Caring for Scleroderma

7/25/2019 Caring for Scleroderma

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Caring for Scleroderma

A Nurses Perspective

Juliann Nederostek, RN,

MAED, CClinical Nurse Specialist

!"omas Je#erson $niversit%

&ospital


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Epidemiolog%

Scleroderma is a rare disease

'ess t"an ()),))) people in t"e $nitedStates

E#ects more *omen t"an men

Possi+le "ormone connection, +ut unclear

Age most commonl% is +et*een (-()

%ears.oung c"ildren, %oung adults and older

adults can also develop scleroderma


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Epidemiolog%

/amil% mem+ers ma% "ave ot"erautoimmune diseases

African Americans and Native Americans

generall% "ave more severe Scleroderma Ever%ones e0perience *it" Scleroderma is

di#erent

C"octa* Native Americans in 1kla"omaare 2) times more likel% t"an t"e generalpopulation, to develop s%stemicscleroderma3


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Pat"olog%

4mmune S%stem 5 is a s%stem of+iological structures and processes*it"in an organism t"at protectsagainst disease3

Autoimmune Disorder 5 is *"en t"eimmune s%stem fails to properl%

distinguis" +et*een self and non-self, and mistakenl% attacks "ealt"%+od% tissue3


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Pat"olog%

1ver production of connective tissue 6likescarring7 on t"e skin under t"e skin

Small arteries, arterioles and capillaries

narro* causing a lack of o0%gen to tissue Cells make collagen as if t"e% are in8ured

and need to +e 90ed, and end up makingtoo muc" collagen

E0tra collagen interferes *it" normalorgan function


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'imited Scleroderma

!"ickened skin-usuall% 8ust t"e9ngers and:or face

Milder form of scleroderma

More common among Caucasians

'ess involvement of +od% organs


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CRES! s%ndrome

C-calcinosis 5 calcium deposits under t"eskin and in tissues 6 "ard *"ite dots7

R-Ra%nauds p"enomenon

E-Esop"ageal d%smotilit% - &eart+urn S-Sclerodact%l% 5 !"ick skin on 9ngers

!-!elangiectasias 5 Enlarged +lood vessels

6Red spots on t"e face and ot"er areas7


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Di#use Scleroderma

!"ickened skin involves arms, legsand trunk

4n;ammation of 8oints, tendons andmuscles

Joint motion +ecomes di


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Di#use Scleroderma 6contd7

Skin *it" pigment c"anges 6lig"t ordark7

&air loss on t"e lim+s

Decreased s*eating

Dr% skin

4nvolvement of 4nternal 1rgans=&eart, 'ungs, >astrointestinal !ract,?idne%s


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'ung 6ventilation defect7

Alveoli-primar% gas e0c"ange unit of t"e lung

Pulmonar% Capillaries-+lood vessels in alveoli

Alveolar Space:Pulmonar% Capillaries allo* for

o0%gen e0c"ange Alveoli e0perience in;ammation t"at MA. lead

to 9+rosis

Result= 4nterference in o0%gen e0c"ange


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'ung 6pulmonar%"%pertension7

Rig"t "eart pumps +lood into t"e lung

'ung:+lood vessel s%stem is a lo*pressure s%stem *it" ver% lo*

resistance

4f +lood vessels or lung tissue +ecomediseased, increased pressure in +ot"

pulmonar% circulation and t"e rig"t"eart result


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Caring for Scleroderma -'ung

S%mptoms=

S"ortness of +reat" graduall%develops overtime

Not a+le to +reat"e easil% *"en %ouare active

Coug" can +e a s%mptom of lungdisease or dr% upper air*a%mem+ranes, > 4 Re;u0

$suall% no c"est pain *it" lungdisease


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Diagnostics=

C"est 0-ra% ma% +e normal in earl%stages

'ung C! identi9es c"anges in lungtissue

Pulmonar% /unction !ests identi9esc"anges in lung function, +ut cantdi#erentiate +et*een old and ne*9+rosis

Cardiac cat"eteri@ation for


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!reatment for pulmonar%"%pertension=

