Duchenne muscular dystrophy: chii zvazviri, zviratidzo uye kurapwa

Zvemukati

• Zviratidzo zvikuru
• Maitiro ekusimbisa kuongororwa
• Kurapwa kunoitwa sei
• 1. Kushandiswa kwemishonga
• 2. Physiotherapy zvikamu
• Ndeipi tarisiro yehupenyu
• Kazhinji zvinowanzoitika zvinetso
• Chii chinokonzeresa iyi mhando ye dystrophy

Duchenne muscular dystrophy chirwere chisingawanzoitika chinobata varume chete uye chinozivikanwa nekushaikwa kweprotein mumhasuru, inozivikanwa sedystrophin, iyo inobatsira kuchengetedza maseru etsandanyama ane hutano. Naizvozvo, chirwere ichi chinokonzeresa kushomeka kunofambira mberi kweshungu dzese dzemuviri, izvo zvinoita kuti zviomere mwana kuti asvike pazviitiko zvakakosha zvekusimudzira, sekugara, kumira kana kufamba.

Muzviitiko zvakawanda, chirwere ichi chinongozivikanwa mushure memakore matatu kana mana ekuberekwa kana mwana achinge achinja munzira yekufamba, kumhanya, kukwira masitepisi kana kusimuka kubva pasi, sezvo nzvimbo dzakatapurwa kutanga mahudyu, zvidya nemapfudzi. Nekukura, chirwere ichi chinobata tsandanyama dzakawanda uye vana vazhinji vanopedzisira vobva pawiricheya pazera remakore gumi nematatu.

Duchenne muscular dystrophy haina mushonga, asi kurapwa kwacho kunobatsira kunonoka kukura kwechirwere, kudzora zviratidzo uye kudzivirira kutanga kwematambudziko, kunyanya pamwoyo uye nemweya wekufema. Nekudaro, zvakakosha zvikuru kuti ubatwe nachiremba wevana kana mumwe chiremba ane hunyanzvi muchirwere ichi.

Zviratidzo zvikuru

Zviratidzo zvikuru zveDuchenne muscular dystrophy zvinowanzo kuzivikanwa kubva pagore rekutanga rehupenyu uye kusvika zera rechitanhatu, zvichiramba zvichiwedzera kuwanda pamusoro pemakore, kudzamara, pazera ramakore gumi nematatu, mukomana anova anoenderana newiricheya.

Zvimwe zvezviratidzo zvakajairika nezviratidzo zvinosanganisira:

• Kunonoka kugona kugara, kumira kana kufamba;
• Kudzedzereka kana kunetseka kukwira masitepisi kana kumhanya;
• Yakawedzera vhoriyamu mumhuru, nekuda kwekutsiva maseru emaseru nemafuta;
• Zvakaoma kufambisa majoini, kunyanya kukotamisa makumbo.

Kubva pakuyaruka, matambudziko ekutanga akanyanya echirwere anogona kutanga kuoneka, kureva, kunetseka kufema nekuda kwekushomeka kwedhayaphira uye dzimwe mhasuru dzekufema, uye kunyangwe matambudziko emwoyo, nekuda kwekushomeka kwetsandanyama yemwoyo.

Kana matambudziko atanga kuoneka, chiremba anogona kugadzirisa kurapwa kuti aedze kusanganisira kurapwa kwematambudziko uye nekuvandudza hupenyu hwehupenyu. Muzviitiko zvakaipisisa, kugara muchipatara kungatodikanwa.

Maitiro ekusimbisa kuongororwa

Muzviitiko zvakawanda, chiremba wevana anofungira Duchenne muscular dystrophy chete nekuongorora zviratidzo nezviratidzo zvinoratidzwa panguva yekukura.Nekudaro, izvo zvinogona zvakare kugadzirwa kubva muropa kuti uone huwandu hwedzimwe enzymes, senge creatine phosphokinase (CPK), iyo inosunungurwa muropa kana paine mhasuru inoputsika.

