Ingabe uhlala ugula? Kungaba ngenxa ye-Primary Immunodeficiency!

Ingabe wena noma othile emndenini wakho ugula njalo? Ingabe ngezinye izikhathi uthola umkhuhlane noma umkhuhlane ovamile, kodwa kuthatha isikhathi eside ukuba ngcono? Noma ingabe uthola ukugula okufanayo ngokuphindaphindiwe? Ngezinye izikhathi isizathu salokhu kungaba ubuthakathaka ohlelweni lwethu lokuzivikela lomzimba, olulwa nezifo. Namuhla sizokhuluma ngesimo esinjalo, okuyi-primary immunodeficiency, noma njengoba sibizwa ngesiNgisi `(Primary Immunodeficiency)`.

Kuyini i-Primary Immunodeficiency?

Kalula nje, ukuntuleka kwamasosha omzimba okuyinhloko kuyiqembu lezimo ezingaphezu kuka-400 ezahlukene lapho amasosha ethu omzimba engasebenzi kahle . Cabanga ngale ndlela: umzimba wethu ufana nenqaba. Kunamasosha okuvikela le nqaba. Lawo masosha ayisimiso sethu somzimba somzimba. Ngakho-ke, uma la masosha ebuthakathaka, noma uma engasebenzi kahle, izitha zangaphandle, okungukuthi, amagciwane athelelanayo (amagciwane, amagciwane, njll.), zingangena kalula emzimbeni futhi zibangele izifo.

Amanye amagama amaningana asetshenziswa kulesi simo:

• Isifo esiyinhloko sokuntuleka kwamasosha omzimba.
• Isifo Sokuntuleka Kwamasosha Omzimba Esiyinhloko (i-PIDD).
• Amaphutha Okuzalwa Nawo Okuzivikela Emzimbeni (IEI).

Lezi zimo zivame ukubangelwa izinguquko ezakhiweni zethu zofuzo, ezibizwa ngokuthi "ukuguqulwa kwezakhi zofuzo." Lezi zivame ukudluliselwa ezizukulwaneni ngezizukulwane, okusho ukuthi zizuzwe njengefa . Kodwa ngezinye izikhathi umuntu angahlakulela lesi simo ngokuphelele ngaphandle komlando womndeni. Odokotela bayayelapha ngokuvimbela nokulawula izifo, nangokufaka ezinye izingxenye zesimiso somzimba sokuzivikela ezintulayo.

Yiziphi izibonelo zokuntuleka kwamasosha omzimba okuyinhloko?

Njengoba ngishilo ngaphambili, kunezinhlobo ezingaphezu kuka-400 zokuntuleka kwamasosha omzimba okuyisisekelo. Uhlobo ngalunye luhlukile, futhi ubukhali balo buyahlukahluka . Yingakho iminyaka lapho lezi zimo zivela khona ingahluka. Ezinye izinhlobo zibangela izinkinga ngokushesha nje lapho umntwana ezalwa, esemncane. Ngakho-ke zingatholakala kusenesikhathi. Kodwa kunezinye izinhlobo ezingezimbi kangako. Ngakho-ke ungase ungazi nokuthi unalesi simo uze ube mdala.

Nazi ezinye izibonelo zalesi simo:

• Ukuntuleka Kwamasosha Omzimba Okuvamile Okuguquguqukayo (CVID)
• I-Ataxia-telangiectasia
• Isifo Esingamahlalakhona Se-Granulomatous (CGD)
• I-DiGeorge syndrome
• I-Hemophagocytic lymphohistiocytosis `(Hemophagocytic lymphohistiocytosis)`
• Ukuntuleka kwe-IgA ekhethiwe
• I-agammaglobulinemia exhumene ne-X

Manje, ungadideki ngala magama. Lawa ngamagama ezokwelapha nje. Into ebaluleke kakhulu ukufuna iseluleko sezokwelapha uma ungaqiniseki ngokuthi unalesi simo.

Ziyini izimpawu zokuntuleka kwamasosha omzimba okuyinhloko?