10%gen at nig"t "elps to rela0 t"esmoot" muscle of t"e pulmonar%arter%

ater pills

lood t"inners

Prostac%clin analogues are "elpful totreat pulmonar% "%pertension, +utmust +e given intravenousl%


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Nursing Considerations-'ung

Nursing Care=

Buit smoking

4mprove re;u0

Compliance *it" medications

Compliance *it" diagnostic studies

&ome o0%gen t"erap% if needed

Researc" in process for ne* pills to treatScleroderma pulmonar% "%pertension


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&eart

&eart disease ma% +e t"e result ofmicrovascular disease, tissue 9+rosis,pericardial disease and in;ammation of t"e

"eart muscle3 Arr"%t"mias 6irregular "eart +eat7,

Pericarditis 6outside mem+rane of t"e"eart7, &eart /ailure

Routine risk factors for "eart diseases"ould also +e evaluated= famil% "istor%,c"olesterol, smoking


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Caring for Scleroderma - Arr"%t"mia

4rregular &eart eat S%mptoms=

lig"t-"eaded or di@@%

Palpitations

irregular pulse fainting

Diagnostic !ools=

EC> &olter &eart Monitor

!reatment=

Referral to Cardiologist 6Electrop"%siolog%7

Medication or Pacemaker


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Nursing Considerations- Arr"%t"mia

Nursing Care=

4f %ou are di@@% or lig"t-"eaded, 9nda safe

place to sit do*n to prevent a fall3

Do not attempt to drive3

4f t"is is a ne* s%mptom, notif% t"edoctor3


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Caring for Scleroderma-Pericarditis

Pericarditis is in;ammation and s*ellingof t"e outside mem+rane of t"e "eart

Pericarditis S%mptoms=

C"est pain *"en %ou +reat" deepl%

S"ortness of +reat"

&ig" fever

Diagnostic !ools= EC>, Ec"ocardiogram

P"%sical E0am


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Caring for Scleroderma-Pericarditis

Severe Pericarditis is rare inScleroderma

Severe Pericarditis treatment isdiagnosed +% t"e doctor

Mild Pericarditis usuall% does notreuire treatment, +ut is monitored

+% t"e p"%sician

Pericarditis is temporar% and gets+etter


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Nursing ConsiderationsPericarditis

Nursing Care=

Notif% t"e doctor to descri+e an% ne*s%mptoms3

Cardiologist referral as needed3

Medical e0amination and diagnosticstudies as directed +% t"e doctor3

Compliance *it" medical care

Pericarditis improves *it" treatment3


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Caring for Scleroderma-&eart /ailure

&eart failure is an uncommon "eartpro+lem t"at could occur in t"e laterstages of Scleroderma

Rig"t sided "eart failure usuall% occurs*it" pulmonar% "%pertension3

S%mptoms of rig"t sided "eart failure=

Di


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Caring for Scleroderma-&eart /ailure

'eft sided "eart failure occurs *"en scartissue develops in t"e "eart muscle andit +ecomes a less e#ective pump3

S%mptoms of 'eft sided "eart failure= Di


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Caring for Scleroderma - &eart/ailure

Diagnostics=

EC>, Ec"ocardiogram, Stress !est, and if

necessar%, a cardiac cat"eteri@ation !reatment=

Referral to Cardiologist

Medications or procedures *ill +e decided

+% t"e doctor3


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Nursing Considerations &eart/ailure

Nursing Care Rig"t Sided /ailure=

Report s%mptoms to doctor

Elevate legs *"en sitting

?eep "ead of +ed up for ease of +reat"ing

Evaluate need for supplemental o0%gen Monitor ;uid intake

Monitor +od% *eig"t


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Nursing Considerations &eart /ailure

Nursing Care 'eft Sided /ailure=

Report s%mptoms to t"e doctor

Elevate "ead of +ed

Pace activit% to allo* for rest periods Monitor s"ortness of +reat" episodes

Evaluate t"e need for o0%gen

Monitor ;uid intake

Monitor +od% *eig"t 6*eig"t gain ma%

indicate ;uid retention 7


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Caring for Scleroderma - ?idne%

