Duchenne muscular dystrophy: ke eng, matšoao le kalafo

Litaba

• Matšoao a mantlha
• Mokhoa oa ho netefatsa ts'oaetso
• Kalafo e etsoa joang
• 1. Ts'ebeliso ea meriana
• 2. Physiotherapy mananeo
• Nako ea bophelo ke eng
• Mathata a atileng haholo
• Ke eng e bakang mofuta ona oa ho senyeha ha maikutlo

Duchenne muscular dystrophy ke lefu le sa tloaelehang la liphatsa tsa lefutso le amang banna feela mme le tšoauoa ka khaello ea protheine ka har'a mesifa, e tsejoang e le dystrophin, e thusang ho boloka lisele tsa mesifa li phetse hantle. Ka hona, lefu lena le baka ho fokola butle-butle ha mesifa eohle ea 'mele, e leng ho thatafalletsang ngoana ho fihlela lintlha tsa bohlokoa tsa kholo, joalo ka ho lula, ho ema kapa ho tsamaea.

Maemong a mangata, lefu lena le tsejoa feela kamora lilemo tse 3 kapa tse 4 ha ngoana a e-na le liphetoho tseleng ea ho tsamaea, ho matha, ho hloa litepisi kapa ho nyoloha fatše, kaha libaka tse amehileng pele ke letheka, lirope. le mahetla. Ka botsofali, lefu lena le ama mesifa e mengata mme bana ba bangata ba qetella ba its'etleha ka setulo sa likooa ba le lilemo tse ka bang 13.

Duchenne muscular dystrophy ha e na pheko, empa kalafo ea eona e thusa ho liehisa kholo ea lefu lena, ho laola matšoao le ho thibela ho qala ha mathata, haholo maemong a pelo le a ho hema. Kahoo, ho bohlokoa haholo ho fumana kalafo le ngaka ea bana kapa ngaka e 'ngoe e sebetsanang le lefu lena.

Matšoao a mantlha

Matšoao a mantlha a Duchenne muscular dystrophy hangata a tsebahala ho tloha selemong sa pele sa bophelo le ho fihlela lilemong tsa 6, a ntse a mpefala hanyane ka hanyane ho theosa le lilemo, ho fihlela, a le lilemo li 13, moshanyana a itšetleha ka setulo sa likooa.

Tse ling tsa matšoao le matšoao a atileng ka ho fetisisa li kenyelletsa:

• Ho lieha ho lula ho lula, ho ema kapa ho tsamaea;
• Ho thekesela kapa ho ba le bothata ba ho hloa litepisi kapa ho matha;
• Keketseho ea molumo oa manamane, ka lebaka la ho nkeloa sebaka ha lisele tsa mesifa le mafura;
• Ho ba thata ho tsamaisa manonyello a hau, haholo ho kobola maoto.

Ho tloha bohlankaneng, mathata a pele a bohloko a lefu lena a ka qala ho hlaha, e leng, bothata ba ho hema ka lebaka la ho fokola ha diaphragm le mesifa e meng ea ho hema, esita le mathata a pelo, ka lebaka la ho fokola ha mesifa ea pelo.

Ha mathata a qala ho hlaha, ngaka e ka fetola kalafo ho leka ho kenyelletsa kalafo ea mathata le ho ntlafatsa bophelo. Maemong a boima ka ho fetisisa, ho ka ba ha hlokahala hore motho a kene sepetlele.

Mokhoa oa ho netefatsa ts'oaetso

Maemong a mangata, ngaka ea bana e belaella Duchenne muscular dystrophy feela ka ho lekola matšoao a hlahisoang nakong ea kholo.Leha ho le joalo, li ka etsoa ka mali ho tseba bongata ba li-enzyme tse ling, tse kang creatine phosphokinase (CPK), e lokolloang maling ha mesifa e senyeha.

Ho boetse ho na le liteko tsa liphatsa tsa lefutso, tse thusang ho fihlela phumano e phethahetseng, mme e shebang liphetoho liphatseng tsa lefutso tse ikarabellang bakeng sa ho qala ha lefu lena.

