palliative care of respiratory symptoms james s. botts, md, facp southwest area medical director...

Palliative Care of Respiratory Symptoms

James S. Botts, MD, FACP

Southwest Area Medical Director

VistaCare

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Outline of Topics… Identification of the Patient with Endstage Pulmonary Disease Dyspnea Cough Pulmonary Infections Hemoptysis Pulmonary Hypertension and Cor pulmonale Primary Pulmonary Hypertension Pulmonary Fibrosis Pulmonary Emboli Stridor Neuromuscular Disorders & Restrictive Pulmonary Disease Bronchiectasis and Cystic Fibrosis α-1 Antitrypsin Deficiency List of Links

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Identification of Endstage Pulmonary Disease No single event or parameter signals end stage

Persistent dyspnea despite optimal medical treatment Dyspnea impairing efforts to leave home Increasing number of hospital admissions Limited improvement after hospitalization Increasing number of physician visits Onset of fear, anxiety or panic attacks Expression of concerns about dying No reference to oxygen saturation or other parameter of

pulmonary function It is difficult to accurately identify those with a

prognosis of six months or less

1. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 903

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Identification of Endstage Pulmonary Disease Using CMS LCD pulmonary guidelines

50% of patients qualifying for pulmonary disease will live six months or less (n = 94)*

Pulse rate > 100 has the best correlation with a prognosis of six months or less in patients with endstage pulmonary disease

65.38% of patients meeting the CMS LCD guidelines for pulmonary disease with a pulse rate > 100 will live less than six months (n = 29)*

* Hospice Eligibility Evaluation Database (HEED) ; J.S. Botts

Palliative Care of Dyspnea

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Definition of Dyspnea (American Thoracic Society)

“A subjective experience of breathing discomfort consisting of qualitatively distinct sensations that vary in intensity. Physiologic, psychologic and environmental factors all may play a role. The severity varies widely among patients.”(2)

2. American Journal of Respiratory and Critical Care Medicine - Jan 1999

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Correlation of the complaint with the pathology of the underlying disease. Little correlation in general Some correlation of the following:

“I am drowning.” – Pulmonary edema with CHF “I can’t get enough air in.” – Interstitial disease or pulmonary

emboli.(2,3)

“Tight”, “Constricted” – a sensation used by those with airways obstruction such as asthma and cystic fibrosis but not COPD

2. Chest. 2005;127:1877-18783. Excerpt: Chest. 2005;127:1877-1878

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MidbrainRespiratory

Center

Respiratory Muscles ofBreathing

Peripheral Chemoreceptors

Aorta and Carotid Arteries CentralChemoreceptors

Medulla

MechanoreceptorsLungs and Chest Wall

Sensory Cortex

Dyspnea

Pathophysiology of DyspneaPathophysiology of Dyspnea

EmotionsPersonality

Sense levels of oxygen,carbon dioxide and pHof the blood. Sense levels of oxygen,

carbon dioxide and pH of the blood.

Motor Cortex

Sense stretching of structures in lungs and chest wall

Cor

olla

ryD

isch

arge

Adapted From: Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 898

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Palliative Care of DyspneaAssessment of Dyspnea Five etiologic categories

CardiacPulmonaryNeuromuscularPsychiatric / Social / SpiritualAny combination of the above

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Palliative Care of DyspneaAssessment of Dyspnea

History and Physical ExaminationFrequently identifies the specific system

responsible for the dyspnea Indicated diagnostic testing follows

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Palliative Care of DyspneaAssessment of Dyspnea - Testing Pulmonary Testing

ABG Chest X-ray Pulmonary Functions Bronchial Challenge High resolution CT Lung scan PET Diaphragmatic Fluoroscopy

Cardiac Testing EKG Echocardiography Coronary angiography Myocardial perfusion scan

Other Sleep studies Esophageal pH monitoring Laryngoscopy

Often hospice and palliative care patients choose not to be tested, placingmore reliance on the history and physical examination.

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Palliative Care of DyspneaAssessment of Dyspnea

Reporting Intensity of DyspneaVerbal numerical scales (0-10)VAS (Visual Analog Scale)Modified Borg Dyspnea Scale

Link to Modified Borg Dyspnea Scale

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Palliative Care of DyspneaAssessment of Dyspnea Common Physiological Measurements of

Respiratory Disease Spirometry

FEV1 is a POOR predictor of dyspnea and improvements in dyspnea after bronchodilators do not match improvements of FEV1

(4,5)

Oxygen saturation – with its limitations(6)

NOT a good predictor of the subjective feeling of dyspnea

4. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 8995. Lareau, S.C. et al. (1999).Dyspnea in patients with chronic obstructive pulmonary disease: does dyspnea worsen longitudinally in the presence of declining lung function? Heart & Lung 28 65-736. eMedicine - Pulmonary Function Testing : Article by Raed A Dweik, MD, FACP, FCCP, FRCPC

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Palliative Care of DyspneaTreatment – Non-Pharmacologic

