Grand Rounds Recap 2.8.23

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Dr. Brian Gibler Visiting Lecture WITH Dr. John Deedda

  • The 1st Surge COVID-19 at Henry Ford Hospital 

  • ED and ICU front line

    • The operational framework of having a robust EM/CCM faculty made a continuous operational unit between EM and ICU care. 

    • MICU, SICU, CVICU, NSICU

  • Opened first ICU COVID pod in MArch 

  • Opened 17th COVID ICU pod in april

  • Lessons Learned

    • We can adapt

      • New medications

      • New policies

      • New processes

      • New covid units 

    • We are agile 

      • Surge Planning

        • Build capacity 

        • Surge team

          • ED,ICU physician and Nursing leadership

      • Daily Surge call

        • Resource needs

        • Expand capacity(where, when,who)

    • We are Collaborative

      • Routine calls with Wuhan, Barcelona, and Milan

      • Critical Care Clinical Standardized COVID-19 Management across all ICUs in the Henry Ford System

    • We are innovative

      • Creating novel care spaces

      • Changing how care is delivered to conserve resources 

    • We are Compassionate

      • A lot of isolation with no visitors and the stay at home order

      • Lots of donations and care from around the hospital and the city

    • We are Courageous and Strong 

      • We worked 

        • Long hours

        • High volume

        • Tough conditions in PPE

    • We are supported

      • Massive outpouring of support from the community and from within the hospital system 

    • We are Human 

      • Range of emotions

        • Lots of death, saves,and tragedies

        • Grief, hope, and everything in between

    • We are proud

      • Pride in the work they were doing help sustain incredibly difficult work conditions

    • We will Endure

      • Sprint became a marathon

      • Physical and emotional strain of vaccination politics

      • Three more surges after the initial surge

      • Crisis that spawned more crises

        • Vaccination as a term of employment

        • Premium labor crippling finances

        • Healthcare will forever be more costly

      • Personal wellness 

  • Leadership I've learned from shoe repair, the caddyshack, the c-suite and places in between

    • Emotional Intelligence

    • Discuss Various styles

      • Leadership styles are strategic choices not personalities

        • Self Awareness

        • Self management

        • Social awareness

        • Social skill 

      • Successful leaders can flex between leadership styles

    • Leadership actions that motivate engagement

    • Discuss courage  the challenges of employing it in the workplaces that cultivate it 

      • Courage is taking worthy actions despite the potential risk

      • Works when

        • Excel at their job

        • Engaged in the organization

        • even handed

      • Pick your battles

        • Is it critical 

        • Is it the right time

        • Does the battle aid or hinder winning the war 

        • Focus on 

          • Frame the issue 

          • Effective use of data

          • Managing emotions in the room

R1 Clinical Diagnostics on TEG WITH Dr. Della Porta

Blood Products

  • General 

    • “A therapeutic substance derived from human blood, including whole blood and other blood components for transfusion, and plasma derived medicinal products.”

    • Disparities in Quality and Access 

      • There is a marked difference in the level of access to blood between low- and high-income countries. The whole blood donation rate is an indicator for the general availability of blood in a country. 

      • Transfusion related transmission of Hepatitis B is 2.81 in Low income countries and 0.02% in high income countries 

    • Components of Blood 

      • pRBCs 45% of Whole blood

        • 12 million units/year in the US

        • 1500 per month at UC

        • Shelf life of 42 days at 4C (fridge)

        • 70% Hct in pRBC compared to 40% Hct in whole blood

        • Increases Hct 3-4% or increases Hgb 1g/dL

        • Can be washed or leukoreduced

        • Indicated for patients needing red cells for oxygen carrying capacity rather than for volume replacement (i.e. sickle cell, symptomatic anemia, Hgb < 7), or as part of MTP

      • Plts <1% of Whole blood 

        • Pooled from donated blood (ie. 5 donors= 1u)

        • Shelf life of 5 days at 22C (room temp)

        • A unit can increase plt count by 30-50k platelets

        • Transfuse SLOWLY to avoid hypotension

        • Indicated or patients with plt counts <10k prophylactically, known platelet disorders with associated signs of bleeding, or patients with thrombocytopenia prior to planned invasive procedure (i.e. <20k prior to CVC placement), as part of MTP

