Interstitial Lung Disease – An update from the Irish Lung Fibrosis Association
Written by Nicola Cassidy, Director of the Irish Lung Fibrosis Association
Then…
A fascinating set of photographs in the British Museum’s Egyptology section describes how tissue from ancient mummies were studied with modern microscopic and analytical techniques. Lung tissue from one mummy showed histological evidence of lung fibrosis providing evidence that this condition has been around since ancient times, yet only in relatively recent times has research and investment in the study of lung fibrosis experienced exceptional growth.
And now…
Lung fibrosis, also known as Interstitial lung diseases (ILD), is an umbrella term for a heterogenous group over 200 types of lung diseases characterised by the process of abnormal tissue repair with resultant disruption and destruction of the lung architecture by scar tissue formation (fibrosis). The most prevalent ILD is idiopathic pulmonary fibrosis (IPF) – a diagnosis of exclusion when all other known ILDs have been ruled out. IPF is a severe, progressive lung disease characterised by breathlessness, hypoxemia, dry and non-productive cough, fatigue, and ultimately respiratory failure. Lung fibrosis is a devastating diagnosis that causes significant physical, mental, and financial burden to patients and their caregivers.1 Psychological distress can develop when lung fibrosis impacts on patients’ abilities to engage in normal activities of daily living and the associated deterioration in quality of life.
ILD is thought to affect around 5,000 patients in Ireland. This figure is extrapolated from international prevalence data as the lack of a disease registry means there are no direct data currently collected in Ireland. This omission will have significant implications for planning and service delivery for the healthcare system going forward as the incidence and prevalence of lung fibrosis are likely to rise with an ageing population. The median survival time from diagnosis is 3-5 years which is worse than many cancers, yet awareness of this serious, life-limiting condition is poor among healthcare professionals and the public.
The cause and pathogenesis of ILD is poorly understood but there has been remarkable progress in our understanding of the disease pathogenesis and treatment options over the last 10-15 years. Risk factors include male gender, aged >60 years, previous history of smoking, connective tissue disease, occupational exposure to dusts, mould, birds and other organic matter, industrial agents, radiation. Exposure to certain medications including nitrofurantoin and amiodarone may be significant in certain cases. Familial lung fibrosis is a well-recognised entity, and inherited genes likely influence the risk of developing ILD in up to 20% of individuals.2
During the Covid-19 pandemic, ILD patients were deemed to be extremely medically vulnerable because of their underlying respiratory health and were prioritised for vaccination.
International studies revealed the stark fact that 50% of lung fibrosis patients admitted to hospital with Covid-19 died.3 Given the pulmonary complications associated with severe covid-19 infection, it was expected that an increase in ILD cases might emerge due to the pandemic. Early data in a recent study has suggested an increased incidence but longitudinal data is needed to understand the emerging impact.4
Diagnosing ILD can be difficult with many patients experiencing long delays as the symptoms are quite non-specific. Many patients are initially misdiagnosed with more common respiratory conditions such as Chronic Obstructive Pulmonary Disease (COPD) and asthma, and congestive cardiac failure (CCF). A thorough medical history should be conducted considering the presenting symptoms, timeline of symptom onset and nature of progression, comorbidities, occupational history, hobbies and pastimes, family medical history, and current and past medical therapies. On physical examination the presence of signs like finger clubbing and crackles on chest examination are significant. General Practitioners should have a high index of suspicion and include ILD on the list of differential diagnoses in patients presenting with worsening respiratory symptoms despite antibiotic therapy, recurrent pneumonia, and chronic unresolving, nonproductive cough. In the first incidence investigations like chest X-Ray and possibly CT Thorax and pulmonary function tests should be requested. Patients with abnormal finding or a worrisome or unexplained presentation should be referred to a respiratory consultant for further assessment. In cases of suspected ILD this would ideally be to an ILD Specialist Centre.