Influenza and pneumonia vaccines Cold facial stimulation (i.e. fan)(6)

Nutrition(7)

Weight gain for malnourished COPD (“pink puffer”) Weight reduction is accompanied by respiratory muscle

weakness. Non-fluid weight gain will help correct this Weight gain is difficult to achieve – poor response to

nutritional supplements

Weight loss for hypercapnic COPD (“blue bloater”)

6. Am Rev Respir Dis. 1987 Jul;136(1):58-61

7. Am Rev Respir Dis. 1990 Aug;142(2):283-8.

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Palliative Care of DyspneaTreatment – Non-Pharmacologic Controlled cough

Deep breath followed by coughing For clearing secretions

Forced expiration – incentive spirometry Good for prevention and treatment of atelectasis Follow with controlled cough to clear secretions

Emotional, spiritual and social counselling These issues are important just as they are in the control of pain Addressing these factors may improve the sensation of dyspnea


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Palliative Care of DyspneaTreatment – Non-Pharmacologic Exercise(8)

Exercise is the best way to strengthen the respiratory muscles

Methods Walking; stair climbing; Upper extremity and shoulder girdle strengthening

These are accessory muscles of breathing Pulmonary rehabilitation Inspiratory resistance breathing

No better than general reconditioning exercise alone in COPD patients


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Palliative Care of DyspneaTreatment – Non-Pharmacologic Controlled Breathing(8)

Purse lipped breathing Improves alveolar ventilation and gas exchange

Slow expiration Useful in overcoming associated panic attacks

Bending forward position Improves diaphragmatic function through

increasing intraabdominal pressure Helps relieve dyspnea


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BiPAP (Bilevel Positive Airways Pressure)Reduces time in ICUReduces need for intubationReduces mortality in COPD exacerbations Improves quality of life in ALS patients (64)

Value of BiPAP in a skilled care setting to “rest” the respiratory muscles is uncertain

8. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 90464. Neurology. 2003 Jul 22;61(2):171-7

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Summary… Immediate treatment

Cold facial stimulation with a fan Controlled cough Forced expiration Pursed lip breathing Slow expiration Bend forward posture

Non-immediate treatment Vaccinations – influenza & pneumococcal Nutritional assessment and treatment Addressing emotional, social and spiritual issues Exercise – walking; stair climbing; shoulder girdle strengthening

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Palliative Care of DyspneaTreatment – Pharmacologic

Bronchodilators Antiinflammatories Oxygen Anxiolytics

Mucolytics Antidepressants Antibiotics

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Palliative Care of DyspneaTreatment – Pharmacologic - Bronchodilators

β2 agonists – in COPD Do not necessarily improve FEV1 or FVC Do improve dyspnea

Anticholinergics Improve FEV1

Reduce dyspnea Phosphodiesterase Inhibitors

Theophylline Leukotriene Antagonists

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Palliative Care of Dyspnea Treatment – Pharmacologic - Bronchodilators

β2 agonists In stable COPD

Short acting levalbuterol (Xopenex®) – In stable COPD patients, no advantage over racemic mixture (albuterol) in prn doses(9)

Long acting β2 agonists salmeterol (Serevent®), formoterol (Foradil®), arformoterol (Brovana®)

9. Chest. 2003 Sep;124(3):844-9

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Anticholinergics Short acting –

Ipratropium (Atrovent®) Long acting

Tiotropium (Spiriva®) Tiotropium (Spiriva®) alone is more effective than long acting β2

agonists alone in COPD patients (10) Tiotropium (Spiriva®) added to a regimen of a long acting β2 agonist

and a corticosteroid significantly improved dyspnea, FEV1 and FVC in COPD patients(11)

Comparing tiotropium alone to fluticasone/salmeterol/tiotropium therapy showed no difference in rates of COPD exacerbation but the combination therapy did improve lung function, quality of life, and hospitalization rates in patients with moderate to severe COPD.(11a)

10. Thorax. 2003 May;58(5):399-40411. Respirology. 2006 Sep;11(5):598-60211a. Annals of Internal Medicine. 2007 April 17; 146( 8):545-555

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Theophylline(12)

A non-selective phosphodiesterase (PDE) inhibitor with antiinflammatory and bronchodilatory effects

Improves dyspnea Improves FEV1 24 hour sustained release preparation may be given once before

bedtime without disturbing sleep (13)

Is now used less because of narrow therapeutic range and risks of toxicity. ? Resurgence due to antiinflammatory effects and lower serum levels (<10mg/L).(35a)

On the horizon, “Cilomilast and roflumilast are selective PDE4 inhibitors that are currently in pre-registration and phase III clinical trials, respectively, for the treatment of COPD (cilomilast and roflumilast) and for treatment of asthma (roflumilast).”(35)

12. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 90313. Chest, Vol 110, 648-65335. Curr Opin Investig Drugs. 2006 May;7(5):412-735a. American Journal of Respiratory and Critical Care Medicine Vol. 167. pp. 813-818, (2003)

