Nusiwo wòle be nànya tso Pompe Dɔléle dada ŋu

Enye nusi sɔ be vɔvɔ̃ naɖo wò eye wòatsi dzodzodzoe ne ɖɔkta gblɔ na wò be Pompe dɔléle le wò ƒomea me, vevietɔ ɖevi sue aɖe. Enye dɔléle si mebɔ o, si tso domenyiŋusẽfianu gbɔ, eyata ele dzɔdzɔme nu be woatsi dzimaɖi. Gake na magblɔ na wò be, mègatsi dzi o. Togbɔ be womekpɔ atike na dɔléle sia fifia o hã la, atikewɔmɔnu nyui geɖe li siwo ate ŋu akpe ɖe dɔnɔwo ŋu woanɔ agbe didi wu eye kuxi ʋɛ aɖewo manɔ wo ŋu o.

Pompe dɔlélea dada vevitɔe nye ERT (Enzyme Replacement Therapy).

Kpuie ko la, Pompe dɔlélea tsoa enzyme aɖe si le vevie na lãmeka siwo le míaƒe ŋutilã me ƒe anyimanɔmanɔ gbɔ. Ne enzyme sia bu la, sukli ƒomevi aɖe si woyɔna be glycogen ƒoa ƒu ɖe lãmenugbagbeviawo me, si gblẽa nu le lãmekawo ŋu. Bu eŋu abe dɔwɔla suewo le dɔ wɔm le míaƒe ŋutilã me ene, eye míeyɔa wo be enzymes. Le Pompe dɔlélea me la, dɔwɔla sia mewɔa dɔ nyuie o.

Eyata, Enzyme Replacement Therapy (ERT) ye nye afisi míenaa enzyme, alo dɔwɔla, si bu la tso ŋutilãa godo. Le esia ta la, wotsɔa atike si me enzyme si wowɔ si woyɔna be `alglucosidase alfa` le la naa ŋutilãa to ʋuka me (IV infusion). Atike sia koe nye atike si dzi woda asi ɖo fifia na Pompe dɔlélea.

Ne womewɔ ERT dɔ na vidzĩ siwo ŋu Pompe dɔlélea le o la, vivivi la, woƒe dzi ƒe lãmekawo nɔa kpekpem, woƒe ŋutilã ƒe lãmeka bubuwo gbɔdzɔna, eye wokuna le woƒe agbenɔƒe gbãtɔ me. Lãmetsi siwo hiã be woagbɔ ya hã meganɔa wo si o. Esi wodze ERT zazã gɔme vɔ la, nɔnɔme sia trɔ kura. Vidzĩ siwo wowɔ ERT-dɔdamɔnu na zi gbãtɔ la le woƒe ƒe 20-awo me fifia.

Nukatae wòle vevie ŋutɔ be woadze atikewɔwɔ gɔme kaba?

Esiae nye nu vevitɔ kekeake. ERT-dɔdamɔnu ate ŋu ana dɔlélea nagagblẽ o, gake mate ŋu atrɔ nusiwo gblẽ le lãmekawo ŋu xoxo o. Esia fia be ne atikewɔwɔa tsi megbe la, ate ŋu adzɔ be màgate ŋu agbɔ ya alo azɔ azɔli o.

Ðɔktawo dzea agbagba be yewoadze ERT zazã gɔme le woƒe agbenɔƒe ƒe ŋkeke ʋɛ gbãtɔwo me , vevietɔ na vidzĩwo, ne wokpɔ dɔlélea vɔ. Ne wohe ɖe megbe vie gɔ̃ hã la, ate ŋu axe mɔ na vidzĩa tegbee be wòagazɔ o.

Le amesiwo ŋu Pompe dɔléle si dzea egɔme megbe le gome la, ɖɔktaa alé ŋku ɖe dzesiawo ŋu edziedzi ahalé ŋku ɖe wo ŋu be yeanya ɣeyiɣi nyuitɔ si woadze ERT gɔme.

