Ukuthini yi-Malignant Hyperthermia? Izimpawu, Izimbangela, & Ukwelashwa

I-Malignant hyperthermia iyisimo esingavamile kodwa esibucayi esingase sibe khona uma usebenzisa imithi ethile yokunciphisa ubuhlungu ngesikhathi sokuhlinzwa. Kwenzeka uma imisipha yakho iphendula ngendlela eyingozi kule mithi, okubangela ukuthi izinga lokushisa lomzimba wakho likhuphuke ngokushesha futhi imisipha yakho ibe lukhuni.

Lesi simo sihlupha cishe umuntu o-1 ku-5,000 kuya ku-1 ku-50,000 abantu abamukela ukunciphisa ubuhlungu okuvamile. Nakuba kuzwakala kukhulu, kuphatheka ngokuphelele uma kubanjwa ngokushesha, futhi amagumbi okuhlinzwa anamuhla akulungele kahle ukuphatha ngokuphephile.

Ziyini izimpawu ze-Malignant Hyperthermia?

Izimpawu ze-malignant hyperthermia ziveleka ngokushesha ngesikhathi noma ngokushesha ngemva kokuba ukhombiswe imithi yokunciphisa ubuhlungu. Iqembu lakho lezokwelapha liqaphela lezi zimpawu ngokucophelela kuwo wonke umkhuba ohilela imithi ebangela leli simo.

Izimpawu zokuqala ezivamile zihlanganisa:

• Ukuphuma okusheshayo kwizinga lokushisa lomzimba (ngezinye izikhathi kufinyelela ku-106°F noma ngaphezulu)
• Ukuqina kwemisipha, ikakhulukazi emiphakathini yemisipha
• Ukuqhubuka okusheshayo kwenhliziyo nokuqhubuka okungavamile kwenhliziyo
• Ukuphuma kwegazi eliphezulu
• Ukuqhubuka okusheshayo, okungavamile kokuphefumula
• Ukukhishwa okukhulu kokubila
• Umbala wesikhumba oqoshiwe noma obomvu

Ezimweni ezinzima kakhulu, ungase ube nokuwohloka kwemisipha, izinkinga zezinso, noma ukuguquka okubi kwezinto zegazi. Izindaba ezinhle ukuthi amaqembu asemagumbini okuhlinzwa aqeqeshwe ukubona lezi zimpawu ngokushesha futhi athathe isinyathelo ngokushesha.

Yini ebangela i-Malignant Hyperthermia?

I-Malignant hyperthermia ibangelwa yisimo sezakhiwo ezihlanganisa imisipha ekulawuleni indlela amaseli emisipha aphatha ngayo i-calcium. Uma ukhombiswe imithi ethile yokunciphisa ubuhlungu, umehluko wezakhiwo ezihlanganisa imisipha uqalisa impendulo engavamile yemisipha.

Izinto ezibangela leli simo yi imithi ethile yokunciphisa ubuhlungu:

• Igesi yokunciphisa ubuhlungu (njenge-sevoflurane, isoflurane, ne-halothane)
• I-Succinylcholine (umthambisi wemisipha)

Uzuza lobu buthakathaka bezakhiwo ezihlanganisa imisipha kubazali bakho. Kuhlotshaniswa nokuguqulwa kwezakhiwo ezihlanganisa imisipha ekulawuleni ukukhishwa kwe-calcium emaseleni emisipha, ikakhulukazi izakhiwo ezihlanganisa imisipha ze-RYR1 ne-CACNA1S. Uma lezi zakhiwo ezihlanganisa imisipha zingasebenzi kahle, ukukhombiswa imithi ebangela leli simo kubangela i-calcium ukuba igcwale emaseleni emisipha ngokulawuleki.

Lesi simo sezakhiwo ezihlanganisa imisipha sihamba emindenini, kodwa ukuba nesakhiwo ezihlanganisa imisipha akusho ukuthi uzoba naleli phendulo. Abanye abantu bayithwala le sakhiwo ezihlanganisa imisipha kodwa abazange babe nazimpawu, kanti abanye bangase babe nemiphumela emibi ekukhombiseni kwabo kokuqala imithi ebangela leli simo.

Nini okufanele ubheke udokotela nge-Malignant Hyperthermia?

Uma uhlelwe ukuhlinzwa, kufanele ukhulume nodokotela wakho onciphisa ubuhlungu ngaphambi kwesikhathi uma unembali yomndeni yezinkinga zokunciphisa ubuhlungu. Le ngxoxo iyasiza iqembu lakho lezokwelapha ukuhlela indlela ephephile kakhulu yokuhlinzwa kwakho.

