Ingabe isikhumba sengane yakho siyaqina futhi siyaqina? Ake sixoxe nge-Juvenile Systemic Scleroderma!

Uke waqaphela ukuthi isikhumba somntwana wakho sishintshe kancane ngokuzumayo? Mhlawumbe isikhumba sesibe siqinile, siqinile, futhi sizwakala sihlukile uma usithinta? Uma ubona into efana naleyo, kufanele ukhathazeke kancane. Ngoba, lokhu ngezinye izikhathi kungaba uphawu lwesifo esibizwa ngokuthi i-Juvenile Systemic Scleroderma , esikhuluma ngaso namuhla. Ungakhathazeki, sizoxoxa ngalokhu ngokuningiliziwe.

Kuyini i-Juvenile Systemic Scleroderma?

Kalula nje, i-Juvenile Systemic Scleroderma (eyaziwa nangokuthi i-Systemic Sclerosis ) yisimo lapho isikhumba sengane yakho siba sikhulu futhi siqine kakhulu kunokujwayelekile. Cabanga ngaso njengeshidi lerabha elikhula kancane kancane libe njengepulasitiki eqinile. Akugcini nje esikhumbeni. Ngezinye izikhathi, lingathinta nezinye izitho zomzimba wengane. Igama elithi 'systemic' lisho ukuthi lingathinta wonke umzimba wengane.

Empeleni lesi yisifo sokuzivikela komzimba . Okusho ukuthi, uhlelo lwethu lokuzivikela lomzimba, uhlelo lokuzivikela komzimba, luqala ngephutha ukuhlasela amangqamuzana alo aphilile. Kulesi simo se-Scleroderma, lapho uhlelo lokuzivikela komzimba luhlasela amangqamuzana esikhumba ngale ndlela, kuvela ukuvuvukala noma ukuvuvukala kwesikhumba. Ngenxa yalokhu kuvuvukala, iphrotheni ebizwa ngokuthi i-collagen, enikeza isikhumba sethu ukuqina nokuqina, iqala ukukhiqizwa ngokweqile. Njengoba nje kwakheka isibazi lapho kwenzeka ukulimala, lokhu kukhiqizwa kwe-collagen ngokweqile kubangela i-fibrosis noma ukuqina kwesikhumba okufana nesibazi.

Ingabe zikhona izinhlobo eziyinhloko ze-Systemic Scleroderma?

Yebo, kunezinhlobo ezintathu eziyinhloko. Ake sibone ukuthi ziyini.

1. I-Diffuse cutaneous systemic sclerosis: Kulolu hlobo, ukuqina kwesikhumba sengane kungabonakala kuwo wonke umzimba. Futhi, kungase kube nezinkinga ngokusebenza kwezitho zangaphakathi zomzimba. Cabanga nje, hhayi izingalo, imilenze, nobuso kuphela, kodwa nezindawo ezifana nesifuba nesisu ziqala ukuqina.

2. I-Limited cutaneous systemic sclerosis (CREST syndrome): Lesi sikhethekile kancane. Sibizwa nangokuthi i-CREST syndrome . I-CREST isifinyezo esakhiwe ngokuhlanganisa izinhlamvu zokuqala zezici ezinhlanu eziyinhloko zalesi simo.

• C - I-Calcinosis: Lokhu kusho ukuthi i-calcium igcina ngaphansi kwesikhumba bese yakha amaqhubu amancane.
• R - Isenzakalo sikaRaynaud: Lokhu kwenzeka lapho iminwe (izandla nezinyawo) iphenduka ibe mhlophe, ibe luhlaza okwesibhakabhaka, bese kuba bomvu uma ibekwe emakhazeni noma uma ingaphansi kokucindezeleka. Kuba nzima ukulawula izinga lokushisa lomzimba.
• Ukungasebenzi kahle kokuhamba kwesisu: Lokhu kusho ukuthi ipayipi elithwala ukudla kusuka emphinjeni liye esiswini (umhubhe) alisebenzi kahle uma sigwinya. Lokhu kungabangela isilungulela.Izinto ezinjalo zingenzeka.
• S - I-Sclerodactyly: Ukuqina nokuqina kwesikhumba eminweni. Iminwe ingavuvukala futhi iqine, njengesoseji.
• I-T - I-Telangiectasia: Imithambo yegazi emincane esikhumbeni iyakhula, ivele njengezintambo zesicabucabu noma amachashazi abomvu.

