MEDICATIONCALCULATI
ONAND
PRI
ORITIZATI
ON
MODULE4HEMATOLOGY
Wh
ati
san
emi
a?
Anemiaisad ef
ici
encyint
henu mberoferyt
hrocyt
es, t
hequant
it
yorqu
ali
tyofhemogl
obi
n
a
nd/
orvol
umeofp a
c k
e dRBCs(hema t
ocr
it
).
• Ulti
matel
yleadtotiss
ueh y
poxiaduetobloodloss,
imp ai
redprodu
cti
onanddest
ruct
ionofe
ryt
hroc
yte
s
-
wh i
chaccount
sf ort
hes i
gnsandsymp t
omsofa nemia.
Cause
:
• Ch roni
cdiseas
e(CHF,cancer,
c h
roni
cr enaldi
sease)
• Bloodloss
• He molyt
icanemia(ac
qu i
red)-
-break
d ownofRBC
Decr
easedRBCProduct
ion E
tio
logy
Dec
rease#ofRBCp r
ecur
s or
s Si
ckl
ece
lla
nemi
a
(Nor
moc y
tic
,nor
mo c
hromi c
) Le
ukemi
a
I
rond
efi
ci
enc
yan
emi
a
De
crea
seHgbSynth
es i
s(Mic
rocyti
c,hyp
ochr
omi
c)
Def
ect
iveDNASy nt
hesis Cobal
amin(
vi
taminB12
)de
fic
ien
cy
(Macr
ocy
tic(
megalobl
ast
ic)normochr
omi
c) Fol
i
ca ci
ddef
ici
ency
Ma
nif
est
ati
ons(
S/S)
Cl
in
ica
l Mi
l
dst
ate
:Hg
b10
-12g
/dLMod
era
tes
tat
e:Hg
b6-
10g
/dLSe
ver
est
ate
:Hg
b<6g
/dL
I
nte
gume
nta
ry Pal
lor(r
e d
uc edHg ba n
dr educedbloodflowtotheskin)Jaundi
ce(hemoly
sisof
RBCresult
ingini ncr
easeds e
rumb il
iru
bin)Pruri
tus(i
ncreas
eds er
uma ndski
nb i
le
sal
tconcentrat
ion s)
Ca
rdi
opulmonar CO2increasesHRa ndSV
y Lowviscosi
tyofb loodleadtohe a
rtmu rmursandb r
uit
s
MI,angi
nap ectori
s,HF,cardi
ome galy,
pu l
mon a
ryands ys
temiccongest
ion,
ascit
es,
andperi
pherale demama yd eve
lopifhea r
tisover
workedforanexten
d e
dp eri
odof
ti
me.
Tachyca
rdia
Tachyp
ne a,or
th opnea,dy
sp ne
aa trest
Pul
mo nary
Short
nessofb re a
th
Ne
urol
ogi
cal He
ada
che
s,v
ert
igo,
ir
ri
tab
il
it
y,d
epr
ess
ion
,imp
air
edt
hou
ghp
roc
ess
GI Anor
exi
a,h
epa
tomegal
y,s
ple
nome
gal
y,d
iff
icu
ltys
wal
l
owi
ng,
sor
emou
th(
glos
sit
is
an
ds moot
hpr
uri
tus
)
,Mu
scu
los
kel
eta
l Bon
epa
in
Ge
ner
al F
ati
gue,se
nsit
ivi
tytocol
d,weightl
oss,
let
har
gy
Nur
sin
gImpl
ementat
ion
• Acut ei
nter
vent
ionma yincl
ude
• bl
oodtransfusi
on(st
ay15mi ns
)*
• dr
ugtherapy(e.
g.,er
yth
ropoi
et
in,v
it
amins
upp
leme
nts
)
• RBCrepl
ac e
me nt
• O2thera
py
• Adequat en u
tri
ti
onali
n t
ake
• Safe
tycaut
ionstop r
eventc
ardi
opul
mon ar
yst
res
s,f
all
s,a
ndi
nj
ury
• Foodsourc
et opromoteRBCma t
ura
tion
:
• Avocadoes,r
edme at(
ri
chinvi
tb12)
Si
ck l
eCel
lDi
sea
se:
Cau
ses
,la
bte
sts
,as
ses
sme
ntf
ind
ing
s,t
rea
tme
nti
ncl
udi
ngme
dic
ati
ons
,
c
omp
li
c a
tions
Agrou
pofin
heri
te
d,aut
osomal
rec
essi
vedi
sor
derc
har
act
eri
zedb
yab
nor
mal
for
mofHg
bin
RBCaHg
bSmu s
tbein
heri
te
dfrombot
hpare
nts
E
tiol
ogy(CAUSE )
• Tri
g ger
edb ylowO2t ensi
oninb l
ood
• Er
y thr
ocytetosti
ffe
na ndelon gat
eat aki
ngonas i
ckl
eds hape
d
• Hyp oxi
aord e
oxyge n
ati
onoft heRBCsc au
sedb y:
• viralorb a
cteri
a li
nfecti
on,hi
gha l
ti
tude, e
mot i
onalorphysic
alstr
ess,surg
e r
y,andblood
l
oss,d ehydr
ation,i
ncreasedhydrogenc oncent
rati
on(acidosi
s),i
ncr
e as
edp l
asma
osmola l
i
ty,decreasedp l
asmav ol
umea n dlowb odytemp.
• SicklecellCrisi
s:
• Sever epainf
ul, a
cutee xac
erbat
ionofRBCs ick
li
n gcausi
ngv as
o¬occ l
usi
vecris
is
• Vasospasmar e s
tri
cti
ngb l
oodflowat iss
u eisc
h e
mi a
,inf
arct
ion,andnecros
is(la
ck
ofox y
ge n)ash ock(O2de pl
eti
ona ndredu ce
dflui
dc i
rcul
ati
on)
Cli
ni
cal
Man i
festa
tions
• Pr
ima r
ysymp tom: Pa i
ndu etotissuehypoxi
a
• Episodesa reaccomp aniedbyfever,
swe l
l
ing,
tender
ness
, t
achypn
ea,
HTN,n/v
• Af fect
sthec hest,ab
dome n,ex
tremit
ie
s
• Ti
s suehyp oxi
ainv olv
esthek i
dneysorl
iver
• Pal
lor(checkmu cou sme mb rane
)
• Sk
inma yh avegrayishcas t
• J
aundi
cebecauseofh emolys i
sa n
dp tbecomemor epronetogal
lst
one(Chol
e l
i
thi
asi
s)
• Hepatomegaly,splenome ga l
y(notspleni
ca t
rophy)
,hyper
bil
ir
ubi
n emia
, Br
ai
n E
ye
•Th
rombos
isorhemorr
hag
e •H
emorrh
age
ca
usi
ngpar
a l
y
si
s,sen
sor
y •R
eti
na
ldet
ach
men
t
def
i
ci
ts
,de
ath •B
li
nd
ness
•R
eti
no
pal
hy
Lu
ng H
ear
t
*A
cut
eche
sts
ynd
rome *H
ear
tfa
il
ur
e
*P
ul
mona
ryhy
pert
ens
i
on
*Pn
eumo
ni
a
Spl
een
L
i
verandga
ll
bl
add
er
• Spl
en
ic at
ro
phy
•Hep
ato
megal
y
(
aul
os
pt
enect
omy
)
*Gal
st
ones
Ki
dn
ey
*H
emat
uri
a
*R
ena
lfa
il
ur
e
Bonesandj
oi
nt
s P
eni
s
•Ha
nd-f
oots
yndr
ome *P
ri
api
s
m
•Ost
eon
ecro
si
s
Sk
in
*St
as
isul
c
ersof
ha
nds,
ankl
es
,an
dfe
el
Compl
icat
ion
s
W/repeat
edepis
od e
sofsic
kli
ng,th
ereisgraduali
nvol
vementofal
lbodysyste
ms ,
e
spec
ial
lyspl
een,l
ungs
,kidney
,andbra
in(
theor ga
nsi nnee
dofh i
ghO2c onsu
mp t
ion)
Pneumoni
a(mostcommoni nf
ect
ion),
Ac ut
ec he
stsyndro
me( acut
epulmonary
c
ompli
cati
on)
,pri
api
sm(pe r
si
sten
tpeni
leerect
ionthatmayoccurwhenvein
sa r
eoc c
luded
)
Di
agn
ost
ics
tud
ies
Pe
rip
her
alb
loods
mea
rma
yre
vea
lsi
ckl
edc
ell
san
dab
nor
mal
ret
ic
uloc
yte
s
•Thepr
e se
n c
eofHg bScanbedi
ag n
osedbythesi
ckl
i
ngtes
t,whic
hu s
esRBCs(
invi
tr
o)and
exposesthe
mt oadeoxy
genat
ionag
e n
t.
Cl
ini
cal
findi
ngsincl
udehe
molysi
s(j
a u
ndi
ce,el
evat
edseru
mb i
l
iru
binle
vels
)anda
bnor
alla
btest
, r
esul
ts.
Hgbe l
ectr
ophor
esi
sma ybed onet
od e
ter
minetheaboutofhgbS,
Skel
etalX-
ray
sd e
mon st
rat
eb onean
djoin
tdefor
miti
esandflat
ten
ing
MRIsma ybeusedtodxastrokecaus
edbyblock
e dcer
ebral
vessel
sfr
oms
ick
ledc
ell
s.Dop
ple
r
stu
dentsusedtoasses
sforDVT,
ChestX-
raytodxinf
ect
ionororganmalf
unct
ionetc.
I
nte
rpr
ofessi
onalcare
Ptwit
hSCDi sd i
re ct
e dtowards:
Pr eve nt
ionofs e queala
ef romt hedi
se a
s e
Al l
evia t
ingma ni
festati
onf orthecomp l
icat
ionofthed i
seas
eMi n
imizi
ng
e nd-orga nd ama ge
Pr omp t
lytreati
ngs eri
ou ss equel
ae,suchasa c
u t
ec h
e s
tsyndr
ome ,t
hatcan
l
eadtoimme diated ea t
h
Tre
atment
Pr oph yl
a cti
cp enici
l
linbe f
orea ge4
or alh ydr
ox yure
a( chemot herapydrug)toreduce#ofp ai
nfulc
ri
sispacke
dRBC
tran sfusions
s edation,
An alg esi
c s
O2t he r
ap y(tocon t
rolhypox ia
)
or al
/IVf luid
s ,F
lu i
dsa nde l
e ct
rol
yt
esa r
ea dminis
teredtore
duceb l
oodvi
scosi
ty
a
ndma int
ainren alfu nct
ion.
irona ndfolicac i
ds uppl
e me nts
An tibioti
c s
a nti
c oagu l
an t
st opreventDVT
Bl oodt ransfusions,chelationthera
py ,
me dic
ation:hydr
oxure
a,Hgb
Giveflui
ds,ABX( p r
eve ntres
pil
lness)
,aroundth
ec l
ockpai
nme ds(Narc
oti
cs)
Nu
rsi
ngMa nageme nt
Pati
entteachi
ng:
Avoidhigha l
ti
tudes
Ma i
ntai
na dequatefluidinta
ke(2-
3Lor8 -
12g l
asses
)
Treati
nfecti
onsp romp tl
y
Treatpai
n
I
mmu ni
zati
ons:
Pneumov ax,Haemo phil
usinfl
uenza,
inf
lue
n z
a,andhepat
it
is
Chroni
clegu l
cers(commonc omp l
ic
ati
on)
Treate
db yrest,anti
bioti
c s
,warms a
li
nesoaks,debr
ide
me n
t,an
dg r
afti
ng
Pr
iapism
Ma nagedwithp a
inme ds,fl
ui
d,andnif
edi
p i
ne(Pr
ocardi
aai
fnotrel
ie
vedina
fewh oursurol
og i
stshouldbec all
e d
Acutechestsyndrome
Treate
dwi thbroad-sp ectr
uma nt
ibi
oti
cs,
o2a ndfl
uidt
hera
py,andpossi
bly
tra
n s
fu s
ion
Or
a lf
oli
ca ci
dr /
th emolysi
s
ONAND
PRI
ORITIZATI
ON
MODULE4HEMATOLOGY
Wh
ati
san
emi
a?
Anemiaisad ef
ici
encyint
henu mberoferyt
hrocyt
es, t
hequant
it
yorqu
ali
tyofhemogl
obi
n
a
nd/
orvol
umeofp a
c k
e dRBCs(hema t
ocr
it
).
• Ulti
matel
yleadtotiss
ueh y
poxiaduetobloodloss,
imp ai
redprodu
cti
onanddest
ruct
ionofe
ryt
hroc
yte
s
-
wh i
chaccount
sf ort
hes i
gnsandsymp t
omsofa nemia.
Cause
:
• Ch roni
cdiseas
e(CHF,cancer,
c h
roni
cr enaldi
sease)
• Bloodloss
• He molyt
icanemia(ac
qu i
red)-
-break
d ownofRBC
Decr
easedRBCProduct
ion E
tio
logy
Dec
rease#ofRBCp r
ecur
s or
s Si
ckl
ece
lla
nemi
a
(Nor
moc y
tic
,nor
mo c
hromi c
) Le
ukemi
a
I
rond
efi
ci
enc
yan
emi
a
De
crea
seHgbSynth
es i
s(Mic
rocyti
c,hyp
ochr
omi
c)
Def
ect
iveDNASy nt
hesis Cobal
amin(
vi
taminB12
)de
fic
ien
cy
(Macr
ocy
tic(
megalobl
ast
ic)normochr
omi
c) Fol
i
ca ci
ddef
ici
ency
Ma
nif
est
ati
ons(
S/S)
Cl
in
ica
l Mi
l
dst
ate
:Hg
b10
-12g
/dLMod
era
tes
tat
e:Hg
b6-
10g
/dLSe
ver
est
ate
:Hg
b<6g
/dL
I
nte
gume
nta
ry Pal
lor(r
e d
uc edHg ba n
dr educedbloodflowtotheskin)Jaundi
ce(hemoly
sisof
RBCresult
ingini ncr
easeds e
rumb il
iru
bin)Pruri
tus(i
ncreas
eds er
uma ndski
nb i
le
sal
tconcentrat
ion s)
Ca
rdi
opulmonar CO2increasesHRa ndSV
y Lowviscosi
tyofb loodleadtohe a
rtmu rmursandb r
uit
s
MI,angi
nap ectori
s,HF,cardi
ome galy,
pu l
mon a
ryands ys
temiccongest
ion,
ascit
es,
andperi
pherale demama yd eve
lopifhea r
tisover
workedforanexten
d e
dp eri
odof
ti
me.
Tachyca
rdia
Tachyp
ne a,or
th opnea,dy
sp ne
aa trest
Pul
mo nary
Short
nessofb re a
th
Ne
urol
ogi
cal He
ada
che
s,v
ert
igo,
ir
ri
tab
il
it
y,d
epr
ess
ion
,imp
air
edt
hou
ghp
roc
ess
GI Anor
exi
a,h
epa
tomegal
y,s
ple
nome
gal
y,d
iff
icu
ltys
wal
l
owi
ng,
sor
emou
th(
glos
sit
is
an
ds moot
hpr
uri
tus
)
,Mu
scu
los
kel
eta
l Bon
epa
in
Ge
ner
al F
ati
gue,se
nsit
ivi
tytocol
d,weightl
oss,
let
har
gy
Nur
sin
gImpl
ementat
ion
• Acut ei
nter
vent
ionma yincl
ude
• bl
oodtransfusi
on(st
ay15mi ns
)*
• dr
ugtherapy(e.
g.,er
yth
ropoi
et
in,v
it
amins
upp
leme
nts
)
• RBCrepl
ac e
me nt
• O2thera
py
• Adequat en u
tri
ti
onali
n t
ake
• Safe
tycaut
ionstop r
eventc
ardi
opul
mon ar
yst
res
s,f
all
s,a
ndi
nj
ury
• Foodsourc
et opromoteRBCma t
ura
tion
:
• Avocadoes,r
edme at(
ri
chinvi
tb12)
Si
ck l
eCel
lDi
sea
se:
Cau
ses
,la
bte
sts
,as
ses
sme
ntf
ind
ing
s,t
rea
tme
nti
ncl
udi
ngme
dic
ati
ons
,
c
omp
li
c a
tions
Agrou
pofin
heri
te
d,aut
osomal
rec
essi
vedi
sor
derc
har
act
eri
zedb
yab
nor
mal
for
mofHg
bin
RBCaHg
bSmu s
tbein
heri
te
dfrombot
hpare
nts
E
tiol
ogy(CAUSE )
• Tri
g ger
edb ylowO2t ensi
oninb l
ood
• Er
y thr
ocytetosti
ffe
na ndelon gat
eat aki
ngonas i
ckl
eds hape
d
• Hyp oxi
aord e
oxyge n
ati
onoft heRBCsc au
sedb y:
• viralorb a
cteri
a li
nfecti
on,hi
gha l
ti
tude, e
mot i
onalorphysic
alstr
ess,surg
e r
y,andblood
l
oss,d ehydr
ation,i
ncreasedhydrogenc oncent
rati
on(acidosi
s),i
ncr
e as
edp l
asma
osmola l
i
ty,decreasedp l
asmav ol
umea n dlowb odytemp.
