Skip to main content
SpringerLink
Log in

An Introduction to Advanced Lung Disease

  • Chapter
  • First Online:
Palliative Care in Lung Disease

Part of the book series: Respiratory Medicine ((RM))

  • 599 Accesses

Abstract

Chronic lung diseases, including chronic obstructive pulmonary disease (COPD) and lung cancer, are common worldwide and among the leading causes of mortality. Other conditions such as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and chronic neuromuscular disease are less common but devastating in the impact they have on the patient’s quality of life and life expectancy. Regardless of the underlying disease, patients with pulmonary disease must manage symptoms including breathlessness, cough, weight loss, and emotional distress. Non-pulmonary comorbidities including sleep-disordered breathing, anxiety, and depression are common and often contribute to reduced quality of life in patients with chronic lung disease. This chapter will introduce common forms of chronic pulmonary disease and describe predictive models used to determine prognosis in each. We will highlight the unique set of symptoms present across these disease processes to highlight the importance of longitudinal palliative care in patients who suffer from chronic lung disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 119.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Societies FIR. The global impact of respiratory diseases - second edition. Sheffield: European Respiratory Society; 2017.

    Google Scholar 

  2. Thannickal VJ, Murthy M, Balch WE, Chandel NS, Meiners S, Eickelberg O, et al. Blue journal conference. Aging and susceptibility to lung disease. Am J Respir Crit Care Med. 2015;191(3):261–9. https://doi.org/10.1164/rccm.201410-1876PP.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Collaborators GMaCoD. Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980-2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet. 2016;388(10053):1459–544. https://doi.org/10.1016/S0140-6736(16)31012-1.

    Article  Google Scholar 

  4. Collaborators GDaH. Global, regional, and national disability-adjusted life-years (DALYs) for 315 diseases and injuries and healthy life expectancy (HALE), 1990-2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet. 2016;388(10053):1603–58. https://doi.org/10.1016/S0140-6736(16)31460-X.

    Article  Google Scholar 

  5. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease (2020 report); 2020.

    Google Scholar 

  6. Blanc PD, Annesi-Maesano I, Balmes JR, Cummings KJ, Fishwick D, Miedinger D, et al. The occupational burden of nonmalignant respiratory diseases. An official American Thoracic Society and European Respiratory Society statement. Am J Respir Crit Care Med. 2019;199(11):1312–34. https://doi.org/10.1164/rccm.201904-0717ST.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Salvi SS, Barnes PJ. Chronic obstructive pulmonary disease in non-smokers. Lancet. 2009;374(9691):733–43. https://doi.org/10.1016/S0140-6736(09)61303-9.

    Article  PubMed  Google Scholar 

  8. Riley CM, Sciurba FC. Diagnosis and outpatient management of chronic obstructive pulmonary disease: a review. JAMA. 2019;321(8):786–97. https://doi.org/10.1001/jama.2019.0131.

    Article  PubMed  Google Scholar 

  9. Fletcher C. Standardised questionnaire on respiratory symptoms: a statement prepared and approved by the MRC Committee on the Aetiology of Chronic Bronchitis (MRC breathlessness score). BMJ. 1960;2:1665.

    Google Scholar 

  10. Bestall JC, Paul EA, Garrod R, Garnham R, Jones PW, Wedzicha JA. Usefulness of the Medical Research Council (MRC) dyspnoea scale as a measure of disability in patients with chronic obstructive pulmonary disease. Thorax. 1999;54(7):581–6. https://doi.org/10.1136/thx.54.7.581.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Sundh J, Janson C, Lisspers K, Ställberg B, Montgomery S. The Dyspnoea, Obstruction, Smoking, Exacerbation (DOSE) index is predictive of mortality in COPD. Prim Care Respir J. 2012;21(3):295–301. https://doi.org/10.4104/pcrj.2012.00054.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Nishimura K, Izumi T, Tsukino M, Oga T. Dyspnea is a better predictor of 5-year survival than airway obstruction in patients with COPD. Chest. 2002;121(5):1434–40. https://doi.org/10.1378/chest.121.5.1434.

