Ingaba izihlunu zakho ziya zisiba buthathaka kancinci kancinci? Usenokungazi ukuba yiPrimary Lateral Sclerosis (PLS)! Masithethe ngale nto!

Ngaba ngamanye amaxesha uziva ngathi imilenze neengalo zakho ziyaphela kancinci kancinci, okanye amandla akho ayaphela? Ngaba uziva ngathi awusenamandla njengangaphambili xa uhamba, mhlawumbi ude ulahlekelwe yibhalansi yakho? Nangona ezi ziimpawu onokuthi unganikeli ngqalelo ingako kuzo, ngamanye amaxesha kunokubakho isizathu esinzulu emva kwazo. Yiyo loo nto iPrimary Lateral Sclerosis, okanye i-PLS ngamafutshane.

Masibone nje ukuba le PLS ithetha ukuthini?

Ngamafutshane, i-PLS sisifo se-neuromuscular esiqhubekayo. Sibangela ukuba izihlunu zakho zibe buthathaka kancinci kancinci kwaye/okanye ziqine. Iimpawu zihlala ziqala emilenzeni yakho. Ekuhambeni kwexesha, obu buthathaka kunye nokuqina kunokusasaza kwezinye izihlunu emzimbeni wakho.

Cinga ngale ndlela: izihlunu zethu zisebenza njengesixhobo sombane. Zifuna ukufumana 'umbane', umyalezo ovela engqondweni, kanye njengoko kufanele. Kwi-PLS, iiseli zemithambo-luvo ezibizwa ngokuba yi-Upper Motor Neurons (UMN), ezifana 'neentambo' ezithwala le miyalezo, zonakele.

Ngelishwa, okwangoku akukho nyango lwe-PLS. Ngoko ke unyango lujolise kakhulu ekulawuleni iimpawu zakho nokukunceda wenze imisebenzi yemihla ngemihla ngokulula. Umzekelo, ukukunceda usebenzise intonga okanye i-walker.

Ixhaphake kangakanani i-PLS?

I-PLS yimeko engaqhelekanga kakhulu. Okwangoku akukho zibalo zichaza ukuba bangaphi abantu abayibangelayo.

Yintoni umahluko phakathi kwe-PLS kunye ne-ALS? Ngaba i-PLS ingaba yi-ALS?

Mhlawumbi ukhe weva nge-ALS, okanye i-Amyotrophic Lateral Sclerosis. Zombini i-PLS kunye ne-ALS zizifo zemithambo-luvo kunye nemisipha. Kodwa kukho umahluko obalulekileyo phakathi kwezi zimbini.

Umahluko ophambili kukuba i-PLS ichaphazela kuphela ii-neurons zakho eziphezulu ze-motor (ii-UMN) . Ezi zii-nerve ezithwala imiyalezo esuka engqondweni ukuya kwi-spinal cord. Kwi-ALS, ezi-UMN nazo ziyachaphazeleka, njengoko kunjalo nee-neurons ezisezantsi ze-motor (ii-LMN) , ezithwala imiyalezo esuka kwi-spinal cord ukuya kwimisipha.

Ngoku usenokuba uzibuza, 'Ngoko ke, ngaba umntu one-PLS angafumana i-ALS kamva?' Ewe, kukho ithuba lokuba inokuba njalo. Ngamanye amaxesha iimpawu zokuqala ze-ALS zinokufana ne-PLS. Ukuba uneempawu ezichaphazela kuphela i-UMN, ekuqaleni unokufunyanwa une-PLS. Kodwa ukuba kamva ufumana iimpawu ze-ALS ezichaphazela zombini i-UMN kunye ne-LMN, ugqirha wakho unokutshintsha ukuxilongwa kwakho kube yi-ALS.

