Ingaba uhlala ugula? Isenokuba ibangelwa yiPrimary Immunodeficiency!

Ngaba wena okanye umntu osapho lwakho ugula rhoqo? Ngaba ngamanye amaxesha ufumana umkhuhlane okanye umkhuhlane, kodwa kuthatha ixesha elide ukuba uphile? Okanye ufumana ukugula okufanayo rhoqo? Ngamanye amaxesha isizathu soku sinokuba bubuthathaka kwinkqubo yethu yokuzikhusela yomzimba, elwa nezifo. Namhlanje siza kuthetha ngemeko enjalo, eyi-primary immunodeficiency, okanye njengoko ibizwa ngesiNgesi `(Primary Immunodeficiency)`.

Yintoni iPrimary Immunodeficiency?

Ngamafutshane, i-primary immunodeficiency liqela leemeko ezahlukeneyo ezingaphezu kwama-400 apho i-immune system yethu ingasebenzi kakuhle . Cinga ngale ndlela: umzimba wethu ufana nenqaba. Kukho amajoni okukhusela le nqaba. Loo majoni yinkqubo yethu yokuzikhusela. Ngoko ke, ukuba la majoni abuthathaka, okanye ukuba awasebenzi kakuhle, iintshaba zangaphandle, oko kukuthi, iintsholongwane ezisulelayo (iibhaktheriya, iintsholongwane, njl.njl.), zinokungena emzimbeni ngokulula zibangele izifo.

Amanye amagama aliqela asetyenziswa kule meko:

• Isifo esiphambili sokungabikho komzimba.
• Ingxaki yokungakhuseleki komzimba (i-PIDD) ephambili.
• Iimpazamo Zokuzalwa Zokuzikhusela (IEI).

Ezi meko zihlala zibangelwa lutshintsho kwiijini zethu, olubizwa ngokuba yi-"genetic mutations." Ezi zihlala zidluliselwa kwizizukulwana ngezizukulwana, oko kuthetha ukuba zizuzwe njengelifa . Kodwa ngamanye amaxesha umntu unokufumana le meko ngokupheleleyo ngaphandle kwembali yosapho. Oogqirha bayayinyanga ngokuthintela nokulawula usulelo, nangokutshintsha iindawo ezithile zenkqubo yomzimba yokuzikhusela ezingonelanga.

Ziziphi imizekelo ye-primary immunodeficiency?

Njengoko benditshilo ngaphambili, kukho iintlobo ezingaphezu kwama-400 ze-primary immunodeficiency. Uhlobo ngalunye lwahlukile, kwaye ubunzima bazo buyahluka . Yiyo loo nto ubudala apho ezi meko zibonakala khona bunokwahluka. Ezinye iintlobo zibangela iingxaki kwangoko nje ukuba umntwana azalwe, esengumntwana. Ngoko ke zinokufunyanwa kwangethuba. Kodwa kukho ezinye iintlobo ezingezizo ezinzulu kangako. Ngoko ke usenokungazi nokuba unale meko de ube ngumntu omdala.

Nantsi eminye imizekelo yale meko:

• Ukungakwazi komzimba okuguquguqukayo okuqhelekileyo (i-CVID)
• I-Ataxia-telangiectasia
• Isifo seGranulomatous esingapheliyo (CGD)
• Isifo sikaDiGeorge
• I-Hemophagocytic lymphohistiocytosis `(Hemophagocytic lymphohistiocytosis)`
• Ukunqongophala kwe-IgA ekhethiweyo
• I-agammaglobulinemia edityanisiweyo ne-X

Kaloku, musa ukubhideka ngala magama. La ngamagama nje ezonyango. Eyona nto ibalulekileyo kukufuna ingcebiso kagqirha ukuba unamathandabuzo malunga nokuba unale meko.

Zithini iimpawu ze-primary immunodeficiency?

Kubantu abaninzi, uphawu lokuqala lokunqongophala kwamasosha omzimba lusulelo oluqhelekileyo, oluhlala luhleli, okanye olungaqhelekanga olunzima ukunyanga . Olu sulelo lunokuba yingozi kakhulu , okanye amanye amalungu osapho anokuba neengxaki ezifanayo.