• SicklecellCrisi
s:
• Sever epainf
ul, a
cutee xac
erbat
ionofRBCs ick
li
n gcausi
ngv as
o¬occ l
usi
vecris
is
• Vasospasmar e s
tri
cti
ngb l
oodflowat iss
u eisc
h e
mi a
,inf
arct
ion,andnecros
is(la
ck
ofox y
ge n)ash ock(O2de pl
eti
ona ndredu ce
dflui
dc i
rcul
ati
on)
Cli
ni
cal
Man i
festa
tions
• Pr
ima r
ysymp tom: Pa i
ndu etotissuehypoxi
a
• Episodesa reaccomp aniedbyfever,
swe l
l
ing,
tender
ness
, t
achypn
ea,
HTN,n/v
• Af fect
sthec hest,ab
dome n,ex
tremit
ie
s
• Ti
s suehyp oxi
ainv olv
esthek i
dneysorl
iver
• Pal
lor(checkmu cou sme mb rane
)
• Sk
inma yh avegrayishcas t
• J
aundi
cebecauseofh emolys i
sa n
dp tbecomemor epronetogal
lst
one(Chol
e l
i
thi
asi
s)
• Hepatomegaly,splenome ga l
y(notspleni
ca t
rophy)
,hyper
bil
ir
ubi
n emia
, Br
ai
n E
ye
•Th
rombos
isorhemorr
hag
e •H
emorrh
age
ca
usi
ngpar
a l
y
si
s,sen
sor
y •R
eti
na
ldet
ach
men
t
def
i
ci
ts
,de
ath •B
li
nd
ness
•R
eti
no
pal
hy
Lu
ng H
ear
t
*A
cut
eche
sts
ynd
rome *H
ear
tfa
il
ur
e
*P
ul
mona
ryhy
pert
ens
i
on
*Pn
eumo
ni
a
Spl
een
L
i
verandga
ll
bl
add
er
• Spl
en
ic at
ro
phy
•Hep
ato
megal
y
(
aul
os
pt
enect
omy
)
*Gal
st
ones
Ki
dn
ey
*H
emat
uri
a
*R
ena
lfa
il
ur
e
Bonesandj
oi
nt
s P
eni
s
•Ha
nd-f
oots
yndr
ome *P
ri
api
s
m
•Ost
eon
ecro
si
s
Sk
in
*St
as
isul
c
ersof
ha
nds,
ankl
es
,an
dfe
el
Compl
icat
ion
s
W/repeat
edepis
od e
sofsic
kli
ng,th
ereisgraduali
nvol
vementofal
lbodysyste
ms ,
e
spec
ial
lyspl
een,l
ungs
,kidney
,andbra
in(
theor ga
nsi nnee
dofh i
ghO2c onsu
mp t
ion)
Pneumoni
a(mostcommoni nf
ect
ion),
Ac ut
ec he
stsyndro
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rip
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ick
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nte
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lu i
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me dic
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,aroundth
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Nu
rsi
ngMa nageme nt
Pati
entteachi
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Avoidhigha l
ti
tudes
Ma i
ntai
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ke(2-
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12g l
asses
)
Treati
nfecti
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y
Treatpai
n
I
mmu ni
zati
ons:
Pneumov ax,Haemo phil
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inf
lue
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a,andhepat
it
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Chroni
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ic
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Treate
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bioti
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,warms a
li
nesoaks,debr
ide
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afti
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Pr
iapism
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ui
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ocardi
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stshouldbec all
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Acutechestsyndrome
Treate
dwi thbroad-sp ectr
uma nt
ibi
oti
cs,
o2a ndfl
uidt
hera
py,andpossi
bly
tra
n s
fu s
ion
Or
a lf
oli
ca ci
dr /
th emolysi
s