Kufanele ukhulume ngokukhethekile uma kukhona umuntu emndenini wakho oqaphele:

• Amathambo aphezulu angachaziwe ngesikhathi sokuhlinzwa
• Ukubuyela emuva okunzima kokunciphisa ubuhlungu
• Izinkinga zemisipha noma ubuthakathaka
• Ukuqaphela okwaziwayo kwe-malignant hyperthermia

Ngesikhathi sokuhlinzwa, iqembu lakho lokunciphisa ubuhlungu likuqapha ngokuqhubekayo, ngakho awudingi ukukhathazeka ngokubona izimpawu ngokwakho. Noma kunjalo, uma ubuye uhlonzwe ngokuhlolwa kwezakhiwo ezihlanganisa imisipha okubonisa ukuqaphela, qiniseka ukuthi ukhombisa noma yimuphi umhlinzeki wezokunakekelwa kwezempilo ngaphambi kokuthola ukunciphisa ubuhlungu noma imithi ethile.

Ziyini izinto ezikhuphula ingozi ye-Malignant Hyperthermia?

Into elikhulu kakhulu elikhuphula ingozi iwumbhalo womndeni we-malignant hyperthermia noma izinkinga ezingachaziwe ngesikhathi sokuhlinzwa. Njengoba lesi simo sezakhiwo ezihlanganisa imisipha siwumndeni, sihlala sihamba emindenini ezizukulwaneni.

Ezinye izinto ezingase zikhuphule ingozi yakho zihlanganisa:

• Ukuba owesilisa (amadoda anethuba elikhulu lokuba naleli phendulo)
• Ukuba musha (abantwana nabantu abadala abasebasha banesilinganiso esiphezulu)
• Ukuba nezifo ezithile zemisipha njenge-central core disease
• Imiphumela engachaziwe ngaphambili yokunciphisa ubuhlungu

Izimo ezithile ezingavamile zemisipha zihlanganiswa nezingozi eziphezulu. Lezi zihlanganisa i-congenital myopathies, i-muscular dystrophies, ne-periodic paralysis syndromes. Uma une-muscle disorder ethile, iqembu lakho lokunciphisa ubuhlungu lizothatha izinyathelo ezengeziwe.

Kubalulekile ukuqaphela ukuthi ukucindezeleka, ukushisa, noma ukuzivocavoca ngokuvamile akuqalisi i-malignant hyperthermia ngokwayo. Impendulo cishe ihlale idinga ukukhombiswa imithi ethile yokunciphisa ubuhlungu kubantu abanezakhiwo ezihlanganisa imisipha eziqaphelayo.

Ziyini izinkinga ezingase zenzeke nge-Malignant Hyperthermia?

Uma kuphathwa ngokushesha, abantu abaningi bayaphila ngokuphelele e-malignant hyperthermia ngaphandle kwemiphumela ehlala njalo. Noma kunjalo, uma impendulo ingabonakali futhi ingaphathwa ngokushesha, ingase iholele ezingxakini ezinzima.

Izinkinga ezinkulu kakhulu zihlanganisa:

• Ukuwohloka kwemisipha (rhabdomyolysis) okungalimaza izinso zakho
• Izinkinga zokugijima kwenhliziyo ezingaba yingozi ebomini
• Ukulimala kwengqondo ngenxa yezinga lokushisa lomzimba eliphezulu kakhulu
• Izifo zokugqwala kwegazi
• Ukushona okukhulu nokungalingani kwe-electrolyte
• Ukwehluleka komzimba ezimisweni eziningi

Ukuvimbela lezi zinkinga kubalulekile ukuqaphela ngokushesha nokwelashwa ngokushesha. Amagumbi okuhlinzwa anamuhla ane-temperature monitoring ne-emergency medications, okwenza izinkinga ezinzima zingavamile kunangaphambili.

Ngokwelashwa okufanele, izinga lokuphila liphezulu kunama-95%. Abantu abaningi ababa ne-malignant hyperthermia baqhubeka bephila impilo evamile ngokuphelele, nakuba bazodinga ukugwema imithi ebangela leli simo ekuhlinzweni kwesikhathi esizayo.

I-Malignant Hyperthermia ihlonishwa kanjani?

I-Malignant hyperthermia ihlolwa ngokuyinhloko ngokusekelwe ezimpawu zakho nendlela ophendula ngayo ekwelapheni ngesikhathi sokuhlinzwa. Iqembu lakho lokunciphisa ubuhlungu lenza lo mkhuba ngokubona izimpawu ezivamile nokubona ukuthi uphendula kanjani emithini yokuphuthuma.

Ngesikhathi sesigameko esisebenzayo, odokotela bafuna ukuxuba okuvamile kokushisa okuphezulu, ukuqina kwemisipha, nokuguquka okuthile kwezinto zegazi. Baqapha futhi impendulo yakho ku-dantrolene, umuthi wokulwa naleli simo.

Ngemva kokuba uphulukile, ukuhlolwa kwezakhiwo ezihlanganisa imisipha kungasiza ukuqinisekisa ukuqaphela kwakho nokuqondisa ukunakekelwa kwezokwelapha kwesikhathi esizayo. Lokhu kuhlolwa kubheka ukuguqulwa kwezakhiwo ezihlanganisa imisipha ezivame ukuxhumana ne-malignant hyperthermia. Noma kunjalo, ukuhlolwa kwezakhiwo ezihlanganisa imisipha akubambi wonke amacala, ngakho-ke umphumela ovamile awuqinisekisi ukuthi awuqapheli.

Kubalandeli bomndeni, ukuhlolwa kwe-muscle biopsy kwakuyindlela evamile yokuhlola. Lokhu kuhilela ukuthatha ingxenye encane yethishu yemisipha nokukhombisa izinto ebangela leli simo elabhorethri. Noma kunjalo, lo mkhuba manje uyatholakala kuphela ezikhungweni ezimbalwa ezikhethekile futhi awusetshenziswa njalo njengoba ukuhlolwa kwezakhiwo ezihlanganisa imisipha kwaba khona.

Ukuphathwa kwe-Malignant Hyperthermia kuyini?

Ukuphathwa kwe-malignant hyperthermia kugxile ekuyekezeni ngokushesha imithi ebangela leli simo nokusebenzisa umuthi wokulwa naleli simo othi dantrolene. Lo muthi usebenza ngokuvinjwa kokukhishwa kwe-calcium emaseleni emisipha, uqeda impendulo eyingozi.

Iqembu lakho lezokwelapha lizothatha izinyathelo eziningi ngokushesha:

1. Yekisa yonke imithi yokunciphisa ubuhlungu ebangela leli simo ngokushesha
2. Nikeza i-dantrolene nge-IV yakho (ngokuvamile i-2.5 mg nge-kg yesisindo somzimba)
3. Phoqelela umzimba wakho ngama-ice packs, amanzi e-IV abandayo, nama-cooling blankets
4. Nikeza i-oksijini bese usekela ukuphefumula kwakho
5. Hlola futhi ulungise noma yiziphi izinkinga zokugijima kwenhliziyo
6. Hlola futhi uqondise izinto zegazi lakho

Ukuphathwa kwe-dantrolene ngokuvamile kudinga ukuphindaphindwa njalo emahoreni ambalwa kuze kube yilapho izimpawu zakho ziqeda ngokuphelele. Abantu abaningi badinga izilinganiso eziningi ezingama-24 kuya kuma-48 amahora ukuvimbela impendulo ukuba ingabuyeli.

Iqembu lakho lezokwelapha lizoqapha futhi umsebenzi wezinso zakho ngokucophelela futhi likupe amanzi amaningi ukusiza ukuhlanza noma yimiphi imikhiqizo yokuwohloka kwemisipha. Ezimweni ezinzima, ungase udinga i-dialysis ukusekela izinso zakho ngesikhathi ziphulukana.

Ungaphatha kanjani ukubuyela emuva kwe-Malignant Hyperthermia?

Ukubuyela emuva kwe-malignant hyperthermia ngokuvamile kwenzeka e-intensive care unit lapho iqembu lakho lezokwelapha lingakuqapha khona eduze. Abantu abaningi baqala ukuzizwa bephilile ngaphakathi kwamahora ambalwa ngemva kokuthola i-dantrolene, nakuba ukubuyela emuva okuphelele kungathatha izinsuku ezimbalwa.

Ngesikhathi sokubuya kwakho, iqembu lakho lezokunakekelwa kwezempilo lizoqhubeka likupa i-dantrolene futhi liqaphe izimpawu zakho eziphilayo, umsebenzi wezinso, nama-muscle enzymes. Cishe uzoba esibhedlela okungenani amahora angu-24 kuya kwangu-48 ukuqinisekisa ukuthi impendulo ayibuyeli.

Uma usukekile ekhaya, uzodinga ukuphumula futhi uvumele umzimba wakho ukuba uphulukane ngokuphelele. Abanye abantu baba nobuhlungu bemisipha noma ubuthakathaka izinsuku ezimbalwa, okuvamile. Ukupha amanzi amaningi kusiza izinso zakho ekuqedeni noma yimiphi imikhiqizo yokuwohloka kwemisipha.

Ingxenye ebaluleke kakhulu yokubuyela kwakho ukuthola amadokhumenti afanele wendlela oqaphele ngayo nokuqondisa komndeni uma kunconywa. Lolu lwazi lubalulekile kunoma yiziphi izinqubo zezokwelapha zesikhathi esizayo nokuqondisa amalungu omndeni angase abe sengozini.

I-Malignant Hyperthermia ingavikeleka kanjani?

Ukuvimbela okungcono kakhulu kwe-malignant hyperthermia ukugwema imithi ebangela leli simo. Uma wazi ukuthi uqaphile noma unembali yomndeni eqinile, iqembu lakho lokunciphisa ubuhlungu lizoqala imithi ehlukile ephephile ngokuphelele kuwe.

Izinketho zokunciphisa ubuhlungu eziphephile zihlanganisa:

• I-Propofol nezinye izidakamizwa ze-IV ezingabangeli leli simo
• I-Local anesthetics njenge-lidocaine
• Ukunciphisa ubuhlungu kwendawo (i-spinal noma i-epidural blocks)
• I-muscle relaxants ezingabangeli leli simo
• I-Nitrous oxide (i-laughing gas)

Uma unembali yomndeni ye-malignant hyperthermia, cabanga ukuhlolwa kwezakhiwo ezihlanganisa imisipha ngaphambi kwanoma yikuphi ukuhlinzwa oluhleliwe. Lokhu kuyasiza iqembu lakho lezokwelapha ukwenza izinqumo eziphephile kakhulu mayelana nokunakekelwa kwakho kokunciphisa ubuhlungu.

Gqoka njalo i-medical alert bracelet noma uthwale ikhadi elikhombisa ukuqaphela kwakho kwe-malignant hyperthermia. Ezimweni zokuphuthuma, lolu lwazi lungasindisa impilo futhi lusisize amaqembu ezokwelapha ukukhetha imithi efanele ngokushesha.

Ukuhlela umndeni, ukuqondisa komndeni kungakusiza ukuqonda izingozi zokudlulisa lesi simo kubantwana bakho nokuxoxa ngezinketho zokuhlola amalungu omndeni.

Kufanele uzilungiselele kanjani i-appointment yakho nodokotela?

Uma ukhathazekile ngengozi ye-malignant hyperthermia, qala ngokuqoqa ulwazi oluqondile ngembali yezokwelapha yomndeni wakho. Gxila ikakhulukazi kunoma yiziphi izinkinga amalungu omndeni abe nazo ngokunciphisa ubuhlungu noma izinkinga ezingachaziwe ngesikhathi sokuhlinzwa.

Ngaphambi kwe-appointment yakho, bhala phansi:

• Noma yimiphi imbali yomndeni yezinkinga zokunciphisa ubuhlungu noma i-malignant hyperthermia
• Ukuhlinzwa okudlule obekukhona futhi indlela obuyile ngayo
• Noma yiziphi izifo zemisipha noma ubuthakathaka emndenini wakho
• Imibuzo mayelana nokuhlolwa kwezakhiwo ezihlanganisa imisipha noma ukuphepha kokunciphisa ubuhlungu
• Imithi yamanje nezifo zokuzalwa

Uma ulungiselela ukuhlinzwa, hlela i-pre-operative consultation nodokotela wakho onciphisa ubuhlungu. Lokhu kukupha isikhathi sokuxoxa ngezinkinga zakho futhi kubavumela ukuhlela indlela ephephile kakhulu yokunciphisa ubuhlungu yokuhlinzwa kwakho.

Letha noma yimaphi amarekhodi ezokwelapha adlule ahlobene nemiphumela yokunciphisa ubuhlungu, imiphumela yokuhlolwa kwezakhiwo ezihlanganisa imisipha, noma imibiko ye-muscle biopsy uma unayo. Lolu lwazi lusisiza iqembu lakho lezokunakekelwa kwezempilo ukwenza izinqumo ezilwemi kakhulu mayelana nokunakekelwa kwakho.

Iyini into ebalulekile mayelana ne-Malignant Hyperthermia?

I-Malignant hyperthermia iyisimo esibucayi kodwa esiphatheka kakhulu esenzeka kubantu abanezakhiwo ezihlanganisa imisipha eziqaphelayo emithini ethile yokunciphisa ubuhlungu. Nakuba ingaba yingozi ebomini uma ingabonakali ngokushesha, ukunakekelwa kwezokwelapha kwamanje kwenze kube lula kakhulu ukuphila ngokwelashwa okufanele.

Into ebaluleke kakhulu okumele uyikhumbule ukuthi ulwazi luyisivikelo sakho esingcono kakhulu. Uma unembali yomndeni yezinkinga zokunciphisa ubuhlungu, khuluma nabaphakeli bakho bezokunakekelwa kwezempilo ngaphambi kwanoma yikuphi ukuhlinzwa. Bangasebenzisa imithi ehlukile ephephile ngokuphelele futhi balungele ukwelashwa okuphuthumayo uma kudingeka.

Ngokwelashwa okufanele nokuqaphela kwezokwelapha, abantu abanezakhiwo ezihlanganisa imisipha eziqaphelayo bangaphila ngokuphephile futhi baphile impilo evamile ngokuphelele. Okubalulekile ukuqinisekisa ukuthi iqembu lakho lezokwelapha liyazi ngengozi yakho ukuze bathathe izinyathelo ezifanele.

Imibuzo evamile ebuzwa mayelana ne-Malignant Hyperthermia

Q1: Ingabe i-malignant hyperthermia ingase ibe khona ngaphandle kokunciphisa ubuhlungu?

I-Malignant hyperthermia cishe ihlale idinga ukukhombiswa imithi ethile ebangela leli simo esetshenziswa ekunciphiseni ubuhlungu. Nakuba kunezimangalo ezimbalwa kakhulu ezibikwe ngokukhombiswa ukushisa okukhulu noma imithi ethile, iningi lemiphumela liyenzeka kuphela ngesikhathi sokuhlinzwa nge-volatile anesthetics noma i-succinylcholine.

Q2: Uma umzali wami wayene-malignant hyperthermia, ngingayithola na ngokuqinisekile?

Akunjalo. Ukuqaphela kwe-malignant hyperthermia kuzuzwa, kodwa akulandeli iphethini elula. Unethuba elingu-50% lokuzuza ukuqaphela kwezakhiwo ezihlanganisa imisipha uma omunye umzali enayo, kodwa ukuba nesakhiwo ezihlanganisa imisipha akusho ukuthi uzoba neli phendulo. Abanye abantu bayithwala le sakhiwo ezihlanganisa imisipha kodwa abazange babe nazimpawu.

Q3: I-malignant hyperthermia ivela ngokushesha kangakanani ngesikhathi sokuhlinzwa?

I-Malignant hyperthermia ingase ivela ngaphakathi kwamaminithi okukhombiswa imithi ebangela leli simo, nakuba ngezinye izikhathi kuthatha isikhathi eside ukuba ibonakale. Impendulo ngokuvamile ibonakala ngaphakathi nehora lokuqala lokunciphisa ubuhlungu, kungakho iqembu lakho lezokwelapha likuqapha eduze ngalesi sikhathi.

Q4: Ngingenza yini umsebenzi wama-dental uma ngiqaphela i-malignant hyperthermia?

Yebo, ungenza umsebenzi wama-dental ngokuphephile. I-Local anesthetics njenge-lidocaine ne-novocaine iphephile ngokuphelele kubantu abanezakhiwo ezihlanganisa imisipha eziqaphelayo. Vele uqinisekise ukuthi ukhombisa udenti wakho ngesimo sakho ukuze bagweme noma yimiphi imithi ebangela leli simo uma kudingeka ukunciphisa ubuhlungu obukhulu.

Q5: Ngizodinga ukugwema yonke imisebenzi yokuhlinzwa yesikhathi esizayo uma ngibe ne-malignant hyperthermia?

Akunjalo neze. Ungaba nokuhlinzwa kwesikhathi esizayo ngokuphephile usebenzisa imithi yokunciphisa ubuhlungu engabangeli leli simo. Iqembu lakho lokunciphisa ubuhlungu lizoqala imithi ehlukile ephephile ngokuphelele kuwe. Abantu abaningi abanezakhiwo ezihlanganisa imisipha eziqaphelayo banezinkonzo eziningi zokuhlinzwa eziphumelelayo ekuphileni kwabo ngezinyathelo ezifanele.