cut) in the front of your neck. The ET tube or trach is attached to the ventilator.

Learn more about Pulmonary Fibrosis (Inpatient Care)