Ka Sehloohong >> Thuto Ea Bophelo >> Lefu la selei ke eng (hona le alafshoa joang)?

Lefu la selei ke eng (hona le alafshoa joang)?

Thuto ea Bophelo

Boloetse ba sele (SCD) ke sehlopha sa mathata a mali ao re a futsitseng a amang lisele tse khubelu tsa mali, haholo-holo molek'hule e liseleng tsena tse bitsoang hemoglobin e fanang ka oksijene liseleng ho pholletsa le 'mele. Ho latela Litsi tsa Tlhokomelo le Thibelo ea Maloetse (CDC), boemo bona bo ama batho ba limilione ho pota lefatše, ho kenyeletsoa batho ba hakanyetsoang ho 100,000 United States.

U ba le lefu la seleng ea sekele joang?

Hore motho a tšoaroe ke lefu la seleng ea sekele, batsoali ka bobeli ba tlameha ho fetisetsa mofuta o fosahetseng oa lefutso la hemoglobin ho ngoana oa bona. Haeba motsoali a le mong feela a fetisa lefutso, ngoana o tla ja lefa la sele ea sekele. Sena se bolela hore mali a bona a ka ba le lisele tse itseng tsa sekele, empa hangata ha ba kule.

Bakuli ba lisele tse kulang ba sothile limolek'hule tsa hemoglobin tse otlollang lisele tse khubelu tsa mali hore li be sekele. Lisele tsena li thata, 'me lera la tsona le senyehile ebile le senyeha habonolo. Le ha sele e khubelu ea mali e tloaelehileng e phela ka har'a 'mele ka matsatsi a 120, lisele tsa sekele li phela matsatsi a ka bang 18 kapa 19. Lisele tse kulang li thibela methapo e menyenyane ea mali (capillaries), e liehisang phallo ea mali ho litho tse fapaneng mme e ka baka tšenyo ea setho sa nako e telele.

Le ha ho sa tloaelehang ho hoholo ho lutse kahare ho sele e khubelu, lefu la sekele le fetoha lefu la methapo e mengata Abdullah Kutlar, MD , ngaka e hodileng ya madi le motsamaisi wa Sickle Cell Center Univesithing ya Augusta. Mathata a bongaka a ama litho tsohle tsa 'mele ho tloha hloohong ho isa monwaneng. E amahanngoa le stroke, e ama mahlo, matšoafo, spleen le masapo.

Boloetse ba lisele tsa boloetse bo ama haholo-holo batho bao baholo-holo ba bona ba tsoang Afrika e ka boroa ho Sahara, libaka tse buang Sepanishe Bophirima ba Lefatše (Amerika Boroa, Caribbean le Amerika Bohareng), India le linaheng tse ling tsa Middle East le Mediterranean. Lefu la Sickle cell le etsahala hangata ho batho ba tsoang likarolong tsa lefats'e moo malaria e atileng — ekaba kajeno kapa nakong e fetileng. Batho ba nang le tšobotsi ea sele ea sekele ba na le monyetla o motle oa ho phela khahlanong le malaria.

Boloetse ba lisele tsa boloetse bo hlaha ho motho a le mong ho ba 365 ba hlahang Afrika Boroa; hoo e ka bang lesea le le leng ho ba 13 ba batšo naheng ea Amerika le tsoaloa le na le lisele tsa sekele.

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Mefuta ea mafu a seleng ea sekele

Boloetse ba lefu la sele ke lentsoe le pharaletseng le kenyeletsang mefuta e fapaneng ea boemo. Tsena ke mefuta e tloaelehileng ea SCD:

Sickle cell anemia (HbSS):Batho ba nang le mofuta ona oa SCD ba ja lefa la sele e le 'ngoe ho motsoali ka mong. Sickle cell anemia hangata ke mofuta o tebileng ka ho fetesisa oa lefu la seleng.
Boloetse ba Sickle hemoglobin C (HbSC):Ka mofuta ona oa SCD, phatsa e le 'ngoe ea sekele e futsitsoeng e tsoa ho motsoali a le mong,' me ho e mong, lefutso la hemoglobin e sa tloaelehang e bitsoang C. Hangata ke mofuta o bobebe oa SCD.
Sickle beta thalassemia (HbS beta thalassemia):Batho ba nang le mofuta ona oa SCD ba rua lefa e le 'ngoe ea sekele ho motsoali a le mong le liphatsa tsa lefutso tsa beta thalassemia ( mofuta wa phokolo ya madi ) ho tsoa ho motsoali e mong. Ho na le mefuta e 'meli ea beta thalassemia: 0 le +. Hbs beta 0-thalassemia ke mofuta o matla haholo oa lefu la sekele, ha Hbs beta + e le bobebe.

Matšoao a lefu la seleng ea sekele

Batho ba nang le lefu la seleng ea sekele hangata ba qala ho bontša matšoao a boemo ba bona ba sa le banyenyane, ba ka ba likhoeli tse hlano. Masea a ka tlase ho lilemo tsena ha a bontše matšoao hobane hemoglobin ea 'fetal' e sireletsa lisele tsa eona tse khubelu tsa mali ho tsoa ho sickling. Ha li ntse li tsofala, hemoglobin ea sekele e nkela sebaka mofuta oa lesea le ka pōpelong.

Matšoao le mathata a SCD a fapane ho motho e mong le e mong mme a ka fapana ho tloha bobeng ho ea ho matla. Bothata bo atileng haholo ba lefu la seleng ea bohloko ke bohloko bo bohloko, bo bitsoang linako tsa bohloko kapa mathata a bohloko, a ka bang bobebe ho isa ho a boima. Ho etsahala ha lisele tsa sekele li feta methapong e menyenyane ea mali, li tšoarella, li thibele phallo ea mali. Sena se bitsoa maqakabetsi a sele ea sekele 'me ke lona sesosa se atileng haholo sa ho amoheloa sepetlele har'a bakuli ba SCD. Mathata a mang a kenyelletsa:

Lefu la maoto a maoto: Ho ruruha hona matsohong le maotong hangata ke matšoao a pele a SCD.
Khaello ea maliHo hloka lisele tse khubelu tsa mali tse phetseng hantle ho jara oksijene ho baka phokolo ea mali e matla moo batho ba nang le SCD ba tla ikutloa ba khathetse, ba tsekela kapa ba le hlooho e bobebe, mme ba thatafalloa ke ho hema maemong a boima.
Tšoaetso :Batho ba nang le SCD, haholo-holo bana ba banyenyane, ba kotsing e kholo ea tšoaetso ka lebaka la tšenyo ea spleen.
Lefu la sefuba le bohloko :Boemo bona bo shebahala ebile bo ikutloa bo tšoana le pneumonia e nang le bohloko ba sefuba, ho khohlela, ho hema ka thata le feberu. E ka beha bophelo kotsing.
Tlhaselo ea Splenic :Boemo bona bo sokelang bophelo bo etsahala ha palo e kholo ea lisele tsa sekele e tšoasoa ke spleen ebe e etsa hore e holise.
Liso tsa maoto :Tsietsi, ts'oaetso, ho ruruha le ho se tsamaee hantle methapong ea mali ho baka lisosa karolong e ka tlase ea leoto.

Boloetse ba sekele ea sekele bo fumanoa joang?

Teko ea mali ke eona feela tsela ea ho fumana lefu la sele ea sekele kapa ho fumana hore na motho o na le lefutso la sele ea sekele eo a ka e fetisetsang ho ngoana.

Mofani oa tlhokomelo ea bophelo o tla kha mali ho tsoa mothapong oa letsoho ho batho ba baholo, le monoana kapa serethe maseeng le baneng ba banyenyane. Naheng ea Amerika, masea a sa tsoa tsoaloa a lula a hlahlojoa mafu a sekele. Kamora hore mali a huloe, laboraka e ntša sampole bakeng sa hemoglobin e nang le sekoli.

