Bone Marrow Failure: Lɛ wi tɔk bɔt dis ditayli!

di bon mכro na di men blכd we de mek yu bכdi. I tan lɛk faktri. Dis faktri de mek tin dɛn lɛk rɛd blɔd sɛl, wayt blɔd sɛl, ɛn pletlɛt we de ɛp wi bɔdi fɔ klɔt. Imajin wetin go apin if dis faktri stɔp fɔ wok wantɛm wantɛm, ɔ if i nɔ wok fayn? Na dat na bon marrow failure. Lɛ wi tɔk bɔt dis smɔl mɔ .

Wetin na Bone Marrow Failure?

Fɔ tɔk am simpul wan, bon mɛro we nɔ de wok fayn na we yu bon mɛro nɔ ebul fɔ mek inof wɛlbɔdi rɛd blɔd sɛl, wayt blɔd sɛl, ɔ pletlɛt fɔ di bɔdi. Ɛni wan pan dɛn kayn sɛl ya gɛt wan patikyula wok we wi de du na wi bɔdi.

• Rɛd blɔd sɛl dɛn na di wan dɛn we de kɛr ɔksijɛn ɔlsay na di bɔdi. If dɛn smɔl, yu kin taya ɛn yu kin gɛt blɛsin.
• Wait blɔd sɛl dɛn tan lɛk di ami we de na wi bɔdi. Dɛn kin fɛt infɛkshɔn. If dɛn smɔl, di chans fɔ mek yu sik kin bɔku.
• Plɛtlɛt dɛn kin ɛp fɔ mek blɔd klɔt, we min se dɛn kin stɔp fɔ blɔd we yu wund. If yu pletlɛt kɔnt go dɔŋ, ivin smɔl wund kin mek yu blɔd bɔku.

Bɔn mכro fεil kin bi wan kכmplikεshכn fכ כda mεdikal kכndyushכn. Bɔt sɔntɛnde, i kin apin we nɔ gɛt ɛni rizin we mek i apin. Dɔktɔ dɛn kin ebul fɔ kɔntrol di sik dɛn wit mɛrɛsin ɛn difrɛn tritmɛnt dɛn. Bɔt di wangren tritmɛnt fɔ dis sik fɔ lɔng tɛm na fɔ transplant allogeneic stem cell .

Wae na de men kayn dis sik?

Tu men kayn tin dɛn de we kin mek yu bɔdi nɔ wok fayn:

1. Acquired: Masta sabi pipul dεm nכ no εksεkεkt wetin de mek dis kכndyushכn. Bɔt risach dɔn sho se sɔm sik dɛn, sɔm kayn kemikal dɛn, ɔ yuz sɔm mɛrɛsin dɛn kin mek i kam. Dis kayn kin de smɔl smɔl as tɛm de go.

2. Inherited: Dis kin apin if yu inherit genetic changes (mutations) frɔm wan ɔ ɔl tu yu mama ɛn papa. Dɔktɔ dɛn kɔl dis Bone Marrow Failure Syndrome .

Aw dis sik kin kɔmɔn?

Dis na rili kɔmɔn sik. Fɔ ɛgzampul, pan ɔl di milyɔn pikin dɛn we dɛn kin bɔn na Amɛrika ɛvri ia, lɛk 65 pan dɛn kin gɛt wan sik we dɛn kin gɛt frɔm dɛn mama ɛn papa. Wan ɔda ɛgzampul na wan sik we pɔsin kin gɛt frɔm in mama ɛn papa we dɛn kɔl ``Fanconi Anemia.'' I kin afɛkt lɛk wan pan fayv pan ɛvri milyɔn. So yu kin si, dis na smɔl tin we nɔ kin apin.

Wetin kin bi di sayn dɛm?