Ezinye iimpawu ezinokubonwa ziquka:

• Kufuneka uthathe amayeza okubulala iintsholongwane amaxesha amaninzi ukuze unyange usulelo.
• Iingxaki ezivela emva kokufumana isitofu sokugonya esiphilayo.
• I-spleen ekhulisiweyo (I-spleen sisitho esikwicala eliphezulu lasekhohlo lesisu sethu esinceda inkqubo yomzimba yokuzikhusela).
• Ii-lymph nodes ezidumbileyo (ezo zivakala ngathi ziqhuma esandleni).
• Ukwehla kobunzima okanye ukungaphumeleli ekukhuleni (ingakumbi kubantwana abancinci).
• Iingxaki zokugaya ukutya rhoqo ezifana norhudo.
• Izifo ezizikhusela ngokwazo emzimbeni ( iimeko apho amajoni omzimba wethu ahlasela iiseli zethu).

Khawucinge nje, umntwana wakho unesifo somkhuhlane kunye nokukhohlela kanye okanye kabini ngenyanga kwaye kufuneka anikwe amayeza okubulala iintsholongwane. Okanye, uhlala eba namaqhuqhuva aqatha kunye namadyungudyungu eluswini lwakhe, athatha ixesha elide ukuphola. Ukuba ezi zinto ziyaqhubeka zenzeka, kufuneka ukhathazeke ngayo.

Yintoni ebangela oku?

Njengoko benditshilo ngaphambili, i-primary immunodeficiency ibangelwa ziinguqu zemfuza ezichaphazela iindawo ezithile zenkqubo yethu yokuzikhusela komzimba, ezifana neeseli kunye neeproteni . Ezi nguqu zemfuza zinokubangela ukuba ezinye iindawo zenkqubo yethu yokuzikhusela komzimba:

• Isenokuba ayisebenzi kangako kunesiqhelo.
• Isenokuba inesiphene .
• Isenokuphela ngokupheleleyo .

Phakathi kwama-50% nama-60% ezi meko zibangelwa ziziphene kwiiseli ezibizwa ngokuba yi-B lymphocytes (iiseli ze-B) . Ezi seli ze-B luhlobo olukhethekileyo lweeseli kwinkqubo yethu yokuzikhusela. Zezo zenza ii-antibodies , iiproteni ezikhethekileyo ezinceda ekutshabalaliseni iintsholongwane ezifana neebhaktheriya kunye neentsholongwane ezingena emizimbeni yethu. Khawucinge nje, ukuba ezi seli ze-B azisebenzi kakuhle, ukuba azivelisi ii-antibodies, imizimba yethu isengozini enkulu yezifo, akunjalo?

Ngubani osengozini enkulu yokuhlaselwa yile meko?

Nabani na unokuba ne-PIDD. Kodwa ukuba umntu kusapho lwakho lwendalo unayo, unokuba nayo . Uninzi lwexesha, ezi zifo ziphambili zokuzikhusela komzimba zibonakala ngaphambi kokuba zibe neminyaka engama-20 ubudala. Kwakhona, kumadodaKuthiwa le meko ixhaphakile.

Zeziphi iingxaki ezinokubangela oku?

Ukunqongophala kwamasosha omzimba okuphambili kunokunyusa umngcipheko weengxaki kamva ebomini. Oku kungabandakanya iingxaki zokuzikhusela komzimba okanye umhlaza othile . Ukuba ayinyangwa, i-PIDD inokukhokelela kwiintsholongwane ezinzima ngokungaqhelekanga .

Oogqirha bayixilonga njani le nto?

Ugqirha wakho uza kuqinisekisa ukuba unayo na i-PIDD ngokuhlola imbali yakho yezonyango kunye neyentsapho yakho, uvavanyo lomzimba, kunye nokwenza iimvavanyo ezininzi zelebhu .

