The Effects of Scleroderma on the GI Tract: A Major Case Study

Alexandra Shear 2/11/14

Introduction: !"! #s a $2 year old male! %#s he#&ht #s $ ft ' #n (1!)$* m+, and h#s adm#tt#n& -e#&ht #s 1.4 l/s (4)!4 0&+! !"! -as adm#tted -#th a 1r#mary 1ro/lem of a/dom#nal 1a#n -#th nausea and 2om#t#n&! Th#s 1at#ent -as chosen for th#s study /ecause h#s treatment #n2ol2ed nutr#t#on su11ort, and he has a se2eral med#cal cond#t#ons contr#/ut#n& to h#s 1oor health! The study /e&an 3anuary 4, 2.14 and ended 4e/ruary 1., 2.14! The focus of th#s study #s on the effects of scleroderma and lu1us on the GI tract and nutr#t#on su11ort! Social History: !"! #s not em1loyed! %e #s d#2orced and has a &#rlfr#end, -ho #s #n2ol2ed #n h#s care! !"! has t-o &ro-n ch#ldren! %e #s #nsured throu&h Med#care, 1art A and 5, and !"! l#2es at home and has home health care! %e had a /r#ef stay at an !"! has Med#ca#d!

extended care fac#l#ty /et-een h#s recent adm#ss#ons! %e #s a current smo0er! smo0ed 1/2 1ac0 a day for *$ years! Normal Anatomy and Physiology of Applicable Body Functions:

The GI tract has four /as#c funct#ons! Mot#l#ty #s the mo2ement of consumed food alon& the GI tract! Secret#on occurs -hen d#&est#2e secret#ons, such as -ater, electrolytes, en6ymes, /#le salts, and mucus, m#x -#th foods throu&h 1ro1uls#2e contract#ons and m#x#n& mo2ements #n order to max#m#6e 1otent#al a/sor1t#on! 7#&est#on con2erts com1lex molecules #nto the#r s#m1lest form, and last, a/sor1t#on 1ro2#des essent#al nutr#ents, l#0e -ater, electrolytes, 2#tam#ns, and m#nerals, to e2ery cell #n the /ody! These 1rocesses /e&#n -#th the oral ca2#ty and end -#th the lar&e #ntest#ne!1 The u11er GI tract cons#sts of the oral ca2#ty, eso1ha&us, and stomach, -h#le the lo-er GI tract cons#sts of the small and lar&e #ntest#nes! The oral ca2#ty #ncludes the l#1s, ton&ue, teeth, and 1alate! 4or mot#l#ty, the teeth /e&#n mast#cat#on, or che-#n&, -h#ch /rea0s do-n food #nto smaller 1#eces, m#xes food -#th sal#2a, and also st#mulates the taste /uds! Sal#2a #s 1roduced #n the 1arot#d, su/mand#/ular, and su/l#n&ual &lands and secreted #nto the oral ca2#ty! 182 l#ters #s 1roduced 1er day! Sal#2a has many funct#ons! Some of these #nclude: Mo#sten#n& and lu/r#cat#n& the food to fac#l#tate s-allo-#n& In#t#at#n& d#&est#on of car/ohydrates

"ro2#d#n& ant#/acter#al 1rotect#on -#th lysosomes 9#ns#n& a-ay food Enhanc#n& taste /y 1ro2#d#n& a solut#on that #nteracts -#th taste /uds :eutral#6#n& ac#d#c foods and 1rotect#n& teeth from dama&e "romot#n& oral hy&#ene /y d#ssol2#n& dead cells and fore#&n su/stances Ass#st#n& -#th s1eech /y allo-#n& the l#1s and ton&ue to mo2e freely

