Ngaba ngamanye amaxesha uziva ngathi amalungu akho omzimba abuthathaka kwaye adiniwe? Ngaba ukufumanisa kunzima ukwenza nezinto ezilula njengokuvuka esitulweni, ukuphakamisa into enzima, okanye ukukama iinwele zakho? Ukuba uziva ngathi izihlunu zakho ziya zisiba buthathaka ngaphandle kwesizathu esicacileyo, musa ukuyithatha njengento eqhelekileyo. Mhlawumbi unobangela wezi mpawu yimeko ebizwa ngokuba yi-'myopathy', esiza kuthetha ngayo namhlanje. Ungakhathazeki, siza kuthetha ngale nto ngendlela elula onokuyiqonda.

Yintoni i-myopathy? Ngamafutshane...

I-Myopathy, ngokufutshane, liqela lezifo ezichaphazela izihlunu ezinamathele emathanjeni ethu. Ezi zifo zonakalisa imicu yezihlunu zethu kwaye zizenze buthathaka. Kanye njengokuba injini yemoto iba buthathaka, kuba nzima ukuqhuba, xa izihlunu ziba buthathaka, kuba nzima kwimizimba yethu ukwenza imisebenzi yemihla ngemihla.

Ngaba kukho iintlobo ezahlukeneyo ze-myopathy?

Ewe, ii-myopathies zinokuhlulwahlulwa ngeendlela ezahlukeneyo. Zahlulwe ngokubanzi zibe ziindidi ezimbini: ii-myopathy ezizuzwe njengelifa kunye nezifunyenweyo . Makhe sijonge uhlobo ngalunye kwezi.

Zithini iiMyopathies ezizuzwe njengelifa?

Ezi ziintlobo ze-myopathy ezibangelwa kukufumana i-gene mutation engaqhelekanga eyenzeka emizimbeni yethu xa sizalwa, rhoqo ivela kumama okanye kutata wethu.

• Iimyopathies zokuzalwa:

Iimpawu zihlala ziqala ekuzalweni okanye ebuntwaneni. Nangona kunjalo, ngamanye amaxesha zinokubonakala xa umntu eselula okanye kamva. Uphawu olwahlukileyo lolu hlobo kukuba ubuthathaka abupheleli nje kwimisipha ekufutshane nombindi womzimba, kodwa bunokuchaphazela imisipha yomzimba wonke, kwaye esi sifo sidla ngokungaqhubeki.

• Iimyopathies zeMitochondrial:

Iiseli zethu zinemizi-mveliso emincinci ebizwa ngokuba yi-mitochondria eyenza amandla. Olu hlobo lwe-myopathy lubangelwa sisiphene kwezi mitochondria. Ukongeza kubuthathaka bemisipha, ezi meko zinokuchaphazela nezinye iinkqubo zomzimba, ezifana nentliziyo, ingqondo, kunye nenkqubo yesisu. Zinokukhula ngenxa yotshintsho lwemfuza, nokuba kukho imbali yosapho okanye akunjalo.

• Iingxaki zeMetabolic Myopathies:

Le myopathy ibangelwa ziziphene kwiijini ezenza ii-enzymes ezinceda izihlunu zisebenze kwaye zihambe kakuhle. Inokubangela ukudinwa ngexesha lokuzilolonga, iintlungu emagxeni nasemathangeni xa uzilolonga, okanye imeko ebizwa ngokuba yi-rhabdomyolysis, apho imicu yezihlunu iyaqhekeka ngaphandle kokwenzakala. Ngamanye amaxesha iimpawu zinokuvela ziphele, kunye nobuthathaka bezihlunu zize zibuyele esiqhelweni.

• Ukungasebenzi kakuhle kwemisipha:

Kwezi meko, izihlunu ziyathamba kancinci kancinci kwaye ziphele ngenxa yokungaqheleki okanye ukunqongophala kweeproteni ezixhasa ulwakhiwo ezifunekayo kwizihlunu. Kwimeko nganye, iingalo kunye/okanye imilenze ziyachaphazeleka ngokwamanqanaba ahlukeneyo. Ngamanye amaxesha izihlunu zamehlo okanye zobuso nazo zinokuchaphazeleka.

Zithini ii-myopathies ezifunyenweyo?

Ezi ziintlobo ze-myopathies ezikhula kamva ebomini. Zinokubangelwa zizinto ezahlukeneyo, kuquka ezinye izifo, usulelo, ukuchatshazelwa ngamayeza athile, kunye nokungalingani kwe-electrolyte emzimbeni.

