Lit­tle Char­lie’s spe­cial gift

Lan­g­ley tod­dler with se­vere sun al­lergy gets liver trans­plant from dad

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Char­lie Lock’s Christ­mas came with a very spe­cial gift.

On Dec. 4, doc­tors re­moved the Lan­g­ley tod­dler’s liver and gave her a piece of her dad’s liver. The trans­plant, per­formed by sur­geons at Sick Kids in Toronto, is one of the first steps in a com­pli­cated plan to give the lit­tle girl with a se­vere sun al­lergy a more nor­mal life.

“The trans­plant went re­ally well,” Char­lie’s mom Bekah Lock told Post­media by phone from Toronto.

“We’re not out of the woods yet, but we’re grate­ful for how well it went.”

The next six months will see more med­i­cal pro­ce­dures for the two-year-old girl and her dad, Kelsey, who was dis­charged from hospi­tal a few days af­ter his surgery, but con­tin­ues to heal as his liver re­gen­er­ates.

In a few months, he’ll give his tiny daugh­ter an­other gift — this time, a bone-mar­row trans­plant — that will help to save her new liver from the rav­ages of por­phyria.

In July, two-year-old Char­lie and her mom Bekah Lock met with Post­media to talk about life with ery­thro­poi­etic pro­to­por­phyria (EPP) — a ge­netic dis­ease that is some­times de­scribed as an al­lergy to the sun.

Even small amounts of ul­tra­vi­o­let light cause the tod­dler’s skin to burn, blis­ter and swell. But it’s the in­vis­i­ble dam­age — the ac­cu­mu­la­tion of por­phyrins in her liver — that can even­tu­ally be life-threat­en­ing.

Char­lie can­not go out­side, not even on the cloud­i­est days, said her mom. The win­dows in the fam­ily’s Lan­g­ley home are coated with UV-block­ing film. To leave, she must be bun­dled into a stroller with a pro­tec­tive cover and then rushed to a ve­hi­cle with sim­i­larly coated win­dows.

She can’t go to a park, or visit a pet­ting zoo, or have a play date at a friend’s house.

Her brief hours out­side — the long walks her mom and dad would take with her be­fore she was di­ag­nosed a few weeks af­ter her first birth­day — have been for­got­ten.

“It’s a very small world that she lives in,” Lock told Post­media in July.

“She’ll stare out the win­dows and point at the leaves in the trees. She knows what’s out there, but she’s never fully ex­pe­ri­enced it.”

Por­phyria, specif­i­cally EPP, can be ex­cru­ci­at­ingly painful. The fam­ily was pre­pared to adapt to a life with­out sun­light, but shortly af­ter Char­lie’s di­ag­no­sis, there was more bad news.

Peo­ple with EPP have a short­age of a par­tic­u­lar en­zyme that me­tab­o­lizes por­phyrins, which help with the pro­duc­tion of he­mo­glo­bin. With­out the en­zyme, por­phyrins ac­cu­mu­late in the blood, re­act­ing with sun­light to cause burns. In a small per­cent­age of peo­ple with EPP, por­phyrins also ac­cu­mu­late in the liver.

Like light­ning strik­ing twice, Char­lie had the rare form of EPP, which destroys the liver. Tests showed scar­ringsim­i­lar­tothato­fanal­co­holic.

Despite the risks as­so­ci­ated with the pro­ce­dure, doc­tors be­gan plan­ning a bone-mar­row trans­plant, which could help the lit­tle girl me­tab­o­lize por­phyrins. But be­cause Char­lie has two rare ge­netic mark­ers, a per­fect match could not be found among fam­ily or the in­ter­na­tional data­base. Doc­tors de­cided Kelsey’s bone mar­row, although not a per­fect match, could at least halt the dam­age to the tod­dler’s liver and pos­si­bly help re­duce the im­pacts of sun ex­po­sure.

Two weeks be­fore the bone-mar­row trans­plant was set to take place, Char­lie be­came se­ri­ously ill. Her en­larged liver was press­ing on her lungs, lead­ing to pneu­mo­nia. Her tiny, sick body would no longer be able to han­dle the chemo­ther­apy needed to de­stroy her own bone mar­row in prepa­ra­tion for the trans­plant.

A new plan was cre­ated. Char­lie needed a new liver, so in­stead of do­nat­ing his bone mar­row, Kelsey do­nated a piece of his liver in­stead. The pro­ce­dure could not be done in B.C., so the fam­ily trav­elled to Toronto in late fall.

When de­scrib­ing Char­lie’s tu­mul­tuous year, Bekah Lock is cheer­ful and op­ti­mistic.

The young mom cel­e­brates the small things, like be­ing able to hear Char­lie’s lit­tle voice — and her laugh­ter — af­ter she was re­moved from a ven­ti­la­tor af­ter the trans­plant.

“We’re not all the way there, but get­ting those lit­tle pieces of her back has been so good,” she said.

Lock ad­mit­ted it was tough for her­self and Kelsey to be away from fam­ily over Christ­mas, but in the same breath, she ex­pressed grat­i­tude for the doc­tors, nurses and staff at the hospi­tal, as well as for Ron­ald McDon­ald House, where the fam­ily has been stay­ing while in Toronto.

“They’re tak­ing great care of us,” she said.

Af­ter Char­lie be­comes stronger and gains some weight, the lit­tle girl will un­dergo chemo­ther­apy in prepa­ra­tion for the bone-mar­row trans­plant. The fam­ily plans to re­main in Toronto for that process and doesn’t ex­pect to re­turn to B.C. for about six months.

“It’s out of our hands,” said Lock. “We know that there will be ups and downs. It never goes per­fectly to plan. But right now things are good, so we’re just go­ing to cel­e­brate that.”

We’re not all the way there, but get­ting those lit­tle pieces of her back has been so good.”

Bekah Lock

NICK PROCAYLO/ PNG FILES

Char­lie Lock, shown with her mother Bekah in this July photo be­fore her sec­ond birth­day, has por­phyria, mean­ing she is al­ler­gic to the sun. The com­pli­ca­tions are many; she just re­ceived a liver trans­plant and will be get­ting a bone­mar­row trans­plant as well.

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