A /olus, or a st#c0y /all of food and sal#2a, #s formed -#th#n the oral ca2#ty, and mo2es to the /ac0 of the ca2#ty!1 The #n#t#at#on of s-allo-#n& #s 2oluntary /ut /ecomes #n2oluntary after the 1rocess /e&#ns! The /olus then mo2es to the eso1ha&us, -hose ma#n funct#on #s mot#l#ty! The eso1ha&us has /oth lon&#tud#nal and c#rcular muscles that alternate contract#ons to mo2e the /olus! The 1ur1ose of the 1haryn&eal 1hase of s-allo-#n& #s to ensure the /olus #s d#rected #nto the eso1ha&us and not the trachea!1 The u2ula seals off the nasal 1assa&e, and the laryn&eal muscles contract to close the &lott#s, -h#ch #s the entrance to the larynx! The e1#&lott#s t#lts u1-ard to ass#st #n 1re2ent#n& food from enter#n& the larynx! The eso1ha&eal 1hase #s the f#nal 1hase of s-allo-#n&! The u11er eso1ha&eal s1h#ncter (;ES+ allo-s the /olus to enter the eso1ha&us and 1re2ents a#r from enter#n& the GI tract! Mucus #s secreted /y the eso1ha&us to lu/r#cate the /olus and a#d #n successful 1assa&e! As ment#oned 1re2#ously, 1er#stalt#c -a2es mo2e the /olus do-n the eso1ha&us and to-ards the stomach! The lo-er eso1ha&eal s1h#ncter (<ES+ controls the release of the /olus #nto the stomach! The <ES also 1rotects eso1ha&eal mucosa from ac#d#c stomach contents and rema#ns closed exce1t dur#n& s-allo-#n&! S-allo-#n& #s com1lete -hen the /olus mo2es throu&h the <ES! If not all the food #s cleared from the eso1ha&us, secondary 1er#stalt#c -a2es are #n#t#ated to remo2e the left o2er food1! The stomach #s the f#nal 1ort#on of the u11er GI! It cons#sts of the fundus, cor1us, antrum, and 1ylorus! Mot#l#ty #n the stomach #n2ol2es f#ll#n&, stora&e of food, m#x#n& -#th &astr#c ju#ces, and em1ty#n& #nto the small #ntest#ne! hen em1ty the 2olume of the stomach #s a/out $. m<, /ut #t can stretch to hold more than 1... m<! 4ood #s stored #n the cor1us and m#xes -#th &astr#c ju#ces #n the antrum! The antrum has much th#c0er muscle #n order for the 1er#stalt#c -a2es to a#d #n m#x#n&! =nce the food m#xes, #t mo2es

to-ard the 1ylorus and 1ylor#c s1h#ncter! The rate of em1ty#n& throu&h the 1ylor#c s1h#ncter #nto the small #ntest#ne #s controlled /y the anatom#c structure of the stomach, nutr#ent content of the food, ner2ous system, and #s #nfluenced /y s1ec#f#c hormones! 1 to * l#ters of &astr#c ju#ces are 1roduced 1er day!1 These #nclude -ater, mucus, hydrochlor#c ac#d, en6ymes, and electrolytes! The mucus 1rotects the l#n#n& of the stomach from dama&e related to mechan#cal mo2ements and ac#d! These secret#ons are 1roduced /efore food enters the stomach to 1re1are for d#&est#on, -h#ch #n2ol2es mechan#cal and chem#cal mechan#sms! The contract#ons #n the stomach d#2#de the /olus and m#x #t -#th &astr#c ju#ces! En6ymes and ac#d secreted /y the stomach a#d #n d#&est#on of car/ohydrates, 1rote#n, and fat! A/sor1t#on #s l#m#ted #n the stomach! =nly small amounts of -ater are a/sor/ed, alon& -#th alcohol and some med#cat#ons!1 '>? of all d#&est#on and a/sor1t#on occurs #n the lo-er GI tract! The small #ntest#ne #ncludes the duodenum, jejunum, and #leum! It #s structured to 1ro2#de max#mum surface area for com1lete d#&est#on and a/sor1t#on! It #s c#rcularly folded, called the folds of @erc0r#n&, -#th f#n&erl#0e 1roject#ons, 2#ll#, r#s#n& from the mucosal surface! M#cro2#ll#, also called the /rush /order, on the surface of the 2#ll# #ncrease the surface area!1 Se&mentat#on of the small #ntest#ne allo-s for m#x#n& of #ntest#nal contents! Chyme from the stomach enters the duodenum and hormones st#mulate the se&mental contract#ons, -h#ch occur e2ery ' m#nutes #n the duodenum and at a slo-er rate farther do-n! Th#s lets the ch#me m#x -#th secret#ons! The M#&rat#n& Mot#l#ty Com1lex (MMC+ occurs -hen the small #ntest#ne #s em1ty to clean out lefto2er /acter#a and -aste! The #leocecal 2al2e controls the rate of mo2ement from the #leum to the ascend#n& colon of the lar&e #ntest#ne! The small #ntest#ne /oth 1roduces and rece#2es secret#ons, such as hormones, d#&est#2e en6ymes, /#car/onate, and /#le! 1!$ l#ters of #ntest#nal ju#ces, -ater and mucus, are secreted da#ly to 1ro2#de the a11ro1r#ate -ater8solu/le en2#ronment for d#&est#on and for 1rotect#on to the mucosa of the small #ntest#ne! hen the chyme mo2es from the stomach to duodenum, hormones st#mulate the release of 1ancreat#c and &all/ladder secret#ons! 5#car/onate from the 1ancreas neutral#6es the ac#d#c chyme to 1rotect the duodenum! "ancreat#c ju#ces also 1ro2#de the 1r#mary d#&est#2e en6ymes for starch, l#1#ds, and 1rote#ns! 4or o1t#mal a/sor1t#on, the 2#llus conta#ns access to c#rculatory and lym1hat#c systems throu&h ca1#llar#es and lym1hat#c 2essels! These 1ro2e

