FACULTY OBJECTIVES 12/4/2017. Women > Men in recent years END-OF-LIFE CARE IN LUNG DISEASE.

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1 END-OF-LIFE CARE IN LUNG DISEASE STEPHEN A. LEEDY, MD MA HMDC FAAHPM FACULTY STEPHEN A. LEEDY, MD MA HMDC FAAHPM PRESIDENT & CEO Helping Hospices Excel OBJECTIVES Leading Causes of Death in Americans 65 and Older IMPACT Acknowledge the impact of end-stage lung on society ILLNESS Describe the pathophysiology and diagnosis of end-stage lung disease MANAGEMENT Describe the role of hospice and palliative care in managing COPD PROGNOSTICATION Discuss the challenges faced and strategies used in prognosticating in advanced COPD Cause of death Percentage Heart disease 44% Cancer 29% Stroke 11% COPD 8% Influenza and pneumonia 4% Diabetes 4% (Federal Forum on Aging-Related Statistics, 2000) 4 TH LEADING CAUSE OF DEATH IN US LIKELY TO BECOME 3 RD LEADING CAUSE OF DEATH GLOBALLY BY 2020 SMOKING IS THE PRIMARY RISK FACTOR AIR POLLUTION, SECOND-HAND SMOKE, HISTORY OF CHILDHOOD RESPIRATORY INFECTIONS, AND HEREDITY ARE OTHER CAUSES 12 MILLION AMERICANS WITH DIAGNOSED COPD Female smokers are >13 times more likely to die of COPD than female non-smokers FEMALE SMOKERS Women > Men in recent years 20 MILLION MORE WITH EARLY EVIDENCE 250,000 ADVANCED COPD DEATHS A YEAR SOURCE: AMERICAN LUNG ASSOCIATION FACT SHEET AUGUST 2006 EPIDEMIOLOGY OF COPD 1

2 CLASSIC DEFINITION OF COPD COPD IS A GROUP OF RESPIRATORY DISORDERS CHARACTERIZED BY CHRONIC, RECURRENT OBSTRUCTION OF AIRFLOW IN THE PULMONARY AIRWAYS NOT FULLY REVERSIBLE WITH INHALED BRONCHODILATORS EMPHYSEMA CHRONIC BRONCHITIS NEW DEFINITION OF COPD COPD IS A PREVENTABLE AND TREATABLE DISEASE STATE CHARACTERIZED BY AIRFLOW LIMITATION THAT IS NOT FULLY REVERSIBLE THE AIRFLOW LIMITATION IS USUALLY PROGRESSIVE AND IS ASSOCIATED WITH AN ABNORMAL INFLAMMATORY RESPONSE OF THE LUNGS TO NOXIOUS PARTICLES OR GASES, PRIMARILY CAUSED BY CIGARETTE SMOKING ALTHOUGH COPD AFFECTS THE LUNGS, IT ALSO PRODUCES SIGNIFICANT SYSTEMIC CONSEQUENCES RESPIRATORY ANATOMY UPPER RESPIRATORY TRACT MOUTH, NOSE, THROAT (PHARYNX), LARYNX, TRACHEA LOWER RESPIRATORY TRACT LUNGS, BRONCHI, ALVEOLI MEDULLA OBLONGATA CONTROLS INSPIRATION AND EXPIRATION RESPIRATORY ANATOMY DIAGNOSING COPD Exposure to risk factors (smoking) Clinical signs and symptoms Spirometry FEV1/FVC < 70% of predicted FEV1 < 80% of predicted 2

3 SPIROMETRY FEV 1 Forced expired volume in the first second FVC Total volume of air that can be exhaled from maximal inhalation to maximal exhalation FEV 1 /FVC% - The ratio of FEV 1 to FVC, expressed as a percentage COPD Classification Based on Spirometry Severity Post-bronchodilator FEV 1 /FVC Post-bronchodilator FEV 1 % predicted At risk >0.7 >80 Mild COPD <0.7 >80 Moderate COPD < Severe COPD < Very Severe COPD <0.7 <30 SPIROMETR Y IS NOT TO SUBSTITUTE FOR CLINICAL JUDGMENT IN THE EVALUATION OF THE SEVERITY OF DISEASE IN INDIVIDUAL PATIENTS CHRONIC BRONCHITIS DIAGNOSIS Mucus-producing cough most days of the month, 3 months of the year for 2 consecutive years (ALA) CHARACTERISTICS OF CHRONIC BRONCHITIS Cough with phlegm Shortness of breath Exercise intolerance Expiratory phase of respiration long Wheezes and crackles on auscultation Hypoxemia, hypercapnia Pulmonary hypertension Right heart failure with peripheral edema EMPHYSEMA DIAGNOSIS Careful History and Physical Examination Pulmonary Function Tests Forced Expiratory Volumes Chest X-rays Arterial Blood Gases 3

