Ingabe kukhona inkinga ngegazi lakho? Ake sifunde nge-hemoglobinopathy ngamagama alula!

Ingabe uhlala uzizwa ukhathele futhi ukhathele? Mhlawumbe isikhumba sakho simhlophe kancane? Noma ingabe ukhona emndenini wakho oke waba nesifo segazi? Khona-ke engizokutshela kona kubaluleke kakhulu kuwe. Namuhla sizokhuluma ngesifo segazi esibizwa ngokuthi i-Hemoglobinopathy, esinegama eliyinkimbinkimbi, kodwa elibonakala kubantu abaningi. Ungakhathazeki, sizokhuluma ngalokhu kalula.

Kuyini i-Hemoglobinopathy? Ake sithole ukuthi kuyini ngempela!

Kalula nje, i-hemoglobinopathy iyiqembu lezifo ezizuzwe njengefa ezithinta iphrotheni egazini lethu ebizwa ngokuthi i-hemoglobin. Manje cishe uyazibuza ukuthi iyini le-hemoglobin, akunjalo?

Cabanga ngale ndlela: i-hemoglobin ifana neqhawe elincane ngaphakathi kwamangqamuzana ethu abomvu egazi. Yiyo ethatha umoya-mpilo emaphashini ethu lapho siphefumula bese iwusabalalisa emizimbeni yethu yonke. Isebenza njengetekisi ye-oxygen. Ngakho-ke uma le hemoglobin ingakhiqizwanga kahle, noma uma ingakhiqizwanga ngokwanele, imizimba yethu ayitholi umoya-mpilo owanele. Yilapho izinkinga ziqala khona.

Lesi simo sizuzwe njengefa, okusho ukuthi sibangelwa ushintsho ezakhini zofuzo esizizuza kubazali bethu. Izigidi zabantu emhlabeni wonke zihlushwa yilesi simo. Eqinisweni, uhlobo oluvame kakhulu lwesifo segazi esizuzwe njengefa. Abacwaningi bathole izinhlobo ezingaphezu kuka-600 ze-hemoglobinopathy!

Uma imizimba yethu ikhiqiza i-hemoglobin engajwayelekile, noma inganele, izimpawu ezifana nobuhlungu bomzimba, ukukhathala, kanye nomonakalo ezithweni ezahlukahlukene zomzimba zingavela. Yingakho kubaluleke kakhulu ukukubona kusenesikhathi. Yingakho odokotela behlola izingane ukuthi zine-hemoglobinopathy ngokushesha nje lapho zizalwa.

Into ebalulekile ukuthi i-Hemoglobinopathy ayisona isifo esingelapheki ngokuphelele. Kodwa-ke, ngokwelashwa okufanele, singalawula izimpawu futhi sivimbele izinkinga.

Yiziphi izinhlobo ze-hemoglobinopathy ezikhona?

Njengoba sishilo ngaphambili, kunezinhlobo eziningi zalokhu. Lezi zinhlobo zivame ukuqanjwa ngamagama esiNgisi. Lezi zinhlamvu zibhekisela ezinhlotsheni ezahlukene zeprotheyini ye-hemoglobin kanye nokulandelana abacwaningi abazithole ngakho. Lokhu kusiza odokotela baqonde ukuhlukahluka okuqondile kwezakhi zofuzo okuhilelekile.

Nazi ezinye zezinhlobo ezivame kakhulu ze-hemoglobinopathy:

• Isifo se-Hemoglobin C: Yilapho i-hemoglobin evamile ithathelwa indawo uhlobo olubizwa ngokuthi i-hemoglobin C.
• Isifo se-Hemoglobin E: Lapha, i-hemoglobin evamile ithathelwa indawo yi-hemoglobin E.
• Isifo se-Hemoglobin D: Lapha, i-hemoglobin evamile ithathelwa indawo yi-hemoglobin D.
• Isifo se-Hemoglobin SC:Lesi simo senzeka uma uthola ifa le-sickle cell gene elilodwa kanye ne-hemoglobin C gene elilodwa.
• Isifo se-Hemoglobin SD: Lapha, i-gene eyodwa ye-sickle cell kanye ne-gene eyodwa ye-hemoglobin D izuzwa njengefa.
• Isifo se-Hemoglobin SE: Lapha, i-gene eyodwa ye-sickle cell kanye ne-gene eyodwa ye-hemoglobin E izuzwe njengefa.
• Isifo se-Sickle cell: Kulesi simo, amangqamuzana akho abomvu egazi aba nesimo se-sickle. Lokhu kuwavimbela ukuthi ahambe kalula emithanjeni yegazi futhi kungabangela ukuthi avaleke.
• I-Thalassemia: Kulesi simo, umzimba wakho awukhiqizi i-hemoglobin eyanele.

