Ngaba nawe unesifo sezintso esine-polycystic (PCK)? Makhe sithethe ngale nto!

Ngaba wakha weva ngesifo apho iingxowa ezincinci, ezizele lulwelo zenzeka ngaphakathi kwezintso? Mhlawumbi kukho umntu kusapho lwakho onaso, okanye ukhe weva ngaso ndaweni ithile. Le yinto esiyibiza ngokuba yiPolycystic Kidney Disease (PKD) . Eli gama lisenokuvakala lisoyikisa kancinci, kodwa ukwazi okungakumbi ngaso kunokuba nentsingiselo enkulu. Ngoko ke, masithethe ngePKD ngokweenkcukacha namhlanje.

Yintoni i-PKD? Ngamafutshane...

Ngamafutshane, iPolycystic Kidney Disease (PKD) yimeko apho ukhula khona ii-cysts ezininzi ezizaliswe lulwelo, ezifana nama-blisters amancinci, ngaphakathi kwezintso zakho . Yimeko yemfuza . Oku kuthetha ukuba kufuneka ube nejini eguquliweyo emzimbeni wakho ukuze uyivelise. Oku kwahluke kakhulu kwii-kidney cysts, ezihlala zenzeka kwizintso zakho zodwa kwaye zihlala zingenabungozi.

Ezi cysts zenzeka ngenxa ye-PKD zinokukhula kancinci kancinci zize zikhule zombini izintso. Khawucinge nje, ngamanye amaxesha ezi cysts zinokunyusa ubunzima bezintso ukuya kwiikhilogram ezingama-30 (malunga neekhilogram ezili-13.5)! Emva koko, izintso zethu azisenakukwazi ukwenza umsebenzi wazo ophambili wokucoca inkunkuma egazini. Ekuhambeni kwexesha, le meko inokukhokelela kwiSifo sezintso esingapheliyo , esinokubangela ukungasebenzi kakuhle kwezintso ekugqibeleni. Enyanisweni, i-PKD yimbangela yokungasebenzi kakuhle kwezintso malunga ne-2% eMelika. Abantu abaninzi abane-PKD baya kufuna i-dialysis okanye ukufakelwa izintso ngaxa lithile ebomini babo.

Ngoko ke, ukuba ugqirha ukuxilonga ukuba une-PKD, eyona nto ibalulekileyo kukungabi naxhala kodwa kukusebenzisana naye ukuze kwenziwe isicwangciso sonyango esifanelekileyo sokulawula iingxaki ezinokubangelwa sesi sifo.

Ziziphi iintlobo eziphambili ze-PKD?

Kukho iintlobo ezimbini eziphambili ze-PKD. Masifunde kancinci ngazo.

1. Isifo sezintso se-Autosomal Dominant Polycystic (ADPKD)

Olu lolona hlobo luqhelekileyo lwe-PKD. Ixhaphake kakhulu xa umntu ekhulile, phakathi kweminyaka engama-30 nama-50. Nangona kunjalo, ngamanye amaxesha inokuvela ebuntwaneni okanye ebusheni. Ukuze kubekho olu hlobo lwe-PKD, omnye wabazali bakho kufuneka abe notshintsho lwezakhi zofuzo. Abantu abane-ADPKD banolu tshintsho kwiizakhi zofuzo ezibizwa ngokuba yi-`PKD1` okanye `PKD2`.

2. Isifo sezintso se-Autosomal Recessive Polycystic (ARPKD)

Olu luhlobo olungaqhelekanga kakhulu. Lukwabizwa ngokuba yi-infantile PKD . Luvela ngexesha umntwana esesesibelekweni, oko kukuthi, ngexesha lokukhula komntwana ongekazalwa., izintso azikhuli kakuhle. Oogqirha badla ngokubona oku ngexesha lokuhlolwa kwe-ultrasound ngaphambi kokukhulelwa ngexesha lokukhulelwa okanye emva kokuba umntwana ezelwe. Ukuze umntwana abe nolu hlobo lwe-ARPKD, bobabini abazali kufuneka babe notshintsho lwe-gene olubizwa ngokuba yi-`PKHD1`, kwaye umntwana kufuneka azuze i-gene kubo bobabini.

Ixhaphake kangakanani i-PKD?

Izibalo zibonisa ukuba kukho malunga ne-500,000 (500,000) izigulane ezine-PKD eMelika kuphela.

