Familyal Hypercholesterolemia - Zomwe Muyenera Kudziwa Zokhudza Izi

Kodi pali anthu m'banja mwanu omwe adadwala matenda a mtima asanakule, kutanthauza kuti, ali ndi zaka 40-50? Kapena ndi zachilendo kuti anthu m'banja mwanu azinena nthawi zonse kuti, "Cholesterol yambiri, cholesterol yambiri"? Nthawi zambiri, timaganiza kuti chifukwa cha izi ndi zinthu monga zomwe timadya ndi kumwa, komanso kusachita masewera olimbitsa thupi. Izi ndi zoona, ndipo zimakhudzanso. Koma nthawi zina, chifukwa chenicheni cha cholesterol yambiri iyi chingakhale vuto mkati mwa thupi lathu, m'majini athu. Ndicho chimene timachitcha 'Familial Hypercholesterolemia' kapena 'FH' mu zamankhwala. Lero, tikambirana izi mosavuta, mwanjira yomwe mungamvetse.

Mwachidule, kodi Familyal Hypercholesterolemia (FH) ndi chiyani?

Matenda a hypercholesterolemia a m'banja ndi matenda a majini omwe amabadwa nawo kuchokera kwa amayi, abambo, kapena onse awiri. Apa ndi pamene cholesterol "yoipa", kapena LDL cholesterol, m'thupi lathu imakhala yokwera kwambiri kuyambira kubadwa.

Taganizirani mitsempha yathu yamagazi ngati mitsempha. Cholesterol ya `LDL` iyi ili ngati chigawo chachikasu, chonga sera chomwe chimamatira kumakoma a mitsemphayo. Nthawi zambiri, izi zimasonkhana pang'onopang'ono. Koma mwa munthu yemwe ali ndi `FH`, kuchuluka kwa cholesterol ya `LDL` kumakhala kwakukulu kwambiri kotero kuti chigawo cha sera ichi chimayamba kusonkhana mwachangu komanso mozama kwambiri mkati mwa mitsempha yamagazi. Timachitcha ichi `atherosclerosis`. Pakapita nthawi, chigawochi chimakula ndikuchepetsa mitsempha yamagazi, nthawi zina kuitseka kwathunthu. Pamenepo ndi pomwe zinthu zoopsa monga matenda amtima ndi sitiroko zimachitika.

Mulingo wa cholesterol wa `LDL` wa munthu wathanzi wabwinobwino uyenera kukhala wochepera 100 mg/dL. Komabe, mwa munthu yemwe ali ndi `FH`, mtengo uwu ukhoza kukhala wokwera kwambiri, monga 160 mg/dL, 190 mg/dL . Nthawi zina zoopsa, zimatha kupitirira 400 mg/dL. Choopsa kwambiri ndichakuti popeza vutoli lakhalapo kuyambira ali mwana, ngati silinachiritsidwe, chiopsezo chotenga matenda a mtima chimakhala chachikulu kwambiri ali wamng'ono kwambiri kuposa munthu wamba.

Nkhaniyi ingamveke ngati yoopsa pang'ono, koma nkhani yabwino ndi yakuti ngati muizindikira msanga, kumwa mankhwala oyenera, ndikusintha moyo wanu, mutha kuyilamulira kwathunthu ndikukhala ndi moyo wathanzi.

Pali mitundu iwiri ikuluikulu ya FH.

Vutoli lagawidwa m'magulu awiri akuluakulu kutengera momwe timakhalira nalo. Izi n'zosavuta kumvetsa.

Tangoganizani kuti matupi athu ali ndi 'malangizo' awiri owongolera cholesterol: Limodzi timalandira kuchokera kwa amayi athu, ndi lina kuchokera kwa abambo athu.

1. Heterozygous FH: Mtundu uwu ndi wofala kwambiri. Chomwe chimachitika apa ndi chakuti pali cholakwika mu 'buku la malangizo' (jini) chomwe chimalandira kuchokera kwa kholo limodzi lokha , kaya mayi kapena bambo. Izi zikutanthauza kuti buku limodzi ndi labwino, ndipo buku lina lili ndi cholakwika pang'ono. Izi zimapangitsa kuti kulamulira kwa cholesterol kukhale kovuta pang'ono.

2. Homozygous FH: Izi sizichitika kawirikawiri, komanso zimakhala zoopsa kwambiri.Mtundu. Chomwe chimachitika apa ndi chakuti 'malangizo' onse awiri omwe amalandira kuchokera kwa amayi ndi abambo ali ndi zolakwika. Kenako njira yowongolera cholesterol imakhala yofooka kwambiri. Milingo ya cholesterol ya `LDL' ya anthu awa ndi yokwera kwambiri.

`FH` ndi matenda a majini omwe amapezeka m'gulu la `autosomal dominant`. Izi zikutanthauza kuti mwana safunika kulandira jini yolakwika kuchokera kwa makolo onse awiri kuti alandire matendawa, koma kungolandira kuchokera kwa kholo limodzi lokha .

Kodi vutoli ndi lofala bwanji? Kodi zizindikiro zake ndi ziti?

Akuti munthu m'modzi pa anthu 250 aliwonse padziko lapansi ali ndi matenda ofanana a 'Heterozygous FH'. Koma chomvetsa chisoni kwambiri n'chakuti anthu 9 pa 10 aliwonse sadziwa kuti ali ndi matendawa .