• I-Autoimmune/inflammatory myopathy: I-Myopathy yokuzikhusela komzimba/yokudumba:

Okwenzekayo koku kukuba inkqubo yomzimba wethu yokuzikhusela ihlasela izihlunu zethu. Ngenxa yoko, ukusebenza kwezihlunu kuyaphazamiseka. Kufana nokuzikhusela kwethu kuhlasela zona.

• I-myopathy enobuthi:

Le meko yenzeka xa ityhefu okanye iyeza liphazamisa ulwakhiwo okanye ukusebenza kwezihlunu.

• Iityhefu: Utywala, i-toluene (into eguquguqukayo efumaneka kwizinto ezifana nepeyinti abanye abantu abayiphefumlelayo ukuze banxile).
• Amayeza: Amayeza e-Checkpoint inhibitor immunotherapy (umz., i-pembrolizumab, i-nivolumab), ii-corticosteroids (umz., i-prednisone), amayeza okwehlisa i-cholesterol (ii-statins), i-amiodarone, i-colchicine, i-chloroquine, ii-antivirals kunye ne-protease inhibitors zosulelo lwe-HIV, i-omeprazole.

• Iimyopathies ze-endocrine:

Le meko yenzeka xa ukusebenza kweehomoni emzimbeni wethu kuchaphazela izihlunu.

• I-thyroid gland: Nangona amanqanaba aphantsi e-thyroid hormone (hypothyroidism) exhaphake kakhulu, iingxaki zinokubakho ngenxa yamanqanaba aphezulu e-thyroid hormone (hyperthyroidism).
• I-Parathyroid gland: I-Hyperparathyroidism ibangela ukunyuka kwamanqanaba e-calcium egazini.
• I-Adrenal gland: `Isifo sika-Addison` kunye `nesifo sikaCushing`.

• Iimyopathies ezisulelayo:

Iintsholongwane ezahlukeneyo zinokuchaphazela ukusebenza kwezihlunu.

• Usulelo olubangelwa ziintsholongwane: `i-HIV`, `umkhuhlane`, `i-Epstein-Barr`.
• Usulelo lweentsholongwane: `i-bacterial pyomyositis`.
• `Isifo seLyme`.
• Usulelo lwezibungu: `i-trichinosis`, `i-toxoplasmosis`, `i-cysticercosis`.
• Usulelo lwefungus: Candida, Coccidiomycosis.

• Ukungalingani kwe-electrolyte:

Ukwanda okanye ukwehla kwamanqanaba e-electrolytes ezibalulekileyo emzimbeni wethu kunokuchaphazela ukusebenza kwemisipha.

• I-Potassium: iyancipha (`hypokalemia`), iyanda (`hyperkalemia`).
• I-Magnesium: Iyanda (`i-Hypermagnesemia`).

• I-myopathy yesifo esibuhlungu kwiyunithi yokhathalelo oluphezulu:

Le yimeko echaphazela amalungu omzimba kunye nemisipha yokuphefumla kubantu abagula kakhulu kwaye bafumana unyango kwiyunithi yokhathalelo olunzulu (ICU) . Ingabangelwa kukungashukumi ixesha elide okanye kukusetyenziswa kwezihlunu eziphumzayo, ii-corticosteroids, kunye neepilisi zokulala ezisetyenziswa ngexesha lonyango.

Ngubani onokuba nethuba elikhulu lokufumana i-myopathy? Ixhaphake kangakanani le meko?

Enyanisweni, nabani na unokuba ne-myopathy, kodwa abanye abantu basengozini enkulu kancinci.

Izinto ezinokunyusa umngcipheko:

• Ukuba kukho umntu kusapho one-myopathy, kukho ithuba elikhulu lokufumana esi sifo njengelifa ngokwemfuza.
• Ukuba yindoda. Ezinye iintlobo ze-myopathy zidluliselwa nge-chromosome X, ngoko ke zinokuchaphazela amadoda ngaphezu kwabasetyhini. Nangona kunjalo, iintlobo ze-myopathy zokuzalwa ezidluliselwa kwezinye ii-chromosome zichaphazela wonke umntu ngokulinganayo.
• Ukuba nesifo se-autoimmune, i-metabolic, okanye i-endocrine.
• Ukuchatshazelwa ziziyobisi okanye iityhefu ezithile (jonga icandelo elimalunga ne-toxic myopathy esixoxe ngayo ngaphambili).

