Cystic fibrosis

Cystic fibrosis ndi matenda omwe amayambitsa mamina okhwima, omata m'mapapu, m'mimba, ndi madera ena amthupi. Ndi matenda omwe amapezeka kwambiri m'mapapo mwa ana komanso achinyamata. Ndi matenda owopsa.

Cystic fibrosis (CF) ndimatenda omwe amapitilira m'mabanja. Amayambitsidwa ndi jini lopunduka lomwe limapangitsa kuti thupi litulutse madzi amtundu wandiweyani komanso womata, wotchedwa ntchofu. Maminawa amakula m'mapapu ndi m'mapiko.

Kuchuluka kwa ntchentche kumabweretsa matenda owopsa a m'mapapo komanso mavuto akulu chimbudzi. Matendawa amathanso kukhudza thukuta la thukuta komanso ziwalo zoberekera za abambo.

Anthu ambiri amakhala ndi jini ya CF, koma alibe zizindikiro. Izi ndichifukwa choti munthu yemwe ali ndi CF ayenera kulandira majini awiri olakwika, 1 kuchokera kwa kholo lililonse. Anthu ena aku America ali ndi jini ya CF. Ndizofala kwambiri pakati pa makolo akumpoto kapena chapakati ku Europe.

Ana ambiri omwe ali ndi CF amapezeka ndi zaka 2, makamaka chifukwa chakuwunika kumene kumene kumachitika ku United States. Kwa ochepa, matendawa sapezeka mpaka azaka 18 kapena kupitilira apo. Ana awa nthawi zambiri amakhala ndi matenda ofatsa.

Zizindikiro za ana akhanda zimatha kuphatikiza:

• Kukula kochedwa
• Kulephera kunenepa bwino nthawi yaubwana
• Palibe kusuntha kwamatumbo m'maola 24 mpaka 48 oyamba amoyo
• Khungu lokoma mchere

Zizindikiro zokhudzana ndi matumbo zimatha kuphatikizira izi:

• Belly kupweteka kwa kudzimbidwa kwakukulu
• Kuwonjezeka kwa mpweya, kuphulika, kapena mimba yomwe imawoneka yotupa (yotayika)
• Nsautso ndi kusowa kwa njala
• Manyowa omwe ali otumbululuka kapena ofiira, onunkhira bwino, ali ndi ntchofu, kapena amayandama
• Kuchepetsa thupi

Zizindikiro zokhudzana ndi mapapu ndi sinus zitha kuphatikizira izi:

• Kutsokomola kapena kuchuluka kwa ntchofu m'machimo kapena m'mapapu
• Kutopa
• Kuchulukana kwa mphuno komwe kumayambitsidwa ndi tizilombo tamphuno
• Nthawi zambiri chibayo (zizindikiro za chibayo mwa munthu amene ali ndi cystic fibrosis zimaphatikizapo malungo, kuchuluka kwa chifuwa ndi kupuma movutikira, ntchofu yowonjezeka, komanso kusowa kwa njala)
• Sinus kupweteka kapena kukakamizidwa chifukwa cha matenda kapena tizilombo ting'onoting'ono

Zizindikiro zomwe zitha kuzindikiridwa pambuyo pake m'moyo:

• Kusabereka (mwa amuna)
• Kutupa kobwerezabwereza kwa kapamba (kapamba)
• Zizindikiro za kupuma
• Zala zopukutidwa

Kuyezetsa magazi kumachitika kuti athandizidwe kupeza CF. Kuyesaku kumayang'ana kusintha kwa jini la CF. Mayesero ena omwe amagwiritsidwa ntchito pozindikira CF ndi awa:

• Mayeso a Immunoreactive trypsinogen (IRT) ndi mayeso owunika a CF. Mulingo wapamwamba wa IRT ukuwonetsa kuthekera kwa CF ndipo kumafuna kuyesedwa kwina.
• Sweat chloride test ndiyeso yofananira yoyezetsa matenda a CF. Kuchuluka kwa mchere mu thukuta la munthuyo ndi chizindikiro cha matendawa.

Mayesero ena omwe amapeza mavuto omwe angakhale okhudzana ndi CF ndi awa:

• X-ray pachifuwa kapena CT scan
• Mayeso amafuta
• Kuyesa kwa mapapo
• Kuyeza kwa kapamba (chopondapo pancreatic elastase)
• Mayeso okondoweza a Secretin
• Trypsin ndi chymotrypsin mu chopondapo
• Upper GI ndi matumbo ang'onoang'ono
• Zikhalidwe zam'mapapo (zopezedwa ndi sputum, bronchoscopy kapena khosi swab)

Kuzindikira koyambirira kwa CF ndi dongosolo la chithandizo kumatha kupulumutsa moyo komanso kukhala ndi moyo wabwino. Kuwunika ndikuwunika ndikofunikira kwambiri. Ngati kuli kotheka, chisamaliro chiyenera kulandiridwa ku chipatala chapadera cha cystic fibrosis. Ana atakula, ayenera kusamukira ku malo enaake achikulire a cystic fibrosis.

