Ingaba ulusu lwakho lubuthathaka kakhulu ekukhanyeni kwelanga? Oku kusenokuba yi (Xeroderma Pigmentosum - XP)!

Namhlanje siza kuthetha ngemeko yempilo engaziwayo kangako, kodwa ebaluleke kakhulu. Ngaba wakha waphawula ukuba abanye abantu batsha lilanga baze babe bomvu nangona sele bebekwe elangeni kancinci? Ngamanye amaxesha oku kuyenzeka nakubantwana abancinci. Esinye isizathu esinokubakho sesi sifo sibizwa ngokuba yiXeroderma Pigmentosum , okanye iXP ngamafutshane. Namhlanje siza kuthetha nje ngokuba yintoni, kutheni isenzeka, kwaye singaphila njani nayo.

Kuthetha ukuthini oku (XP)? Masiqonde kakuhle!

Ngamafutshane, iXeroderma Pigmentosum (XP) yimeko engaqhelekanga yemfuza . Xa usiva igama elithi "imfuza," mhlawumbi ucinga ngento efana nale. Oko kukuthi, yinto esiyizuza njengelifa kubazali bethu, okanye kubo bobabini. Abantu abanale meko banolusu olubuthathaka kakhulu kwimitha ye-ultraviolet (UV) evela elangeni. Ngokuchanekileyo, ulusu lwabo lunzima ukufunxa le mitha ye-UV.

Ngokwesiqhelo, iimpawu zesi simo (XP) zivela kwiindawo zomzimba ezihlala zichatshazelwa lilanga. Umzekelo, ebusweni, ezandleni, nasemilebeni . Ezi mpawu zihlala ziqala ukubonakala esemncinci, oko kukuthi, ebuntwaneni . Khawucinge nje, nomntwana omncinci angachatshazelwa lilanga imizuzu embalwa, kwaye ulusu lwakhe lunokuba namadyungudyungu kwaye lutshiswe (ukutsha lilanga okuqhumayo).

Ingaba umhlaza (XP) ngumbuzo abantu abaninzi abanawo!

Hayi, i-XP ayingomhlaza ngqo . Kodwa nantsi ingongoma ebalulekileyo. Umntu one-XP usemngciphekweni omkhulu wokuba nomhlaza wolusu kunabanye.

Nangona eli bali liyoyikisa kancinci, kubalulekile ukuba uqaphele. Jonga ezi zibalo:

• Umntu one-XP unamathuba aphindwe ka-10,000 okuba nomhlaza wesikhumba ongengomelanoma , njenge- basal cell carcinoma okanye i-squamous cell carcinoma, kunomntu oqhelekileyo.
• Kwakhona, umngcipheko wokuba ne -melanoma, umhlaza wolusu onzima, uphezulu ngokuphindwe kayi-2,000 .

Ukongeza kwezi ntlobo zomhlaza wolusu, ezinye izifundo zifumanise ukuba abantu abane-XP banomngcipheko omkhulu wokufumana ezinye iintlobo zomhlaza. Umzekelo:

• Iithumba zobuchopho (i-Astrocytoma) , (i-Glioblastoma)
• Umhlaza webele
• Umhlaza wezintso
• I-Leukemia
• Umhlaza wemiphunga
• Umhlaza wePancreatic
• Umhlaza wesisu
• Umhlaza wamatyhalarha
• Umhlaza we-thyroid
• Umhlaza wesibeleko

Musa ukothuka lolu luhlu. Ayizizo zonke ezi cancer eziya kuvela kwisigulane ngasinye (XP). Nangona kunjalo, kubalulekile ukuqaphela iingozi.

Ixhaphake kangakanani le meko?

I-Xeroderma Pigmentosum yimeko engaqhelekanga kakhulu . Kuqikelelwa ukuba ngumntu omnye kuphela kwisigidi kumazwe afana ne-United States ne-Europe onayo le meko (XP).