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Leukotriene Receptor Antagonists Zafirlukast (Accolade®)–

Has bronchodilation effect in COPD and asthma There is no additive effect when added to inhaled steroids (34)

May reduce pulmonary hypertension in COPD(35)

Montelukast (Singulair®) There is long term benefit in elderly COPD patients with

moderate to severe disease(36)

34. Pulm Pharmacol Ther. 2000;13(6):301-535. Chin Med J (Engl). 2003 Mar;116(3):459-61

36. Respir Med. 2004 Feb;98(2):134-8

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Palliative Care of Dyspnea Treatment – Pharmacologic - Antiinflammatories

Corticosteroids in the treatment of COPD / Dyspnea Short term oral corticosteroids:

Acute exacerbation of COPD Long term inhaled corticosteroids:

Reduces all cause mortality in moderate to severe COPD(14)

Not a first line drug in mild COPD(15)

Long term oral corticosteroids: Only in those not responding to inhaled corticosteroids Sometimes beneficial in hospice patients with malnutrition

Identification of those who will benefit from long term use: Remains controversial One method:

Check FEV1 then give a trial of 20-40 mg prednisone per day for 14 days, then repeat the FEV1. A ≥ 20% increase indicates the patient will benefit from inhaled steroids(16)

14. Thorax. 2005 Dec;60(12):992-7. Epub 2005 Oct 1415. Curr Opin Pulm Med. 2004 Mar;10(2):113-916. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 903

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Palliative Care of Dyspnea Treatment – Pharmacologic - Antiinflammatories

Nebulized IndomethacinMay be of value in reduction of mucus

secretions in bronchiectasis and chronic bronchitis(52,53)

Inhibits production of a proteolytic enzyme, neutrophil elastase

May have long term beneficial effect on progression of bronchiectasis

Dyspnea was improved(52)

52. Am Rev Respir Dis. 1992 Mar;145(3):548-52

53. Eur Respir J. 1995 Sep;8(9):1479-87

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Palliative Care of Dyspnea Treatment – Pharmacologic - Oxygen

Indications Resting O2 saturation ≤ 89% with or without dyspnea Those with dyspnea relieved by O2 despite the resting

oxygen saturation. Studies have shown ↑ survival with use of long

term oxygen, as well as improvement in health related quality of life measures including dyspnea (17,18)

The level of O2 saturation does not correlate with the degree of dyspnea (17)

17. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 90318. Curr Opin Pulm Med. 2004 Mar;10(2):120-7

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Palliative Care of Dyspnea Treatment – Pharmacologic - Oxygen

Beware!Patients on oxygen with high oxygen

saturation and confusion or lethargy may have C02 retention

Treat with discontinuation or reduction in oxygen flow and close observation

Titrate to the flow of oxygen that does not cause the confusion or lethargy

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Palliative Care of Dyspnea Treatment – Pharmacologic - Opioids

Meta-analysis concludes that opioids in modest doses are effective in treating dyspnea(28)

Dose – as little as 2.5 mg of MS q4h(29)

Sustained release morphine reduces dyspnea(27)

(Don’t start on the sustained release forms.)

27. BMJ. 2003 Sep 6;327(7414):523-8

28. Thorax. 2002 Nov;57(11):939-44


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Palliative Care of Dyspnea Treatment – Pharmacologic - Opioids

No clear evidence that inhaled morphine is effective in the relief of dyspnea(30)

30. Eur Respir J. 1997 May;10(5):1079-83

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Palliative Care of Dyspnea Treatment – Pharmacologic - Anxiolytics

Benzodiazepines Scant literature on the use of benzodiazepines in the

treatment of dyspnea but they are commonly used (19,

20)

Opioids are first line anxiolytic drugs for dyspnea secondary to advanced disease of any cause(21)

19. Q J Med. 1980 Winter;49(193):9-20

20. Am J Hosp Palliat Care. 1998 Nov-Dec;15(6):322-30

21. Can Fam Physician. 2003 Dec;49:1611-6.

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Palliative Care of Dyspnea Treatment – Pharmacologic - Anxiolytics

Buspirone (BuSpar®) Conflicting reports of its effect on dyspnea(22,23)

Concerns about respiratory depression in COPD patients receiving anxiolytics is unfounded.(24)

Anxiolytics can be beneficial in some patients with dyspnea, even those without appreciable anxiety.(24)

22. Respiration. 1993;60(4):216-2023. Chest. 1993 Mar;103(3):800-424. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 904

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Palliative Care of Dyspnea Treatment – Pharmacologic - Mucolytics

N-Acetylcysteine (Mucomyst®) by mouth or inhalation will help patients with excessive or viscous mucous clear these secretionsEffect on dyspnea has not been studiedEvidence is conflicting as to its reduction of

COPD exacerbations(31,32)