      • Plasma 

        • Water 92% of Plasma 

        • Protein 7% of plasma

          • Albumin

          • Clotting factors 

          • Products 

            • FFP

              • Portion of whole blood that remains after platelets and RBC are removed

              • 1000u/month at UC

              • Increases each clotting factor by 2-3%

              • Takes 45 minutes to thaw, pre-thawed available for emergency use

              • Shelf life of 12 months at -25C 

              • Indicated for patients with INR > 1.7 and planned invasive procedure, >1.4 and need for oral anticoagulant reversal, as part of MTP

            • Cryoprecipitate

              • Contains factor VIII, XIII, fibrinogen and vWF, ADAMTS13

              • 1u/5-7 kg can increase fibrinogen levels by 100 mg/dL

              • Shelf life of 12 months at -25C 

              • Indicated for the correction of hypofibrinogenemia, usually levels < 100-150 mg/dL in conditions such as hemophilia, vWD, DIC, and HELLP

        • Whole Blood Transfusion

          • More than the sum of its parts

          • Whole blood has more Hct (40 vs 30%), more Plts, representation of all factors, and more fibrinogen than component resuscitation. 

  • Coagulation measurement 

    • TEG (Thromboelastography) and ROTEM (rotational Thromboelastomotry)

    • TEG components

      • R time - clot initiation which is heavily dependent on clotting factors and fibrinogen

        • If long consider FFP

      • Alpha Angle and K - Clot propagation dependent on clotting factors and fibrinogen

        • If prolonged K or shallow alpha angle consider FFP and Cryoprecipitate 

      • Max Amplitude - the strength and size of the clot which is dependent on platelets 

        • If low consider giving platelets

      • Lys30 which assess fibrinolysis

        • If long consider giving TXA

R4 Case FOllow up:PPROM and NRP WITH Dr. Crawford

  • PROM Premature rupture of Membranes: 

    • Rupture of membranes before the onset of labor 

  • PPROM = Preterm PROM

    •  complicates 2-3% of pregnancies in the US

    • a leading cause of neonatal morbidity and mortality and is associated with 30% of preterm deliveries. 

    • Pre-Term

      1. The onset of labor before 37 weeks gestation. 

      2. Signs and Symptoms including:

        • regular uterine contractions

        • effacement and dilatation of the cervix

        • Accounts for 10% of all deliveries 

      3. Perform an ultrasound for fetal position

      4. Monitor mother and fetus

      5. Consult an obstetrician for admission 

      6. Consider tocolytics, antibiotics and steroids

  • Risk Factors

    • History of preterm birth

    • Infections

    • Vaginal bleeding

    • Smoking

    • Short cervix 

    • Multiple gestations

  • Diagnosis is confirmed by: (AVOID digital exam to help decrease infection risk (chorioamnionitis)

    • finding a pool of fluid in the posterior fornix

    • pH greater than 7.0 

    • Ferning pattern on smear.

    • Commercial Assays (Amnisure)

    • US looking for oligohydramnios 

  • Treatment (is controversial in OB!)

    • Antibiotics

    • Steroids

    • Betamethasone 12 mg 2x

    • Consider RhoGam

    • Expectant management vs intervention

  • Treatment overall depends on:

    • Gestational age

    • Presence or absence of maternal/fetal infection

    • Presence or absence of labor

    • Fetal presentation

    • Fetal well-being

    • Expectation of fetal lung maturity based on gestational age

  • Umbilical Prolapse 

    • Umbilical cord prolapse is when the umbilical cord exits the cervical os before the fetal presenting part

    • It is dangerous because compression of the cord results in decreased blood flow and fetal hypoxia

    • Risk Factors: 

      • Malpresentation (breech, transverse, oblique, or unstable lie)

      • Preterm gestational age

      • Low birth weight

      • Rare  0.1%

    • Management involved elevating the presenting part

      • Minimize manipulating a prolapsed cord and avoid exposing it to the cold environment, which may exacerbate poor perfusion

      • Gently replace an overtly prolapsed cord in the vagina and keep it moist with wet gauze