Kubantu abaningi, uphawu lokuqala lokuntuleka kwamasosha omzimba okuyinhloko ukutheleleka okuvamile, okuqhubekayo, noma okungavamile okunzima ukukwelapha . Lokhu kutheleleka kungaba kubi kakhulu , noma amanye amalungu omndeni angase abe nezinkinga ezifanayo.

Ezinye izimpawu ezingase zibonakale zifaka:

• Ukuthatha ama-antibiotic izikhathi eziningi ukuze welaphe izifo.
• Izinkinga eziphakama ngemva kokuthola umuthi wokugomela ophilayo.
• Ubende olukhulisiwe (Ubende luyisitho esitholakala ohlangothini olungaphezulu kwesobunxele lwesisu sethu esiza amasosha omzimba).
• Ama-lymph node avuvukele (lawo azwakala sengathi amaqhubu esandleni).
• Ukwehla kwesisindo noma ukwehluleka ukukhula (ikakhulukazi ezinganeni ezincane).
• Izinkinga zokugaya ukudla ezivame ukwenzeka njengohudo.
• Izifo ezizenzakalelayo ( izimo lapho amasosha omzimba wethu ehlasela khona amangqamuzana ethu).

Cabanga nje, ingane yakho inomkhuhlane nokukhwehlela kanye noma kabili ngenyanga futhi kufanele inikwe ama-antibiotic. Noma, ihlala ihluma amabala amakhulu esikhumbeni sayo, okuthatha isikhathi eside ukuphola. Uma lezi zinto ziqhubeka zenzeka, udinga ukukhathazeka ngakho.

Yini ebangela lokhu?

Njengoba ngishilo ekuqaleni, ukuntuleka kwamasosha omzimba okuyinhloko kubangelwa izinguquko zofuzo ezithinta izingxenye zesimiso sethu somzimba, njengamaseli namaprotheni . Lezi zinguquko zofuzo zingabangela ezinye izingxenye zesimiso sethu somzimba omzimba ukuthi:

• Kungase kungasebenzi kahle kunokujwayelekile.
• Kungase kube nephutha .
• Kungase kunyamalale ngokuphelele .

Phakathi kuka-50% no-60% walezi zimo kubangelwa ukukhubazeka kumaseli abizwa ngokuthi ama-B lymphocytes (amaseli e-B) . Lawa maseli e-B awuhlobo olukhethekile lweseli ohlelweni lwethu lokuzivikela komzimba. Yiwo enza ama-antibodies , amaprotheni akhethekile asiza ekubhubhiseni amagciwane afana namagciwane namagciwane angena emizimbeni yethu. Ngakho-ke cabanga, uma lawa maseli e-B engasebenzi kahle, uma engakhiqizi ama-antibodies, imizimba yethu isengozini enkulu yezifo, akunjalo?

Ubani osengozini enkulu yokuthola lesi simo?

Noma ubani angaba ne-PIDD. Kodwa uma othile emndenini wakho wemvelo enayo, kungenzeka kakhulu ukuthi uyithole . Esikhathini esiningi, lokhu kuntuleka kwamasosha omzimba okuyinhloko kuvela ngaphambi kweminyaka engama-20. Futhi, kwabesilisaKuthiwa futhi lesi simo sivamile.

Yiziphi izinkinga ezingabangelwa yilokhu?

Ukuntuleka kwamasosha omzimba okuyinhloko kungandisa ingozi yezinkinga kamuva ekuphileni. Lokhu kungafaka phakathi izifo zokuzivikela komzimba noma umdlavuza othile . Uma kungelashwa, i-PIDD ingaholela ezifweni ezinzima kakhulu .

Odokotela bakuthola kanjani lokhu?

Udokotela wakho uzonquma ukuthi unayo yini i-PIDD ngokubuyekeza umlando wakho wezokwelapha kanye nowomndeni, ukuhlolwa ngokomzimba, kanye nokwenza izivivinyo eziningana zelebhu .

