PAIN MANAGEMENT & VASO-OCCLUSIVE CRISIS IN SICKLE CELL DISEASE
By: Hima Khamar, PGY3 & Dr. Patel, PGY16
Sickle cell disease (SCD) is a common disease process we encounter in the ED. There are multiple complaints related to SCD that patients present with such as: pain, fever, stroke like symptoms, anemia. In this pediatric pearl article, we will discuss pain management during vaso-occlusive crisis (VOC) in the pediatric sickle cell patient.
We as emergency medicine physicians often tend to undertreat pain in SCD patients, we tend to be even more cautious with pediatric patients. The goal of this pediatric pearl article is to emphasize trusting patient reported pain, treating pain adequately, realizing opioids are the main stay for VOC, discussing dosing of various pain medications along with routes, and hopefully making you more comfortable treating VOC in pediatric patients.
Primary Goal on Arrival (provided patient’s ABCs are stable and the patient is neurologically intact): PAIN ASSESSMENT
Assess, document pain – TRUST the PATIENT’S self-reported score
Recent pain medication, dose, time of last dose
Allergies to any medication (if they need Benadryl, ONLY PO, avoid use of IV or IM. DO NOT re-dose with every administration of pain medication. Dose every 4-6 hours as you normally do when using Benadryl.)
Order pain medication immediately (ideally should be administered within 30 mins of arrival)
History and Physical
– Pain typical of previous VOE?
– Location, quality, duration, intensity Prior Hx of Complications of SCD
– Acute chest syndrome
– Aplastic crisis (Parvovirus Infection)
– Documented bacteremia
– Stroke, TIA
– Splenic sequestration
– Gall bladder disease
– Osteomyelitis, septic arthritis
Past Medical History
– Baseline pulse oximetry reading
– Previous admissions, ICU admissions
– Transfusion history
– Vaccination history
– Penicillin prophylaxis
– Folic acid
– Chronic pain medications AllergiesPhysical Exam
– VS, pulse oximetry reading
– General appearance, jaundice
– Pain assessment
– Respiratory, Circulatory, Neurologic status
– Evidence of focal infection
– Spleen size
– Presence of rash or petechiae
CBC w/ differential – determine if there is any hemolytic or inflammatory process
Reticulocyte count – determine if there is any hemolytic or inflammatory process
Type and Screen
Pale, persistent tachycardia, ill appearing
Suspected splenic sequestration
Acute chest syndrome
Focal neurologic findings
Hgb < 5 g/dL or Hgb drop > 2 g/dL from baseline Hgb
Chest Radiograph – new hypoxia, chest pain, clinical suspicion for pneumonia/acute chest syndrome
HCG – any females ≥ 12 years or < 12 with menarche
Blood cultures – if infection is suspected
Recommended onlyif O2 saturation is less than 92%
Recommended: D5 ½ NS.
As clinically indicated for: clinical dehydration/intravascular volume depletion
If need to place patient on maintenance fluids because they are not tolerating adequate PO, then place on hypotonic solution, not hypertonic or isotonic solution. Note: IVF offers no therapeutic benefit for patients with VOE who have no signs of volume depletion.
IVP or IM
Ensure patient has no history of renal insufficiency
Great for those patients who are in a lot of pain, while you are trying to get IV access.
(Max 8 mg/dose)
(Max 2 mg/dose)
No indication to give IM or IV, unless patient truly can’t tolerate PO, but may try giving PO after giving anti-emetic.
< 6 yrs: 0.15 mg/kg/dose up to 2.5 mg
6-12 yrs: 0.2 mg/kg/dose up to 5 mg
> 12 yrs: 0.2 mg/kg/dose up to 10 mg
For outpatient management. Give up to 3 days, until they can see their heme-onc doctor.
Use as an adjunct to decrease opioid use provided there is no contraindication.
Use as an adjunct to decrease opioid use provided there is no contraindication.
Incentive Spirometry – RECOMMENDED FOR ADMITTED PATIENTS
Provide to all patients who are admitted for VOC.
This will help decrease development of acute chest syndrome. Acute chest syndrome normally develops during inpatient stay. (We will discuss this in depth in another pearl.)
Based on studies, high dose methylprednisolone may help in the acute event, but has been associated with high rate of rebound pain after stopping steroids.
Currently, it is not routinely recommended for VOC.
Ketamine – need more studies
In ED recommend 0.25-1 mg/kg; inpatient can place on continuous infusion of 3-5 mcg/kg/min
Has been shown to be effective in some case series, but need more data and large, randomized control trials
Nitrous oxide – needs more studies
May try as another modality to control pain
Has been shown to be effective in some case series, but need more data and large, randomized control trials
Patient controlled analgesia (PCAs) – RECOMMENDED FOR ADMITTED PATIENTS
Pain management, which is under anesthesia can help with setting this up. You can get pager number from the operator. Recommended for patients who are admitted, and pain is not controlled with opioid pushes. Discuss with heme-onc team and may consider starting in ED, especially if patient is not going to get a bed for some time.
