So wo ba kumaa no koma benkum nnyin yiye? (Hypoplastic Left Heart Syndrome - HLHS) Momma yɛnka eyi ho asɛm!

Ɛnnɛ yɛrebɛka komayareɛ bi a ɛyɛ den kakra, na ɛntaa nsi paa, nanso ɛho hia paa ho asɛm. Eyi yɛ tebea bi a wɔfrɛ no Hypoplastic Left Heart Syndrome , anaa (HLHS) tiawa. Ebia wobɛte ehu nka bere a wote eyi, nanso mma ɛnhaw wo. Momma yɛnka eyi ho asɛm tiawa, wɔ ɔkwan a wubetumi ate ase so. Efisɛ, ɛho hia paa sɛ yɛma yɛn ani da hɔ wɔ nneɛma a ɛte sɛɛ ho.

Dɛn ne koma benkum mu yare a ɛma mogya tu (HLHS)?

Sɛ yɛbɛka no tiawa a, (HLHS) yɛ komayare a wɔde awo obi a ɛwɔ hɔ bere a wɔawo no . Wɔ eyi mu no, akokoaa no koma benkum fã bi nnyin yiye. Fa no sɛ ɛte sɛ pɔmpɛ. Pɔmpɛ yi benkum so na ɛde mogya a oxygen pii wom kɔ nipadua no nyinaa mu. Enti, sɛ benkum fam yi ntumi nkɔ yiye a, ɔhaw bi sɔre. Wɔ eyi mu no, koma no benkum so afã horow yi titiriw na ɛka:

• Benkum so ahurututu : Eyi ne ɔdan titiriw a ɛwɔ koma no benkum fam ase. Ɛde mogya a oxygen wom kɔ aorta no mu . Wɔ HLHS mu no, ɛtaa yɛ ketewaa dodo sɛ ɛbɛyɛ adwuma yiye.
• Aorta : Eyi ne mogya ntini a ɛsõ sen biara wɔ yɛn nipadua mu. Ɛde mogya fi koma no mu kɔ nipadua no nyinaa mu. Wɔ (HLHS) mu no, ebia eyi nso renkɔ so yiye.
• Mitral ne aortic valves: Eyinom te sɛ apon. Wɔma mogya sen kɔ ɔkwan biako pɛ so. Aortic valve no ma mogya fi benkum ventricle no mu kɔ aorta no mu. Mitral valve no ma mogya fi ɔdan a ɛwɔ soro (atrium) a ɛwɔ koma no benkum so no mu kɔ ɔdan a ɛwɔ fam (mitral valve) no mu . Wɔ HLHS mu no, ebia saa valve yi rennyɛ adwuma yiye anaasɛ ɛbɛyɛ ketewaa dodo.

Ɛtɔ mmere bi a, nkokoaa a wɔwɔ HLHS no betumi anya tokuru ketewaa bi wɔ ɔfasu no mu wɔ koma no atifi adan abien no ntam (atrial septal defect) . Mpɛn pii no, ɛsɛ sɛ eyi yɛ ɔfasu a ɛyɛ den.

Nsonsonoe bɛn na ɛda koma a ɛteɛ ne koma a ɛwɔ (HLHS) ntam?

