Ngaba uhlala uziva iintlungu zomzimba ezingaqhelekanga? Masifunde okungakumbi ngale (Systemic Mastocytosis)!

Ngaba ngamanye amaxesha uba namabala abomvu, arhawuzelelayo eluswini lwakho? Okanye ngaba uneentlungu zesisu kunye neentloko ezivele ziphele? Ukuba ezi zinto zenzeka ngokuphindaphindiweyo, isenokuba yimeko engaqhelekanga ebizwa ngokuba yi-"Systemic Mastocytosis" esiza kuthetha ngayo namhlanje. Ungakhathazeki, esi ayisosifo sichaphazela abantu abaninzi. Kodwa kubalulekile ukuqaphela oku.

Ngoko ke, yintoni le "Systemic Mastocytosis"?

Ngamafutshane, `(Systemic Mastocytosis)` sisifo esingaqhelekanga esinxulumene negazi lethu. Okwenzekayo koku kukuba uhlobo oluthile lweseli olubizwa ngokuba yi`(Mast cells)` emzimbeni wethu lukhula ngendlela engaqhelekanga. Cinga ngayo, ezi mast cells zifana namajoni amancinci emzimbeni wethu. Ngabo, njengenxalenye yenkqubo yethu yokuzikhusela, abaqaphela kwaye balwe nabahlaseli bamanye amazwe (njengeentsholongwane, ii-allergens, njl.njl.). Xa kufika umhlaseli wasemzini onjalo, ezi mast cells zikhupha iikhemikhali ezifana ne`(Histamine)`. Le `(Histamine)` yiyo ebangela ezo mpawu ze-allergy (ezifana nokubomvu, ukurhawuzelela, ukudumba). Ngokwesiqhelo, iimast cells ziyeka ukukhupha le `(Histamine)` xa umsebenzi ugqityiwe. Nangona kunjalo, emzimbeni womntu one`(Systemic Mastocytosis)`, ezi mast cells ezingaqhelekanga zikhula kakhulu kwaye zikhupha i`(Histamine)` rhoqo. Kufana nomntu oshiye impompo yamanzi aze alibale ukuyicima. Yiyo loo nto sihlala sineempawu ezifana ne-allergy. Ayinakuchaphazela ulusu lwethu kuphela, kodwa kunye namalungu ethu angaphakathi njengesibindi, i-spleen, namathumbu. Le meko, ebizwa ngokuba yi-"Systemic Mastocytosis," kuthiwa ichaphazela abantu abali-13 kuphela kwabayi-100,000 ehlabathini. Oko kuthetha ukuba ayiqhelekanga kakhulu. Nangona kunjalo, abantu abanale meko basengozini enkulu ye- anaphylaxis ( i-allergy reaction enzima, ngequbuliso, nesongela ubomi). Amaxesha amaninzi, i-"Systemic Mastocytosis" inokuguquka ibe ngumhlaza. Oogqirha abanakuyinyanga ngokupheleleyo le meko. Nangona kunjalo, kukho unyango olunokukunceda ukulawula iimpawu zakho kwaye uphile ubomi obuqhelekileyo.

Ziziphi iintlobo eziphambili ze-`(Systemic Mastocytosis)`?

Kukho iintlobo ezintandathu eziphambili ze-"Systemic Mastocytosis". Zisichaphazela ngeendlela ezahlukeneyo. Ngokubanzi, xa unee-mast cells ezingaqhelekanga emzimbeni wakho, kokukhona unokuba neengxaki. Makhe sijonge ukuba zeziphi ezo ntlobo: 1. Uhlobo olukhula kancinci (``Indolent systemic mastocytosis``):

• Olu lolona hlobo luqhelekileyo. Njengomthi okhula kancinci, kungathatha iminyaka ukuba iimpawu zibonakale. Inani leeseli ezingaqhelekanga zemast liyanda ngokuhamba kwexesha. Oku kunokubangela iimpawu kulusu, isibindi , ipleen , kunye nenkqubo yesisu (oko kukuthi, amathumbu).

2. Uhlobo oluphucuke kancinci (`i-systemic smoldering mastocytosis`):

• Kolu hlobo, iiseli zemast ezingaqhelekanga ziqokelelana ngamanani amakhulu esibindini nakwispleen. Ekuhambeni kwexesha, oku kunokubangela ukuba isibindi okanye ispleen idumbe. Cinga ngento eyenzekayo xa indlu encinci ixinene kakhulu.

3. Uhlobo oluza nezinye izifo zegazi (`I-Systemic mastocytosis ene-hematologic neoplasm enxulumeneyo`):

• Abantu abanolu hlobo banokuba nezinye iimeko ezinxulumene negazi, ezifana ne -myeloproliferative neoplasms kunye ne-myelodysplastic syndrome . Olu hlobo luchaphazela malunga nomntu omnye kwabahlanu abaneSystemic Mastocytosis.

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

• Oku kubi ngakumbi kuba kunokuchaphazela umongo wethambo (apho kwenziwa khona iiseli zegazi) kunye namathambo. Iiseli zemast ezingaqhelekanga zinokukhula ngaphakathi kwamathambo, ziwenze buthathaka kwaye ziwenze aphuke lula.

5. Uhlobo oluguquka lube yi-leukemia (`i-Mast cell leukemia`):

• Kunqabile kakhulu, iSystemic Mastocytosis inokukhula ibe yimeko ebizwa ngokuba yiMast Cell Leukemia . Olu luhlobo lweAcute Myeloid Leukemia (AML) .

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

• Kulapho ke iithumba (iithumba) ezenziwe ngeeseli zemast ezingaqhelekanga zonakalisa izicubu ezahlukeneyo emzimbeni. Inani elincinci kakhulu labantu abane-"Systemic Mastocytosis" lifumana le meko ebizwa ngokuba yi-"Mast cell sarcoma".

Zithini iimpawu zesi sifo (i-Systemic Mastocytosis)?

Iimpawu zingahluka kumntu nomntu. Kuxhomekeke ekubeni zikuphi emzimbeni apho iiseli ze-mast ezingaqhelekanga zixinene khona. Umzekelo, ukuba uneeseli ze-mast ezininzi esiswini sakho, unokuba nezilonda kunye nentlungu yesisu. Kwakhona, ukuba iiseli ze-mast ziqokelelana kumongo wakho wethambo, oku kunokuchaphazela ukuveliswa kweeseli zegazi. Nazi ezinye iimpawu eziqhelekileyo:

• I-anemia ( ukungabikho kwegazi )
• Intlungu yethambo
• Ukopha kakhulu (umz., igazi elininzi elivela kwinxeba elincinci)
• Ndiziva ndidiniwe ngalo lonke ixesha (` Ukudinwa` )
• Ulusu oluhlanjululweyo
• Ukubetha kwentliziyo
• Iihives (ezirhawuzelayo, eziphakanyisiweyo eluswini)
• Ulusu olurhawuzelelayo
• Utshintsho lweemvakalelo okanye uxinezelekoukudakumba
• Amabala amnyama, arhawuzelelayo eluswini (`Urticaria pigmentosa`)

Ngamanye amaxesha, abantu abaneSystemic Mastocytosis banokufumana ezinye zezi mpawu ngaxeshanye. Oogqirha bakubiza oku ngokuthi "uhlaselo lwe-mastocytosis" okanye "ukugqabhuka."

Kubalulekile: Asinguye wonke umntu onezi mpawu one `(Systemic Mastocytosis).` Nangona kunjalo, ukuba unezinye zezi mpawu ngaxeshanye, kwaye ziyaqhubeka, ngokuqinisekileyo kuya kufuneka ubone ugqirha ukuze akunike ingcebiso.

Kutheni le nto `(Systemic Mastocytosis)` isenzeka?

Uphando lufumanise ukuba malunga ne-80% yabantu abane-`(Systemic Mastocytosis)` bane -`KIT gene variant` . Le `KIT` gene inceda iintlobo ezithile zeeseli emizimbeni yethu, ingakumbi iiseli zegazi kunye neeseli zemast, ukuba zikhule. Le gene variant yenzeka emva kokuba sikhulelwe esibelekweni. Oku kuthetha ukuba ayizuzwa njengelifa.

Zeziphi izinto ezibangela okanye ezibangela ukuba esi sifo sibe sibi kakhulu (`Iingxaki`)?

Zininzi izinto ezinokubangela iimpawu ze-"Systemic Mastocytosis." Asinguye wonke umntu ochaphazeleka zizinto ezifanayo. Nangona kunjalo, kukho izinto ezimbalwa eziqhelekileyo:

• Utywala
• Ukutya okunongwe
• Amanye amayeza: Umzekelo , ii-NSAID ( iintlungu zentlungu), iziphumzi zemisipha, kunye ne-anesthesia.
• Ukuzilolonga kunye nomsebenzi womzimba ( kwabanye kuphela)
• Ukulunywa zizinambuzane (ingakumbi iimbovane, iinyosi, kunye neenyosi)
• Uxinzelelo lomzimba okanye lweemvakalelo
• Ukukhuhla okanye ukukhuhlana kolusu lwakho
• Utshintsho olukhawulezileyo kubushushu (umz., ukutsiba ngequbuliso emanzini abandayo)

Ukuba unesi sifo, kubaluleke kakhulu ukubuza ugqirha wakho ukuba zeziphi izinto obuthathaka kuzo kunye nezinto omele uziphephe.

Ungayixilonga njani ngokuchanekileyo le sifo? (Ukuxilongwa)

Xa usiya kugqirha, into yokuqala abaya kuyenza kukuvavanya umzimba. Baza kukubuza ngeempawu zakho kunye nazo naziphi na izifo oye wazifumana ngaphambili. Emva koko, banokwenza olunye okanye ngaphezulu kwezi vavanyo zilandelayo ukuze babone ukuba uneSystemic Mastocytosis:

• Uvavanyo lwegazi: Oku kujonga ubungakanani be -tryptase egazini lakho. I-Tryptase yi- enzyme ekhethekileyo ekhutshwa ziiseli ze-mast xa zisabela kwizinto zangaphandle.
• Ukuhlolwa kwamathambo: Jonga umonakalo emathanjeni.
• I-Bone marrow biopsy: Oku kuquka ukuthatha isampuli encinci yomongo wakho wethambo uze uwuvavanye ukuze ubone ukuba kukho iiseli zemast ezingaqhelekanga kuwo, kwaye zingaphi.
• Uvavanyo lwemfuza: Jonga utshintsho kwimfuza ethi `KIT` ekhankanyiweyo ngaphambili.

Iphathwa njani?

Oogqirha banyanga iSystemic Mastocytosis ngokulawula iimpawu kunye neengxaki. Umzekelo, ukuba une-asidi ephezulu esiswini, banokukunika ii-H2 blockers (uhlobo lwe-antacid). Ukuba i-anemia ibangelwa sisifo, iya kunyangwa. Nazi ezinye zeendlela eziphambili zonyango:

• Ii-Antihistamines: Zinciphisa iimpawu zolusu ezifana nokurhawuzelela nokuba bomvu.
• IiCorticosteroids: Zinciphisa ukudumba kunye nokuvuvukala emzimbeni.
• IiBisphosphonates: Zinceda ukuqinisa amathambo ukuba abuthathaka.
• Unyango olujoliswe kulo: Oku kuquka ukujolisa kwiproteni ethile efumaneka kwiiseli zemast ezingaqhelekanga.
• I-Chemotherapy: Oku kusetyenziswa kuphela xa i-Systemic Mastocytosis iguquke yaba ngumhlaza.
• I-Splenectomy: Ukuba i-spleen idumbile kakhulu, ingasuswa ngotyando.
• Ukufakelwa kwethambo lomongo: Oku kwenzelwa abantu abanesifo esibi kakhulu, okanye abo bakwinqanaba lokugqibela.

Khumbula: Ukuba uneSystemic Mastocytosis, kufuneka uhlale uphethe i-EpiPen . I-EpiPen liyeza elingasetyenziswa ngoko nangoko xa kukho i-allergy enzima, njenge-Anaphylaxis ekhankanyiweyo ngaphambili.

Ngaba le "Systemic Mastocytosis" ayinakuthintelwa?

Njengoko besitshilo ngaphambili, i-"Systemic Mastocytosis" ibangelwa kukuguquka kwezakhi zofuzo. Ngenxa yoko, akunakwenzeka ukuyithintela ukuba ingakhuli. Nangona kunjalo, ungasilawula esi sifo ngokuphepha "izinto ezibangela ukuba iimpawu zikhule."

Luhlobo luni lwekamva lomntu one-`(Systemic Mastocytosis)`?

Oku kuxhomekeke ngokwenene kuhlobo lwe-`(Systemic Mastocytosis)` onalo. Abantu abanohlobo olukhula kancinci (`Indolent systemic mastocytosis`) badla ngokulawula esi sifo kakuhle ngonyango. Banokuphila ubomi obuqhelekileyo. Nangona kunjalo, abantu abanezinye iintlobo ezinzima banokuba nobomi obufutshane kancinci. Nangona kunjalo, `(Systemic Mastocytosis)` banokunyangwa ngokupheleleyo ngonyango olubizwa ngokuba yi-`Bone marrow transplant``. Nangona kunjalo, oogqirha banika olo nyango kuphela kubantu abakwinqanaba elinzima kakhulu lesi sifo.

Ndingazinyamekela njani ngelixa ndiphila ne-`(Systemic Mastocytosis)`?

Ukwazi ukuba yintoni ebangela iimpawu zakho kunye nokuziphepha yeyona nto ibalulekileyo onokuyenza xa uphila neSystemic Mastocytosis. Ukuba unale meko, qaphela ezi zinto:

• Soloko uphethe i-EpiPen. Nyanga ngokukhawuleza xa kukho i-allergy enzima.
• Yazi kakuhle ukuba zeziphi izinto ezikubangelayo kwaye uziphephe kangangoko unako.
• Lawula uxinzelelo. Ziqhelise ukucamngca okanye ezinye iindlela zokuzikisa ingqondo.
• Nxiba isongo sesilumkiso sezonyango esidwelisa amayeza ongenakuwasebenzisa. Oku kubaluleke kakhulu kuba kunceda oogqirha bazi imeko yakho ukuba awukwazi ukuthetha xa kukho imeko engxamisekileyo.

Ndifanele ndimbone nini ugqirha?

Ukuba uneSystemic Mastocytosis, kuya kufuneka ubone ugqirha wakho ukuze ahlolwe rhoqo. Kwakhona, qiniseka ukuba uyamxelela ugqirha wakho ukuba ubonakalisa naziphi na iimpawu ezintsha, okanye ukuba naziphi na iimpawu zakho zibonakala zisiba mandundu.

Ndingayibuza yiphi imibuzo ugqirha?

Ngenxa yokuba i-Systemic Mastocytosis sisifo esingaqhelekanga, usenokuba nemibuzo emininzi. Nazi ezinye zemibuzo onokuzibuza ugqirha wakho:

• Luhlobo luni lwe-"Systemic Mastocytosis" endinalo?
• Ziziphi ezinye iimpawu ezinokubonisa ukuba imeko yam iya isiba mandundu?
• Ziziphi iindlela zonyango ezikhoyo zohlobo lwam?
• Ngaba unyango luza kuziphelisa ngokupheleleyo iimpawu zam?
• Ngaba kukho naziphi na iimvavanyo zeklinikhi endinokuthi ndithathe inxaxheba kuzo?

Ukufunyaniswa une-Systemic Mastocytosis ngamava atshintsha ubomi. Kuya kufuneka ubone ugqirha wakho rhoqo, wenze uvavanyo lwegazi, kwaye ufunde ukuqaphela nokuphepha izinto ezibangela oku. Ukuphila nale meko luhlengahlengiso olukhulu. Ngoko ke, gcina ubudlelwane obuhle nogqirha wakho. Bakho ukukunceda. Baza kukunika ulwazi kunye namaqela enkxaso anokukunceda. Oku kuya kukunika amandla okwenza izigqibo malunga nempilo yakho.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ngoko ke sithethe kakhulu nge-`(Systemic Mastocytosis)`, akunjalo? Khumbula, nangona esi sisifo esingaqhelekanga, kubaluleke kakhulu ukuba usiqaphele.

Eyona nto ibalulekileyo kukubona ugqirha ukuba unale mpawu. Ukuba isifo sifunyenwe kwangethuba, unyango lunokuqaliswa kwaye iimpawu zinokulawulwa.

Awuwedwa. Oogqirha bakho, usapho, kunye nabahlobo bakho bakhona ukukunceda. Ngokwazi ulwazi olufanelekileyo nokulandela icebiso likagqirha wakho, ungaphila kakuhle nale meko. Hlala uzolile kwaye ucinge kakuhle!