Uke wabona uphawu olukhulu, olubomvu olumnyama, olunombala wewayini emzimbeni wengane yakho lapho izalwa? Noma, njengoba ingane yakho ikhula, ingabe umlenze owodwa ubonakala umude futhi ujiyile kunomunye? Ungase uqaphele nokuthi kunemithambo egobile. Uma ingane yakho inesinye noma ngaphezulu salezi zimpawu, kubalulekile ukuthi uqaphele isimo esingavamile esikhuluma ngaso namuhla esibizwa ngokuthi i-Klippel-Trenaunay Syndrome (KTS). Nakuba kungase kuzwakale kuyinkimbinkimbi, masikwenze kube lula.
Kalula nje, iyini i-Klippel-Trenaunay Syndrome (KTS)?
I-KTS yisimo esingavamile kakhulu sokuzalwa esenzeka lapho umuntu ezalwa. Sithinta cishe umuntu oyedwa kwabayisigidi emhlabeni jikelele. Ngakho-ke asivamile kakhulu. Kunezinkinga ezintathu eziyinhloko ezingabonakala kumuntu onalesi simo.
1. Uphawu lokuzalwa olubomvu esikhumbeni: Lokhu kubizwa nangokuthi "ibala lewayini le-Port" ngoba lifana nombala wewayini elincane.
2. Imithambo ye-Varicose: Lesi yisimo esisibiza ngokuthi ``imithambo ye-Varicose''.
3. Ingalo noma umlenze owodwa okhula ube mkhulu kunomunye: Umlenze owodwa ikakhulukazi ungakhula ube mude noma ube mkhulu kunomunye.
Ngaphezu kwalezi zimpawu ezintathu eziyinhloko, abanye abantu bangase babe nezinkinga ohlelweni lwe-lymphatic, okusiza ukugcina ibhalansi yoketshezi emizimbeni yethu.
Odokotela ngezinye izikhathi bakubiza lokhu ngokuthi "i-CLVM." Lokhu kusho ukuthi lesi simo sithinta izingxenye eziningana zomzimba wethu.
- C (Ama-Capillaries): Lena yimithambo yegazi emincane kakhulu exhumanisa imithambo yethu nemithambo yegazi. Lolo phawu olubomvu lubangelwa izinguquko kulezi.
- L (Uhlelo lwe-lymphatic): Lokho kusho uhlelo lwe-lymphatic. Ingxenye yesimiso sethu sokuzivikela komzimba.
- V (Imithambo): Lokho kusho imithambo yegazi. Imithambo yegazi ethwala igazi iye enhliziyweni.
- M (Ukukhubazeka): Lokhu kusho ukuthi ingxenye ayizange ikhule ngendlela evamile.
Lesi simo saqala ukutholwa odokotela ababili baseFrance ngo-1900. Yingakho lesi sifo siqanjwe ngabo.
Yiziphi izimpawu zomuntu one-KTS?
Ake sibheke kabanzi ukuthi lesi simo sithinta kanjani imithambo yegazi, izicubu ezithambile, amathambo, kanye nemithambo ye-lymphatic yomuntu one-KTS. Ukuze uqonde lezi zimpawu ngokucacile, bheka ithebula elingezansi.
