Matenda a Marfan ndi Mtima Wanu: Tiyeni Tikambirane Izi!

Mwina mwawonapo anthu aatali kwambiri, okhala ndi miyendo yayitali, komanso owonda kuposa ena. Aliyense ali ndi vutoli, koma si matenda. Koma nthawi zina, mtundu uwu wa thupi ukhoza kukhala chizindikiro cha matenda a majini otchedwa Marfan Syndrome . Uwu ndi mkhalidwe womwe umachitika pamene minofu yolumikizana m'thupi lathu sikukula bwino. Mwachidule, minofu yolumikizana iyi ndi yomwe imagwirizanitsa ziwalo zosiyanasiyana za thupi lathu ndikuzipatsa mphamvu. Izi zikafooka, ziwalo zambiri za thupi zimatha kukhudzidwa, makamaka mtima, maso, mitsempha yamagazi, ndi mafupa . Lero, tikambirana momwe matenda awa otchedwa Marfan Syndrome amakhudzira mtima.

Kodi Marfan Syndrome imakhudza bwanji mtima?

Mukakhala ndi matenda a Marfan, ziwalo ziwiri zazikulu za mtima zimakhudzidwa kwambiri.

1. Mtsempha wa magazi: Uwu ndi mtsempha waukulu, waukulu kwambiri womwe umanyamula magazi okhala ndi mpweya kuchokera mumtima mwathu kupita ku thupi lonse. Uli ngati chitoliro chachikulu chomwe chimanyamula madzi kuchokera mu thanki yamadzi kupita kunyumba kwanu.

2. Ma valve a mtima: Izi ndi ziwalo zomwe zimagwira ntchito ngati zitseko pakati pa zipinda zamkati mwa mtima ndi m'mitsempha yayikulu yomwe imanyamula magazi kuchokera mumtima. Zimaonetsetsa kuti magazi akuyenda mbali imodzi yokha.

Tiyeni tsopano tiwone chilichonse mwa izi padera kuti tiwone zomwe zikuchitika.

Kodi chimachitika ndi chiyani ku aorta?

Matenda a Marfan amachititsa kuti minofu yolumikizana m'makoma a mtima ifooke. Monga chubu chakale cha rabara, imataya mphamvu ndi kusinthasintha kwake. Izi zingayambitse mavuto awiri akuluakulu:

• Kutambasuka kwa mtsempha wamagazi: Mtsempha wamagazi umayamba kukula pang'onopang'ono.
• Aortic aneurysm: M'malo ena, khoma la mtima limakhala lofooka ndipo limatha kuphulika kunja ngati baluni.

Tangoganizirani, aneurysm iyi ili ngati tayala la njinga lomwe limatuluka pamalo amodzi pamene chubucho chafooka.

Makamaka, gawo la mtima lomwe lili pafupi ndi mtima, lotchedwa `muzu wa mtsempha wamagazi`, ndi gawo lomwe nthawi zambiri limakula kapena kukulirakulira motere mwa anthu omwe ali ndi matenda a Marfan. Imeneyi ndi vuto loopsa, chifukwa ngati `aneurysm` iyi ikukula ndikuphulika (`aortic dissection` kapena break), ikhoza kukhala pachiwopsezo cha moyo.

Matenda ena monga Ehlers-Danlos syndrome, Loeys-Dietz syndrome, bicuspid aortic valve, ndi Turner syndrome angayambitsenso kukula kwa mtima, koma amatha kuchitika m'mbali zina za mtima.

Kodi chimachitika ndi chiyani ku ma valve a mtima?

Matenda a Marfan angayambitsenso mavuto ndi ma valve a mtima. Ngati ma valve awa sagwira ntchito bwino, mtima uyenera kugwira ntchito molimbika kuti upompe magazi. Pakapita nthawi, izi zingayambitse kulephera kwa mtima.Zingachitike mbali zonse ziwiri. Matenda awiri akuluakulu a valve amapezeka mogwirizana ndi Marfan syndrome:

• Kubwerera kwa valavu ya mtsempha wamagazi: Pamene valavu pakati pa aorta ndi chipinda chakumanzere cha mtima (ventricle yakumanzere) siitseka bwino, magazi ena omwe amapopedwa amabwerera mumtima. Zimakhala ngati pompo yotuluka madzi.
• Kutsika kwa valavu ya Mitral: Valavu ya mitral, yomwe ili pakati pa chipinda chapamwamba (atrium yakumanzere) ndi chipinda chapansi (ventricle yakumanzere) kumbali yakumanzere ya mtima, sitseka bwino, ndipo chipinda chapamwamba chimatuluka kunja. Izi nthawi zina zingayambitse magazi kutuluka m'mbuyo (mitral regurgitation).