4 CHARACTERISTICS OF EMPHYSEMA Dyspnea, slowly progressive Hypoxia, hypercapnia Use accessory muscles Weight loss Sputum production in morning, scant Minimal cough Loss of lung elasticity Destruction of alveoli walls and capillary beds COPD TYPES PINK PUFFER BLUE BLOATER PINK PUFFER: EMPHYSEMA OVER VENTILATE TO MAINTAIN RELATIVELY NORMAL ABG S UNTIL LATE IN DISEASE RED FACE BLUE BLOATER: CHRONIC BRONCHITIS BRONCHIAL SECRETIONS AND AIRWAY OBSTRUCTION CAUSE POOR VENTILATION AND PERFUSION UNABLE TO COMPENSATE LEADING TO HYPOXIA AND CYANOSIS CLUBBING OF FINGERS CIRCUMORAL CYANOSIS BARREL CHESTED COPD patients often have barrel-shaped chests Why? These patients have a loss of lung elasticity. Airways collapse during expiration because pressure in lung tissue exceeds airway pressure. Air gets trapped causing increase in anteroposterior dimensions of the chest. Simply: Their lungs are chronically over-inflated with air PURSED LIP BREATHING COPD patients purse their lips to breath Why? Pursing your lips increases the resistance to the outflow of air. It helps to prevent airway collapse by increasing pressure. Simply: Pucker up. Try to blow air out. Feel the resistance? 4

5 GOLD STRATEGY HOSPICE FOR COPD PATIENTS UNDERUTILIZED MISUNDERSTOOD CLINICALLY COMPLEX COMPARED TO CANCER, COPD IS MORE LIFE THREATENING AND SYMPTOM MANAGEMENT IS MORE CHALLENGING DYSPNEA 94% of COPD patients experience dyspnea in the last year of life COPD patients are more likely to die with poor control of dyspnea than lung cancer patients Short-Acting Beta-2-Agonists ALBUTEROL (VENTOLIN, PROVENTIL) METAPROTERENOL (ALUPENT) LEVALBUTEROL (XOPENEX) LESS BETA-1 ACTIVITY SO (SUPPOSEDLY) LESS SIDE EFFECTS PIRBUTEROL (MAXAIR) ALL ARE 2 PUFFS 3 TO 4 TIMES A DAY SIDE EFFECTS: TREMOR, TACHYCARDIA Long-Acting Beta-2-Agonists SALMETEROL (SEREVENT) 2 PUFFS TWICE A DAY FORMOTEROL (FORADIL) 1 CAP INHALED TWICE A DAY SLOWER ONSET OF ACTION NOT FOR ACUTE SYMPTOMS LESS SIDE EFFECTS DUE TO LONGER ONSET OF ACTION ANTICHOLINERGIC AGENTS IPRATROPIUM (ATROVENT) 2 PUFFS 4 TIMES A DAY TIOTROPIUM (SPIRIVA) 1 CAP INHALED ONCE A DAY IPRATROPIUM/ALBUTEROL (COMBIVENT) 2 PUFFS 4 TIMES A DAY MINIMAL CARDIAC STIMULATORY EFFECTS SIDE EFFECTS: DRY MOUTH, DIZZINESS 5

