Bɔku tɛm yu kin gɛt strenj pen na yu bɔdi? Lɛ wi lan mɔ bɔt dis (Systemic Mastocytosis)!

Sɔntɛnde yu kin gɛt rɛd ɛn it spat na yu skin? Ɔ yu gɛt bɛlɛ we de at ɛn ed we jɔs de kam ɛn go? if dεn tin ya apin bכku tεm, i kin bi wan rare kכndyushכn we dεn kכl ``Systemic Mastocytosis'' we wi go tכk bכt tide. Nɔr wɔri, dis nɔr to sik wae de ambɔg bɔrku pipul. Bɔt i impɔtant fɔ no bɔt dis.

So, wetin na dis ``Systemic Mastocytosis''?

Fɔ tɔk am simpul wan, `(Systemic Mastocytosis)` na wan sik we nɔ kin apin so ɔltɛm we gɛt fɔ du wit wi blɔd. wetin de apin insay dis na we wan spεshal tכp sεl we dεn kכl `(Mast cells)` na wi bכdi de divεlכp abnכmal wan. Tink bɔt dis, dɛn mast sɛl ya tan lɛk smɔl sojaman dɛn na wi bɔdi. na dεn wan dεm we, as pat pan wi imyun sistεm, de no εn fכt fכrin invayda dεm (lεk jεm, alergen, εtk). we dis kayn fכrin invayda kam, dεn mast sεl dεm ya de rilis kεmikכl dεm lεk `(Histamine)`. Dis `(Histamine)` na in de mek yu gɛt dɛn alɛji simptom dɛm (lɛk fɔ rɛd, it, swel). nכmal wan, di mast sεl dεm de stכp fכ rilis dis `(Histamine)` we dεn dכn du di wok. כltu, insay di bכdi fכ pכsin we gεt `(Systemic Mastocytosis)`, dεn abnכmal mast sεl dεm ya de divεlכp pasmak εn de rilis `(Histamine)` kכntinyu. I tan lɛk se sɔmbɔdi lɛf wata tap ɛn fɔgɛt fɔ ɔf am. Dis na di rizin we mek wi kin kɔntinyu fɔ gɛt sayn dɛn we tan lɛk alɛji. I nɔ kin afɛkt wi skin nɔmɔ, bɔt i kin afɛkt wi insay ɔgan dɛm lɛk wi liva, splin, ɛn intestinal dɛm. dis kכndyushכn we dεn kכl ``Systemic Mastocytosis,'' dεn se i de afekt כnli 13 pan εvri 100,000 pipul dεm na di wכl. Dat min se i rili rare. Bɔt pipul dɛm wae gɛt dis sik kin gɛt bɔrku risk fɔ gɛt anafilaksis ( wan bad bad alɛji wae kin kam wit dɛn layf). na sכm tεm, ``Systemic Mastocytosis'' kin tכn bak to kεnsar. Dɔktɔ dɛn nɔ kin ebul fɔ mɛn dis sik ɔltogɛda. Bɔt sɔm tritmɛnt dɛn de wae kin ɛp yu fɔ kɔntrol yu sik ɛn liv nɔrmal layf.

Wetin na di men kayn `(Systemic Mastocytosis)`?

siks men tכp dεm de fכ ``Systemic Mastocytosis``. Dɛn kin afɛkt wi difrɛn we dɛn. In jɛnɛral, di mɔ abnɔmal mast sɛl dɛn we yu gɛt na yu bɔdi, na di mɔ yu go gɛt prɔblɛm dɛn. Lɛ wi luk wetin na dɛn kayn ya: 1. di kayn we we de gro sloslo (``Indolent systemic mastocytosis``):

• Dis na di kayn we we pipul dɛn kin gɛt mɔ. Lɛk tik we de gro sloslo, i kin tek sɔm ia fɔ mek di sayn dɛn we de sho se i gɛt di sik. di nכmba fכ di abnכmal mast sεl dεm de inkrεs as tεm de go. dis kin mek di simptom dεm na di skin, liva , splin , εn di gεstrointestinal sistεm (i.e., di intestinal dεm).

2. sכm mכr advans tכp (`Systemic smoldering mastocytosis`):

• insay dis kayn, abnכmal mast sεl dεm de kכmכt bכku bכku wan na di liva εn splin. As tɛm de go, dis kin mek di liva ɔ di splin swel. Tink bɔt am lɛk wetin kin apin we smɔl os ful-ɔp.

3. Tayp we de kam wit כda bכdi sik dεm (`Systemic mastocytosis with associated hematologic neoplasm`):

• Pipul wae gɛt dis kayn sik kin gɛt ɔda kayn sik wae gɛt fɔ du wit blɔd, lɛk myeloproliferative neoplasms ɛn myelodysplastic syndrome . dis kayn de afekt bכt wan pan fayv pipul dεm we gεt Systemic Mastocytosis.

4. siriכs tכp (`Agresiv sistεmik mastosaytosis`):

• Dis kin siriɔs smɔl bikɔs i kin afɛkt di bon mɛro (we dɛn kin mek blɔd sɛl) ɛn di bon dɛn. di abnכmal mast sεl dεm kin gro insay di bon dεm, we kin mek dεn wik εn mek dεn brok izi wan.

5. Di kayn we de tɔn to lukimiya (`Mast cell leukemia`):

• Na sכm tεm, Sεstεmik Mastosaytosis kin divεlכp to wan kכndyushכn we dεn kכl Mast Sεl Lukimiya . Dis na wan kayn sik we dɛn kɔl Acute Myeloid Leukemia (AML) .

6. Tayp fכ di tכmכro (`Mast sel sarkoma`):

• dis na we di tכmכro dεm (tכmכro) we de fכm frכm abnכmal mast sεl dεm de pwεl difrεn tisu dεm na di bכdi. wan rili sכm nכmba fכ pipul dεm we gεt ``Systemic Mastocytosis`` de divεlכp dis kכndyushכn we dεn kכl ``Mast cell sarcoma``.

Wetin na di sayn dɛm fɔ dis sik (Systemic Mastocytosis)?

Di sayn dɛm kin difrɛn frɔm wan pɔrsin to ɔda pɔrsin. i dipכnt pan usay insay di bכdi di abnכmal mast sεl dεm de mכst kכnsantreshכn. Fɔ ɛgzampul, if yu gɛt bɔku mast sɛl dɛn na yu bɛlɛ, yu kin gɛt ɔlsa ɛn yu bɛlɛ kin pen. Dɔn bak, if di mast sɛl dɛn gɛda na yu bon mɛro, i kin afɛkt di we aw blɔd sɛl dɛn de mek. Na sɔm kɔmɔn sayn dɛn ya:

• Anemia ( we pɔsin nɔ gɛt blɔd ) .
• Bɔn pen
• Blɔd we de kɔmɔt pasmak (e.g., bɔku blɔd we de kɔmɔt na smɔl wund) .
• Fɔ fil taya ɔltɛm (` Fatigue` ) .
• Skin we dɔn flash
• Di at we de blo
• Hiv (itchy, raised bumps pan di skin) .
• Skin we de it
• Di mud chenj ɔr pwɛl hatdipreshɔn `) .
• Dak, it we de it na di skin (`Urticaria pigmentosa`) .

Sɔmtɛm, pipul dɛm wae gɛt Systemic Mastocytosis kin gɛt sɔm pan dɛn sik ya wan tɛm. dכkta dεn kכl dis "mastocytosis attack" כ "flare."

Impɔtant: Nɔto ɔlman we gɛt dɛn sik ya gɛt `(Systemic Mastocytosis).` Bɔt if yu gɛt sɔm pan dɛn simptom ya wan tɛm, ɛn dɛn de kɔntinyu, yu fɔ rili go to dɔktɔ fɔ advays.

Wetin mek dis `(Systemic Mastocytosis)` de apin?

rεsכch dεn fכnshכn se bכt 80% pan di pipul dεm we gεt `(Systemic Mastocytosis)` gεt wan `KIT jin vεryכnt` . dis `KIT` jin de εp sכm kayn sεl dεm na wi bכdi, spεshal wan bכdi sεl dεm εn mast sεl dεm, fכ divεlכp. dis jin vεryכnt de apin afta wi bכn na di bεlε. Dis min se i nɔ gɛt frɔm dɛn gret gret granpa dɛn.

Wetin na di tin dɛm wae kin mek di sik wɔs ɔr kin mek pɔrsin fil bad (`Triggers`)?

bכku tin kin trigεr di simptom dεm fכ ``Systemic Mastocytosis.`` Nɔto ɔlman kin afɛkt di sem tin. Bɔt sɔm tin dɛn de we bɔku pipul dɛn kin du:

• Rum
• Fɔd dɛn we gɛt spays
• Sɔm mɛrɛsin dɛn: Fɔ ɛgzampul , NSAID ( painkillers), mɔsul rilaks, ɛn anestesia.
• Ɛksesaiz ɛn fyzikal aktiviti ( fɔ sɔm nɔmɔ) .
• Insɛkt dɛn we de bit (espɛshali ant, wasp, ɛn bi) .
• Stret pan bɔdi ɔ filin
• Rub ɔ frikshɔn pan yu skin
• Wan tɛm we di tɛmpracha chenj (e.g., we yu jomp wantɛm wantɛm insay kol wata) .

If yu gɛt dis sik, i rili impɔtant fɔ aks yu dɔktɔ wetin yu wik ɛn wetin yu fɔ avɔyd.

Aw yu kin no dis sik kɔrɛkt wan? (Diagnosis) .

We yu go si dɔktɔ, di fɔs tin we dɛn go du na fɔ du yu bɔdi ɛgzam. Dɛn go aks bɔt yu sayn dɛm ɛn ɛni sik wae yu bin gɛt bifo. Dɔn, dɛn kin du wan ɔ mɔ pan dɛn tɛst ya fɔ no if yu gɛt Systemic Mastocytosis:

• Blɔd tɛst: Dis de chɛk ɔmɔs triptaz de na yu blɔd. Tryptase na spεshal εnzym we di mast sεl dεm de rilis we dεn de rεspכnd to fכrin tin dεm.
• Bon skan: Chɛk fɔ si if yu bon dɔn pwɛl.
• Bone marrow biopsy: Dis na fɔ tek smɔl sampul pan yu bon marrow ɛn tɛst am fɔ si if ɛni abnɔmal mast sɛl de insay, ɛn ɔmɔs.
• jεnεtik tεst dεm: Chεk fכ mכtεshכn na di `KIT` jin we wi bin dכn mεnshכn bifo.

Aw dɛn kin trit am?

Dɔktɔ dɛn kin trit Systemic Mastocytosis bay we dɛn kin kɔntrol di simptom ɛn kɔmplikeshɔn dɛn. fכ egzampl, if yu gεt hכy bεlε asid, dεn kin gi yu H2 blכk dεm (wan kayn antasid). If anemia na bikɔs ɔf di sik, dɛn go trit am. Na sɔm pan di men tritmɛnt dɛn ya:

• Antihistamines: Ridyus di skin simptom dɛm lɛk it ɛn rɛd.
• Kɔtikosterɔyd: I de ridyus di swel ɛn inflamɛns na di bɔdi.
• Bisphosphonates: Dɛn kin ɛp fɔ mek di bon dɛn strɔng if dɛn wik.
• Tεrapi we dεn de tכk: Dis involv fכ tכk to wan spεsifi k protin we de insay di abnכmal mast sεl dεm.
• Kimotɛrapi: Dɛn kin yuz dis nɔmɔ if di Systemic Mastocytosis dɔn tɔn to kansa.
• Splenectomy: If di spleen dɔn swel bad bad wan, dɛn kin du ɔpreshɔn fɔ pul am.
• Bɔn mɛrɔ transplant: Dɛn kin du dis fɔ pipul dɛn we gɛt siriɔs sik, ɔ di wan dɛn we de na di las stej.

Mɛmba se: If yu gɛt Systemic Mastocytosis, yu fɔ kɛr wan EpiPen ɔltɛm. EpiPen na mɛrɛsin we yu kin yuz wantɛm wantɛm if yu gɛt siriɔs alɛji, lɛk di Anaphylaxis we wi bin dɔn tɔk bɔt.

yu nכ kin ebul fכ sכpכt dis ``Systemic Mastocytosis''?

as wi bin dכn tכk bכt, ``Systemic Mastocytosis`` de kכz fכ wan jεnεtik mכtεshכn. So, i nɔ pɔsibul fɔ mek i nɔ de divɛlɔp. Bɔt yu kin kɔntrol di sik bay we yu avɔyd ``trig`` we de mek di sik bɔku.

Us kayn fiuja pɔsin we gɛt `(Systemic Mastocytosis)` gɛt?

dis rili dipכnt pan us kayn `(Systemic Mastocytosis)` yu gεt. Pipul dεm we gεt di tכp we de gro sloslo (`Indolent systemic mastocytosis`) kin כlwayz kכntro di sik fayn fayn wan wit tritmεnt. Dɛn kin liv nɔmal layf span. Bɔt pipul dɛm wae gɛt di ɔda kayn sik kin gɛt shɔt layf smɔl. כltu, `(Systemic Mastocytosis)` kin kכmplit kכl wit wan tritmεnt we dεn kכl ``Bכn mכro transplant``. Bɔt, dɔktɔ dɛn kin jɔs gi da tritmɛnt de to pipul dɛn we de na di say we di sik rili bad.

Aw a kin tek kia ɔf misɛf we a de liv wit `(Systemic Mastocytosis)`?

Fɔ no wetin de trig yu simptom dɛm ɛn fɔ avɔyd dɛm na di impɔtant tin wae yu kin du wae yu de liv wit Systemic Mastocytosis. If yu gɛt dis sik, no bɔt dɛn tin ya:

• Ɔltɛm yu fɔ kɛr wan EpiPen wit yu. Trit wan siriɔs alɛji kwik kwik wan.
• No gud gud wan wetin na di tin dɛn we de mek yu trit yu ɛn avɔyd dɛn as yu ebul.
• Manej di strɛs. Praktis fɔ tink gud wan ɔ ɔda we dɛn fɔ tink gud wan.
• Wear wan mɛdikal alert breslɛt we gɛt di list fɔ di mɛrɛsin dɛn we yu nɔ go ebul fɔ tek. Dis na impɔtant tin bikɔs i kin ɛp dɔktɔ dɛn fɔ no yu sik if yu nɔ ebul fɔ tɔk na imejensi.

Ustɛm a fɔ go to di dɔktɔ?

If yu gɛt Systemic Mastocytosis, yu go nid fɔ go to yu dɔktɔ fɔ mek dɛn chɛk yu ɔltɛm. Dɔn bak, mek shɔ se yu tɛl yu dɔktɔ if yu gɛt ɛni nyu sik, ɔ if i tan lɛk se ɛni wan pan yu sik dɛn de wɔs.

Us kwɛstyɔn dɛn a fɔ aks di dɔktɔ?

Bikɔs Systemic Mastocytosis na sik wae nɔr kin bɔrku, yu kin gɛt bɔrku kwɛstyɔn. Na sɔm kwɛstyɔn dɛn we yu fɔ aks yu dɔktɔ:

• Us kayn ``Systemic Mastocytosis'' a gɛt?
• Us ɔda sayn dɛn we go sho se mi sik de wɔs?
• Us tritmɛnt dɛn de fɔ mi tayp?
• Yu tink se di tritmɛnt go pul mi sik dɛn kpatakpata?
• Ɛni klinik trial de we a kin tek pat pan?

Fɔ no se yu gɛt Systemic Mastocytosis na tin we kin chenj yu layf. Yu go gɛt fɔ go to yu dɔktɔ ɔltɛm, du blɔd tɛst, ɛn lan fɔ no ɛn avɔyd di tin dɛn we kin mek yu gɛt dis sik. Fɔ liv wit dis kɔndishɔn na big ajɔstmɛnt. So, kɔntinyu fɔ gɛt gud padi biznɛs wit yu dɔktɔ. Dɛn de de fɔ ɛp yu. Dɛn go gi yu infɔmeshɔn ɛn sɔpɔt grup dɛn we go ɛp yu. Dɛn tin ya go gi yu pawa fɔ disayd bɔt yu wɛlbɔdi biznɛs.

Fɔ dɔn, tin dɛn fɔ mɛmba (Take-Home Message) .

Okay, so wi don tok plenti boht `(Systemic Mastocytosis)`, no so? Mɛmba se pan ɔl we dis na sik we nɔ kin bɔku, i rili impɔtant fɔ no bɔt am.

De tin wae impɔtant pas ɔl na fɔ go to dɔktɔ if yu gɛt dɛn kayn sik ya. If dɛn no di sik kwik kwik wan, dɛn kin bigin fɔ trit am ɛn kɔntrol di sayn dɛm.

Nɔto yu wan de. Yu dɔktɔ dɛn, yu fambul dɛn, ɛn yu padi dɛn de fɔ ɛp yu. If yu no di rayt tin ɛn fala yu dɔktɔ in advays, yu go ebul fɔ liv fayn wit dis sik. Stay kol ɛn tink gud wan!