Di sayn dɛm kin difrɛn difrɛn wan bay wetin mek di bon mɛro nɔ wok fayn. Fɔ ɛgzampul, pɔrsin wae gɛt bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔdi kin bigin fɔ sho di sik as i ol tu ia. Bɔt, risach dɔn sho se pɔrsin wae gɛt di kayn sik wae dɛn dɔn gɛt kin bigin fɔ sho di sik as ali as bitwin 20 ɛn 25 ia, ɔr as let as 65. Ilɛk ustɛm i bigin, di sayn dɛm wae kin kam mɔs na:

• Pen na di bon dɛn
• blɔd we de kɔmɔt pasmak (e.g. blɔd we de kɔmɔt na yu nos, blɔd we de kɔmɔt na yu gɔm, we yu de blɔd bɔku tɛm we yu de gɛt mɔnt) .
• Taya pasmak ɛn taya pasmak
• Fiva we kin kam ɔltɛm
• Baktri infekshɔn dɛn we kin kam bɔku tɛm
• Ed de at
• I nɔ izi fɔ blo (Dyspnea) .
• Skin kala de kam paler pas aw i kin bi
• Smɔl smɔl rɛd spat dɛn ɔnda di skin (Petechiae) .
• Brus na di bɔdi we dɛn nɔ no ɛni rizin

If yu kɔntinyu fɔ gɛt wan ɔ mɔ pan dɛn sayn ya, mek shɔ se yu go to dɔktɔ.

Wetin mek dis bon mɛro nɔ de wok? Wetin na di tin dɛn we kin mek i apin?

Dɛn tin ya kin mek yu gɛt prɔblɛm wit yu bon mɛrɔ:

• Yu gɛt Bone Marrow Failure Syndrome (we yu kin gɛt frɔm yu mama ɛn papa).
• Yu gɛt blɔd kansa, ɔda blɔd sik, ɔ sɔm blɔd sik, kansa, ɔ infɛkshɔn (dɛn tin ya kin bigin leta, dat na, dɛn de pan di `(Acquired)` tayp).
• εksposhכn to sכm kεmikכl εn mεdikeshכn dεm (dis de fכl כnda di `(Acquired)` kεtכgrεf bak).

Sɔntɛnde, dis sik kin apin fɔ no rizin. Dɔktɔ dɛn kin kɔl am bak " idiopathic bone marrow failure." Risach pipul dεm biliv se lεk de bitwin כtoimyuniti εn bon mכro fεil. Awtoimyuniti na we yu bɔdi in yon imyun sistɛm mistek atak yu bon mɛrɔ.

Inhɛrit Bɔn Mɛrɔ Fayla Sindrɔm dɛn

Dis na sɔm kayn sik wae kin kam wit am, wae gɛt fɔ du wit am, ɔr gɛt di sem kayn sayn dɛm:

• Congenital Agranulocytosis (Kostmann Syndrome): Dis kin afɛkt wan kayn wayt blɔd sɛl we dɛn kɔl nyutrofil.
• Congenital Amegakaryocytic Thrombocytopenia (CAMT): pan dis kכndyushכn, di nכmba fכ di sεl dεm we dεn kכl megakaryocytes, we de εp fכ mek pletlet dεm, de dכn na yu bon mכro.
• Diamond-Blackfan Anemia (Diamond-Blackfan Anemia): Dis na wan sik we nɔ de mɛn we kin afɛkt di we aw dɛn de mek rɛd blɔd sɛl.
• Diskɛratosis Kɔnjɛnita (Diskɛratɔsis Kɔnjɛnita - `(Diskɛratɔsis Kɔnjɛnita)`):Di sayn dɛm fɔ dis sik na we di skin kin chenj, we kin bi wan fɔs sayn fɔ se di bon mɛro nɔ de wok fayn.
• Fanconi Anemia (Fanconi Anemia): Dis na di sik wae kin kam pan yu bon mכro fεil.
• Rεtikulכr Disjεnεsis (Rεtikulכr Disjεnεsis): Dis na wan fכm fכ Sεvεri Kכmbayn Imyun Dεfisiεns (SCID). pan dis kכndyushכn, yu bכdi nכ de mek inof machכ T-sεl dεm.
• Sivεr Congenital Neutropenia (Severe Congenital Neutropenia): Insay dis kכndyushכn, yu nyutrofil dεm, we na wan kayn wayt blכd sεl, de lכs pas aw i nכmal.
• Shwachman-Diamond Syndrome (Shwachman-Diamond Syndrome): Dis sik kin afɛkt yu pankrias, yu bon mɛro, ɛn yu bon dɛm.