the necessary routes for a/sor/ed nutr#ents!1 End 1roducts of d#&est#on #n the small #ntest#ne, such as &lucose, &alactose, and am#no ac#ds, are 1r#mar#ly a/sor/ed throu&h act#2e trans1ortA ho-e2er, fructose a/sor1t#on #s affected /y the 1resence of other end 1roducts and #s a/sor/ed throu&h fac#l#tated or carr#er8med#ated trans1ort! <#1#ds are more d#ff#cult to a/sor/ due to the#r #nsolu/#l#ty #n -ater! <#1#ds are carr#ed to the enterocytes throu&h m#celles, -h#ch then form chylom#crons and are 1ass#2ely a/sor/ed #nto the lym1hat#c system! Most nutr#ents are a/sor/ed #n the duodenum and jejunum, /ut the #leum can accommodate a/sor1t#on of nutr#ents! The exce1t#on of th#s #s 2#tam#n 512, -h#ch can only /e a/sor/ed at s1ec#f#c s#tes #n the #leum!1 The lar&e #ntest#ne cons#sts of the ascend#n&, trans2erse, descend#n&, and s#&mo#d colon, -h#ch ends #n the rectum -#th the anal s1h#ncter! The mucosa of the lar&e #ntest#ne form three stra#&ht sect#ons #nstead of c#rcular folds, l#0e #n the small #ntest#ne! To 1ro2#de mo2ement, there are re1eat#n& /ands of lon&#tud#nal s0eletal muscle that follothe len&th of the colon and c#rcular smooth muscle that co2ers the ent#re or&an! Methods of mot#l#ty #nclude haustrat#on, 1ro1uls#on, mass mo2ement, and defecat#on! %austrat#on, or small sacs (called haustra+ formed /y c#rcular muscle, holds the chyme as #t m#xes -#th the secret#ons of the colon! The haustra can form and 2an#sh -hen #ntest#nal contents mo2e throu&h the colon! "ro1uls#on occurs -hen alternat#n& -a2es of relaxat#on and contract#on of smooth muscle ena/le contents of the lar&e #ntest#ne to mo2e #n /oth d#rect#ons! Th#s allo-s for adeBuate a/sor1t#on of flu#d and electrolytes, and ty1#cally lasts se2eral m#nutes! Mass mo2ement #s s#&n#f#cant contract#on of a lar&e 1ort#on of the colon! It occurs se2eral t#mes 1er day and mo2es lar&e amounts of the contents of the colon! <astly, defecat#on #s the d#stent#on of the rectum and relaxat#on of the anal s1h#ncter! Th#s #s usually under 2oluntary control!1 There are relat#2ely fe- secret#ons #n the lar&e #ntest#ne! Mucus #s 1roduced to 1rotect the e1#thel#um and a#d #n format#on of feces! "otass#um and /#car/onate are also released for flu#d and electrolyte a/sor1t#on! There #s no en6ymat#c d#&est#on #n the lar&e #ntest#ne! The 1r#mary funct#on of the colon #s to 1ro2#de a s#te for rea/sor1t#on of -ater, electrolytes, and some 2#tam#ns! Chyme enters as l#Bu#d, and dur#n& mo2ement, -ater #s rea/sor/ed, -h#ch results #n dr#er fecal matter! Th#s 1rocess normally ta0es 128)2 hours! 1 Past Medical History:

Chron#c o/struct#2e 1ulmonary d#sease (C="7+ Colla1sed r#&ht lun& %y1ertens#on Cachex#a "ulmonary hy1ertens#on "neumon#a Anx#ety 7e1ress#on Scleroderma M#xed connect#2e t#ssue d#sease <u1us Arthr#t#s Eso1ha&eal reflux Irre&ular heart /eat Cere/ral aneurysm

!"! has /een adm#tted to the hos1#tal 1C t#mes #n the 1ast year, -h#ch #ncludes h#s most recent adm#ss#on! %#s -as adm#tted from 1/4 throu&h 1/1C, 1/*1 throu&h 2/), and 2/' throu&h 1resent! %e #s a *.8day readm#t! a&a#nst med#cal ad2#ce se2eral t#mes! Present Medical Status and Treatment: Scleroderma #s a chron#c auto#mmune d#sease and #s cate&or#6ed #nto t-o ty1es!2 System#c scleros#s #n2ol2es a chron#c harden#n& of the s0#n and 2#sceral or&ans, character#6ed /y accumulat#on of f#/rous connect#2e t#ssue!* An #ncrease #n hydroxylys#ne aldehyde colla&en cross8l#n0a&es and #nflammatory cyto0#nes result #n f#/ros#s of the t#ssues! <#m#ted cutaneous scleros#s ty1#cally affects the s0#n and lun&s! Th#s ty1e of scleroderma #s also referred to as C9EST syndrome /ecause #t #s assoc#ated -#th Calc#um de1os#ts, 9aynaudDs 1henomenon (reduced /lood flo- #n res1onse to cold or stress caus#n& d#scolorat#on of f#n&ers and toes+, eso1ha&eal dysfunct#on, s0#n dama&e on f#n&ers, and telan&#ectas#a (s1#der 2e#ns+, -h#ch form the acronym C9EST! C9EST !"! has also s#&ned h#mself out of the hos1#tal