| Isibonakaliso | Incazelo elula |
|---|---|
| Ibala lewayini le-Port-wine | Lokhu kungaba uphawu lokuqala lwe-KTS. Kubangelwa ukuvuvukala kwemithambo yegazi emincane (ama-capillary) ngaphansi kwesikhumba. Kungahluka ngombala kusukela kopinki okhanyayo kuya kobomvu bewayini obumnyama. Njengoba ukhula, leli bala lingase libe mnyama noma likhanye. Ngezinye izikhathi, kuvela amabhamuza amancane endaweni, angaqhuma futhi aphume igazi. |
| Imithambo ye-Varicose | Lesi simo singathinta cishe wonke umuntu one-KTS. Uma lezi zinkinga zenzeka emithanjeni esebusweni besikhumba, imithambo esemilenzeni, ikakhulukazi emathangeni nasemabeleni, iba nombala oluhlaza okwesibhakabhaka. Ngezinye izikhathi, ingathinta nemithambo egijima ngaphakathi emzimbeni. Uma lokhu kwenzeka, kunengozi yokuqhekeka kwegazi (Deep Vein Thrombosis - DVT). |
| Ukukhula okungajwayelekile kwengalo noma umlenze | Ngokuvamile, ingalo noma umlenze owodwa kuphela okhula ube mkhulu kunomunye. Lokhu kungaqala ebuntwaneni. Ngokuvamile kuthinta umlenze owodwa. Umlenze owodwa ungase ube mude noma ube mkhulu kunomunye. Lokhu kungabangela ubunzima bokuhamba nokunyakaza. |
| Izinkinga zesistimu ye-lymphatic | Abanye abantu bangase babe nemithambo ye-lymph eyengeziwe noma engajwayelekile. Uma le mithambo ingasebenzi kahle, ingavuza uketshezi lwe-lymphatic, ibangele ukuvuvukala emilenzeni, noma ibangele izinkinga ze-pelvic, esinyeni, noma zamathumbu. |
| Ubuhlungu | Imithambo ingase ilume noma ibuhlungu. Izinkinga zokujikeleza kwegazi zingabangela imilenze ukuba ivuvuke futhi ibe buhlungu. Futhi, imilenze ekhulisiwe ingase izwakale isinda noma ibuhlungu. |
Kungani lesi simo senzeka ngempela?
Esikhathini esiningi, i-KTS ibangelwa ushintsho kwenye yezakhi zethu zofuzo. Uma sikhuluma iqiniso, ibangelwa ukuguquka kwezakhi zofuzo okubizwa nge-PIK3CA.
Into ebalulekile ukuthi lokhu akuyona into ezuzwe njengefa kumama kuya enganeni. Lokhu kuguquka kwezakhi zofuzo kwenzeka ngezikhathi ezithile, ngaphandle kwesizathu. Ngakho-ke, njengabazali, asikho isizathu sokuzizwa udabuke noma uzisole ngalokhu.
Ngezinye izikhathi abantu abangenalo ushintsho ku-PIK3CA gene nabo bane-KTS, ngakho abacwaningi bakholelwa ukuthi ezinye izinguquko zofuzo zingaba yimbangela.
Yiziphi ezinye izinkinga ezingaba khona ngenxa ye-KTS?
Uma i-KTS ingaphathwa kahle, kungase kube nezinkinga ezithile, futhi kubalulekile ukuzazi.
- Amahlule egazi: Amahlule egazi (i-DVT) angakheka emithanjeni ejulile yemilenze. Ingozi enkulu ukuthi uma leli hlule legazi liqhuma bese libhajwa emthanjeni emaphashini, kungaba yisimo esisongela ukuphila (i-Pulmonary Embolism).
- Ukutheleleka kwesikhumba: Ukutheleleka ngamagciwane okwenzeka ngaphansi kwesikhumba (i-cellulitis) kungenzeka.
- Ukuvuvukala: Izinkinga ngohlelo lwe-lymphatic zingabangela uketshezi ukuthi luqoqeke emzimbeni futhi lubangele ukuvuvukala (i-Lymphedema).
- Ukopha kwangaphakathi: Ukopha kungase kwenzeke esiswini, emathunjini, esinyeni, noma kwabesifazane, ohlelweni lokuzala.
Akuvamile kakhulu, abanye abantu abane-KTS bangase babe nezinkinga zokuzalwa ezihilela iminwe noma izinzwane.
- Ukuba neminwe eyengeziwe (i-Polydactyly)
- I-Syndactyly (iminwe ihlanganiswe ndawonye)
Udokotela uyixilonga kanjani i-KTS ngokunembile?
