CC: “ I couldn ’ t breathe ”

1. CC: “I couldn’t breathe” Presented by: Ha Nguyen, MS3

3. CC: “I couldn’t breathe” Presented by: Ha Nguyen, MS3

4. History of Present Illness Mr. J.R. is a 39 YO WM, with a history of asthma, who presented to the HH ER with SOB, wheezing, and cough productive of sputum. For ~2 wks prior to admission, he experienced progressive SOB and began to use a Primatene Mist inhaler; his SOB progressed to where he needed to use his inhaler every few minutes. (cont’d…)

6. HPI (cont’d) • On the night of admission, Mr. J.R. was so SOB that he could not speak or breathe. He was so fearful of dying that he became incontinent. Upon arrival to the ER, pt was given 3 breathing treatments and Solu-Medrol, after which he felt much better. His SaO2 was 84% on RA and 96% on 8L of NC. Pt says that this is the most severe asthma attack that he has ever had.

7. Pertinent ROS • Gen: 2 wk hx of fatigue and decreased PO intake, resulting in a 10 lb wt loss. No fever/chills • Resp: Asthma precipitated by stress, weeds/grasses. Pt unsure of what triggered his SOB this time. His cough over the past 2 wks have been productive of sputum with brown flecks.

8. Pertinent ROS • GI: past hx of excessive flatulence. Presently, no diarrhea, constipation, nausea, or vomiting. He does not eat as much as he should and is frustrated with being too thin. • Theories about food energies and type A blood. • No beef, breads, dairy, tomatoes, other foods with “warm energies”. • Skin: no rash or infection • CV: No chest pain

9. Past Medical History • 1 YO: bilateral subdural hematomas and traumatic hydrocephalus. An V-P shunt was inserted, then replaced with a V-A shunt due to recurrent infections. The V-A shunt was later found to be non-functional but was left in place for cosmetic reasons. • 10 YO: Dx w/ asthma after frequent episodes of SOB requiring epinephrine shots up to 2x/wk. Theo-Dur was prescribed—pt compliant for 1 yr. (cont’d…)

10. PMHx (cont’d) • 23 years w/o maintenance therapy or rescue inhaler for asthma. Pt still had episodes of wheezing, but sx were not severe enough to seek medical attention. Pt said that he believed in more “natural” ways maintain health and focused on diet changes to control asthma. (cont’d…)

11. PMHx(cont’d) • 1998- admitted to HH for asthma exacerbation and pneumonia; eosinophilia was noted. D/C with home O2. Self-weaned off O2 after 6 wks with use of herbs/diet. • Malnutrition. He has been extremely thin most of his life.

12. Social History • History of child abuse, lives alone, but has friends from church. Little family support. • Eastern Medicine vs. Western medicine: • focused on understanding the body and its energy balance; • believes that most illnesses can be cured by diet or other natural means. • Western medicine does more harm than good. • Unstable employment • construction, electrician, shipping & receiving

13. Social History • ⊝ smoking hx • ⊝ alcohol use • ⊝ other substance abuse • Has not traveled outside of U.S.

14. Medications • None. • Pt takes herbal supplements as needed for various symptoms (H/A, SOB, etc.)

15. Complete ROS • Gen: 2 wk hx of fatigue and decreased PO intake, resulting in a 10 lb wt loss. No fever/chills. • HEENT: no headache, rhinorrhea, • Skin: no rashes or infections • Resp: Asthma precipitated by stress, weeds/grasses. Pt unsure of what triggered his SOB this time. His cough over the past 2 wks have been productive of sputum with brown flecks. • CV: No chest pain, no hx of MI, no valvular defects

16. Complete ROS • GI: past hx of excessive flatulence. Presently, no diarrhea, constipation, nausea, or vomiting. He does not eat as much as he should and is frustrated with being too thin. • GU: no dysuria, no increased frequency, no hematuria, no STDs, not sexually active. • Vascular: no leg edema, claudication, or hx of thrombi • Neuro: No tingling, no numbness, no weakness • Musculoskeletal: No weakness, no arthritis • Hematologic: no transfusions • Psychiatric: sad and frustrated about health

17. Physical Exam • T=98.6oF; BP=122/84; HR=120; RR= 20 SaO2=84% on RA, 96% on 8L NC • Gen: Pt is in NAD, currently on breathing treatments, and is cachectic • Ht: 5’9”, 94lbs (62% IBW) • HEENT: normocephalic, atraumatic, PERRLA, oropharynx is clear. • NECK: no lymphadenopathy, a .5cm tube running w/ R IJV vein is palpable.