Ukuqinisekisa ukuxilongwa, ugqirha wakho unokuyalela iimvavanyo ezifana nezi:

• Uvavanyo lwegazi: Olu vavanyo lunokubona iingxaki ezithile kwinkqubo yomzimba yokuzikhusela (umzekelo, amanqanaba aphantsi e-antibody okanye amanani aphantsi eeseli zomzimba zokuzikhusela).
• Uvavanyo lwe-genetic: Ukufumanisa iziphene kwi-genes, oko kukuthi, utshintsho.
• I-Flow cytometry: Le yindlela yokuhlola iisampulu zeeseli zenkqubo yomzimba yokuzikhusela kusetyenziswa imisebe ekhethekileyo yelaser.

Ukongeza, kwamanye amazwe, afana ne-United States, uvavanyo losana olusandul’ ukuzalwa lwenziwa phantse kuwo onke amasana ukuze kuhlolwe uhlobo lwe-severe combined immunodeficiency (SCID). Oku kuvumela ukuchongwa kwangethuba kunye nonyango lweentsana ezinale meko inzima.

Zithini iindlela zonyango zoku?

Ukuba ufunyaniswe une-primary immunodeficiency, iinjongo eziphambili zonyango kukulawula usulelo lwangoku kunye nokuthintela usulelo lwexesha elizayo. Unyango oluchanekileyo olufumanayo luya kuxhomekeka kuhlobo losulelo onalo kunye nohlobo lwe-PIDD.

Ugqirha wakho angakunika amayeza anjengala:

• Ii-antibiotics: Thintela okanye unyange usulelo lweentsholongwane.
• Ii-Antivirals: Zinceda ekupholeni kwiintsholongwane ezibangelwa ziintsholongwane.
• I-immune globulin: Ezi zinokunikwa ngemithambo yegazi (IV) okanye nge-subcutaneous. Zisebenza ngokutshintsha ezinye zeendawo zenkqubo yomzimba yokuzikhusela ezingekhoyo (ezifana nee-antibodies eziphantsi kakhulu).

Ngamanye amaxesha, utyando lunokufuneka ukulawula iingxaki ezibangelwa lusulelo. Umzekelo, ukuba kukho ithumba (ingqokelela yobovu ngaphakathi kwilungu), linokususwa ngotyando ukuze kukhutshwe ubovu. Oku kunokunceda ukunciphisa iintlungu nokukhawulezisa ukuphiliswa.

Kwiimeko ezinzima ngakumbi, ugqirha unokwenza utyando lwe-stem cell.Kungacetyiswa. Oku kuquka ukutshintsha iiseli zenkqubo yomzimba ezingasebenzi kakuhle okanye ezilahlekileyo ngeeseli ezintsha. Kule meko, iiseli zesiseko (ezinokukhula zibe zezinye iintlobo zeeseli) ezithathwe kumnikeli ziyafakwa emzimbeni wakho. Ekuhambeni kwexesha, ezi seli zesiseko ziyakhula zibe ziiseli zenkqubo yomzimba yomzimba eziqhelekileyo.

Unyango lwe-gene luye lwaba yindlela yonyango ephumelelayo kwezinye iintlobo ze-PIDD.

Ngaba ukungakwazi ukuzikhusela komzimba okuqhelekileyo kungathintelwa?

Ekubeni uninzi lwe-PIDDs lubangelwa kukuguquka kwezakhi zofuzo , akukho ndlela yokuzithintela. Nangona kunjalo, ukuba kukho umntu kusapho lwakho onesifo sokungakhuseleki kwamasosha omzimba, ungacinga ngokucebisa ngezakhi zofuzo . Oku kunokukunika ulwazi oluthe kratya kunye nesikhokelo.