Udokotela uzoqala ngokuxilonga lesi simo ngokubheka ukubukeka komntwana ngokomzimba. Uma okungenani kunezimpawu ezimbili kwezithathu eziyinhloko esixoxe ngazo ngaphambili (izimpaphe zokuzalwa, imithambo yesicabucabu, kanye nezitho ezikhulisiwe), kusolwa ukuthi kungenzeka ukuthi yi-KTS. Njengoba lezi zimpawu zivame ukubonakala lapho kuzalwa, kunezikhathi lapho zingaxilongwa khona ngaphambi kokuba umntwana aye ekhaya evela esibhedlela.
Udokotela angase acele ukuhlolwa okuhlukahlukene ukuqinisekisa lesi simo nokuthola ukuthi yimiphi imiphumela emzimbeni.
| Ukuhlolwa | Ubonani kulokhu? |
|---|---|
| I-CT scan noma i-MRI scan | Hlola isimo sezicubu namathambo athambile omzimba, kanye nokukhula okungavamile. |
| I-MRA (i-Magnetic Resonance Angiogram) | Lolu uhlobo olukhethekile lwe-MRI scan. Lungabona kahle isimo semithambo yegazi nemithambo yegazi. |
| I-Doppler Ultrasound | Bheka indlela igazi eligeleza ngayo emithanjeni nasemithanjeni, bese ubona ukuthi kukhona yini ukuvaleka noma ukugeleza kwegazi okubangelwa yigazi. |
Yiziphi izindlela zokwelapha i-KTS?
Akukho ukwelashwa kwe-KTS. Kodwa-ke, kunezindlela eziningi zokwelapha ezingasiza abantu ukuthi baphathe izimpawu nezinkinga zabo futhi baphile impilo evamile. Ukwelashwa kuncike ezimpawini zomuntu ngamunye.
| Indlela yokwelapha | Kwenzekani kuyo? |
|---|---|
| Ama-anticoagulant (ama-anticoagulant) | Lo muthi usiza ekunciphiseni ingozi yokuqhekeka kwegazi. |
| Umuthi iSirolimus | Lo muthi wehlisa noma umisa ukukhula kwemithambo yegazi engajwayelekile, ngaleyo ndlela unciphise izimpawu nezinkinga. |
| Amasokisi Okucindezela | Lokhu kusiza ekunciphiseni ukuvuvukala nobuhlungu emilenzeni, futhi kunciphisa ingozi yokuqhekeka kwegazi. Kusiza ukugeleza kwegazi kusuka emilenzeni kuya enhliziyweni. |
| Ama-lift ezicathulo | Uma imilenze yakho ingalingani ngobude, ukufaka ingxenye ephezulu esicathulweni esisodwa kungenza ukuhamba kube lula. Lokhu kusiza futhi ukunciphisa ingozi ye-scoliosis. |
| Ukwelashwa nge-laser | Lokhu kwelashwa kusetshenziselwa ukukhanyisa umbala wophawu lokuzalwa 'lwe-port-wine stain' esikhumbeni. |
| Ukwelashwa nge-Sclerotherapy | Kufakwa uketshezi olukhethekile emithanjeni enenkinga, oluyivala futhi luyivale. Lokhu kuyindlela yokwelapha ephumelelayo yemithambo ekhuphukile. |
| Ukuhlinzwa | Ukuhlinzwa kungenziwa ukuze kulungiswe izinkinga ezinkulu zemithambo yegazi, kulinganiswe ubude bomlenze, noma kuncishiswe usayizi wengalo/umlenze okhulisiwe ngokususa izicubu ezisele. |
Yiziphi ezinye izinto okufanele uziqaphele kakhulu uma uphila ne-KTS?
Uma uphila ne-KTS, kubaluleke kakhulu ukuqonda kahle umzimba wakho nokunakekela izinto ezithile.
- Nakekela isikhumba sakho kahle: Ukugcina isikhumba sakho sihlanzekile futhi singenazo izingozi kunganciphisa ingozi yokutheleleka kwesikhumba.
- Hlala usebenza ngangokunokwenzeka: Ungahlali endaweni efanayo isikhathi eside kakhulu. Ukuzivocavoca njalo nokuhamba kunganciphisa ingozi yokuqhekeka kwegazi.