Uhlobo esithethe ngalo ngaphambili, i-ADPKD, luxhaphake kakhulu kune-ARPKD. Malunga nama-90% abantu abane-PKD bane-ADPKD. I-ARPKD sisifo esingaqhelekanga kakhulu. Sichaphazela umntu omnye kwabangama-25,000.

Ziziphi iimpawu eziqhelekileyo ze-PKD?

Iimpawu zinokwahluka ngokuxhomekeke kuhlobo lwe-PKD onayo.

Iimpawu ze-ADPKD

Abantu abane-ADPKD, uhlobo oluqhelekileyo, basenokungabi nazimpawu bade babe ngabantu abadala. Ngamanye amaxesha, ii-cysts ezikhula kwizintso zisenokungabonakali zide zibe nkulu kakhulu. Ukuba iimpawu ziyavela, zingabandakanya:

• Intlungu emqolo okanye ecaleni (ekwabizwa ngokuba yi-"flank pain")
• Igazi elonyukayo
• Intloko ebuhlungu rhoqo
• Igazi kumchamo (iHematuria)
• Usulelo oluqhelekileyo lwendlela yomchamo (UTIs)
• Amatye ezintso

Iimpawu ze-ARPKD

Iintsana ezine-ARPKD, uhlobo olungaqhelekanga, zinokubonisa iimpawu ekuzalweni okanye ngexesha lobuntwana. Ngamanye amaxesha, ugqirha unokubona amaqhuqhuva kwizintso zomntwana ongekazalwa ngexesha le-ultrasound yokukhulelwa.

Ukuba umntwana osandul’ ukuzalwa une-ARPKD, unokufumana iimpawu ezifana nezi:

• Ubunzima obuphantsi bokuzalwa
• Isisu esidumbileyo
• Ukuba noxinzelelo lwegazi oluphezulu ekuzalweni
• Ubunzima bokuphefumla

Ukuba une-ARPKD ebuntwaneni, unokufumana iimpawu ezifana nezi:

• Ukusilela kokukhula (oku kubizwa ngokuba ``ukusilela kokukhula'')
• Usulelo oluqhelekileyo lwendlela yomchamo (UTIs)
• Igazi elonyukayo
• Intlungu esiswini okanye emqolo osezantsi

Ukuba i-ARPKD ikhona kwimveku engekazalwa, iiskeni zingabonisa oku kulandelayo:

• Izintso ezinkulu kuneziqhelekileyo
• Inqanaba eliphantsi lolwelo lwe-amniotic

Ngaba iimpawu ze-PKD zihlala zibonakala ngokukhawuleza?

Hayi, akunjalo. Esi sifo sidla ngokungathethiyo ngokwezonyango, singenazo iimpawu . Oko kuthetha ukuba esi sifo sinokukhula ngaphakathi emzimbeni ngaphandle kweempawu zangaphandle. Khawucinge nje, malunga nesiqingatha sabantu abanale sifo basenokungazi nokuba banaso ebomini babo. Iimpawu zihlala zibonakala emva kokuba ezo blister (ii-cysts) zikhule.

Yintoni ebangela i-PKD?

Njengoko besitshilo ngaphambili, unobangela oyintloko we-PKD kukuguquka kwezakhi zofuzo . Uyazi ukuba izakhi zofuzo zifana nezakhi ezisisiseko zomzimba wethu. Sifumana ezi zakhi zofuzo kubazali bethu. Ezi zakhi zofuzo zimisela indlela umzimba wethu omele ukhule kwaye usebenze ngayo. Ngamanye amaxesha, ngexesha lesigaba somntwana osandul' ukuzalwa, esi sakhi sofuzo siyatshintshatshintsha kwaye asikopishwa kakuhle. Ukuba unezakhi zofuzo ezinjalo, zinokudluliselwa kubantwana bakho. I-PKD idla ngokukhula ngale ndlela.

Nangona kunjalo, kunqabile kakhulu, nokuba abazali bobabini abanalo olu jini, esi sifo sinokuvela ngenxa yotshintsho olungacwangciswanga kwijini.

Ziziphi izinto ezibangela umngcipheko wale meko?

Ngenxa yokuba i-PKD yimeko yemfuza, eyona nto ibangela ukuba ube nayo yimbali yosapho. Oko kukuthi, omnye wabazali bakho unesifo (ingakumbi kwimeko ye-ADPKD) okanye bobabini abazali banesi sifo (kwimeko ye-ARPKD).

