Thalassemia: kuyini, izimpawu kanye nezinketho zokwelashwa

-Delile

• Izimpawu eziyinhloko
• Izinhlobo zeThalassemia
• 1. I-Alpha Thalassemia
• 2. Thalassemia Beta
• Ungakuqinisekisa kanjani ukuxilongwa
• Yelashwa kanjani
• 1. IThalassemia encane
• 2. I-thalassemia ephakathi
• 3. I-Thalassemia enkulu
• Izinkinga ezingenzeka

I-Thalassemia, eyaziwa nangokuthi yi-Mediterranean anemia, yisifo esizuzwe njengefa esivezwa ukukhubazeka ekukhiqizeni i-hemoglobin, okuyiyona ebhekele ukuhambisa i-oxygen ezicutshini.

Ukubonakaliswa komtholampilo kwe-thalassemia kuncike enanini lamaketanga athintekile ku-hemoglobin kanye nohlobo lokuguquka kwezakhi zofuzo olwenzekile, okungaholela ekukhathaleni, ukubambezeleka kokukhula, i-pallor ne-splenomegaly, ngokwesibonelo.

I-Thalassemia yisifo sofuzo nesofuzo, esingathathelani noma esibangelwe ukuswela kokudla, kepha-ke, uma kunezinhlobo ezithile ze-thalassemia, ukwelashwa kungafaka ukudla okufanele. Bona ukuthi ukudla kwe-thalassemia kwenziwa kanjani.

Izimpawu eziyinhloko

Ngokuvamile, uhlobo oluncane lwe-thalassemia, okuyisimo esisesincane kakhulu sesifo, lubangela kuphela i-anemia ne-pallor emnene, okungajwayele ukubonwa yisiguli. Kodwa-ke, ifomu elikhulu, okuluhlobo oluqine kakhulu lwesifo, lingadala:

• Ukukhathala;
• Ukuthukuthela;
• Amasosha omzimba abuthakathaka nokuba sengozini yokutheleleka;
• Ukubambezeleka kokukhula;
• Ukuphefumula okufushane noma okusebenza kanzima kalula;
• Pallor;
• Ukungabi nesifiso sokudla.

Ngaphezu kwalokho, ngokuhamba kwesikhathi lesi sifo singadala nezinkinga ebuchosheni, esibindini, enhliziyweni nasemathanjeni, ngaphezu kwe-jaundice, okuwumbala ophuzi wesikhumba namehlo.

Izinhlobo zeThalassemia

I-Thalassemia ihlukaniswe ngama-alpha ne-beta ngokochungechunge lweglobin oluthintekile. Endabeni ye-alpha thalassemia, kuncipha noma ukungabikho kokukhiqizwa kwamaketanga e-alpha hemoglobin, kuyilapho ku-beta thalassemia kuncipha noma ukungabikho kokukhiqizwa kwamaketanga e-beta.

1. I-Alpha Thalassemia

Kubangelwa ushintsho ku-alpha-globin molecule yama-hemoglobin egazi, futhi ingahlukaniswa:

• Isici se-Alpha thalassemia: ibonakala nge-anemia emnene ngenxa yokwehla kweketanga elilodwa kuphela le-alpha-globin;
• Isifo seHemoglobin H: okuyinto ebonakala ngokungabikho kokukhiqizwa kwezakhi zofuzo ezi-3 kwezingu-4 ezihlobene nochungechunge lwe-alpha globin, kubhekwa njengenye yezindlela ezinzima zesifo;
• I-hemoglobin hemoglobin hydrops fetal syndrome: uhlobo olubi kakhulu lwe-thalassemia, ngoba lubonakala ngokungabikho kwazo zonke izakhi zofuzo ze-alpha, okuholela ekufeni kombungu ngisho nangesikhathi sokukhulelwa;

2. Thalassemia Beta

Kubangelwa ushintsho ku-beta-globin molecule yama-hemoglobin egazi, futhi ingahlukaniswa:

• I-Thalassemia encane (encane) noma isici se-Beta-thalassemia: okungenye yezinhlobo ezimnene zesifo, lapho umuntu engazizwa khona izimpawu, ngakho-ke kutholakala kuphela ngemuva kokuhlolwa kwe-hematological. Kulokhu, akunconywa ukwenza ukwelashwa okuthile kuyo yonke impilo, kepha udokotela angancoma ukusetshenziswa kwe-folic acid supplement ukuze kuvinjelwe ama-anemias amancane;
• I-Beta-Thalassemia Intermediate: kubangela i-anemia emnene kuya kokubi kakhulu, futhi kungadingeka ukuthi isiguli sithole ukumpontshelwa igazi ngezikhathi ezithile;
• I-beta thalassemia enkulu noma enkulu: isithombe esibucayi kakhulu somtholampilo se-betas thalassemia, njengoba kungekho ukukhiqizwa kwamaketanga e-beta globin, okudinga ukuthi isiguli sithole ukumpontshelwa igazi njalo ukunciphisa izinga lokushoda kwegazi. Izimpawu ziqala ukuvela onyakeni wokuqala wokuphila, zibonakala nge-pallor, ukukhathala ngokweqile, ukozela, ukucasuka, amathambo obuso avelele, amazinyo angahambisani kahle nesisu esivuvukele ngenxa yezitho ezikhulisiwe.