3. I-systemic sclerosis sine scleroderma: Lokhu kwenzeka lapho izitho zangaphakathi zomzimba zithinteka ngaphandle kokuqina noma ukuqina kwesikhumba. I-'Sine scleroderma' isho ukuthi 'ngaphandle kwe-scleroderma (ukuqina kwesikhumba)'. Lokhu kusho ukuthi nakuba kungase kungabi khona izinguquko ezibalulekile esikhumbeni, izitho zangaphakathi zingathinteka.

Ubani ongase abe nesifo se-Juvenile Systemic Scleroderma? Sivame kangakanani?

Lesi simo esibizwa ngokuthi i-Juvenile Systemic Scleroderma singathinta noma ubani wanoma yimuphi ubudala, kungakhathaliseki ubulili. Kodwa-ke, akuvamile kakhulu ukuthi izingane ezincane, okungukuthi, izingane ezingaphansi kweminyaka eyi-10, zisithole. Igama elithi 'intsha' ngokwalo lisho ukuthi lesi yisimo esithinta izingane. Uma ubheka izibalo ezweni elifana ne-United States, kuthiwa kunezingane eziphakathi kuka-5,000 no-7,000 ezine-Scleroderma. Cishe u-2% wabantu bonke abane-Scleroderma bathintwa yilesi sifo ngaphambi kweminyaka eyi-10. Abanye abangu-7% babhekana nalesi simo phakathi kweminyaka eyi-10 no-19. Ngakho-ke, lesi sithathwa njengesifo esingavamile kakhulu .

Ziyini izimpawu ze-Juvenile Systemic Scleroderma?

Kulesi simo, izimpawu zingathinta isikhumba sengane kanye nezitho zangaphakathi. Ngaphezu kwalokho, izingane eziningi ezine-Systemic Scleroderma nazo zibhekana nesimo esibizwa ngokuthi i-Raynaud's phenomenon .

Izimpawu ezithinta isikhumba nezicubu

Okokuqala, ake sibheke ukuthi lokhu kuthinta kanjani isikhumba kanye nezicubu ezihlobene:

• Isikhumba silahlekelwa ukunwebeka kwaso. Siba lukhuni, siqine, futhi singabukeki kahle.
• Umsebenzi wezandla uyancipha. Isikhumba siyaqina, ikakhulukazi eminweni nasezintendeni zezandla, okwenza kube nzima ukugoba izandla nokubamba okuthile.
• Imithambo yegazi ebomvu ivela esikhumbeni, ikakhulukazi ezandleni, ebusweni, kanye nasezinziphoni (telangiectasias). Lokhu kungabonakala njengezintambo ezincane zesicabucabu.
• I-Calcinosis ukwakheka kwamaqhubu amancane e-calcium deposits ngaphansi kwesikhumba noma kwenye indawo. Lokhu ngezinye izikhathi kungazwakala kunzima.

Ekuqaleni, isikhumba ezandleni nasezinyaweni singase sibonakale sivuvukele futhi sikhulile. Ngokuhamba kwesikhathi, isikhumba sengane singase siqine, sibe sikhulu, futhi singase sibe nemibimbi, izindawo ezicwile, noma imigodi emincane. Isibonelo, ezinye iminwe yezingane ingavuvukala njengesoseji, bese iba lukhuni futhi icwebezele.

Izimpawu ezithinta izitho zangaphakathi

I-Systemic Scleroderma ingathinta nezitho zangaphakathi zengane. Izimpawu zingase zihlanganise:

• Ukuvuvukala kwamalunga, ukuqina, kanye nobuhlungu. Njenge-arthritis.
• Izilonda ezinyaweni. Lezi zilonda zithatha isikhathi eside ukuphola.
• Izinkinga zesistimu yokugaya ukudla. Isibonelo, isilungulela, ubunzima bokugwinya, indle ekhululekile (uhudo), kanye nobuhlungu besisu.
• Izinkinga zokuphefumula. Ungase ube nokukhwehlela okuqhubekayo noma ubunzima bokuphefumula.
• Izinkinga zezinso. Lokhu kungabangela nomfutho wegazi ophakeme (umfutho wegazi ophakeme) .
• Ukuzizwa ukhathele ngaso sonke isikhathi. Ukukhathala ngokushesha ngisho nangemva kokwenza into encane.
• Ubuthakathaka bemisipha.

