Mwana wako haasi kufema zvakanaka here? Ngatidzidzei nezve Congenital Central Hypoventilation Syndrome (CCHS)!

Dzimwe nguva, vabereki vanonetseka zvishoma pavanoona shanduko diki mumafemerero evana vavo, handiti? Zvakajairika kunzwa kutya zvishoma, kunyanya kana mwana wako achiita seari kudzipwa kana kufema zvakanyanya pavanenge vakarara. Nhasi, tichataura nezvechirwere chisingawanzoitika icho munhu wese anofanira kuziva. Vanachiremba vanodana ichi kuti `Congenital Central Hypoventilation Syndrome`, kana kuti tichachidana kuti `(CCHS)` muchidimbu.

Chii chinonzi CCHS? Ngatinzwisisei chaizvo.

Zvichitaurwa zviri nyore, `(CCHS)` chirwere chisingawanzoitika chinowanikwa pakuzvarwa uye chinokanganisa muviri wese. Chinhu chikuru ndechekuti muviri haufeme zvakakwana, kana kuti kufema kunodzikira. Dambudziko iri rinonyanya kuoma panguva yekurara. Vanachiremba vanoti izvi `(Hypoventilation)` (hypoventilation). Fungidzira, patinofema kazhinji, mapapu edu anofema uye anogumira kuti atore okisijeni uye abudise carbon dioxide. Asi mumunhu ane `(CCHS)`, kufema uku, kunyanya kufema kunoitwa nemuviri, hakuitike nemazvo. Nekuda kweizvozvo, huwandu hweokisijeni muropa hunoderera uye huwandu hwecarbon dioxide hunowedzera zvisina kufanira. Izvi zvinogona kukanganisa masisitimu mazhinji mumuviri.

Mamiriro aya ekuti `(CCHS)` anokanganisa sisitimu yetsinga yemuviri wedu `(Autonomic Nervous System)` . Iye zvino ungave uchishamisika kuti iyi sisitimu yetsinga yemuviri inoitei. Fungidzira, patinotyaira mota, tinoita zvinhu zvakaita sekuchinja `giya` uye `bhureki` nemaune. Asi zvimwe zvinhu mumuviri wedu zvinongoerekana zvaitika, uye hatitombofungi nezvazvo. Sekufema (mapapu edu anoshanda kunyangwe patinorara!), kugaya chikafu, kurova moyo wedu, kudzora tembiricha yemuviri wedu, kudikitira, uye kusimba kweiris kana chiedza chichirova maziso edu. Zvese izvi zvinodzorwa nesisitimu yetsinga yemuviri inozviita yoga. Kana tiine `(CCHS)`, maitiro aya ega, anokosha anokanganiswa. Nokudaro, pamusoro pekufema, matambudziko anogonawo kuitika muzvinhu zvakaita se:

• Kudzora BP.
• Basa rematumbu.
• Kurova kwemoyo.
• Kudzora tembiricha yemuviri.

Dambudziko iri rinonzi ``(CCHS)`` nemamwe mazita akati wandei. Chiremba wako anogona kushandisa rimwe remazita aya, saka zvakanaka kuziva nezvawo:

• "Kufema Kwemhepo Kwekuzvarwa Nako"
• "Kukundikana Kwekuzvarwa Nako Kwekudzora Kuzvitonga"
• "Kutadza Kuberekwa Kwekufema Kwemweya"
• 'Haddad Syndrome'
• Dzimwe nguva inonziwo 'Ondine Syndrome', 'Ondine-Hirschsprung Disease', kana kuti 'Ondine's Curse'.

CCHS yakajairika zvakadii?

Kutaura zvazviri, CCHS chirwere chisingawanzoitiki.Fungidzira, nhamba diki chete yezviitiko, zvingangoita chiuru kusvika chiuru nemazana maviri, zvakawanikwa pasi rose. Zvisinei, masayendisiti anotenda kuti dzimwe nguva, nekuda kwezvirwere zvisingazivikanwe zve `(CCHS)`, pane fungidziro yekuti dzimwe dzerufu rwevacheche, kureva kuti, `Sudden Infant Death Syndrome (SIDS),` dzinogona kuitika. Izvi zvinoreva kuti, kunyangwe zvingave `(SIDS)`, chikonzero chikuru chingavewo `(CCHS)`. Saka, zvakakosha kuziva izvi.

Ndezvipi zviratidzo zveCCHS? Unozviziva sei?

