Ngaba nawe wopha kakhulu kwinxeba elincinci? Makhe sithethe ngeBernard-Soulier Syndrome (BSS)!

Ngaba wakha waphawula ukuba abanye abantu bopha kakhulu nokuba banomkrwelo omncinci okanye inxeba nje, okanye ukuba izilonda zikhawuleza kwaye zithatha ixesha elide ukuphola? Ngamanye amaxesha oku kusenokubangelwa yimeko esingakayiva kangako, kodwa kubaluleke kakhulu ukuyazi. Enye yezi meko yiBernard-Soulier Syndrome, okanye ``(BSS)`` ngamafutshane. Makhe sithethe ngale nto ngeenkcukacha ezithe vetshe namhlanje.

Yintoni iBernard-Soulier Syndrome (BSS)?

Ngamafutshane, iBernard-Soulier Syndrome `(BSS)` yimeko engaqhelekanga kakhulu. Okwenzekayo koku kukuba inkqubo yokujiya kwegazi emzimbeni wethu, oko kuthetha ukuba ukopha kuyayeka xa senzakele, kuyaphazamiseka. Khawuthelekelele, unenxeba elincinci esandleni sakho. Emva kwexesha elithile, ukopha kuyayeka, akunjalo? Kaloku, into encedayo ngaloo nto ziiplatelets, uhlobo lweseli encinci egazini lethu, kunye neplasma, eyinxalenye yolwelo egazini.

Umntu one-Bernard-Soulier Syndrome (BSS) une-platelet ezimbalwa kuneziqhelekileyo. Oogqirha bayibiza le meko ngokuthi yi-thrombocytopenia. Kwakhona, ii-platelet ezikhoyo zinkulu kunee-platelet eziqhelekileyo. Ngenxa yezi zizathu, abantu abane-BSS bopha kwaye bagruzuke lula.

Ingaba le meko inqabile kangakanani?

Esi sisifo esingaqhelekanga kakhulu. Ngokutsho kweengcali zonyango, esi sifo sichaphazela umntu omnye kuphela kwisigidi kwihlabathi liphela. Oku kuthetha ukuba naseSri Lanka, bambalwa kakhulu abantu abaphethwe sesi sifo.

Zithini iimpawu zeBernard-Soulier Syndrome (BSS)?

Amaxesha amaninzi, iintsana ezine-BSS ziqala ukubonisa ezi mpawu kwasebuntwaneni. Jonga ukuba wena okanye umntu omaziyo unazo na ezi mpawu:

• Ufumana amanxeba lula, kwaye kuthatha ixesha elide ukuphola: Nokuba sisilonda nje esitulweni sinokushiya amanxeba amakhulu. Kungathatha iiveki ukuba loo manxeba aphole.
• Ukopha kakhulu nokuba kubangelwa zizilonda ezincinci: Nokuba ufumana isilonda esincinci ngelixa ucheba okanye usike isandla sakho ngemela ngelixa usika imifuno, kunzima ukuyeka ukopha.
• Ukopha rhoqo kwempumlo: Abanye abantu bayibiza le nto ngokuthi `(epistaxis)`. Ukuba ukopha rhoqo ngaphandle kwesizathu, loo nto nayo yimpawu.
• Ukopha phantsi kolusu: Oku kubonakala njengamachaphaza amancinci abomvu okanye amfusa phezu kolusu. Oogqirha babiza la machaphaza ngokuthi "petechiae".
• Ukopha kakhulu ngexesha lokuya exesheni kwabasetyhini: Ukuba ukopha kuthatha iintsuku ezininzi kunesiqhelo, okanye ukuba kukhulu (i-menorrhagia), oko nako kuyingxaki.

Musa ukoyika i-BSS kuba nje unempawu enye okanye ezimbini kwezi. Kodwa ukuba ezi mpawu ziyaqhubeka, kungcono ukufuna ingcebiso kagqirha.

Yintoni ebangela iBernard-Soulier Syndrome (BSS)?

Le yeyona ngongoma ibalulekileyo. `(BSS)` sisifo semfuza.Oko kukuthi, imeko eyenzeka xa sizalwa ngenxa yotshintsho (ukuguqulwa) kwizakhi zofuzo zethu. Oku kukufuzwa , oko kuthetha ukuba abantwana banokulufumana olu tshintsho kwizakhi zofuzo kubazali babo.

Le meko (BSS) ibangelwa kukuguquka kwezakhi zofuzo `GP1BA`, `GP1BB` okanye `GP9` ngokukodwa. Zonke ezi zakhi zofuzo zibaluleke kakhulu kwinkqubo yokujiya kwegazi emzimbeni wethu. Ngoko ke xa kukho ingxaki ngezi zakhi zofuzo, ukujiya kwegazi akwenzeki kakuhle.

Uyazi njani ukuba uneBernard-Soulier Syndrome (BSS)?

Ukuba unale mpawu, into yokuqala ugqirha aza kuyenza xa ekubona kukubuza umbuzo oneenkcukacha. Emva koko, baya kuyalela uvavanyo lwegazi oluninzi ukuqinisekisa imeko.

