Cystic fibrosis

Cystic fibrosis chirwere chinokonzera mukobvu, unonamira mamasi kuvaka mumapapu, nzira yekugaya, uye dzimwe nzvimbo dzemuviri. Icho chimwe chezvirwere zvakajairika zvemapapu muvana nevakuru vechidiki. Icho chirwere chinotyisidzira hupenyu.

Cystic fibrosis (CF) chirwere chinopfuudzwa kuburikidza nemhuri. Iyo inokonzerwa neyakaremara geni rinoita kuti muviri uburitse zvisina kujairika gobvu uye inonamira mvura, inonzi mamota. Iyi mhezi inovaka munzvimbo dzekufema dzemapapu uye mupancreas.

Kuwanda kwemakonzo kunoguma nehupenyu hunotyisidzira hutachiona hwemapapu nematambudziko akakomba ekugaya. Chirwere ichi chingakanganisawo magwere eziziwe nehurume hwekubereka.

Vanhu vazhinji vanotakura CF geni, asi havana zviratidzo. Izvi zvinodaro nekuti munhu ane CF anofanirwa kugara nhaka magene maviri asina kukwana, 1 kubva kumubereki mumwe nemumwe. Vamwe vekuAmerican vane CF geni. Izvo zvinowanzoitika pakati peavo vekuchamhembe kana epakati vekuEurope.

Vazhinji vana vane CF vanoonekwa nezera rechipiri, kunyanya sekuongororwa kwevana vachangozvarwa kunoitwa muUnited States. Kune chiverengero chidiki, chirwere hachionekwe kusvika zera gumi nemasere kana kupfuura. Vana ava vanowanzo kuve nechimiro chakareruka chechirwere.

Zviratidzo muvacheche zvinogona kusanganisira:

• Kunonoka kukura
• Kukundikana kuwana uremu zvakajairika panguva yehucheche
• Hapana ura hunofamba mukutanga 24 kusvika 48 maawa ehupenyu
• Ganda rine munyu

Zviratidzo zvine hukama nebasa remukati zvinogona kusanganisira:

• Belly kurwadziwa kubva kwakanyanya kupatira
• Kuwedzera gasi, kuputika, kana dumbu rinoratidzika kunge rakazvimba (rakapararira)
• Nausea uye kurasikirwa kwechido
• Masturu akacheneruka kana kuti ane ruvara rwevhu, kunhuwirira, ane mamasi, kana izvo zvinoyangarara
• Kuonda

Zviratidzo zvine hukama nemapapu uye zvitadzo zvinogona kusanganisira:

• Kukosora kana kuwedzera kamasi muzvitadzo kana mumapapu
• Kuneta
• Kumhanyisa kwemhino kunokonzerwa nemumhino polyps
• Dzokororo yezvikamu zvemabayo (zviratidzo zvebayo mune mumwe munhu ane cystic fibrosis inosanganisira kupisa muviri, kuwedzera kukosora nekufema kwekufema, kuwedzerwa mamasi, uye kurasikirwa nechido)
• Sinus kurwadziwa kana kumanikidzwa kunokonzerwa nehutachiona kana polyps

Zviratidzo zvinogona kucherechedzwa gare gare muhupenyu:

• Kusabereka (mune varume)
• Kudzokorora kuzvimba kwepancreas (pancreatitis)
• Zviratidzo zvekufema
• Yakagerwa minwe

Kuongororwa kweropa kunoitwa kubatsira kuona CF. Muedzo unotarisa shanduko muCF gene. Mimwe bvunzo inoshandiswa kuongorora CF inosanganisira:

• Immunoreactive trypsinogen (IRT) bvunzo yakajairwa bvunzo yekuzvarwa kweCF. Iyo yakakwira mwero we IRT inoratidza inogona CF uye inoda kumwe kuyedzwa.
• Sweat chloride bvunzo ndiyo yakajairwa kuongororwa bvunzo yeCF. Musanganiswa wemunyu mudikita remunhu mucherechedzo wechirwere.

Mimwe miedzo inoratidza matambudziko anogona kunge aine hukama neCF anosanganisira:

• Chest x-ray kana CT scan
• Fecal mafuta bvunzo
• Lung basa bvunzo
• Kuyerwa kwepancreatic basa (chetsoka pancreatic elastase)
• Secretin kukurudzira bvunzo
• Trypsin uye chymotrypsin muzvigaro
• Kumusoro GI uye mudiki ura akateedzana
• Tsika dzemapapu (dzakawanikwa ne sputum, bronchoscopy kana huro swab)

Kuongororwa kwekutanga kweCF uye chirongwa chekurapa kunogona kusimudzira zvese kurarama uye hupenyu hwehupenyu. Kutevera uye kuongorora kwakakosha kwazvo. Pazvinokwanisika, kutarisirwa kunofanirwa kugamuchirwa kune cystic fibrosis yakasarudzika kiriniki. Kana vana vasvika pakuva vanhu vakuru, vanofanirwa kuendesa kune cystic fibrosis yakasarudzika nzvimbo yevakuru.