      • Prompt delivery: c-section 

  • Breech 

    • Represents about ~4% of singleton deliveries at term, about 25% of cases before 30 weeks

    • Prematurity, small fetal size, excessive amniotic fluid, multiple pregnancies 

    • Delivery 

      • For frank and complete breech delivery

        1. Let the delivery progress naturally, pulling too early can cause the fetal head to go into extension which will cause issues with the delivery later on 

        2. Allow the baby to be delivered until the umbilicus 

        3. Usually baby is face down and their back is flat

        4. After spontaneous expulsion to the level of the umbilicus, external rotation of each thigh with pressure in the popliteal fossa with delivery of the foot

        5. A towel is wrapped around the fetus for better traction

        6. Apply pressure to the pelvis

        7. Don’t grab the abdomen

        8. When the scapula appears under the symphysis, the operator reaches over the left shoulder, sweeps the arm across the chest and delivers the arm

        9. You may have to rotate side to side to get arm out 

        10. If arms are crossed, there is a good chance the arms will delivery spontaneously 

        11. Delivery of the head is accomplished with continued expulsive forces from above, suprapubic pressure, and gentle traction. 

        12. Cephalic flexion is maintained by pressure (heavy arrow) on the fetal maxilla (not mandible!).

        13. Pull up at a 45 degree angle 

        15. If this is unsuccessful there is a : symphysiotomy to help increase the diameter of the canal

        16. Zavanelli maneuver – the child is replaced back into the uterus to allow for emergent C-section. 

  • Neonatal Resuscitation Program 

    • Cornerstone of NRP is respiratory support, with is different than PALS and ACLS

    • If the answer is no the initial assessment the baby should be brought the the radiant warmer , clamp and cut cord 

    • The Algorithm

      • Position the baby with head in sniffing position to open the airway, consider a shoulder roll

      • If needed clear secretions from the airway , suction, mouth  before nose (M comes before N in the alphabet)

      • Dry and stimulate 

      • If HR > 100 and breathing OK continue to observe

      • If there is labored breathing or cyanosis 

        • Reposition airway and clear airway

        • Apply pulse-ox- remember O2 sats are low at birth and slowly rise 

        • Administer O2 as needed  21-30% FiO2, may need oxygen blender depending on pulse ox

          • Usually starting at 21% and titrating up as needed, 10 LPM to meet target saturations

        • If the baby as persistently low O2 sats or labored breathing, move on to CPAP 

          • If there is apnea or gasping or the HR is less than 100

            • Prepare for PPV

            • Using ”breath” two three, administering a breath each time you say breath 

        • If HR remained less than 100, MR SOPA, considering a more advanced airway, laryngeal mask or ETT

      • If HR less than 60

        • Intubate 

        • Starting chest compression , and then administering epinephrine 

        • Epi every 3-5 minutes with CPR and ventilations 

R2 CPC: Blast Crisis and Leukostasis WITH Dr. Brower and Dr. Minges

Hyperleukocytosis WBC > 50K- 100K

  • Leukostasis = symptomatic hyperleukocytosis (aka Hyperviscosity syndrome) 

  • Pathophys

    • thought to be secondary to two mechanisms: hyperviscosity and local hypoxemia

      • Increased blood viscosity occurs as a direct complication of a large population of leukemic blasts that are less deformable than mature leukocytes resulting in plugs in the microcirculation

      • Local hypoxemia then may be exacerbated by the high metabolic activity of the dividing blasts and the associated production of various cytokines 

  • Presentation

    •  the main clinical symptoms of leukostasis and causes of early death are related to involvement of the central nervous system and lungs, which affect ~40% and ~30% of patients, respectively

    • Neurologic 

      • visual changes, headache, dizziness, tinnitus, gait instability, confusion, somnolence, and coma

      • increased risk of intracranial hemorrhage that persists for at least a week after the reduction of white cell count, possibly secondary to reperfusion injury to areas of the brain that were ischemic from leukostasis

    • Pulmonary 

      • dyspnea and hypoxia with or without diffuse interstitial or alveolar infiltrates on imaging studies

      • ~80 percent of patients are febrile, which may be due to inflammation associated with leukostasis or concurrent infection

