Yintoni i-AL Amyloidosis? Makhe sithethe ngesi sifo siyingozi!

Ngaba wakha weva ngesi sifo esibizwa ngokuba yi-'AL Amyloidosis'? Mhlawumbi akunjalo, akunjalo? Oku kunqabile, oko kuthetha ukuba asisosifo esichaphazela wonke umntu. Nangona kunjalo, ukuba siyenzeka, sinokuba sibi kakhulu. Ngamafutshane, lutshintsho kuhlobo lweseli olubizwa ngokuba yi-"Plasma cells" esinalo emizimbeni yethu, kwaye ezinye zeeproteni eziveliswa zizo ziyaphuma kulawulo kwaye zihlale kwizitho ezahlukeneyo zomzimba wethu, zizonakalisa. Kufana nomatshini osebenza kakuhle kwaye ngequbuliso waqala ukwenza iindawo ezingalunganga. Makhe sithethe ngale nto ngokweenkcukacha ezithe vetshe, kuba kubaluleke kakhulu ukuqaphela oku.

Yintoni kanye kanye i-'AL Amyloidosis'?

Le `AL amyloidosis` yenye yeentlobo zezifo ezibizwa ngokuba yi`Amyloidosis`. I`Amyloidosis` sisifo esingaqhelekanga esenzeka xa kukho utshintsho, okanye 'uguquko', 'kwiiseli zeplasma' kumongo wethu wethambo. Ezi seli zeplasma ezitshintshileyo zivelisa uhlobo olungaqhelekanga lweproteni. Ezi proteni, njengeebhola zentambo ezidityanisiweyo, ziyahlangana, kwaye zihlale kwizitho zethu ezibalulekileyo kunye nezicubu.

Kwi-AL amyloidosis, uhlobo lweproteni oluphazamiseka ngolu hlobo yiproteni yetsheyini yokukhanya. Ezi proteni zetsheyini yokukhanya ziyinxalenye yee-antibodies ezisetyenziswa yimizimba yethu ukulwa nezifo. Ezi antibodies zenziwa ziiseli zeplasma endizikhankanyileyo ngaphambili.

Ngokwesiqhelo, i-AL amyloidosis ichaphazela kakhulu intliziyo kunye/okanye izintso zethu. Nangona kunjalo, ngamanye amaxesha inokuchaphazela isisu, amathumbu, imithambo-luvo kunye nolusu.

Eyona nto ingcono kukuba ukuba esi sifo siqatshelwa kwangethuba size sinyangwe ngokukhawuleza , ngamanye amaxesha sinokulawulwa njengesifo esingapheliyo. Nangona kunjalo, ukuba asinyangwa, sinokukhokelela kwiimeko ezisongela ubomi kakhulu kwanokufa. Ngoko ke oku akuyonto ifanele ithathwe lula.

Zithetha ukuthini ezi nobumba babini `AL` kweli gama?

Kukho iintlobo ezahlukeneyo ze-amyloidosis. Uhlobo ngalunye lubizwa ngegama leproteni engaqhelekanga ebangela esi sifo. Kwi-AL amyloidosis, unobumba u-A umele i-amyloidosis kwaye unobumba u-L umele iproteni ebizwa ngokuba yi-light chain ebangela esi sifo. Ezinye iintlobo ezaziwayo ze-amyloidosis yi-AA amyloidosis (ebangelwa yi-serum amyloid A protein) kunye ne-ATTR amyloidosis (ebangelwa yi-transthyretin).

I-'AL amyloidosis' ichaphazela njani umzimba wam?

Ngamafutshane, i-AL amyloidosis sisifo seeseli ze-plasma. Iiseli ze-plasma ziyinxalenye yenkqubo yethu yomzimba yokuzikhusela. Umsebenzi wazo ophambili kukwenza ii-antibodies zokulwa nosulelo. Khawuthelekelele ukuba emizimbeni yethu, kukho amawaka ee-clones zeeseli ze-plasma ezenza iintlobo ezahlukeneyo zee-antibodies ukulwa nezifo ezahlukeneyo. Iseli enye ye-plasma iyahlukana kwaye yenze iiseli ezininzi ngakumbi.

Kwizifo ezibandakanya 'iiseli zeplasma', iqela lezi 'seli zeplasma' lahlukana ngokungalawulekiyo, livelisa inani elikhulu lohlobo olufanayo 'lwee-antibodies'.

Kwi-AL amyloidosis, xa kwenziwa ii-antibodies, kwenziwa iiproteni ezimbini ezibizwa ngokuba zii-heavy chains kunye nee-light chains. Okwenzekayo apha kukuba iiseli zeplasma zenza iiproteni ezininzi kakhulu ze-light chain. Ezi zibane zidibana kwaye zihlangana. Ezi proteni zidibeneyo zibizwa ngokuba zii-amyloid fibrils. Ezi fibrils zifakwa kwizitho zethu zomzimba. Kulapho ke kuqala khona umonakalo omkhulu kuloo malungu, ngamanye amaxesha ude ube yingozi ebomini.