Kurapa kwematambudziko emapapu kunosanganisira:

• Mishonga inorwisa mabhakitiriya inodzivirira uye yekurapa hutachiona hwemapapu uye sinus. Vanogona kutorwa nemuromo, kana kupihwa mutsinga kana nekurapwa kufema. Vanhu vane CF vanogona kutora mishonga inorwisa mabhakitiriya chete pazvinenge zvichidikanwa, kana nguva dzese. Maitiro anowanzo kuve akakwirira kupfuura akajairwa.
• Inhaled mishonga yekubatsira kuvhura nzira dzekufema.
• Mimwe mishonga inopihwa nemushonga wekufema kuhutete hutete uye kuita kuti zvive nyore kukosora ndeye DNAse enzyme kurapa uye yakanyatsogadziriswa mhinduro yemunyu (hypertonic saline).
• Flu jekiseni uye pneumococcal polysaccharide jekiseni (PPV) gore rega (bvunza wako mupi wezvehutano).
• Kugadzira mapapu ndeimwe sarudzo mune zvimwe zviitiko.
• Oxygen therapy inogona kudiwa sezvo chirwere chemapapu chiri kuwedzera kuipa.

Matambudziko emapapu anorapwa zvakare nemishonga yekurapa kutapudza mhuru. Izvi zvinoita kuti zvive nyore kukosora mamasi kubva mumapapu.

Idzi nzira dzinosanganisira:

• Chiito kana kurovedza muviri zvinoita kuti ufeme zvakadzama
• Midziyo inoshandiswa mukati mezuva kubatsira kubvisa iyo nzira yemumhepo yeakawandisa mamasi
• Chinyorwa chechipfuva kurova (kana chipfuva physiotherapy), umo nhengo yemhuri kana murapi anoombera zvishoma chipfuva chemunhu, musana, nenzvimbo iri pasi pemaoko

Kurapa kwematambudziko uye matambudziko ezvehutano kunogona kusanganisira:

• Kudya kwakakosha kune mapuroteni uye macalorie evana vakura nevakuru
• Pancreatic enzymes kubatsira kutora mafuta uye mapuroteni, ayo anotorwa pamwe nekudya kwese
• Vitamin zvinowedzera, kunyanya mavitamini A, D, E, uye K
• Wako mupi anogona kuraira mamwe marapirwo kana iwe uine zvakaoma zvakanyanya zvituru

Ivacaftor, lumacaftor, tezacaftor, uye elexacaftor mishonga inorapa mamwe marudzi eCF.

• Ivo vanovandudza mashandiro eimwe yeakaremara majini anokonzera CF.
• Kusvika ku90% yevarwere vane CF uye vanokodzera mumwe kana yakawanda yemishonga iyi yega kana musanganiswa.
• Nekuda kweizvozvo, pane kushomeka kwekuvaka kwemukati mamukisi mumapapu. Zvimwe zviratidzo zveCF zvinovandudzika futi.

Kuchengeta uye kuongorora pamba kunofanirwa kusanganisira:

• Kudzivirira utsi, guruva, tsvina, utsi, makemikari epamba, hutsi hwehuni, uye chakuvhuvhu kana chakuvhe.
• Kupa zvinwiwa zvakawanda, kunyanya kune vacheche uye vana munguva inopisa, kana paine manyoka kana zvituru zvisina kusununguka, kana panguva yekuita zvekuita.
• Kuita 2 kana katatu nguva pasvondo. Kushambira, kumhanya, uye kuchovha bhasikoro zvakanaka sarudzo.
• Kuchenesa kana kuunza mamese kana zvakavanzika kubva kunzira dzemumhepo. Izvi zvinofanirwa kuitwa ka1 kana kanomwe pazuva. Varwere, mhuri, uye vanochengeta vanofanirwa kudzidza nezvekuita muchipfuva uye nepashure pemadhiraini kubatsira kuchengetedza nzira dzepamhepo dzakajeka.
• Hapana kusangana nevamwe vanhu vane CF inokurudzirwa sezvo ivo vachigona kuchinjanisa hutachiona (haishande kunhengo dzemhuri).

