Patient Activation for Self-Management in Patients with Idiopathic Pulmonary Fibrosis or Sarcoidosis.

BACKGROUND: Self-management is considered important in the management of patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis. However, data about the degree of activation for self-management is lacking. OBJECTIVES: The aim of the study was to determine the degree of activation for self-management in patients with IPF or sarcoidosis using the Patient Activation Measure (PAM) and to evaluate the association between PAM scores, clinical characteristics, and health-related outcomes. STUDY DESIGN AND METHODS: This cross-sectional prospective study assessed besides the PAM also demographics, lung function, dyspnea (modified Medical Research Council [mMRC]), fatigue (Checklist Individual Strength-Fatigue [CIS-Fatigue]), anxiety/depression (Hospital Anxiety and Depression Scale [HADS-A/HADS-D]), and generic health status (EuroQol five-dimensional-five-level [EQ-5D-5L]). RESULTS: Mean PAM was 55.0 (9.1) points in patients with IPF (n = 59) and low levels of patient activation for self-management (PAM ≤55.1 points) were present in 56% of the patients. PAM Scores correlated significantly (p < 0.05) with mMRC (ρ = -0.476), HADS-A (ρ = -0.326), HADS-D (ρ = -0.459), and EQ-5D-5L (ρ = 0.393). In patients with sarcoidosis (n = 59), the mean PAM score was 55.7 (11.0) points, and 46% of the patients reported low PAM levels. Significant correlations were found with mMRC (ρ = -0.356), HADS-A (ρ = -0.394), HADS-D (ρ = -0.478), and EQ-5D-5L (ρ = 0.313). CONCLUSION: About half of the outpatients with IPF or sarcoidosis have a low degree of activation for self-management, and these patients generally report more dyspnea, anxiety, depression, and a lower health status. Whether patients with a low degree of activation can be successful in self-managing their disease remains to be determined.

Respiratory and non-respiratory symptoms in patients with IPF or sarcoidosis and controls.

INTRODUCTION: Besides dyspnoea and cough, patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis may experience distressing non-respiratory symptoms, such as fatigue or muscle weakness. However, whether and to what extent symptom burden differs between patients with IPF or sarcoidosis and individuals without respiratory disease remains currently unknown. OBJECTIVES: To study the respiratory and non-respiratory burden of multiple symptoms in patients with IPF or sarcoidosis and to compare the symptom burden with individuals without impaired spirometric values, FVC and FEV1 (controls). METHODS: Demographics and symptoms were assessed in 59 patients with IPF, 60 patients with sarcoidosis and 118 controls (age ≥18 years). Patients with either condition were matched to controls by sex and age. Severity of 14 symptoms was assessed using a Visual Analogue Scale. RESULTS: 44 patients with IPF (77.3% male; age 70.6±5.5 years) and 44 matched controls, and 45 patients with sarcoidosis (48.9% male; age 58.1±8.6 year) and 45 matched controls were analyzed. Patients with IPF scored higher on 11 symptoms compared to controls (p<0.05), with the largest differences for dyspnoea, cough, fatigue, muscle weakness and insomnia. Patients with sarcoidosis scored higher on all 14 symptoms (p<0.05), with the largest differences for dyspnoea, fatigue, cough, muscle weakness, insomnia, pain, itch, thirst, micturition (night, day). CONCLUSIONS: Generally, respiratory and non-respiratory symptom burden is significantly higher in patients with IPF or sarcoidosis compared to controls. This emphasizes the importance of awareness for respiratory and non-respiratory symptom burden in IPF or sarcoidosis and the need for additional research to study the underlying mechanisms and subsequent interventions.

Perceptions of fatigue in patients with idiopathic pulmonary fibrosis or sarcoidosis.

BACKGROUND: Fatigue is highly prevalent in patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis. However, the difference in fatigue perceptions for these patients is unknown and this may be important to better understand what fatigue means to the individual patient. METHODS: This cross-sectional quantitative study aims to determine the different perceptions of fatigue as 'frustrating', 'exhausting', 'pleasant', 'frightening' using the Fatigue Quality List and to assess determinants related to these perceptions of fatigue. Beside the fatigue quality connotations, demographics, lung function, fatigue severity (Checklist Individual Strength subscale Fatigue), dyspnea (modified-Medical Research Council), fatigue catastrophizing (Fatigue Catastrophizing Scale), anxiety/depression (Hospital Anxiety and Depression Scale) and general health status (EuroQoL 5-dimension 5-level) were assessed. RESULTS: Mean frequency score of fatigue-related perceptions in patients with IPF was 3.4 points and in patients with sarcoidosis 4.0 points. Severely fatigued patients with IPF reported their fatigue less 'pleasant' significantly more often than patients without severe fatigue. Fatigue severity, dyspnea, catastrophizing and general health were significantly correlated with the negative connotation categories of the Fatigue Quality List in patients with IPF. Severely fatigued sarcoidosis patients reported their fatigue perceptions significantly more often as 'frustrating', 'exhausting', 'frightening' and less 'pleasant' than patients without severe fatigue. Moreover, in patients with sarcoidosis fatigue severity, dyspnea, catastrophizing and depression were significantly associated with all four categories of the Fatigue Quality List that describe the experienced fatigue (P<0.05). CONCLUSIONS: The current findings of experiences of fatigue in patients with IPF or pulmonary sarcoidosis provide insights for professionals treating these patients. Although similarities were found in the several experiences of fatigue across non-severely and severely fatigued patients, differences were also evident and could be mapped for IPF and sarcoidosis.