Esikhathini esiningi, lesi simo asibuhlungu. Ngakho-ke, ezinye izingane zingase zingazi nokuthi zine-Scleroderma. Yingakho ingane etholakale ine-Systemic Sclerosis kufanele ibone udokotela njalo ukuze ihlole umfutho wegazi ophakeme, amaphaphu, izinso, izinkinga zesisu kanye nenhliziyo.

Isenzakalo sikaRaynaud

Lesi yisibonakaliso esiyingqayizivele. Isenzakalo sikaRaynaud yilapho iminwe nezinzwane zengane zishintsha umbala lapho zibekwe emakhazeni noma lapho zicindezelekile. Okokuqala ziba mhlophe , bese kuba luhlaza okwesibhakabhaka , bese ekugcineni zibomvu . Lesi yisibonakaliso esingabonakala ezigabeni zokuqala zesifo. Ngezinye izikhathi lokhu kungaba yinkomba yokuqala yokuthi kukhona i-Systemic Sclerosis.

Ezinye izimpawu ezingase zenzeke nge-Raynaud's phenomenon zifaka:

• Ukuzizwa ukhathele ngaso sonke isikhathi
• Ubuhlungu bamalunga
• Ubunzima bokugwinya ukudla
• Isisu esibuhlungu
• Ukuvuvukala kwesifuba
• Uhudo
• Ubunzima bokuphefumula

Kungani kwenzeka i-Juvenile Systemic Scleroderma?

Empeleni, imbangela eqondile ye-Juvenile Systemic Scleroderma ayikakaziwa . Kodwa-ke, ucwaningo lukhombisile ukuthi umonakalo kumaseli angaphakathi kwemithambo yegazi yethu ubangela ukuthi amaseli ezicubu ezixhumene esikhumbeni, abizwa ngokuthi ama-fibroblast, asebenze ngokweqile. Lawa ma-fibroblast amaseli akhiqiza amaprotheni afana ne-collagen. Ngakho-ke, izimpawu ze-Systemic Scleroderma zibangelwa yilokhu kukhiqizwa ngokweqile kwe-collagen.

Kungani isikhumba somntwana siqinile kangaka?

Ku-Systemic Scleroderma, isikhumba siba siqinile ngenxa yokwanda kweprotheni ebizwa ngokuthi i-collagen ezicutshini zengane. Njengoba sesixoxile ngaphambili, umsebenzi wesimiso sokuzivikela komzimba ukuvikela umzimba ekuthelelekeni nasezifweni. Kodwa-ke, enganeni ene-Scleroderma, isimiso sokuzivikela somzimba siyasabela ngokweqile futhi sizame ukuvikela umzimba ngamaseli ayo anempilo - okungukuthi, sihlasela amaseli aso . Ngenxa yalokho, amaseli engane aqala ukukhiqiza i-collagen eningi kakhulu. Le collagen eningi ifakwa esikhumbeni sengane nasezithweni zangaphakathi, idale ukubukeka okuqinile nokujiyile, okufana nesilonda.

I-Systemic Scleroderma ihlolwa kanjani?

Udokotela wengane yakho uzoxilonga i-Systemic Scleroderma ngokucabangela izici eziningana.

• Izimpawu nezimpawu zomzimba, njengokuqina kwesikhumba kanye noshintsho eminweni.
• Izinguquko ezithile esikhumbeni, njengezindawo ezijiyile, usayizi wazo, ukuma kwazo, nombala wazo.
• Umlando ophelele wezokwelapha kanye nokuhlolwa ngokomzimba kuzohlinzeka ngolwazi mayelana nezifo zengane zangaphambilini nokuthi ngabe kukhona yini emndenini oke waba nezimo ezifanayo.

Yiziphi izivivinyo ezisetshenziswa ukuxilonga i-Systemic Scleroderma?