Laxatives should be provided around the clock to prevent side effects of opioids.
Antiemetics as needed.
Antipyretics as needed.
CALCULATING PARENTERAL DOSE BASED ON HOME MEDICATIONS
The website listed below can help you convert a patient’s home medication into the parenteral dose you should be starting at in the ED. You can also do it manually using the conversion table provided below. On the right side is an example case on how to use the table. Whenever you are converting opioids, always convert to morphine and then convert to another opioid agent.
Patients with pain relief: After 1 – 3 doses of IV analgesia
Patient continues to be pain free at least 60 minutes after PO analgesia
Discharge with 3 days of PO analgesia until they can follow up with heme-onc doctor
Absence of other complications of sickle cell disease
TRUST the patient’s self-reported pain score
Vital signs can be normal even if patient is in pain, so do not rely on vital signs as an indicator of pain.
There is no lab test to definitively say a patient is having VOC or not.
Acute VOC is considered acute, not chronic pain, and opioids are indicated and should be used to treat pain.
Rapidly initiate analgesic therapy within 30 minutes of triage or within 60 minutes of registration.
In adults and children with SCD and a VOC associated with mild to moderate pain who report relief with NSAIDS in the absence of contraindications to the use of NSAIDS, continue treatment with NSAIDS.
In adults and children with SCD and a VOC associated with severe pain, rapidly initiate treatment with parenteral opioids.
Calculate the parenteral (IV or subcutaneous) opioid dose based on total daily short-acting opioid dose currently being taken at home to manage the VOC.
Administer parenteral opioids using the subcutaneous route when intravenous access is difficult.
Reassess pain and re-administer opioids if necessary for continued severe pain every 15–30 minutes until pain is under control per patient report.
Maintain or consider escalation of the dose by 25 percent until pain is controlled.
Reassess after each dose for pain relief and side effects.
In adults and children with SCD and a VOC, do not use meperidine unless it is the only effective opioid for an individual patient.
In adults and children with a VOC, administer oral NSAIDS as an adjuvant analgesic in the absence of contraindications.
In adults and children with a VOC who require antihistamines for itching secondary to opioid administration, prescribe agents orally, and do not re-administer with each dose of opioid in the acute VOC management phase. Re-administer every 4 to 6 hours if needed.
To reduce the risk of acute chest syndrome in adults and children hospitalized for a VOC. Encourage use of incentive spirometry while awake. Encourage ambulation and activity as soon as possible.
In adults and children with VOC, use adjunctive nonpharmacologic approaches to treat pain such as local heat application and distraction.
In euvolemic adults and children with SCD and a VOC who are unable to drink fluids, provide intravenous hydration at no more than maintenance rate to avoid over-hydration.
Assess all patients with sickle cell disease who present with acute pain to determine whether their pain is being caused by an acute painful sickle cell episode or whether an alternative diagnosis is possible, particularly if pain is reported as atypical by the patient.
Patients who experience three or more acute painful episodes per year that require treatment with parenteral opioids in a medical facility are considered to have severe disease.
As physicians, we all decided to help others. Those of us in Emergency Medicine decided to help others at their most ill, at the most traumatic moment in their lives, or at their wits end as far as where to seek answers. Sometimes we can help, and sometimes we cannot. Regardless of what their reason or our ability to meet their expectations, patients come to offload some of their burdens of life. Maybe they can no longer care for themselves because their sepsis has drained them of all their energy. Or their addiction to substances (despite the number of times you’ve counseled them on breaking their habit) has brought them back to us either for either intoxicant effects or the pangs of being without for too long.
Regardless, we can feel the burden and it manifests as burnout. Burnout is a syndrome exemplified by emotional exhaustion, depersonalization and a sense of lack of accomplishment. It manifests in those whose careers aim at working with others. While stress is a normal part of life and work, when we cannot defuse the stress between shifts thereby allow it to build up will we be in danger of experiencing burnout. The syndrome can lead to us making clouded medical decisions which can have disastrous consequences.
Some studies suggest 1 of 3 physicians are experiencing burnout at any given time. The Medscape Physician Lifestyle Survey shows an increase of self-reported burnout from 39.8% in 2013 to 46% in 2015. Whether we don’t have enough time off between shifts, work too many shifts to pay off loans, or simply keep our feelings inside, it exists and knowledge of it is essential. Many of these habits perhaps are inculcated from medical pedagogy since day one of our education.
But there is a cure. Recognize it. In ourselves. In others. Talk about it. Talk to each other. Be there for one another. Recommend healthy coping strategies. Provide an ear. The National Academy of Medicine has validated tools for you to determine if you are burnt out.
