‘Brush against a wall tears skin like tis­sue pa­per’

Par­ents of tod­dler with se­vere skin-blis­ter­ing con­di­tion tell of their daugh­ter’s or­deal

The Irish Times - Tuesday - Health - - Skin Condition - Áilín Quin­lan

Ev­ery Mon­day, Wed­nes­day, Fri­day and Sun­day, 2½-year-old Alana un­der­goes an ex­cru­ci­at­ing or­deal which con­tin­ues for hours.

It starts with the peel­ing away of the ban­dages which cover her lit­tle body from shoul­ders to toes, pro­tect­ing the tis­suethin skin and the wounds which criss-cross it.

Next, the tod­dler is placed in a bath of Mil­ton and salt. It stings cru­elly, but it’s a cru­cial step to pro­tect her bro­ken skin from in­fec­tion.

Af­ter that, Alana is car­ried to what used to be the spare room in her par­ents’ home in Knock­raha, Co Cork, but which has been turned into a spe­cial ban­dag­ing room with padded sur­faces.

Alana’s ban­dages must be re­placed, but, be­fore that can hap­pen, the huge blis­ters which ap­pear all over her body must be popped, with a ster­ilised nee­dle.

The blis­ters, ex­plain her mother Rachel Reid (35), can get very big. They re­sult from any fric­tion, such as a mis­ap­plied ban­dage, but Alana can also get them from walk­ing, play­ing with toys or eat­ing.

“It takes three of us to hold her, pop the blis­ters and ap­ply the ban­dages,” ex­plains Rachel, who par­tic­i­pates in this rit­ual – which can take up to three hours – along­side her part­ner Greg and Alana’s HSE-sup­plied carer and per­sonal as­sis­tant, Caro­line, three days a week. On the fourth day, Sun­day, Alana has the ban­dages changed on her hands and right leg.

There is noth­ing nor­mal, as Rachel ob­serves, about pin­ning your baby down while she screams in pain as you pop blis­ters with nee­dles and wrap her whole body in ban­dages.

“Four days a week this takes place – she has to be heav­ily med­i­cated be­fore we start. It’s aw­ful. I think if you stop and think about what you’re do­ing you wouldn’t be able to do it. You can’t stop and walk away – the ban­dages have to put back on, be­cause if she touches any­thing while she has no ban­dages she will be cov­ered in blis­ters.”

Se­vere

There are days, Rachel says, when Alana screams so much that ev­ery­one is in tears. “There are days she stares me dead in the eyes scream­ing, beg­ging me to make it stop but we have to keep go­ing, re­mind­ing your­self that one day she will un­der­stand why we are do­ing this.”

Lit­tle Alana has the most se­vere form of a rare ge­netic dis­ease, epi­der­mol­y­sis bul­losa (EB), a hor­rific skin-blis­ter­ing con­di­tion, for which there is no cure, and which was once de­scribed by a med­i­cal ex­pert as “the worst thing you can live with”.

This week is Na­tional EB Aware­ness Week and De­bra Ire­land, the char­ity that sup­ports EB pa­tients and their fam­i­lies, have but­ter­fly tat­toos avail­able at Ap­ple­green ser­vice sta­tions. Al­ter­na­tively, you can text the word But­ter­fly to 50300 to make a ¤4 do­na­tion.

The lit­tle Cork girl is the only per­son in Ire­land to have gen­er­alised in­ter­me­di­ate re­ces­sive dys­trophic epi­der­mol­y­sis bul­losa, though about 300 peo­ple in Ire­land (and about 500,000 world­wide) have var­i­ous forms of EB, which causes a break­down in the nat­u­ral pro­teins that hold the skin to­gether. Even the slight­est fric­tion can cause se­vere pain, blis­ter­ing and sores.

Like most ac­tive tod­dlers, Alana falls down and bumps into things. Un­like most tod­dlers, how­ever, when this hap­pens she gets blis­ters im­me­di­ately and her par­ents in­stantly leap into ac­tion. “We take off the ban­dages – on the knees, el­bows or hands to pop the blis­ters – if she brushes against a wall for ex­am­ple the skin tears like tis­sue pa­per,” says Rachel.

If Alana eats any­thing hard, sharp or rough like ce­real or a piece of toast it will tear her tongue, gums and the in­side of her oe­soph­a­gus. She has to have spe­cial med­i­ca­tion to en­sure her stools stay soft or they will tear her on the in­side dur­ing a bowel move­ment.

Mean­while, Rachel and Greg, who are lucky to get two or three hours of sleep a night, try to give their other daugh­ter, nine-year-old Chloe, some­thing ap­prox­i­mat­ing a nor­mal life.

Alana Reid has the most se­vere form of a rare ge­netic dis­ease, epi­der­mol­y­sis bul­losa, a hor­rific skin-blis­ter­ing con­di­tion, for which there is no cure

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