?idne% disease or renal crises occursearl% in patients *it" di#usescleroderma3

Renal +lood vessels constrictdropping t"e +lood ;o* to t"ekidne%3

$ntreated, t"e lo* +lood ;o* leadsto kidne% damage and failure3

Reversi+le condition *it" treatment

no* availa+le3 6ace in"i+itors7


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Caring for Scleroderma -?idne%

Scleroderma Renal Crisis occurs *"en t"e+lood pressure can go from normal levels todangerousl% "ig" levels in a matter of da%s3

?idne% damage can occur in a matter of"ours or da%s3

4n t"e earl% stages, t"ere are no s%mptoms3

After t"e 9rst fe* da%s, malignant p"ase

"%pertension can cause= "eadac"es, nausea,and vomiting


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Nursing Considerations -?idne%

Nursing Care= 6&o* to Prevent ?idne%Damage7

Detect and treat "ig" +lood pressureuickl%3

$se a "ome +lood pressure monitor3

Call %our doctor 4MMED4A!E'. if %our+lood pressure is "ig"3 64f t"e topnum+er goes over () or t"e +ottomnum+er goes over )7


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Nursing Considerations -?idne%

>et %our +lood pressure do*n rig"ta*a%

Medication dose *ill +e c"anged

'a+orator% studies to c"eck kidne%function

4f necessar%, "ospitali@ation toac"ieve rapid control of +loodpressure

F!ake %our +lood pressure at t"e

same time ever% da%


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>astrointestinal !ract -Mout"

Dr% mout" is result of in;ammation:9+rosis of t"esalivar% glands

Saliva is GH *ater, en@%mes, electrol%tes, mucous,and anti-+acterial compounds

Saliva lu+ricates and protects t"e tongue, teet", andtissues of t"e mout"

Saliva +egins t"e digestive process and +reaks do*nfood caug"t in t"e teet"

Dr% mout" results in increased plaue, toot" deca%and gum disease

Decreased mout" opening ma% +e t"e result of facialskin tig"tness3


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>astrointestinal - Mout"

Nursing Care=

Decreased mout" opening causes di


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>astrointestinal 5 Esop"agus 5Stomac" 5 Small or 'arge o*el

>astrointestinal tract is involuntar%smoot" muscle t"at can +e a#ected3

Peristalsis is t"e normal esop"ageal,stomac" and +o*el *all motion t"atmoves food t"roug" t"e upper andlo*er digestive tract

4n;ammation and 9+rosis results indecreased

motilit% or d%smotilit% of t"is smoot"

muscle


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>astrointestinal S%mptoms

D%sp"agia is di


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>astrointestinal S%mptoms

!%pical gastrointestinal re;u0 is"eart+urn3

At%pical gastrointestinal re;u0 is c"est

pain, gagging, or dr% coug"3 4f untreated, ma% result in esop"ageal

in;ammation, esop"ageal stricture, or

gastrointestinal +leeding3 Alteration in +o*el elimination presents

as constipation or diarr"ea3


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Caring for Scleroderma 5>astrointestinal

Diagnostics=

Endoscop% 5 direct visuali@ation oft"e upper

gastrointestinal tract:esop"agus

Colonoscop% 5 direct visuali@ation oft"e lo*er gastrointestinal tract:smalland large intestine

>astroenterologist "as t"e a+ilit% toalso complete +iopsies along t"e

tract to furt"er evaluate an% c"anges


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Caring for Scleroderma ->astrointestinal

Medications=

Antacids 6M%lanta, Maalo0, !ums, Di->el7

&2 lockers 6!agamet, Iantac,Pepcid, A0id7

Proton Pump 4n"i+itors 6Prilosec,Prevacid7

1t"ers 6Carafate, Propulsid7


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Nursing Considerations ->astrointestinal