As physicians, we have an obligation to patients. But as humans, we have an obligation to ourselves.
HPI: 26 year old male with no past medical history presents to the Emergency Room with complaints of palpitations. Patient states that while exercising at the gym just prior to arrival, he suddenly developed palpitations along with associated lightheadedness. He reported one similar episode in the past that self-remitted. He denies chest pain, shortness of breath, nausea or vomiting but was experiencing intermittent lightheadedness. He denies taking any medication prior to arrival. Patient states he drinks ETOH infrequently with his last drink several days ago. He denies tobacco or illicit drug use. Patient also denies any family history of heart disease or arrhythmias.
Vitals: HR: 221 BP: 117/74 RR: 20 T: 98.8 Pulse Ox 100% on Room Air
General: Patient is awake, appears uncomfortable
Respiratory: No respiratory distress. Lungs are clear to auscultation.
Cardiovascular: +S1/+S2, tachycardic, irregular. No murmurs, rubs or gallops.
Abdomen: Soft, non-tender, non-distended
Extremities: Palpable, rapid pulses. No cyanosis, clubbing, or edema.
Neurological: Alert and oriented to person, place, time, and situation, following all commands, strength and sensation intact and equal bilaterally.
Lab work and radiography including troponins, complete blood count, complete metabolic panel, thyroid stimulating hormone, chest x-ray, and urine drug screen were all within normal limits. Image 1 is the EKG on presentation. The patient quickly became hypotensive 90/52 and complained of lightheadedness. The patient was quickly sedated and cardioverted with 150 joules. He returned to sinus rhythm at which time the underlying conduction was more apparent.
What EKG abnormalities do you notice?
EMS requests permission to administer 6mg of Adenosine to treat the arrhythmia.
What is your recommended treatment plan?
This EKG demonstrates Wolff-Parkinson-White Syndrome with Atrial Fibrillation.
Advise EMS not to treat with Adenosine or other A-V nodal blocking agents as this may precipitate ventricular tachycardia or ventricular fibrillation.
Wolff-Parkinson-White Syndrome is a pre-excitation syndrome where myogenic bundles known as the Bundles of Kent directly link the atria to the ventricles, bypassing the atrioventricular (AV) node and the organized infranodal system [source: Tintinalli]. Atrial Fibrillation (AFib) in Wolff-Parkinson-White Syndrome (WPW) is a presentation of this syndrome in up to 20% of cases.
Features include an irregularly irregular rhythm, very rapid ventricular rates often in the range of 250 to 300, and widened QRS complexes that differ in duration and morphology from one to the next.The accessory pathway present in patients with WPW allow for the multitude of additional ectopic atrial impulses present in AFib to bypass the AV node and conduct to the ventricles, accounting for the unusually rapid ventricular rates. The variation in QRS duration and morphology can also be attributed to simultaneous conduction through the traditional AV node-His-Purkinje pathway as well as the Bundle of Kent-Ventricle route.
WPW with AFib is often misdiagnosed as a supraventricular tachycardia (SVT), ventricular tachycardia (VT) or atrial fibrillation with a bundle branch block, all of which can be safely treated with AV nodal blockers such as Adenosine, Diltiazem, and Amiodarone. WPW in the presence of atrial fibrillation makes degeneration into ventricular fibrillation more likely and thus synchronized cardioversion, delivering 0.5 – 2 Joules/kilogram, is the first-line treatment. Second-line treatments include Procainamide or Ibutilide whose mechanism prolongs the refractory period of the accessory pathway and whose dosing is weight-based. Stable WPW in the presence of narrow-complex SVT can be treated with the traditional vagal maneuvers, adenosine, and calcium channel- or betablockers [source: Tintinalli]. Patients with WPW should undergo ablation as this syndrome can lead to sudden cardiac death by means of ventricular fibrillation.
Take Home Pearls:
Wolff-Parkinson-White with Atrial Fibrillation presents as an irregularly irregular tachycardia with widened QRS complexes that vary in morphology and result in ventricular rates often greater than 250.
If the QRS complex is widened, avoid use of any AV nodal blockers as they can precipitated ventricular arrhythmias.
Procainamide and synchronized Cardioversion are the treatment of choice for WPW with AFib.
