Ngaba uqaphele uphawu olukhulu, olubomvu mnyama, olunombala wewayini emzimbeni womntwana wakho xa wayezalwa? Okanye, njengoko umntwana wakho ekhula, ngaba umlenze omnye ubonakala umde kwaye utyebile kunomnye? Ungaphawula nokuba kukho imithambo ejijekileyo. Ukuba umntwana wakho unenye okanye ngaphezulu kwezi mpawu, kubalulekile ukuba uqaphele imeko engaqhelekanga esithetha ngayo namhlanje ebizwa ngokuba yiKlippel-Trenaunay Syndrome (KTS). Nangona isenokuvakala inzima, masiyigcine ilula.
Ngamafutshane, yintoni iKlippel-Trenaunay Syndrome (KTS)?
I-KTS yimeko engaqhelekanga kakhulu yokuzalwa eyenzeka xa umntu ezalwa. Ichaphazela umntu omnye kuphela kwisigidi kwihlabathi liphela. Ngoko ke ayiqhelekanga kangako. Kukho iingxaki ezintathu eziphambili ezinokubonwa kumntu onesi sifo.
1. Uphawu olubomvu eluswini: Oku kukwabizwa ngokuba yi-"Port-wine stain" kuba kufana nombala wewayini encinci.
2. Imithambo yeVaricose: Le yimeko esiyibiza ngokuba yi-"Varicose veins".
3. Ingalo okanye umlenze omnye ukhula ube mkhulu kunomnye: Umlenze omnye ngokukodwa ungakhula ube mde okanye ube mkhulu kunomnye.
Ukongeza kwezi mpawu zintathu ziphambili, abanye abantu banokuba neengxaki kwinkqubo ye-lymphatic, nto leyo enceda ekugcineni ulungelelwano lolwelo emizimbeni yethu.
Oogqirha ngamanye amaxesha bayibiza le nto ngokuthi "i-CLVM." Oku kuthetha ukuba le meko ichaphazela amalungu aliqela omzimba wethu.
- C (iiCapillaries): Le yimithambo yegazi emihle kakhulu edibanisa imithambo yethu kunye nemithambo yegazi. Elo phawu lokuzalwa elibomvu libangelwa kukutshintsha kwezi zinto.
- L (Inkqubo yeLymphatic): Oko kuthetha inkqubo ye-lymphatic. Inxalenye yenkqubo yethu yokuzikhusela komzimba.
- V (Imithambo): Oko kuthetha imithambo yegazi. Imithambo yegazi ethwala igazi ukuya entliziyweni.
- M (Ukukhubazeka): Oku kuthetha ukuba inxalenye ayiphuhlanga kakuhle.
Le meko yaqala ukufunyanwa ngoogqirha ababini baseFransi ngo-1900. Yiyo loo nto esi sifo sibizwa ngegama labo.
Zithini iimpawu zomntu one-KTS?
Makhe sijonge ngokusondeleyo indlela le meko echaphazela ngayo imithambo yegazi, izicubu ezithambileyo, amathambo, kunye nemithambo ye-lymphatic yomntu one-KTS. Ukuze uqonde ezi mpawu ngokucacileyo, jonga itheyibhile engasezantsi.
