Ingaba ulusu lomntwana wakho luyaqina kwaye luqina? Masithethe nge-Juvenile Systemic Scleroderma!

Ngaba ukhe waphawula ukuba ulusu lomntwana wakho lutshintshe kancinci ngequbuliso? Mhlawumbi ulusu luye lwaba rhabaxa, lwaqina, kwaye luvakala lwahlukile xa luchukunyiswa? Ukuba uqaphela into efana naleyo, kufuneka ukhathazeke kancinci. Kuba, oku ngamanye amaxesha kunokuba luphawu lwesifo esibizwa ngokuba yiJuvenile Systemic Scleroderma , esithetha ngaso namhlanje. Ungakhathazeki, siza kuthetha ngale nto ngokweenkcukacha.

Yintoni iJuvenile Systemic Scleroderma?

Ngamafutshane, i-Juvenile Systemic Scleroderma (ekwaziwa ngokuba yiSystemic Sclerosis ) yimeko apho ulusu lomntwana wakho luba lukhuni kwaye luqine ngaphezu kwesiqhelo. Yicinge njengephepha lerabha elikhula kancinci kancinci libe yiplastiki eqinileyo. Ayipheleli nje eluswini. Ngamanye amaxesha, inokuchaphazela nezinye izitho zomzimba womntwana. Igama elithi 'systemic' lithetha ukuba inokuchaphazela wonke umzimba womntwana.

Esi sisifo esibangelwa kukuzikhusela komzimba . Oko kukuthi, inkqubo yokuzikhusela yomzimba wethu, inkqubo yokuzikhusela yomzimba, iqala ngempazamo ukuhlasela iiseli zayo eziphilileyo. Kwimeko yeScleroderma, xa inkqubo yokuzikhusela yomzimba ihlasela iiseli zolusu ngale ndlela, kuvela ukudumba okanye ukudumba kolusu. Ngenxa yokudumba, iproteni ebizwa ngokuba yi-collagen, enika ulusu lwethu ukuqina kunye namandla, iqala ukuveliswa ngokugqithisileyo. Kanye njengokuba isilonda senzeka xa kukho ukwenzakala, oku kuveliswa kwe-collagen engaphezulu kubangela i-fibrosis okanye ukuqina kolusu okufana nesilonda.

Ngaba kukho iintlobo eziphambili zeSystemic Scleroderma?

Ewe, kukho iintlobo ezintathu eziphambili. Masibone ukuba zeziphi.

1. I-Diffuse cutaneous systemic sclerosis: Kolu hlobo, ukuqina kolusu lomntwana kunokubonakala emzimbeni wonke. Kwakhona, kunokubakho iingxaki ngokusebenza kwamalungu angaphakathi omzimba. Khawuthelekelele, kungekuphela nje iingalo, imilenze, nobuso, kodwa neendawo ezifana nesifuba nesisu ziqala ukujiya.

2. I-Limited cutaneous systemic sclerosis (CREST syndrome): Le ikhethekile kancinci. Ikwabizwa ngokuba yi -CREST syndrome . I-CREST sisifinyezo esenziwe ngokudibanisa oonobumba bokuqala beempawu ezintlanu eziphambili zale meko.

• C - I-Calcinosis: Oku kuthetha ukuba i-calcium igcina phantsi kolusu ize yenze amaqhuma amancinci.
• R - Isiganeko sikaRaynaud: Oku kwenzeka xa iminwe (izandla neenyawo) zijika zibe mhlophe, zibe luhlaza okwesibhakabhaka, zize zibe bomvu xa zibekwe emakhazeni okanye xa ziphantsi koxinzelelo. Kuba nzima ukulawula ubushushu bomzimba.
• E - Ukungasebenzi kakuhle kokuhamba kwesisu: Oku kuthetha ukuba umbhobho othwala ukutya ukusuka emqaleni ukuya esiswini (umqala) awusebenzi kakuhle xa siginya. Oku kunokubangela isitshiso sentliziyo.Izinto ezinjalo zinokwenzeka.
• S - Sclerodactyly: Ukujiya nokuqina kolusu kwiminwe. Iminwe ingadumba ize iqine njengeesoseji.
• I-T - I-Telangiectasia: Imithambo yegazi emincinci eluswini iyakhula, ibonakale njengeentambo zesigcawu okanye amachaphaza abomvu.