Zviratidzo zvekutanga zveCCHS zvinowanzoonekwa pakuzvarwa kana mukati memwedzi mishoma yekutanga yehupenyu. Izvi ndizvo zviratidzo zvikuru zvinogona kuonekwa:

• Kuchinja ruvara rwebhuruu rwemiromo kana ganda (Cyanosis): Izvi zvakafanana nekushaikwa kweokisijeni. Izvi zvinonyanya kuonekwa kana mwana akarara.
• Kufema zvisina kurongeka panguva yekurara (Hypoventilation): Kufema kunogona kunzwa kushoma kana kukurumidza. Dzimwe nguva zvinogona kutoita sekunge kufema kunomira kwenguva yakati.

Zvakakosha: Kana ukaona zviratidzo izvi, unofanira kuona chiremba nekukurumidza.

Asi dzimwe nguva, kana chirwere chisina kunyanya kuipa, zviratidzo izvi zvinogona kuoneka gare gare muhupenyu. Kana, zvimwe zviratidzo zvinogona kutanga. Tarisa kana mwana wako aine chimwe chezvizvi:

• Zvisina kumira zvakanaka mumaziso: Semuenzaniso, maitiro anoita iris kuchiedza neruzha anogona kudzikira, maziso anogona kupatsanurwa (strabismus), kana kuti maonero aunoita uri kure anogona kuchinja (kuchinja maonero ekudzika).
• Kuderedza marwadzo: Kunyangwe kukuvara kudiki kunogona kusanyanya kurwadza.
• Kushaikwa kwehomoni yekukura: Kukura kwemwana kunogona kunonoka kupfuura vamwe vana.
• Kungodikitira zvakanyanya pasina chikonzero.
• Matambudziko emudumbu (GI): Kudzoka kwemvura mumuviri, kuvimbiwa nguva nenguva, uye dzimwe nguva kuvharika kwematumbu madiki kana kuvharika kwematumbu makuru. Chirwere cheHirschsprung (CCHS) chinogonawo kuonekwa neCCHS.
• Tembiricha yemuviri yakaderera: Muviri unogona kunzwa kutonhora nguva dzese.
• Matambudziko etsinga: Zvinhu zvakaita sekurema kwekudzidza.
• Kuomerwa nekudzora kurova kwemoyo neBP.

Sei CCHS iyi ichiitika? Chii chinokonzera?

Zvakanaka, ngationei kuti sei chirwere ichi chinonzi `(CCHS)` chichiitika. Chikonzero chikuru cheizvi kuchinja kwemajini edu. Jini iri rinonzi `PHOX2B` (Fox-2-B) gene.Jini iri re'PHOX2B' rakakosha zvikuru pakugadzira puroteni inobatsira masero etsinga, kunyanya masero ehurongwa hwetsinga hwe autonomic hwatakataura nezvahwo, kukura zvakanaka paanenge achikura mudumbu ari mwana ari mudumbu.

Kune vanhu vazhinji vane CCHS, kuchinja kwemajini uku ikuchinja kutsva, kunoitika nguva nenguva. Izvi zvinoreva kuti kuchinja uku hakugamuchirwi kubva kuna amai kana baba, asi kuchinja kutsva mumuviri wemwana. Zvisinei, muzviitiko zvishoma, kunogona kupfuudzwa kubva kuchizvarwa kuenda kune chimwe. Izvi zvinoreva kuti mumwe wevabereki ane kuchinja kwemajini uku uye mwana anogona kunge akakugara nhaka.

Ungaziva sei kuti une CCHS? (Kuongororwa)

Nzira chete uye inonyanya kujeka yekusimbisa kana uine CCHS ndeyekuita bvunzo dzemajini kuti uone kana paine shanduko mujini rePHOX2B. Iyi ndiyo nzira chete iyo vanachiremba vanogona kuva nechokwadi che100% kana uine chirwere ichi kana kwete.

Zvisinei, vanachiremba vanogona kuita mimwe miedzo yakati wandei kuti vanzwisise mhedzisiro yechirwere ichi pamuviri uye kuti vawane chikonzero chezviratidzo zvacho. Semuenzaniso:

• Kuongororwa kweRopa: Tarisa zvinhu zvakaita se oxygen ne carbon dioxide mumuviri.
• Kana uine zviratidzo zvematambudziko ekugaya chikafu, ita kuti vaongororwe necolonoscopy , pamwe ne biopsy .
• `Echocardiogram` yekutarisa mashandiro emoyo.
• Kuongororwa kwemifananidzo kwakadai seX-ray, CT scan, kana MRI kuti vatarise mukati mechipfuva nedumbu kuti vaone kana paine zvipingamupinyi.
• Kuongororwa kwemaziso kuti uone mamiriro emaziso.
• Zvidzidzo zveKurara : Iyi bvunzo inokosha zvikuru. Muizvi, mwana anoradzikwa mukamuri rakakosha muchipatara uye zvinhu zvakasiyana-siyana zvakaita semafambiro ekufema, kurova kwemoyo, uye mwero weokisijeni zvinoongororwa nemichina.