Enyanisweni, iimeko ezininzi ezibonakala ngathi yi-PLS zinokuba ziimpawu zokuqala ze-ALS ezichaphazela i-UMN (`i-UMN-predominant ALS`). Yiyo loo nto oogqirha besithi ukuze ufunyaniswe une-PLS ngokuqinisekileyo, kufuneka ube neempawu ubuncinane iminyaka emithathu ukuya kwemine .

Zithini iimpawu ze-PLS?

Iimpawu ze-PLS zibonakala kancinci kakhulu.Awuyi kuva mahluko mkhulu kwangoko.

Iimpawu ezinokubonakala kwizigaba zokuqala:

Ekuqaleni ungaziva izinto ezinje:

• Ukuqina kwemilenze. Kusenokuvakala ngathi imilenze yakho ibambekile.
• Izihlunu emilenzeni yakho ziyabuthathaka. Ungaziva ngathi awusenamandla njengangaphambili.
• Kuba nzima ukuhamba, okanye ukugcina ibhalansi yakho. Ungakhubeka okanye uwe.
• Kuvela ukuqaqamba kwemisipha okanye iintlungu ezibuhlungu.

Iimpawu ezinokubonakala njengoko isifo siqhubeka:

Njengoko isifo siqhubeka, ezinye iimpawu ezifana nezi zinokuvela:

• Izihlunu zeminwe, izandla, neengalo nazo ziyaqina kwaye zibe buthathaka. Kunokuba nzima ukubamba izinto ezincinci okanye ukubhala.
• Ubunzima bokulawula ukuchama. Oku kunokubangela ukuba umchamo ukhawuleze kwaye ungalawuleki.
• Intlungu yenzeka emqolo ongezantsi nasentanyeni.

Iimpawu ezingaqhelekanga ezichaphazela izihlunu zolwimi:

Oku kunqabile kakhulu, kodwa abanye abantu banokuchaphazeleka yimisipha yolwimi lwabo. Ukuba oku kuyenzeka:

• Intetho engacacanga (dysarthria) iyenzeka. Kuba nzima ukubiza amagama ngokucacileyo.
• Ubunzima bokuginya (dysphagia). Usenokuziva ngathi uyakrwitshwa xa uginya ukutya okanye isiselo.

Yintoni ebangela i-PLS?

Kwiimeko ezininzi, asikazi ukuba yintoni kanye kanye imbangela ye-PLS. Yiyo leyo inyaniso.

Nangona kunjalo, kunqabile kakhulu ukuba kubekho uhlobo lwe-PLS oluchaphazela abantwana abancinci kunye nabantu abadala abancinci (`i-juvenile primary lateral sclerosis`). Oku kubangelwa kukutshintsha kwe-DNA yabo ngexesha lokukhulelwa.

Kwenzeka ntoni emizimbeni yethu ngenxa ye-PLS?

I-PLS yimeko ye-neuromuscular. Ichaphazela kakhulu ii-motor neurons engqondweni yakho kunye nee-nerve fibers ezisuka kwezo neurons ukuya kwi-spinal cord. Ezi zikwabizwa ngokuba zii-upper motor neurons (UMN).

Khawuthelekelele, xa ufuna ukuhambisa umlenze wakho, ingqondo yakho ithumela umyalezo. Lo myalezo uhamba ngemithambo-luvo ukuya kwii-neurons kumqolo, uze ukusuka apho uye kwimisipha ezenzekelayo emlenzeni wakho. Xa izihlunu zifumana lo myalezo, ziyashukuma.

Kwi-PLS, ezi seli zemithambo-luvo ze-UMN ziyafa kancinci kancinci (ukuwohloka). Emva koko, izihlunu azifumani imiyalezo efunekayo ngokufanelekileyo, ngoko ke izihlunu azisebenzi njengoko kulindelekile.

Ngaba esi sisifo esizuzwe njengelifa?

I-PLS ayiqhelekanga ukuba yimfuza. Oku kuthetha ukuba unokuyifumana nokuba akukho mntu kusapho lwakho owakha wayifumana. Uninzi lwexesha, yenzeka ngequbuliso, kungekho mbali yesifo kusapho.