31. Lancet. 2005 Apr 30-May 6;365(9470):1552-60

32. Eur Respir J. 2003 May;21(5):795-8

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Palliative Care of Dyspnea Treatment – Pharmacologic - Mucolytics

Additional agents that may assist in mucolysis and expectoration of thick sputum: Normal or hypertonic saline nebulizations Inhaled mannitol powder (66)

Inhaled atropine Corticosteroids β2 agonists Indomethacin Theophylline

Glycerol guaiacolate Of limited value

33. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 90466. Respirology. 2005 Jan;10(1):46-56

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Palliative Care of Dyspnea Treatment – Pharmacologic - Antidepressants

SSRIs; Tricyclics – In depressed patients with endstage lung disease Beneficial for anxiety Benefit for dyspnea is not conclusive (25,26)

25. Psychosomatics. 1998 Jan-Feb;39(1):24-9.


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Palliative Care of Dyspnea Treatment – Pharmacologic - Antibiotics

Treatment of Exacerbations Antibiotics

Fluoroquinolones (37,38)

Amoxicillin almost as effective and cheaper(39)

Short acting β2 agonists → long acting

Short acting anticholinergics → long acting Oral prednisone → Inhaled corticosteroid

37. Clin Microbiol Infect. 2006 May;12 Suppl 3:42-54

38. Chest. 2004 Mar;125(3):953-64

39. American Family Physician Vol. 70/No. 4 (August 15, 2004)

Palliative Treatment of Cough

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Palliative Care of Cough Assessment

Many patients will not want the usual diagnostic tests

A thorough history and physical examination is often our best and only tool for assessing the cause of the cough

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Palliative Care of Cough Assessment

Causes Acute infections Chronic Infections Airways Disease Cardiovascular Parenchymal Disease Irritant Recurrent Aspiration Drug Induced Pleural Disease Vocal Cord Disease

Examples Pneumonia; Acute Bronchitis Chronic bronchitis; Bronchiectasis COPD; Asthma LV failure; pulmonary edema Interstitial Fibrosis GERD; Foreign body Stroke; Motor neuron disease ACE Inhibitors; inhaled drugs Pneumothorax; pleural effusion Paralysis; nodules on cords


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Palliative Care of Cough Treatment of the Underlying Cause

Acute and chronic infections Antibiotics

Asthma and COPD Bronchodilators and anti-

inflammatories Left ventricular failure

Diuretics, ACE inhibitors, ± β- blockers

Recurrent aspiration Postioning of patient;

swallowing evaluation → alter food consistency

Drug induced (ACE inhibitors) Discontinue drug

Pleural disease Correct pneumothorax; drain

pleural effusion Vocal cord dysfunction

ENT evaluation and treatment GERD

PPIs; metoclopramide; positioning of patient

Post-nasal drip Decongestants; antihistamines


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Palliative Care of CoughTreatment – Protussive and Antitussive

Protussive TreatmentsMeasures to improve cough effectiveness

and secretion clearance Antitussive Treatments

Measures to prevent or eliminate cough


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Palliative Care of CoughTreatment – Protussive Treatments

Measures to make cough more effective(40)

Adequate hydration – po fluids; steam inhalations; saline nebulizations

Physiotherapy – only in select patients with COPD and bronchiectasis (41)

Forced exhalations Airways vibrations Postural drainage Assisted cough techniques


41. Chron Respir Dis. 2006;3(2):83-91

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Measures to make cough more effective(40)

Pharyngeal suctioningMini-tracheostomyFor thick, excessive, infected sputum

Steroids Antibiotics Inhaled mannitol powder or hypertonic saline (42,43)


42. Cochrane Database Syst Rev. 2005 Jul 20;(3):CD001506

43. J Aerosol Med. 2002 Fall;15(3):331-41

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Increase of secretion clearance (40,44)

Liquification of secretions N-acetylcysteine Recombinant human DNAse Arginine – not as effective as N-acetylcysteine Uridine-5'-triphosphate – useful for getting sputum samples

from mild chronic bronchitics (67)

Bronchodilators β2 – agonists (albuterol)

40. Derek, D. et al. (2004). Oxford Textbook of Palliative Medicine, pp. 90044. Expert Opin Pharmacother. 2004 Feb;5(2):369-77 67. Chest. 2002 Dec;122(6):2021-9

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Palliative Care of CoughTreatment – Antitussive Treatments

Antitussive Treatment Used when cough is not reversibleUsed primarily for dry non-productive cough

Opioids Oral local anesthetics Nebulized local

anesthetics

Other antitussive agents Antimuscarinics


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ACP Medicine 2006

Antitussive Treatment of Cough

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Opioids Morphine is the strongest antitussive (47)

Useful especially in the terminal patient

Codeine is used widely In its OTC form codeine has no more antitussive effect than

the demulcent vehicle (47)

Dextromethorphan – an opioid derivative No analgesic effect in antitussive doses As effective as codeine for cough suppression

