Isifo seSickle cell

Isifo seSickle cell sisifo esidluliswa ziintsapho. Iiseli ezibomvu zegazi ezihlala zimile njengediski zithatha irhengqa okanye imilo yecrescent. Iiseli ezibomvu zegazi zithwala ioksijini emzimbeni wonke.

Isifo seSickle cell sibangelwa luhlobo olungaqhelekanga lwehemoglobin ebizwa ngokuba yihemoglobin S.Ihemoglobin yiprotein ngaphakathi kweeseli ezibomvu zegazi ezithwala ioksijini.

• IHemoglobin S itshintsha iiseli ezibomvu zegazi. Iiseli ezibomvu zegazi ziethe-ethe kwaye zimile okwekresenti okanye irhengqa.
• Iiseli ezingaqhelekanga zihambisa ioksijini encinci kwizicubu zomzimba.
• Banokuxinga ngokulula kwimithambo yegazi encinci kwaye baphule iziqwenga. Oku kunokuphazamisa ukuhamba kwegazi okusempilweni kwaye kunqumle nangakumbi kwinani leoksijini engena kwizicwili zomzimba.

Isifo seSickle cell sivela kubazali bobabini. Ukuba ufumana iseli yemfuza yeseli kumzali omnye kuphela, uya kuba neempawu zeseli erhengayo. Abantu abaneempawu zerhengqa abanazo iimpawu zesifo serhengqa.

Isifo seSickle cell sixhaphake kakhulu kubantu abavela eAfrika naseMeditera. Ikwabonwa nakubantu abavela eMzantsi nakuMbindi Merika, kwiCaribbean, nakuMbindi Mpuma.

Iimpawu zihlala zingenzeki kude kube semva kweenyanga ezi-4 ubudala.

Phantse bonke abantu abanesifo se-sickle cell baneziqendu ezibuhlungu ezibizwa ngokuba ziintlekele. Ezi zinokuhlala kwiiyure ukuya kwiintsuku. Iingxaki zinokubangela iintlungu kumqolo osezantsi, emlenzeni, kumalungu kunye nasesifubeni.

Abanye abantu banesiqendu esinye rhoqo kwiminyaka embalwa. Abanye baneziqendu ezininzi ngonyaka. Iintlekele zinokuba nzima ngokwaneleyo ukufuna ukuhlala esibhedlele.

Xa i-anemia iba nzima, iimpawu zingabandakanya:

• Ukudinwa
• Ubumhlophe
• Inqanaba lentliziyo elikhawulezayo
• Ukuqhawukelwa ngumphefumlo
• Ukuthungwa kwamehlo kunye nolusu (i-jaundice)

Abantwana abancinci abanesifo se-sickle cell bahlaselwa zintlungu zesisu.

Ezi mpawu zilandelayo zinokwenzeka ngenxa yokuba imithambo yegazi encinci ivaliwe ziiseli ezingaqhelekanga:

• Ulungiso olubuhlungu kunye nolude (priapism)
• Ukungaboni kakuhle okanye ukungaboni
• Iingxaki ngokucinga okanye ukudideka okubangelwa yimivumbo emincinci
• Izilonda kwimilenze esezantsi (kulutsha nakubantu abadala)

Ixesha elingaphezulu, udakada uyayeka ukusebenza. Ngenxa yoko, abantu abanesifo se-sickle cell banokuba neempawu zosulelo ezinje:

• Usulelo lwe-Bone (osteomyelitis)
• Usulelo lwe-gallbladder (cholecystitis)
• Usulelo lwemiphunga (inyumoniya)
• Usulelo lwe-Urinary tract

Ezinye iimpawu kunye neempawu zibandakanya:

• Ukulibaziseka kokukhula kunye nokufikisa
• Amalungu abuhlungu abangelwa sisifo samathambo
• Intliziyo okanye ukusilela kwesibindi ngenxa yentsimbi eninzi (ukusuka kutofelo-gazi)

Uvavanyo oluqhelekileyo olwenziwe ukuxilonga kunye nokubeka iliso kubantu abanezifo zerhengqa ziquka:

• Bilirubin
• Ukugcwaliswa kweoksijini yegazi
• Gcwalisa ubalo lwegazi (CBC)
• IHemoglobin electrophoresis
• I-serum creatinine
• I-potassium yeSerum
• Uvavanyo lweseli yokugula

Injongo yonyango kukulawula kunye nokulawula iimpawu, kunye nokunciphisa inani leengxaki. Abantu abanesifo se-sickle cell bafuna unyango oluqhubekayo, nokuba bengenangxaki.

Abantu abakule meko kufuneka bathathe izongezo ze-folic acid. I-Folic acid inceda ukwenza iiseli ezibomvu zegazi.

Unyango lwengxaki yeseli erhengayo ibandakanya:

• Utofelo-gazi (lunokunikwa rhoqo ukuthintela ukubetha)
• Amayeza entlungu
• Uninzi lwamanzi

Olunye unyango lwezifo zesigulo zingabandakanya:

• I-Hydroxyurea (Hydrea), enceda ukunciphisa inani leziqendu zentlungu (kubandakanya iintlungu zesifuba kunye neengxaki zokuphefumla) kwabanye abantu
• Amayeza okubulala iintsholongwane, anceda ukuthintela usulelo lwebacteria oluqhelekileyo kubantwana abanesifo serhengqa
• Amayeza anciphisa ubungakanani bentsimbi emzimbeni
• Amayeza amatsha okunciphisa ubungakanani kunye nobukrakra beengxaki zentlungu zivunyiwe

Unyango olunokufuneka ekulawuleni iingxaki zesifo se-sickle cell zibandakanya:

• I-Dialysis okanye ukufakelwa kwezintso kwisifo sezintso
• Iingcebiso malunga neengxaki zengqondo
• Ukususwa kwe-gallbladder kubantu abanesifo se-gallstone
• Ukutshintshwa kwe-Hip ye-avascular necrosis yomlenze
• Utyando lweengxaki zamehlo
• Unyango lokusetyenziswa gwenxa okanye ukusetyenziswa gwenxa kwamayeza entlungu
• Ukunyangwa kwenxeba kwizilonda zomlenze

Umongo wethambo okanye ukufakelwa kweeseli zeziqu kunokunyanga isifo serhengqa, kodwa olu nyango alunakukhethwa ngabantu abaninzi. Abantu abanesifo se-sickle cell bahlala bengenakufumana abaxhasi abaneseli be-stem.

Abantu abanezifo zerhengqa kufuneka babe nogonyo olulandelayo ukunciphisa umngcipheko wosulelo:

• Isitofu sokugonya seHaemophilus influenzae (Hib)
• Ugonyo lwePneumococcal conjugate (PCV)
• Ugonyo lwe-pneumococcal polysaccharide (PPV)

Ukujoyina iqela lenkxaso apho amalungu abelana ngemicimbi eqhelekileyo kunokususa uxinzelelo lwesifo esinganyangekiyo.

Kwixesha elidlulileyo, abantu abane-sickle cell isifo babedla ngokusweleka phakathi kweminyaka engama-20 nengama-40. Ngononophelo lwangoku, abantu ngoku banokuphila ukuya kwiminyaka engama-50 nangaphezulu.

Izizathu zokufa zibandakanya ukusilela komzimba kunye nosulelo.

Shayela umboneleli wakho wezempilo ukuba unayo:

• Naziphi na iimpawu zosulelo (umkhuhlane, iintlungu emzimbeni, intloko ebuhlungu, ukudinwa)
• Iingxaki zentlungu
• Ulwakhiwo olubuhlungu kunye nexesha elide (emadodeni)

Anemia - iseli irhengqa; Isifo seHemoglobin SS (Hb SS); Isifo seanemia

• Iiseli ezibomvu zegazi, iiseli zerhengqa
• Iiseli ezibomvu zegazi-ziqhelekileyo
• Iiseli ezibomvu zegazi-iiseli ezininzi zeerheng
• Iiseli ezibomvu zegazi - iiseli zerhengqa
• Iiseli ezibomvu zegazi - irhengqa kunye nePappenheimer
• Izinto ezenziwe ngegazi
• Iiseli zegazi

Isifo seseli sikaHoward J. Sickle kunye nezinye iihemoglobinopathies. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 154.

Meier ER. Unyango olunokukhethwa kunyango lwesifo selebheli. Iiklinikhi zePediatriki eMantla Am. 2018; 65 (3) 427-443. I-PMID 29803275 ipapashwe.ncbi.nlm.nih.gov/29803275/.

I-National Heart Lung kunye newebhusayithi yeZiko leGazi. Ulawulo olusekwe kubungqina bokugula kwesifo serhengqa: Ingxelo yephaneli yengcali, 2014. www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease. Ukuhlaziywa kukaSeptemba 2014. Kufumaneka ngoJanuwari 19, 2018.

USaunthararajah Y, Vichinsky EP. Isifo seSickle cell: Iimpawu zeklinikhi kunye nolawulo. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 42.

USmith-Whitley K, Kwiatkowski JL. IHemoglobinopathies. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 489.