Sickle Cell Anemia-Hospitalist Treatment Protocol
Every admit for sickle cell anemia should have the following parameters analyzed
and treated accordingly. You will find the evidence based explanation in each subheading.
I.
General Prophylaxis
1. Infection control and prophylaxis: Immunizations are a cornerstone
of infection prevention in SCD. Children with SCD should be immunized
against S. pneumoniae, H. influenzae type B, N. meningitides, Hepatitis B
Virus, and Influenza. All children with HbSS should be on Penicillin VK
125mg po BID, this should be continued until the child is 2-3 years of age.
After 2-3 years of age, the oral dose should be increased to 250mg PO BID
until the age of 5. After the age of five years, some parents, with
consultation of their clinician, elect to stop penicillin prophylaxis, while
others will continue. 5 is the cutoff b/c at this point the child has
completed the immunization series with the pneumococcal
polysaccharide vaccine (PPV23).
2. Nutrition: growing evidence suggests that these individuals have
vitamin and micronutrient deficiencies. Therefore, the following meds are
used
-Folate: 1mg PO Q daily; Folate def has been found in SCD pts
numerous times due to an increase in folate consumption by ongoing
hemolytic anemia. Never been shown to increase HCT, but does show dec
in MCV and less dactylitis.
-MVI: Any MVI can be used as long as its without Iron; depletion of
antioxidant vitamins occur from oxidative injury of chronic hemolysis
-Vit D: if found to be Vit D deficient, should supplement with oral vit D
and calcium. These deficiencies contribute to osteopenia and
osteoperosis, which affect 80% of SC pts. There is also an incompletely
understood relationship between Vit D Def and chroni pain in children
with SCD.
3. Hydroxyurea: 15mg/kg/day, if CrCl <60mL/min, the dose should
be 7.5mg/kg/day. The dose is then incrementally increased every 612 weeks by their Hem/Onc. Hydroxyurea increases HbF production;
Mainstay in overall mgmt of individuals with SCD, since it reduces the
incidence of acute painful episodes and hospitalization rates, and
prolongs survival.
4. Pain Control: This refers to chronic pain control, acute pain control is
covered under pain crisis (below). Similar to chronic pain management,
long acting oral morphine, oxycodone, methadone and transdermal
fentanyl. Look up for dosing. Methadone is superior to all others, but is
rarely used.
5. Transcranial Doppler: Date of last Doppler? Should be started at 2, In
children < 16 yoa with hemoglobin SS or hemoglobin S beta thalassemia,
cerebral blood flow should be evaluated by transcranial Doppler (TCD)
annually. This is done b/c children at risk for CVAs can be identified with
this and the incidence of stroke can be reduced by the use of regular
blood transfusion aimed at maintaining hemoglobin S levels <30 %.
You will not give the transfusion in the hospital, but instead will use this
information to pass on to his/her Hem/Onc.
6. Hem/Onc Physician: Need to call and let them know they are in the
hospital for whatever reason and the plan. Mostly listen and learn from
this conversation.
7. Chronic Transfuions: Has the child had chronic transfusions? Do
they have Excessive Iron stores? Chelation therapy needed? The
presence of excessive iron stores from chronic transfusion has resulted in
clinically important hemosiderosis in many SCD pts. These repetitive
transfusions cause hepatic fibrosis and cirrhosis. Serial serum Ferritin
concentrations (since acute phase reactant and elevated in vaso-occlusion
crisis) should be drawn if pt has had transfusions for the past 2 years. If
the ferritin is >1500 g/L, need chelation. Chelation is done with
Deferoxamine (look up dosing schedule) or Deferasirox (PO iron chelator
that has similar efficacy to deferoxamine in dec iron stores in liver)
II.
Pain Crisis (Vaso-Occlusion Crisis)
1. Optimal Hydration: very important in children
2. Aggressive Pain Relief: If not on chronic pain management, IV
morphine sulfate or IV hydromorphone (Dilaudid). If pain not relieved
with intermittent dosing, a continuous infusion of morphine or
dilaudid, preferably PCA, can be given. Also, Toradol is a great choice.
If renal or hepatic dysfunction, fentanyl is used.
3. Blood Transfusion: NOT INDICATED TO ABATE THE ACUTE PAIN
EPISODE; indicated ONLY if pt hemodynamically unstable in a vasoocclusion crisis (see transfusion below)
III.
Acute on Chronic Anemia: Only 3 causes
1. Aplastic anemia: retic count <1.0%; Parvovirus B19. Must give
transfusion and reticulocytes will typically appear in 2-14 days.
