Kodi muli ndi mavuto amenewa ndi mapapo anu? Tiyeni tikambirane za LAM (Lymphangioleiomyomatosis)

Kodi mudamvapo za matendawa omwe ali ndi dzina lalitali lotchedwa LAM (lymphangioleiomyomatosis)? Mungaone dzinali ngati lachilendo. Koma ndi matenda osowa omwe amakhudza mapapo. Lero, tikambirana za matendawa mosavuta, m'njira yomwe mungamvetse. Musadandaule, tidzafotokoza zonse.

Kodi LAM (Lymphangioleiomyomatosis) kwenikweni ndi chiyani?

Mwachidule, LAM ndi matenda osowa omwe amachititsa kuti ma cysts odzaza ndi madzi apangidwe m'mapapu anu. Izi zitha kuwononga mapapu anu. Nthawi zina zingayambitsenso ma cysts mu impso zanu ndi dongosolo la lymphatic. Tangoganizirani zomwe zingachitike ngati maselo osalala a minofu m'thupi lanu ayamba kukula mwadzidzidzi. Ndicho chimene chimachitika apa. Zimayambitsidwa ndi kusintha kwa majini.

Matendawa, otchedwa LAM (otchedwa “limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , nthawi zambiri amakhudza akazi . Nthawi zambiri amakhudza akazi azaka zapakati pa 20 ndi 40, kapena akazi omwe atha msinkhu ndipo akuyandikira kusamba. Chifukwa cha izi, madokotala amaganiza kuti mahomoni otchedwa estrogen kapena kukhalapo kwa chiberekero kungakhale ndi chifukwa choyambitsa matenda a zotupa izi.

Kodi mitundu ikuluikulu ya LAM ndi iti?

Pali mitundu iwiri ikuluikulu ya LAM:

1. TSC-LAM: Azimayi ena ali ndi vuto la majini lotchedwa ``Tuberous Sclerosis Complex - TSC''. Ngati ayamba LAM, imatchedwa TSC-LAM.

2. Kusadya nyama nthawi ndi nthawi: Izi zimachitika mwachisawawa, kutanthauza kuti kusintha kwa majini kumachitika mwachisawawa pa moyo. Chofunika ndichakuti mtundu uwu wa Kudya nyama nthawi ndi nthawi usaperekedwe kwa ana anu.

Kodi matenda amenewa amatchedwa LAM ndi ofala bwanji?

Kutsegula m'mimba kwenikweni ndi matenda osowa kwambiri . Akuti pafupifupi mkazi m'modzi mwa akazi 140,000 akhoza kukhala ndi matendawa. Komabe, pakati pa 30% ndi 80% ya akazi omwe ali ndi ``Tuberous Sclerosis`` akhoza kukhala ndi kutsegula m'mimba.

Kodi zizindikiro za matenda a LAM ndi ziti?

Munthu amene ali ndi LAM angakumane ndi zizindikiro monga:

• Kuvuta kupuma (kupuma movutikira) : Nthawi zina izi zimatha kuwonjezeka pang'onopang'ono.
• Kupuma movutikira ndi phokoso lochokera pachifuwa .
• Kupweteka pachifuwa .
• Chifuwa .
• Nthawi zina magazi kapena chyle (`(chyle)` - madzi am'mimba ochokera m'mimba) amatuluka pamodzi ndi ntchofu .

Musaganize kuti muli ndi vuto la kutsekula m'mimba chifukwa chakuti muli ndi zizindikirozi, chifukwa zingakhalenso zizindikiro za matenda ena a m'mapapo. Chifukwa chake, ndikofunikira kufunafuna upangiri wa dokotala.

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

Chifukwa chachikulu cha LAM ndi kusintha kwa majini awiri m'thupi lathu otchedwa `(TSC1)` ndi `(TSC2)`. Majini awiriwa ali ngati majini oletsa chotupa omwe amaletsa zinthu monga maselo a khansa kukula mosafunikira m'thupi lathu. Chifukwa chake, ngati pali cholakwika mu majini awiriwa, maselo osalala a minofu omwe ndatchula kale amayamba kukula mosalamulirika. Zotsatira zake, izi ndi izi:

• Matenda a m'mapapo (pulmonary lymphangioleiomyomatosis) amayamba.
• Matenda a angiomyolipomas amapezeka mu impso.
• Ma cysts amapangika mu dongosolo la lymphatic.

Kodi munthu amadwala bwanji matenda a LAM?