45. Chest. 2006 Jan;129(1 Suppl):284S-286S

46. Pulm Pharmacol Ther. 2004;17(6):459-62

47. Thorax. 2004 May;59(5):438-40



47. Thorax. 2004 May;59(5):438-40

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Oral Local Anesthetics Benzonatate (Tessalon Perles ®)

Peripheral acting / opiates largely central acting Often effective in opiate resistant cough (47)

Levodropropizine – not available in USA Widely used in Europe Peripheral acting and useful in cancer related cough (47)



47. Thorax. 2004 May;59(5):438-40

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Nebulized Local AnestheticsRisk is aspiration 2-4 hours after a treatment

Patient should not eat or drink for 1 hour after RxNebulized lidocaine is effective in reduction of

cough (48, 49) (5mg/kg in normal saline)Bupivacaine and Lidocaine have been

associated with bronchoconstriction in patients with reactive airways. Consider giving with salmeterol (50)

48. Am J Emerg Med. 2001 May;19(3):206-7

49. Emerg Med J. 2005 Jun;22(6):429-32

50. Canadian Family Physician. May 2002

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Other Antitussive Agents If cause is bronchospasm, inflammation, or

tumor… Theophylline β2 –agonists Anti-inflammatories

Steroids Sodium cromoglycate


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Other Antitussive Agents OTC Marketed as Antitussive but Not Proven

Effective Pseudoephedrine Dexbrompheniramine Guaifenesin


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Antimuscarinics Ipratropium bromide

Good in chronic bronchitis Reduces secretions without reduction in mucus viscosity

Hyoscine .2-.4mg sc prn or Glycopyrronium bromide .2-.4 mg IM prn

Good for the death rattle and associated cough May cause ataxia and hallucinations in the elderly


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Antimuscarinics (68)

Ophthalmic Atropine 1% drops Give sublingually or po

Scopolamine Patch ® Hyoscine in a patch Not effective for about 12 hours

68. AAHPC Fast Fact and Concept #109: Death rattle and oral secretions

Palliative Care of Respiratory Infections

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Treatment – Establishing Goals

Above all - goals must be discussed and formulated with the patient and familyThe patient or POA may ultimately decide

against antibiotic therapy If antibiotics are not chosen as a treatment,

symptomatic treatment of fever, dyspnea and cough should be the plan

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Treatment – Antibiotic Selection

COPD with FEV1 < 50% (Most hospice patients with end stage lung disease) exacerbations should be treated with a quinolone

COPD with FEV1 > 50% use ampicillin, tetracycline or trimethoprim/sulfa

51. ACP Medicine Chapter 14:Respiratory Medicine: III Chronic Obstructive Disease of the Lung

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Bronchiectasis and Cystic FibrosisCoverage of anaerobic bacteria and

pseudomonas are importantAntibiotics should be given in high doses,

sometimes rotated and for 3-4 week courses Ciprofloxacin Metronidazole Augmentin


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Bronchiectasis and Cystic FibrosisNebulized antibiotics

Gentamicin (300 mg bid) Tobramycin (300 mg bid)


Palliative Care of Hemoptysis

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Palliative Treatment of HemoptysisAssessment

Majority of cases are mild to moderate <20% are massive (> 500 cc per day) Most common causes

Infection ~ 80% TB Abscesses Bronchiectasis

Malignancy ~ 20%


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Palliative Treatment of HemoptysisAssessment

History and Physical Examination Examination of the sputum

Presence of food particles Hematemesis T/E fistula

Purulent sputum Infection

Laboratory and X-Ray Studies Chest x-ray CT with contrast Bronchial artery or pulmonary artery arteriogram


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Palliative Treatment of HemoptysisTreatment - Anticipatory

Anticipation - If resuscitation is or is not the goal Education of patient, family and caregivers Goals must be established Dark colored towels Morphine Anxiolytics

Lorazepam Diazepam Midazolam


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Palliative Treatment of HemoptysisTreatment of Massive Hemoptysis

If resuscitation is the goal… Patent airway + oxygen

Intubation and ventilation if needed Position

Lateral decubitus Head down Bleeding lung down

Determine the site of bleeding Avoid excessive manipulation Cough suppression (codeine 30-60 mg po q6h)


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Palliative Treatment of HemoptysisTreatment of Massive Hemoptysis – Goal Resuscitation

If resuscitation is the goal…(continued) Immediate bronchoscopy

If source identified, lavage with iced saline and adrenalin (10cc of 1:10,000 dilution)

Topical thrombin Balloon catheter tamponade Vasopressin Bronchial stent placement

If source not found CT with contrast Bronchial or pulmonary angiography


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Palliative Treatment of HemoptysisTreatment of Massive Hemoptysis – Goal Resuscitation

If resuscitation is the goal…(continued) Bronchial arterial embolization

Successful in 70-100% of cases Especially good in those with dilated bronchial arteries

(bronchiectasis) Complications

Rebleeding - common Anterior spinal artery infarction and paraplegia – 5% Ischemic necrosis of the bronchus Arterial dissection

