semifinals 1

8/19/2019 Semifinals 1

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NURSING CARE OF A FAMILY

EXPERIENCING POSTPARTALCOMPLICATIONSBY: AMPE C. ASUNCION, R.N., M.A.N.


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Problems with the Psyche

Ab!"#o"me"t !tte"tio" h!s shi$te# $romthe clie"t to the b!by% $!ther m!y com&l!i"th!t the clie"t h!s "o time $or him

'is!&&oi"tme"t b!by #oes "ot t(r" o(t !se)&ecte#% comme"t o" the chil#*s +oo#&oi"ts

Post&!rt!l ,l(es -./.0 #!ys A,1 occ(rs i"

20 3 o$ !ll births% te!r$(l"ess4 $eeli"+s o$i"!#e5(!cy4 moo# l!bility4 !"ore)i!4 !"#slee& #ist(rb!"ce% #(e to hormo"!l ch!"+es

or stress o$ li$e ch!"+es6


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Ofer support, empathy, compassionand understanding

POSTPARTAL DEPRESSION (1-12months AB):(p. 695)Woman experiences overwhelming eeling o

sadness, ina!ility to stop crying, increased anxietya!out her own and inant"s health, insecurity,psychosomatic symptoms, and either depressive ormanic mood #uctuations.$ssess or hx. o previous depression, hormonal

response or lac% o social support.&rovide counselling or drug therapy'is% actors include hx o depression, trou!ledchildhood, low sel esteem, stress at homewor%,

lac% o efective support people


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POSTPARTAL PSYC7OSIS Occ(rs withi" . ye!r !$ter birth

'el(sio"s or h!ll(ci"!tio"s o$ h!rmi"+ i"$!"tor sel$

.3/83 o$ !ll births

Possible !cti9!tio" o$ me"t!l ill"ess4 hormo"!lch!"+es4 $!mily h) o$ bi&ol!r #isor#er

Loss o$ co"t!ct with re!lity4 wom!" m!y #e"y

bei"+ &re+"!"t Pro9i#e &sychother!&y4 #r(+ ther!&y4 re$er to

&sychi!tric c!re

S!$e+(!r# mother $rom i":(ry to sel$ or to

"ewbor"% "e9er le!9e wom!" !lo"e with chil#


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POSTPARTAL 7EMORR7AGE -&6;2;1

Possible threat throughout pregnancy andimmediate postpartal period.

Blood loss from uterus 500- 1,000 ml/24hours

1 !arly- "rst 24 hours

2 #ate- any time after the "rst 24 hoursduring the remaining days of the $ %ee&-

PPP.

''' (auses are uterine atony, lacerations,

retained placental fragments, uterine


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Uteri"e Ato"y

Most $re5(e"t c!(se o$ (teri"e blee#i"+

Co"#itio"s th!t i"cre!se ris< $or PP7

Co"#itio"s th!t c!(se O9er#iste"sio" o$(ter(s

Co"#itio"s th!t c!(se cer9ic!l or (teri"el!cer!tio"s

Co"#itio" o$ 9!rie# &l!ce"t!l site or

!tt!chme"t I"!bility o$ (ter(s to co"tr!ct

I"!#e5(!te bloo# co!+(l!tio"


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N(rsi"+ #i!+"oses $or hemorrh!+e

='e>cie"t ?(i# 9ol(me rel!te# to e)cessi9ebloo# loss !$ter birth

/ &eri"e!l &!# s!t(r!tio" is !bo(t 8@/@0 ml

-8@/@0+1

NURSING INTERENTIONS

.1Pre9e"tio" is &!l&!ti"+ the $("#(s !tre+(l!r i"ter9!ls4 sho(l# o"ly be #o"e whe"

$("#(s is bo++y681Assess $("#ic hei+ht !"# lochi! re+(l!rly $or

the "e)t B ho(rs


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1 'il(te o)ytoci" is (s(!lly or#ere# .0/B0UD.L4 $ollowe# by 7em!b!te re&e!te# 5.@/0mi"(tes (& to #oses4 Mether+i"e4e9ery 8/

B ho(rs (& to @ #oses6B1 Rect!l Miso&rostol is +i9e"

@1 Oer be#&!" to em&ty bl!##er

;1 O)y+e" by m!s< !t B LDmi" $or res&ir!tory#istress

21,im!"(!l com&ressio" to #etect ret!i"e#

&l!ce"t!l $r!+me"ts1Prost!+l!"#i" !#mi"istr!tio"

1,loo# tr!"s$(sio"


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S(bi"9ol(tio"

Occ(rs !t B/; wee


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*) W+- O/ &O/0&$'0$1 213/ (&&2)4OO-1 O443'7

8) W+$0 / 0+ &'$'4$3/ O: &&27

;) W+- O/ &O/0&$'0$1 &/4+O//(&&&)O443'7

-0O- :O' &&&7

6) $O3-0 O: 21OO 1O// -&O/0&$'0$1 +O''+$=

?,@,9 4$3// O: &O/0&$'0$1

+O''+$=


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*A) O/0 :'B3-0 4$3/ O: 30'-21-=

**) => O- 4$3/ O: 4'>4$1

1$4'$0O- &O/0&$'03

*8) => O- C$&1 O: $ &1$4-0$14O-0O-0+$0 4$- 4$3/ 21-=

&O/0&$'03*;) &'O'0 -/= -0'>-0O- :O' &&

+O''+$=

*


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*?)-&--0 -3'/-=-0'>-0O- 0O $ - 30'-4O-0'$40O- (-O0 $//$=)

*@) O/ O: 0+'=- =>- :O'/32->O130O-

*98A) 4$3// O: /32->O130O-


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P(er&er!l I"$ectio" -&;81 Pro+"osis #e&e"#s o"

›

ir(le"ce o$ i"9!#i"+ microbes› Hom!"*s +e"er!l he!lth

› Port!l o$ e"try

› 'e+ree o$ i"9ol(tio" !t time o$ i"$ectio"

› Prese"ce o$ l!cer!tio"s i" re&ro#(cti9e tr!ct

.1 E"#ometritis-2/.0 #!ys1 !ssoci!te# withchorio!m"io"itis !"# CS% $e9er e9i#e"t o" the r#

or Bth #!y% =.006B*F $or 8 co"sec(ti9e 8B ho(rs6/ Cli"#!myci"4 Mether+i"e !"# !"!l+esics !re+i9e"% !mb(l!te clie"t to #r!i" lochi!% t(b!lsc!rri"+ !"# $(t(re i"$ertility m!y occ(r i$

("tre!te#6


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81 Ho("# i"$ectio" &!i"4 he!t !"# swelli"+ is&rese"t% $e9er m!y or m!y "ot be &rese"t6

/ s(t(re m!y be remo9e# to !llow #r!i"!+e6

/ !"tibiotics !"# !"!l+esics m!y be +i9e"

/Sit b!th4 moist w!rm com&ress !&&lic!tio"

/Fre5(e"t ch!"+e o$ &eri&!#s !"#

h!"#w!shi"+ sho(l# be #o"e

/ wi&e $rom $ro"t to b!c< to !9oi# $(rtherco"t!mi"!tio"

1 Throm&hlebitis i"?!mm!tio" with$orm!tio" o$ bloo# clots


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C!(ses o$ Thrombo&hlebitis

= i"cre!se# >bri"o+e" le9els c!(si"+i"cre!se# clotti"+

'il!t!tio" o$ lower e)tremity 9ei"s #(e to&ress(re o$ $et!l he!#

I"!cti9ity or (se o$ stirr(&s m!y le!# to&ooli"+4 st!sis !"# clotti"+ o$ bloo# i"e)tremities

Obesity #(e to i"!cti9ity !"# wei+ht +!i" Ci+!rette smo


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Pre9e"tio" o$ TP

.1 Goo# !se&tic tech"i5(e

81 E!rly !mb(l!tio"

1 Limit stirr(&s (se !"# (se them well/&!##e#

B1 He!r s(&&ort stocrst 8 w


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N(rsi"+ I"ter9e"tio"s $or Femor!l TP

.1Ele9!te le+ !"# (se be# cr!#le4 "o wri"


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Pel9ic TP -&;1

/ I"9ol9es the o9!ri!"4 (teri"e or hy&o+!stric

9ei"s !"# $ollows ! mil# e"#ometritis !"#occ(rs l!ter th!" $emor!l TP4 o$te" !ro("#the .B/.@th #!y PP6

/ clie"t e)hibits s(##e" ill"ess4 hi+h $e9er4chills4 +e"er!l m!l!ise6

/ Se9ere i"$ectio" c!" le!# to 9ei" "ecrosis!"# &el9ic !bscess

/ Systemic i"$ectio" c!" c!(se l("+4 he!rt4 or


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'ise!se r("s its co(rse $or ;/ wee


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Uri"!ry Tr!ct I"$ectio" -&6;81

C!theteri!tio" i"cre!ses ris< $or #e9t o$ UTI6

Sym&toms i"cl(#e b(r"i"+ o" (ri"!tio"4hem!t(ri!4 $re5(e"cy4 lower !b#omi"!l &!i"4low/ +r!#e $e9er6

N(rsi"+ I"ter9e"tio"s

Amo)icilli" or Am&icilli" is +i9e" !s or#ere# $or@/2 #!ys to com&letely er!#ic!te the i"$ectio"

O"e +l!ss o$ w!terDhr to ?(sh o(t to)i"s

Acet!mi"o&he" to re#(ce #ys(ri!

" er y ( er y &


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" er y ( er y &6.2.1

Hhe" &re+"!"cy #oes "ot occ(r !$ter . ye!r o$

("&rotecte# se).1 Prim!ry/ "o &re9io(s co"ce&tio"s h!9e occ(rre#

81 Seco"#!ry/ &re9io(s 9i!ble &re+"!"cy b(t co(&leis ("!ble to co"cei9e !t &rese"t

M!le F!ctors

I"!#e5(!te s&erm co("t -NJ 80 MDml or @0 MDe:

@03 sho(l# be motile4 03"orm!l i" sh!&e !"#$orm

Chro"ic i"$ectio"s li


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I"cre!se# scrot!l he!t -#es< :obs4 #ri9i"+4$re5(e"t (se o$ hot t(bs1

Co"+e"it!l !"om!lies -cry&torchi#ism or

9!ricocele1 Im&!ire# s&erm motility #(e to obstr(ctio"s

c!(se# by m(m&s4 orchitis4 +o"orrhe!4

!sce"#i"+ i"$ectio"s4 9!sectomies4 hy&oDe&is&!#i!s

Erectile #ys$("ctio"s -&rim!ry/ "e9er!chie9e# erectio" !"# e:!c(l!tio"Dseco"#!ry/&rese"t #iKc(lty i" !chie9i"+e:!c(l!tio"

Prem!t(re e:!c(l!tio"/ "orm!l i"

!#olesce"ts


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F l F t $ S b$ tilit


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Fem!le F!ctors o$ S(b$ertility

*) $novulation most commo" c!(se% m!y be#(e to T(r"er*s sy"#rome -hy&o+o"!#ism1 or!bse"ce o$ o9!ries% hy&othyroi#ism% o9!ri!"t(mors% e)&os(re to X/r!ys% 9!ri!tio"s i"o9(l!tory &!tter"s

8) 0u!al transport pro!lems #(e to t(b!lsc!rri"+ #(e to !&&e"#ectomy4 !b#omi"!ls(r+ery or i"$ectio" or ,TL% PI' #(e to ST' or!$ter me"ses whe" b!cteri!l +rowth is hi+hest4

or IU' (se;) 3terine pro!lems c!(se# by >brom!s4

#e$ormities4 e"#ometriosis or "o#(les i"'o(+l!s* c(l/#e/s!c4 o9!ries4 (teri"e li+!me"ts4

!"# o(ter s(r$!ces o$ (ter(s !"# bowel


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B1 4ervical pro!lems c!(se# by "o"


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B1 4ervical pro!lems c!(se# by "o"/sy"chro"i!tio" o$ coit(s with time o$o9(l!tio" -.8/28 ho(rs14 i"?!mm!tio" o$

cer9i)4 obstr(ctio" or ti+hte"i"+ o$ os4m(lti&le 'C4 cer9ic!l co"i!tio"

@1 >aginal pro!lems i"$ectio" o$ the 9!+i"!

c!(si"+ !ci#otic &74 s&erm/ immobilii"+bloo# &l!sm!-a! 846)

iagnostic testsD

.1 Assessme"t o$ he!lth history/ #iet4e)ercise4 !lcohol4 #r(+4 tob!cco (se4co"+e"it!l &roblems4 #ise!ses4

co"tr!ce&ti9e (se4 se)(!l &r!ctices4 (se o$


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81 &hysical exam to r(le o(t obstr(ctio" i"


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81 &hysical exam to r(le o(t obstr(ctio" i"re&ro#(cti9e t(b(les

1 :ertility testing seme" !"!lysis4 o9(l!tio"

mo"itori"+4 t(b!l &!te"cy !ssessme"t6A6 emen analysis/ !$ter !bsti"e"ce $or 8/B#!ys4 seme" !ssesse#4 mi"im(m is 80MDml6%re&e!te# !$ter 8/ mo"ths4 s&erm m!t(rity is

re!che# i" 0/0 #!ys

Norm!l @0/800 MDml

A9er!+e e:!c(l!tio" 86@/@ ml

,6 )ulation monitoring/ #o"e by recor#i"+ ,,T$or B mo"ths4 tem& rise sho(l# l!st !t le!st .0#!ys% Fertel me!s(res FS7 le9els i" 0 mi"(tes

C ubal patency (ltr!so("# !"# X r!y


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C. ubal patency / (ltr!so("# !"# X/r!yim!+i"+ !re (se# to !ssess $or obstr(ctio"s

I"ter9e"tio"s

.1 I"cre!se s&erm co("t !"# motility by

=!bsti"e"ce $or 2/.0 #!ys

=li+!tio" o$ 9!ricocele

=ch!"+es i" li$estyle - we!r looser clothi"+bo)ers i"ste!# o$ brie$s4 !9oi# &rolo"+e#sitti"+4 hot b!ths

81 Re#(ce i"$ectio"1 7ormo"e ther!&y/ G"R74 Clomi# (se

B1S(r+ery4 i"semi"!tio"4 i" 9itro F4 !#o&tio"4

s(rro+!te motherhoo#4 chil#/$ree li9i"+

N(rsi"+ C!re o$ 7i+h Ris< Newbor"


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N(rsi"+ C!re o$ 7i+h/ Ris< Newbor"-&6201

*) /$11 :O' =/0$0O- $= below the .0th

&erce"tile #(e to IUGR -/84@00 + or @6 o("ces1%8B/BBth wee<

=&reterm/ below th wee< +est!tio"

=term/ betwee" / B8 wee


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Assessme"t

'o"e by me!s(ri"+ $("#!l hei+ht4(ltr!so("#4 NST4 !m"iotic ?(i# !mo("t6%

Ces!ri!" birth is birth metho# o$ choice6 arly deprivation #ecre!se# wei+ht4

le"+th !"# he!# circ(m$ere"ce

1ate deprivation re#(ce# wei+ht6 O9er!ll w!ste# !&&e!r!"ce4 &oor s


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L!b >"#i"+s


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L!b >"#i"+s

= hem!tocrit #(e to l!c< o$ ?(i# i" (tero

= R,C/ !"o)i! i"cre!ses &ro#(ctio" o$ R,C%

c!(ses 9iscosityJ !crocy!"osis6

;0/203 / hem!tocrit4 e)ch!"+e tr!"s$(sio"is "ee#e# to #il(te bloo#6

hy&o+lycemi! -B@ m+D#l1/ I +l(cose6

8) 1=$ (macrosomia1/ !bo9e 0th &erce"tile


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8) 1=$ (macrosomia1/ !bo9e 0th &erce"tile#(e to o9er&ro#(ctio" o$ +rowth hormo"e i"(tero- a!ove


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=ICP #(e to l!r+e he!# c!" c!(se &ress(re


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=ICP #(e to l!r+e he!# c!" c!(se &ress(reto res&ir!tory ce"ter6

A #i!&hr!+m!tic &!r!lysis m!y occ(r

bec!(se cer9ic!l "er9e tr!(m! !s the he!#is be"t si#ew!ys to !llow $or birth o$ l!r+esho(l#ers which &re9e"ts !cti9e l("+

motio" o" !ecte# si#e6 Imme#i!te bre!st$ee#i"+ is "ee#e# to

&re9e"t hy&o+lycemi!6

1 &reterm nant less th!" 2 wee


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1 &reterm nant less th!" 2 wee


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Assessme"t


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Assessme"t

#is&ro&ortio"!te sie o$ he!# -= cm +re!terth!" chest sie14 r(##y s


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1 Persistent patent ductus arteriosus #(e to&(lmo"!ry hy&erte"sio" which &re9e"tsclos(re o$ 'A6

'6O6C6/ +buprofen or +ndomethacin -oli+(ri!1is +i9e" to close P'A6

B1 Peri)entricular/ +ntra)enticular

hemorrhage/ #(e to $r!+ile cerebr!lc!&ill!ries !"# imm!t(re cerebr!l 9!sc(l!r#e9elo&me"t% r(&t(re o$ c!&ill!ries le!# tohy#roce&h!l(s $rom blee#i"+ i"to the

!5(e#(ct o$ Syl9i(s6

@1 R'S4 !&"e!4 reti"o&!thy o$ &rem!t(rity

100 ygen/c!" c!(se &(lmo"!ry e#em! or

bli"#"ess o$ &rem!t(rity

. mlDD


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. mlDD


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PostTerm I"$!"t bor" !$ter B8 wee


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Res&ir!tory 'istress Sy"#rome -7M'1


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Res&ir!tory 'istress Sy"#rome -7M'1

Commo" i" &reterm i"$!"ts4 i"$!"ts o$

#i!betic mothers4 those bor" thr( CS4 orthose with #ecre!se# bloo# &er$(sio" to thel("+s

7y!li"e/ li


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Assessme"t

/'iKc(lty i"iti!ti"+ res&ir!tio"s !t birth%/low bo#y tem&

/N!s!l ?!ri"+/ster"!l !"# s(bcost!l retr!ctio"s

/t!chy&"e! = ;0 RR

/cy!"otic m(co(s membr!"es

/e)&ir!tory +r("ti"+ i" se9er!l ho(rs

/sees!w res&ir!tio"s

/7e!rt $!il(re show" by oli+(ri! !"# e#em! o$ e)tremities

/P!le +r!y s


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'i!+"osis o$ R'S +r("ti"+4 ce"tr!l cy!"osis i" room !ir4 t!chy&"e!4

"!s!l ?!ri"+4 retr!ctio"s4 !"# shoc


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1 &y&ancuronium( &avulon1% 9e"til!tory!ssist!"ce is (se# i" c!se o$ &ower $!il(re6

@1 xtracorporeal mem!rane oxygenation-ECMO1

bloo# is remo9e# $rom b!by by +r!9ity (si"+9e"o(s c!theter !#9!"ce# thr( ri+ht !tri(m

!"# is o)y+e"!te# !"# rew!rme# !"# ret(r"e#thr( c!theter% (se# $or B/2 #!ys6

;1 1iEuid ventilation/ (se o$ &er?(oroc!rbo"s

21 -itric oxide/ &(lmo"!ry 9!so#il!tio"i"cre!ses bloo# ?ow


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econium $spiration /yndrome


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p y

/&rese"t i" !s e!rly !s .0 wee


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/ low APGAR4 #iKc(lty i" st!rti"+ res&ir!tio"s4

t!chy&"e!4 retr!ctio"s4 !"# cy!"osis occ(r6

/ /$:4 s(ctio" i"$!"t with b(lb syri"+e !t&eri"e(m4 be$ore birth o$ sho(l#ers4 to !9oi#

meco"i(m !s&ir!tio"6/#o "ot +i9e O8 by m!s< to &re9e"t &l(++i"+!l9eoli with meco"i(m

/!ir tr!&&i"+ m!y c!(se b!rrel chest

/Chest r!#io+r!&h will show bil!ter!l co!rsei">ltr!tes

/#i!&hr!+m is &(she# #ow"w!r#s

herapeutic 3anagement


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p g

.1 Am"ioi"$(sio" to #il(te meco"i(m i" !m"iotic?(i#

81CS1A"tibiotics

B1 S(r$!ct!"t

@1Obser9e $or !ir tr!&&i"+4 r(&t(re o$ !l9eoli c!"c!(se &"e(mothor!) - rele!se o$ !ir i"to&le(r!l s&!ce1

;1Chest &hysiother!&y with cl!&&i"+ !"#

9ibr!tio" to e"co(r!+e remo9!l o$ meco"i(mrem"!"ts


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SU''EN INFANT 'EAT7 SYN'ROME


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&e!< i"ci#e"ce is 8/B mo"ths o$ !+e

Commo" i" i"$!"ts o$ !#olesce"tmomsDclosely/ s&!ce# &re+"!"cies4 !"#("#erwei+htD&reterm i"$!"ts% i"$!"ts withbro"cho&(lmo"!ry #ys&l!si!4 twi"s4 Es


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+ &

'ecre!se# !ro(s!l res&o"ses

Possible l!c< o$ s(r$!ct!"t i" !l9eoli

Slee&i"+ i" room with "o mo9i"+ !irc(rre"ts- re/bre!thi"+ c!rbo" #io)i#e1

i"$!"ts !&&e!r well/"o(rishe#4 #ies

so("#lessly #(e to l!ry"+os&!sm4 some !re$o("# with 9omit(s or bloo#/?ec


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slee& !ssessme"t #o"e withi" the >rst 8 wee


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Risi"+ le9el o$ !"tibo#ies -I"#irect Coomb*s test1 #(ri"+&re+"!"cy

Co">rme# by !"tibo#ies o" $et!l erythrocytes i" cor#bloo# -&ositi9e #irect Coomb*s test1

Mother will !lw!ys be Rh-/1 !"# i"$!"t will be Rh -1

Se9ere !"emi! will c!(se he&!tome+!ly !"#s&le"ome+ly

E)treme e#em! c!(ses hy#ro&s $et!lis

V!("#ice o" >rst 8B ho(rs o$ li$e

,re!st$e# b!bies will show more :!("#ice #(e to&re+"!"e#iol which i"ter$eres with co":(+!tio" o$i"#irect bilir(bi"

Norm!l I,L i" cor# bloo#J 0/ m+D.00 ml

=80 i" termD=.8 m+D.00 ml i" &retermJ br!i" #!m!+e

Ther!&e(tic M!"!+eme"t


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.1 E!rly $ee#i"+/ bowel elimi"!tio" o$ bilir(bi"

81 Photother!&y/e)&os(re to li+ht c!(ses li9er to&rocess bilir(bi"% 5(!rt h!lo+e"4 cool white li+htor s&eci!l bl(e ?(oresce"t li+ht is &l!ce# .8/0 i"!bo9e b!ssi"et

.0/.8 m+D#l i" 8B ho(rsJ le9el !t which t) is "ee#e#by term b!bies

,ri+ht +ree" stoolsJ #(e to e)cretio" o$ e)cessi9ebilir(bi" -(ri"e is #!r


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'URING INFANCY.6 I"t(ss(sce&tio"

86 F!il(re to Thri9e6 Colic

B6 Trisomy 8.

@6 Cle$t P!l!te

;6 Im&er$or!te A"(s26 7irschs&r("+*s 'ise!se

6 S&i"! ,i>#!

6 7y#roce&h!l(s

.06 Otitis Me#i!

..6 Me"i"+itis

.86 Febrile Sei(res

.6 A(tismD A'7'

4let 1ip and &alate -&62B;1


65/354

C#'t L/ $!il(re o$ the m!)ill!ry !"# me#i!""!s!l &rocesses to close betwee" wee


66/354

anagement


67/354

S(r+ery o" ! cle$t li& is #o"e o" the i"iti!lhos&it!l st!y or betwee" 8/.0 wee


68/354

O"e o$ the most commo" #e9elo&me"t!l#isor#ers o$ the "er9o(s system !t birth%

E)cess !cc(m(l!tio" o$ CSF i" the 9e"tricles orthe s(b!r!ch"oi# s&!ce c!(si"+ e"l!r+eme"to$ the s


69/354

.1 A +rowi"+ t(mor

81 Obstr(ctio" o$ the &!ss!+e o$ ?(i# i" the

!5(e#(ct o$ Syl9i(s/ most commo" c!(se%i"$ectio"s li


70/354

$ssessment


71/354

Se&!r!te# s(t(re li"e4 &romi"e"t sc!l& 9ei"s4i"cre!se# he!# circ(m$ere"ce4 bossi"+ o$

$orehe!#4 s("set eyes4 leth!r+y or irrit!bility4shrill cry4 hy&er!cti9e re?e)es4

Tr!"sill(mi"!tio" re9e!ls ?(i# r!ther t!" soli#br!i"

/igns o F 4&

=&(lse4 res&ir!tio"s

tem&er!t(re4 ,P/str!bism(s4 o&tic !tro&hy4 FTT

me!s(re chil#*s he!# !t birth $or b!seli"e #!t!

!"# !ssess !ll chil#re" ("#er 8 ye!rs $or he!#

anagement


72/354

e!rly #etectio" to &re9e"t br!i" #!m!+e4motor !"# me"t!l #eterior!tio"6

iamox (diuretic) i$ c!(se# byo9er&ro#(ctio" o$ ?(i#

Remo9!l o$ t(mor i$ #(e to obstr(ctio"

>entriculoperitoneal shuntto remo9e

e)cess CSF $rom 9e"tricles !"# sh("t it to the&erito"e(m i"to the bo#y circ(l!tio"

Assess $or W ICP !$ter s(r+ery% !ssess $or si+"s

o$ i"$ectio" or me"i"+itis s(ch !s sti "ec< orm!r


73/354

/&-$ 2:$ -P6 2;@1


74/354

'i9i#e# s&i"e #(e to l!c< o$ $(sio" o$

&osterior s(r$!ce o$ embryo i" e!rlyi"tr!(teri"e li$e6

Occ(rs !s ! &oly+e"ic i"herit!"ce &!tter" or$olic !ci# #e>cie"cy6

;00 mc+ o$ $olic !ci# $or &re+"!"t wome" to&re9e"t S,6

MSAFP is obt!i"e# !t wee< .@ o$ &re+"!"cy to

r(le o(t S, i" the 8"# chil# Am"ioce"tesis is #o"e to !ssess AFP i"

!m"iotic ?(i#

0ypes o /pina 2iGda


75/354

.1 A"e"ce&h!ly/ !bse"ce o$ cerebr!l

hemis&heres whe" (&&er e"# o$ "e(r!l t(be$!ils to close i" e!rly i"tr!(teri"e li$e% commo"breech &rese"t!tio"% m!y s(r9i9e ! $ew #!ys#(e to i"t!ct me#(ll!

81 Microce&h!ly/ c!(se# by i"tr!(teri"ei"$ectio"s li


76/354

1 S&i"! bi>#! occ(lt! / occ(rs commo"ly !t the@th l b # .st l l l #i li


77/354

@th l(mb!r !"# .st s!cr!l le9el% #im&li"+ or!b"orm!l t($ts o$ h!ir4 #iscolore# s


78/354


79/354

$ssessment ' i i # h $


80/354

'etectio" is #o"e thr( so"o+r!m4 $etosco&y4!m"ioce"tesis or MSAFP

CS is #o"e to !9oi# &ress(re !"# i":(ry to thes&i"!l cor#

Assess $or s&o"t!"eo(s mo9eme"t o$ lowere)tremities !"# bowel !"# bl!##er $("ctio"

Norm!l chil# is (s(!lly #ry $or 8/ ho(rs % i"$!"twith S, 9oi#s co"ti"(!lly6

See T!ble 826./ Motor $("ctio" !bility i"

chil#re" with myelome"i"+ocele -T;/.8Jcom&lete ?!cci# &!r!lysis o$ the lowere)tremities4


81/354

For me"i"+ocele4 myelome"i"+ocele ore"ce&h!locele4 Imme#i!te s(r+ery -withi" 8B/

B ho(rs A,1 to re&l!ce co"te"ts !"# close s


82/354

/strict(re o$ the !"(s% the lower bowel e"#s i" !

bli"# &o(ch='etecte# by so"o+r!m% i"s&ectio" !t birth will

re9e!l ! membr!"e >lle# with bl!c< meco"i(mc!" be see" &rotr(#i"+ $rom the !"(s

=Also ob9io(s by the i"!bility to i"sert ! rect!lc!theter i"to the !"(s4 "o stool will be &!sse#

=F!il(re to &!ss stools !$ter the >rst 8B ho(rs

=Collect ! (ri"e s&ecime"4 &rese"ce o$meco"i(m me!"s th!t there mi+ht be ! recto/bl!##er >st(l!

anagement A t i $ t # b l li t i$


83/354

A"!stomosis o$ se&!r!te# bowel li+!me"ts i$rect(m e"#s close to the &eri"e(m

All re&!irs !re com&lic!te# by >st(l!s to thebl!##er or 9!+i"!

Tem&or!ry colostomy is cre!te# $or e)te"si9ere&!irs4 >"!l re&!ir is #o"e whe" i"$!"t is ;/.8

mo"ths N(rsi"+ ') Im&!ire# tiss(e i"te+rity !t rect(m

RT s(r+ic!l i"cisio"

O(tcome e9!l(!tio" i"cisio" li"e rem!i"s $reeo$ erythem! or #r!i"!+e ("til it he!ls by !bo(t#!y 2 !$ter s(r+ery

Rect!l #il!t!tio" ./8) #!y to e"s(re &!te"cy o$

rect!l s&hi"cter

4ommon +ealth &ro!lems 0hat evelopuring nancy -& .1


84/354

uring nancy -&6 .1

I"t(ss(sce&tio" I"9!+i"!tio" o$ o"e &ortio" o$ the i"testi"e i"to

!"other4 (s(!lly i" the 8"# h!l$ o$ the >rst ye!r

. ye!rJ i#io&!thic c!(se =. ye!r4 le!# &oi"t - Mec


85/354

Chil# s(##e"ly #r!ws (& le+s !"# cry !s i$ i"

se9ere &!i"4 m!y 9omit% &!i" s(bsi#es !"# thechil# &l!ys !+!i"% sym&toms rec(rs !$ter!ro("# .@ mi"(tes% 9omit(s will co"t!i" bile%c(rr!"t :elly li


86/354

S(r+ic!l emer+e"cy to &re9e"t "ecrosis

I"still!tio" o$ w!ter/ sol(ble4 sol(tio"4 b!ri(me"em!4 or !ir i"to bowel

Obser9e# !$ter 8B ho(rs $or rec(rre"ce%

s(r+ery is #o"e $or !##itio"!l re#(ctio"

7irschs&r("+* 'ise!se -A+!"+lio"icMe+!colo"1


87/354

Me+!colo"1

Abse"ce o$ +!"+lio"ic i""er9!tio" to the m(scles o$ !sectio" o$ the bowel/ lower &!rt o$ si+moi# colo" :(st!bo9e the !"(s% "o &erist!ltic w!9es6

Chro"ic co"sti&!tio" !"# ribbo"/ li


88/354

Two/st!+e s(r+ery

Tem&or!ry colostomy the" bowel re&!ir !t .8/. mo"ths

Perm!"e"t colostomy i$ chil# h!s "o !"!l "er9ee"#i"+s6

COLIC &6


89/354

P!ro)ysm!l !b#omi"!l &!i" i" i"$!"ts ("#er

ye!rs !"# is m!r


90/354

= !s< &!re"ts !bo(t #(r!tio" !"# $re5(e"cy

= !s< wh!t h!&&e"s be$ore colic occ(rs

='oc(me"t "(mber !"# ty&e o$ bowelmo9eme"t

=!ssess $ee#i"+ &!tter" -bre!stDbottle $e#1

=!ssess m!ter"!l #iet -+!s/ $ormi"+ $oo#s1M!"!+eme"t

=$ee# the b!by (&ri+ht

=(se bottles with #is&os!ble b!+

=t!


91/354

Most $re5(e"tly occ(rri"+ chromosom!l!b"orm!lity

Co+"iti9ely/ ch!lle"+e#% I6 o$ @0/20 Pro$o("#ly !ecte# chil#re"*s I6 is less th!"

80

7e!# sie is sm!ller th!" the .0/80th &erce"tileo$ ! "orm!l chil#

Altere# imm("e $("ctio"4 &ro"e to URTI

Atrio9e"tric(l!r #e$ects4 #(o#e"!l ste"osis or

!tresi!4 str!bism(s !"# c!t!r!ct #isor#ers !re!lso commo"

ALL is 80 3 more commo" i" these chil#re"

Li$es&!" is o"ly @0/;0 ye!rs4 $!ster !+i"+

Assessme"t


92/354

=bro!#4 ?!t "ose% e)tr! e&ic!"th!l $ol#4

&!l&ebr!l >ss(re sl!"ts l!ter!lly (&w!r#4,r(sh>el#*s s&ots4 &rotr(#i"+ to"+(e4 ?!t b!c<o$ the he!#4 short "ec"+ers with little >"+er c(r9e#i"w!r#% wi#e s&!ce betwee" >rstDseco"#>"+ers !"# toes4 simi!" cre!se

M!"!+eme"t E"rolme"t i" e!rly e#(c!tio"!l !"# &l!y


93/354

E"rolme"t i" e!rly e#(c!tio"!l !"# &l!y&ro+r!ms

7!"#w!shi"+ to #ecre!se i"$ectio"s Fee# slowly to &re9e"t cho


94/354

FE,RILE SEIZURES

= most commo"ly see" i" &reschool chil#re" -@


95/354

= most commo"ly see" i" &reschool chil#re" -@

mos/@ yrs1 or !s e!rly !s mo"ths to 2 ye!rs

=$e9er !s hi+h !s .0B/ .0B*F=sei(res show !" !cti9e to"ic/ clo"ic &!tter"which l!st $or .@/80 seco"#s or ./8 mi"(tes

= !ssess $or me"i"+itis

Pre9e"tio"

=


96/354

/s&o"+e with te&i# w!ter !$ter !" e&iso#e

/#o "ot +i9e !cet!mi"o&he" &ost/ict!l to

&re9e"t !s&ir!tio"/l(mb!r &("ct(re to r(le o(t me"i"+itis

/!"ti&yretic #r(+s to re#(ce $e9er

/!"tibiotics to tre!t i"$ectio"

OTITIS ME'IA


97/354

= i"?!mm!tio" o$ the mi##le e!r4 most

commo" #ise!se o$ chil#hoo# !$ter RTI= occ(rs !t ;/; mo"ths !"# !+!i" !t !+eB/; o$ !+e

= commo" i" Al!s

Assessme"t Occ(rs !$ter ! res&ir!tory tr!ct i"$ectio"


98/354

Occ(rs !$ter ! res&ir!tory tr!ct i"$ectio"

Chil# h!s ! col#4 rhi"itis4 !"# low +r!#e

$e9er $or ! $ew #!ys S(##e" $e9er o$ .08*F !"# sh!r& co"st!"t

e!r!che

Stre& or 76 i"?(e"! Irrit!bility4 &(lli"+ o$ !ecte# e!r

Ther!&e(tic M!"!+eme"t

A"!l+esicsD !"ti&yretics

N!s!l #eco"+est!"ts $or #!ys o"ly

Ce&h!los&ori"s $or &ersiste"t i"$ectio"

:ailure to 0hrive I"$!"t $!lls below @th &erce"tile $or wei+ht


99/354

I"$!"t $!lls below @th &erce"tile $or wei+ht!"# hei+ht

CAUSES.1 or+!"ic/ c!(se# by #ise!ses

81 No"/ or+!"ic/ #ist(rbe# &!re"t/ chil#

rel!tio"shi&% m!ter"!l role i"s(Kcie"cy%i"$!"t is &hysic!lly !"# emotio"!lly#e&ri9e#

t!


100/354

leth!r+y with &oor m(scle to"e4 loss o$ sc $!t or s


101/354

M!rcits !"# i"ter$ere"ce withmet!bolism

I"$!"t is &(t o" ! #iet ri+ht $or their i#e!l wei+ht% r!&i#wei+ht +!i" is #i!+"ostic o$ "o"or+!"ic $!il(re to thri9e

$00-0O- :40 +&'$40>0/O''


102/354

/O''

/ persistent pattern of inattention and/or hyperacti)ity-

impulsi)eness re)ealed before 9 years and occursmore fre:uently in boys

6elated to child neglect, lead poisoning, and drugeposure in utero

; 3a disorgani?ation, repetition,

compulsion

81 !ri!bility/ loses t!s


103/354

1 Se5(e"ci"+/ &rocess o$ rel!ti"+ thi"+s to o"e !"otheri" time or s&!ce% #iKc(lty $ollowi"+ #irectio"s

B1 Se5(e"ci"+ o$ wor#s/ #iKc(lty o$ (si"+ co":("ctio"s!"# &re&ositio"s

@1 So$t "e(rolo+ic si+"s/ i"!bility to hol# ! &e"cil%i"!bility to reco+"ie ! sh!&e tr!ce# o" the s


104/354

.1 St!ble e"9iro"me"t $ree o$ stim(li/ &!stel colors4

ro(ti"e81 Gi9e ste&/by/ste& i"str(ctio"s

1 P("ishme"t sho(l# $ollow !" oe"se 5(ic


105/354

M!rcits i" l!"+(!+e4

&erce&t(!l4 !"# motor #e9elo&me"t% #e$ecti9ere!lity testi"+% i"!bility to $("ctio" i" soci!lsetti"+s6

L!c< o$ res&o"si9e"ess to other &eo&le4 +ross

im&!irme"t i" comm("ic!tio" s


106/354

F!il(re to #e9elo& soci!l rel!tio"s

Stereoty&e# beh!9ior s(ch !s h!"# +est(res

E)treme resist!"ce to ch!"+e i" ro(ti"e Ab"orm!l res&o"ses to se"sory stim(li

'ecre!se# se"siti9ity to &!i"

I"!&&ro&ri!te or #ecre!se# emotio"!le)&ressio"s

S&eci>c4 limite# i"tellect(!l &roblem/ sol9i"+!bilities

Im&!ire# !bility to i"iti!te or s(st!i" !co"9ers!tio"

+$10+ &'O21/ 4OO- -0O1'/D


107/354

.6 CERE,RAL PALSY -&6.B@186 ,URNS -&6 .@;;1

6 POISONING -&6 .@@1

B6 C7IL' A,USE -&6.;1@6 MENINGITIS

4'2'$1 &$1/D


108/354

"o"/ &ro+ressi9e (&&er motor "e(ro"

im&!irme"t th!t res(lts i" motor #ys$("ctio"4s&eechD oc(l!r &roblems4 sei(res4 co+"iti9ech!lle"+es or hy&er!cti9ity4 m(scle s&!sticity

CAUSES:

I"tr!(teri"e !"o)i!J Lbw4 &l!ce"t!l #e$ects4"(tritio"!l #e>cie"cies4 #r(+s4 !"# m!ter"!li"$ectio"s -CMD to)o&l!smosis1

Commo" i" L,HD SGA i"$!"ts !"# thosebor" i" OP &ositio"s

Chil# !b(se4 S,S4 tr!(m! or se9ere

#ehy#r!tio" me"i"+itis or e"ce&h!litis

0&/D*. /pastic type e)cessi9e4 hy&erto"ic


109/354

*. /pastic type e)cessi9e4 hy&erto"icm(scles4 !b"orm!l clo"(s4 e)!++er!tio" o$

'TRs4 !b"orm!l re?e)es -,!bi"s


110/354

;) $taxic !w


111/354

& & &to/toe4 Romber+*s test4 resti"+ tremors4

>"+er/to/"ose test4 heel/to/shi" test1


112/354

Ris< $or imb!l!"ce# "(tritio"4 less th!"bo#y re5(ireme"ts4 RT #iKc(lty i" s(c


113/354

I":(ries c!(se# by e)cessi9e he!t +re!terth!" .0B*F -B0*C16

8"# c!(se o$ ("i"te"tio"!l i":(ry i" chil#re"./B yDo

r# c!(se i" chil#re" @/.B yDo

$ssessmentD

HWhere and what is the extent and

depth7I !s< the c!(se4 seco"#!ry he!lth &roblems4!s< !bo(t other chil#re"


114/354


115/354


116/354

*st egree !&&ly ice to cools


117/354

oi"tme"t !"# ! +!(e

b!"#!+e

8nd egree #o "ot r(&t(reblisters4 co9er with sil9ers(l$!#i!i"e !"# +!(e

;rd egree ?(i# ther!&y4

systemic !"tibiotics4 &!i"m!"!+eme"t4 !"# &hysic!lther!&y

-ursing iagnoses Ris< $or i"eecti9e bre!thi"+ &!tter"s RT to


118/354

+ &res&ir!tory e#em! $rom b(r" i":(ry -RR

rem!i"s withi" .;/80 b&m4 l("+!(sc(lt!tio" re9e!ls "o r!les1

Ris< $or i"eecti9e tiss(e &er$(sio" RTc!r#io9!sc(l!r !#:(stme"ts !$ter b(r" i":(ry-chil#*s S st!ys withi" "orm!l limits% ho(rly(ri"e o(t&(t rem!i"s +re!ter th!" . mlD


119/354

+ ycle!"i"+ !+e"ts4 cosmetics4 OTC #r(+s%

s(s&ect s(ici#e !ttem&t i" !" ol#er chil#

-ursing iagnosis Ris< $or i":(ry RT

m!t(r!tio"!l !+e o$ chil# !"# &rese"ce o$&oiso"s -&!re"ts i#e"ti$y &oiso"o(s !"#to)ic items !t home !"# #escribe s!$estor!+e% st!tes loc!l &oiso" co"trol "(mber%

#escribes me!s(res to see< hel&imme#i!tely1


120/354

$cetaminophen &oisoning= (se# to tre!t chil#hoo# $e9ers% c!(ses


121/354

!"ore)i!4 N4 :!("#ice4 te"#er li9er4

ele9!te# li9er e"ymes !s l!r+e #oses c!"c!(se li9er #estr(ctio"

= Acetylcystei"e -!cti9!te# ch!rco!l1 bi"#swith the bre!


122/354

my Ne+lect - "ot $e#4 clothe#4 s(&er9ise#

&ro&erly4 "ot oere# me#ic!l c!re ore#(c!tio"!l o&&ort("ities1

Psycholo+ic!l or emotio"!l - chil# is m!#e

to $eel ("i"telli+e"t or i"!#e5(!te1 A+&s# "h#n !#:

More !"+ry4 "o"com&li!"t4 !"# hy&er!cti9e

Show &oor sel$/co"trol4 low sel$/ esteem More with#r!w" !"# h!9e ! ?!tter !ect

7!9e ("#i!+"ose# me#ic!l &roblems-!"emi!4 otitis me#i!4 le!# &oiso"i"+4 ST's


123/354


124/354

0+O'/ O: 4+1 $23/D.6 A &!re"t h!s the &ote"ti!l to !b(se the


125/354

chil# -s&eci!l &!re"t1% .03 h!9e ! h) o$

me"t!l ill"ess% "o s(&&ort &erso"s% !b(se#!s chil#re"% isol!te#4 !lcoholic

86 A chil# is see" !s #iere"t i" some w!yby the &!re"t -s&eci!l chil#1%m!y h!9e birth#e$ects4 m!y !ss(me role re9ers!l

6 A" e9e"t or circ(mst!"ce bri"+s !bo(t the!b(se -s&eci!l circ(mst!"ce1% stress $rom

wor


126/354

&e"!lty or loss o$ "(rsi"+ lice"se

.1 st!te re5(ires hos&it!l to hol# the chil#$or 28 ho(rs% #etermi"!tio" is #o"e by co(rtwhether chil# is rele!se# to &!re"ts*c(sto#y or


127/354

&lot hei+ht !"# wei+ht i" ! +rowth ch!rt

81 Ab(se# chil#re" h!9e hi+her i"ci#e"ces o$h!"# i":(ries% #ors!l h!"# b(r"s or sc!l#s!re commo"% ci+!rette b(r"s he!l withsc!rri"+% h(m!" bite m!r


128/354

mo"ths o$ !+e

I" the U6S64 &e!< i"ci#e"ce is !t wi"ter #(eto Stre& &"e(mo"i!e or +ro(&/ , stre&

I" chil#re" yo("+er th!" 8 mo"ths4 c!(se#

by G, stre& or E6 Coli C!(se# by b!cteri!4 9ir(s thro(+h !" URTIor #irect i"tro#(ctio" thro(+h ! l(mb!r&("ct(re or s


129/354

+l!"# c!(si"+ e#em!

$////-0

8/ #!ys o$ URTI4 i"cre!si"+ly irrit!bility #(e

to he!#!ches4 sh!r& &!i" o" be"#i"+ theirhe!# $orw!r#

-1 Qer"i+*sD,r(#i"s


130/354

9ir(s4 !"# e"tero9ir(s


131/354

+


132/354

+


133/354


134/354


135/354

-ew!orn $ssessmentD/ Poor s(c


136/354

shoc


137/354

81 I"tr!thec!l !"tibiotics to &!ss thro(+h the bloo#/

br!i" b!rrier

1 Am&icilli"/ '6O6C $or =. inuen?ae

4 Ce$ot!)imeD ce$tri!)o"e/ /.0 #!ys

@1 Corticosteroi#s or m!""itol to re#(ce ICP !"#

hel& &re9e"t he!ri"+ loss;1 Res&ir!tory &rec!(tio"s $or 8B ho(rs to !$ter

!"tibiotic ther!&y is st!rte# to &re9e"ttr!"smissio"

21 Pro&hyl!ctic !"tibiotics $or $!mily members

1 Positio" +e"tly4 me!s(re he!# circ(m$ere"ce%chec< I !"# sDs o$ ICP4 wei+h% chec< SG o$ (ri"e

7EALT7 PRO,LEMS COMMON INPRESC7OOLERS


138/354

.6 LEUQEMIA -&6.@B186 HILM*S TUMOR

6 AST7MA

B6 UTI


139/354

13J$ (p. *59


140/354

4$3//D radiation exposure to chemicals, genetics ncreased incidence in twins, those withown :anconi syndromesL exposure to

radiation/&0O/D-/tarts with pallor, lowgrade ever, anemia,!leeding a!normalities, easy !ruising

-/plenomegaly and hepatomegaly causesa!dominal pain, vomiting, and anorexia


141/354

invasion o lymphocytes invade !oneperiosteum causing !one and Moint pain4-/ invasion leads to headache orunsteady gait

&ainless swelling o su!maxillary andcervical nodeslevated leu%ocytes, low platelets andhematocrit !ut normal '24

0/0/D*. 2one marrow aspiration at the iliac crestD

(N) 85K !last cells1um!ar tapD presence o !last cells in


142/354

isease 4lassiGcationD*. 2 1ymphocytes cell typesD @5K (4$11$PNQ)

8. t 1ymphocytes cell typesD*58AK

0+'$&304 $-$=-0D*. nduction phaseD complete remission or

a!sence o leu%emia cells * month tx with vincristine prednisone,

1 asparaginase, doxoru!icin through acentral venous port (nonirritating)Lallopurinol (:)


143/354

8. 4onsolidation or /anctuary phaseDintrathecal administration omethotrexate or oral

6mercaptopurine to prevent leu%emic

cells to #ourish in the 4-/ P222Q(less usedtoday due to / o minimal learningdisorders)

;. elayed ntensive therapyD intensiGeddosage >&1a,o,4yclophosphamide,cytosine ara!inoside ($'$4) or 6thioguanine


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do not give oral and intrathecalmethotrexate at the same time

Ommaya reservoirD silicon tu!ing inserted

into cere!ral ventricle and threaded underthe scalp to chec% 4/: or samplingL nomore repeated lum!ar punctures


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Jidney involvementD can limit the use o

chemotx0esticular invasionD invasion o testes !yleu%emic cells can cause sterilityL sperm!an%ing !eore therapy is started i patientis past pu!ertyN&sn D/: 'is% or inection '0 nonunctioning W24s and immunosuppresive

efects o therapyO&t"om# E0!&!tonD 4hild"s temperatureremains lower than [email protected]":(;?"4)L no areaso erythema or drainage present on s%in


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N&sn D/ D 'is% or deGcient #uid volume'0 increased chance o hemorrhage rompoor platelet production

O&t"om# #0!&!ton: -O evidence ohemorrhage at present (no epistaxis,hematuria, or hematemesis), &', '' W-1or age group


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8. $cute yeloid 1eu%emia ($1)Dprolieration o granulocytes (neu, !aso,and eo) seen in 8AK

in late adolescence

same symptoms with $11L increasedsuscepti!ility to 3'0 !eore dxgmt.D*. nduction phaseD4ytara!ine and

aunoru!icin or *8 months or ullremission


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8. 4onsolidationsanctuaryD 4yclo and 6thioguanine or 69 months

2one marrow transplant to ensure new

growth o normal granulocytes

SSremission is more diTcult with $1

4O-=-0$1 +$'0 /$/ (4+)D

C7's !re str(ct(r!l #e$ects o$ the he!rt4


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4+re!t 9essels4 or both th!t !re &rese"t $rom

birth/ 8"# o"ly to &rem!t(rity !s ! c!(se o$ #e!th i"the >rst ye!r o$ li$e

Cli"ic!l Cl!ssi>c!tio" o$ Co"+e"it!l he!rt #ise!se

1. cyanotic P'A4 AS'4 S'

2. (yanotic TOF4 TG4 Tr("c(s !rterios(s;. bstructi)e Co!rct!tio" o$ Aort!4 AS4 PS

ACYANOTIC CHD (PDA)1. PATENT DUCTUS ARTERIOSUS (PDA)•results when the fetal ductus arteriosus fails to


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close completely after birth

Pathophysiology:

•blood flows from the aorta through the PDA andback to the pulmonary artery and lungs, causing >LV workload and pulmonary vascular congestion

41-4$1 $-:/0$0O-/D=i$ #e$ect is sm!ll4 chil# m!y be !ysm&tom!tic! lo(# m!chi"e li


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•child may have CHF with poor feeding, fatigue,hepatosplenomegaly, poor weight gain,tachypnea and irritability, widened pulse

db d l b


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pressure and bounding pulse rate maybe

detected

1$2O'$0O' $- $=-O/04 :--=/ECG "orm!l b(t m!y show 9e"tricle

e"l!r+eme"t i$ the sh("t is l!r+eN(rsi"+ m!"!+eme"t.6 Pro9i#e $!mily te!chi"+ !bt6 tre!tme"t o&tio"ssome close s&o"t% others c!" be close# s(r+ic!llyor "o"/s(r+ic!lly86 I" &rem!t(re i"$!"ts4 P'A c!" be close# (si"+&rost!+l!"#i" sy"thet!se i"hibitors

-I"#ometh!ci"1 wDc stim(l!te clos(re o$ the

an a!normal communication !etween theatriaL results when the atrial septal tissuedoes not use properly during em!ryonicd t


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devt.

&athophysiologyD

•pressure is higher in the let atrium than

the right, causing !lood to shunt rom letto right•the '> and &$ enlarge !ecause they arehandling more !lood$ssessment GndingsD*. most inants tend to !e aysmptomaticuntil early childhood and many deects

close spont !y 5 yo


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Laboratory and diagnostic findings:

•ECG – normal but may show ventricle enlargement if the shunt is large

2. slow weight gain and frequent respiratoryinfections may occur3. systolic ejection murmur may be auscultated,


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LABORATORY AND DIAGNOSTIC STUDYFINDINGS:

1. Echocardiography with Doppler gen. reveals the

enlarged R side of the heart and the inc.pulmonary circulation2. Cardiac catheterization demonstrates theseparation of the R atrial septum and the inc.

oxygen saturation in the R atrium

y j yusually most prominent at the 2nd ICS

Nursing Management:1. Provide family teaching abt. treatment options:


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y g pa. defects are usually repaired in girls due to

possibility of clot formation during child bearingyearsb. small ASDs are left open in boys, larger ones arerepairedc. surgical closure is performed during the schoolage years

C. VENTRICULAR SEPTAL DEFECT- the most common CHD where there is anabnormal opening between the right and left


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ventricles

- the degree of this defect vary from a pinholebetween the R & L ventricles to an absentseptumPathophysiology:

•pressure from the LV causes blood to flowthrough the defect to RV, resulting in increasedpulmonary vascular resistance and right heartenlargement•'> and &$ pressures increase, leadingeventually to o!structive pulmonaryvascular disease


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Assessment findings:1.symptoms vary with the size of the defect, age andamtofresistance,usuallythechildis


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amt of resistance, usually the child isasymptomatic.2. failure to thrive, excessive sweating, fatigue3. more susceptible to pulmonary infections4. may exhibit s/s of CHF

1a!oratory and diagnostic study Gndings

.6 Echoc!r#io+r!&hy with 'o&&ler UDS or MRI

re9e!ls R7 !"# &ossible PA #il!t!tio" $rom thei"c6 bloo# ?owECG shows R7

NURSING MANAGEMENT:


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1. provide family teaching about treatment options

2. some VSDs close spontaneously3. others are closed with a Dacron patch,recommended for large defects, PA hypertension,

CHF, recurrent resp. infxns. FTT

4$-O04 4+00'$1O= O: :$11O0 (0O:)

co"sists o$ B m!:or !"om!lies


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/ co"sists o$ B m!:or !"om!lies

!6 S' c6 PSb6 R7 #6 o9erri#i"+ !ort!

&athophysiology

•PS im&e#es the ?ow o$ bloo# to the l("+s4c!(si"+ i"cre!se# &ress(re i" the R4 $orci"+#eo)y+e"!te# bloo# thro(+h the se&t!l #e$ect tothe L

•the i"cre!se# wor


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of the VSD and the degree of PS.

1. Acute episodes of cyanosis (“tet spells”) andtransient cerebral ischemia;“Tet spells” are char.by irritability, pallor, and blackouts or

convulsions.2. Cyanosis at rest (as PS worsens)3. Squatting (a char. posture of older children thatserves to decrease the return of poorly

oxygenated venous blood from the lowerextremities and to inc. SVR, w/c increasespulmonary blood flow and eases respiratory effort)

4. slow weight gain5. clubbing, exertional dyspnea, fainting, or fatigueslownessduetohypoxia


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slowness due to hypoxia

6. a pansystolic murmur may be heard at the mid-lower left sternal border

LABORATORY AND DIAGNOSTIC STUDY

FINDINGS1. echocardiography and ECG show the enlargedchambers of the right side of the heart2. echocardiography also demonstrates the

decrease in the size of the PA and the reducedblood flow through the lungs

3. cardiac catheterization and angiography allowdefinitive evaluation of the extent of the defect,particularlythePSandtheVSD


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particularly the PS and the VSD

4. CBC reveals polycythemia, ABG demonstratereduced oxygen saturation

NURSING MANAGEMENT:

1. Provide family teaching about treatment options elective repair is usually performed during theinfant’s 1st year of life, but palliative repairs maybe warranted for infants who cannot undergo

primary repairtotal repair involvesVSD closure,infundibular stenosis resection, and pericardial

patch to enlarge RV outflow tract2. Provide preoperative and postoperative care

B. TRANSPOSITION OF GREAT VESSELS (TGV)-inTGV,thePAleavestheLVandtheaorta


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in TGV, the PA leaves the LV and the aorta

exits the RV, there is no communication betweenthe systemic and pulmonary circulationsPathophysiology•this defect results in two separate circulatory

patterns; the right heart manages systemiccirculation and the left manages pulmonarycirculation to sustain life, the child must have anassociated defect.

- Associated defects such as septal defects or aPDA, permit oxygenated blood into the systemiccirculation but cause increased cardiac workload.


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-POTENTIAL COMPLICATIONS : CHF, infective endocarditis, brain abscess, and

cerebralvascularaccidentsresulting from


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cerebral vascular accidents resulting from

hypoxia or thrombosis.ASSESSMENT FINDINGS:Clinical manifestations vary, depending onassociated defects

1. In infants with minimal communication (noassociated defects), severe respiratory depressionand cyanosis, will be evident at birth

2. In infants with associated defects, there is lesscyanosis but the infant may have symptoms ofCHF,easily fatigued, FTT

LABORATORY AND DIAGNOSTIC STUDYFINDINGS1echocardiographyrevealsanenlargedheart


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1. echocardiography reveals an enlarged heart

2. cardiac catheterization reveals low O2 saturationresulting from the mixing of blood in the chambers

NURSING MANAGEMENT

1. Provide family teaching about the treatmentoptions2. Prostaglandin E is administered to maintain aPDA and further blood mixing.

3. An arterial switch procedure within the 1st weekof life is the surgical procedure of choice

C. TRUNCUS ARTERIOSUS- failure of normal septation and division of theembryonic bulbar trunk into the PA and aorta,

lti i i l ltht id bth


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resulting in a single vessel that overrides both

ventriclesPathophysiology•blood ejected from the ventricles enters thecommon artery and flows either the lungs or aortic

arch.pressure in both ventricles is high and blood flowto the lungs is markedly increased.

Assessment findings:1. neonates with this defect appear normal;however, as pulmonary vascular resistancedecreases after birth, severe pulmonary edema and

CHFcommonldevelo


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2. marked cyanosis, especially on exertion; S/Sof CHF; LVH, dyspnea, marked activity intolerance,d t dd th


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and retarded growth

> loud systolic murmur best heard at the lowerleft sternal border and radiating throughout thechestLaboratory and diagnostic study findings:1. echocardiography reveals the defectNURSING MANAGEMENT1. surgical repair is necessary in the 1st few

months of life, the mortality rate associated withsurgery is greater than 10%; w/o surgery, childrendie w/in 1 yr.

COARCTATION OF AORTA (COA)•a defect that involves a localizednarrowingoftheaorta


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narrowing of the aorta

Pathophysiology•COA is char. by inc. pressure proximalto the defect and decreased pressuredistal to it

restricted blood flow through thenarrowed aorta increases the pressureon the LV and causes dilation of theproximal aorta and LVH, w/c may lead

to LVF•eventually, collateral vessels developto bypass the coarctated segment andsupply circulation to the LE

Assessment findings:Clinical manifestations1. the child may be asymptomatic or mayexperiencetheclassicdifferenceinBP


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experience the classic difference in BP

and pulse quality between the upper andlower ext. – the BP is elevated in the UEand dec. in the LE while the pulse is bounding in the UE and dec. or absent

in the LE. Thus femoral pulse are weakor absent2. epistaxis, headaches, fainting andlower leg cramps3. a systolic murmur may be heard overthe left anterior chest and between thescapula posteriorly; rib notching may be

observedinanolderchild


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Laboratory and dagnostcfindings:

1. ECG, echocardiography, andchestx-raymayrevealleftsided


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chest xray may reveal left sided

heart enlargement resulting from back pressurethe radiograph may alsodemonstrate rib notching from

enlarged collateral vesselsNursing management:1. repair involves surgical removal ofthe stenotic area

2. nonsurgical repair via balloonangioplasty

Aortic

2. $O'04 /0-O// ($/)-! #e$ect th!t &rim!rily i"9ol9es !"b t ti t th L t? $ th l


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obstr(ctio" to the L o(t?ow o$ the 9!l9e

&$0+O&+/O1O=D

•L &ress(re i"c6 to o9ercome resist!"ce o$the obstr(cte# 9!l9e !"# !llow bloo# to?ow i"to the !ort!4 e9e"t(!lly &ro#(ci"+

L7•MI m!y #e9elo& !s the i"c6 O8 #em!"#s o$the hy&ertro&hie# L +o ("met

ASSESSMENT FINDINGS:CLINICALMANIFESTATIONS:


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CLINICAL MANIFESTATIONS:

1. faint pulse, hypotension, tachycardia, andpoor feeding pattern

2. exercise intolerance, chest pain, anddizziness when standing for long periods3. a systolic ejection murmur may be heardbest at the 2nd ICS

LABORATORY AND DIAGNOSTIC STUDYFINDINGS:


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1. ECG or echocardiography reveals LVH2. cardiac catheterization demonstratesdegree of the stenosisNURSING MANAGEMENT:1. if the child’s symptoms warrant, surgicalaortic valvulotomy or prosthetic valvereplacement is necessary;

balloon angioplasty can be used to dilate thenarrow valve


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C. PULMONIC STENOSIS (PS)-a defect that involves obstruction of bloodflow from the right ventricleh h il


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Pathophysiology:•RV pressure increases leading to RVH andeventually RV failure may occur Assessment findings:

Clinical manifestations1. may be asymptomatic or may have mildcyanosis or CHF2. a systolic murmur may be heard over the

pulmonic area; a thrill may be heard ifstenosis is severe;decreased exercisetolerance, dyspnea, precordial pain andgeneralized cyanosis may occur

LABORATORY AND DIAGNOSTICFINDINGS:1. ECG or echocardiography reveals RVH2cardiaccatheterizationdemonstratesthe


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2. cardiac catheterization demonstrates the

degree of stenosisNURSING MANAGEMENT:1. provide family teaching about treatmentoptions2. Balloon angioplasty techniques are being widely used to treat PS3. Surgical valvulotomy may be performed

(although the need for surgery isuncommon due to the widespread use of balloon angioplasty techniques)4. provide preoperative and postoperative


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RHEUMATIC HEART FEVER•Aninflammatoryautoimmunedisease


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• An inflammatory autoimmune disease

that affects the connective tissues of theheart, joints, subcutaneous tissues, and blood vessels of the central nervoussystem

•Rheumatic heart disease is the mostserious complication•

Presents 2-6 weeks following anuntreated or partially treated Group A beta-hemolytic streptococcal infection ofthe upper respiratory tract


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Assessment:1. Fever: low grade fever that spikes in thelate afternoon2. Elevated antistreptolysin O titer

3. Elevated sedimentation rate4. Elevated C-reactive protein5. Aschoff bodies (lesions): Found in theheart, blood vessels, brain and seroussurfaces of the joints and pleura6. Leukocytosis


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Ascho*s bo#ies

INTERVENTIONS :•Assess vital signs•Control joint pain and inflammation with massageand alternating hot and cold applications as prescribed

d bd d fb l h


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•Provide bed rest during acute febrile phase•Limit physical exercise in the child with carditis•Administer antibiotic (Penicillin) as prescribed•Administer salicylates and anti-inflammatory agents(prednisone) as prescribed

•Initiate seizure precautions if the child is experiencingchorea•Instruct the parents about the importance of follow-upand the need for antibiotic prophylaxis for dental work,

infection and invasive procedures•Instruct child to inform parents of strep infxns inschool

SEIZURE DISORDERS


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Assessment:>Obtain information from the parents about thetime of onset, precipitating events, and behaviorand after seizure

>Determine the child’s history related to seizures Aura, Apnea, cyanosisPost-seizure: Disoriented, sleepyAbsence seizures: Occur in children between 4 to

12 years of age, last 5 to 10 seconds, and the childappears inattentive

Interventions:•Ensure airway patency


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•

Have suction equipment and oxygen available•Time the seizure episode•Ease the child down to floor, placing the child in aside-lying position (standing or sitting)

•Place a pillow or folded blanket under the child’shead; if no bedding is available, place your ownhands under the child’s head or place the child’shead in your lap

•Loosen restrictive clothing

•Remove eyeglasses from the child if present•Cleartheareaofanyhazardsorhardobjects


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•Clear the area of any hazards or hard objects

•Allow the seizure to proceed and end withoutinterference•If vomiting occurs, turn the child to one side asone unit•Do not restrain the child, place anything in thechild’s mouth, or give any food or liquids to thechild•Prepare to administer medication as ordered

DRUGS: ANTICONVULSANTDepress abnormal neuronal discharges and

prevent the spread of seizures to adjacent neurons


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>Hydantoins: Ethotoin (Peganone), Fosphenytoin(Cerebyx), Phenytoin (Dilantin)

>Barbiturates: Phenobarbital; alcohol (Luminal),Amobarbital ( Amytal), Mephobarbital ( Mebaral)>Benzodiazepines: Clonazepam (Klonopin),Diazepam (Valium), Lorazepam (Ativan)

Nsg. Interventions:•Iitit i ti


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•Initiate seizure precautions•Monitor urinary output•Monitor liver and renal function tests andmedication blood serum•

Monitor for signs of toxicity: CNS depression,ataxia, N & V, drowsiness, dizziness, restlessnessand visual disturbances•Remain with the child until he/she fully recovers

•Observe for incontinence, which may haveoccurred during the seizure•Document occurrence

•Sezure Precautons:a. Raise the side rails when the child is sleeping orrestingb. Pad the side rails and other hard objects


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c. Place a waterproof mattress or pad, on the bedor cribd. Instruct the child to wear or carry medicalcertification

e. Instruct the child in precautions to take duringpotentially hazardous activities.f. Instruct the child to swim with a companiong. Instruct the child to use a protective helmet and

padding when engaged in bicycle riding,skateboarding, and in-line skatingh. Alert caregivers to the need for any specialprecautions

ATTENTION DEFICIT HYPERACTIVITYDISORDER (ADHD)

-Developmentaldisordercharacterizedby


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Developmental disorder characterized by

inappropriate degrees of inattention, over-activity,and impulsivity- Childhood problems include lowered intellectualdevelopment, some minor physical abnormalities,

sleeping disturbances, behavioral or emotionaldisorders- Diagnosis is established based on self-reports,parent and teacher reports, and psychologicalassessments

Assessment:•Fidgets with hands or feet or squirms in the seat


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•

Easily distracted with external or internal stimuli•Assessment:•Difficulty with following through on instructions•Poor attention span•Shifting from one uncompleted activity to another•Talking excessively•Interrupting or intruding on others•Engaging in physically dangerous activities

without considering the possible consequences

Interventions:•Provide environmental and physical safetymeasures


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measures•Enhance capabilities and self-esteem•Encourage support groups for parents•Administer prescribed medication;

Methylphenidate hydrochloride (Ritalin), pemoline(Cylert), and dextroamphetamine

SICKLE CELL ANEMIADescriptionThisconstitutesagroupofdiseasestermed


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- This constitutes a group of diseases termed

hemoglobinopathies, in which hemoglobin A ispartly or completely replaced by abnormal sicklehemoglobin S.- It is caused by the inheritance of a gene for astructurally abnormal portion of the hemoglobinchain.-Hemoglobin Sis insensitive to changes in the

oxygen content of the red blood cell.- Insufficient oxygen causes the cells to assume asickle shape and the cells become rigid andclumped together, obstructing capillary blood flow

SITUATIONS THAT PRECIPITATE SICKLING-emotional or physical stress; any condition thatincreases the need for oxygen or alters the


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transport of oxygen can result in sickle cell crisis(acute exacerbation).RISK FACTORS:> parents heterozygous for hemoglobin S or

being African American descent. > reversible under conditions of adequateoxygenation and hydration; after repeated sickling,the cell becomes permanently sickled.

> The clinical manifestations primarily occur asa result of obstruction caused by sickled red bloodcells and increased red blood cell destruction.

- Sickle cell crises are acute exacerbations ofthe disease, which vary considerably in


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severity and frequency; these include vaso-occlusive crisis, splenic sequestration, andaplastic crisis.Care focuses on the prevention (preventing

exposure to infection and maintaining normalhydration) and treatment (oxygen, hydration,pain management, and bed rest) of the crisis.

INTERVENTIONS:1. Maintain adequate hydration and blood flowwith intravenously administered normal saline asprescribed,andwithoralfluids.


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prescribed, and with oral fluids.

2. Administer oxygen and blood transfusions asprescribed to increase tissue perfusion.3. Administer analgesics as prescribed (round theclock); administration of meperidine (Demerol) isavoided because of the risk of normeperidine-induced seizures.4. Assist the child to assume a comfortable

position so that the child keeps the extremitiesextended to promote venous return; elevate thehead of the bed no more than 30 degrees, avoidputting strain or painful joints, and do not raise

th k th fth bd

anagementSt!rt !"!l+esics &rom&tly to &ro9i#e eecti9e relie$ o$&!i"6


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ild painP!r!cet!mol -80m+D


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+ +

Some &!tie"ts m!y re5(ire hi+her i"#i9i#(!l#oses4 b!se# o" &rior history or m!y be"e>t$rom co"ti"(o(s i"$(sio" 9i! PCA -P!tie"tCo"trolle# A"!l+esi!16

M!y "ee# bloo# tr!"s$(sio" (si"+ H,C >ltere#bloo#

&aracetamol80 m+D


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ib(&ro$e" 86@ .0 m+Drst to see eect4the" re&e!t !t @ / .0 mi"(te i"ter9!ls !s re5(ire# (& tom!)im(m tot!l #ose16R#s!to$ ##sson s / re#(ce #oses i$combi"e# with se#!ti9es6 M!y +et #el!ye# res&ir!tory#e&ressio" !$ter tre!ti"+ c!(se o$ &!i"6

5. Encourage consumption of a high-calorie, high-protein diet, with folic acid supplementation.6. Administer antibiotics as prescribed to preventinfection.


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7. Monitor for signs of complications, includingincreasing anemia, decreased perfusion, and shock(mental status changes, pallor, vital sign changes).8. Instruct the child and parents about the early

signs and symptoms of crisis and the measures toprevent crisis.9. Ensure that the child receives pneumococcal,Haemophilus influenza type B, and meningococcalvaccines because of the susceptibility to infectionfrom functional asplenia.10. Inform the parents of the hereditary aspects of

th di d

APLASTIC ANEMIA:- This is a deficiency of circulating erythrocytesand all other formed elements of blood, resulting


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from the arrested development of cells within thebone marrow.CAUSES :chronic exposure to myelotoxic agents, viruses,

infection, autoimmune disorders, and allergicstates.- The definitive diagnosis is determined by bonemarrow aspiration (demonstrates conversion of redbone marrow to fatty red bone marrow).

-Therapeutic management:-restoring function to the bone marrow andinvolves immunosuppressive therapy and bonemarrowtransplantation(treatmentofchoiceifa


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marrow transplantation (treatment of choice if a

suitable donor exists).

-If the cause is myelotoxic medication that is beingadministered for another purpose, the medicationmay be discontinued to improve bone marrowfunction.

ASSESSMENT:1.Pancytopenia:


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planned.

•Administer immunosuppressive medications as prescribed;antilymphocyte globulin or antithymocyte clobulin may beprescribed to suppress the autoimmune response.•Colo"y/stim(l!ti"+ $!ctors m!y be &rescribe# to e"h!"ce

bo"e m!rrow &ro#(ctio"6•Corticosteroi#s !"# cyclos&ori"e m!y be &rescribe#6•Gi9e ,T i$ &rescribe# !"# HOF tr!"s$(sio" re!ctio"s•Mo"itor $or si+"s RT #ise!se !"# to t)•A#9ice &!re"ts to obt!i" ! Me#ic/ Alert br!celet $or thechil#


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HEMOPHILIA

- Refers to a group of bleeding disorders resulting


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from a deficiency of specific coagulation proteins.- Identifying the specific coagulation deficiency isimportant so that definitive treatment with thespecific replacement agent can be implemented;

aggressive replacement therapy is initiated toprevent the chronic crippling effects from jointbleeding.

TYPES:1.Factor VIII deficiency (hemophilia A or classichemophilia)

2.Factor IX deficiency (hemophilia B or Christmas disease


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- Hemophilia is transmitted as an X-linked recessivedisorder (it may also occur as a result of a gene mutation).- It is most frequently transmitted by the union of anunaffected male with a trait-carrier female; however, it canresult from the union between an affected male and anormal female or a carrier female, leading to offspring suchas an affected son, affected daughter, carrier daughter, ornormal son.TREATMENT:

•. replacement of the missing clotting factor;•. pain meds may be prescribed depending on the source ofbleeding from the disorder.

ASSESSMENT•Abnormal bleeding in response to trauma orsurgery (sometimes is detected after circumcision)•Epistaxis(nosebleeds)


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Epistaxis (nosebleeds)•Joint bleeding causing pain, tenderness, swelling,and limited range of motion•Tendency to bruise easily•

Results of tests that measure platelet function arenormal; results of tests that measure clotting factorfunction may be abnormal.

INTERVENTIONS:•Monitor for bleeding and maintain bleedingprecautions.


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•Prepare/ administer replacement factors asprescribed.•Monitor for joint pain; immobilize the affectedextremity if joint pain occurs.

•Assess neurological status (child is at risk forintracranial hemorrhage).•Monitor urine for hematuria.•Control joint bleeding by RICE(15 minutes) forsuperficial bleeding.

•Instruct the child and parents about the signs ofinternal bleeding.•Instruct the parents in how to control thebleeding.


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bleeding.

•Instruct the parents regarding activities for thechild, emphasizing the avoidance of contact sportsand the need for protective devices while learningto walk.•Instruct the child to wear protective devices suchas helmets and knee and elbow pads whenparticipating in sports such as bicycling and

skating.Instruct the parents to obtain a Medic-Alertbracelet or medallion

VON WILLEBRAND DISEASE:


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- A hereditary bleeding disorder characterized by adeficiency of or a defect in a protein termedvonWillebrand factor(vWF)- The disorder causes platelets to adhere to

damaged endothelium; the vWF protein also servesas a carrier protein for factor VIII.- It is characterized by an increased tendency tobleed from mucous membranes.

ASSESSMENT•Epistaxis•Gum bleeding•Easy bruising


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y g

•Excessive menstrual bleeding

INTERVENTIONS•Treatment and care are similar to those measuresimplemented for hemophilia, including theadministration of clotting factors.•Provide emotional support to the child and

parents, especially if the child is experiencing anepisode of bleeding.

β-THALASSEMIA MAJOR

-Autosomal recessive disorder characterized by the


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reduced production of one of the globin chains inthe synthesis of hemoglobin (both parents must becarriers to produce a child with β-thalassemiamajor).

- The incidence is highest in individuals ofMediterranean descent, such as Italians, Greeks,

Syrians, or their offspring.

-TREATMENT:-supportive; the goal of therapy is to maintainnormalhemoglobinlevelsbytheadministrationof


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normal hemoglobin levels by the administration ofblood transfusions.- A splenectomy may be performed in a child withsevere splenomegaly who requires repeated

transfusion (assists in relieving abdominalpressure and may increase the life span ofsupplemental red blood cells).

ASSESSMENT•Frontal bossing•Maxillary prominence


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•Wide-set eyes with a flattened nose•Greenish-yellow skin tone•Hepatosplenomegaly•Severe anemia

•Microcytic, hypochromic red blood cells

INTERVENTIONS•Administer blood transfusions as prescribed;monitor for transfusion reactions.


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•Monitor for iron overload,; chelation therapy withdeferoxamine (Desferal) may be prescribed totreat iron overload and to prevent organ damagefrom the elevated levels of iron caused by the

multiple transfusion therapy.•If the child has had a splenectomy, instruct theparents to report any signs of infection because ofthe risk of sepsis.•Provide genetic counseling.

HEMOLYTIC-UREMIC SYNDROME (HUS) >bacterial toxins, chemicals, and viruses that cause acuterenal failure in children.infants and small children between the ages of 6 months


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and 5 years.

3 CLINICAL FEATURES OF THE DISEASE1. acquired hemolytic anemia/ thrombocytopenia

2. renal injury,3. central nervous system symptoms.ASSESSMENT•Triad of anemia, thrombocytopenia, and renal failure isdiagnostic

•Proteinuria, hematuria, and presence of urinary casts•Blood urea nitrogen and serum creatinine levels areelevated; hemoglobin and hematocrit levels are decreased

•Hemodialysis or peritoneal dialysis may be prescribed ifthe child is anuric•Hemodialysis requires venous access (arteriovenous [AV]shunt, fistula, or graft) and treatment is usually 3 to 8hours in length (three times per week); peritoneal dialysis


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INTERVENTIONS

g ( p );p y

requires surgical placement of an abdominal catheter

•(correction of fluid and electrolyte imbalance is slower thanhemodialysis).

