What is Pulmonary Fibrosis?
By Brenda Kimble
Pulmonary fibrosis (PF) is a respiratory disease where the lungs slowly lose the ability to function due to scarring. This scarring causes the passageways to become thick and hard, which inhibits the lung’s ability to move oxygen through the walls of the alveoli and into the bloodstream. Patients with pulmonary fibrosis find themselves unable to catch their breath due to improper oxygenation of the blood.
Pulmonary fibrosis is treatable, but there is currently no cure for the disease. Treatments that are offered to PF patients will not reverse the damage that has already been done to the lungs, nor will it make the disease go away. They can, however, both improve the symptoms associated with PF and possibly delay the progression of more internal scarring.
What are the Stages of Pulmonary Fibrosis?
Pulmonary fibrosis has four stages. Each stage marks the patient’s symptoms growing progressively worse. The symptoms of pulmonary fibrosis include shortness of breath, fast and shallow breathing, a dry hacking cough, chronic fatigue, aching joints and muscles, and unintended weight loss. These symptoms should be discussed with a doctor. The MD may ask about the frequency with which the patient loses their breath or how often they find themselves working through a coughing fit.
Staging is also diagnosed by how well a patient scores on something called a forced vital capacity test or FVC. This test measures the quantity of air that’s exhaled during a forced breath. A spirometer measures the volume of air, and depending on the score, the stage of pulmonary fibrosis is then assigned. The FVC test is quick and painless, taking approximately three minutes to complete.
FVC test scoring is as follows:
- Greater than 75% = mild (stage 1 PF)
- 50% – 75% = moderate (stage 2 PF)
- 25% – 49% = severe (stage 3 PF)
- Less than 25% = very severe (stage 4 PF)
Unfortunately, this staging system has come under some scrutiny. Some researchers believe these stages are arbitrary because pulmonary fibrosis does not progress on a linear timeline, and all patients progress differently.
New Scoring Gap Model
A new scoring system called the GAP Model has been developed. GAP uses the gender, age and physiology of the patient while also including FVC scores to determine the stage of pulmonary fibrosis.
The patient may also be asked to do a six-minute walk test, which measures individual exercise capacity. During this test, the patient is asked to walk at a normal pace for six minutes. This measures the patient’s aerobic exercise capacity and helps the doctor make an accurate diagnosis of how severe pulmonary fibrosis has advanced.
Pulmonary fibrosis is a terminal disease, but there is no set lifespan for patients. Every individual handles the disease differently and the severity of symptoms and the speed at which the disease progresses is very much dependent on the person’s age, lifestyle and health. Some people live with pulmonary fibrosis for many years, while others experience a quick decline in health.
Anticipating Depression
Being diagnosed with such a severe disease takes a physical toll and a mental one as well. After learning of their diagnosis, many individuals respond with anger and grief. This is considered a normal reaction. 
But often, the patient’s grief spirals into depression. Caregivers should be aware of the signs of depression and seek out professional help if necessary. Baylor University Medical Center researchers believe severe depression affects nearly 77 percent of terminally ill patients.
If you suspect that your loved one is struggling with depression, don’t hesitate to seek out the help of a therapist. Look for a counselor with experience dealing with cases of terminal illness. You should not try to be a counselor yourself.
A few markers of depression you should keep an eye out for include: trouble sleeping, a loss of interest in favorite activities, touchiness and a loss of appetite. Patients suffering from severe depression may also show an emerging dark side. They may become preoccupied with death or may constantly use phrases like “after I’m gone.” While it’s true that their remaining time may be limited, it’s important for patients with any terminal illness to try to enjoy the time they have left–not obsess over what will happen when it’s “over.”
In Case of Exacerbation
Depression can exacerbate an illness and this is particularly dangerous with pulmonary fibrosis. When PF patients have a sudden worsening of symptoms, the scarring of the lungs increases as well. As this scarring intensifies, the patient experiences even more difficulty breathing.
During acute exacerbation, physicians use oxygen levels, CT scans and a variety of other tests to help determine the severity of and discover the reason for the exacerbation. Unfortunately, during acute exacerbation of pulmonary fibrosis, many patients find themselves in the hospital. If the exacerbation is severe enough, patients may be put on a ventilator to assist with breathing.
If this occurs, the mortality rate of the patient increases. As a caregiver, you should talk with your loved one as soon as possible about their wishes. Then help them create a living will. That way, you’ll be prepared to be a strong advocate for their wishes while transitioning through end of life and after death.
What About Hospice Care?
Taking care of someone with a terminal illness is a difficult task. It’s sometimes too difficult to be done properly–especially if the caregiver has a full-time job, kids to take care of and other pressing matters to attend to. Hospice care van be the best option for someone in advanced stages of pulmonary fibrosis. Hospice offers high quality care, supervision, and caregiver education to manage symptoms to support dignity, quality of life and comfort. Emotional support is also available from trained counselors and social workers to assist patients and caregivers during this transition. If you’d like to look into hospice care, a good time frame to start is once your loved one’s doctor believes there are only six months to a year left in their life. Have the physician order an evaluation from a hospice agency.
A Note to Caregivers
As a caregiver, you have one of the most important jobs when it comes to your loved one’s quality of life. While it’s imperative to know how to offer them the comfort and support they need, it’s a challenging task when you must deal with your own grief. It’s crucial to remember that even if the person becomes withdrawn or lashes out in anger due to their own sorrow, they’re still the same person you knew before they were diagnosed with pulmonary fibrosis.
Most terminally ill patients want to be treated the same way they’ve always been. Constantly focusing on their illness only makes dealing with it more difficult. Look back on the good times and encourage your loved one to enjoy the time you have together.
Brenda Kimble is the Pulmonary Fibrosis Now Program Coordinator at pulmonaryfibrosisnow.com
