Kei te pupuhi, kei te pakaru rānei tō kiri i te pā noa iho? Tērā pea he Epidermolysis Bullosa (EB)?

He tino tairongo tō tātou kiri i ētahi wā, kāore? Ahakoa ka pā mai tētahi mea iti, ka haehae rānei tētahi kākahu, ka ngāwari te pāngia o ētahi tāngata e ngā mirumiru me ngā whara kiri. Ina koa he tino tairongo te kiri o ngā pēpi nohinohi, he tino whakamataku te kite i ēnei mea. Nō reira, i tēnei rā ka kōrero tātou mō tētahi mate e pā ana ki tēnei momo kiri, he mea onge engari he mea nui kia mōhiotia. Ko te epidermolysis bullosa, ko te EB rānei te whakarapopototanga.

He aha te epidermolysis bullosa (EB)?

He māmā noa iho, he mate tuku iho te epidermolysis bullosa (EB) . Ka pakarukaru te kiri, ka pupuhi, ka ngawari te tihore. I ētahi wā, ahakoa ka mirimiri koe i tō kiri ki te kākahu, ka pāngia rānei koe e tetahi wāhi, ka puta he pupuhi me he mate kiri. Mō ētahi tāngata, he ngawari pea, ā, ka puta noa he pupuhi mamae ki ngā ringaringa, ki ngā tuke, ki ngā turi, me ngā waewae. Heoi, i ngā wā tino kino, ka puta ēnei pupuhi me ngā mate kiri ki ngā wāhi katoa o te tinana. Ka rerekē ngā tohu o te EB mai i te ngawari ki te tino kino .

Kia maumahara, i ētahi wā ka puta ēnei pupuhi i roto i te waha, i te korokoro (esophagus), i ngā kanohi, me ētahi atu okana o roto o te tinana. Ina ora ēnei pupuhi, ka waiho pea he koha mamae. I ngā wā tino kino, ka taea e ēnei pupuhi me ngā koha te kino i ngā okana o roto o te tinana, ka mate pea.

He aha ngā momo matua o te EB?

E whā ngā momo matua o te EB, e tautuhia ana e te paparanga kiri e pāngia ana.

1. EB Simplex (EBS): Koinei te momo EB tino noa . Ka rerekē i roto i ētahi tāngata mai i te ngawari, kāore i te tino mamae, ki te tino mamae, te kino. Ka puta ngā pupuhi i te paparanga o runga o te kiri, arā, te kiriuhi kiri . He onge te puta o ngā koha i muri i te ora o ēnei pupuhi.

2. Junctional EB (JEB): I roto i te JEB, ka puta he pupuhi i roto i te waha me te ara manawa. He ahua onge tēnei, ā, ka rerekē mai i te ngawari (he ahua kore whakamarie, he ahua mamae) ki te kino.

3. Dystrophic EB (DEB): I roto i te DEB, ka puta he pupuhi i te paparanga o waenganui o te kiri, arā, te dermis . Ka rerekē pea tēnei mai i te ngawari ki te kino.

4. Te Mate Pukupuku Kindler: I roto i te Mate Pukupuku Kindler, ka puta he pupuhi i te nuinga o ngā paparanga kiri. He tino onge tēnei.

Ka wehewehea e ngā tākuta te EBS, te JEB, me te DEB i runga i ngā paparanga o tō kiri e pāngia ana. I roto i te mate Kindler, ka puta ngā mirumiru puta noa i ngā paparanga rerekē o tō kiri.

Ko wai te hunga e tino pāngia ana e tēnei mate? He pēhea te noa o tōna pānga?

Ka pāngia te tangata e te EB. Ka taea e te tangata o tetahi iwi, iwi, ira tangata rānei te pāngia e te EB. Heoi anō,Mena kei tētahi o ō mātua te mate, he nui ake te tūponotanga ka pāngia koe e te EB.

Ahakoa te noa, e ai ki ngā tatauranga i te Hononga o Amerika, kotahi anake i roto i te 50,000 tāngata e pāngia ana e te EB. Ko te tikanga he mate tino onge tēnei.

