Learn about prescription IPF treatment

Symptom management

Managing patients with IPF:Symptom management

Symptom management should be a treatment priority

Because of the progressive nature of IPF, health care providers should assess patients regularly for symptoms and implement treatment strategies in a timely manner¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Symptoms of IPF significantly impair patients’ quality of life

Patients find many symptoms of IPF to significantly impair their QOL^2,3
–Dyspnea
–Chronic cough
–Fatigue
–Depression

QOL, quality of life.

2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
3. Glaspole IN et al. Respirology. 2017;22(5):950-95.

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 patient¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

A strong provider–patient partnership is key to optimal symptom management (cont.)

Mutual trust and respect and an understanding of the patient's values and preferences are essential to a strong provider–patient partnership¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

A strong provider–patient partnership is key to optimal symptom management (cont.)

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 preferences¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

A strong provider–patient partnership is key to optimal symptom management (cont.)

Goals of care should be discussed at the time of diagnosis and periodically thereafter, as the goals of care are likely to change over time¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Dyspnea

Dyspnea is nearly universal in patients with IPF

In IPF, dyspnea often develops gradually and may occur at rest as well as on exertion^2,5
Symptoms of dyspnea discourage patients from physical activity and create the need to take breaks from simple tasks to catch their breath²

2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
4. Bjoraker JA et al. Am J Respir Crit Care Med. 1998;157(1):199-203.
5. NHLBI. Signs and Symptoms of IPF. Available at https://www.nhlbi.nih.gov/health/health-topics/topics/ipf/signs. Accessed June 7, 2017.

Managing dyspnea

Evaluate for comorbidities

Certain comorbidities may contribute to dyspnea, including¹:
–Pulmonary hypertension
–Sleep-disordered breathing
–Muscle weakness
–Psychosocial factors
–Increased weight

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Managing dyspnea (cont.)

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

6. Allen S et al. Palliat Med. 2005;19(2):128-130.

Managing dyspnea (cont.)

Pulmonary rehabilitation

According to current guidelines, most patients with IPF should be treated with pulmonary rehabilitation²

2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.

Managing dyspnea (cont.)

Pulmonary rehabilitation

The main goals of pulmonary rehabilitation are to reduce symptoms, optimize functional status, and increase participation in activities of daily living⁷

7. Nici L et al. Am J Respir Crit Care Med. 2006;173(12):1390-1413.

Managing dyspnea (cont.)

Pulmonary rehabilitation

Components of pulmonary rehabilitation programs include patient assessment, exercise training, education, nutritional intervention, and psychosocial support⁷

7. Nici L et al. Am J Respir Crit Care Med. 2006;173(12):1390-1413.

Managing dyspnea (cont.)

Pulmonary rehabilitation

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

8. Holland AE et al. Thorax. 2008;63(6):549-554.
9. Nishiyama O et al. Respirology. 2008;13(3):394-399.

Managing dyspnea (cont.)

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 therapy¹⁰

10. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.

Managing dyspnea (cont.)

Oxygen supplementation

For patients who exhibit desaturation during exercise ( <90%), ambulatory oxygen is recommended if it clearly improves exercise capacity or dyspnea¹¹

11. Bradley B et al. Thorax. 2008;63 Suppl 5:v1-58.

Cough

Chronic cough is common among patients with IPF

Some patients have a nagging desire to constantly cough with no feeling of relief after coughing²

2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
10. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.

Managing cough

Evaluate for comorbidities

Common etiologies should be excluded before diagnosing IPF-induced cough in patients who have both chronic cough and IPF¹²
–Upper airway cough syndrome (previously referred to as postnasal drip syndrome)
–Asthma
–GERD

GERD, gastroesophageal reflux disease.

12. Brown KK. Chest. 2006;129(1 Suppl):180S-185S.

Managing cough (cont.)

Evaluate for comorbidities

A clinical study evaluated cough in nonsmoking patients with IPF who did not have airway hyperreactivity, chronic rhinitis, or GERD¹³

13. Hope-Gill BDM et al. Am J Respir Crit Care Med. 2003;168(8):995-1002.

Managing cough (cont.)

Evaluate for comorbidities

The investigators found that patients had upregulated function of sensory neurons to substance P and capsaicin, as well as increased sputum levels of NGF and BDNF¹³

NGF, nerve growth factor; BDNF, brain-derived neurotrophic factor.

13. Hope-Gill BDM et al. Am J Respir Crit Care Med. 2003;168(8):995-1002.

Managing cough (cont.)

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

14. Smith SM et al. Cochrane Database Syst Rev. 2014; 11:CD001831.
15. Vigeland CL et al. Respir Med.2017; 123:98-104.

Managing cough (cont.)

Pharmacologic treatment

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 GERD¹³

13. Hope-Gill BDM et al. Am J Respir Crit Care Med. 2003;168(8):995-1002.

Managing cough (cont.)

Pharmacologic treatment

Because corticosteroids are associated with significant side effects, their use requires an analysis of the benefit and risk in each individual patient¹²

12. Brown KK. Chest. 2006;129(1 Suppl):180S-185S.

Fatigue

Fatigue is a common symptom for patients with IPF

Fatigue may be a primary symptom for some patients with IPF and may affect them as much as breathlessness^1,2

Patients feel a consistent lack of energy and the need to economize energy throughout the day²

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.

