Ingabe uvame ukuzwa ubuhlungu obungavamile emzimbeni? Ake sifunde kabanzi ngalokhu (I-Systemic Mastocytosis)!

Ingabe ngezinye izikhathi uthola amabala abomvu, alumayo esikhumbeni sakho? Noma ingabe unezinhlungu zesisu kanye nekhanda elivele lidlule? Uma lezi zinto zenzeka ngokuphindaphindiwe, kungaba yisimo esingavamile esibizwa ngokuthi "i-Systemic Mastocytosis" esizokhuluma ngaso namuhla. Ungakhathazeki, lesi akusona isifo esithinta abantu abaningi. Kodwa kubalulekile ukuqaphela lokhu.

Ngakho-ke, iyini le "Systemic Mastocytosis"?

Kalula nje, i-`(Systemic Mastocytosis)` yisifo esingavamile esihlobene negazi lethu. Okwenzekayo kulokhu ukuthi uhlobo olukhethekile lweseli olubizwa ngokuthi `(Mast cells)` emzimbeni wethu lukhula ngendlela engavamile. Cabanga ngakho, lawa maseli e-mast afana namasosha amancane emzimbeni wethu. Yiwo, njengengxenye yesimiso sethu somzimba sokuzivikela, abona futhi alwe nabahlaseli bakwamanye amazwe (njengamagciwane, izinto ezibangela ukungezwani komzimba, njll.). Lapho kufika umhlaseli wangaphandle onjalo, lawa maseli e-mast akhipha amakhemikhali afana ne-`(Histamine)`. Le `(Histamine)` yiyo ebangela lezo zimpawu zokungezwani komzimba (njengokubomvu, ukulunywa, ukuvuvukala). Ngokuvamile, amaseli e-mast ayayeka ukukhipha le `(Histamine)` uma umsebenzi usuqediwe. Kodwa-ke, emzimbeni womuntu one-`(Systemic Mastocytosis)`, lawa maseli e-mast angavamile akhula ngokweqile futhi akhulule i-`(Histamine)` njalo. Kunjengomuntu oshiye impompi yamanzi bese ekhohlwa ukuyicisha. Yingakho siqhubeka nokuba nezimpawu ezifana nokungezwani komzimba. Kungathinta hhayi isikhumba sethu kuphela, kodwa nezitho zethu zangaphakathi njengesibindi, udende, namathumbu. Lesi simo, esibizwa ngokuthi "i-Systemic Mastocytosis," kuthiwa sithinta abantu abayi-13 kuphela kwabayi-100,000 emhlabeni. Lokho kusho ukuthi asivamile kakhulu. Kodwa-ke, abantu abanalesi simo basengozini enkulu ye- anaphylaxis ( ukusabela okubi kakhulu, okungazelelwe, okusongela impilo). Akuvamile kakhulu, "i-Systemic Mastocytosis" nayo ingaphenduka umdlavuza. Odokotela abakwazi ukwelapha lesi simo ngokuphelele. Kodwa-ke, kunezindlela zokwelapha ezingakusiza ukulawula izimpawu zakho futhi uphile impilo evamile.

Yiziphi izinhlobo eziyinhloko ze-`(Systemic Mastocytosis)`?

Kunezinhlobo eziyisithupha eziyinhloko ze-"Systemic Mastocytosis". Zisithinta ngezindlela ezahlukene. Ngokuvamile, uma unawo amaseli e-mast angajwayelekile emzimbeni wakho, kulapho amathuba okuba nezinkinga anda khona. Ake sibheke ukuthi yiziphi lezo zinhlobo: 1. Uhlobo olukhula kancane (``Indolent systemic mastocytosis``):

• Lolu uhlobo oluvame kakhulu. Njengomuthi okhula kancane, kungathatha iminyaka ukuthi izimpawu zivele. Inani lamaseli e-mast angavamile liyakhula ngokuhamba kwesikhathi. Lokhu kungabangela izimpawu esikhumbeni, esibindini , e-spleen , kanye nasesimisweni sesisu (okungukuthi, emathunjini).

2. Uhlobo oluthuthuke kancane (`I-mastocytosis eshunqayo ehlelekile`):

• Kulolu hlobo, amaseli e-mast angavamile aqoqana ngobuningi esibindini nasesinqeni. Ngokuhamba kwesikhathi, lokhu kungabangela isibindi noma isinqe ukuthi sivuvuke. Cabanga ngalokho okwenzekayo lapho indlu encane igcwele kakhulu.

