Nukae Nye Cystic Fibrosis? Mina míase egɔme bɔbɔe!

Ðewohĩ èse be domenyiŋusẽfianu ƒe dɔléle aɖee wodzia ame aɖewo, alo? Cystic Fibrosis (CF) nye domenyiŋusẽfianu ƒe dɔléle. Ame geɖe susuna be esia nye dɔléle si gblẽa nu le lãkusiwo ŋu ko, elabena gbɔgbɔtsixetsixe kple aɖuɖɔtoedɔléle enuenu bɔ ɖe dɔléle sia me. Gake le nyateƒe me la, ŋutinyaa sesẽ vie wu ema. Na míalé ŋku ɖe nusi wònye ŋu.

Nuka tututue nye cystic fibrosis (CF)?

Kpuie ko la, cystic fibrosis (CF) nye domenyiŋusẽfianu ƒe dɔléle si nana be ʋuʋudedi gbogbo aɖewo siwo léna ɖe nu ŋu la ƒoa ƒu ɖe míaƒe ŋutilã ƒe ŋutinu aɖewo me. Aɖuɖɔ ɣi sia ate ŋu agblẽ nu le ŋutinu mawo ŋu eye wòagblẽ nu le woƒe dɔwɔwɔ ŋu.

Zi geɖe la, ʋuʋudedi si le míaƒe lãkusiwo kple ŋɔti me la ƒuna vie eye wòle sue. Enaa teƒe mawo xɔa dzo eye wòléa ɖinuwo kple gbeɖuɖɔwo ɖe mɔ̃ me. Gake le amesiwo ŋu CF le gome la, domenyiŋusẽfianu ƒe tɔtrɔ trɔa alesi wowɔa ʋuʋudedi siae. Protein aɖe si gbɔ esia tso la bu alo mewɔa dɔ nyuie o. Esia wɔnɛ be dze siwo nana ʋuʋudedi nɔa lãmenugbagbeviawo me la gena ɖe lãmenugbagbeviawo me, si wɔnɛ be ʋuʋudedia nɔa ʋeʋẽm ŋutɔ eye wòléna ɖe nu ŋu.

Togbɔ be ame geɖe bua eŋu be enye lãkusidɔ hã la, CF xɔ ŋkɔ elabena enaa ʋuʋudedi kple abi (fibrosis) nɔa dɔgboa me. Nusiwo gblẽ le eŋu sia, tsɔ kpe ɖe nusi le eme ƒe ƒoƒo ŋu, ate ŋu axe mɔ na ʋuka siwo ɖea nuɖuɖumeŋusẽ siwo kpena ɖe mía ŋu míeɖua nuɖuɖu si míeɖuna la. Esia ate ŋu ana ŋutilãa nagaxɔ nunyiame siwo le nuɖuɖu me nyuie o. Tsɔ kpe ɖe lãkusi kple dɔgbo ŋu la, ate ŋu agblẽ nu le aklã, aɖuɖɔtoe, dɔgbo, kple gbɔdɔdɔ ƒe ŋutinuwo hã ŋu.

CF nye domenyiŋusẽfianu ƒe dɔléle si wodzina kple ame. Esia fia be enye agbemeŋkekewo katã ƒe nɔnɔme si ate ŋu agblẽ ɖe edzi le ɣeyiɣi aɖe megbe. Ðewohĩ amesiwo ŋu CF le ƒe agbenɔƒe anɔ kpuie wu amesiwo ŋu CF mele o.

Ðe cystic fibrosis (CF) ƒomevi vevi aɖewo lia?

Ẽ, woate ŋu ade dzesi `CF` ƒomevi vevi eve:

1. Classic cystic fibrosis: Zi geɖe la, esia gblẽa nu le ŋutilãa ƒe ŋutinu geɖe ŋu. Zi geɖe la, wokpɔa dɔlélea le eƒe agbenɔƒe ʋɛ gbãtɔwo me.

2. Atypical cystic fibrosis: Esia nye CF si mesẽ boo o, si mesẽ o. Ate ŋu adze ŋutinu ɖeka ko dzi, alo dzesiwo ate ŋu ava eye woadzo. Zi geɖe la, ɖevi tsitsiwo alo sɔhɛ tsitsiwo ŋue wokpɔnɛ le.

Nukae nye cystic fibrosis (CF) ƒe dzesiwo?