Kodi matenda a mtima ndi ofala bwanji kwa anthu omwe ali ndi matenda a Marfan?

Ndipotu, anthu omwe ali ndi matenda a Marfan ali pachiwopsezo chachikulu chotenga matenda a mtima. Kafukufuku wasonyeza kuti anthu asanu ndi anayi mwa khumi omwe ali ndi matenda a Marfan amakhala ndi vuto linalake la valavu ya mtima kapena aorta. Ndicho chifukwa chake ndikofunikira kudziwa izi.

Kodi zizindikiro za matenda a Marfan zomwe zimakhudza mtima ndi ziti?

Ngati muli ndi matenda a Marfan, monga aortic aneurysm kapena matenda a valve, mutha kukhala ndi zizindikiro zina. Komabe, anthu ena akhoza kukhala ndi matendawa popanda zizindikiro zilizonse. Ichi ndichifukwa chake mayeso azachipatala ndi ofunikira.

Zizindikiro zomwe zingawonekere nthawi zambiri ndi izi:

• Kupweteka pachifuwa kapena kupweteka kumbuyo
• Kutsokomola magazi (ichi ndi chizindikiro choopsa pang'ono)
• Kuvuta kumeza chakudya (ngati mtima waukulu ukukankha pa mmero )
• Kumva chizungulire kapena mutu wopepuka
• Kumva kutopa kwambiri kapena kufooka
• Kumva kugunda kwa mtima kosazolowereka (kugunda kwa mtima)
• Mawu omveka bwino (ngati mtima ukukankha mitsempha yolumikizidwa ndi zingwe zolumikizira mawu)
• Kuvuta kupuma , makamaka mukatopa kapena mukagona pansi
• Kutupa , makamaka m'miyendo ndi m'mapazi
• Kupuma movutikira (kuimba mluzu)

Tangoganizirani kuti muli ndi mnzanu wamtali komanso woonda kwambiri. Nthawi zambiri amadandaula kuti ali ndi ululu pachifuwa komanso amavutika kuyenda . Ngati ndi choncho, zingakhale bwino kupita kwa dokotala kuti akaone ngati vutoli likugwirizana ndi matenda a Marfan.

Kodi n’chiyani chimayambitsa matenda a Marfan kukhudza mtima?

Monga tanenera kale, Marfan syndrome ndi matenda olumikizana ndi minofu.Sizimapangidwira bwino. Minofu yolumikizana bwino imapezeka paliponse m'thupi lathu. Imapereka mphamvu, mawonekedwe, ndi kusinthasintha kwa ziwalo ndi mitsempha yamagazi. Minofu yolumikizana iyi imapezeka m'makoma a mtima ndi mitsempha yamagazi, makamaka aorta, ndi m'ma valve amtima.

Minofu yolumikizana iyi ikafooka chifukwa cha matenda a Marfan, imataya mphamvu ndi kusinthasintha kwawo. Izi zimapangitsa kuti mtima ukhale wotambasuka komanso wotupa, osatha kupirira kuthamanga kwa magazi. Izi zimapangitsanso kuti ma valve a mtima akhale ndi matenda komanso osatha kutsegula kapena kutseka bwino.

Ndi mayeso ati a mtima omwe amathandiza kuzindikira matenda a Marfan?

Matenda a Marfan nthawi zina amakhala ovuta kuwazindikira, chifukwa zizindikiro zimasiyana malinga ndi munthu ndipo zimatha kufanana ndi matenda ena. Anthu ambiri amapeza kuti ali ndi matendawa ali aang'ono kapena akuluakulu.

Ngati dokotala akukayikira kuti muli ndi matenda a Marfan, adzachita zinthu monga:

• Kuyezedwa thupi lonse kudzachitika. Kutalika kwanu, kulemera kwanu, kutalika kwa mkono wanu ndi mwendo wanu, mawonekedwe a chifuwa chanu, maso anu, ndi mtundu wa khungu lanu zidzawunikidwa.
• Adzakufunsani za mbiri ya matenda a m'banja mwanu . Adzafufuza ngati pali aliyense m'banja mwanu amene wakhalapo ndi matenda a Marfan kapena zizindikiro zina zofanana ndi zimenezi.
• Ma scan angapo apadera (`mayeso ojambula`) omwe amafufuza mtima amalamulidwa. Ma scan akuluakulu ndi awa:
• Echocardiogram (Echo): Izi zimakupatsani mwayi wowona bwino kukula, mawonekedwe, ndi ntchito ya mtima ndi mitsempha yamagazi, monga momwe zimachitikira ndi ultrasound scan ya mtima.
• Electrocardiogram (EKG): Iyi imayesa ntchito yamagetsi ya mtima, kutanthauza kayimbidwe ka kugunda kwa mtima.
• Kuyesa majini: Kuyesa kumeneku kungachitike kuti kutsimikizire ngati muli ndi kusintha kwa majini (mu jini la FBN1) komwe kumayambitsa matenda a Marfan.