Yɛn koma a ɛteɛ no wɔ afã abien. Ɔfã nifa no de mogya a oxygen nnim fi nipadua no mu kɔ ahurututu no mu ma ɛboaboa mframa pa ano. Afei, mogya a oxygen wom no kɔ benkum so na wɔde kɔ nipadua no fã a aka no mu. Nanso, wɔ akokoaa a ɔwɔ HLHS mu no, koma no benkum yɛ ketewaa araa ma entumi mfa mogya a ɛdɔɔso nkɔ nipadua no fã a aka no mu. Afei, ventricle nifa no gye dibea. Ɛbɔ mmɔden sɛ ɛde mogya bɛkɔ ahurututu ne nipadua no fã a aka no mu. Wɔnam ductus arteriosus so na ɛyɛ eyi.Ɛdenam mogya ntini titiriw bi a wɔfrɛ no ductus arteriosus so. Eyi (ductus arteriosus) yɛ mogya ntini a akokoaa biara wɔ wɔ awotwaa mu. Mpɛn pii no, ɛto mu nna kakraa bi wɔ awo akyi. Nanso wɔ akokoaa a ɔwɔ (HLHS) mu no, sɛ mogya ntini yi to mu, sɛ wɔansa a, ebetumi de ne nkwa ato asiane mu. Esiane sɛ benkum fam no ntumi nyɛ adwuma nti, ɔkwan biako pɛ a mogya fa so kɔ nipadua no mu no siw.

(HLHS) abu so dɛn?

(HLHS) yɛ tebea a ɛntaa nsi koraa. Sɛnea akontaabu a ɛwɔ United States kyerɛ no, ɛka nkokoaa 3,800 biara mu bɛyɛ biako afe biara. (HLHS) yɛ komayare a wɔde wo awo (CHD) nyinaa mu 2% kosi 3%. Ɛtaa ba mmarimaa so sen mmeawa.

Dɛn ne akokoaa a ɔwɔ (HLHS) ho sɛnkyerɛnne?

Ebia akokoaa a ɔwɔ (HLHS) ho sɛnkyerɛnne renna adi ntɛm ara. Ebia wobepue wɔ awo akyi nnɔnhwerew kakraa bi anaa nna kakraa bi. Nsɛnkyerɛnne titiriw a ɛkyerɛ ne sɛ:

• Cyanosis : Ɛyɛ kɔla a ɛyɛ bruu a ɛyɛ fitaa a ɛwɔ honam ani, anofafa, ne nsateaa nnadewa mu. Eyi betumi ada adi sɛ kɔla a ɛyɛ fitaa wɔ nkokoaa a wɔn honam ani yɛ tuntum mu. Ebia ɛbɛda adi sɛ kɔla bruu wɔ nkokoaa a wɔn honam ani yɛ hare no mu. Eyi fi nipadua no a ennya mframa pa a ɛdɔɔso.
• Ɔhome a ɛyɛ den : Ebia akokoaa no bɛte nka sɛ ɛyɛ den sɛ ɔbɛhome.
• Ɛyɛ den sɛ ɔbɛnom nufusu: Ebia akokoaa no mpɛ sɛ ɔbɛnom nufusu, anaasɛ ebia ɔbɛteɛteɛm bere a ɔrenom nufusu no.
• Ɔbrɛ : Ebia ɛbɛyɛ te sɛ nea akokoaa no ada na wayɛ ɔbrɛ.
• Koma a ɛbɔ ntɛmntɛm.
• Fifiri , honam ani a ɛyɛ nwunu, anaasɛ awɔw a wɔte nka.
• Apɔw-mu-teɛteɛ a ɛyɛ mmerɛw .

Sɛ wuhu saa sɛnkyerɛnne ahorow yi bi a, ɛho hia sɛ wohwehwɛ aduruyɛfo afotu ntɛm ara. Mma wo ho nnpopo, na mmom yɛ ho biribi ntɛm.

Dɛn ne nneɛma a ɛde (HLHS) ba?

Mpɛn pii no, biribiara nni hɔ pɔtee biara a ɛde HLHS ba . Ɛtɔ mmere bi a, ebetumi aba sɛ efi awosu mu nneɛma. Nkokoaa a wɔwɔ awosu mu nkwaadɔm mu nsakrae bi , te sɛ GJA1 anaa NKX2-5, wɔ asiane kɛse mu sɛ wobenya HLHS. Afei nso, nkokoaa a wɔwɔ awosu mu tebea horow bi te sɛ Turner syndrome anaa Trisomy 18 betumi anya HLHS.

