Isifo se-Ehlers-Danlos: yintoni, iimpawu kunye nonyango

Umxholo

• Zithini iimpawu?
• Izizathu ezinokubangela
• Zeziphi iintlobo
• Yintoni isifo
• Unyango lwenziwa njani

Isifo se-Ehlers-Danlos, esaziwa ngcono ngokuba sisifo samadoda eselastiki, sichazwa liqela lokuphazamiseka kwemfuza okuchaphazela izicwili ezinxulumene nolusu, amalungu kunye neendonga zemithambo yegazi.

Ngokubanzi, abantu abanesi sifo banamalungu, iindonga zemithambo yegazi nolusu olwandayo kunesiqhelo kwaye lubuthathaka ngakumbi, kuba sisihlunu esidibeneyo esinomsebenzi wokubanika ukuxhathisa kunye nokuguquguquka, kwaye kwezinye iimeko, umonakalo omkhulu we-vascular yenzeka.

Esi sifo sidlulela kubazali sisiya ebantwaneni asinanyango kodwa sinokunyangwa ukunciphisa umngcipheko weengxaki. Unyango luquka ukulawulwa kwamachiza e-analgesic kunye ne-antihypertensive, unyango lomzimba kwaye kwezinye iimeko, utyando.

Zithini iimpawu?

Iimpawu kunye neempawu ezinokuthi zenzeke kubantu abane-Ehlers-Danlos syndrome zikhulu kakhulu kwiindawo ezidibeneyo, ezikhokelela ekuhambeni okubanzi kunokuba ziqhelekile kwaye zibangele iintlungu zendawo kunye nokulimala, ukunyaniseka okukhulu kwesikhumba okwenza kube lula kunye nokugqithisa kunye nokuqhekeka okungaqhelekanga.

Ukongeza, kwiimeko ezinzima kakhulu, apho i-Ehlers-Danlos syndrome inemithambo yegazi, abantu banokuba neempumlo ezibhityileyo kunye nomlomo ongasentla, amehlo aqaqambileyo kunye nolusu olucekeceke olonzakala ngokulula. Imithambo esemzimbeni nayo ibuthathaka kakhulu kwaye kwabanye abantu umthambo i-aortic usenokuba buthathaka kakhulu, onokuthi uqhekeke kwaye ukhokelele ekufeni. Iindonga zesibeleko kunye namathumbu nazo zibhityile kwaye zinokwaphuka ngokulula.

Ezinye iimpawu ezinokuvela zezi:

• Ukukhutshwa rhoqo kunye nokuphazamiseka, ngenxa yokungazinzi ngokudibeneyo;
• Ukudibana kwezihlunu;
• Ukudinwa okungapheliyo;
• IArthrosis nesifo samathambo ziselula;
• Buthathaka kwezihlunu;
• Iintlungu kwezihlunu kunye namalungu;
• Ukuxhathisa okukhulu kwiintlungu.

Ngokubanzi, esi sifo sifunyaniswa ebuntwaneni okanye kwinqanaba lokufikisa ngenxa yokuqhawuka rhoqo, ukuqina kunye nokubaxwa okubaxekileyo okunazo kwizigulana, okuphelela ekubizeni umdla wosapho kunye nodokotela wabantwana.

Izizathu ezinokubangela

Isifo se-Ehlers-Danlos sisifo semfuza esizuzwe njengelifa esenzeka ngenxa yokutshintsha kwemfuza encode iintlobo ezahlukeneyo ze-collagen okanye ii-enzymes eziguqula i-collagen, kwaye zinokudluliselwa zisuka kubazali ziye ebantwaneni.

Zeziphi iintlobo

I-Ehlers-Danlos syndrome ihlelwa kwiindidi ezi-6 eziphambili, ukuba luhlobo lwe-3, okanye ukunganyamezeleki, eyona nto ixhaphakileyo, ebonakaliswa luluhlu olukhulu lokunyakaza, lulandelwa luhlobo 1 kunye no-2, okanye iklasikhi, olutshintsho lwayo lunika uhlobo lwe-collagen I kunye uhlobo IV kwaye ichaphazela ulusu ngakumbi.

Uhlobo lwe-4 okanye lwe-vascular lunqabile kunangaphambili kwaye lwenzeka ngenxa yotshintsho kuhlobo III lwe-collagen echaphazela imithambo yegazi kunye namalungu, anokuqhekeka ngokulula.

Yintoni isifo

Ukwenza uxilongo, yenza nje uvavanyo lomzimba kwaye uvavanye imeko yamalungu. Ukongeza, ugqirha unokwenza uvavanyo lwemfuzo kunye nolusu biopsy ukufumanisa ubukho be-collagen fibers.

Unyango lwenziwa njani

Isifo i-Ehlers-Danlos asinalo unyango, kodwa unyango lunokunceda ekuthomalaliseni iimpawu nasekukhuseleni iingxaki kwesi sifo. Ugqirha unokuyalela iipilisi zentlungu, ezinje ngeparacetamol, ibuprofen okanye naproxen, umzekelo, ukunciphisa iintlungu kunye namayeza okwehlisa uxinzelelo lwegazi, kuba iindonga zemithambo yegazi zibuthathaka kwaye kunyanzelekile ukunciphisa amandla igazi elidlula kuwo.

Ukongeza, i-physiotherapy nayo ibaluleke kakhulu, kuba iyanceda ukomeleza izihlunu kunye nokuzinzisa amalungu.

Kwiimeko ezinzima kakhulu, apho kufuneka ukuba kulungiswe ilungu elidibeneyo, apho isitya segazi okanye iqhekeza lomzimba, utyando lunokuba yimfuneko.