The key topics of discussion during a new webinar for people with pulmonary fibrosis (PF) focused on strategies — ranging from getting appropriate vaccines and managing individual symptoms to taking advantage of palliative and support care — for improving patient quality of life.
The talk was given by Marlies Wijsenbeek, MD, PhD, from the Erasmus University Medical Center in the Netherlands, whose research has addressed patient-centered outcome measures in lung disease. It was the fourth monthly webinar hosted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF), as part of a lead-in to the first European PF Patient Summit that will take place next month.
While a person’s quality of life is determined by a variety of factors, including self-esteem and interpersonal relationships, physical health is one major determinant. For this reason, a chronic disease like PF can have a major impact on one’s overall quality of life.
“There’s so many ways that a disease can influence your life,” Wijsenbeek said. “Some people say, ‘I’m tired, I’m more easily upset.’ The others say, ‘I cannot work in my garden, I’m really short of breath … I worry for my family, I have cough, I feel embarrassed.’”
Consistently, several studies have shown that PF patients generally have a poorer quality of life than people without the disease. Plus, people with the disease are not the only ones whose well-being is affected.
“Let’s not forget about the system around someone — the family, the partners,” Wijsenbeek said, noting that loved ones of people with PF often experience stress and anxiety related to the disease.
Wijsenbeek shared several self-management strategies for improving the quality of life for those with PF. She noted, however, that there is little research to support these recommendations, and explained they are mainly based on expert opinion.
Some of these management strategies were self-evident, like quitting smoking, as smoking is a known trigger of fibrosis (tissue scarring).
She also encouraged people with PF to avoid other possible sources of airway irritation, such as those found in certain work environments, and she highlighted the importance of vaccination to prevent other diseases that also may affect the lungs, including the flu and COVID-19.
When possible, “mechanical stress” on the lungs should be avoided, she said, as this can promote the release of pro-fibrotic signaling molecules. Simply put, this means that people with PF should, whenever possible, avoid surgeries that require general anesthesia, since mechanical ventilation used during such procedures can put stress on the lungs.
“For instance, if you break your ankle and you could have local anesthesia, that would [be preferred],” Wijsenbeek said.
Proper dietary management also may be important in PF, as studies have shown that people with fibrosis who lose more weight over the course of the disease tend to have worse clinical outcomes, or lower survival rates. Specifically, those who have more than 5% weight loss in a year lost more lung function, one study found.
“Currently, we all advise [that] if you lose a lot of weight and cannot manage to eat yourself, to go and see a dietitian,” Wijsenbeek said.
In recent years, technological advances have made it possible for some people with PF to monitor their health at home, at a level that was previously only possible in a hospital. Early data suggests that engaging in this kind of at-home monitoring can improve quality of life, particularly patients’ psychological well-being, according to Wijsenbeek.
She also said that participating in a clinical trial — in addition to being a crucial part of research to advance the development of new treatments and PF management — can help improve patients’ quality of life. Wijsenbeek encouraged patients to learn ask their physicians about upcoming or ongoing trials or even do a little bit of research themselves, online, to see what specific topics are currently being studied.
“We do know from research that patients that participate in a trial report that they were more hopeful, felt more empowered, and felt that they contributed,” Wijsenbeek said, noting that these effects are independent of any actual treatments given during a trial.
As such, she also emphasized the need for healthcare providers to inform patients about trials in which they could choose to participate.
Given that PF can cause a variety of symptoms that impact qualify of life in many ways, Wijsenbeek said it’s important to explore therapies aimed at managing them. Such strategies can ease these symptoms, which in turn would be expected to improve patients’ quality of life.
Certain medications, namely opioids and benzodiazepines, may ease breathlessness in some patients, though clear data on their potential benefits are still lacking, she said.
“This is more a trial-and-error, without very good evidence in pulmonary fibrosis, but it might be worth to actually try it [treatment with opioids or benzodiazepines] if you struggle with dyspnea,” she said.
Wijsenbeek said the use of these medications is “absolutely safe” and not linked to any complications or poor outcomes.
Another common symptom is coughing, which can be difficult to manage.
“Cough is a really frustrating symptom, both for patients as well as for doctors, because there’s no good evidence-based therapy in IPF,” Wijsenbeek said.
Current management strategies for cough include over-the-counter cough suppressants, low-dose steroids, painkillers, opioids, and speech therapy. Generally, the type of treatment selected for each patient is based on a trial-and-error approach.
“But I do want to tell you, there is hope,” Wijsenbeek said. “There are some drugs now coming from chronic cough or cough in cancer, that seem beneficial in those areas, and the pharmaceutical companies are starting to investigate these also for fibrosis cough.”
Despite not being a specific symptom of PF, many people with the disease will experience mental health problems, such as anxiety, which can substantially impact their quality of life. Support programs and mindfulness can help alleviate these mental health issues, Wijsenbeek said.
Wijsenbeek also stressed that individual symptoms are all interconnected. For example, she said, a person might experience cough, which in turn leads to breathlessness, which then triggers feelings of anxiety. Given these links, she emphasized the need for holistic management, and for patients to communicate openly and honestly with their physicians.
She also noted that it is important to treat other co-occurring conditions that may further worsen PF symptoms, such as dyspnea or cough.
Palliative and end-of-life care
Providing early palliative care — what she prefers to call “comfort care or support care” — can improve quality of life, Wijsenbeek said. She said the term “palliative care” may sound scary, as it is often associated with end-of-life care. However, while there is a certain degree of overlap between the two, they are not interchangeable.
“The definition and the goal of palliative care are to prevent and relieve suffering by controlling symptoms, and to provide support to patients and families in order to maintain and improve their quality of life. So, it is about supporting and about comfort,” she said.
In addition to palliative care, it is important for patients to have open and honest conversations with their healthcare providers and loved ones about end-of-life care. People with PF are living longer than ever before, with the advent of new treatments, Wijsenbeek said. Yet, PF is still a fatal disease.
Wijsenbeek acknowledged that these conversations can be very challenging — for both patients and their healthcare providers — and that they are somewhat culture-specific. But they are nonetheless important, she said.
According to surveys of her own patients, many people would generally prefer to have these discussions early on in the course of the disease, rather than later once it has progressed substantially, she said.
“You should not be scared of bringing this [end-of-life care] up with your doctor, and you have the right to ask about it. Because sometimes you have worries that are not necessary, so discuss your worries. Make your wishes clear; make your limitations clear. Tell the doctor what you need, and don’t be modest. These are things that you really should share, and we [healthcare providers] are there to support you,” she said.
Ultimately, optimizing quality of life in people with PF is a joint team effort, Wijsenbeek said, that requires the participation and direct involvement of family members, loved ones, and clinicians.
We are sorry that this post was not useful for you!
Let us improve this post!
Tell us how we can improve this post?