If wan pan yu mama ɛn papa gɛt di sik ɛn di simptom dɛm (dɛn kɔl dis ``Autosomal Dominant''), yu gɛt 50% chans fɔ gɛt di sik ɛn gɛt bon mɛrɔ failure syndrome.

כltu, if yu mama εn papa כl tu kכri jin mכtεshכn we riliyt to di sem kayn bon mכro fεil sεndrכm, bכt nכn pan dεm nכ gεt simptom (dεn kכl dis ``Autosomal Recessive''), yu risk fכ divεlכp di kכndyushכn de rεdכks to 25%.

Blɔd Kansa dɛn

Yu de pan big risk fɔ mek yu bon mɛrɔ nɔ wok fayn if yu gɛt wan pan dɛn kayn kansa ya:

• Limfoma we dɛn kɔl Limfoma
• Mɔltipɛl Mayloma
• Maylodisplastik Sindrom we dɛn kɔl di sik

Ɔda Blɔd Disɔda dɛn

Sɔm pipul dɛn kin bɔn wit dɛn kayn tin ya. Ɔda pipul dɛn kin gɛt dɛn as tɛm de go. Dɛn tin ya na:

• Aplastic Anemia (Aplastic Anemia): Dis na wan sik we nɔ kin apin na di blɔd we nɔ kin apin so ɔltɛm. I de pwɛl yu bon mɛrɔ ɛn mek i nɔ mek nyu blɔd sɛl ɛn pletlɛt. Dɔktɔ dɛn kin yuz di wɔd "aplastic anemia" we dɛn de tɔk bɔt bon mɛro we nɔ de wok fayn.
• Saytopenia (Cytopenias): Dis inklud kכndyushכn dεm lεk כtoimyun nyutropenia, idiopatik nyutropenia, εn big granul limfosayt lukimiya.
• Paroxysmal Nocturnal Hemoglobinuria (Paroxysmal Nocturnal Hemoglobinuria): Insay dis kɔndishɔn, yu imyun sistɛm de pwɛl di rɛd blɔd sɛl dɛn.
• Pure Red Cell Aplasia (Pure Red Cell Aplasia): Dis na sik bak we pɔsin kin gɛt frɔm in mama ɛn papa we kin afɛkt di we aw dɛn de mek rɛd blɔd sɛl dɛn.

Vayral Infɛkshɔn dɛn

Dɛn vayral infɛkshɔn ya kin mek yu gɛt mɔ risk bak:

• Saytomegalovayrɔs `(Saytomegalovayrɔs)`
• Epstein-Barr Vayrɔs we dɛn kɔl Epstein-Barr
• Yuman Imyunɔdefisiɛns Vayrɔs (HIV) .
• Parvovayrɔs B19 `(Parvovayrɔs B19)`
• Vayral Ɛpatitis we pɔsin kin gɛt

Kimotɛrapi ɛn/ɔ redyushɔn tɛrapi fɔ kansa kin mek yu gɛt mɔ bon mɛrɔ we nɔ wok fayn bak. Dɔn bak, if yu gɛt kemikal ɛn sɔlvɛnt dɛn we dɛn kin yuz fɔ mek sɔm insektisayd ɛn peshɛnt sayd dɛn, dat kin mek yu gɛt mɔ prɔblɛm.

Wetin na de prɔblɛm wae kin kam wit dis sik?

