Uyazazi izinkinga ze-Sickle Cell? Ake sixoxe ngalesi simo esibuhlungu!

Uma unesifo se-sickle cell, kungenzeka ukuthi uke wezwa noma wabhekana nokuhlaselwa ubuhlungu okungazelelwe, okunzima. Lokhu yilokho esikubiza ngokuthi inkinga ye-sickle cell. Kungaba yinto ekhathazayo futhi ebuhlungu kakhulu. Kodwa ungakhathazeki, sizoxoxa ngakho ngokuningiliziwe namuhla.

Iyini le nkinga ye-sickle cell?

Kalula nje, isifo se-sickle cell yilapho 'amaseli esikela' omzimba wakho, noma amaseli abomvu egazi amise okwesikela, ebhajwa emthanjeni wegazi, ehlisa noma emisa ukugeleza kwegazi. Uyazi, amaseli ethu abomvu egazi yiwo athwala umoya-mpilo kuwo wonke umzimba. Ngakho-ke, uma unesifo se-sickle cell, izicubu zakho nezitho zomzimba azitholi umoya-mpilo owanele. Lokhu kungabangela ubuhlungu obukhulu kanye nezinye izinkinga .

Cabanga ngakho njengokuthi uma ukungcola okuncane kubhajwa epayipini lamanzi, ukugeleza kwamanzi kuyehla. Kodwa okubhajwa lapha yilawo maseli abomvu egazi amise okwesikela. Lawa awagobi kalula njengamaseli abomvu egazi ajwayelekile ayindilinga, futhi awasheleli ezikhaleni ezincane. Yingakho ezama ukubhajwa.

Ngezinye izikhathi lesi simo singaphathwa ekhaya, kodwa uma izimpawu zinzima, kungadingeka uhlale esibhedlela izinsuku ezimbalwa ukuze welashwe.

Yiziphi izinhlobo zenkinga ye-sickle cell?

Kunezinhlobo ezine eziyinhloko zenkinga ye-sickle cell. Ake sibheke ukuthi ziyini:

• Inkinga yokuvaleka kwemithambo yegazi: Lolu uhlobo oluvame kakhulu. Okwenzekayo lapha ukuthi amaseli esikela abhajwe kuma-capillary akho noma kweminye imithambo yegazi emincane, okunciphisa ukugeleza kwegazi ezicutshini nasezithweni zakho zomzimba. Yilokhu okubangela ubuhlungu obukhulu. Ezinye izinhlobo zithinta izindawo ezithile zomzimba.

• Inkinga ye-Aplastic: Lokhu kwenzeka lapho umongo wakho wethambo (indawo engaphakathi kwamathambo akho eyenza amangqamuzana egazi) ngokuzumayo uyeka ukukhiqiza amangqamuzana egazi abomvu. Lokhu kuvame ukubangelwa izifo ezithile zamagciwane, ikakhulukazi i-parvovirus B19.

• Inkinga ye-Hyperhemolytic: Lokhu kwenzeka lapho izinga le-hemoglobin yakho (iphrotheni esemaselini abomvu egazi ethwala umoya-mpilo) lehla ngokuzumayo. Lokhu kungenxa yokuthi amaseli akho abomvu egazi aphuka ngokushesha kakhulu. Lokhu kungenzeka futhi ngemva kokumpontshelwa igazi, okuyindlela evamile yokwelapha isifo se-sickle cell.

• Ukususwa kwe-splenic: Lokhu kwenzeka lapho amaseli esikela ebhajwa e-spleen yakho (isitho esitholakala ohlangothini olungaphezulu kwesobunxele lwesisu sakho), okubangela ukuthi i-spleen ikhule ngokuzumayo futhi ivuvuke kabuhlungu . Lokhu kungaba yingozi kakhulu ezinganeni ezincane, njengoba inani elikhulu legazi lingaqongelela e-spleen, okubangela ukwehla kwegazi.

Ziyini izimpawu zalokhu?

Izimpawu zesifo se-sickle cell zingahluka kuye ngokuthi sithinteke kuphi emzimbeni. Izimpawu ezivamile zifaka:

• Isiyezi.
• Ukukhathala okukhulu, ukukhathala.
• Ikhanda elibuhlungu.
• I-jaundice (ukuphuzi kwesikhumba namehlo).
• Ubuhlungu (lesi yisibonakaliso esiyinhloko nesinzima kakhulu).
• Ubunzima bokuphefumula.
• Ubuthakathaka.