Kuchiza kwa mavuto am'mapapo kumaphatikizapo:

• Maantibayotiki kupewa ndi kuchiza matenda am'mapapo ndi sinus. Amatha kutengedwa pakamwa, kapena kuperekedwa m'mitsempha kapena kupuma. Anthu omwe ali ndi CF amatha kumwa maantibayotiki pokhapokha ngati akufunikira, kapena nthawi zonse. Mlingo nthawi zambiri umakhala wapamwamba kuposa wabwinobwino.
• Mankhwala opumira kuti athandizire kutsegula njira zapaulendo.
• Mankhwala ena omwe amaperekedwa ndi kupuma kwa mamina owonda ndikupangitsa kuti kukokoloka kukhale kosavuta ndi mankhwala a ma enzyme a DNAse ndi mayankho amchere kwambiri (hypertonic saline).
• Katemera wa chimfine ndi katemera wa pneumococcal polysaccharide (PPV) pachaka (funsani wothandizira zaumoyo wanu).
• Kuika m'mapapo ndi njira zina.
• Thandizo la oxygen lingafunike matenda am'mapapo akukulirakulira.

Mavuto am'mapapo amathandizidwanso ndimankhwala ochepetsa mamina. Izi zimapangitsa kukhala kosavuta kutsokomola mamina m'mapapu.

Njirazi ndi monga:

• Zochita kapena zolimbitsa thupi zomwe zimakupangitsani kupuma mwamphamvu
• Zipangizo zomwe zimagwiritsidwa ntchito masana kuti zithandizire kutulutsa mamina ochuluka kwambiri
• Kuphulika pachifuwa pamanja (kapena chifuwa cha physiotherapy), momwe wachibale kapena wothandizira amawombetsa mopepuka pachifuwa, kumbuyo, ndi m'manja mwake

Chithandizo cha mavuto am'mimba ndi zakudya zimatha kuphatikizira izi:

• Chakudya chapadera chokhala ndi mapuloteni ambiri ndi zopatsa mphamvu kwa ana achikulire ndi akulu
• Mavitamini a Pancreatic othandiza kuthandizira mafuta ndi mapuloteni, omwe amatengedwa ndi chakudya chilichonse
• Mavitamini owonjezera, makamaka mavitamini A, D, E, ndi K
• Wothandizira anu akhoza kulangiza mankhwala ena ngati muli ndi mipando yolimba kwambiri

Ivacaftor, lumacaftor, tezacaftor, ndi elexacaftor ndi mankhwala omwe amathandizira mitundu ina ya CF.

• Amathandizira kugwira ntchito kwa jini imodzi yolakwika yomwe imayambitsa CF.
• Kufikira 90% ya odwala omwe ali ndi CF ndipo akuyenera kulandira mankhwala amodzi kapena angapo okha kapena osakanikirana.
• Zotsatira zake, pamakhala mapapo ochepa am'mapapo. Zizindikiro zina za CF zimasinthidwanso.

Kusamalira ndi kuwunika kunyumba kuyenera kuphatikizapo:

• Kupewa utsi, fumbi, dothi, utsi, mankhwala apanyumba, utsi wamoto, ndi nkhungu kapena cinoni.
• Kupereka madzi ambiri, makamaka kwa ana ndi ana nthawi yotentha, pakakhala matenda otsekula m'mimba kapena zotchinga, kapena nthawi yochita zolimbitsa thupi.
• Kuchita masewera olimbitsa thupi kawiri kapena katatu sabata iliyonse. Kusambira, kuthamanga, ndi kupalasa njinga ndi njira zabwino.
• Kutsuka kapena kubweretsa ntchofu kapena zotulutsa kuchokera munjira zopumira. Izi ziyenera kuchitika kamodzi kapena kanayi tsiku lililonse. Odwala, mabanja, ndi osamalira anzawo ayenera kuphunzira za kuphulika pachifuwa komanso ngalande zapambuyo kuti athandizire kuwonekera bwino.
• Palibe kuyanjana ndi anthu ena omwe ali ndi CF ndikulimbikitsidwa chifukwa amatha kusinthana ndi matenda (sikugwira ntchito kwa abale awo).

