Frag­ile life

About 100,000 peo­ple in China suf­fer from os­teo­ge­n­e­sis im­per­fecta, a ge­netic and in­her­ited dis­or­der char­ac­ter­ized by brit­tle bones. Liu Zhi­hua finds out what life is like for suf­fer­ers of this cruel af­flic­tion.

China Daily (Hong Kong) - - FRONT PAGE - Con­tact the writer at li­uzhi­hua@chi­nadaily.com.cn.

About 100,000 peo­ple in China suf­fer from a ge­netic and in­her­ited dis­or­der char­ac­ter­ized by brit­tle bones. Life is hard for th­ese peo­ple, who are also called “china dolls”.

Six-year-old Wu Yul­in­g­long, a girl from a small county in Hanzhong city, Shaanxi prov­ince, looks like a 2-yearold. Her limbs are thin and her legs are so de­formed she can barely walk. “When she was a baby, her bones just broke for no rea­son,” says her fa­ther Wu Liang. “We dare not hold her for fear of hurt­ing her.”

The girl is one of about 100,000 “china dolls” in the coun­try. The term is used to de­scribe those who suf­fer from os­teo­ge­n­e­sis im­per­fecta, a ge­netic and in­her­ited dis­or­der char­ac­ter­ized by frag­ile bones.

Ac­cord­ing to China-Doll Cen­ter for Rare Dis­ease, a non­govern­men­tal or­ga­ni­za­tion, 70 per­cent of suf­fer­ers live in less-priv­i­leged ru­ral ar­eas.

Their bones break eas­ily with­out any spe­cific cause, and an in­di­vid­ual can suf­fer dozens to hun­dreds of sig­nif­i­cant frac­tures in a life­time, leav­ing not only pain but also bone de­for­mity.

A sur­vey re­port re­cently re­leased by the cen­ter shows that al­though sit­u­a­tions vary, “china dolls” face sim­i­lar prob­lems in life — poor ac­cess to med­i­cal care, ed­u­ca­tion and job op­por­tu­ni­ties.

“Os­teo­ge­n­e­sis im­per­fecta is treat­able. With timely and ef­fi­cient in­ter­ven­tion, OI chil­dren can grow up healthily to live a nor­mal life,” says Wang Yi’ou, founder and di­rec­tor of the cen­ter, who is also an OI pa­tient.

“But, it is a pity that for the ma­jor­ity of OI pa­tients, it is of­ten too late when they re­ceive di­ag­no­sis and treat­ment.”

Wu Liang says in the first two years of her life, his daugh­ter suf­fered from dozens of frac­tures and de­vel­oped de­for­mi­ties as she grew up.

The girl would frac­ture her­self even while ly­ing in bed. Lo­cal doc­tors were clue­less about the con­di­tion.

When the girl turned 3, doc­tors in a hos­pi­tal in Xi’an fi­nally made a di­ag­no­sis: She be­came the hos­pi­tal’s first OI case.

But doc­tors told the fa­ther there was no treat­ment, nor hope for re­cov­ery.

The girl’s de­for­mity de­vel­oped rapidly. Al­though she’s ob­vi­ously smarter than her peers, she was re­fused by kinder­gartens and schools.

Last year, she fi­nally un­der­went OI treat­ment in a hos­pi­tal in Tian­jin, with the help of Wang’s cen­ter, which the fa­ther chanced upon when surf­ing the In­ter­net.

Cur­rently, to in­crease bone den­sity and re­duce the num­ber of frac­tures, the girl re­ceives drug in­fu­sions ev­ery three to four months, cost­ing about 2,000 yuan ($326) each time.

The fa­ther also plans to send her for surgery to cor­rect her de­for­mity.

Wu Yul­in­g­long is luck­ier than many OI pa­tients in China as her fam­ily is able to af­ford her treat­ment.

OI is a life­long con­di­tion and needs con­tin­u­ous treat­ment, in­clud­ing med­i­ca­tion to in­crease the den­sity of the bones. Oth­er­wise, the bones and mus­cles will de­form rapidly, es­pe­cially dur­ing child­hood. to self-study, or re­main il­lit­er­ate.

“When we grow up, we can­not do la­bor work be­cause of our phys­i­cal con­di­tion, and we can­not ob­tain good po­si­tions in com­pa­nies be­cause of lack of ed­u­ca­tion. As a re­sult, we earn too lit­tle for a liv­ing, not to men­tion to pay for treat­ment.”

Wang Lin works as a re­cep­tion­ist in an ad­ver­tis­ing firm. But her other friends who suf­fer from the same con­di­tion are un­em­ployed.

“So­ci­ety should be more aware of the suf­fer­ings and needs of OI pa­tients, and ex­tend a help­ing hand to them,” Wang Yi’ou says.

But few hos­pi­tals in big cities know how to treat OI, let alone those in less­de­vel­oped ru­ral ar­eas, ac­cord­ing to Qi Ming, a ge­netic dis­eases spe­cial­ist with Zhe­jiang Univer­sity, who is also a pro­fes­sor with Depart­ment of Pathol­ogy and Lab­o­ra­tory Medicine, Rochester Univer­sity in the United States.

He says in many de­vel­oped coun­tries, such as the United States, the cost of OI treat­ment is cov­ered by var­i­ous med­i­cal in­sur­ance pro­grams.

There are also a lot of non­govern­men­tal or­ga­ni­za­tions and foun­da­tions en­gaged in ac­tiv­i­ties pro­tect­ing and help­ing OI pa­tients, such as fund­ing for OIre­lated re­search and launch­ing leg­is­la­tion cam­paigns to pro­tect the in­ter­ests of OI pa­tients. But there are few in China, Qi adds.

In ad­di­tion, the high cost of treat­ment and low in­sur­ance cov­er­age pre­vent most pa­tients from get­ting med­i­cal care.

With­out treat­ment, OI adults can only grow to the height of a young child, with bowed limbs and pro­trud­ing chest bones. Most of them are not able to move around with­out crutches or wheel­chairs.

The con­di­tion also af­fects their hear­ing, teeth and blood ves­sels.

“It is a vi­cious cir­cle,” says Wang Lin, 26, an OI pa­tient from Dalian, Liaon­ing prov­ince.

“With­out money and treat­ment, we will be­come dis­abled. No schools are will­ing to ac­cept a dis­abled child, and we have

PHO­TOS BY ZHU XINGXIN / CHINA DAILY

A wed­ding cer­e­mony is one of the high­lights for os­teo­ge­n­e­sis im­per­fecta pa­tients as more than 300 suf­fer­ers gath­ered in Bei­jing on Sun­day.

Jiang Yingxi, from Jiangxi prov­ince, per­forms the erhu (Chi­nese two-string fid­dle) at an event in Bei­jing.

Wu Yul­in­g­long, a 6-year-old from Shaanxi prov­ince, joins a draw­ing con­test or­ga­nized by China-Doll Cen­ter for Rare Dis­ease.

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