Wetin na Vɔn Hipɛl-Lindau Sindrom (VHL)? Lɛ wi tɔk bɔt am simpul wan.

Yu dɔn ɛva yɛri bɔt Von Hippel-Lindau Syndrome? I go mɔs bi se yu nɔ dɔn du am. I kin tan lɛk se i kɔmplikt smɔl, bɔt na tin we nɔ kin apin to pɔsin we gɛt jɛnɛtiks. Fɔ tɔk am simpul wan, wan muteshon na wan jin we dɛn kɔl VHL kin mek tumor ɛn sist dɛn we ful-ɔp wit wata fɔm na difrɛn pat dɛn na di bɔdi. Nɔ fred we yu yɛri dis. Lɛ wi tɔk bɔt am klia wan ɛn simpul wan.

Wetin rili na VHL sindrom?

VHL na wan kכndyushכn we de kכz fכ chenj na wi jin dεm. Dis kin mek difrɛn pat dɛn na yu bɔdi, fɔ ɛgzampul, .

• Yay
• Bren
• Spinal kɔd
• Insay yes
• Di adrenal gland dɛn we de na di bɔdi
• Pankrias we de na di bɔdi
• Kidni dɛn
• Ɔgan dɛn we de mek uman bɔn pikin

Tumɔs kin fɔm na ples dɛn lɛk...

Di bɛst pat na dat bɔku pan dɛn tin ya we de gro nɔ kin fayn . Dat min se dɛn nɔ gɛt kansa. Bɔt pɔsin we gɛt dis sik kin gɛt smɔl kansa na in kidni ɛn pankrias. Na dat mek i impɔtant fɔ no bɔt dis.

Wetin mek dis VHL sindrom kin apin?

nכmal wan, di VHL jin na wi bכdi de kכntro di tכmכro dεm we de gro bay we i de mek di sεl dεm nכ divayd we wi nכ de kכntro. I tan lɛk gad na wi bɔdi. Bɔt pan pɔsin we gɛt VHL sindrom, dis jin kin chenj ɔ chenj. Dɔn da gad de in wok nɔ de wok fayn. dis kin mek di sεl dεm we nכ nכmal de gro we dεn nכ de kכntro εn bigin fכ fכm tכmכro.

Tu men we dɛn de we dis tin kin apin:

1. Inhɛritɛshɔn frɔm mama ɛn papa: Na lɛk 8 pan 10 pipul dɛm we gɛt VHL kin gɛt am frɔm dɛn mama ɔ papa. i de inhεrit insay wan כtosom dכminant patεn, we min se if wan mama εn papa gεt di kכndyushכn, εvri wan pan dεn pikin dεm gεt 50% chans fכ inhεrit am.

2. Random occurrence: Na lɛk 2 pan 10 kes dɛm, dis nɔto sɔntin we di mama ɛn papa kin gɛt. dis kכndishכn kin apin bikoz fכ wan random chenj na di jin dεm ( spontaneous mutation) di fכs stej dεm fכ divεlכpmεnt as εmbriyo.

Wetin na di sayn dɛm fɔ VHL?

Di sayn dɛm fɔ VHL kin difrɛn bad bad wan frɔm wan pɔsin to ɔda pɔsin. Dis na bikɔs di sayn dɛm de dipen pan usay di tumbu de na yu bɔdi. Sɔm pipul kin go fɔ lɔng lɔng tɛm ɛn nɔr kin gɛt ɛni sayn.

Pan ɔl we bɔku tɛm di sik kin bigin arawnd 26 ia, sɔm pipul dɛn nɔ kin gɛt big big sik te dɛn rich 65. Di ej we di sik kin bigin kin difrɛn bak fɔ di kayn tumbu. Fɔ ɛgzampul:

• di ay tכmכro dεm (rεtina hεmangioblastomas): 12-25 ia ol
• di tכmכro dεm na di bכdi vεsul dεm na di bren כ spεnal kכd (hεmangioblastomas): 18-35 ia ol
• di kidni tכmכro כ kεnsar (rεnal sεl): bitwin 25-50 ia ol
• di insay ia tכmכro dεm (εndolimfatik sak tכmכro dεm): bitwin 24-35 ia ol

Aw dɛn kin no se pɔsin gɛt VHL?

If pɔsin na yu famili gɛt dis sik, ɔ if yu sik de mek yu dɔktɔ tink se yu gɛt VHL, i go rifer yu fɔ mek dɛn du yu jenɛtik tɛst . Dɛn kin du dis wit blɔd sɛmpul. dis na di onli we fכ no fכ sכri if yu gεt chenj na di VHL jin.

Di kɔmɔn tin dɛn we dɔktɔ kin sɔprayz se i gɛt VHL na:

• Fɔ fɛn ay tumor (ay hemangioblastoma) we yu yɔŋ.
• Kidni kansa bifo i ol 40 ia.
• fכ gεt plεnti tכmכro dεm (hεmangioblastomas) na di bren כ di spεnal kכd.
• Fɔ gɛt bɔku bɔku tumbu ɔ sist na di kidni ɔ pankrias.