Ukuqinisekisa ukuxilongwa, udokotela wakho angase acele ukuhlolwa okufana nalokhu:

• Ukuhlolwa kwegazi: Lokhu kungathola ukukhubazeka okuthile ohlelweni lokuzivikela komzimba (isibonelo, amazinga aphansi e-antibody noma inani eliphansi lamaseli omzimba).
• Ukuhlolwa kwezakhi zofuzo: Ukuthola amaphutha ezakhi zofuzo, okungukuthi izinguquko.
• I-Flow cytometry: Lena indlela yokuhlola amasampula amaseli omzimba kusetshenziswa imisebe ekhethekile ye-laser.

Ngaphezu kwalokho, kwamanye amazwe, njenge-United States, ukuhlolwa kwezinsana kwenziwa cishe kuzo zonke izingane ezisanda kuzalwa ukuze kuhlolwe uhlobo lwe-severe combined immunodeficiency (SCID). Lokhu kuvumela ukuhlonza nokwelapha izingane ezinalesi simo esibucayi kusenesikhathi.

Yiziphi izindlela zokwelapha lokhu?

Uma kutholakala ukuthi unesifo sokuntuleka kwamasosha omzimba okuyisisekelo, imigomo eyinhloko yokwelashwa ukulawula ukutheleleka kwamanje nokuvimbela ukutheleleka kwesikhathi esizayo. Ukwelashwa okuqondile okutholayo kuzoncika ohlotsheni lokutheleleka onalo kanye nohlobo lwe-PIDD.

Udokotela wakho angase akunike imithi efana nalokhu:

• Ama-antibiotic: Vimbela noma welaphe izifo ezibangelwa amagciwane.
• Ama-antiviral: Asiza ekululameni ezifweni ezibangelwa amagciwane.
• I-immune globulin: Lezi zinganikezwa ngemithambo yegazi (IV) noma ngaphansi kwesikhumba. Zisebenza ngokufaka ezinye izingxenye zesimiso sokuzivikela komzimba ezingekho (njengama-antibodies aphansi kakhulu).

Ngezinye izikhathi, kungase kudingeke ukuhlinzwa ukuze kulawulwe izinkinga ezibangelwa ukutheleleka. Isibonelo, uma kukhona ithumba (iqoqo lobomvu ngaphakathi kwesitho), lingasuswa ngokuhlinzwa ukuze kukhishwe ubomvu. Lokhu kungasiza ekunciphiseni ubuhlungu futhi kusheshise ukuphulukiswa.

Ezimweni ezimbi kakhulu, udokotela angenza ukufakelwa kweseli le-stem.Kunganconywa. Lokhu kuhilela ukufaka amaseli amasha esikhundleni samaseli omzimba anephutha noma alahlekile. Kulokhu, amaseli okuqala (angakhula abe ezinye izinhlobo zamaseli) athathwe kumnikeli afakwa emzimbeni wakho. Ngokuhamba kwesikhathi, lawa maseli okuqala akhula abe amaseli ajwayelekile omzimba.

Ukwelashwa ngezakhi zofuzo nakho sekuyindlela yokwelapha ephumelelayo kwezinye izinhlobo ze-PIDD.

Ingabe ukuntuleka kwamasosha omzimba okuyinhloko kungavinjelwa?

Njengoba iningi lama-PIDD libangelwa ukuguquka kwezakhi zofuzo , ayikho indlela yokuzivimbela. Kodwa-ke, uma othile emndenini wakho enesifo sokuzivikela komzimba esiyinhloko, ungase uthande ukucabangela ukwelulekwa kwezakhi zofuzo . Lokhu kungakunikeza ulwazi olwengeziwe kanye nesiqondiso.

