Miyendo yayitali komanso yayitali modabwitsa... Mwina iyi ndi Marfan Syndrome!

Kodi mumamvanso kuti ndinu wamtali kwambiri ndipo muli ndi manja ndi miyendo yayitali kuposa anzanu? Kapena kodi mafupa anu amasweka mosavuta? Kodi munavala magalasi kuyambira muli ana? Ngati chimodzi mwa zinthuzi chikukukhudzani, kungakhale kofunikira kwambiri kuti mudziwe matenda omwe tikukamba lero, otchedwa Marfan Syndrome. Ngakhale kuti izi sizikudziwika bwino, tiyeni tikambirane mwachidule.

Mwachidule, kodi Marfan Syndrome ndi chiyani?

Taganizirani thupi lathu ngati nyumba yomangidwa bwino. Njerwa, makoma, ndi denga la nyumbayi zimagwirizanitsidwa pamodzi ndikulimba ndi simenti. Mofananamo, pali mtundu wapadera wa minofu yomwe imalumikiza chilichonse m'thupi lathu, monga ziwalo, mafupa, minofu, ndi mitsempha yamagazi, ndikuzipatsa mphamvu ndi kusinthasintha komwe zimafunikira. Mu zamankhwala, timatcha izi "minofu yolumikizana." Izi zili ngati 'chingamu' m'thupi lathu.

Matenda a Marfan Syndrome ndi matenda a majini. Munthu amene ali ndi vutoli amakhala ndi minofu yolumikizana yofooka, kapena, yomasuka kwambiri. Izi zikutanthauza kuti minofu yolumikizanayo siili yolimba komanso yotanuka. Chifukwa minofu yolumikizana iyi imapezeka m'thupi lonse, Matenda a Marfan Syndrome amatha kukhudza ziwalo zingapo za thupi lathu. Amatha kukhudza makamaka mtima, mitsempha yamagazi, maso, mafupa, ndi mafupa .

Ngakhale kuti vutoli ndi lobadwa nalo, nthawi zina zizindikiro zimawonekera ndipo matendawa amapezeka ali aang'ono.

Kodi zizindikiro za izi zingakhale zotani?

Chofunika kumvetsetsa apa ndichakuti si aliyense amene ali ndi Marfan Syndrome amene amakumana ndi zizindikiro zofanana. Zimasiyana kwambiri kuchokera kwa munthu ndi munthu. Anthu ena akhoza kukhala ndi zizindikiro zambiri, pomwe ena akhoza kukhala ndi zochepa chabe. Ndicho chifukwa chake madokotala amatcha vutoli kukhala ndi "kusintha kwa mawonekedwe."

Komabe, pali makhalidwe ena ofanana omwe angaoneke. Tiyeni tiwagaŵanitse m'magawo awiri.

Kuwonjezera pa makhalidwe awiri akuluakulu awa, makhalidwe ena okhudzana ndi mawonekedwe a thupi nthawi zambiri amaonekera.

Kodi ndi mavuto otani omwe angabwere chifukwa cha Marfan Syndrome?

Ngati vutoli silikuthandizidwa bwino, mavuto aakulu angabuke pakapita nthawi.

Zotsatira zomwe zingachitike pa mtima ndi mitsempha yamagazi

Izi ndi zovuta zomwe zimafunika chisamaliro chachikulu mu Marfan Syndrome.

• Kuduladula kwa mtsempha wamagazi: Uwu ndiye mkhalidwe woopsa kwambiri. Khoma la mtsempha wamagazi limapangidwa ndi zigawo zingapo. Kung'ambika mwadzidzidzi mkati mwa khomali kungayambitse kutuluka kwa magazi pakati pa zigawozo. Uwu ndi mkhalidwe woopsa womwe umafuna opaleshoni yadzidzidzi.
• Matenda a valavu ya mtima: Mavalavu a mtima amakhala ofooka ndipo sangatseke bwino, zomwe zimapangitsa kuti magazi atuluke m'mbuyo.
• Mtima wokulira: Minofu ya mtima imatha kukula ndikufooka chifukwa mtima umayenera kugwira ntchito molimbika pakapita nthawi.
• Kusakhazikika kwa kugunda kwa mtima (Arrhythmia): Kugunda kwa mtima kungakhale kosakhazikika.
• Ma aneurysms a ubongo: Malo ofooka mu mtsempha wamagazi mkati kapena mozungulira ubongo amatha kutupa ngati buluni.

Zotsatirapo pa maso

• Matenda a Cataract
• Glaucoma
• Kuchotsedwa kwa retina

Zotsatirapo pa mapapo

Kufooka kwa minofu yolumikizirana kungakhudzenso mapapo.

• Mphumu
• Matenda a m'mapapo (bronchitis, chibayo)
• Mapapo osweka (Pneumothorax)

N’chifukwa chiyani matenda a Marfan amachitika? Kodi chifukwa chake n’chiyani?

Chifukwa chachikulu cha izi ndi kusintha kwa majini, Fibrillin-1, yomwe imapezeka m'maselo olumikizana a thupi lathu.Pali jini yomwe imalamulira kupanga puloteni yotchedwa ``fibrillin.`` Imatchedwa jini ya ``FBN1''. Anthu omwe ali ndi Marfan Syndrome ali ndi kusintha kwina, kapena chilema (chosiyana), mu jini iyi ya ``FBN1''. Izi zimapangitsa thupi kupanga puloteni yofooka ya fibrillin.

