Ngaba uhlala uphuma igazi empumlweni? Okanye ngaba unamabala amancinci abomvu eluswini lwakho, ingakumbi ebusweni bakho, emilebeni, nakwiiminwe? Mhlawumbi umama wakho, utata wakho, okanye omnye umntu kusapho lwakho ukhe waba nezi ngxaki. Sisenokungazinaki kakhulu ezi zinto, sithi, "Owu, sisizukulwana sethu nje eso." Kodwa, ngamanye amaxesha kunokubakho isizathu sezonyango emva koku esifanele sisazi. Namhlanje sithetha ngemeko engaqhelekanga kodwa ebaluleke kakhulu ekufuneka siyiqaphele. Leyo yi-HHT, okanye i-Hereditary Hemorrhagic Telangiectasia .
Kalula nje, yintoni i-HHT?
I-HHT yimeko yemfuza edluliselwa kwizizukulwana ngezizukulwana. Ichaphazela kakhulu indlela imithambo yegazi yethu, okanye imithambo yegazi, eyakheka ngayo. Cinga ngenkqubo yemithambo yegazi emzimbeni wethu njengothungelwano lweendlela. Kukho imithambo yegazi, njengeendlela ezinkulu, kunye nemithambo yegazi, njengeendlela ezinkulu. Iindlela ezincinci, ezincinci ezisecaleni ezidibanisa ezi ndlela zimbini zinkulu zibizwa ngokuba zii-capillaries.
Okwenzekayo kumntu one-HHT kukuba olu nxibelelwano lubuthathaka olubizwa ngokuba zii-capillaries alwenziwanga kakuhle. Endaweni yoko, kukho unxibelelwano oluthe ngqo, olungaqhelekanga, nolubuthathaka phakathi kwemithambo yegazi kunye nemithambo yegazi. Kwizonyango, olu nxibelelwano silubiza ngokuba zii-Arteriovenous Malformations (AVMs) .
Ezi AVM zinokukhula nakweyiphi na ilungu emzimbeni wethu, umzekelo empumlweni, emiphungeni, emathunjini, okanye nasebuchotsheni. I-AVM encinci kakhulu ekhula phezu kolusu ibizwa ngokuba yiTelangiectasia . La ngamabala amancinci abomvu avela ngaphakathi kolusu lwakho, njengemilebe. Le mithambo yegazi ebuthathaka ingaqhuma ngokulula. Xa iqhuma kwaye yopha, inokubangela neemeko ezinzulu, kuxhomekeke kwindawo ekuyo.
Into ebalulekileyo kukuba abantu abaninzi abane-HHT baphila iminyaka emininzi bengazi. Nangona kungekho nyango, kukho unyango oluninzi olusebenzayo olufumanekayo namhlanje lokulawula iimpawu nokuthintela izifo ezinzulu.
Esi sifo sichaphazela umntu omnye kwabayi-5,000 kwihlabathi liphela. Kodwa ngenxa yokuba abantu abaninzi bengafumani sigulo, kukholelwa ukuba banokubakho abaguli abaninzi. Sinokwenzeka nakubantu balo naliphi na ixesha kunye nohlanga. Sikwabizwa ngokuba yi -Osler-Weber-Rendu syndrome .
Ziziphi iimpawu eziphambili zesifo se-HHT?
Iimpawu ze-HHT zinokwahluka kumntu nomntu. Kuxhomekeke ekubeni iphi emzimbeni imithambo yegazi engaqhelekanga (ii-AVM) esithethe ngazo ngaphambili. Abanye abantu basenokungabi nazo iimpawu ezibonakalayo. Kodwa abanye banokuba neempawu ezinzulu kakhulu.
Masizihlele ezi mpawu kwitheyibhile engezantsi.
| Uhlobo lwempawu | Ingcaciso kunye nemizekelo |
|---|---|
| Iimpawu eziqhelekileyo kubantu abaninzi |
|
| Iimpawu zokopha ngaphakathi | |
| Iimpawu ezibangelwa yi-AVM kwizitho eziphambili ezifana nemiphunga nengqondo | |
| Iingxaki ezingaqhelekanga kodwa ezinzima |
Kutheni i-HHT isenzeka?
Oku kuxhomekeke kwimfuza ngokupheleleyo.Ibangelwa yinto ethile. Oko kukuthi, ayisosifo esosulelayo. Yinto ezuzwe ngabazali ukuya ebantwaneni. I-HHT sisifo esibangelwa yi-gene elawulayo (isifo esilawulayo). Oko kuthetha ukuba, ukuba omnye wabazali bakho wayenaso esi sifo, nawe unethuba lama-50% lokufumana esi sifo.
I-HHT ibangelwa kukuguquka kwezakhi zofuzo ezimbini (i-`ENG` gene kunye ne-`ACVRL1` gene), kwaye izazinzulu zisaphanda oku.
Ukuba une-HHT, kubaluleke kakhulu ukuthetha nogqirha wakho malunga nokuba abantwana bakho baya kuyizuza njengelifa.
Ifunyaniswa njani i-HHT?
Ugqirha unokukukrokrela oku emva kokuva ngeempawu zakho kunye nembali yosapho lwakho. Nangona uvavanyo lwemfuza lunokwenziwa ukuqinisekisa ukuxilongwa, ukuxilongwa kudla ngokwenziwa ngokusekelwe kwiimpawu.