Lizobaphathelani ikusasa labo abanalesi simo? (Isibikezelo)

Ngokwelashwa okufanele, abantu abaningi abane-PIDD bangaphila impilo enempilo. Kwezinye izimo, kungadingeka ukuthi uphuze imithi impilo yakho yonke. Kufanele futhi wenze konke okusemandleni akho ukugwema izifo . Nanka amanye amathiphu angakusiza:

• Zenze umkhuba ukugeza izandla zakho kahle — geza izandla zakho kahle ngensipho namanzi. Qiniseka ukuthi ugeza izandla zakho ngaphambi nangemva kokudla, ngemva kokusebenzisa indlu yangasese, ngemva kokuthinta izilwane, nangemva kokuthinta noma yini engcolile.
• Hlala kude nezindawo ezinabantu abaningi kanye nabantu abagulayo .
• Landela imiyalelo kadokotela wakho mayelana nokugonywa ngqo.
• Phumula ngokwanele .
• Landela uhlelo lokudla okunempilo kanye nokuzivocavoca olukufanele.

Uma nginayo, noma ngisola ukuthi nginayo, i-primary immunodeficiency, kufanele ngimbone nini udokotela?

Uma unesifo esingapheli, sibi kakhulu, noma siqhubeka sibuya, bona udokotela ukuze ahlole i-PIDD. Uma usuvele unayo i-PIDD, tshela udokotela wakho ngokushesha uma unomkhuhlane noma yiziphi izimpawu zokutheleleka . Lokhu kubalulekile ukuvimbela izinkinga.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Uma usola lesi simo, noma uma uthola ukuthi unaso, ungabuza udokotela wakho imibuzo efana nale:

• Uhlobo luni lokuntuleka kwamasosha omzimba enginalo?
• Ingabe lokhu kungazuzwa njengefa ngabantwana bami?
• Hlobo luni lokwelashwa olunconywayo?
• Yimiphi imiphumela emibi engaba khona yokwelashwa? Kufanele ngikhathazeke kanjani ngayo?
• Yiziphi izinkinga ezingaba khona ngenxa yalesi simo?

Ukuphila nezimo ezihambisana nokuntuleka kwamasosha omzimba okuyinhloko kungaba yinselele. Ukutheleleka okuvamile, okuqhubekayo, nokunzima ukukwelapha kungaba yingozi ngempela, futhi kungaba nomthelela omkhulu enhlalakahleni yakho yengqondo.

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Kodwa akudingeki ukuthi udlule kulolu hambo wedwa. Cela udokotela wakho akusize uthole iqembu lokusekelana lapho ungaxoxa khona futhi wabelane ngemibono nabanye abake badlula kokuhlangenwe nakho okufanayo. Lokhu kungaba umthombo omkhulu wamandla.

Okokugcina, khumbula lokhu (Umyalezo Wokuya Ekhaya)

Kulungile, nansi eminye yemicikilisho okufanele uyikhumbule mayelana ne-Primary Immunodeficiency esikhulume ngayo namuhla:

• Lesi yisifo esibangelwa ubuthakathaka ohlelweni lwethu lokuzivikela komzimba , ngokuvamile olusekelwe ezizakhi zofuzo .
• Esinye sezimpawu eziyinhloko ukutheleleka okuvamile, okunzima okuthatha isikhathi eside ukuphulukiswa .
• Kunezinhlobo eziningi zalokhu, ngakho-ke izimpawu kanye nobunzima kungahluka kumuntu nomuntu.
• Kubaluleke kakhulu ukuxilonga nokuthola ukwelashwa okufanele kusenesikhathi .
• Ngokwelashwa, ukutheleleka kungalawulwa futhi abantu abaningi bangaphila impilo evamile .
• Kubalulekile ukuthatha izinyathelo zokuzivikela ezifweni .

Uma wena noma othile omaziyo enanoma yiziphi zalezi zimpawu, ungangabazi ukufuna iseluleko sezokwelapha. Uma usheshe utholakale ukuthi unesifo, kulapho kungenzeka khona ukuthi ukwelashwa kuphumelele. Impilo enhle kubo bonke!

Ukuntuleka Kwamasosha Omzimba Okuyinhloko , Isimiso Sokuzivikela Emzimbeni, Ukutheleleka, Ukuguqulwa Kwezakhi Zofuzo, I-PIDD, Ukwelashwa, Izimpawu