• Uvavanyo lokubalwa kweeplatelet: Olu vavanyo lujonga inani leeplatelet egazini lakho kunye nokuba ziphantsi na.
• Uvavanyo lokuguquka kwezakhi zofuzo: Oku kunokufumanisa ukuba kukho na utshintsho kwiizakhi zofuzo ze-`GP1BA`, `GP1BB`, `GP9` ezikhankanyiweyo ngaphambili ezibangela `(BSS)`.
• Uvavanyo lwee-platelet nge-microscopic: Isampuli yegazi lakho iyathathwa ize ihlolwe phantsi kwe-microscope ukuze kubonwe ukuba ii-platelet zikhulu ngokungaqhelekanga na.
• I-cytometry yokuhamba kweeplatelet: Nangona inegama elintsonkothileyo, olu vavanyo lulinganisa ubungakanani beeglycoproteins ezibizwa ngokuba yi-`GP1BA`, `GP1BB`, `GP9` kumphezulu weeplatelets. Ukuba ezi ziphantsi, luphawu lwe-`(BSS)`.

Ngokusekelwe kwiziphumo zezi mvavanyo ugqirha uza kukuxelela ngokuchanekileyo ukuba unayo i-BSS okanye akunjalo.

Ziziphi iindlela zonyango lweBernard-Soulier Syndrome (BSS)?

Okwangoku akukho nyango lwe-BSS, kuba yimeko yemfuza. Nangona kunjalo, zininzi izinto onokuzenza ukuthintela ukopha kakhulu kunye nokunciphisa ezinye iingxaki ezinokuvela. Unyango lujolise kakhulu kuloo nto.

Izinto onokuzenza ukunciphisa ukopha:

Ezi zizinto ezilula, kodwa ezibaluleke kakhulu onokuzongeza kubomi bakho bemihla ngemihla.

• Ziphephe iipilisi ezinyusa umngcipheko wokopha: Ezinye iipilisi zokuthomalalisa iintlungu, ezifana nee-nonsteroidal anti-inflammatory drugs (NSAIDs), i-aspirin, kunye nezinye ii-antihistamines zinokunyusa umngcipheko wokopha. Ngoko ke, musa ukuzithatha ezi pilisi ngaphandle kokubonisana nogqirha.
• Sebenzisa ibhrashi yamazinyo ethambileyo xa uhlamba amazinyo akho: Oku kunokunciphisa ukopha kweentsini.
• Funda indlela echanekileyo yokuthintela ukopha kwempumlo: buza ugqirha wakho malunga nendlela yokubamba impumlo yakho kunye nendlela yokubamba intloko yakho.
• Nciphisa ukutya neziselo ezithile: Ukutya okunotywala obuninzi kunye neeswekile ezicociweyo kunokuchaphazela inani leeplatelet zakho, ngoko ke kungcono ukuzinciphisa kangangoko kunokwenzeka.
• Bhalisa kwiziko elibonelela ngononophelo olungxamisekileyo olusebenza iiyure ezingama-24: Oku kuya kukunceda ufumane unyango ngokukhawuleza xa kukho imeko engxamisekileyo, efana nokopha kakhulu.
• Thatha izongezo zesinyithi ukuba unesiphene sesinyithi: Ukopha rhoqo kunokubangela ukungabikho kwesinyithi. Thatha iipilisi zesinyithi njengoko ugqirha wakho ekuyalele.
• Nxiba isongo okanye ithegi yokuchonga unyango: Kubaluleke kakhulu xa kukho imeko engxamisekileyo ukunxiba isongo okanye ithegi echaza ngokucacileyo ukuba unayo `(BSS)`.

Unyango lwezonyango:

Kwezinye iimeko, ingakumbi kwiimeko zokopha ngequbuliso okanye xa ulungiselela utyando, oogqirha banokubonelela ngonyango olufana nolu:

• Utofelo-gazi oluvela kumntu onikela ngegazi: Olu lunyango lokuqala xa kukho imeko engxamisekileyo yokopha. Iiplatelets ezivela kumntu onikela ngegazi zifakwa emzimbeni wakho ngomgca we-IV. Ezi platelets zinceda ukujiya kwegazi lakho.
• Unyango lwe-antifibrinolytic: La ngamayeza anceda ukujiya kwegazi. Umzekelo, i-tranexamic acid, osenokuba uyivile, ikwafumaneka phantsi kwegama lophawu elithi ``Cyklokapron®''. Oku kunceda ukulawula ukopha kwegazi.

Ngaba iBernard-Soulier Syndrome (BSS) ingathintelwa?

Ngelishwa, akukho ndlela yokuthintela i-BSS ekubeni ikhule kuba yimeko yemfuza.

Nangona kunjalo, ukuba kukho umntu kusapho lwakho one-BSS, okanye ukuba ucinga ukuba unokuba nale nguqu yezakhi zofuzo, ungacinga ngokuvavanya izakhi zofuzo . Kubalulekile nokufuna iingcebiso ngezakhi zofuzo . Umcebisi ngezakhi zofuzo angakuxelela ngakumbi ngale meko aze achaze umngcipheko wokuyidlulisela kubantwana bakho.