- Gqoka izingubo zokucindezela njengoba kuqondiswe udokotela wakho: Uma udokotela wakho ekuyalele, gqoka njalo amasokisi noma izingubo ezikhethekile ezinciphisa ukuvuvukala.
- Gwema izindlela ezithile zokuvimbela inzalo: Khuluma nodokotela wakho ngokugwema izinto ezifana namaphilisi okuvimbela inzalo aqukethe i-hormone i-estrogen, njengoba andisa ingozi yokuqhekeka kwegazi.
- Ngesikhathi sokukhulelwa nangaphambi kokuhlinzwa: Phakathi nalezi zikhathi, kungadingeka ukuthi uphuze imithi yokunciphisa igazi ukuze uvimbele ukujiya kwegazi, njengoba ingozi yokujiya kwegazi iphakeme. Khuluma nodokotela wakho ngalokhu bese uthola iseluleko.
Kufanele ngibone nini udokotela? Zingaki izikhathi zokuya e-ETU?
Kubalulekile ukuthi umuntu one-KTS ahlolwe njalo impilo yakhe yonke. Yilapho isimo singaqashwa khona, ukwelashwa kungabonakala kuyasebenza, futhi noma yiziphi izinguquko ezidingekayo zingenziwa.
Nokho, ngezinye izikhathi kungadingeka uye esibhedlela uma kunesimo esiphuthumayo. Qaphela lezi zimpawu.
Lezi yizimo lapho udinga ukuya e-ETU (i-Emergency Treatment Unit) ngokushesha:
* Uma ubona izimpawu zokuqhekeka kwegazi: Uma umlenze owodwa ngokuzumayo uba buhlungu, uvuvukele, ubomvu, futhi uzizwa ufudumele uma uwuthinta, kungaba yi-deep vein thrombosis (DVT).
* Uma unezimpawu zokuqhekeka kwegazi emaphashini: Ubuhlungu besifuba obungazelelwe, ubunzima bokuphefumula, ukuphefumula okusheshayo, noma ukukhwehlela igazi, kungaba yi-pulmonary embolism. Lesi yisimo esiphuthumayo.
* Uma kukhona ukopha okukhulu: Uma kukhona ukopha okungalawuleki okuvela noma kuphi emzimbeni.
Uma ubona noma yiziphi zalezi zimpawu, ungachithi isikhathi bese uya eMnyangweni Wezimo Eziphuthumayo (ETU) wesibhedlela esiseduze ngokushesha.
I-KTS iyisimo esiyinkimbinkimbi, ngakho-ke ungase ube nemibuzo eminingi. Njalo uma ubona udokotela wakho, lalela konke okusengqondweni yakho. Ungacabangi ukuthi, "Lena inkinga nje okungafanele ngibhekane nayo." Kungcono ukuvuleleka nokwethembeka kudokotela wakho.
Umlayezo Wokuya Nawe Ekhaya
- I-Klippel-Trenaunay Syndrome (KTS) iyisici esingavamile sokuzalwa esingabangelwa yinoma yiliphi iphutha labazali.
- Izimpawu ezintathu eziyinhloko yilezi: uphawu lokuzalwa olubomvu olumnyama (ibala le-port-wine), imithambo evuvukele, kanye nengalo noma umlenze owodwa omkhulu kunomunye.
- Nakuba lesi simo singelapheki ngokuphelele, kunezindlela eziningi zokwelapha ezingakusiza ukuthi ulawule izimpawu futhi uphile impilo enhle kakhulu.
- Izinkinga ezibaluleke kakhulu okufanele uziqaphele amahlule egazi (i-DVT, i-Pulmonary Embolism) kanye nokopha ngokweqile. Hlala uqaphele izimpawu eziyisixwayiso zalokhu.
- Kubaluleke kakhulu ukuhlala uthintana nodokotela wakho impilo yakho yonke futhi uye njalo ekuhlolweni.
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