Dis na siriɔs sik we kin mek pɔsin in layf de pan denja . Ivin afta dɛn dɔn trit am, dɛn prɔblɛm ya kin apin:

• Baktri ɔ vayral infɛkshɔn
• Blɔd we de kɔmɔt
• Blɔd kansa, fɔ ɛgzampul, akyu mayloid lukimiya ɔ maylodisplasia
• Ɔda kansa tɔŋ dɛm, fɔ ɛgzampul ɔstiosarkoma ɛn rabdomyosarkoma
• Squamous Cell Carcinoma we gɛt di sik we dɛn kɔl Squamous Cell Carcinoma

Aw yu kin no bɔt dis?

Dɔktɔ go aks yu fɔs bɔt yu sik, yu mɛdikal histri, ɛn yu famili in mɛdikal histri. Dɛn go du ɛgzam bak fɔ dɛn bɔdi. Dɛn kin ɔda bak fɔ mek dɛn du blɔd tɛst ɛn fɔ mek dɛn tek pikchɔ dɛn.

Tɛst fɔ Blɔd

Blɔd tɛst kin inklud:

• Tɛst fɔ Sɛruloplasmin
• Kɔmplit Blɔd Kɔnt (CBC) .
• Fɛritin Tɛst
• Fɔlayt Defisiɛns Tɛst
• Test fɔ di wan dɛn we nɔ gɛt vitamin B12

Tɛst dɛn we dɛn kin yuz fɔ tek pikchɔ

Di tɛst dɛn we dɛn kin du fɔ tek pikchɔ kin inklud:

• Magnεtik Rεsכnans Imej (MRI - `(Magnetik Rεsכnans Imej)` skan)
• Positron Emission Tomografi (PET - `(Positron Emission Tomografi)` skan)
• Ultrasound egzamin `(Ultrasaund)`

Bay di rizɔlt dɛn we dɛn kin gɛt frɔm di blɔd tɛst ɛn di imej tɛst, yu dɔktɔ kin tɛl yu fɔ tek yu bon mɛro bayɔpsi . I kin tɔk bak se dɛn fɔ tɛst di jɛnɛtiks fɔ no di chenj dɛn we de apin na di jɛnɛtiks we de mek di bon mɛro nɔ wok fayn.

Yu dɔktɔ kin ɔda bak fɔ mek dɛn du tɛst fɔ no if yu gɛt sɔm sik dɛn ɔ fɔ pul ɔda tin dɛn we de apin. Dɛn go ɛksplen to yu wetin mek dɛn de du ɛni tɛst ɛn wetin di tɛst dɛn kin sho.

Wetin na di tritmɛnt dɛn?

Di tritmɛnt we yu go gɛt go dipen pan bɔku tin dɛn, lɛk:

• di kayn bon mכro fεil (i.e. if na inhεrit כ we dεn gεt am).
• Di kayn we aw yu sik kin tranga.
• Yu ej ɛn ɔl yu wɛlbɔdi.
• Yu simptom dɛm.

Di tritmɛnt we dɛn kin pik kin bi:

• Antibayɔtik , antifungal, ɛn antivayrɔs mɛrɛsin fɔ fɛt infɛkshɔn.
• Blɔd transfyushɔn fɔ mek di rɛd blɔd sɛl dɛn bɔku ɛn fɔ ridyus di sayn dɛn lɛk fɔ blɔd ɛn taya.
• di tin dεm we de mek yu bon mכro de εp yu bon mכro fכ mek mכr bכdi sεl dεm.
• Immunosuppressants dεm de εp fכ stכp yu imyun sistεm fכ atak yu bon mכro.
• Allojenik Stem Sεl (Bכn Mכro) Transplant .

Ɛni sayd ɛfɛkt ɔ kɔmplikeshɔn de fɔ di tritmɛnt? (Kɔmplikeshɔn dɛn we kin apin we pɔsin de trit am)

Di kɔmplikeshɔn ɛn sayd ɛfɛkt dɛn kin difrɛn difrɛn wan bay di tritmɛnt, bɔt we dɛn transplant stem sɛl (bon mɛro) na in kin mek bɔku prɔblɛm dɛn kam. Dɛn tin ya na graft-versus-host disease (GVHD) ɛn infɛkshɔn.