Ubuhlungu besifo se-sickle cell bungaba buthuntu, bubukhali, buhlabe, noma buhlabe . Bungahlala amahora, izinsuku, noma ngisho namasonto. Ubuhlungu bungenzeka noma kuphi emzimbeni, endaweni eyodwa noma ezindaweni eziningana. Isibonelo:

• Ezandleni noma entendeni yesandla.
• Esifubeni.
• Emilenzeni noma ezinyaweni.
• Emhlane ongezansi.
• Esiswini.

Kungani kwenzeka le nkinga yamaseli esikele?

Isifo se-sickle cell senzeka lapho amangqamuzana abomvu egazi amise okwesikela ehlangana ndawonye futhi evimba imithambo yegazi. Kodwa-ke, ochwepheshe abazi njalo ukuthi yini ebangela lokhu.

Kodwa-ke, kunezinye izimbangela ezingaba nomthelela kulesi simo:

• Ukuphelelwa amanzi emzimbeni : Uma umzimba ungenawo amanzi, igazi liyaqina futhi amaseli avame ukuvaleka.
• Ukuya ezindaweni eziphakeme (ngoba kunomoya-mpilo omncane ezindaweni eziphakeme).
• Ukutheleleka : Noma yikuphi ukutheleleka, njengomkhuhlane noma umkhuhlane.
• Ukukhulelwa.
• Ukubhema noma ukuphuza i-vaping.
• Umsebenzi womzimba onzima kakhulu.
• Ukucindezeleka : Ukukhathazeka ngokweqile nokwesaba.
• Ukushintsha kwezinga lokushisa okukhulu ngokuzumayo (isib., ukugeza ngamanzi abandayo ngemva kokuba sendaweni eshisayo kakhulu).
• Ukuntuleka kwamavithamini athile, isibonelo i-folate.

Yiziphi izinkinga ezingabangelwa yilokhu?

Ubunzima benkinga yamaseli esikele buxhomeke ekutheni umthambo wegazi uvalekile kuphi. Uma imithambo yegazi ivalekile ezicutshini noma ezithweni zakho zomzimba, izinkinga ezifana nalezi:

• I-Acute chest syndrome : Lesi yisimo esibi nesisongela ukuphila esenzeka esifubeni. Izimpawu zifaka phakathi umkhuhlane, ubuhlungu besifuba, kanye nobunzima bokuphefumula.
• Amahlule egazi.
• Ubuhlungu obungapheli (ubuhlungu obuqhubekayo).
• Ukwanda kwe-spleen nezinye izifo ezihlobene ne-spleen.
• Amanxeba angapholi emilenzeni.
• Isifo sesibindi.
• Izifo zamaphaphu, isibonelo, umfutho wegazi ophezulu emaphashini.
• I-Necrosis : Ukufa kwethambo noma izicubu (ikakhulukazi ezindaweni ezifana ne-hip) ngenxa yokulahlekelwa ukugeleza kwegazi.
• Ukuma okubuhlungu kwepenisi (okubizwa nangokuthi 'i-priapism' kwabesilisa).
• Isifo sohlangothi : Sibangelwa ukuvaleka noma ukuqhekeka komthambo wegazi ohambisa igazi ebuchosheni.

Ukubona kanjani lokhu?

Uma unesifo se-sickle cell, cishe usuvele ubona udokotela wakho njalo ezinyangeni ezi-6 noma kanye ngonyaka ukuze uhlolwe igazi. Kodwa uma unenkinga ye-sickle cell, kuzodingeka ube nesethi ephelele yezivivinyo zelebhu. Lokhu kungafaka:

• Ukuhlolwa kohlobo lwegazi (uma udinga ukumpontshelwa igazi).
• I-CBC (Inani Eliphelele Legazi) : Lokhu kulinganisa amazinga e-hemoglobin kanye nenani lamaseli egazi abomvu namhlophe.
• I-CMP (Iphaneli Ephelele Ye-Metabolic) : Isethi ephelele yezivivinyo ze-metabolic ezibheka ukusebenza kwezitho ezifana nezinso nesibindi.
• Ukuhlolwa kokusebenza kwesibindi .
• Inani lama-reticulocyte : Lokhu kulinganisa inani lamaseli abomvu egazi asanda kwakheka. Lokhu kunganikeza umbono wokuthi umnkantsha usebenza kahle kangakanani.

Ngaphezu kwalokho, udokotela wakho angenza ukuhlolwa okuphelele ngokomzimba kanye nokuhlolwa kwezithombe okufana nalokhu:

• I-Doppler ultrasound : Bona ukuthi igazi ligeleza kanjani emithanjeni yegazi.
• I-MRI : Ikakhulukazi uma kukhona okusolwayo ngesimo esifana nesifo sohlangothi.
• I-Ultrasound : Ukubheka izinto ezifana nezitho zesisu kanye ne-spleen.
• I-X-ray yesifuba : Ukuhlola isimo esifana nesifo esibuhlungu sesifuba.

