IPF Symptom Management

Symptoms Associated With IPF Significantly Impair Patients’ Quality of Life35

A study of patients' perspectives on how IPF affects the quality of their lives found that dyspnea, chronic cough, fatigue, and depression were significant impairments to overall quality of life.35 Among patients with IPF, dyspnea is nearly universal.36 Symptoms of dyspnea discourage patients from physical activity and create the need to take breaks from simple tasks to catch their breath.35 Chronic cough is also common among patients with IPF.36 Some patients have a nagging desire to constantly cough with no feeling of relief after coughing.35 Fatigue may be a primary symptom for some patients with IPF and may be as bothersome as breathlessness.3536 Muscle deconditioning further worsens the symptom of fatigue, creating a cycle of progressive fatigue and deconditioning that is challenging to break.36 Patients feel a consistent lack of energy and the need to economize energy during the day.35 Finally, symptoms of depression are experienced by many patients with IPF.35 Patients may also have fear, worry, anxiety, and panic related to having IPF due to its poor prognosis.3536

Symptom Management Should be a Treatment Priority36

Because of the progressive nature of IPF, health care providers must regularly assess for symptoms and implement treatment strategies in a timely manner.36

Treatment of Dyspnea

Oxygen Therapy
Current guidelines recommend that patients with clinically significant resting hypoxemia (commonly defined by a resting oxygen saturation of <88%) should be treated with long-term oxygen therapy.35 For patients who desaturate to <90% during exercise, ambulatory oxygen is recommended if a clear beneficial effect on exercise capacity or dyspnea is seen.37

Pulmonary Rehabilitation
According to current guidelines, most patients with IPF should be treated with pulmonary rehabilitation.35 An improvement in walk distance, symptoms, and quality of life has been demonstrated in 2 controlled trials of pulmonary rehabilitation in IPF.3839 The goals of pulmonary rehabilitation, among others, are to reduce symptoms, optimize functional status, and increase participation in activities of daily living.40 Components of programs include patient assessment, exercise training, education, nutritional intervention, and psychosocial support.40

Pharmacological Treatment
For patients with severe dyspnea, opioids such as morphine should be considered. A small study of a low-dose opioid in elderly patients with end-stage IPF found that it effectively reduced breathlessness without causing a decrease in oxygen saturation.41

Evaluate for Comorbidities
Certain comorbidities may contribute to dyspnea. These include pulmonary hypertension, sleep-disordered breathing, muscle weakness, psychosocial factors, and increased weight.36

Treatment of Cough

Cough Secondary to IPF
In a well-conducted study of nonsmoking patients with IPF in whom airway hyperresponsiveness, chronic rhinitis, and gastroesophageal reflux disease (GERD) were excluded, an increased sensitivity to substance P and capsaicin, and increased sputum levels of nerve growth factor and brain-derived neurotrophic factor were found. This suggests a functional up-regulation of sensory neurons of the lung.42 A small prospective controlled study in nonsmoking patients with IPF who did not have reversible airflow limitation, chronic rhinitis, or GERD demonstrated that oral corticosteroids decreased cough severity and sensitivity to capsaicin and substance P in all patients.42 Because corticosteroids are associated with significant side effects, their use requires an analysis of the benefit and risk in each individual patient.

Cough Secondary to Comorbid Conditions
A diagnosis of IPF as the cause of cough should be considered a diagnosis of exclusion―before diagnosing IPF as the sole cause, more common etiologies such as upper airway cough syndrome (previously referred to as postnasal drip syndrome), asthma, and GERD should be considered.42 Identification of the specific disorder is key to successful management.

Treatment of Fatigue

Pulmonary Rehabilitation
In patients with IPF, dyspnea and fatigue worsen as fibrosis advances.43 Patients are increasingly unable to perform physical activities such as dressing and bathing without becoming severely breathless resulting in a reduction in physical activities. This can lead to aerobic and skeletal muscle deconditioning. A pilot study of pulmonary rehabilitation in patients with IPF consisting of 18 sessions over 6-8 weeks found that the Fatigue Severity Scale score improved significantly, declining an average 1.5 ±0.5 points from baseline.43

Evaluate for Comorbidities
Certain comorbidities may contribute to fatigue. These include obesity, sleep disordered breathing and hypoxemia.36

Treatment of Depression

Pharmacological Treatment and Psychological Counseling
A single-center, prospective study of 118 patients with IPF found that 49% of participants met the criteria for depression on the Wakefield Self-assessment of Depression Inventory.44 Duration since diagnosis, age, and number of comorbidities were not significantly correlated with depression score. Given the high prevalence of depression in patients with IPF, depression should be actively screened, and if present, managed with antidepressant medication and psychological counselling.44

Pulmonary Rehabilitation
Two studies of pulmonary rehabilitation in patients with IPF found a positive effect of pulmonary rehabilitation on depression; however, the change from baseline was not statistically significant in 1 of the studies.4345

Evaluate for Comorbidities
Certain comorbidities may contribute to depression. These include dyspnea and fatigue. Further, polypharmacy may contribute to depression.36

A Strong Provider-Patient Partnership is Key to Optimal Symptom Management

Optimal symptom management relies on a foundation of active engagement and collaboration between the health care provider and the patient.36 Mutual trust and respect and an understanding of the patient's values and preferences are essential to a strong provider–patient partnership. A detailed assessment of the goals of care is critical and management options must be tailored to the individual patient based on the patient's age, comorbidities, and values and preferences. Goals of care should be discussed at the time of diagnosis and periodically thereafter, as the goals of care are likely to change over time.

Once goals of care are established, education and self-management strategies should be implemented.36 Enabling patients to set realistic goals and remain in control of his or her care allows them to enjoy a higher quality of life and prepare for the future. Education may be provided in the physician's office, an IPF center, online, and at educational seminars offered in the community. Further, outstanding ways for patients to learn about symptom management are IPF support groups and pulmonary rehabilitation programs, provided facilitators target the program to patients with IPF.

Conclusions

In patients with IPF, symptoms are significant impairments to overall quality of life.35 Therefore, it is imperative that health care providers regularly assess for symptoms and make management a treatment priority.36 Establishing a strong provider-patient partnership, setting management goals, and educating patients on self-management are critical to optimal symptom management.36