Aw layf go tan lɛk wit dis kɔndishɔn? (Prɔgnosis) .

Wetin yu kin ɛkspɛkt afta tritmɛnt (prognosis) kin dipen pan sɔm tin dɛm, lɛk di kayn sik, yu ej ɛn ɔl yu wɛl bɔdi, ɛn aw yu bɔdi de ansa to tritmɛnt.

Bɔku tɛm, pipul dɛn we gɛt bon mɛrɔ we nɔ de wok fayn nid fɔ kɔntinyu fɔ gɛt mɛrɛsin ɛn sɔpɔt . Bɔt nɔto ɔlman na di sem. No yu sik gud gud wan, ɛn aks yu dɔktɔ wetin go apin nɛks afta yu fɔs tritmɛnt.

Yu tink se we yu bon mɛrɔ nɔ wok fayn na sik we kin kil pɔsin? (Na tɛminal?)

Dis na wan sik we rili siriɔs ɛn we kin mek pɔsin in layf de pan denja . Bɔrku pipul dɛm wae gɛt bon mɛrɔ wae nɔr de woke fayn, na spɛshal pipul dɛm wae gɛt ɛkspiriɛns lɛk ɔnkɔlɔjis ɛn ɛmatɔlɔjis kin trit dɛm.

Wetin wi kin se bɔt di layf we dɛn sik pipul ya kin liv? (Layf Ɛkspɛkt)

Di layf we pɔsin kin liv na fɔ no aw lɔng pɔsin go liv afta dɛn dɔn trit am fɔ wan patikyula sik. De layf wae pipul dεm wae gεt bon mכro fεil kin liv frכm fכs mכnt to dεn layf.

Bɔt, yu spɛshal, ɛn yu ɛkspiriɛns kin difrɛn frɔm ɔlman in yon. Aks yu dɔktɔ bɔt wetin yu kin ɛkspɛkt, ɛn aw lɔng yu kin ɛkspɛkt fɔ liv. Dɛn na yu bɛst sɔs fɔ infɔmeshɔn, bikɔs dɛn no yu ɛn yu kɔndishɔn.

Yu tink se dɛn kin ridyus di prɔblɛm? (Rɛdyus di Risk) .

Bɔt i sɔri fɔ no se, no we nɔ de fɔ ridyus di prɔblɛm we pɔsin kin gɛt we i gɛt fɔ du wit di bon mɛrɔ. Bɔt if yu avɔyd di kemikal dɛn we dɛn no se kin mek yu bon mɛrɔ nɔ wok fayn, dat kin mek yu nɔ gɛt sɔm bon mɛrɔ we yu dɔn gɛt. Dɔn bak, tritmɛnt kwik kin ɛp fɔ mek yu nɔr gɛt di sik ɛn fɔ mek yu liv bɛtɛ layf.

Aw a kin kia fɔ misɛf? (We yu de kia fɔ yusɛf) .

Di bɛst tin fɔ du na fɔ kia fɔ yu ɔl yu wɛlbɔdi . Fɔ ɛgzampul, nɔ yuz tabak, stɔp ɔ stɔp fɔ drink rɔm. Na sɔm ɔda advays dɛn ya:

• Si yu dɔktɔ ɔltɛm. Bɔn mɛrɔ we nɔ de wok fayn kin bi tin we pɔsin kin gɛt fɔ ɔl in layf. Yu kin nid fɔ kɔntinyu fɔ gɛt tritmɛnt. I kin mek wi gɛt nyu wɛlbɔdi prɔblɛm bak. So i impɔtant fɔ mek yu de chɛk yu ɔltɛm. Yu dɔktɔ go wach yu ɔl yu wɛlbɔdi ɛn trit ɛni nyu prɔblɛm.
• It tin dɛn we go mek yu gɛt wɛlbɔdi. Yu sayn dɛm ɛn sayd ɛfɛkt dɛm wae yu gɛt frɔm yu tritmɛnt kin mek i nɔr izi fɔ yu fɔ it ɔr yu nɔr want fɔ it. If yu nɔ shɔ if yu de gɛt di it we yu nid, tɔk to pɔsin we sabi bɔt it. Dɛn kin gi yu di rayt advays dɛn.
• Gɛt sɔm ɛksesaiz. Fɔ gɛt siriɔs wɛl bɔdi prɔblɛm lɛk wae yu bon mɛro nɔr de woke kin mek yu strɛs. Ɛksesaiz na fayn we fɔ mek yu nɔ strɛs. Mek shɔ se yu tɔk to yu dɔktɔ bifo yu bigin fɔ du nyu ɛksesaiz.

Ustɛm yu nid fɔ go na di Imejɛnsi Tritmɛnt Yunit (ETU) ?

If yu gɛt bon mɛrɔ we nɔ de wok fayn, yu go gɛt bɔku infɛkshɔn ɛn prɔblɛm wit blɔd. Yu fɔ go na di imejensi rum if yu gɛt ɛni wan pan dɛn tin ya:

• Fɔ gɛt fiva we nɔ de go dɔŋ ivin afta yu tek wan kɔmɔn pen kil
• Fɔ fil kol ɛn shek shek (Chills) .
• Blɔd we nɔ go ebul fɔ stɔp

Us kwɛstyɔn dɛn a fɔ aks di dɔktɔ?

Yu kin gɛt bon mɛrɔ we nɔ de wok fayn bikɔs ɔf sɔm sik dɛn we yu kin gɛt frɔm yu mama ɛn papa. Bɔt sɔntɛnde, i kin apin fɔ no rizin. Yu kin aks yu dɔktɔ kwɛstyɔn dɛn lɛk dɛn wan ya:

• yu no wetin mek a geht dis bon marrow failure?
• Us tritmɛnt opshɔn dɛn a gɛt?
• A go gɛt fɔ tek tritmɛnt sote go?
• Yu tink se dɛn go ebul fɔ mɛn dis?

If yu gɛt bon mɛrɔ we nɔ de wok fayn, yu go dɔn ɔlrɛdi gɛt siriɔs wɛlbɔdi prɔblɛm, lɛk sik we yu gɛt frɔm yu mama ɛn papa, blɔd prɔblɛm, ɔ kansa. Dis nyu diagnosis kin de mek yu gɛt bɔrku nyu kwɛstyɔn ɛn wɔri, wae kin tranga. Na nɔmal tin fɔ fil wɔri bikɔs dis kin bi wan sik wae de mek yu layf de pan denja. Tɔk to yu mɛdikal tim bɔt wetin fɔ ɛkspɛkt ɛn wetin fɔ du nɛks. Dɛn go gladi fɔ ansa yu kwɛstyɔn dɛn.

Mɛmba as sɔmari (Tek-Home Message) .

Bɔn mɛrɔ we nɔ de wok fayn na siriɔs ɛn kɔmpleks kɔndishɔn . Bɔt mɛmba se bɔku tin dɛn de we kin mek pɔsin gɛt dis sik, ɛn bɔku difrɛn tritmɛnt dɛn de ɛn aw fɔ mɛn am. Di tin we impɔtant pas ɔl na fɔ gɛt diagnosis kwik kwik wan, go to tritmɛnt frɔm di rayt spɛshal pipul dɛn, ɛn kɔntinyu fɔ fala dɛn instrɔkshɔn dɛn de.If yu gɛt ɛni kwɛstyɔn ɔ wɔri bɔt dis, nɔ fred fɔ tɔk to yu dɔktɔ. Dɛn de de fɔ ɛp yu.

` Bon Mכro Fεil, Rεd Blכd Sεl dεm, Wait Blכd Sεl dεm, Pletlet dεm, Stem Sεl Transplant, Bon Mכro Sik dεm