Ame si ŋu CF le ate ŋu akpɔ dzesiwo abe:

• Aɖuɖɔtoedɔléle enuenu (e.g., aklãdɔ alo aɖuɖɔtoedɔléle si gbugbɔna) .). Bu eŋu kpɔ, ɖevi sue aɖewo ƒe ŋɔti me tsi, kɔdzi, kple ɣlidodo le woƒe akɔta ɣesiaɣi, eye ne wozã atike gɔ̃ hã la, wogatrɔna vaa wo gbɔ zi gbɔ zi geɖe gbɔdzɔe aɖeke manɔmee. Aleae wòle.
• Aɖuɖɔ siwo me mekɔ o alo esiwo me ami le la toto.
• Yagbɔgbɔ sesẽ.
• Gbɔgbɔtsixe nye gbeɖiɖi si ɖina enuenu, si ɖina tso akɔta.
• Sinus me dɔlékuiwo xɔxɔ enuenu.
• Kɔdzidede si nɔa anyi ɖaa.
• Tsitsi nɔa blewu.
• Ne èdo kpo nu le dzidzedzekpɔkpɔ me, togbɔ be èɖua nu nyuie eye nèkpɔa nuɖuɖumeŋusẽ si wò ŋutilã hiã hã la, nye nɔnɔme si me mèlolona alo mezua ame tsɛ o.

Dzesiwo le atypical cystic fibrosis (Atypical CF) ƒe dɔléle ŋu .

Dzesi siwo míeyɔ ɖe etame la dometɔ aɖewo ate ŋu anɔ amesiwo ŋu CF si mebɔ o le hã ŋu. Le ɣeyiɣi aɖe megbe la, woate ŋu ato nusiwo le abe:

• Sinusitis si nɔa anyi didi.
• No me ʋuʋudediwo.
• Elektrikŋusẽ ƒe agbɔsɔsɔ si mesɔ o le ŋutilãa me ate ŋu ana tsi nagbɔdzɔ alo dzoxɔxɔ nadze ame dzi.
• Dɔmetɔtrɔ.
• Dɔgbo ƒe dɔléle.
• Lolo dzi ɖeɖe kpɔtɔ le manyamanya me.

Nukae hea cystic fibrosis (CF) vɛ?

Nusi koŋ gbɔ CF tsonae nye domenyiŋusẽfianu si woyɔna be CFTR ƒe tɔtrɔ (trɔtrɔ alo tɔtrɔ). CFTR domenyiŋusẽfianu wɔa protein si wɔa dɔ abe ion-mɔ̃ ene le lãmenugbagbeviwo ƒe anyime. Bu eŋu abe agbo sue aɖe si le lãmenugbagbeviwo ƒe lãkusi me ene. Agbo siawo ɖea mɔ na nu suesuesue aɖewo toa eme.

Zi geɖe la, protein si `CFTR` domenyiŋusẽfianu wɔna la wɔa dɔ abe agbo ene na chloride ions (dze ƒomevi aɖe si me elektrikŋusẽ gbegblẽ le). Ne chloride ions do le lãmenugbagbevi la me la, wohea tsi hã yia eme. Gake le amesiwo ŋu `CF` le gome la, nuwɔna sia medzɔna nyuie o le tɔtrɔ siwo va le `CFTR` domenyiŋusẽfianu me ta. Emegbe chloride ions-awo gena ɖe lãmenugbagbeviawo me, si wɔnɛ be ʋuʋudedia nɔa ʋeʋẽm eye wòléna ɖe nu ŋu.

CFTR domenyiŋusẽfianu ƒe tɔtrɔ ƒomevi vovovowo li (ƒomevi I va ɖo VI). Dzɔdzɔmenugbagbevi aɖewo mewɔa protein aɖeke o, ɖewo wɔa protein sue aɖe ko, eye ɖewo mewɔa protein si wowɔna o.

Ðe cystic fibrosis (CF) nye dɔléle si wodzina?

Ẽ, `CF` nye domenyiŋusẽfianu ƒe nɔnɔme si ƒe dome wonyi tso edziɣi. Ame si ŋu `CF` le nyia `CFTR` domenyiŋusẽfianu sia si trɔ la ƒe kɔpi eve dome – ɖeka tso dadaa gbɔ, eye ɖeka tso fofoa gbɔ (woyɔa esia be `autosomal recessive` domenyinyi).

