1. Living and Dying with Chronic Lung Disease
Dr Brian Bradley December 7th 2011

2. Respiratory Service Clinics / Respiratory Nursing Team at home
Dr Downes Palliative Care Clinic
Chronic Disease Management Clinic – Clinical Psychologist
Pulmonary Rehabilitation

3. Examples - Joyce
73 year Severe COPD and Bronchiectasis 2-3 admissions / year Always asks “Why am I breathless”
Clinic: “ How Long can this go on for ? “
Follow up at home – Respiratory Nurse
Eventually died at home

4. Example - Ronald 1
54 year old fencer. Extrinsic Allergic Alveolitis Progressive Fibrosis over 14 years Respiratory Failure on LTOT
IHD with MI and stent insertion Severe Aortic Stenosis and re-stenosis Gross Right Heart Failure
Numerous Conversations – never took it on board – died in Hospital

5. Example – Ronald 2
79 year old + IPF which was stable for 5 years CABG and then lung function deteriorated over 6 months
Wife very anxious pushed him to do things Would not contemplate opiates, had Oxygen – did not use it
Had pulmonary Emboli
Palliative Care team / Respiratory – deterioration, not for resuscitation
Died in Hospital

6. Living and Dying with Chronic Lung Disease
National Policy on End of Life Care
Can we predict the end of life?
What can we do for patients? (symptoms / support)
How are these issues best discussed?

7. COPD Deaths Increasing in USA
Of the six leading causes of death in the United States, only COPD has been increasing steadily since 1970
Deaths from:
Heart Disease Cancer
Stroke
Diabetes Accidents
Decreasing or steady
Source: Jemal A. et al. JAMA 2005

8. Deaths from Respiratory Disease

9. Cause of Death : number of deaths in England, 2007/09

10. Cause of Death : proportion of deaths in England 07/09

11. Chronic lung diseases : number of deaths in England 07/09

12. Number of death by Region in England, 2007/9

15. G OL D
lobal Initiative for Chronic bstructive ung isease

16. Four Components of COPD Management
Assess and monitor disease
Reduce risk factors
Manage stable COPD
Education
Pharmacologic
Non-pharmacologic
Manage exacerbations

17. Living and Dying with Chronic Lung Disease
National Policy on End of Life Care
Can we predict the end of life?
What can we do for patients? (symptoms / support)
How are these issues best discussed?

18. Illness Trajectories Ellershaw’s trajectory from BMJ
Typical illness trajectories for people with progressive chronic illness. Adapted from Lynn and
Adamson, With permission from RAND Corporation, Santa Monica, California, USA.
Published in BMJ Murray, S. A et al. BMJ 2005;330:

19. What is the prognosis?
Demonstrates that predicting prognosis is very difficult.
Christakis, N.A and J.J. Escare (1996). “Survival of Medicare Patients after Enrolment in Hospice Programs.”
N Engl J Med 335(3):

20. COPD

21. Predicting Prognosis
But we have the Fletcher Peto diagram which provides some indication but….
Fletcher CM, P. R. (1977). "The natural history of chronic airflow obstruction." British Medical Journal(i):

22. Classification of COPD Severity -Spirometry
Stage I: Mild FEV1/FVC < 0.70
FEV1 > 80% predicted
Stage II: Moderate FEV1/FVC < 0.70
50% < FEV1 < 80% predicted
Stage III: Severe FEV1/FVC < 0.70
30% < FEV1 < 50% predicted
Stage IV: Very Severe FEV1/FVC < 0.70
FEV1 < 30% predicted or
FEV1 < 50% predicted plus chronic respiratory failure

23. Indicative markers in COPD:
Severe airflow obstruction (FEV1 <30% predicted)
Respiratory failure
Low BMI (less than 19)
House bound (MRC dyspnoea score 5)
History of two or more admissions for exacerbations during the previous year
Need for non-invasive ventilation for an acute exacerbation
Eligibility for long-term home oxygen therapy

24. Predicting prognosis – acute exacerbation of COPD
Age >70 FEV1 <30% predicted pH 7.26 PaCO2 >8 kPa PaO2 <7.3 kPa No of admissions Other co-morbidity
Hospital type/resources
SaO2 on admission
PaO2/FiO2
Diffusion capacity
Body mass index <18
Albumin
Factors thought to be important in determining the outcome of an admission with an acute exacerbation – these can even be plugged into mathematical equations (Apache score) to produce an estimated outcome from an ITU admission