Kalafo e etsoa joang

Le ha Duchenne muscular dystrophy e se na pheko, ho na le liphekolo tse thusang ho thibela ho mpefala ha eona ka potlako le ho lumellang taolo ea matšoao, hammoho le ponahalo ea mathata. Tse ling tsa kalafo ena li kenyelletsa:

1. Ts'ebeliso ea meriana

Maemong a mangata, kalafo ea Duchenne muscular dystrophy e etsoa ka ts'ebeliso ea meriana ea corticosteroid e kang prednisone, prednisolone kapa deflazacort. Meriana ena e lokela ho sebelisoa bophelo bohle, 'me e be le ts'ebetso ea ho laola sesole sa' mele, e sebetsa e le anti-inflammatory mme e liehise tahlehelo ea mesifa.
Leha ho le joalo, ts'ebeliso ea nako e telele ea li-corticosteroids hangata e baka litla-morao tse 'maloa tse kang ho eketsa takatso ea lijo, ho nona, ho nona haholo, ho boloka mokelikeli, ho fokola ha masapo, seemo se sekhuts'oane, khatello ea kelello le lefu la tsoekere, mme li lokela ho sebelisoa tlasa taolo ea ngaka. Hlahloba ho eketsehileng ka hore na li-corticosteroids ke eng le hore na li ama bophelo bo botle joang.

2. Physiotherapy mananeo

Mefuta ea physiotherapy eo hangata e sebelisetsoang ho phekola Duchenne muscular dystrophy ke motor le phefumoloho kinesiotherapy le hydrotherapy, e ikemiselitseng ho liehisa ho se khone ho tsamaea, ho boloka matla a mesifa, ho kokobetsa bohloko le ho thibela mathata a ho hema le ho robeha ha masapo.

Nako ea bophelo ke eng

Tšepo ea bophelo ea Duchenne muscular dystrophy e ne e le pakeng tsa lilemo tse 16 le 19, leha ho le joalo, ka tsoelo-pele ea bongaka le ho hlaha ha liphekolo tse ncha le tlhokomelo, tebello ena e eketsehile. Kahoo, motho ea kenang kalafo e khothalelitsoeng ke ngaka a ka phela ho feta lilemo tse 30 mme a phela bophelo bo tloaelehileng, ka linyeoe tsa banna ba phelang lilemo tse fetang 50 ba tšoeroe ke lefu lena.

Mathata a atileng haholo

Mathata a mantlha a bakoang ke Duchenne muscular dystrophy ke:

• Scoliosis e matla;
• Ho phefumoloha ho thata;
• Nyomonea;
• Ho se sebetse ha pelo;
• Botenya kapa khaello ea phepo e nepahetseng.

Ho phaella moo, bakuli ba nang le lefu lena ba ka 'na ba fokola kelellong ka mokhoa o itekanetseng, empa tšobotsi ena ha e amane le nako kapa boima ba lefu lena.

Ke eng e bakang mofuta ona oa ho senyeha ha maikutlo

Joaloka lefu la liphatsa tsa lefutso, Duchenne's muscular dystrophy e etsahala ha phetoho e hlaha ho o mong oa liphatsa tsa lefutso o ikarabellang bakeng sa ho baka 'mele ho hlahisa protheine ea dystrophin, lefutso la DMD. Protheine ena e bohlokoa haholo hobane e thusa lisele tsa mesifa ho lula li phetse hantle ha nako e ntse e feta, ho li sireletsa likotsing tse bakiloeng ke ho honyela hoa mesifa le boikhathollo.

Kahoo, ha liphatsa tsa lefutso tsa DMD li fetoloa, ha ho hlahisoe liprotheine tse lekaneng mme mesifa e qetella e fokolisa le ho utloa bohloko ha nako e ntse e feta. Protheine ena e bohlokoa molemong oa mesifa e laolang motsamao, hammoho le mesifa ea pelo.