Iphathwa kanjani?

Uma unenkinga ye-sickle cell, ungadinga ama-painkiller ukusiza ukulawula izimpawu ezingakhululekile. Ezimweni ezincane, ungakuphatha lokhu ekhaya ngemithi etholakala ngaphandle kwemithi kadokotela njenge-acetaminophen (isib. i-Panadol®, i-Tylenol®) noma i-ibuprofen (isib. i-Advil®, i-Brufen®).

Kodwa-ke, uma ubuhlungu obukhulu bukhona, kungadingeka uhlale esibhedlela izinsuku ezimbalwa. Odokotela bangakunikeza imithi enamandla yokudambisa izinhlungu (njenge-morphine), uketshezi lwe-IV (ukugcina igazi lakho ligeleza kahle), kanye nomoya-mpilo uma kudingeka.

Ngaphezu kwalokho, ukwelashwa kwesifo se-sickle cell kuyahlukahluka kuye ngezingxenye zomzimba ezithintekile kanye nobukhali bezimpawu. Odokotela bangase basebenzise imithi, ukumpontshelwa igazi, ukufakelwa umnkantsha, noma inhlanganisela yalezi zindlela zokwelapha.

Imithi yokuphathwa kwesikhathi eside

Kunezinhlobo eziningana zemithi odokotela abayisebenzisayo ukuphatha izinkinga ze-sickle cell isikhathi eside nokunciphisa ukuvama kwazo:

• I-Hydroxyurea: Lo muthi wenza amangqamuzana akho abomvu egazi abe makhulu, wandisa ikhono lawo lokugoqa, futhi wandisa uhlobo lwe-hemoglobin olubizwa ngokuthi i-fetal hemoglobin. Lokhu kunciphisa ukujiya kwamangqamuzana.
• I-Crizanlizumab `(Crizanlizumab)`:Lolu uhlobo lwe-antibody ye-monoclonal. Lungavimbela amaseli akho abomvu egazi ukuthi anamathele ezindongeni zemithambo yegazi yakho.
• I-L-glutamine: Lena yi-amino acid esiza ekunciphiseni umonakalo kumaseli abomvu egazi.
• I-Voxeletor: Lo muthi uvimbela amangqamuzana akho abomvu egazi ukuthi angabanjwa futhi anamathele ndawonye.

Ukumpontshelwa igazi

Odokotela bangase basebenzise ukumpontshelwa igazi ukwelapha izinkinga ze-sickle cell ezingasebenzi kahle noma ezibangelwa yi-hyperhemolytic. Lokhu kwelashwa kungagcwalisa amangqamuzana akho abomvu egazi aphilile futhi kunciphise ingozi yakho yezinkinga ze-sickle cell. Abanye abantu kungadingeka ukuthi bampontshelwe igazi kaningi.

Ukufakelwa izitshalo

Ukufakelwa umnkantsha wethambo noma ukufakelwa i-stem cell kungaba yindlela kuphela yokwelapha isifo se-sickle cell kwezinye izimo. Kodwa-ke, ukuthola umuntu onikelayo ofanayo (njengomfowabo noma udadewabo) kungathatha isikhathi. Futhi, ukufakelwa umnkantsha wethambo kungabangela ezinye izinkinga ezinkulu, njengesifo se-graft vs. host (GvHD). Ngenxa yalesi sizathu, odokotela abavamile ukwenza ukufakelwa umnkantsha wethambo lapho belapha izinkinga ze-sickle cell. Kunalokho, umgomo oyinhloko ukwelapha izimpawu zakho nokunciphisa ubuhlungu bakho.

Ingabe lokhu kungavinjelwa?

Izinkinga zamaseli esikele azikwazi ukuvinjelwa ngaso sonke isikhathi. Kodwa-ke, kunezinto ezithile ezingasiza ekunciphiseni ingozi:

• Gwema ukubhema ngokuphelele.
• Uma usendaweni ebandayo, gqoka ngokufudumele. Umkhuhlane ungavimba imithambo yegazi futhi ubangele inkinga.
• Nciphisa noma uyeke ngokuphelele ukuphuza utshwala . Utshwala bungabangela ukugcinwa kwamanzi.
• Phuza okungenani izingilazi zamanzi ezingu-8-10 ngosuku (hlala unamanzi).
• Thatha amaphilisi e-folic acid nsuku zonke ukuze wandise ukukhiqizwa kwamangqamuzana abomvu egazi (lokhu kufanele kwenziwe ngokweseluleko sikadokotela).
• Geza izandla zakho ngensipho njalo ukuze unciphise ingozi yokutheleleka.
• Thola yonke imijovo edingekayo ngesikhathi (isib. umjovo womkhuhlane, umjovo we-pneumonia).
• Gwema ukuzivocavoca okunzima ngokweqile, kodwa ukuzivocavoca okulingene kuhle.
• Thola izindlela zokunciphisa ukucindezeleka (isib. i-yoga, ukuzindla).