Mehiã be CF nanɔ dziwòlawo si hafi nàxɔ CF o. Le nyateƒe me la, ƒome akpa gãtɔ ƒe ƒome ŋutinya mexɔ CF o. Woyɔa amesi si domenyiŋusẽfianu si trɔ ƒe kɔpi ɖeka koe le be domenyiŋusẽfianu si tsɔa domenyiŋusẽfianu . Amesiwo tsɔe meɖea CF ƒe dzesiwo fiana o.

Ðe ame tsitsiwo hã ate ŋu axɔ cystic fibrosis (CF)a?

Ao, wodzi wò kple domenyiŋusẽfianu ƒe tɔtrɔ si hea CF vɛ. Gake ne dzesiawo nu mesẽ kura o, alo ne wova yina la, ate ŋu adzɔ be womakpɔ dɔlélea le ame aɖewo ŋu o vaseɖe esime wova tsi, ɖewohĩ va ɖo woƒe tsitsime gɔ̃ hã.

Nukawoe nye kuxi siwo ate ŋu ado tso CF me?

CF ate ŋu ahe kuxiwo abe:

• Dɔlékuiwo: Aɖuɖɔtoe si le ɣie léa dɔlékuiwo ɖe wò lãkusiwo kple yaƒoƒomɔ̃wo me, si wɔnɛ be esesẽna na wo be woado go. Esia ate ŋu ana ame naxɔ dɔlékuiwo enuenu.
• Vas deferens ƒe akpa eve ƒe anyimanɔmanɔ si wodzina (CBAVD): Le nɔnɔme sia me la, vas deferens, si nye mɔ̃ si me tsinyenye tsɔna, mele ŋutsuwo si o. Eyata wohiã kpekpeɖeŋu le vidzidzi ƒe atikewɔwɔ me ne wodi be yewoadzi vi.
• Suklidɔ: Suklidɔ si do ƒome kple cystic fibrosis ate ŋu adzɔ le esi dɔgboa gblẽ ta.
• Nunyuimakpɔɖui: Esi ʋuʋudedi ɣiwo mele nuɖuɖumeŋusẽ me o kple dɔgbo ƒe enzyme siwo kpena ɖe nuɖuɖu ŋu be wòaɖu nu la nana nunyuimakpɔɖu ƒe afɔkua dzina ɖe edzi.
• Ƒumeŋuɖui kple Ƒumeŋuɖui: Dɔléle siwo nana ƒu gbɔdzɔna ate ŋu ado mo ɖa le esi womete ŋu xɔa nunyiame siwo le nuɖuɖumeŋusẽ me nyuie o ta.
• Fufɔfɔ ƒe kuxiwo: CF ate ŋu agblẽ nu le nuɖuɖumeŋusẽ ŋu eye wòana nunyuimakpɔɖu. Esia dzia fufɔfɔ ƒe kuxiwo ƒe afɔkua ɖe edzi. Vidzidzi do ŋgɔ na ɣeyiɣi si woɖo ɖie nye kuxi si bɔ wu.

Aleke wokpɔa dɔléle si nye cystic fibrosis (CF) dzea sii?

Zi geɖe la, ɖɔktawo doa vidzĩ yeyewo kpɔna be CF le wo me hã le vidzĩ yeyewo dodokpɔ me. Wowɔa esia to ʋu ʋee aɖewo ɖeɖe le vidzĩa ƒe afɔkpodzi me. Le dodokpɔxɔa me la, wodoa ʋu sia kpɔna be atike aɖe si woyɔna be immunoreactive trypsinogen (IRT) hã le eme hã. Esia tsoa dɔgboa me. IRT sɔ gbɔ ɖe amesiwo ŋu CF le ƒe ʋu me. Wodoa vidzĩwo kpɔna be woate ŋu akpɔ IRT hã le wo dzidzi me eye wogadoa wo kpɔna le kwasiɖa ʋɛ aɖewo megbe.

Gake nɔnɔme aɖewo, abe vidzidzi kaba ene hã ate ŋu ana IRT ƒe agbɔsɔsɔ nadzi ɖe edzi. Eyata ne wodo IRT kpɔ be enyo la, mefia kokoko be CF le vidzĩ ŋu o. Ne vidzĩa ƒe IRT ƒe agbɔsɔsɔme sɔ gbɔ wu alesi wokpɔ mɔe la, ɖɔktaa aɖe gbe be woagawɔ dodokpɔ bubuwo atsɔ akpɔ dɔlélea mlɔeba.