Ɔkwan bɛn so na wohu HLHS?

Nnuruyɛfo betumi de mfoninitwa nhwehwɛmu a ɛnyɛ nea wɔde hyɛ nipadua mu ahu HLHS. Wobetumi ayɛ eyi bere a wɔyem anaasɛ wɔawo akokoaa no akyi. Bere a wɔyem no:

• Prenatal ultrasound : Eyi yɛ scan a wɔtaa yɛ bere a wɔyem.
• Fetal echocardiogram: Eyi yɛ scan titiriw bi a ebetumi ahwɛ sɛ ebia ɔwɔ komayare bere a akokoaa no da so ara wɔ awotwaa mu no.

Bere a wɔawo akokoaa no akyi: Nnuruyɛfo nam yare no ho sɛnkyerɛnne ahorow a wɔhwɛ na wɔhwɛ nea efii nhwehwɛmu no mu bae so hu tebea no. Ɛtɔ mmere bi a, sɛ wode afiri a wɔde tie asɛm retie akokoaa no koma a, ebia wobɛte koma mu nwiinwii . Eyi kyerɛ sɛ mogya no nkɔ yiye.

Nhwehwɛmu bɛn na wɔde hu (HLHS)?

• Chest X-ray: Eyi betumi ahwɛ sɛnea akokoaa no koma ne n’ahurututu yɛ kɛse ne sɛnea ɛte.
• Echocardiogram: Eyi nso yɛ ultrasound nhwehwɛmu. Wotumi hu nneɛma a ɛwɔ koma no mu no pefee.
• Electrocardiogram (EKG/ ECG ): Wɔde eyi susuw anyinam ahoɔden nsakrae a ɛba bere a koma rebɔ no.
• Pulse oximetry screening: Eyi betumi ahu oxygen dodow a ɛwɔ akokoaa no mogya mu.

So aduru bi wɔ hɔ a wɔde sa (HLHS)?

Yiw, ayaresa ahorow wɔ hɔ. Nea edi kan no, ɛsɛ sɛ wɔma akokoaa no aduru bi a wɔfrɛ no prostaglandin . Eyi ma ductus arteriosus no kɔ so bue. Eyi ma wonya ɔkwan bi a mogya fa nipadua no mu. Nea ɛka ho no, wobetumi de nnuru afoforo ama akokoaa no koma ma ayɛ adwuma yiye. Ebia ɛho behia nso sɛ wɔboa akokoaa no ma ɔhome. Afei, wobetumi ayɛ oprehyɛn ahorow pii. Wɔde oprehyɛn ahorow yi di dwuma de dan mogya a ɛkɔ ahurututu ne nipadua no mu. Wɔ oprehyɛn ahorow yi akyi no, ɛkame ayɛ sɛ wɔde koma no adwuma nyinaa kɔ nifa so ahurututu no mu. Ɛno na ɛde mogya kɔ nipadua no nyinaa mu.

Oprehyɛn bɛn na wɔyɛ ma (HLHS)?

Nnuruyɛfo yɛ oprehyɛn atitiriw abiɛsa: Norwood oprehyɛn , Glenn oprehyɛn , ne Fontan oprehyɛn . Wɔyɛ saa akwan horow yi nnidiso nnidiso. 1. Norwood adeyɛ: Wɔyɛ saa adeyɛ yi wɔ adapɛn abien a edi kan wɔ nkwa nna mu ma nkokoaa a wɔwɔ HLHS. Wɔ saa adeyɛ yi mu no, nnuruyɛfo a wɔyɛ oprehyɛn:

• Wɔsan sesa akokoaa no aorta a ennyaa nkɔso no sɛnea ɛbɛyɛ a mogya bɛsen akɔ nipadua no mu.
• Wɔde shunt , afiri ketewaa bi hyɛ mu de dan mogya fi nifa so ahurututu no mu, sɛ́ ebia efi aorta no mu, kɔ ahurututu mu ntini a ɛkɔ ahurututu no mu no mu.
• Ɛma nkitahodi ba koma no atifi adan (atria) no ntam. Eyi boa ma nipadua no nya mogya a oxygen pii wom fi ahurututu mu.