He aha ngā pānga o te EB ki te tinana?

I ngā wā tino kino o te EB, ka pupuhi ngā kanohi, ka ngaro pea te tirohanga . I ētahi wā, ka puta he whara nui me te āhua rerekē o te kiri me ngā uaua, ka kore e taea te neke tika i ngā maihao, ngā ringaringa, ngā waewae, me ngā hononga. Ko ētahi tāngata whai EB he nui ake te tūponotanga ki te whakawhanake i tētahi momo mate pukupuku kiri e kiia nei ko te squamous cell carcinoma .

I ngā wā tino kino, ka mate pea te tamaiti i te wā e pēpi ana . Nā ngā mate pēnei i ngā mate kino (hei tauira , te sepsis ), te uaua ki te manawa nā te aukati i te ara manawa, te matewai, me te kore kai pai.

Ka taea te whakamate i te EB?

Kei te momo EB kei a koe tēnei. Kāore e mate ngā momo EB ngawari, iti iho te kino. Heoi, ko te roanga o te oranga o te hunga e pāngia ana e ngā momo EB kino ka rerekē mai i te pēpi ki te 30 tau te pakeke. Nō reira, he mate tēnei e hiahia ana kia aro nui ki a rātou.

He aha ngā tohu o te EB?

He rerekē ngā tohu o te EB i runga i te momo. Ka puta mai ēnei tohu i te wā e pēpi ana koe, e tamariki ana rānei . He mea noa ētahi tohu i waenga i ngā momo rerekē. Anei ētahi o ngā tohu matua o te EB:

• He mirumiru kei te kiri (ringa, waewae, tuke, turi) kei roto rānei i te tinana.
• Te mātotoru o te kiri i ngā nikau me ngā kapu o te waewae, te āhua nei he kiri mārō.
• Te hekenga o ngā toto whero (Anemia).
• Ngā maihao e piri ana tetahi ki tetahi (ngā maihao, ngā maihao rānei kua honoa).
• Ngā maikuku o ngā ringaringa me ngā maikuku kua pēpeke, kua matotoru rānei.
• Ko te putanga mai o ngā puku ma iti, he rite ki te mirumiru (milia) ki te kiri.
• Te uaua ki te horomi kai (dysphagia).
• Te korenga o te tipu e tumanakohia ana i roto i te tamaiti.
• Kāore ngā niho e whanake ana e ai ki te whakaaro ( hypoplasia ).

He aha ngā take o te EB?

Ko te tino take o te EB he whakarerekētanga, he hapa rānei i roto i tētahi o ngā ira 18. Kei te hunga whai mate nei he ira ngaro, he ira pakaru rānei e āwhina ana ki te hanga i tētahi pūmua e kiia nei ko te collagen . E mōhio ana koe, ko te collagen te mea e homai ana te kaha me te hanganga ki ngā kiko honohono pērā i tō tātou kiri. Nō reira, nā tēnei hapa ira, kāore ngā paparanga e rua o te kiri, te epidermis me te dermis, e hono tahi ana. Nō reira, ka tino pakarukaru te kiri, ka ngawari te pupuhi, ka whara.

He mate tuku iho te EB, arā, mēnā kei tētahi o ngā mātua tēnei mate, ka taea hoki e ngā tamariki te whiwhi.

Heoi, he tino onge, ka puta mai hoki te EB hei mate pūnaha ārai mate kua riro mai .

Ko te mea nui, ehara te EB i te mate hopuhopu. He mate tuku iho i te nuinga o te wā. Nō reira, ki te pā atu koe ki tētahi tangata kua pangia e te EB, e kore koe e pangia.

Me pēhea koe e tino mōhio ai mēnā kei a koe te EB? (Tauātanga)

Ka taea e ngā tākuta te tautuhi i te EB mā te mahi i tētahi koiora kiri , arā, ka tangohia e te tākuta tētahi tauira iti o te kiri ka tirotirohia i raro i tētahi karu hiko.

Hei tāpiritanga, ka taea e te whakamātautau ira te tautuhi i te ira hapa me te whakaū i te momo EB. Ka taea hoki e ngā mātua e whakamahere ana ki te whānau tamariki te whai whakamātautau ira i mua i te whānautanga kia kitea ai mēnā kei te mōrearea rātou ki te whānau tamariki me te EB.

He aha ngā maimoatanga mō te EB?

Engari, kāore anō kia kitea he rongoā mō te EB i tēnei wā . Heoi anō, ka taea e ngā maimoatanga te āwhina:

• Āwhina ki te ārai i ngā mirumiru.
• Mā te tiaki pai i ngā mirumiru me te kiri ka ārai i ngā raruraru.
• He rongoā mō ngā raruraru kai e puta mai ana i ngā pupuhi i roto i te waha, i te korokoro rānei.
• Te whakahaere i te mamae.

Hei ārai i te kino o te kiri me te hanganga o ngā pupuhi me ngā mate pāwera nā te waku, ka tūtohu ngā tākuta i ēnei e whai ake nei:

• Kia mau ki ngā kākahu ngohengohe, kore e tau ki roto, he mea hanga ki ngā muka tūturu. Mā te mau i ngā kākahu mai roto ki waho ka āwhina i te whakaiti i te nui o ngā tuitui e mirimiri ana ki tō kiri.
• Āraia te wera nui; kia noho rite, kia whakamarie hoki te pāmahana o ngā rūma.
• Kaua e haere ki waho i te rā, kaua hoki e mau i te pare rā i a koe e whakamahi ana i ngā mōhiti kanohi me ngā pōtae.
• Whakamahia ngā momo takai motuhake hei tiaki i te kiri – whakamahia ngā takai me te rīpene kāore e piri, he tauera porotaka, me ētahi atu.

Hei rongoā i ngā mirumiru, ka tūtohu pea tō tākuta i ngā mea penei:

• Pania he hinu ki ngā patunga i ia rā.
• Whakamahia ngā takai rongoā hei āwhina i te whakaora i ngā mirumiru me te ārai i te mate.
• Tangohia he rongoā hei whakahaere i te mamae.

Hei rongoā i ngā mate pāwera, ka tūtohu pea tō tākuta i ēnei e whai ake nei:

• Tangohia he rongoā paturopi ā-waha, pania rānei he kirīmi rongoā paturopi.
• Whakamahia he hipoki patunga motuhake mō ngā patunga kāore e ora.

Hei ārai i ngā raruraru kai me te uaua ki te kai nā te pupuhi o te waha, o te korokoro rānei, ka tūtohu pea tō tākuta i ēnei e whai ake nei:

• I te wā e whāngai ana i tāu pēpi, whakamahia he pounamu pēpi me tētahi uma motuhake .
• Whāngaihia te pēpi ki te whakamahi i te pata wai, i te ngira rānei.
• Tāpirihia he wai iti ki te kai kua kuruhia hei whakawaiwai, hei whāngai hoki. Mā tēnei ka māmā ake te horomi.
• Kai atu i ngā kai ngohengohe pērā i te hupa, ngā kai penupenu, te pūtini, me te aporo ranu .
• Ka tukuna te kai i te pāmahana mahana (kāore i te tino wera).
• Tirohia tētahi tohunga kai hei matapaki i ō hiahia kai motuhake.

I ngā wā tino kino o te EB, me mahi pokanga pea koe. Mena ka āraia te esophagus (te ngongo e kawe ana i te kai mai i te waha ki te puku) e ngā pupuhi me ngā koha, ka taea te mahi pokanga hei whakawhānui ake. Tērā pea ka taea e ētahi tāngata te karo katoa i te esophagus ka whakaurua he ngongo whāngai hei whāngai tika i te kai ki roto i te puku. Ka taea hoki te mahi pokanga hei wehe i ngā maihao e piri ana nā ngā pupuhi.

Me pēhea te tiaki a te tangata whai EB i a ia anō?

