Expanding hemophilia care in developing countries

The World Federation of Haemophilia (WFH) estimates that worldwide, approximately 70% of patients with hemophilia are underdiagnosed and untreated. Most live in developing countries. Although in developed countries the life expectancy of hemophiliacs is close to that of healthy persons, this is not the case in developing countries. Great disparity also exists in the treatment of hemophiliacs, especially when this relates to available factor concentrates. There are many reasons for the inadequate care of hemophilic patients: the perception of rarity of the disease; lack of laboratory facilities to diagnose the disorder; lack of understanding of the disorder by patients, their relatives, and even healthcare providers; poorly developed blood bank facilities; and lack of adequate factor supply are just some examples. The WFH attempts to address many of these issues by establishing hemophilia care programs and by educating and training healthcare practitioners so that a healthcare team can be organized that attempts to ameliorate these problems and provides treatment options. In the last few years, a considerable number of developing countries have been organized to deliver at least a minimum of care, and attempts have been made to obtain support from appropriate governmental sources.     

Demographics of hemophilia in developing countries

Demographic datasets pertaining to populations are extremely valuable tools in healthcare planning. They are vital in setting priorities, allocation of resources, measurement of outcomes, and comparison of alternate approaches. Countries with emerging economies especially need information regarding targeted populations when initiating programs designed to deliver care to persons with chronic conditions such as hemophilia. The problems associated with data collection in these countries are huge but surmountable. The World Federation of Haemophilia (WFH) Global Survey provides a valuable synopsis of current global data on hemophilia patients and has provided insight into the extent of the problem with hemophilia worldwide. More and more countries recognize the uses of these data and have established or are in the process of establishing registries for persons with hemophilia to try and improve the quality of the information provided to the WFH. This information will most certainly assist in guiding the future of hemophilia care in these countries with emerging economies.     

Transfusion-transmitted infection in hemophilia in developing countries

Treatment of patients with bleeding disorders (especially those with hemophilia) with blood products has been associated with infections with blood-borne viruses. Of these, hepatitis B and C viruses (HBV and HCV, respectively) and the human immunodeficiency virus (HIV) have created major health problems. Although virus-inactivation procedures have virtually eliminated these viruses from newer factor concentrates since 1985, the risk remains in developing countries where there is no ready access to these concentrates. Although a few of these countries have established their own fractionation facilities and in others the respective governments make concentrates available, the large majority of countries still face the problems of blood-borne infections. HCV will invariably lead to liver damage and many hemophiliacs who were exposed to the HCV virus will succumb to cirrhosis. Only approximately 10% of hemophilic patients infected with HCV will clear the infection naturally. Coinfection with HIV shortens the life expectancy. The HIV epidemic in hemophiliacs began in the mid-1980s. Patients in developed countries were especially affected because they were predominantly treated with factor concentrates that were manufactured from thousands of blood donors. Hemophiliacs in developing countries have considerably less HIV infection, although it does exist and depends largely on the source of the plasma fractions. Progress has been made not only in the purification of factor concentrates, but also in the understanding of the HIV virus and in the development of antiretroviral treatment modalities. However, there are still several challenges in delivering antiretroviral treatment that must be addressed before the full impact of these transmitted infections is known.     

Carrier detection and prenatal diagnosis of hemophilia in developing countries

Hemophilia A and B, inherited as X-linked recessive traits, are the most common hereditary hemorrhagic disorders caused by a deficiency or dysfunction of coagulation factor VIII (FVIII) or FIX, respectively. Hemophilia is prevalent worldwide, without ethnic or geographic limitations, and remains a life-threatening and often disabling condition. Advances in molecular medicine in this century have markedly improved hemophilia treatment. However, management is still largely inadequate in developing countries. Therefore, carrier detection and prenatal diagnosis remain the key steps for the prevention of the birth of children with hemophilia in developing countries where patients with this coagulation disorder rarely live beyond childhood. Carrier detection and prenatal diagnosis are possible by direct or indirect genetic analysis of the F8 or F9 genes. In countries with more advanced molecular facilities and higher budget resources, the most appropriate choice in general is a direct strategy for mutation detection by prescreening techniques or direct mutation detection. However, in countries with limited facilities and low budget resources, carrier detection and prenatal diagnosis are usually performed by linkage analysis with genetic markers. This article suggests the possibilities of genetic diagnosis and a feasible strategy for carrier detection and prenatal diagnosis in families with hemophilia A and B in developing countries.     