A specialist review will include a high-resolution CT scan of the thorax-this is a key study in the diagnosis of all ILDs. Pulmonary function tests are used to quantify any physiological abnormalities in the size or function of the lungs. Blood tests including a full biochemistry panel with immunological and rheumatological profiles are also needed.
An accurate and timely diagnosis requires a multidisciplinary team approach involving radiologists and respiratory clinicians supported by pathologists, rheumatologists, and cardiothoracic surgeons. There is no cure for lung fibrosis, but anti-fibrotic medications (Nintedanib and Pirfenidone) have been shown to slow the rate of disease progression. A significant percentage of patients may not tolerate these due to side effects or other contra-indications. Hence continued research and drug development in this area is crucial. Lung transplantation can provide longer term survival for some individuals, but it is available only to a small number of patients who meet strict health criteria. Supportive therapies including pulmonary rehabilitation and targeted exercise are key components of the treatment for all patients with ILD and ideally should be universally available. Similarly, access to supplemental oxygen therapy is important for certain patients. Respiratory clinical nurse specialists, palliative care professionals and allied healthcare professionals particularly physiotherapists, dietitians, social workers play an essential role in supporting patients but there is evidence that ILD patients frequently have poor access to these healthcare professionals.5
The Irish Lung Fibrosis Association (ILFA) is a patient organisation founded in 2002 to support individuals with lung fibrosis and their families, and to promote research and education in lung fibrosis. Over the years, ILFA has experienced an exceptional increase in demand for our support services, advocacy activities, and educational opportunities. ILFA is firmly established as a leading lung fibrosis patient organisation in the national and international respiratory community and was a founding member of the European Union Pulmonary Fibrosis Federation (EUPFF).
The National Patient Charter for Idiopathic Pulmonary Fibrosis was developed by ILFA in collaboration with key stakeholders in 2015 to ensure that lung fibrosis patients have
- Early and accurate diagnosis with multi-disciplinary team input.
- Clear and concise information about PF in plain language.
- Access to appropriate medicines and oxygen therapy.
- Early referral to pulmonary rehabilitation and exercise programmes.
- Early referral to the National Lung Transplant Unit for lung transplant assessment, with a minimal emphasis on age.
- Access to social, practical and emotional supports.6
Today, unfortunately, the vast majority of ILD patients are still forced to fight a battle on two fronts – against the progression of their disease, and against an inequitable ‘healthcare system’ that denies them access to the care they need. Equitable healthcare as was envisioned in Sláintecare for ILD patients can only be realised through the establishment of a Clinical Care Programme. While ILFA works toward this goal, there are interim priorities that ILFA have requested the Irish Government fund. These priorities include access to pulmonary rehabilitation, tax rebates to offset increased electricity costs, the establishment of an ILD patient registry, and resourcing the Clinical Care Pathway.
September is Pulmonary Fibrosis Awareness Month, an international global campaign that aims to raise awareness of lung fibrosis. In Europe, the campaign is led by the EUPFF in association with its member organisations including ILFA. The theme for the 2024 campaign is “Oxygen is a fundamental human right”. This theme aligns with ILFA’s work ensuring that patients must be spared financial hardship and having to make agonising health choices if they cannot afford to purchase and run oxygen via electricity due to high costs. We want all patients to receive supplemental oxygen for free under the Drug Payment Scheme eliminating the cumbersome payment and reimbursement system currently in operation. Patients should also be entitled to an automatic tax rebate on their electricity costs. ILFA is also working with other patient organisations to ensure that the new home oxygen contracted introduced by the HSE Community Funded Schemes, Service Improvement Programme is efficient, fit for purpose, and patient centred. Oxygen is lifepreserving and patients requiring supplemental oxygen should have the right to breathe easier.
If you are involved in the care of ILD patients, please refer them to the Irish Lung Fibrosis Association where they can access information, resources, and peer support through our network. www.ilfa.ie