Yonke into ofuna ukuyifumana malunga ne-ADPKD

Umxholo

• Iimpawu ze-ADPKD
• Unyango lwe-ADPKD
• Iziphumo ebezingalindelekanga zonyango lwe-ADPKD
• Uvavanyo lwe-ADPKD
• Ukuchongwa kwe-ADPKD
• Oonobangela be-ADPKD
• Iingxaki
• Ukulindela ubomi kunye nembono

Isifo sezintso esine-Autosomal esilawulayo (ADPKD) sisifo esinganyangekiyo esenza ukuba ii-cysts zikhule kwizintso.

I-National Institute of Diabetes kunye neDigestive and Kidney Diseases inika ingxelo yokuba ichaphazela umntu oqikelelwa kubantu abangama-400 ukuya ku-1 000.

Funda ukuze ufunde ngakumbi malunga nayo:

• iimpawu
• oonobangela
• unyango

Iimpawu ze-ADPKD

I-ADPKD inokubangela iimpawu ezahlukeneyo, kubandakanya:

• intloko ebuhlungu
• iintlungu emqolo
• iintlungu emacaleni enu
• igazi kumchamo wakho
• ukwanda kwesisu
• imvakalelo yokugcwala esiswini sakho

Iimpawu zihlala zikhula ebudaleni, phakathi kweminyaka engama-30 ukuya kwengama-40 ubudala, nangona zinokubonakala zikwiminyaka yokuqhubela phambili. Ngamanye amaxesha, iimpawu zivela ebuntwaneni okanye ebusheni.

Iimpawu zale meko zihlala zisiba mandundu ngokuhamba kwexesha.

Unyango lwe-ADPKD

Akukho nonyango eyaziwayo ye-ADPKD. Nangona kunjalo, unyango lukhona ukunceda ukulawula isifo kunye neengxaki ezinokubakho.

Ukunceda ukucothisa ukukhula kwe-ADPKD, ugqirha wakho unokumisela i-tolvaptan (Jynarque).

Yiyo kuphela amayeza avunyiweyo yi-Food and Drug Administration (FDA) ngokukodwa ukunyanga i-ADPKD. La mayeza anokunceda ukulibaziseka okanye ukuthintela ukusilela kwezintso.

Ngokuxhomekeke kwimeko ethile kunye neemfuno zonyango, ugqirha wakho unokudibanisa enye okanye ngaphezulu kwezi zilandelayo kwisicwangciso sakho sonyango:

• utshintsho kwindlela yokuphila ukunceda ukunciphisa uxinzelelo lwegazi kunye nokukhuthaza impilo yezintso
• amayeza okunceda ukunciphisa uxinzelelo lwegazi, ukunciphisa iintlungu, okanye ukunyanga usulelo olunokwenzeka kwizintso, kumchamo, okanye kwezinye iindawo
• utyando lokususa amaqhakuva abangela iintlungu
• ukusela amanzi imini yonke kunye nokuthintela i-caffeine ukunciphisa ukukhula kwama-cysts (abaphandi bafunda indlela i-hydration echaphazela ngayo i-ADPKD)
• ukutya iinxalenye ezincinci zeprotein esemgangathweni
• ukunciphisa umda ityuwa, okanye isodiyam, ekutyeni kwakho
• ukunqanda i-potassium eninzi kunye nefosforasi ekudleni kwakho
• ukunciphisa ukusela utywala

Ukulawula i-ADPKD kunye nokunamathela kwisicwangciso sakho sonyango kunokuba ngumceli mngeni, kodwa kubalulekile ekunciphiseni ukuqhubela phambili kwesi sifo.

Ukuba ugqirha wakho umisela i-tolvaptan (Jynarque), kuyakufuneka ukuba ube neemvavanyo rhoqo ukuvavanya impilo yesibindi sakho kuba amayeza anokubangela ukonakala kwesibindi.

Ugqirha wakho uya kujonga ngononophelo impilo yezintso zakho ukuze abone ukuba imeko izinzile okanye iyaqhubeka.

Ukuba uhlakulela ukungaphumeleli kwezintso, kuya kufuneka ufumane i-dialysis okanye ukufakelwa kwezintso ukubuyisela ukulahleka komsebenzi wezintso.

Thetha nogqirha wakho ukuze ufunde okungakumbi malunga nonyango lwakho, kubandakanya izibonelelo ezinokubakho, umngcipheko kunye neendleko zendlela ezahlukeneyo zonyango.

Iziphumo ebezingalindelekanga zonyango lwe-ADPKD

Uninzi lwamayeza anokuqwalaselwa ngugqirha wakho ukunceda ukunyanga okanye ukulawula i-ADPKD ithwala umngcipheko weziphumo ebezingalindelekanga.

