A continuation of Part 1, Part 2 outlines the following topics covered in the second half of the video by Dr. Case:
Anxiety and depression
(The PFF reminds listeners that the information presented is for educational purposes only and is not a replacement for professional medical advice by a personal physician.)
Treating cough (28:31)
Rather than just applying a “band aid,” it is important to first identify other possible causesof the cough besides PF. If acid reflux or allergies are present, for example, those triggers should first be addressed.
Secondly, whether the cough is new or has changed is important to consider and to not assume that it is PF progression.
Once the cause has been identified, the cough reflex can be targeted. Treatments may include:
Non-specific cough suppressants like guaifenesin (over the counter), which has had some success with PF, and benzonatate (by prescription, non-narcotic).
Low-dose steroids, sometimes considered just for PF-related cough, while they may be used for ongoing disease management in other types of PF.
Gabapentin, which targets the nerves involved in the cough reflex.
Opiates (narcotics) in the form of prescription cough syrups, particularly for more severe cases.
There are also clinical trials underway for experimental cough treatments:
Thalidomide has been shown to decrease cough and increase quality of life in people with idiopathic pulmonary fibrosis (IPF) in a small randomized controlled trial. While the drug has serious side effects including birth defects and is not easily accessible, it can have a place in treating cough.
RVT-1601 is an experimental cough treatment being studied in people with PF in the ongoing SCENIC trial.
Gefapixant has been in development for general chronic cough and has now been studied for IPF, with no results yet published.
Acid reflux (31:40)
Acid reflux, or gastroesophageal reflux disease (GERD), is more than a common cause of cough. There is evidence that silent, recurrent reflux and aspiration of acidic stomach contents can lead to ongoing injury of lung tissue and eventually affect lung function. This is particularly a risk factor in people susceptible to lung fibrosis or inflammation. With or without cough, treating acid reflux may be beneficial if reflux is believed to be contributing to PF progression.
Research supports taking an acid-reducing medicine for reducing loss of lung function. One study of people with IPF showed those taking either a proton pump inhibitor (PPI) or H2 receptor blocker experienced less decline in forced vital capacity (FVC) over 30 weeks than those not taking an acid-reducing treatment.
Another study of acid reflux treatment in IPF is the WRAP-IPF trial. This study showed that people with IPF receiving a WRAP surgical procedure to correct GERD had less adverse disease-related outcomes after 48 weeks than those not receiving the procedure.
After breathlessness and cough, fatigue, or lack of energy, stands at #3 on the list of top problematic symptoms in PF.
There are many factors contributing to fatigue in PF:
PF disease itself, especially if the underlying cause is a systemic issue (example, autoimmune)
Low oxygen levels, day or night and with or without sleep apnea (sleep apnea common with PF and not always easily detectable with snoring and frequent waking)
Deconditioning and weakness
Mental or social stress
Emotional stress (anxiety, depression)
Comorbid conditions (for example, pulmonary hypertension) or other conditions unrelated to PF
Medication side effects
Excessive caffeine or alcohol intake
Treating fatigue (39:20)
Pulmonary rehabilitation is very effective at helping to reverse the downward spiral of lack of physical activity and deconditioning leading to increased shortness of breath and lack of energy.
Supplemental oxygen is very helpful when oxygen levels are low (hypoxia) and should be used during these times. (Again, the lungs are not able to “store” oxygen with sporadic use.) Levels should be tested during rest, exertion, and sleeping.
The underlying causes of fatigue must be investigated and addressed, whether sleep apnea, anemia (a lack of healthy red blood cells to carry oxygen), thyroid issues, emotional problems, etc. They may or may not be related to PF.
Sleep apnea (41:05)
An important cause of fatigue, presence of any sleep apnea should be determined and treated. Untreated obstructive sleep apnea (OSA) may lower survival in people with PF.
OSA is very common with IPF. One study of 50 people with IPF found some type of OSA in nearly 80% of people and moderate-to-severe OSA in 64% of the people.
OSA, in conjunction with normal sleep-related factors like the brain resetting itself and shallow breathing, can reduce oxygenation of the body and affect sleep quality, leading to daytime fatigue, even if supplemental oxygen is not needed during exertion..
There are a multitude of symptoms of OSA:
Gasping or choking while sleeping
Excessive tiredness when awake
Dry mouth or sore throat upon waking
Breathing interruptions during sleep testing
Not all of these symptoms are always present in PF, and the tools for detecting OSA are not always accurate. A sleep study is needed for diagnosis, assessing severity, and determining treatment.
OSA impacts not just PF but can worsen other conditions, as well: cardiovascular, neurological, blood sugar, etc. So it is important to treat.
CPAP (continuous positive airway pressure) is the primary treatment for sleep apnea, though other therapies exist depending on severity.
Psychological support (for example, “talk therapy” and cognitive behavioral therapy)
Support groups (talking with like-minded peers for emotional support and practical disease management tips)
Medications (including those aimed at panic disorders) prescribed by a psychiatrist
Palliative care (47:23)
Palliative care is not hospice care in most cases, but rather symptom management aimed at quality of care at the right time and place.
There are several categories of palliative care:
Hospice (given at end of life)
Pain management, which often overlaps hospice and curative or life-prolonging treatment
Comfort care, often overlapping hospice and infusion therapy
Implementation of symptom-based palliative care generally begins slowly in progressive chronic disease and ramps up as a person moves from early disease onset with minimal symptoms to experiencing worsening symptoms and frequent disease complications. At that point, curative or life-prolonging care gives way to quality of life care that culminates in hospice.
Quality of life can be affected by many factors in PF, including but not limited to:
Depression and anxiety
A quick recap of treatments for each of these factors is given.
Following the presentation, beginning at 52:25, Dr. Case addressed two listener questions:
“I am on O2 therapy (3 lpm) and when I move around my O2 level falls into the low 80s or even 70s. When I notice it’s that low, I stop what I’m doing and wait until my O2 level rises back to at least 90. My question is are these routine episodes of low O2 levels doing any damage to my heart, brain, etc.?”
“How do we participate in pulmonary rehab under COVID conditions and are there any guidelines?”