WITNESSED or UNWITNESSED
Time of ingestion
Description of object: Size, Shape, Length, Width Sharp end, similar object for comparison
Last meal time
History of GI anomaly, surgery or disease
Refusing oral intake
Drooling, dysphagia, gagging
Choking or coughing with PO intake
Respiratory symptoms: Stridor, Hoarseness
Abdominal pain, vomiting (signs of perforation, obstruction)
Drooling, oral lesions
Neck crepitus, stridor
Wheezing, unequal breath sounds
Check ears and nose, especially if FB not confirmed on X-ray
Signs suggesting acute abdomen
AP and lateral view of chest, neck, and abdomen
Flat object location on AP:
-Esophagus: Coin appears circular
-Trachea: Coin appears as a slit
-Double ring on AP view
-Step-off between the anode and cathode on lateral view
-Difficult to reliably distinguish single from multiple magnets
-Avoid GI contrast studies for suspected esophageal FBs: May obscure visualization on endoscopy and also increases the risk of aspiration if there is an esophageal FB
-CT scan may be considered in special circumstances
-Sharp longer objects in stomach with no symptoms
– FB > 2cm wide
– FB > 5cm long
BUTTON BATTERY INGESTION
Button Battery Facts
-Serious burns can occur within 2 hrs of ingestion
-Symptoms may be delayed
-If mucosal injury is present after removal, observe for delayed complications (esophageal perforation, TEF, vocal cord paralysis, tracheal stenosis, mediastinitis, aspiration pneumonia, perforation into a large vessel)
-Complications may be delayed weeks, months
-Lithium cell batteries are most frequently involved in esophageal injuries
-Determination of battery diameter prior to removal or passage is unlikely in at least 40% of cases
-Assume hearing aid batteries are < 12 mm
-X-ray overestimates the diameter
Coins/Blunt Objects Facts
-Items within the stomach:
—Width > 2 cm, length > 5 cm (less likely to pass pylorus/duodenum)
Within 2-3 weeks if Age < 2 yrs or Quarter
Within 1 week if Cylindrical battery
Sooner if symptomatic
Items beyond the stomach:
-Return if symptoms
Coins usually appear larger on the X-ray due to magnification
-Quarter à 24 mm
-Nickel à 21 mm
-Penny à 19 mm
-Dime à 18 mm
30-year-old male with no past medical history presented with acute onset left ocular pain and double vision x1d. Patient states that last night he turned off the light and had immediate symptoms. Patient denies using glasses or contact lenses. Patient has never felt this before. Patient has not taken anything for the symptoms. No primary care physician. Denies any associated fever, chills, blurry vision, redness of the eye, discharge from the eye, nausea or vomiting, focal weakness, dysphagia, dysarthria, parasthesias, sob, chest pain, cough, abd pain, trauma.
Pertinent PE and Vitals
T: 36.4 °C HR: 86 RR: 20 BP: 146/79 SpO2: 98%
Constitutional: Alert, awake, comfortable
Head/Face: Normocephalic, atraumatic, no temporal artery tenderness
Eye: OD 2 mm reactive, OS 6 mm nonreactive; APD present; EOMI, no nystagmus; intraocular pressure OD 21, OS error ×3; acuity OD 20/20, OS 20/40
ENT: Moist mucous membranes; Posterior Pharynx is without erythema and exudates, Airway is patent
Neck: ROM is full and without pain, no spinous process tenderness, trachea midline
Cardiovascular: Regular rate, regular rhythm, S1, S2, no murmurs, gallops, or rubs
Respiratory: No respiratory distress, breath sounds are equal bilaterally, no rales, no rhonchi, no wheezing.
Abdomen/Gi: Appears normal, Bowel Sounds present; Soft, non-tender, no guarding or rigidity.
Neuro: Oriented to person, place and time, Lucid thought process, follows commands. Strength 5/5 all extremities, Sensation intact, cerebellar signs absent
Musculoskeletal: Full active ROM without pain, 2+ b/l DP, PT.
Skin: Warm, dry, no rash or injuries
Pertinent Labs, Pertinent Imaging/EKG (if any)
Acute Angle Closure Glaucoma
ED & Hospital Course
Ophthalmology was consulted and requested acetazolamide 500 mg IV, mannitol 50 g IV, Combigan gtt (brimonidine/timolol), and pilocarpine gtt. Pt to f/u in office for YAG iridotomy. Patient was given drops with resultant resolution of anisocoria and APD. Patient had brother pick him up and drive him to ophthalmology office.
Pearls & Takeaways
Ø Glaucoma is characterized by increased intraocular pressure which will compress the optic nerve and can cause vision disturbances and, if left untreated, blindness.
Ø Aqueous humor normally drains from the posterior chamber, through the iris/lens interface into the anterior chamber, through the trabecular meshwork and out through the canal of Schlemm and into the episcleral vein.
Individuals with shallow anterior chambers are more susceptible to closure of the angle because the iris and lens have a greater area of contact with subsequent bowing of the iris, blocking drainage.
