Interstitial Lung Disease


Patient presentation:
K.P., 55 year old male presented to ED with complains of increased shortness of breath (SOB) over the past few days. No known sick contacts. In ER pt increasingly SOB and hypoxic (PaO2 55 on 100% Non-Rebreather) and soon required intubation. K.P. was then sent to the ICU for further management and ventilator support.

PMH: HTN, DM, hyperlipidemia, Sarcoidosis on home O2 and steroid treatment

Labs:
Na+: 140, Cl-: 90, K+: 4.0, BUN: 40, Creatinine: 1.2, Glucose: 187
WBC: 19,000, Hgb: 10, Hct: 30, PLT: 200,000
ABG: pH 7.46, PaCO2 47, PaO2 60, HCO3 26 on 100% FiO2 post intubation AC rate 14 Vt 450 PEEP 8
Vitals: BP: 132/80, HR: 95 - sinus tachycardia, RR: 14, O2sat: 82%, Temp: 37.7 C axillary
CXR: lower lung consolidation

Diagnosis:
Interstitial lung disease and possible pneumonia

Treatment:
Once K.P. arrives to ICU, A-Line placed for frequent ABGs. K.P. is started on broad spectrum IV antibiotics and IV steroids. Pt respiratory status continues to decline and becomes increasingly hypoxic. PEEP is eventually increased to 14 on ventilator with FiO2 of 100%.  O2 reserve is low and is not tolerating activity. Pt is also bucking the ventilator further compromising his respiratory status. K.P. has high airway pressures indicative of poor lung compliance. K.P. requires paralytics (vecuronium gtt) and heavy sedation (Versed® and fentanyl drips). Train of four is used to monitor degree of neuromuscular blockade.  K.P. also becomes hemodynamically unstable requiring multiple pressors (norepinephrine, dopamine, vasopressin drips). A pulmonary arterial catheter is placed, showing severe pulmonary HTN with PA pressures of 80’s/30’s and PA wedge pressure 28. Pt placed on sildenafil (Viagra®) with minimal effect. Pt is then started on Remodulin® infusion for pulmonary HTN.

Outcome:
Even with the Remodulin®, pulmonary HTN remains severe and pt continues to require high ventilator settings. After being told that K.P. has a very poor prognosis, the family opts for withdrawal of care and comfort measures.