Hemophilia is a chronic medical disorder that causes internal bleeding and can result in deformity, joint mobility impairment, chronic pain, and narcotic medication abuse. There is no cure for hemophilia, so psychological interventions are necessary to help the affected individuals manage their condition and emotionally cope with the consequences of the disorder. Even though psychological interventions are considered an essential part of care delivery to hemophilia patients and their families, most of the interventions focus on acceptance, coping strategies, and cognitive-behavioral changes while neglecting potential psychiatric problems associated with hemophilia. This paper will discuss the importance of psychological interventions for improving quality of life in hemophilia patients and their families, but it will also discuss the barriers to effective psychosocial care delivery to hemophilia patients.
Hemophilia is a rare, incurable hereditary blood clotting disorder, but spontaneous gene mutation also accounts for up to 40% of hemophilia cases (Cassis et al., 2012, p. 2). The clinical manifestations of hemophilia usually include bleeding in the joints or muscles, but the bleeding can also occur in other areas, such as soft tissue and the gastrointestinal tract. If untreated, hemophilia results in chronic arthropathy, and the patient experiences reduced mobility, deformity, and chronic pain (Cassis et al., 2012, p. 2). To prevent complications, the current medical treatment for hemophilia is clotting factor replacement therapy, which is administered intravenously upon hemorrhage or routinely as prophylaxis in severe cases.
However, worldwide surveys show that only 37% of hemophilia patients received prophylactic therapy while only 54% of them received on-demand treatment (Coppola et al., 2010, p. 25). While lifestyle, cost, and clotting factor concentrate availability determine the adherence rates to treatment, it is also considered that implementing a multi-disciplinary approach to hemophilia treatment by educating patients and providing psychological support is required to understand the needs of the patients and improve treatment compliance rates (Coppola et al., 2010, p. 25). Although patients with hemophilia require medical attention to prevent physical complications, their illness also affects their psychosocial condition, so it is reasonable to suggest that combining psychological interventions and medical treatments will improve the quality of life in affected individuals.
Because there is no cure for hemophilia, the patients and their families can experience various adverse events when they are unable to cope with the cost of treatments or the consequences of the disorder. For example, suicide is a common occurrence among hemophilia patients because they fail to cope with chronic pain, the abuse of high narcotic medication doses, and socioeconomic caused by their inability to continue working or performing other daily activities (Fakhari & Dolatkhah, 2004, n. pag.). Therefore, providing emotional support to the affected individuals through psychological interventions is critical in healthcare delivery.
The psychological interventions used can depend on various factors (e.g. treatment objectives, patient characteristics, disease severity, and the physical and emotional state of the patients), and they usually include cognitive-behavioral therapy (CBT), psycho-education, systematic therapy, and psychotherapy (Cassis et al., 2012, p. 3). These types of interventions are the most prevalent, but some approaches are not control-oriented. The acceptance of hemophilia through acceptance and commitment therapy (ACT) is an alternative approach that emphasizes acceptance over control, and it is also considered an effective psychological intervention in hemophilia (Cassis et al., 2012, p. 3).
Although control-oriented and acceptance models are contradictory, each type of intervention can be effective depending on the circumstances. For example, psycho-education therapy is a common intervention immediately after the diagnosis. It is an essential intervention when the disease is diagnosed in infants and toddlers because parents can experience guilt for passing a hereditary disorder to their child, anxiety, disappointment, or anger (Cassis, 2007, p. 2). Psycho-education can teach parents the basic knowledge they need to ensure their child’s safety and to help the child build confidence instead of being overprotective. Over time, the psychosocial support gradually shifts the attention from the parents to the children to help them achieve and maintain normal cognitive and social development (Cassis, 2007, p. 5).
Besides psycho-education, CBT and ACT are two distinct approaches designed to help hemophilia patients cope with chronic pain. The CBT approach focuses on reducing physical pain by modifying sensations, avoiding catastrophic thinking, and transforming maladaptive behaviors (Wetherell et al., 2011, p. 2098). While the CBT model is control-oriented, the ACT treatment model proposes different objectives (i.e. non-judgmental acceptance of all experiences, identification of personal values, and life actions aimed towards satisfying those values) to improve quality of life and decrease pain (Wetherell et al., 2011, p. 2098).
Despite the contradictory viewpoints proposed by the different approaches, both approaches appear effective. Both CBT and ACT showed similar positive outcomes (i.e. reduced depression, improved pain interference, and reduced anxiety) on the quality of life and psychological well-being in hemophilia patients (Wetherell et al., 2011, p. 2104). From the patients’ perspectives, CBT was considered more credible than ACT because of the reasoning behind the interventions, but ACT was described as more satisfying than CBT because of the higher patient engagement in the treatment (Wetherell et al., 2011, p. 2104).
However, the exact mechanisms responsible for the positive outcomes of psychological interventions in hemophilia patients are still unknown. Current research indicates that the importance of pain acceptance in hemophilia might be overemphasized because mediation analyses showed that the patients’ sense of control over pain was a more important determinant of well-being than acceptance of pain in both CBT and ACT interventions (Wetherell et al., 2011, p. 2105). Therefore, further research is required to clarify the role and effects of both acceptance and control in pain management for hemophilia patients. It is possible that ACT interventions were effective for pain management because of the emphasis on value-driven activities rather than the non-judgmental attitude towards chronic pain. Another possibility is that acceptance is only the first stage of coping with hemophilia that enables patients to learn how they can manage the symptoms. While both approaches show high success rates, identifying the exact reason for psychological well-being improvement could refine the available interventions and facilitate care delivery.
