Pain, Fever, and ACS in Sickle Cell Disease
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Transcript Pain, Fever, and ACS in Sickle Cell Disease
Pain, Fever, and ACS in Sickle
Cell Disease
Sirish Palle, M.D.
July 26, 2011
Adenine to thymidine at codon 6
in beta Hb on Chromosome 11 leads
to substitution of glutamic acid by valine
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
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
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 & 5 yo
Those that have already received their 4 doses
of Prevnar 7, get additional dose of Prevnar 13
Continue to vaccinate yearly against influenza
At 3 yo, change to PCN VK 250 mg PO BID
May stop PCN VK at 5 yo
≤16yo need TCD to assess for risk of CVA and
need for chronic transfusion to prevent
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 (<7), 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 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)?
Labs to order
CBC with differential
Don’t be surprised if the WBC count is elevated
due to stress response
Reticulocyte count
Expect it to be elevated
Chem 14
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
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
Parenteral Pain Medications
Ketorolac (Toradol): NSAID
0.5mg/kg IV/IM Q6 hours, max dose 30 mg IM,
15 mg IV for pediatrics
Don’t use if concerns for bleeding
Don’t 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
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 child’s maturity
Counsel family that they cannot push the button! This
counteracts the built in safety of a PCA.
Morphine
Oral
MS Contin (extended release)
0.3-0.6 mg/kg PO Q 12 hours
Don’t 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 unless acute
chest, risk of stroke, acute multi-organ failure,
heart failure, reticulocytopenia (eg ParvoB19),
hepatic/splenic sequestration or preoperative
Fever
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
Haemophilus influenzae type b
Streptococcus pneumoniae
Salmonella typhi
Neisseria meningitides
Group B streptococcus
Klebsiella pneumoniae
Bottom Line
Any patient with sickle cell disease
and T>101 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 (MRI)
Medications
Ceftriaxone (Rocephin):
50-75 mg/kg/day IV divided Q12-24 hours
75-100 mg/kg/day IV divided Q12-24 hours if cephalosporin resistant S
pneumo suspected/prevalent in area
Vancomycin:
Consider adding if clinically ill
Commonly used vancomycin doses outside of the neonatal period
range from 40 to 60 mg/kg/d divided every 6 to 8 hours
The exact dose will depend on the clinical presentation and may need to
be altered based on the results of therapeutic monitoring
Level prior to 5th dose – trough
Some evidence suggests that keeping vancomycin trough levels >10
μg/mL may help prevent the development of heteroresistance in MRSA,
level closer to 15-20 in CNS, bone/joint infections and/or suspected
MRSA pulmonary infections
Pediatric Infectious Disease Journal:
May 2010 - Volume 29 - Issue 5 - pp 462-464
Adjusting Vancomycin
Overall, nephrotoxicity in adult patients is estimated to be <5%
However, there are reports of an increased risk of nephrotoxicity
when vancomycin is combined with an aminoglycoside
Use with caution in renal insufficiency (BUN/Creat) & GFR
Although initially reported to be associated with ototoxicity, this
association has been called into question
Many initial reports of ototoxicity occurred in patients with potential
confounding factors (eg, concurrent meningitis and aminoglycoside
use)
Red Man's Syndrome an association of erythema and flushing of
the face, neck, and torso occurs in a significant number of patients
(up to 50% in some series) and is believed to be secondary to
histamine release
stop/slow infusion rate and administer Benadryl
Other Medications
Hydroxyurea:
Continue at the patient’s 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 one or
more of the following:
Fever (>38.5)
Chest pain
Tachypnea, wheezing, cough or appearance of increased work
of breathing (retractions)
Hypoxemia relative to baseline measurements
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
Febrile pt with SCD need xray up to 61% not clinically suspected
prior to radiograph evidence of infiltrate
Repeat CXR on kids in VOC pain crisis if resp symptoms
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 IV or other 3rd generation Cephalosporin
(consider S pneumo resistance)
Azithromycin
10mg/kg PO/IV day one, then 5 mg/kg daily for days 2-5
Continue Folate and Hydroxyurea
Treatment
When should you give oxygen?
When your patient has desaturations <90-92%
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
Keep O2 Sats <95% once O2 started
Treatment
When should you give blood?
Keep H/H around 10/30
Transfuse with leukocyte poor, sickle trait negative
PRBCs; consider extended antigen match in chronic
transfusion patients to avoid alloimmunization
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
If dehydrated, correct with isotonic solution
D5 ½ NS with 20 meq KCl/L at 100%
maintenance for continued fluid needs
Daily weights
Monitor in and outs closely
Adjust fluids as needs
Furosemide if fluid overload suspected
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
Case 1
Kevin is a 13 yo AAM 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.
History
What further questions?
Fever?
SOB? Cough?
Priapism?
HA?
Injury or wounds?
PE
Well developed, NAD, mild scleral icterus
T=99, HR=80, RR=16, BP 110/70, O2 sats
97% RA
1/6 SEM
Leg without erythema or wounds, mild
TTP, CR brisk
Labs
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 to treat the
pt in the hospital?
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 AAF with HbSS disease
who comes to your office as a new patient
with T=102. 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.
What do you want to do?
Admit her to the hospital
Labs?
Medications? She weighs 15 kg and has
no allergies.
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?
In her room…
You find Alexis to be sleepy but awakes
easily. She is fussy when you awaken her
and is somewhat consolable by her
mother. T=104.3, BP is 85/50, O2
sats=100% RA, RR=30. PE: CR is 2-3
seconds, lungs are clear.
What do you do?
Follow-up
A nurse calls you with Alexis’s Vancomycin
level of 14 at 2100 the next night. She is
worried.
What do you do?
What if the level was 22?
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 AAM 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 allergies
Other therapies?