The role of physical therapy and rehabilitation in the management of hemophilia in developing countries

Without replacement therapy, patients with severe hemophilia (PWH) will have five damaged joints by the age of 20, which lead to limitation of activities and prevent normal participation in society. Of all PWH, 80% live in developing countries, where access to factor replacement is limited. Physiotherapy and rehabilitation help to prevent disabilities, stimulate activities and participation, and help to preserve autonomy. Rehabilitation should be organized by creating awareness that physiotherapy and rehabilitation (next to availability of safe clotting factor) are very important in developing countries. Locally available medical and social systems and materials should be used for both education and aids and adaptations. Trainers must be trained to develop local treatment protocols. PWH and their families need to be educated and trained to do exercises. Important treatment modalities are management of pain, active muscle strengthening exercises, combined measures and exercises to regain range of motion, training proprioception and coordination, functional training, and orthotics and shoe adaptations.     

Management of chronic synovitis in patients with hemophilia: with special reference to developing countries

Chronic synovitis in severe hemophilia is a painful debilitating condition eventually affecting greater than 20% severe hemophilia patients in developing countries. Though this complication is all but eliminated in the countries with advanced hemophilia care and having access to generous factor concentrate replacement, the same can not be said for 80% of the hemophilia patients in the world who live in developing countries. In developing countries chronic synovitis can be treated conservatively with short course of steroid, factor replacement, physiotherapy and splintage. Failing this, chemical or radioactive synoviorthesis give worthwhile remission and relief in 70–80% of the severely affected patients who fail to respond to conservative therapy. We found a short course of Cox-2 (etoricoxib) inhibitor to be an extremely useful adjunct. The role of d-Pencillamine, Thalidomide and inhibitors of matrix metalloproteinases needs to be explored. HLA-B27 was found to be strongly associated with chronic synovitis in hemophilia in one of the studies and this marker in a hemophilia patient may suggest need for more intensive replacement and other therapy in these patients to prevent chronic synovitis.     

Management of hemophilia with minimal factor replacement in developing countries: role of ancillary therapy

Compared with developed countries, the care of hemophiliacs in still relatively poor in developing countries. There are three major factors that influence this problem: inadequate knowledge, lack of laboratories to diagnose the disorder, and inadequate supply of factor concentrates. It is important that healthcare providers and family members of these patients be adequately educated about all of the aspects of hemophilia. Basic laboratories could be established at a reasonable price, technical personnel could be trained, and quality-assurance programs could be initiated. Even in the absence of high-quality factor concentrates, which are expensive, there are affordable means to manage these patients, although certainly not optimally. To overcome these problems, hemophilia care services need to be established and integrated to facilitate the basic needs for these patients.     

Training programmes for developing countries

Summary Unprecedented gains have been made in the early detection and prevention of childhood metabolic diseases. Over the last 12 years under the International Atomic Energy Agency (IAEA) Technical Cooperation Programme there have been newborn screening (NBS) projects in all the key regions—Asia, Africa, Latin America and Europe. A total of 29 projects have been undertaken at an estimated total cost of US$6.7 million, mainly focused on establishing and supporting NBS for congenital hypothyroidism. Under these projects professional training has been one of the key objectives. This short report provides a summary of the training efforts under these projects. Competing interests: None declared     

Aging in developing countries

After presenting informations about the general life conditions in the less developed regions, the author concentrates on the term "syndrome of poverty", which have a great impact on the life conditions and the status of the elderly. Demographic data concerning the world population of all ages and the aged population in the more developed and the less developed regions follow.     

Sarcoidosis in developing countries

Sarcoidosis is seen in different parts of India and other developing countries with almost similar frequency as in the West. It was largely due to lack of awareness and non-availability of investigations for diagnosis that the disease was reported to be rare in the past. A combination of clinical, radiologic, and histologic criteria are used to diagnose sarcoidosis. A confident exclusion of other causes of granuloma formation, especially tuberculosis, is required. Absence of mycobacteria and of caseation in the histologic specimens and presence of skin anergy to tuberculin help make a diagnosis. Transbronchial lung biopsy obtained with the help of fiberoptic bronchoscopy is positive in about 80% of patients. Corticosteroids are used to treat patients with symptoms and those showing active organ involvement. Aggressive treatment is required for patients with acute and severe pulmonary, cardiac, ocular, or neurologic involvements.     