Esia menye ame ɖeka ƒe mɔzɔzɔ o - kpekpeɖeŋu tso eŋutinunyalawo ƒe ƒuƒoƒo aɖe gbɔ

Togbɔ be ERT-dɔdamɔnu ate ŋu aɖe ame ƒe agbe hã la, mesɔ gbɔ o. Be ame aɖe si ŋu Pompe dɔlélea nanɔ agbe nyuitɔ kekeake la, ehiã be woawɔ dɔ vovovowo ƒe ƒuƒoƒo si me dɔwɔla bibiwo le. Zi geɖe la, domenyiŋusẽfianu ŋuti nunyala si kpɔa dɔlélea gbɔe nyea ame vevitɔ si kpɔa atikewɔwɔ dzi.

Kpɔ kplɔ̃ si le ete hena eŋutinunyala bibi ƒomevi siwo ate ŋu ahiã na esia kple kpekpeɖeŋu si ƒomevi woana.

Atikewɔwɔ bubuwo tsɔ kpe ɖe ERT ŋu

Atike siwo gblẽa nu le dɔlélenutsiŋutete ŋu

Vidzĩ aɖewo ƒe ŋutilã dea dzesi enzyme therapy (ERT) sia si wozãna le gota be enye ‘dutatɔ’ eye wodzea dɔlékuiwutikewo wɔwɔ gɔme ɖe eŋu. Woyɔa esia be `CRIM-negative` nɔnɔme. Ne edzɔ alea la, ERT-dɔdamɔnua mawɔ dɔ nyuie o.

Be ɖɔktawo naxe mɔ ɖe esia nu la, wodzea dɔlélenutsiŋutete ƒe mɔɖeɖe ɖe nu ŋu ƒe dɔdamɔnu (ITI) zazã gɔme . Le esia me la, wonaa atike geɖe siwo kpɔa dɔlélenutsiŋutete dzi tsɔ kpe ɖe ERT zazã ŋu.

• Rituximab ƒe atike
• Methotrexate ƒe atike
• Dɔlékuiwutike si wodona ɖe ʋuka me (IVIG) .

Nusi atike siawo wɔna enye be "woblea" dɔlélenutsiŋutete be woasusu be ERT nye nane si nye ŋutilã tɔ. Ekema ŋutilãa mawɔ avu kple ERT o.

Lãmesẽfefewɔwɔ kple dɔwɔwɔ ƒe atikewɔwɔ

Esi wònye be lãmekawo ƒe gbɔdzɔgbɔdzɔ nye Pompe dɔlélea ƒe akpa vevi aɖe ta la, kamedede hiã vevie. Ate ŋu akpe ɖe lãmekawo ŋu wòado ŋusẽ eye ŋutilã nawɔ dɔ nyuie. Dɔwɔwɔ ƒe atikewɔwɔ ate ŋu akpe ɖe ame ŋu hã be woatu aɖaŋu siwo hiã be woawɔ gbesiagbedɔwo le wo ɖokui si la ɖo.

Nuƒoƒo kple nunyiame ƒe atikewɔwɔ

Esi wònye be lãmeka siwo hiã be woatsɔ aɖu nuɖuɖu ahami nu ate ŋu agbɔdzɔ ta la, nuƒoƒoŋutinunyala ate ŋu akpe ɖe ame ŋu le kamedede mimi me. Azɔ hã, ate ŋu ahiã ɣeaɖewoɣi be woazã mɔ̃ si wotsɔ naa nuɖuɖu vidzĩa be wòana nunyiame si hiã la vidzĩa. Nuɖuɖuŋutinunyala ate ŋu ana mɔfiame si hiã na esia.

Wò akpa hã le vevie ŋutɔ.

Ne èle agbe kple Pompe dɔlélea la, wò akpa si nàwɔ abe dɔnɔ alo dzila ene le vevie ŋutɔ.

Eyata ele be nàwɔ dɔ kple wò ɖɔkta be nàse wò nuhiahiãwo gɔme eye nàxɔ ŋgɔ le atikewɔwɔ nyuitɔ kekeake xɔxɔ me. Mègavɔ̃ gbeɖe be yeabia nya aɖeke wò ɖɔkta tso dɔléle sia alo atikewɔwɔ sia ŋu o.