Kodi njira zothanirana ndi mavuto a mtima omwe amayamba chifukwa cha matenda a Marfan ndi ziti?

Ngakhale kuti matenda a Marfan sangachiritsidwe kwathunthu, pali njira zochizira matenda a mtima komanso kupewa mavuto aakulu. Izi zimabwera m'mitundu iwiri: njira zochizira popanda opaleshoni ndi njira zochizira opaleshoni.

Dokotala angakulangizeni opaleshoni pazochitika zotsatirazi:

• Ngati m'mimba mwake mwa mtsempha wa mtima (aorta) muli masentimita 5 (pafupifupi mainchesi 1.97) kapena kuposerapo.
• Ngati kugunda kwa mtima kukukwera ndi 0.5 centimita (pafupifupi 0.197 inches) kapena kupitirira apo mkati mwa chaka (izi zimatchedwa `rapid expansion`).
• Ngati achibale anu a m'magazi (a m'banja lanu) adachitidwapo opaleshoni yamtunduwu (mwina chifukwa cha majini, opaleshoniyo ingafunike kuchitidwa ngakhale pa mulifupi wocheperako).

Anthu omwe ali ndi matupi ang'onoang'ono nthawi zambiri amakhala ndi mitsempha yamagazi yaying'ono, kotero angafunike opaleshoni ngakhale kukula kwake kuli kochepa.

Mankhwala osagwiritsa ntchito opaleshoni

Izi ndi njira zoyamba zothandizira matenda a Marfan.

• Kusintha moyo: Muyenera kupewa zinthu zomwe zimaika mtima pamavuto.
• Si bwino kuchita zinthu monga kunyamula zolemera kapena kukankhira.
• Masewera amphamvu monga mpira wamiyendo, rugby, ndi nkhonya si oyenera.
• Lankhulani ndi dokotala wanu kuti mudziwe masewera olimbitsa thupi omwe ndi otetezeka komanso oyenera kwa inu.
• Mankhwala:
• Madokotala amapereka mankhwala otchedwa `Angiotensin II receptor blockers (ARBs)` kapena `beta-blockers` . Mankhwalawa amathandiza kuchepetsa kuthamanga kwa magazi m'mitsempha ya mtima komanso kuwongolera kuthamanga kwa magazi.
• Mayeso okhazikika a kujambula zithunzi za mtima:
• Kukula kwa mtima ndi momwe ma valve alili ziyenera kufufuzidwa nthawi zonse ndi dokotala. Izi zitha kuchitika pogwiritsa ntchito ma scan monga `transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, kapena `magnetic resonance angiography (MRA)`.
• Mayeso amenewa amatha kuzindikira kusintha kwa mtima usanaphulike (kuduladula).

Mankhwala opaleshoni

Nthawi zina, mavuto a mtima sangathe kuthetsedwa ndi mankhwala ndi kusintha moyo kokha. Pamenepo ndi pamene opaleshoni imafunika.

Cholinga chachikulu cha opaleshoni ya mtima ya Marfan syndrome ndi kukonza gawo lofooka la valavu ya mtima, kulichotsa ndikuyika valavu yopangira, kapena kukonza valavu ya mtima yowonongeka kapena kuisintha ndi yatsopano.

Cholinga chachikulu cha opaleshoniyi ndikuletsa ngozi zomwe zingawononge moyo monga kusweka kwa mtsempha wamagazi.

Nthawi zambiri, opaleshoni imeneyi imakonzedwa ndipo imachitidwa pa tsiku lokhazikitsidwa kale (opaleshoni yosankha). Komabe, ngati mtsempha wa mtima waphulika mwadzidzidzi kapena kusweka, iyenera kuchitidwa ngati njira yadzidzidzi.

Pali mitundu ingapo ikuluikulu ya opaleshoni ya mtima yomwe imachitidwa pa matenda a Marfan:

• Kubwezeretsa mizu ya mtsempha wamagazi: Iyi ndi opaleshoni yofala kwambiri chifukwa gawo ili la mtima nthawi zambiri limatupa kapena kukula.
• Kukonza kapena kusintha ma valve a mtsempha wamagazi.
• Kukonza kutupa kwa mtsempha wamagazi pafupi ndi mtima (`Kukonzanso kwa aneurysm ya aorta yokwera`).
• Kukonza kapena kusintha ma valve a Mitral.