Ka makhetlo a mangata, batho ba fumanoang ba jere lisele tsa sekele ba isoa ho moeletsi oa liphatsa tsa lefutso ea thusang bakuli le ba malapa a bona ho utloisisa tlhaiso-leseling ea lefutso le hore e ama tlhokomelo ea bona ea bongaka kapa ho laola maemo a bona.

Na sele ea sekele e ka phekoloa?

Ho phela ka lefu la sekele e ka ba phephetso, empa ho ntse ho ka etsahala hore o be le bophelo bo phethahetseng le bo phetseng hantle. Lilemong tse 20 ho isa ho tse 30 tse fetileng, lipatlisiso tsa bongaka, tlhokomelo le thuto mabapi le lefu la sele ea sekele li thusitse ho ntlafatsa pono ea batho ba nang le boemo bona.

Palo ea lefu la SCD ho batho ba bacha e na le e theohile haholo Lilemong tse mashome a 'maloa tse fetileng, ho tloha ho 9.3% ka 1975 ho fihlela 2.6% ka 1989 . HO boithuto ba morao-rao e bonts'a palo ea mafu a bana ba Maafrika a Amerika a theohileng ka 68% nakong ea lilemo tse 16. Bafuputsi ba re phokotso e entsoe ke ente ea pneumococcal e lokollotsoeng ka 2000. Batho ba nang le SCD ha ba sireletsoe hanyane mafung a tebileng le a bolaeang a kang nyumonia.

Le ha bana ba nang le lefu la sekele ba ntse ba phela nako e telele, boemo bona bo ntse bo hokahane le a Nako e khuts'oane ea bophelo ho batho ba baholo . Phuputso e bonts'a karolelano ea nako ea bophelo ke lilemo tse 42 bakeng sa basali le lilemo tse 38 bakeng sa banna.

Le ha tlhokomelo ea bana e potlakile ka litsi tse ncha tsa kalafo le lingaka tse mafolofolo, bakuli ba baholo ba ntse ba tobana le likheo tsamaisong ea tlhokomelo ea bophelo.

Kamora hore motho ea nang le sekele a fihle ho lilemo tse 18 kapa 19, o tlameha ho tloha sebakeng sa bana, mme ka bomalimabe ke moo batho ba bang ba oang selomong, ho bolela Barbara Harrison, moeletsi oa liphatsa tsa lefutso le motsamaisi oa Community Outreach and Education for Setsi sa Mafu a Sickle Cell Sepetleleng sa Howard University. Ha ho na litsebi tsa mafu a likokoana-hloko tse nang le tsebo tse lekaneng tse koetliselitsoeng mafu a sekele 'me li tseba mathata a batho ba baholo.

Phephetso e 'ngoe: lichelete tsa lipatlisiso. Lefu la boloetse ba boloetse ke e 'ngoe ea maloetse a kotsi ka ho fetisisa liphatseng tsa lefutso ho la U.S. Leha ho le joalo, lea amohela chelete e tlase haholo ea lipatlisiso ho feta mathata a mang a liphatsa tsa lefutso joalo ka cystic fibrosis, phapang e bang molato ho khethollo ea morabe litabeng tsa bophelo bo botle. Motheo oa limolek'hule le liphatsa tsa lefutso bakeng sa lefu la sekele e ne e le e 'ngoe ea tsa pele tse fumanoeng maloetseng a batho, leha ho le joalo, re ntse re le morao haholo mabapi le kalafo le mehato e nkiloeng ho thusa ho folisa boemo boo, Harrison o re.

Hona joale, ho kenyelletsa sele ea stem (eo hape e bitsoang ho kenyelletsa 'moko oa masapo) ke eona feela pheko ea lefu la sele ea sele. Nakong ea ts'ebetso ena, lingaka li kenya lisele tse phetseng hantle (li-stem cell) 'meleng ho nka sebaka sa moko oa masapo o senyehileng. 'Mele o etsa lisele tsa mali masong a masapo bohareng ba lesapo. Hangata, lingaka li sebelisa ho fetisetsa mali le 'moko ho tsoa ho bafani ba phetseng hantle ho folisa SCD, empa bakuli ba hloka mofani ho tsoa ho motho ea nang le moko oa masapo o tšoanang. Lisebelisoa ka linako tse ling li ka baka litla-morao tse tebileng esita le lefu, 'me ha li fumanehe hohle ka ntle ho US le Europe.

Pheko e 'ngoe e ka bang teng haufinyane ke phekolo ea liphatsa tsa lefutso . Ho kenyelletsa ho tlosa lisele tse bakoang tse etsang mali le lisele tsa 'mele tsa ho itšireletsa mafung ho tsoa mokong oa masapo kapa mali le ho eketsa lefutso le nang le phoso ho batho ba nang le lefu la sele ea sekele. Ka mor'a moo lingaka li khutlisetsa lisele ho mokuli, e leng se lebisang 'meleng ho hlahisa hemoglobin ea anti-sickling. Bafuputsi ba ntse ba etsa liteko tsa bongaka, empa liphetho tsa pele li shebahala li ts'episa.

Hajoale, meriana e sebetsang e bohlokoa. Re hloka litlhare tse ling ha re ntse re sebetsa ka litheknoloji tse phahameng, tse nang le lisebelisoa tse phahameng tse kang ho kenya lisele tsa STEM le kalafo ea liphatsa tsa lefutso, Dr. Kutlar o re.

Phekolo ea lisele tse kulang

Mohato oa pele oa ho phela bophelo bo bolelele le SCD ke ho fumana tlhokomelo ea boleng bo phahameng ho tsoa ho litsebi tsa boemo bona. Joale, meriana e nepahetseng bakeng sa matšoao a hau a ikhethang.

Hydroxyurea

Kalafo e tloaelehileng ea lefu la seleng ea sekele ke hydroxyurea , meriana eo bafani ba tlhokomelo ea bophelo ba neng ba e sebelisa pele ho phekola mofetše. Batho ba nang le SCD ba nka tekanyetso e nyane, 'me e matlafatsa hemoglobin ea lesea ha e ntse e thibela mathata a mangata a boemo-ho theola likoluoa tsa lisele tsa sekele, ho ea sepetlele, liketsahalo tsa pneumonia le tlhoko ea litšelo tsa mali.

Lilemong tsa morao tjena, lik'hamphani tsa meriana li se li thahasella ho theha mekhoa e mecha ea kalafo bakeng sa lefu la seleng ea sekele. Karolo e 'ngoe e bakoa ke khaello ea kalafo, mme hobane lefu la sekele ke mohlala oa ho phekola maemo a mang a kang ho ruruha le maloetse a methapo ea mali.

Endari (L-glutamine phofo ea molomo)

Ho tloha ka 2017, the Tsamaiso ea Tsamaiso ea Lijo le Lithethefatsi ea U.S. (FDA) e amohetse kalafo e ncha bakeng sa lefu la sekele, e leng ea pele lilemong tse mashome. Endari ( L-glutamine phofo ea molomo ) ke ea batho ba nang le SCD ba lilemo li 5 le ho feta. Lithethefatsi, phofo e nkiloeng ka molomo, e sebetsa e le antioxidant ho fokotsa mathata a tebileng a amanang le boemo boo. Bafuputsi ba fumane hore batho ba nkang Endari ba na le likoluoa tse fokolang tsa lisele tsa sekele le maeto a ho ea sepetlele, hape ba lula nako e khuts'oane sepetlele.

