Cystic fibrosis

Cystic fibrosis cuta ce da ke haifar da dattin ciki mai kauri a cikin huhu, hanyar narkar da abinci, da sauran bangarorin jiki. Yana daya daga cikin cututtukan huhu na yau da kullun ga yara da matasa. Cuta ce ta barazanar rai.

Cystic fibrosis (CF) cuta ce da ke sauka ta cikin iyalai. Hakan na faruwa ne ta sanadiyyar kwayar halitta wacce ke sanya jiki samar da ruwa mai kauri mara kyau mara kyau, wanda ake kira gamsai. Wannan lakar tana toshewa a cikin hanyoyin numfashi na huhu da kuma cikin pancreas.

Yawan gamsai yana haifar da cututtukan huhu masu barazanar rai da matsaloli masu narkewa. Haka kuma cutar na iya shafar glandon gumi da tsarin haihuwa na mutum.

Mutane da yawa suna ɗaukar kwayar CF, amma ba su da alamun bayyanar. Wannan saboda mutum mai cutar CF dole ne ya gaji kwayoyin cuta 2, 1 daga kowane mahaifa. Wasu Amurkawa suna da kwayar CF. Ya fi yawanci tsakanin waɗanda ke asalin arewacin ko tsakiyar Turai.

Yawancin yara da ke da CF ana bincikar su ne daga shekara 2, musamman yayin da ake yin gwajin haihuwa a duk faɗin Amurka. Don karamin lamba, ba a gano cutar har sai ya kai shekara 18 ko sama da haka. Wadannan yara galibi suna da saukin cutar.

Kwayar cututtuka a cikin jarirai na iya haɗawa da:

• Rashin jinkiri
• Rashin samun karin nauyi kullum yayin yarinta
• Babu motsawar hanji a farkon awa 24 zuwa 48 na rayuwa
• Fata mai dandano mai gishiri

Kwayar cutar da ke da alaƙa da aikin hanji na iya haɗawa da:

• Ciwon ciki daga tsananin maƙarƙashiya
• Gasara yawan gas, kumburin ciki, ko ciki wanda ya bayyana kumbura (an ba shi)
• Tashin zuciya da rashin cin abinci
• Kujerun da suke launuka ne masu launi ko launuka, ƙamshi mai daɗi, suna da ƙanshi, ko kuma suna iyo
• Rage nauyi

Kwayar cutar da ke da alaƙa da huhu da sinus na iya haɗawa da:

• Tari ko ƙaruwa a cikin sinus ko huhu
• Gajiya
• Cutar hanci da hanci ya haifar da polyps na hanci
• Maimaitattun maganganu na ciwon huhu (alamun cututtukan huhu a cikin wanda ke da ƙwayar cuta ta ciki sun haɗa da zazzaɓi, ƙarar tari da ƙarancin numfashi, ƙarar gamsai, da ƙarancin abinci)
• Sinus zafi ko matsin lamba sanadiyyar kamuwa da cuta ko polyps

Kwayar cututtukan da za a iya lura da su daga baya a rayuwa:

• Rashin haihuwa (a cikin maza)
• Maimaita kumburi na pancreas (pancreatitis)
• Alamun numfashi
• Yatsun kafa

Ana yin gwajin jini don taimakawa gano CF. Jarabawar tana neman canje-canje a cikin kwayar CF. Sauran gwaje-gwajen da aka yi amfani da su don tantance CF sun haɗa da:

• Immunoreactive trypsinogen (IRT) gwajin gwaji ne na daidaitaccen haihuwa don CF. Babban matakin IRT yana nuna yiwuwar CF kuma yana buƙatar ƙarin gwaji.
• Gwaji chloride gwajin shine daidaitaccen gwajin cutar CF. Babban gishiri a cikin gumin mutum alama ce ta cutar.

Sauran gwaje-gwajen da ke gano matsalolin da zasu iya alaƙa da CF sun haɗa da:

• Kirjin x-ray ko CT scan
• Gwajin mai kiba
• Gwajin aikin huhu
• Gwajin aikin pancreatic (stool pancreatic elastase)
• Gwajin gwaji na sirri
• Trypsin da chymotrypsin a cikin kujeru
• Babban GI da ƙananan jerin hanji
• Al'adun huhu (wanda aka samo ta sputum, bronchoscopy ko swab makogwaro)

Sanarwar asali na CF da shirin kulawa na iya inganta rayuwa da ingancin rayuwa. Kulawa da sa ido suna da matukar mahimmanci. Idan ya yiwu, ya kamata a samu kulawa a asibitin musamman na cystic fibrosis. Lokacin da yara suka balaga, ya kamata su canza zuwa cibiyar keɓaɓɓiyar cystic fibrosis don manya.

Jiyya don matsalolin huhu sun haɗa da:

• Maganin rigakafi don hanawa da magance cututtukan huhu da sinus. Ana iya ɗaukar su ta baki, ko a ba su a cikin jijiyoyi ko kuma ta hanyar maganin numfashi. Mutanen da ke da CF na iya ɗaukar maganin rigakafi kawai lokacin da ake buƙata, ko koyaushe. Allurai sun fi yawa fiye da al'ada.
• Magunguna masu shaƙa don taimakawa buɗe hanyoyin iska.
• Sauran magunguna da ake bayarwa ta hanyar numfashiwa zuwa laushin bakin ciki kuma yana sauƙaƙa tari shi ne maganin enzyme na DNAse da haɓakar gishiri mai ƙarfi (sarkar hawan jini)
• Alurar rigakafin mura da pneumococcal polysaccharide (PPV) kowace shekara (tambayi mai ba da lafiyar ku).
• Dasawar huhu wani zaɓi ne a wasu yanayi.
• Ana iya buƙatar maganin oxygen a yayin da cutar huhu ke ƙara muni.

Hakanan ana magance matsalolin huhu tare da hanyoyin kwantar da bakin. Wannan yana sa a sauƙaƙa tari tari na huhu.

Wadannan hanyoyin sun hada da:

• Aiki ko motsa jiki wanda ke sa ku numfasawa sosai
• Na'urorin da ake amfani dasu yayin rana don taimakawa share hanyoyin iska daga yawan gamsai
• Bugun kirji na hannu (ko kuma ilimin lissafi na kirji), wanda memba na iyali ko mai ilimin hanyoyin kwantar da hankali suna tafa kirjin mutum, baya, da yanki a ƙarƙashin makamai

Jiyya don hanji da matsalolin abinci mai gina jiki na iya haɗawa da:

• Abinci na musamman wanda ya kunshi furotin da adadin kuzari don yara da manya
• Enzymes na Pancreatic don taimakawa sha mai da furotin, waɗanda ake ɗauka tare da kowane abinci
• Kayan bitamin, musamman bitamin A, D, E, da K
• Mai ba ku sabis zai iya ba da shawara ga sauran jiyya idan kuna da ɗakuna masu wuya

Ivacaftor, lumacaftor, tezacaftor, da elexacaftor su ne magunguna waɗanda ke kula da wasu nau'ikan CF.

• Suna inganta aikin ɗayan lalatattun kwayoyin halitta da ke haifar da CF.
• Har zuwa 90% na marasa lafiya tare da CF kuma sun cancanci ɗayan ko fiye daga waɗannan magungunan su kaɗai ko a hade.
• A sakamakon haka, akwai ƙarancin ƙarancin dattin ciki a cikin huhu. Sauran cututtukan CF sun inganta kuma.

Kulawa da kulawa a gida yakamata su haɗa da:

• Guje wa hayaƙi, ƙura, datti, hayaƙi, sinadaran gida, hayaƙin murhu, da ƙyalli ko fumfuna.
• Bayar da ruwa mai yawa, musamman ga jarirai da yara a cikin yanayin zafi, lokacin da ake gudawa ko kujerun mara, ko yayin karin motsa jiki.
• Motsa jiki sau 2 ko 3 kowane mako. Iyo, tseren guje guje, da keke su ne zaɓuɓɓuka masu kyau.
• Sharewa ko kawo gamsai ko ɓoyewa daga hanyoyin iska. Dole ne ayi hakan sau 1 zuwa 4 kowace rana. Marasa lafiya, iyalai, da masu kulawa dole ne su koya game da buga kirji da magudanan ruwa don taimakawa tsaftace hanyoyin iska.
• Babu wata ma'amala da wasu mutane masu CF wanda aka ba da shawarar saboda suna iya musayar cututtuka (ba ya shafi membobin gidan).

Kuna iya sauƙaƙa damuwar rashin lafiya ta hanyar haɗuwa da ƙungiyar tallafi na cystic fibrosis. Yin tarayya da wasu waɗanda suke da masaniya da matsaloli na yau da kullun na iya taimaka wa dangin ku kada su ji da kansu.