    Article  PubMed  Google Scholar 

  13. Jones PW, Harding G, Berry P, Wiklund I, Chen WH, Kline LN. Development and first validation of the COPD Assessment Test. Eur Respir J. 2009;34(3):648–54. https://doi.org/10.1183/09031936.00102509.

    Article  CAS  PubMed  Google Scholar 

  14. Maltais F, Decramer M, Casaburi R, Barreiro E, Burelle Y, Debigaré R, et al. An official American Thoracic Society/European Respiratory Society statement: update on limb muscle dysfunction in chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2014;189(9):e15–62. https://doi.org/10.1164/rccm.201402-0373ST.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Vestbo J, Anderson W, Coxson HO, Crim C, Dawber F, Edwards L, et al. Evaluation of COPD longitudinally to identify predictive surrogate end-points (ECLIPSE). Eur Respir J. 2008;31(4):869–73. https://doi.org/10.1183/09031936.00111707.

    Article  CAS  PubMed  Google Scholar 

  16. Agusti A, Calverley PM, Celli B, Coxson HO, Edwards LD, Lomas DA, et al. Characterisation of COPD heterogeneity in the ECLIPSE cohort. Respir Res. 2010;11:122. https://doi.org/10.1186/1465-9921-11-122.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Celli BR, Cote CG, Marin JM, Casanova C, Montes de Oca M, Mendez RA, et al. The body-mass index, airflow obstruction, dyspnea, and exercise capacity index in chronic obstructive pulmonary disease. N Engl J Med. 2004;350(10):1005–12. https://doi.org/10.1056/NEJMoa021322.

    Article  CAS  PubMed  Google Scholar 

  18. Puhan MA, Garcia-Aymerich J, Frey M, ter Riet G, Antó JM, Agustí AG, et al. Expansion of the prognostic assessment of patients with chronic obstructive pulmonary disease: the updated BODE index and the ADO index. Lancet. 2009;374(9691):704–11. https://doi.org/10.1016/S0140-6736(09)61301-5.

    Article  PubMed  Google Scholar 

  19. Yohannes AM, Kaplan A, Hanania NA. Anxiety and depression in chronic obstructive pulmonary disease: recognition and management. Cleve Clin J Med. 2018;85(2 Suppl 1):S11–S8. https://doi.org/10.3949/ccjm.85.s1.03.

    Article  PubMed  Google Scholar 

  20. Budhiraja R, Siddiqi TA, Quan SF. Sleep disorders in chronic obstructive pulmonary disease: etiology, impact, and management. J Clin Sleep Med. 2015;11(3):259–70. https://doi.org/10.5664/jcsm.4540.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Wedzicha JA, Seemungal TA. COPD exacerbations: defining their cause and prevention. Lancet. 2007;370(9589):786–96. https://doi.org/10.1016/S0140-6736(07)61382-8.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Seemungal TA, Donaldson GC, Paul EA, Bestall JC, Jeffries DJ, Wedzicha JA. Effect of exacerbation on quality of life in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1998;157(5 Pt 1):1418–22. https://doi.org/10.1164/ajrccm.157.5.9709032.

    Article  CAS  PubMed  Google Scholar 

  23. Rothnie KJ, Müllerová H, Smeeth L, Quint JK. Natural history of chronic obstructive pulmonary disease exacerbations in a general practice-based population with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2018;198(4):464–71. https://doi.org/10.1164/rccm.201710-2029OC.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Ahmadi Z, Lundström S, Janson C, Strang P, Emtner M, Currow DC, et al. End-of-life care in oxygen-dependent COPD and cancer: a national population-based study. Eur Respir J. 2015;46(4):1190–3. https://doi.org/10.1183/09031936.00035915.