3. I-Systemic sclerosis sine scleroderma: Oku kuxa amalungu angaphakathi omzimba echaphazeleka ngaphandle kokuqina okanye ukuqina kwesikhumba. I-'Sine scleroderma' ithetha 'ngaphandle kwe-scleroderma (ukuqina kwesikhumba)'. Oku kuthetha ukuba nangona kusenokungabikho tshintsho lubalulekileyo eluswini, amalungu angaphakathi anokuchaphazeleka.

Ngubani onokuthi abe ne-Juvenile Systemic Scleroderma? Ixhaphake kangakanani?

Le meko ibizwa ngokuba yiJuvenile Systemic Scleroderma inokuchaphazela nabani na walo naliphi na ubudala, nokuba ungowasiphi na isini. Nangona kunjalo, kunqabile kakhulu ukuba abantwana abancinci, oko kukuthi, abantwana abangaphantsi kweminyaka eli-10 ubudala, bayifumane. Igama elithi 'ulutsha' ngokwalo lithetha ukuba le yimeko echaphazela abantwana. Ukuba ujonga izibalo kwilizwe elifana ne-United States, kuthiwa kukho abantwana abaphakathi kwama-5,000 nama-7,000 abaneScleroderma. Malunga ne-2% yabantu bonke abaneScleroderma bachatshazelwa sesi sifo ngaphambi kokuba bafikelele kwiminyaka eli-10 ubudala. Abanye abangama-7% bajongana nale meko phakathi kweminyaka eli-10 neli-19 ubudala. Ngoko ke, esi sisifo esingaqhelekanga kakhulu .

Ziziphi iimpawu ze-Juvenile Systemic Scleroderma?

Kule meko, iimpawu zinokuchaphazela ulusu lomntwana kunye namalungu angaphakathi. Ukongeza, abantwana abaninzi abaneSystemic Scleroderma nabo bafumana imeko ebizwa ngokuba yiRaynaud's phenomenon .

Iimpawu ezichaphazela ulusu kunye nezicubu

Okokuqala, makhe sijonge indlela oku okuchaphazela ngayo ulusu kunye nezicubu ezinxulumene noko:

• Ulusu luphelelwa kukuqina. Luba rhabaxa, luqine, kwaye lungabonakali.
• Umsebenzi wezandla uyancipha. Ulusu luyaqina, ingakumbi kwiminwe nasezintendeni zezandla, nto leyo eyenza kube nzima ukugoba izandla nokubamba izinto.
• Imithambo yegazi ebomvu ivela eluswini, ingakumbi ezandleni, ebusweni, nakwiinzipho (telangiectasias). Ezi zinokubonakala njengeentambo ezincinci zesigcawu.
• I-Calcinosis kukwakheka kwamaqhuqhuva amancinci e-calcium phantsi kolusu okanye kwenye indawo. Ngamanye amaxesha anokuvakala ngathi aqinile.

Ekuqaleni, ulusu lwezandla neenyawo lunokubonakala ludumbile kwaye lukhulisiwe. Ekuhambeni kwexesha, ulusu lomntwana lunokuqina, lube lukhuni, kwaye lunokuba nemibimbi, iindawo ezitshonileyo, okanye imingxunya emincinci. Umzekelo, ezinye iiminwe zabantwana zinokudumba njengeesoseji, emva koko zibe lukhuni kwaye zibengezele.

Iimpawu ezichaphazela amalungu angaphakathi

I-Systemic Scleroderma inokuchaphazela nezitho zangaphakathi zomntwana. Iimpawu zingabandakanya:

• Ukudumba kwamalungu, ukuqina kwawo, kunye nentlungu. Njenge-arthritis.
• Izilonda ezikwiminwe. Ezi zilonda zithatha ixesha elide ukuphola.
• Iingxaki zenkqubo yokugaya ukutya. Umzekelo, isitshiso sentliziyo, ubunzima bokuginya, indle ekhululekileyo (urhudo), kunye nokuqaqamba kwesisu.
• Iingxaki zokuphefumla. Usenokuba nokukhohlela okungapheliyo okanye ubunzima bokuphefumla.
• Iingxaki zezintso. Oku kunokubangela uxinzelelo lwegazi oluphezulu (uxinzelelo lwegazi oluphezulu) .
• Ukudinwa ngalo lonke ixesha. Ukudinwa ngokukhawuleza nangona sele wenze into encinci.
• Ubuthathaka bemisipha.