18. Physical Exam • CV: RRR, -MGR • LUNGS: Diffuse bilateral rhonchi, rales, and wheezes with good air flow • ABD: non-distended, active bowel sounds, non-tender. • EXTREMETIES: no cyanosis, clubbing, or edema • NEURO: CN II-XII grossly intact • SKIN: warm and dry, no lesions or infections

19. Labs • Na: 137 mEq/L • K: 4.2 mEq/L • Cl: 101 mEq/L • HCO3: 27 mEq/L • BUN: 13 mg/dL • Cr: 0.9 mg/dL • Glucose: 178 mg/dL (cont’d…)

20. Labs (cont’d) • WBCs: 14.28 x 103/L • L17.0, N65.6, M5.9, E9.8, B0.05 • Hb: 14.9 g/dL • Hct: 44.1 % • Platelets: 258 x 103/L

21. Radiology • Portable AP, semi-upright chest plain film: Bilateral infiltrates, R more extensive than L. Patchy infiltrate of RUL. Azygous lobe noted. Diffuse fibrotic changes bilaterally. • CT w/ contrast: Fairly severe bronchiectasis involving upper and lower lobes bilaterally. Acute infiltrative change in lateral aspects of RUL

22. PFTs • FEV1 = 1.07L; 27% of predicted • FVC = 1.90L; 39% of predicted • FEV1/FVC= 56%; 68% of predicted.

23. Additional Tests • Peripheral blood eosinophils=3,420/mm3 (nl <450) • Sweat chloride test in both arms x 2 :40-48 mE/L (borderline) • ⊝ common CFTR gene mutations • Serum AAT level: 127 (nl: 110-200) • 24 hr fecal fat: 2.4 (nl: 2-7) • D-xylose abs: 35% of 25g dose(nl >16% excretion) • IgE= 12,660 KU/L (nl: <100 IU/mL) • ⊕ Aspergillus skin test • Aspergillus IgG AB > 200 (nl: 0-110) • ⊝ PPD skin test

24. Differential Diagnosis • Asthma exacerbation • Cystic Fibrosis • Tuberculosis • pneumonia • Alpha-1 Antitrypsin deficiency • Pulmonary aspergillosis • Bacterial Endocarditis w/ PE

25. Assessment • ABPA and asthma exacerbation. • CF, AAT deficiency, & TB have been sufficiently ruled out with screening tests. No evidence of PE on CT. • Pt’s history of eosinophilic asthma with ↑IgE, ↑anti-aspergillus IgG, and ⊕ Aspergillus skin test, along with bronchiectasis all support the dx of ABPA. • Brown flecks in sputum most likely aspergillus

26. Aspergillosis

27. Aspergillus • Ubiquitous soil-dwelling filamentous fungi. • Found in dust, compost, foods, spices, rotted plants, organic debris. • Small spores (2-3 m) easily inhaled and deposited deep in lungs- found in sputum of healthy individuals.

28. Pulmonary Aspergillosis Inhalation of aspergillus spores colonization Normal Host Cavitary Lung dz Chroniclung dz or mild ICH ICH asthma Chronic Necrotizing aspergillosis IPA No Sequela ABPA Aspergilloma

29. ABPA clinical picture • Asthma complicated by recurrent episodes of bronchial obstruction, fever, malaise, expectoration of brownish mucous plugs, peripheral blood eosinophilia, and hemoptysis.

30. ABPA: Diagnostic Criteria

31. ABPA Staging • Stage 1: Acute presentation • Stage 2: Remission • Stage 3: exacerbation • Stage 4: corticosteroid-dependent • Stage 5: Irreversible lung damage-fibrosis

33. ABPA treatment • Oral corticosteroids (tapering or long term low-dose) • suppress immunologic response to Aspergillus antigens • Suppress secondary inflammatory rxn • relief of bronchospasm, clearing of pulmonary infiltrates, and a decrease in IgE levels and decreased peripheral eosinophilia • Preservation of lung fxn unknown (cont’d)

34. ABPA treatment • Oral corticosteroids (tapering or long term low-dose) • Control acute inflammation • Limit progressive lung injury • Prevent acute episodes of ABPA (cont’d)

35. ABPA treatment (cont’d) • Itraconazole • Few side effects • Lower fungal load • Reduction of corticosteroid use • improvement of pulmonary infiltrates • Reduction of IgE levels • Expensive! ($210/wk x 16 wks =$3360)

36. Plan • Maintain SaO2 > 92% • Tequin 400mg #1 PO daily x 7 days • Albuterol neb 90 mcg 2 puffs daily • Prednisone 40mg #1 PO daily • Itraconazole 200mg #1 PO twice daily • Flutter valve to clear sputum • Follow-up care w/ pulmonologist

37. Pt’s clinical course • CPPD improved sputum production and breathing. • Rales, rhonchi, and wheezes bilaterally never resolved. • Pt’s SaO2 at d/c was 90% on RA; did not qualify for home O2. • Very poor prognosis, given he is has progressed to stage V of ABPA with severe impairment of respiratory function.