    Article  CAS  PubMed  Google Scholar 

  25. Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44–68. https://doi.org/10.1164/rccm.201807-1255ST.

    Article  PubMed  Google Scholar 

  26. Fernández Pérez ER, Swigris JJ, Forssén AV, Tourin O, Solomon JJ, Huie TJ, et al. Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest. 2013;144(5):1644–51. https://doi.org/10.1378/chest.12-2685.

    Article  PubMed  PubMed Central  Google Scholar 

  27. King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92. https://doi.org/10.1056/NEJMoa1402582.

    Article  CAS  PubMed  Google Scholar 

  28. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82. https://doi.org/10.1056/NEJMoa1402584.

    Article  CAS  PubMed  Google Scholar 

  29. Nathan SD, Albera C, Bradford WZ, Costabel U, Glaspole I, Glassberg MK, et al. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis. Lancet Respir Med. 2017;5(1):33–41. https://doi.org/10.1016/S2213-2600(16)30326-5.

    Article  CAS  PubMed  Google Scholar 

  30. Richeldi L, Cottin V, du Bois RM, Selman M, Kimura T, Bailes Z, et al. Nintedanib in patients with idiopathic pulmonary fibrosis: combined evidence from the TOMORROW and INPULSIS(®) trials. Respir Med. 2016;113:74–9. https://doi.org/10.1016/j.rmed.2016.02.001.

    Article  PubMed  Google Scholar 

  31. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718–27. https://doi.org/10.1056/NEJMoa1908681.

    Article  CAS  PubMed  Google Scholar 

  32. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824. https://doi.org/10.1164/rccm.2009-040GL.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Nathan SD, Shlobin OA, Weir N, Ahmad S, Kaldjob JM, Battle E, et al. Long-term course and prognosis of idiopathic pulmonary fibrosis in the new millennium. Chest. 2011;140(1):221–9. https://doi.org/10.1378/chest.10-2572.

    Article  PubMed  Google Scholar 

  34. Paterniti MO, Bi Y, Rekić D, Wang Y, Karimi-Shah BA, Chowdhury BA. Acute exacerbation and decline in forced vital capacity are associated with increased mortality in idiopathic pulmonary fibrosis. Ann Am Thorac Soc. 2017;14(9):1395–402. https://doi.org/10.1513/AnnalsATS.201606-458OC.

    Article  PubMed  Google Scholar 

  35. Ley B, Bradford WZ, Weycker D, Vittinghoff E, du Bois RM, Collard HR. Unified baseline and longitudinal mortality prediction in idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1374–81. https://doi.org/10.1183/09031936.00146314.

    Article  PubMed  Google Scholar 

  36. Ley B, Ryerson CJ, Vittinghoff E, Ryu JH, Tomassetti S, Lee JS, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684–91. https://doi.org/10.7326/0003-4819-156-10-201205150-00004.

    Article  PubMed  Google Scholar 

  37. Ryerson CJ, Vittinghoff E, Ley B, Lee JS, Mooney JJ, Jones KD, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest. 2014;145(4):723–8. https://doi.org/10.1378/chest.13-1474.

    Article  PubMed  Google Scholar 

  38. Campo I, Zorzetto M, Bonella F. Facts and promises on lung biomarkers in interstitial lung diseases. Expert Rev Respir Med. 2015;9(4):437–57. https://doi.org/10.1586/17476348.2015.1062367.

    Article  CAS  PubMed  Google Scholar 

  39. Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. Am J Respir Crit Care Med. 2016;194(3):265–75. https://doi.org/10.1164/rccm.201604-0801CI.

    Article  CAS  PubMed  Google Scholar 

  40. Collard HR, Richeldi L, Kim DS, Taniguchi H, Tschoepe I, Luisetti M, et al. Acute exacerbations in the INPULSIS trials of nintedanib in idiopathic pulmonary fibrosis. Eur Respir J. 2017;49(5). https://doi.org/10.1183/13993003.01339-2016.