Surgical resection of the bleeding tissue


Palliative Care of Pulmonary

Hypertension and Cor Pulmonale

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Palliative Care of Pulmonary Hypertension and Cor Pulmonale

Clinical ManifestationsDependent edemaRight ventricular hypertrophyRight ventricular dilatation

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Etiology and Pathophysiology Most chronic pulmonary diseases can ultimately cause

pulmonary hypertension and cor pulmonale Pathophysiology (56)

COPD – severe pulmonary hypertension only in a small percentage of COPD patients

Hypoxia → constriction of pulmonary arterial vasculature – However…

Poor correlation between arterial p02 and pulmonary artery pressure in COPD

Chronic inflammation Repeated hyperinflation of the lungs Cigarette smoking

Pulmonary Emboli and Pulmonary Fibrosis Obstruction of the pulmonary vasculature

Primary Pulmonary Hypertension Etiology unknown


56. The Proceedings of the American Thoracic Society 2:20-22 (2005)

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Pathophysiology

Pathophysiology of Edema in COPD Exercise → ↑ right ventricular end diastolic pressure → ↑ stretching of the right atrium → ↑ sympathetic tone → ↑ renin angiotensin aldosterone production → ↑ renal distal tubular retention of water and sodium → ↑ edema (56)

C02 retention → ↑ renal proximal tubular sodium retention → ↑ edema

56. The Proceedings of the American Thoracic Society 2:20-22 (2005)

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Treatment

Treat the underlying pulmonary disease Oxygen

Long term oxygen therapy in COPD Only produces a small decrease in pulmonary

artery pressure

In acute exacerbations of COPD Delivered with BiPAP , reduces pulmonary artery

pressure


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Treatment

β2 – agonists Reduce pulmonary artery pressure Increase right ventricular ejection fraction

Diuretics – the primary treatment of edema Edema is secondary to –

Hypoxic renal dysfunction Excessive release of pituitary hormones Not caused by right heart failure Caution: hypochloremic alkalosis → ↓ ventilation and C02 retention

Calcium Channel Blockers Only short term effect on pulmonary hypertension May produce ventilation-perfusion mismatch and worsen oxygen

saturation May produce systemic hypotension


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Treatment

ACE InhibitorsCause systemic hypotensionNo improvement in pulmonary vascular

resistance, gas exchange or ventilatory parameters


Palliative Care of Primary Pulmonary

Hypertension

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Palliative Care of Primary Pulmonary Hypertension

Treatment

Endothelin antagonists Bosentan (Tracleer®) (57) –

Oral endothelin receptor blocker Mild improvement in dyspnea 36 meter increase in 6 minute walking distance

Approved for use in pulmonary arterial hypertension May be used in patients with COPD and severe pulmonary

hypertension, but these patients are difficult to identify in an end of life setting. Clinical trials are ongoing.(58)

Caution – Numerous drug interactions

57. U.S. Pharmacist Vol. No: 30:05 Posted: 5/16/05

58. Curr Opin Pulm Med. 2003 Mar;9(2):139-43

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Treatment

Prostacyclin Analogs Epoprostenol (Flolan®) and Treprostinil

(Remodulin®) Improves exercise tolerance Must be given as a continuous infusion

Iloprost (Ventavis®) Inhaled Improves exercise tolerance

Beraprost – Not available in USA Inhaled Improvement in symptoms


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Treatment

Phosphodiesterase V Inhibitors Sildenafil (Viagra®)

Improves exercise tolerance Other phosphodiesterase V inhibitors are being evaluated

Tadalafil (Cialis®) – only once daily dosing

Anticoagulants Warfarin –

To prevent microthrombi formation in pulmonary circulation To prevent thrombophlebitis in the lower extremities Keep INR at 1.5 - 2.5 Reduces progression of the disease and those symptoms that will

worsen with progression of the disease


Palliative Care of Pulmonary Fibrosis

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Palliative Care of Pulmonary FibrosisTreatment

Pneumoconioses – Most Common Cause Idiopathic Pulmonary Fibrosis

Treatment with interferon gamma-1b Conflicting evidence of effectiveness (59,60) Metaanalysis suggests it does prolong life ( 61)

In general pulmonary fibrosis patients do not retain CO2High flows of oxygen may be used

59. Mayo Clin Proc. 2003 Sep;78(9):1082-7

60. Ann Pharmacother. 2005 Oct;39(10):1678-86. Epub 2005 Sep 13

61. Chest. 2005 Jul;128(1):203-6

Palliative Care of Pulmonary Emboli

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Most deaths from PE are a result of inadequate prophylaxis

Which end of life patients should receive prophylaxis?End stage cardiopulmonary patientsCancer patients with prothrombotic tumorsMinimal data on prophylactic treatment VTE in

end of life outpatients

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Current VTE Prophylaxis Hydration Not crossing legs Traditional stockings probably not effective Encouraging mobility Drug therapy

Low molecular weight heparin is preferred No prothrombin time needed Once daily injection