Lokhu kungase kuzwakale kuyinkimbinkimbi kancane, kodwa udokotela angakuchazela kabanzi.

Ziyini izimpawu zalokhu? Bheka ukuthi nawe unazo lezi...

Izimpawu ze-hemoglobinopathy zingahluka kuye ngomuntu, kodwa kunezinye izimpawu ezivamile:

• Izandla nezinyawo ezibandayo
• Umchamo omnyama (ukuba mnyama)
• Ukukhathala okuvamile
• Ukukhula okulibazisekile ezinganeni
• Ukuphuzi kwesikhumba namehlo (i-Jaundice)
• Isikhumba esikhanyayo
• Ubunzima bokuphefumula, ukukhwehlela
• Ukulala kakhulu kunokujwayelekile
• Ukuvuvukala kwamalungu

Izinsana ezine-hemoglobinopathy enzima zingase zibonise izimpawu lapho zizalwa. Ngezinye izikhathi, izimpawu zivela ebuntwaneni. Kwabantu abadala, lezi zimpawu zingase zibe zimbi kakhulu ngezikhathi zokubhebhetheka kwesifo (njengenkinga ye-sickle cell).

Kungani lokhu kwenzeka ngenxa ye-hemoglobinopathy?

Isizathu esiyinhloko salokhu ukuthi kukhona ukuhlukahluka kwezakhi zofuzo okuthinta i-hemoglobin yakho. Ungakuthola lokhu kuhlukahluka kwezakhi zofuzo kumama wakho, kuyihlo, noma kokubili. Lokhu kusho ukuthi lokhu akuyona into esingayilawula, kuyinto evela esizalweni.

Ubani onamathuba amaningi okuthuthukisa lokhu? (Izici eziyingozi)

Abanye abantu banamathuba amaningi okuthola le hemoglobinopathy. Ake sibheke ukuthi bangobani:

• Uma uzalo lwakho luvela e-Afrika, eMedithera, eNingizimu-mpumalanga ye-Asia, noma eNtshonalanga ye-Asia. (Lezi zimo zingabonakala naseSri Lanka)
• Uma umama wakho, ubaba, noma umfowenu ene-hemoglobinopathy.
• Uma uhlala endaweni lapho ukuhlolwa kwe-hemoglobinopathy yosana kungenziwa njalo.

Uma une-hemoglobinopathy, kungenzeka ukuthi nengane yakho izoba nalesi sifo. Ngakho-ke, kubaluleke kakhulu ukufuna iseluleko sezokwelapha ngaphambi kokuqala umndeni.

Yiziphi izinkinga ezingabangelwa yilokhu?

Uma i-hemoglobinopathy ingalawulwa kahle, kungavela izinkinga ezahlukahlukene. Ezinye zazo yilezi:

• I-anemia: Ezinye izinhlobo ze-hemoglobinopathy zingabangela i-anemia, okungaholela ekunciphiseni kwamangqamuzana abomvu egazi. Lokhu kungaholela ekukhathaleni okukhulu kanye nobuthakathaka.
• Ukutheleleka okuvamile: I-Hemoglobinopathy ingaholela ekutheni amasosha omzimba abe sengozini , okwandisa ingozi yokugula nokutheleleka.
• Ukulimala kwezitho zomzimba: I-Hemoglobinopathy ibangela ukwehla kwamazinga omoya-mpilo egazini. Uma igazi elinomoya-mpilo lingalethwanga kahle isikhathi esithile, izicubu zethu nezitho zomzimba zingalimala.
• Iziqephu zobuhlungu: Kwezinye izinhlobo ze-hemoglobinopathy, njengesifo se-sickle cell, amahlule egazi angakheka ngaphakathi kwemithambo yegazi futhi ayivimbe. Lokhu kungaholela ebuhlungwini obukhulu kanye nokwehla kokugeleza kwegazi.

Uyithola kanjani ngokunembile lesi sifo? (Ukuxilongwa)

Odokotela benza izivivinyo eziningana ukuze bathole ukuthi une-hemoglobinopathy yini.

