Case Studies from the Pulmonary Function Laboratory Focus Conference on Respiratory Care and Sleep Medicine, May 2013 Nashville, TN Angela Lorenzo, MS,

Case Studies from the Case Studies from the Pulmonary Function Pulmonary Function

LaboratoryLaboratory

Focus Conference on Respiratory Care Focus Conference on Respiratory Care and Sleep Medicine, May 2013 Nashville, and Sleep Medicine, May 2013 Nashville,

TNTN

Angela Lorenzo, MS, RRT, RPFT

Respiratory Care DivisionSchool of Health Sciences

Indications for PFTsIndications for PFTs

Establish baseline of pulmonary functionEstablish baseline of pulmonary function Determine presence/severity of diseaseDetermine presence/severity of disease Monitor disease Monitor disease

progression/improvementprogression/improvement Monitor response to therapyMonitor response to therapy Pre-operative assessmentPre-operative assessment Disability evaluationsDisability evaluations Occupational lung diseaseOccupational lung disease

Case 1: Ankylosing Spondylitis Case 1: Ankylosing Spondylitis and MAC c/o DOEand MAC c/o DOE

53 y/o male, Ht 72 “, Wt 90 lbs, Smoker- 53 y/o male, Ht 72 “, Wt 90 lbs, Smoker- 39 pk/yr hx, c/o DOE39 pk/yr hx, c/o DOE

4 Months ago released from hospital for 4 Months ago released from hospital for hemoptysis, Rx’s with antibiotics & Home hemoptysis, Rx’s with antibiotics & Home O2O2

Chest Imaging: LUL cavitating lesion with Chest Imaging: LUL cavitating lesion with an enlarging mycetoma, new densities in an enlarging mycetoma, new densities in RUL & LLL, widespread pulmonary RUL & LLL, widespread pulmonary fibrosisfibrosis

Sputum + for M. xenopi in past (now -)Sputum + for M. xenopi in past (now -)

Case 1: Ankylosing Case 1: Ankylosing Spondylitis and MAC c/o Spondylitis and MAC c/o

DOEDOE Also has Crohn’s Disease and Also has Crohn’s Disease and

treated with 6MP (mercaptopurine) treated with 6MP (mercaptopurine) which in rare cases can cause which in rare cases can cause pulmonary fibrosispulmonary fibrosis

Pt had been treated with Remicade Pt had been treated with Remicade in past (anti-TNF) associated with in past (anti-TNF) associated with TB infectionsTB infections

Case 1: Ankylosing Spondylitis Case 1: Ankylosing Spondylitis and MACand MAC

FVC: 1.47 L (28%)FVC: 1.47 L (28%) FEV1: 1.37 L FEV1: 1.37 L

(33%)(33%) FEV1%: 93%FEV1%: 93% MIP: 52% MIP: 52%

predictedpredicted MEP: 26% MEP: 26%

predictedpredicted

Unable to perform Unable to perform DLCO or N2 DLCO or N2 washout due to washout due to leakleak

Limited ability to Limited ability to open mouth-could open mouth-could not use not use mouthpiecemouthpiece

Case 2: Alpha-1 Antitrypsin Case 2: Alpha-1 Antitrypsin DeficiencyDeficiency

64 y/o male with history of A-1AD64 y/o male with history of A-1AD Has pulmonary and liver involvementHas pulmonary and liver involvement

Up to 15% pts have liver involvementUp to 15% pts have liver involvement Currently taking Aralast 5 g IV weeklyCurrently taking Aralast 5 g IV weekly

Alpha-1 proteinase inhibitorAlpha-1 proteinase inhibitor Increased exercise tolerance since Increased exercise tolerance since

beginning A1PI therapy beginning A1PI therapy Progressive weight loss has stabilizedProgressive weight loss has stabilized

Case 2: A1ADCase 2: A1AD FVC: FVC: 4.47 L 4.47 L (98%)(98%) FEV1: FEV1: 2.63 L 2.63 L (74%)(74%) FEV1%: FEV1%: 59%59% FEF FEF 25-75%25-75% 0.99 L/sec0.99 L/sec (29%)(29%) PEFR PEFR 8.09 L/sec8.09 L/sec (93%)(93%) TLC TLC 7.64 L7.64 L (111%)(111%) FRC PL FRC PL 5.00 L5.00 L (141%)(141%) RV RV 3.09 L3.09 L (129%)(129%) DLCO 70%; DLCO/Hb 67%; DLCO 70%; DLCO/Hb 67%;