Nursing Care=

Dietar% Advice to avoid certain foods anddrink t"at mig"t make "eart+urn *orse=

Alco"ol and C"ocolate Acidic foods 6oranges, tomato sauce7

/ried foods 6"ig" fat, fast food, nuts, dair%7

Ra* vegeta+les:1nions Spic% food


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Nursing Care=

Dont eat *it"in 2 "ours of +edtime

Eat slo*l% and sitting up C"e* food carefull% and s*allo*

+efore t"e ne0t +ite

Drink sips of *ater +et*een +ites Eat small freuent meals


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Nursing Care=

Elevate t"e "ead of %our +ed at least inc"es on *ooden +locks 6!"is lifts

%our esop"agus a+ove %our stomac"7 Decrease pressure on %our stomac"

6limit +ending, vigorous e0ercise or

tig"t clot"ing7 !r% to keep %our +od% *eig"t in a

"ealt"% range3


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Skin

Assessment is completed utili@ing askin score3

Palpation of skin determines skint"ickening

Determines classi9cation of patientsdisease as limited or di#use

scleroderma Active disease is manifested +%

in;ammator% signs suc" as

edematous skin

C i f S l d


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Caring for Scleroderma -Skin

S%mptoms=

Dr% skin-collagen in t"e inner la%er of t"e skindestro%s s*eat and oil glands3 !"e outer la%er oft"e skin "as fatt% su+stances t"at moisten t"e

skin3 4n Scleroderma, t"is outer la%er gets t"innerso t"ere is less oil3

4tc"ing

$lcers

Calcium deposits

Discoloration

Enlarged +lood vessels 6telangiectsias7


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Nursing Considerations 5 Skin

Stop Dr% Skin=

!urn t"e t"ermostat do*n3

$se a mild soap3

'imit soap to certain areas36$ndert"e arms and groin7 Rinse *ell3

$se *arm, not "ot *ater3 $se +a+% oil for +at"ing36Caution=

!u+ *ill +e slipper%7

N i C id ti


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Nursing Considerations -Skin

$se special soaps and s"ampoos3

6&%poallergenic, fragrance free cosmetics7

$se moisturi@ers and moisturi@e often3

$se gloves 6"en "andling "ouse"old cleaners7

Avoid certain ointments:medications3

6Dermoplast or anti"istamines as t"e% dr%7

Avoid electric +lankets e0cept to *arm %our+ed3

N i C id ti


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Moisturi@ers=

Eucerin Cream or 'otion

Neutroderm

'u+riderm

Alp"a- ?eri 'otion

Eutra 'otion

N i C id ti


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Canderm%l Cream

Neutrogena 5 Nor*egian /ormula

Auap"or 1intment

'acticare 'otion

Moisturel

Comple0 5 (


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land Soaps=

Alp"a ?eri

Aveeno ar 6Dr% Skin7

Neutrogena6Dr% Skin7 asis $nscented ar

Eucerin Dr% Skin Cleansing ar

Moisturel Sensitive Skin Cleanser

Dove

Cetap"il


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S"ampoos=

Neutrogena Regular S"ampoo

D&S Clear S"ampoo

Duple0

Progaine


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Sunscreen=

1nl% use sunscreen containing SunProtection /actor 6SP/7 greater t"an or

eual to ( Avoid t"e sun if %ou are on medications

t"at make %ou sun sensitive

Avoid= All sunscreens t"at contain Pa+aor Pa+a Ester skin reactions ma% occur3

N i C id ti


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'aundr% Detergents=

All Po*der

Das" Po*der

4vor% Sno*

Safeskin

Rinse out of clot"es *ell so reducesitc"ing and irritation


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Musculoskeletal

Muscle *eakness is due to=

Autoimmune mediated in;ammator%m%ositis

Non-in;ammator% 9+rotic m%opat"% Disuse or *asting from deconditioning

Malnutrition

Side e#ect from medications suc" ascorticosteroids or lipid lo*ering agents

!endon friction ru+s and contractures

i id i


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Nursing Considerations 5Musculoskeletal

1ccupational !"erapist=

&and and 9nger e0ercises

&and splints

Range of Motion

Para9n +at"s

Massage

i C id i


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Nursing Considerations 5Musculoskeletal