Kawirikawiri, FH siisonyeza zizindikiro zilizonse zoyambirira. Simungadziwe kuti muli ndi FH mpaka mutadwala matenda a mtima, monga kupweteka pachifuwa. Koma kenako zingakhale mochedwa. Izi zili choncho chifukwa chakuti cholesterol m'mitsempha yamagazi ya munthu amene ali ndi FH imayamba kupangika kuyambira tsiku lomwe anabadwa.

Pakapita nthawi, kuchuluka kwa cholesterol m'magazi kungayambitse zizindikiro zotsatirazi.

Kodi matenda a FH amachiritsidwa bwanji?

Ngati dokotala wanu akukayikira kuti muli ndi FH, adzayang'ana zinthu zingapo kuti atsimikizire matendawa.

• Kuyezetsa magazi: Ichi ndi chofunika kwambiri. Kuyezetsa magazi kotchedwa `Lipid Profile`, komwe kumayesa kuchuluka kwa cholesterol yanu, kumayang'ana kuchuluka kwa cholesterol ya `LDL` yokwera kwambiri. Ngati ndi yoposa 190 mg/dL mwa munthu wamkulu, kapena yoposa 160 mg/dL mwa mwana, `FH` imaganiziridwa.
• Mbiri ya matenda a m'banja: Izi ndizofunikira kwambiri. Dokotala wanu adzakufunsani mafunso monga, "Kodi abambo anu, amayi anu, agogo anu, kapena abale anu adadwalapo matenda a mtima ali aang'ono?" "Kodi pali aliyense m'banja mwanu amene ali ndi cholesterol yambiri?"
• Kuwunika thupi: Dokotala adzayang'ana thupi lanu ngati pali zizindikiro zilizonse za xanthomas (zotupa pakhungu) kapena cornea arcus (mabwalo a maso) zomwe zatchulidwa kale.
• Kuyesa majini (kuyesa DNA): Nthawi zina, kuyesa majini kungachitike kuti kutsimikizire matendawa. Izi zitha kuwona zolakwika m'majini akuluakulu omwe amayambitsa FH (monga APOB, LDLR, kapena PCSK9).

Kodi mankhwala ake ndi otani?

`FH` si matenda omwe tingathe kuchiza. Chifukwa ndi chinthu chomwe chimabwera ndi majini athu. Koma chingathe kulamulidwa bwino kwambiri . Cholinga chachikulu cha chithandizo ndikuchepetsa kuchuluka kwa cholesterol ya `LDL` momwe tingathere, kuchepetsa chiopsezo cha matenda a mtima.

1. Mankhwala (mitundu ya mankhwala)

Kusintha moyo kokha sikukwanira kulamulira cholesterol kwa munthu amene ali ndi `FH`. Chifukwa chake , mankhwala amodzi kapena angapo ayenera kumwedwa moyo wawo wonse .

• Ma statins:Mankhwala awa ndi ofala kwambiri komanso oyamba. Amagwira ntchito pochepetsa kupanga kwa cholesterol m'chiwindi.
• Mankhwala oletsa PCSK9: Awa ndi mankhwala atsopano omwe amaperekedwa jakisoni. Ndi othandiza kwambiri kwa anthu omwe kuchuluka kwa cholesterol m'thupi lawo sikungathe kulamulidwa ndi ma statin okha.
• Ezetimibe: Mankhwalawa amagwira ntchito pochepetsa kuyamwa kwa cholesterol kuchokera muzakudya zomwe timadya. Nthawi zambiri amaperekedwa pamodzi ndi ma statins.
• Mankhwala ena: Kuphatikiza apo, palinso mankhwala ena monga `Bile acid sequestrants` ndi `Fibrates`. Dokotala wanu adzasankha mankhwala oyenera kwambiri kwa inu.

2. Kusintha kwa moyo

Kuphatikiza pa kumwa mankhwala, ndikofunikiranso kutsatira moyo wathanzi. Izi sizimangowonjezera mphamvu ya mankhwalawo, komanso zingachepetse chiopsezo cha matenda a mtima.

3. Mankhwala ena enieni

Kwa anthu omwe ali ndi matenda a FH oopsa kwambiri, mankhwala okha sangathe kulamulira cholesterol yawo. Kwa anthu awa, chithandizo chotchedwa LDL apheresis chimachitika. Izi zili ngati dialysis. Magazi ochepa amatengedwa, amadutsa mu makina, omwe amachotsa cholesterol yoyipa ya LDL, kenako amabwereranso m'thupi. Izi zimachitika kamodzi kapena kawiri pa sabata.

Ngati muli ndi FH, muyenera kuchita chiyani?

Ngati mwapezeka ndi `FH`, si nkhani ya inu nokha. Ndi uthenga wofunika kwambiri kwa banja lanu lonse.

Ngati mwana ali ndi mbiri ya banja la `FH`, cholesterol imatha kuyesedwa ngakhale ali ndi zaka ziwiri. Chifukwa chithandizo cha vutoli chikayamba msanga, mavuto omwe angakhalepo angachepe mtsogolo.

Ngati muli ndi `FH`, musachite mantha. Lumikizanani ndi dokotala wanu. Imwani mankhwala anu pa nthawi yake. Sinthani moyo wanu. Mukatero inunso mutha kukhala ndi moyo wautali komanso wathanzi monga wina aliyense.