2. Glenn shunt oprehyɛn a wɔde fa akwan abien so: Ma akokoaa noƐsɛ sɛ wɔyɛ oprehyɛn a ɛto so abien no wɔ bɛyɛ asram 4 kosi 6 mu. Bere a wɔreyɛ Glenn oprehyɛn no:

• Wɔreyi shunt dedaw no afi hɔ.
• Wɔde shunt foforo hyɛ mu na ɛde akokoaa no superior vena cava (SVC) (a ɛde mogya a oxygen nnim fi nipadua no atifi kɔ koma no mu) no bata ahurututu mu ntini no ho.
• Saa shunt yi brɛ adesoa a ɛwɔ ventricle nifa no so no ase, efisɛ ɛma mogya kɔ ahurututu no mu tẽẽ.

3. Fontan nhyehyɛe: Wɔyɛ ayaresa a etwa to no bere a wɔadi asram 18 kosi mfe 4 . Saa adeyɛ yi dan mogya a ɛsan fi nipadua no mu kɔ ahurututu no mu nyinaa, na ɛtwa koma no ho hyia. Wɔ Fontan nhyehyɛe no mu no:

• Akokoaa no inferior vena cava (IVC) (a ɛde mogya a oxygen nnim fi nipadua no ase kɔ koma no mu) no ne ahurututu mu ntini no di nkitaho.

So nsɛnnennen bi wɔ ayaresa no mu?

Yiw, nkokoaa binom mpo tumi hwere wɔn nkwa bere a wofi oprehyɛn biako mu kɔ foforo mu no. Afei nso, ɔhaw ahorow bi betumi aba wɔ oprehyɛn biara akyi. Nhwɛsoɔ:

• Ɔhaw ahorow a ɛfa ventricle nifa a ɛnyɛ adwuma yiye ho.
• Aortic valve a ɛretu.
• Mmɛn mu yare.
• Koma mu nnyigyei a ɛnyɛ ne kwan so.
• Mogya a ɛtwetwe.
• Nsaneɛ.
• Adidi a ɛyɛ den.
• Akisikuru a ɛma obi ho kyere no.
• Asaabo mu haw ahorow.
• Koma a ɛyɛ den.

Eyinom yɛ nneɛma a ɛyɛ hu sɛ wobɛte, nanso nnuruyɛfo bɛka asiane ahorow yi ho asɛm akyerɛ wo na wɔabɔ mmɔden sɛ wɔbɛma wo ayaresa a eye sen biara.

So koma a wɔde ma obi foforo no yɛ ayaresa pa?

Ɛtɔ mmere bi a, ebia nnuruyɛfo a wɔyɛ oprehyɛn bɛkamfo akyerɛ sɛ wɔmfa koma foforo mma sen sɛ wɔbɛyɛ saa oprehyɛn abiɛsa no. Nanso, ɛho behia sɛ nkokoaa a wɔama wɔn koma foforo no nom nnuru (nnuru a ɛma nipadua no tumi ko tia nyarewa) wɔn nkwa nna nyinaa.

So wobetumi asa HLHS ansa na wɔawo?

Mprempren ɛrentumi nyɛ yiye sɛ wɔbɛsa HLHS koraa denam oprehyɛn so bere a wɔyem no. Nanso, ɛtɔ mmere bi a, awotwaa mu oprehyɛn ho animdefo betumi ayɛ nneɛma bi de asiw nsunsuanso bɔne bi a ebetumi aba wɔ akokoaa a ɔrenyin a ɔwɔ HLHS (e.g., aortic valve ketewaa bi) no bi ano. Nanso eyi te saa wɔ nsɛm titiriw bi mu nkutoo.