Ngubani onokuthi afumane esi sifo?

I-Amyloidosis sisifo esingaqhelekanga. Kuqikelelwa ukuba sichaphazela abantu abaphakathi kwe-9 ne-14 kwisigidi eMelika kunye nabantu abaphakathi kwe-5 ne-12 kwisigidi kwezinye iindawo zehlabathi. I-AL amyloidosis ixhaphake kancinci kumadoda kunakwabasetyhini. Ikwachaphazela abantu abangaphezulu kweminyaka engama-60 ubudala . Ubudala obuqhelekileyo bokuxilongwa bumalunga neminyaka engama-64.

Zithini iimpawu ze-'AL amyloidosis'?

I-'AL amyloidosis' ayinakuchaphazela kuphela amalungu omzimba wethu, ukusuka entloko ukuya ezinyaweni, kodwa nakwimilenze. Rhoqo, iimpawu zesi sifo zinokufana neempawu zezinye izifo ezingabalulekanga kangako. Kwakhona, iimpawu zibonakala kancinci kakhulu . Ngoko ke usenokungaluboni utshintsho emzimbeni wakho ngoko nangoko.

Iimpawu ezinxulumene nentloko nentamo:

• Ngaba uziva unesiyezi okanye unentloko ekhaphukhaphu (`intloko ekhaphukhaphu`) xa uvuka?
• Ngaba unerhashalala emfusa ejikeleze amehlo okanye kwiinkophe zakho?
• Ngaba ulwimi lwakho luvakala lukhulu kunesiqhelo ?

Iimpawu ezinxulumene nezandla neenyawo:

Izandla zinokuba nezi mpawu:

• Ngaba uziva urhawuzelelwa, ukutshisa, okanye ukurhawuzelelwa ezandleni zakho? Ezi zinokuba ziimpawu zesifo esibizwa ngokuba yi-"Peripheral Neuropathy".
• Ngaba uziva urhawuzelelwa kwaye ungenazinzwane kwiminwe yakho? Ezi zisenokuba ziimpawu ze-"Carpal Tunnel Syndrome".

Iimpawu emilenzeni nasezinyaweni zingabandakanya:

• Ingaba imilenze okanye iinyawo zakho zidumbile ?
• Ngaba imilenze yakho ayibonakali ngathi iphilile ?

Ukongeza, ungaqaphela ukuba ugruzuka lula , wopha lula, okanye unombala omfusa wesikhumba apho kukho imibimbi.

Iimpawu ezibonisa iingxaki zentliziyo nemiphunga:

Iimpawu ezingezantsi zingaba ziimpawu zezinye izifo, kodwa lumka:

• Ukubetha kwentliziyo: Ukuziva ngathi intliziyo iyabetha ngamandla kwaye ibetha ngamandla.
• Ukuphefumla kancinci (ukuphelelwa ngamandla): Ukuziva uxinene esifubeni, ngokungathi awukwazi ukuphefumla nzulu.
• Intlungu yesifuba : Intlungu naphi na esifubeni. Le ntlungu inokuba bukhali, ingabi namdla, okanye idlule. Intlungu yesifuba inokuba luphawu lokuhlaselwa yintliziyo, ngoko ke ukuba unentlungu yesifuba ehlala imizuzu engaphezu kwemihlanu kwaye ingapheli ngokuphumla okanye amayeza, fowunela u-911 ngoko nangoko okanye ucele umntu akuse esibhedlele.
• Ukudinwa : Ukuziva udiniwe kangangokuba awukwazi nokwenza imisebenzi yakho yemihla ngemihla, ngokungathi umzimba wakho awunabomi.

Iimpawu ezibonisa iingxaki zesisu okanye zamathumbu:

I-AL amyloidosis inokuchaphazela nendlela otya ngayo. Usenokuba neempawu ezifana nezi:

• Ukungathandi ukutya : Umnqweno wokutya wonke umntu uyatshintsha ngamanye amaxesha. Kodwa ukuba uphelelwa kukuthanda ukutya kangangeentsuku ezininzi ngaphandle kwesizathu, oko kungaba luphawu lwengxaki yamathumbu enxulumene ne-AL amyloidosis.
• Ukudumba okanye igesi egqithisileyo : Ukudumba kunye negesi zizinto eziqhelekileyo, kodwa ngamanye amaxesha zibangela iintloni. Nangona kunjalo, ukuba unegesi engapheliyo, isenokuba luphawu lwengxaki yesisu enxulumene ne-AL amyloidosis.
• Ukuqhina : Ukuqhina kuthetha ukuba nesisu esingaphantsi kwesithathu ngeveki. Ukuba uqhina kangangeeveki ezingaphezu kwezintathu, yingxaki ekufuneka uthethe nogqirha wakho ngayo.
• Urhudo : Indle enamanzi. Ukuba urhudo luyaqhubeka, kufuneka uxelele ugqirha wakho.