Ø Acute angle closure attacks are mostly precipitated by pupillary dilation, increased iris/lens contact, blocked aqueous flow into the anterior chamber and increased posterior chamber pressure that causes worsening bowing or the iris against the canal.
o Pupillary dilators: dim light, mydriatics, antihistamines, anticholinergics, TCAs, adrenergics, emotional stress, antiparkinsonians
Ø Far-sightedness is a risk factor due to anatomic flatter corneas, narrower angles, and shorter Anterior to Posterior lengths.
Ø Family history, Asian descent, female, age (lenses thicken and anterior chambers narrow) are additional risk factors
Ø The increase in IOP will cause corneal edema and cause the cornea to appear hazy and the patient to complain of blurred vision and/or halos.
Ø Conjunctival injection, mid-dilated (5-6mm) pupil, hardness of globe (may use contralateral eye or nose as reference), decreased acuity are some exam findings
Ø Fundoscopic exams are more difficult due to corneal edema but may show a pale, cupped optic disc with cup-to-disc ration > 0.6
Ø Slit lamp will show shallow anterior chamber and corneal cloudiness
Ø Oblique flashlight test: if shadow appears on nasal iris when penlight shone from temporal side with beam parallel to iris angle is narrow (sensitivity 76%, specificity 81%)
Ø IOP > 22 is hallmark.
o Tonopen: Be kind and anesthetize the eye. Instruct patient to look straight ahead. Contact cornea for 10 applanations; you do not need to indent the cornea and it may lead to incorrect readings and corneal injury. Error codes are displayed. Two numbers will appear: the first is the IOP measurement and the second (smaller) number is the statistical confidence indicator (95 is acceptable, repeat if 80 or 80-). Tonopen tip should be cleaned with canned air for 3 seconds and should occur monthly if 10 pts/week.
o Shiotz: Measures via indentation tonometry and is dependent upon scleral rigidity.
§ Check calibration by testing tonometer against spherical mould and 5.5g weight: pointer should read 0.
§ Clean, anesthetize, have patient look straight at fixed point (their own outstretched finger)
§ With 5.5g weight, gently lower tonometer disc onto corneal surface and note reading.
§ If 2 or less, replace weight with 7.5g weight and repeat. If again 2 or less, use 10g weight.
o Goldmann applanation: gold standard
§ Calibrate with the dial at 0mmHg, insert control weight. Set measuring drum at 0mmHg. If prism does not tilt forward and backward with the turning of the measuring drum up to +2 and -2, then calibration required. (Beyond the scope of this review)
§ Anesthetize and instill fluorescein, dab the eyes dry.
§ Patient positions into slit lamp with eyes level with black alignment marker
§ Maximum brightness of light
§ Blue light
§ Touch central surface of cornea with prism and then pull back
§ View cornea through miscroscope. 2 semicircular rings (mires) will be seen.
§ Adjust drum until mires line up. IOP is 10x the drum reading.
Ø TREATMENT: should be in co-ordination with specialist but based on following principle:
o Decrease production
§ Topical Beta-blocker: timolol 0.5% 3 drops q15m x3
§ Topical alpha-2 agonist: brimonidine 0.15% or apraclonidine 1% 3 drops q15m x3
§ Topical CA inhibitor: dorzolamide 2% 3 drops q15m x3
§ Systemic CA inhibitor: acetazolamide 500mg IV/PO
§ Topical anti-inflammatory: prednisolone acetate 1%
o Increase drainage
§ Topical prostaglandin: latanoprost 0.005%
§ Systemic diuretic: mannitol 50g IV
§ Topical muscarinic (miotic): pilocarpine 1-2%
§ Surgical iridotomy (YAG laser typically) is typically curative if done early
· Cordero I. Understanding and caring for a Schiotz tonometer. Community Eye Health. 2014;27(87):57.
· Stevens S. How to measure intraocular pressure: Schiötz tonometry. Community Eye Health. 2008;21(66):34.
· TonoPen Avia User Guide. http://doclibrary.com/MSC167/PRM/68E3892-Rev-J-UG-AVIA4540.pdf
· Shikino K, Hirose Y, Ikusaka M. Oblique Flashlight Test: Lighting Up Acute Angle-Closure Glaucoma. Journal of General Internal Medicine. 2016;31(12):1538. doi:10.1007/s11606-016-3737-8.
· Chang DF. Chapter 2. Ophthalmologic Examination. In: Riordan-Eva P, Cunningham ET, Jr. eds. Vaughan & Asbury’s General Ophthalmology, 18e New York, NY: McGraw-Hill; 2011. http://accessmedicine.mhmedical.com/content.aspx?bookid=387§ionid=40229319. Accessed September 08, 2018.