25. Making a prognosis in COPD
Mortality after admission to hospital in severe COPD is between 36% and 50% at 2 years
The mortality data are after an admission with severe COPD (respiratory failure),
Almagro excludes the 11% who died whilst inpatients in the Connors study, the figures are remarkably similar.
Connors et al. Am J Respir Crit Care Med 1996;154(4 Pt 1):959-67
Almagro et al. Chest 2002;121(5):1441-8

26. Interstitial Lung Disease
Idiopathic Pulmonary Fibrosis

27. ILD – Idiopathic Pulmonary Fibrosis Prognosis and Survival
Carbon monoxide transfers (TLCO) < 40%
Fall in FVC of 10% over 6-12 months > 15% in TLCO
Desaturation during 6 minute walk at presentation
A TLCO of <39% of predicted, combined with HRCT scores, had an 80% sensitivity and specificity for death within 2 years.(Mogulkoc et al)
TLCO levels of <35% were associated with a mean survival of 24 months (Latsi et al)

28. ILD / IPF - Treatment
To date there is no therapy proven to improve survival or otherwise significantly modify the clinical course of IPF
Disease Modifying Drugs – Steroids, Azathioprine, N AcetylCysteine – very dissappointing
Lung transplantation if appropriate.

30. ILD/IPF -Best supportive care
Proactive approach to symptomatic treatment: Oxygen therapy Pulmonary rehabilitation Opiates Antireflux therapy Stop steroids & other immunosuppressants Smoking Cessation
Early recognition of terminal decline and liaison with palliative care specialists.

31. Living and Dying with Chronic Lung Disease
National Policy on End of Life Care
Can we predict the end of life?
What can we do for patients? (symptoms / support)
How are these issues best discussed?

32. Symptom prevalence in advanced COPD / ILD
Breathlessness 60%–88%
Fatigue 68%–80%
Anxiety 51%–75%
Pain 34%–77%
Depression 37%–71%
Insomnia 55%–65%
Anorexia 35%–67%
Constipation 27%–44% Solano et al. 2006

35. Treatment & devices

36. Treating dyspnoea in COPD- bronchodilators
2agonist better Placebo better
Dullinger
Guyatt
Hansen
Shah
Pooled
Bronchodilators for the relief of dyspnoea
Bausewein C, Booth S, et al. Cochrane Database Syst Rev is a review of non – pharmacological interventions for treating dyspnoea
This review by Sestini in stable COPD - does show benefit.
Sestini P, Renzoni E, Robinson S, Poole P, Ram FSF. Short-acting beta2-agonists for stable chronic obstructive pulmonary disease. Cochrane Database of Systematic Reviews 2002, Issue 3. Art. No.: CD DOI: / CD001495
Sestini P, et al . Short-acting beta2-agonists for stable chronic obstructive pulmonary disease. Cochrane Database of Systematic Reviews 2002, Issue 3. Art. No.: CD001495

37. Treating dyspnoea - opiates
Opiates for the relief of dyspnoea
Opioids better Placebo better
Woodcock
Johnson
Eiser
Brurera
Light
Chua
Poole
Pooled
his review supports the continued use of oral and parenteral opioids to treat dyspnoea in patients with advanced disease. The results of the subgroup analysis of the COPD studies were essentially similar to the results of the main analysis. There are insufficient data from the meta-analysis to conclude whether nebulised opioids are effective, but the results from included studies that did not contribute to the meta-analysis suggest that they are no better than nebulised normal saline.
Side effects:
constipation
nausea
drowsiness
but no increased hypoxia
Jennings A-L. Thorax 2002; 57:

38. Opiates & Benzodiazepines
Morphine
Low dose
Oral
Consider side effects
IV or SC Diamorphine in extreme distress
2nd or 3rd line
Diazepam or Lorazepam
Used when no response to other therapies

39. Treatment of breathlessness in advanced COPD – non pharmacological
Breathing training
Walking aids
Neuromuscular electrical stimulation
Chest wall vibration
Hand-held fan
Anxiety management
Physiotherapy
DRUGS
Booth S, Moosavi SH, et al. Nat Clin Pract Oncol (2):90-100
Booth S, Farquhar M, et al. Palliat Support Care 2006;4(3):287-93