Ngubani osengozini enkulu yokufumana i-PLS?

I-PLS inokuchaphazela nabani na. Idla ngokufunyaniswa ukuba inesifo esimalunga neminyaka engama-50 ubudala. Nangona kunjalo, inokuchaphazela abantu abangaphantsi kweminyaka engama-50 ubudala, abantu abadala, kwanabantwana. Ixhaphake kancinci kumadoda.

Ziziphi iingxaki ezinokwenzeka ngenxa ye-PLS?

Njengoko i-PLS iqhubeka, unokufumana kunzima ukuhamba ngaphandle koncedo. Usenokufuna ukusebenzisa intonga, i-walker, okanye isitulo esinamavili. Ukongeza, njengoko izihlunu zakho zibuthathaka, unokulahlekelwa yibhalansi yakho kwaye uwe lula. Ukuba wenza njalo, unokwenzakala.

Ifunyanwa njani i-PLS?

Ugqirha uxilonga i-PLS emva kovavanyo lomzimba, uvavanyo lwemithambo-luvo, kunye nolunye uvavanyo olukhethekileyo. Baza kuhlola iimpawu zakho baze bazame ukuthintela ezinye iimeko ezineempawu ezifanayo, ezifana ne-ALS okanye i-multiple sclerosis. Uvavanyo lungabandakanya:

• Uvavanyo lwegazi.
• Uvavanyo lwe-Electrodiagnostic - Olu lujonga ukuba imithambo-luvo kunye nemisipha yakho isebenza kakuhle kangakanani. Imizekelo ibandakanya izifundo zokuqhuba imithambo-luvo kunye nezifundo ze-needle electrode.
• Uvavanyo lwe-MRI (Magnetic Resonance Imaging) lwengqondo kunye nomqolo.
• Uhlalutyo lwe-Cerebrospinal fluid (CSF). Oku kwenziwa ngokuthatha ulwelo oluncinci oluvela kumqolo wakho (ukubhobozwa kwe-lumbar okanye i-spinal tap) ukujonga iingxaki ezithile kwizifo ze-neuromuscular.

Ziziphi iindlela zonyango ze-PLS?

Unyango lwe-PLS lujolise ekukuncedeni ukulawula iimpawu zakho. Oku kungabandakanya:

• Amayeza okunciphisa ukuqina kwemisipha, ukuqhwalela, kunye nobunzima bokuginya.
• Unyango lomzimba lokunciphisa ubuthathaka bemisipha, ukuphucula ukuguquguquka kwemisipha, kunye nokuphucula ukuhamba kwamalungu.
• Izixhobo ezinceda kwimisebenzi yemihla ngemihla kunye nokuhambahamba. Umzekelo, intonga, i-walker, okanye isitulo esinamavili.
• Unyango lokuthetha okanye izixhobo zokunceda kwiingxaki zokuthetha.

Amayeza anikwayo kwiimpawu ze-PLS:

Amanye amayeza aqhelekileyo anikwa iimpawu ze-PLS aquka:

• Ukuqina kwemisipha: `(Baclofen)` kunye `(Tizanidine)`
• Ukuqaqamba kwemisipha: `(Quinine)`
• Ukukhulula izihlunu: `(Diazepam)`

Ngaba kukho naziphi na iziphumo ebezingalindelekanga zonyango?

Ewe, njengawo onke amayeza, eli linokuba nemiphumo emibi. Iziphumo ebezingalindelekanga ziyahluka ngokuxhomekeke kwiyeza. Kungoko ke, kubaluleke kakhulu ukuthetha nogqirha wakho malunga nemiphumo emibi enokubakho ngaphambi kokuba uqalise naliphi na iyeza.

Ngaba i-PLS ingathintelwa okanye inyangeke?

Ekubeni unobangela ochanekileyo we-PLS ungaziwa, okwangoku akukho ndlela yokuyithintela ukuba ingaphuhli.

