World Hemophilia Day 2021: Challenges for Hemophilia patients during the COVID-19 pandemic and the future of treatment in Thailand
Thailand – World Hemophilia Day, which coincides with April 17 of every year, is the day in which the world comes together to generate awareness on the challenges of living with Hemophilia to the public and relevant stakeholders in order to better understand the importance of delivering efficient treatment to patients. Compounded by the COVID-19 pandemic, patients are faced with mounting complications when it comes to accessing adequate treatment and care at the hospital, as there is potential exposure to the virus.
Hemophilia is considered a rare disease, whereby the World Federation of Hemophilia has estimated the number of cases worldwide at approximately 320,000. It is estimated that Thailand has 5,750 cases of Hemophilia, but in reality only 2,138 or a mere 37% of all patients are officially registered in the Thailand Hemophilia Registry. The remaining 62% of patients with mild to moderate symptoms have not been registered. In other words, the majority of patients are not able to access adequate care and efficient treatments. Symptoms of hemophilia can be detected from a very young age; from surface bruises to joint and muscle bleeds. Some bleedings can present a significant health concern, especially when they involve vital organs, like brain and abdomen. The recurrent bleeds may lead to deformities or cost patients their lives.
Hemophilia is a serious, inherited bleeding disorder in which a person’s blood does not clot properly, leading to prolonged and uncontrolled bleeding, either spontaneously or after minor injuries, which can result in severe bleeds and death. Hemophilia is categorised into two main types namely Hemophilia A and Hemophilia B. The former is more common and accounts for 80-85%, while the latter accounts for the remaining 15-20% of all Hemophilia patients. Additionally, since Hemophilia is carried on the X chromosome as a recessive trait, it is usually transmitted to male offspring with the prevalence at 1: 12,000.
The severity levels of Hemophilia A can be classified into three: mild, moderate, and severe, depending on the degree of deficiency of an essential clotting protein in blood known as factor VIII. The World Hemophilia Federation revealed that in 2019, the Factor VIII consumption per capita in Thailand was 0.622, which despite exceeding the rate for Southeast Asian countries, but is still lagging behind Singapore (2.219) Malaysia (1.458) Korea (5.729). The difference is especially stark when compared to developed countries, such as The United States (7.105) and the United Kingdom (9.026).1 After receiving factor VIII concentrate to reduce the severity of their bleedings for a certain period of time, some Hemophilia A patients can develop factor VIII inhibitor. As a result, medical professionals cannot treat them the same way, and thus caring for this group of patients gets more complicated and challenging. Without appropriate treatment approaches, they may be at risk of disability or even death.
According to the World Federation of Hemophilia, 72% of Hemophilia patients in Thailand are children and adolescent under 18. Such children-to-adult patient ratio is considerably high when compared to other countries, such as the United States at 45%, Malaysia at 39%, the United Kingdom at 26%, and Singapore and Korea at 21%. It also correlates with the average life expectancy of Thai Hemophilia patients at 17.2 years of age. In the past two years, Thailand sees between 5 and 6 deaths of Hemophilia patients per annum.
Additionally, the World Federation of Hemophilia recorded the number of adult Hemophilia patients (aged 45 and above) by geographical regions, and found that Europe has the highest proportion of adult patients at 31%, followed by Africa at 13% and Eastern Mediterranean at 8%. On the other hand, in Southeast Asia, where Thailand belongs to, the proportion of adult patients is only 7%. Therefore, it can be inferred that the higher the ratio of adult-to-children patients is an indicator of comprehensive care.
Apiwat Akarapattananukul, M.D., a severe Hemophilia A patient shared his experience of living with this rare condition, as quoted: “Unlike most boys that enjoy maximum freedom to romp around outdoors or do adventurous activities, I was always told to be extra careful while engaging in any kind of everyday activities. This is due to the disease that I was born with. Hemophilia causes me recurrent and uncontrolled bleedings than other people. In the past, this disease can have a considerable impact on everyday life. Imagine a child who had to find veins for regular injections or was absent from school consistently for hospital visits. Without my parents’ continual support and medical professionals’ adequate treatments and care, I would not be able to fulfill my dreams and do what I love today.”
Furthermore, the COVID-19 pandemic, which has been around for over a year, has posed mounting challenges on the lives of Hemophilia patients who require episodic treatment or hospitalization. “If possible, I want to see a future approach of Hemophilia treatment pivoting to prophylaxis, or the home-based regular injections of clotting factor concentrates in an attempt to prevent bleedings. Prophylaxis minimizes the risk of bleeding and subsequently reduce the frequency of doctor visits. This will in turn lessen the workload of medical professionals as well,” Apiwat added.
In terms of the COVID-19 vaccination, which has been rolled out in many countries including Thailand, the World Hemophilia Federation has publicly announced that Hemophilia patients are not at greater risk of experiencing side effects from the COVID-19 vaccine. In moderate and severe cases, the factor VIII injection should be given before receiving the vaccine to prevent muscle bleeds. Additionally, the smallest gauge needle available (25-27 gauge) should be used. Pressure should be applied to the site for at least 10 minutes to reduce bleeding or swelling. Patients should not be alarmed in case of discomfort around the arm area for 1-2 days, unless the symptoms worsen or any swelling occurs, then they are advised to seek immediate medical help.
Currently there is no cure for hemophilia, but medical advancements makes a meaningful difference in improving patients and caregivers’ quality of lives. Prophylaxis allows them to remain active and participate more fully in daily life, despite being extra careful.
Farid Bidgoli, General Manager, Roche Thailand, Myanmar, Cambodia and Laos said in a closing statement, “For more than 20 years, Roche has been researching and delivering innovative treatments for people with blood diseases of the blood. We have worked closely with various Hemophilia related associations and stakeholders in Thailand and abroad. Thus, we are well aware of the many challenges faced by patients and caregivers. As a result, Roche is committed to improving their quality of lives by making positive impact in the treatment outcomes and access to treatment. We strive to support Hemophilia patients of all ages, so that they can live happy, fulfilling, and long lives and become productive members of society in the future.”
Although rare, it is fortunate that relevant organisations, such as Thai Society of Hematology, the National Hemophilia Foundation of Thailand, Thai Hemophilia Patient Club, and International Hemophilia Training Center-Bangkok, work relentlessly to provide public information on proper physical and mental care. Additionally, there are 52 Hemophilia treatment centres across Thailand to further accommodate patients. Stay abreast of further updates or activities to help spread awareness of World Hemophilia Day 2021 by visiting the Thai Hemophilia Patient Club website at http://www.thaihemophilia.org/ and https://www.roche.co.th/th/disease-areas/haemophilia.html
 WFH. Global Survey 2019
 Thailand Hemophilia registry
 WFH. Guidelines for the management of haemophilia. 2012
 Roche. Hemophilia https://www.roche.co.th/th/disease-areas/haemophilia.html
 Ampaiwan Chuansumrit, et al. (2021), Real-world evidence on health resource use among patients with haemophilia and inhibitor exhibiting severe bleeding episodes, The Official Journal of the World Federation of Hemophilia, 27 (1): 69-80.
 Guidance from the World Federation of Hemophilia (WFH), European Association for Haemophilia and Allied Disorders (EAHAD), European Haemophilia Consortium (EHC), and U.S. National Hemophilia Foundation (NHF). https://news.wfh.org/covid-19-vaccination-guidance-for-people-with-bleeding-disorders/