Pali njira ziwiri zomwe munthu angakulitsire LAM. Njira yoyamba ndi kulandira jini yolakwika kuchokera kwa kholo lomwe lili ndi vuto mu jini ya `(TSC1)` kapena `(TSC2)`. Cholowa ichi chimabweretsa vuto la `(Tuberous Sclerosis)`, ndipo limabwera ndi chiopsezo chotenga LAM. Azimayi oposa 30% omwe ali ndi `(Tuberous Sclerosis)` ali ndi LAM.

Njira ina ndi yakuti kusintha kwa majini a `(TSC2)` kumachitika mwangozi, popanda kulumikizana kulikonse kobadwa nako. Chifukwa chenicheni cha izi sichikudziwika. LAM yomwe imayamba motere imatchedwa Sporadic LAM. Pankhaniyi, zizindikiro zina za `(Tuberous Sclerosis)` sizimawonekera.

Ndani ali pachiopsezo chachikulu chotenga LAM?

Ngakhale izi n'zodabwitsa, LAM nthawi zambiri imapezeka mwa akazi . Amuna ochepa okha ndi omwe apezeka ndi LAM mpaka pano. Mwamuna m'modzi yekha ndi amene wanenedwa kuti ali ndi LAM nthawi ndi nthawi.

Komanso, LAM ikhoza kukhala yoopsa kwambiri panthawi ya mimba komanso mukamagwiritsa ntchito mankhwala okhala ndi estrogen .

Kodi mavuto omwe angakhalepo chifukwa cha LAM ndi ati?

Vuto lofala kwambiri la LAM ndi pneumothorax . Monga momwe baluni imaphulikira, mpweya umatuluka m'mapapo ndipo umapangitsa kuti mapapo agwe. Azimayi oposa theka omwe ali ndi LAM amakhala ndi pneumothorax kamodzi pa moyo wawo. Nthawi zina izi zimatha kuchitika mobwerezabwereza. LAM nthawi zambiri imapezeka pambuyo pa nthawiyi.

Mavuto ena ndi awa:

• Kusonkhanitsa madzi otchedwa chyle mozungulira mapapo (chylothorax).
• Kuchulukana kwa madzi m'mapapo (pleural effusion).
• Kutsekeka kwa dongosolo la lymphatic.

Kodi matenda a LAM amapezeka bwanji?

Kuti adziwe matenda a LAM, dokotala adzayamba wakuyezani kaye ndikukufunsani za zizindikiro zanu. Kenako adzayitanitsa mayeso ndi mayeso ojambulira kuti adziwe zotsatirazi:

• Onani momwe mapapu anu akugwirira ntchito bwino.
• Yang'anani kuchuluka kwa mpweya m'magazi.
• Yang'anani ngati pali kusintha kulikonse m'mapapo.
• Yang'anani ngati pali "mapuloteni" ena ochulukirapo kuposa omwe ali m'magazi.

Popeza zizindikiro za LAM zimafanana ndi za matenda ena ofala m'mapapo, nthawi zina zimakhala zovuta kuti madokotala azindikire.

Ndi mayeso ati omwe amagwiritsidwa ntchito pozindikira LAM?

Mungafunike kuchita mayeso monga awa:

• Mayeso a ntchito ya mapapo : Izi zimatsimikizira momwe mapapu anu amakuthandizireni kupuma ndi kutuluka.
• Kuwona kuchuluka kwa mpweya m'magazi (`(pulse oximetry)`) : Kuyesa kosavuta komwe kumaphatikizapo kulumikiza chinthu chonga chogwirira chala chanu.
• Mayeso ojambulira : Izi zikuphatikizapo ma CT scan (Computed Tomography scans), MRI (Magnetic Resonance Imaging), kapena ma HRCT (High-Resolution Computed Tomography). Mayeso ojambulira a HRCT amatha kupereka chithunzi chomveka bwino cha malo ang'onoang'ono a mapapo, zomwe zimapangitsa kuti zikhale zosavuta kuzindikira matenda ena a m'mapapo.
• Kuyezetsa kwa VEGF-D : Dokotala wanu angachite mayeso a magazi kuti aone ngati ali ndi matenda, momwe ziwalo zina zanu zikugwira ntchito, komanso kuchuluka kwa puloteni yomwe imatchedwa VEGF-D (Vascular Endothelial Growth Factor-D). Anthu ambiri omwe ali ndi LAM ali ndi puloteni iyi yambiri, koma si aliyense amene amachita zimenezo.
• Kufufuza za mapapo : Izi zimaphatikizapo kutenga kachidutswa kakang'ono ka minofu kuchokera m'mapapo ndikukayesa mu labotale. Chitsanzochi chikhoza kutengedwa pogwiritsa ntchito bronchoscopy (njira yomwe chubu chokhala ndi kamera chimalowetsedwa m'mapapo) kapena kudzera mu opaleshoni ya pakhosi yothandizidwa ndi kanema (VATS).