Warfarin INR should be 2-3 Difficult to regulate in the end of life patient because of other

drug therapies and fluctuating liver functions

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On the horizon…Ximelagatran

Oral medication As effective as low dose warfarin with enoxaparin Not yet approved because of potential

hepatotoxicity and ↑ incidence of coronary events Idraparinux

Once weekly injection In phase III trials

62. Semin Vasc Med. 2005 Aug;5(3):276-84

Palliative Care of Stridor

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Palliative Treatment of Stridor Causes

Infection – epiglottitis, diphtheria Tumor Aspirated objects

Thick sputum Blood clots Foreign bodies Dislodged tumor particles

Crohn's Disease – rare – resistant to dexamethasone (54)

Diffuse Idiopathic Skeletal Hyperostosis (DISH) Forestier’s Disease – from large cervical spine osteophytes compressing the trachea (55)

Achalasia – megaesophagus compression of trachea (56)

Myasthenia gravis – presenting with exertional stridor (57)

Psychogenic stridor (58)

Drug hypersensitivity – amphotericin (60)


54. Chest. 2006 Aug;130(2):579-81

55. J Laryngol Otol. 1999 Jan;113(1):65-7

56. Eur J Gastroenterol Hepatol. 1997 Nov;9(11):1125-8

57. Thorax. 1996 Jan;51(1):108-9

59. Gen Hosp Psychiatry. 1994 May;16(3):213-23

60. Ann Allergy Asthma Immunol. 2003 Nov;91(5):460-6

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Palliative Treatment of StridorTreatment – Non-pharmacologic and Pharmacologic

Treatment Postural manipulation Heimlich maneuver – for acute onset stridor Physiotherapy Bronchoscopy or laryngoscopy Tracheostomy Stents Medications

Dexamethasone 16 mg po qd for edema or inflammation Oxygen / Helium 4:1 Mixture Infliximab – for Crohn’s Disease (54)


54. Chest. 2006 Aug;130(2):579-81

Palliative Care of Neuromuscular and

Restrictive Pulmonary Disorders

Main Menu…

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Palliative Care of Neuromuscular Disorders and Restrictive Pulmonary Disease

Hypercapnia and sleep disorders are very common in neuromuscular disorders

MS and ALS – bulbar disorders result in dysphagia and frequent aspiration and pneumonia

Long term anticoagulation is often prescribed for thromboembolic prophylaxis

Glossopharyngeal breathing is a good technique to improve ventilation in patients with high cervical injuries

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Palliative Care of Neuromuscular Disorders and Restrictive Pulmonary Disease

Non-invasive mechanical ventilationRocking bedsAbdominal pneumatic beltsNegative pressure ventilatorsNasal CPAP

Palliative Care of Bronchiectasis and

Cystic Fibrosis

Main Menu…

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Palliative Care of Bronchiectasis and Pulmonary Fibrosis

Nebulized Deoxyribonuclease (DNAse)Hydrolysis of extranuclear DNA that

accumulates with neutrophil degradation in infected airways

Useful in cystic fibrosis and to a lesser extent in bronchiectasis


Palliative Care of α-1 Antitrypsin Deficiency

Main Menu…

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Palliative Care of α-1 Antitrypsin Deficiency

“AAT replacement therapy is for enzyme deficient patients with impaired FEV-1 (35-65% of predicted value), who have quit smoking and are on optimal medical therapy but continue to show a rapid decline in FEV-1 after a period of observation of at least 18 months.”(63)

63. Treat Respir Med. 2005;4(1):1-8

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Happy Trails from Lea County, NM

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Links - 1 Spiriva Cost Spiriva vs. Serevent Respiratory. 2006 Sep;11(5):598-602 Is a long-acting inhaled bronchodilator the first agent to use in stable chronic obstructive pulmonary disease? Emerging drugs for the treatment of chronic obstructive pulmonary disease. Pharmacologic treatment of chronic obstructive pulmonary disease: past, present, and future. Names of leukotriene related drugs Effect of Intravenous Magnesium Sulfate on Chronic Obstructive Pulmonary Disease Addition of anticholinergic solution prolongs bronchodilator effect of beta 2 agonists Comparison of the bronchodilating effect of salmeterol and zafirlukast in combination Retrospective evaluation of systemic corticosteroids for the management of acute exacerbations Efficacy and safety of inhaled corticosteroids in patients with COPD Roflumilast for the treatment of chronic obstructive pulmonary disease Corticosteroids and Chronic Obstructive Pulmonary Disease Theophylline in chronic obstructive pulmonary disease: new horizons. Corticosteroid resistance in chronic obstructive pulmonary disease: inactivation of histone deacetylase. Inhaled corticosteroids and mortality in chronic obstructive pulmonary disease. Inhaled corticosteroids in chronic obstructive pulmonary disease: is there a long-term benefit? Health-related quality of life in individuals with chronic obstructive pulmonary disease. Improving health-related quality of life in chronic obstructive pulmonary disease.