•Dialysate solution is prescribed to meet the child’selectrolyte needs.• Strict monitoring of fluid balance is necessary; fluidrestrictions may be prescribed if the child is anuric.•Institute measures to prevent infection.•Provide adequate nutrition.


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BLADDER EXSTROPHY- congenital anomaly characterized by extrusion

oftheurinarybladdertotheoutsideofthebody


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of the urinary bladder to the outside of the bodythrough a defect in the lower abdominal wall.-The cause is unknown.

- Treatment requires surgical management andoccurs in a series of staged reconstructions.- Initial surgery for closure of the abdominal defectshould occur within thefirst few days of life.

- The goal of subsequent operations is toreconstruct the bladder and genitalia and enablethe child to achieve urinary continence.

Assessment•Exposed bladder mucosa•Widened symphysis pubis•Defects of the external genitalia


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INTERVENTIONS:•Monitor urinary output.•Monitor for signs of urinary tract or woundinfection.•


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•Maintain the integrity of the exposed bladdermucosa.•Prevent the bladder tissue from drying, whileallowing the drainage of urine, until surgical

closure is performed.•The bladder is covered with sterile, non-adherentclear plastic wrap or a sterile thin film dressingwithout adhesive.

•Petroleum jelly is avoided because it tends to dryout, adhere to the bladder mucosa, and damagethe delicate tissues when the dressing is removed.

•Monitor laboratory values and urinalysis to assessfor renal function.•Administer antibiotics as prescribed.•Provide emotional support to the parents, and


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encourage verbalization of their fears andconcerns.

NEPHROBLASTOMA (WILM’S TUMOR)-Most common intra-abdominal and kidney tumorof childhood; it may present unilaterally and


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localized or bilaterally, sometimes with metastasisto other organs- Peak incidence is at 3 years of age-The occurrence is associated with a genetic

inheritance and with several congenital anomalies- - Therapeutic management includes a combinedtreatment of surgery (partial or total nephrectomy)and chemotherapy with or without radiation,depending on the clinical stage and histologicalpattern of tumor

ASSESSMENT•Swelling or mass in the abdomen (mass ischaracteristically firm, non-tender,


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confined to one side, deep within the flank• Abdominal pain•Urinary retention or hematuria• Anemia•Pallor, anorexia, lethargy•Hypertension• Weight loss and fever•Sxs. Of lung involvement:

•Dyspnea•Shortness of breath•Pain in the chest


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Pre-operative Interventions•Monitor v/s particularly bp•Avoid palpation of the abdomen•Place a sign at the bedside, “Do not palpate


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abdomen”•Measure abdominal girth at least once daily

Post-operative Interventions

•Monitor temperature and blood pressure closely•Monitor for signs of hemorrhage and infection•Monitor strict I & O closely•Monitor for abdominal distention, monitor bowel

sounds, and other signs of GI activity (risk forobstruction)

CYSTIC FIBROSIS (CF)-chronic multisystem disorder (autosomal recessivetrait disorder) characterized by exocrine glanddysfunction.


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- The mucus produced by the exocrine glands isabnormally thick, tenacious, and copious, causingobstruction of the affected organs, particularly inthe respiratory, gastrointestinal, and reproductivesystems.- The most common symptoms are pancreaticenzyme deficiencycaused by duct blockage,

progressive chroniclung disease associated withinfection, andsweat gland dysfunction resulting inincreased sodiumand chloride sweatconcentrations.

r

most reliable diagnostic test, the sweat chloride test

- CF is a fatal genetic disorder and respiratory failure is themost common cause of death.RESPIRATORY SYSTEM:Symptoms are produced by the stagnation of mucus in the


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airway, leading to bacterial colonization and destruction oflung tissue.Emphysema and atelectasis occur in the airways becomeincreasingly obstructed.Chronic hypoxemia causes contraction and hypertrophy ofthe muscle fibers in pulmonary arteries and arterioles,leading to pulmonary hypertension and eventual corpulmonale.Pneumothorax from ruptured bullae and hemoptysis from

erosion of the bronchial wall occur as the diseaseprogresses.

OTHER RESPIRATORY SYMPTOMS:Wheezing and dry nonproductive cough,dyspnea, c yanosisclubbing of the fingers and toes,barrel chest, r epeatedepisodes of bronchitis and pneumonia


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Gastrointestinal systems:Meconium ileus in the neonate Intestinal obstruction (distal intestinal obstructive

syndrome) caused by thick intestinal secretions;signs include pain, abdominal distention, nausea,and vomiting.Steatorrhea (frothy, foul-smelling stools)

Deficiency of thefat-soluble vitamins A, D, E, andK, which causeseasy bruising and anemiaMalnutrition andfailure to thrive

Demonstration of hypoalbuminemia fromdiminished absorption of protein, resulting ingeneralized edemaRectal prolapse that can result from the large,


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gbulky stools and lack of supportive fat pads aroundthe rectumIntegumentary system

Abdominally high concentrations of sodium andchloride in sweatParents reporting that the infant tastes “salty”when kissed

Dehydration and electrolyte imbalances, especiallyduring hyperthermic conditionsReproductive systemCystic fibrosis can delay puberty in girls.

Fertility can be inhibited by the highly viscous cervicalsecretions, which act at a plug and block sperm entry.Males are usually sterile, caused by the blockage of the vasdeferens by abnormal secretions or by failure of normal


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y ydevelopment of duct structures.

Diagnostic tests:Quantitative sweat chloride test: >60 mg (+);


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•Chest physiotherapy(percussion and postural drainage)on awakening and in the evening (more frequently duringpulmonary infection); not done before or immediately after ameal

•Bronchodilator medication by aerosol opens the bronchifor easier expectoration (administered before the chestphysiotherapy when the child has reactive airway disease oris wheezing).

•Use of a Flutter Mucus Clearance Device(a small, hand-held plastic pipe with a stainless steel ball on the inside)that facilitates removal of mucus; store away from smallchildren because if the device separates, the steel ball posesa choking hazard.


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•Use o a T AIRapy vest devcethat provide high-

frequency chest wall oscillation to help loosen secretions.•Administering medications as prescribed to decrease theviscosity of mucus.•Instruct the parents not to give cough suppressants such


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as guaifenesin (Robitussin) because they will inhibitexpectoration of secretions and promote infection.•Teach the child forced expiratory technique (huffing) tomobilize secretions.•Develop a physical exercise program with the aim of

establishing an effective habitual breathing pattern.•Administer antibiotics as prescribed, which may beprescribed prophylactically or when pulmonary symptomsdevelop.

•Aerosolized antibiotics may be prescribed and areadministered after chest physiotherapy is performed, orantibiotics may be prescribed and administeredintravenously at home through a central venous accessdevice.

•Administer oxygen as prescribed during acuteepisodes; monitor closely foroxygen narcosis(signsinclude nausea and vomiting, malaise, fatigue,numbness and tingling of extremities, substernal


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distress).•Monitor for hemoptysis;more than 300 mL in 24hours for the older child (less for a younger child)needs to be treated immediately.•Hemoptysis may be controlled by bed rest, coughsuppressants, antibiotics, and vitamin K; ifhemoptysis persists, the site of bleeding may be

cauterized or embolized.•Lung transplantation is a final therapeutic optionfor the child with end-stage disorder.

Gastrointestinal systemThe goal of treatment for pancreatic insufficiency is toreplace pancreatic enzymes; this is administered withall meals and all snacks to ensure that digestive


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enzymes are mixed with food in the duodenum.•The amount of pancreatic enzymes administered isadjusted to achieve normal growth and a decrease inthe number of stools to two or three daily.

•Enteric-coated pancreatic enzymes should not becrushed or chewed.•Pancreatic enzymes should not be given if the child isNPO.

•Encourage a well-balanced, high-protein, high-caloride diet; multivitamins and vitamins A, D, E, andK are also administered.

•Assess weight and monitor for failure to thrive.•Monitor for constipation and intestinal obstruction.•Ensure adequate salt intake and fluids that provide anadequate supply of electrolytes during extremely hotweather and if the child has a fever.


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Home care•Instruct the child and family about the prescribedtreatment measures and their importance.

•Instruct the parents and caregivers to be sureimmunizations are up to date.•Inform the parents and caregivers that the child should bevaccinated yearly for influenza; pneumococcus vaccine may

also be prescribed.Inform the child and family about the Cystic FibrosisFoundation.

wilm

ASTHMA:- chronic inflammatory disease of the airwaysmarked by airway inflammation and

hyperresponsiveness to a variety of stimuli or


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triggers- Commonly caused by physical and chemicalirritants such as foods, pollens, dust mites,cockroaches, smoke, animal dander, temperature

changes, respiratory infection, activity and stress- Mast cell release of histamine leads tobronchoconstrictive process- Common symptom is coughing in the absence of

respiratory infection, especially at night

STATUS ASTHMATICUS- Child displays respiratory distress despite vigoroustreatment measures; a medical emergency that can resultin respiratory failure and death if left untreated


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Assessment:•episodes of wheezing, breathlessness, dyspnea, chesttightness, and cough particularly at night and or in theearly morning•Child may present with prodromal itching localized at the

front of the neck or over the upper part of the back•Exacerbations are episodes of progressively worseningshortness of breath, cough, wheezing, chest tightness,decreases in expiratory airflow secondary to bronchospasm,

mucosal edema, and mucous plugging; air is trappedbehind occluded or narrow airways and hypoxemia occurs

•Asthmatic episode:•The episode begins with irritability, restlessness,headache, feeling tired, and or chest tightness•Respiratory symptoms include a hacking, irritable,


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non-productive cough caused by bronchial edema•Accumulated secretions stimulate the cough;cough becomes rattling, and there is production of

frothy, clear, gelatinous sputum•Retractions•Hyperresonance on percussion of the chest isnoted

Breath sounds are coarse and loud, with crackles,coarse rhonchi, and inspiratory and expiratorywheezing; expiration is prolonged

•Exercise-induced bronchospasm-cough,

shortness of breath, chest pain or tightness,wheezing, and endurance problems occur duringexercise•Severe spasm or obstruction-breath sounds and


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crackles may become inaudible, and the cough isineffective (lack of air movement)•Ventilatory failure and asphyxia- shortness of

breath, with air movement in the chest restricted tothe point of absent breath sounds accompanied bya sudden rise in respiratory rate•Child may be pale or flushed, and the lips may

have a deep, dark red color that may progress tocyanosis observed in the nail beds and skin,especially in the mouth

•Restlessness apprehension and diaphoresis occur•Younger children assume tripod sitting position;older children sit upright with a shoulders in ahunched-over position, the hands on the bed or a

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chair, and the

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