P"%sical !"erap%=

6Prior to t"e start of p"%sical t"erap%,pain and fatigue "ave to +e

controlled37

>eneral e0ercise

ater aero+ics

FAll e0ercise must +e supervised +% adoctor and p"%sical t"erapist


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Ps%c"ological

Emotional response to t"e disease=

Mood disorders 5 depression

Self image 5 a#ects t"e face and t"e"ands

An0iet%:panic 5 fear of t"e disease,distress

over t"e unkno*n

Pain 5 impacts on mood, sleep c%cle,and


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Ps%c"ological

Societal role 5 maintain relations"ips*it"in t"e famil% and societ%

C"ronic disease takes control a*a% from

t"e patient and adds fear of t"e unkno*n

Patient s"ould +e a recogni@ed participantin t"eir care as part of t"e "ealt" care

team


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Se0ualit%

Se0ual function is often impaired, +utrarel% discussed3

Males ma% "ave erectile d%sfunction

secondar% to microvascular c"angesand tissue 9+rosis3

/emales ma% "ave dr% mem+ranes,

causing discomfort or pain *it"se0ual intercourse3

Professional counseling can +e

"elpful3


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Communication >uide

Prepare a list of uestions for %ourdoctors appointment3

Communicate *"at %ou *ould like to

discuss3 4nitiate %our uestions as t"e doctor

ma% not cover t"is information in

%our visit3 'et doctor kno* if %ou cant follo* plan

of care t"at is outlined3


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Communication >uide

Revie* %our treatment *it" t"edoctor3 Do not c"ange medicationsor dose *it"out speaking to t"e

doctor3 Discuss 9nancial issues as needed3

!alk to t"e doctor *"en %ou are

dressed3 4mportant medication information=

6name, purpose, dose and sc"edule,

s"ould 4 avoid certain foods or


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Communication >uide

4dentif% t"e possi+le side e#ects oft"e medication3

4dentif% t"e contact num+er for t"e

o


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Bualit% of 'ife

&ealt" care needs s"ould consider t"epatients a+ilit% to "ave control and en8o%ot"er aspects of t"eir lives3

Bualit% of life depends on personalit%traits of t"e patient, t"e p"%sician:patientrelations"ip, and social:famil% support3

Patient education, e0pert care, andcompre"ensive medical managementpositivel% in;uence t"e ualit% of life3


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References and Resources

4nternational Scleroderma Net*ork

***3sclero3org

Jo"ns &opkins Scleroderma Center

National 4nstitute of Art"ritis and

Musculoskeletal and Skin Diseases4nformation Clearing"ouse

"ttp=::***3niams3ni"3gov:&ealt" 4nfo:Scleroderma:default3asp
http://www.sclero.org/http://www.niams.nih.gov/Health%20Info/Scleroderma/default.asphttp://www.niams.nih.gov/Health%20Info/Scleroderma/default.asphttp://www.niams.nih.gov/Health%20Info/Scleroderma/default.asphttp://www.niams.nih.gov/Health%20Info/Scleroderma/default.asphttp://www.sclero.org/


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National 4nstitute of Art"ritis andMusculoskeletal L Skin Diseases 6N4AMS7

***3niams3ni"3gov

Sclerodema Clinical !rials Consortium

***3sctc-online3org

Scleroderma Researc" /oundation

***3sclerodermaresearc"3org
http://www.niams.nih.gov/http://www.sctc-online.org/http://www.sclerodermaresearch.org/http://www.sclerodermaresearch.org/http://www.sctc-online.org/http://www.niams.nih.gov/


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!"e Art"ritis /oundation

***3art"ritis

!"e Scleroderma /oundation***3scleroderma3org

***3sclero3org:c"apter:dela*arevalle%:
http://www.arthritis/http://www.scleroderma.org/http://www.sclero.org/chapter/delaware_valley/http://www.sclero.org/chapter/delaware_valley/http://www.scleroderma.org/http://www.arthritis/

Caring for Scleroderma

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