Ixesha elininzi, le meko ayibuhlungu. Ngenxa yoko, abanye abantwana basenokungazi nokuba baneScleroderma. Yiyo loo nto umntwana ofunyaniswe eneSystemic Sclerosis kufuneka abonane nogqirha rhoqo ukuze ahlole uxinzelelo lwegazi oluphezulu, imiphunga, izintso, amathumbu, kunye neengxaki zentliziyo.

Isiganeko sikaRaynaud

Olu luphawu olwahlukileyo. Ingxaki kaRaynaud kukuba xa iminwe yomntwana kunye neenzwane zakhe zitshintsha umbala xa ebanda okanye xa exinezelekile. Okokuqala zijika zibe mhlophe , emva koko zibe luhlaza okwesibhakabhaka , kwaye ekugqibeleni zibe bomvu . Olu luphawu olunokubonwa kwizigaba zokuqala zesifo. Ngamanye amaxesha olu lunokuba luphawu lokuqala lokuba kukho iSystemic Sclerosis.

Ezinye iimpawu ezinokwenzeka ngesifo sikaRaynaud ziquka:

• Ndiziva ndidiniwe ngalo lonke ixesha
• Intlungu yamalungu
• Ubunzima bokuginya ukutya
• Isisu esibuhlungu
• Ukudumba kwesifuba
• Urhudo
• Ubunzima bokuphefumla

Kutheni i-Juvenile Systemic Scleroderma isenzeka?

Enyanisweni, imbangela echanekileyo ye-Juvenile Systemic Scleroderma ayikaziwa . Nangona kunjalo, uphando lubonise ukuba umonakalo kwiiseli ezingaphakathi kwemithambo yegazi yethu ubangela ukuba iiseli zezicubu ezinxibelelanayo eluswini, ezibizwa ngokuba yi -fibroblasts, zisebenze kakhulu. Ezi fibroblast ziiseli ezivelisa iiproteni ezifana ne-collagen. Ngoko ke, iimpawu ze-Systemic Scleroderma zibangelwa kukuveliswa kakhulu kwe-collagen.

Kutheni ulusu lomntwana luqinile kangaka?

KwiSystemic Scleroderma, ulusu luqina ngenxa yeproteni egqithisileyo ebizwa ngokuba yi-collagen kwizicubu zomntwana. Njengoko besitshilo ngaphambili, umsebenzi wenkqubo yokuzikhusela kukukhusela umzimba kwizifo nezifo. Nangona kunjalo, kumntwana oneScleroderma, inkqubo yokuzikhusela isabela ngokugqithisileyo ize izame ukukhusela umzimba ngeeseli zayo eziphilileyo - oko kukuthi, ihlasela iiseli zayo . Ngenxa yoko, iiseli zomntwana ziqala ukuvelisa i-collagen eninzi kakhulu. Le collagen igqithisileyo ifakwa eluswini lomntwana nakwizitho zangaphakathi, idala inkangeleko eqinileyo, efana nesilonda.

Ifunyanwa njani iSystemic Scleroderma?

Ugqirha womntwana wakho uza kuxilonga iSystemic Scleroderma ngokuqwalasela izinto ezahlukeneyo.

• Iimpawu kunye neempawu zomzimba, ezinje ngokukhula kolusu kunye notshintsho kwiminwe.
• Utshintsho oluthile eluswini, njengeendawo ezijiyileyo, ubungakanani bazo, imilo, kunye nombala wazo.
• Imbali epheleleyo yezonyango kunye novavanyo lomzimba luya kubonelela ngolwazi malunga nezifo zomntwana zangaphambili kunye nokuba kukho nabani na kusapho lwakhe okhe waba neemeko ezifanayo.

Ziziphi iimvavanyo ezisetyenziselwa ukufumanisa iSystemic Scleroderma?

