Malungo ndi ziphuphu pakhungu pafupipafupi? Tiyeni tikambirane za CAPS (Cryopyrin-Associated Periodic Syndromes)

Tangoganizani, popanda chifukwa chomveka, nthawi zonse mumamva malungo, kupweteka kwa mafupa, ndi ziphuphu zazing'ono m'thupi lanu lonse. Izi zimatha pakatha masiku awiri kapena atatu, kenako n’kubwereranso. Ngati chinthu chonga ichi chichitika kwa inu kapena mwana wanu, chifukwa chake chingakhale matenda osowa omwe anthu ambiri sanamvepo. Lero tikulankhula za matenda amodzi otere, CAPS.

Kodi CAPS imatanthauza chiyani?

Mwachidule, matenda a Cryopyrin-Associated Periodic Syndromes, kapena CAPS, ndi gulu la matenda osowa kwambiri omwe amakhudza chitetezo chathu chamthupi. Nthawi zambiri, chitetezo chathu chamthupi chimatiteteza ku majeremusi ndi matenda ena. Komabe, mu mkhalidwe uwu wa CAPS, chifukwa cha vuto la chitetezo chamthupi, umayamba kuukira thupi lathu. Izi zimatchedwa 'matenda odzipha okha' mu sayansi ya zamankhwala. Izi zimayambitsa kutupa kosafunikira m'thupi, zomwe zimayambitsa zizindikiro monga ziphuphu pakhungu, malungo, kupweteka kwa mafupa ndi kutupa, ndi maso ofiira.

Pali mitundu itatu ikuluikulu ya ma CAPS. Tiyeni tiwone zomwe zili.

Kodi n’chiyani chimayambitsa ma CAPS?

Chifukwa chachikulu cha izi ndi kusintha kwa jini m'thupi lathu. Kunena zoona, matendawa amayamba chifukwa cha kusintha kwa jini yotchedwa `NLRP3` .

Ganizirani za jini iyi ya `NLRP3` ngati chithunzi chomwe chimalangiza thupi lathu kupanga puloteni yotchedwa `cryopyrin`. Nthawi zambiri, puloteni iyi ya `cryopyrin` imathandiza thupi lathu kupanga chinthu chotchedwa `interleukin-1 (IL-1)`, chomwe chimathandiza thupi lathu kulimbana ndi matenda.

Koma chifukwa chakuti jini ya `NLRP3` mwa munthu yemwe ali ndi CAPS yasintha, puloteni ya `cryopyrin` sigwiranso ntchito bwino. Imapereka chizindikiro nthawi zonse ku thupi kuti lipange `IL-1` yochuluka. Kupanga kwambiri kwa `IL-1` kumeneku ndi komwe kumayambitsa kutupa kosafunikira m'thupi komanso kumayambitsa zizindikiro za CAPS.

Matendawa angachitike ngati mwana walandira jini yosinthika kuchokera kwa kholo limodzi lokha, kaya mayi kapena bambo. Nthawi zina, jiniyo imatha kusintha yokha m'mimba, popanda makolo.

Kodi zizindikiro zake ndi ziti?

Chizindikiro chachikulu komanso chodziwika bwino cha CAPS ndi ziphuphu zomwe zimawoneka ngati matuza ang'onoang'ono, osayabwa thupi lonse . Izi nthawi zambiri zimawonekera pobadwa. Anthu ena amakhala ndi zilondazi nthawi zonse, koma zimakhala zoopsa kwambiri akamatuluka. Zizindikiro zina zimasiyana malinga ndi mtundu wa CAPS womwe muli nawo.

Zizindikiro zomwe zingawonekere mu FCAS

Zizindikiro zimenezi nthawi zambiri zimakhalapo kwa masiku 1-2.

• Malungo
• Maso otupa
• Kuzizira
• Kupweteka kwa mafupa
• Kusanza
• Mutu

Zizindikiro zomwe zimawonedwa mu MWS

Zizindikiro zimenezi zimabwera ndi kutha. Zitha kukhalapo kwa masiku 1-3 nthawi imodzi.

• Kutupa pakhungu
• Malungo ndi kuzizira
• Kufiira kwa maso
• Kupweteka kwa mafupa
• Mutu waukulu
• Kutaya kumva (ana omwe ali ndi MWS angataye kumva konse kapena gawo lina lake akamakula)
• Kuwawa kwam'mimba

Zizindikiro zomwe zimawonedwa mu NOMID

Izi ndi zizindikiro zoopsa kwambiri.

• Mutu waukulu
• Maso otupa
• Kupweteka kwa mafupa ndi kutupa
• Kusanza
• Kutaya masomphenya
• Kutaya kumva

Kodi mungazindikire bwanji matendawa?

Mukapita kwa dokotala wanu ndi zizindikiro izi, iye adzakufunsani kaye za mbiri yanu yachipatala komanso ya banja lanu. Kenako, adzakuyesani ndipo, ngati pakufunika, angakutumizireni kuti mukayesedwe, monga:

• Mayeso a magazi ndi mkodzo: Mayesowa amayang'ana kuchuluka kwa maselo oyera amagazi ndi mapuloteni a C-reactive, omwe ndi zizindikiro za kutupa, komanso mapuloteni ochulukirapo mu mkodzo.
• Kufufuza khungu: Chitsanzo chaching'ono kwambiri cha khungu lanu chimatengedwa ndikuyang'aniridwa ndi maikulosikopu.
• Kuyesa majini: Kuyesa kumeneku kungatsimikizire ngati muli ndi kusintha kwa majini anu a NLRP3. Komabe, nthawi zina anthu omwe ali ndi CAPS amatha kukhala ndi majini awa nthawi zonse.
• Kuyesa maso ndi khutu: Kuyesa kumeneku kumachitika kuti muwone ngati maso anu kapena kumva kwanu kwakhudzidwa.
• Kuboola kwa msana: Madzi ochepa a msana amatengedwa kuchokera ku msana ndikuwunikidwa ngati pali zizindikiro za kutupa.
• Kujambula kwa MRI: Izi zitha kujambula zithunzi zatsatanetsatane za kapangidwe ka ubongo ndi makutu kuti zione ngati pali kusintha kulikonse.

Mafunso oti mufunse dokotala

Ndi bwino kulemba mafunso ena omwe muli nawo papepala mukapita kukaonana ndi dokotala. Mwanjira imeneyi, simudzaiwala chilichonse. Mutha kufunsa mafunso ngati awa:

• Kodi ine (kapena mwana wanga) ndili ndi ma CAPS amtundu wanji?
• Kodi matenda anga angayambitse zizindikiro zotani?
• Kodi ndi mankhwala ati omwe mumalimbikitsa? Kodi angandithandize bwanji?
• Kodi pali zotsatirapo zilizonse za mankhwalawa?
• Kodi ndingachite chiyani kunyumba kuti ndichepetse zizindikiro?
• Ndi mayeso ena ati omwe ndiyenera kuchita? Kodi ndiyenera kuwayesa liti?
• Kodi ndikufunika kuonana ndi katswiri wa majini?

Kodi mankhwala ake ndi otani?

Ndikofunikira kwambiri kuyamba chithandizo cha matendawa mwachangu, chifukwa maso, makutu, mafupa, kapena ubongo zikawonongeka, zimakhala zovuta kwambiri kuzibwezeretsa m'malo abwino.

Chithandizo chachikulu ndi mankhwala omwe amaletsa kupanga mopitirira muyeso kwa chinthu chotchedwa IL-1 m'thupi. Mankhwalawa amaletsa kutupa.

• Anakinra (Kineret)
• Canakinumab (Ilaris)
• Rilonacept (Arcalyst)

Kuwonjezera pa izi, dokotala wanu angakulangizeninso njira zina zochiritsira, monga:

• Kuvala ma splints oteteza kupweteka ndi kutupa kwa mafupa
• Kuchiza thupi
• Mankhwala monga NSAIDs, steroids, kapena methotrexate kuti achepetse malungo ndi kutupa
• Zipangizo zothandizira kumva kwa anthu ovutika kumva
• Opaleshoni ngati mafupa awonongeka

Kodi mungadzisamalire bwanji?

Pamodzi ndi chithandizo, kusintha pang'ono moyo wanu kungathandize kwambiri kuthetsa zizindikiro.

• Pewani chimfine: Ngati muli ndi vuto ngati la FCAS, nyengo yozizira ingakulitse zizindikiro zanu. Choncho yesetsani kupewa zinthu monga kukhala m'zipinda za AC ndi kukhala m'malo ozizira.
• Chepetsani kupsinjika maganizo: Zinthu monga kusinkhasinkha, kuchita masewera olimbitsa thupi opumira kwambiri, ndi yoga zingathandize kwambiri pa izi.
• Moyo wathanzi:
• Gonani bwino.
• Idyani chakudya chopatsa thanzi.
• Pewani kusuta fodya, mowa, ndi zakudya zopangidwa kale.