Zeziphi ezinye izinto ekufuneka uziqaphele xa uphila neBernard-Soulier Syndrome (BSS)?

Uninzi lwabantu abane-BSS banokuphila ubomi obuqhelekileyo. Nangona kunjalo, kufuneka balumke ngakumbi ukuze banciphise umngcipheko wokwenzakala.

Umzekelo, kungcono ukungabandakanyeki kwimidlalo edlalwa ngabantu abanxibelelanayo, kuba inokubangela ingozi enkulu yokwenzakala nokopha kakhulu.

Kubalulekile nokubonana nogqirha wakho rhoqo ukuze ugcine impilo yakho iyonke. Ukuba utyando, kusenokufuneka ukuba ulucwangcise kwangaphambili kwaye mhlawumbi ufakwe iiplatelet ngaphambi kokuba wenze utyando.

Ukuba uneBernard-Soulier Syndrome (BSS), yeyiphi imibuzo omele uyibuze ugqirha wakho?

Ukuba ucinga ukuba unayo (BSS) okanye ufunyaniswe unayo, kuya kuba luncedo kakhulu ukubuza ugqirha wakho imibuzo efana nale:

• Ziziphi iimpawu zokuqala zeBernard-Soulier Syndrome?
• Zeziphi iimvavanyo ekufuneka ndizenze ukuze ndifumanise ukuba ndinayo na iBernard-Soulier Syndrome?
• Ziziphi iindlela zonyango lweBernard-Soulier Syndrome?
• Ukuba ndineBernard-Soulier Syndrome, ndingenza ntoni ukuthintela ukopha kakhulu?
• Zeziphi izinto endingafanele ndizenze kuba ndineBernard-Soulier Syndrome?
• Ngawaphi amathuba okuba abantwana bam bafumane iBernard-Soulier Syndrome kum?

Ukongeza kule mibuzo, ungoyiki ukuthetha nogqirha ngayo nayiphi na into efika engqondweni yakho.

Ngaba ukuba neBernard-Soulier Syndrome (BSS) ngexesha lokukhulelwa kuyingozi kumntwana?

Ukuba ukhulelwe kwaye une-BSS, uya kufuna unyango olukhethekileyo (unyango olunobungozi obukhulu lokubeleka kunye ne-hematology) . Inani leeplatelet zakho kunye nempilo iyonke kuya kufuneka zijongwe rhoqo. Ngexesha lokubeleka, usenokungakwazi ukwenza iintlobo ezithile ze-anesthesia ezandisa umngcipheko wokopha.

Kwanasemva kokuba umntwana ezelwe, inani leeplatelet zomntwana kufuneka lihlolwe rhoqo. Kuba kukho ithuba lokuba umntwana wakho azuze i-genetic mutation ebangela ``(BSS)'' kuwe. Ngoko ke, kubalulekile ukuthetha nogqirha wakho ngale nto kwaye uthathe amanyathelo afunekayo.

Yintoni ingxaki ephambili ebangelwa yiBernard-Soulier Syndrome (BSS)?

Ngamafutshane, ingxaki ephambili ebangelwa yi-"BSS" kukungabi namandla kwegazi ukujiya . Yiyo loo nto ukopha kakhulu kunye nokukrweleka okulula zezona mpawu zixhaphakileyo.

Ziziphi ezinye iimeko ezibangela iimpawu ezifana neBernard-Soulier Syndrome (BSS)?

Kukho ezinye iimeko ezininzi ezibangela iingxaki zokopha kunye neempawu ezifana neze-BSS. Kulungile ukuzazi ngamagama kuphela ezi zinto:

• 'I-Hemophilia'
• `UMeyi-Hegglin ongaqhelekanga`
• `Isifo sedama lokugcina izinto`
• `I-Glanzmann thrombasthenia`
• `Isifo sikaVon Willebrand`

Ngokusekelwe kwiimpawu zakho, ngugqirha kuphela onokumisela ukuba zeziphi ezi zifo.

Amazwi ethu okugqibela

I-Bernard-Soulier Syndrome `(BSS)` yimeko engaqhelekanga kakhulu efunyanwa ngabazali bakho. Ukuba unale meko, unokuba neengxaki ezithile zokujiya kwegazi. Nangona kunjalo, akuthethi ukuba awunakuphila ubomi obuqhelekileyo. Eyona nto ibalulekileyo kukuphepha iimeko ezinobungozi ezinokukhokelela ekwenzakaleni kwaye uhlale ulandela icebiso likagqirha wakho.

Bona ugqirha wakho wosapho rhoqo. Angaphendula imibuzo yakho, akuncede ulawule le meko, aze asebenzisane nawe ukugcina impilo yakho iyonke. Musa ukoyika, ukuqonda kunye nokukhathalela zezona zinto zibalulekileyo kuthi xa siphila nezi meko.

Isifo sikaBernard -Soulier, i-BSS, ukujiya kwegazi, iiplatelets, ukopha okugqithisileyo, izifo zemfuza, ukukrweleka