• Kawirikawiri (pafupifupi 75%) jini yolakwika imatengedwa kuchokera kwa kholo limodzi. Ngati kholo lililonse lili ndi vutoli, mwana aliyense ali ndi mwayi wa 50% wolandira vutoli.
• Mu 25% yotsala ya milandu, mwanayo akhoza kukhala ndi kusintha kwa majini ngakhale makolo ake alibe vutoli. Chifukwa chenicheni sichinapezekebe.

Kodi madokotala amapeza bwanji izi?

Popeza Marfan Syndrome imakhudza ziwalo zambiri za thupi, zingafunike gulu la madokotala ochokera kumadera osiyanasiyana kuti adziwe matenda molondola. Dokotala wanu nthawi zambiri amatsatira njira izi:

• Kufunsa za mbiri yanu yachipatala ndi zizindikiro: Kufunsa za kusapeza bwino kulikonse komwe mukukumana nako, mavuto a masomphenya, kapena kupweteka kwa mafupa.
• Kuwunika thupi lonse: kuyeza kutalika, kulemera, kutalika kwa miyendo, ngati pali kupweteka kwa msana, ndi mawonekedwe a chifuwa.
• Kufunsa za mbiri ya matenda a m'banja: Kufunsa za zinthu monga ngati pali aliyense m'banjamo amene wakhalapo ndi zizindikirozi, kapena ngati pali wina amene wamwalira chifukwa cha kulephera kwa mtima mwadzidzidzi.
• Kutumiza mayeso apadera:
• Echocardiogram (Echo test): Iyi ndi njira yofunika kwambiri yoyezera mtima ndi mtsempha wamagazi, komanso momwe ma valve amagwirira ntchito.
• Electrocardiogram (ECG): Yang'anani ngati mtima sukuyenda bwino.
• Kujambula kwa CT kapena MRI: Onani kutalika konse kwa mtsempha wamagazi ndi mitsempha ina yamagazi mwatsatanetsatane.
• Kuyezetsa maso: Dziwani malo a lenzi ya diso ndi mavuto ena.
• Kuyezetsa majini: Chitsanzo cha magazi chikhoza kutengedwa kuti mudziwe ngati pali cholakwika mu jini ya `FBN1`.

Kodi mankhwala ochizira izi ndi otani?

Choyamba, palibe mankhwala a Marfan Syndrome. Koma musadandaule. N'zotheka kuthana nayo bwino, kupewa mavuto, ndikukhala ndi moyo wabwinobwino komanso wathanzi. Cholinga chachikulu cha chithandizo ndikuwongolera zizindikiro ndikupewa mavuto oopsa.

Mankhwala

• Beta-blockers: Mankhwalawa amachepetsa kugunda kwa mtima pang'ono, amachepetsa kuthamanga kwa magazi, komanso amachepetsa kuthamanga kwa magazi m'mitsempha yamagazi. Izi zitha kuchepetsa kuthamanga kwa magazi komwe mtsempha wamagazi umakula.
• Angiotensin II Receptor Blockers (ARBs): Mankhwalawa amathandizanso kuteteza mtsempha wamagazi.

Kuwunika Kwachizolowezi

Iyi ndi gawo lofunika kwambiri pa chisamaliro. Muyenera kuonana ndi dokotala nthawi zonse ndikuyesedwa.

• Mtima ndi mitsempha yamagazi: Kuyesa kwa echo kumachitika kamodzi pachaka kuti muwone ngati pali kusintha kwa kukula kwa mtsempha wamagazi.
• Maso: Muyenera kuyesedwa maso anu nthawi zonse ndi dokotala wa maso.
• Dongosolo la mafupa: Muyeneranso kusamala ndi zinthu monga msana wanu.

Malangizo pa masewera olimbitsa thupi

Kuchita masewera olimbitsa thupi ndi kwabwino kwa munthu amene ali ndi Marfan Syndrome, koma si masewera olimbitsa thupi onse omwe ndi abwino. Masewera olimbitsa thupi ena amphamvu amatha kukakamiza mtsempha wamagazi. Chifukwa chake, ndikofunikira kulankhula ndi dokotala wanu kuti mudziwe masewera olimbitsa thupi omwe ali oyenera kwa inu.

Opaleshoni ya Mtima

Ngati mtsempha wa mtima wakula kwambiri, madokotala angakulangizeni opaleshoni yochotsa gawo lofooka ndikuyika chogwiriracho chisanasweke. Ngati ma valve a mtima awonongeka, opaleshoni ikhoza kuchitidwa kuti ikonzedwe kapena kusinthidwa.

Moyo ndi thanzi la maganizo ndi Marfan Syndrome

Kukhala ndi Marfan Syndrome nthawi zina kungakhale kovuta. Kuonana ndi madokotala nthawi zonse, kumwa mankhwala, komanso kusintha moyo wanu kungakhale kotopetsa.

Ndiponso,

• Kuganizira za mawonekedwe a thupi lanu kungayambitse nkhawa.
• Kupweteka thupi pafupipafupi komanso kutopa.
• Ngati simungathe kusewera masewera, kuthamanga, ndi kusewera ngati anthu ena, mungamve ngati kuti mwapatulidwa ndi anthu ena.
• Zinthu monga tsogolo ndi kuyambitsa banja zingayambitse mantha.

Pazifukwa izi, pali chiopsezo chotenga matenda amisala monga nkhawa ndi kuvutika maganizo .

Chifukwa cha chidziwitso chomwe tili nacho chokhudza Marfan Syndrome ndi mankhwala atsopano, nthawi ya moyo ya munthu amene ali ndi vutoli tsopano ikufanana kwambiri ndi ya munthu wamba. Chofunika kwambiri ndi kuzindikira matendawa msanga ndikuthana nawo bwino.