Relationship between body composition, exercise capacity and health-related quality of life in idiopathic pulmonary fibrosis.

INTRODUCTION: Bioelectrical impedance analysis (BIA) can be used to estimate Fat-Free Mass Index (FFMI). However, the use of directly measured BIA variables, such as phase angle (PhA), has gained attention. The frequency of low FFMI and PhA and its associations with exercise capacity and health-related quality of life (HRQL) in patients with idiopathic pulmonary fibrosis (IPF) have been scarcely studied. OBJECTIVES: To investigate the frequency of low FFMI and PhA and their associations with exercise capacity and HRQL in patients with IPF. METHODS: Patients underwent assessment of lung function, body composition, exercise capacity by the 6 min walk distance (6MWD), and HRQL by the Medical Outcomes Study Short-Form 36-item Questionnaire (SF-36). Patients were classified as presenting normal or low PhA or FFMI, accordingly to the 10th percentiles of age-sex-body mass index (BMI)-specific reference values. RESULTS: 98 patients (84 males, age: 68±8 years, forced vital capacity: 64%±18%predicted) were included. 24 patients presented low PhA. They were characterised by worse lung function, exercise capacity and HRQL compared with patients with normal PhA. 10 patients presented low FFMI, but despite differences in body composition, no differences were found between these patients and patients with normal FFMI. In a single regression analysis, age, lung function and body composition variables (except FFMI) were related to 6MWD and SF-36 Physical Summary Score (R²=0.06-0.36, p<0.05). None of the variables were related to SF-36 Mental Summary Score. CONCLUSION: One-fourth of the patients with IPF with normal to obese BMI present abnormally low PhA. Patients classified as low PhA presented worse lung function, exercise capacity and HRQL.

Severe Fatigue is Highly Prevalent in Patients with IPF or Sarcoidosis.

In patients with interstitial lung disease (ILD) next to dyspnea, fatigue is expected to be the most prevalent symptom. Surprisingly, the prevalence of severe fatigue has been scarcely studied in ILD patients and limited information on its associated factors is available. This study aimed to determine the prevalence of severe fatigue in patients with idiopathic pulmonary fibrosis (IPF) or pulmonary sarcoidosis and to identify the relationship between fatigue, patient characteristics, and clinical parameters. In this cross-sectional study, fatigue (checklist individual strength-fatigue (CIS-Fat)), demographics, lung function, dyspnea (modified-Medical Research Council (mMRC)), sleepiness (Epworth Sleepiness Scale), anxiety/depression (hospital anxiety and depression scale (HADS-A/HADS-D)), catastrophizing (fatigue catastrophizing scale (FCS)), functional activity impairment (respiratory illness quality-of-life (QoL-RIQ-Activity)), and health status (EuroQol five-dimensional descriptive system (EQ-5D-5L)) were assessed in outpatients with ILD. Mean CIS-Fat scores were 34.1 (SD ± 11.2) in 59 IPF patients and 40.0 (12.3) in 58 sarcoidosis patients. Severe fatigue (SD ± ≥36 points) was present in IPF patients (47.5%) and sarcoidosis (69%). In IPF, CIS-Fat correlated strongly (ρ > 0.5; p < 0.01) with FCS, QoL-RIQ-Activity, and EQ-5D-5L-Health and moderately (0.3 < ρ < 0.5; p < 0.01) with EQ-5D-5L-Index, mMRC, and HADS-D. In sarcoidosis, CIS-Fat correlated strongly with EQ-5D-5L-Health, QoL-RIQ-Activity, EQ-5D-5L-Index, HADS-D, and mMRC and moderately with FCS and hospitalization <12 months. Severe fatigue is highly prevalent in ILD patients and is associated with dyspnea, depression, catastrophizing, functional activity impairments, and QoL.

Validation of 4-meter-gait-speed test and 5-repetitions-sit-to-stand test in patients with pulmonary fibrosis: a clinimetric validation study.

Background and objective: Patients with pulmonary fibrosis (PF) have a clear exercise intolerance. The 4-meter-gait-speed (4MGS) test and the 5-repetitions-sit-to-stand (5STS) test are easy, inexpensive and reliable measures of functional performance. Both tests have been validated in healthy adults and patients with chronic obstructive pulmonary disease. 4MGS test and 5STS test have not been studied in patients with PF. Methods: In this cross-sectional clinimetric validation study 51 PF patients conducted in random order the 4MGS test, 5STS test and the 6-min walk test (6MWT) on a single day. Additionally, body weight, height, lean body mass, health-related quality of life, disease severity, handgrip strength, dyspnoea and leg fatigue were assessed. The setting was a tertiary referral center for Interstitial Lung Diseases. Results: Patients had a diagnosis of idiopathic pulmonary fibrosis (IPF, 37%), PF other than IPF (47%), or unclassified (16%). Patients walked 453±111m in six minutes. Moreover, it took the patients 2.0±0.5s to walk 4 m, and 12.0±3.8s for the 5STS test. The 4MGS test (r = 0.77; p<0.01) and the 5STS test (r = -0.41; p<0.01) correlated significantly with the distance walked in 6MWT. Indeed, 4MGS combined with handgrip strength and Medical Research Council dyspnoea grade could explain 75% of the variance in 6MWD. Conclusions: 4-meter-gait-speed and 5-repetitions sit-to-stand are significantly and independently correlated with the 6-minute walk distance in patients with pulmonary fibrosis. Indeed, 4-meter-gait-speed test may serve as a simple initial field test to assess exercise performance in patients with pulmonary fibrosis. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 317-326).