Kuyini ukushoda kwe-Alpha-1 antitrypsin?

Ukushoda kwe-Alpha-1 antitrypsin, okuvame ukubizwa ngokuthi i-Alpha -1, kuyisimo sofuzo esingadluliselwa kusuka kubazali kuya ezinganeni zabo. Abantu abane-Alpha-1 banamazinga aphansi ephrotheni ebizwa ngokuthi i-AAT, evikela amaphaphu. EYurophu nase-United States, cishe abantu abangu-100,000 1 ezweni ngalinye bahlaselwa i-Alpha-XNUMX.

I-Alpha -1 ingabangela i-emphysema (amasaka omoya asemaphashini akho alimele), ukusha kwesibindi (izibazi esibindini), kanye ne-panniculitis (isimo sesikhumba esingavamile esingenza isikhumba sibe lukhuni, sibe buhlungu, sibomvu, noma sibe mnyama kwezinye izindawo).

Kuba kubi kakhulu ngokuhamba kwesikhathi. Nakuba lingekho ikhambi le-Alpha-1, ukwelashwa kungasiza izimpawu ezithile. Abantu abaningi abane-Alpha-1 bangaphila isikhathi esivamile, ikakhulukazi uma bengabhemi.

Izimpawu zamaphaphu: Izimpawu zamaphaphu zivame ukubukeka njengezifo ezingamahlalakhona ezivimbela amaphaphu (COPD), njenge:

  • Ukukhwehlela okungapheli ngephlegm
  • Ukushisa
  • Ukuphelelwa umoya
  • Imikhuhlane yesifuba ephindaphindiwe

Izimpawu zesibindi:

  • Isikhumba namehlo aphenduka aphuzi (i-jaundice)
  • Ukukhathala
  • Ukuvuvukala kwesisu

Ngezinye izikhathi, abantu bangaba nesimo sesikhumba esingajwayelekile, i-panniculitis. Kungase kubukeke njengamaqhubu abuhlungu, abomvu anganyakaza emzimbeni wakho futhi angaqhuma futhi avuze ubomvu noma uketshezi.

Amaphaphu abantu abane-Alpha-1 angonakala ngoba igazi labo alinayo iphrotheni ebizwa ngokuthi i-alpha-1 antitrypsin (AAT). Lelo phrotheni lenziwa esibindini futhi livikela amaphaphu ekuvuvukeni lapho echayeka entuthuni kagwayi noma ezinye izinto eziwucasulayo. Ngaphandle kwaleso sivikelo emaphashini, angalimala ngemva kokuhogela izinto ezicasulayo.

Isibindi sabantu abane-Alpha-1 singalimala lapho i-AAT ingakwazi ukukhululwa esibindini
isilinganiso esivamile. Inganqwabelana esibindini futhi ibangele isifo sesibindi.

I-Alpha-1 ivame kakhulu phakathi kwabantu abanozalo lwaseYurophu noma lwaseNyakatho Melika. Kodwa ezinye izinhlanga noma izinhlanga nazo zingathola i-Alpha-1. Abantu abasemathubeni okuthuthukisa i-Alpha-1 yilabo abanabazali abanamazinga aphansi we-AAT protein.

Uma othile ene-Alpha-1, izinto ezithile zingandisa ingozi yokuba nesifo samaphaphu.

  • Ukubhema
  • Ukuchayeka entuthuni kagwayi
  • Ukuba nomlando womndeni we-emphysema noma isifuba somoya
  • Ukuba nezifo zamaphaphu eziphindaphindiwe
  • Ukuhlala noma ukusebenza ezindaweni ezinothuli

Khuluma nodokotela wakho mayelana nokuhlolwa uma ucabanga ukuthi une-Alpha-1, unezimpawu, noma uma wena noma amalungu omndeni wakho ninomlando wesibindi noma isifo samaphaphu. Udokotela wakho angenza ukuhlolwa kwegazi ukuze ahlole izinga leprotheyini ye-AAT egazini lakho kanye nokuhlolwa kofuzo ukuze abone ukuguqulwa kofuzo.