· Laser iridotomy. http://www.littlerockeye.com/laser-iridotomy/
· Murphy-Crews, M. 2017. Angle Closure Glaucoma. Taming the Sru. http://www.tamingthesru.com/blog/annals-of-b-pod/b-pod-case/angle-closure-glaucoma
HPI: ALS box call to St. Joseph Wayne ED # 1: 92 year old female from nursing home with PMH of Alzheimer Disease and failure to thrive, RUE DVT on Eliquis with complaints of SOB. BiPAP was initiated in the nursing home with minimal improvement. On arrival, Patient was in severe respiratory distress, tripoding and with an O2 saturation of 75% on BiPAP. Patient was tachycardic with initial HR of 130bpm, BP 100/80 with a RR of 30.
Med control: Intubation was advised with 50 mcg of Fentanyl, 18 mg of Etomidate, 75 mg of Succinylcholine, and 1 mg of Ativan for post-intubation sedation. NS IV Fluids were started and patient was to be transported to PCI Center St. Joseph Regional Center Paterson for Cardiac catheterization.
ALS box call to St. Joseph Wayne (SJW) ED #2: Patient los pulses after intubation. CPR was in progress.
Med control: Transfer to SJW, continue ACLS
Patient arrives in SJW with return of spontaneous circulation (ROSC) status-post Epinephrine x 2 and total CPR time 3-4 minutes.
Palpable Carotid Pulses. Endotracheal Tube (ETT) was confirmed
BP 81 / 68 HR140 RR20 T: 96.5 F SaO2 94%.
Intubated, GCS 3T, Pupils 4mm equal and reactive bilaterally
Respiratory exam: Equal but diminished breath sounds throughout.
GI: Abdomen soft
Extremities: 2+ femoral pulses B/L, no peripheral edema.
A blind subxiphoid pericardiocentesis was performed. 50cc of frank blood was aspirated and no coagulation of aspirate was noted. Repeat vitals signs were BP 94 /74; Pulse 103; RR 20; Pulse Ox 98%.
Ultrasound was used to confirm the presence of the guide wire in the pericardial space. A triple lumen was passed over the guide wire and secured into placed. A total of 350cc was aspirated with repeat vitals: BP 111/73; Pulse 74; RR 20; Temp 97.6; Pulse Ox 99%.
A subclavian triple lumen catheter was placed and patient was given 2 PRBCs, 2 FFPs, 10mg IV Vitamin K, and 1L NS. Cardiologist from St. Joseph in Paterson was called and he accepted the transferred to their CCU. Patient was started on Fentanyl and Ativan Drip. Levophed gtt was also started for transport.
While in the CCU, patient was continued on antibiotics and vasopressors for septic shock.
Cardiac ECHO showed LV EF 65 to 70%, impaired relaxation pattern of LV diastolic filling, moderate concentric LVH, moderate pericardial effusion is present, no hemodynamic compromise, echogenic material adherent to the visceral pericardium, especially anteriorly measuring approximately 0.5 cm in thickness.
On Hospital day 2 (HOD), Cardiothoracic was consulted. They determined “patient currently very ill and unable to tolerate OR. Not stable for OR at this time.” Pericardial fluid was sent for evaluation.
Pericardial fluid results: Exudative as LDH 1613 with RBC count 1 million; ANA positive, RF negative.
Patient was additionally seen by palliative care. It was also determined there was no surgical interventions needed for the effusion. Sputum culture grew MRSA.
On Hospital day #4, Patient was following commands and was taken off the vasopressors. She self extubated and failed a trial on BiPAP and was re-intubated.
Patient was extubated on HOD #9 and discharged back to Nursing Home on HOD#15.
EKG post ER interventions: NSR, improved amplitude, prolonged QT
CXR post procedure: Enlarged heart, LLL infiltrate with obscured L hemi-diaphragm, ETT above carina, R subclavian central line, Pericardardial drain
Pearls & Takeaways
o Obstructive shock from impaired cardiac filling due to pericardial accumulation
o As fluid accumulates, pericardial pressure become greater than the cardiac relaxing pressures
o Incidence 2/10,000 in USA.
o TB #1 cause in developing countries. Viral and postsurgical complications #1 cause in developed countries. Differential includes malignancy, infectious including HIV, idiopathic, atrogenic, trauma, uremia, hypothyroid, rheumatologic, and post radiation.
o Symptom development depends on rate of fluid accumulation and amount. Pericardial compliance accommodates a slower rate of volume expansion.
o Signs include persistent tachycardia, JVD, muffled heart sounds, pericardial friction rub, hepatomegaly, peripheral edema, pulsus paradoxus. Hypotension and bradycardia precede cardiac arrest.
o Pulsus paradoxus reportedly present in 98% of tamponade cases even prior to onset of hypotension.
o Pulsus paradoxus (decrease in SBP >10% during inspiration) occurs because the negative intrathoracic pressure during inspiration increases venous return and pulmonary vasculature compliance with subsequent pulmonary vascular pooling. RV engorges with septal shift causing decreased LV filling, SV, and SBP.
o Pulsus paradoxus can be seen on plethysmogram. 1998 study by Frey of 57 PICU patients shows sensitivity of 89% and specificity of 90% to detect pulsus paradoxus 10% change with a pleth variation of 8mm.
o EKG findings include low voltage, tachycardia, electrical alternans, PR depression, nonspecific ST/T changes
o CXR may show enlarged silhouette
o Bedside ECHO
· Trivial is seen only in systole
· Mild < 10mm
· Moderate 10-20mm
· Severe >20mm
§ Collapse of chambers during relaxation
· Atrial collapse precedes ventricular collapse
· Atrial collapse longer than 1/3 of cardiac cycle has 100% sensitivity and specificity of tamponade
· M Mode with EKG tracing through collapsing wall
· RV indentation is more sensitive and specific than pulsus paradoxus
§ IVC size > 20mm is 92% sensitive
· Pericardiocentesis: Indicated when hemodynamic compromise is present
§ US may guide you if necessary: if only RA collapse and RV unaffected, may wait to CTS consult
o Needle aspiration with drain placement for repeat aspiration if re-accumulation occurs
§ Triple lumen catheter is most readily available in the ER though CTS prefers pigtail catheters due to decreased clotting
o Subxiphoid approach (blind or US guided)
§ Insert needle at xiphoid process at 45 degree angle and aim towards tip of left scapula/left areola. Advance slowly until fluid aspirated.
§ US guided may be limited by habitus. Needle will enter pericardium inferior to the RV.
o Parasternal (US)
§ Probe on left parasternal position at 4th intercostal space (Long axis view). Needle should be inserted in-plane with probe at a 45 degree angle. Will enter pericardial space anterior to RV.
§ Preferred over subxiphoid due to closer proximity to effusion and better ability to avoid liver and lung
§ Aspirated blood should not clot due to anticoagulative factors in space
HPI: 46-year-old female w/ PMH of asthma, SLE, RA and seizure disorder presents to the emergency department by BLS for evaluation. Patient states, “I don’t like people accusing me of doing things I didn’t do, I only took 2 Percocet and that’s it!” Patient states that she has been feeling depressed and “everything I do is never good enough for my kids, I buy them car, and I don’t even have car myself.” Patient appears to have multiple scratches over her face and left wrist, but is not willing to elaborate how she sustained them or provide any additional history. Denies any suicidal ideations, homicidal ideations, or hallucinations. There is no known psychiatric history.
Per family and EMS, patient was in her bedroom with the door shut, “not acting right, she was asking for us to pass her the key under the door to get out of the room but there is no lock on the door. She did this once before but it went away, today is more severe.” Pt was found in her bedroom by EMS with 2 empty beer cans and bottles of Percocet nearby. She had a recent URI for which she was taking Zzz-quil.
Working Diagnosis: Anion gap metabolic acidosis, most likely due to ethylene glycol poisoning
ED/Hospital course: In the ED patient became increasingly altered. Upon arrival patient was coherent, AAOx3, tearful. Upon reevaluation she was found in room shaking back and forth talking to herself and banging her hands on the bed rails, more confused, inappropriate in her thoughts and actions. A short time later she was found on the floor next to stretcher and was AAOx1, agitated, combative, not following commands, fighting with staff, incontinent of urine. Lab results returned at this time and the patient was subsequently intubated, received 1 amp sodium bicarbonate prior to intubation and admitted to MICU. Repeat labs in MICU showed persistent anion gap metabolic acidosis with Bicarbonate of 5 and anion gap of 26. She was placed on bicarbonate drip and received emergent HD. After HD, the acidosis improved with a bicarbonate value of 19 afterwards. Additionally placed on fomepizole. Received 2 rounds of HD. Subsequently extubated and transferred to floor. Upon extubation she continued to deny any SI or toxic ingestion. Methanol and ethylene glycol levels (-), however, they were drawn after HD was performed.
Pearls: Brief differential diagnosis of anion gap metabolic acidosis:
Visual symptoms include photophobia, “snowstorm” vision, and blindness
Supportive measures including airway management
Severe acidosis is treated with bicarbonate to reduce diffusion of formate into the CNS and protect the optic nerve
Alcohol dehydrogenase inhibitor (ethanol or fomepizole) to block further metabolism of methanol
Fomepizole Fomepizole (4-methylpyrazole) is preferred iv.
