Kodi tikambirane za LAM, matenda omwe amakhudza makamaka akazi? (Lymphangioleiomyomatosis)

Kodi nthawi zina mumamva kupuma movutikira, kutopa, kapena kupweteka pachifuwa? Nthawi zambiri timaganiza izi ngati zizindikiro za matenda ofala monga mphumu. Komabe, pali matenda osowa kwambiri m'mapapo omwe amayambitsa zizindikirozi, makamaka mwa akazi. Amatchedwa Lymphangioleiomyomatosis. Dzinali ndi lalitali pang'ono, kotero tidzalitcha "LAM" mwachidule. Ngati mukuona kuti dzinali ndi lovuta kulitchula, ganizirani motere: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Ngakhale kuti ndi lovuta pang'ono, zingakhale zofunikira kwambiri kuti mumvetse matendawa mosavuta.

Mwachidule, kodi LAM ndi chiyani?

LAM ndi vuto losowa kwambiri lomwe maselo osazolowereka amakula m'mapapo mwanu, ndikupanga ma cysts ang'onoang'ono. Izi zimachitika chifukwa cha kusintha kwa majini komwe kumapangitsa maselo osalala a minofu m'thupi lanu kukula mopanda mphamvu. Izi zitha kuwononga mapapo anu. Sikuti mapapo okha, komanso nthawi zina impso ndi dongosolo la lymphatic zimathanso kupanga zotupa izi.

Matendawa nthawi zambiri amapezeka mwa akazi azaka zapakati pa 20 ndi 40. Izi zikutanthauza kuti, akatha msinkhu komanso asanasambe. Chifukwa chake, madokotala amakhulupirira kuti mahomoni achikazi otchedwa estrogen amatenga gawo pakukula kwa matendawa.

Pali mitundu iwiri ikuluikulu ya matenda a LAM.

Matenda a LAM akhoza kugawidwa m'magulu awiri akuluakulu, kusiyana pang'ono pakati pa awiriwa.

Kodi zizindikiro za matenda a LAM ndi ziti?

Zizindikiro za LAM zimafanana kwambiri ndi za matenda ena a m'mapapo, monga mphumu ndi bronchitis. Izi nthawi zina zimatenga nthawi kuti zidziwike. Ndikofunikira kudziwa izi.

• Kuvuta kupuma (kulephera kupuma): Ichi ndiye chizindikiro chachikulu komanso chofala kwambiri. Ngakhale poyamba chingamveke pokhapokha mutachita masewera olimbitsa thupi pang'ono, vutoli likhoza kuipiraipira pakapita nthawi.
• Kupuma movutikira: Kulira moyimbira mumtima munthu akamapuma, mofanana ndi munthu amene ali ndi mphumu.
• Kupweteka pachifuwa: Kupweteka kumeneku kungamveke makamaka mukapuma mpweya wozama.
• Chifuwa: Pakhoza kukhala chifuwa chouma chosatha.
• Kutsokomola magazi kapena 'chyle': Chyle ndi madzi a mucous omwe amachokera m'mimba mwathu. Ali ndi mtundu wa mkaka. Ngati chinthu chonga ichi chichitika, ndibwino kuti musachedwe konse .

N’chifukwa chiyani LAM iyi imachitika? Kodi chifukwa chake n’chiyani?

Mwachidule, chifukwa chachikulu cha izi ndi kusintha (kusintha kwa majini) komwe kumachitika m'majini awiri m'thupi lathu otchedwa TSC1 ndi TSC2 . Majini awiriwa ali ngati alonda a thupi lathu. Ntchito yawo yayikulu ndikuletsa maselo ena kugawikana ndi kuchulukana mosafunikira popanda kulamulira. Timatcha majini oletsa chotupa.

Tangoganizirani ngati panali vuto mu imodzi mwa majini awiriwa oteteza. Chomwe chimachitika ndichakuti maselo osalala a minofu salandira chizindikiro choti, "Chabwino, zakwana, siyani kugawa." Choncho maselowo amayamba kugawa mosalamulirika. Zotsatira zake ndi izi:

• Ma cysts amapangika m'mapapo.
• Matenda a angiomyolipomas amapezeka mu impso.
• Ma cysts amapangika mu dongosolo la lymphatic.

Mungathe kulandira jini lolakwikali kuchokera kwa makolo anu (lomwe limayambitsa chifuwa chachikulu), kapena mutha kusintha majini awa mwachisawawa nthawi yonse ya moyo wanu popanda chifukwa chomveka (lomwe limayambitsa LAM yosakhazikika).

Zovuta ndi zoopsa za matenda a LAM

Vuto lofala kwambiri la LAM ndi mapapu ogwa , omwe amadziwika kuti pneumothorax .Azimayi oposa theka omwe ali ndi LAM adzakumana ndi vutoli kamodzi pa moyo wawo. Nthawi zina limatha kubwereranso. Ndipotu, akazi ambiri amapezeka ndi LAM koyamba atakhala ndi thumba la m'mapapo ndipo agonekedwa m'chipatala.

Kuphatikiza apo, mavuto ena angachitike.

Kodi matenda a LAM amapezeka bwanji?

Pambuyo pomvera zizindikiro zanu, ngati dokotala wanu akukayikira kuti muli ndi matendawa, adzalamula kuti muyesedwe kangapo kuti atsimikizire.

