Ingabe nawe unesifo sezinso se-polycystic (PCK)? Ake sixoxe ngalokhu!

Wake wezwa ngesifo lapho kwakheka khona amasaka amancane agcwele uketshezi ngaphakathi kwezinso? Mhlawumbe othile emndenini wakho unaso, noma uke wezwa ngaso ndawana thize. Lokhu yilokho esikubiza ngokuthi i-Polycystic Kidney Disease (PKD) . Igama lingase lizwakale liyesabisa kancane, kodwa ukwazi okwengeziwe ngaso kungenza umqondo omkhulu. Ngakho-ke, ake sixoxe nge-PKD ngokuningiliziwe namuhla.

Kuyini i-PKD? Kalula nje...

Kalula nje, i-Polycystic Kidney Disease (PKD) yisimo lapho ukhula khona ama-cyst amaningi agcwele uketshezi, afana nama-blister amancane, ngaphakathi kwezinso zakho . Kuyisimo sofuzo . Lokhu kusho ukuthi kumele ube nesakhi sofuzo esiguquliwe emzimbeni wakho ukuze usikhule. Lokhu kuhluke kakhulu kuma-cyst ezinso, avame ukwakheka ezinso zakho zodwa futhi ngokuvamile awayona ingozi.

Lawa ma-cyst akhiwa ngenxa ye-PKD angakhula kancane kancane futhi andise izinso zombili. Cabanga nje, ngezinye izikhathi lawa ma-cyst angandisa isisindo sezinso sibe amakhilogremu angu-30 (cishe amakhilogremu angu-13.5)! Ngemuva kwalokho, izinso zethu azisakwazi ukwenza umsebenzi wazo oyinhloko wokuhlunga imfucuza egazini. Ngokuhamba kwesikhathi, lesi simo singakhula sibe yiSifo Sezinso Esingapheli , okungagcina kuholele ekuhlulekeni kwezinso . Eqinisweni, i-PKD iyimbangela cishe yama-2% okwehluleka kwezinso e-United States. Abantu abaningi abane-PKD bazodinga i-dialysis noma ukufakelwa izinso ngesikhathi esithile empilweni yabo.

Ngakho-ke, uma udokotela ekuxilonga ukuthi une-PKD, into ebaluleke kakhulu ukungathuki kodwa ukusebenzisana naye ukuze kwakheke uhlelo oluhle lokwelapha ukuze kulawulwe izinkinga ezingase zivele ngenxa yalesi sifo.

Yiziphi izinhlobo eziyinhloko ze-PKD?

Kunezinhlobo ezimbili eziyinhloko ze-PKD. Ake sifunde kancane ngazo.

1. Isifo Sezinso Esivame Ukutholakala Esikhumbeni Se-Autosomal (ADPKD)

Lolu uhlobo oluvame kakhulu lwe-PKD. Luvame ukutholakala uma umuntu esemdala, phakathi kweminyaka engama-30 no-50. Kodwa-ke, ngezinye izikhathi lungabonakala ebuntwaneni noma ebusheni. Ukuze athuthukise lolu hlobo lwe-PKD, omunye wabazali bakho kumele abe nokuguqulwa kwezakhi zofuzo. Abantu abane-ADPKD banalolu guquko ku-genes olubizwa ngokuthi `PKD1` noma `PKD2`.

2. Isifo Sezinso Esiphindaphindayo Esibangelwa Yi-Autosomal Recessive Polycystic (ARPKD)

Lolu uhlobo olungavamile kakhulu. Lubizwa nangokuthi i-infantile PKD . Luvela ngesikhathi umntwana esesesibelethweni, okungukuthi, ngesikhathi sokukhula kwengane., izinso azikhuli kahle. Odokotela bavame ukukubona lokhu ngesikhathi sokuhlolwa kwe-ultrasound ngaphambi kokukhulelwa ngesikhathi sokukhulelwa noma ngemva kokuzalwa komntwana. Ukuze ingane ibe nalolu hlobo lwe-ARPKD, bobabili abazali kumele babe nokuguqulwa kwezakhi zofuzo okubizwa ngokuthi `PKHD1`, futhi ingane kumele izuze ifa lazo zombili izakhi zofuzo.

Ivame kangakanani i-PKD?