Hemodialysis to remove methanol and formic acid indicated if methanol level >50 mg/dL, metabolic acidosis (arterial pH ≤ 7.25), severe visual or CNS symptoms
Found in antifreeze and brake fluids
Converted by alcohol dehydrogenase to glycolaldehyde which is metabolized to glycolic acid
Glycolic acid is primarily responsible for the anion gap metabolic acidosis; it is metabolized to multiple metabolites including oxalic acid
Oxalic acid forms calcium oxalate crystals in the kidney, brain and liver
Initial CNS changes suggestive of ethanol intoxication progressing over 9-12 hours to possible seizures, stupor, coma; toxicity may progress to pulmonary edema and myocardial dysfunction
Late toxicity is characterized by renal failure
Positive birefringent calcium oxalate crystals in the urine; often absent initially commonly described as “envelope-shaped”
May see hypocalcemia although not always present
Alcohol dehydrogenase inhibitors: Fomepizole or ethanol
Hemodialysis with same indications as methanol above
Thiamine and pyridoxine can be administered to decrease production of oxalic acid
Isopropyl (rubbing) alcohol
More intoxication/CNS depression but generally less severe sequlae than methanol and ethylene glycol 80% is absorbed from the stomach within 30 minutes and metabolized by alcohol dehydrogenase to acetone
Presentation: CNS depression, hypotension in severe cases, hemorrhagic gastritis/tracheobronchitis
Positive serum acetone and acetonuria
Anion gap metabolic acidosis is generally not a feature of isopropanol toxicity (unless you have significant hypotension with subsequent lactic acidosis)
Supportive care, including airway management
Alcohol dehydrogenase inhibitors are not indicated
Hemodialysis indicated for refractory hypotension or serum levels >400-500 mg/dL
Some Additional Points:
Lactic acid on lab analysis may be falsely elevated and vary depending on how the sample is analyzed. You will note that ABG lactic acid and serum lactic acid analyzed by lab are very different values, this is due to the fact that a metabolite of ethylene glycol’s metabolism has a very similar chemical structure to lactic acid, causing a falsely positive elevated lactic acid
The differential diagnosis of anion gap metabolic acidosis listed above is a limited list of the most commonly noted causes of AG metabolic acidosis; a comprehensive list is much broader
An osmolar gap >50 is highly suggestive of toxic alcohol ingestion; a level >100 is virtually pathognomonic—there is really nothing else that can cause such a severely elevated osmolar gap other than toxic alcohol
Case presented by Dr. GregnCassidy
Special thanks to Dr. Kashani for his guidance in preparing this case.
HPI: 7 day old female presents to the Emergency Dept. (ED) after being seen earlier in clinic. Mother is rom Nigeria and arrived to the U.S a few weeks prior to delivery. Prenatal care is unclear. Patient was delivered via C-Section at 39 weeks at another nearby hospital. Both mother and baby spent one day in the hospital after delivery being discharged home. Mother states that since last night, the baby appears to “ not breathing right.” She also hasn’t’ been eating much and is crying more often than usual, during which her lips start to turn blue.
Obvious respiratory distress, hypoxic on RA, saturating in the middle to low 80’s on NC 3 L
HR 176, RR 60. Accuchek 96.
BP: (RA) 69/45 BP (LA) 91/64 BP (LL) 84/64 BP (RL) 71/58
ECG in the ED:
Working Diagnosis: Shock secondary to congenital cardiac pathology.
ED/Hospital course: Patient was admitted to PICU, underwent Echocardiogram and further imaging and was diagnosed with Coarctation of the Aorta. The patient was transferred to Mt. Sinai for operative repair.
7 day olds are terrifying, especially if they are sick.
Take a breath! Start with your ABCs.
Coarctation of Aorta(CoA) is a congenital aortic narrowing which most commonly occurs at the level of ductus arteriosis. There are Pre-ductal and Post-ductal types. Pre-Ductal CoA is a Truncus dependent lesion and most patients present within 2 weeks of birth once duct closes. Aortic narrowing leads to increased LV function and dilation. Post-ductal are commonly identified in adulthood.
Obtain a BP/pulse Ox in all 4 extremities
Perform the Hyperoxia test
Obtain ABG on RA. Then, apply 100% supplemental oxygen with a NRB for 10-15 minutes. Repeat the ABG on the NRB.
On repeat ABG
If PaO2 > 250mmHg = Lung problem
If PaO2 < 100mmHg = Congenital Heart Disease
If PaO2 100-250: Assume the worst situation first
You may also apply supplemental oxygen and assess the infant’s response. If saturation increases to at least 5-10%, it’s most likely a Lung problem. If it remains the same, think about Congenital Heart Disease
Prostaglandin (pt is less than 4 weeks old typically 1-2 weeks of life) start at 0.05 mcg/kg/min.
Dr. Hochman rule #11, call consultants early “don’t be the captain of a sinking ship”
Additional medications: Lasix 1 mg/kg if evidence of volume overload.Ductal dependent lesions, pulmonary presentation cyanosis/hypoxia use phenylephrine . If systemic SX, shock, pulmonary congestion on CXR Milrinone (decrease afterload + inotropic support).