Ngaba nawe uyarhawuzelelwa emzimbeni wakho ngamanye amaxesha? Ngaba ubuso bakho nentamo yakho zijika zibe bomvu ngequbuliso uze uzive ngathi ufumana ubushushu obuphezulu? Ngaba unesisu esibuhlungu, isiyezi, kunye nentliziyo ebetha ngamandla? Nokuba ucinga ukuba ezi zinto zizinto nje zokunganyamezelani, ngamanye amaxesha kunokubakho imeko eyahlukileyo emva koku, esingazange sive ngayo kakhulu. Namhlanje sithetha ngesifo esingaqhelekanga, iSystemic Mastocytosis. Ungakhathazeki, masiqonde oku ngokulula.
Yintoni i-systemic mastocytosis?
Ngamafutshane, esi sisifo esibangelwa kukwanda okungaqhelekanga kohlobo lweseli olubizwa ngokuba yi
-'mast cells' emzimbeni wethu. Ii-mast cells luhlobo lweeseli ezimhlophe zegazi. Cinga ngezi seli ze-mast njengamajoni kwinkqubo yethu yokuzikhusela. Umsebenzi wazo ophambili kukusikhusela kwizifo nokusinceda siphilise xa
senzakele . Kodwa kwesi sifo, ezi seli ze-mast ziqokelelana ngokugqithisileyo nangokungaqhelekanga kwizitho ezahlukeneyo ezifana nolusu lwethu,
umongo wethambo , amathambo, kunye namathumbu. Kwenzeka ntoni emva koko? Ngenxa yesizathu esithile, ezi seli ze-mast ezigqithisileyo zisebenza ngequbuliso kwaye zikhuphe inani elikhulu leekhemikhali ezifana ne
-'histamine' emzimbeni. Kulapho ke iimpawu endizikhankanyileyo ngaphambili, ezinje ngokurhawuzelela, ukurhawuzelela,
kunye ne-eczema , zivela khona.
Kwezinye iimeko ezinzima kakhulu, le mpendulo inokuba yimpendulo enzima nesongela ubomi ebizwa ngokuba yi -anaphylaxis . Le yimeko engxamisekileyo. Kwiimeko ezinjalo, kufuneka uye kwisebe likaxakeka lesibhedlele (ETU) ngoko nangoko. Kufuneka usebenzise ne -epinephrine auto- injector ukuba ugqirha wakho uyicebisile.
Yintoni ebangela esi sifo?
Abantu abaninzi bayazibuza ukuba esi sisifo esidluliselwa ngelifa. Kodwa esi asisosifo esidluliselwa ngelifa. Uninzi lwexesha, senzeka
kwi-gene ebizwa ngokuba yi-KIT, efumaneka kwiiseli zethu ze-mast.Ibangelwa lutshintsho olungacwangciswanga (uguquko). Le gene inceda ekwenzeni iproteni elawula ukukhula kweeseli. Ngoko ke xa oku kutshintsha, iiseli zemast ziqala ukukhula ngendlela engalawulekiyo. Nangona ezi mpawu zinokuvela nakweyiphi na iminyaka, zixhaphake kakhulu kubantu abadala, ingakumbi
phakathi kweminyaka engama-40 nama-50 .
Izinto ezibangela ukuba iimpawu zibe mandundu
Ayingabo bonke abantu abanesi sifo abafumana iimpawu ezifanayo. Kwakhona, izinto ezibangela esi sifo zinokuhluka kumntu nomntu. Nangona kunjalo, ezinye zezinto ezibangela esi sifo zidweliswe kwitheyibhile engasezantsi. Kubalulekile ukuzazi ezi zinto.
| Izinto ezibangela ukuba iimpawu zibe mandundu | Inkcazo |
|---|
| Utywala | Ingakumbi izinto ezifana newayini ebomvu. |
| Ukutya okunongwe | Ukutya okuqulethe iitshili kunye nepepile. |
| Utshintsho kubushushu | Ukuchaphazeleka ngequbuliso yingqele okanye bubushushu obugqithisileyo. |
| Ukulunywa zizinambuzane | Izinto ezifana nokulunywa ziinyosi kunye neenyosi. |
| Uxinzelelo okanye ixhala | Uxinzelelo lomzimba okanye lwengqondo. |
| Amanye amayeza | I-aspirin, ii-opioid, kunye nee-NSAID zizinto ezithomalalisa iintlungu. |
| Utyando kunye nokugonywa | Uxinzelelo ngaphambi nasemva kotyando kunye nezinye iintlobo zemijovo. |
Kubalulekile ukukhumbula ukuba ngamanye amaxesha iimpawu zinokuvela ngaphandle kwesizathu esithile.
Ziziphi iintlobo eziphambili kunye neempawu zesi sifo?
Kukho iintlobo ezintlanu eziphambili zeSystemic Mastocytosis. Ugqirha wakho uza kukuvavanya aze akuxelele ukuba loluphi uhlobo onalo.
- Indolent Systemic Mastocytosis (ISM): Olu lolona hlobo luqhelekileyo nolungabi nzima kangako. Esi sifo siqhubeka kancinci kwaye asibangeli monakalo mkhulu kumalungu omzimba.
- I-Smoldering Systemic Mastocytosis (SSM): Inzima kancinci kune-ISM. Iimpawu zinzima ngakumbi kwaye zinokuchaphazela amalungu omzimba ngokuhamba kwexesha.
- I-Systemic Mastocytosis ene-Associated Hematological Neoplasm (SM-AHN): Kule meko, i-mastocytosis ihamba nesinye isifo esinxulumene negazi (umz. i-leukemia ).
- I-Aggressive Systemic Mastocytosis (ASM): Olu hlobo lukhula ngokukhawuleza kwaye lunokonakalisa amalungu omzimba afana nesibindi, ipleen, kunye namathambo.