Kodi matenda a LAM amachiritsidwa bwanji?

Tsoka ilo, pakadali pano palibe mankhwala a LAM. Komabe, mankhwala otchedwa sirolimus (omwe amadziwikanso kuti rapamycin, dzina lodziwika bwino la Rapamune®) angathandize kulimbitsa matendawa ndikuletsa kuti asakule kwambiri. Sirolimus ingathandize kuchepetsa zotupa za impso (angiomyolipomas), kuchepetsa zotupa ndi kudzikundikira kwa madzi m'thupi, komanso kuchepetsa zizindikiro zanu.

Monga mankhwala ena:

• Chithandizo cha okosijeni : Kwa iwo omwe akuvutika kupuma.
• Ma bronchodilator opumira : Izi zili ngati ma inhalers. Amatsegula njira zopumira m'mapapo ndikupangitsa kuti kupuma kukhale kosavuta.
• Kubwezeretsa m'mapapo : Iyi ndi pulogalamu yomwe imaphatikiza masewera olimbitsa thupi, maphunziro, ndi uphungu.
• Kuika mapapo : Monga njira yomaliza kwa iwo omwe matenda awo afika pamlingo wapamwamba kwambiri.

Tiyeni tiphunzirenso za zotsatirapo zake ndi mavuto omwe amabwera chifukwa cha chithandizo.

Sirolimus nthawi zina ingayambitse kuwonongeka kwakukulu kwa impso ndikuwonjezera chiopsezo cha matenda oopsa . Chifukwa chake, ndikofunikira kwambiri kukambirana zabwino ndi zoyipa zogwiritsira ntchito mankhwalawa ndi dokotala musanapange chisankho.

Kodi munthu amene ali ndi vuto la LAM angayembekezere tsogolo lotani?

LAM ikhoza kuipiraipira pakapita nthawi. Ndondomeko yanu ya chithandizo ingasinthe kutengera chithandizo chomwe chili choyenera kwa inu. Mudzagwira ntchito ndi katswiri wanu wa mapapu kuti adziwe njira yabwino yowunikira matenda anu. Iye adzayang'ana ntchito ya mapapo anu nthawi zonse. Ndondomeko yanu ya chithandizo idzakhazikitsidwa pa:

• zaka zanu.
• Mkhalidwe wanu wa kusamba (kaya mwadutsa kale kusamba kapena ayi).
• Kuchepa kwa ntchito ya mapapo mwachangu.
• Kaya impso zanu kapena dongosolo lanu la lymphatic zakhudzidwa.

Kodi LAM imafalikira mofulumira bwanji?

Kufalikira kwa LAM mwachangu kumasiyana malinga ndi munthu ndi munthu . Pali zinthu zingapo zomwe zingakhudze izi:

• Mtundu wa LAM womwe muli nawo : Ngakhale kuti anthu omwe ali ndi `(TSC-LAM)` ali ndi ntchito yabwino kwambiri ya mapapo kuposa omwe ali ndi `(Sporadic LAM)`, kuchuluka kwa kuchepa kwa ntchito ya mapapo kumakhala kofanana kwambiri pa zonsezi.
• Zaka zanu : Kwa anthu ena, LAM imasiya kufalikira pambuyo pa kusamba, pamene kuchuluka kwa estrogen m'thupi kumachepa.
• Momwe mumachitira mukalandira chithandizo : Mankhwala a Sirolimus amatha kulimbitsa matendawa mwa anthu ambiri.

Kodi ndi liti pamene kuyika mapapo ndikofunikira?

Anthu ambiri omwe ali ndi LAM (pafupifupi 64%) amatha kukhala ndi moyo zaka 20 kapena kuposerapo atapezeka ndi matendawa popanda kufunikira kuikidwa mapapo. Komabe, chifukwa maselo a LAM sayamba m'mapapo, ma cysts amatha kukula ngakhale atayikidwa mapapo .

Kodi nthawi yoyembekezera moyo ndi yotani ndi LAM? (Chiyembekezo cha Moyo)

Kutalika kwa nthawi yomwe mungakhale ndi LAM kumadalira kuopsa kwa matendawa komanso momwe amafalikira mofulumira. Koma, monsemo, zinthu zili bwino kwambiri tsopano kuposa kale. Anthu opitilira 90% omwe ali ndi LAM amakhala ndi moyo zaka 10 atapezeka ndi matendawa.