Nangona kunjalo, ixhaphake kakhulu kwezinye iindawo zehlabathi. Umzekelo, eJapan, le meko ichaphazela malunga nomntu omnye kwabangama-22,000. Ixhaphake kakhulu nakuMntla Afrika nakuMbindi Mpuma. Nangona kungekho datha ichanekileyo ngokuxhaphaka kwayo eSri Lanka, ithathwa njengesifo esingaqhelekanga.

Zithini iimpawu ze-(XP)? Masizihlalutye!

(XP) Iimpawu zinokwahluka kumntu nomntu, kodwa ikakhulu zichaphazela ulusu, amehlo kunye nenkqubo yemithambo-luvo .

Iimpawu zolusu

Ezi zezona mpawu zibonakala kakhulu:

• Ukutsha kwelanga okutshayo: Nokuba emva kwexesha elifutshane lokuchatshazelwa lilanga, ulusu luyadumba kwaye lutshiswe.
• Ulusu olomileyo noluncinci (Xerosis): Ulusu lomile kakhulu, kwaye lunokuba luncinci njengephepha lesikhumba.
• IiFreckles (iiLentigos) ngaphambi kweminyaka emi-2 ubudala: Ngokwesiqhelo, abantwana abancinci abaphuhlisi iiFreckles kwangethuba.
• Amabala ombala wolusu owandileyo nowehlayo (iPoikiloderma): Ezinye iindawo zinokuba mdaka, ngelixa ezinye zinokuba mhlophe.
• Ukuncitshiswa kolusu (Atrophy).
• Imigca ebomvu eluswini ebangelwa kukwanda kwemithambo yegazi - iTelangiectasia: Ibonakala njengemigca ebomvu kuba imithambo yegazi emincinci ibonakala phezu komphezulu.

Ukongeza, abantu abane-XP basengozini enkulu yokufumana izilonda zesikhumba ezibizwa ngokuba yi -actinic keratoses, ezinokuba ngumhlaza .

Iimpawu zamehlo

Rhoqo, abantu abane-XP banokuba neempawu zamehlo ngaphambi kokuba bafikelele kwiminyaka eli-10 :

• Amehlo omileyo.
• Ukuwohloka/ukushwabana kwamehlo.
• Ukudumba kwe-cornea yakho (iKeratitis).
• Ukungabikho kokukhanya kwileya engaphandle yeliso lakho - Ukukhanya kwe-Cornea: Oku kuthetha ukuba inxalenye efana nesipili yeliso iba namafu.
• Uvakalelo lokukhanya (Photophobia): Ubunzima bokunyamezela nokukhanya okuncinci.
• Ukulahlekelwa ziinkophe.

Ekuhambeni kwexesha, ezi mpawu zinokubangela ukulahleka kokubona, kwanobumfama . Abantu abane-XP nabo basengozini yokuba nomhlaza wamehlo .

Iimpawu ze-neurological

I-XP inokuchaphazela nenkqubo yakho yemithambo-luvo. Malunga nekota yabantu abane-XP baya kuba neempawu ezinxulumene nenkqubo yemithambo-luvo. Imizekelo ibandakanya:

• I-Dysphagia - ubunzima bokuginya.
• Ukulahlekelwa yi-reflexes.
• Ukulawulwa kakubi kwemisipha (i-Ataxia) kunye nokuqina (i-Spasticity).
• Ukulahlekelwa okuqhubekayo kwezakhono zokucinga (zokuqonda).
• Ukulahlekelwa kukuva okuqhubekayo okubangelwa kukonakala kwemithambo-luvo yangaphakathi endlebeni.
• Ubukhulu bentloko obuncinci (iMicrocephaly).
• Ukukhubazeka kwentambo yelizwi.

Iingcali zikholelwa ukuba ezi mpawu zibangelwa kukutshatyalaliswa kweeseli zemithambo-luvo engqondweni.

Yintoni isizathu sokwenziwa kwe (XP)?

Njengoko besitshilo ngaphambili, iXeroderma Pigmentosum sisifo semfuza . Oku kuthetha ukuba le meko ibangelwa lutshintsho olungaqhelekanga (iinguqu) oluvela kwiijini emizimbeni yethu. Olu tshintsho lwemfuza lunokuzuzwa kumama wakho, kutata wakho, okanye kuzo zombini. Ngamafutshane, lubangelwa bubuthathaka kwiijini ezinceda ulusu lwethu ukulungisa umonakalo obangelwa yimitha ye-UV.