Diabetes in developing countries

There has been a rapid escalation of type 2 diabetes (T2D) in developing countries, with varied prevalence according to rural vs urban habitat and degree of urbanization. Some ethnic groups (eg, South Asians, other Asians, and Africans), develop diabetes a decade earlier and at a lower body mass index than Whites, have prominent abdominal obesity, and accelerated the conversion from prediabetes to diabetes. The burden of complications, both macro‐ and microvascular, is substantial, but also varies according to populations. The syndemics of diabetes with HIV or tuberculosis are prevalent in many developing countries and predispose to each other. Screening for diabetes in large populations living in diverse habitats may not be cost‐effective, but targeted high‐risk screening may have a place. The cost of diagnostic tests and scarcity of health manpower pose substantial hurdles in the diagnosis and monitoring of patients. Efforts for prevention remain rudimentary in most developing countries. The quality of care is largely poor; hence, a substantial number of patients do not achieve treatment goals. This is further amplified by a delay in seeking treatment, “fatalistic attitudes”, high cost and non‐availability of drugs and insulins. To counter these numerous challenges, a renewed political commitment and mandate for health promotion and disease prevention are urgently needed. Several low‐cost innovative approaches have been trialed with encouraging outcomes, including training and deployment of non‐medical allied health professionals and the use of mobile phones and telemedicine to deliver simple health messages for the prevention and management of T2D.     

Prevention in developing countries

Developing countries have implemented primary health care programs directed primarily at prevention and management of important infectious and nutritional problems of children. Successful programs have emphasized the need for individual and community involvement and have been characterized by responsible government policies for equitable implementation of efficacious and cost-effective health interventions. Unfortunately, developing countries must also face increases in the chronic disease and social problems commonly associated with industrialized countries. Prevention efforts, for example, to reduce tobacco smoking, to modify the diet, to reduce injuries, or to avert environmental contamination, are needed to contain future morbidity and rapidly increasing medical care costs. Developing countries can build on their successful approaches to program implementation and add other measures directed at preservation of health and prevention of disease in adult as well as child populations. Received from the Department of International Health, The Johns Hopkins University School of Hygiene and Public Health, 615 North Wolfe Street, Baltimore, Maryland 21205.     

Information technology for health in developing countries

Poverty has deepened the crisis in health-care delivery in developing countries, particularly sub-Saharan Africa, which is a region facing a disease burden that is unmatched in the world. Whether access to proven and powerful information and communication technologies (ICTs) can improve health indicators is an ongoing debate. However, this brief review shows that in the last decade there has been significant growth in Internet access in urban areas; health-care workers now use it for communication, access to relevant health-care information, and international collaboration. The central message learned during this period about the application of ICTs is that infrastructural and cultural contexts vary and require different models and approaches. Thus, to harness the full potential of ICTs to the benefit of health systems, health workers, and patients will demand an intricate mix of old and new technologies.     

Hypertension in developing countries

Data from different national and regional surveys show that hypertension is common in developing countries, particularly in urban areas, and that rates of awareness, treatment, and control are low. Several hypertension risk factors seem to be more common in developing countries than in developed regions. Findings from serial surveys show an increasing prevalence of hypertension in developing countries, possibly caused by urbanisation, ageing of population, changes to dietary habits, and social stress. High illiteracy rates, poor access to health facilities, bad dietary habits, poverty, and high costs of drugs contribute to poor blood pressure control. The health system in many developing countries is inadequate because of low funds, poor infrastructure, and inexperience. Priority is given to acute disorders, child and maternal health care, and control of communicable diseases. Governments, together with medical societies and non-governmental organisations, should support and promote preventive programmes aiming to increase public awareness, educate physicians, and reduce salt intake. Regulations for the food industry and the production and availability of generic drugs should be reinforced.     

Osteoporosis in developing countries

Osteoporosis poses a huge challenge in developing countries due to demographic transition and aging of the population coupled with limited availability of resources. The exact disease burden is difficult to quantify because of the paucity of data. Ethnicity affects bone density as well as fracture risk. Population-specific normative data for bone density are lacking in large parts of the world. Vitamin D deficiency is common even in sunny countries. The WHO has developed an algorithm for estimation of 10-year fracture risk which may be used even in the absence of bone mineral density.