• Ukubalwa kwegazi okuphelele (i-CBC): Lokhu kuvame ukuba ukuhlolwa kwegazi kokuqala okwenziwayo. Kungakutshela ngezinhlobo zamaseli egazini lakho, okungukuthi, ukuthi mangaki ohlotsheni ngalunye lweseli onalo.
• Ukuhlolwa kwezakhi zofuzo: Lokhu kuhlolwa kungakhomba ngqo ukuthi yiziphi izinguquko zezakhi zofuzo ezihilelekile.
• I-Hemoglobin electrophoresis: Lokhu kuhlolwa kuhlukanisa ama-molecule e-hemoglobin nesampula yegazi lakho futhi kubheka ukukhubazeka.
• Izifundo ze-iron: Lokhu kuhlolwa kungenziwa ukuze kutholakale ukuthi une-anemia yokuntuleka kwe-iron.
• Ukuhlolwa kwezinsana: Emazweni amaningi afana neCanada neMelika, izinsana ezisanda kuzalwa zihlolwa i-hemoglobinopathy. Ezinye izibhedlela eSri Lanka nazo zinalesi sikhungo.
• Ukuhlolwa Kwangaphambi Kokubeletha: Lokhu kuhlolwa kungasetshenziswa ukuthola ukuthi ingane esanda kuzalwa ine-hemoglobinopathy ngesikhathi sokukhulelwa.

Yiziphi izindlela zokwelapha?

Kunezindlela eziningana zokwelapha i-hemoglobinopathy, kodwa lezi zindlela zokwelapha ziyahlukahluka kuye ngohlobo kanye nobunzima besifo.

• Ukumpontshelwa igazi: Ezinye izimo, njenge-thalassemia nesifo se-sickle cell, zithinta inani le-hemoglobin egazini. Ukumpontshelwa igazi kungadingeka ukuze kugcinwe amazinga e-hemoglobin ajwayelekile.
• Amaphilisi e-folic acid: Lawa angasiza ekwandiseni ukukhiqizwa kwamangqamuzana abomvu egazi.
• Ukwelashwa nge-Gene:Lokhu kuyindlela entsha yokwelapha. Ososayensi bathatha amangqamuzana athintekile emzimbeni wakho, bawalungise besebenzisa amasu akhethekile, bese bewabuyisela emzimbeni wakho. Kufana nokubhala kabusha imiyalelo ezizakhini zakho zofuzo.
• Ukwelashwa nge-iron chelation: Uma une-iron eningi emzimbeni wakho, lokhu kwelashwa kungasusa i-iron eningi. Lo muthi unikezwa ngomlomo noma njengomjovo.
• Ukwelashwa nge-oxygen: Udokotela wakho angase akuncome ukwelashwa nge-oxygen kanye nezinye izindlela zokwelapha. Umgomo walokhu ukwandisa amazinga e-oxygen egazini lakho nokunciphisa ubuhlungu.
• Ukufakelwa kwamaseli e-stem: Lokhu kuhilela ukufaka amaseli abomvu egazi angajwayelekile endaweni yamaseli abomvu egazi anempilo. Kodwa-ke, lokhu akuvamile kakhulu, njengoba kunzima ukuthola umnikeli ofanele. Kuphinde kube nengozi yesifo se-graft-versus-host (GvHD) .

Khumbula, kuye ngohlobo lwe-hemoglobinopathy onayo, ungase ungadingi ukwelashwa nhlobo. Ngakho-ke kungcono ukukhuluma nodokotela wakho ubuze ukuthi yini engcono kakhulu kuwe.

Ngizozizwa ngingcono ngokushesha kangakanani ngemva kokwelashwa?

Kuya ngohlobo lwe-hemoglobinopathy onayo kanye nobunzima bayo. Iningi labantu liqala ukuzizwa lingcono phakathi namasonto noma izinyanga ezimbalwa zokuqala ukwelashwa.

I-Hemoglobinopathy iyisimo esihlala isikhathi eside, ngakho-ke uzodinga ukuhlolwa njalo kwezokwelapha kanye nokuqapha. Udokotela wakho uzokusiza ukuphatha izimpawu zakho.

Liyini ikusasa ngalabo abaphila nalesi sifo?

Ngokwelashwa okufanele, ikusasa lakho lingaba lihle. Abantu abangaphezu kuka-90% abane-hemoglobinopathy basinda baze babe badala.

Kodwa-ke, uma kungelashwa, izinhlobo eziningi ze-hemoglobinopathy zingaholela ekufeni eminyakeni embalwa yokuqala yokuphila. Ngakho-ke, ukuxilongwa nokwelashwa kusenesikhathi kuyindlela engcono kakhulu yokuvimbela izinkinga ezinkulu.

Lokhu akunakuvinjelwa?

Ngeshwa, cha. Njengoba i-hemoglobinopathy iyinto oyizuza njengefa, ayikho indlela yokuyivimbela. Kodwa-ke, singakwazi ukulawula izimpawu ngokwelashwa okufanele.

Ngizinakekela kanjani?

Ukuphila ne-hemoglobinopathy kungaba yinselele, kodwa uma uzinakekela kahle, unganciphisa ingozi yobuhlungu kanye neminye imiphumela emibi.

