Getting patients to ILD Specialist Centres is vital to preserving lung capacity and function1-4
Delayed access costs patients vital capacity
For patients with chronic progressive fibrotic interstitial lung diseases, time lost due to referral delays equates to lost function. A decline in forced vital capacity (FVC) of 5–10% over 6 months significantly increases a patient’s risk of death.5-8 Timely access to specialist care programmes and treatment is vital to minimise the irreversible damage to lung tissue caused by fibrotic disease.1-4
Awareness, identification and prompt referral play key roles throughout the interstitial lung disease (ILD) referral pathway. Whilst significant progress has been made, a patient survey reported that patients with idiopathic pulmonary fibrosis (IPF) were experiencing median referral delays of over 2 years (ranging from 0.9 to 5 years).4 For patients who experience long referral delays, the impact on their survival is devastating.9
Impact of delayed referral on survival
Two key contributing factors to diagnostic delays are when referring patients from GP to local hospital and from local hospitals to ILD Specialist Centres.4 This may be due to regional differences in awareness and approach or clinical complexity.
An ideal pathway for chronic progressive fibrotic ILDs would likely reflect the one established for IPF. The broader referral base for chronic progressive fibrotic ILD, reflective of the wider presentation routes for the different ILDs, will necessitate greater vigilance to engage in early dialogue with specialist MDT teams and refer on first suspicion. Recognising the benefits of specialist anti-fibrotic treatment and interventions for this patient group will also be key.
Ideal IPF pathway
The current IPF pathway is likely to form a basis for chronic progressive fibrotic ILD referral
How can you preserve chronic progressive fibrotic ILD patients’ capacity for living?
When should you suspect pulmonary fibrosis?
- Cosgrove GP et al. BMC Pulm Med 2018;18(1):9
- Cottin V and Cordier VC. Eur Respir J 2012;40(3):519-521.
- Molina-Molina M et al. Exp Rev Resp Med 2018;12(7):537-539.
- Hoyer N et al. Respir Res 2019;20(1):103.
- Richeldi L et al. Thorax 2012;67(50):407-411.
- Collard HR et al. Am J Respir Crit Care Med 2003;168(5):538-542.
- du Bois RM et al. Am J Respir Crit Care Med 2011;184(4):459-466.
- Zappala CJ et al. Eur Respir J 2009;35:830-836
- Lamas DJ et al. Am J Respir Crit Care Med 2011;184(7):842-847.
- Kreuter M et al. Lancet Respir Med 2017;5(12):968-980.
- Duck A. Nursing Times 2008;104(9):46-49. Available at: www.nursingtimes.net/nursingpatients-withinterstitial-lung- disease/850840.article (Accessed July 2023).
- OFEV® 100 mg and 150 mg soft capsules Summary of Product Characteristics. Boehringer Ingelheim.
Adverse events should be reported.
UK: Reporting forms and information can be found at www.mhra.gov.uk/yellowcard. Adverse events should also be reported to Boehringer Ingelheim Drug Safety on 0800 328 1627 (freephone) or by email to PV_local_uk_ireland@boehringer-ingelheim.com.
IE: Reporting forms and information can be found at www.hpra.ie/homepage/about-us/report-an-issue. Adverse events should also be reported to Boehringer Ingelheim Drug Safety on 01 291 3960 or by email to PV_local_uk_ireland@boehringer-ingelheim.com.
OFEV® is indicated in adults for the treatment of idiopathic pulmonary fibrosis (IPF) and for the treatment of other chronic fibrosing interstitial lung diseases (ILDs) with a progressive phenotype. The recommended dose is one 150 mg capsule taken twice daily.12