Oxbreaker (voxelotor)

Ho 2019, tumello ea FDA e fumanehang ka potlako bakeng sa Mokelikeli (voxelotor), moriana oa pele oa ho phekola lisosa tsa anemia ea sickle cell ho fapana le matšoao a eona feela. Ka Oxbryta, lisele tsa sekele ha li na monyetla oa ho tlamahana le ho etsa sebopeho sa sekele, se ka bakang maemo a tlase a hemoglobin ka lebaka la tšenyo ea lisele tse khubelu tsa mali, Richard Pazdur, MD, motsamaisi oa Oncology Center of Excellence ea FDA (OCE) o itse ho phatlalatso ea litaba e phatlalatsang kalafo . Kalafo ena e fana ka khetho e ncha ea kalafo bakeng sa bakuli ba nang le boemo bona bo tebileng le bo sokelang bophelo. Oxbryta ke ea bana le batho ba baholo ba lilemo li 12 le ho feta.

Adakveo (crizanlizumab-tmca)

FDA e amohetse sethethefatsi se seng ho elella bofelong ba 2019 , Adakveo (crizanlizumab-tmca), e theolang palo ea likoluoa tsa lisele tsa sekele ho batho ba baholo le bana ba ka holimo ho lilemo tse 16. Meriana ke ente, eo batho ba nang le SCD ba e fuoang nako le nako, libeke tse ling le tse ling tse peli pele ebe ba ea ho eona hang ka khoeli.

Sickle Cell Center ea Augusta University e ne e le karolo ea teko ea naha ena bakeng sa kalafo ena, mme Dr. Kutlar o re karabelo ea bakuli e ne e le ntle haholo. Kamora hore thuto e qete, bakuli ba 'maloa ba ile ba tla ho rona mme ba re,' Ke ne ke ikutloa ke le betere haholo ha ke ne ke fumana infusions tseo. Na ho na le tsela eo u ka mpusetsang ho eona? 'Kahoo, ka bokhutšoanyane, eo e bile boiphihlelo bo nepahetseng ho latela pono ea mokuli.

Kalafo ea maloetse a sele
Lebitso la lithethefatsi	Sehlopha sa lithethefatsi	Tsela ea Tsamaiso	Tekanyo e tloaelehileng	Litla-morao	Fumana setlankane
Hydroxyurea (Droxia, Hydrea, Siklos)	Antimetabolite	Molomo	K'hapsule e le 'ngoe ea 200-400 mg ka letsatsi	Ho tsekela, ho otsela, bohloko ba mpeng, ho sokela, letšollo, ho nyekeloa ke pelo, ho hlatsa le ho felloa ke takatso ea lijo	Fumana setlankane
L-glutamine phofo ea molomo Endari )	Asiti ea Amino	Molomo	10-30 dikgerama habeli ka letsatsi e kopantsoe le lijo kapa lino	Ho sokela, ho nyekeloa ke pelo, ho opeloa ke hlooho, bohloko ba mpeng, ho khohlela, ho opeloa ke bohloko bo boholo, mokokotlo le bohloko ba sefuba	Fumana setlankane
Voxelotor (Oxbryta)	Hemoglobin oksijene-kamano ea modulator	Molomo	Letlapa le le leng la 1,500 mg ka letsatsi le nkiloeng le se na lijo	Hlooho, letšollo, bohloko ba mpeng, ho nyekeloa ke pelo, mokhathala, ho phatloha le feberu	Fumana setlankane
Crizanlizumab-tmca (Metsi)	P-selectin inhibitor	Ente	Liente tsa 5 mg libekeng tsa 0 le 2, ebe libeke tse ling le tse ling tse 4	Ho nyekeloa ke pelo, bohloko ba manonyeletso, bohloko ba mokokotlo le feberu	Ithute haholoanyane

Batho ba nang le maloetse a likokoana-hloko le bona ba nka li-counter kapa meriana ea ngaka bakeng sa bohloko bo sa foleng 'me e ka hloka litšelo tsa mali ho phekola mathata a kang phokolo ea mali kapa tšoaetso.

Lisebelisoa tsa lisele tse kulang

Khonkerese ea United States e khethile Loetse e le Khoeli ea Naha ea Tlhokomeliso ea Lisele tsa Sickle ho thusa ho tlisa tlhokomeliso lipatlisisong le kalafong ea lefu la lisele tsa sekele. Ho ithuta haholoanyane ka boemo bona, etela: