I-JHD ihluke njani kwi-Adult-Onset Huntington's Disease?
Nangona isifo sabantwana baseHuntington sisifo esilukhuni ukufumana, kuya kunceda ukufunda oko unokulindela kunye nendlela yokujamelana nayo nemiphumo. Isifo se-Juvenile Huntington sisimo sengqondo esenza ukuba iingxaki zengqondo, iingxaki zengqondo kunye nokuhamba okungalawulwayo komzimba. Igama elithi "abantwana" libhekisela ebuntwaneni okanye ebusheni; Isifo sikaHuntington sibalwa njengabantwana xa kuqhubeka phambi komntu oneminyaka engama-20 ubudala.
Amanye amagama kwisifo se-Huntington sesantwazana siquka i-JHD, i-juvenile iqala i-HD, isifo se-Huntington, umntwana we-HD, nesifo se-Huntington,
Ukukhula
Ucwaningo lophando luqikelele ukuba malunga neepesenti ezi-5 ukuya kwi-10 ekhulwini lwee-cases ze-Huntington zezifo zichazwe njengabantwana. Okwangoku, malunga nama-30,000 aseMelika ahlala nesifo sikaHuntington, malunga ne-1,500 ukuya ku-3 000 kubo bangaphantsi kweminyaka engama-20.
Iimpawu
Iimpawu ze-JHD zihlala zihluke ngokusuka kumntu omdala ukuqala kwesifo sikaHuntington. Ukufunda malunga nabo kuyaqondakala. Yazi ukuba akusiyo yonke into eyenzekayo kwaye ukuba iindlela ezahlukeneyo zokwelapha ziyafumaneka ukunceda ukulawula abo benzayo ukuze kugcinwe umgangatho wobomi.
Izinguqu ezinokwenkcazo zingabandakanya ukuhla kweememori , ukucotha okucothayo, ubunzima bwanamhlanje kwimisebenzi yesikolo, kunye nemingeni ekuqaleni okanye ukugqiba umsebenzi ngempumelelo.
Utshintsho lwezenzo luhlala lukhula kwaye luquka ulaka, ukrakra , ukunyaniseka, ukuxinezeleka, ukuxhalaba, ukuphazamiseka okunyanzelekileyo , kunye neengcinga .
Utshintsho oluthile luquka imilenze elukhuni, ukuxhamla, ukugwinya iingxaki, ukuncipha kwentetho, nokuhamba kwiindawo eziphambili. Ngethuba ukuqala kwabantu abadala abantu abahlala e-Huntington bavame ukubonisa ukunyanzelisa, ukungalawulwa okungalawulwayo (okubizwa ngokuba yi-chorea), abo abaneJHD banakho ukubonakalisa iimpawu ezifana nesifo sikaParkinson, njengokunciphisa, ukuqina, ukulingana okungafanelekanga kunye nokuxhamla.
Iingxaki zenzeke nakwabanye abantu abatsha abaneJHD. Ukongezelela, utshintsho ekubhalweni ngesandla lungabonwa kwangaphambili kwi-JHD.
Abafana kunye ne-Adult Onset HD
I-JHD ineemingeni ezahlukahlukeneyo ezivela kwi-HD ekukhuleni kwabantu abadala. Ziquka ezi zilandelayo.
Iingxaki ezikolweni
I-JHD inokubangela ubunzima ekufundeni nasekukhunjuleni ulwazi kwimeko yesikolo, nangaphambi kokuba i-JHD ifumaneke. Oku kungenza kube nzima kubafundi kunye nabazali abangenako ukuqonda ukuba kutheni ukusebenza kwesikolo kunqabile kakhulu.
Ngentlalo, isikolo kunye ne-JHD sinokuba nzima ukuba ngaba iimpazamo zingalunganga kwaye ukusebenzisana kwentlalo kuchaphazelekayo.
Ubungqina Endaweni yeCorea
Nangona iimeko ezininzi ze-HD ziqala ukubandakanya i-chorea (ukunyakaza okungazibandakanyi), i-JHD ihlala ichaphazela iziphumo ngokuyenza ibe nzima kwaye ingapheli. Ngaloo ndlela, unyango luhlala luhluke kwiJHD.