Liza kubaphatha njani ikamva abo banale meko? (Uqikelelo)

Ngonyango olufanelekileyo, uninzi lwabantu abane-PIDD banokuphila ubomi obusempilweni. Kwezinye iimeko, kunokufuneka uthathe amayeza ubomi bakho bonke. Kufuneka wenze konke okusemandleni akho ukunqanda usulelo . Nazi ezinye iingcebiso zokunceda:

• Ziqhelise ukuhlamba izandla zakho kakuhle — hlamba izandla zakho kakuhle ngesepha namanzi. Qiniseka ukuba uhlamba izandla zakho ngaphambi nasemva kokutya, emva kokusebenzisa indlu yangasese, emva kokuchukumisa izilwanyana, nasemva kokuchukumisa nantoni na engcolileyo.
• Sukuma kwiindawo ezixineneyo nabantu abagulayo .
• Landela imiyalelo kagqirha wakho malunga nokugonywa ngokuchanekileyo.
• Phumla ngokwaneleyo .
• Landela ukutya okunempilo kunye nesicwangciso sokuzilolonga esikufaneleyo.

Ukuba ndinayo, okanye ndicinga ukuba ndinayo, i-primary immunodeficiency, kufuneka ndiye kugqirha nini?

Ukuba unentsholongwane engapheliyo, imbi kakhulu, okanye ihlala ibuya, bona ugqirha ukuze ahlole i-PIDD. Ukuba sele unayo i-PIDD, xelela ugqirha wakho ngoko nangoko ukuba unomkhuhlane okanye naziphi na iimpawu zosulelo . Oku kubalulekile ukuthintela iingxaki.

Ndingayibuza yiphi imibuzo ugqirha wam?

Ukuba ukrokrela ukuba le meko, okanye ukuba ufumanisa ukuba unayo, ungabuza ugqirha wakho imibuzo efana nale:

• Luhlobo luni lokungasebenzi kakuhle kwamasosha omzimba endinalo?
• Ngaba oku kungenziwa ngabantwana bam njengelifa?
• Luhlobo luni lonyango olucetyiswayo?
• Zithini iziphumo ebezingalindelekanga zonyango? Ndingaxhalaba njani ngazo?
• Ziziphi iingxaki ezinokwenzeka ngenxa yale meko?

Ukuphila neemeko ezinxulumene nokusilela kwamasosha omzimba okuphambili kunokuba nzima. Usulelo oluhlala luhleli, oluhlala luhleli, nolunzima ukulunyanga lunokuba buthathaka ngokwenene, kwaye lunokuba nefuthe elikhulu kwimpilo-ntle yakho yengqondo.

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Kodwa akufuneki uhambe wedwa kolu hambo. Cela ugqirha wakho akuncede ufumane iqela lenkxaso apho unokuthetha khona kwaye wabelane ngezimvo nabanye abaye badlula kumava afanayo. Oku kunokuba ngumthombo omkhulu wamandla.

Okokugqibela, khumbula oku (Umyalezo Wokuya Ekhaya)

Kulungile, ke, nazi izinto ekufuneka uzikhumbule ngokufutshane malunga nePrimary Immunodeficiency esithethe ngayo namhlanje:

• Esi sisifo esibangelwa bubuthathaka kwinkqubo yethu yokuzikhusela komzimba , sidla ngokusekelwe kwizizathu zemfuza .
• Enye yeempawu eziphambili kukwanda kosulelo oluqatha noluthatha ixesha elide ukuphola .
• Kukho iintlobo ezininzi zale nto, ngoko ke iimpawu kunye nobunzima zinokwahluka kumntu nomntu.
• Kubaluleke kakhulu ukuxilonga nokufumana unyango olufanelekileyo kwangethuba .
• Ngonyango, usulelo lunokulawulwa kwaye abantu abaninzi banokuphila ubomi obuqhelekileyo .
• Kubalulekile ukuthatha amanyathelo okuzikhusela kwiintsholongwane .

Ukuba wena okanye umntu omaziyo unenye yezi mpawu, ungathandabuzi ukufuna ingcebiso kugqirha. Okukhona ufunyaniswa unesifo ngokukhawuleza, kokukhona kunokwenzeka ukuba unyango luphumelele. Impilo entle kubo bonke!

Ukunqongophala komzimba okuphambili , Inkqubo yoKhuseleko loMzimba, iiNtsholongwane, iiNguqu zoFuzo, i-PIDD, unyango, iimpawu