So wobetumi atew asiane a ɛwɔ (HLHS) mu no so?

Esiane sɛ wonnim nea ɛde HLHS dodow no ara ba nti, ɛyɛ den sɛ wɔbɛka ɔkwan a wɔbɛfa so asiw ano koraa. Nanso, ɛho hia bere nyinaa sɛ wudi suban pa akyi bere a wonyinsɛn no:

• Kwati asanom ne sigaretnom.
• Ayaresa mu tebea afoforo te sɛ Asikreyare a wobedi so .
• Aduan a ahoɔden wom a wobedi.
• Vitamin a wɔde di dwuma ansa na wɔawo a anyɛ yiye koraa no folic acid microgram 400 (400 mcg) wom da biara da .

Sɛ wo anaa wo hokafoɔ wɔ abusua abakɔsɛm a ɛfa HLHS ho a, ɛyɛ papa sɛ wo ne awosuo ho ɔfotufoɔ bɛkasa ansa na woanyinsɛn.

Sɛ me ba no anya (HLHS) a, dɛn na ɛsɛ sɛ mehwɛ kwan?

Sɛ wo ba no sa HLHS wie a, anyɛ yiye koraa no, ɛho behia sɛ ɔkɔ komayare ho oduruyɛfo nkyɛn pɛnkoro afe biara wɔ ne nkwa nna nyinaa mu. Eyi bɛma ahwɛ ahu sɛ ne koma, n’ahurututu, ne ne akwaa afoforo reyɛ adwuma yiye. Bere a wo ba no nyin no, ɛho behia sɛ ɔkɔ oduruyɛfo bi a ɔhwɛ komayare a wɔde awo mpanyimfo so nkyɛn . Mmofra pii a wɔwɔ HLHS no behia sɛ wɔnom koma mu nnuru wɔn nkwa nna nyinaa. Ɛho behia nso sɛ wɔnom nnuru a ekum ɔyare mmoawa ansa na wɔayɛ wɔn oprehyɛn biara, a wɔn sẽ oprehyɛn ka ho. Eyi betumi atew asiane a ɛwɔ hɔ sɛ obi benya endocarditis (yare mmoawa a ɛba koma no mu) so.

Dɛn ne anidasoɔ a ɛwɔ hɔ ma (HLHS) gyinabea?

Sɛ wɔansa a, HLHS yɛ tebea a ebetumi ama obi awu wɔ nna anaa adapɛn bi akyi wɔ awo akyi. Wɔ ayaresa mu no, adwene a ɛwɔ hɔ no gyina sɛnea komayare a ɛwɔ akokoaa no mu no yɛ den no so. Bisa wo ba no oduruyɛfo no asiane ahorow a ɛwɔ oprehyɛn biara mu. Ebia mmofra binom ntumi nteɛteɛ wɔn apɔw mu wɔ wɔn nkwa nna nyinaa mu. Nnuruyɛfo taa kamfo kyerɛ sɛ wɔmfa apɔw-mu-teɛteɛ a emu yɛ den te sɛ akansi agumadi ano hye.

Nkokoaa a wɔwɔ (HLHS) nkwa nna yɛ dɛn?

Eyi yɛ nokwasɛm a ɛyɛ awerɛhow kakra, nanso ɛho hia sɛ wuhu. Nkokoaa a wɔwɔ (HLHS) no mu 20% kɔsi 60% na wɔte ase wɔ wɔn nkwa afe a ɛdi kan no mu. Ɛno akyi no, nkwa a wɔbɛnya wɔ mfeɛ anum, du, ne dunum a ɛdi hɔ no mu no bɛyɛ 40%. Nkokoaa a wɔwo wɔn wɔ awo mu duru a ɛfata, a wɔanwo wɔn ntɛm no, fi mu ba yiye sen nkokoaa a wɔwo wɔn a wɔn mu duru sua. Nhwehwɛmu bi daa no adi sɛ nkokoaa pii a wonyaa nkwa afe a edi kan no da so te ase bere a wɔadi mfe 18 no.

