Kodi kugona ndi maloto chabe? Tiyeni tikambirane za Kusowa Insomnia kwa Anthu Odwala Matenda a Mimba (FFI).

Ndizachilendo kuti nthawi zina sitingathe kugona usiku. Mwina zimachitika titagwira ntchito molimbika kapena tikakhala ndi vuto linalake m'maganizo mwathu. Koma, kodi mungaganizire matenda oopsa omwe sangakupangitseni kugona bwino, ndipo nawonso ndi obadwa nawo? N'zovuta kuganiza, sichoncho? Lero tikambirana za matenda osowa koma oopsa kwambiri. Izi zimatchedwa Fatal Familyal Insomnia (FFI) . Dzinalo likumveka ngati loopsa pang'ono, sichoncho? Koma tiyeni tidziwe bwino lomwe.

Kodi FFI iyi ndi chiyani? Mwachidule...

Mwachidule, FFI (Fatal Family Insomnia) ndi matenda osowa kwambiri omwe amapezeka m'majini. Amakhudza mwachindunji ubongo wanu ndi dongosolo lanu la mitsempha. Matendawa amakupangitsani kuti musagone bwino, zomwe zikutanthauza kuti muli ndi vuto lalikulu la kusowa tulo . Kuphatikiza apo, pang'onopang'ono mumataya kukumbukira, zomwe zikutanthauza kuti muli ndi vuto la dementia . Muthanso kugwedezeka kwa minofu mosadzifunira, komwe kumatchedwa myoclonus .

FFI ndi vuto lomwe limayamba pang'onopang'ono, kapena kufooka . Izi zikutanthauza kuti zizindikiro zimakula pakapita nthawi. Mwatsoka, zizindikirozo zimaika moyo pachiswe, ndipo pakadali pano palibe mankhwala. Komabe, asayansi akupitiliza kufufuza njira zomwe zingachepetse kufalikira kwa matendawa ndikuwonjezera moyo wa omwe akhudzidwa.

Ndani amene ali ndi mwayi waukulu wopanga FFI?

FFI imayamba chifukwa cha majini. Izi zikutanthauza kuti imayambitsidwa ndi anthu omwe amalandira jini ya matendawa kuchokera kwa mmodzi wa makolo awo. Nthawi zambiri, ngati wina m'banjamo adakhalapo ndi matendawa kale, banja limenelo limakhala ndi mwayi woti lidwale matendawa. Chifukwa, ngakhale kukhala ndi kopi imodzi ya jini yosinthika ndikokwanira kuyambitsa zizindikirozi. Izi zimatchedwa autosomal dominant pattern mu mankhwala.

Komabe, nthawi zambiri, munthu amene alibe mbiri ya banja lake ya matendawa amatha kukhala ndi FFI. Izi zimachitika pamene kusintha kwa majini (de novo mutation) kwachitika. Izi zikutanthauza kuti vuto la majini limapangidwa mwatsopano m'thupi la munthuyo.

Kodi matendawa amatchedwa FFI ndi osowa bwanji?

FFI kwenikweni ndi matenda osowa kwambiri. Akuti munthu m'modzi kapena awiri okha pa anthu miliyoni ali ndi matendawa. Popeza FFI ndi ya majini, mabanja pafupifupi 50 mpaka 70 okha padziko lonse lapansi ndi omwe apezeka ndi kusintha kwa majini komwe kumayambitsa matendawa. Kotero mutha kuganiza momwe zimakhalira zachilendo.

Kodi matenda a FFI amakhudza bwanji thupi lathu?

FFI imayang'ana mwachindunji ubongo wathu ndi dongosolo lathu la mitsempha. Mapuloteni ena omwe amayambitsidwa ndi kusintha kwa majini amakhudza thalamus, gawo la ubongo wathu lomwe limayang'anira ntchito za thupi monga kugona.Zimasonkhana pamalo pomwe zanenedwa. Tangoganizirani kuti m'malo mopinda zovala zanu bwino ndikuziyika mu kabati yanu, mumaziphwanya, kuzipinda, ndikuzikanikiza. Patapita kanthawi, kabatiyo imakhala yodzaza ndi zovala kotero kuti simungathe kutseka, sichoncho? Umu ndi momwe mapuloteni opindika molakwikawa amasonkhana mu thalamus ya ubongo. Mapuloteni osonkhanitsidwawa amawononga ndikuwononga maselo a mitsempha.