syndrome causes less dama&e to #nternal or&ans, -h#ch #s -hy #t #s cons#dered less se2ere than system#c scleros#s!2 The follo-#n& d#&est#2e system com1l#cat#ons result from system#c scleroderma! )$8'.? of scleroderma 1at#ents ex1er#ence GI sym1toms! Secondary to the s0#n, the d#&est#2e system #s the most commonly affected or&an system! 4 The GI sym1toms affect mot#l#ty, d#&est#ons, and a/sor1t#on and are ma#nly due to the f#/ros#s caused /y the d#sease! In add#t#on to the f#/ros#s, the muscles of the GI tract are -ea0ened due to lac0 of /lood flo- to the ner2es that st#mulate the d#&est#2e system! Th#s leads to a slo- and uncoord#nated mot#on #n the GI tract!$ The sym1toms may /e&#n #n the oral ca2#ty! 2.? of scleroderma 1at#ents de2elo1 Sjor&renDs syndrome, -h#ch #s assoc#ated -#th dry eyes and dry mouth! There may also /e d#ff#culty s-allo-#n& and 1oss#/le de2elo1ment of &um d#sease or ca2#t#es! The eso1ha&us #s affected #n ).8'.? of scleroderma 1at#ents -#th GI sym1toms! The most common sym1tom #s &astro8eso1ha&eal reflux (GE97+, -h#ch #s due to stomach ac#d /ac0#n& u1 #nto the eso1ha&us! GE97 #s often referred to as heart/urn and feels l#0e a /urn#n& sensat#on /eh#nd the /reast/one! Th#s #s caused /y the -ea0en#n& of the <ES, -h#ch normally only allo-s food to mo2e from the eso1ha&us to the stomach! 4 hen the <ES #s -ea0ened, #t doesnDt close as t#&htly as #t should, and ac#d#c &astr#c ju#ces mo2e from the stomach to the eso1ha&us! The 1oor mot#l#ty of the eso1ha&us does not clear the &astr#c contents, -h#ch can lead to further dama&e! A str#cture, or scarr#n& of the eso1ha&us from re1eated ac#d dama&e, may form and cause narro-#n& of the eso1ha&us and lead to dys1ha&#a, #f untreated! There #s also a h#&h chance for scleroderma 1at#ents to de2elo1 5arrettDs eso1ha&us #f the GE97 sym1toms are left untreated or uncontrolled!$ 5arrettDs eso1ha&us occurs -hen the cells that form the l#n#n& of the eso1ha&us are transformed /y chron#c ex1osure to stomach ac#d #nto cells s#m#lar to those #n the l#n#n& of the stomach!C =ther eso1ha&eal sym1toms #nclude: 7ry cou&h "ers#stent hoarse 2o#ce Asthma, -hee6#n& and shortness of /reath 9e&ur&#tat#on of food 7#ff#culty s-allo-#n&

Mouth ulcers

Gastro1ares#s, the delayed em1ty#n& of food from the stomach #nto the small #ntest#ne, #s the ma#n &astr#c sym1tom found #n 1at#ents -#th scleroderma! 9eta#n#n& food #n the stomach leads feel#n&s of nausea, fullness, /loat#n&, d#stent#on, and 2om#t#n&!) Th#s can result #n com1lete food #ntolerance and #ncrease the se2er#ty of GE97 sym1toms!4 Gastro1ares#s results from the f#/ros#s of the muscles that create 1er#stalt#c mo2ement #n the stomach and lac0 of /lood flo- to the ner2es that st#mulate th#s mot#on!> The stomach can also ha2e d#lated /lood 2essels, called s1#der 2e#ns or telan&#ectas#a! These /lood 2essels may cause chron#c GI /leed#n& #n scleroderma 1at#ents, -h#ch can lead to anem#a!4 Scleroderma affects the small and lar&e #ntest#ne #n s#m#lar -ays as the stomach! There #s /loat#n& and d#stent#on -#th nausea and 2om#t#n&, and a/dom#nal 1a#n may also occur! 9educed /lood flo- and /acter#al o2er&ro-th from sta&nant food may contr#/ute to mala/sor1t#on!$ The /acter#a normally colon#6e #n small amounts /ut mult#1ly #n the food that s#ts #n the small #ntest#ne! The lar&e amounts of /acter#a then com1ete -#th the /ody for nutr#ents, 2#tam#ns, and m#nerals! Th#s leads to #na/#l#ty to &a#n -e#&ht, -e#&ht loss, 2#tam#n and m#neral def#c#enc#es, and 1oss#/ly d#arrhea!4 A 1seudo8o/struct#on #s another 1oss#/le s#de effect of scleroderma! There #s no /loc0a&e, /ut the small #ntest#ne just sto1s -or0#n& due to -ea0en#n& and f#/ros#s of the muscles and #m1a#red mot#l#ty! Const#1at#on #s def#ned as less than three /o-el mo2ements 1er -ee0 and #s the ma#n sym1tom of scleroderma found #n the lar&e #ntest#ne! Th#s #s also due to the -ea0en#n& and f#/ros#s of the muscles and delayed trans#t! Add#t#onally, stool #ncont#nence #s another 1otent#al sym1tom and affects a/out 4.? of #nd#2#duals -#th scleroderma!$ The anal s1h#ncter #s -ea0ened and #s una/le to close t#&htly, -h#ch allo-s feces to mo2e throu&h #n2oluntar#ly!4 There #s no treatment to cure or re2erse the f#/ros#s of t#ssue caused /y scleroderma! The d#sease #s usually 1ro&ress#2e, so treatment #s a#med at l#m#t#n& dama&e and rel#e2#n& sym1toms! To hel1 -#th dry eyes and mouth, lu/r#cants can /e /ou&ht o2er the counter or 1rescr#/ed /y a doctor! Su&ar8free &um and lo6en&es can also hel1 #ncrease sal#2a 1roduct#on to treat dry mouth! If oral #nta0e #s feas#/le, mo#st foods -#thout added s1#ces and #ncreased flu#d consum1t#on #s recommended!*

