Ngaba uyayazi ingxaki yeSickle Cell? Makhe sithethe ngale meko ibuhlungu!

Ukuba unesifo se-sickle cell, usenokuba ukhe weva okanye wahlaselwa yintlungu ngequbuliso. Oku sikubiza ngokuba yi-sickle cell crisis. Kungaba ngamava abuhlungu kakhulu. Kodwa ungakhathazeki, siza kuthetha ngayo ngokweenkcukacha namhlanje.

Yintoni le ngxaki ye-sickle cell?

Ngamafutshane, isifo se-sickle cell sithetha xa 'iiseli zesickle' zomzimba wakho, okanye iiseli ezibomvu zegazi ezimile okwesickle, zibhajwa emthanjeni wegazi, zicothisa okanye ziyeke ukuhamba kwegazi. Uyazi, iiseli zethu ezibomvu zegazi zezona zithwala ioksijini emzimbeni wonke. Ngoko ke, xa unesifo se-sickle cell, izicubu zakho kunye namalungu omzimba awafumani ioksijini eyaneleyo. Oku kunokubangela iintlungu ezinzima kunye nezinye iingxaki .

Cinga nje ngokuba xa uthuli oluncinci lubhajwe kumbhobho wamanzi, ukuhamba kwamanzi kuyehla. Kodwa okubhajwe apha zezo seli zibomvu zegazi ezimile okwesikela. Ezi azigobi lula njengeeseli ezibomvu zegazi eziqhelekileyo, kwaye azityibiliki kwiindawo ezincinci. Yiyo loo nto zizama ukubhajwa.

Ngamanye amaxesha le meko inokulawulwa ekhaya, kodwa ukuba iimpawu zinzima, kunokufuneka uhlale esibhedlele iintsuku ezimbalwa ukuze ufumane unyango.

Ziziphi iintlobo zengxaki ye-sickle cell?

Kukho iintlobo ezine eziphambili zengxaki ye-sickle cell. Makhe sijonge ukuba zithini:

• Ingxaki yokuvaleka kwemithambo yegazi: Olu lolona hlobo luxhaphakileyo. Okwenzekayo apha kukuba iiseli zesikele zibambeke kwii-capillaries zakho okanye kwezinye imithambo yegazi emincinci, nto leyo enciphisa ukuhamba kwegazi kwiithishu zakho kunye namalungu omzimba. Yiyo le nto ibangela iintlungu eziqatha. Ezinye iintlobo zichaphazela iindawo ezithile zomzimba.

• Ingxaki ye-Aplastic: Oku kwenzeka xa umongo wakho wethambo (indawo engaphakathi emathanjeni akho eyenza iiseli zegazi) ngequbuliso uyeka ukuvelisa iiseli ezibomvu zegazi. Oku kudla ngokubangelwa zizifo ezithile zentsholongwane, ingakumbi i-parvovirus B19.

• Ingxaki ye-Hyperhemolytic: Oku kwenzeka xa inqanaba le-hemoglobin yakho (iproteni ekwiiseli ezibomvu zegazi ezithwala ioksijini) yehla ngequbuliso. Oku kungenxa yokuba iiseli zakho ezibomvu zegazi ziyaqhekeka ngokukhawuleza. Oku kunokwenzeka nasemva kotofelo-gazi, olu lunyango oluqhelekileyo lwesifo se-sickle cell.

• Ukususwa kwe-splenic: Oku kwenzeka xa iiseli ze-sickle zinamathela kwi-spleen yakho (ilungu elikwicala eliphezulu lasekhohlo lesisu sakho), nto leyo ebangela ukuba i-spleen ikhule ngequbuliso ize idumbe kabuhlungu . Oku kunokuba yingozi kakhulu kubantwana abancinci, njengoko inani elikhulu legazi linokuqokelelana kwi-spleen, nto leyo ebangela ukuba igazi linciphe.

Zithini iimpawu zoku?

Iimpawu zesifo se-sickle cell zinokwahluka ngokuxhomekeke ekubeni sichaphazeleke phi emzimbeni. Iimpawu eziqhelekileyo ziquka:

• Isiyezi.
• Ukudinwa kakhulu, ukudinwa.
• Intloko ebuhlungu.
• I-Jaundice (ukutyheli kolusu namehlo).
• Intlungu (le yeyona mpawu iphambili neyona inzima).
• Ubunzima bokuphefumla.
• Ubuthathaka.