Kwenziwa izivivinyo ezahlukahlukene ukuze kunqunywe ezinye izimo ezifana ne-scleroderma, ukuthola ukuthi i-scleroderma isebenza kangakanani, kanye nokunquma ukuthi ezinye izitho ngaphandle kwesikhumba ziyathinteka yini. Ukuhlolwa kwamaprotheni okuzivikela komzimba kunganikeza umbono othile wendlela lesi sifo esiqhubeka ngayo. Akuvamile kakhulu, odokotela benza i-biopsy yesikhumba .

Ezinye izivivinyo ezengeziwe ze-Systemic Scleroderma yilezi:

• Ukuhlolwa kwegazi: Kala amazinga amaprotheni okuzivikela komzimba kanye nokusebenza kwezinso.
• Ukuhlolwa kwe-X-ray: Bheka izinguquko esikhumbeni sengane, emathanjeni, nasezithweni zangaphakathi (njengamaphaphu namathumbu).
• Ukuhlolwa kokuhlola inqubo yokugwinya emhubheni: Lokhu kusho ukubheka umsebenzi wepayipi elithwala ukudla kusuka emlonyeni kuya esiswini.
• Ukuhlolwa kokusebenza kwamaphaphu: Ukuhlolwa kokuphefumula ukuze kubonakale ukuthi amaphaphu engane asebenza kahle yini.
• I-ultrasound yenhliziyo (i-Echocardiography): Bona ukuthi inhliziyo yengane yakho isebenza kanjani.

Ubani oxilonga i-Juvenile Systemic Scleroderma?

I-Systemic Scleroderma ihlolwa, ihlolwe, futhi yelashwe udokotela wengane yakho, udokotela wamathambo ochwepheshe kulezi zimo, udokotela wesikhumba , noma iqembu lodokotela abangochwepheshe ezingxenyeni ezithile zomzimba (uhlelo lokugaya ukudla, inhliziyo, amaphaphu, nezinso).

Iphathwa kanjani i-Juvenile Systemic Scleroderma?

Imigomo eyinhloko yokwelashwa kwe-scleroderma ehlelekile ukuvimbela ukuvuvukala, ukuvimbela lesi simo ukuba singabi sibi, nokuvimbela ukulimala kwezitho zangaphakathi.

Ukuvikela isikhumba

Ukuvikela isikhumba sakho kusiza ukwandisa ukugeleza kwegazi esikhumbeni sakho nasemalungeni. Lokhu kubaluleke kakhulu ezinganeni ezinesifo sikaRaynaud . Nazi ezinye izinto ongazenza ukuze uvikele isikhumba sengane yakho:

• Gwema ukulimaza izindawo ezithintekile, ikakhulukazi izinzipho zeminwe nezinzwane.Ngisho nokudabuka okuncane kungaba yinkinga enkulu.
• Vikela izandla nezinyawo zengane yakho emakhazeni. Gcina igumbi lifudumele. Ebusika (ezindaweni ezibandayo njengeziqhingi zezwe lethu), gqokisa ingane yakho ungqimba olwengeziwe lwezingubo, njengesigqoko esinezindwangu zokumboza ama-earflaps, ama-mittens, namasokisi afudumele. Uboya bufudumele kunokotini noma izindwangu zokwenziwa. Futhi, kungcono ukugqoka izendlalelo eziningana ezincane zezingubo kunongqimba olulodwa olujiyile.
• Gwema ukubhema noma ukuveza ingane yakho ukuthi ibheme.
• Gwema ukunikeza ingane yakho imithi yomkhuhlane equkethe isithako se-pseudoephedrine.
• Vikela ingane yakho elangeni eliningi. Sebenzisa ama-sunscreen njengoba kunconywe udokotela wakho.
• Ungasebenzisi izinto eziqeda ukuvuvukala, izikhuhla zomzimba noma zobuso, noma izihlanzi eziqinile esikhumbeni sengane yakho ezomisa isikhumba.
• Sebenzisa ama-lotion ukuze ugcine isikhumba sengane yakho sibushelelezi njengoba udokotela ekuyalele.

Ukwelashwa ngokomzimba

Izinhlelo zokwelula eziyisisekelo kanye nokuzivocavoca okuqondiswayo, ezenziwa abelaphi bomzimba kanye nabelaphi bomsebenzi, zingasiza ukugcina ukuguquguquka kwengane, ububanzi bokunyakaza emalungeni, amandla emisipha, kanye nokugeleza kwegazi ezindaweni ezithintekile. Lokhu kwelashwa kungasiza futhi ekuvimbeleni ukugoba kwamalunga, okuqinisa amalunga. Uma kudingeka, udokotela angase atuse ama-splint noma ama-braces.