  41. Molyneaux PL, Cox MJ, Wells AU, Kim HC, Ji W, Cookson WO, et al. Changes in the respiratory microbiome during acute exacerbations of idiopathic pulmonary fibrosis. Respir Res. 2017;18(1):29. https://doi.org/10.1186/s12931-017-0511-3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Sakamoto S, Homma S, Mun M, Fujii T, Kurosaki A, Yoshimura K. Acute exacerbation of idiopathic interstitial pneumonia following lung surgery in 3 of 68 consecutive patients: a retrospective study. Intern Med. 2011;50(2):77–85. https://doi.org/10.2169/internalmedicine.50.3390.

    Article  PubMed  Google Scholar 

  43. Ghatol A, Ruhl AP, Danoff SK. Exacerbations in idiopathic pulmonary fibrosis triggered by pulmonary and nonpulmonary surgery: a case series and comprehensive review of the literature. Lung. 2012;190(4):373–80. https://doi.org/10.1007/s00408-012-9389-5.

    Article  PubMed  Google Scholar 

  44. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37(2):356–63. https://doi.org/10.1183/09031936.00159709.

    Article  CAS  PubMed  Google Scholar 

  45. Al-Hameed FM, Sharma S. Outcome of patients admitted to the intensive care unit for acute exacerbation of idiopathic pulmonary fibrosis. Can Respir J. 2004;11(2):117–22. https://doi.org/10.1155/2004/379723.

    Article  PubMed  Google Scholar 

  46. Suzuki A, Kondoh Y, Brown KK, Johkoh T, Kataoka K, Fukuoka J, et al. Acute exacerbations of fibrotic interstitial lung diseases. Respirology. 2020;25(5):525–34. https://doi.org/10.1111/resp.13682.

    Article  PubMed  Google Scholar 

  47. Kreuter M, Bendstrup E, Russell AM, Bajwah S, Lindell K, Adir Y, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968–80. https://doi.org/10.1016/S2213-2600(17)30383-1.

    Article  PubMed  Google Scholar 

  48. Ahmadi Z, Wysham NG, Lundström S, Janson C, Currow DC, Ekström M. End-of-life care in oxygen-dependent ILD compared with lung cancer: a national population-based study. Thorax. 2016;71(6):510–6. https://doi.org/10.1136/thoraxjnl-2015-207439.

    Article  PubMed  Google Scholar 

  49. Goobie GC, Guler SA, Johannson KA, Fisher JH, Ryerson CJ. YouTube videos as a source of misinformation on idiopathic pulmonary fibrosis. Ann Am Thorac Soc. 2019;16(5):572–9. https://doi.org/10.1513/AnnalsATS.201809-644OC.

    Article  PubMed  Google Scholar 

  50. King CS, Nathan SD. Idiopathic pulmonary fibrosis: effects and optimal management of comorbidities. Lancet Respir Med. 2017;5(1):72–84. https://doi.org/10.1016/S2213-2600(16)30222-3.

    Article  PubMed  Google Scholar 

  51. Bray F, Ferlay J, Soerjomataram I, Siegel RL, Torre LA, Jemal A. Global cancer statistics 2018: GLOBOCAN estimates of incidence and mortality worldwide for 36 cancers in 185 countries. CA Cancer J Clin. 2018;68(6):394–424. https://doi.org/10.3322/caac.21492.

    Article  PubMed  Google Scholar 

  52. SEER Database; 2020.

    Google Scholar 

  53. Goldstraw P, Chansky K, Crowley J, Rami-Porta R, Asamura H, Eberhardt WE, et al. The IASLC lung cancer staging project: proposals for revision of the TNM stage groupings in the forthcoming (eighth) edition of the TNM classification for lung cancer. J Thorac Oncol. 2016;11(1):39–51. https://doi.org/10.1016/j.jtho.2015.09.009.