Kune zvekare mageneti bvunzo, ayo anobatsira kusvika pane imwezve yakajeka kuongororwa, uye iyo inotarisa shanduko mumajeni anokonzera kutanga kwechirwere.

Kurapwa kunoitwa sei

Kunyangwe Duchenne muscular dystrophy isina mushonga, pane mishonga inobatsira kudzivirira kukurumidza kwayo kuwedzera uye iyo inobvumidza kudzora kwezviratidzo, pamwe nekuonekwa kwezvinetso. Mimwe yemishonga iyi inosanganisira:

1. Kushandiswa kwemishonga

Muzviitiko zvakawanda, kurapwa kweDuchenne muscular dystrophy kunoitwa nekushandiswa kwemishonga yecorticosteroid yakadai sa prednisone, prednisolone kana deflazacort. Iyi mishonga inofanirwa kushandiswa kwehupenyu hwese, uye iine chiito chekugadzirisa immune system, ichiita seanorwisa-kuzvimba uye kunonoka kurasikirwa kwemhasuru basa.
Nekudaro, kushandiswa kwenguva refu kwecorticosteroids kazhinji kunokonzeresa mhedzisiro yakawanda senge kuwedzera kudya, kuwedzera huremu, kufutisa, kuchengetedza kwemvura, osteoporosis, chimiro chipfupi, BP uye chirwere cheshuga, uye zvinongofanirwa kushandiswa pasi pekutarisirwa nachiremba. Tarisa uone zvakawanda nezve izvo corticosteroids zviri uye kuti zvinokanganisa sei hutano.

2. Physiotherapy zvikamu

Mhando dze physiotherapy dzinowanzo shandiswa kurapa Duchenne muscular dystrophy mota uye yekufema kinesiotherapy uye hydrotherapy, inovavarira kunonotsa kutadza kufamba, kuchengetedza simba remhasuru, kubvisa marwadzo uye kudzivirira matambudziko ekufema uye mapfupa kutsemuka.

Ndeipi tarisiro yehupenyu

Iyo tarisiro yeupenyu yeDuchenne muscular dystrophy yaive pakati pemakore gumi nematanhatu negumi nemapfumbamwe, zvisinei, nekufambira mberi kwemishonga uye kubuda kwemishonga mitsva nekutarisira, tarisiro iyi yakawedzerwa. Nekudaro, munhu anoenda kunorapwa kwakakurudzirwa nachiremba anogona kurarama anopfuura makore makumi matatu uye ave nehupenyu hwakajairika, nenyaya dzevarume vanorarama makore anodarika makumi mashanu nechirwere ichi.

Kazhinji zvinowanzoitika zvinetso

Matambudziko makuru anokonzerwa neDuchenne muscular dystrophy ndeaya:

• Yakaoma scoliosis;
• Kunetseka kufema;
• Chirwere chemabayo;
• Kusakwana kwemoyo;
• Kufutisa kana kushaya zvinovaka muviri.

Uye zvakare, varwere vane iyi dystrophy vanogona kusangana nekudzikisira kwepfungwa zvine mwero, asi hunhu uhwu hauna hukama nehurefu kana kuomarara kwechirwere.

Chii chinokonzeresa iyi mhando ye dystrophy

Sechirwere chechirwere, Duchenne's muscular dystrophy inoitika kana shanduko ikaitika mune imwe yemageneti anokonzeresa kuti muviri uburitse dystrophin protein, iyo DMD geni. Protein iyi yakakosha nekuti inobatsira maseru emhasuru kuti agare aine hutano nekufamba kwenguva, achiadzivirira kubva mukukuvara kunokonzerwa neyakajairika mhasuru inodzvinyirira uye kutandara.

Nekudaro, kana iyo DMD geni yashandurwa, haina kukwana mapuroteni anogadzirwa uye mhasuru dzinopedzisira dzave kupera simba uye kutambura kukuvara nekufamba kwenguva. Iyi protein yakakosha kune ese mhasuru inodzora kufamba, pamwe nemusuru wemwoyo.