Izibalo zibonisa ukuthi kuneziguli ze-PKD ezingaba ngu-500,000 (500,000) e-United States kuphela.

Uhlobo esikhulume ngalo ngaphambili, i-ADPKD, luvame kakhulu kune-ARPKD. Cishe abantu abangu-90% abane-PKD bane-ADPKD. I-ARPKD iyisimo esingavamile kakhulu. Ithinta cishe umuntu oyedwa kwabangu-25,000.

Yiziphi izimpawu ezivamile ze-PKD?

Izimpawu zingahluka kuye ngohlobo lwe-PKD onayo.

Izimpawu ze-ADPKD

Abantu abane-ADPKD, uhlobo oluvame kakhulu, bangase bangabi nazimpawu baze babe badala. Ngezinye izikhathi, ama-cyst akha ezinso angase angabonakali aze abe makhulu kakhulu. Uma kuvela izimpawu, zingase zihlanganise:

• Ubuhlungu emhlane noma ohlangothini (obizwa nangokuthi "ubuhlungu besifuba")
• Umfutho wegazi ophakeme
• Ubuhlungu bekhanda obuvamile
• Igazi emchameni (i-Hematuria)
• Ukutheleleka okuvamile komgudu womchamo (UTIs)
• Amatshe ezinso

Izimpawu ze-ARPKD

Izingane ezine-ARPKD, uhlobo olungavamile, zingase zibonise izimpawu lapho zizalwa noma ngesikhathi sezinsana. Ngezinye izikhathi, udokotela angase abone ama-cyst ezinso zomntwana ngesikhathi sokuhlolwa kwe-ultrasound ngaphambi kokubeletha.

Uma umntwana osanda kuzalwa ene-ARPKD, angase abe nezimpawu ezifana nalezi:

• Isisindo esiphansi sokuzalwa
• Isisu esivuvukile
• Ukuba nomfutho wegazi ophezulu ngesikhathi sokuzalwa
• Ubunzima bokuphefumula

Uma une-ARPKD ebuntwaneni, ungase ube nezimpawu ezifana nalezi:

• Ukwehluleka kokukhula (lokhu kubizwa ngokuthi "ukwehluleka kokukhula")
• Ukutheleleka okuvamile komgudu womchamo (UTIs)
• Umfutho wegazi ophakeme
• Ubuhlungu esiswini noma emhlane ongezansi

Uma i-ARPKD ikhona embungwini, amaskeni angabonisa okulandelayo:

• Izinso ezinkulu kunezivamile
• Izinga eliphansi loketshezi lwe-amniotic

Ingabe izimpawu ze-PKD zihlala zivela ngokushesha?

Cha, akunjalo. Lesi sifo sivame ukungakhulumi ngokwezokwelapha, ngaphandle kwezimpawu . Lokho kusho ukuthi lesi sifo singakhula ngaphakathi emzimbeni ngaphandle kwezimpawu zangaphandle. Cabanga nje, cishe ingxenye yabantu abanalesi sifo kungenzeka bangazi nokuthi banaso ngesikhathi sokuphila kwabo. Izimpawu zivame ukuvela ngemva kokuba lawo maqhubu anamanzi (ama-cyst) esekhulile.

Yini ebangela i-PKD?

Njengoba sishilo ngaphambili, imbangela eyinhloko ye-PKD ukuguquka kwezakhi zofuzo . Uyazi ukuthi izakhi zofuzo zifana nezakhi zomzimba wethu eziyisisekelo. Sithola lezi zakhi zofuzo kubazali bethu. Lezi zakhi zofuzo zinquma ukuthi imizimba yethu kufanele ikhule futhi isebenze kanjani. Ngezinye izikhathi, ngesikhathi sokukhulelwa, lesi sakhi sofuzo siyashintsha futhi asikopishwa kahle. Uma unezakhi zofuzo ezinjalo, singadluliselwa nasezinganeni zakho. I-PKD ivame ukukhula ngale ndlela.

Kodwa-ke, akuvamile kakhulu, noma ngabe bobabili abazali abanalo leli jini, lesi sifo singakhula ngenxa yokuguquka okungahleliwe kwejini.

Yiziphi izinto ezibangela lesi simo?