2. Splenic sequesteration crisis: retic count elevated above baseline,
thrombocytopenia and rapidly enlarging spleen; must give
transfusion but only ½ of normal transfusion (so ½ of recommended
dose); remove spleen after this occurs for the first time b/c is
recurrent in 50%
3. Hyperhemolytic crisis: retic count elevated above baseline but
spleen WNL; transfusion needed
IV.
V.
Acute Chest Syndrome
a. Adequate Analgesia: Spine, thoracic, and abdominal pain in
important to prevent hypoventilation and atelectasis. Ketorolac
should be used first line. Morphine or Fentanyl if NSAID not enough,
but remember will suppress respiratory system and cause more
hypoventilation with atelectasis. Dose conservatively.
b. Respiratory Support: keep O2% > 92, do this with supplemental O2,
frequent incentive spirometry, and CPAP (if needed); Bronchodilatory
if hx of RAD or wheezing on exam; Short course steroids if
SYMPTOMATIC ASTHMA, but should be tapered to reduce the risk of a
vasoocclusive rebound phenomenon.
c. Infections: is the MCC of ACS, so all pts need rocephin and
azithromycin
d. Transfusion: Transfusion therapy is used to improve oxygenation
and should be considered, weight pros vs cons; good rule is if
respiratory failure is imminent, ACS is worsening, or the Hct
continues to drop despite other interventions listed above=>
transfuse.
e. Long Term Management: decrease risk of infection (prophy
penicillin, immunizations), manage RAD/Asthma, decrease risk of RBC
sickling (hydroxyurea and transfusion)
Blood Transfusion
a. Therapeutic: acute stroke, acute multi organ failure, splenic/hepatic
sequesteration, acute chest syndrome with clinical deterioration
and/or respiratory failure; acute symptomatic anemia with or without
reticulocytopenia
b. Prophylactic: In the Stroke Prevention Trial (STOP), which randomly
assigned children with SCD and Abnl transcranial Doppler U/S to
receive chronic transfusion or obs, transfusion reduced risk of stroke,
reduced rates of hospitalization for ACS and acute pain episodes. Also,
those who got the transfusion for 2 years had improved growth. With
that being said here are the following guidelines:
i. Transfusion therapy to prevent pain or acs episodes in
severely affected SCD: option for select pts. NEVER USE IN
UNCOMPLICATED PAIN episodes
ii. Pre-operative pts: goal Hgb of 10 mg/dL
iii. Primary Stroke Prevention: if mean velocity > 200 cm/sec
on 2 TCD studies within a 2-4 week period, they should get
prophylactic transfusions. Once entered into the prophylactic
transfusion program, it should usually be continued
indefinitely unless other preventative measures are taken, b/c
the risk of stroke returns if transfusion are stopped.
iv. Secondary Stroke Prevention: Exchange transfusion is used
with a goal of achieving a HbS fraction of <30 % and a
VI.
hemoglobin level of approx 10 mg/dL (NOT GREATER).
Chronic transfusion therapy should be continued.
Fever: If fever > 101.5 F by history or measurement while in ER, the
following should be done:
a. Labs: CBC with diff, Blood culture, and reitc count; LP if suspected
meningitis, UA and culture if symptoms of UTI; type and cross match
b. Empiric Parenteral ABx: after labs done, start:
i. Rocephin
ii. Vancomycin (if hemodynamicall unstable or suspected
meningitis)
iii. Clindamycin (if allergic to cephalosporins)
c. Admit to the Hospital: can be managed out patient, but we admit all
SCD pts with fever
How to Put This All Together:
1. Are they taking all their meds that are standard of care? If not, be sure
they are started at admit or discharge.
2. Are they UTD on their immunizations? If not, give what they need while
inpt.
3. Are they UTD on their transcranial Doppler? If not, order before D/c.
4. Are they admitted for a vaso-occlusion (pain) crisis? If so, optimize
hydration, control pain, and transfuse if hemodynamically stable OR
have acute on chronic anemia.
5. Do they have an acute severe anemia on their chronic hemolytic
anemia? If so, transfuse. See “Pedi transfusion guidelines”. Define
etiology.
6. Do they have any finding on CXR? If so, treat for ACS
7. Do they have an acute severe anemia? Are they Pre-Op? Do they have
acute chest syndrome? Do they have multiorgan failure? Respiratory
Failure? If so, transfuse to a goal Hgb of 10 mg/dL.
8. Do they have a Fever? If so get labs and start abx