| Uphawu | Ingcaciso elula |
|---|---|
| Ibala lewayini yePort-wine | Oku kungaba luphawu lokuqala lwe-KTS. Kubangelwa kukudumba kwemithambo yegazi emincinci (ii-capillaries) phantsi kolusu. Ingahluka ngombala ukusuka kupinki okhanyayo ukuya kubomvu obomvu wewayini. Njengoko ukhula, eli bala linokuba mnyama okanye likhanye. Ngamanye amaxesha, kuvela amaqhuqhuva amancinci kuloo ndawo, anokugqabhuka aze aphume igazi. |
| Imithambo yeVaricose | Le meko inokuchaphazela phantse wonke umntu one-KTS. Xa ezi ngxaki zisenzeka kwimithambo ephezu kolusu, imithambo esemilenzeni, ingakumbi emqolo nasemathangeni, iba luhlaza okwesibhakabhaka. Ngamanye amaxesha, inokuchaphazela nemithambo ebaleka nzulu emzimbeni. Ukuba oku kuyenzeka, kukho umngcipheko wokuqhekeka kwegazi (Deep Vein Thrombosis - DVT). |
| Ukukhula okungaqhelekanga kwengalo okanye umlenze | Amaxesha amaninzi, ingalo okanye umlenze omnye kuphela okhula ube mkhulu kunomnye. Oku kunokuqala ebuntwaneni. Kudla ngokuchaphazela umlenze omnye. Umlenze omnye unokuba mde okanye ube mkhulu kunomnye. Oku kunokubangela ubunzima bokuhamba nokuhamba. |
| Iingxaki zenkqubo ye-lymphatic | Abanye abantu banokuba nemithambo ye-lymph engaphezulu okanye engaqhelekanga. Xa le mithambo ingasebenzi kakuhle, inokuvuza ulwelo lwe-lymphatic, ibangele ukudumba emilenzeni, okanye ibangele iingxaki ze-pelvic, ze-bladder, okanye zamathumbu. |
| Intlungu | Imithambo isenokurhawuzelela okanye ibuhlungu. Iingxaki zokujikeleza kwegazi zinokubangela ukuba imilenze idumbe kwaye ibe buhlungu. Kwakhona, imilenze ekhulisiweyo isenokuvakala ngathi inzima okanye ibuhlungu. |
Kutheni le meko isenzeka ngokwenene?
Ixesha elininzi, i-KTS ibangelwa kukutshintsha kwenye yezakhi zofuzo zethu. Ngokuchanekileyo, ibangelwa kukuguquka kwezakhi zofuzo okubizwa ngokuba yi-PIK3CA.
Into ebalulekileyo kukuba oku akuyonto izuzwe ngumama ukuya kumntwana. Olu tshintsho lwemfuza lwenzeka ngamaxesha athile, ngaphandle kwesizathu. Ngoko ke, njengabazali, akukho sizathu sokuba buhlungu okanye uzibeke ityala ngale nto.
Ngamanye amaxesha abantu abangenalo utshintsho kwi-PIK3CA gene nabo bane-KTS, ngoko ke abaphandi bakholelwa ukuba olunye utshintsho kwi-genetic lunokuba luxanduva.
Ziziphi ezinye iingxaki ezinokubakho ngenxa ye-KTS?
Ukuba i-KTS ayiphathwa kakuhle, kukho iingxaki ezithile ezinokubakho, kwaye kubalulekile ukuba uzazi.
- Amahlwili egazi: Amahlwili egazi (i-DVT) anokwenzakala kwimithambo enzulu yemilenze. Eyona ngozi inkulu kukuba ukuba eli hlwili legazi liyaqhekeka lize libhajwe emthanjeni emiphungeni, linokuba yimeko esongela ubomi (i-Pulmonary Embolism).
- Usulelo lwesikhumba: Usulelo lweebhaktheriya oluvela phantsi kwesikhumba (i-cellulitis) lunokwenzeka.
- Ukudumba: Iingxaki kwinkqubo ye-lymphatic zinokubangela ukuba ulwelo luqokelele emzimbeni luze lubangele ukudumba (i-Lymphedema).
- Ukopha ngaphakathi: Ukopha kusenokuvela esiswini, emathunjini, kwisinyi, okanye kubasetyhini, kwinkqubo yokuzala.
Kunqabile kakhulu, abanye abantu abane-KTS banokufumana iziphene zokuzalwa ezibandakanya iminwe okanye iinzwane.
- Ukuba neminwe eyongezelelweyo (i-Polydactyly)
- I-Syndactyly (iminwe idibene)
Ugqirha uyixilonga njani ngokuchanekileyo i-KTS?
