Ngaba kukho umntu osondeleyo kuwe onayo le nguqu? Masifunde ngeSifo sikaPick

Sihlala simangaliswa xa sibona utshintsho olungalindelekanga kwindlela esiziphatha ngayo nesithethi sabanye babantu esibathandayo, akunjalo? Namhlanje siza kuthetha ngemeko yezonyango enokuba yimbangela yotshintsho olunjalo. Nangona le yingongoma enzima, masizame ukuyiqonda ngokulula.

Yintoni isifo sikaPick? Sahluke njani kwisifo sikaNiemann-Pick?

Ngamafutshane, iSifo sikaPick sisifo esichaphazela ingqondo, sibulala iiseli kancinci kancinci. Luhlobo lwesifo sengqondo esibizwa ngokuba yi -frontotemporal dementia (FTD) . Sidla ngokubonakala kubantu abangaphantsi kweminyaka engama-65 ubudala. Ngaphambili, isifo sengqondo esibizwa ngokuba yi-frontotemporal dementia sasibizwa ngokuba sisifo sikaPick. Kodwa ngoku, oogqirha basebenzisa eli gama kuphela kwiimeko apho kuhlangatyezwana neemfuno ezithile.

Ngoku usenokuba ucinga, "Owu... ingaba isifo sikaPick sifana nesifo sikaNiemann-Pick?" Hayi, zizifo ezimbini ezahlukeneyo ngokupheleleyo. Nangona inxalenye yegama elithi "Pick" ifana ngengozi, ezi zifo zimbini azinxulumananga.

• Isifo sikaPick: Sithiywe ngoArnold Pick, ingcali yezifo zengqondo yaseCzech kunye nengcali yezengqondo owaqala ukuchonga esi sifo ngo-1892. Luhlobo lwe-frontotemporal dementia (FTD), oko kuthetha ukuba ichaphazela kuphela ingqondo yakho.
• Isifo sikaNiemann-Pick: Sithiywe ngoogqirha ababini baseJamani, uAlbert Niemann noLudwig Pick, esi sifo sibonakala ngokuqokelelwa kwamafutha emzimbeni kunye nokuphazamiseka kwindlela alawulwa ngayo. Xa amafutha eqokelelana, anokuchaphazela amalungu amaninzi, kuquka ingqondo, isibindi, ipleyini, umongo wamathambo kunye nemiphunga.

Uyayiqonda na umahluko? Enye yinto elinganiselweyo engqondweni, enye yinto echaphazela amalungu ahlukeneyo omzimba.

Ngubani ofumana esi sifo? Sixhaphake kangakanani?

Isifo sikaPick sidla ngokufunyaniswa sisebancinci kunezinye izifo ezifana ne-dementia. Sidla ngokufunyaniswa kubantu abakwiminyaka yabo yama-50 nama-60. Nangona kunjalo, ngamanye amaxesha sinokufunyaniswa kubantu abaneminyaka engama-20 okanye abaneminyaka engama-80.

Kukho ubungqina bokuba esi sifo sinokungena kwiintsapho. Abaphandi bafumanise ubuncinane utshintsho lwezakhi zofuzo ezintathu olunxulumene naso. Nangona kunjalo, kwiimeko ezininzi, esi sifo asihlali sisenzeka rhoqo. Oku kuthetha ukuba umntu unokusifumana ngaphandle kwembali yosapho.

Kunzima ukutsho ngqo ukuba esi sifo sixhaphake kangakanani. Iingcali ziqikelela ukuba kukho iimeko eziphakathi kwe-15 kunye ne-22 kubantu abayi-100,000. Nangona kunjalo, kukho amathandabuzo malunga nokuchaneka kwezi zibalo, kuba isifo kunzima kakhulu ukusifumanisa ebomini, kwaye ngamanye amaxesha nasemva kokufa, kunzima ukusifumanisa. Ngoko ke inani langempela lezigulana linokuba phezulu.

Isifo sikaPick siwuchaphazela njani umzimba?