Ndingafumanisa njani ukuba ndinayo (XP)?

Ugqirha wakho usenokucinga ukuba une-XP ngokweempawu zakho. Ukuqinisekisa ukurhanelwa, baza kwenza uvavanyo lwegazi . Olu vavanyo lwegazi lujonga utshintsho lwezakhi zofuzo olubangela i-Xeroderma Pigmentosum.

Ngaba ikho indlela epheleleyo yokunyanga (XP)?

Ngelishwa, okwangoku akukho nyango lwe-Xeroderma Pigmentosum. Nangona kunjalo, ugqirha wakho unokucebisa iindlela ezahlukeneyo zonyango ukunceda ukunciphisa iimpawu nokwenza ubomi bakho bube lula:

• Amaconsi amehlo: Amaconsi aqabisayo anganikwa ukunciphisa ukudumba komhlophe weliso.
• Izixhobo zokuncedisa ukuva: Ukuba kukho ukulahleka kokuva, izixhobo zokuncedisa ukuva zinokunikwa. Kwezinye iimeko ezinzima , ii-implants ze-cochlear zinokucetyiswa.
• Utyando: Ukuba unesifo somhlaza wolusu, ungadinga utyando ukuze ususwe. Okanye ungadinga utyando ukuze unyange iimeko ezithile zamehlo, ezinje ngeenkophe ezigobileyo (i-ptosis) okanye iingxaki ngomhlophe weliso. Kwezinye iimeko ezinzima , ukufakelwa i-cornea kungafuneka.
• Izongezo zeVitamin D:Uninzi lwabantu lufumana i-vitamin D ekukhanyeni kwelanga nasekutyeni. Abantu abane-XP kufuneka baphephe ukukhanya kwelanga phantse ngokupheleleyo, ngoko ke kufuneka bathathe isongezelelo se-vitamin D.

Ngaba i-XP ingathintelwa?

Ngenxa yokuba i-XP yimeko yemfuza, akukho ndlela yokuyithintela ukuba ingakhuli . Ukuba ugqirha wakho ukrokrela ukuba une-gene mutation ebangela i-XP, ungenza uvavanyo lwemfuza .

Uvavanyo lwemfuza lunokukuxelela ukuba unayo na i-gene mutation. Umcebisi ngezemfuza uza kukuchazela iziphumo, iingozi ezibandakanyekileyo, kunye namathuba okuba nomntwana one-XP.

Yintoni ekufuneka uyiqwalasele xa uhlala ne-(XP)?

Ukuba une-Xeroderma Pigmentosum, kukho izinto ezimbalwa ekufuneka uziqwalasele ukuze ugcine impilo yakho iyonke. Nazi izinto ezibaluleke kakhulu:

• Ukukhuselwa lilanga: Eli lelona nyathelo libalulekileyo ekulawuleni i-XP. Kubalulekile ukukhusela ulusu lwakho kwimitha ye-UV.
• Nxiba impahla ekhusela ilanga . Oku kuthetha iihempe ezineemikhono emide, iibhulukhwe ezinde, iikawusi kunye neminqwazi.
• Nxiba iiglasi zelanga ezithintela i-UV .
• Sebenzisa i- broad-spectrum sunscreen ene- SPF 35+ nangaphezulu yonke imihla. Nokuba ayibonakali inelanga, imisebe ye-UV isenokufikelela emafini.
• Iingcali zicebisa ukuba usebenzise imitha yokukhanya ye-UV ukuze ufumane apho indawo okuyo ichaphazeleka khona kakhulu yimitha ye-UV kwaye uyiphephe. ​​Ezinye izibane ezingaphakathi ekhayeni lakho zinokukhupha imitha ye-UV, ngoko ke qaphela loo nto.