Mena kei a koe te EB, mā ēnei tohutohu koe e āwhina ki te tiaki i a koe anō:

• Moe i runga i ngā whāriki me ngā urunga i hangaia ki ngā muka ngohengohe, māori, pērā i te hiraka, te satin rānei.
• Kia pai ō hu, kaua e whakawhāiti i tō tinana.
• Kaua e tū, e hikoi rānei mō te wā roa.
• Kaua e rakuraku, e ngau rānei i te kiri. Tāpirihia he rongoā ārai-mākūkū hei whakaiti i te mākūkū.
• Kia tūpato ki te taiao e karapoti ana i a koe, ā, kaua e pā atu, e rakuraku rānei i tō kiri.
• Whakamakukuhia tō kiri i ngā wā katoa hei whakaiti i te waku.
• Ki te puta he mirumiru, hohoro te pupuhi ki te ngira kua horoia, ki te kutikuti ma rānei.
• Whakamahia he takai kāore e piri. Tērā atu rānei, pania he hinu pēpi (Vaseline™) he hinu kiri rānei (pērā i te Aquaphor™) ki te takai kia kore ai e piri ki te kiri.

Me pēhea te tiaki i tētahi tamaiti me te EB? Ētahi tohutohu mō ngā mātua

Mena he EB tāu tamaiti, he rerekē ōna hiahia i ērā atu. Tērā pea kāore e taea e rātou te whakapuaki i tōna mamae. Mā ēnei tohutohu ka āwhina i tāu tamaiti kia noho humarie:

• Horoia pai ō ringaringa i mua i te pā atu ki te kiri o tāu pēpi.
• Kaua e whakamahi i ngā karapu rapa, nā te mea ka nui ake te waku.
• I te wā e kaukau ana koe i tāu pēpi, kaua e maka katoa te tinana ki roto i tētahi peihana kī tonu i te wai i te wā kotahi, engari me kaukau takitahi ia wāhanga o te kiri.
• Engari i te whakanoho i tāu pēpi hou ki roto i ngā nāpi, whakaarohia te tāpiri i ngā papa mimiti ki runga i a ia.
• Whakamahia ngā nāpi me ngā whitiki Velcro®. Tērā pea ka piri ngā whitiki/rīpene piri ki te kiri o tāu pēpi.
• Tapahia, ka tangohia te taura here i te wāhi e piri ana ngā waewae o te nāpi.
• Whakatakotoria he mea pēnei i te pepa tiēre silicone ki roto i te nāpi hei ārai i te kiri kei piri ki ngā mirumiru kua puta.
• Kia tino tupato i te wā e hiki ana i te tamaiti.Kaua e hiki mā te whakatakoto i ō ringaringa ki raro i ngā kēkē (axilla). Mena he pupuhi, he patunga rānei tō tamaiti i ōna huha me tōna tuara, hikihia ia ki te ringa kotahi e karapoti ana i ōna huha, me te ringa tuarua hei tautoko i tōna tuara.
• Whakatenatenahia tāu tamaiti kia kaha ki te mahi i ngā mahi e taea ana e tōna mate EB. Mā te noho tonu ka pā mai ētahi atu raruraru, pērā i te mate puku me te ngoikore o ngā uaua. He pai te kaukau, he iti noa te pānga.
• Kaua e akiaki i ngā mahi ā-tinana uaua, i ngā kēmu rānei e werawera ai tāu tamaiti, e werawera ai rānei.

He uaua pea te aro atu ki ngā tohumate o tāu tamaiti i ētahi wā. Tērā pea ka pōuri koe, ka ngenge hoki. Pātai atu ki tō tākuta mō ētahi atu tohutohu hei pupuri i te whakamarie o tāu tamaiti – me koe anō hoki. Mā te whakauru atu ki ngā rōpū tautoko mātua , ka taea e koe te whakapuaki i ō wheako me te ako i ngā huarahi hou hei whakahaere i te mate EB o tāu tamaiti.

Ka taea te ārai i te EB?

Nā te mea he ira te EB, kāore e taea te ārai . Ko te hunga he hītori whānau tō rātou mō te EB, ā, e whakamahere ana kia whānau tamariki, tera pea ka kitea he tino āwhina te tohutohu ira ki te whakatau me pēhea te whakamahere i tō rātou whānau.