Ngizinakekela kanjani (uma kuvela inkinga)?

Uma wena noma othile oseduze nawe enenkinga ye-sickle cell, ukwenza lezi zinto kungasiza ekunciphiseni izimpawu:

• Hlala ufudumele. Ukugeza ngamanzi ashisayo noma ukubeka i-heating pad endaweni ebuhlungu kuzokhulisa imithambo yegazi yakho futhi kuthuthukise ukuhamba kwegazi. Ukushisa kuzosiza futhi ekuthokoziseni imisipha ebuhlungu.(Ungafaki amaphakethe eqhwa. Umkhuhlane ungabangela inkinga yobuhlungu. Ngakho-ke kungcono ukuhlala ufudumele ngangokunokwenzeka.)
• Phuza amanzi amaningi. Uma uphuza amanzi anele, amangqamuzana akho abomvu egazi anamathelana kancane, okwenza akwazi ukuhamba kalula emigudwini.
• Thola ukubhucungwa. Ukubhucungwa okuthambile kungandisa ukugeleza kwegazi, kuphumuze imisipha, futhi kunciphise ubuhlungu.
• Zijwayeze ukuqaphela. Ucwaningo lukhombisile ukuthi ukugxila ingqondo yakho emgomweni owodwa isikhathi esifushane kungasiza ekuphazamiseni ubuchopho bakho futhi kunciphise ubuhlungu obuhambisana nesifo se-sickle cell. Ngisho nento elula njengokugxila ekuphefumuleni kwakho ingasiza.
• Phuza imithi yokubulala izinhlungu ethengiswa ngaphandle kwemvume kadokotela. Lokhu kufaka phakathi i-paracetamol, i-ibuprofen, noma i-naproxen (isib. i-Aleve®). Kodwa ungathathi okungaphezu kwalokho okunqunywe udokotela wakho.

Kufanele uye nini e-Emergency Treatment Unit (ETU) ?

Uma unezinye zalezi zimpawu, hamba ngokushesha eMnyangweni Wezimo Eziphuthumayo (ETU) :

* Uma unobuhlungu obungazelelwe, obukhulu obungenakulawuleka ngemithi yasekhaya.

* Ubunzima bokuphefumula noma ubuhlungu besifuba.

* Umkhuhlane ophezulu (ngaphezu kuka-101°F noma u-38.3°C).

* Ubuthakathaka obungazelelwe, ubunzima bokukhuluma, ukugoba kolunye uhlangothi lobuso (izimpawu zesifo sohlangothi).

* Ubuhlungu obukhulu besisu noma ukuqunjelwa.

* Uma i-jaundice ikhula.

* Kwabesilisa, ukuvuka kowesifazane okubuhlungu kuthatha amahora angaphezu kwamane.

Ungalibali. Ukuqala ukwelashwa kusenesikhathi kunganciphisa ingozi yezinkinga zesikhathi eside.

Izinto ezibaluleke kakhulu okudingeka uzikhumbule

Uma unesifo se-sickle cell, umcabango wenkinga ungakuphazamisa kancane. Lezi zimo zingabangela izimpawu ezingathandeki kanye nezinkinga zokuphila kwakho konke. Uma uke waba nenkinga ye-sickle cell, uyazi ukuthi isikhathi siyigugu kangakanani.

Uma izimpawu zakho zingathuthuki ngemithi yokwelapha izinhlungu etholakala ngaphandle kwemithi kadokotela noma imithi yakho evamile yasekhaya, sekuyisikhathi sokubona udokotela wakho. Balapha ukuzokusiza. Ungesabi ukukhuluma nodokotela wakho ngesimo sakho. Ukwelashwa kusenesikhathi kungcono kakhulu. Sebenzisana nodokotela wakho ukuze wakhe uhlelo lokulawula izinhlungu olukusebenzelayo.

Inkinga Yesikele , Isifo Sesikele, Amaseli Egazi Abomvu, Ubuhlungu, Imithambo Yegazi, I-Oxygen