Ezimweni ze-thalassemia enkulu, usengabona kuhamba kancane kunokukhula okujwayelekile, okwenza ingane ibe mfushane futhi ibe ncanyana kunalokho obekulindelwe ngokweminyaka yayo. Ngaphezu kwalokho, ezigulini ezithola ukumpontshelwa igazi njalo, ukusetshenziswa kwemithi evimbela i-iron eyeqile emzimbeni kuvame ukukhonjiswa.

Ungakuqinisekisa kanjani ukuxilongwa

Ukuxilongwa kwe-thalassemia kwenziwa ngokuhlolwa kwegazi, okufana nokubalwa kwegazi, ngaphezu kwe-hemoglobin electrophoresis, ehlose ukuhlola uhlobo lwe-hemoglobin ejikeleza egazini. Bona ukuthi uhumusha kanjani i-hemoglobin electrophoresis.

Ukuhlolwa kofuzo nakho kungenziwa ukuhlola izakhi zofuzo ezibhekele lesi sifo nokuhlukanisa izinhlobo zethalassemia.

Ukuhlolwa kwesithende akufanele kwenziwe ukuthola i-thalassemia, ngoba lapho kuzalwa i-hemoglobin ejikelezayo yehlukile futhi ayinazinguquko, kungenzeka ukuthi ihlolwe i-thalassemia kuphela ngemuva kwezinyanga eziyisithupha zokuphila.

Yelashwa kanjani

Ukwelashwa kwe-thalassemia kumele kuqondiswe udokotela futhi kuvame ukuhluka ngokobukhulu besifo:

1. IThalassemia encane

Lolu uhlobo olumnene kunalo lonke lwesifo futhi aludingi ukwelashwa okuthile. Ngokuvamile, umuntu akazitholi izimpawu, kodwa kufanele azi ngokuwohloka kwe-anemia ezimweni ezinjengokuhlinzwa, izifo ezinzima, izimo zokuxineka kakhulu noma ngesikhathi sokukhulelwa.

Ngokuvamile, udokotela wakho angancoma ukusetshenziswa kwe-folic acid supplements, ivithamini evuselela ukukhiqizwa kwamaseli egazi futhi isize ukuqeda i-anemia. Bona ukudla okunothe nge-folic acid nokuthi ukudla kungasiza kanjani ekwelapheni i-thalassemia.

2. I-thalassemia ephakathi

Ngokuvamile, ukwelashwa kwaleli fomu le-thalassemia kwenziwa ngokumpontshelwa igazi ngesikhathi sobuntwana, uma kwenzeka ingane inenkinga yokukhula, noma ezimeni lapho kukhona khona ubende nesibindi esandisiwe.

3. I-Thalassemia enkulu

Kuyindlela ebucayi kakhulu yesifo, lapho umuntu edinga ukumpontshelwa igazi impilo yakhe yonke, njalo emavikini amabili kuya kwamane, kuya ngezinga le-anemia. Uma kuqala ukwelashwa ngokushesha, ziyancipha izinkinga zesifo esizayo.

Abantu abane-thalassemia enkulu bangagcina benensimbi eyeqile emzimbeni ngenxa yokumpontshelwa igazi njalo, ngakho-ke udokotela angaphinde anikeze izidakamizwa ezihlafuna insimbi, ezibopha insimbi emzimbeni futhi zivimbele ukweqisa kwayo. Le mithi inganikezwa ngqo emthanjeni izikhathi ezi-5 kuye kwezi-7 ngeviki noma ngamaphilisi.

Izinkinga ezingenzeka

Izinkinga zeThalassemia zivela kuphela phakathi nezinhlobo ezinzima zesifo, ikakhulukazi lapho singelashwa kahle.

Ngendlela ephakathi yesifo, izinkinga zingaba:

• Ukukhubazeka kwamathambo namazinyo;
• Ukukhumuzeka kwamathambo;
• Gall amatshe;
• Izilonda zomlenze, ngenxa yokushoda komoya-mpilo emaphethelweni omzimba;
• Izinkinga zezinso;
• Ingozi ephezulu ye-thrombosis;
• Izinkinga zenhliziyo.

Ezimweni ezinzima, izinkinga ezinjengokukhubazeka kwamathambo namazinyo, ukukhuliswa kwesibindi kanye nobende nokuhluleka kwenhliziyo kungenzeka.