Ukuxhaphaka kwe-myopathy kuyahluka ngokuxhomekeke kuhlobo lwayo. Umzekelo, i-myopathy ekhula kamva ebomini:

• I-myopathies yokudumba kunye ne-endocrine ixhaphake kakhulu kunezinye iintlobo, kwaye ixhaphake kakhulu kwabasetyhini kunakwamadoda.
• Phakathi kwabantu abayi-9 ukuya kwabayi-32 kwi-100,000 nganye banesifo sokudumba kwemithambo yegazi.
• Phakathi kwama-25% nama-79% abantu abadala abane-hypothyroidism banokuba neempawu ezinxulumene nemisipha, kodwa ukwenzeka kwe-myopathy ecacileyo kunokuba ngaphantsi kwe-10%.

Ezona myopathies zixhaphakileyo zokuzalwa zii-muscular dystrophies, eziqheleke kakhulu kumadoda.

• I-Duchenne's kunye ne-Becker's muscular dystrophies zezona ntlobo zixhaphakileyo, zichaphazela malunga ne-7 kubantu abayi-100,000 kwihlabathi liphela.
• Izifo ze-Mitochondrial zichaphazela umntu omnye kwabayi-5,000, kwaye uninzi lwazo luchaphazela izihlunu zamathambo.
• Ezinye iintlobo ze-myopathies zokuzalwa azixhaphakanga kakhulu.

Ngoko ke, zithini iimpawu ze-myopathy?

Iindidi ezininzi ze-myopathy zineempawu ezininzi eziqhelekileyo. Ezi zezi:

• Ubuthathaka bemisipha.Ingakumbi izihlunu zamagxa, iingalo eziphezulu, namathanga (ezi zezona zichaphazeleka kakhulu kwaye zinzima kakhulu).
• Ukuqaqamba kwemisipha, ukuqina, kunye nokuqaqamba.
• Ukudinwa ngokukhawuleza xa usenza umthambo okanye usenza umsebenzi omncinci.
• Ukuziva ungenabomi kwaye ungenawo amandla.

Injani ngokwenene i-myopathy?

Kwiimeko ezininzi ze-myopathies, izihlunu kumacala omabini omzimba (ezilinganayo) zibuthathaka ngokulinganayo. Izihlunu ezikufutshane nombindi womzimba (izihlunu ezikufutshane), ezifana namagxa, iingalo eziphezulu, izinqe, namathanga, zichaphazeleka kakhulu. Ngenxa yoko, unokufumana iimpawu ezifana nezi:

• Ubunzima bokwenza imisebenzi yemihla ngemihla, njengokuhlamba, ukunxiba, kunye nokukama iinwele zakho.
• Ubunzima bokuvuka esitulweni, ukunyuka izinyuko, okanye ukwenza imisebenzi efuna ukufikelela ngaphezu kwentloko yakho (umz., ukutshintsha ibhalbhu yesibane eluphahleni).
• Ukuziva ngathi izihlunu ziyashukuma kwaye ziyashukuma.
• Izihlunu ziyadinwa ngokukhawuleza xa zisenza umsebenzi othile.
• Ubunzima bokuphefumla ngexesha lokuzilolonga.

Ngokwesiqhelo ayichaphazeli izihlunu zezandla neenyawo.

Ezinye iimpawu ziyahluka ngokuxhomekeke kuhlobo lwe-myopathy.

• Ubuthathaka bemisipha bunokuba yinto eqhubekayo (engaqhubekiyo) okanye eqhubekeka kancinci kancinci. Kwezinye izifo, ubuthathaka bemisipha bunokufika bubuye buphele, ngelixa ngamanye amaxesha kunokubakho amandla aqhelekileyo.
• Ukulibaziseka kophuhliso lwezakhono zomzimba (umz. ukuhamba, ukutsiba, ukunyuka izinyuko, ukubamba icephe okanye ipensele) kubantwana abancinci.
• Ukungakwazi ukuhambisana nabanye abantwana xa bedlala imidlalo okanye bedlala ngaphandle (umz., bedlala ngokubamba ibhola).
• Iingxaki zezihlunu ezinceda ekuginyeni nasekuthetheni. Oku kunokubangela ukuba ukutya kubambeke kwaye intetho ingabi mnandi.

Ndingenza ntoni xa ndicinga ukuba ndine-myopathy?

Ukuba unale mpawu, kufuneka uqale uthethe nogqirha wakho oyintloko. Ngokuxhomekeke kwiimpawu zakho, angakuthumela kwingcali, njengogqirha wezifo zengqondo okanye ugqirha wamathambo.

Ifunyaniswa njani i-myopathy?

Ugqirha wakho uza kukubuza ngembali yakho yezonyango, nokuba kukho umntu kusapho lwakho okhe waba neemeko ezifanayo, ngawaphi amayeza owasebenzisayo, kunye neempawu zakho. Emva koko baza kwenza uvavanyo lomzimba. Baza kujonga ulusu lwakho, i-reflexes, amandla emisipha, ibhalansi, kunye neemvakalelo zakho.

Iindidi zovavanyo ezinokuthi zi-odolwe ngugqirha:

• Uvavanyo lwegazi:
• Kwezinye izifo ze-myopathies, amanqanaba eeenzyme zemisipha afana ne-creatine kinase (CK) okanye i-aldolase anokunyuka ngenxa yokuqhekeka kweefayibha zemisipha.
• Amanqanaba e-electrolyte afana ne-sodium, i-magnesium, i-potassium, i-calcium, kunye ne-phosphorus.
• Uvavanyo lwezifo ezizikhuselayo emzimbeni: Umzekelo, `Antinuclear Antibodies (ANA), `Rheumatoid Factor', `Sedimentation Rate' kunye ne `C-reactive protein'.
• Uvavanyo lwe-endocrine gland: umzekelo, amanqanaba e-thyroid hormone.
• Izifundo ze-Electromyography (EMG) kunye nokuqhuba imithambo-luvo: Ezi mvavanyo zilinganisa uhlobo kunye nobungakanani bomonakalo wemisipha ngokulinganisa indlela umbane ohamba ngayo kwimithambo-luvo kunye nokufaka iinaliti ezincinci kwimisipha.
• Uvavanyo lwe-magnetic resonance imaging (MRI) lwemisipha .
• Uvavanyo lwemfuza.
• I-biopsy yemisipha: Oku kuquka ukususa iqhekeza elincinci lezicubu zemisipha ngotyando ukuze lihlolwe.

Iphathwa njani i-myopathy?

Xa sele kuchongiwe uhlobo oluchanekileyo lwe-myopathy onayo, ugqirha wakho uya kuphuhlisa isicwangciso sonyango esilungiselelwe iimpawu zakho.

Uninzi lonyango luquka unyango lomzimba, unyango lomsebenzi, kunye nohlobo oluthile lomthambo . Olunye unyango luhambelana ngqo nohlobo lwe-myopathy. Ngokubanzi, uninzi lwe-myopathies ezifunyenweyo zinokulawulwa kakuhle, zinciphise ubuthathaka kunye neempawu. Ezinye i-myopathies zokuzalwa zineendlela ezithile zonyango ezinokucothisa ukuqhubela phambili kwesi sifo. Okwangoku, akukho nyango luthile lwe-myopathies zokuzalwa, kodwa unyango lomzimba kunye nezinye iintlobo zomthambo zinokunceda.

Iimyopathies ezinxulumene nokudumba kunye nokuzikhusela komzimba

Injongo yonyango lwezi zinto kukunciphisa ukudumba kunye nokusabela komzimba kwi-autoimmune. Unyango oluqhelekileyo lwezi myopathies luquka:

• Amayeza okunciphisa umzimba/okuthintela umzimba: Umzekelo, i-methotrexate, i-cyclosporine, i-tacrolimus, i-azathioprine, i-mycophenolate, i-rituximab, kunye ne-immunoglobulin ye-intravenous (IVIg) okanye ye-subcutaneous (SubQIg).
• `Ii-Corticosteroids`: Umzekelo `i-prednisone` okanye `i-methylprednisolone`.

I-myopathies ezuzwe njengelifa kunye neyofuzo

Iimyopathies ezininzi zokuzalwa kunye nezemfuza azinanyango luthile okanye unyango olupheleleyo.Ulawulo luqhutywa ikakhulu kulawulo lweempawu kunye neendlela ezahlukeneyo zonyango. Inani lezilingo zonyango ziyaqhubeka kwiindawo ezahlukeneyo zophando malunga nonyango kunye nonyango lwezakhi zofuzo.

Iimeko ezibizwa ngokuba yiDuchenne muscular dystrophy kunye nesifo sePompe zinokunyangwa ngamayeza athile.