- I-Mast Cell Leukemia (MCL): Olu lolona hlobo lungaqhelekanga nolunzima kakhulu. Kukho inani elikhulu leeseli ze-mast kwi-bone mongo nasegazini.
Iimpawu ozifumanayo zixhomekeke kuhlobo lwesifo onaso kunye nokuba zininzi phi iiseli zemast ezingaqhelekanga emzimbeni wakho. Nazi ezinye zeempawu eziqhelekileyo:
- Ukurhawuzelela, ukurhawuzelela , ukufutha
- Intlungu yesisu, ukudumba, urhudo
- Isicaphucaphu okanye ukuhlanza
- Intloko ebuhlungu kunye nokudinwa
- Ukubetha kwentliziyo ngokukhawuleza
- Uxinzelelo lwegazi oluphantsi, isiyezi, ukulahlekelwa zingqondo
- Ukudumba kwesibindi, ipleen, kunye ne-lymph nodes
- I-anemia kunye neemeko zokopha
- Ukubuthathaka kwamathambo (i-Osteoporosis)
- Engqondweni, iimeko ezinje ngobunzima bokugxila, ukuxhalaba rhoqo, okanye ukudakumba.
Ungasixilonga njani esi sifo? (Ukuxilongwa)
Ugqirha wakho uza kuqala akubuze ngeempawu zakho aze enze uvavanyo lomzimba. Emva koko, banokucebisa iimvavanyo ezininzi ukuqinisekisa ukuxilongwa:
- I-biopsy yolusu: Iqhekeza elincinci lesikhumba lithathwa kwindawo echaphazelekayo lize lihlolwe nge-microscope ukuze kubonwe ukuba kukho iiseli ezininzi ze-mast.
- I-Bone Marrow Biopsy: Ingcali ye-hematologist isebenzisa inaliti encinci ukuthatha isampuli encinci yomongo wakho wethambo aze awuhlole.
- Uvavanyo lwegazi nomchamo:Uvavanyo lwegazi olupheleleyo (i-CBC) lungasetyenziswa ukujonga ezinye iimeko, ezifana ne-anemia.
- Uvavanyo lweMfuzo: Isampuli yegazi okanye yethishu iyavavanywa ukuze kubonwe ukuba kukho naluphi na utshintsho kwi-gene ye-KIT ekhankanyiweyo ngaphambili.
- Uvavanyo lwemifanekiso: Ii-X-ray, ii-ultrasound, kunye nee-CT scans zinokunceda ekufumaneni ubuthathaka bamathambo kunye nokudumba kwamalungu anjengesibindi kunye ne-spleen.
- I-Endoscopy okanye iColonoscopy: Ityhubhu encinci enekhamera eqhotyoshelwe kuyo isetyenziselwa ukuhlola ngaphakathi kwesisu okanye amathumbu ukujonga naziphi na iingxaki, ezifana nezilonda.
Zithini iindlela zonyango?
Ugqirha uza kugqiba isicwangciso sonyango ngokusekelwe kwiimpawu zakho, amalungu omzimba achaphazelekileyo, kunye nohlobo lwesifo.
- Ii-Antihistamines: La mayeza anceda ukulawula izinto ezinje ngokurhawuzelela ulusu, ukuba bomvu, kunye nokuphazamiseka kwesisu.
- I-Epinephrine: Yi-hormone esetyenziswa xa kukho i-allergy enzima (i-anaphylaxis).
- IiProton Pump Inhibitors: Lawula i-asidi yesisu kwaye unciphise izinto ezifana nokudumba kwesisu.
- IziQinisekisi zeMast Cell: Amayeza athintela ukukhululwa kwe-histamine kwiiseli zemast.
- IiSteroids: Zinokunceda ngeempawu zesikhumba kunye nokuphefumla.
- Unyango Olujoliswe Kulo: Amayeza ajoliswe kwijini ethile okanye kwiproteni ebandakanyekayo kwesi sifo. La mayeza anokufuneka kubantu abaneempawu eziqatha.
- Unyango lweKhemotherapy: Olu nyango lunokufuneka kwiimeko apho isifo sisasazeka ngokukhawuleza kwaye sibangela umonakalo omkhulu kumalungu omzimba.
- Ukufakelwa kweeseli zeStem: Unyango oluqwalaselwa kwiimeko ezingaqhelekanga kakhulu.
Umyalezo Wokuya Ekhaya
- I-Systemic Mastocytosis sisifo esingaqhelekanga esibangelwa kukwanda okungaqhelekanga kweeseli ze-mast.
- Iimpawu zinokuba mandundu zizinto ezifana notywala, ukutya okuneziqholo, kunye noxinzelelo. Chonga kwaye uziphephe izinto ezibangela ukuba iimpawu zakho zibe mandundu.
- Oku kusenokungabi yi-allergy nje kuphela. Ukuba iimpawu ziyaqhubeka, qiniseka ukuba ubona ugqirha kwaye wenze uvavanyo olufunekayo.
- Kukho umngcipheko wokuba ne-allergy enkulu (i-anaphylaxis). Soloko uphethe i-epinephrine auto-injector ukuba ugqirha wakho uyicebisile.
- Nangona esi sifo singenakunyangwa ngokupheleleyo, unyango lunokulawula kakhulu iimpawu kwaye luvumele abantu ukuba baphile ubomi obuqhelekileyo.
- Kubalulekile ukuhlala uqinile engqondweni xa uphila nesi sifo esifana nesi ixesha elide. Funa inkxaso kusapho, kubahlobo, okanye kumaqela enkxaso.
I-Systemic Mastocytosis, ii-mast cells, i-skin rash, i-anaphylaxis, i-anaphylaxis, i-KIT gene, i-histamine
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