Mutha kuchepetsa nkhawa zamatenda polowa mgulu lothandizira la cystic fibrosis. Kugawana ndi ena omwe akukumana nawo komanso omwe ali ndi mavuto ambiri kumatha kuthandiza banja lanu kuti lisasungulumwe.

Ana ambiri omwe ali ndi CF amakhala ndi thanzi labwino mpaka atakula. Amatha kutenga nawo mbali pazochitika zambiri ndikupita kusukulu. Achinyamata ambiri omwe ali ndi CF amaliza maphunziro awo kukoleji kapena amapeza ntchito.

Matenda am'mapapo amayamba kukulira mpaka kuti munthu akhale wolumala. Masiku ano, nthawi yayitali ya moyo wa anthu omwe ali ndi CF omwe akukalamba mpaka pano ndi pafupifupi zaka 44.

Imfa imayambitsidwa ndimatenda am'mapapo.

Vuto lofala kwambiri ndi matenda opuma opuma.

Zovuta zina ndizo:

• Mavuto am'matumbo, monga ma gallstones, kutsekeka m'matumbo, komanso ma rectal prolapse
• Kutsokomola magazi
• Matenda kupuma kulephera
• Matenda a shuga
• Kusabereka
• Matenda a chiwindi kapena kulephera kwa chiwindi, kapamba, biliary cirrhosis
• Kusowa zakudya m'thupi
• Tizilombo ting'onoting'ono m'mphuno ndi sinusitis
• Osteoporosis ndi nyamakazi
• Chibayo chomwe chimabwerera mmbuyo
• Pneumothorax
• Kulephera kwa mtima kumanja (cor pulmonale)
• Khansa yoyipa

Itanani omwe akukuthandizani ngati khanda kapena mwana ali ndi zizindikiro za CF, komanso zokumana nazo:

• Kutentha thupi, kuchuluka kwa kutsokomola, kusintha kwa sputum kapena magazi mu sputum, kusowa kwa njala, kapena zizindikilo zina za chibayo
• Kuchuluka kwa kuchepa thupi
• Kusuntha matumbo pafupipafupi kapena ndowe zomwe zimanunkha kapena zimakhala ndi mamina ambiri
• Kutupa m'mimba kapena kuchuluka kwam'mimba

Itanani omwe akukuthandizani ngati munthu yemwe ali ndi CF ayamba kukhala ndi zisonyezo zatsopano kapena ngati matenda akuwonjezereka, makamaka kupuma kovuta kwambiri kapena kutsokomola magazi.

CF silingapewe. Kuwunika omwe ali ndi mbiri yakubadwa kwa matendawa kumatha kuzindikira mtundu wa CF mwa omwe amanyamula.

CF

• Zakudya zolimbitsa thupi - kusamalira ana
• Gastrostomy yodyetsa chubu - bolus
• Momwe mungapume mukakhala ndi mpweya wochepa
• Thumba lodyetsera la Jejunostomy
• Ngalande zapambuyo pake

• Kalabu
• Ngalande zapambuyo pake
• Zala zopukutidwa
• Cystic fibrosis

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Eagan INE, Schechter MS, Voynow JA. Cystic fibrosis. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: mutu 432.

Farrell PM, White TB, Ren CL, et al. (Adasankhidwa) Kuzindikira kwa cystic fibrosis: malangizo ogwirizana ochokera ku Cystic Fibrosis Foundation. J Wodwala. Chizindikiro. 2017; 181S: S4-S15.e1. PMID: 28129811 pubed.ncbi.nlm.nih.gov/28129811/. (Adasankhidwa)

Graeber SY, Dopfer C, Naehrlich L, ndi al. Zotsatira za mankhwala a lumacaftor / ivacaftor pa CFTR ikugwira ntchito mu Phe508del odwala homozygous odwala cystic fibrosis. Ndine J Respir Crit Care Med. 2018; 197 (11): 1433-1442. (Adasankhidwa) PMID: 29327948 pubed.ncbi.nlm.nih.gov/29327948/.

Grasemann H. Cystic fibrosis. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 83.

Rowe SM, Hoover W, Solomon GM, Sorscher EJ. Cystic fibrosis. Mu: Broaddus VC, Mason RJ, Ernst JD, et al, olemba. Murray ndi Nadel's Bookbook of Respiratory Medicine. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier Saunders; 2016: chap 47.

Taylor-Cousar JL, Munck A, McKone EF, ndi al. Tezacaftor-ivacaftor mwa odwala cystic fibrosis homozygous ya phe508del. N Engl J Med. 2017; 377 (21): 2013-2023 PMID: 29099344 adatulutsidwa.ncbi.nlm.nih.gov/29099344/.