GE97 can /e treated throu&h the follo-#n& med#cat#ons! "roton 1um1 #nh#/#tors: #nh#/#ts ac#d secret#on %2 /loc0ers: /loc0s the act#on of h#stam#ne on 1ar#etal cells, -h#ch decreases ac#d 1roduct#on "ro0#net#c a&ents: #ncreases contract#l#ty of the stomach and shortens &astr#c em1ty#n& t#me Ant#secretory: #nh#/#ts release of #nsul#n and other &ut hormones, slo-s rate of &astr#c em1ty#n& and small #ntest#ne trans#t t#me, #ncreases #ntest#nal -ater and sod#um a/sor1t#on Ant#dum1#n&: delays car/ohydrate d#&est#on Ant#&as a&ents: lo-ers surface tens#on of &as /u//les Antac#ds: /uffers &astr#c ac#d

It #s recommended to l#m#t or a2o#d a&&ra2at#n& foods or su/stances, such as alcohol, caff#ene, chocolate, coffee, on#ons, s1#ces, or car/onated /e2era&es! Smo0#n& cessat#ons has also /een 1ro2en to #m1ro2e GE97!2 Ele2at#n& the head of the /ed and eat#n& 2 to * hours /efore /ed may hel1 /y allo-#n& &ra2#ty to 0ee1 the stomach contents farther a-ay from the <ES! Small, freBuent meals may also hel1!* To ass#st -#th dys1ha&#a, or d#ff#culty s-allo-#n&, #f #s su&&ested to chan&e the cons#stency of food, -h#ch may reBu#re a mechan#cal soft or 1ureed d#et, de1end#n& on the se2er#ty of the dys1ha&#a!$ Eat#n& small freBuent meals and mo#st foods -#th sauces or &ra2#es ha2e also /een found to hel1 -#th s-allo-#n&! A2o#d#n& dry foods that eas#ly crum/le #s #m1ortant /ecause the crum/s could /e more eas#ly cho0ed on! Cooler tem1erature foods ha2e /een /etter tolerated -#th dys1ha&#a than hot foods! :utr#t#on su11ort may /e reBu#red, #f oral #nta0e #s not adeBuate or not a11ro1r#ate! Gastro1ares#s can /e treated -#th 1ro0#net#c a&ents, such as 9e&lan, Erythromyc#n, or ;rechol#ne! It #s su&&ested that 1at#ents s#t u1r#&ht dur#n& meals and a2o#d h#&h f#/er foods, as those may cause food to s#t #n the stomach lon&er! Small, freBuent meals are also recommended for &astro1ares#s! A soft or l#Bu#d d#et may /e /etter tolerated than sol#ds!4 If th#s /ecomes a 1ers#stant 1ro/lem, a 38tu/e may /e necessary to /y1ass the stomach!

5acter#al &ro-th #s ty1#cally treated -#th ant#/#ot#cs, and often an ant#/#ot#c med#cat#on must /e ta0en once a month to 0ee1 /acter#al le2els lo-! "romot#l#ty a&ents may also /e /enef#c#al! 9e&ular consum1t#on of yo&urt -#th 1ro/#ot#cs, or /o-el8fr#endly /acter#a, may 1ro2#de sym1tomat#c rel#ef from /acter#al o2er&ro-th and hel1 to 1re2ent future o2er&ro-th!$ The only treatment for a 1seudo8o/struct#on #s com1lete rest of the small #ntest#ne! Se2eral stud#es ha2e sho-n that the small /o-el can /enef#t from 2 to * months of rest -#th 1arenteral nutr#t#on to meet nutr#t#onal needs! The 1romot#l#ty a&ent, =ctereot#de, has /een found successful at treat#n& attac0s and 1re2ent#n& future 1seudo8 o/struct#ons #n some 1at#ents! A 38tu/e may also /e used to 1ro2#de E2ent#n&F and release a#r from small #ntest#ne to 1re2ent d#stent#on$ Const#1at#on can /e rel#e2ed us#n& stool softeners or laxat#2es and consum#n& adeBuate flu#ds, l#0e -ater or 1rune ju#ce! <astly, classes to teach /#ofeed/ac0 thera1y to #m1ro2e 2oluntary sBuee6#n& and the funct#on of the anal s1h#ncter are offered at scleroderma centers to treat stool #ncont#nence! "rel#m#nary stud#es ha2e also found that sacral ner2e st#mulat#on may /e hel1ful!4 !"!Ds sym1toms u1on adm#ss#on -ere a/dom#nal 1a#n -#th d#stent#on, nausea, 2om#t#n&, -e#&ht loss, and se2ere malnutr#t#on! These are all sym1toms l#0ely caused /y the f#/ros#s and muscle -ea0en#n& found #n scleroderma 1at#ents! <a/oratory f#nd#n&s can /e found #n a11end#x A! Med#cat#ons can /e found #n a11end#x 5! =/ser2a/le 1hys#cal and 1sycholo&#cal chan&es I 1at#ent Med#cal treatment #ncluded: 1/4: S8ray #n the E7, sho-ed a small /o-el o/struct#on and an unchan&ed "EG tu/e 1os#t#on! 1/4: Electrocard#o&ram (E@G+ #n the E7, sho-ed s#nus rhythm -#th f#rst de&ree /loc0, 2entr#cular rate of >) /eats 1er m#nute -#th "9 #nter2al 22$ ms, G9S durat#on 1.$ ms, GTC *'' ms, lo- 2olta&e G9S, and nons1ec#f#c ST/T -a2e chan&es! 1/): E@G, -as unreada/le due to 1at#ent sha0#n&! 1/*1: "all#at#2e care consult o "t decl#ned hos1#ce or extended care fac#l#ty!

1/*1: S1eech thera1y e2aluat#on, found m#ld 1haryn&eal dys1ha&#a o 9e2#e-ed 2#deo s-allo- from C/2.1* 2/*: Eso1ha&o&astroduodenosco1y (EG7+, found se2ere eso1ha&eal #nflammat#on 2/4: "sych consult, found 1at#ent mentally com1etent 2/1.: A/dom#nal x8ray #n E7, found d#stent#on of se2eral loo1s of /o-el 2/1.: CT scan of 1el2#s #n E7, found 1art#al small /o-el o/struct#on -#th d#stended 1rox#mal small /o-el loo1s 2/1.: E@G #n E7, found atr#al tachycard#a -#th 9H9

Sur&#cal treatment #ncluded: 1/)/14: Ex1loratory la1arotomy -#th 38tu/e 1lacement !"! -as on an enteral nutr#t#on re&#men 2#a G8tu/e at home! %e stated he -as us#n& =smol#te 1!$ Calor#e at a cont#nuous rate E24/),F so he could tolerate the formula /etter! %e sa#d he l#0es to eat, /ut he al-ays thro-s #t /ac0 u1! "at#ent sa#d he mostly a2o#ds food /ecause of the nausea and 2om#t#n&! ;1on adm#ss#on on 1/4, eaten or &#2en formula -#th#n 24 hours! !"!Ds current 1rescr#/ed d#et #s :"=! %e #s on total 1arenteral nutr#t#on (T":+, 2.? dextrose, 4!2$? am#no ac#d formula at $. m</hr -#th 2$. m< 2.? l#1#ds da#ly! Th#s 1ro2#des 1$C. 0cals and $1 &rams of 1rote#n! !"! -as made :"= on 2/* /ecause of se2ere eso1ha&eal #nflammat#on! "re2#ously, he -as on a re&ular d#et and #nstructed to eat for 1leasureA ho-e2er, he often could not tolerate oral #nta0e and -ould ha2e nausea and emes#s after the meal! 5elo- #s a l#st of the hos1#tal! 1/4: !"! made :"= #n E7 !"!Ds d#etary chan&es -h#le he has /een #n !"! had not

Medical Nutrition Therapy:

1/C: "": #n#t#ated /ecause 1at#ent -as una/le to tolerate oral #nta0e or tu/e feed o 1.? dextrose, 4!2$? am#no ac#d at >. m</hr -#th 2$. m< 2.? l#1#ds da#ly and mult#2#tam#n * t#mes 1er -ee0 14)' 0cals and >2 & 1rote#n 1ro2#ded throu&h "":

1/): "": held for sur&ery 1/>: "": restarted 1ost8sur&ery

1/1.: Tu/e feed (T4+ 2#a ne-ly 1lace 38tu/e #n#t#ated and "": s-#tched to T": o T4: =smol#te 1!$ Cal at 2. m</hr -#th a &oal rate of 4$ m</hr )2. 0cals and *. & 1rote#n 1ro2#ded

o T":: 2.? dextrose, 4!2$? am#no ac#ds I $. m</hr -#th no l#1#ds 1.2. 0cals, $1 & 1rote#n 1ro2#ded