Ukuhlinzwa

Akuvamile kakhulu ukuthi ukuhlinzwa kwezandla kwamathambo noma ukuhlinzwa kobuhle kungadingeka ukuze kulungiswe ukukhubazeka okukhulu kwamalunga noma kwesikhumba noma izibazi. Kodwa-ke, ngaphambi kokuhlinzwa, lesi simo kumele siphumule , okusho ukuthi sesisebenze iminyaka eminingana.

Udokotela wengane yakho angase acabangele ukufakelwa umnkantsha wethambo we-autologous njengendlela yokwelapha. Ezinye izindlela zokwelapha ziyahlolwa okwamanje.

Yimiphi imithi enikezwayo ye-Systemic Scleroderma?

Udokotela wakho angase akunike imithi ehlukahlukene yokwelapha isimo sengane yakho. Isibonelo:

• Ama-Corticosteroids: Yehlisa ukuvuvukala emisipheni, emalungeni nasesikhumbeni. Ama-steroid angasiza futhi ekwelapheni izigaba zokuqala zokuvuvukala ezithweni zangaphakathi. Kodwa-ke, ama-steroid ngokuvamile awasebenzi kahle ezigabeni zakamuva ze-fibrotic ze-systemic sclerosis.
• Imithi engeyona i-steroidal elwa nokuvuvukala (ama-NSAID): Isibonelo , i-ibuprofen ne -naproxenImithi efana nama-NSAID ngezinye izikhathi isetshenziselwa ukunciphisa ukuvuvukala kwamalunga ezinganeni ezine-arthritis. Kodwa-ke, kufanele kuqashelwe ukuthi ungasebenzisi ama-NSAID ngokweqile uma ukusebenza kwezinso kuphazamiseka.
• Eminye imithi yokulawula amasosha omzimba: Le mithi inikezwa ukunciphisa impendulo yokuzivikela komzimba nokuvimbela ukuvuvukala kanye nezibazi ezilandelayo.
• Imithi ekhulisa imithambo yegazi futhi ithuthukise ukugeleza kwegazi: Le mithi isetshenziselwa ukwelapha isifo sikaRaynaud .

Uma ingane yami ine-Systemic Scleroderma, yini okufanele ngiyilindele?

Akukho ukwelashwa okuqondile kwe-scleroderma. Kodwa-ke, ungasiza ingane yakho ukuthi ilawule lesi simo futhi iphile naso.

I-Systemic Scleroderma iyisifo esingamahlalakhona , esihlala isikhathi eside, futhi esiqhubeka kancane kancane. Singathatha izinyanga noma iminyaka. Umbono wengane yakho uncike ekutheni izimpawu zisakazeke kangakanani nokuthi kuthinteka kangakanani umzimba (ikakhulukazi isikhumba nezitho zangaphakathi).

Izingane ezinezinkinga zamaphaphu, zenhliziyo, noma zezinso zisengozini enkulu yezinkinga, njengokuqina kwamalunga kanye noshintsho lobuhle. Lesi simo singathinta nokukhula kwamathambo engane.

I-Systemic Scleroderma ngokuvamile ayipheli ngokuphelele, kodwa ingahlala ezingeni elifanayo iminyaka eminingana ngaphandle kokuba ibe yimbi kakhulu.

Ungaphila isikhathi esingakanani ne-Juvenile Systemic Scleroderma?

Isikhathi sokuphila kwengane etholakale ine-Systemic Scleroderma siyahlukahluka kuye ngokuthi lesi simo sibucayi kangakanani, ikakhulukazi ukuthi ngabe sithinte izitho zayo zangaphakathi. Kubalulekile ukukhuluma nodokotela wakho ngalokhu ukuze uthole ukuqonda okucacile.

Ingabe i-Juvenile Systemic Scleroderma ingavinjelwa?

Njengoba imbangela eqondile ingaziwa, ayikho indlela eqondile yokuvimbela i-Systemic Scleroderma.

Ngingayinakekela kanjani ingane yami etholakale ine-Systemic Scleroderma?