Dokotala wa opaleshoni ya mtima adzakambirana nanu ndikusankha opaleshoni yomwe ili yoyenera kwa inu.

Kodi matenda a mtima angapewedwe ngati muli ndi matenda a Marfan?

Mwatsoka, matenda a Marfan sangapewedwe, chifukwa ndi a majini. Mofananamo, matenda a mtima omwe amayambitsa sangapewedwe kotheratu.

Komabe, pali zinthu zomwe mungachite kuti muchepetse chiopsezo chanu cha mavuto aakulu, monga kugawanika kwa mtsempha wamagazi:

• Kupewa zinthu zomwe zimaika munthu pamtima nkhawa (monga tafotokozera kale, kupewa kunyamula zinthu zolemera komanso masewera olimbitsa thupi).
• Kuyezetsa matenda a mtima kumachitika pa nthawi yake monga momwe dokotala wanenera.
• Kumwa mankhwala onse olembedwa molondola komanso pa nthawi yake.

Chinthu chimodzi choyenera kukumbukira ndichakuti ngati ndinu mkazi yemwe ali ndi matenda a Marfan ndipo mukuganiza zokhala ndi pakati, muyenera kulankhula ndi dokotala wanu za vutoli. Kafukufuku wasonyeza kuti akazi okwana 40% omwe ali ndi matenda a Marfan angakumane ndi mavuto panthawi ya mimba. Chifukwa chake, ndikofunikira kwambiri kufunafuna upangiri wa dokotala musanayambe kutenga pakati.

Kodi tsogolo la munthu amene ali ndi matenda a mtima chifukwa cha matenda a Marfan ndi lotani?

Ngati munthu amene ali ndi matenda a Marfan syndrome akuyang'aniridwa bwino, kutanthauza kuti, akatsatira malangizo a dokotala komanso akalandira chithandizo choyenera, akhoza kukhala ndi moyo wabwinobwino, mwina zaka 70 kapena 80.

Komabe, mavuto a mtima ndi omwe amachititsa imfa zambiri pakati pa anthu omwe ali ndi matenda a Marfan, choncho ndikofunikira kuti nthawi zonse muzilankhulana ndi dokotala wanu wa mtima kuti akuthandizeni kuyang'anira matenda anu.

Kodi ndiyenera kupita kwa dokotala liti? Kodi zadzidzidzi ndi ziti?

Ngati mukukumana ndi zizindikiro izi, zikhoza kukhala chizindikiro cha kuphulika kwa aneurysm ya mtsempha wamagazi. Muyenera kupeza chithandizo chamankhwala mwachangu:

• Kutaya mwadzidzidzi chidziwitso.
• Kusanza ndi kusanza.
• Kusanza kulikonse m'thupi.
• Kulephera kusuntha ziwalo zina za thupi.
• Kuvuta kwambiri kupuma.
• Kupweteka mwadzidzidzi komanso kosapiririka - pachifuwa, kumbuyo chakumtunda, kapena m'mimba.
• Kutuluka thukuta kwambiri.
• Khungu limakhala lofiirira kapena lozizira kwambiri.
• Kugunda kwa mtima kofooka kwambiri.

Ngati muwona chikwangwani chonga ichi, musachedwe ngakhale mphindi imodzi.

Pomaliza, zomwe muyenera kukumbukira (Uthenga Wopita Nawo Kunyumba)

Matenda a Marfan ndi matenda obadwa nawo omwe amakhudza minofu yolumikizana ya thupi. Amatha kukhudza mtima makamaka. Koma musadandaule. Ngati matendawa atapezeka msanga ndikuthandizidwa bwino, mutha kukhala ndi moyo wabwinobwino.

Chofunika kwambiri ndi ichi:

• Pitani kwa dokotala nthawi zonse.Kenako akhoza kuyang'anira vuto lanu ndikupereka mayeso ndi chithandizo chofunikira panthawi yake.
• Sinthani moyo wanu moyenera. Pewani zinthu zomwe zimakuvutitsani maganizo.
• Imwani mankhwalawo monga momwe mwalangizidwira.
• Ngati dokotala akulangiza opaleshoni, kambiranani mosamala ndipo sankhani chomwe chili chabwino kwa inu.

Mukadziwa zambiri za vuto lanu, zimakhala zosavuta kuti muthane nalo. Chifukwa chake, tikukhulupirira kuti izi zikuthandizani. Ngati muli ndi mafunso aliwonse, musazengereze kufunsa dokotala wanu.

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