    Article  PubMed  Google Scholar 

  54. Nicholson AG, Chansky K, Crowley J, Beyruti R, Kubota K, Turrisi A, et al. The International Association for the Study of Lung Cancer Lung Cancer Staging Project: proposals for the revision of the clinical and pathologic staging of small cell lung cancer in the forthcoming eighth edition of the TNM classification for lung cancer. J Thorac Oncol. 2016;11(3):300–11. https://doi.org/10.1016/j.jtho.2015.10.008.

    Article  PubMed  Google Scholar 

  55. Lassen U, Osterlind K, Hansen M, Dombernowsky P, Bergman B, Hansen HH. Long-term survival in small-cell lung cancer: posttreatment characteristics in patients surviving 5 to 18+ years--an analysis of 1,714 consecutive patients. J Clin Oncol. 1995;13(5):1215–20. https://doi.org/10.1200/JCO.1995.13.5.1215.

    Article  CAS  PubMed  Google Scholar 

  56. Howlader N, Forjaz G, Mooradian MJ, Meza R, Kong CY, Cronin KA, et al. The effect of advances in lung-cancer treatment on population mortality. N Engl J Med. 2020;383(7):640–9. https://doi.org/10.1056/NEJMoa1916623.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Walling AM, Weeks JC, Kahn KL, Tisnado D, Keating NL, Dy SM, et al. Symptom prevalence in lung and colorectal cancer patients. J Pain Symptom Manage. 2015;49(2):192–202. https://doi.org/10.1016/j.jpainsymman.2014.06.003.

    Article  PubMed  Google Scholar 

  58. Iyer S, Roughley A, Rider A, Taylor-Stokes G. The symptom burden of non-small cell lung cancer in the USA: a real-world cross-sectional study. Support Care Cancer. 2014;22(1):181–7. https://doi.org/10.1007/s00520-013-1959-4.

    Article  PubMed  Google Scholar 

  59. Morrison EJ, Novotny PJ, Sloan JA, Yang P, Patten CA, Ruddy KJ, et al. Emotional problems, quality of life, and symptom burden in patients with lung cancer. Clin Lung Cancer. 2017;18(5):497–503. https://doi.org/10.1016/j.cllc.2017.02.008.

    Article  PubMed  PubMed Central  Google Scholar 

  60. Bourke SC, Shaw PJ, Gibson GJ. Respiratory function vs sleep-disordered breathing as predictors of QOL in ALS. Neurology. 2001;57(11):2040–4. https://doi.org/10.1212/wnl.57.11.2040.

    Article  CAS  PubMed  Google Scholar 

  61. Westeneng HJ, Debray TPA, Visser AE, van Eijk RPA, Rooney JPK, Calvo A, et al. Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. Lancet Neurol. 2018;17(5):423–33. https://doi.org/10.1016/S1474-4422(18)30089-9.

    Article  PubMed  Google Scholar 

  62. Ackrivo J, Hansen-Flaschen J, Wileyto EP, Schwab RJ, Elman L, Kawut SM. Development of a prognostic model of respiratory insufficiency or death in amyotrophic lateral sclerosis. Eur Respir J. 2019;53(4). https://doi.org/10.1183/13993003.02237-2018.

  63. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5(2):140–7. https://doi.org/10.1016/S1474-4422(05)70326-4.

    Article  PubMed  Google Scholar 

  64. Phukan J, Elamin M, Bede P, Jordan N, Gallagher L, Byrne S, et al. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012;83(1):102–8. https://doi.org/10.1136/jnnp-2011-300188.

    Article  PubMed  Google Scholar 

  65. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1). https://doi.org/10.1183/13993003.01913-2018.

  66. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023–30. https://doi.org/10.1164/rccm.200510-1668OC.

    Article  PubMed  Google Scholar 

  67. Ling Y, Johnson MK, Kiely DG, Condliffe R, Elliot CA, Gibbs JS, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790–6. https://doi.org/10.1164/rccm.201203-0383OC.

