blood disorders 35 36

7/25/2019 Blood Disorders 35 36

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F I U S C H O O L O F N U R S I N G

L A U R A M . H E R N A N D E Z , C P N A R N P , M S N

Chapter 16, 35 & 36

Hematologic Disorders


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Immunodeficiency


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Immunodeficiency (p. 15!"15#$

%eere Com'ined Immunodeficiency %yndrome(%CID$ and is)ott"*ldrich %yndrome"the 'ody isuna'le to mount an immune response.

+he immune systems function is to recogni-eself/ from nonself/ and to initiate a response toeliminate the nonself/ or antigen.

Cell surface mar)ers0'ody cells ith specific cell

surface mar)ers uni2ue to the indiidual


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rotectie 4echanisms

Intact s)in

%alia, seat, tears, stomach acids

%nee-ing, coughing

rimary lymphoid organs (thymus,4,lier$%econdary lymphoid organs (spleen, lymph nodes,

*7+$


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HI89*ID%

15.: million omen and !.5 million children lessthan 15 years of age are liing ith the HI89*ID%

orldide (H;, !


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Western &Central Europe

1400[


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2009 Global HIV and I!" #stimatesChildren (


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HI89*ID% in E%* for Children =13


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Fe'orn Infection Gates in the E%


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+ransmission


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+he pidemic in lorida, !


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8ertical +ransmission (perinatal$

HI8 L mother to her ne'orn

*ntepartum

Intrapartum?M deliery

ostpartum? ia 'reastfeeding"minimal in E.%.,carries a ris) as high as !#>


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Clinical 4anifestations

7ymphadenopathy

Hepatosplenomegaly

Diarrhea Achronic9recurrent

;ral candidiasisailure to thrie

De. Delay

arotitis


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Its the 7a/

4E%+ counsel & offer testing to those ho appearat deliery ith F; record of an HI8 test duringpregnancy

If a oman declines HI8 testing, a signedo'Nection 4E%+ 'e attempted

;ffer testing to a child, hose mother has not 'eentested

Childs HI8 status is strictly confidential, schoolsmay not hae info unless parents allo


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+esting9+reatment

;raOuic) Gapid HI8"1 *nti'ody +est

(D* approed 119$

%hould 'e confirmed ith

DF*"CG9anti'ody testing"ne'orns9infantsreention, antiretroiral agents (PD8, *P+, F8"

iramune, etc.$


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+@ oals & ;'Necties

%loing the groth of HI8

romoting or restoring normal &D

reenting complicating infections and cancers

Improing 2uality of liferolonging surial

act? Children d@d *ID% as a result of C in the

1st

year of life 9Aencephalopathy hae a shorter lifee@pectancy


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+herapeutic 4gmt.

*ssess compliance

*ssuring adherence to med. schedule

romoting eight gain"clinical improements

include groth retardation improements, decreasehepatospleno, encephalopathy, and immune sys. @.

Futritional mgmt to help ++


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N U + R I + I O N A L D E F I C I E N C I E S

D E S + R U C + I O N O F R E D L O O D C E L L SP O I S O N I N G

*nemia


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Iron Deficiency *nemia"(p.11:$

4ost common nutritional pro'lem in E%

Caused 'y any num'er of factors that decrease thesupply of e, impair its a'sorption, increase the

'odys need for e, or affect the synthesis ofhemoglo'in (%ee o@ 35"3, p. 1!!$

7arge mil) inta)e ith lo solid food source

Gapid groth

Inade2uate diet, menses, impaired a'sorption


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athophysiology acts

irth" full term infant esupply03


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4il) 'a'y"oereight 4il) is a poor source of iron, 8it.C, -inc & louride

ale, porcelain"li)e s)inEndereightoor muscle de,edema, retarded groth,Irrita'le, tachycardic, fatigue, )oilonychia%ocial and cognitie a'ilities


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Iron Deficiency *nemia

Spooning(koilonychia):

concaveshapeofnails

occursi!h"#$


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4gmt9Fursing

reention9%creening9+eaching

Dietary counseling

Iron ortified formula9cereal up to 1! m

Iron rich foods upon solidse supplementation (errous sulfate$

"gie '9 meals98it C to a'sorption

"Qeep no more than 1m supply0to@ic


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*nemia

Ot-er (utriti)(al au!e! ) a(emia

olic acid deficiency and pernicious anemia

+o@ic effects to 'one marro, nerous system,)idneys

ehaioral pro'lems and learning difficulties,encephalopathy, sei-ures, and 'rain damage


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%ic)le Cell *nemia (p. 1!5$

*utosomal recessie disorder

Formal adult hemoglo'in is partly or completelyreplaced 'y a'normal sic)le Hg' (Hg'%$

%C* is the most common of the %CDCommon in *frican *mericans 1?1! carry the trait


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%ic)le Cell *nemia

Ender conditions of dehydration, acidosis, hypo@ia,and temperature eleations, Hg'% changes itsstructure in the cell mem'rane from a plia'le dis) toa crescent or sic)le shaped GC.