Kwenziwa uvavanyo olwahlukeneyo ukuze kuthintelwe ezinye iimeko ezifana ne-scleroderma, ukufumanisa ukuba i-scleroderma isebenza kangakanani, nokufumanisa ukuba ezinye izitho ngaphandle kolusu ziyachaphazeleka na. Uvavanyo lweeproteni zokuzikhusela komzimba lunokunika umbono othile ngendlela esi sifo esiqhubeka ngayo. Amaxesha amaninzi, oogqirha benza i-skin biopsy .

Ezinye iimvavanyo ezongezelelweyo zeSystemic Scleroderma zezi:

• Uvavanyo lwegazi: Linganisa amanqanaba eeprotheyini ezikhusela umzimba kunye nokusebenza kwezintso.
• Uvavanyo lwe-X-ray: Khangela utshintsho eluswini lomntwana, emathanjeni, nakwizitho zangaphakathi (ezifana nemiphunga namathumbu).
• Uvavanyo lokujonga inkqubo yokuginya kwi-esophagus: Oku kuthetha ukujonga umsebenzi wetyhubhu ethwala ukutya ukusuka emlonyeni ukuya esiswini.
• Uvavanyo lokusebenza kweMiphunga: Uvavanyo lokuphefumla ukuze kubonwe ukuba imiphunga yomntwana isebenza kakuhle na.
• I-ultrasound yentliziyo (i-Echocardiography): Jonga indlela esebenza ngayo intliziyo yomntwana wakho.

Ngubani oxilonga i-Juvenile Systemic Scleroderma?

I-Systemic Scleroderma ixilongwa, ijongwe, kwaye inyangwa ngugqirha womntwana wakho, ingcali yamathambo egxile kwezi meko, ingcali yesikhumba , okanye iqela loogqirha abagxile kwiindawo ezithile zomzimba (inkqubo yokugaya ukutya, intliziyo, imiphunga, izintso).

Inyangwa njani iJuvenile Systemic Scleroderma?

Iinjongo eziphambili zonyango lwe-systemic scleroderma kukuyeka ukudumba, ukuthintela imeko ukuba ingabi mandundu, kunye nokuthintela umonakalo kwizitho zangaphakathi.

Ukukhusela ulusu

Ukukhusela ulusu lwakho kunceda kakhulu ekuphuhliseni ukuhamba kwegazi eluswini nasemalungeni akho. Oku kubaluleke kakhulu kubantwana abanesi sifo sikaRaynaud . Nazi ezinye izinto onokuzenza ukukhusela ulusu lomntwana wakho:

• Kuphephe ukwenzakalisa iindawo ezichaphazelekileyo, ingakumbi iincam zeminwe kunye neenzwane.Nokuba kukukrazuka okuncinci kunokuba yingxaki enkulu.
• Khusela izandla neenyawo zomntwana wakho ekubandeni. Gcina igumbi lifudumele. Ebusika (kwiindawo ezibandayo ezifana neentaba zelizwe lethu), nxibisa umntwana wakho umaleko owongezelelweyo wempahla, njengomnqwazi onezigqubuthelo zeendlebe, iiglavu, kunye neekawusi ezifudumeleyo. Uboya bufudumele kunekotoni okanye amalaphu enziweyo. Kwakhona, kungcono ukunxiba amaleko amaninzi amancinci empahla kunomaleko omnye otyebileyo.
• Kuphephe ukutshaya okanye ukumbangela ukuba umntwana wakho atshaye.
• Kuphephe ukunika umntwana wakho amayeza omkhuhlane aqulethe isithako se-pseudoephedrine.
• Khusela umntwana wakho elangeni eligqithisileyo. Sebenzisa izithambisi zelanga njengoko ugqirha wakho ekucebisile.
• Musa ukusebenzisa izinto ezithintela ulusu, izinto zokuhlikihla umzimba okanye ubuso, okanye izicoci eziqinileyo eluswini lomntwana wakho ezomisa ulusu.
• Sebenzisa iiloshini ukugcina ulusu lomntwana wakho luthambile njengoko ugqirha emyalele.

Ulungiso lwenyama

Iinkqubo zokuzilolonga ezisisiseko kunye neenkqubo zokuzilolonga ezikhokelwayo, ezenziwa ziingcali zonyango lomzimba kunye neengcali zonyango lomsebenzi, zinokunceda ukugcina ukuguquguquka komntwana, uluhlu lokushukuma kwamalungu, amandla emisipha, kunye nokuhamba kwegazi kwiindawo ezichaphazelekayo. Olu nyango lunokunceda ekuthinteleni ukuqina kwamalungu, okubangela ukuqina kwamalungu. Ukuba kuyimfuneko, ugqirha unokucebisa ii-splint okanye ii-braces.

Utyando

Kunqabile kakhulu ukuba utyando lwezandla lwamathambo okanye utyando lobuhle lunokufuneka ukuze kulungiswe ukukhubazeka okanye amanxeba anzulu amalunga okanye ulusu. Nangona kunjalo, ngaphambi kotyando, imeko kufuneka iphumle , oko kuthetha ukuba ibingasebenzi kangangeminyaka eliqela.

Ugqirha womntwana wakho usenokucinga ngokufakelwa umthambo wethambo le-autologous njengendlela yonyango. Kukho ezinye iindlela zonyango ezisaphandwayo okwangoku.

Ngawaphi amayeza anikwa iSystemic Scleroderma?

Ugqirha wakho angakunika amayeza ahlukeneyo okunyanga imeko yomntwana wakho. Umzekelo:

• IiCorticosteroids: Zinciphisa ukudumba kwimisipha, amalungu, kunye nolusu. Iisteroids zinokunceda ekunyangeni amanqanaba okuqala okudumba kwizitho zangaphakathi. Nangona kunjalo, iisteroids zihlala zingasebenzi kakuhle kumanqanaba okugqibela e-fibrotic e-systemic sclerosis.
• Amayeza angengowe-steroidal alwa nokuvuvukala (ii-NSAID): Umzekelo , i-ibuprofen kunye ne -naproxenAmayeza afana ne-NSAID ngamanye amaxesha asetyenziselwa ukunciphisa ukudumba kwamalungu kubantwana abane-arthritis. Nangona kunjalo, kufuneka kuthathwe unonophelo ukuba ungasebenzisi kakhulu ii-NSAID xa ukusebenza kwezintso kuphazamiseka.
• Amanye amayeza okulawula amasosha omzimba: La anikwa ukunciphisa impendulo yokuzikhusela komzimba kunye nokuthintela ukudumba kunye nokulimala okulandelayo.
• Amayeza andisa imithambo yegazi aze aphucule ukuhamba kwegazi: La mayeza asetyenziselwa ukunyanga isigulo sikaRaynaud .

Ukuba umntwana wam uneSystemic Scleroderma, ndimele ndilindele ntoni?

Akukho ndlela ithile yonyango lwe-scleroderma. Nangona kunjalo, ungamnceda umntwana wakho ukuba alawule esi sifo aze aphile naso.

I-Systemic Scleroderma sisifo esinganyangekiyo , esihlala ixesha elide, nesiqhubeka kancinci kancinci. Sinokuhlala iinyanga okanye iminyaka. Imbono yomntwana wakho ixhomekeke ekubeni iimpawu zisasazeke kangakanani kwaye umzimba wonke (ingakumbi ulusu kunye nezitho zangaphakathi) zichaphazeleka kangakanani.

Abantwana abaneengxaki zemiphunga, zentliziyo, okanye zezintso basengozini enkulu yokufumana iingxaki, ezifana nokuqunjelwa kwamalungu kunye notshintsho kubuhle. Le meko inokuchaphazela nokukhula kwamathambo omntwana.

I-Systemic Scleroderma ayipheli ngokupheleleyo, kodwa inokuhlala ikwinqanaba elifanayo iminyaka eliqela ngaphandle kokuba mandundu.

Ungaphila ixesha elingakanani neJuvenile Systemic Scleroderma?

Ixesha lokuphila komntwana ofunyaniswe eneSystemic Scleroderma liyahluka ngokuxhomekeke kubukhali bemeko, ingakumbi nokuba ichaphazele amalungu akhe angaphakathi. Kubalulekile ukuthetha nogqirha wakho ngale nto ukuze ufumane ukuqonda okucacileyo.

Ngaba i-Juvenile Systemic Scleroderma ingathintelwa?

Ekubeni unobangela ochanekileyo ungaziwa, akukho ndlela ithile yokuthintela iSystemic Scleroderma.

Ndingamnyamekela njani umntwana wam oye wafunyaniswa ukuba uneSystemic Scleroderma?