Isifo sikaPick sisifo esibangela ukonakala kwengqondo, uhlobo lwe-dementia olubizwa ngokuba yi-frontotemporal dementia (FTD). Ngamafutshane, iiseli zemithambo-luvo (ii-neurons) ebuchotsheni ziyayeka ukusebenza kancinci kancinci. Iindawo ezichaphazelekayo zengqondo ziqala ukushwabana (ukuwohloka). Emva koko, ulahlekelwa zizakhono ezazisetyenziswa zezo ndawo ukulawula. Nangona zifana nesifo sika-Alzheimer, isifo sikaPick sidla ngokuqala usemncinci, kwaye kukho umahluko obalulekileyo.

Ngenxa yokuba isifo sikaPick sichaphazela iindawo ezithile zobuchopho kuphela, iimpawu eziphambili kukutshintsha kwendlela yokuziphatha okanye izakhono zolwimi . Rhoqo, abantu abanesifo sikaPick abakwazi ukubona ukuba banengxaki okanye imeko ethile. Oku kungenxa yokuba iingqondo zabo azikwazi ukucubungula ulwazi malunga nemeko yabo. Oku kubizwa ngokuba "kukuswela ukuqonda."

Zithini iimpawu zeSifo sikaPick?

Isifo sikaPick senzeka ngeendlela ezimbini eziphambili: enye yi -behavioral variant frontotemporal dementia (bvFTD) , kwaye enye yi -primary progressive aphasia (PPA), echaphazela iindawo zengqondo ezinxulumene nolwimi.

Uhlobo oluthile lokuziphatha olubizwa ngokuba yi-frontotemporal dementia (bvFTD)

Iimpawu zale meko ye-bvFTD zinokwahlulwa zibe ziindidi ezintandathu eziphambili:

• Ukulahlekelwa kukungathinteli: "Ukungathinteli" yinxalenye yengqondo esixelela ukuba "musa ukwenza oku." Xa ezi ndawo zonakele, asinakuzibamba ekuthetheni okanye ekwenzeni izinto ezingafanelekanga.
• Ukungabikho "kwesihluzo" sento ethethwayo: Ukuthetha into esengqondweni yakho, ngamanye amaxesha ngendlela ebangela intlungu kwabanye, ayilunganga, okanye ayifanelekanga. Umzekelo, ukuthuka umntu ngequbuliso kwindibano yosapho.
• Ukungahloniphi abanye: Basenokungahloniphi ubumfihlo babanye, banokubachukumisa bengafuni, basenokuzama ukuziphatha ngendlela engafanelekanga ngokwesondo, baze ngequbuliso babe nomsindo okanye bacaphuke.
• Izenzo zokuziphatha ezingakhathaliyo: Oku kungabandakanya ukuchitha imali ngokungakhathali, mhlawumbi nokuba evenkileni.

• Ukungakhathali: Oku kunokubonakala ngathi kukudandatheka, kodwa kwahlukile kancinci.
• Ukulahlekelwa ngumdla kuyo nayiphi na into: Ukulahlekelwa ngumdla kwizinto obukade uzonwabela (izinto ozithandayo, ukuchitha ixesha nabahlobo).
• Ukuzikhupha ebantwini: Ukuphepha ukuba nabahlobo kunye nosapho.
• Ukuncipha kokuzinyamekela: Ukulahlekelwa ngumdla wokuhlamba, ukutshintsha impahla, nokuhlala ucocekile.

• Ukulahlekelwa luvelwano:Oku kukwabizwa ngokuba "kukufiphalisa iimvakalelo." Kuba nzima ukuqonda iimvakalelo zabanye. Ababonisi nkxaso kwiintlungu okanye uvuyo lwabanye abantu njengoko babesenza ngaphambili.

• Iindlela zokuziphatha ezinyanzelekileyo: Abantu abanale ngxaki babonisa iindlela zokuziphatha ezahluke kakhulu kwabanye.
• Iintshukumo eziphindaphindwayo: Ukwenza iintshukumo ezincinci ngokuphindaphindiweyo, njengokuqhwaba izandla, ukunyathela iinyawo ngeenyawo, nokuhamba usiya phambili nangasemva.
• Iindlela zokuziphatha ezintsonkothileyo okanye ezingokwesiko: ukubukela iimuvi ezifanayo ngokuphindaphindiweyo, ukufunda iincwadi ezifanayo, ukuqokelela izinto ezifanayo. Oku kuquka ukugcina imali.
• Ukuphindaphinda: Ukuphinda-phinda izandi ezifanayo, amagama, kunye nezivakalisi ezifanayo ngokuphindaphindiweyo.

• Utshintsho kwindlela yokutya okanye ukuziphatha okugxile emlonyeni: Abantu abanesifo banokufumana imeko ebizwa ngokuba yi-"hyperorality," enomdla ongaqhelekanga kwizinto ezinxulumene nomlomo.
• Utshintsho kwindlela yokutya: Abantu badla ngokutya kakhulu ukutya abakuthandayo, ingakumbi ukutya okulula, iilekese, kunye notywala. Oku kunokukhokelela ekutyebeni.
• Iindlela zokuziphatha zomlomo ezinyanzelisayo: Ukutshaya ngokugqithisileyo, ukutya ngokugqithisileyo, njl. Kwakhona, ukuphonononga ngomlomo. Nangona oku kuqhelekile ukuba abantwana abancinci bakwenze xa behlola ihlabathi, kuqhelekile ukuba abantu abadala bakwenze.
• I-Pica: Oku kukutya ngokunyanzelekileyo izinto ezingatyiwayo nezingenasondlo (iingqekembe, uthuli, amatye amancinci).

• Ukulahlekelwa ngumsebenzi wolawulo kodwa ezinye izakhono azisekho: "Umsebenzi wolawulo" ubhekisa kubuchule bokucwangcisa usuku, ukusombulula iingxaki, kunye nokwenza izinto. Nangona ezi zakhono ziphazamisekile kubantu abanesi sifo, ezinye izakhono, ezifana nenkumbulo kunye nokucubungula okubonakalayo, azichaphazeleki kangako kwizigaba zokuqala. Olu luphawu oluphambili oluhlula i-frontotemporal dementia kwisifo se-Alzheimer.

I-Aphasia ePhambili ePhambili (i-PPA)

I-PPA inokubangelwa sisifo sikaPick. Oku kubangela ukulahleka kancinci kancinci kwezakhono zolwimi, njengokukwazi ukuthetha nokuqonda oko kuthethwa ngabanye. Nangona kukho iintlobo ezintathu eziphambili ze-PPA, iintlobo ezimbini eziphambili ezinokunxulunyaniswa nesifo sikaPick zezi:

• I-Nonfluent variant PPA (nfvPPA): Kolu hlobo, kunzima ukufanisa amagama nokusebenzisa igrama echanekileyo. Nangona amagama ahlukeneyo kunye nezivakalisi ezilula zinokuqondwa, izivakalisi ezintsonkothileyo zinobunzima bokuqonda.
• Uhlobo lweSemantic lwePPA (svPPA):Olu hlobo lunobunzima bokukhetha igama elifanelekileyo okanye ukuqonda intsingiselo yamagama. Ezinye izinto ezithethwayo zisenokungabi nantsingiselo, kwaye kunzima ukuqonda oko kuthethwa ngabanye. Kusenokuba neengxaki zokufunda nokubhala, kodwa zinokuphinda oko kuthethwa ngabanye ngendlela efanayo.

Khawucinge nje, utata wakho ngequbuliso utshintsha umntu awayenguye ngaphambili, akhalimele wonke umntu, achithe imali ngokungakhathali, okanye acule ingoma enye imini yonke. Okanye, xa uthetha naye, amazwi akhe ayaxabana, aze azive ngathi akaqondi into oyithethayo. Le yimizekelo yezinto ebesithetha ngazo.

Yintoni ebangela le meko? Ingaba iyasulela?

Isifo sikaPick luhlobo lwe-frontotemporal dementia (FTD) olunesizathu esithile. Iingqondo zethu kunye neeseli zemithambo-luvo ziqulethe iproteni ekhethekileyo ebizwa ngokuba yi-tau . Ezi proteni kufuneka zibe nemo ethile, kungenjalo aziyi kusebenza kakuhle.

Xa iiproteni ze-tau zingasebenzi kakuhle, zidibana, kwaye ziqokelelana ngaphakathi kwee-neurons, ezo seli ziyayonakala kwaye zitshatyalaliswe. Ii-neurons ezonakeleyo zibizwa ngokuba yi- "Pick cells." Ziyadumba kwaye zimile njengebhaluni. Amaqoqo eeproteni ze-tau ezidityaniswe ngaphakathi kweeseli abizwa ngokuba yi- "Pick bodies." Ezi zinokubonwa phantsi kwe-microscope. Okubalulekileyo kukuba, ezi seli ze-Pick kunye nee-Pick bodies azibonwa nakwesiphi na esinye isifo.

Isizathu sokuba ezi proteni ze-tau zingasebenzi kakuhle siseyimfihlakalo. Nangona abaphandi bedibanise ezinye iinguqu zemfuza noku, kwiimeko ezininzi esi sifo asizuzisi.

Esi sifo asisuleli. Asidluliselwa ukusuka komnye umntu ukuya komnye. Nangona kukho ithuba lokuba sidluliselwe kwizizukulwana ngezizukulwana, asenzeki kwiimeko ezininzi.

Isifo sikaPick sifunyanwa njani? Zeziphi iimvavanyo ezenziwayo?

Oogqirha banokuxilonga i-frontotemporal dementia (FTD) ngokuhlolwa ngokomzimba nangokwemithambo-luvo, uvavanyo lokuxilonga kunye nolwemifanekiso, kwaye iziphumo zinokumisela ukuba yi-FTD yokuziphatha okanye uhlobo oluthile lwe-PPA.

Nangona kunjalo, indlela ekuphela kwayo yokwazi ngokuqinisekileyo ukuba isifo sengqondo esibizwa ngokuba yi-frontotemporal dementia (FTD) somntu sibangelwa sisifo sikaPick kukukhangela imizimba yePick kunye neeseli zePick kwizicubu zobuchopho bakhe. Oku kunokubonwa kuphela ngokujonga isampuli yezicubu zobuchopho phantsi kwe-microscope. Oku kuthetha ukuba isifo sikaPick sinokuchongwa ngokuqinisekileyo kuphela emva kokuba umntu eswelekile, ngexesha lovavanyo lwesidumbu.

Uvavanyo olufana nolu lunokwenziwa ukuze kufunyaniswe i-FTD:

• Uvavanyo lwegazi: Olu vavanyo lukhangela iimpawu zeekhemikhali ezibizwa ngokuba zii-"biomarkers" ezinokunceda ekuxilongeni izifo ezithile.
• Uvavanyo lolwelo lwe-cerebrospinal:Oku kwenziwa ngokuthatha ulwelo oluvela kumqolo (umqolo otyhafileyo / ukubhobozwa kwe-lumbar).
• I-CT scan (i-computerized tomography - i-CT scan)
• I-EEG (i-Electroencephalogram)
• Uvavanyo lwemfuza
• Iskeni se-MRI (Umfanekiso we-Magnetic resonance - i-MRI)
• Iskeni yePET (iPositron emission tomography - iskeni yePET)

Ngaba ikho indlela yonyango lweSifo sikaPick? Ngaba sinokunyangeka?

Ngelishwa, akukho nyango, unyango, okanye uthintelo lwesifo sikaPick. Oogqirha banokunika amayeza okunceda ukulawula iimpawu ezinje ngokudakumba, ukungakhathali, kunye nokuziphatha okurhabaxa. Nangona kunjalo, ekubeni olu nyango lwahlukile kumntu nomntu, ugqirha wakho ngoyena mntu ulungileyo onokuthetha naye.

Ngaba umngcipheko wokufumana esi sifo ungancitshiswa?

Isifo sikaPick senzeka ngeendlela ezingalindelekanga nezingaziwayo. Ngoko ke, okwangoku akukho ndlela yokusithintela okanye yokunciphisa umngcipheko wokusifumana.

Luhlobo luni lwekamva umntu onesi sifo anokulilindela?

Kuzo zonke iintlobo ze-FTD, kuquka neSifo sikaPick, ingqondo iyawohloka kancinci kancinci. Njengoko isenza njalo, iindawo zengqondo ezichaphazelekayo ziyalahlekelwa kukusebenza kwazo. KwiSifo sikaPick, oku kudla ngokuchaphazela iindawo zengqondo ezilawula ukuziphatha okanye iindawo zengqondo ezilungelelanisa intetho kunye nokukwazi ukuqonda oko kuthethwa ngabanye.

Usenokulahlekelwa nayimbono yakho ngemeko okuyo. Oko kukuthi, usenokulahlekelwa yingqiqo yeempawu kunye nento eyenzekayo kuwe ngenxa yesi sifo.

Abantu abane-frontotemporal dementia (FTD) kancinci kancinci balahlekelwa kukwazi ukuphila ngokuzimela. Njengoko isifo siqhubeka, banokufuna unyango lweeyure ezingama-24 oluvela kubantu ababathandayo okanye kwiingcali eziqeqeshiweyo. Oku kunokufuna nonyango lwexesha elide.

Abantu abane-FTD banokufumana ubunzima bokuginya (i-dysphagia) , nto leyo enokwenza kube nzima ukutya, ukusela nokuthetha. Oku kwandisa umngcipheko wokufumana izifo ezifana ne-pneumonia okanye ukungaphumeleli kokuphefumla.

Zonke iintlobo ze-FTD, kuquka nesifo sikaPick, ziimeko zobomi bonke. Esi sifo sinzima, sitshabalalisa kancinci kancinci iindawo eziphambili zobuchopho. Iingxaki zinokuba nzima kwaye zide zibulale. Ngenxa yokuba ixesha lokuphila liyahluka kumntu nomntu, kunzima ukutsho ngqo ukuba esi sifo siza kukuchaphazela njani kwaye siza kuhlala ixesha elingakanani. Ugqirha wakho (okanye ugqirha onyanga umntu omthandayo) unokukuxelela okungakumbi ngale nto.

Ungayisebenzisa njani intando yakho ukuba awukwazi ukuzenzela izigqibo?

Ukuba ufunyaniswe une-frontotemporal dementia (FTD) kwisigaba sayo sokuqala, kubalulekile ukuthetha nogqirha wakho, usapho lwakho okanye abantu obathandayo, kunye nabo ubathembayo abanokwenzela izigqibo ezibalulekileyo ngokukhawuleza. Le ngxoxo inokuba nzima, kodwa ukuthetha ngezi zinto kwangethuba kunokuba kudlule ixesha kuya kunceda abantu obathandayo bazi ukuba zithini iminqweno yakho ukuba awukwazi ukuthetha ngemicimbi yakho okanye ukwenza izigqibo.

Ukongeza kuloo ngxoxo, kubalulekile nokuba neminqweno nezigqibo zakho zibhalwe phantsi. Ukuba awukwazi ukuzinyamekela, okanye ukuba awukwazi ukwenza izigqibo malunga nokhathalelo lwakho okanye impilo-ntle yakho, cinga ngokulungiselela amaxwebhu asemthethweni. Ungafumana uncedo kwigqwetha ukulungiselela la maxwebhu, kodwa ungazilungiselela nawe amanye awo (kuxhomekeke kumthetho okwindawo okuyo, anokufuna imvume yenotari okanye elinye igosa).

Wenza ntoni ukuba umntu osondeleyo kuwe ubonakalisa ezi mpawu?

Abantu abane-FTD badla ngokungaziqondi iimpawu okanye imeko yabo. Ngenxa yokuba bengaboni ukuba banengxaki, abakholelwa ukuba bafuna uncedo lwezonyango. Oku kungaqondi kunokuba yinto ecaphukisayo neyoyikisayo kumntu oneempawu kunye nabo basondeleyo kubo.

Ukuba umntu osondeleyo kuwe ubonisa iimpawu ze-FTD okanye imeko efanayo, ungazama ukunceda ngezi ndlela:

• Buza ukuba unganceda njani: Nangona abantu abane-FTD bebonisa iimpawu, basenokungazi ukuba ezi ziimpawu zengxaki enkulu yobuchopho. Ngokubaphulaphula, ukubanika inkxaso yakho, banokuziva benxulumene nabantu ababathembayo, kwaye banokufumana nenkuthazo abayidingayo yokubona ugqirha.
• Bakhuthaze ukuba bafune uncedo: Nangona i-frontotemporal dementia (FTD) yimeko engenanyango okanye unyango, kukho iindlela zokunyanga nokulawula ezinye zeempawu. Le mizamo inokunceda kakhulu ekuphuculeni umgangatho wobomi bomntu onesi sifo. Ukubona ugqirha kunokunceda umntu one-FTD ukuba axilongwe. Unyango olukhethekileyo lunokubonelela ngoncedo kwezinye zeempawu ezingathandekiyo kubantu abanesifo kunye neentsapho zabo.
• Hlala uzolile kwaye ungazithatheli izinto ngendlela yakho: Abantu abane-FTD badla ngokungakwazi ukulawula oko bakuthethayo okanye abakwenzayo. Oku kungabonakala ngathi bazama ngabom ukucaphukisa, ukuhlazisa, okanye ukwenzakalisa abanye, kodwa eneneni yingxaki yezonyango.
• Musa ukoyika ukucela uncedo: Ukunyamekela umntu one-FTD kunokuba nzima kakhulu, ingakumbi njengoko imeko iba mandundu ngokuhamba kwexesha. Musa ukoyika ukucela uncedo okanye izixhobo. Iiarhente ezininzi zikarhulumente nezabucala zibonelela ngeenkqubo zenkxaso kunye neenkonzo, ezinje ngokhathalelo lwabantu abadala emini, ukhathalelo lokuphumla, kunye nokhathalelo lwabahlengikazi abanobuchule emakhaya.
• Ukhathalelo lwexesha elide lunokuba lolona khetho lubalaseleyo: Kwabaninzi, ukunyamekela umntu omthandayo one-FTD kunokuvakala ngathi ngumsebenzi wexesha elizeleyo. Asinguye wonke umntu onokunikela ixesha elininzi okanye umgudu ongako ekunyamekeleni umntu omthandayo. Ke ngoko, kubalulekile ukuqwalasela ukuba umntu omthandayo ufuna unyango kwiziko lokhathalelo lwexesha elide elinamaziko okunakekela abantu abanobuchule. Nangona oku kunokuba sisigqibo esinzima ukusenza, olo khathalelo lunokuba yeyona ndlela ilungileyo yokuqinisekisa ukuba bakhuselekile, bakhululekile, kwaye banabanakekeli abaqeqeshiweyo nabafanelekileyo.

Okokugqibela, umyalezo oya ekhaya

Isifo sikaPick sisifo esibizwa ngokuba yi-frontotemporal dementia (FTD), esiluhlobo lwe-dementia eyonakalisa ingqondo kancinci kancinci xa isencinci kunezinye izifo zengqondo ezinxulumene nobudala. I-FTD ikwachaphazela iindawo zengqondo ezinceda umntu aqonde ukufaneleka nokungafaneleki kokuziphatha kwakhe. Oku kunokubangela ukukhungatheka, ukungaqondani, kunye nokunye ukuphazamiseka okukhulu kubomi babo bonke ababandakanyekayo. Nangona ezinye iimpawu zinokunyangwa, akukho nyango lunokuguqula isifo ngokwaso, kwaye abantu abanale meko badla ngokufuna ukhathalelo oluqhubekayo, iiyure ezingama-24.

Ukuba wena okanye umntu osondeleyo kuwe uhlangabezana nezi mpawu, eyona nto ingcono onokuyenza kukufuna ingcebiso kagqirha. Khumbula, awuwedwa, kwaye kukho iindawo zokufumana uncedo.

Isifo sikaPick , i-Frontotemporal Dementia, i-Dementia, isifo sobuchopho, i-Neurodegenerative, i-Tau Protein, utshintsho kwindlela yokuziphatha