Unogona kudzikisira kushushikana kwehurwere nekujoinha cystic fibrosis rutsigiro boka. Kugovana nevamwe vane zvakajairika zviitiko uye matambudziko zvinogona kubatsira mhuri yako kuti isazonzwa kusurukirwa.

Vazhinji vana vane CF vanogara muhutano hwakanaka kudzamara vakura. Ivo vanokwanisa kutora chikamu muzviitiko zvakawanda uye kuenda kuchikoro. Vazhinji vechidiki vechidiki vane CF vanopedza koreji kana kuwana mabasa.

Chirwere chemapapu chinopedzisira chichiwedzera kusvika padanho rekuremara. Nhasi, hupenyu hwepakati hwevanhu vane CF vanorarama kusvika pakuva vanhu vakuru ingangoita makore makumi mana nemana.

Rufu runowanzo kukonzerwa nematambudziko emapapu.

Dambudziko rakajairika kusingaperi chirwere chekufema.

Zvimwe zvinetso zvinosanganisira:

• Matambudziko emuviri, akadai sematombo, kuvharwa kwematumbo, uye rectal prolapse
• Kukosora ropa
• Kukundikana kusingaperi kwekufema
• Chirwere cheshuga
• Kushaya mbereko
• Chiropa chirwere kana kutadza kwechiropa, pancreatitis, biliary cirrhosis
• Kushaya zvokudya zvinovaka muviri
• Nasal polyps uye sinusitis
• Osteoporosis uye arthritis
• Mabayo anoramba achidzoka
• Pneumothorax
• Kurudyi-sided moyo kutadza (cor pulmonale)
• Kenza yakajeka

Fonera wako mupi kana mucheche kana mwana ane zviratidzo zveCF, uye zviitiko:

• Fivhiri, kuwedzera kukosora, shanduko mukukosora kana ropa musputum, kurasikirwa nechido, kana zvimwe zviratidzo zvemabayo
• Kuwedzera kuonda
• Kunyanya kuwanda ura kana zvituru zvinonhuwa-zvinonhuwirira kana zvine mamwe mamasi
• Dumbu rakazvimba kana kuwedzera kubhowa

Fonera mupiki wako kana munhu ane CF achiratidzira zviratidzo zvitsva kana zviratidzo zvikatowedzera, kunyanya kunetseka kwakanyanya kwekufema kana kukosora ropa.

CF haigoni kudzivirirwa. Kuongorora avo vane nhoroondo yemhuri yechirwere ichi kunogona kuona iyo CF geni mune vakawanda vanotakura.

CF

• Chikafu chemukati - mwana - kugadzirisa matambudziko
• Gastrostomy yekudyisa chubhu - bolus
• Nzira yekufema kana iwe uchinge uchinge uchishaya mweya wekufema
• Jejunostomy yekudyisa chubhu
• Postural abudise

• Kutamba
• Postural abudise
• Yakagerwa minwe
• Cystic fibrosis

Donaldson SH, Pilewski JM, Griese M, et al. Tezacaftor / ivacaftor mune zvidzidzo zvine cystic fibrosis uye F508del / F508del-CFTR kana F508del / G551D-CFTR. Am J Respir Crit Kuchengeta Med. 2018; 197 (2): 214-224. PMID: 28930490 pubmed.ncbi.nlm.nih.gov/28930490/.

Eagan INI, Schechter MS, Voynow JA. Cystic fibrosis. Mune: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chitsamba 432.

Farrell PM, White TB, Ren CL, et al. Kuongororwa kwecystic fibrosis: mazano ekubvumirana kubva kuCystic Fibrosis Foundation. J Pediatr. 2017; 181S: S4-S15.e1. PMID: 28129811 pubmed.ncbi.nlm.nih.gov/28129811/.

Graeber SY, Dopfer C, Naehrlich L, uye al. Mhedzisiro ye lumacaftor / ivacaftor kurapa paCFTR basa muPhe508del homozygous varwere vane cystic fibrosis. Am J Respir Crit Kuchengeta Med. 2018; 197 (11): 1433-1442. PMID: 29327948 pubmed.ncbi.nlm.nih.gov/29327948/.

Grasemann H. Cystic fibrosis. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chits. 83.

Rowe SM, Hoover W, Soromoni GM, Sorscher EJ. Cystic fibrosis. Mune: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray naNadel Chinyorwa cheKufema Kwemishonga. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016: chits. 47.

Taylor-Cousar JL, Munck A, McKone EF, uye al. Tezacaftor-ivacaftor mune varwere vane cystic fibrosis homozygous ye phe508del. N Engl J Med. 2017; 377 (21): 2013-2023 PMID: 29099344 yakashambadzirwa.ncbi.nlm.nih.gov/29099344/.