Ɛyɛ den sɛ wubehu akontaabu ahorow yi. Nanso kae sɛ aduruyɛ ho nyansahu renya nkɔso da biara da. Ayaresa foforo, mfiridwuma foforo reba. Enti ɛho hia sɛ yɛma anidaso kɔ so tra ase.

Mɛyɛ dɛn ahwɛ me ba?

Mmofra a wɔwo wɔn a wɔwɔ saa tebea yi betumi atra ase yiye bere a wɔde komayare ho oduruyɛfo ahwɛ wɔn bere tenten no. Wubetumi ahwɛ wo ba denam:

• Fa influensa (flu) aduru ne COVID-19 aduru ma wo ba afe biara.
• Fa wo ba kɔ komayare ho oduruyɛfo nkyɛn asram asia biara anaa pɛnkoro afe biara .
• Ma wo ba no aduru a wɔakyerɛw ama wo no bere ano .
• To wo ba no apɔw-mu-teɛteɛ a emu yɛ den ano hye , sɛnea wo duruyɛfo kamfo kyerɛ no.
• Sɛ wo ba no wɔ adesua mu nsɛnnennen a , boa no.

Nsɛm bɛn na ɛsɛ sɛ mibisa me duruyɛfo?

Sɛ wo ba no wɔ HLHS a, wubetumi abisa oduruyɛfo no nsɛm te sɛ:

• Asiane bɛn na ɛwɔ oprehyɛn mu?
• So nsɛnnennen bi wɔ hɔ a ɛsɛ sɛ wɔhwɛ yiye wɔ ho bere a wɔayɛ oprehyɛn awie no?
• Nnuru bɛn na me ba hia? So nsunsuanso bɔne bi wɔ saa nnuru no so?
• Ɔkwan bɛn so na tebea no (HLHS) bɛka me ba no asetra?
• Ɔhwɛ bɛn na ɛsɛ sɛ me ba no di akyi bere a wɔayɛ no oprehyɛn akyi?
• Sɛ mewo akokoaa foforo a, so asiane bi wɔ hɔ sɛ saa akokoaa no nso bɛwo (HLHS)?

Abofra a ɔwɔ HLHS a wobɛwo no betumi ayɛ osuahu a ɛma wote nka kɛse na ɛyɛ ankonam . Sɛ woredi wo ba komayare ho adwennwen ne nea wontumi nsi pi ho dwuma a, ɛho hia sɛ wohwehwɛ kuw bi a wɔboa anaa baabi foforo a wubenya nkate fam mmoa. Sɛ wokɔ so yɛ den wɔ w’adwene mu a, ɛbɛboa wo ma woadi wo ba no akwahosan mu nkɔso ne ɔhaw ahorow ho dwuma.

Nkrasɛm a Wɔde Kɔ Fie

(HLHS) yɛ komayare a emu yɛ den a wonya fi awo mu. Nanso, sɛ wohu mmofra ntɛm na wɔsa wɔn yare yiye a, wonya hokwan bɔ bra pa. Eyi hwehwɛ sɛ wɔyɛ oprehyɛn a emu yɛ den pii na wɔde bere tenten hwɛ aduruyɛ so. Nea ehia paa ne sɛ wubehu sɛ ɛnyɛ wo nkutoo na wowɔ hɔ. Nnuruyɛfo, ayarehwɛfo, ne akwahosan ho adwumayɛfo afoforo wɔ hɔ sɛ wɔbɛboa wo ne wo ba no. Ɛho hia paa nso sɛ wubenya mmoa afi awofo afoforo a wɔanya osuahu a ɛte saa ara hɔ. Mma akokoduru nnhwere. Afɔre a wode bɔ afɔre ma wo ba no som bo.