Ugqirha uza kuqala ayixilonge le meko ngokujonga inkangeleko yomntwana. Ukuba kukho ubuncinane iimpawu ezimbini kwezi zintathu eziphambili esixoxe ngazo ngaphambili (iimpawu zokuzalwa, imithambo yesigcawu, kunye namalungu amakhulu), kucingelwa ukuba isenokuba yi-KTS. Ekubeni ezi mpawu zihlala zibonakala ekuzalweni, kukho amaxesha apho zinokuxilongwa ngaphambi kokuba umntwana aye ekhaya evela esibhedlele.
Ugqirha angayalela iimvavanyo ezahlukeneyo ukuqinisekisa le meko aze afumanise ukuba yintoni kanye kanye imiphumo ebangwa yile nto emzimbeni.
| Uvavanyo | Ubona ntoni kule nto? |
|---|---|
| I-CT scan okanye i-MRI scan | Jonga imeko yezicubu ezithambileyo zomzimba kunye namathambo, kunye nobungakanani bokukhula okungaqhelekanga. |
| I-MRA (i-Magnetic Resonance Angiogram) | Olu luhlobo olukhethekileyo lwe-MRI scan. Lunokuyibona kakuhle imeko yemithambo yegazi kunye nemithambo yegazi. |
| I-Doppler Ultrasound | Jonga indlela igazi elihamba ngayo ngemithambo yegazi, uze ubone ukuba akukho nto ivalelekileyo okanye ukubuya kwegazi na. |
Ziziphi iindlela zonyango ze-KTS?
Akukho nyango lwe-KTS. Nangona kunjalo, kukho unyango oluninzi olunokunceda abantu ukuba balawule iimpawu zabo kunye neengxaki zabo baze baphile ubomi obuqhelekileyo. Unyango luxhomekeke kwiimpawu zomntu ngamnye.
| Indlela yonyango | Kwenzeka ntoni kuyo? |
|---|---|
| Izinto zokunciphisa igazi (i-Anticoagulants) | Eli yeza linceda ekunciphiseni umngcipheko wokuqhekeka kwegazi. |
| Isiyobisi iSirolimus | Eli yeza licothisa okanye limise ukukhula kwemithambo yegazi engaqhelekanga, ngaloo ndlela linciphisa iimpawu kunye neengxaki. |
| Iisokisi zokucinezelwa | Ezi zinceda ekunciphiseni ukudumba kunye nentlungu emilenzeni, kwaye zinciphise umngcipheko wokuqhekeka kwegazi. Zinceda ukuhamba kwegazi ukusuka emilenzeni ukuya entliziyweni. |
| Iilifti zezihlangu | Ukuba imilenze yakho ayilingani ngobude, ukongeza icandelo eliphezulu kwisihlangu esinye kunokwenza kube lula ukuhamba. Oku kunceda ekunciphiseni umngcipheko we-scoliosis. |
| Unyango lwelaser | Olu nyango lusetyenziselwa ukukhanyisa umbala wophawu lokuzalwa olubizwa ngokuba yi-'port-wine stain' eluswini. |
| Unyango lweSclerotherapy | Ulwelo olukhethekileyo lufakwa kwimithambo enengxaki, oluyivala kwaye luyivale. Olu lunyango olusebenzayo lwemithambo eneentlungu. |
| Utyando | Utyando lunokwenziwa ukulungisa iingxaki ezinkulu zemithambo yegazi, ukulinganisa ubude bomlenze, okanye ukunciphisa ubukhulu bengalo/umlenze okhulisiweyo ngokususa izicubu ezingaphezulu. |
Zeziphi ezinye izinto ekufuneka uzilumkele ngakumbi xa uhlala ne-KTS?
Xa uphila ne-KTS, kubaluleke kakhulu ukuba uwuqonde kakuhle umzimba wakho kwaye unakekele izinto ezithile.
- Lukhathalele kakuhle ulusu lwakho: Ukugcina ulusu lwakho lucocekile kwaye lungenazintlungu kunokunciphisa umngcipheko wosulelo lolusu.
- Hlala usebenza kangangoko unako: Musa ukuhlala kwindawo efanayo ixesha elide. Ukuzilolonga rhoqo nokuhambahamba kunokunciphisa umngcipheko wokuqhekeka kwegazi.
- Nxiba iimpahla zokucinezela njengoko ugqirha wakho ekuyalele: Ukuba ugqirha wakho ukunike umyalelo, soloko unxiba iikawusi ezikhethekileyo okanye iimpahla ezinciphisa ukudumba.
- Ziphephe iindlela ezithile zokulawula ukuzala: Thetha nogqirha wakho malunga nokuphepha izinto ezifana neepilisi zokulawula ukuzala eziqulethe i-hormone ye-estrogen, njengoko zonyusa umngcipheko wokuqhekeka kwegazi.
- Ngexesha lokukhulelwa nangaphambi kotyando: Ngala maxesha, kunokufuneka uthathe amayeza okunciphisa igazi ukuthintela ukuqhekeka kwegazi, njengoko umngcipheko wokuqhekeka kwegazi uphezulu. Thetha nogqirha wakho ngale nto kwaye ufumane ingcebiso.
Ndifanele ndimbone nini ugqirha? Ngawaphi amaxesha okuya kwi-ETU?
Kubalulekile ukuba umntu one-KTS ahlolwe rhoqo ubomi bakhe bonke. Kulapho imeko inokujongwa khona, unyango lunokubonwa lusebenza, kwaye naluphi na utshintsho olufunekayo lunokwenziwa.
Nangona kunjalo, ngamanye amaxesha kunokufuneka uye esibhedlele xa kukho imeko engxamisekileyo. Qaphela ezi mpawu.
Nazi iimeko apho kufuneka uye kwi-ETU (iYunithi yoNyango oluNgxamisekileyo) ngokukhawuleza:
* Ukuba ubona iimpawu zokuqhekeka kwegazi: Ukuba umlenze omnye ngequbuliso uba buhlungu, udumbe, ube bomvu, kwaye uzive ufudumele xa uwuchukumisa, isenokuba yi-deep vein thrombosis (DVT).
* Ukuba uneempawu zokuqhekeka kwegazi emiphungeni: Iintlungu zesifuba ngequbuliso, ubunzima bokuphefumla, ukuphefumla ngokukhawuleza, okanye ukukhohlela igazi, isenokuba yi-pulmonary embolism. Le yimeko engxamisekileyo.
* Ukuba kukho ukopha okukhulu: Ukuba kukho ukopha okungalawulekiyo okuvela naphi na emzimbeni.
Ukuba ubona naziphi na kwezi mpawu, musa ukuchitha ixesha kwaye yiya kwiSebe lezeMpilo eziNgxamisekileyo (ETU) lesibhedlele esikufutshane ngoko nangoko.
I-KTS yimeko enzima, ngoko unokuba nemibuzo emininzi. Ngalo lonke ixesha ubona ugqirha wakho, mamela yonke into esengqondweni yakho. Musa ukucinga ukuba, "Le yingxaki endingafanele ndiyijongane nayo." Kungcono ukuba uthethe ngokukhululekileyo nangokunyaniseka nogqirha wakho.
Umyalezo Wokuya Ekhaya
- I-Klippel-Trenaunay Syndrome (KTS) sisifo esingaqhelekanga sokuzalwa esingabangelwanga yimpazamo yabazali.
- Iimpawu ezintathu eziphambili zezi: uphawu lokuzalwa olubomvu obumnyama (i-port-wine stain), imithambo erhabaxa, kunye nengalo okanye umlenze omnye omkhulu kunomnye.
- Nangona le meko ingenakunyangwa ngokupheleleyo, kukho iindlela ezininzi zonyango ezinokukunceda ulawule iimpawu zakho kwaye uphile ubomi obuhle kakhulu.
- Iingxaki ezibalulekileyo ekufuneka uziqaphele ziingxaki zokuqhekeka kwegazi (i-DVT, i-Pulmonary Embolism) kunye nokopha kakhulu. Hlala uqaphela iimpawu ezilumkisayo zezi.
- Kubaluleke kakhulu ukuhlala unxibelelana nogqirha wakho ubomi bakho bonke kwaye uye rhoqo kwiimvavanyo.
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