Symptom Management

Symptom management should be a treatment priority.¹ Because of the progressive nature of IPF, health care providers should assess patients regularly for symptoms and implement treatment strategies in a timely manner.¹

SYMPTOMS

Symptoms of IPF significantly impair patients’ quality of life (QOL).²

Symptoms of IPF that can significantly impair QOL:^2,3

• Dyspnea
• Chronic cough
• Fatigue
• Depression

A strong provider—patient partnership is key to optimal symptom management.¹

Optimal symptom management relies on a foundation of active engagement and collaboration between the health care provider and the patient.¹

Mutual trust and respect and an understanding of the patient's values and preferences are essential to a strong provider—patient partnership.¹

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.¹

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.¹

DYSPNEA

Dyspnea is nearly universal in patients with IPF.⁴

• In IPF, dyspnea often develops gradually and may occur at rest as well as on exertion^3,5
• Symptoms of dyspnea discourage patients from physical activity and create the need to take breaks from simple tasks to catch their breath³

MANAGING DYSPNEA

Evaluate for comorbidities

Certain comorbidities may contribute to dyspnea, including:¹

• Pulmonary hypertension
• Sleep-disordered breathing
• Muscle weakness
• Psychosocial factors
• Increased weight

Pharmacological treatment

• For patients with severe dyspnea, opioids such as morphine should be considered⁶
• In a small study, low-dose opioids effectively reduced breathlessness in elderly patients with end-stage IPF without causing a decrease in oxygen saturation⁶

Pulmonary rehabilitation

According to current guidelines, most patients with IPF should be treated with pulmonary rehabilitation³

The main goals of pulmonary rehabilitation are to reduce symptoms, optimize functional status, and increase participation in activities of daily living.⁷

Components of pulmonary rehabilitation programs include patient assessment, exercise training, education, nutritional intervention, and psychosocial support.⁷

In 2 controlled trials, pulmonary rehabilitation programs improved walk distance, symptoms, and QOL in patients with IPF.^8,9

Oxygen supplementation

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.¹⁰

For patients who exhibit desaturation during exercise (<90%), ambulatory oxygen is recommended if it clearly improves exercise capacity or dyspnea.¹¹

COUGH

Chronic cough is common among patients with IPF.¹⁰

Some patients have a nagging desire to constantly cough with no feeling of relief after coughing.³

MANAGING COUGH

Evaluate for comorbidities

Common etiologies should be excluded before diagnosing IPF-induced cough in patients who have both chronic cough and IPF:¹²

• Upper airway cough syndrome (previously referred to as postnasal drip syndrome)
• Asthma
• Gastroesophageal reflux disease (GERD)

A clinical study evaluated cough in nonsmoking patients with IPF who did not have airway hyperreactivity, chronic rhinitis, or GERD.¹³

The investigators found that patients had upregulated function of sensory neurons to substance P and capsaicin, as well as increased sputum levels of nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF).¹³

Pharmacologic treatment

Treatments for acute cough, such as cough suppressants, expectorants, mucolytics, and antihistamines, have shown little efficacy in treating IPF-induced cough.^14,15 Using these agents can produce systemic side effects with limited and/or transient effects.^14,15

A small prospective controlled study demonstrated that oral corticosteroids decreased cough severity and sensitivity to capsaicin and substance P in nonsmoking patients with IPF who did not have reversible airflow limitation, chronic rhinitis, or GERD.¹³

Because corticosteroids are associated with significant side effects, their use requires an analysis of the benefit and risk in each individual patient.¹²

FATIGUE

Fatigue is a common symptom for patients with IPF.^1,3 It may be a primary symptom and may affect them as much as breathlessness.^1,3

• Patients feel a consistent lack of energy and the need to economize energy throughout the day³

Muscle deconditioning from inactivity worsens fatigue symptoms and creates a cycle of progressive fatigue and deconditioning that is challenging to break.¹

Evaluate for comorbidities

Certain comorbidities may contribute to fatigue:¹

• Obesity
• Sleep-disordered breathing
• Hypoxemia

Pulmonary rehabilitation

• In patients with IPF, dyspnea and fatigue worsen as fibrosis advances³
• Patients are increasingly unable to perform physical activities such as dressing and bathing without becoming severely breathless, resulting in a reduction in physical activities³
• This can lead to aerobic and skeletal muscle deconditioning¹

Pulmonary rehabilitation may improve symptoms of IPF and patients’ perceptions of QOL.^8,9,16

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.¹⁶

DEPRESSION

Finally, many patients with IPF experience symptoms of depression.^1,3,17

Patients may also have fear, worry, anxiety, and panic related to having IPF due to its poor prognosis.¹

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.¹⁸

Depression scores did not correlate with duration since diagnosis, age, or number of comorbidities.¹⁸

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.¹⁸

Pulmonary rehabilitation

Two studies of pulmonary rehabilitation in patients with IPF found a positive effect of pulmonary rehabilitation on depression.^16,19 However, the change from baseline was not statistically significant in 1 of the 2 studies.¹⁶

Evaluate for comorbidities

• Certain comorbidities (e.g., dyspnea, fatigue) may contribute to depression¹
• Polypharmacy may also contribute to depression¹

PATIENT EDUCATION

Patient education and self-management are important components of care.¹ Once goals of care are established, education and self- management strategies should be implemented.¹

Enabling patients to set realistic goals and remain in control of his or her care allows them to enjoy a better QOL and to prepare for the future.¹

Education may be provided in the physician's office, an IPF center, online, and at educational seminars offered in the community.¹

Patient resources are available to help patients educate themselves and manage their disease.

• LungsandYou.com
• PatientsLikeMe
• Pulmonary Fibrosis Foundation support groups
• IPFandYou^TM call center
  • 1-844—473-2638
  • Monday— Friday, 9am-6pm ET

Patients can also learn about symptom management through IPF support groups and pulmonary rehabilitation programs, as long as the programs are targeted to patients with IPF.¹

REFERENCES