Yintoni ukusilela kwe-Alpha-1 antitrypsin?

Ukunqongophala kwe-Alpha-1 ye-antitrypsin, edla ngokubizwa ngokuba yi-Alpha -1, yimeko yemfuza ezuzwe njengelifa enokuthi idluliselwe kubazali ukuya kubantwana babo. Abantu abane-Alpha-1 banamazinga aphantsi eprotheyini ebizwa ngokuba yi-AAT, ekhusela imiphunga. EYurophu naseUnited States, abantu abamalunga ne-100,000 1 kwilizwe ngalinye bachatshazelwa yiAlpha-XNUMX.

I-Alpha-1 inokubangela i-emphysema (iingxowa zomoya emiphungeni yakho zonakele), i-cirrhosis yesibindi (i-scarring esibindini), kunye ne-panniculitis (imeko yesikhumba enqabileyo enokwenza ulusu lube lukhuni, lubuhlungu, lubomvu, okanye lube mnyama kwezinye iindawo).

Iya iba mandundu ngokuhamba kwexesha. Nangona lungekho unyango lwe-Alpha-1, unyango lunokuzinceda ezinye iimpawu. Uninzi lwabantu abane-Alpha-1 banokuphila ubomi obuqhelekileyo, ngakumbi ukuba abatshayi.

Iimpawu zemiphunga: Iimpawu zemiphunga zihlala zijongeka njengezo zesifo esinganyangekiyo semiphunga (COPD), njengale:

  • Ukukhohlela okungapheliyo kunye nephlegm
  • Ukugubha
  • Ukuqhawukelwa ngumphefumlo
  • Izifuba eziphindaphindiweyo

Iimpawu zesibindi:

  • Ulusu kunye namehlo ajika abe tyheli (jaundice)
  • Ukukhathala
  • Ukudumba kwesisu

Ngamanye amaxesha, abantu banokuba nemeko yesikhumba engaqhelekanga, i-panniculitis. Isenokubonakala ngathi ngamaqhuma abuhlungu, abomvu anokujikeleza umzimba wakho kwaye anogqabhuka kwaye avuze ubovu okanye ulwelo.

Imiphunga yabantu abane-Alpha-1 inokonakala ngenxa yokuba igazi labo alinayo iprotheni ebizwa ngokuba yi-alpha-1 antitrypsin (AAT). Loo proteni yenziwa esibindini kwaye ikhusela imiphunga ekudumbeni xa ichanabeke kumsi wecuba okanye ezinye izinto ezicaphukisayo. Ngaphandle kolu khuseleko kwimiphunga, inokonakala emva kokuphefumla izinto ezicaphukisayo.

Isibindi sabantu abane-Alpha-1 singonakala xa i-AAT ingenako ukukhululwa esibindini
izinga rhoqo. Inokwakheka esibindini kwaye ibangele isifo sesibindi.

I-Alpha-1 ixhaphake kakhulu phakathi kwabantu abanomnombo wosapho waseYurophu okanye eMntla Melika. Kodwa olunye uhlanga okanye amaqela ezizwe nawo anokufumana i-Alpha-1. Abantu abanokuthi baphuhlise i-Alpha-1 ngabo banabazali abanamazinga aphantsi eprotheni ye-AAT.

Xa umntu ene-Alpha-1, izinto ezithile zinokunyusa umngcipheko wokuba nesifo semiphunga.

  • ukutshaya
  • Ukuba sesichengeni kumsi wecuba
  • Ukuba nembali yosapho ye-emphysema okanye i-asthma
  • Ukuba nosulelo oluphindaphindiweyo lwemiphunga
  • Ukuhlala okanye ukusebenza kwiindawo ezinothuli

Thetha nogqirha wakho malunga novavanyo ukuba ucinga ukuba une-Alpha-1, uneempawu, okanye ukuba wena okanye amalungu osapho lwakho anembali yesibindi okanye isifo semiphunga. Ugqirha wakho unokwenza uvavanyo lwegazi ukujonga inqanaba leprotheyini ye-AAT egazini lakho kunye novavanyo lofuzo ukuchonga ukuguqulwa kofuzo.

Iimvavanyo zesiqhelo zemiphunga (PFTs) ziyacetyiswa ukujonga ukusebenza kwemiphunga. Uvavanyo lomsebenzi wesibindi kunye nezifundo zokucinga zinokuba yimfuneko ukuvavanya impilo yesibindi.

Unyango loNyango:

  • Igazi elinikelwe elinamanqanaba aqhelekileyo e-Alpha-1 unokunikwa ngu-IV ukunyusa amanqanaba egazini nakwimiphunga, ejolise ekuthobeni isifo semiphunga. Oku ikakhulu kubantu abane-emphysema ngenxa ye-Alpha-1.

Unyango lweempawu:

  • I-Bronchodilators kunye ne-corticosteroids e-inhaled: isetyenziselwa ukulawula iimpawu zemiphunga kunye nokuphucula ukusebenza kwemiphunga.
  • Unyango lweoksijini: lunokuba yimfuneko kubantu abaneoksijini ephantsi egazini labo.

Utshintsho kwindlela yokuphila:

  • Ukuyeka ukutshaya: ukutshaya kukhawulezisa umonakalo kwimiphunga yakho. Kubalulekile ukuqhubeka uzama ukuyeka ukutshaya, nokuba awuzange uphumelele ngaphambili.
  • Ugonyo: Ugonyo loMkhuhlane (umkhuhlane) kunye ne-pneumococcal (inyumoniya) luyacetyiswa ukuthintela usulelo lwemiphunga.
    Funda nzulu Apha malunga namandla okugonya kubantu abanesifo esingapheliyo sokuphefumla.
  • Ukuvuselelwa kwemiphunga kunokukunceda ukuba uphile kwaye uphefumle ngcono. Yinkqubo yezonyango ephantsi kweliso enokukunceda ukuba ufumane amandla, yenze kube lula ukusebenza okanye ukuya kwimisebenzi yentlalo, kwaye unciphise ukudakumba kunye nexhala.

Ulawulo lweSifo sesibindi:

  • Ugqirha wakho uya kufuna ukujonga kunye nawe rhoqo ukujonga iingxaki zesibindi. Kwiimeko eziphambili, ukutshintshwa kwesibindi kunokuqwalaselwa.

Unyango olutsha kunye noPhando:

  • Unyango lwemfuza kunye nokhetho lwamachiza olutsha luyaphandwa njengonyango olunokwenzeka lwexesha elizayo lwe-alpha-1.

Musa ukoyika ukubuza ugqirha wakho imibuzo. Ukuthetha nogqirha wakho kunye nokuqonda iAlpha-1 kunokukunceda ufumane olona khathalelo lungcono. Nantsi eminye imibuzo onokuyibuza ugqirha wakho, kodwa qiniseka ukuba ubuza imibuzo ngayo nantoni na ongayiqondiyo.

  • Ngaba kufuneka ndivavanye i-Alpha-1?
  • Zeziphi iindlela endinazo zonyango?
  • Zeziphi ezinye iimeko zempilo ekufuneka ndazi ngazo?
  • Ngaba ndingaqhubeka ndisebenza ndize ndibandakanyeke kwizinto endizithandayo?
  • Ndinokuyeka njani ukutshaya?
  • Ingaba kufuneka ndicwangcise nini utyelelo lukagqirha olulandelayo?
  • Ngaba amalungu osapho lwam kufuneka afumane uvavanyo lwegazi lwe-AAT?
  • Ndingathetha njani namalungu entsapho yakowethu ngeAlpha-1?

Awunako ukuthintela i-Alpha-1 kuba sisifo sofuzo. Kodwa, unokuwuthoba umngcipheko wokufumana isifo sesibindi okanye semiphunga ukuba une-Alpha-1.

  • Musa ukutshaya okanye vape.
  • Gwema utywala.
  • Yiphephe nayiphi na into enokucaphukisa imiphunga yakho, njengemichiza, uthuli, kunye nezinye izinto eziphazamisayo.
  • Zilolonge rhoqo.
  • Yitya ukutya okusempilweni.

Resources

ISikhokelo seNgxoxo seSifo sesibindi se-Alpha-1 (IsiNgesi)
Isikhokelo seNgxoxo se-Alpha-1 yeSifo sesibindi (ngesiFrentshi)
ISikhokelo seNgxoxo seSifo sesibindi se-Alpha-1 (esiJamani)
Isikhokelo sokuxoxa ngeSifo sesibindi se-Alpha-1 (isiPhuthukezi)

Ndingazifumana phi ezinye?

Isiseko se-Alpha-1
Umbutho waseMelika weLung
IAlpha-1 yaseYurophu iManyano
Isiseko semiphunga yaseYurophu
Alpha-1 Association of Australia

Ubhaliso

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

Iimpawu:

  1. Yintoni uAlpha-1? Isiseko se-Alpha-1. Ifikeleleke ngo-Okthobha 14, 2024. https://alpha1.org/what-is-alpha1/
  2. Ukunqongophala kwe-Alpha-1 Antitrypsin-Isebenza Njani (ividiyo). Isiseko se-Alpha-1. Ifikeleleke nge-14 ka-Okthobha ngo-2024. https://vimeo.com/247506044
  3. Ukunqongophala kwe-Alpha-1 Antitrypsin. Iklinikhi yaseCleveland. Ifikeleleke ngo-Okthobha 14, 2024.
    https://my.clevelandclinic.org/health/diseases/21175-alpha-1-antitrypsin-deficiency
  4. Uvavanyo lweDermatopathology lwePanniculitis. Iziko leSizwe loLwazi lwe-Biotechnology, iThala leeNcwadi leSizwe leMithi, amaZiko ezeMpilo eSizwe. Ifikeleleke nge-14 ka-Okthobha ngo-2024. https://www.ncbi.nlm.nih.gov/books/NBK606121/
  5. Ukuvuselelwa kwemiphunga. Intliziyo yeSizwe, iZiko lemiphunga kunye neGazi. Ifikeleleke nge-14 ka-Okthobha ngo-2024. https://www.nhlbi.nih.gov/health/pulmonary-rehabilitation
  6. Ukunqongophala kwe-Alpha-1 Antitrypsin - Iimpawu kunye nezizathu. Pennmedicine.org. Ipapashwe ngo-2022. Ifikeleleke ngoNovemba 4, 2024. https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/alpha-1-antitrypsin-deficiency
  7. Umbutho wamaLung waseMelika. Funda Ngokunqongophala kwe-Alpha-1 Antitrypsin. Lung.org. Ipapashwe ngo-2024 Ifikeleleke ngoNovemba 4, 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/alpha-1-antitrypsin-deficiency/learn-about-alpha-1-antitrypsin-defiency
  8. Umbutho wamaLung waseMelika. Iimpawu zokunqongophala kwe-Alpha-1 ye-Antitrypsin, oonobangela kunye neMicimbi yoMngcipheko. Lung.org. Ipapashwe ngo-2024. Ifikeleleke ngoNovemba 4, 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/alpha-1-antitrypsin-deficiency/symptoms-diagnosis
  9. Oonobangela – Alpha-1 Foundation. Isiseko se-Alpha-1. Ipapashwe ngoJulayi 13, 2023. Ifikeleleke ngoNovemba 4, 2024. https://alpha1.org/about-alpha-1-causes/
  10. Umbutho wamaLung waseMelika. Ukunqongophala kwe-Alpha-1 ye-Antitrypsin: Iimpendulo kwiMibuzo emi-5 eQhwa ukubuzwa. Lung.org. Ipapashwe ngo-2023. Ifikeleleke ngoNovemba 4, 2024. https://www.lung.org/blog/alpha-1-deficiency-faqs
  11. Attaway A, Majumdar U, Sandhaus RA, Nowacki AS, Stoller JK. Uhlalutyo lweqondo lokuvumelana phakathi kwezikhokelo zamazwe ngamazwe malunga nokunqongophala kwe-alpha-1 ye-antitrypsin. Ijenali yeHlabathi yeCOPD. 2019; Umqulu 14:2089-2101. doi:https://doi.org/10.2147/copd.s208591
  12. Izaguirre DE. I-Alpha1-Antitrypsin (AAT) Izikhokelo zokuSilela: Isishwankathelo sezikhokelo. Medscape.com. Ipapashwe ngoMeyi 29, 2024. Ifikeleleke ngoNovemba 4, 2024. https://emedicine.medscape.com/article/295686-guidelines?form=fpf

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