3. Uhlobo oluhambisana nezinye izifo zegazi (`I-systemic mastocytosis ene-hematologic neoplasm ehlobene`):

• Abantu abanalolu hlobo bangase babe nezinye izimo ezihlobene negazi, njenge- myeloproliferative neoplasms kanye ne-myelodysplastic syndrome . Lolu hlobo luthinta cishe umuntu oyedwa kwabahlanu abane-Systemic Mastocytosis.

4. Uhlobo olubi kakhulu (`I-Aggressive systemic mastocytosis`):

• Lokhu kuyingozi kakhulu ngoba kungathinta umongo wamathambo (lapho kwenziwa khona amangqamuzana egazi) kanye namathambo. Amaseli e-mast angajwayelekile angakhula ngaphakathi kwamathambo, awenze abe buthaka futhi aphuke kalula.

5. Uhlobo oluphenduka i-leukemia (`i-Mast cell leukemia`):

• Akuvamile kakhulu, i-Systemic Mastocytosis ingakhula ibe yisimo esibizwa ngokuthi i-Mast Cell Leukemia . Lolu uhlobo lwe- Acute Myeloid Leukemia (AML) .

6. Uhlobo lwesimila (`i-Mast cell sarcoma`):

• Yilapho izimila (amathumba) akhiwe ngamaseli e-mast angavamile elimaza izicubu ezahlukene emzimbeni. Inani elincane kakhulu labantu abane-"Systemic Mastocytosis" liba nalesi simo esibizwa ngokuthi i-"Mast cell sarcoma".

Yiziphi izimpawu zalesi sifo (i-Systemic Mastocytosis)?

Izimpawu zingahluka kumuntu nomuntu. Kuya ngokuthi amangqamuzana e-mast angavamile agxile kuphi emzimbeni. Isibonelo, uma unamaseli amaningi e-mast esiswini sakho, ungase ube nezilonda kanye nobuhlungu besisu. Futhi, uma amaseli e-mast eqoqana emnkantsheni wakho wamathambo, kungathinta ukukhiqizwa kwamaseli egazi. Nazi ezinye izimpawu ezivamile:

• I-anemia ( ukuntuleka kwegazi )
• Ubuhlungu bethambo
• Ukopha ngokweqile (isib., igazi eliningi elivela esilondeni esincane)
• Ukuzizwa ukhathele ngaso sonke isikhathi (` Ukukhathala` )
• Isikhumba esicwebezelayo
• Ukushaya kwenhliziyo
• Amaqhubu (okulumayo, amaqhubu aphakanyisiwe esikhumbeni)
• Isikhumba esilumayo
• Ukushintsha kwesimo sengqondo noma ukucindezelekaukucindezeleka `)
• Amabala amnyama, alumayo esikhumbeni (`Urticaria pigmentosa`)

Ngezinye izikhathi, abantu abane-Systemic Mastocytosis bangase babe nezimpawu eziningana ngesikhathi esisodwa. Odokotela bakubiza lokhu ngokuthi "ukuhlasela kwe-mastocytosis" noma "ukugqabhuka."

Okubalulekile: Akuwona wonke umuntu onalezi zimpawu one-`(Systemic Mastocytosis).` Kodwa-ke, uma unezimpawu eziningana ngesikhathi esisodwa, futhi ziqhubeka, kufanele nakanjani ubone udokotela ukuze akunike iseluleko.

Kungani lokhu `(i-Systemic Mastocytosis)` kwenzeka?

Ucwaningo luthole ukuthi cishe abantu abangu-80% abane-`(Systemic Mastocytosis)` bane -`KIT gene variant` . Le `KIT` gene isiza izinhlobo ezithile zamaseli emzimbeni wethu, ikakhulukazi amaseli egazi namaseli e-mast, ukuba akhule. Le gene variant yenzeka ngemva kokuba sikhulelwe esibelethweni. Lokhu kusho ukuthi ayizuzwa njengefa.

Yiziphi izinto ezibhebhethekisa noma ezivusa lesi sifo (`Izibangeli`)?

