Pain, Fever, and ACS in Sickle Cell Disease - PowerPoint PPT Presentation

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Pain, Fever, and ACS in Sickle Cell Disease


Do they have SOB, chest pain, cough (worry about ACS)? If they are male, do they have priapism? ... maybe you have to have chest pain. Or maybe you have to ... – PowerPoint PPT presentation

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Title: Pain, Fever, and ACS in Sickle Cell Disease

Pain, Fever, and ACS in Sickle Cell Disease
  • Susan E. Haynes, MD
  • July 17, 2007

  • Alabama newborn screen tests for sickle cell
  • If a screen is positive, order Hb electrophoresis
    for verification
  • If a child has trait, counsel the family
    regarding risk in future children
  • Check Hb electrophoresis at one year old

  • If diagnosed with sickle cell disease
  • Start prophylactic PCN VK at 125 mg PO BID
    before 2 months old to decrease risk of death
    from pneumococcal infection
  • Refer to Hematology, refer family to Genetics for
  • Give Prevnar (pneumococcal conjugate vaccine) at
    2, 4 and 6 mos old
  • Give influenza vaccine at starting at 6 mos

Infants and Toddlers
  • Start Folic Acid 1 mg PO daily by one year old
  • Give Prevnar (conjugate) at 15 mos
  • Give Pneumovax (polysaccharide) at 2 yo and 5 yo
  • Continue to vaccinate yearly against influenza
  • At 3 yo, change to PCN VK 250 mg PO BID. May stop
    PCN VK at 5 yo.

  • Severity is generally based on Hb variant
  • SS S-betao thalassemia S-beta thalassemia
  • Median life expectancy for HbSS is 45 years, for
    HbSC is 65 years
  • High WBC, low Hb, and VOC crisis in the first
    year of life are the known risk factors for
    severe morbidity

Vaso-Occlusive Crises
  • AKA Pain Crisis

VOC (Vaso-Occlusive Crisis)
  • Triggers
  • Infection
  • Temperature extremes
  • Dehydration
  • Stress
  • Idiopathic

History to obtain
  • Location of pain, severity on 0/10 scale
  • Is this their typical pain or is it different?
  • Do they have significant headache (worry about
  • Do they have SOB, chest pain, cough (worry about
  • If they are male, do they have priapism?
  • Do they have a fever (worry about ACS, sepsis,

Labs to order
  • CBC with differential
  • Dont be surprised if the WBC count is elevated
    due to stress response
  • Reticulocyte count
  • Expect it to be elevated
  • Chem 14

  • Hydration D5 ½ NS with 20 meq KCl/L to run at
    150 maintenance (unless concerns for ACS or for
    long periods of time)
  • Heating packs
  • PT consult for ROM, TENS unit, whirlpool
  • Encourage OOB, incentive spirometry

Oral Pain Medications
  • Acetaminophen with Codeine 120 mg/12 mg/5 mL
    0.5 mg/kg/dose of codeine PO Q 4-6 hours max
    dose 60 mg codeine per dose watch acetaminophen
  • Acetaminophen/Hydrocodone (Lortab) 500 mg/7.5
    mg/15 mL dose depends on weight, from 3.75 mL to
    15 mL PO Q 4-6 hours watch acetaminophen

Parenteral Pain Medications
  • Ketorolac (Toradol) NSAID
  • 0.5mg/kg IV/IM Q6 hours, max dose 30 mg IM, 15 mg
    IV for pediatrics
  • Dont use if concerns for bleeding
  • Dont use with other NSAIDS
  • Caution if renal impairment
  • Watch for gastric irritation
  • Use for 5 days only

Parenteral Pain Medications
  • Nalbuphine (Nubain) opioid agonist/antagonist
  • 0.1-0.3 mg/kg/dose IV Q 2-4 hours
  • Max dose 20 mg
  • Monitor for respiratory depression
  • Good for patients who have pruritis with morphine

  • Parenteral
  • 0.1-0.2 mg/kg/dose SC/IM/IV Q2-4 hours
  • Max dose 15 mg
  • Can give via PCA if 5 years or older, depends on
    childs maturity
  • Counsel family that they cannot push the button!
    This counteracts the built in safety of a PCA.