Muscle deconditioning from inactivity worsens fatigue symptoms

Muscle deconditioning creates a cycle of progressive fatigue and deconditioning that is challenging to break¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Fatigue

Evaluate for comorbidities

Certain comorbidities may contribute to fatigue¹
–Obesity
–Sleep-disordered breathing
–Hypoxemia

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Fatigue (cont.)

Pulmonary rehabilitation

In patients with IPF, dyspnea and fatigue worsen as fibrosis advances²
Patients are increasingly unable to perform physical activities such as dressing and bathing without becoming severely breathless, resulting in a reduction in physical activities²
This can lead to aerobic and skeletal muscle deconditioning¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.

Fatigue (cont.)

Pulmonary rehabilitation

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

8. Holland A et al. Thorax. 2008;63:549-554.
9. Nishiyama O et al. Respirology 2008;13:394-399.
16. Swigris JJ et al. Respir Care. 2011;56(6):783-789.

Fatigue (cont.)

Pulmonary rehabilitation

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 baseline¹⁶

16. Swigris JJ et al. Respir Care. 2011;56(6):783-789.

Depression

Finally, many patients with IPF experience symptoms of depression^1,2,17
Patients may also have fear, worry, anxiety, and panic related to having IPF due to its poor prognosis¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
2. Swigris JJ et al. Health Qual Life outcomes. 20005;3:61.
17. Ryerson CJ et al. Chest. 2011;139(3):609-616.

Depression (cont.)

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 Inventory¹⁸

18. Akhtar AA et al. Chron Respir Dis. 2013;10(3):127-133.

Depression (cont.)

Pharmacological treatment and psychological counseling

Depression scores did not correlate with durationsince diagnosis, age, or number of comorbidities¹⁸

18. Akhtar AA et al. Chron Respir Dis. 2013;10(3):127-133.

Depression (cont.)

Pharmacological treatment and psychological counseling

Given the high prevalence of depression in patients with IPF, depression should be actively screened, and if present, managed with antidepressant medication and psychological counselling¹⁸

18. Akhtar AA et al. Chron Respir Dis. 2013;10(3):127-133.

Depression (cont.)

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 studies¹⁶

16. Swigris JJ et al. Respir Care. 2011;56(6):783-789.
19. Ferreira A et al. Chest. 2009;135(2):442-447.

Depression (cont.)

Evaluate for comorbidities

Certain comorbidities (eg, dyspnea, fatigue) may contribute to depression¹
Polypharmacy may also contribute to depression¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Patient Education

Patient education and self-management are important components of care

Once goals of care are established, education and self-management strategies should be implemented¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Patient education and self-management are important components of care (cont.)

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 future¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Patient education and self-management are important components of care (cont.)

Education may be provided in the physician's office, an IPF center, online, and at educational seminars offered in the community¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

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

BreathlessIPF.com
LungsandYou.com
PatientsLikeMe
- https://www.patientslikeme.com/conditions/1954-pulmonary-fibrosis
Pulmonary Fibrosis Foundation support groups
- http://www.pulmonaryfibrosis.org/supportgroups/online
IPFandYou™ call center
- 1-844-473-2638
- Monday – Friday, 9am-6pm ET

Patient education and self-management are important components of care (cont.)

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 IPF¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.

Summary

In patients with IPF, symptoms significantly impair overall QOL²
Therefore, health care providers should regularly assess for symptoms and make symptom management a treatment priority¹
Establishing a strong provider–patient partnership, setting management goals, and educating patients on self-management are critical to optimal symptom management¹

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
2. Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.

References

1. Lee JS et al. Curr Opin Pulm Med. 2011;17(5):348-354.
2 .Swigris JJ et al. Health Qual Life Outcomes. 2005;3:61.
3. Glaspole IN et al. Respirology. 2017;22(5):950-956.
4. Bjoraker JA et al. Am J Respir Crit Care Med. 1998;157(1):199-203.
5. NHLBI. Signs and Symptoms of IPF. Available at https://www.nhlbi.nih.gov/health/health-topics/topics/ipf/signs. Accessed June 7, 2017.
6 Allen S et al. Palliat Med. 2005;19(2):128-130.
7. Nici L et al. Am J Respir Crit Care Med. 2006;173(12):1390-1413.
8. Holland AE et al. Thorax. 2008;63(6):549-554.
9. Nishiyama O et al. Respirology. 2008;13(3):394-399.
10. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
11. Bradley B et al. Thorax. 2008;63 Suppl 5:v1-58.
12. Brown KK. Chest. 2006;129(1 Suppl):180S-185S.
13 Hope-Gill BDM et al. Am J Respir Crit Care Med. 2003;168(8):995-1002.
14. Smith SM et al. Cochrane Database Syst Rev. 2014; 11:CD001831.
15. Vigeland CL et al. Respir Med. 2017;123:98-104.
16. Swigris JJ et al. Respir Care. 2011;56(6):783-789.
17. Ryerson CJ et al. Chest. 2011;139(3):609-616
18. Akhtar AA et al. Chron Respir Dis. 2013;10(3):127-133.
19. Ferreira A et al. Chest. 2009;135(2):442-447.