Kodi ndimadzisamalira bwanji?

Lankhulani ndi katswiri wa matenda a m'mapapo (pulmonologist) za zomwe mungayembekezere komanso momwe mungapangire dongosolo lokhalira ndi LAM. Onani dokotala wanu nthawi yake, imwani mankhwala anu monga momwe mwalangizidwira, ndipo uzani dokotala wanu nthawi yomweyo ngati muli ndi zizindikiro zatsopano, ngati zizindikiro zanu zikuipiraipira, kapena ngati muli ndi zotsatirapo zoyipa chifukwa cha chithandizo chanu.

Kodi muyenera kupita kuchipatala liti? Kodi muyenera kupita kuchipatala chadzidzidzi liti?

Ngati mukuvutika kupuma, chifuwa, kapena zizindikiro zina zodetsa nkhawa, kaya zikupitirira kapena zikuipiraipira, onetsetsani kuti mwaonana ndi dokotala.

Nthawi yoti mupite ku Emergency Treatment Unit (ETU) :

Ngati muli ndi zizindikiro za kugwa kwa mapapo (pneumothorax) kapena zizindikiro zina zazikulu, pitani kuchipatala nthawi yomweyo:

• Ngati mukuvutika kupuma.
• Ngati muli ndi ululu waukulu pachifuwa .
• Ngati mutsokomola magazi .
• Ngati khungu, milomo, ndi misomali zikusintha kukhala zabuluu (`(cyanosis)`) .

Kodi ndi mafunso otani ofunika kufunsa dokotala?

Zingakhale zothandiza kufunsa dokotala wanu mafunso monga awa:

• Kodi ndili ndi `(Sporadic LAM)` kapena `(TSC-LAM)`?
• Kodi ndi njira ziti zochiritsira zomwe ndili nazo?
• Kodi ndiyenera kugwiritsa ntchito bwanji mankhwala anga?
• Ndi zizindikiro ziti zatsopano kapena zoipitsitsa zomwe ndiyenera kuyang'anitsitsa?
• Kodi ndikufunika kuikidwa mapapo?

Kodi LAM ndi khansa?

LAM nthawi zambiri si khansa . Komabe, m'njira zina imakhala ngati khansa yomwe imafalikira kuchokera pamalo amodzi m'thupi kupita kwina (khansa yofalikira). Izi zikutanthauza kuti maselo amayamba pamalo amodzi ndikuyenda kudzera m'mitsempha yamagazi ndi dongosolo la lymphatic kupita ku mapapo ndi impso. Mtundu uwu wa kukula nthawi zina umatchedwa benign cell metastasis.

Koma asayansi sakudziwabe komwe maselowa amayambira. Komanso, zotupazi sizikuoneka kuti zikufalikira kwambiri ku ziwalo zina, monga chiwindi kapena ubongo, monga khansa. Akayang'aniridwa ndi maikulosikopu, maselowa amaoneka ngati maselo abwinobwino kuposa maselo a khansa.

Chifukwa cha mankhwala atsopano komanso kumvetsetsa bwino za LAM, anthu tsopano akukhala ndi moyo wautali popanda kuikidwa mapapo. Koma zimatenga nthawi kuti azolowere moyo watsopanowu. Kuphunzira za matenda omwe mungakhale nawo kwa moyo wanu wonse kungamveke ngati nthaka yachotsedwa pansi pa mapazi anu. Koma gulu lanu lachipatala - ndi gulu lanu lothandizira - lingakuthandizeni kuyambiranso kuyenda bwino.

Pomaliza, zomwe muyenera kukumbukira

LAM ikhoza kukhala matenda ovuta, koma simuli nokha. Chifukwa cha kupita patsogolo kwa sayansi ya zamankhwala, pali njira zambiri zothanirana ndi matendawa. Chofunika kwambiri ndikukhala ndi chiyembekezo, kutsatira malangizo a dokotala wanu, komanso osataya chiyembekezo. Lankhulani ndi dokotala wanu ndi okondedwa anu za nkhawa zanu ndi mantha anu. Kumbukirani, pali anthu ambiri omwe angakuthandizeni paulendowu.

` LAM, matenda a m'mapapo, matenda osowa, kupuma movutikira, sirolimus, kusamutsa mapapo, kusintha kwa majini