Ugqirha wakho uya kucinga ukuba une-HHT ukuba unezi zinto zintathu kwezi zilandelayo:
- Ukopha impumlo okuphindaphindiweyo.
- Ukubakho kwee -telangiectasias ezininzi kwiindawo zesikhumba apho kudla ngokuvela khona amabala (ubuso, imilebe, iminwe).
- Ukuba ne-AVM okanye i-Telangiectasia kwisitho esingaphakathi emzimbeni (njengemiphunga, ingqondo, isibindi).
- Ukuba nelungu losapho elisondeleyo (umama, utata, umntakwenu) one-HHT.
Uvavanyo lokuqinisekisa isifo
Ugqirha wakho unokucebisa ukuba wenze iimvavanyo ezithile, ezifana nezi:
- I-Ultrasound Scan: Oku kunceda ukubona ukuba kukho ii-AVM esibindini.
- I-MRI (Magnetic Resonance Imaging) Scan: Iluncedo kakhulu, ingakumbi ekujongeni ii-AVM engqondweni.
- I-CT (Computed Tomography) Scan: Inokufumana imifanekiso ecacileyo yezitho zangaphakathi zomzimba.
- Uvavanyo lweBubble (Echocardiogram): Olu luvavanyo olukhethekileyo. Lusetyenziselwa ukufumanisa ukuba kukho ii-AVM emiphungeni.
Ziziphi iindlela zonyango ze-HHT?
Into yokuqala ekufuneka uyikhumbule kukuba akukho nyango lwe-HHT. Nangona kunjalo, kukho unyango oluninzi olusebenzayo olunokunceda ukulawula iimpawu, ukunciphisa umngcipheko weengxaki ezinkulu, kunye nokukunceda uphile ubomi obuqhelekileyo.
Ngelixa unyanga iimpawu zakho zangoku, ugqirha wakho uya kujonga naziphi na ii-AVM ezifihlakeleyo ezisenokuba azinazo iimpawu okwangoku.
Nazi ezinye iindlela zonyango:
- Xa uphuma igazi rhoqo: Sebenzisa amafutha okugcoba kunye ne-saline spray ukuze ugcine impumlo imanzi.
- Kwi-Anemia: Nika iipilisi zokutshintsha isinyithi okanye, ukuba kuyimfuneko, utofelo-gazi.
- Ukuyeka ukopha: Unyango lwe-laser (`Ablation`) lutshabalalisa imithambo yegazi emincinci ephuma igazi.
- Ukufakwa kwegazi emzimbeni: Oku kuquka ukuvala umthambo wegazi okhokelela kwi-AVM esengozini yokopha okanye ukuqhuma yinto ethile ekhethekileyo.
- Utyando okanye unyango lwemitha: Ezi zisetyenziselwa ukususa okanye ukunciphisa ezinye ii-AVM.
- Ukuyeka amayeza athile: Amayeza anjenge-aspirin anciphisa ukujiya kwegazi angadinga ukuyekiswa ngokwengcebiso kagqirha.
Kubaluleke kakhulu: Ukuba une-HHT, ingakumbi ukuba uyazi ukuba une-AVM emiphungeni yakho, kusenokufuneka uthathe amayeza okubulala iintsholongwane ngaphambi kotyando lwamazinyo, njengokukhupha amazinyo. Oku kwenzelwa ukuthintela usulelo olufana namathumba ebuchotsheni. Qiniseka ukuba uthetha nogqirha wakho ngale nto.
Izinto ekufuneka uziqwalasele xa uhlala ne-HHT
Umntu one-HHT unokufuna ukujongwa nonyango ubomi bakhe bonke, kodwa ngonyango olufanelekileyo, abaguli abane-HHT banobomi obuqhelekileyo.
Yintoni enokwenziwa ukuthintela ukopha empumlweni?
- Thetha nogqirha wakho kwaye uphephe amayeza anyusa ukopha (umz. i-aspirin, ii-NSAIDs).
- Gcina impumlo yakho imanzi ngamaxesha onke. Ukusebenzisa isixhobo sokufumisa umswakama ekhaya, ukusebenzisa izitshizi zempumlo ezinetyuwa, kunye nokusebenzisa amafutha amiselwe ngugqirha wakho kubaluleke kakhulu.
- Gcina idayari ukuze ubone ukuba ukutya okanye imisebenzi ethile iyakwandisa na umngcipheko wakho wokopha empumlweni.
Ukuba ufunyaniswe une-HHT, kubaluleke kakhulu ukucebisa bonke abantu bosapho lwakho ukuba nabo baye kuvavanywa, kuba okukhona isifo sifunyanwa ngokukhawuleza, kokukhona iingxaki ezinkulu zinokuthintelwa.
Umyalezo Wokuya Ekhaya
- I-HHT yimeko yemfuza ebangela ubuthathaka kwimithambo yegazi.
- Iimpawu eziphambili kukuphuma kwegazi rhoqo kunye namabala abomvu (telangiectasias) eluswini, emilebeni, nakwiminwe.
- Ukuba wena okanye umntu osapho lwakho unezi mpawu, kubaluleke kakhulu ukuthetha nogqirha ngazo.
- Nangona kungekho nyango lupheleleyo lwesi sifo, kukho unyango olusebenzayo olunokulawula iimpawu nokuthintela iingxaki ezinzulu (ezifana nesifo sohlangothi kunye nokopha kakhulu).
- Ngokufunyaniswa kwangoko kunye nonyango olufanelekileyo, ungaphila ubomi obuqhelekileyo nobusempilweni.
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