(No Transcript)
  • Oral
  • MS Contin (extended release)
  • 0.3-0.6 mg/kg PO Q 12 hours
  • Dont chew
  • Comes as 15,30,60 mg
  • MSIR (immediate release)
  • 0.2-0.5 mg/kg PO Q 4-6 hours PRN severe pain
  • Comes as 15 mg, 30 mg pills, or as 10 mg/5mL, 20
    mg/10 mL solution

Transition from IV to PO Morphine
  • Oral dose is 3x the IV dose
  • If a patient is comfortable on Morphine 3 mg IV Q
    3 hours, total daily dose is 24 mg/day
  • Oral dose 3 x 24 72 mg/day
  • Can give MS Contin 30 mg PO Q12 hours
  • Can give MSIR for breakthrough pain 3-5 mg PO Q
    4-6 hours PRN severe pain

Other Medications
  • If patient is on hydroxyurea at home, continue it
    at the same dose
  • Folate 1 mg PO daily
  • Consider transfusion in the case of severe
    anemia typically not done in VOC

  • Functional asplenia from sickling in the
    microcirculation in 90 of patients by age 6
  • unless chronic transfusions which may prolong the
    function of the spleen
  • Increased risk of infection with encapsulated
    organisms (S.pneumo and HIB)

Bottom Line
  • Any patient with sickle cell disease and T101 F
    (38.4 C) gets parenteral antibiotics, even if you
    have a source!

Labs that you must have
  • CBC with differential
  • Reticulocyte count
  • CRP
  • Blood culture
  • Chest X-ray

Labs that you might want
  • CP14
  • UA, urine culture
  • Parvovirus B19 titers if a drop in Hb and
    inappropriately low reticulocyte count
  • Look for elevated IgM, if , needs isolation from
    pregnant women
  • If swollen painful limb, consider osteomyelitis
    (may want an MRI)

  • Ceftriaxone (Rocephin)
  • 50-75 mg/kg/day IV divided Q12-24 hours
  • Vancomycin
  • Consider adding if clinically ill
  • 10 mg/kg/dose IV Q6 hours check trough before
    4th dose, should be 5-15
  • Red Man syndrome stop/slow infusion rate and
    administer Benadryl

Adjusting Vancomycin
  • If you have a patient on Vancomycin, you must
    check the level to avoid renal failure and
  • If level is high (15), hold the dose and recheck
    in 6 hours
  • If normal, make the doses less frequent
  • If still high, continue to hold and consider
    checking BUN/Cr
  • If level is low (and recheck level before 4th new dose

Other Medications
  • Hydroxyurea
  • Continue at the patients home dose
  • Hold if leukopenia, neutropenia, or
    thrombocytopenia is noted
  • Folate 1 mg PO daily

Acute Chest Syndrome
  • AKA new infiltrate on CXR and a fever

Acute Chest Syndrome
  • Definition is debated
  • New infiltrate on CXR (the best film is an old
    film) AND fever
  • Or maybe you have to have chest pain
  • Or maybe you have to have hypoxia
  • Bottom Line keep ACS forefront in your mind if
    your patient has any pulmonary complaints cough,
    SOB, chest pain, chest wall pain, hypoxia on exam

  • Incentive spirometry with older patients (10
    breaths per hour while awake) or have younger
    children blow bubbles
  • Albuterol nebulizer/MDI Q 4 hours ATC
  • Chest PT, Mucomyst nebs
  • Ceftriaxone (Rocephin) IV
  • Azithromycin 10mg/kg PO/IV day one, then 5 mg/kg
    daily for days 2-5
  • Continue Folate and Hydroxyurea

  • When should you give oxygen?
  • When your patient has desaturations
  • Starting oxygen when not needed ( for comfort)
    can decrease reticulocytosis
  • If you have to start oxygen, get an ABG first
    unless oxygen need is urgent

  • When should you give blood?
  • Keep H/H around 10/30
  • Transfuse with leukopoor, sickle trait negative
    PRBCs premedicate with Tylenol and Benadryl
  • Do not transfuse to 12/36 as this can increase
    the risk for stroke
  • Consider exchange transfusion if worsening even
    with H/H 10/30
  • Need double lumen central line Red Cross involved

Consent for Blood
Hydration Status
  • Overhydration can worsen ACS
  • D5 ½ NS with 20 meq KCl/L at 100 maintenance
  • Monitor in and outs closely

  • Children with sickle cell disease and acute chest
    syndrome can get ill very quickly and go into
    respiratory failure
  • When called to evaluate children with sickle cell
    disease with shortness of breath, ALWAYS go to
    the bedside to assess them

Case 1
  • Kevin is a 13 yo BM with Hb SS disease who is
    admitted with left leg pain for the last 2 days,
    unresponsive to Lortab 5mg at home. Pain is
    7/10, nonradiating, and is similar to previous
    pain crises.

  • What further questions?
  • Fever?
  • SOB? Cough?
  • Priapism?
  • HA?
  • Injury or wounds?

  • Well developed, NAD, mild scleral icterus
  • T99, HR80, RR16, BP 110/70, O2 sats 97 RA
  • 1/6 SEM
  • Leg without erythema or wounds, mild TTP, CR brisk

  • H/H 10/30
  • Retic count 5
  • CP14 with T bili mildly elevated

What medications?
  • He weighs 45 kg and has no allergies
  • What else do you want to order?

Hospital course
  • Hospital Day 2 You are called at 0200 his leg
    pain is 2/10 but he has abdominal pain.
  • What do you want to do?
  • Hospital Day 3 his pain is 2/10 and he is
    tolerating food well.
  • What do you want to do?

Hospital Course
  • His leg and abdominal pain resolves and you
    discharge him home.
  • What advice should you give his parents?
  • Importance of yearly influenza vaccine
  • Importance of fever
  • Folate 1 mg PO daily
  • Pain management and concerns for addiction

Case 2
  • Alexis is a 2 yo BF with HbSS disease who comes
    to your office as a new patient with T102. Her
    parents report that she has had rhinorrhea for 3
    days and has been pulling at her left ear. She
    has been eating and drinking well and has normal
    UOP. You examine her and diagnose left otitis

What do you want to do?
  • Admit her to the hospital
  • Labs?
  • Medications? She weighs 15 kg and has no

On call
  • You are paged at 0100 on hospital day 2. Alexis
    has vomited once and her parents say that she is
    not acting right. The nurse reports that she
    seems a little sleepy, but it is past her
  • What do you do?

In her room
  • You find Alexis to be sleepy but arousable. She
    is fussy when you awaken her and is somewhat
    consolable by her mother. T104.3, BP is 85/50,
    O2 sats100 RA, RR30. PE CR is 2-3 seconds,
    lungs are clear.
  • What do you do?

  • A nurse calls you with Alexiss Vancomycin level
    of 14 at 2100 the next night. She is worried.
  • What do you do?
  • What if the level was 17?
  • What if the level was 4?

Discharge Advice
  • Yearly influenza vaccine
  • Has she had Pneumovax (due at 2 yo)?
  • PCN VK 125 mg PO BID
  • Folate 1 mg PO daily
  • Follow up in your office

Case 3
  • Tony is a 16 yo BM with HbSS disease who comes
    into the ER with a cough for 24 hours and pain in
    his left chest.
  • He denies fever at home, denies SOB, denies
    priapism. He is on hydroxyurea at home. He has
    received multiple transfusions.

What next?
  • Physical exam!
  • Are there crackles? Is he tachypneic? Is he
    febrile? What is his O2 sat?
  • CXR left lower lobe infiltrate

What next?
  • Medications? He weighs 70 kg and has no
  • Other therapies?
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