A blog by Daisy J.A. Janssen, MD PhD

25 July, 2018

I am sitting next to her on my knees. She just managed to make a few steps out of her bed and is sitting on a chair, trying to catch her breath. First, we are both silent. She is too much out of breath to speak and I don’t know what to say. Almost two years ago she came to our specialised centre, to our unit for palliative and supportive care for patients with endstage lung disease. In between she has been at home for a few months. Well, at home, she mostly was in the hospital, because she can’t be at home anymore. She wishes that she could live at home, with her husband and her pets. She is still young and has been suffering from very severe COPD for many years. She has been on the waiting list for lung transplantation for five years. Gradually, she is deteriorating.

When she is able to talk a bit we talk about her severe breathlessness, everything that we have tried to manage her breathlessness, and we are searching for something we didn’t try yet. But we don’t find anything. Her COPD is pharmacologically optimally treated. There are no surgical options, except lung transplantation. She is too frail for endoscopic lung volume reduction. We are offering her all components of a pulmonary rehabilitation program she is still able to participate in. She is using oxygen therapy and non-invasive ventilation. The psychosocial team is intensively involved to help her cope with her situation and treat breathlessness-related anxiety. The speech and language therapist pays attention to her breathlessness while she is speaking and eating. She is using opioids and benzodiazepines. We treat her comorbidities and every time, when her breathlessness is so severe, we rule out other treatable causes. The nurses apply a personal crisis plan to manage episodes of acute breathlessness. Now we talk about the lung transplantation. Will it be in time? And what if it won’t? Would it have been worthwile for her to have stayed on our unit for so long, trying to get out of bed each day, trying to walk, trying to follow her therapies and to control her weight gain? Even if she she will die before lungs are available for her?

For more than ten years I have dedicated my work as physician and clinical researcher to the treatment of patients like her. Frequently I give lectures to healthcare professionals about how to offer personalised care to patients with advanced lung disease, integrating disease-oriented and palliative care. And now I am sitting next to her, on my knees. I see her daily struggle with her breathlessness, despite everything we do to manage it. I remember the times that I was standing next to her during episodes of acute breathlessness. We talk about what makes life still worthwhile to her. A single tear appears in her eye. We talk about how difficult it is to not have the breath to be able to scream and get the frustration out. Despite my knowledge and experience I am standing here empty-handed. The only thing my hands can do, is hold hers.

The story of this patient underlines both the importance as well as the challenge of managing severe breathlessness in endstage COPD. Living with severe breathlessness has consequences for all life domains resulting in impaired quality of life.[1] Breathlessness is a complex symptom, deriving from multiple physiological, psychological, social and environmental factors and inducing secondary physiological, as well as behavioral, responses.[2] Systemic opioids are effective for severe breathlessness[3], but a single palliative intervention won’t be able to completely manage such a complex and multifactorial symptom. Indeed, optimal management of severe breathlessness starts with optimal treatment of the underlying disease, somatic and psychological comorbidities and intercurrent diseases aggrevating breathlessness. Palliative management consists of pharmacological interventions (e.g. opioids) as well as non-pharmacological interventions (for example a hand-held fan, activity pacing, mobility aids).[4] A personal crisis plan with a stepwise approach for episodes of acute breathlessness is needed.[5] Severe breathlessness requires interdisciplinary treatment. The chest physician, palliative care physician, nurse, physiotherapist, occupational therapist, speech and language therapist, psychologist and art therapist contribute to the personal care plan of this patient. Despite all our efforts, breathlessness remains severe for this patient. However, palliative care doesn’t stop after applying all available pharmacological and non-pharmacological interventions. Palliative care is holistic care and goes beyond physical symptoms. Offering palliative care is also about treating the whole person, building relationships, maintaining dignity, confirming non-abandonment, but also aknowledgement of limitations and discussing realistic expectations. A team-approach is not only needed for management of complex problems, but also to support each other in coping with the witnessing of suffering.[6]

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About the author

Dr Daisy J.A. Janssen is elderly care physician and palliative care physician in Ciro, a centre offering specialised treatments for patients with chronic lung diseases in Horn, the Netherlands. She is also research coordinator at the Centre of Expertise for Palliative care at Maastricht University Medical Centre in Maastricht, the Netherlands.

Her field of expertise is palliative care and advance care planning for patients with advanced non-malignant disease, including COPD. In addition, she studies the psychosocial needs of patients with advanced chronic lung diseases.

Further, she is (co-)author of more than 70 peer-reviewed English-language articles related to palliative care and psychosocial needs of patients with advanced chronic lung diseases.

Dr Janssen has been a member of ERS for over 10 years, during which time she has played an active role in ERS Taskforces.

References for this article

  1. Janssen DJ, Wouters EF, Spruit MA. Psychosocial consequences of living with breathlessness due to advanced disease. Curr Opin Support Palliat Care 2015; 9: 232-237.
  2. Parshall MB, Schwartzstein RM, Adams L, Banzett RB, Manning HL, Bourbeau J, Calverley PM, Gift AG, Harver A, Lareau SC, Mahler DA, Meek PM, O'Donnell DE. An official American Thoracic Society statement: update on the mechanisms, assessment, and management of dyspnea. Am J Respir Crit Care Med 2012; 185: 435-452.
  3. Ekstrom M, Bajwah S, Bland JM, Currow DC, Hussain J, Johnson MJ. One evidence base; three stories: do opioids relieve chronic breathlessness? Thorax 2018; 73: 88-90.
  4. Maddocks M, Lovell N, Booth S, Man WD, Higginson IJ. Palliative care and management of troublesome symptoms for people with chronic obstructive pulmonary disease. Lancet 2017; 390: 988-1002.
  5. Mularski RA, Reinke LF, Carrieri-Kohlman V, Fischer MD, Campbell ML, Rocker G, Schneidman A, Jacobs SS, Arnold R, Benditt JO, Booth S, Byock I, Chan GK, Curtis JR, Donesky D, Hansen-Flaschen J, Heffner J, Klein R, Limberg TM, Manning HL, Morrison RS, Ries AL, Schmidt GA, Selecky PA, Truog RD, Wang AC, White DB, Crisis ATSAHCoPMoD. An official American Thoracic Society workshop report: assessment and palliative management of dyspnea crisis. Annals of the American Thoracic Society 2013; 10: S98-106.
  6. Swetz KM, Harrington SE, Matsuyama RK, Shanafelt TD, Lyckholm LJ. Strategies for avoiding burnout in hospice and palliative medicine: peer advice for physicians on achieving longevity and fulfillment. J Palliat Med 2009; 12: 773-777.