Chizindikiro chachikulu cha izi ndi kusowa tulo , komwe kungakhudze kwambiri luso lanu la maganizo ndi magwiridwe antchito a thupi. Zizindikiro zimatha kukhala zoopsa komanso kukulirakulira pakapita nthawi.

Kodi zizindikiro za FFI ndi ziti?

Pali zizindikiro zingapo za FFI, zomwe zimayamba kuonekera pang'onopang'ono.

• Kusowa tulo kopitirira muyeso: Poyamba, zingawoneke ngati mukuvutika kugona, koma pambuyo pake simungagone konse.
• Kuthamanga kwambiri kwa mitsempha: Izi zikuphatikizapo zinthu monga kuthamanga kwa magazi, kugunda kwa mtima mofulumira kuposa momwe zimakhalira, komanso nkhawa kwambiri.
• Kutaya kukumbukira: Pang'onopang'ono umayamba kuiwala zinthu.
• Kuona zinthu m'maganizo: Kuona kapena kumva zinthu zomwe sizilipo kwenikweni.
• Kugwedezeka kapena kugwedezeka kwa minofu mosadzifunira (myoclonus): Kugwedezeka kapena kugwedezeka kwa mkono kapena mwendo mosadzifunira.

Zizindikiro za FFI nthawi zambiri zimayamba pakati pa zaka 20 ndi 70. Zaka zofala kwambiri za matendawa ndi pafupifupi zaka 40.

Chofunika kwambiri n'chakuti, zizindikiro zoyambirira za FFI nthawi zina zimatha kufanana ndi za matenda a dementia kapena matenda a Alzheimer's . Chifukwa chake, ngati muli ndi zizindikirozi, ndikofunikira kupita kwa dokotala kuti akakupatseni chithandizo choyenera. Monga momwe dzinalo likusonyezera, zizindikirozi zitha kukhala zoopsa pamoyo.

Kodi n’chiyani chimayambitsa FFI?

Chifukwa chachikulu cha FFI ndi kusintha kwa majini a PRNP . Jini iyi ya PRNP imapanga puloteni ya prion PrPC . Puloteni iyi ya PrPC imapezeka muubongo wathu, makamaka mu thalamus, gawo la ubongo lomwe limayang'anira ntchito za thupi monga kugona.

Pakakhala kusintha kwa majini a PRNP, ma amino acid omwe amapanga puloteni ya PrPC samalandira malangizo olondola opangira puloteniyi molondola. Zili ngati kupindika shati molakwika. Ngati simukudziwa momwe mungapindire T-sheti molondola, mumayipinda ndikuyiyika mu kabati. Pakapita nthawi, mulu wa T-sheti zomwe mwapinda molakwika zimapangitsa kuti zikhale zosatheka kutseka kabati. Mofananamo, puloteni ya PrPC yopindika molakwika iyi imasonkhana muubongo ndikuwononga maselo a mitsempha, zomwe zimayambitsa zizindikiro.

Kodi FFI ikhoza kukhala cholowa?

Inde, FFI ikhoza kutengera cholowa. Kusintha kwa majini kumenekuImafalikira mu autosomal dominant pattern. Izi zikutanthauza kuti ngakhale mutalandira jini yomwe yakhudzidwa kuchokera kwa kholo limodzi lokha, mutha kulandirabe matendawa.

Komabe, monga tanenera kale, FFI nthawi zambiri simakhala yodziyimira payokha, zomwe zikutanthauza kuti palibe aliyense m'banjamo amene adakhalapo ndi matendawa kale, ndipo amathanso kuchitika ngati kusintha kwa majini. Ngati zimenezo zitachitika, kusintha kwatsopano kumeneku kungapatsiridwe kuchokera kwa inu kupita kwa ana anu amtsogolo.