Yawancin yara masu cutar CF suna kasancewa cikin koshin lafiya har sai sun balaga. Suna iya shiga cikin yawancin ayyukan kuma suna halartar makaranta. Yawancin samari da yawa tare da CF sun gama kwaleji ko samun ayyuka.

Cutar huhu daga ƙarshe tana ta'azzara har ta kai ga mutum ya naƙasa. A yau, matsakaiciyar rayuwar mutanen da ke tare da CF waɗanda ke rayuwa zuwa girma kusan shekaru 44 ne.

Yawanci ana samun mutuwa ne saboda rikicewar huhu.

Rikicin mafi yawan mutane shine kamuwa da cutar numfashi na yau da kullun.

Sauran matsalolin sun hada da:

• Matsalar hanji, kamar duwatsun gall, toshewar hanji, da ɓarkewar dubura
• Tari da jini
• Rashin numfashi na kullum
• Ciwon suga
• Rashin haihuwa
• Ciwon hanta ko gazawar hanta, pancreatitis, biliary cirrhosis
• Rashin abinci mai gina jiki
• Hancin hanci da sinusitis
• Osteoporosis da amosanin gabbai
• Ciwon huhu da ke ci gaba da dawowa
• Pneumothorax
• Bugun zuciya mai dama (cor pulmonale)
• Cutar kansa

Kira mai ba ku sabis idan jariri ko yaro yana da alamun CF, da gogewa:

• Zazzaɓi, ƙarar tari, canje-canje a cikin ruwan azzakari ko jinni a cikin fitsarin, rashin cin abinci, ko wasu alamun ciwon huhu
• Lossara asarar nauyi
• Movementsarin yawan hanji ko kujerun hanji waɗanda ke wari ko kuma suna da ƙoshi
• Ciki ya kumbura ko kumburin ciki

Kirawo mai ba da sabis idan mutumin da ke da CF ya sami sababbin alamomi ko kuma idan alamun cutar sun ƙara muni, musamman mawuyacin numfashi mai wahala ko tari na jini.

Ba za a iya hana CF ba. Binciken waɗanda ke da tarihin iyali na cutar na iya gano kwayar CF a cikin masu ɗauka da yawa.

CF

• Abincin abinci na yau da kullun - matsalolin yara
• Gastrostomy ciyar da bututu - bolus
• Yadda ake numfashi lokacin da kake karancin numfashi
• Jejunostomy yana ciyar da bututu
• Maganganun bayan gida

• Klub
• Maganganun bayan gida
• Yatsun kafa
• Cystic fibrosis

Donaldson SH, Pilewski JM, Griese M, et al. Tezacaftor / ivacaftor a cikin batutuwa tare da cystic fibrosis da F508del / F508del-CFTR ko F508del / G551D-CFTR. Am J Respir Masu Kula da Kulawa. 2018; 197 (2): 214-224. PMID: 28930490 pubmed.ncbi.nlm.nih.gov/28930490/.

Eagan ME, Shirye-shiryen MS, Voynow JA. Cystic fibrosis. A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 432.

Farrell PM, White TB, Ren CL, et al. Ganewar asali na cystic fibrosis: jagororin yarjejeniya daga Cystic Fibrosis Foundation. J Pediatr. 2017; 181S: S4-S15. 1. PMID: 28129811 pubmed.ncbi.nlm.nih.gov/28129811/.

Graeber SY, Dopfer C, Naehrlich L, et al. Hanyoyin maganin lumacaftor / ivacaftor akan aikin CFTR a cikin Phe508del homozygous marasa lafiya tare da cystic fibrosis. Am J Respir Masu Kula da Kulawa. 2018; 197 (11): 1433-1442. PMID: 29327948 pubmed.ncbi.nlm.nih.gov/29327948/.

Grasemann H. Cystic fibrosis. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: babi na 83.

Rowe SM, Hoover W, Solomon GM, Sorscher EJ. Cystic fibrosis. A cikin: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Littafin rubutu na Murray da Nadel na Magungunan numfashi. Na 6 ed. Philadelphia, PA: Elsevier Saunders; 2016: babi na 47.

Taylor-Cousar JL, Munck A, McKone EF, et al. Tezacaftor-ivacaftor a cikin marasa lafiya tare da cystic fibrosis homozygous don phe508del. N Engl J Med. 2017; 377 (21): 2013-2023. Â PMID: 29099344 pubmed.ncbi.nlm.nih.gov/29099344/.