Ngenxa yokuthi i-PKD iyisifo esibangelwa ufuzo, isici esiyinhloko esiyingozi sokuba ube naso umlando womndeni. Okusho ukuthi, omunye wabazali bakho unesifo (ikakhulukazi uma une-ADPKD) noma bobabili abazali bangabathwali baso (uma une-ARPKD).

Yiziphi izinkinga ezingaba khona ze-PKD?

I-PKD ingabangela izinkinga ezinkulu zempilo kubantu abadala kanye nezingane ezincane. Ngezinye izikhathi lokho esikubona njengezimpawu ze-PKD kungaba yizinkinga. Isibonelo:

• Amatshe ezinso
• Umfutho wegazi ophakeme
• Ukutheleleka komgudu womchamo (UTIs)

Ngaphezu kwalokho, i-PKD ingabangela ezinye izinkinga ezinkulu, njenge:

• Ukuqhuma kwemithambo yegazi ebuchosheni (okubizwa ngokuthi "ama-Brain aneurysms")
• Izinkinga zekholoni, isibonelo, i-diverticulitis, isimo lapho kwakheka khona izikhwama ezincane emakholoni bese zitheleleka
• Izinkinga ze-valve yenhliziyo
• Ukwehluleka kwezinso
• Ama-cyst esibindi kanye nama-cyst e-pancreatic
• Umfutho wegazi ophezulu ngesikhathi sokukhulelwa (obizwa ngokuthi "i-Preeclampsia")

Lokhu kungaba yingozi ezinganeni ezine-ARPKD enzima. Inyanga yokuqala yokuphila ibaluleke kakhulu ezinganeni ezizalwa zine-ARPKD, njengoba zingaba nokucindezeleka okukhulu kokuphefumula kanye nokwehluleka kwezinso phakathi naleso sikhathi.

Itholakala kanjani i-PKD?

Udokotela wezinso (udokotela ogxile ezifweni zezinso) uvame ukudlala indima ebalulekile ekuxilongeni nasekwelapheni i-PKD. Uzohlola ngokucophelela izimpawu zakho kanye nomlando womndeni, futhi angase acele ukuhlolwa kwezithombe ukuze kuhlolwe izinso zakho, njengokuthi:

• I-ultrasound yezinso: Lena yindlela evame ukusetshenziswa kakhulu futhi engenabuhlungu.
• I-ultrasound yangaphambi kokubeletha: Lokhu kusiza ekuboneni ukuthi ingane esibelethweni ine-PKD.
• I-CT scan (i-computed tomography scan): Lokhu kungaveza izithombe ezicacile zezinso kanye nesinye.
• I-MRI (i-magnetic resonance imaging): Lokhu kusiza futhi ukuthola izithombe ezinemininingwane zezinso.

Ngaphezu kwalokho, udokotelaUkuhlolwa kofuzo nakho kunganconywa. Lokhu kuhlolwa kungahlola ukuba khona kofuzo oluguquliwe olubangela i-PKD egazini noma esampula yamathe. Lokhu kungaqinisekisa nokuthi uluhlobo luni lwe-PKD onalo.

Yiziphi izindlela zokwelapha i-PKD?

Eqinisweni, alikho ikhambi le-PKD okwamanje. Ukwelashwa kuhloswe kakhulu ekunciphiseni ukuqhubeka kwesifo, ukulawula izimpawu, nokuvimbela izinkinga. Ukwelashwa okuyinhloko kwe-PKD yile:

• Ukuphathwa komfutho wegazi: Umfutho wegazi ophakeme uyisitha esikhulu se-PKD. Ngakho udokotela wakho uzokusiza ukulawula umfutho wegazi lakho ngemithi, ukudla okunempilo okunosawoti omncane, kanye nokuzivocavoca. Ukugcina umfutho wegazi lakho usezingeni eliphephile kunganciphisa ingozi yakho yokuthola izifo ezingathi sína njengesifo senhliziyo kanye nesifo sohlangothi.
• Ukusekelwa kokuphefumula: Izingane ezine-ARPKD, amaphaphu azo angakakhuli ngokugcwele, futhi ezinenkinga yokuphefumula zingadinga ukusekelwa umshini wokuphefumula wemishini .
• I-Dialysis: Uma izinso zakho zingasebenzi kahle futhi zingasakwazi ukwenza umsebenzi wazo, kungase kudingeke ukuthi wenze i-"Dialysis" (inqubo yokuhlanza igazi lakho ngokwenziwa). Kule nqubo, umshini obizwa ngokuthi i-"Hemodialysis" uhlunga igazi lakho ngaphandle komzimba wakho. Ku-"Peritoneal dialysis" uhlunga igazi lakho usebenzisa uketshezi olukhethekile kanye ne-membrane ebizwa ngokuthi i-peritoneum emboze isisu sakho.
• Ukwelashwa Kokukhula: Izingane ezine-ARPKD ezingaphansi kwesisindo futhi ezikhule ngokungakhuli kahle zingadinga usizo lokukhula. Udokotela angase ancome ukwelashwa okukhethekile okunomsoco noma i-hormone yokukhula komuntu .
• Ukufakelwa kwezinso: Uma i-ADPKD iqhubeka nokwehluleka kwezinso , ungase udinge ukufakelwa izinso . Ukufakelwa izinso inqubo yokuhlinzwa lapho kususwa khona izinso ezingasebenzi kahle bese kufakwa izinso eziphilile ezivela kumnikeli.
• Ukulawulwa kobuhlungu: Imithi ingasetshenziswa ukulawula ubuhlungu obubangelwa ukutheleleka, amatshe ezinso, noma ama-cyst aqhuma ngenxa ye-PKD. Kodwa-ke, noma yimiphi imithi yobuhlungu oyithathayo kufanele ivunyelwe udokotela wakho . Amanye ama-painkiller (ikakhulukazi ama-NSAID) angandisa ukulimala kwezinso.

Ingakanani iminyaka yokuphila komuntu one-PKD?

Ungase uzizwe wesaba kancane uma uzwa lokhu, kodwa kubalulekile ukwazi iqiniso.

Uma abantu abane-ADPKD bethola ukwelashwa okufanele, bephatha lesi sifo kahle, futhi belandela indlela yokuphila enempilo,Ungaphila impilo ende negcwele. Cishe ingxenye yabantu abane-ADPKD bazoqala ukwehluleka kwezinso lapho beneminyaka engu-70 futhi bazodinga i-dialysis noma ukufakelwa izinso .

Kodwa isibikezelo sezingane ezine-ARPKD asisihle kangako. Cishe ingxenye yesithathu yezingane ezizalwa zine-ARPKD, ikakhulukazi lezo ezinezinkinga ezinkulu zokuphefumula, ziyafa ziseyizinsana. Izingane ezisindayo zivame ukudinga ukwelashwa nokunakekelwa okukhethekile kukho konke ukuphila kwazo. Cishe ingxenye yezingane ezisindayo ngemva kokuzalwa ziba nokwehluleka kwezinso phakathi kweminyaka eyi-15 nengama-20.

Ingabe i-PKD ingavinjelwa?

Ngeshwa, i-PKD iyisifo esibangelwa ufuzo, ngakho-ke ayinakuvinjelwa ngokuphelele . Kodwa-ke, ngokulandela indlela yokuphila enempilo, ukulawula umfutho wegazi, nokulandela iseluleko sezokwelapha ngokucophelela, unganciphisa ijubane lokuqhubeka kwalesi sifo noma ubambezele ukukhula kwezinkinga ezifana nokwehluleka kwezinso.

Kubaluleke kakhulu ukuthi abantu abane-PKD abahlela ukuba nezingane bakhulume nomeluleki wezakhi zofuzo ukuze baziswe ngengozi yokuthi izingane zabo zizuze lesi sifo kanye nezinyathelo ezingathathwa ukuze zisivimbele.

Yini engingayenza ukuze ngiphile ne-PKD kube lula?

