Yɛn nyinaa ka srade ho asɛm bere nyinaa, na yɛhaw yɛn ho wɔ ho, ɛnte saa? Nanso susuw ho hwɛ, so woate yare bi a wonya fi awo mu a mogya mu srade dodow kɔ soro wɔ ɔkwan a ɛnteɛ so fi mmofraase, ebia wɔ mmofraase mpo ho asɛm pɛn? Ɛnnɛ yɛbɛka yareɛ a ɛte saa a ɛntaa nsi nanso ɛho hia paa ho asɛm. Wɔfrɛ eyi sɛ Homozygous Familial Hypercholesterolemia (HoFH). Ɛwom sɛ edin no yɛ den kakra de, nanso momma yɛnte ase kɛkɛ.
Dɛn ne Homozygous Family Hypercholesterolemia?
Sɛ yɛbɛka no tiawa a, eyi yɛ awosu mu yare a ɛma ɛyɛ den ma yɛn nipadua sɛ ebeyi srade "bɔne" a ɛne LDL (Low-Density Lipoprotein) srade afi yɛn mogya mu. Yɛn nyinaa nim sɛ srade yɛ ade a ɛte sɛ waxy a yɛn nkwammoaa hia. LDL srade adwuma ne sɛ ɛde saa srade yi bɛfa mogya no mu akɔ nipadua no nyinaa mu.
Nanso sɛ LDL dodow kɔ soro dodo a, srade a ɛboro so yi fi ase boaboa ano wɔ mogya ntini a ɛde mogya kɔ yɛn koma, ntini ahorow no mu no mu. Bere kɔ so no, saa nneɛma yi betumi asiw ntini ahorow no ano, atwa mogya ne mframa pa a ɛkɔ koma no mu, na ama asiane a ɛwɔ hɔ sɛ obi benya komayare no ayɛ kɛse .
Saa yare yi fi awo mu. Eyi kyerɛ sɛ wubetumi anya srade a ɛdɔɔso fi wo mmofraase. Eyi yɛ tebea a emu yɛ den a wontumi nsa na ɛsɛ sɛ wodi ho dwuma wɔ nkwa nna nyinaa mu.
Sɛ wɔansa mmarima a wɔwɔ saa tebea yi yare a, wobetumi anya komayare bere a wɔadi mfe 40 ne mmea a wɔadi mfe 50. Enti, ɛho hia yiye sɛ wuhu eyi na wohwehwɛ ayaresa a ɛfata.
Dɛn nti na ɛte saa?
Yɛfrɛ eyi awosu mu yare. Sɛ yɛbɛka no katee a, sɛ wunya awosu mu abɔde bi a ɛnyɛ adwuma yiye no bi abien nkutoo a, biako fi wo maame hɔ na biako fi wo papa hɔ nkutoo a .
Mpɛn pii no, yɛn mmerɛbo yi LDL srade a ɛboro so fi mogya no mu. Mmɛn no wɔ ‘LDL agyefo’ soronko ma eyi. Eyinom te sɛ magnet a ɛkyere srade a ɛwɔ mogya mu na eyi fi mogya no mu. Nanso wɔ obi a ɔwɔ saa yare yi mu no, esiane awosu mu abɔde a ɛtɔ sin a wonya fi awo mu nti, saa ntini ahorow yi ntumi nyɛ adwuma yiye. Nea afi mu aba ne sɛ srade dodow kɔ soro a wontumi nni so.
Ebia woate ‘Heterozygous’ Abusua mu Cholesterolemia nso ho asɛm. Eyi ne bere a wonya awosu mu abɔde a asɛe no fi ɔwofo biako pɛ hɔ. Saa tebea yi mu nyɛ den te sɛ ‘Homozygous’.
Dɛn ne yare yi ho sɛnkyerɛnne?
Nsɛnkyerɛnne atitiriw pii wɔ hɔ a wobetumi de ahu yare yi. Momma yɛnhwɛ wɔn wɔ pon bi so.
| Su a ɛwɔ hɔ | Nkyerɛmu |
|---|---|
| Cholesterol dodow a ɛkɔ soro yiye | Cholesterol dodow nyinaa betumi ayɛ 600 mg/dL anaa nea ɛboro saa (apɔwmuden dodow nnu 200). |
| Nneɛma a ɛwɔ honam ani (Xanthomas) . | Nkuruwankuruwa kɔkɔɔ a ɛte sɛ waxy a ɛwɔ honam ani wɔ nsateaa, nkotodwe, ne akyi. Eyinom yɛ srade a ɛwɔ nipadua no mu. |
| Nneɛma a ɛwɔ aniwa no anim (Xanthelasmas) . | Srade kɔkɔɔ a ɛwɔ aniwa no so. |
| Aniwa mu nsakrae (Arcus cornealis) . | Nkatabo a ɛyɛ fitaa anaa fitaa a atwa aniwa tuntum no ho ahyia. |
| Koma mu sɛnkyerɛnne ahorow | Nsɛnkyerɛnne te sɛ koko mu yaw (angina), ahome a ɛyɛ tiaa, ne koma a ɛbɔ ntɛmntɛm betumi aba wɔ ne mmofraase. |
Ɔkwan bɛn so na wobetumi ahu yare no?
Sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, ɛho hia sɛ wokɔ wo duruyɛfo nkyɛn. Oduruyɛfo no bedi kan ayɛ nipadua mu nhwehwɛmu na wabisa wo nsɛm kakraa bi.
- So woahyɛ biribi te sɛ nsensanee kɔkɔɔ wɔ wo honam ani nsow?
- So wowɔ koko mu yaw?
- So wote nka sɛ wo home yɛ wo yaw?
- So w’awofo nso wɔ srade a ɛdɔɔso?
Afei, wo duruyɛfo no bɛyɛ mogya mu nhwehwɛmu de ahwɛ sɛnea wo srade a ɛwɔ wo mu no te. Ebia wɔbɛyɛ awosu mu nhwehwɛmu nso de ahwɛ sɛ wowɔ awosu mu abɔde a ɛnteɛ a ɛde yare no ba anaa. Ebia wo duruyɛfo nso bɛsɔ w’abusuafo a wɔbɛn wo no ahwɛ.
Akwan a wɔfa so sa yare ne sɛnea wɔde di dwuma
Botae titiriw a ɛwɔ ayaresa mu ne sɛ ɛbɛtew wo LDL srade dodow so na ɛbɛtew asiane a ɛwɔ hɔ sɛ wubenya komayare no so.Wɔbɛtaa de wo akɔ oduruyɛfo a ne ho akokwaw wɔ srade nyarewa te sɛ eyi mu (Lipidologist).
Nsakrae a ɛba asetra kwan ne aduan a wodi mu
Ade a edi kan a ɛsɛ sɛ woyɛ ne sɛ wubegye aduan a ɛma koma nya ahoɔden a srade a ɛdɔɔso, srade, ne asikre kakraa bi na ɛwom. Ɛho hia nso sɛ wugyae sigaretnom, woto nsa ano hye, na woteɛteɛ w’apɔw mu da biara da.
Nnuru a wɔde yɛ nnuru
Esiane sɛ wo srade a ɛwɔ wo mu no dɔɔso wɔ ɔkwan a ɛnteɛ so nti, ebia wobefi ase anom statin dodow a ɛkɔ soro , a ɛyɛ adwuma denam mmerɛbo no a esiw srade a ɛba no ano so.
Nea ɛka ɛno ho no, wobetumi de nnuru afoforo a ɛtew srade dodow a wɔtwe fi aduan mu so (e.g. Ezetimibe , PCSK9 inhibitors , Lomitapide ) aka ho.
Ayaresa pɔtee bi
Sɛ wontumi mfa nnuru nkutoo nsiw srade a ɛwɔ mogya mu ano a, ɛho behia sɛ wosusuw akwan foforo a wɔfa so sa yare ho.
- Apheresis: Eyi yɛ adeyɛ a ɛte sɛ dialysis a wɔyɛ ma ayarefo a wɔwɔ asaabo no. Wɔde wo bata afiri bi ho wɔ ayaresabea, woyi wo mogya fi wo nipadua mu, woyi LDL srade no fi mu, na wɔsan de mogya a wɔahohoro no kɔ wo nipadua mu. Eyi yɛ ayaresa a egye nnɔnhwerew pii na ɛsɛ sɛ wɔyɛ no daa.
- Berɛbo a Wɔde Ma: Sɛ atifi hɔ ayaresa no mu biara antumi anyɛ yiye a, ebia mmerɛbo a wɔde bɛma obi foforo ne ɔkwan a etwa to. Mmerɛbo foforo a ɛwɔ apɔwmuden no wɔ LDL agyefo, a ebetumi aboa ma wɔadi srade a ɛwɔ mogya mu no so. Eyi yɛ oprehyɛn a anibere wom yiye, na sɛ obi ho bɛtɔ no a, ebetumi agye asram 6 kosi afe biako.
Sɛ wɔreyɛ ayaresa a anibere wom saa a, ɛho hia paa sɛ wɔhwehwɛ nkate fam mmoa fi abusuafo ne nnamfo hɔ. Wo ne wo duruyɛfo nka eyi ho asɛm pefee.
Nkrasɛm a Wɔde Kɔ Fie
- Homozygous Familial Hypercholesterolemia yɛ awosu mu tebea a emu yɛ den, nanso ɛntaa nsi, a ɛma srade dɔɔso yiye.
- Nea enti a ɛte saa ne sɛ wonya awosu mu nkwaadɔm abien a asɛe fi wɔn maame ne wɔn papa nyinaa hɔ.
- Sɛ wohu na wofi ayaresa ase wɔ mmofraase so a, wobetumi atew asiane a ɛwɔ hɔ sɛ obi benya komayare wɔ mmofraase no so.
- Ayaresa no yɛ nkwa nna nyinaa na ebetumi ayɛ nnuru, aduan, apɔw-mu-teɛteɛ, ne ebia ayaresa titiriw (apheresis).
- Sɛ obi a ɔwɔ w’abusua mu anya komayare anaa srade a ɛdɔɔso wɔ ne mmofraase a, nyansa wom sɛ wobɛma wɔahwɛ wo srade mu sɛnea wo duruyɛfo de afotu ama wo no.
- Ɛnyɛ wo nkutoo na wowɔ saa akwantu yi mu. Wo ne wo duruyɛfo, abusua, ne mmoa akuw nyɛ adwuma na ama woadi tebea yi ho dwuma yiye.
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