In January of 2010, I traveled to Panama City, Panama with a group of peers to help the Crystal Children Foundation raise awareness of their mission: to improve the quality of life for children, youth and adults affected by Osteogenesis Imperfecta, and raise awareness of the disease to promote patients' development and integration into society. Osteogenesis Imperfecta, or OI, is a rare genetic disease characterized by fragile bones prone to breaking and fracturing. Our goal was to collect stories from those suffering from this disease, speak to doctors about how they are treating the disease, and find more answers from the government as to why an important drug that has been approved in the US and other nations that has proven to alleviate OI symptoms, wasn't approved in Panama. From these stories we developed a web presence for the Foundation so they could continue to tell their story and push change in their country for those affected by OI.
Below are some of the stories we collected.
Kenneth is suffering from Type II Osteogenesis Imperfecta, the most type severe type there is. At the young age of 10, he has already had 172 bone fractures.
Soon after Kenneth was born he was diagnosed with OI and his father abandoned the family, leaving them living in extreme poverty alone. Raw sewage and rats ran rampant in their apartment. Kenneth’s mother, Eneida reached out to Dayana Castilla, Director at the Crystal Children’s Foundation because she knew Kenneth needed a better life in order to have any chance of surviving with OI. Through a government-housing program, the Foundation assisted Eneida and her family to move to their home in Colón.
Eneida and her new husband have not been able to find full time work and the cost for getting Kenneth to his appointments in Panama and paying the medical expenses is a constant struggle. Kenneth does not have health insurance, and with the severity of his OI the medical bills add up fast. "When Kenneth has to go to Panama, my husband tries to have all the money we need for the visit," Eneida says. “We have to pay for x-rays but sometimes we do not have the money for them and they do not let me do a payment option.”
Kenneth and his mother live with the constant fear that he will break a bone. The slightest movement, a bump in the road on the way to school, a shake of the hand can all cause a fracture for Kenneth.
Pamidronate is a drug that helps the body to produce collagen, a protein that strengthens bones and therefore helps decrease the severity of OI. This drug is approved for use in the United States, Europe and other countries and has shown positive results in OI patients, however the Panamanian government is reluctant to approve the drug in the country. Eneida explains that the doctors have been very sympathetic to Kenneth’s plight and have done everything possible to give him a better life without this drug.
"What we need now is the medicine,” said Eneida. "Before he would break once a month, but now he is constantly fracturing more, and when I have no money, there is no way to pay for medical appointments. It is a problem. Every time this happens, I have to call and reschedule. I don’t like missing appointments because the only one who gets hurt is Kenneth"
The Crystal Children’s Foundation has provided Kenneth and his family with some of their basic living needs. “We really need and appreciate all the support the Foundation has given us," says Eneida.
Despite his situation, Kenneth continues to live with a smile on his face. His favorite things to do are play video games and watching his favorite soccer team,Barca, with his brothers and friends.
At 4 am, Zuleika is awakened by the cries of her baby girl, Jocelyn. She rises from bed to find that her baby has broken a bone and needs to be taken to the hospital. Unfortunately, the hospital is hours away and not an easy journey to get there.
Zuleika and Jocelyn live in a hut made of wood and palm tree leaves east of Panama City, in a remote village in the jungle near Chepo. The hut is on top of a hill with boulders, trees, streams and uneven ground that is surrounded by rainforest. The area is no place to raise a child with brittle bones.
Whenever Jocelyn fractures or has an appointment with the doctor, Zuleika has to walk about two miles to a taxi that takes her the river in Puerto Coquira. On the river, she catches a water taxi to cross to the other side. From there she takes a bus to Chepo, then another bus to Felipillo, and the last bus leaves her close to the Hospital del Niño in Panama City. Jocelyn remains safely in Zuleika’s arms the whole trip. The journey is exhausting and takes several hours, and depending on how many fractures Jocelyn gets, Zuleika has to repeat this several times a month.
“I have to leave here”, Zuleika said. "It's tough coming and going when she fractures. If she fractures in the afternoon, she has to go through much pain during the night and wait until the next morning when I can take her to the hospital. That's why we need a house in Panama City, but I have no means to achieve this.”