1/1.: T4 sto11ed due to /loody and foul smell#n& res#duals from 38tu/e 1/11: T4 restarted -#th same formula at 2. m</hr 1/1481/1C: T4 #ncreased to *$ m</hr -#th same formula 1/1C: !"! refused T4 /ecause he Efelt fullF

1/1C: T": sto11ed after /a& com1leted 1/*1: #tF 2/1: T4 2#a 38tu/e and "": formula throu&h central l#ne started o =smol#te 1!$ Calor#e at 1$ m</hr da#ly 1112 0cals, $1 & 1rote#n 1ro2#ded $4. 0cals, 2* & 1rote#n 1ro2#ded !" refused T4 at EC4 and at hos1#tal /ecause h#s Estomach cannot tolerate

o 1.? dextrose, 4!2$? am#no ac#d at $. m</hr -#th 2$. m< 2.? l#1#ds

2/1: 9efused T4 shortly after start#n& due to nausea 2/*: :"= status recommended /y M7 due to se2ere eso1ha&eal #nflammat#on 2/$: "": #ncreased to ). m</hr o 12$) 0cals, )1 & 1rote#n 1ro2#ded 2/$82/): T4 started at 1. m</hr and sto11ed #nterm#ttently 2/C: Cycl#c "": tr#al o ). m</hr for 12 hours o 14. m</hr for 12 hours

2/): 2/):

!"! refused T4 due to /ro-n, foul smell#n& emes#s !"! to cont#nue cycl#c "": u1on d#schar&e to home

2/1.: T": #n#t#ated o 2.? dextrose, 4!2$? am#no ac#ds at $. m</hr -#th 2$. m< 2.? l#1#ds da#ly 1$2. 0cals, $1 & 1rote#n 1ro2#ded !"!Ds d#etary treatment are -e#&ht &a#n and 1re2ent#n& further

The 1r#mary o/ject#2es of malnutr#t#on!

!"! has /een de1ressed -h#le adm#tted! %e has as0ed for food -h#le /e#n& :"=, and he has refused E: /ut has acce1ted nutr#t#on 2#a T":! There -as a s#&n#f#cant amount of -e#&ht loss from h#s d#schar&e on 1/1C to readm#ss#on 1/*1, /ut there has /een some -e#&ht &a#n s#nce readm#ss#on on 1/*1, and h#s 5MI #ncreased from 11 to 12! "ES STATEME:T The follo-#n& are !"!Ds nutr#t#on d#a&noses throu&hout h#s adm#ss#ons! Malnutr#t#on related to scleroderma as e2#denced /y $? -e#&ht loss #n 1 month -#th se2ere muscle and fat loss! o Malnutr#t#on related to scleroderma as e2#denced /y 2C? -e#&ht loss #n 2 months and 5MI of 11!$! o Malnutr#t#on related to scleroderma as e2#denced /y 2.? -e#&ht loss #n 2 months and 5MI of 12!)*! ;nder-e#&ht related to #nadeBuate ener&y #nta0e as e2#denced /y 5MI 14!)*! !"Ds 1resent nutr#t#onal status #s 1oor! %e #s extremely under-e#&ht and una/le to tolerate tu/e feed#n& or oral #nta0e! =n h#s f#rst adm#ss#on on 1/4, !"!Ds nutr#t#on needs -ere calculated us#n& *.8*$ 0cal/0& /ody-e#&ht (142281C4' 0cals+, 181!2 & 1rote#n/0& /ody-e#&ht (4)8$) & 1rote#n+, and 1 m</0cal flu#d (142281C4' m<+! %e needs are noh#&her due to -e#&ht loss! ;1on adm#ss#on on 1/*1, h#s needs -ere est#mated ust#n *$84. 0cals/0& /ody-e#&ht (1*'C81$'C 0cal+, 1!$82 & 1rote#n/0& /ody-e#&ht ($'8)' & 1rote#n+, and 1 m</0cal flu#d (1*'C81$'C m<+! !"!Ds T": #ncludes a mult#2#tam#n/m#neral * t#mes 1er -ee0! /een a/le to tolerate oral #nta0e or enteral nutr#t#on, !"! -ould /enef#t from da#ly adm#n#strat#on, /ut the nat#onal shorta&e 1re2ents th#s! 5ecause he has not !"! needs T": to meet h#s est#mated nutr#t#onal needs and 1re2ent -e#&ht loss! The current T": re&#men #s meet#n& 1..? of h#s calor#e needs and >'? of h#s 1rote#n needs!