DLCO/VA 42%DLCO/VA 42%

Case 2: A1ADCase 2: A1AD Alpha-1 antitrypsin is Alpha-1 antitrypsin is

produced in the liverproduced in the liver Protects the lungs Protects the lungs

from the effects of from the effects of elastaseelastase

A-1A inactivates A-1A inactivates elastase carried on elastase carried on WBCs in the lungsWBCs in the lungs

Elastase destroys Elastase destroys alveolialveoli

Prolastin, Zemaira, Prolastin, Zemaira, AralastAralast

Case 2: A1ADCase 2: A1AD

Year 2009 1/2010 12/2010 2012

FVC 3.53 4.47 4.14 92%

4.05 92%

FEV1 2.38 2.63 2.50 70%

2.51 73%

FEV1/FVC 67% 59% 60% 62%

FRC 4.30 121%

4.53 129%

RV 3.31 3.09 3.17 132%

3.62 149%

DLCO 58% 70% 61% 58%

Case 2: A1ADCase 2: A1AD

Diagnosed 6 years ago presenting Diagnosed 6 years ago presenting with DOE, reactive erythrocytosis with DOE, reactive erythrocytosis 2/2 hypoxia2/2 hypoxia

On A1PI 5 yearsOn A1PI 5 years Liver cirrhosis diagnosed 7 years Liver cirrhosis diagnosed 7 years

ago, stable at this timeago, stable at this time Weight loss has stabilizedWeight loss has stabilized No limitations on activityNo limitations on activity

Case 3 CREST/ILDCase 3 CREST/ILD

64 y/o male with CREST is being 64 y/o male with CREST is being evaluated for ILD. evaluated for ILD.

Pt also has goiter. Pt also has goiter. Previous PFTs WNL when pt tested Previous PFTs WNL when pt tested

to see if goiter was obstructing to see if goiter was obstructing tracheatrachea

Case 3: CREST/ILDCase 3: CREST/ILD

FVC 2.60 L 60%FVC 2.60 L 60% FEV1 2.11 L 62%FEV1 2.11 L 62% FEV1% 81%FEV1% 81% FEF25-75% 2.15 FEF25-75% 2.15

67%67% PEF 7.56 89%PEF 7.56 89% No bronchodilatorNo bronchodilator Post-loops assessed Post-loops assessed

effect of goiter on effect of goiter on airwayairway

Case 3: CREST/ILDCase 3: CREST/ILD TLC 4.45L 66%TLC 4.45L 66% VC 2.75 L 63%VC 2.75 L 63% FRC N2 2.63L 74%FRC N2 2.63L 74% ERV 0.94 L 65%ERV 0.94 L 65% RV 1.69 L 69%RV 1.69 L 69% RV/TLC 38% 98%RV/TLC 38% 98% DLCO 58%DLCO 58% DLCO/VA 52%DLCO/VA 52%

Case 4: Asthma, Case 4: Asthma, Allergies, DM2Allergies, DM2

54 y/o asthmatic with hx DM tested54 y/o asthmatic with hx DM tested Pulmonary Medication profile: Pulmonary Medication profile:

advair singulair, albuterol prn (used advair singulair, albuterol prn (used 5 canisters in a 6 month time frame)5 canisters in a 6 month time frame)

At age 49 pt sought treatment for a At age 49 pt sought treatment for a suspected latex allergysuspected latex allergy

Pt had known seasonal allergies. Pt had known seasonal allergies.