Ukuphila ne-PKD kungaba yinselele, kodwa ngokulandela indlela yokuphila enempilo nokuqaphela lesi sifo, ungenza uhambo lube lula. Nazi ezinye izeluleko zokukusiza:

• Yidla ukudla okulungele izinso . Lokhu kufanele kugxile ekudleni okunosawoti omncane, i-potassium, ne-phosphorus. Thola iseluleko esiqondile kudokotela wakho noma kuchwepheshe wezokudla ngalokhu.
• Zivocavoce ngendlela ekufanele, okungenani imizuzu engama-30 ngosuku, izinsuku eziningi zesonto.
• Gcina isisindo somzimba siphilile.
• Hlola umfutho wegazi lakho njalo bese uwulawula ngokwemiyalelo kadokotela wakho.
• Uma ubhema noma usebenzisa eminye imikhiqizo kagwayi, yeka ngokushesha.
• Gwema iziphuzo ezidakayo ngangokunokwenzeka.
• Thola izindlela zokunciphisa ukucindezeleka. Izinto ezifana nokuzindla kanye ne-yoga zingasiza.
• Phuza amanzi amaningi kanye neziphuzo ezingenayo i-caffeine (itiye elincane, ikhofi) usuku lonke. Kodwa-ke, uma udinga ukunciphisa uketshezi kunoma yisiphi isigaba sesifo sezinso, landela iseluleko sikadokotela wakho.
• Lala kahle okungenani amahora ayisikhombisa njalo ebusuku.
• Thatha yonke imithi oyinikezwe udokotela wakho ngqo, ngesikhathi, nangesilinganiso osinqunyiwe. Ungayeki ukuthatha noma yimuphi umuthi ngaphandle kweseluleko sikadokotela.

Kufanele ubonane nini nodokotela mayelana ne-PKD?

Uma une-PKD, kufanele ukuthi usuvele ungaphansi kokuqashwa ngudokotela. Kodwa-ke, uma kwenzeka ngokuzumayo noma yiziphi zalezi zimpawu , bona udokotela noma uye esibhedlela ngokushesha :

• Uma imilenze yakho, amaqakala, noma izinyawo zakho zivuvukala ngokuzumayo (lokhu kubizwa ngokuthi i-edema)
• Uma unezinhlungu zesifuba ezinzima noma umuzwa wokuqina esifubeni sakho
• Uma unobunzima bokuphefumula ngokuzumayo
• Uma ungakwazi ukuchama
• Uma kukhona ukopha ngokweqile ngomchamo
• Uma uzwa ikhanda elibuhlungu noma ushintsho lombono

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Kuvamile ukuba nemibuzo eminingi ngalesi sifo. Ungesabi ukubuza udokotela wakho imibuzo efana nale:

• Uhlobo luni lwe-PKD enginalo? (i-ADPKD noma i-ARPKD?)
• Iyini ingozi yokuthi izingane zami zizuze lesi sifo kimi?
• Ingabe ngidinga ukwenza ezinye izivivinyo ezikhethekile?
• Iyiphi indlela yokwelapha engcono kakhulu kimi?
• Yiziphi izinguquko okufanele ngizenze ekudleni kwami, ikakhulukazi mayelana nosawoti, i-potassium, ne-phosphorus?
• Ingabe kudingeka ngenze noma yiziphi izinguquko endleleni yami yokuphila (ukuzivocavoca umzimba, ukulala, njll.)?
• Ngingadinga yini i-dialysis noma ukufakelwa izinso esikhathini esizayo?
• Yimiphi imiphumela emibi noma izinkinga okufanele ngiziqaphele ngokukhethekile?
• Yimiphi imiphumela emibi yemithi engiyithathayo?

Okokugcina, okufanele ukukhumbule (Umyalezo Wokuya Ekhaya)

Manje usuyazi ukuthi i-Polycystic Kidney Disease (PKD) yisimo sofuzo esibangela ukwakheka kwama-cyst ezinso. Lawa ma-cyst angabangela ukuthi izinso zikhule futhi ziphazamise ukusebenza kwazo. Ngokuvamile kuthinta abantu abadala, kodwa akuvamile ukuba kube nohlobo oluyingozi lwe-PKD ezinganeni.

Nakuba kungekho ikhambi le-PKD, ungakhathazeki. Ngokuphatha izimpawu zakho ngendlela efanele, ukulandela iseluleko sikadokotela wakho, nokuphila impilo enempilo, ungaphila kahle nalesi simo. Into ebaluleke kakhulu ukusebenzisana eduze nodokotela wakho futhi uthole izivivinyo kanye nokwelashwa okudingayo. Awuwedwa, futhi kunodokotela, umndeni, nabangani abangakusiza kulolu hambo.

Isifo Sezinso Esinama - Polycystic, i-PKD, isifo sezinso, ama-cyst ezinso, izifo zofuzo, i-ADPKD, i-ARPKD