6 OTHER NEBULIZED MEDICATIONS MORPHINE CONFLICTING EVIDENCE OF BENEFIT OVER SALINE COYNE PJ, VISWANATHAN R, SMITH TJ. NEBULIZED FENTANYL CITRATE IMPROVES PATIENTS' PERCEPTION OF BREATHING, RESPIRATORY RATE, AND OXYGEN SATURATION IN DYSPNEA. J PAIN SYMPTOM MANAGE. 2002;23(2): MAY WORSEN BRONCHOSPASM VIA HISTAMINE RELEASE FROM PULMONARY MAST CELLS THEOPHYLLINE USED LESS OFTEN THESE DAYS MANY DRUG INTERACTIONS A ND ADVERSE EFFECTS FUROSEMIDE SHIMOYAMA N, SHIMOYAMA M. NEBULIZED FUROSEMIDE AS A NOVEL TREATMENT FOR DYSPNEA IN TERMINAL CANCER PATIENTS. J PAIN SYMPTOM MANAGE. 2002;23(1):73-6. INHALED ORAL PARENTERAL GEL STEROIDS DECREASE INFLAMMATION IMPROVE FEELING OF WELL BEING OPIOIDS OPIOIDS RELIEVE DYSPNEA BY: ALTERING THE PERCEPTION OF DYSPNEA DECREASING VENTILATORY DRIVE TO BOTH HYPOXIA AND HYPERCAPNIA REDUCING OXYGEN CONSUMPTION AT REST AND DURING EXERCISE BRUERA E, MACMILLAN K, PITHER J ET AL. THE EFFECTS OF MORPHINE ON DYSPNEA OF TERMINAL CANCER PATIENTS. J PAIN AND SYMPTOM MANAGEMENT 1990; 5: OPIOIDS CONTROL COUGH LOWER DOSES THAN NEEDED TO CONTROL PAIN BENZOS BENZODIAZEPINES USEFUL TO RELIEVE REFRACTORY ANXIETY AND DYSPNEA AFTER BRONCHODILATORS AND OPIOIDS ARE MAXIMIZED NOT HELPFUL WITH DYSPNEA IF ANXIETY AND PANIC ARE ABSENT OTHER INTERVENTIONS SMOKING CESSATION ONLY INTERVENTION THAT SLOWS LOSS OF LUNG FUNCTION INFLUENZA VACCINE YEARLY PNEUMOCOCCAL VACCINE 6

7 FAN BEDSIDE FAN DIRECTED AT PATIENT S FACE CAN HELP ALLEVIATE DYSPNEA ROUSSEAU PC. NON-PAIN SYMPTOM MANAGEMENT IN TERMINAL CARE. CLIN GERIATRICS MED 1996; 12: THOUGHT TO BE MEDIATED BY STIMULATION OF THE THERMAL AND MECHANICAL RECEPTORS OF THE TRIGEMINAL NERVE IN THE CHEEK AND NASOPHARYNX ENCK RE. THE MEDICAL CARE OF THE TERMINALLY ILL PATIENTS. BALTIMORE: JOHNS HOPKINS UNIVERSITY PRESS, 1994 DUDGEON DJ, ROSENTHAL S. MANAGEMENT OF DYSPNEA AND COUGH IN PATIENTS WITH CANCER. HEMATOL ONCOL CLIN NORTH AM 1996; 10: OXYGEN Supplemental oxygen may not relieve dyspnea Patients can have dyspnea with normal oxygen saturation Masks may be constricting and frightening, especially to confused patients SECRETION MANAGEMENT ANTIBIOTICS Atropine ophthalmic drops sublingually (0.5mg/drop, 2 drops sublingually every 2 hours prn) Hyoscyamine liquid (0.125mg sublingually every 4 hours prn) Scopolamine patches (1.5mg transdermally every 3 days) Glycopyrrolate ( mg orally or parenterally 3 times a day) Not usually indicated at endof-life ( terminal pneumonia ) May have palliative purpose earlier in disease to treat bacterial pneumonia or bronchitis DIURETICS VENTILATION: INVASIVE & NON-INVASIVE Patients often discharged on CPAP after being weaned from ventilator Patients may have fluid overload from concomitant CHF 7