Izinto eziningi zingabangela izimpawu ze-"Systemic Mastocytosis." Akuwona wonke umuntu othintwa yizinto ezifanayo. Kodwa-ke, kunezinto ezimbalwa ezivamile:

• Utshwala
• Ukudla okunongwe
• Eminye imithi: Isibonelo , ama-NSAID ( ama-painkiller), ama-muscle relaxer, kanye ne-anesthesia.
• Ukuzivocavoca kanye nomsebenzi womzimba ( kwabanye kuphela)
• Ukulunywa yizinambuzane (ikakhulukazi izintuthwane, iminyovu, nezinyosi)
• Ukucindezeleka ngokomzimba noma ngokomzwelo
• Ukuhlikihla noma ukukhuhlana esikhumbeni sakho
• Izinguquko ezingazelelwe ekushiseni (isb., ukugxuma ngokuzumayo emanzini abandayo)

Uma unalesi sifo, kubaluleke kakhulu ukubuza udokotela wakho ukuthi ubuthakathaka bakho buyini nokuthi yini okufanele uyigweme.

Uyithola kanjani ngokunembile lesi sifo? (Ukuxilongwa)

Uma uya kudokotela, into yokuqala abazoyenza ukukuhlola ngokomzimba. Bazokubuza ngezimpawu zakho kanye nanoma yiziphi izifo oke waba nazo ngaphambilini. Bese, bangenza olunye noma ngaphezulu kwezivivinyo ezilandelayo ukuze bathole ukuthi une-Systemic Mastocytosis:

• Ukuhlolwa kwegazi: Lokhu kuhlola inani le -tryptase egazini lakho. I-Tryptase iyi- enzyme ekhethekile ekhishwa amaseli e-mast lapho esabela ezintweni zangaphandle.
• Ukuskena kwamathambo: Hlola umonakalo wamathambo.
• Ukuhlolwa komnkantsha wethambo: Lokhu kuhilela ukuthatha isampula encane yomnkantsha wakho wethambo bese uwuhlola ukuze kubonakale ukuthi kukhona yini amaseli e-mast angavamile kuwo, nokuthi mangaki.
• Ukuhlolwa kwezakhi zofuzo: Hlola ukuguquka kwezakhi zofuzo ze-'KIT' okukhulunywe ngazo ngaphambilini.

Iphathwa kanjani?

Odokotela belapha i-Systemic Mastocytosis ngokulawula izimpawu nezinkinga. Isibonelo, uma une-asidi ephezulu esiswini, bangase bakunike ama-H2 blockers (uhlobo lwe-antacid). Uma i-anemia iwumphumela wesifo, izokwelashwa. Nazi ezinye zezindlela zokwelapha eziyinhloko:

• Ama-Antihistamine: Yehlisa izimpawu zesikhumba ezifana nokulunywa nokubomvu.
• Ama-Corticosteroids: Yehlisa ukuvuvukala nokuvuvukala emzimbeni.
• Ama-Bisphosphonates: Asiza ekuqiniseni amathambo uma ebuthakathaka.
• Ukwelashwa okuqondiwe: Lokhu kuhilela ukuqondisa iphrotheni ethile etholakala kumaseli e-mast angavamile.
• Ukwelashwa ngamakhemikhali: Lokhu kusetshenziswa kuphela uma i-Systemic Mastocytosis isiphenduke umdlavuza.
• Ukususwa kwe-splenectomy: Uma i-splen ivuvukele kakhulu, ingasuswa ngokuhlinzwa.
• Ukufakelwa umnkantsha: Lokhu kwenzelwa abantu abanesifo esibi kakhulu, noma labo abasesigabeni sokugcina.

Khumbula: Uma une-Systemic Mastocytosis, kufanele uhlale uphethe i-EpiPen . I-EpiPen umuthi ongasetshenziswa ngokushesha uma kwenzeka ukusabela okukhulu kwe-allergy, njenge-Anaphylaxis eshiwo ngenhla.

Ingabe lokhu "ku-Systemic Mastocytosis" akunakuvinjelwa?

Njengoba sixoxile ngaphambili, i-"Systemic Mastocytosis" ibangelwa ukuguquka kwezakhi zofuzo. Ngakho-ke, akunakwenzeka ukuyivimbela ukuthi ingakhuli. Kodwa-ke, ungalawula lesi sifo ngokugwema "izinto ezibangela" ezikhulisa izimpawu.

Luhlobo luni lwekusasa umuntu one-`(Systemic Mastocytosis)` analo?

Lokhu kuncike kakhulu kuhlobo luni lwe-`(Systemic Mastocytosis)` onalo. Abantu abanohlobo olukhula kancane (`Indolent systemic mastocytosis`) bavame ukulawula lesi sifo kahle ngokwelashwa. Bangaphila isikhathi sokuphila esijwayelekile. Kodwa-ke, abantu abanezinye izinhlobo ezinzima bangase babe nesikhathi sokuphila esifushane kancane. Kodwa-ke, i-`(Systemic Mastocytosis)` ingelapheka ngokuphelele ngokwelashwa okubizwa ngokuthi ``Bone marrow transplant``. Kodwa-ke, odokotela banikeza kuphela lokho kwelashwa kubantu abasezigabeni ezinzima kakhulu zesifo.

Ngingazinakekela kanjani ngenkathi ngiphila ne-`(Systemic Mastocytosis)`?

Ukwazi ukuthi yini ebangela izimpawu zakho nokuzigwema kuyinto ebaluleke kakhulu ongayenza uma uphila ne-Systemic Mastocytosis. Uma unalesi simo, qaphela lezi zinto:

• Hlala uphethe i-EpiPen. Yelapha ngokushesha ukusabela okukhulu kokungezwani komzimba.
• Yazi kahle ukuthi yiziphi izinto ezikubangelayo futhi uzigweme ngangokunokwenzeka.
• Lawula ukucindezeleka. Prakthiza ukuzindla noma ezinye izindlela zokucabanga.
• Gqoka isongo lesexwayiso sezokwelapha elinohlu lwemithi ongeke uyithathe. Lokhu kubaluleke kakhulu ngoba kusiza odokotela bazi isimo sakho uma ungakwazi ukukhuluma esimweni esiphuthumayo.

Kufanele ngimbone nini udokotela?

Uma une-Systemic Mastocytosis, kuzodingeka ubone udokotela wakho ukuze ahlolwe njalo. Futhi, qiniseka ukuthi utshela udokotela wakho uma uthola izimpawu ezintsha, noma uma ezinye zezimpawu zakho zibonakala ziba zimbi kakhulu.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Ngenxa yokuthi i-Systemic Mastocytosis iyisifo esingavamile, ungase ube nemibuzo eminingi. Nazi ezinye zemibuzo okufanele uyibuze udokotela wakho:

• Uhlobo luni lwe-"Systemic Mastocytosis" enginalo?
• Yiziphi ezinye izimpawu ezingase zibonise ukuthi isimo sami siya siba sibi kakhulu?
• Yiziphi izindlela zokwelapha ezitholakalayo zohlobo lwami?
• Ingabe ukwelashwa kuzoqeda ngokuphelele izimpawu zami?
• Ingabe kukhona izivivinyo zemitholampilo engingahlanganyela kuzo?

Ukutholwa une-Systemic Mastocytosis kuyinto eshintsha impilo. Kuzodingeka ubone udokotela wakho njalo, uhlolwe igazi, futhi ufunde ukuqaphela nokugwema izinto ezibangela lokhu. Ukuphila nalesi simo kuyisinqumo esikhulu. Ngakho-ke, gcina ubuhlobo obuhle nodokotela wakho. Bakhona ukukusiza. Bazokunikeza ulwazi kanye namaqembu okusekela angakusiza. Lokhu kuzokunika amandla okwenza izinqumo mayelana nempilo yakho.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ngakho-ke sesikhulume kakhulu nge-`(Systemic Mastocytosis)`, akunjalo? Khumbula, yize lesi kuyisifo esingavamile, kubaluleke kakhulu ukuqaphela.

Into ebaluleke kakhulu ukubona udokotela uma unalezi zimpawu. Uma lesi sifo sitholakala kusenesikhathi, ukwelashwa kungaqalwa futhi izimpawu zingalawulwa.

Awuwedwa. Odokotela bakho, umndeni wakho, nabangani bakho bakhona ukukusiza. Ngokwazi ulwazi olufanele nokulandela iseluleko sikadokotela wakho, ungaphila kahle nalesi simo. Hlala uzolile futhi ucabange kahle!