Kwakhona, okubuhlungu kukuba, okwangoku akukho nyango lunokuyinyanga ngokupheleleyo i-PLS.

Ingakanani iminyaka yobomi bomntu one-PLS? Yintoni abanokuyilindela?

I-PLS ayichaphazeli ngqo ixesha ophila ngalo. Umntu one-PLS unokuphila ubomi obuqhelekileyo, njengomntu ongenaso esi sifo.

Indlela ezibonakala ngayo ngokukhawuleza iimpawu zakho kunye nendlela ezibonakala ngayo ngokukhawuleza ziyahluka ukusuka kumntu ukuya komnye. Ngokubanzi, isifo se-primary lateral sclerosis sihamba kancinci kakhulu, ngokuhamba kweminyaka, ngamanye amaxesha amashumi eminyaka. Kodwa ukuba uziva ngequbuliso iimpawu zakho zisiba mandundu, bonana nogqirha wakho ngoko nangoko.

Amayeza anokunceda ukunciphisa iimpawu zakho kwaye akuncede wenze imisebenzi yakho yemihla ngemihla ngaphandle kokuphazamiseka. Usenokufuna ukucinga ngokusebenzisa intonga, i-walker, okanye isitulo esinamavili ukuze uzive ukhuselekile kwaye uzithembile xa uhambahamba.

Ufanele uye nini kugqirha?

Ukuba ubona naluphi na utshintsho oluhamba kancinci kancinci kwizihlunu zakho, njengokuqina okanye ubuthathaka, thetha nogqirha wakho. Ukuba ufunyenwe une-PLS, ukuba naluphi na unyango olusebenzisayo lubangela ukuba iimpawu zakho zibe mandundu, okanye ukuba ufumana iziphumo ebezingalindelekanga, xelela ugqirha wakho ngaloo nto.

Eyona nto ibalulekileyo: Ukuba uyawa okanye wenzakele kwingozi, yiya kwigumbi longxamiseko ngoko nangoko.

Imibuzo omele uyibuze ugqirha wakho

Ukuba une-PLS, uya kuba nemibuzo emininzi ekufuneka uyibuze ugqirha wakho. Umzekelo:

• Zeziphi iindlela zonyango ezinokunceda ukulawula iimpawu zam?
• Zithini iziphumo ebezingalindelekanga zonyango?
• Ndingazi njani ukuba imeko yam iya kuba mandundu ngokuhamba kwexesha?
• Zeziphi iindlela zokuzilolonga endinokuzenza ukuze ndigcine izihlunu zam zisebenza kakuhle?
• Ngaba kufuneka ndisebenzise intonga okanye i-walker?

Ekugqibeleni, umyalezo wokubuyela ekhaya

Ukuziqhelanisa nemeko eqhubekayo echaphazela amandla akho okushukuma umzimba wakho kunokuba nzima kakhulu. Ezinye zezinto obukade uzonwabela ukuzenza zinokuba nzima kancinci kancinci. Nangona i-primary lateral sclerosis (PLS) iqhubeka kancinci kancinci, inokuchaphazela ubomi bakho bemihla ngemihla.

Ukuba olu tshintsho lomzimba luchaphazela nendlela ovakalelwa ngayo, ungafumana isiqabu ngokuthetha nengcali yezempilo yengqondo .Akukho nto inokubangela iintloni ngayo, abantu abaninzi bafuna uncedo ngamaxesha anje.

Ukuba ubona utshintsho kumandla emisipha yakho, bonana nogqirha ngoko nangoko. Banokukunika amayeza okanye izixhobo zokuhamba ukuze bakuncede uzimele kwaye ukhululeke ngakumbi. Khumbula, awukho kolu hambo wedwa.

I- Primary lateral sclerosis, i-PLS, ubuthathaka bemisipha, i-neuropathy, ii-motor neurons, i-ALS, iimpawu