Zuleika was raised in the area where they live today, which is far away from civilization. She lives with her two daughters and her father. They use the river to bathe, a fire to cook, they do their own hunting. During the rainy season everything gets wet and floods, and when the river overflows the clothes can’t be washed because the water is dirty.
“I want to live there (in Panama City), but I have to live here because I only have a home here,” she said. "My father is here too.” Unable to get a job to afford an apartment in the city, Zuleika contacted the Crystal Children Foundation. The Foundation provides her with milk, clothing, food and other basic needs, and is working to get her into affordable housing closer to the city.
With type III OI, Jocelyn has suffered many fractures from birth, beginning with two broken legs during labor. She will continue to suffer fractures without the proper medical treatment. As she grows and begins to play, her risk of fracture increases. "I need help to get a house, " Zuleika says. "That will make it easier for me to take my daughter to the hospital and live with her in peace."
Osvaldo Garcia, best known as Arturo, is proof that patients with Osteogenesis Imperfecta can lead normal, healthy lives. By the time he turned 15, Arturo had suffered from 29 broken bones. At age 28, he has already passed the toughest days of the disease, childhood and adolescence, and has not had a fracture related to OI for several years.
Arturo is one of 12 members of his family that has the genetic disease. His parents did not suffer from OI, but many of his aunts, uncles and cousins do. His sister does not have symptoms but is likely a carrier and could pass it on to her children someday.
Today Arturo uses a wheelchair but his outlook on life is positive and inspiring. After meeting a Peace Corps member, he became involved in volunteering and his attitude changed. “I realized that we have many obstacles in life,” Arturo said. “We have many problems, and hundreds of things happening to us, but the only thing that prevents us from doing something is ourselves. If you think you cannot do something, then you probably don’t do it. "
Arturo’s childhood was difficult, as it is for most children with OI. “Every time I fractured a bone, I had to go to the hospital,” Arturo says, “It was frustrating because I’d be in a cast for a month or a month and a half, and then another month after that in rehab before I could start moving again.”
These constant fractures caused him to miss school and added a few additional years to his education. That did not stop him from learning though. Arturo recently earned a degree in Biology and is currently working on his thesis. His willingness to lead a normal, full life is an enlightening example for all people with OI.
"I do not know if I am an example because I do not consider myself one,” Arturo said. “As everyone, I have a downside. I have bad habits, I have made mistakes, but I do know is that I try to live a good life without regrets. " He says he owes many thanks to his family and friends, as they have had a major impact on all that he has accomplished. He explains that if you have a strong support system and a positive outlook it can make all the difference in the fight against OI.
Tays Milena Núñez Betancourt is nine years and was suspended from school for the constant hospital visits, fractures and absences due to Osteogenesis Imperfecta. Despite her constant struggle with OI, which is out of her control, the school system did not hesitate to expel Tays, a strong student who loves math and is aspiring to be a doctor.
"It takes a long time to get to hospital," says Alina Núñez Yeyna, Tays' mother. When Tays fractures, she has to go to many doctor visits and it take a long time to get there. The wheelchair makes traveling by bus complicated and lengthy. The trek is 2-3 hours from her home to San Miguelito, and another hour and a half to the hospital. During the school year, these doctor visits result in absences from school. Tays missing school is not ideal for her or the family, but there is no other option.
The Crystal Children Foundation fought an uphill battle with the school administration for Tays to be accepted back at school. "I had to fight for it because this should never have happened," says Dayana Castillo, director of the Crystal Children Foundation. "Children with Osteogenesis Imperfecta break bones, but mentally they are normal people and the school administration has taken away her right to prosper and develop." Tays was truly touched by the efforts of the Foundation.
Unlike other children with OI, Tays has public health insurance, which covers certain things but not all. Surgery in 2004 to add pins to her femur strengthened her legs and her spirit. The most important medicine Tays needs though is not available to her in Panama. Despite the daily struggles, Tays has built a great support system. Yeyna says the house is always filled with friends and family visiting Tays, as you can see in the photos below. This support system, and the support of the Foundation have all helped to keep the beautiful smile on Tays’ face.