Pediatric Pearl – January

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 Assessment
–          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

Medications
–          Penicillin prophylaxis
–          Hydroxyurea
–          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

LABS

  • 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
    • Reticulocyte count < 1% (unless hemoglobin > 10 g/dL)
  • 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

TREATMENT

Oxygen

  • Recommended onlyif O2 saturation is less than 92%

IVF

    • 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.
PAIN MEDICATIONS
MEDICATION ROUTE DOSAGE COMMENTS
Ketorolac IVP or IM 0.5 mg/kg
(Max 30mg/dose)
Ensure patient has no history of renal insufficiency
Fentanyl IN 0.2 mcg/kg
(Max 100mcg/dose;
Max volume
1ml/nare)
Great for those patients who are in a lot of pain, while you are trying to get IV access.
Morphine IV 0.1-0.15 mg/kg/dose
(Max 8 mg/dose)
Hydromorphone IV 0.015-0.02 mg/kg/dose
(Max 2 mg/dose)
Diphenhydramine PO 1mg/kg/dose
(Max 50mg/dose)
No indication to give IM or IV, unless patient truly can’t tolerate PO, but may try giving PO after giving anti-emetic.
Oxycodone PO < 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.
Tylenol PO 10-15mg/kg/dose
(Max 975mg/dose)
Use as an adjunct to decrease opioid use provided there is no contraindication.
Motrin PO 10 mg/kg/dose
(Max 600mg/dose)
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.)

Steroids

      • 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.

Other adjuncts

      • 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.

https://opioidcalculator.practicalpainmanagement.com/conversion.php

ADMISSION vs. DISCHARGE CRITERIA

Admission:

  • < 3 months of age
  • Focal neurologic findings
  • Acute chest syndrome
  • Splenic sequestration
  • Reticulocyte count < 1 % (unless Hgb > 10 g/dL)
  • Severe anemia (Hgb < 5 g/dL)

Discharge:

  • 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

PEARLS

  • 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.

Physician burnout

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.

Pediatric Pearl

OCTOBER Foreign Body Ingestion – Hima Khamar

FOREIGN BODY INGESTION

By: Hima Khamar M.D., PGY3

HISTORY
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

Symptoms:
FB sensation
Refusing oral intake
Drooling, dysphagia, gagging
Choking or coughing with PO intake
Respiratory symptoms: Stridor, Hoarseness
Chest pain
Abdominal pain, vomiting (signs of perforation, obstruction)
GI Bleeding

PHYSICAL EXAM
Vital signs
Drooling, oral lesions
Tripod position
Neck crepitus, stridor
Wheezing, unequal breath sounds
Check ears and nose, especially if FB not confirmed on X-ray
Signs suggesting acute abdomen

 IMAGING TIPS

AP and lateral view of chest, neck, and abdomen

Flat object location on AP:
-Esophagus: Coin appears circular
-Trachea: Coin appears as a slit

Batteries:
-Double ring on AP view
-Step-off between the anode and cathode on lateral view

Magnets:
-Difficult to reliably distinguish single from multiple magnets

Non-radiopaque FB:
-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
-Endoscopy favored

-CT scan may be considered in special circumstances

WHO NEEDS ENDOSCOPY & WHEN 

EMERGENT URGENT NON-URGENT
Esophageal location
-Button battery – NO DELAY
-Obstructive symptoms
-Respiratory distress
-Significant pain
-Sharp pointed objects
-Multiple magnetsStomach location
-Multiple magnets
Esophageal location
-Minimal symptoms
-Sharp longer objects in stomach with no symptoms
Stomach location
–        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)
—Repeat X-ray:
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

 COINS/BLUNT OBJECTS INGESTION

MAGNETS INGESTION
 

RADIOPAQUE/SHARP RADIOTRANSLUSCENT OBJECT INGESTION

COW #19

Presenting Resident Nicholas Mota, DO PGY3
Chief Complaint Double vision
Brief HPI 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) n/a
Working Diagnosis 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

References
·       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&sectionid=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