Intlungu yesifo se-sickle cell inokuba buthuntu, ibukhali, ihlaba, okanye ihlabe . Inokuhlala iiyure, iintsuku, okanye iiveki. Intlungu ingenzeka naphi na emzimbeni, kwindawo enye okanye kwiindawo ezininzi. Umzekelo:

• Ezandleni okanye entendeni yesandla.
• Esifubeni.
• Emilenzeni okanye ezinyaweni.
• Kwindawo esezantsi yomqolo.
• Esiswini.

Kutheni le nto kukho le ngxaki ye-sickle cell?

Isifo seSickle cell senzeka xa iiseli ezibomvu zegazi ezimile okwesickle zihlangana zize zivale umthambo wegazi. Nangona kunjalo, iingcali azisoloko zisazi ukuba yintoni ebangela oku kuqale.

Nangona kunjalo, kukho ezinye izinto ezinokubangela esi sifo:

• Ukuphelelwa ngamanzi emzimbeni : Xa umzimba ungenamanzi, igazi liyaqina kwaye iiseli zinokuba sengozini yokuvaleka.
• Ukuya kwiindawo eziphakamileyo (kuba kukho ioksijini encinci kwiindawo eziphakamileyo).
• Izifo : Naluphi na usulelo, olufana nomkhuhlane okanye umkhuhlane.
• Ukukhulelwa.
• Ukutshaya okanye ukutshaya i-vaping.
• Umsebenzi womzimba onzima kakhulu.
• Uxinzelelo : Ukukhathazeka kakhulu kunye noloyiko.
• Utshintsho olukhulu ngequbuliso kubushushu (umz., ukuhlamba ngamanzi abandayo emva kokuba kwindawo eshushu kakhulu).
• Ukunqongophala kweevithamini ezithile, umzekelo i-folate.

Zeziphi iingxaki ezinokubangela oku?

Ubunzima bengxaki ye-sickle cell buxhomekeke ekubeni iphi imithambo yegazi evalekileyo. Ukuba imithambo yegazi ivalekile kwizicwili okanye kumalungu omzimba wakho, iingxaki ezifana nezi:

• I-Acute chest syndrome : Le yimeko enzima nesongela ubomi eyenzeka esifubeni. Iimpawu ziquka umkhuhlane, iintlungu esifubeni, kunye nobunzima bokuphefumla.
• Amahlwili egazi.
• Intlungu engapheliyo (intlungu eqhubekayo).
• Ukwanda kwepleen kunye nezinye izifo ezinxulumene nepleen.
• Amanxeba angapholiyo emilenzeni.
• Isifo sesibindi.
• Izifo zemiphunga, umzekelo, uxinzelelo lwegazi oluphezulu emaphaphu.
• I-Necrosis : Ukufa kwethambo okanye izicwili (ingakumbi kwiindawo ezifana ne-hip) ngenxa yokulahlekelwa kukuhamba kwegazi.
• Ukuqhwalela okubuhlungu kwepenis (okubizwa ngokuba yi-'priapism' kumadoda).
• Istroke : Ibangelwa kukuvaleka okanye ukuqhekeka komthambo wegazi ohambisa igazi engqondweni.

Uyibona njani le nto?

Ukuba unesifo se-sickle cell, mhlawumbi sele ubona ugqirha wakho rhoqo kwiinyanga ezi-6 okanye kube kanye ngonyaka ukuze ahlolwe igazi. Kodwa ukuba unengxaki ye-sickle cell, kuya kufuneka wenze uvavanyo olupheleleyo lwelabhoratri. Ezi zingabandakanya:

• Uvavanyo lohlobo lwegazi (ukuba udinga utofelo-gazi).
• I-CBC (Ubalo lwegazi olupheleleyo) : Oku kulinganisa amanqanaba e-hemoglobin kunye nenani leeseli zegazi ezibomvu nezimhlophe.
• I-CMP (Iphaneli yeMetabolic eNgqinisisayo) : Iseti epheleleyo yovavanyo lwemetabolism olujonga ukusebenza kwamalungu anjengezintso nesibindi.
• Uvavanyo lokusebenza kwesibindi .
• Inani lee-reticulocyte : Oku kulinganisa inani leeseli ezibomvu zegazi ezisandula ukwenziwa. Oku kunokunika umbono wokuba umongo wethambo usebenza kakuhle kangakanani.

Ukongeza, ugqirha wakho unokwenza uvavanyo olupheleleyo lomzimba kunye novavanyo lwemifanekiso olufana noku:

• I-Doppler ultrasound : Jonga indlela igazi elihamba ngayo ngemithambo yegazi.
• I-MRI : Ingakumbi ukuba kukho isikrokro semeko efana nestroke.
• I-Ultrasound : Ukujonga izinto ezifana nezitho zesisu kunye ne-spleen.
• I-X-ray yesifuba : Ukujonga imeko efana ne-acute chest syndrome.