Izingane ezine-Systemic Scleroderma kufanele ziphile impilo evamile ngangokunokwenzeka. Kufanele ziye esikoleni, zidlale, futhi zihlanganyele emisebenzini. Ngokuvamile, akukho mingcele enanini lomsebenzi womzimba izingane ezingawenza (uma nje kuphephile). Ukuzivocavoca kusiza ukuvimbela ubuthakathaka futhi kwandisa amandla emisipha, ukuguquguquka, kanye nokukhuthazela.

Kufanele ngiyise nini ingane yami kudokotela?

Ingane yakho kufanele iyiswe njalo ukuze iyohlolwa. Qaphela kakhulu izimpawu ezithinta izitho zangaphakathi. Uma ingane yakho isezinhlungwini ezinzima , inenkinga yokunyakaza izingxenye zomzimba wayo, noma inezimpawu ezithinta impilo yayo yansuku zonke,Bonana nodokotela ngokushesha.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Ukubuza imibuzo efana nale kuzokusiza:

• Ingakanani inkinga yengane yami?
• Ngingasiza kanjani ekuvikeleni isikhumba sengane yami ekhaya?
• Ingane yami kufanele iye kangaki ekwelashweni ngokomzimba?
• Yiziphi izimpawu okufanele ngiziqaphele uma lesi sifo siba sibi kakhulu?
• Ingabe ingane yami idinga ukuhlinzwa?

Zikhona yini ezinye izinhlobo ze-scleroderma?

Ngaphezu kwezinhlobo ze-Systemic Scleroderma, kunezinhlobo ezimbili eziyinhloko ze-Scleroderma. Lezi yizi:

• I-scleroderma etholakala endaweni ethile: Lolu hlobo luthinta isikhumba kuphela ezindaweni ezithile zomzimba. Lungase lusakazekele nasemisipheni noma emathanjeni angaphansi, noma lungase lungabi khona. Akuvamile ukuthinta izitho zangaphakathi.
• I-Systemic scleroderma (eyaziwa nangokuthi i-systemic sclerosis): Yilokhu ebesikhuluma ngakho kulo lonke lesi sihloko. Kulesi simo, isikhumba singaba lukhuni noma kuphi emzimbeni. Ngaphezu kokushintsha kwesikhumba, izicubu zesilonda zingakhula ezithweni zangaphakathi njengezinso, inhliziyo, amaphaphu, kanye nohlelo lokugaya ukudla. Izingane ezine-systemic sclerosis zingase zibe nokunyakaza okulinganiselwe kwamalunga ngenxa yokushintsha kwesikhumba okusabalele.

Izinto ezibaluleke kakhulu okudingeka uzikhumbule kulesi sihloko

I-Juvenile Systemic Scleroderma yisimo esingavamile ezinganeni esibangela ukuba isikhumba siqine futhi siqine, ngezinye izikhathi sithinte izitho zangaphakathi. Kuyisifo esibangelwa ukuzivikela komzimba.

• Izimpawu: Ukuqina kwesikhumba, ukuvuvukala kweminwe, isimanga sikaRaynaud (iminwe ishintsha umbala uma kubanda), ubuhlungu bamalunga, izinkinga zokugaya ukudla, kanye nobunzima bokuphefumula kungase kube khona.
• Imbangela: Nakuba ingaziwa kahle, kucatshangwa ukuthi ibangelwa ukungasebenzi kahle kwesimiso somzimba sokuzivikela kanye nokukhiqizwa ngokweqile kwe-collagen .
• Ukwelashwa: Lawula lesi sifo, uvikele isikhumba, sebenzisa ukwelashwa ngokomzimba, futhi uma kudingeka, imithi. Nakuba kungekho ukwelashwa okuphelele, izimpawu zingalawulwa.
• Okubaluleke kakhulu: Uma ingane yakho inezimpawu, funa iseluleko sezokwelapha ngokushesha. Kubaluleke kakhulu ukuthumela ingane yakho ukuze iyohlolwa njalo futhi uyisize iphile impilo evamile. Ungakhathazeki, ungabhekana nalesi simo ngosizo lodokotela.

I- Scleroderma, izifo zesikhumba zezingane, ukuqina kwesikhumba, i-collagen, izifo zokuzivikela komzimba, isenzakalo sikaRaynaud, i-CREST syndrome