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Links - 2 Diazepam in the treatment of dyspnea in the 'Pink Puffer' syndrome. The palliation of dyspnea in terminal disease More research needed An approach to dyspnea in advanced disease. Opioids are first line drugs Buspirone effect on breathlessness and exercise performance in patients with chronic obstructive pulmonary dise

ase. Effects of buspirone on anxiety levels and exercise tolerance in patients with chronic airflow obstruction and mild

anxiety. Sertraline effects on dyspnea in patients with obstructive airways disease Randomized, double blind, placebo controlled crossover trial of sustained release morphine for the management

of refractory dyspnea A systematic review of the use of opioids in the management of dyspnea Disabling dyspnea in patients with advanced disease: lack of effect of nebulized morphine Roflumilast for the treatment of chronic obstructive pulmonary disease Effects of N-acetylcysteine on outcomes in chronic obstructive pulmonary disease (Bronchitis Randomized on NA

C Cost-Utility Study, BRONCUS): a randomized placebo-controlled trial N-acetylcysteine reduces the risk of re-hospitalization among patients with chronic obstructive pulmonary disease Short-term effects of montelukast in stable patients with moderate to severe COPD Therapeutic responses in asthma and COPD. Bronchodilators Review of effects of PDE4 Inhibitors and LRAs Long-term montelukast therapy in moderate to severe COPD--a preliminary observation Current and future pharmacologic therapy of exacerbations in chronic obstructive pulmonary disease and asthma. Should patients with acute exacerbation of chronic bronchitis be treated with antibiotics? Advantages of the use of

fluoroquinolones.

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Links - 3 Short-term and long-term outcomes of moxifloxacin compared to standard antibiotic treatment in acute exacerbati

ons of chronic bronchitis Moxifloxacin vs. Alternatives for Chronic Bronchitis Palliative Home Care for Advanced Lung Disease Is there a role for airway clearance techniques in chronic obstructive pulmonary disease? Nebulized hypertonic saline for cystic fibrosis Osmotic stimuli increase clearance of mucus in patients with mucociliary dysfunction Potential future therapies for the management of cough: ACCP evidence-based clinical practice guidelines Potential new cough therapies. Current and future drugs for the treatment of chronic cough Comparison of lidocaine and bronchodilator inhalation treatments for cough suppression in patients with chronic o

bstructive pulmonary disease. Lidocaine inhalation for cough suppression Effect of indomethacin on bronchorrhea in patients with chronic bronchitis, diffuse panbronchiolitis, or bronchiecta

sis In vivo study of indomethacin in bronchiectasis: effect on neutrophil function and lung secretion Stridor in Crohn disease and the use of infliximab

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Links - 4 An unusual case of stridor due to osteophytes of the cervical spine: (Forestier's disease). Myasthenia gravis presenting with stridor Achalasia presenting as acute stridor Psychogenic stridor Amphotericin-induced stridor: a review of stridor, amphotericin preparations, and their immunoregulatory effects Use of the Dumon Y-stent in the management of malignant disease involving the carina: a retrospective review of

86 patients Thoracic embolotherapy for life-threatening hemoptysis: a pulmonologists perspective Bronchial and non bronchial systemic artery embolization for life-threatening hemoptysis: a comprehensive review Pulmonary hypertension and right heart failure in chronic obstructive pulmonary disease Advances in the treatment of secondary pulmonary hypertension Overview of treprostinil sodium for the treatment of pulmonary arterial hypertension Sildenafil for pulmonary hypertension Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension:

comparison with inhaled nitric oxide Treatment of Pulmonary Hypertension Interferon gamma-1b as therapy for idiopathic pulmonary fibrosis. An intra-patient analysis. Interferon gamma-1b therapy for advanced idiopathic pulmonary fibrosis Interferon gamma-1b in the treatment of idiopathic pulmonary fibrosis Interferon-gamma1b therapy in idiopathic pulmonary fibrosis: a metaanalysis Emphysema in alpha1-antitrypsin deficiency: does replacement therapy affect outcome? Ximelagatran vs low-molecular-weight heparin and warfarin for the treatment of deep vein thrombosis: a

randomized trial.

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Links - 5 Is long-term low-molecular-weight heparin acceptable to palliative care patients in the treatment of cancer related

venous thromboembolism? A qualitative study. Acceptability of low molecular weight heparin thromboprophylaxis for inpatients receiving palliative care:

qualitative study. Treating patients with venous thromboembolism: initial strategies and long-term secondary prevention. Inhaled mannitol for the treatment of mucociliary dysfunction in patients with bronchiectasis: effect on lung

function, health status and sputum. Improved sputum expectoration following a single dose of INS316 in patients with chronic bronchitis.

palliative care of respiratory symptoms james s. botts, md, facp southwest area medical director...

Documents

pulmonary edema

language of dyspnea

interstitial disease

underlying disease

cms lcd pulmonary guidelines

botts slide

language patients

different disease states