40. Positioning, remember to focus on breathing when it is helpful!!

41. Provision of Domicilary Oxygen
Aims of oxygen therapy
To correct or prevent potentially harmful hypoxaemia
To alleviate breathlessness (only if hypoxaemic)
Oxygen has no effect on breathlessness if the oxygen saturation is normal

42. Types of Domicillary Oxygen
Long Term Oxygen Therapy (LTOT)
Ambulatory Oxygen
Short Burst Oxygen

45. Why use LTOT? Improved survival Reduce secondary polycythemia
Improve Neuropsychological health
Prevention of progression of pulmonary hypertension
Improved sleep quality
Reduction in cardiac arrhythmias
Increased renal blood flow

46. Criteria for LTOT Severe COPD with PaO2 <7.3kPa
paO2 < 8kPa with nocturnal hypoxemia and peripheral oedema
Provision of oxygen therapy for a minimum of 15hrs/24hrs administred at a flow rate sufficient to raise the PaO2 to above 8kPa

48. Ambulatory oxygen Effective in increasing exercise capacity
Reduces breathlessness on exertion
For patients who de-saturate by 4% to less than 90%
Improves concordance

49. Short Burst Oxygen Intermittent use of oxygen for 10-20 minutes
No evidence
No formal assessment

51. COPD and Co-Morbidities
COPD has significant extrapulmonary
(systemic) effects including:
Weight loss
Nutritional abnormalities
Skeletal muscle dysfunction

52. Living and Dying with Chronic Lung disease
How successful is our care now?
Do patients want to know ?
Who should do it and when ?
How !!

53. End of life care - to live “as well as possible” until they die
Enables the supportive and palliative care needs of both patient and family to be identified and met throughout the last phase of life and into bereavement
It includes the management of pain, breathlessness and other symptoms and provision of psychological, social, spiritual and practical support.

54. Appropriate care near the end of life.
Appropriate care near the end of life. Adapted from Lynn and Adamson, With permission from RAND Corporation, Santa Monica, California, USA
Murray S A et al. BMJ 2005;330:
©2005 by British Medical Journal Publishing Group

56. Current Service Provision
One third of patients lacked regular follow up in hospital or in the community
One third of patients saw their GP less than 3 monthly or never
Lack of home-based services although many patients were housebound
Elkington, White, et al Pall Med :

57. How are people who die from COPD affected?
In the last year of life
40% had breathlessness unrelieved
68% had low mood unrelieved
51% had pain unrelieved
20% did not know they might die
70% died in hospital (for 25% of whom it was not the best place to die)
To conclude we have found that….
The reference from this was from discussion with informants (next of kin / spouse) of patient who had died from COPD.
Elkington, White, et al Pall Med :

58. How are people who die from COPD affected?
“I’m that short of breath,
I get breathless even
going to the toilet”
Skilbeck. Palliative Care 1998; 12:
82% housebound
36% chair bound
Gore, J. M., C. J. Brophy, et al. (2000). "How well do we care for patients with end stage chronic
obstructive pulmonary disease (COPD)? A comparison of palliative care and quality of life in COPD
and lung cancer." Thorax 55(12):

59. COPD compare with lung cancer?
Gore. Thorax 2000; 55:
Worse quality of life

60. COPD compare with lung cancer?
No differences in the total number of symptoms in final year / week of life but COPD patients have these for longer
Lung Cancer
COPD
Moderate – severe breathlessness
78%
94%
Pain
25%
63%
Depression
55%
Edmonds, P., S. Karlsen, et al. (2001). "A comparison of the palliative care needs of patients dying from chronic respiratory diseases and lung cancer." Palliative Medicine 15(4):

61. Provision of care – not just medication!
It may be other things that have big differences in patients and carers lives.
Gore. Thorax 2000; 55:

62. Living and Dying with Chronic Lung disease
How successful is our care now?
Do patients want to know ?
Who should do it and when ?
How !!