Allergy skin testing + for pollens, grass, Allergy skin testing + for pollens, grass, trees, dust mites, feathers, dogs, cats, trees, dust mites, feathers, dogs, cats, tree nuts, shell fish, some other foodstree nuts, shell fish, some other foods

Blood tests were – for latex x 2, + some Blood tests were – for latex x 2, + some foodsfoods

Pt agreed to Immunotherapy. Pt agreed to Immunotherapy. 11stst PFT 4 years prior to beginning IT PFT 4 years prior to beginning IT 22ndnd PFT 2 years IT PFT 2 years IT FeNO 4 years ITFeNO 4 years IT


FVC 2.68L FVC 2.68L 85%85%

FEV1 1.98L FEV1 1.98L 75%75%

FEV1% 74%FEV1% 74% FEFFEF25-7525-75 1.44 1.44

27%27% L/SECL/SEC

PEFR 6.32 PEFR 6.32 104%104%

Pre/Post

Case 4: Asthma, Case 4: Asthma, Allergies, DM2 Allergies, DM2

FVCFVC 2.58L 85% 2.58L 85% FEV1 2.52L FEV1 2.52L

79%79% FEV1% 80%FEV1% 80% FEFFEF25-75 25-75 2.82 2.82

57%57% L/SECL/SEC

PEFR 5.96 PEFR 5.96 134%134%


20032003 FVC 2.68L FVC 2.68L

85%85% FEV1 1.98L FEV1 1.98L

75%75% FEV1% 74%FEV1% 74% FEFFEF25-7525-75 1.44 1.44 27% 27% L/SECL/SEC

PEFR 6.32 PEFR 6.32 104%104%

L/SECL/SEC

20092009 FVCFVC 2.58L 85% 2.58L 85% FEV1 2.52L FEV1 2.52L

79%79% FEV1% 80%FEV1% 80% FEFFEF25-75 25-75 2.82 2.82

57%57% L/SECL/SEC

PEFR 5.96 PEFR 5.96 134%134%

L/SECL/SEC


2012 worsening symptoms after URI2012 worsening symptoms after URI 2 weeks after URI, sinus complaints, 2 weeks after URI, sinus complaints,

productive cough. No fever, chillsproductive cough. No fever, chills Baseline FeNO 17 ppbBaseline FeNO 17 ppb Peak symptoms FeNO 54 ppbPeak symptoms FeNO 54 ppb Advair increased to 250/50: FeNO 34 ppbAdvair increased to 250/50: FeNO 34 ppb Advair increased to 500/50: FeNO 17 ppbAdvair increased to 500/50: FeNO 17 ppb Baseline dose 100/50 resumed: FeNO 17 Baseline dose 100/50 resumed: FeNO 17

ppbppb


Advair Dose FeNO Resting Blood Sugar

100/50 15-19 100-110

100/50 54 160’s

250/50 34 140’s

500/50 17 100

100/50 17-19 90-110

Case 5Case 5

72 y/o physician “curious” to see 72 y/o physician “curious” to see what cigarettes have done to her what cigarettes have done to her lungs and what a PFT feels like.lungs and what a PFT feels like.

Approx 75 pack year historyApprox 75 pack year history No respiratory complaintsNo respiratory complaints

Case 5Case 5

Case 5Case 5

Case 6: BronchiectasisCase 6: Bronchiectasis

49 y/o male with hx severe COPD, pulm TB 49 y/o male with hx severe COPD, pulm TB for which he began tx 1994 for which he began tx 1994

TB relapsed due to noncompliance. TB relapsed due to noncompliance. Completed multi-drug tx > 1 yr 1996Completed multi-drug tx > 1 yr 1996 Now has stable biapical scarring/cavitationNow has stable biapical scarring/cavitation Sputum + MAC 3 of 4 cultures, no MAI txSputum + MAC 3 of 4 cultures, no MAI tx 25 pack year smoking hx, quit 8 years ago25 pack year smoking hx, quit 8 years ago

Case 6: BronchiectasisCase 6: Bronchiectasis

Presents for f/up and sputum inductionPresents for f/up and sputum induction No wt loss, night sweats, fever, chills No wt loss, night sweats, fever, chills Good appetite, good ET >3-4 flights stairsGood appetite, good ET >3-4 flights stairs + phlegm varies clear to brownish, no + phlegm varies clear to brownish, no

blood blood Chest CT: extensive bilat old Chest CT: extensive bilat old

granulomatous disease with bullous and granulomatous disease with bullous and cavitary changes, significant volume loss in cavitary changes, significant volume loss in upper lobes, bronchiectatic changes, upper lobes, bronchiectatic changes, fibrosis/scarring notedfibrosis/scarring noted

