IPF Patients Voice Frustrations with Disease and Public’s Poor Understanding of It

Magdalena Kegel avatar

by Magdalena Kegel |

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IPF patient survey

A European survey of patients with idiopathic pulmonary fibrosis (IPF) found that patients well understand the disease’s significant physical and emotional impact on their quality of life, and are frustrated by a perceived lack of quality IPF information and public understanding. The study also underscored the beneficial role of patients’ caregivers and specialist nurses, where available.

The study was titled Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients’ perspectives of the disease and treatment and performed by Anne-Marie Russell from the Imperial College and Royal Brompton Hospital, London, along with colleagues from Italy. It was published in the journal BMC Pulmonary Medicine.

“Living with IPF and an exploration of Esbriet” was an in-depth, exploratory, qualitative, real-world survey of European patients with IPF treated with pirfenidone (Esbriet, Genentech, Roche) before it was commercially available. It included 45 patients from the U.K., Germany, and Italy. Researchers performed qualitative interviews, where patients were asked about their experiences of living with IPF and of treatment with pirfenidone.

Most patients, 87 percent, reported that it took them more than one year to receive a diagnosis, and half of the patients reported that IPF had a substantial negative impact on their quality of life. Most patients (82 percent) were troubled by fatigue. Other symptoms present in more than half the group were a loss of appetite — often due to nausea — difficulty in lifting objects, and incessant phlegm and coughing throughout the day.

Patients often reported that the physical disabilities impacted them on an emotional level, with feelings of depression and reduced mental strength to fight the disease. Many patients reported fear of disease progression as the most troublesome feature, but 36 percent also reported feeling frustrated and angry because of other people’s poor knowledge of IPF and the lack of self-acceptance of their disease. Almost one-fifth of the patients also reported feeling isolated.

Some patients mentioned they felt like a burden, being reliant on their family. The study found a gender difference associated with identity — 90 percent of the women in the study reported feeling a loss of identity as the main family support figure. Another gender difference was that 15 percent of the men stated that IPF had a detrimental impact on their sex lives, both physically and emotionally.

The use of oxygen treatment — reported in 90 percent of the patients — was often associated with a perceived limitation of freedom and feelings of less hope, as well as shame as the disease became visible to others.

One important difference between the study countries was the role of the main medical contact for IPF care. In the U.K., 90 percent of patients had a specialist nurse as their main contact. In Germany, nurses played a minor role and physicians were the main contacts, whereas nurses were not at all involved in IPF care in Italy.

This difference seemed to lead to different perceptions of support from medical professionals. U.K. patients appeared to be more satisfied, as can be understood from a patient quote in the article: “She [the nurse] is everything for me, she is a shoulder to cry on, she answers any question I ask her, I hope she never leaves!”

Specialist nurses in the U.K. helped patients to access medications and to manage side effects, and also provided lifestyle advice, information on the disease, and knowledge about patient support organizations.

One of the main features lacking in IPF care, according to most patients, was the psychological support needed to face IPF-related difficulties. Only one-fifth of the patients reported receiving professional psychological support.

“The doctors helped me, but I have never received any psychological support, I really need it,” said a German patient cited in the study.

More than half of the patients felt they needed a caregiver in their household who could remind them to take medications, accompany them to medical appointments, and help to research information on IPF.

About one-third of patients felt that they lacked information or awareness of IPF, and 74 percent were searching for information about the disease. Many, however, said that the material found was often of poor quality and out-of-date. Information was most often available only in English, presenting difficulties for the German and Italian patients.

U.K. and German patients usually understood the severity of their disease and prognosis, as well as available treatments, while Italian patients often reported being less well-informed about IPF. They also relied only on information provided by their doctor to a greater extent. One Italian patient said in the study: “No idea about the name of this disease, I call it lung infection.”

Patients were most often interested in receiving information on IPF, the use of pirfenidone, and news of research and treatment updates. Patients also reported a lack of expert advice relating to lifestyle, diet, oxygen treatment, and physical exercise. Many participants would also like to know how many other people in their area were affected by IPF, and wanted the general public to be better informed about the disease.

Patients’ perceptions on pirfenidone were largely positive but influenced by the level of side effects, including nausea and photosensitivity, they experienced. Side effects negatively impacted the psychological well-being of patients, who perceived little awareness of their treatment working. Conversely, patients experiencing minimal or no pirfenidone side effects felt their condition had stabilized, which gave them a sense of hope and greater confidence.

Recent reports suggest that adverse effects of pirfenidone can be ably managed, but patients need guidance to minimize such risks. The study reports that strategies that have proved successful include using sun protection to avoid photosensitive skin reactions, and taking pirfenidone during or after a meal to lessen its gastrointestinal impact.

The research team concluded that patients often felt isolated, embarrassed and stigmatized, highlighting the importance of raising awareness about IPF in the community.

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