Ko CCHS inogona kurapwa zvizere here?

Mubvunzo unobvunzwa nevanhu vazhinji uye unofungidzirwa mupfungwa dzavo ndewekuti `(CCHS)` inogona here kurapwa zvizere. Kutaura chokwadi, pari zvino hapana mushonga chaiwo we`CCHS`. Idambudziko rehupenyu hwese. Asi, musazvidya mwoyo. Kurapa kwakagadzirirwa kudzora zviratidzo, kuderedza matambudziko angangoitika, uye kubatsira murwere kurarama zvakachengeteka, zvakanaka, uye zvakanaka sezvinobvira.

Kurapwa kwemunhu ane CCHS ndekupi?

Vanhu vazhinji vane CCHS vanoda rubatsiro rwekufema kwehupenyu hwavo hwese.Inodiwa. Izvi zvinonzi `Ventilator`. Vamwe vanhu vanoida zuva rese, nguva dzese. Asi vamwe vanoida chete pavanenge vakarara. `(Ventilator)` iyi inobatsira muviri kufema nenzira yakati, sezvinodiwa. Izvi zvinogona kuchengetedza mwero weokisijeni muropa zvakanaka uye kumisa mwero wecarbon dioxide kuti usawedzera.

Pamusoro pezvo, kune mamwe marapirwo anotsigira. Marapirwo aya anosiyana zvichienderana nezviratidzo zvemunhu wega wega:

• Matambudziko ekuona anogona kugadziriswa nekushandisa magirazi, magirazi ekuona, kana kuvhiyiwa. Izvi zvinogonawo kubatsira kudzivirira maziso kubva kuchiedza chakawandisa.
• Kurapa neGrowth Hormone (GHT) yekukura kwenguva pfupi.
• Mishonga yekuderedza zviratidzo zvemudumbu (semuenzaniso, `Reflux`, `Constipation`).
• Dzora BP uye kurova kwemoyo uchishandisa mishonga kana dzimwe nzira dzekurapa .
• Kuongororwa kwezvirwere nguva dzose kunoitwa kuti zvionekwe zvimwe zvinetso zvehutano zvine chekuita nazvo nguva isati yakwana.

Maitiro aya ekurapa angada rutsigiro rwenyanzvi dzakasiyana . Fungidzira, kune nyanzvi yakakodzera matambudziko emunhu wega wega. Zvakakosha kuti nyanzvi dzakasiyana dzakadai dzibatane pamwe chete dzibatane sechikwata. Nekuti `(CCHS)` hachisi chinhu chinongokanganisa chikamu chimwe chete chemuviri. Saka kana munhu wega wega achishandisa hunyanzvi hwake kugadzirisa matambudziko akasiyana-siyana emwana, kutarisirwa kunoitwa mwana kunenge kwakakwana. Semuenzaniso:

• Vanachiremba vemwoyo vezvirwere zvemwoyo.
• Matambudziko enzeve, mhino, nepahuro anorapwa nenyanzvi dze ENT .
• Nyanzvi dzeEndocrinologists inyanzvi mu endocrine glands dzematambudziko ane chekuita nemahormone.
• Vanachiremba vezvekudya inyanzvi dzezvirwere zvemudumbu zvematambudziko emudumbu.
• Vanachiremba vetsinga inyanzvi mumhedzisiro yehuropi nekudzidza.
• Vanachiremba vemaziso vanorapa zviratidzo zvine chekuita nemaziso.
• Vanachiremba vekutanga (vakadai sevanachiremba vevana).
• Vanachiremba vemapapu inyanzvi dzematambudziko ekufema .
• Nyanzvi dzePathology dzeKutaura neMutauro.
• Vashandi veSocial (vanopa mhuri rutsigiro rwepfungwa nerwemagariro evanhu).

Pane nzira yekudzivirira CCHS here?

Kazhinji patinotaura nezvechirwere, tinotaurawo nezvenzira dzekuzvidzivirira kubva kwachiri. Zvisinei, panyaya ye `(CCHS)`, hapana nzira yakawanikwa yakasimbiswa yekudzivirira kuitika kwacho.Nekuti inonyanya kukonzerwa nechikonzero chemajini (kuchinja kwemajini e'PHOX2B'), zvinonetsa kudzivirira. Zvisinei, kana mumwe munhu mumhuri aine '(CCHS)', zvakanaka kutaura nachiremba nezvemajini ekupa mazano uye kuongororwa kwevamwewo.