Ukuhlolwa kokusebenza kwamaphaphu okuvamile (PFTs) kuyanconywa ukuze kuqashwe ukusebenza kwamaphaphu. Ukuhlolwa kokusebenza kwesibindi nezifundo zokucabanga kungase kudingeke ukuze kuhlolwe impilo yesibindi.

Ukwelashwa kwe-Augmentation:

  • Igazi elinikelwe elinamazinga ajwayelekile e-Alpha-1 unganikezwa wena nge-IV ukuze kwandiswe amazinga egazini nasemaphashini, okuhloswe ngawo ukunciphisa isifo samaphaphu. Lokhu ikakhulukazi kubantu abane-emphysema ngenxa ye-Alpha-1.

Ukwelashwa Kwezimpawu:

  • Ama-bronchodilator kanye ne-corticosteroids ephefumulelwe: esetshenziselwa ukuphatha izimpawu zamaphaphu kanye nokwenza ngcono ukusebenza kwamaphaphu.
  • I-Oxygen Therapy: kungase kudingeke kubantu abanomoya-mpilo omncane egazini labo.

Izinguquko Zendlela Yokuphila:

  • Ukuyeka ukubhema: ukubhema kusheshisa umonakalo emaphashini akho. Kubalulekile ukuqhubeka uzama ukuyeka ukubhema, noma ngabe awuzange uphumelele esikhathini esidlule.
  • Ukugoma: Imijovo yomkhuhlane (umkhuhlane) kanye ne-pneumococcal (inyumoniya) iyatuswa ukuvimbela izifo zamaphaphu.
    Funda kabanzi lapha mayelana namandla okugoma kubantu abanesifo esingelapheki sokuphefumula.
  • Ukuvuselelwa kwamaphaphu kungakusiza uphile futhi uphefumule kangcono. Uhlelo lwezokwelapha olugadiwe olungakusiza ukuthi uthole amandla, lwenza kube lula ukusebenza noma ukuya emisebenzini yomphakathi, futhi luphinde lwehlise ukudangala nokukhathazeka.

Ukulawulwa Kwezifo Zesibindi:

  • Udokotela wakho uzofuna ukungena nawe njalo ukuze abuke izinkinga zesibindi. Ezimweni ezithuthukile, ukufakelwa kwesibindi kungase kucatshangelwe.

Izindlela Zokwelapha Ezintsha Nocwaningo:

  • Ukwelashwa kwezakhi zofuzo kanye nezinketho zezidakamizwa ezintsha ziyacwaningwa ngendlela engenzeka yokwelapha yesikhathi esizayo ye-alpha-1.

Ungesabi ukubuza udokotela wakho imibuzo. Ukukhuluma nodokotela wakho nokuqonda i-Alpha-1 kungakusiza uthole ukunakekelwa okungcono kakhulu ngangokunokwenzeka. Nansi eminye imibuzo okufanele uyibuze udokotela wakho, kodwa qiniseka ukuthi ubuza imibuzo nganoma yini ongayiqondi.

  • Ingabe kufanele ngihlolelwe i-Alpha-1?
  • Yiziphi izindlela engingazikhetha zokwelashwa?
  • Yiziphi ezinye izimo zezempilo okufanele ngiyazi ngazo?
  • Ngingakwazi ukuqhubeka nokusebenza futhi ngihlanganyele emisebenzini engiyijabulelayo?
  • Ngingakuyeka kanjani ukubhema?
  • Kufanele ngihlele nini ukuvakashela udokotela ozolandela?
  • Ingabe amalungu omndeni wami kufanele ahlolelwe igazi le-AAT?
  • Ngingakhuluma kanjani namalungu omndeni wami nge-Alpha-1?

Awukwazi ukuvimbela i-Alpha-1 ngoba iyisifo sofuzo. Kodwa, unganciphisa ingozi yokuthola isifo sesibindi noma samaphaphu uma une-Alpha-1.

  • Ungabhemi noma uvape.
  • Gwema utshwala.
  • Gwema noma yini engacasula amaphaphu akho, njengamakhemikhali, uthuli, nezinye izinto ezingezwani nomzimba.
  • Zivocavoce njalo.
  • Yidla ukudla okunempilo.