    Article  PubMed  Google Scholar 

  68. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46(4):903–75. https://doi.org/10.1183/13993003.01032-2015.

    Article  CAS  PubMed  Google Scholar 

  69. Anand V, Roy SS, Archer SL, Weir EK, Garg SK, Duval S, et al. Trends and outcomes of pulmonary arterial hypertension-related hospitalizations in the United States: analysis of the nationwide inpatient sample database from 2001 through 2012. JAMA Cardiol. 2016;1(9):1021–9. https://doi.org/10.1001/jamacardio.2016.3591.

    Article  PubMed  Google Scholar 

  70. Stein PD, Matta F, Hughes PG. Scope of problem of pulmonary arterial hypertension. Am J Med. 2015;128(8):844–51. https://doi.org/10.1016/j.amjmed.2015.03.007.

    Article  PubMed  Google Scholar 

  71. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142(2):448–56. https://doi.org/10.1378/chest.11-1460.

    Article  PubMed  Google Scholar 

  72. Gall H, Felix JF, Schneck FK, Milger K, Sommer N, Voswinckel R, et al. The Giessen pulmonary hypertension registry: survival in pulmonary hypertension subgroups. J Heart Lung Transplant. 2017;36(9):957–67. https://doi.org/10.1016/j.healun.2017.02.016.

    Article  PubMed  Google Scholar 

  73. Morris NR, Kermeen FD, Holland AE. Exercise-based rehabilitation programmes for pulmonary hypertension. Cochrane Database Syst Rev. 2017;1:CD011285. https://doi.org/10.1002/14651858.CD011285.pub2.

    Article  PubMed  Google Scholar 

  74. Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, Bush A. Cystic fibrosis. Nat Rev Dis Primers. 2015;1:15010. https://doi.org/10.1038/nrdp.2015.10.

    Article  PubMed  PubMed Central  Google Scholar 

  75. MacKenzie T, Gifford AH, Sabadosa KA, Quinton HB, Knapp EA, Goss CH, et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry. Ann Intern Med. 2014;161(4):233–41. https://doi.org/10.7326/M13-0636.

    Article  PubMed  PubMed Central  Google Scholar 

  76. Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J. 2007;29(3):522–6. https://doi.org/10.1183/09031936.00099506.

    Article  CAS  PubMed  Google Scholar 

  77. Kerem E, Viviani L, Zolin A, MacNeill S, Hatziagorou E, Ellemunter H, et al. Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS patient registry. Eur Respir J. 2014;43(1):125–33. https://doi.org/10.1183/09031936.00166412.

    Article  PubMed  Google Scholar 

  78. Goss CH, Edwards TC, Ramsey BW, Aitken ML, Patrick DL. Patient-reported respiratory symptoms in cystic fibrosis. J Cyst Fibros. 2009;8(4):245–52. https://doi.org/10.1016/j.jcf.2009.04.003.

    Article  CAS  PubMed  Google Scholar 

  79. Sawicki GS, Rasouliyan L, McMullen AH, Wagener JS, McColley SA, Pasta DJ, et al. Longitudinal assessment of health-related quality of life in an observational cohort of patients with cystic fibrosis. Pediatr Pulmonol. 2011;46(1):36–44. https://doi.org/10.1002/ppul.21325.

    Article  PubMed  Google Scholar 

  80. Smith BA, Modi AC, Quittner AL, Wood BL. Depressive symptoms in children with cystic fibrosis and parents and its effects on adherence to airway clearance. Pediatr Pulmonol. 2010;45(8):756–63. https://doi.org/10.1002/ppul.21238.

    Article  PubMed  Google Scholar 

  81. Riekert KA, Bartlett SJ, Boyle MP, Krishnan JA, Rand CS. The association between depression, lung function, and health-related quality of life among adults with cystic fibrosis. Chest. 2007;132(1):231–7. https://doi.org/10.1378/chest.06-2474.