Ezinye i-myopathies ezifunyenweyo

Oogqirha banyanga i-myopathies ebangelwa yi-endocrine, ityhefu, kunye nezizathu ezisulelayo ngokunyanga isifo esibangela i-myopathy. I-myopathies enobuthi inyangwa ngokuyekisa into ebangela ukungasebenzi kakuhle (umz., utywala, i-toluene) okanye iziyobisi (umz., ii-statins). Iimpawu zemisipha ezibangelwa ziibhaktheriya, iintsholongwane, okanye ezinye izinto ezibangela ukugula zihlala ziphucuka xa usulelo lunyangwa ngokuthe ngqo ngamayeza okubulala iintsholongwane.

Ndingayinyamekela njani impilo yam?

Nokuba i-myopathy yimeko yokuzalwa, efunyenweyo, okanye yimeko yexesha elide (engapheliyo), kukho izinto onokuzenza ukuze ugcine impilo yakho kwaye ulawule le meko. Ezi ziquka:

• Yitya ukutya okunempilo nokunesondlo. Faka iziqhamo nemifuno emininzi eyahlukeneyo ekutyeni kwakho.
• Hlala usebenza ngokuzivocavoca okuncinci kwentliziyo. Ngokuxhomekeke kuhlobo lwakho lwe-myopathy, unokucetyiswa ukuba ungenzi iintlobo ezithile zokuphakamisa iintsimbi. Ngoko ke thetha nogqirha wakho ngaphambi kokuba uqalise nayiphi na inkqubo yokuzilolonga.
• Gcina ubunzima bakho busempilweni.
• Ukuba unerhashalala eluswini ebangelwa yi-dermatomyositis, khusela ulusu lwakho elangeni. Oku kunokwenza irhashalala ibe mandundu. Nxiba impahla yokuzikhusela kunye nomnqwazi nanini na kunokwenzeka. Ungalibali ukufaka i-sunscreen ubuncinane ene-SPF 30 ngaphambi kokuba uphume ngaphandle.
• Ukuba unengxaki yokuginya, yitya ukutya okuthambileyo okanye okuqinileyo kancinci. Ungakucoca ukutya kwakho. Ukuba ungumntu ohlala ebhedini, yitya uhleli ebhedini.
• Thatha onke amayeza owanikwe ngugqirha ngexesha elifanelekileyo.
• Ukuba unyango olufana nonyango lomzimba, unyango lomsebenzi, okanye unyango lokuthetha luyacetyiswa, thatha inxaxheba kuzo.

Khumbula, kuba kukho iintlobo ezininzi ze-myopathy, ugqirha wakho kuya kufuneka enze isicwangciso sonyango esihambelana ngqo nohlobo lwakho lwe-myopathy kunye neempawu zakho. Ngoko ke kubalulekile ukulandela imiyalelo yakhe ngokuchanekileyo. Kwakhona, nikela ingqalelo emzimbeni wakho. Qaphela naluphi na utshintsho kwimeko yakho okanye ubukhali beempawu zakho. Bona ugqirha wakho kwishedyuli eqhelekileyo (okanye ngokukhawuleza ukuba ubona naluphi na utshintsho). Ngale ndlela, ungenza utshintsho kwisicwangciso sakho sonyango ngokukhawuleza nje ukuba iimpawu zibonakala.

Okokugqibela, ezona zinto zibalulekileyo ekufuneka uzikhumbule

I-Myopathy ayisiyonto yokoyika, kodwa ayiyonto yokungayinaki.

• Ukuba ufumana ubuthathaka bemisipha obungapheliyo, iintlungu, okanye ukudinwa, qiniseka ukuba ubona ugqirha.
• Kukho iintlobo ezininzi ze-myopathy, ngoko ke ukuxilongwa ngokuchanekileyo kubaluleke kakhulu.
• Kukho unyango kunye neendlela zokulawula iintlobo ezininzi ze-myopathy.
• Ngokulandela imiyalelo kagqirha wakho, ukuthatha amayeza amiselweyo, nokuphila ubomi obusempilweni, ungaphila ubomi obuhle xa unesi sifo.

Ndiyathemba ukuba olu lwazi luza kukunceda. Ukuba uneminye imibuzo, ungoyiki ukuthetha nogqirha nangaliphi na ixesha.

I- Myopathy, ubuthathaka bemisipha, iintlungu zemisipha, izifo zemfuza, izifo zokuzikhusela komzimba, iimpawu zemisipha, unyango lwe-myopathy