Ɣeaɖewoɣi (anɔ abe 5% ene), ɖewohĩ vidzĩ yeyewo dodokpɔ mate ŋu ade dzesi IRT ƒe agbɔsɔsɔ si dzi ɖe edzi le amesi ŋu CF le ŋu o. Alo, ɖewohĩ womedo wò kpɔ edziedzi be CF le ŋuwò le vidzidzi me o. Ne CF ƒe dzesiwo le mia alo viwòa ŋu la, ɖɔkta awɔ fifia dodokpɔ eye ne ehiã la, awɔ dodokpɔ bubuwo hã.

Dodokpɔ siwo wowɔna le cystic fibrosis (CF) ŋu .

• Fifia dodokpɔ:Esia dzidzea chloride ƒe agbɔsɔsɔ si le wò fifia me. Chloride sɔ gbɔ ɖe amesiwo ŋu CF le ƒe fifia me. Esiae nye dodokpɔ si koŋ wowɔna tsɔ dea dzesi CF. Gake ate ŋu asɔ le amesiwo ŋu CF si mebɔ o le gome.
• Domenyiŋusẽfianu dodokpɔ: Woɖea ʋu tsɔ léa ŋku ɖe tɔtrɔ siwo va domenyiŋusẽfianu siwo hea CF vɛ ŋu.
• Nɔnɔmetatawo dodokpɔ: Wozãa nuwo abe X-ray siwo woɖena le ŋɔti me kple akɔta tsɔ ɖoa ​​kpe CF ƒe dzesi dzi alo doa alɔe. Nɔnɔmetatawo dodokpɔ siawo ɖeɖe mate ŋu ade dzesi CF o.
• Aklã ƒe dɔwɔwɔ dodokpɔ (PFT): Esiawo dzidzea alesi wò aklã le dɔ wɔm nyuie.
• Aɖuɖɔtoe ƒe dekɔnu: Wò ɖɔkta aɖe aɖuɖɔ si dona le wò lãkusi me ne èkɔ akɔta eye wòdoe kpɔ be dɔlékuiwo le eme hã. Dɔlékui ƒomevi aɖewo, abe Pseudomonas ene, bɔ ɖe amesiwo ŋu CF le me wu.
• Dɔgbo ƒe lãmenugbagbeviwo ɖeɖe: Esia wɔnɛ be wò ɖɔkta kpɔae ɖa be ʋuʋudedi alo abi aɖewo le wò dɔgboa me hã.
• Nasal potential difference (NPD): Esia dzidzea elektrikŋusẽ sue si nɔa ŋɔti ƒe lãkusi me zi geɖe. Ionwo ƒe ʋuʋu ye hea dzoxɔxɔ sia vɛ. Ame siwo ŋu CF le la ƒe ionwo meʋãna boo o le alesi CF kpɔa ŋusẽ ɖe ion ƒe mɔwo dzii ta.
• Intestinal current measurement (ICM): Be ɖɔkta nawɔ dodokpɔ sia la, eɖea dɔgbo ƒe lãkusi ƒe kpɔɖeŋu. Nudokpɔƒea dzidzea chloride agbɔsɔsɔme si dona tso kpɔɖeŋu sia me.

Aleke wodaa cystic fibrosis (CF) ƒe dɔléleae?

Nublanuitɔe la, atike aɖeke meli na CF o. Gake ne CF ŋuti nunyala bibi aɖe kple ame bubu siwo le wò lãmesẽnyawo gbɔ kpɔha me kpe ɖe ŋuwò la, àte ŋu akpɔ dɔlélea kple eƒe dzesiwo gbɔ. Dɔdzikpɔkpɔ sia ƒe akpa aɖewoe nye:

• Yaƒoƒomɔ̃wo me kɔ eye woaʋu to gbɔgbɔ ƒe mɔnuwo kple mɔ̃ siwo tsia ʋuʋudedi me zazã me.
• Atike siwo kpena ɖe ame ŋu wòɖɔa kuxi siwo le `CFTR` protein (`CFTR modulators`) ŋu ɖo.
• Atike siwo ɖea dzesi tɔxɛwo dzi kpɔtɔna.
• Kpɔ egbɔ be yekpɔ nuɖuɖumeŋusẽ si sɔ eye wòsɔ gbɔ tso nuɖuɖu me.
• Amekoko.