Ngati mukuyembekezera mwana ndipo mukufuna kudziwa za chiopsezo chanu chopatsira mwana wanu matenda a majini, ndi bwino kulankhula ndi dokotala wanu kuti mudziwe zambiri zokhudza kuyezetsa majini .

Kodi n’chiyani chimachititsa kuti anthu omwe ali ndi FFI amwalire?

Chifukwa chachikulu cha imfa mwa anthu omwe ali ndi FFI ndi kuwonongeka kwa ubongo ndi dongosolo lamanjenje. Kuwonongeka kumeneku, komwe kumachitika chifukwa cha kuchuluka kwa mapuloteni a prion mu thalamus, kumayambitsa zizindikiro zoopsa monga kusowa tulo komanso kusokonezeka kwa maganizo.

N’chifukwa chiyani tulo ndi lofunika kwambiri kwa ife?

Kugona mokwanira kumatithandiza kukhala athanzi. Kugona bwino usiku kumathandiza kuti thanzi lathu la maganizo ndi thupi likhale labwino. Mukagona, ubongo wanu umagwira ntchito limodzi ndi thupi lanu, zomwe zimakupatsani mwayi wodzuka muli ndi mphamvu komanso mphamvu m'mawa. Ngati simugona mokwanira, ubongo wanu umataya mphamvu zake zolimbitsa thupi. Zimakhudza momwe mumaganizira komanso momwe thupi lanu limagwirira ntchito. Ubongo wanu ukakhala kuti sukugwira ntchito 100%, umakhudza momwe thupi lanu limamvera komanso momwe limachitira zinthu.

Anthu omwe ali ndi FFI sangathe kugona, zomwe zimasokoneza kwambiri ntchito ya ubongo wawo. Izi zingayambitse zotsatirapo zoyipa zomwe zingawopseze moyo wawo komanso kuwononga thanzi lawo lonse.

Kodi FFI imapezedwa bwanji?

Dokotala wanu adzazindikira FFI mwa kuwunikanso zizindikiro zanu ndikuchita mayeso angapo kuti atsimikizire matendawa. Mayeso awa angaphatikizepo:

• Polysomnography: Iyi ndi mayeso okhudzana ndi tulo.
• Electroencephalogram (EEG): Izi zimayesa mawonekedwe a mafunde a ubongo.
• Kusanthula kwa Cerebrospinal Fluid (CSF): Kuyesaku kumafufuza madzi mu ubongo ndi msana kuti adziwe matenda omwe amakhudza ubongo ndi msana.
• Kuyezetsa majini kuti mudziwe jini yomwe imayambitsa zizindikiro.
• Mayeso ojambulira: Izi zitha kuphatikizapo MRI (Magnetic Resonance Imaging) , CT scan (Computed Tomography scan) , kapena PET scan (Positron Emission Tomography scan) .
• Kuchuluka kwa Magazi (CBC) , mayeso a chiwindi , ndi kuchuluka kwa magazi m'thupiMayeso a labotale monga.

Kodi FFI imachiritsidwa bwanji?

Cholinga chachikulu cha chithandizo cha FFI ndikuchepetsa zizindikiro ndikukupangitsani kukhala omasuka momwe mungathere. Njira zina zochiritsira, makamaka mankhwala, zingathandize kuchepetsa zizindikiro kwakanthawi. Komabe, pakadali pano palibe mankhwala a FFI.

Njira zochiritsira zingaphatikizepo:

• Kupereka mankhwala oti agone tulo tofa nato (monga (gamma-hydroxybutyrate) , (phenothiazines) ).
• Chiritsani kupweteka kwa minofu mwa kupereka mankhwala monga clonazepam .
• Kupereka mavitamini ( B6, B12, iron, folic acid ).
• Ngati pali mankhwala omwe akuwonjezera zizindikiro, sinthani mlingo wawo kapena muwasiye.
• Chithandizo cha maganizo: Kupereka chithandizo chamaganizo kwa wodwala ndi banja lake.
• Chisamaliro cha odwala ovutika kwambiri / Chisamaliro cha anthu ovutika: Kupangitsa kuti masiku omaliza a wodwalayo akhale omasuka.

Kafukufuku akupitilizabe kupeza njira zatsopano zothandizira anthu omwe ali ndi FFI. Kafukufuku wina adapeza kuti mankhwala opha tizilombo otchedwa doxycycline awonetsa kupambana pang'ono pakutalikitsa moyo wa odwala a FFI.

Ndi mankhwala ati omwe sagwira ntchito pochiza FFI?

Mankhwala ena omwe amakuthandizani kugona, monga mankhwala owonjezera a melatonin , amagwira ntchito kwakanthawi kokha pochiza FFI. Kafukufuku wapeza kuti mankhwala ochepetsa ululu , monga barbiturates ndi benzodiazepines , si mankhwala othandiza.

Kodi munthu amene ali ndi FFI angayembekezere chiyani?

Palibe mankhwala a FFI. Mukapezeka ndi matendawa, chithandizo chimayang'ana kuti mukhale omasuka komanso kuti musamavutike ndi zizindikiro zanu. Izi zikutanthauza kuti chisamaliro chochepetsa ululu ndicho chofunikira kwambiri. Munthu amene ali ndi FFI amakhala ndi moyo wautali - makamaka zizindikiro zikayamba, nthawi yopulumuka imatha kukhala kuyambira miyezi ingapo mpaka zaka ziwiri. Matendawa amakula pang'onopang'ono.

Nthawi ngati izi, ndikofunikira kwambiri kuti mabanja azisonkhana pamodzi, osaganizira za chisamaliro chomwe wodwalayo amafunikira, komanso thanzi lawo la maganizo, kukonzekera imfa yadzidzidzi ya wokondedwa wawo, ndikupempha thandizo lamankhwala ngati pakufunika kutero.

Kodi FFI ingapewedwe?

FFI singathe kupewedwa. Popeza imayamba chifukwa cha kusintha kwa majini, nthawi zina imatha kuchitika yokha, popanda wachibale aliyense amene wakhalapo ndi matendawa kale.

Kodi muyenera kupita kwa dokotala liti?

Ngati mukuvutika kugona, makamaka masana, ndipo sikukutha, muyenera kuonana ndi dokotala. Zizindikiro za FFI ndi monga matenda a dementia ndi matenda a Alzheimer's.Ngati mukuvutika kugona, monga kuiwala zinthu komanso kulephera kuwongolera magwiridwe antchito a thupi, chifukwa cha matenda monga khunyu, muyenera kuwonana ndi dokotala.

Ndi mafunso ati omwe ndiyenera kufunsa dokotala wanga?

• Kodi mungalimbikitse chisamaliro cha hospice panthawiyi pamene matendawa akupezeka?
• Kodi zizindikiro zake ndi zoopsa bwanji?
• Kodi pali njira zina zochiritsira zomwe zingathandize kuchepetsa zizindikiro?

Pomaliza, zomwe muyenera kukumbukira (Uthenga Wopita Nawo Kunyumba)

Kupezeka ndi matenda a FFI kungakhale kovuta kwambiri, makamaka pamene zizindikiro zikuipiraipira pang'onopang'ono pakapita miyezi ingapo. Komabe, simuli nokha panthawiyi. Funani thandizo kwa madokotala anu, abale anu, anzanu, ndi akatswiri azaumoyo wamaganizo. Chisamaliro cha opuwala ndi njira zomwe zilipo pano zingathandize kuchepetsa zizindikiro zanu kwakanthawi. Kafukufuku akupitilira kuti apeze mankhwala othandiza kwambiri omwe angawonjezere miyoyo ya anthu omwe ali ndi matendawa. Chifukwa chake, musataye mtima.

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