Although it is clear that psychological interventions in hemophilia are necessary to address the emotional and psychological needs of the patients, there is no unified model for adjusting care delivery to hemophilia patients at different stages of development. One systematic review was designed to perform a subgroup analysis to identify confounding variables (i.e. treatment, age, inhibitor status, and life-stage during treatment) associated with positive treatment outcomes (Cassis et al., 2012, p. 6). However, the results are not yet published, so the associations between the confounding variables and intervention outcomes is still not identified and mapped. Therefore, planning interventions in hemophilia at the moment relies on evaluating broad life-stage objectives and personality characteristics rather than empirical evidence that shows the most effective treatment models for different age groups.
Furthermore, most studies address the coping strategies for issues associated with hemophilia and those interventions are often implemented as a part of the routine hemophilia care, but clinical diagnoses of psychological disorders in hemophilia patients are often neglected in both research and practice (Fakhari & Dolatkhah, 2004, n. pag.). Hemophilia patients often suffer from a variety of psychological issues, including anxiety (64.55%), depression (75%), aggressive traits (47.9%), obsessive-compulsive disorder (77.05%), and somatization (64.56%) (Fakhari & Dolatkhah, 2004, n. pag.). With the high prevalence of psychological issues among hemophilia patients, it is clear that psychotherapy might be necessary for a lot of hemophilia patients, so proper screening protocols also need to be included in routine care delivery.
The development of new medical diagnostic tests and treatment options for hemophilia also brings up new choices and dilemmas for physicians, patients, and parents. For example, it is possible to order prenatal testing with chorionic villus sampling (CVS) to identify the disorder in the first trimester, but several short-term psychological consequences, such as negative self-perception and depression, have been associated with CVS testing (Coppola et al., 2010, p. 22). Although there are no long-term psychological effects associated with CVS, a positive test result opens the possibility of terminating the pregnancy during the first trimester (Coppola et al., 2010, p. 22). That option poses a significant moral, religious, and legal dilemma for the mother who needs to decide between giving birth to a child with a chronic illness or violating personal beliefs and values.
There are also various cultural differences that need consideration when planning psychological interventions at different stages of development. For example, different cultures can have different perceptions of the stages of development, so adolescents in some societies are expected to marry young and start working instead of continuing education (Cassis, 2007, p. 9). Therefore, the objectives of counseling or psychosocial support will depend on both the stage of development and cultural background of the patient.
The most significant barrier to receiving proper hemophilia treatment, both medical treatment and psychosocial support, is geographic location. In some regions of the world, the learning materials about hemophilia are outdated while the medical staff is often unprepared to provide care to hemophilia patients (Cassis, 2007, p. 11). The lack of understanding of hemophilia can contribute to social stigmatization, which leads to isolation and decreased psychological well-being (Cassis, 2007, p. 11). Even in the developed regions of the world, some people still lack access to proper care because they live in remote areas. Without access to medication and healthcare facilities specializing in hemophilia treatments, people find it difficult to manage chronic pain, manage adverse symptoms, and receive social or emotional support (Cassis, 2007, p. 11).
In conclusion, psychological interventions and emotional support should be an integral part of hemophilia care delivery, but there are several obstacles that need to be identified and resolved to improve care delivery to hemophilia patients. Although various psychological interventions proved effective, further research is warranted to improve psychological intervention planning. Most treatments focus on emotional and psychosocial support delivery, but it is also important to consider that hemophilia might contribute to the development of severe psychological disorders, so psychiatric screening with clinical questionnaires should be routinely administered to identify individuals at risk for psychiatric disorders and implement timely interventions. In addition to assessing the needs of the patients and their families, hemophilia care delivery staff must also understand the cultural differences to effectively plan psychological interventions. Finally, the implications of technology in medicine for remote psychosocial care delivery should also be investigated to improve emotional and social support delivery to patients living in remote locations.
References
Cassis, F. R. M. Y. (2007). Psychosocial care for people with hemophilia. Treatment of Haemophilia Monograph Series, (44), 1-12.
Cassis, F., Emiliani, F., Pasi, J., Palareti, L., & Iorio, A. (2012). Psychological interventions for people with hemophilia. Cochrane Database of Systematic Reviews, (11),
Coppola, A., Cerbone, A. M., Mancuso, G., Mansueto, M. F., Mazzini, C., & Zanon, E. (2010). Confronting the psychological burden of haemophilia. Haemophilia, 17(1), 21-27.
Fakhari, A., & Dolatkhah, R. (2004). Psychiatric disorders in hemophilic patients. Anxiety, 14(43.75), n.pag. Retrieved from http://priory.com/fam/hemophil.htm
Wetherell, J. L., Afari, N., Rutledge, T., Sorrell, J. T., Stoddard, J. A., Petkus, A. J., & Hampton Atkinson, J. (2011). A randomized, controlled trial of acceptance and commitment therapy and cognitive-behavioral therapy for chronic pain. Pain, 152(9), 2098-2107.