Case 6: BronchiectasisCase 6: BronchiectasisYear 2007 2009

FVC 3.12L 63% 3.89L 76%

FEV1 1.27L 32% 1.10L 27%

FEV1/FVC 41% 28%

SVC 92% 3.89L 76%

IC 75% 2.70L

ERV 153% 1.19L 70%

FRCpleth 130% 4.35L 122%

RV 145% 3.17L 144%

DLCO/VA WNL 64%

R/A Pulsox 97% 98%

Pt began Tx with spiriva, foradil, asmanex and albuterol prn in 2007. Asmnex d/c’d 2009

Case 7: Parkinson’s Case 7: Parkinson’s DiseaseDisease

62 y/o male with hx Parkinson’s disease 62 y/o male with hx Parkinson’s disease (1998) eosinophilic PNA 2006-nml eos since (1998) eosinophilic PNA 2006-nml eos since 20082008

Worsening SOB at rest, band-like radiating Worsening SOB at rest, band-like radiating chest tightness that has increased over the chest tightness that has increased over the last several weeks and gets worse as his last several weeks and gets worse as his Parkinson’s meds wear offParkinson’s meds wear off

Parkinson’s meds: carbidopa levodopa Parkinson’s meds: carbidopa levodopa (Sinemet) q 2h (past 8 years), Selegiline (4 (Sinemet) q 2h (past 8 years), Selegiline (4 years); past use comtan d/c’d 2/2 side effectsyears); past use comtan d/c’d 2/2 side effects

Case 7: Parkinson’s Case 7: Parkinson’s DiseaseDiseaseAfter Parkinson’s Meds

2 hours later, feeling SOB

FVC 3.98 L 1.79 L

FEV1 2.98 L 1.77 L

FEV1FVC 75% 99%


Mask facies, tachyneic, speaks in Mask facies, tachyneic, speaks in short sentences, lungs clear with short sentences, lungs clear with shallow respirationsshallow respirations

As dopa wore off, PFT pattern As dopa wore off, PFT pattern changed to restricted- muscle changed to restricted- muscle rigidity affecting respiratory muscles rigidity affecting respiratory muscles which is rarewhich is rare

Dyspnea is a described side effect of Dyspnea is a described side effect of carbidopa levodopacarbidopa levodopa


Case 8: AmiodaroneCase 8: Amiodarone

79 y/o male with h/o cardiomyopathy 79 y/o male with h/o cardiomyopathy and subsequent Afib. and subsequent Afib.

Pt now on amiodaronePt now on amiodarone Pt c/o DOEPt c/o DOE The following tests were ordered: The following tests were ordered:

spirometry and static lung volumesspirometry and static lung volumes DLCO was not orderedDLCO was not ordered Amiodarone known to cause pulmonary Amiodarone known to cause pulmonary

fibrosisfibrosis

Case 8 : AmiodaroneCase 8 : Amiodarone

FVC 2.68L FVC 2.68L 85%85%

FEV1 1.98L FEV1 1.98L 75%75%

FEV1% 74%FEV1% 74% FEFFEF25-7525-75 1.44 1.44

27%27% L/SECL/SEC

PEFR 6.32 PEFR 6.32 104%104%

L/SECL/SEC

Case 8 : AmiodaroneCase 8 : Amiodarone TLC 7.49L TLC 7.49L

94%94% VC 4.68L VC 4.68L

95%95% FRC PL 3.80L FRC PL 3.80L

95%95% ERV 0.99L ERV 0.99L

61%61% RV 2.81L RV 2.81L

95%95% RV/TLC 38% 84%RV/TLC 38% 84%

Case 8 : AmiodaroneCase 8 : Amiodarone

DLCO: 41% predictedDLCO: 41% predicted Discussion followed: was decreased Discussion followed: was decreased

DLCO due to interstitial edema 2DLCO due to interstitial edema 2oo to to cardiac failure or lung disease?cardiac failure or lung disease?