    Article  PubMed  Google Scholar 

  82. Nick JA, Chacon CS, Brayshaw SJ, Jones MC, Barboa CM, St Clair CG, et al. Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis. Am J Respir Crit Care Med. 2010;182(5):614–26. https://doi.org/10.1164/rccm.201001-0092OC.

    Article  PubMed  PubMed Central  Google Scholar 

  83. Sawicki GS, Sellers DE, Robinson WM. High treatment burden in adults with cystic fibrosis: challenges to disease self-management. J Cyst Fibros. 2009;8(2):91–6. https://doi.org/10.1016/j.jcf.2008.09.007.

    Article  PubMed  Google Scholar 

  84. Cleveland RH, Lee EY. Pediatric interstitial (diffuse) lung disease. Imag Pediatr Pulmonol. 2019(September):145–97.

    Google Scholar 

  85. Panitch HB. Respiratory implications of pediatric neuromuscular disease. Respir Care. 2017;62(6):826–48. https://doi.org/10.4187/respcare.05250.

    Article  PubMed  Google Scholar 

  86. Ivy D. Pulmonary hypertension in children. Cardiol Clin. 2016;34(3):451–72. https://doi.org/10.1016/j.ccl.2016.04.005.

    Article  PubMed  PubMed Central  Google Scholar 

  87. Michael Z, Spyropoulos F, Ghanta S, Christou H. Bronchopulmonary dysplasia: an update of current pharmacologic therapies and new approaches. Clin Med Insights Pediatr. 2018;12:1179556518817322. https://doi.org/10.1177/1179556518817322.

    Article  PubMed  PubMed Central  Google Scholar 

  88. Quittner AL, Goldbeck L, Abbott J, Duff A, Lambrecht P, Solé A, et al. Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: results of The International Depression Epidemiological Study across nine countries. Thorax. 2014;69(12):1090–7. https://doi.org/10.1136/thoraxjnl-2014-205983.

    Article  PubMed  Google Scholar 

  89. Willis LD. Transition from pediatric to adult care for young adults with chronic respiratory disease. Respir Care. 2020;65(12):1916–22. https://doi.org/10.4187/respcare.08260.

    Article  PubMed  Google Scholar 

  90. Chambers DC, Cherikh WS, Harhay MO, Hayes D, Hsich E, Khush KK, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-sixth adult lung and heart-lung transplantation Report-2019; Focus theme: donor and recipient size match. J Heart Lung Transplant. 2019;38(10):1042–55. https://doi.org/10.1016/j.healun.2019.08.001.

    Article  PubMed  PubMed Central  Google Scholar 

  91. Kulkarni HS, Cherikh WS, Chambers DC, Garcia VC, Hachem RR, Kreisel D, et al. Bronchiolitis obliterans syndrome-free survival after lung transplantation: an International Society for Heart and Lung Transplantation Thoracic Transplant Registry analysis. J Heart Lung Transplant. 2019;38(1):5–16. https://doi.org/10.1016/j.healun.2018.09.016.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. University of Chicago, Department of Medicine, Section of Pulmonary and Critical Care Medicine, Chicago, IL, USA

    Renea Jablonski & Mary Strek

Authors
  1. Renea Jablonski
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mary Strek
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mary Strek .

Editor information

Editors and Affiliations

  1. College of Nursing, Medical University of South Carolina, Charleston, SC, USA

    Kathleen O. Lindell

  2. Department of Medicine, Johns Hopkins University, Baltimore, MD, USA

    Sonye K. Danoff

Rights and permissions

Reprints and permissions

Copyright information

© 2021 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Jablonski, R., Strek, M. (2021). An Introduction to Advanced Lung Disease. In: Lindell, K.O., Danoff, S.K. (eds) Palliative Care in Lung Disease. Respiratory Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-81788-6_2

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-81788-6_2

  • Published:

  • Publisher Name: Humana, Cham

  • Print ISBN: 978-3-030-81787-9

  • Online ISBN: 978-3-030-81788-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation