cystic fibrosis stmu

8/18/2019 Cystic Fibrosis STMU

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Care client’s with respiratory disorder

CYSTIC FIBROSIS

MUHAMMAD SHOAIB

STMU ISAMABAD


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INTRODUCTION

• Cystic fibrosis also known as CF or mucoviscidosis is a

common recessive genetic disease which affects the

entire body,causing progressive disability and often early

death.

• Approximately 30,000 Americans

have C, making it one of the

most common life!shorteninginherited diseases in "nited #tates.


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DEFINITON

• Cystic fibrosis is a disease passed down through families

that cause thick, sticky mucus to build up in the lungs,

digestive tract and other areas of the body.

• $t is one of the most common

chronic lungs disease in children

and young adults.


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ETIOLOGY

• Caused by a mutation in the gene for the protein

cystic fibrosis transmembrane conductance

regulator %C&'(.

• &his mucus builds up in the

breathing passages of

the lungs and in the pancreas, the organ that helps to break

down and absorb it.


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RISK FACTOR

• Family history. )ecause cystic fibrosis is an inherited

disorder, it tends to run in families.

• Race. Although cystic fibrosis occurs in all races, it is

most common in white people of northern *uropean

ancestry.


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PATHOPHYSIOLOGYCFTR loss of its function as a chloride ion transporter caused by misfolding protein

Abnormal sodium and chloride transport across cell membrane,causing thick

tenacious secrection in lung and pancrease

Thick,sticky respiratory secretions that are difficult to remove

cause airway obstruction,resulting in air trapping and frequent respiratoryinfection.


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Cellular Causes of Cysti

Fi!rosisCysti Fi!rosis is ause" !y a #utatio$ of t%e &e$e t%at o$tai$st%e i$strutio$s to reate ysti '!rosis tra$s#e#!ra$eo$"uta$e re&ulator (CFTR). T%is #utatio$ auses t%e se*ue$eof a#i$o ai"s i$ t%is +rotei$ to !e asse#!le" ,ro$&- ,%i% i$

tur$ auses t%e e$tire +rotei$ $ot to fol" orretly. %e$ t%ei$orretly fol"e" +rotei$ e$ters t%e E$"o+las#i Retiulu# (ER)-t%e ER reo&$i/es t%e i$orret fol"i$& of t%e +rotei$ a$" #ar0s itto !e "e&ra"e". T%us- t%e +rotei$ $e1er e1e$ rea%es t%e Gol&i-let alo$e t%e ell #e#!ra$e ,%ere it !elo$&e". Si$e t%e +rotei$

,as i$te$"e" to !e a %lori"e io$ %a$$el t%rou&% t%e +las#a#e#!ra$es of ells t%at li$e #a2or +assa&e,ays i$ t%e !o"y(su% as air,ays a$" i$testi$es) - t%e so"iu#3%lori"eo$e$tratio$s are t%ro,$ o4 t%rou&%out t%e +erso$5s !o"y. T%isulti#ately auses t%e $or#ally t%i$ #uus ,it%i$ o$e5s !o"y to

tur$ t%i0 a$" sti0y- a$" t%erefore i##o1a!le !y ilia t%at li$et%e +assa&e,ays.


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CON5T6

ymptoms in newborns may include !

+elayed growthailure to gain weight

o bowel movement %meconium(

#alty!tasting skin


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Sy#+to#s of CystiFi!rosis

• Res+iratory7 - A +ersiste$t ou&% t%at +ro"ues t%i0 #uus

- %ee/i$& or la0 of !reat%

- A lo,ere" a!ility to "o e8erise

- Re+etiti1e lu$& i$fetio$s

- A +ersiste$t stu4y $ose a$" i$9a#e" $asal +assa&es

- Di&esti1e7• Foul s#elli$& a$" &reasy stools

- U$usually s#all a#ou$t of ,ei&%t &ai$ or &ro,t%

- I$testi$al !lo0i$&- es+eially i$ $e,!or$s

- Se1ere o$sti+atio$

- Ot%er7• I$fertility is o##o$ i$ !ot% #ales a$" fe#ales- t%ou&% #ore fre*ue$tly i$

#ales

- Salty tasti$& s0i$ a$" s,eat.


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Here is a#ore 1isualre+rese$tatio$ of #a$y oft%e +ro!le#sause" !y

CystiFi!rosis.


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Dia&$osis

T%ere are #a$y ,ays for a "otor to "ia&$ose CF. No, alli$fa$ts are sree$e" for it i$ t%e U$ite" States- !ut as t%is,as o$ly ree$tly i#+le#e$te" ol"er %il"re$ or a"ultssu4eri$& fro# t%e +re1iously #e$tio$e" sy#+to#s #ay!e reo##e$"e" to !e sree$e" as ,ell.

;et%o"s of Dia&$osis7 - a$ !e +erfor#e" testi$& a sa#+le of s,eat.A!$or#ally %i&% le1els of salt a$ i$"iate t%at t%e +erso$%as CF.

- Ge$eti tests a$ also s%o, CF !y "otors o!ser1i$& t%es+ei' #alfu$tio$s o$ t%e &e$e atually res+o$si!le for t%e

"isor"er.


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Cellular Causes lea" toSy#+to#s

• T%i0 #uus !uil" u+ ours"ue to ell #alfu$tio$i$&fro# out of !ala$e so"iu#a$" %lori"e o$e$tratio$s.

T%e #uus !uil"s u+ i$ lu$&sa$" ot%er +assa&e,ays!eause it a$ $ot !e #o1e"!y ilia. As t%e #uus !uil"su+ it tra+s !ateria ,%i%

ause %ro$i lu$& i$fetio$sto "e1elo+. T%e !uilt3u+#uus also !lo0s air,aysa$" i$testi$es reati$&"i?ulty !reat%i$& a$"

se1ere o$sti+atio$.


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CON5T6

• Computeried tomography %C&(.

• /agnetic resonance imaging %/'$(

• Chest x!rays


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"#$%CA& TR#AT"#'T

.Antibiotics to prevent and treat lung and sinus

infections #g!A(ithromycin


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CO!’T"

1.Anti!inflammatory medications may help

reduce the inflammation in your lungs that iscaused by ongoing infections.

#g!teroid and %buprofen.


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CO!’T"

3.$nhaled medicines to help open theairways. )eg!"ucolytics like )isolven2(


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C*'+T

.4ene therapy. attempts to place a normal copy

of the C&' gene into affected cells

5.6xygen therapy may be needed as lung

disease gets worse.


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SURGICAL TREAT;ENT

• 7ungs transplantation re8uires surgery to

replace one or both of your lungs with

healthy lungs from a human donor.


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CO;PLICATION

• /any different types of complication can occur

with C. &he most complications affect the

respiratory,and digestive tract.

Respiratory system include !

)ronchiectasis

Chronic infectionCollapsed lungs

asal polyps


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CON5T6

• Di#esti$e co%plication

• Nutritio$al "e'ie$ies

• Dia!etes•


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PRE@ENTION

• Actually,there is no way to prevent cystic fibrosis

because it is genetic disorder.

• )ut,if people or their partner has close relatives whohave cystic fibrosis,they both might want to undergo

genetic testing before having children of they own.

• $f woman already pregnant and the test shows thather baby may be at risk of cystic fibrosis, doctor

can conduct additional tests on her developing child.


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HEALTH TEACHING

• *ncourage patient avoid smoke, dust, dirt,

fumes, household chemicals, fireplace smoke.

• &each patient clearing or bringing up mucus or

secretions from the airways.


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CON5T6

• *ncourage patient

drinking plenty of fluids.

• *ncourage patient to do exercising two orthree time a week.


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!URSI!& CAR' (A!

FOR CI'!T)ITH CYSTIC FIBROSIS


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NURSING CARE PLAN

• 'ursing diagnosis 9 $neffective airway

clearance related to excess tenacious mucus.

• #-pected outcome 9 &o improve pulmonary, 4$and pancreatic status.

Nursi$& I$ter1e$tio$ Ratio$al


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E$oura&e re&ular e8erise E8erise %el+s #ai$tai$ +%ysial,ell$ess a$" su++le#e$ts t%e+atie$ts air,ays leara$e strate&ies!y %el+i$& to loose$ +ul#o$ary

seretio$.A"#i$ister o8y&e$ t%era+y a$"reei1e "i&o8i$ a$"Bor "iuretis

To "erease t%e lu$&s ,or0.

E$oura&e ou&%i$&- "ee+ !reat%i$&e8erise a$" fre*ue$t +ositio$

%a$&es

Pro#ote lu$&s e8+a$sio$-#o!ili/atio$- a$" "rai$a&e of

seretio$s.;o$itor res+iratory rate- "e+t% a$",or0 of !reat%i$&

Res+iratory #ay re#ai$ so#e,%atra+i" !eause %y+ert%yroi" state- !ut"e1elo+#e$t of res+iratory "istress isi$"iati1e of tra%eal o#+ressio$fro# e"e#a or %e#orr%a&e.

Assist ,it% re+ositio$i$&- "ee+!reat%i$& e8erises- a$" ou&%i$& asi$"iate"

;ai$tai$s lear air,ay a$"1e$tilatio$.


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NURSING CARE PLAN

• 'ursing diagnosis 9 $mbalanced nutrition

related to poor appetite and increased calorie

expenditure.

• #-pected outcome 9 &he patient:s weight will be

stable at desired weight for height



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;o$itor foo" i$ta0e a$" ,ee0ly,ei&%t.

Re&ular #o$itori$& a$ %el+ i"e$tify$utrito$ +ro!le# t%at are se1ere.

If t%e +atie$t is too "ys+$ei to eat-s%e"ule rest +erio"s a$"!ro$%o"ilator treat#e$ts !efore#eals.

Eati$& ta0es a lot of e$er&y a$"resti$& a$ %el+ o$ser1e e$er&y!efore #eal.

Create a +leasa$t eati$&

e$1iro$#e$t.

U$+leasa$t 1ie,s or o"ors a$ s+oil

a$ a++etite.

Pro1i"e s#aller a$" #ore fre*ue$t#eals of t%e +atie$t5s fa1ourite foo".

Eati$& a lot at o$e ti#e a$ 'll u+ t%esto#a% a$" re"ue t%e roo# forlu$&.

E$oura&e fa#ily #e#!ers to !ri$&fa1ourite foo"s fro# %o#e for%os+itali/e" +atie$t.

A lar&e tray of u$a++eti/i$& foo" #ay!e #ore t%a$ a +atie$t a$ %a$"lea$" #ay s+oil t%e a++etite.


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NURSING CARE PLAN

• 'ursing diagnosis 9 Anxiety related to dyspnea.

• #-pected outcome 9

&he patient will state anxiety is controlled.

&he patient will be able to use techni8ue tocontrol dyspnea and anxiety when they occur .



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Re#ai$ ,it% t%e +atie$t ,%o is autely"ys+$ei a$" a$8ious.

Feeli$& alo$e "uri$& e+iso"es of"ys+$ea a$ i$rease" a$8iety.

Cal#ly re#i$" t%e +atie$t to !reat%slo,ly i$ t%rou&% t%e $ose a$" outt%rou&% +urse" li+s.

Duri$& aute e+iso"es of "ys+$ea-t%e +atie$t #ay for&et t%at !reat%i$&e8erises a$ %el+.

Tea% "ee+ !reat%i$& e8erises "uri$&ti#es ,%e$ a$8iety is #i$i#al a$"re#i$" t%e +atie$t to use t%e# "uri$&aute a$8iety.

Rela8atio$ a$ %el+ re"ue #uslete$sio$ a$" "istrat t%e +atie$t.

A"#i$ister a$ti a$8iety #e"iatio$. ;e"iatio$ a$ re"ue a$8iety !uta$ also "e+ress res+iratio$- sos%oul" !e use ,it% autio$.


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CONCLUSION

• Cysti '!rosis tra$s#e#!ra$e o$"uta$ere&ulator(CFTR) +lay i#+orta$t role i$fu$tio$ as a %lori"e io$ tra$s+orter.

• Alt%ou&% t%ere is $o ure for CF- treat#e$ts%a1e i#+ro1e" &reatly i$ ree$t years. U$tilt%e s- #ost "eat%s fro# CF ourre" i$

%il"re$ a$" tee$a&ers. To"ay- ,it% i#+ro1e"treat#e$ts- so#e +eo+le ,%o %a1e CF areli1i$& i$to t%eir forties- 'fties- or ol"er.


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REFERENCE

+, Te-ts Boo./

a. Li$"a S.ILLIA;S- +aula D.HOPPER.- U$"ersta$"i$&#e"ial sur&ial $ursi$& 1ol - r" e"itio$ i$ter$atio$al

e"itio$.

0,The we1side a1o2t cystic 31rosis/

a.%tt+7BB,,,.#e"ii$e$et.o#Bysti'!rosisBartile.%t#

!.%tt+7BB0i"s%ealt%.or&Btee$B"iseaseso$"itio$sB"i&esti1eBysti'!rosis.%t#l

.%tt+7BB,,,.4.or&Btreat#e$tsBT%era+iesB

".%tt+7BB,,,.youtu!e.o#B


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