• Ukuhlolwa kwamehlo: Yiya kugqirha wamehlo ukuze ahlolwe amehlo akho rhoqo. Uza kujonga izinto ezifana neenkophe ezivuzayo kunye nokudumba kwamehlo. Jonga amehlo akho ubuncinane kanye ngonyaka, okanye rhoqo njengoko ugqirha wakho ecebisa.

• Ukuhlolwa kolusu: Bona ugqirha wesikhumba rhoqo emva kweenyanga ezi-6 ukuya kwezili-12, okanye rhoqo njengoko ugqirha wakho ecebisa. Baza kujonga iimpawu zomhlaza okanye ii-moles ezinokuba ngumhlaza. Ngelixa useofisini kagqirha, jonga ulusu lwakho ubuncinane kanye ngenyanga . Ukuba ubona amabala okanye amaqhuma amatsha, bona ugqirha wesikhumba ngoko nangoko.

• Ukhathalelo lwe-neurological: Abantu abane-(XP) kufuneka babone ugqirha we-neurological.Kufuneka udibane nabo rhoqo ukuze uhlole indlela abasabela ngayo, indlela abava ngayo, njl. Ukuba ugqirha ubona naluphi na utshintsho kule misebenzi, uya kucebisa unyango lokulawula okanye lokuthomalalisa imeko embi.

Khumbula, konke oku kukuzikhusela. Ukuba ulandela ezi zinto ngokufanelekileyo, ungaphila ubomi obuphumelelayo nangona une-(XP).

Ungathini ngobomi bomntu one-(XP)?

Ixesha lokuphila komntu one-Xeroderma Pigmentosum linokwahluka kumntu nomntu. Kwiimeko ezininzi, umhlaza wolusu unokufunyaniswa eselula kakhulu, nto leyo enokunciphisa ixesha lokuphila. Nangona kunjalo, ukuthatha amanyathelo okuthintela umonakalo wolusu olubangelwa lilanga ukususela ebuntwaneni kunokwandisa amathuba akho okuphila ubomi obude . Ke ngoko, kubaluleke kakhulu ukulandela amanyathelo okhuseleko akhankanywe apha ngasentla.

Imibuzo omele uyibuze ugqirha wakho

Ukuba wena okanye umntwana wakho une-Xeroderma Pigmentosum, okanye ukuba ukrokrela ukuba unayo, ungabuza ugqirha wakho le mibuzo:

• Ndingenza ntoni ukunciphisa umngcipheko wokuba nomhlaza wolusu?
• Loluphi uhlobo lovavanyo lomhlaza ekufuneka lwenziwe?
• Ziziphi iimpawu zokuqala zomhlaza wolusu?
• Olu xilongo luza kuyichaphazela njani yonke intsapho yam?
• Ngaba kufuneka ndicinge ngovavanyo lwemfuza?
• Angakanani amathuba okuba ndibe nomnye umntwana one (Xeroderma Pigmentosum)?

Okokugqibela, izinto ekufuneka uzikhumbule

Kulungile, ngoko ke esi sifo besithetha ngaso (i-Xeroderma Pigmentosum - XP) sisifo esingaqhelekanga, esibangelwa lufuzo. Uphawu oluphambili lwale nto kukuba novakalelo olukhulu kwimitha ye-(UV). Abantu abaninzi banengxaki yokukhula kwamehlo kunye nenkqubo yemithambo-luvo. Ukuba une-(XP), kufuneka uphephe ukukhanya kwelanga okuninzi, oko kukuthi, imitha ye-(UV), kangangoko kunokwenzeka . Ukukhusela ulusu lwakho elangeni kunye nokufumana uvavanyo lomhlaza wolusu rhoqo lelona thuba lakho lilungileyo lokuhlala usempilweni.

Ndiyathemba ukuba olu lwazi luza kukunceda. Ukuba unemibuzo, ungathandabuzi ukubuza ugqirha wakho. Baza kukunceda.

I- Xeroderma Pigmentosum, i-XP, izifo zesikhumba, izifo zemfuza, ukuva ukukhanya kwelanga, umhlaza wesikhumba, imisebe ye-UV