Mayeso omwe dokotala amachita

• Mayeso a Ntchito ya Mapapu: Izi zimaphatikizapo kupuma ndi kutulutsa mpweya kudzera mu chubu cholumikizidwa ndi makina. Izi zimatha kuyeza momwe mapapu anu akugwirira ntchito komanso kuchuluka kwa mpweya womwe angasunge.
• Pulse Oximetry: Chipangizo chaching'ono, chofanana ndi clip, chimalumikizidwa ku chala chanu ndipo chimayesa kuchuluka kwa mpweya m'magazi anu. Iyi ndi njira yosavuta komanso yopanda ululu.
• Zojambulajambula:
• Kujambula kwa CT (Kujambula Tomography):Izi zitha kutenga zithunzi za 3D za mapapo anu. Izi zingathandize kuona bwino ngati pali ma cysts omwe ali enieni kwa LAM.
• Kujambula kwa HRCT (High-Resolution Computed Tomography): Iyi ndi njira yapamwamba kwambiri yojambulira CT. Imatha kujambula zithunzi zomveka bwino za gawo laling'ono kwambiri la mapapo. Izi ndizofunikira kwambiri pozindikira matenda a LAM.
• Kuyezetsa magazi kwa VEGF-D: VEGF-D ndi puloteni yomwe imapezeka m'magazi mwathu. Anthu ambiri omwe ali ndi LAM ali ndi puloteni iyi m'magazi mwawo kwambiri kuposa masiku onse. Choncho kuyezetsa magazi kumeneku kumathandiza kwambiri kuzindikira matendawa.
• Kufufuza za Mapapo: Nthawi zina, ngati mayeso ena sakutsimikizika 100%, dokotala wanu angasankhe kutenga chidutswa chaching'ono kwambiri cha minofu kuchokera m'mapapo mwanu kuti akayesedwe. Izi zitha kuchitika pogwiritsa ntchito njira yotchedwa bronchoscopy (chubu chopyapyala chokhala ndi kamera yoyikidwa m'njira zopumira) kapena VATS (kanema wothandizidwa ndi thoracic surgery) .

Kodi mankhwala a matenda a LAM ndi ati?

Choyamba, palibe mankhwala a LAM pakadali pano. Koma musadandaule. Tsopano pali mankhwala othandiza kwambiri omwe angathe kuletsa matendawa, kuchepetsa zizindikiro, ndikuchepetsa kukula kwa matendawa.

Chithandizo chachikulu ndi mankhwala otchedwa sirolimus . Amatchedwanso rapamycin. Mankhwalawa amagwira ntchito poletsa kukula kwa maselo omwe amakula mosalamulirika. Izi zingathandize kuwongolera kuwonongeka kwa matendawa kwambiri. Zingathandizenso kuchepetsa zotupa mu impso ndi lymphatic system ndikuchepetsa zizindikiro.

Kuphatikiza apo, dokotala wanu angakulangizeni njira zina zochiritsira kutengera vuto lanu.

• Chithandizo cha Oxygen: Ngati mpweya m'magazi uli wochepa, matanki a okosijeni amaperekedwa kuti agwiritsidwe ntchito kunyumba.
• Mankhwala opumira a bronchodilator: Mankhwalawa, omwe amaperekedwa kudzera mu inhaler, angathandize kuchepetsa vuto la kupuma.
• Kubwezeretsa M'mapapo: Izi zili ngati pulogalamu yapadera yochitira masewera olimbitsa thupi m'mapapo. Zimathandiza kukonza moyo wanu kudzera mu masewera olimbitsa thupi opumira komanso masewera olimbitsa thupi abwino.
• Kusamutsa mapapo:Matendawa akakula kwambiri ndipo sangathe kuthetsedwa ndi mankhwala ena aliwonse, njira yomaliza ndiyo kuyika mapapo ena.

Kodi matendawa amakula mofulumira bwanji?

Izi zimasiyana malinga ndi munthu ndi munthu. Kuchuluka kwa LAM komwe kukukula kumadalira zinthu zingapo.

• Mtundu wa LAM womwe muli nawo: Anthu omwe ali ndi LAM yosakhazikika akhoza kukhala ndi ntchito yochepa ya mapapo poyerekeza ndi omwe ali ndi TSC-LAM.
• Zaka zanu: Kwa akazi ena, akasiya kusamba, kupita patsogolo kwa matendawa kumasiya kapena kuchedwa pamene kuchuluka kwa estrogen m'thupi kumachepa.
• Yankho lanu ku chithandizo: Anthu ambiri amatha kuwongolera matendawa bwino ndi mankhwala monga Sirolimus.

Zinthu zili bwino kwambiri tsopano kuposa kale. Ndi mankhwala amakono, anthu opitilira 90% omwe ali ndi LAM amakhala ndi moyo zaka 10 atapezeka ndi matendawa. Anthu ambiri (pafupifupi 64%) amatha kukhala ndi moyo zaka 20 kapena kuposerapo popanda kufunikira kuikidwa mapapo ena.

Nthawi yoti mukaonane ndi dokotala wanu ndikupita ku ETU

Mukakhala ndi LAM, ndikofunikira kwambiri kudziwa nthawi yoti mupemphe upangiri wa dokotala komanso nthawi yoti mupite ku Emergency Treatment Unit (ETU).

Anthu ena amafunsa ngati LAM ndi khansa. LAM nthawi zambiri si khansa. Komabe, m'njira zina, monga momwe maselo amachokera kwina kupita ku mapapo, amakhala ndi makhalidwe ofanana ndi khansa. Komabe, akayang'aniridwa ndi maikulosikopu, maselowa samawoneka ngati maselo a khansa.

Ndizachibadwa kudabwa komanso kuchita mantha ukadziwa kuti uli ndi matenda oopsa ngati awa. Koma kumbukirani kuti simuli nokha. Ndi chithandizo cha madokotala anu, banja lanu, ndi anzanu, mudzapeza mphamvu zothana ndi vutoli.