Ziziphi iingxaki ezinokubakho ze-PKD?

I-PKD inokubangela iingxaki zempilo ezinzulu kubantu abadala nakwiintsana. Ngamanye amaxesha oko sikubona njengeempawu ze-PKD kunokuba yingxaki. Umzekelo:

• Amatye ezintso
• Igazi elonyukayo
• Usulelo lwendlela yomchamo (UTIs)

Ukongeza, i-PKD inokubangela ezinye iingxaki ezinkulu, ezifana nezi:

• Ukugqabhuka kwemithambo yegazi engqondweni (ebizwa ngokuba yi-"Brain aneurysms")
• Iingxaki zekholoni, umzekelo, i-diverticulitis, imeko apho iingxowa ezincinci zenzeka khona kwikholoni zize zosuleleke
• Iingxaki zevalvu yentliziyo
• Ukungasebenzi kakuhle kwezintso
• Iicysts zesibindi kunye neecysts zepancreatic
• Uxinzelelo lwegazi oluphezulu ngexesha lokukhulelwa (olubizwa ngokuba yi-'Preeclampsia'')

Oku kunokuba yingozi kwiintsana ezine-ARPKD enzima. Inyanga yokuqala yobomi ibaluleke kakhulu kwiintsana ezizelwe zine-ARPKD, njengoko zinokuba neengxaki zokuphefumla ezinzima kunye nokungasebenzi kakuhle kwezintso ngelo xesha.

Ifunyaniswa njani i-PKD?

Ingcali yezifo zezintso (ugqirha ogxile kwizifo zezintso) idla ngokudlala indima ephambili ekuxilongeni nasekunyangeni i-PKD. Uza kujonga ngononophelo iimpawu zakho kunye nembali yosapho lwakho, kwaye angayalela ukuvavanywa kwemifanekiso ukuze kuhlolwe izintso zakho, okufana noku:

• I-ultrasound yezintso: Le yindlela esetyenziswa kakhulu, engenabuhlungu.
• I-ultrasound yangaphambi kokuzalwa: Oku kunceda ekuchongeni ukuba umntwana osesiswini une-PKD na.
• I-CT scan (i-computed tomography scan): Oku kunokuvelisa imifanekiso ecacileyo yezintso kunye nesinyi.
• I-MRI (magnetic resonance imaging): Oku kunceda ekufumaneni imifanekiso eneenkcukacha zezintso.

Ukongeza, ugqirhaUvavanyo lwemfuza nalo lunokucetyiswa. Olu vavanyo lunokujonga ubukho bejini eguquliweyo ebangela i-PKD kwisampulu yegazi okanye yamathe. Oku kunokuqinisekisa nokuba loluphi uhlobo lwe-PKD onayo.

Ziziphi iindlela zonyango ze-PKD?

Enyanisweni, akukho nyango lwe-PKD okwangoku. Unyango lujolise kakhulu ekunciphiseni ukuqhubela phambili kwesi sifo, ukulawula iimpawu, kunye nokuthintela iingxaki. Unyango oluphambili lwe-PKD zezi:

• Ulawulo loxinzelelo lwegazi: Uxinzelelo lwegazi oluphezulu lutshaba olukhulu lwe-PKD. Ngoko ke ugqirha wakho uya kukunceda ulawule uxinzelelo lwakho lwegazi ngamayeza, ukutya okunempilo okunetyuwa encinci, kunye nokuzilolonga. Ukugcina uxinzelelo lwakho lwegazi lukwinqanaba elifanelekileyo kunokunciphisa umngcipheko wokuba nezifo ezinzulu ezifana nesifo sentliziyo kunye nestroke.
• Inkxaso yokuphefumla: Iintsana ezine-ARPKD, ezinemiphunga engakakhuli ngokupheleleyo, kwaye ezinengxaki yokuphefumla zisenokufuna ukuxhaswa yi -mechanical ventilator .
• I-Dialysis: Ukuba izintso zakho azisebenzi kakuhle kwaye azisakwazi ukwenza umsebenzi wazo, kunokufuneka wenze i-"Dialysis" (inkqubo yokucoca igazi lakho ngokwenziwa). Kule nkqubo, umatshini obizwa ngokuba yi-"Hemodialysis" uhluza igazi lakho ngaphandle komzimba wakho. Kwi-"Peritoneal dialysis" uhluza igazi lakho usebenzisa ulwelo olukhethekileyo kunye ne-membrane ebizwa ngokuba yi-peritoneum ejikeleze isisu sakho.
• Unyango lokukhula: Iintsana ezine-ARPKD ezingaphantsi komzimba kwaye ezikhula kancinci zingadinga uncedo lokukhula. Ugqirha unokucebisa unyango olukhethekileyo lwesondlo okanye i-human growth hormone .
• Ukufakelwa kwezintso: Ukuba i-ADPKD iqhubeka nokungasebenzi kakuhle kwezintso , ungadinga ukufakelwa izintso . Ukufakelwa kwezintso yinkqubo yotyando apho izintso ezingasebenzi kakuhle zisuswa khona zize zithatyathelwe indawo yintso ephilileyo evela kumntu onikelayo.
• Ulawulo lweentlungu: Amayeza angasetyenziselwa ukulawula iintlungu ezibangelwa zizifo, amatye ezintso, okanye amaqhuma aqhumayo ngenxa ye-PKD. Nangona kunjalo, naziphi na iintlungu ozithathileyo kufuneka zivunywe ngugqirha wakho . Ezinye iintlungu (ingakumbi ii-NSAID) zinokunyusa umonakalo wezintso.

Ingakanani iminyaka yobomi bomntu one-PKD?

Usenokuziva usoyika kancinci xa usiva oku, kodwa kubalulekile ukwazi inyaniso.

Ukuba abantu abane-ADPKD bafumana unyango olufanelekileyo, balawula esi sifo kakuhle, baze balandele indlela yokuphila esempilweni,Ungaphila ubomi obude nobupheleleyo. Malunga nesiqingatha sabantu abane-ADPKD baya kungasebenzi kakuhle kwezintso xa beneminyaka engama-70 ubudala kwaye baya kufuna i-dialysis okanye ukufakelwa izintso .

Kodwa ingqikelelo yabantwana abane-ARPKD ayilunganga kangako. Malunga nesinye kwisithathu seentsana ezizalwa zine-ARPKD, ingakumbi ezo zineengxaki ezinkulu zokuphefumla, ziyafa ziseseziintsana. Iintsana ezisindayo zihlala zifuna unyango kunye nokhathalelo olukhethekileyo ubomi bazo bonke. Malunga nesiqingatha sabantwana abasindayo emva kokuzalwa baba nokungasebenzi kakuhle kwezintso phakathi kweminyaka eli-15 nengama-20 ubudala.

Ngaba i-PKD ingathintelwa?

Ngelishwa, i-PKD sisifo esibangelwa lufuzo, ngoko ke asinakuthintelwa ngokupheleleyo . Nangona kunjalo, ngokulandela indlela yokuphila enempilo, ukulawula uxinzelelo lwegazi, kunye nokulandela iingcebiso zonyango ngononophelo, ungalibazisa ukuqhubela phambili kwesi sifo okanye ulibazise uphuhliso lweengxaki ezifana nokungasebenzi kakuhle kwezintso.

Kubaluleke kakhulu kubantu abane-PKD abaceba ukuba nabantwana ukuba bathethe nomcebisi wezemfuza ukuze baziswe ngomngcipheko wokuba abantwana babo bafumane esi sifo njengelifa kunye namanyathelo anokuthathwa ukuthintela esi sifo.

Ndingenza ntoni ukuze kube lula ukuphila ne-PKD?

Ukuphila ne-PKD kunokuba nzima, kodwa ngokulandela indlela yokuphila enempilo kunye nokwazi ngesi sifo, ungenza uhambo lube lula. Nazi ezinye iingcebiso zokukunceda:

• Yitya ukutya okulungele izintso . Oku kufanele kugxile ekutyeni okunetyuwa encinci, i-potassium, kunye ne-phosphorus. Fumana ingcebiso ethile kugqirha wakho okanye kwingcali yokutya malunga noku.
• Yenza umthambo okulungeleyo, ubuncinane imizuzu engama-30 ngosuku, uninzi lweentsuku zeveki.
• Gcina ubunzima bomzimba busempilweni.
• Jonga uxinzelelo lwegazi lwakho rhoqo uze ululawule ngokwemiyalelo kagqirha wakho.
• Ukuba utshaya okanye usebenzisa ezinye iimveliso zecuba, yeka ngoko nangoko.
• Ziphephe iziselo ezinxilisayo kangangoko unako.
• Fumana iindlela zokunciphisa uxinzelelo. Izinto ezifana nokucamngca kunye neyoga zinokunceda.
• Sela amanzi amaninzi kunye neziselo ezingenayo i-caffeine (ithi encinci, ikofu) imini yonke. Nangona kunjalo, ukuba ufuna ukunciphisa ulwelo nakweyiphi na inqanaba lesifo sezintso, landela icebiso likagqirha wakho.
• Lala kakuhle ubuncinane iiyure ezisixhenxe ubusuku ngabunye.
• Sela onke amayeza owanikwe ngugqirha wakho ngokuchanekileyo, ngexesha, nangomlinganiselo omiselweyo. Musa ukuyeka ukuthatha naliphi na iyeza ngaphandle kwengcebiso kagqirha.

Ufanele uye nini kugqirha malunga ne-PKD?

Ukuba une-PKD, kufuneka ukuba sele uphantsi kweliso likagqirha. Nangona kunjalo, ukuba ngequbuliso ufumanisa nayiphi na kwezi mpawu , bona ugqirha okanye uye esibhedlele ngoko nangoko :

• Ukuba imilenze yakho, amaqatha, okanye iinyawo zakho zidumba ngequbuliso (oku kubizwa ngokuba yi-edema)
• Ukuba unentlungu eqatha esifubeni okanye uziva uxinezelekile esifubeni sakho
• Ukuba unengxaki yokuphefumla ngequbuliso
• Ukuba awukwazi ukuchama
• Ukuba kukho ukopha okugqithisileyo ngomchamo
• Ukuba ufumana intloko ebuhlungu okanye utshintsho lombono

Ndingayibuza yiphi imibuzo ugqirha wam?

Kuqhelekile ukuba nemibuzo emininzi ngesi sifo. Musa ukoyika ukubuza ugqirha wakho imibuzo efana nale:

• Luhlobo luni lwe-PKD endinalo? (ADPKD okanye ARPKD?)
• Ingakanani ingozi yokuba abantwana bam bafumane esi sifo kum?
• Ngaba kufuneka ndenze naluphi na olunye uvavanyo olukhethekileyo?
• Yeyiphi indlela yonyango engcono kum?
• Zeziphi iinguqu endifanele ndizenze kwindlela enditya ngayo, ingakumbi malunga netyuwa, i-potassium, kunye ne-phosphorus?
• Ngaba kufuneka ndenze naluphi na utshintsho kwindlela yam yokuphila (ukuzilolonga, ukulala, njl.njl.)?
• Ngaba ndingadinga i-dialysis okanye ukufakelwa izintso kwixesha elizayo?
• Zeziphi iziphumo okanye iingxaki ekufuneka ndiziqaphele ngokukodwa?
• Zithini iziphumo ebezingalindelekanga zamayeza endiwasebenzisayo?

Okokugqibela, yintoni ekufuneka uyikhumbule (Umyalezo Wokuya Ekhaya)

Ngoku uyazi ukuba iPolycystic Kidney Disease (PKD) yimeko yemfuza ebangela ukuba kubekho ii-cysts kwizintso. Ezi cysts zinokubangela ukuba izintso zikhule kwaye ziphazamise ukusebenza kwazo. Ihlala ichaphazela abantu abadala, kodwa kunqabile ukuba kubekho uhlobo oluyingozi lwe-PKD ebantwaneni.

Nangona kungekho nyango lwe-PKD, ungakhathazeki. Ngokulawula iimpawu zakho ngokufanelekileyo, ukulandela iingcebiso zikagqirha wakho, nokuphila ubomi obusempilweni, unokuphila kakuhle nale meko. Eyona nto ibalulekileyo kukusebenza ngokusondeleyo nogqirha wakho kwaye ufumane iimvavanyo kunye nonyango olufunekayo. Awuwedwa, kwaye kukho oogqirha, usapho, kunye nabahlobo abanokukunceda kolu hambo.

Isifo sezintso sePolycystic , i-PKD, isifo sezintso, ii-kidney cysts, izifo zemfuza, i-ADPKD, i-ARPKD