If dɛn no se yu gɛt VHL, di nɛks tin we impɔtant pas ɔl na fɔ wach yu aktif wan.

Wetin na Aktiv Sɔvɛlayshɔn?

Dis kin tan lɛk big tin, bɔt fɔ tɔk am simpul wan, i min se ivin if yu nɔ gɛt di sik, yu mɛdikal tim go de wach yu ɔltɛm. Dis min se if nyu tumbu kam ɔ wan ol wan big, dɛn kin no am kwik kwik wan ɛn dɛn kin bigin di tritmɛnt we dɛn nid. Dis na di at fɔ VHL manejmɛnt.

Dɛn tɛst ya kin difrɛn difrɛn wan bay yu ej.

• Frɔm we yu smɔl (1-4 ia): Mek dɔktɔ we de mɛn yu yay chɛk yu yay ɛvri 6 to 12 mɔnt. Afta 2 ia, mek dɛn chɛk yu blɔd prɛshɔn ɛvri ia.
• Pikin (5-10 ia): Dɛn kin kɔntinyu fɔ du di ɛgzam fɔ di yay. כlso, dεn kin du urine כ bכdi tεst (mεtanεfrin) fכ chεk fכ adrenal gland tכmכro dεm (pheochromocytomas).
• Adolescence (frɔm 11 ia): Ɛvri sɔm ia, dɛn kin du MRI skan fɔ luk di bren ɛn di spɛnal kɔd. Dɛn kin chɛk bak aw pɔsin kin yɛri.
• We pɔsin big (frɔm 15 ia): Dɛn kin du MRI skan fɔ di bɛlɛ ɛvri tu ia fɔ chɛk di kidni, pankrias, ɛn adrenal gland dɛn.

Yu dɔktɔ go mek dis schedule fɔ fit yu.

Wetin na di tritmɛnt dɛm fɔ VHL?

di tritmεnt fכ VHL dipכnt pan di kayn, saiz, εn di say we di tכmכro de. If di tumbu nɔ gɛt kansa, i smɔl, ɛn i nɔ fayn, fɔ wach di bɔdi kin du fɔ yu if yu nɔ gɛt ɛni tritmɛnt.

Bɔku men we dɛn de fɔ trit pɔsin:

1. Mεdikeshכn: Di dכg we dεn jכs introdyus belzutifan (Welireg) dεn bin rili saksesful fכ shrink εn stכp di spred fכ wan nכmba כf VHL-rεlatεd tכmכro dεm (kidni kεnsar, hεmangioblastomas).

2. Ɔpreshɔn: Di tritmɛnt we dɛn kin yuz mɔ fɔ VHL na ɔpreshɔn. Dɛn kin ɔpreshɔn di sist dɛn we de mek pɔsin gɛt di sik, we de gro, ɔ we de sho sayn dɛn fɔ gɛt kansa.

3. Radiation therapy: Na di yus fɔ ay-ɛnaji rayt fɔ pwɛl di tumbu dɛn. Dɛn kin yuz dis mɔ fɔ sɔm tumbu dɛn we kin apin na di bren ɛn yes.

4. Ɔda tritmɛnt dɛn: Mɔdan tritmɛnt dɛn de fɔ kidni kansa lɛk Ablation (we de pwɛl di tumbu we yu de yuz ɔt pasmak ɔ kol pasmak) ɛn Immunotherapy .

Yu mɛdikal tim go disayd us tritmɛnt go fayn fɔ yu.

Liv wit VHL ɛn mental wɛlbɔdi

Na nɔmal tin fɔ fil frayd, wɔri, ɛn kɔnfyus we yu lan se yu gɛt wan sik we nɔ kin apin lɛk VHL. Di strɛs ("scanxiety") kin pasmak we yu de rɛdi fɔ skan ɛn wet fɔ di rizɔlt.

Bɔrku tin de wae yu kin du fɔ liv wɛl ɛn gladi layf wit dis sik:

• It fayn: It fayn it wit bɔku vɛjitebul, frut, mit we nɔ gɛt bɔdi, ɛn prɔtin lɛk fish. Nɔ smok kpatakpata.
• Ɛksesaiz: Simpul ɛksesaiz lɛk fɔ waka ɛvride kin mek yu nɔ strɛs ɛn mek yu bɔdi gɛt wɛlbɔdi.
• Mental rilaks: Du tin dɛm lɛk yoga, meditashɔn, ɛn ɔda tin dɛm Mek tɛm fɔ tin dɛm wae de briŋ yu pis na yu maynd (lɛk fɔ rid gud buk, lisin to siŋ).
• Aks fɔ ɛp: Tɔk to yu fambul ɛn yu tayt padi dɛn bɔt dis. Tɔk bɔt aw yu de fil. If yu nid ɛp (fɔ go to dɔktɔ, ɛp fɔ du homwok), nɔ fred fɔ tɔk dat.
• Trɔst yu mɛdikal tim: Aks yu dɔktɔ ɛni kwɛstyɔn ɔ tin we de mɔna yu. I kin fayn bak fɔ kip wan jɔnal fɔ yu sik dɛn ɛn di tɛst rizɔlt.