Ukuxhamla
Ukuqubuzana akuqhubi ngokuqhelekileyo kwi-HD ephakamileyo kodwa bakhula malunga nama-25 ukuya kuma-30 ekhulwini lamatyala e-JHD. Ezi zithintelo zinokubeka abantu ngabomngcipheko omkhulu wokuwa kunye nokulimala.
Isizathu kunye neeGenetics
Yazi ukuba akukho nantoni na eyenziwa okanye ayizange yenze ukuba umntwana akhulise i-JHD. Ngokuqhelekileyo kubangelwa ukuguquka komzimba kwi-chromosome ezine ezuzwe kubazali.
Enye inxalenye yemfuza, ebizwa ngokuba yiCAG (i-cytosine-adenine-guanine) iphinda iphinda ibekwe ngumngcipheko we-HD. Ukuba inani le-CAG liphinda lingaphezulu kwama-40, loo mntu uya kuhlola i-HD. Abantu abaphuhlisa i-JHD banomdla wokuphindaphinda kwama-50 we-CAG kwi-chromosome yabo.
Amaphesenti angama-90 ama-JHD amatyala azuze ilifa ku-ntata, nangona i-gene ingadluliselwa kubomi bobabini abazali.
Unyango
Ekubeni ezinye iimpawu zihluke kwi-HD, i-JHD ihlala iphathwa ngokungafani ne-HD. Izinyango zonyango zihlala zixhomekeka kwiimpawu ezikhoyo kwaye zijoliswe ekuncedeni ukugcina umgangatho wobomi obuhle.
Ukuba izibilini zikhona, amayeza e-anticonbulsant mayinganyelwe ukuba abalawulwe. Nangona abanye abantwana basabela kakuhle kula mayeza, abanye banemiphumo emibi ebuthathaka yokulala, ukulungelelaniswa okungafanelekanga kunye nokudideka. Abazali bafanele baxoxe ngeengozi zokufumana amayeza anogqirha wengane.
I-Psychotherapy inconywa ngabantu abahlala ne-JHD, banikezelwa utshintsho olubalulekileyo olufunekayo yi-JHD. Ukuthetha nge-JHD kunye nemiphumo yayo kunokunceda abantwana kunye namalungu entsapho ukuba bahlangabezane, bafunde ukuchazela abanye, baqinisekise ukuba zeziphi iimfuno, kunye nokudibanisa neenkonzo ezixhasayo kuluntu.
Uphando olusisigxina kunye nomsebenzi luyacetyiswa kwaye lungasetyenziselwa ukuncedisa kwaye uphumule iingalo kunye nemilenze eqinile, kunye nokulungelelanisa izixhobo ukuhlangabezana neemfuno.
Umtya wesondlo unokunceda ngokumisela ukutya okuphezulu kweekhalori ukulwa nokulahleka kwesisindo esivame ukujamelana nabantu abaneJHD.
Ekugqibeleni, intetho kunye nolwimi lwezilwanyana lunokukunceda ngokuchonga iindlela zokuqhubela phambili ukunxibelelana neemfuno kunye nabo bajikeleze ukuba kuthetha kunzima.
Prognosis
Emva kokuxilongwa nge-JHD, ukulindela ubomi kuba malunga neminyaka eyi-15. I-JHD ihamba phambili, ithetha ukuba ngokuhamba kwexesha, iimpawu zanda kunye nokusebenza kuyaqhubeka nokuhla.
Nangona kunjalo, khumbula ukuba kukho uncedo kubo bobabini kunye neentsapho zabo njengoko befumana i-JHD. Ukuba awuqinisekanga ukuba ungaqala kuphi, qha ga mshelana ne-Huntington's Disease Society of America ngolwazi kunye nenkxaso kufuphi nawe.
> Imithombo:
> Huntington's Disease Society of America. I-Young Onset HD. http://hdsa.org/living-with-hd/juvenile-onset-hd/
> I-Huntington's Disease Youth Organization. Iisiseko zeJHD. https://en.hdyo.org/jhd/articles/52
> ULiou, S. (2010) Iprojekthi ye-Outreach ye-Outreach yeMfundo e-Stanford. Izifo ze-Juvenile Huntington.
> AmaZiko eNational Health. Iziko leNgcaciso loLuntu lweGenesis neRareases. Izifo Zengqungquthela zaseHuntington. https://rarediseases.info.nih.gov/diseases/10510/juvenile-huntington-disease