Hei tāpiritanga, kāore ngā tohunga i te mōhio he aha te take o te momo EB kua riro mai. Nō reira, kāore ngā tākuta i te mōhio me pēhea te ārai.

He aha te momo āpōpō ka taea e te tangata whai EB te tumanako atu?

Ko te heke mai o te hunga whai EB e whakawhirinaki ana ki te momo EB kei a rātou me tōna kaha. Ka taea e ngā momo kino o te mate te whakararu i te mamae nui, te hanga i ngā āhua rerekē, te hauātanga, ngā patunga e kore e ora, tae atu ki te mate wawe .

Heoi, ka taea e ngā tākuta te āwhina i a koe ki te whakahaere i ō tohumate. Ka taea tēnei mā te maimoatanga tika, me te rongoā mamae mēnā e tika ana. Mā te pupuri i te kiri kia kapi pai ki ngā takai tiaki kore-piripiri i ia rā, te kaukau pinepine, me te tiaki patunga ka āwhina i te whakaiti i te pānga o te EB ki tō oranga.

Mena kei a koe te EB, āhea koe me toro atu ki te tākuta?

Kia mahara ki te toro atu ki tō tākuta i roto i ēnei āhuatanga:

• Mena he uaua ki te manawa.
• Mena he uaua ki a koe te horomi i te kai.
• Mena kei te ahua pangia tō patunga (kei te whero, te papura, te hina, te mā rānei te kiri; kei te mumura, kei te pupuhi rānei).
• Mena ka puta mai ngā tohu hou.

He aha ngā pātai nui hei pātai atu ki te tākuta?

Ina haere koe ki te tākuta, he mea pai kia pātai koe i ēnei pātai:

• Me pēhea e taea ai e koe te mōhio mēnā kei a au te EB?
• Mena kāore he EB i ahau, he aha atu ngā mate kiri ka pā mai ki ahau?
• Me pēhea taku whakahaere i aku tohumate?
• He aha ngā rongoā e taunaki ana koe? He pānga taha kei roto?
• He aha ngā maimoatanga kāinga e taunaki ana koe?
• He aha atu anō taku mahi hei whakapai ake i aku tohu?
• He kirīmi, he hinu rānei ka taea e koe te tohu?
• Me toro atu ahau ki tētahi tākuta kiri, ki tētahi atu tohunga rānei?

He aha te rereketanga o te epidermolysis bullosa (EB) me te bullous pemphigoid?

He mate kiri onge, he mate pūnaha ārai mate te bullous pemphigoid . Ka taea e ia te puta he mākūkū, he rite ki te pītiti, he pupuhi wai rānei. Ko te bullous pemphigoid te nuinga o te wā ka pā ki te hunga neke atu i te 60 tau te pakeke , ā, ka ngaro i roto i ngā tau e rima. Heoi, i ngā take kino rawa atu, ka taea e ngā pupuhi me ngā koha te kino i ngā whekau o roto, ā, ka mate.

Ahakoa he mate pūnaha ārai mate te EB i ētahi wā, he maha ngā wā ka puta mai i te whakarerekētanga ira e pā ana ki te collagen i roto i tō kiri. Ka puta te EB i te wā pēpi, i te wā tamarikitanga rānei . Nā te mea kāore he rongoā mō te EB, me noho pea koe me ngā tohu mō te toenga o tō oranga.

Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)

He uaua te mate Epidermolysis bullosa (EB) i ētahi wā. Heoi, mā te tohutohu hauora tika, te maimoatanga, me te manaaki aroha, ka taea e koe te whakahaere i ngā tohu me te ora pai. Kia maumahara, ehara i te mea ko koe anake. Kei reira ngā tākuta, te whānau, me ngā rōpū tautoko hei āwhina i a koe. Nō reira, kaua e mataku, kia kaha, kia anga atu ki tēnei āhuatanga.

Mena he pātai anō āu mō te EB, me kōrero ki tētahi tākuta kiri.

` Te mate kiri pūkahukahu, EB, mate kiri, pupuhi, mate ira, mate kiri o te tamarikitanga, ngoikore o te kiri