Resources

I-Alpha-1 Liver Discussion Guide (IsiNgisi)
I-Alpha-1 Liver Discussion Guide (French)
I-Alpha-1 Liver Discussion Guide (isiJalimane)
I-Alpha-1 Liver Discussion Guide (isiPutukezi)

Ngingakuthola kuphi okwengeziwe?

I-Alpha-1 Foundation
I-American Lung Association
I-Alpha-1 Europe Alliance
I-European Lung Foundation
I-Alpha-1 Association yase-Australia

Ukubhaliswa

Iphothali ye-Alpha-1 Registry Research (US)
I-European Alpha-1 Research Collaboration (EARCO)

Izilinganiso:

  1. Iyini i-Alpha-1? I-Alpha-1 Foundation. Ifinyelele ngo-Okthoba 14, 2024. https://alpha1.org/what-is-alpha1/
  2. Ukushoda kwe-Alpha-1 Antitrypsin - Indlela Esebenza ngayo (ividiyo). I-Alpha-1 Foundation. Ifinyelele ku-Okthoba 14, 2024. https://vimeo.com/247506044
  3. Ukuntuleka kwe-Alpha-1 Antitrypsin. Umtholampilo waseCleveland. Ifinyelele ngo-Okthoba 14, 2024.
    https://my.clevelandclinic.org/health/diseases/21175-alpha-1-antitrypsin-deficiency
  4. Ukuhlolwa kwe-Dermatopathology kwePanniculitis. Isikhungo Sikazwelonke Solwazi Lwe-Biotechnology, Umtapo Wezincwadi Kazwelonke Wezokwelapha, Izikhungo Zikazwelonke Zezempilo. Ifinyelele ngo-Okthoba 14, 2024. https://www.ncbi.nlm.nih.gov/books/NBK606121/
  5. Ukuvuselelwa kwamaphaphu. National Heart, Lung and Blood Institute. Ifinyelele ngo-Okthoba 14, 2024. https://www.nhlbi.nih.gov/health/pulmonary-rehabilitation
  6. Ukushoda kwe-Alpha-1 Antitrypsin – Izimpawu Nezimbangela. Pennmedicine.org. Ishicilelwe ngo-2022. Ifinyelele Novemba 4, 2024. https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/alpha-1-antitrypsin-deficiency
  7. I-American Lung Association. Funda Ngokushoda kwe-Alpha-1 Antitrypsin. Lung.org. Ishicilelwe ngo-2024 Ifinyelele ngoNovemba 4, 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/alpha-1-antitrypsin-deficiency/learn-about-alpha-1-antitrypsin-defiency
  8. I-American Lung Association. Izimpawu Zokuntula kwe-Alpha-1 Antitrypsin, Izimbangela kanye Nezici Zengozi. Lung.org. Ishicilelwe ngo-2024. Ifinyelele Novemba 4, 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/alpha-1-antitrypsin-deficiency/symptoms-diagnosis
  9. Izimbangela - Isisekelo se-Alpha-1. I-Alpha-1 Foundation. Ishicilelwe Julayi 13, 2023. Ifinyelele Novemba 4, 2024. https://alpha1.org/about-alpha-1-causes/
  10. I-American Lung Association. Ukushoda kwe-Alpha-1 Antitrypsin: Izimpendulo Zemibuzo emi-5 Evame Ukubuzwa. Lung.org. Ishicilelwe ngo-2023. Ifinyelele Novemba 4, 2024. https://www.lung.org/blog/alpha-1-deficiency-faqs
  11. Attaway A, Majumdar U, Sandhaus RA, Nowacki AS, Stoller JK. Ukuhlaziywa kwezinga lekhonkodensi phakathi kwemihlahlandlela yamazwe ngamazwe mayelana nokushoda kwe-alpha-1 antitrypsin. Ijenali yamazwe ngamazwe ye-COPD. 2019; Umqulu 14:2089-2101. doi:https://doi.org/10.2147/copd.s208591
  12. Izaguirre DE. Izinkombandlela Zokuntula kwe-Alpha1-Antitrypsin (AAT): Isifinyezo Semihlahlandlela. I-Medscape.com. Ishicilelwe ngoMeyi 29, 2024. Ifinyelelwe ngoNovemba 4, 2024. https://emedicine.medscape.com/article/295686-guidelines?form=fpf

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