Iphathwa njani?

Ukuba unengxaki ye-sickle cell, ungadinga iintlungu zokunciphisa iintlungu ukunceda ukulawula iimpawu ezingathandekiyo. Kwiimeko ezincinci, ungayilawula le nto ekhaya ngamayeza athengiswa ngaphandle kwemvume kagqirha anjenge-acetaminophen (umz. iPanadol®, iTylenol®) okanye i-ibuprofen (umz. i-Advil®, iBrufen®).

Nangona kunjalo, kwiimeko zeentlungu eziqatha, kunokufuneka uhlale esibhedlele iintsuku ezimbalwa. Oogqirha banokukunika amayeza okudambisa iintlungu anamandla (njenge-morphine), ulwelo lwe-IV (ukugcina igazi lakho lihamba kakuhle), kunye neoksijini ukuba kuyimfuneko.

Ukongeza, unyango lwesifo se-sickle cell luyahluka ngokuxhomekeke kumalungu omzimba achaphazelekileyo kunye nobukhali beempawu. Oogqirha banokusebenzisa amayeza, utofelo-gazi, ukufakelwa umongo wethambo, okanye indibaniselwano yale mithi.

Amayeza okulawula ixesha elide

Kukho iintlobo ezahlukeneyo zamayeza asetyenziswa ngoogqirha ukulawula iingxaki ze-sickle cell ixesha elide nokunciphisa ukwenzeka kwazo:

• I-Hydroxyurea: Eli yeza lenza iiseli zakho ezibomvu zegazi zibe nkulu, landisa amandla azo okugoba, kwaye landisa uhlobo lwe-hemoglobin olubizwa ngokuba yi-fetal hemoglobin. Oku kunciphisa ukujiya kweeseli.
• Crizanlizumab `(Crizanlizumab)`:Olu luhlobo lwe-antibody ye-monoclonal. Ingathintela iiseli zakho ezibomvu zegazi ukuba zinganamatheli eludongeni lwemithambo yegazi yakho.
• I-L-glutamine: Le yi-amino acid enceda ekunciphiseni umonakalo kwiiseli ezibomvu zegazi.
• I-Voxeletor: Eli yeza lithintela iiseli zakho ezibomvu zegazi ukuba zingabanjwa zize zinamathelene.

Utofelo-gazi

Oogqirha banokusebenzisa utofelo-gazi ukunyanga iingxaki ze-sickle cell ezibangelwa yiplastiki okanye i-hyperhemolytic. Olu nyango lunokugcwalisa iiseli zakho ezibomvu zegazi ezisempilweni kwaye lunciphise umngcipheko weengxaki ze-sickle cell. Abanye abantu banokufuna utofelo-gazi rhoqo.

Ukufakelwa kwezityalo

Ukufakelwa kwethambo lomongo okanye ukufakelwa i-stem cell kunokuba yindlela ekuphela kwayo yokunyanga isifo se-sickle cell kwezinye iimeko. Nangona kunjalo, ukufumana umntu oza kumnika imali efanayo (njengomntakwenu) kungathatha ixesha. Kwakhona, ukufakelwa kwethambo lomongo kunokubangela ezinye iingxaki ezinkulu, ezifana nesifo se-graft vs. host (GvHD). Ngenxa yesi sizathu, oogqirha abafane benze ukufakelwa kwethambo lomongo xa benyanga iingxaki ze-sickle cell. Endaweni yoko, injongo ephambili kukunyanga iimpawu zakho kunye nokunciphisa iintlungu zakho.

Ngaba oku kungathintelwa?

Iingxaki zeSickle cell azinakuthintelwa rhoqo. Nangona kunjalo, kukho izinto ezithile ezinokunceda ukunciphisa umngcipheko:

• Kuphephe ukutshaya ngokupheleleyo.
• Ukuba ukwindawo ebandayo, nxiba shushu. Ukubanda kunokuxinanisa imithambo yegazi kwaye kubangele ingxaki.
• Nciphisa okanye uyeke ngokupheleleyo ukusela utywala . Utywala bunokubangela ukugcinwa kwamanzi.
• Sela ubuncinane iiglasi ezisi-8-10 zamanzi ngosuku (hlala unamanzi aneleyo).
• Sela iipilisi ze-folic acid yonke imihla ukuze wonyuse ukuveliswa kweeseli ezibomvu zegazi (oku kufuneka kwenziwe ngokwengcebiso kagqirha).
• Hlamba izandla zakho ngesepha rhoqo ukuze unciphise umngcipheko wokosuleleka.
• Fumana zonke iigonyo ezifunekayo ngexesha elifanelekileyo (umz. igonyo lomkhuhlane, igonyo le-pneumonia).
• Kuphephe ukwenza umthambo onzima kakhulu, kodwa ukwenza umthambo ophakathi kulungile.
• Fumana iindlela zokunciphisa uxinzelelo (umz. i-yoga, ukucamngca).

