Few would disagree that haemophilia presents challenges for everyone who lives with it. Perhaps the greatest of these is being denied the opportunities available to other people through fear of bleeding and joint damage. The regular infusions and clinic appointments that characterise treatment are certainly burdensome, and sometimes traumatic for children and parents alike; but being told ‘People with haemophilia can’t do this…’ is damning in its finality. It is enough to make you demand, ‘Why not?’ - which is exactly what Clive Smith and a growing number of people in the haemophilia community are asking.
Sickle cell disease and haemophilia are rare inherited disorders that require intensive management. Few cases of people with both disorders have been reported. This case study describes the impact of living with these disorders and coping with their management on a boy and his mother.
Karolina Mielko, Ewelina Soroka, Karolina SprawkaD and Marcin Olajossy
Introduction The authors present an overview of current views on the treatment of obsessive-compulsive disorder refractory to pharmacological and psychological treatment.
Aim: To review the mechanisms of stimulation of deep brain structures and to evaluate the effectiveness of therapy in obsessive-compulsive disorder.
Method: Review and analysis of the Polish and foreign scientific articles from the years 1999-2016.
Conclusions: According to the literature considered, in half of the examined patients there was an improvement of over 35% on the Y-BOCS scale, in some patients even a reduction of symptoms reaching 81-83% was described. Previous studies have been carried out on small groups of patients. Since 2009, the method of invasive treatment with deep brain stimulation of the obsessive-compulsive syndrome is registered in the EU. In spite of the above, additional studies are necessary on a larger group of patients in order to precisely estimate the effectiveness of the procedure and elaborate the criteria for qualifying patients for inclusion in the procedure.
Courtney Schroeder, Richard Fw. Barnes, Lena Volland, Sonha Nguyen and Annette von Drygalski
Background: Low impact physical activity is important for patients with haemophilic arthropathies, but is often considered boring with suboptimal adherence. There is therefore a need for physical activities that motivate patient engagement. Aims: To evaluate the benefits of top rope climbing, increasingly used in other musculoskeletal disorders, as an engaging sports discipline in haemophilia. Materials/Methods: Six adult arthropathic patients with haemophilia (PWH) completed 12 sessions of tailored top rope climbing training. Functional and clinical joint status, climbing skills, quality of life (QoL), annual bleed rate (ABR) and joint findings with musculoskeletal ultrasound/power doppler (MSKUS/PD) were assessed before and after climbing. Results: Haemophilia joint health scores, dorsiflexion in arthropathic joints and climbing skills all improved. ABRs were comparable before and during climbing, and QoL remained high. MSKUS evaluation demonstrated no detrimental effects on synovial and cartilage health, with a decrease of inflammatory PD signal in some joints. Conclusion: We conclude that top rope climbing therapy (known as “Haemophilia Vertical”) can improve joint health in PWH with arthropathies. Haemophilia Vertical therefore emerges as an innovative athletic concept to promote physical activity among PWH. Further study investigating the longer-term impact in a larger cohort is warranted.
Introduction: Gamma-hydroxybutyric acid (GHB) is commonly known as a recreation drug or the so-called “date rape drug”. It is also used in medicine to treat narcolepsy and alcohol addiction. GHB has an affinity for two types of receptors: GABAB and the relatively recently discovered GHB receptors. GHB receptors were first cloned in 2003 in mice and then in 2007 in humans. So far, evidence has been presented for their impact on dopaminergic transmission, which may imply that they play a role in the pathogenesis of diseases such as schizophrenia. At the same time, it has been demonstrated that benzamide antipsychotic drugs have an affinity for GHB receptors, which is why it is postulated that some of the effects of these drugs may result precisely from this affinity.
Aim: The study presents the current state of knowledge about GHB receptors and their potential role in the pathogenesis of schizophrenia, and discusses drugs which show an affinity for this receptor.
Material and method: The literature review was based on a search of articles indexed between 1965 and 2018 in Medline, Google Scholar, ScienceDirect and Research Gate databases. The following search terms were used: GHB receptor, GHB, sulpiride, and amisulpride.
Result and discussion: 1. It is possible that GHB receptors are involved in the pathogenesis of schizophrenia, although more research is needed in this area. 2. Part of the effects of some benzamide antipsychotic drugs (such as amisulpride) may be due to their affinity for GHB receptors.
Galila Mokhtar, Amal El-Beshlawy, Mohssen El Alfy, Magdy El Ekiaby, Naglaa Omar, Khaled Abd El-Azim Eid, Hoda Hassab, Osama El Safy, Ahmed Mansour, Rasha El Ashry, Naglaa Shaheen and Azza Abdel Gawad
These guidelines have been developed by an expert panel of haemophilia treaters to support the appropriate management of people with haemophilia in Egypt. Although the guidelines are based primarily on the World Federation of Hemophilia (WFH) Guidelines for the Management of Hemophilia, they aim to address unmet needs and local requirements in the Egyptian setting.
This article describes a case of wrongly managed haemophilic arthropathy in a 15-year-old boy in Uganda. Delayed diagnosis of haemophilia and misdiagnosis of haemophilic arthropathy resulted in unnecessary corrective surgery and potentially severe complications. After haemophilia was diagnosed, he was managed by haematologists using factor VIII and regular physiotherapy. The physiotherapy treatment approach focused in particular on functional activities, which was beneficial in achieving quicker results and made the patient’s rehabilitation more meaningful. This case indicates that there is a need for greater awareness of the presentation of haemophilia in developing countries.
