Pain, Fever, and ACS in Sickle Cell Disease

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Pain, Fever, and ACS in Sickle Cell Disease

• Susan E. Haynes, MD
• July 17, 2007

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Identification

• Alabama newborn screen tests for sickle cell
  disease
• 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

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Newborns

• 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
  counseling
• Give Prevnar (pneumococcal conjugate vaccine) at
  2, 4 and 6 mos old
• Give influenza vaccine at starting at 6 mos

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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.

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Outcomes

• Severity is generally based on Hb variant
• SS S-betao thalassemia S-beta thalassemia
  SC
• 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

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Vaso-Occlusive Crises

• AKA Pain Crisis

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VOC (Vaso-Occlusive Crisis)

• Triggers
• Infection
• Temperature extremes
• Dehydration
• Stress
• Idiopathic

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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
  stroke)?
• Do they have SOB, chest pain, cough (worry about
  ACS)?
• If they are male, do they have priapism?
• Do they have a fever (worry about ACS, sepsis,
  osteomyelitis)?

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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

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Therapies

• 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

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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
  component
• 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
  component

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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

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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

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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.

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Morphine

• 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

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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

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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

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Fever
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Fever

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

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Bottom Line

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

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Labs that you must have

• CBC with differential
• Reticulocyte count
• CRP
• Blood culture
• Chest X-ray

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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)

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Medications

• 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

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Adjusting Vancomycin

• If you have a patient on Vancomycin, you must
  check the level to avoid renal failure and
  deafness
• 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

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Other Medications

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

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Acute Chest Syndrome

• AKA new infiltrate on CXR and a fever

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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

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Treatment

• 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

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Treatment

• 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

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Treatment

• 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

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Consent for Blood
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Hydration Status

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

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Remember

• 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

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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.

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History

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

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PE

• 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

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Labs

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

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What medications?

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

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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?

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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

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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
  media.

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What do you want to do?

• Admit her to the hospital
• Labs?
• Medications? She weighs 15 kg and has no
  allergies.

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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
  bedtime.
• What do you do?

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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?

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Follow-up

• 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?

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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

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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.

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What next?

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

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What next?

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