8 NICOTINE NICOTINE CRAVING CAN BE AN ISSUE IN DYING SMOKERS CONSIDER NICOTINE REPLACEMENT FOR PALLIATION CLONIDINE PATCH CAN DECREASE CRAVING AND SERVES AS AN ADJUVANT ANALGESIC PROGNOSIS WHY IS AN ACCURATE PROGNOSIS IMPORTANT? STEDMAN S DEFINITION: THE FORETELLING OF THE PROBABLE COURSE OF A DISEASE; A FORECAST OF THE OUTCOME OF A DISEASE DERIVATION OF WORD: PRO = BEFORE + GIGNOSKO = TO KNOW HELPS PATIENTS AND FAMILIES PLAN THEIR TIME HELPS PHYSICIANS AND PROGRAMS ANTICIPATE CARE NEEDS QUALIFIES PATIENTS FOR THE MEDICARE HOSPICE BENEFIT 8

9 SO HOW GOOD IS OUR CRYSTAL BALL? DON'T QUIT YOUR DAY JOBS! Systematic review of 1563 predictions from 8 studies: in 27% of terminal cancer cases, survival was over-estimated by 4 weeks, and only correct to within 1 week in 25% of cases. Glare P, et al. A systematic review of physicians survival predictions in terminally ill cancer patients. BMJ. 2003;327: Prospective cohort study: Only 20% of prognostic predictions are correct. Survival was over-estimated by a factor of up to 5.3. Lamont & Christakis. Physician factors in the timing of cancer patient referral to hospice palliative care. Cancer. 2002;94(10): Numerous studies document that physicians are inaccurate when determining prognosis and frequently overestimate survival Overestimation of survival can have a profoundly negative impact on the patient s end-of-life experience and the family s perception of whether the death was good or bad SO HOW CAN WE GET BETTER? CANCER Understanding Death Trajectories Glaser and Strauss introduced the concept of death trajectories in 1965 They found that the pattern of disease process leading to a patient s death had a profound impact on the experience for patients, families and clinicians Glaser BG, Strauss AL. Awareness of Dying. 1965, Aldine: Chicago NON-CANCER WOULD YOU BE SURPRISED? ONCOLOGISTS WERE ASKED TO ANSWER NO OR YES TO THE QUESTION WOULD YOU BE SURPRISED IF THIS PATIENT DIED IN THE NEXT YEAR?. A NO RESULTED IN A 7 TIMES GREATER HAZARD OF DEATH IN THE NEXT YEAR THAN A YES SAME STUDY DESIGN WITH NEPHROLOGISTS AND DIALYSIS PATIENTS: A NO RESULTED IN A 3.5 TIMES GREATER HAZARD OF DEATH IN THE NEXT YEAR THAN A YES MOSS AH, ET AL. PROGNOSTIC SIGNIFICANCE OF THE SURPRISE QUESTION IN CANCER PATIENTS. JOURNAL OF PALLIATIVE MEDICINE. 2010;13: MOSS AH, ET AL. UTILITY OF THE SURPRISE QUESTION TO IDENTIFY DIALYSIS PATIENTS WITH HIGH MORTALITY. CLIN J AM SOC NEPHROL. 2008;3:

10 PROGNOSTICATION IN COPD PROGNOSTICATION IN COPD PROGNOSTICATION IN COPD IS DIFFICULT USING NHPCO CRITERIA, 50% OF PATIENTS WERE STILL ALIVE AT 6 MONTHS (FOX 1999) COPD IS A HETEROGENEOUS DISEASE WITHOUT A SIMPLE PROGNOSTIC TRAJECTORY 1996 NHPCO CRITERIA (THE BASIS OF THE LCD S) WERE NOT ACCURATE PREDICTORS OF 6-MONTH COPD MORTALITY DISABLING DYSPNEA AT REST, POORLY RESPONSIVE TO BRONCHODILATORS FEV1, AFTER BRONCHODILATOR, OF LESS THAN 35% OF PREDICTED 25% OF PATIENTS WITH FEV1 < 35% OF PREDICTED WILL DIE WITHIN 2 YEARS, 55% WITHIN 4 YEARS (CELLI 2004) SERIAL DECREASES OF FEV1 OF >40 ML/YEAR INCREASING ER VISITS AND HOSPITALIZATIONS FOR PULMONARY INFECTIONS AND/OR RESPIRATORY FAILURE BODE INDEX PROGNOSTICATION IN COPD HYPOXEMIA AT REST ON ROOM AIR AS EVIDENCED BY: PO2 < 55 MMHG OR O2 SATURATION < 88% PCO2 > 50 MMHG 10% OF PATIENTS WILL DIE ON INDEX HOSPITALIZATION, 33% WILL DIE IN 6 MONTHS, 43% DIE IN 1 YEAR (CONNORS 1996) RESTING TACHYCARDIA > 100/MIN PROGNOSTICATION IN COPD PROLONGED OR RECURRENT MECHANICAL VENTILATION IS A POOR PROGNOSTIC SIGN MECHANICAL VENTILATION FOR > 48 HOURS HAD A 50% ONE-YEAR SURVIVAL (CELLI 2004) COPD CASE 84-YEAR-OLD FEMALE WITH A COPD EXACERBATION IS IN THE ICU AND INTUBATED. THIS IS HER 5 TH HOSPITALIZATION IN THE LAST 6 MONTHS AND 3 HAVE REQUIRED ICU STAYS. SHE IS CHRONICALLY OXYGEN AND STEROID DEPENDENT, AND DESPITE THIS HAS BASELINE DYSPNEA AT REST. WHEN YOU MENTION TO THE FAMILY THAT HER CONDITION IS SERIOUS AND SHE MAY DIE THIS TIME, THEY RESPOND WITH CONFIDENCE THAT SHE HAS BEEN IN THIS SAME POSITION MANY TIMES BEFORE AND HAS ALWAYS RECOVERED. HOW SHOULD YOU RESPOND? A. MAYBE YOU RE RIGHT. SHE MUST BE A TOUGH OLD BIRD. B. THIS TIME IS DIFFERENT AND YOU BETTER GET READY. C. MAYBE WE SHOULD GET A SECOND OPINION D. UNFORTUNATELY, THE NATURE OF HER DISEASE IS SUCH THAT THERE IS A CHANCE OF DEATH WITH EACH EXACERBATION. 10

11 QUESTIONS? REFERENCES American Lung Association website. Retrieved March 22, 2011 from Bruera E., et al. (1990). The effects of morphine on dyspnea of terminal cancer patients. Journal of Pain and Symptom Management; 5: Celli B.R., et al. (2004). The body-mass index, airflow obstruction, dyspnea and exercise capacity index in chronic obstructive pulmonary disease. N Eng J Med; 350(10): Connors A.F., et al. (1995). Outcomes following acute exacerbation of severe chronic obstructive lung disease. The SUPPORT investigators (Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatments). Am J Respir Crit Care Med; 154: Dudgeon D.J. and Rosenthal S. (1996). Management of dyspnea and cough in patients with cancer. Hematol Oncol Clin North Am; 10: Enck R.E. (1994). The Medical Care of the Terminally Ill Patient. Baltimore: Johns Hopkins University Press. Fox E., et al. (1999). Evaluation of prognostic criteria for determining hospice eligibility in patients with advanced lung, heart, or liver disease. JAMA; 282(17): Glare P., et al. (2003). A systematic review of physicians survival predictions in terminally ill cancer patients. BMJ;327: Glaser B.G. and Strauss A.L. (1965). Awareness of Dying. Aldine: Chicago. REFERENCES Hallenbeck J. (2003). Palliative Care Perspectives. London: Oxford University Press. HPM FAST Prognostication. Lau F., et al. (2007). A systematic review of prognostic tools for estimating survival time in palliative care. Journal of Palliative Care; 23: Lynn J. (1996). Caring at the end of our lives. NEJM; 335: Moss A.H., et al. (2008). Utility of the surprise question to identify dialysis patients with high mortality. Clin J Am Soc Nephrol; 3: Moss A.H., et al. (2010). Prognostic significance of the surprise question in cancer patients. Journal of Palliative Medicine; 13: Rousseau PC. (1996). Nonpain symptom management in terminal care. Clin Geriatrics Med; 12: SUPPORT Investigators. (1995). A Controlled Trial to Improve Care for Seriously Ill Hospitalized Patients. The Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatments (SUPPORT). JAMA; 274(20): Teno J.M., et al. (2001). Dying trajectory in the last year of life: does cancer trajectory fit other diseases? Journal of Palliative Medicine; 4(4): yofdying.htm 11