Umzekelo, iJynarque inokubangela ukunxanwa okugqithisileyo, ukuchama rhoqo, kwaye kwezinye iimeko, umonakalo omkhulu wesibindi. Kukho iingxelo zokungaphumeleli kwesibindi okufuna ukufakelwa isibindi kubantu abathatha iJynarque.

Olunye unyango olujolise kwiimpawu ezithile ze-ADPKD lunokubangela iziphumo ebezingalindelekanga. Ukufunda ngakumbi malunga neziphumo ebezingalindelekanga zonyango, thetha nogqirha wakho.

Ukuba ucinga ukuba unokuba neziphumo ebezingalindelekanga kunyango, yazisa ugqirha wakho ngoko nangoko. Banokucebisa utshintsho kwisicwangciso sakho sonyango.

Ugqirha wakho kunokwenzeka ukuba a-odole uvavanyo oluqhelekileyo ngelixa usenziwa unyango oluthile ukujonga iimpawu zomonakalo wesibindi okanye ezinye iziphumo ebezingalindelekanga.

Uvavanyo lwe-ADPKD

Isifo sezintso sePolycystic (PKD) sisifo esingofuzo.

Uvavanyo lwe-DNA luyafumaneka, kwaye zimbini iintlobo ezahlukeneyo zovavanyo:

• Uvavanyo lokudibana kweGene. Olu vavanyo luhlalutya amanqaku athile kwi-DNA yamalungu osapho ane-PKD. Ifuna iisampulu zegazi ezivela kuwe kunye namalungu osapho aliqela achaphazelekayo kwaye angachaphazeleki yi-PKD.
  
• Uhlalutyo lokutshintsha ngokuthe ngqo / ukulandelelana kweDNA. Olu vavanyo lufuna isampulu enye kuphela evela kuwe. Ihlalutya ngokuthe ngqo iDNA yohlobo lwe-PKD.

Ukuchongwa kwe-ADPKD

Ukufumanisa i-ADPKD, ugqirha wakho uya kukubuza malunga:

• iimpawu zakho
• imbali yezonyango
• Imbali yonyango yosapho

Banoku-odola i-ultrasound okanye olunye uvavanyo lokujonga ukuba ujonge ii-cysts kunye nezinye izinto ezinokubangela iimpawu zakho.

Banokuyalela ukuvavanywa kwemfuza ukuze bafunde ukuba unenguquko yemfuza ebangela i-ADPKD. Ukuba une-gene echaphazelekayo kwaye unabantwana, banokubakhuthaza ukuba bafumane uvavanyo lwezofuzo ngokunjalo.

Oonobangela be-ADPKD

I-ADPKD yimeko yofuzo.

Kwiimeko ezininzi, iziphumo zivela kuguquko lwe-PKD1 gene okanye i-PKD2 yemfuza.

Ukuphuhlisa i-ADPKD, umntu kufuneka abe nekopi enye yohlobo oluchaphazelekayo. Bahlala belifa lemfuza elichaphazelekayo komnye wabazali babo, kodwa kwiimeko ezinqabileyo, utshintsho lwofuzo olunokwenzeka ngokuzenzekelayo.

Ukuba unayo i-ADPKD kwaye iqabane lakho alinalo kwaye uthatha isigqibo sokuqalisa usapho kunye, abantwana bakho banokuba nethuba leepesenti ezingama-50 zokuphuhlisa esi sifo.

Iingxaki

Le meko ikubeka emngciphekweni weengxaki, ezinje:

• igazi elonyukayo
• usulelo lomchamo
• cysts kwisibindi sakho okanye injeke
• Iintsholongwane zentliziyo ezingaqhelekanga
• I-aneurysm yengqondo
• ukusilela kwezintso

Ukulindela ubomi kunye nembono

Ukulindelwa kobomi bakho kunye nembonakalo ye-ADPKD kuxhomekeke kwizinto ezininzi, kubandakanya:

• utshintsho oluthile lwemfuza olubangela i-ADPKD
• naziphi na iingxaki ozivelisayo
• unyango olufumanayo kunye nendlela onamathela ngayo kwisicwangciso sakho sonyango
• impilo yakho yonke kunye nendlela ophila ngayo

Thetha nogqirha wakho ukuze ufunde okungakumbi malunga nemeko kunye nembono yakho. Xa i-ADPKD ifunyaniswa kwangoko kwaye ilawulwa ngokufanelekileyo, abantu banokukwazi ukugcina ubomi obupheleleyo, obusebenzayo.

Umzekelo, abantu abaninzi abane-ADPKD abasasebenza xa befunyanisiwe bayakwazi ukuqhubeka nemisebenzi yabo.

Ukuziqhelanisa nemikhwa esempilweni kunye nokulandela isicwangciso sonyango esinconyelwe ngugqirha kunokunceda ukuthintela iingxaki kunye nokugcina izintso zakho zisempilweni ixesha elide.