Yameʋudzeƒewo ɖeɖeɖa ƒe mɔnuwo

Ne CF le ŋuwò la, mɔ geɖe li siwo dzi nàto ana wò yaƒoƒomɔ̃wo nakɔ:

• Kɔdzi kple gbɔgbɔ ƒe mɔnuwo: Lãmesẽdɔwɔla si bi ɖe CF me ate ŋu afia aɖaŋu siwo nàtsɔ aʋu wò yaƒoƒomɔ̃wo eye nàɖe ʋuʋudedi ɖa la wò.
• Yagbɔgbɔ ƒe nyaƒoɖeamenu nyui (PEP) ƒe mɔ̃wo:Woateŋu ado `PEP` mɔ̃ siawo le nu me alo abe `mask` ene ɖe mo. Wonaa tsitretsitsi, eyata ele be nàku kutri geɖe wu hafi agbɔ ya. Esia ʋua yaƒoƒomɔ̃wo eye wòtutua ʋuʋudedi la doa goe. Ʋuʋudedi `PEP` mɔ̃wo (e.g. `Flutter®`, `Acapella®`, `AerobikA®`, `RC-Cornet®` ) nye `PEP` ƒomevi tɔxɛ siwo ƒoa ʋuʋudedi kple ŋutete be woaɖe ʋuʋudedi nu ƒu ɖekae.
• Yameʋuɖoɖo ƒe ʋuƒo: Wogayɔnae be akɔta ƒe gli ƒe ʋuʋu ƒe mɔ̃ si wozãna zi geɖe, eye esia nyea ya si wotsɔna ƒoa yae si wotsɔ doa ka kple mɔ̃. Vest la ʋuʋuna eye wògbãa ʋuʋudedi.
• Tsi si dona le nɔnɔme me kple ƒoƒo: Esia nye kamedede ƒe mɔnu. Èʋuna yia nɔnɔme siwo kpena ɖe ŋuwò nèɖea ʋuʋudedi ɖa le wò lãkusiwo me. Ame bubu ƒoa wò akɔta kple/alo wò megbe be wòakpe ɖe ŋuwò nàɖe ʋuʋudedia ɖa. Woate ŋu awɔ esia kpe ɖe kɔdzidɔ ƒe mɔnuwo ŋu.

CFTR ƒe tɔtrɔwo na cystic fibrosis

CFTR modulators nye atikewo ƒe hatsotso aɖe si kpena ɖe ame ŋu wòɖɔa kuxi siwo le protein siwo CFTR domenyiŋusẽfianu si trɔ la wɔ ŋu ɖo eye wodzia protein si wɔa dɔ nyuie le lãmenugbagbeviwo ƒe anyime ƒe agbɔsɔsɔ ɖe edzi. Womedaa CF o. Gake ame aɖewo ƒe dzesiwo kple woƒe agbenɔƒe nyo ɖe edzi ŋutɔ. Gake modulator-dɔdamɔnu siawo mesɔ na amesiame si ŋu CF le o, eye wo dometɔ aɖewo mate ŋu ado dzi atsɔ wo o.

`CFTR modulators` ƒe kpɔɖeŋu aɖewo:

• `Kalydeco® (nu si wotsɔ wɔa dɔe)`
• ``Orkambi® (vacaftor/lumacaftor)'' ƒe dziƒoe nye esia.
• `Symdeko® (vakaftor/tezacaftor)`
• `Trikafta® (vakaftor/tezacaftor/elexacaftor)`

Atike bubu siwo wozãna na cystic fibrosis

Wò ɖɔkta ate ŋu aŋlɔ atike bubuwo hã na wò be wòaɖe abi dzi akpɔtɔ, ada dɔlékuiwo, alo akpɔ dzesiawo gbɔ. Esiawo dometɔ aɖewoe nye:

• Dɔlékuiwutikewo: Wò ɖɔkta ate ŋu aŋlɔ dɔlékuiwutikewo na wò be woatsɔ ada dɔlékuiwo alo axe mɔ ɖe enu.
• Aɖuɖɔtoekekemɔ̃ siwo wogbɔna: Aɖuɖɔtoe kekeɖenudɔlékuiwo ʋua yaƒoƒomɔ̃wo heɖea dzi ɖi, si wɔnɛ be gbɔgbɔ nɔa bɔbɔe.
• Inhaled hypertonic saline: Dze si le saline solutions me hea tsi, si nana tsi si le lãme na ame la nɔa sue eye wònana wònɔa bɔbɔe be ame nakɔ.
• Atike siwo tsia abi nu: Atike siawo ɖea abi dzi kpɔtɔna. Esiawo dometɔ aɖewoe nye corticosteroids kple nonsteroidal anti-inflammatory drugs (NSAIDS) .
• Dɔgbo me dɔlékuiwo: Esiawo kpena ɖe nuɖuɖu ŋu wòɖua nu eye wòxɔa nunyiame siwo le eme.
• Aɖuɖɔ falɛfalɛ: Esiawo kpena ɖe nɔnɔmewo abe dɔmeɖui ene ŋu eye wònana wònɔa bɔbɔe be woato aɖuɖɔ me.

Nuɖuɖu na cystic fibrosis (CF) ƒe dɔléle .

Ne CF le ŋuwò la, wò nuɖuɖu ƒe hiahiãwo to vovo na amesi si CF mele o tɔ. Le CF ta la, ɖewohĩ wò dɔgbo mate ŋu awɔ enzyme siwo kpena ɖe ŋuwò nèɖua nuɖuɖu alo ɖea wo ɖa o. Esia fia be ɖewohĩ wò dɔgbowo mate ŋu axɔ nunyiame kple ami siwo le wò nuɖuɖu me nyuie o.

Wò CF ŋuti nunyala alo nuɖuɖuŋutinunyala si woŋlɔ ŋkɔ na aɖo aɖaŋu na wò be nàwɔ ɖoɖo ɖe nunyiame ŋu na wò. Ate ŋu anye:

• Calorie si woɖuna gbesiagbe. Esia ate ŋu asɔ gbɔ wu amesi si CF mele o ƒe teƒe eve.
• Nuɖuɖu si me ami sɔ gbɔ ɖo ɖuɖu. Esia le vevie be vitamin siwo me ami le wu nasu ame si.
• Ŋutilã ƒe kpekpeme si lolo wu esi sɔ me léle ɖe asi tso ɖevime ke. Esia ate ŋu akpe ɖe ŋuwò nàtsi eye wò lãkusiwo nakeke. Esia ate ŋu akpe ɖe ŋuwò le dzesiwo me ne èle tsitsim.
• Enzyme kpeɖeŋutɔ ƒe atikekuiwo nono. Enzyme kpeɖeŋutɔ kpena ɖe nuɖuɖumeŋusẽ ŋu.
• Dze geɖe tsɔtsɔ de wò nuɖuɖu me. Esia kpena ɖe ame ŋu be wòaxɔ ɖe dze si sɔ gbɔ si wò ŋutilã bu to fifia me la teƒe. Esia le vevie ŋutɔ le yame ƒe dzoxɔxɔ kple fafaɣi kple ne èle kame dem. Bia wò ɖɔkta tso dze agbɔsɔsɔme si nèhiã gbesiagbe ŋu.

Amekoko na dɔléle si nye cystic fibrosis (CF) .

Ðewohĩ ahiã be woawɔ dɔ na wò le CF alo CF ƒe kuxi aɖe ta. Esia ate ŋu alɔ:

• Amekoko siwo ku ɖe ŋɔti alo ŋɔti me.
• Amekoko be woaɖe dɔgboa ƒe mɔxenuwo ɖa.
• Aklã dodo na ame.
• Aklã dodo na ame.

Ðe cystic fibrosis (CF) nye dɔléle si ate ŋu ade ame ƒe agbe afɔku mea?

Ẽ, CF nye dɔléle si ate ŋu ade ame ƒe agbe afɔku me. Nusi koŋ gbɔ ku tsonae nye lãkusi ƒe gbegblẽ si tso ʋuʋudedi ɣi kple lãkusi me dɔlékuiwo xɔxɔ enuenu gbɔ.

Nukae nye ame si ŋu cystic fibrosis (CF) le ƒe agbenɔƒe?

Le eŋutinunyalawo ƒe nya nu la, le ƒe ʋɛ siwo va yi me la, amesi wodzi kple CF ƒe agbenɔƒe anɔ abe ƒe 50 ene. Ƒe ʋɛ aɖewoe nye esi va yi la, ame ƒe agbenɔƒe nɔa ƒe 30 va ɖo ƒe 40, gake esia dzi ɖe edzi le ƒe ʋɛ siwo va yi me le ŋgɔyiyi siwo wowɔ le atikewɔwɔmɔnu me ta.

