Life, love, and bleeding hearts at hemophilia camp
MANILA, Philippines - Erickson Kayle Acosta is a good-looking boy. Precocious at nine years old, he can carry on an animated one-sided conversation indefinitely. This is his first trip outside the country — his first time to ride an airplane, as a matter of fact. He is as excited as any other boy in his shoes would be. But he is not like most other boys. His mom, Myra , needs to keep an eye on him constantly. Erickson is a hemophiliac.
Erickson was one of 20 children and youths with hemophilia who participated in the first-ever regional Asia Pacific Hemophilia Camp, which was held recently at the picturesque Pullman Putrajaya Lakeside hotel complex, located south of Kuala Lumpur in Malaysia. All winners of the “Design Your Own Hemophilia Heroes” cartoon drawing and video contest, the boys came from four countries in Southeast Asia, five from Indonesia, seven from Malaysia, four from Thailand, and four from the Philippines, together with their moms, dads, or caregivers. They were also joined by representatives from the medical and patient associations from the four countries as well as from Taiwan and New Zealand.
Hosted by the Hemophilia Society of Malaysia and supported by Bayer Schering Pharma Asia Pacific, the three-day camp had for its theme, “Love, Learn, Live,” with expert speakers and resource persons as well as fun-filled activities intended to raise awareness and encourage patients and parents support group dialogue. It called for “a more proactive management of hemophilia to give children and youths with the disease better opportunities to lead the lives they choose.” The aim is to improve the standard of hemophilia care in the region.
Lifelong Disorder
Hemophilia is a lifelong blood clotting disorder that affects primarily one in every 5,000 males. The blood fails to clot normally because of a deficiency or an abnormality of one of the clotting factors. The two most common types are hemophilia A, where there is little or no clotting factor VIII, and hemophilia B, where there is little or no clotting factor IX.
“The absence or lack of the clotting factor can cause prolonged or spontaneous bleeding, especially in the muscles, joints, or internal organs,” explains Dr. Ernesto Yuson, clinical hematologist and head of the Hematology-Oncology unit at the Fe Del Mundo Medical Center in the Philippines. “A person with hemophilia does not bleed more profusely or faster than normal, but bleeding may last longer. Without treatment, hemophilia can cause crippling pain, severe joint damage, disability, and even early death.”
According to the World Federation of Hemophilia (WFH), approximately 400,000 people around the world have hemophilia A, which accounts for 85 percent of people with hemophilia, but only 25 percent is currently receiving adequate treatment. “There are about 1,100 with hemophilia in Malaysia as well as in Indonesia, 1,780 in Thailand, and 850 in the Philippines who are registered with their local patient groups and associations,” Dr. Tun Maizura Mohd Fathullah, head of the Transfusion Medicine division at the National Blood Centre in Malaysia, notes. Actual prevalence, however, is generally unknown due to under-diagnosis or lack of reporting.
“Over 5,000 people are estimated to have hemophilia in our country,” says Marietta Charvet, president of HAPLOS (Hemophilia Association of the Philippines for Love and Science), “but only 853, 70 percent of which are 18 years old and below, are registered in the local patients registry.” Two of Marietta’s three grown sons have hemophilia. One is married. “A certain social stigma accompanies the disorder,” she observes. Many find the prospect of marriage bleak. It is usually inherited and most commonly found among males, females are rarely affected but are known to be carriers.
There is low awareness and the disorder is commonly misunderstood. Some mistake it for anemia. In Taiwan , it is mistaken for leukemia, since in Taiwanese, only one letter in the word spells the difference.
Effective Treatments
Although there is no cure for hemophilia, there are effective treatments available, and with adequate care, people born with hemophilia do not have to die young. Hemophilia experts are concerned about the urgency of educating medical professionals as well as parents and caregivers on the latest in the management of the disorder.
Original therapies of hemophilia A provided factor VIII replacement through whole blood and plasma. This, however, required hospitalization and carried the risk of blood-borne pathogen transmission and infection. Recombinant human factor VIII treatments are now available. On-demand treatments are given after bleeding incidents occur, while prophylaxis, the regular use of clotting factor concentrates on an ongoing basis, can prevent bleeds before they start, helping reduce or prevent joint damage. Today, most bleeding episodes can be treated at home. Gene therapy is also being investigated as possible cure.
The standard of care in Asia varies widely. WFH estimates that more than 75 percent of people with hemophilia in Asia do not receive adequate care. “In Taiwan, most of the problems we had in the past have been solved or improved after the first Hemophilia Treatment Center was established in 1984 at the National Taiwan University Hospital,” says Prof. Ming-Ching Shen, associate chairman at the Changhua Christian Hospital department of internal medicine in Taiwan. “Hemophilia care is now fully supported by national health insurance.”