Hupenyu huchava hwakaita sei neCCHS? Zvinhu zvakakosha zvekuziva

Nguva yekurarama uye kuremara kunogona kuitika kumunhu anorarama neCCHS zvinogona kusiyana zvakanyanya. Zvinoenderana nezvinhu zvakaita sekuoma kwechirwere, nguva inotorwa kutanga kurapwa, uye kubudirira kwekurapwa .

Zvisinei, kana vakaongororwa nekukurumidza uye vakarapwa nemazvo uye nguva dzose , vanhu vane CCHS isina kusimba vanogona kurarama hupenyu hunobudirira, hunobudirira, uye hunofadza. Vanogona kuenda kuchikoro, kutamba, uye kunyange kushanda kana vakura. Zvisinei, kana chirwere chacho chakakomba, kana kurapwa kukasapihwa nemazvo kana kunonoka, kuremara kwakakura, matambudziko ehutano, uye kupfupikiswa kwenguva yekurarama zvinogoneka. Nokudaro , kuongororwa nenguva uye kurapwa nguva dzose kwakakosha zvikuru.

Izvi zvakakosha kurangarira: Vanhu vane CCHS vane mukana wekuwana mamwe marudzi emabundu etsinga. Nokudaro, zvakakosha kugara uchiongororwa mhando idzi dzemabundu. Kana akaonekwa nekukurumidza, zvinova nyore kurapa. Unofanira kungwarira zvikuru nezvemhando idzi dzemabundu:

• Neuroblastoma
• Ganglioneuroblastoma
• `Ganglioneuroma`

Saka, musaregeredza kuramba muchiongororwa sezvakataurwa nachiremba.

Mibvunzo inokosha yekubvunza chiremba wako nezveCCHS

Kana iwe kana mumwe munhu wemumhuri mako akaonekwa kuti ane chirwere cheCCHS, iva nechokwadi chekubvunza chiremba wako mibvunzo iyi. Iyi mibvunzo ichakubatsira kunzwisisa chirwere chacho uye kuronga matanho ako anotevera:

• "Ko `(CCHS)' yangu/mwana wangu yakakomba zvakadii?"
• "Ndeapi masisitimu emuviri (semuenzaniso, kufema, moyo, ura) anokanganiswa nemamiriro emwana wangu/wangu e `(CCHS)`?"
• "Ndeapi manyanzvi andichada/atichada kuona? Ndinofanira kuvaona kangani?"
• "Ini/mwana wangu ndinoda `Ventilator` here? Kana zvakadaro, ndinofanira kuishandisa nguva dzese, kana kuti pandinenge ndakarara chete?"
• "Ndinofanira kuongororwa kangani kuti ndiongorore maziso angu, moyo, mapapu, kugaya chikafu, nedzimwe nhengo dzemuviri?"
• "Ndinofanira kuongorora kangani mabundu andambotaura?"
• "Zvakanaka here kuti mhuri yangu yese (semuenzaniso vanun'una, vabereki) vaongororwewo majini?"

Pakupedzisira, zvinhu zvekurangarira (Mharidzo Yekuenda Kumba)

Kunyange hazvo CCHS iri chirwere chinotyisa uye chisingawanzoitiki, chinogona kudzorwa kana ukaziviswa zvakanaka uye ukatanga kurapwa nenguva. Chinhu chikuru ndechekutsvaga rubatsiro rwechiremba nekukasika paunongoona zviratidzo, kunyanya kana ukaona chimwe chinhu chisingawanzoitika mukufema kwemwana wako, senge cyanosis. Chinhu chakakosha hakusi kutya, asi kuita chimwe chinhu nekukurumidza.

Kurarama nechirwere ichi kunogona kunetsa mhuri, ichokwadi. Asi yeuka, hausi wega. Vanachiremba, vanamukoti, varapi, nevamwe vashandi vehutano varipo kuti vakubatsire nekukutungamira iwe nemwana wako. Nekutarisira kwakakodzera kwehutano, kutarisirwa kwemhuri nerudo, uye mafungiro akanaka, mwana ane CCHS anogona kurarama hupenyu hunofadza, hwakajairika sezvinobvira. Usatya kubvunza mibvunzo, kutsvaga ruzivo, uye kupa mwana wako zvakamunakira.

` CCHS, Congenital Central Hypoventilation Syndrome, congenital respiratory distress syndrome, autonomic nervous system, PHOX2B gene, respiratory support, baby baby health, cyanosis, genetic testing