Age: 13.0
Sex: F
Vaccine: COVID19, COVID19
Symptoms: Pneumothorax, Positive airway pressure therapy, Positive expiratory pressure therapy, Procalcitonin, Respiratory distress
Clinical narrative: Brief HPI: Patient is a 13-year-old girl with complex PMH significant for kabuki syndrome, moderate/severe mitral valve stenosis, C VID on home IgG infusions, s/p liver transplant in 2017 and immunosuppressed, ESRD on MWF HD, steroid-induced diabetes, HTN, and ILD with home oxygen requirement of 0.5 L who presented in the setting of tachycardia and increased home oxygen requirements and with known COVID-19 infection.    She presented on 1/26 after 1 week of symptoms of myalgias and cough and having already tested positive for COVID-19 on 1/24/2022.  She has home oxygen needs 0.5 L by nasal cannula but had increased needs up to 1 L by nasal cannula.  At her dialysis prior to admission she was tachycardic and increased respiratory support she will pursue further evaluation in the ED.  She was initially fluid resuscitated but given her mitral stenosis this was stopped given concern for worsening interstitial fluid on chest x-ray.  She was started on maintenance fluids.  Empirically started on meropenem with concern for sepsis.  Laboratory studies showed leukopenia of 3.3, pro-Cal 1.9 and CRP at 19.8.  She was initially admitted to C4 for further management.   She fired sepsis alert on the floor.  At 0430 on 1/27 she had an aspiration event related to trying to drink while coughing.  She desaturated to the 60s with this episode.  She was noted to have increased work of breathing and higher oxygen needs and the decision was made to admit her to the PICU for high flow nasal cannula support.   Brief PICU course:   Respiratory: She was admitted to the PICU on HFNC 25 L at 60% FiO2.  Initially, she was tachypneic in the high 40s-low 50s with accessory muscle use and bilateral coarse rhonchorous breath sounds bilaterally with occasional expiratory wheezing.  She was maintained on her pulmonary sick plan  and received treatment for COVID-19 pneumonia with remdesivir and dexamethasone.  She continued on empiric meropenem until blood cultures were negative >48 hours. We were able to wean from HFNC to NC on 1/29/22. On 2/1 she had acute and profound decompensation of her respiratory status with desaturations to 60%, minimally responsive to CPAP. Given level of respiratory distress, decision was made to intubate to allow for optimal lung recovery given severity of Covid pneumonia. Over the coming weeks she had progressively worsening compliance and progression of her respiratory failure, which acutely worsened on the evening of 2/13 into 2/14.  She developed subcutaneous air and pneumothoraces.  She had a right sided chest tube placed without improvement in ventilation.  Also attempted iNO without improvement in cardiac output or arterial saturations.  She was transitioned to the oscillator without success and she developed a new left sided pneumothorax.  The family had previously gave patient an altered code status after discussion with Dr.  Despite attempt to decompress her left pneumothorax, patient had progressive desaturation leading to bradycardia and hypotension. At that point we asked her family to be with her.  At 1158 on 2/14 she was pronounced deceased.     CV: Initially on arrival to PICU was maintaining reasonable indices of perfusion however did require inotropic support throughout course. Inotropic support was escalated with worsening compliance as high ventilatory pressures impeded output. On 2/7, patient had an acute event with worsening tachycardia and worsening mixed venous saturation. Sedation was increased and neuromuscular blockade performed with associated drops in NIRs. Bedside ECHO performed and notable for findings of new right sided atrial and ventricular dilation as well as decreased right sided function concerning for new pulmonary hypertension. Patient initially anticoagulated with heparin drip until stable for CTA which was ultimately negative for PE. A trial of iNO was also done without clinical improvement. During acute worsening on 2/14 and repeat ECHO was obtained and notable for similar findings, but not acutely worse.    FEN/GI: She was maintained on home feeding regimen upon arrival to PICU but required transition to NG feeds after intubation but enteral feeds were not tolerated well. Thus patient started TPN/IL to meet nutritional requirements while intubated. From a transplant perspective, patient's sirolimus levels were closely monitored while inpatient and necessary dose adjustments were made per hepatology recommendations. She continued on bowel regimen and scheduled zofran.    Renal: Patient was transitioned from hemodialysis to CRRT. She overall did well with this. Some concern that event on 2/7/22 was related to citrate lock, but patient tolerated transition back to citrate without further incidence.    ID: She received dexamethasone and remdesivir for Covid. Blood, urine and ETT cultures initially no growth to date. She was initially on meropenem for 48 hours for rule out of superimposed bacterial pneumonia and was discontinued with clinical improvement. However, upon intubation and respiratory support escalation, she was re-started on meropenem for suspected superimposed bacterial pneumonia. She was switched to Avycaz per ID and completed a 10 day course. Blood cultures were obtained during her acute worsening on 2/7/22 and ultimately grew enterococcus faecium. She was started on vancomycin. ETT also cultured at that time with polymicrobial growth. Decision made to not treat this growth unless clinically worsening. Patient was given a therapeutic dose of fluconazole on 2/14 due to worsening of respiratory status. She continued on her dapsone, valgancyclovir and fluconazole for her baseline infection prophylaxis.     Endocrine: She was aintained on stress dose steroids, hydrocortisone 50 mg 3 times daily.  Her home dose of prednisone 5 mg twice daily was held while on stress dose steroids. Also received dexamethasone 3.5 mg daily in the setting of her Covid pneumonia. She was transitioned to her home dose steroids on 2/11/22 with wean of hydrocortisone complete on 2/13/22. She also received one dose of stress dose steroids on the morning of 2/14 due to acute decompensation. She required an insulin drip while on stress dose steroids due to poor glycemic control.