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170 Chronic Obstructive Pulmonary Disease – Current Concepts and Practice

Another complicating factor is that what is acceptable to patients may change over time as they adjust to severe illness and this may influence discussions and the willingness of GPs to initiate advance care planning (Halpin et al., 2008). It is typical of people to normalise their experience of even severe day-to-day symptoms and see themselves a sick only during acute exacerbations. This may be, in part, a coping strategy, but is also a result of the long illness trajectory. Whilst in cancer narratives, there is a definite beginning and developing plot to the 'cancer story', COPD is more likely to be insidious in its beginning and intertwined with the person's 'life story'. The unpredictability of exacerbations creates a chaotic component to the person's experience of illness, yet they may have a sense of relative wellness between these crises (Pinnock et al., 2011). Whilst people may feel that each acute exacerbation may be their last (Oliver, 2001) the threat of death recedes after a COPD crisis, or perhaps the threat of death is also normalised. The result is that death is less likely to be considered imminent and so wishes are rarely discussed with professional carers, friends or family (Pinnock et al., 2011). Where end-of-life discussions do occur, they may be poorly documented and so patient wishes may not be visible to family or other members of a multi-

Having end-of-life discussions with COPD patients and families constitutes significant emotional work for clinicians and requires 'conscious emotional management'. This comes with experience as professionals learn to feel their way with an individual, and apply emotional intelligence and empathetic skills in their discussions (Crawford, 2010). Some ways to approach these difficult conversations include beginning discussions early in the disease course, using the uncertain disease trajectory to ease discussions and building a caring and respectful relationship with patients. It is useful to have a team approach with recognition of the collective responsibility of GPs, respiratory nurses and physicians to proactively identify and use opportunities to talk about prognosis (Halliwell

The aim of good end-of-life discussions is to inform without removing hope, and to bring to the forefront the wishes of the patient and family. Research participant, Mary, described how she appreciated the honesty and sensitivity of the discussions after her husband had an ICU admission: *"The doctor did tell us the dangers of intubation …then when he was moved to ward said, 'You've come through this okay…Perhaps in the future it might happen again…You need to think what you want done, you and your family.' Just nicely … And I* 

Discussing prognosis broadly in terms of a diagnostic population rather than directing it at the individual leaves room for hopeful possibilities. Physicians can foster hope by giving a 'commitment to non-abandonment', by addressing people's fears, such as fear of pain at end-of-life, and by having a management plan that addresses their changing situation (Curtis et al., 2008). Helping people to identify realistic goals and discussing their concerns about day-to-day living can also be useful (Clayton et al., 2005). The ideal is for a formal Advance Care Plan to be documented early. Again, the uncertain disease course of COPD makes this more complex, and means physicians are less comfortable with initiating such plans (Halpin et al., 2008). Fins et al (2005) point out that the process can be simplified by creating possibilities for revision of the plan, and by trying to understand and be true to the patient's core values whilst remaining flexible around practical details such as where they

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**9** 

*Ireland* 

**Alpha-1 Antitrypsin Deficiency – A Genetic Risk Factor for COPD** 

Alpha-1 antitrypsin deficiency (AATD) is a hereditary disorder characterised by low circulating levels of the key antiprotease alpha-1 antitrypsin (AAT) and is associated with the development of chronic obstructive pulmonary disease (COPD), often by the 3rd or 4th decade, and liver disease. The two most common SERPINA1 mutations associated with AATD are the Z and S mutations, and the vast majority of AATD individuals diagnosed with COPD are ZZ homozygotes. AATD is an under-diagnosed condition with the majority of cases misdiagnosed as COPD. The World Health Organisation (WHO) and the American Thoracic Society/European Respiratory Society (ATS/ERS) advocate a targeted screening approach for the detection of AATD in patients with COPD, non-responsive asthma, cryptogenic liver disease and first degree relatives of known AATD patients (Alpha 1 antitrypsin deficiency: memorandum from a WHO meeting 1997; American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. 2003). It is our contention that a diagnosis of AATD gives the clinician a vital and unique opportunity for early medical intervention and the possible prevention of COPD in both the affected individual and first-degree relatives. Unfortunately, despite huge strides in awareness and

AAT is a secretory glycoprotein produced by the liver and is the most abundant serum antiprotease in circulation (Kueppers 1971). While the majority of AAT in the body is hepatocyte-derived, it is actively transcribed and secreted by other cell types including monocytes (Carroll et al. 2010), macrophages (Mornex et al. 1986), neutrophils (Bergin et al. 2010), intestinal epithelial cells (Perlmutter et al. 1989), and various epithelial cells in the lung (Hu and Perlmutter 2002; Venembre et al. 1994; Cichy, Potempa, and Travis 1997), albeit in smaller quantities. In keeping with its role as an acute phase reactant, the

understanding of this condition, this opportunity is too often missed.

**2. Alpha-1 antitrypsin deficiency (AATD)** 

**2.1 Alpha-1 antitrypsin (AAT)** 

**1. Introduction** 

Tomás P. Carroll, Catherine A. O'Connor, Emer P. Reeves and Noel G. McElvaney

*Department of Medicine,* 

*Beaumont Hospital, Dublin,* 

*Royal College of Surgeons in Ireland,* 