Se2eral of the 1hys#c#ans noted th#s 1at#ent as noncom1l#ant and un-#ll#n& to follo- #nstruct#ons! !"! has s#&ned h#mself out a&a#nst med#cal ad2#ce #n the 1ast, /ut #t #s unclear #f he #s currently noncom1l#ant -#th treatment! %e does refuse T4 /ecause he says he cannot tolerate them, or he feels too full, -h#ch #s l#0ely due to h#s 1ro&ress#2e scleroderma! %e has a su11ort#2e fam#ly, as /oth h#s &#rlfr#end and s#ster are #n2ol2ed #n h#s care and ha2e /een at h#s /eds#de throu&hout h#s hos1#tal adm#ss#on! 9ecommendat#ons for home T": to rest small #ntest#ne ha2e /een made throu&h 1at#entDs charted notes! made! General cond#t#ons u1on d#schar&e Prognosis: The 1hys#c#ans ha2e noted !"!Ds 1ro&nos#s as &uarded and 1oor due to Eon&o#n& med#cal nonadherence!F The 1at#ent #s not -#ll#n& to cons#der an extended care fac#l#ty or hos1#ce! %e says he #s Etry#n& to do e2eryth#n& to &et -ell!F 4rom a nutr#t#on stand1o#nt, !"Ds 1ro&nos#s #s &uarded, due to h#s -e#&ht loss, extremely lo- 5MI, and #ntolerance of enteral nutr#t#on and oral #nta0e! Summary and onclusion: Th#s -as a 2ery challen&#n& case study! I 0ne- #t -as &o#n& to /e com1l#cated -hen I chose th#s 1at#ent, /ut I d#dnDt ant#c#1ate ho- much t#me and research I -as &o#n& to 1ut #nto #t! IDm &lad I had the o11ortun#ty to ha2e a 1at#ent -#th scleroderma, s#nce #tDs a rare d#sease, and IDm so &lad I -as a/le to 1resent my case study to the 97s! -as /enef#c#al and can hel1 h#m heal and #m1ro2e! !"!Js case 1ro2#ded a &reat learn#n& o11ortun#ty for me, and I ho1e my #n1ut #n h#s treatment !"! #s currently adm#tted, so no d#schar&e follo-8u1 1lans ha2e /een

5#/l#o&ra1hy 1+ :elms M, Sucher @, 9oth S<! :utr#t#on Thera1y and "atho1hys#olo&y! 2nd ed! Cen&a&e <earn#n&A 2..C! 2+ Escott8Stum1 S! :utr#t#on and 7#a&nos#s89elated Care! )th ed! 5alt#more M7: <#11#ncott #ll#ams K #l0#nsA 2..>!

*+ <! @athleen Mahan MS 97 C7E, Syl2#a Escott8Stum1 MA 97 <7:, 3an#ce < 9aymond MS 97 C7! @rauseJs 4ood and the :utr#t#on Care "rocess! 1*th ed! St! <ou#s, M=: SaundersA 2.12! 4+ @hanna 7! Med#cal 9e1ort: 7#&est#2e System (Gut, Gastro8#ntest#nal+ In2ol2ement #n Scleroderma! Scleroderma 9esearch 4oundat#on: ;n#2ers#ty of M#ch#&anA 2.12! $+ 4or/es A, Mar#e I! Gastro#ntest#nal com1l#cat#ons: the most freBuent #nternal com1l#cat#ons of system#c scleros#s! 9heumatolo&y (=xford+! 2..'A4> Su11l *(Su11lement *+:###*C8'! C+ 5arrettDs Eso1ha&us! Medl#ne"lus: ;!S! :at#onal <#/rary of Med#c#ne and :at#onal Inst#tutes of %ealth! A2a#la/le at: htt1://---!nlm!n#h!&o2/medl#ne1lus/ency/ art#cle/..114*!htm! Accessed 4e/ruary ', 2.14! )+ Mc:earney T, Sallam %, %unn#cutt S, et al! Gastr#c slo- -a2es, &astro#ntest#nal sym1toms and 1e1t#des #n system#c scleros#s 1at#ents! :euro&astroenterol Mot#l! 2..'A21(12+:12C'8e12.! >+ Gastro1ares#s! 3ohn %o10#ns ;n#2ers#ty! A2a#la/le at: htt1://---! ho10#nsmed#c#ne!or&/&astroenterolo&yLhe1atolo&y/L1dfs/eso1ha&usLstomach/&astro1ares #s! Accessed 4e/ruary ', 2.14!

'+ Scleroderma! Amer#can Colle&e of 9heumatolo&y! A2a#la/le at: htt1://---!rheumatolo&y!or&/"ract#ce/Cl#n#cal/"at#ents/7#seasesLAndLCond#t#ons/Scler odermaL(alsoL0no-nLasLsystem#cLscleros#s+/! Accessed 4e/ruary ', 2.14! 1.+ "rons0y MM, Cro-e 3"! 4ood Med#cat#on Interact#ons! 1)th ed! 5#rchrun2#lle, "A: 4ood8Med#cat#on Interact#onsA 2.12!