Nazi ezinye izeluleko ezijwayelekile ezingakusiza:

• Phuza amanzi amaningi. Zama ukuphuza okungenani amalitha angu-2-3 amanzi ngosuku.
• Yiba matasa ngokomzimba, kodwa ungaweqisi. Lalela umzimba wakho. Uma uzizwa ukhathele, phumula.
• Yidla ukudla okucebile nge-iron, amavithamini, kanye namaminerali. Faka okuluhlaza, izithelo, imifino, inyama, kanye nenhlanzi ekudleni kwakho.
• Lawula ukucindezeleka kwakho. Sebenzisa ukuzindla, i-yoga, noma ezinye izindlela zokucabanga ozijabulelayo.
• Geza izandla zakho njalo. Lokhu kusiza ekuvikeleni ezifweni.

Kufanele ngimbone nini udokotela?

Uma unezimpawu ezifana nokukhathala okukhulu, ubunzima bokuphefumula, noma isikhumba esimpunga, qiniseka ukuthi ubona udokotela bese umtshela ngakho.

Uma usuvele utholakale une-hemoglobinopathy, bona udokotela wakho ngokushesha uma izimpawu zakho zibuya noma ziba zimbi kakhulu. Angalungisa ukwelashwa kwakho.

Yimiphi imibuzo okufanele uyibuze udokotela?

Uma wena noma ingane yakho ninesifo se-hemoglobinopathy, kuwumqondo omuhle ukubuza udokotela wakho le mibuzo:

• Uhlobo luni lwe-hemoglobinopathy enginalo mina/ingane yami?
• Hlobo luni lokwelashwa olunconywayo?
• Ingabe kuzodingeka ngibone abanye ochwepheshe ukuze ngithole ukwelashwa?
• Ingabe ngidinga ukushintsha indlela engidla ngayo noma imikhuba yami yokuzivocavoca?
• Angakanani amathuba okuba izingane zami zibe nalesi simo?
• Ingabe unalo olunye ulwazi ngalokhu? (isib. izincwadi, amawebhusayithi)

Kunini lapho kudingeka uye e-Emergency Treatment Unit (ETU) ?

Uma unezinye zezimpawu ezilandelayo, shayela ku-911 (1990 eSri Lanka) noma uye ekamelweni lezimo eziphuthumayo eliseduze ngokushesha:

• Ubuhlungu besifuba
• Isiyezi
• Umkhuhlane ophakeme ngaphezu kwama-degrees Fahrenheit angu-103 (ama-degrees Celsius angu-39.4)
• Ubuhlungu obukhulu obungapheli naphezu kokuthatha imithi
• Izimpawu zesifo sohlangothi, isibonelo, ukulahlekelwa ingqondo ngokuzumayo, ubunzima bokukhuluma.

I-hemoglobinopathy iyithinta kanjani i-A1C yami?

I-Hemoglobinopathy ingabangela imiphumela yakho yokuhlolwa kwe -A1C ukuthi inganembile. Kuye ngokuthi i-genetic variant yakho injani, ingabonisa inani eliphansi noma eliphakeme kunalelo onalo ngempela.

Uma une-hemoglobinopathy, udokotela wakho angase akuncome ukuhlolwa okungasiza ekunciphiseni le nkinga. Le A1C ibaluleke kakhulu kubantu abanesifo sikashukela, ngakho ungakhohlwa ukukhuluma nodokotela wakho ngalokhu.

Okokugcina, izinto ezibaluleke kakhulu okufanele uzikhumbule!

Kuvamile ukuzizwa wesaba futhi ukhathazekile lapho uthola ukuthi wena noma ingane yakho inesifo segazi esihlala isikhathi eside. Imibuzo efana nokuthi "I-hemoglobinopathy izoyithinta kanjani impilo yami?", "Ingabe ngizoba nobuhlungu obungapheli?", "Lesi sifo sizoyithinta kanjani ingane yami?" ingase ivele engqondweni yakho.

Kodwa izindaba ezinhle ukuthi ngokwelashwa okufanele nokulawula izimpawu, ungaphila impilo enempilo ngangokunokwenzeka.Into engcono kakhulu ongayenza ukukhuluma nodokotela wakho. Ungalibali. Ukuxilongwa kusenesikhathi kanye nokwelashwa kuyisihluthulelo sempilo ende nenempilo.

Ungakhathazeki, awuwedwa. Odokotela, abahlengikazi, nomndeni wakho bazokusiza kulolu hambo. Yiba nesibindi!

I- Hemoglobinopathy, izifo zegazi, izifo ezizuzwe njengefa, i-hemoglobin, i-anemia, isifo se-sickle cell, i-thalassemia