    • Less common signs or symptoms 

      • electrocardiographic signs of myocardial ischemia or right ventricular overload, worsening renal insufficiency, priapism, acute limb ischemia, or bowel infarction

  • Management

    • In addition to ABC’s and supportive care, the goals of management are cytoreduction, prevention of tumor lysis syndrome, and antibiotics

      • Cytoreduction 

        • achieved via induction chemotherapy, hydroxyurea, and/or leukapheresis

        • Induction chemotherapy or other immunologic agents is the preferred treatment for symptomatic hyperleukocytosis with leukostasis and typically substantially reduces the WBC count within 24 hours, though also increases the risk of precipitating tumor lysis syndrome

        • Hydroxyurea is typically reserved for patients with asymptomatic hyperleukocytosis who are unable to receive immediate induction chemotherapy

        • leukapheresis remains a controversial adjunct for treatment, with several retrospective analyses demonstrating conflicting evidence on early mortality rates. However, this treatment remains an option for critical patients

      • Prevention of tumor lysis syndrome primarily involves IV fluids, allopurinol or rasburicase for hyperuricemia, correction of electrolyte abnormalities, and potentially renal replacement therapy

      • Patients should be treated with broad-spectrum antibiotics as they are functionally neutropenic. Cefepime monotherapy or in combination with vancomycin is typically the preferred regimen

Pediatrics lecture: Inborn Errors of Metabolism WITH Dr. Yu

  • In-Born Errors of Metabolism

    • Defect in an enzyme or pathway leads to a deficit in the necessary product, build up of toxic substrate or activating of a different or secondary pathway leading to build up of other toxic compounds 

  • Consider in any neonate/infant who is critically ill without known etiology 

    • Often have a period of normalcy, progressing to poor feeding, frequent vomiting, failure to thrive, lethargy, seizures, hypothermia, ALTE/BRUE 

  • Crisis caused by a variety of catabolic stress: acute illness, fever, vomiting/diarrhea, surgery 

  • Types

    •  Protein* 

      • Amino acidopathies 

        • Tyrosinemia 

        • Phenylketonuria 

      • Organic acidemias 

        • Maple syrup urine disease 

      • Urea cycle defects*** 

    •  Glucose*** 

      • Carbohydrate intolerance disorders* 

        • Galactosemia 

        • Fructosemia 

      • Carbohydrate production/utilization disorders 

        • Glycogen storage diseases 

    • Fat 

      • Fatty acid oxidation defects*** 

    • Mitochondrial disorders 

    • Lysosomal Disorders 

    • Peroxisomal Disorders 

    • * present in crisis (catabolic state) before 1 month of age 

    • ***present in crisis after 1 month of age  

  • Evaluation and Management 

    • NPO (don't know what substrate is the culprit) 

    • IV fluids with electrolytes and treatment of hypoglycemia 

      • Normal saline (no lactate ringers because some patients have profoundly elevated lactate) 

    • D10 bolus for hypoglycemia (<40 for newborn, <50 for infants or older, or symptomatic) 

      •  "rule of 50" 

        • D10 x 5ml/kg 

        • D25 x 2ml/kg 

        • D50 x 1ml/kg 

        •  D10 1/2 NS for infants at 1.5x maintenance fluids to maintain BG with goal 120-170 

      •  Need this continuous source of fuel regardless of whether or not they are hypoglycemic to avoid catabolic state 

    • Treatment of acidosis 

      • NPO and Dextrose containing fluids still first line 

      • If need acute stabilization can administer sodium bicarb, but this is a temporary bandaid and will not impact the underlying derangements 

      • Avoid too-rapid correction of acidosis 

    • Treatment of hyperammonemia 

      • NPO 

      • Dextrose and intralipids 

      • Consider ammonia scavengers 

      • If >300, can require dialysis or ECMO 

    • Antibiotics to cover for infection (sepsis always on ddx for these kids, or may be the trigger for the crisis) 

    •  Labs 

      •  ISTAT, glucose, CBC, CMP, ammonia (increase indicates issue with protein metabolism - urea cycle defect), lactate, urinalysis 

  • Resource for metabolic emergencies