Iimpawu ezibonisa iingxaki zezintso okanye zesinyi:

Usenokubona utshintsho kwindlela umchamo wakho obonakala ngayo okanye ukuba kufuneka uchame kangaphi. Ezi mpawu zingabandakanya:

• Ngaba ubona amaqamza amaninzi kumchamo wakho kunesiqhelo?
• Ingaba uchama kancinci kunesiqhelo, okanye kufuneka uvuke ebusuku ukuze uchame ?

Yintoni ebangela i-AL amyloidosis?

Sele sithethe ngale nto kancinci ngaphambili. I-'AL amyloidosis' ibangelwa 'ziiseli zeplasma' ezenza 'ii-antibodies', iiproteni ezibizwa ngokuba 'ziintambo ezinzima' kunye 'neentambo ezikhanyayo', xa ezo protein 'zentambo ezikhanyayo' zenziwe ngokugqithisileyo. Ezi 'ntambo ezikhanyayo' ziyaphambana, zihlangane, kwaye zenze 'ii-amyloid fibrils' ezithi emva koko zibekwe kwizitho zethu zomzimba. Kulapho ke iingxaki ziqala khona.

Oogqirha bayixilonga njani i-AL amyloidosis?

Oogqirha basebenzisa iimvavanyo ezininzi ukuxilonga i-AL amyloidosis. Olona vavanyo lubalulekileyo kukuthatha isampuli encinci yezicubu okanye isitho esicingelwa ukuba sichaphazelekile sesi sifo. Oku kubizwa ngokuba yi-biopsy. Ii-Biopsies zingahlulwa ngolu hlobo lulandelayo:

• I-Bone marrow biopsy : Oku kuquka ukuthatha isampuli encinci yomongo wethambo ngaphakathi kwethambo.
• I-biopsy yezintso : Kunokuthathwa iziqwenga ezimbalwa ezincinci zezicubu zezintso.
• I-biopsy yentliziyo : Kunokuthathwa iziqwenga ezimbalwa ezincinci zemisipha yentliziyo.
• I-biopsy ye-Fat pad : Kuthathwa iqhekeza elincinci lezicubu ezinamafutha esiswini. Olu luvavanyo olulula kakhulu.

Uvavanyo olongezelelweyo

Ugqirha wakho angenza olunye uvavanyo ukuze abone ukuba amalungu akho omzimba asebenza kakuhle kangakanani na. Oku kungabandakanya:

• Uvavanyo lwegazi : Olu lwenziwa ukujonga indlela ezisebenza ngayo izintso zakho, intliziyo, nesibindi, kunye nokujonga ubungakanani bemixokelelwane yokukhanya egazini lakho.
• Uvavanyo lomchamo : Olu luvavanyo oluhlala iiyure ezingama-24 lokuqokelelwa komchamo. Uqokelela umchamo wakho ekhaya uze uzise isampuli kugqirha wakho. Olu vavanyo lujonga ukuba izintso zakho zichaphazelekile yi-amyloidosis.
• I-Electrocardiogram (ECG): I-ECG ilinganisa umsebenzi wombane wentliziyo.
• I-Echocardiogram : Ngamanye amaxesha ibizwa ngokuba yi-ultrasound yentliziyo, oku kusebenzisa amaza esandi asebenza rhoqo ukulinganisa iintshukumo zentliziyo.
• I-Cardiac MRI (Cardiac Magnetic Resonance Imaging) : Olu vavanyo lungathatha imifanekiso ecacileyo neneenkcukacha zentliziyo.

Inyangwa njani i-AL amyloidosis?

Oogqirha banyanga i-AL amyloidosis ukunciphisa iimpawu zakho, ukulawula umonakalo wamalungu omzimba, kunye nokucothisa okanye ukumisa ukwakheka kweeproteni ze-amyloid ezingaqhelekanga.

Oogqirha banokunyanga i-AL amyloidosis nge-chemotherapy, i-immunotherapy, okanye ii-steroids. Uninzi lwabantu lunikwa iyeza elinye okanye amabini e-chemotherapy, adla ngokudityaniswa nee-steroids. La mayeza asebenza kunye ukutshabalalisa iiseli ze-plasma ezenza iiproteni ze-light chain.

Okubalulekileyo kukuba, nangona la mayeza enokuthintela okanye anciphise ukuqhubela phambili kwe-AL amyloidosis, awanakukwazi ukususa ii-amyloid fibrils esele ziqokelelene kwizitho zakho zomzimba. Xa unyango luqala, inkqubo yakho yomzimba ingazisusa kancinci kancinci ezi proteni zingaqhelekanga. Abaphandi bakwakhangela ii-antibodies ezintsha ze-monoclonal ezinokususa ezi fibrils.