Introduction. The basic purpose of this article is to present Jacob Levy Moreno’s psychodrama method as the psychotherapeutic technique useful in work with patients during individual and group psychotherapy.
Material and Method: The author presents the analysis of case studies of patients treated in individual and group psychotherapy. He instances the examples of psychodramatic work on the stage as well as monodramatic and reports their importance in the process of patient psychotherapy. He discusses the effects of these act ivities in the context of changes in the emotional and interpersonal functioning of patients.
Results: The given examples of monodrama and psychodramatic works illustrate the mechanisms of the changes offered by the method, e.g. insight, abreaction, acceptance of internal impulses, confrontation with the feelings of other people, training of alternative behaviors. In the article one can follow each subsequent step of analyzing intrapsychic conflicts of patients, which, thanks to the play on the stage, can be named and experienced by them.
Conclusions: Psychodrama, used in the psychotherapeutic work of the group and in individual work with the patient (in the form of a monodrama), gives great opportunities to broaden the insight of the patient into very complicated internal mechanisms of conflicts and deficits. Psychotherapist - leader encourages patients to be creative and to spontaneous development of their blocked personality elements. The specificity of psychodrama is a relatively quick resolution of many years of ongoing conflict and permanent reparation of traumatic experiences, even from early childhood.
Positive psychology directs its research interests primarily to healthy people. The most important goal is to build a positive attitude towards yourself and the surrounding world. Recently, positive psychology has set a new area of research interest, which is clinical psychology. In recent years, several positive psychotherapy programs have been developed for people with schizophrenia experience. The article presents the latest trends in positive psychotherapy for people with schizophrenia. They involve taking into account the individual differences of each patient and the specificity of his / her psychopathology. As far as the therapeutic goals are concerned, there are interventions focused on strategies for enhancing positive emotions and wellbeing or the method of activating the strengths of character. Taking into account the methods of therapeutic work, they can be divided into training methods or those of the behavioral-cognitive psychotherapy as well as those that take into account the various aspects of meditation. The article presents the distribution of therapeutic programs in terms of the range of therapeutic goals in which the most important are: intensification of positive experiences, building of strengths of character and well-being. Therapeutic programs have been shown to focus not only on breaking down negative attitudes towards one’s own illness and life, but also on those that try to deal with the unsolved schizophrenia problem - negative symptoms.
Background: Scarcity of FVIII concentrate compels caregivers in poor countries to use multiple transfusions of fresh whole blood (FWB), fresh frozen plasma and cryoprecipitate for managing haemophilia A. FWB is the most frequently transfused blood product due to ease of production and its ability to simultaneously stop active bleeding and treat anaemia. Iron deficiency anaemia is common among haemophiliacs in poor tropical countries such as Nigeria, due to the combined effects of bleeding, malnutrition, and haemorrhagic parasitic diseases. Multiple FWB transfusion is usually initiated at local sub-tertiary hospitals before eventual referral to tertiary hospitals. The Nigerian blood transfusion service is underdeveloped, donor screening is rudimentary and transfusion safety is poor. The prevalence of transfusion transmissible viral infections (TTVIs), including HIV, and hepatitis B and C viruses (HBV and HCV), is therefore predicted to be high among Nigerian haemophiliacs. Aims: To determine prevalence and pattern of TTVIs (HIV, HBV, HCV infections) among paediatric haemophiliacs who have received multiple FWB transfusions in Nigeria. Materials and methods: Retrospective analyses of demographic and clinical data, disease severity, number of previous transfusions of FWB, and prevalence and pattern of TTVIs (HIV, HBV and HCV infections) of newly referred haemophiliacs as seen in five tertiary hospitals in northern Nigeria. Prevalence rates of TTVIs were expressed as percentages. Comparisons of parameters (age, disease severity and number of previous transfusions) between patients with and without TTVIs were performed using Students t-test for mean values and Fisher’s exact test for percentages, with p-values of less than 0.05 taken as significant. Results: Of 97 haemophiliacs studied, 24 (24.7%) were infected with TTVIs. The pattern and frequencies of TTVIs among the infected patients revealed HBV infection in 10 (41.7%), HIV-1 infection in five (20.8%), HCV infection in four (16.7%), HBV and HIV co-infection in three (12.5%), and HBV and HCV co-infection in two (8.3%). In comparison with haemophiliacs without TTVIs, haemophiliacs with TTVIs had a significantly lower mean age (4.9 vs. 7.8; p=0.007); a higher proportion of severe disease (62.5% vs. 26%; p=0.009), and a higher mean number of transfusions per patient (27.5 vs. 15.3; p=0.006). Conclusions: The prevalence of TTVIs among haemophiliacs in Nigeria is high, and the risk is correlated with disease severity and number of previous transfusions. There is need for the national transfusion service to be upgraded and for standard haemophilia care centres with an adequate supply of FVIII concentrates for optimal care to be set up. Haemophilia healthcare providers in Nigeria can minimise multiple transfusions by incorporating regular screening and treatment of haemorrhagic parasitic diseases, iron supplementation, and the use of pharmacological agents in the standard of care for haemophilia.