Abantwana abaneSystemic Scleroderma kufuneka baphile ubomi obuqhelekileyo kangangoko banako. Bafanele baye esikolweni, badlale, kwaye bathathe inxaxheba kwimisebenzi. Ngokubanzi, akukho mida kubungakanani bomsebenzi womzimba abantwana abanokuwenza (lo gama nje ukhuselekile). Ukuzilolonga kunceda ukuthintela ubuthathaka kwaye kwandisa amandla emisipha, ukuguquguquka, kunye nokunyamezela.

Ndingamsa nini umntwana wam kugqirha?

Umntwana wakho kufuneka asiwe rhoqo ukuze ahlolwe. Ingakumbi qaphela iimpawu ezichaphazela amalungu angaphakathi. Ukuba umntwana wakho unentlungu enzulu , unengxaki yokushukuma amalungu omzimba wakhe, okanye uneempawu ezichaphazela ubomi bakhe bemihla ngemihla,Bona ugqirha ngokukhawuleza.

Ndingayibuza yiphi imibuzo ugqirha wam?

Ukubuza imibuzo efana nale kuya kukunceda:

• Ingxaki yomntwana wam inzulu kangakanani?
• Ndinganceda njani ukukhusela ulusu lomntwana wam ekhaya?
• Umntwana wam ufanele aye kangaphi kwi-physiotherapy?
• Zeziphi iimpawu ekufuneka ndizijonge ukuba isifo siya sibi?
• Ngaba umntwana wam udinga utyando?

Ngaba kukho ezinye iintlobo ze-scleroderma?

Ukongeza kwiintlobo zeSystemic Scleroderma, kukho iintlobo ezimbini eziphambili zeScleroderma. Nazi:

• I-Localized scleroderma: Olu hlobo luchaphazela ulusu kwiindawo ezithile zomzimba kuphela. Lunokusasazeka nakwimisipha okanye amathambo angaphantsi, okanye lusenokungabikho. Aluchaphazeleki rhoqo amalungu angaphakathi.
• I-Systemic scleroderma (ekwaziwa ngokuba yi-systemic sclerosis): Yile nto besithetha ngayo kulo lonke eli nqaku. Kule meko, ulusu lunokuqina naphi na emzimbeni. Ukongeza kutshintsho lolusu, izicubu ezibomvu zinokukhula kwizitho zangaphakathi ezifana nezintso, intliziyo, imiphunga, kunye nenkqubo yesisu. Abantwana abane-systemic sclerosis banokuba nentshukumo encinci yamalungu ngenxa yotshintsho lolusu olusasazekayo.

Ezona zinto zibalulekileyo ekufuneka uzikhumbule kweli nqaku

I-Juvenile Systemic Scleroderma yimeko engaqhelekanga ebantwaneni ebangela ukuba ulusu lube lukhuni kwaye luqine, ngamanye amaxesha luchaphazela amalungu angaphakathi. Sisifo esibangelwa kukuzikhusela komzimba.

• Iimpawu: Ukuqina kolusu, ukudumba kweminwe, isigulo sikaRaynaud (iminwe itshintsha umbala xa kubanda), iintlungu zamalungu, iingxaki zokugaya ukutya, kunye nobunzima bokuphefumla bunokubakho.
• Unobangela: Nangona kungaziwa kakuhle, kucingelwa ukuba kubangelwa kukungasebenzi kakuhle kwenkqubo yomzimba yokuzikhusela kunye nokuveliswa kakhulu kwe-collagen .
• Unyango: Lawula isifo, khusela ulusu, sebenzisa unyango lomzimba, kwaye ukuba kuyimfuneko, sebenzisa amayeza. Nangona kungekho nyango lupheleleyo, iimpawu zinokulawulwa.
• Okubaluleke kakhulu: Ukuba umntwana wakho uneempawu, funa ingcebiso kagqirha ngoko nangoko. Kubaluleke kakhulu ukumthumela umntwana wakho ukuba aye kuhlolwa rhoqo ngugqirha kwaye umncede aphile ubomi obuqhelekileyo. Ungakhathazeki, ungamelana nale meko ngoncedo loogqirha.

I- Scleroderma, izifo zesikhumba zabantwana, ukuqina kwesikhumba, i-collagen, izifo zokuzikhusela komzimba, isiganeko sikaRaynaud, isifo sikaCREST