The National Blood Centre in Malaysia was established as the country’s center of excellence for the management of hemophilia and provides free treatment and medication. Treatments of factor concentrate are fully funded by the government. In Indonesia, reimbursement for medical treatment is available to government employees as well as financial assistance through the Indonesia Health for Poor People while in Thailand, financial support is provided to civil servants as well as through the National Health Service and handouts from social security. They have 29 regional hospitals and six university hospitals equipped to treat hemophilia. In 1984, the WFH established an International Hemophilia Training Centre in Thailand which is jointly run by the Hemophilia Society of Thailand and serves as a training center for Southeast Asia .
The Philippines has 104 hematologists and five treatment centers nationwide, but there is no form whatsoever of government reimbursement or insurance coverage for hemophilia treatment. Only 10 percent of registered patients are able to afford the needed factor VIII or factor IX products. It could mean a huge drain on the pocket. When Jared, 16-year-old son of Malu Formalejo, who is married to a doctor, suffered cranial bleeding, one week of treatment cost close to P1 million.
Patient associations, such as HAPLOS, HAP-C (Hemophilia Association of the Philippines, Cebu ) and PHF (Philippine Hemophilia Foundation), rely heavily on donations and aid in order to purchase medication for patients. Lidivin de la Rama Balio, a policewoman in Davao whose husband is a taxi driver, is a member of HAND (Hemophilia Association in Davao ). When her 12-year-old son Karl Dave arrived with the group in Kuala Lumpur, he needed a wheelchair due to spontaneous internal bleeding in his knee. Later, he was able to walk normally after a single infusion of factor VIII, which would have cost at least P4,000 back home. The treatment is not only expensive in the Philippines but is also not readily available. “We need the support of government,” Dr. Yuson observes. “We can learn from the experience of our Asian neighbors.”
“An effective and safe routine prophylaxis treatment should be encouraged to promote the standard of hemophilia care in the region,” Dr. Tun remarks. “There has been tremendous progress over the past few years to greatly improve the quality of life of people with bleeding disorders, helping them to be more independent and productive.”
Winning Video
“I don’t want to be different. I want to be treated equally,” says a 13-year-old Indonesian boy in his winning video. His mother, a teacher at the university whom he considers his hero, says, “It’s important to give them the confidence that they can do daily chores.”
The winning video from Malaysia showed a boy riding his bicycle through the streets of his neighborhood. “We cannot always be with him,” says the voice-over. “He must take the journey by himself.” Just going out and doing some exercise is also a form of heroism.
Cycling is a good exercise to protect joints and maintain a fitness level. But care must also be taken, as in other sports such as bowling, running, and basketball. Contact sports such as football and soccer are definitely not recommended. “They can take part in any physical activity, but like any other, there should be sufficient warm-up and cool-down,” says Puan Sarkuna Devi, physiotherapist at Hospital Kuala Lumpur. “Keeping yourself fit and healthy means that you are always ready for anything that life throws at you.”
Devi showed how to tone muscles with the use of Thera-Band, elastic resistance bands that have been proven to increase strength, mobility, and function, as well as reduce joint pain. “Strong muscles will protect your joints,” says Devi. “The enemy of hemophiliacs is muscle weakness, which may lead to arthritis. Do the treadmill or walk regularly. Swimming is the best exercise as it takes care of all the joints in the body.”
Jared, who is in third year high school at La Salle, Greenhills, is eager to join a swimming club for the disabled in Valle Verde, which he heard about recently. Being different does not bother him. “If you think about it, abnormal actually means above normal,” he says. In his winning video, he declares, “If others can do it, I can do it better.”
“Behind every hero, we have another hero, the mother,” someone observed. “Bringing up a child with hemophilia is exhausting,” a mother says. There’s a thin line between being protective and solicitous, and actually spoiling the child. Elisa Olivera, who works in a casino, has two sons with hemophilia, while her only daughter is a carrier. “I want my sons to become priests and my daughter, a nun,” she says while keeping a poker face. In her 13-year-old son’s winning video, Patrick Bryan states, “Anybody can be a hero, even without wings or special powers. Heroes make others proud and happy. I can be one. It’s not about the blood. It’s about the life you give others.”
The activities at the camp were designed to build self-confidence and determination in the young participants with hemophilia. It also fostered healthy relationships. Patrick and Erickson were strangers until they met at the camp. Now, they call each other “best friends forever.”