Ame siwo ŋu CF si mebɔ o le la nɔa agbe didi wu amesiwo ŋu CF si mebɔ o le.

Nukae wòle be makpɔ mɔ na ne CF le ŋunye?

Atike aɖeke meli na CF o. Mia alo viwòa ahiã be woawɔ atike na wò le wò agbemeŋkekewo katã me hafi nàte ŋu akpɔ egbɔ. Esia lɔ dɔlékuiwo dada, nunyiame me léle ɖe asi, kple CF eŋutinunyala gbɔ yiyi edziedzi ɖe eme. Gake atikewɔwɔ yeyewo kpe ɖe ɖevi siwo ŋu CF le ŋu wonɔ agbe nyuie vaseɖe woƒe tsitsime eye woƒe agbenɔnɔ nyo wu.

Atikewɔwɔ wɔa dɔ nyuie wu ne wokpɔ CF kaba. Esia tae vidzĩ yeyewo dodokpɔ le vevie ŋutɔ ɖo. Atikewɔwɔ abe CFTR modulators ene gɔmedzedze le ɖevime ate ŋu ana lãmesẽ si anɔ anyi didi nanyo ɖe edzi eye wòana ame ƒe agbenɔƒe nadzi ɖe edzi.

Ðe woate ŋu axe mɔ ɖe CF nua?

Esi wònye be CF nye nusi wodzi wò kple ta la, mɔ aɖeke meli si dzi nàto axe mɔ ɖe enu o. Ne CFTR domenyiŋusẽfianu ƒe tɔtrɔ le ŋuwò la, àte ŋu abia wò ɖɔkta tso domenyiŋusẽfianu dodokpɔ do ŋgɔ na vidzidzi kple alesi wòanya wɔ be viwòwo naxɔ CF ŋu.

Aleke mawɔ alé be na ɖokuinye?

Beléle na ɖokuiwò kple CF fia be nàwɔ dɔ kple wò lãmesẽnyawo gbɔ kpɔha be nàwɔ atikewɔwɔ ƒe ɖoɖo. Be nànɔ lãmesẽ me la, ele be nàwɔ ɖe ɖoɖo sia dzi nyuie ŋutɔ. Eƒe akpa aɖewoe nye:

• Wɔ ɖe wò gbɔgbɔtsixetsixe ŋuti kɔklɔ ƒe ɖoɖoa dzi pɛpɛpɛ.
• Atikewo nono abe alesi woŋlɔe ene.
• Yi edzi nànɔ atikewɔƒewo kple wò CF atikewɔlawo ƒe ƒuƒoƒoa.

Xɔ aɖaŋuɖoɖo tso wò ɖɔktawo gbɔ ku ɖe nuɖuɖu nyui ƒe ɖoɖo kple kamedede si me afɔku mele o si sɔ na wò ŋu. Bia wò ɖɔkta nenye be aklã ƒe ɖɔɖɔɖo nye susu nyui aɖe na wò.

Àte ŋu aɖe dɔlékuiwo xɔxɔ ƒe afɔkua dzi akpɔtɔ ne èƒo asa na amesiwo dze dɔ, asiklɔ ƒe mɔnu nyuiwo zazã, kple atikewɔmɔnu siwo wokafu na wò .

Àte ŋu akpɔ gome le dɔdamɔnu dodokpɔ siwo doa CF ƒe atike yeyewo kpɔna hã me. Bia wò ɖɔkta ne ɖewo li siwo sɔ na wò. Kpɔ egbɔ be yexɔ nyatakaka blibo tso viɖe kple afɔku siwo le dɔdamɔnu dodokpɔwo me ŋu.

Ɣekaɣie wòle be makpɔ nye ɖɔkta?

De atikewɔƒe siwo katã woɖo ɖi kple wò lãmesẽnyawo gbɔ kpɔha me tɔwo. Ƒo nu kple wò ɖɔkta ne nya aɖe le asiwò ku ɖe wò atikewɔwɔ ƒe ɖoɖoa alo wò dzesiwo ŋu. Bia wo be nukae woawɔ ne dɔlékui aɖe ƒe dzesiwo le ŋuwò. Àte ŋu agblɔe na wo hã ne èhiã kpekpeɖeŋu le hadome alo seselelãmekuxiwo me.

Ɣekaɣie wòle be mayi Dɔwɔƒe si Kpɔa Nudzɔdzɔ Kpatawo Gbɔ (ETU) ?

