ADOPTING ORPHAN DISEASES RARE INTERSTITIAL LUNG DISEASES

adoptingorphandiseaseskapak

END-OF-LIFE CARE INRARE INTERSTITIAL LUNG DISEASES

Burcu Özalp1 Meltem Uyar2

1Ege University, Faculty of Medicine, Department of Algology, İzmir, Türkiye
2Ege University, Faculty of Medicine, Department of Algology, İzmir, Türkiye

Ozalp B, Uyar M. End-of-Life Care in Rare Interstitial Lung Diseases. In: Altinisik G, McCormack FX, editors. Adopting Orphan Diseases: Rare Interstitial Lung Diseases. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.219-232.

ABSTRACT

Rare interstitial lung diseases (ILD) represent a heterogeneous group of respiratory diseases characterized by inflammation and fibrosis in the lung interstitium. Despite being perceived as rare diseases, ILD is the 40th most common cause of death globally, with a 52% increase in mortality over the last 10 years. The majority of these diseases progress over time, leading to respiratory failure and the need for comprehensive palliative care.

Increased dyspnea at rest, refractory hypoxemia, increased hospitalizations, decreased functional capacity are the most frequently observed signs of clinical deterioration. These signs indicate that it is time for end-of-life planning for the patients and families. Patients with advanced ILD experience a high burden of symptoms, especially at the end of life. Early integration of palliative care can significantly improve quality of life.

A comprehensive needs assessment in individuals with interstitial lung disease (ILD) is defined as a key component of the palliative care process. This assessment should address the physical, psychosocial and practical needs of not only patients but also informal caregivers and family members, including the timing and coordination of support services.

A multidisciplinary and individualized needs assessment approach rather than prognosis-based approaches is recommended in patients with ILD. It is important that this approach is carried out during stable phases of the disease rather than during acute exacerbations.

Advance Care Planning (ACP) is essential in ILD and should define patient values, preferences, and goals in advance and has been identified as an essential component of the palliative care process. Patients’ choices for end-of-life management (e.g., mechanical ventilation, resuscitation) are shaped by prognostic understanding, disease burden, spiritual and existential concerns.

End-of-life care in rare ILDs requires a multidisciplinary and individualized approach centered on the needs of the patient and their family. The experience and collaboration of healthcare professionals is important in key areas such as symptom management, communication and advanced care planning. Early integration of palliative care not only improves symptom control and quality of life but also enhances active participation of patients and families in the care process.

In this context, incorporating current clinical guidelines and new pharmacological/psychosocial support approaches will improve the effectiveness of care. Furthermore, additional studies on the roles of multidisciplinary teams and the experiences of patients and their families will contribute to the development of patient-centered care models in the future.

Keywords: Palliative care; End-of-life care; Interstitial lung diseases; Rare interstitial lung diseases; Patient centered care

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