Ndingazinyamekela njani (xa kuvela ingxaki)?

Ukuba wena okanye umntu osondeleyo kuwe unengxaki ye-sickle cell, ukwenza ezi zinto kunokunceda ekunciphiseni iimpawu:

• Zigcine ushushu. Ukuhlamba ngamanzi ashushu okanye ukubeka i-heating pad kwindawo ebuhlungu kuya kuyikhulisa imithambo yegazi yakho kwaye kuphucule ukuhamba kwegazi. Ubushushu buya kunceda ekuthomalaliseni izihlunu ezibuhlungu.(Musa ukufaka iipakethe zomkhenkce. Ukubanda kunokubangela intlungu. Ngoko ke kungcono ukuhlala ufudumele kangangoko.)
• Sela amanzi amaninzi. Xa usela amanzi aneleyo, iiseli zakho ezibomvu zegazi azinamatheli kakhulu, nto leyo evumela ukuba zihambe ngokulula kwiindlela ezijikelezayo.
• Fumana umasaji. Umasaji othambileyo unokunyusa ukuhamba kwegazi, uphumze izihlunu, kwaye unciphise iintlungu.
• Ziqhelise ukuqaphela. Uphando lubonise ukuba ukujolisa ingqondo yakho kumgomo omnye ixesha elifutshane kunokunceda ukuphazamisa ingqondo yakho kwaye kunciphise iintlungu ezinxulumene nesifo se-sickle cell. Nokuba yinto elula njengokugxila ekuphefumleni kwakho inokunceda.
• Sela amayeza okubulala iintlungu athengiswa ngaphandle kwemvume kagqirha. La aquka i-paracetamol, i-ibuprofen, okanye i-naproxen (umz. i-Aleve®). Kodwa musa ukuthatha amayeza angaphezu kwalawo amiselwe ngugqirha wakho.

Ufanele uye nini kwiYunithi yoNyango oluNgxamisekileyo (i-ETU) ?

Ukuba unayo nayiphi na kwezi mpawu, yiya kwiSebe leeNgxamiseko (ETU) elikufutshane ngoko nangoko :

* Ukuba unentlungu ebuhlungu ngequbuliso, engenakulawuleka ngamayeza asekhaya.

* Ubunzima bokuphefumla okanye iintlungu esifubeni.

* Umkhuhlane ophezulu (ngaphezulu kwe-101°F okanye i-38.3°C).

* Ubuthathaka ngequbuliso, ubunzima bokuthetha, ukugoba kwelinye icala lobuso (iimpawu zestroke).

* Intlungu ebuhlungu yesisu okanye ukudumba.

* Ukuba i-jaundice iyanda.

* Kumadoda, ukuxhoma okubuhlungu kuthatha iiyure ezingaphezu kwezine.

Musa ukulibazisa. Ukuqala unyango kwangethuba kunokunciphisa umngcipheko weengxaki ezihlala ixesha elide.

Izinto ezibaluleke kakhulu ekufuneka uzikhumbule

Ukuba unesifo se-sickle cell, ingcinga yengxaki inokuba yinto ekuphazamisayo. Ezi meko zinokubangela iimpawu ezingathandekiyo kunye neengxaki zobomi bonke. Ukuba wakha waba nengxaki ye-sickle cell, uyazi ukuba ixesha lixabiseke kangakanani.

Ukuba iimpawu zakho aziphucuki ngee-painkillers ezithengiswa ngaphandle kwe-counter okanye amayeza akho aqhelekileyo asekhaya, lixesha lokubona ugqirha wakho. Balapha ukukunceda. Ungoyiki ukuthetha nogqirha wakho ngemeko yakho. Unyango lwangethuba lolona lulungileyo. Sebenzisana nogqirha wakho ukuze uyile isicwangciso sokulawula iintlungu esikusebenzelayo.

Ingxaki yeSickle Cell, Isifo seSickle Cell, Iiseli zeGazi eziBomvu, Intlungu, Imithambo yegazi, iOksijini