Age: 7.0
Sex: M
Vaccine: COVID19-2
Symptoms: Hypofibrinogenaemia, Hypotension, Hypotonia, Hypoxia, Increased need for sleep
Clinical narrative: Primary Care Physician at Discharge: DO  Admission Date: 12/23/2023 Discharge Date: 1/5/2024 DETAILS OF HOSPITAL STAY: PRESENTING PROBLEM: Acute respiratory failure with hypoxia (HCC) [J96.01] Acute on chronic respiratory failure with hypoxemia (HCC) [J96.21]   HOSPITAL COURSE: Presentation: Patient is a 7 y.o. male with scoliosis s/p repair, chronic lung disease with trach & nocturnal vent dependence, Lennox-Gastaut syndrome (ketogenic diet), dysphagia with Gtube, developmental delay who was admitted (12/23/23) with acute hypoxemic respiratory failure secondary to COVID as well as pneumonia and possible UTI causing septic shock, he also has presumed chronic hypoalbuminemia .    Per mother has been sick for many weeks. Was diagnosed with Pneumonia and prescribed a 10 day course of Amoxicillin.  He seemed to improve on this course but over the last week has become more hypoxic requiring home mech ventilation almost all day with increased O2 needs.  Secretions are not increased though.  He has also been noted to be more tired with increased sleeping periods compared to baseline.  After the Abx course he developed diarrhea which is better now but the buttocks are quite raw and there is a pretty deep sacral wound per mother. Additionally, he has had b/l pedal, hand and facial edema for the last few months without apparent etiology.  He has also had worsening lagophthalmos with exposure keratitis and chemosis for which he was being seen by an ophthalmologist outpatient.     Admitted from the ER after presenting with severe hypoxia and hypotension. Was immediately given BVM ventilation, home cuffless tracheostomy tube changed to hospital cuffed tracheostomy tube and placed on hospital ventilator on full mechanical ventilator support. CXR showed stable chronic changes b/l. Blood gas reasonable. But quite hypoxic needing 100% FiO2 to keep O2sats in upper 80% range. Also received 20ml/kg NS bolus for hypotension. Blood culture was drawn and IV Vancomycin and Ceftriaxone were given. Quadplex screen + COVID-19. WBC with leucocytosis and left shift. Thrombocytosis most likely reactive.    On arrival to the PICU:   RESP: He was maintained on the hospital ventilator. Inhaled nitric oxide utilized due to persistent hypoxemia. Both were decreased as he clinically improved. 10-day course of decadron completed for COVID. He was able to transition back to home ventilator with home settings, however remained on the ventilator 24hrs/day. Transitoned back to home uncuffed trach 1/4/24. Remained on q6hr treatments.   CV: Fluid was given for hypotension after arrival. He remained hypotensive and was started on Epinephrine infusion. Later in his course this was discontinued and he remained hemodynamically stable until a gradual worsening bradycardia/hypotension starting 12/31 through discharge with prolonged QT interval.    FEN/GI/Renal/Metabolic:  Initially NPO. Later his home ketogenic diet via G-tube was restarted. BHB level extremely high (>9) with some improvement following RD's recommendations of increasing levocarnitine dose and changing fat: protein ratio. Albumin given for hypoalbuminemia and GI consulted due to chronic hypoalbuminemia with concern for PLE, which was later ruled out. Hyperammonemia identified following encephalopathic exam including absent cough/gag reflex. Lactulose started and increased without effect. Amminul started despite that it would break ketosis and hyperammonemia persisted ultimately requiring placement of HD line and CRRT started. Etiology of hyperammonemia unclear, likely secondary to receiving remdesivir. Eventually able to come off HD AND Ammunol, initially stopped lactulose but restarted due to slowly elevating ammonia levels. Family elected to discontinue ketogenic diet and he was started on new formula regimen per specialty registered dietician recommendations. He developed hypernatremia of unclear etiology as well that improved with IV hydration and initiation of feeds. There are still some pending metabolic labs that the metabolic team will follow-up on outpatient.   ENDO: Intermittent insulin infusion started due to elevated BHB and hypertriglyceridemia.    Hematology: Prophylactic lovenox started and discontinued on 12/25 due to mild bleeding. The bleeding continued on 12/27 and coags were checked with hypofibrinogenemia (53) and coagulopathy (INR 3.8). Cryoprecipitate, FFP and PRBCs given. Three doses of vitamin K given. Thrombocytopenia requiring platelet transfusions.    Neurologic: home Fycompa & Sabril. Progressive encephalopathy and hypotonia outpatient, worse this hospital admission. EEG started due to risk of seizures when breaking ketogenic diet. No seizures initially on EEG, but severely depressed. Head CT showed decrease in extra-axial fluid spaces and increase in ventricular size. Later subclinical seizures identified required keppra load. MRI brain obtained showing severe white matter loss with new concerns of restricted diffusion to entire cortex, in the setting of more attenuated/worse EEG.   Pain/Sedation: PRN Fentanyl for pain/sedation. Held home Clonidine (used for sleep) while hypotensive; never restarted due to encephalopathic exam/bradycardia.    ID: He was + COVID.  Sputum culture and urine culture without speciation. CXR with concerns for pneumonia. He was started empirically on Cefepime and Vancomycin. Later vancomycin discontinued and he completed a course of cefepime for presumed bacterial pneumonia. Remdesivir given for COVID. Later in his course he acutely developed severe leukocytosis (30K), afebrile but at this time he was on CRRT so temperature was being regulated via the circuit. Blood and urine cultures obtained (no growth) and he was started empirically on cefepime; discontinued following 48hr rule out. Cryptococcus antigen sent and was negative as a possible etiology for hyperammonemia.    Skin/eyes:  Severe chemosis and keratitis requiring ophthalmology consult. Initially recommend eye antibiotics and taping eyes shut. Decubitus Ulcer, developing and diaper dermatitis requiring WOC consult, also with severe scrotal edema. Tarsorhaphy procedure performed 12/28/23. On 1/4/24, removed tarsorrhaphy per parents request, discussed with Dr. With plan to continue lubrication at home as needed; tobradex BID and prn for any swelling, discussed with family.    Disposition at discharge: Upon discussion between neurology and palliative care 1/4/24, family elected to progress with discharge home with hospice. Code status and de-escalation of technology has not been adjusted prior to discharge.