Ne dzesi sesẽ siawo le ŋuwò la, yi xɔ si me wowɔa dɔ kpata le enumake:

• Asrã sesẽ (si wua dzidzenu Fahrenheit 103/Celsius 40).
• Yagbɔgbɔ sesẽ.
• Aɖuɖɔ si dona le lãme na ame aɖeke meli o alo mesɔ gbɔ kura o.
• Vevesese si nɔa anyi ɖaa le akɔta alo ƒodo me.
• Taɖuame.
• Tɔtɔ.
• Lãmetsiwo ƒe vevesese sesẽ alo gbɔdzɔgbɔdzɔ.
• Alɔ̃madɔmadɔ.
• Ŋutilã, nuyi alo asibidɛ ƒe amadede blɔ (`cyanosis` - esia ateŋu anye dzesi be oxygen ƒe agbɔsɔsɔ si le wò ʋu alo lãkusiwo me mede o).
• Ne asrã alo kɔdzidɔa nu sẽ alo nu yi la, ekema egagblẽ ɖe edzi ake.

Nya kawoe wòle be mabia nye ɖɔkta?

Àte ŋu abia wò ɖɔkta nya siawo abe esiawo ene:

• Atikewɔwɔmɔnu kawoe le asinye?
• Nukae nye nuɖuɖu nyui ƒe ɖoɖo si dzi mate ŋu awɔ ɖo?
• Nukae mate ŋu awɔ be makpɔ nye dɔlélea ƒe dzesiwo gbɔ?
• Dɔlékui ƒe dzesi kawo ŋue wòle be malé ŋku ɖo?
• Ɣekaɣie wòle be magakpɔ wò ake?
• Dzesi kawoe wòle be mayi xɔ si me wowɔa dɔ kpata le?
• Ðe nèdi be yealé ŋku ɖe ƒomea me tɔ bubuwo ŋua?

Nukatae mele be amesiwo ŋu CF le naka asi wo nɔewo ŋu o?

Zi geɖe la, lãmesẽdɔwɔlawo kafua amesiwo ŋu cystic fibrosis (CF) le be woaƒo asa na kadodo kplikplikpli kple ame bubuwo. Esia le alea elabena amesiwo ŋu CF le ate ŋu axɔ dɔlékui siwo dzi ame bubuwo ate ŋu aɖu bɔbɔe. Woate ŋu akaka dɔlékuiwo hã ɖe ame bubu siwo ŋu CF le (siwo hã ŋu dɔlékui siwo dzi ɖuɖu sesẽna le) hã le. Ele be amesiwo ŋu CF le hã naƒo asa na amesiame si le dɔ lém.

Mlɔeba la, nane (Take-Home Message) .

Enye nusi sɔ be nàse le ɖokuiwò me be yegbɔdzɔ vie ne wokpɔe be dɔléle aɖe le ŋuwò le wò agbemeŋkekewo katã me. Gake atikewɔwɔ yeyewo kple cystic fibrosis gɔmesese nyuie wu fia be ɣeyiɣi geɖe sua asiwò nàtsɔ axɔ nuwo gbesiagbe. Fifia ame geɖe siwo ŋu CF le ate ŋu akpɔ mɔ be yewoanɔ agbe bliboe vaseɖe tsitsime. Anɔ eme be atikewɔmɔnu geɖe wu asu ɖevi si ŋu wokpɔ CF le egbea le etsɔme.

Ƒo wò lɔlɔ̃tɔwo kple atikewɔla siwo dzi nèka ɖo ƒe ƒuƒoƒo aɖe nu ƒu be woase nusi nàkpɔ mɔ na gɔme, be woakpe ɖe ŋuwò nànɔ te ɖe gbesiagbegbenɔnɔ ƒe kuxiwo nu. Eye, mègavɔ̃ be yeaxɔ nukpɔsusu evelia le mɔa ƒe akpa aɖeke o. Menye wò ɖeka koe o, eye ame geɖewo li siwo ate ŋu akpe ɖe ŋuwò le mɔzɔzɔ sia me.

` Cystic fibrosis, domenyiŋusẽfianu ƒe dɔlélewo, lãkusidɔ, ʋuʋudedi, CFTR domenyiŋusẽfianu, ɖeviwo ƒe lãmesẽ, gbɔgbɔtsixe ƒe kuxiwo