Low Pulmonary Rehabilitation, Palliative Care Referrals for Patients With IPF Persist

Despite improvements over the past decade, referrals to pulmonary rehabilitation (PR) programs and palliative care (PC) services among patients with idiopathic pulmonary fibrosis (IPF) in England remained low in 2019, according to a study published in NPJ Primary Care Respiratory Medicine.¹

Typically, once IPF is diagnosed, the prognosis is poor, with an average survival time of just 3 to 5 years among those in the United Kingdom (UK) who did not receive treatment.² However, increasing research supports a holistic approach to managing those diagnosed with IPF, with PR and PC considered important components of such an approach.¹

Originally, PR was created to support patients with chronic obstructive pulmonary disease (COPD), but, in recent years, evidence has emerged of its benefits among those with IPF.³ Similarly, several studies have demonstrated that access to integrated PC services improves the quality of life of patients with IPF.¹

Conversely, the researchers noted that PR and PC service provision is poor among those with IPF, with referral rates typically much lower than those with other respiratory conditions, like COPD or lung cancer. National registry data from several countries, including the US, Germany, and Spain, demonstrated that less than 20% of those diagnosed with IPF are offered a PC referral and less than 10% attend PR.⁴

To add to this data, the researchers conducted a study to examine UK trends in referrals to PR and PC for patients with IPF.¹ They also investigated demographic and patient factors linked to referrals.

Referrals to pulmonary rehabilitation and palliative care for patients with idiopathic pulmonary fibrosis remain significantly lower than for other respiratory conditions. | Image Credit: Vitalii Vodolazskyi - stock.adobe.com

The study used the Clinical Practice Research Datalink (CPRD) Aurum database, a curated set of patient-level electronic health records from 1345 participating UK general practitioner (GP) practices⁵; it is representative of the UK population in terms of age, ethnicity, and sex.

Eligible patients were registered at linkage-eligible CPRD Aurum GP practices and diagnosed with IPF on or after the start of the study period (January 1, 2010) but before the end of the study period (December 31, 2019).¹ They also needed to be at least 40 years old at the time of their IPF diagnosis, meet CPRD-defined data quality criteria, and have at least 1 year of continuous registration with their current GP before their IPF diagnosis.

For each patient, follow-up began on the latest of the following dates: the start of the study period, the date of registration with their GP practice, the date of their 40th birthday, or the date of IPF diagnosis. Then, follow-up concluded on the earliest of the following dates: the end of the study, the date of death, or the last day of either practice registration or data collection.

The researchers considered patients to have been referred to PR or PC if their primary care record included evidence that they had been considered and/or offered a referral to PR or PC after their IPF diagnosis and within their follow-up period. Additionally, the selected risk factors comprised demographic characteristics, smoking status, lifestyle characteristics, and the presence of selected comorbid diseases; these included COPD, lung cancer, and asthma.

The study population consisted of 17,071 patients with IPF, most of whom were male (62.6%), White (92.3%), and had a history of smoking (88.5%). The median age at diagnosis was 76.7 years, and the most common comorbidities were ischemic heart disease (IHD; 27.2%), depression/anxiety (25.7%), and diabetes (23.7%).

During follow-up, 2042 patients (12.0%) were referred to PR. More men than women were referred to PR (13.0% vs 10.2%), and nearly half of all referrals occurred in those between the ages of 70 and 79 (43.6%). Also, 22.3% of those referred were current smokers. Compared with the non-referral group (non-PR group), comorbidities like COPD, asthma, and depression/anxiety, were more common among the referral group (PR group). For example, 40.9% of the PR group vs 12.9% of the non-PR group also had COPD.

Through temporal analyses, the researchers discovered that, in 2010, only 1.2% of patients were offered PR (15 of 1246 patients). By 2019, this proportion rose to 6.5% (574 of 8811 patients). Although referral proportions were higher in those who received a previous diagnosis of COPD, they observed a steady referral proportion increase over time, even among those without a COPD diagnosis.

After adjusting for potential confounders, smoking history, a prior COPD diagnosis, and male sex were strongly positively associated with PR referral. Conversely, potential contributory factors to non-referral include older age (over 80 years old), co-existing dementia, and the presence of IHD.

On the other hand, 19.4% (2159 men; 1156 women) of the study population were referred to PC support services; nearly 80% of referrals occurred in those over 70 years old. Lung cancer and pre-existing cardiovascular conditions were among the comorbidities more prevalent among the PC group. Of those diagnosed with lung cancer (n = 137; 0.8%), about a third (n = 47; 34%) received a referral to PC support. However, less than a fifth of those without a lung cancer co-diagnosis (n = 16,934) were offered PC (n = 3268; 19.3%).

Overall, during the study period, the proportion of patients with IPF and a PC referral increased from 2.9% in 2010 to 7.5% in 2019. Throughout the study period, women lagged behind men for PR and PC referrals. Therefore, in the fully-adjusted analysis, female sex and Black ethnicity were most strongly associated with non-referral, while older age and co-existing lung cancer, dementia, and cardiovascular comorbidities remained associated with PC referral.

Before the end of the study period, just under half of the population died (n = 7918), 2658 (33.6%) of whom received a referral to PC services. The median time lapse between PC referral and death was 73 days (IQR, 20-229 days). About 70% were referred within 6 months of their death, while only 15% had a referral more than a year before they died. Additionally, patients who also had a COPD diagnosis (n = 515) were more likely to receive an “early” PC referral, meaning at least a year pre-death (relative risk ratio [RRR], 1,69; 95% CI, 1.27-2.26).

Lastly, they acknowledged their limitations, including their inability to reliably identify referrals to PR and PC services in routinely collected primary care data; this is because they relied on the quality of patient consultation coding by GPs and practice staff. Despite their limitations, the researchers used their findings to suggest areas for future action.

“While for this patient population, the benefits of PR and early referral to PC services are increasingly being recognized and embodied in current guidelines, it is evident that there needs to be substantial investment in service provision if the currently unmet demand for comprehensive and patient-centered PR and PC services is to be met,” the authors concluded.

References

1. Morgan AD, Khan H, George PM, Quint JK. Referral to pulmonary rehabilitation and palliative care services in people with idiopathic pulmonary fibrosis in England, 2010-2019. NPJ Prim Care Respir Med. 2024;34(1):27. doi:10.1038/s41533-024-00387-6
2. Strongman H, Kausar I, Maher TM. Incidence, Prevalence, and survival of patients with idiopathic pulmonary fibrosis in the UK. Adv Ther. 2018;35(5):724-736. doi:10.1007/s12325-018-0693-1
3. Gomes-Neto M, Silva CM, Ezequiel D, Conceição CS, Saquetto M, Machado AS. Impact of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. J Cardiopulm Rehabil Prev. 2018;38(5):273-278. doi:10.1097/HCR.0000000000000273
4. Oliveira A, Fabbri G, Gille T, et al. Holistic management of patients with progressive pulmonary fibrosis. Breathe (Sheff). 2023;19(3):230101. doi:10.1183/20734735.0101-2023
5. Wolf A, Dedman D, Campbell J, et al. Data resource profile: Clinical Practice Research Datalink (CPRD) Aurum. Int J Epidemiol. 2019;48(6):1740-1740g. doi:10.1093/ije/dyz034