Ugqirha wakho uza kuthetha nawe malunga nokuba ungazuza na "kukufakelwa i-bone marrow" okanye "ukufakelwa i-stem cell." Nangona ezi ziindlela zonyango ezinzima ngakumbi, zinokuba yimpumelelo enkulu kwabanye abantu.

Ngaba uphuhliso lwe-'AL amyloidosis' lunokuthintelwa?

Ngelishwa, hayi. Akukho ndlela yokuthintela i-AL amyloidosis okanye naluphi na olunye uhlobo lwe-amyloidosis. I-AL amyloidosis yenzeka xa iseli enye yeplasma iba yinto engaqhelekanga kwaye iqala ukwahlukana ngokungalawulekiyo. Le asiyonto esinokuyilawula.

Ungaphila ixesha elingakanani une-'AL amyloidosis'?

Oogqirha ngoku banceda abantu abane-AL amyloidosis baphile ixesha elide. Kwabanye, i-AL amyloidosis yimeko yexesha elide, okanye engapheliyo, enokulawulwa ngamayeza. Nangona kunjalo, kubalulekile ukukhumbula ukuba le yimeko enzulu kakhulu kwaye inokukhokelela kwiimeko zonyango ezisongela ubomi.

Ngenxa yokuba i-AL amyloidosis sisifo esingaqhelekanga, kunzima koogqirha ukwazi ukuba umntu onesifo angaphila ixesha elingakanani. Ukuba une-AL amyloidosis, buza ugqirha wakho malunga noqikelelo lwakho emva konyango. Ugqirha wakho angakunika ulwazi olungcono malunga nokuba ungalindela ntoni.

Ndingazinyamekela njani ?

Ukuba une-AL amyloidosis, ugqirha wakho uza kugxila kakhulu ekunyangeni iimpawu zakho kunye nokuthintela okanye ukucothisa ukuqhubela phambili kwesi sifo. Kusenokufuneka uqhubeke usela amayeza, kwaye loo mayeza anokuba nemiphumo emibi. Enye indlela onokuzinyamekela ngayo kukufunda ngemiphumo emibi yonyango lwakho kunye nendlela yokuyilawula. Nazi ezinye iingcebiso:

• Ezinye iindlela zonyango zinokuchaphazela umnqweno wakho wokutya.Ukuba kunjalo, buza ugqirha wakho malunga neendlela zokutya ezisempilweni eziya kukunceda uhlale unamandla ngexesha lonyango. Ukutya ukutya okunesondlo kubaluleke kakhulu.
• Zama ukuphumla ngokwaneleyo . Ukuphumla kubalulekile emzimbeni xa ulwa nesifo esifana nesi.
• Ukuzilolonga yindlela entle yokugcina ingqondo yakho yonwabile kwaye ikuncede ugcine ubunzima obusempilweni. Nangona kunjalo, cela iingcebiso kugqirha wakho malunga nokuzilolonga okungayi kubangela uxinzelelo olukhulu emzimbeni wakho.
• Ngenxa yokuba i-AL amyloidosis sisifo esingaqhelekanga, ungaziva unesizungu kwaye ungodwa . Abanye basenokungasiqondi esi sifo. Buza ugqirha wakho malunga namaqela enkxaso okanye iinkqubo zabantu abane-AL amyloidosis. Ukuthetha nabantu abanjalo kunokuba ngumthombo omkhulu wamandla.

Okokugqibela, amanqaku ambalwa abalulekileyo (Umyalezo Wokuya Ekhaya)

I-AL amyloidosis (i-Amyloid light chain okanye i-primary amyloidosis) sisifo esingaqhelekanga esibangelwa kukufakwa kweeproteni ze-light chain ezingaqhelekanga kwizitho zomzimba wethu. Isenokuba sisifo esibi kakhulu , ngamanye amaxesha siphathwa njengesifo esingapheliyo, kodwa sinokubangela neemeko zempilo ezisongela ubomi.

Kodwa ungakhathazeki, oogqirha bafumana iindlela ezintsha zokunceda abantu abane-AL amyloidosis baphile ixesha elide kwaye baphile ubomi obungcono. Ukuba une-AL amyloidosis, buza ugqirha wakho malunga novavanyo olutsha lweklinikhi kunye nophando lokunyanga esi sifo. Ukufunyanwa kwangoko kunye nonyango kubalulekile. Kubalulekile nokuba uhlale unethemba kwaye ulandele iingcebiso zikagqirha wakho.

I- AL amyloidosis, i-primary amyloidosis, iiseli zeplasma, iiproteni ze-light chain, ii-amyloid fibrils, isifo sezintso, isifo sentliziyo