Isifo se-Sickle cell

Isifo se-Sickle cell yisifo esidluliselwa emindenini. Amaseli abomvu egazi ajwayele ukwakheka njengediski athatha isikela noma ukwakheka kocezu. Amaseli abomvu egazi athwala umoya-mpilo emzimbeni wonke.

Isifo se-Sickle cell sibangelwa uhlobo olungajwayelekile lwe-hemoglobin okuthiwa i-hemoglobin S. Hemoglobin yiprotheni ngaphakathi kwamaseli abomvu egazi athwala umoya-mpilo.

• IHemoglobin S iguqula amangqamuzana abomvu egazi. Amaseli abomvu egazi abuthaka futhi abumbe njengama-crescent noma amasikela.
• Amaseli angajwayelekile aletha umoya-mpilo omncane ezicutshini zomzimba.
• Bangakwazi futhi ukubambeka kalula emithanjeni yegazi emincane bese bephuka babe yizicucu. Lokhu kungaphazamisa ukugeleza kwegazi okunempilo futhi kunciphise nangaphezulu inani leoksijini eligelezela ezicutshini zomzimba.

Isifo se-Sickle cell sitholwa ngabazali bobabili. Uma uthola ufuzo lweseli lesikela kumzali oyedwa kuphela, uzoba nesici sesikela lesikela. Abantu abanesici se-sickle cell abanazo izimpawu zesifo se-sickle cell.

Isifo se-Sickle cell sivame kakhulu kubantu abavela e-Afrika naseMedithera. Kubuye kubonakale kubantu abavela eNingizimu Melika naseMelika Ephakathi, eCaribbean naseMpumalanga Ephakathi.

Izimpawu ngokuvamile azenzeki kuze kube ngemuva kwezinyanga ezine ubudala.

Cishe bonke abantu abanesifo se-sickle cell baneziqephu ezibuhlungu ezibizwa ngezinhlekelele. Lokhu kungahlala kusuka emahoreni kuye ezinsukwini. Izinkinga zingadala ubuhlungu emhlane ongezansi, emlenzeni, emajoyintini nasesifubeni.

Abanye abantu banesiqephu esisodwa njalo eminyakeni embalwa. Abanye baneziqephu eziningi unyaka ngamunye. Izinkinga zingaba nzima ngokwanele ukudinga ukuhlala esibhedlela.

Lapho i-anemia iba namandla kakhulu, izimpawu zingafaka:

• Ukukhathala
• Ukudinwa
• Ukushaya kwenhliziyo okusheshayo
• Ukuphelelwa umoya
• Ukuphuzi kwamehlo nesikhumba (i-jaundice)

Izingane ezincane ezinesifo se-sickle cell zihlaselwa ubuhlungu besisu.

Izimpawu ezilandelayo zingavela ngoba imithambo yegazi emincane ivinjwe amaseli angajwayelekile:

• Ukwakhiwa okubuhlungu nokuhlala isikhathi eside (priapism)
• Ukungaboni kahle noma ukungaboni
• Izinkinga zokucabanga noma ukudideka okubangelwa yimivimbo emincane
• Izilonda emilenzeni engezansi (kwentsha nakubantu abadala)

Ngokuhamba kwesikhathi, ubende luyeka ukusebenza. Ngenxa yalokhu, abantu abanesifo se-sickle cell bangaba nezimpawu zezifo ezinjenge:

• Ukutheleleka kwamathambo (i-osteomyelitis)
• Ukutheleleka kwe-gallbladder (cholecystitis)
• Ukutheleleka kwamaphaphu (i-pneumonia)
• Ukutheleleka ngomgudu womchamo

Ezinye izimpawu nezimpawu zifaka:

• Ukukhula okubambezelekile nokuthomba
• Amalunga abuhlungu abangelwa yisifo samathambo
• Ukuhluleka kwenhliziyo noma kwesibindi ngenxa yensimbi eningi (kusuka ekumpontshelweni igazi)

Ukuhlolwa okuvame ukwenziwa ukuthola nokuqapha abantu abanezifo ze-sickle cell kufaka:

• I-Bilirubin
• Ukugcwaliswa komoya-mpilo wegazi
• Qedela ukubalwa kwegazi (CBC)
• I-hemoglobin electrophoresis
• I-serum creatinine
• I-potassium yeSerum
• Ukuhlolwa kwamaseli okugula

Inhloso yokwelashwa ukuphatha nokulawula izimpawu, nokunciphisa inani lezinkinga. Abantu abanesifo se-sickle cell badinga ukwelashwa okuqhubekayo, noma ngabe abanankinga.

Abantu abanalesi simo kufanele bathathe izithasiselo ze-folic acid. I-Folic acid isiza ekwenzeni amangqamuzana abomvu egazi amasha.

Ukwelashwa kwenkinga yeseli yesikela kubandakanya:

• Ukumpontshelwa igazi (kungabuye kunikezwe njalo ukuvimbela isifo sohlangothi)
• Imithi yezinhlungu
• Iziphuzo eziningi

Ezinye izindlela zokwelapha isifo se-sickle cell zingafaka:

• I-Hydroxyurea (Hydrea), esiza ukunciphisa inani leziqephu zobuhlungu (kufaka phakathi ubuhlungu besifuba nezinkinga zokuphefumula) kwabanye abantu
• Ama-antibiotic, asiza ukuvimbela ukutheleleka kwamagciwane okuvamile ezinganeni ezine-sickle cell disease
• Imithi eyehlisa inani lensimbi emzimbeni
• Izindlela zokwelashwa ezintsha zokunciphisa imvamisa nobukhulu bezinhlungu ezibuhlungu zivunyiwe

Ukwelashwa okungadingeka ukuphatha izinkinga zesifo se-sickle cell kufaka:

• I-Dialysis noma ukufakelwa izinso kwesifo sezinso
• Ukwelulekwa ngezinkinga ezingokwengqondo
• Ukususwa kwe-gallbladder kubantu abanesifo senyongo
• Ukushintshwa kwe-Hip kwe-avascular necrosis ye-hip
• Ukuhlinzwa ngezinkinga zamehlo
• Ukwelashwa kokusetshenziswa ngokweqile noma ukuhlukunyezwa kwemithi yobuhlungu be-narcotic
• Ukulimala kwezilonda zomlenze

Ukufakwa komnkantsha wamathambo noma ukuvela kwamaseli we-stem kungelapha isifo se-sickle cell, kepha lokhu kwelashwa akuyona inketho kubantu abaningi. Abantu abanesifo se-sickle cell imvamisa abakwazi ukuthola abanikeli abalingani be-stem cell.

Abantu abanesifo se-sickle cell kufanele babe nale mijovo elandelayo yokwehlisa ubungozi bokutheleleka:

• Umuthi wokugoma we-Haemophilus influenzae (Hib)
• Umuthi wokugoma we-pneumococcal conjugate (PCV)
• Umuthi wokugoma we-pneumococcal polysaccharide (PPV)

Ukujoyina iqembu lokusekelwa lapho amalungu abelana ngezinkinga ezivamile kunganciphisa ukucindezeleka kwesifo esingelapheki.

Esikhathini esedlule, abantu abanesifo se-sickle cell babevame ukufa phakathi kweminyaka engama-20 nengama-40. Ngenxa yokunakekelwa kwanamuhla, abantu manje bangaphila iminyaka engama-50 nangaphezulu.

Izimbangela zokufa zibandakanya ukwehluleka komzimba nokutheleleka.

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma une:

• Noma yiziphi izimpawu zokutheleleka (umkhuhlane, ubuhlungu bomzimba, ikhanda elibuhlungu, ukukhathala)
• Izinkinga zezinhlungu
• Ukwakhiwa okubuhlungu nokwesikhathi eside (emadodeni)

I-anemia - iseli lesikela; Isifo se-Hemoglobin SS (Hb SS); I-sickle cell anemia

• Amaseli abomvu egazi, iseli lesikela
• Amaseli abomvu egazi - ejwayelekile
• Amaseli abomvu egazi - amaseli amaningi amasikela
• Amaseli abomvu egazi - amangqamuzana amasikela
• Amaseli abomvu egazi - isikela nePappenheimer
• Izakhi ezakhiwe zegazi
• Amaseli egazi

Isifo samangqamuzana kaHoward J. Sickle namanye ama-hemoglobinopathies. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 154.

Meier ER. Izinketho zokwelashwa kwesifo se-sickle cell. Umtholampilo Wezingane eNyakatho Am. I-2018; 65 (3) 427-443. I-PMID 29803275 pubmed.ncbi.nlm.nih.gov/29803275/.

I-National Heart Lung ne-Blood Institute iwebhusayithi. Ukuphathwa okusekelwe ebufakazini besifo se-sickle cell: umbiko wephaneli lochwepheshe, 2014. www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease. Kubuyekezwe uSepthemba 2014. Kufinyelelwe ngoJanuwari 19, 2018.

USaunthararajah Y, Vichinsky EP. Isifo seSickle cell: izici zokwelashwa kanye nokuphathwa. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 42.

USmith-Whitley K, Kwiatkowski JL. Ama-hemoglobinopathies. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 489.