63. COPD Patient’s perspectives on Physician skill in End of Life Care
More education about their lung disease and short and long term prognosis
Strategies for dealing with the present, and personal goals
Maintenance of hope
Patients expect physicians to raise these topics
Continued emphasis on symptom control (Randall Curtis et al Chest 2002;122:356-62)

64. COPD Patients and Relatives
Unaware that COPD is life threatening (Spathis & Booth 2008)
More want to discuss prognosis and end of life care than currently do (Dean 2007)
Fear of uncontrolled symptoms contribute to high number of hospital deaths
30% Lung Cancer and only 8% of COPD patients were told they could die from the disease – Physicial and psychosocial needs are at least as intensive as for Lung Cancer – but not as holistic (Edmonds 2001)
Evidence suggests that patients (and carers) require “early phased support” and ongoing assessment of need throughout the “lifetime journey with COPD”. (Pinnock et al 2011)

65. Do patients want to know?
73% of patients would like to discuss prognosis 1
64% of clinicians felt it was difficult to start a discussion 2
In severe COPD, when offered an appointment with a palliative care specialist to discuss prognosis / end of life care issues (on top of usual care) 29% took up the offer 3
Elkington, H., P. White, et al. (2001). "GPs' views of discussions of prognosis in severe COPD." Fam. Pract. 18(4):
Matthews A et al, End of life planning for severe COPD: a pilot study (P230 – abstract; Winter BTS, 2007 available on Thorax Supplement) – this showed that only 15/52 (29% took up the offer)
It is important to emphasis that although most would like to discuss – it is not all that want to.
Also the timing has to be right for them to want to know.
1 – Elkington et al, Elkington et al, – Matthews et al, 2007

66. Provides an opportunity to discuss
The future
The patient’s understanding of prognosis
Resuscitation status
ICU admission
Advance care plans
When ?
Stable
During Outpatient’s Visit
During Pulmonary Rehabilitation

67. Some prompts
“You’ve been quite poorly recently – what are your thoughts about all of this?”
“This is the third time you’ve been in hospital this year, it seems to be getting more frequent – what are your thoughts (feelings) about this?”
‘Would you find it helpful to talk about the future and how you would want to be cared for?”
Well worth asking the participants what they would use? Perhaps if the opportunity is there they can practice or role play

68. Potential outcomes from ACP discussions
Documentation of patients wishes
Patient and/or family has better understanding of illness and treatment options of issues like DNAR and ventilation including non-invasive ventilation
Opportunity for patient to improve communication with family
Patient may appoint an Lasting Power of Attorney
Patient may make an Advance Directive
Professional has better understanding of the patients fears, beliefs and wishes

69. Barriers to communication about end of life issues in COPD / ILD
Patient Barriers
Healthcare Professional
Difficulty in timing discussions because of uncertain prognosis
Lack of time during consultations
Concern about taking away patients’ hope
Belief that patients are not ready to discuss end of life issues
Expectation that healthcare professionals will initiate discussions
Societal taboos with regard to discussing death
Uncertainty about which professionals will be involved during end of life phase
Lack of certainty about the type of care that would be wanted when less well

70. What is Advanced Care Planning?
“Advanced care planning is a voluntary process of discussion between an individual and their care providers irrespective of discipline. If the individual wishes, their family and friends may be included in the discussions. With the individual's agreement, this discussion should be recorded, regularly reviewed and communicated to key persons involved in their care.”
DH, 2008

71. Advanced care planning should address
the individual's concerns
their important values or personal goals for care
their understanding about their illness and prognosis
preferences for types of care or treatment that may be beneficial in the future and the availability of these

72. Advanced Care Planning
Unpredictable timing of should not lead to communication paralysis.
Healthcare professionals should initiate discussions about end of life care as early as possible in the disease trajectory.
Honest & compassionate ACP involves a dual approach of optimism and realism, “hoping for the best and preparing for the worst.”
Make clear to patients that limiting life sustaining treatment does not equate to limiting care.

73. Remember Need to be realistic – but give hope
Be collaborative – give patient dignity and control of their life

74. Respiratory Service
Collaborative work – works well for patient Can provide best care Respecting patients choice
Need to focus more on the relevant groups in Primary and secondary care Take the opportunities to address the issues Incorporate it more into standard
Use the services that are available more: Palliative Care Team Clinical Psychology/Chronic Disease Management Pulmonary Rehabilitation GPWSI