%ic)ling response is reersi'le ith o@ygenation &hydration


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%ic)le Cell +rait

Hae more Hg*, therefore asymptomatic

Fon"painful gross hematuria is the maNorcomplication (teenage9adult years$

@treme or prolonged deo@ygenation (airplane,military training$ causes splenic se2uestration"profound anemia"death


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Gesults from ? ;%+GEC+I;F &D%+GEC+I;F 8*%;";CC7E I9I (C77 D*+H$


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%C98*%;";CC7E%I8 CGI%I%

8asocclusie, splenic se2uestration, aplastic,hyperhemolytic, stro)e, chest syndrome, infection

7ocali-ed9generali-ed pain*cute a'dominal pain (isceral hypo@9gallstones$riapism7o grade +


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Fursing 4gmt.? reent & +@ Crisis

Detection

*ssessment 8%, neuro, ision, hearing, resp.,I,E, musculos)eletal, pain.

reent tissue hypo@ia (actiity, stress, altitude$romote hydration

*nalgesics for pain, heat, rest, transfusions, *R

Fo Demerol

%urgery"GI%Q


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7ead oisoning

%ources? old paint, nesprint, inyl mini'linds, ceramicare, lead curtain eights, stained glass, lipstic)s,playground e2uipment.

ica

Ingestion e@ceeds e@cretion

%tored in 'one and e@creted ery sloly ia )idneys andI

%hos in 'lood first

Diagnosed 'y 'lood test of iron status, !erumerriti(, serum e, +fe 'inding cap,


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7ead oisoning ffects


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uidelines

CDC guidelines? recommend uniersal or targetedscreening on the 'asis of each states determinationof need . +his need is esta'lished using 'lood leadsureillance and other ris) factor data collected oer

time to esta'lish the status and ris) .Oues. House 'efore 1:5:S ;r 1:# 9 ongoingrenoationsS ;r H@ of lead in family9friendS


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7ead oisoning" 4anagement

Chelation therapy?remoes lead 'ycom'ining ith anotheragent

ien 9inN., rotate site ,47*(painful$

4onitor )idney f@

+reat sei-ures, anemia


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I + P

H E M O P H I L I A

Defects in Hemostasis


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Clotting Disorders


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Clotting Disorders


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Clotting Disorders"I+

Idi)pat-i t-r)m/)0t)pe(ia purpura 'I+P*ruising, petechiae, purpura

1" ee)s after a iral infection

4* to r9o malignancylatelet count = !


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Idiopathic +hrom'ocytopenic urpura (I+$

*n ac2uired hemorrhagic disorder characteri-ed 'y +hrom'ocytopeniaTe@cessie destruction of platelets urpuraTdiscoloration caused 'y petechiae 'eneath the s)in Formal 'one marro ith usual increase in large immature

platelets


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I+


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Hemophilia *

R lin)ed recessiedisorder

Carried on femalechromosomeU males are

affectedDeficiency of actor #,

produced 'y the lier,necessary for the

formation ofthrom'oplastin forclotting


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Hemophilia *"%eerity Degrees

%eere"%pontaneously 'leed 9o trauma4oderate"leed ith trauma

4ild"seere trauma9surgery


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Hemophilia *

rolonged 'leeding anyhere from or in the 'ody%O9I4 'leeding

Hemarthrosis? stiffness, tingling, pain folloed 'y

decrease G;4, armth, redness, selling"ony changes and deformities common due tothis

etechiae, %pontaneous hematuria, epista@is


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Hemophilia

$%rsing&riority'%reven! &lee'ing (

)oin!s as i! maycause chronic

crippling*


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DR9 Fursing 4gmt.

H@ of 'leeding, actor #&: assays, ++, DF*testing

reention? strengthening muscle e@., safety,etc.Clotting factor deficiency replacement ('lood

factor infusion$ducation"family, 'leeding episode GIC, + after

acute phase";F7VWW

Fatl Hemophilia oundation & eneticCounseling


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Hemophilia *"*cute hase

F; G;4WW"Koint capsule may 'e stretched and'leeding may recur

V% *G;4WW"atient controls his on pain toleranceand strength


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Clotting Disorders

rotect toddlers? soft helmets, )nee pads, coer corners%imming, 'ase'all, 'as)et'all, and 'icycling

*oid contact sports (foot'all, restling, soccer, andhigh diing.$

*oid trampoline use and riding *+8s.

*micar if oral surgery is indicated (antifri'inolyticagent$

actor # replacement*pply pressure for actie 'leeding

Gefer to Fational Hemophilia oundation (FH$


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7eu)emia


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7eu)emia (p. 1#


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athophysiology

Enrestricted proliferation of immature C in the 'loodforming tissues of the 'ody, depressing 4 production,anemia, infe@., 'leeding

Infiltration and replacement of any tissue ith leu)emiccells occurs esp. lier and spleen

%ometimes diagnosed hen a cold or minor infectionlingers, fe'rility, listlessness, pallor, anore@ia, t. 7oss,petechiae, 'ruising ithout cause, 'one9Noint pain.

%9% mimic KG*9mono

+he inasion of the 4 ith leu)emic cells gradually causesa ea)ening of the 'one and a tendency for f@.

*s leu)emic cells inade the periosteum, increasing pressurecauses seere pain


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rognosis

*77"initial C count(=5


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Diagnosis

one marro aspiration or 'iopsyEsually 4 hypercellular ith 'last cells

;nce d@d, 7 done to CF% inolement


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4gmt.9ollo rotocols

Chemotherapy(antineoplastic agents$GadiotherapyImmunotherapy phases?

Induction Intensification9consol. CF% prophyla@is 4aintenance

4+?allogeneic9autologous

repare families for d@9t@procedures

roide cont emotionalsupport

Geerse isolationVI04* center of the

'one is hollo andcontains the cells thatlater 'ecome or)ing/'lood cells or leu)emiccells


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Fursing Care

4anage pro'lems ofirradiation and drugto@icity 0 F98, anore@ia,mucosal ulceration,

neuropathy, hemorrhagiccystitis, alopecia, moonface, mood changes, pain

roide faored foods 4outh care, soft

tooth'rush, mouthash,hydrogen pero@ide, saline

8incristine, num'nesstemp

+emporary, igs, hats

Decrease Fa inta)e, loo)

healthy *nalgesics

blood disorders 35 36

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