Side effects:Side effects: Interstitial pneumonitis, fibrosisInterstitial pneumonitis, fibrosis Dyspnea, non-productive cough - Dyspnea, non-productive cough -

common presenting symptomscommon presenting symptoms Decreased TLC and DLCODecreased TLC and DLCO

Case 9: DOE, CLL, Case 9: DOE, CLL, LymphomaLymphoma

63 y/o male with Small Cell Lymphoma and 63 y/o male with Small Cell Lymphoma and Chronic Lymphocytic Leukemia c/o DOE and Chronic Lymphocytic Leukemia c/o DOE and persistent unprod cough, 20 pack year hx, persistent unprod cough, 20 pack year hx, quite 11 years agoquite 11 years ago

RituximabRituximab: cardiac= angina, arrhythmia; : cardiac= angina, arrhythmia; pulm = Obliterans bronchiolitis, pneumonitispulm = Obliterans bronchiolitis, pneumonitis

FludarabineFludarabine: pulm- cough, hypersensitivity : pulm- cough, hypersensitivity reactions, pulmonary fibrosis, interstitial reactions, pulmonary fibrosis, interstitial infiltratesinfiltrates

CytoxanCytoxan: cardiac- cardiomyopathy; pulm – : cardiac- cardiomyopathy; pulm – interstitial pneumoniainterstitial pneumonia

Case 9: DOE with CLL, Case 9: DOE with CLL, LymphomaLymphoma

FVCFVC 2.90L 59%2.90L 59% FEV1 2.12L FEV1 2.12L

56%56% FEV1% 73% FEV1% 73% FEFFEF25-7525-75 1.50

43% PEFRPEFR 6.026.02

67%67%

Case 9: DOE with CLL, Case 9: DOE with CLL, LymphomaLymphoma

TLCTLC 4.74L 63% 4.74L 63% VCVC 2.97L 60% 2.97L 60% FRC PL 2.54L 68%FRC PL 2.54L 68% ERV 0.77L 47%ERV 0.77L 47% RV 1.77L 70%RV 1.77L 70% RV/TLC 37% 90%RV/TLC 37% 90% DLCO (Hb 10.8) 44%DLCO (Hb 10.8) 44% DLCO/VA 49%DLCO/VA 49%

Anti-Neoplastic DrugsAnti-Neoplastic Drugs

Hypersensitivity reactionsHypersensitivity reactions: : characterized by cough, SOB, characterized by cough, SOB, bronchospasm, urticaria, allergic bronchospasm, urticaria, allergic alveolitis/pneumonitisalveolitis/pneumonitis Procarbazine, Azathioprine (Imuran), Procarbazine, Azathioprine (Imuran),

AsparginaseAsparginase Pulmonary fibrosisPulmonary fibrosis: :

Busulfan (Myleran), Cyclophosphamide Busulfan (Myleran), Cyclophosphamide (Cytoxan), Bleomycin, Chlorambucil (Cytoxan), Bleomycin, Chlorambucil (Leukeran), Melphalan(Leukeran), Melphalan

Case 10: Lung Case 10: Lung Cancer/ResectionCancer/Resection

68 y/o male referred to PFT Lab for 68 y/o male referred to PFT Lab for pre-op evaluation for LUL lung pre-op evaluation for LUL lung cancercancer

Pt is a 40 pack year ex-smoker who Pt is a 40 pack year ex-smoker who quit 10 years ago. quit 10 years ago.

DOE, Productive cough DOE, Productive cough He presented nearly one year later He presented nearly one year later

for follow up PFTsfor follow up PFTs

Case 10: Lung Case 10: Lung Cancer/ResectionCancer/Resection

Pre-op s/p Left Upper Lobectomy

FVC 2.44L 53% 2.27L 50%

FEV1 1.55L 44% 1.47L 42%

FEV1/FVC 64% 65%

FEF 25-75 0.58L/Sec 18% 0.73L/Sec

TLC 4.18L 59% 3.89L 54%

VC 2.56L 59% 2.34L 51%

FRCpleth 2.29L 62% 2.24L 61%

ERV 0.66L 44% 0.69L 46%

RV 1.63L 63% 1.55L 60%

RV/TLV 39% 40%

DLCO

Case Studies from the Pulmonary Function Laboratory Focus Conference on Respiratory Care and Sleep Medicine, May 2013 Nashville, TN Angela Lorenzo, MS,

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