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Možnosti fyzioterapie u pacientů s hemofilií
PETRÁKOVÁ, Barbora
My bachelor thesis addresses the influence of physiotherapy over the condition of the musculoskeletal system of patients with hemophilia. The aim of this work is to map the possibilities of physiotherapy in patients with hemophilia and subsequently design a physiotherapeutic plan for individual probands, and to assess the effect of therapy. The work is divided into theoretical and practical part. The theoretical part describes the physiology of blood clotting, which is disturbed due to the absence of clotting factors in hemophiliacs. Furthermore, hemophilia disease, its occurrence, types, etiology, diagnosis and its clinical picture are characterized. It also explains in detail the origin of hemophilic arthropathy, which is the final stage of joint damage in this disease. The last part describes the possibilities of physiotherapy in patients with hemophilia, the possibilities are divided into physiotherapy in the acute and subacute stages of the disease. The practical part is focused on the use of physiotherapy methods, which are described in the theoretical part. The researched group consists of three patients with severe hemophilia in the range of the age of 49 to 53 years, who undergo 10 individual therapies. Case studies include the initial kinesiological analyzes, described therapy, and the final kinesiological analyzes. The data collection also includes the standardized Hemophilia Joint Health Score and the A36 Hemophilia - QoL quality of life test. These analyzes also show the changes that occurred after the completed therapies. According to the results, the therapy had positive effects on the probands. I believe that this work could help physiotherapists in their clinical practice as an aid in finding physiotherapeutic methods used in patients with hemophilia. The educational material can also be used by patients as a source of information about this disease or as an aid in self-therapy.
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Impact of Pain in Patients with Hemophilia on their Emotional Experience and Quality of Life
Půčková, Romana ; Raudenská, Jaroslava (advisor) ; Křivková, Jana (referee)
The Bachelor's thesis deals with the issue of the impact of pain on emotional experience and quality of life in haemophiliac patients and the significance of pain management for nursing practice. The aim of the thesis was to tackle problems concerning the disease in adult haemophiliacs in connection with pain, emotional experience and quality of life, describe relations between these variables and determine the main factors influencing pain and quality of life in haemophiliacs. For that purpose, nine basic hypotheses were set and verified by the means of statistical processing of data obtained from a questionnaire survey of 105 adult haemophiliacs of age among 18 to 79 addressed across the whole Czech Republic. The respondents represented patients of both, A and B haemophilia and of all kinds of haemophilia severity. Four instruments were used to carry out the survey - a standardized questionnaire A36 Hemofilia-QoL for determining quality of life, a standardized questionnaire BDI - II for determining depressive symptoms, a standardized questionnaire GAD 7 for determining the level of anxiety and a visual-analogical scale, so-called Thermometer Scale of Emotions. The survey included a semi- structured questionnaire for demographic-anamnesis data. By the means of the instruments mentioned above,...
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The role of peripheral quantitative CT in early diagnostics of osteoporosis in chronically ill children
Souček, Ondřej ; Šumník, Zdeněk (advisor) ; Hoza, Jozef (referee) ; Zapletalová, Jiřina (referee)
Patients with Turner syndrome (TS) have increased fracture risk and decreased bone density, patients with severe hemophilia have low bone density. The etiology of these changes hasn't been completely elucidated in any of the two diseases. Our aimes were to assess bone density and geometry at the radius using a new method peripheral quantitative CT densitometry (pQCT) and to describe associations between densitometry parameters and estrogen treatment in TS and laboratory as well as clinical markers of disease severity in haemophilia. Sixty-seven girls with TS (median age 14.3 years, range 6.0-19.4) and 42 boys with haemophilia (median age 12.7 years, range 6.6-19.2) have been measured using peripheral quantitative CT at the radius. The results have been compared to published reference data. Girls with TS had decreased cortical bone density and thinner cortex. These parameters were positively correlated to the length of estrogen treatment. Trabecular bone density was normal in prepubertal girls but it was decreased after puberty. There was no association between trabecular bone density and the estrogen treatment. Boys with haemophilia had decreased trabecular density and low muscle area at the forearm. Densitometry parameters were influenced neither by the clotting factor VIII/IX level nor by the frequency of...
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Hemophilia in dogs and use of a dog as a model for analogous disease in human
Marvanová, Veronika ; Hofmanová, Barbora (advisor) ; Dokoupilová, Adéla (referee)
This compilation deals about hemophilia in dogs and options, how to apply findings obtained from studies on dogs to cure people. Hemophilia is suitable for research of modern healing methods, because it has been known for centuries and thanks to this its thoroughly researched. As a hereditary, sex-linked coagulation disorder, which is normally cured by replacement therapy, is hemophilia ideal model for examining the genetics determinants and nowadays it significantly helps in development of modern methods of treatment.
Hemophilia in dogs and humans is caused by gene mutation, which brings the absence or dysfunction of coagulation factor VIII or FIX. These factors in a healthy individual participates on coagulation cascade and on blood clot creation. Hemophilia mutations increase incidence of spontaneous hemorrhage, or hemorrhage after minor trauma, and patients suffer by hematomas, blood loss, dangerous internal bleeding, difficulties in movement and other related problems.
The fact, that hemophiliac dogs naturally occurs, in addition with possibility to compare them with people despite the differences in karyotype, because of binding to sex chromosome, and finally in connection with people desire to treat these dogs, just confirms that hemophilia in dogs is suitable for studying. This explain why dog models were involved in all significant discoveries in hemophilia treatment. For example, dogs assisted in development of replacement therapy with plasma and plasma concentrates, where studies confirmed safety and efficacy of these substances. Within recent years dog models enables safety transfer of gene therapy to human patients.
Thanks to these studies hemophilia possibly become the first genetically conditioned disorder, which would be permanently cured by gene therapy.
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Determination of coagulation factor VIII in haemophilia A patients;Evaluation of different methods of measurement depending on the type of mutations in the gene for factor VIII.
HOUSKOVÁ, Kateřina
Hemostasis is essential to life; it is the ability of organism to stop bleeding and to maintain the fluidity of the blood in an intact vascular bed at one time. Factor VIII is called antihemophilic globulin or antihemophilic factor. It is a plasma glycoprotein composed of two noncovalently associated chains. One chain is a heavy one, comprising domains A1- A2- B and the light chain composed of the domains A3- C1-C2. FVIII activity was measured in two ways from historical reasons a) a one-phase method, where a mixture of FVIII deficient plasma and patient plasma is analyzed using APTT assay; the absence of FVIII in the patient plasma leads to lengthening of the time, b) two-phase method, where the first step leads to formation of FVIIIa and FXa and in the second phase there are thrombin and fibrin created. Two-phase method is difficult to implement in routine laboratory, and therefore it was stopped using during time. This method was substituted by the chromogenic method after development of the chromogenic substrates, where there is in the first step created FVIIIa and FXa in the presence of FIXa, phospholipids and Ca2+, and in the second step there is formed a yellow coloration by the addition of the chromogenic substrate. FVIII deficiency causes a severe bleeding disorder, hemophilia A. I performed measurements from May to September 2013 at Coagulation laboratory at the Institute of Hematology and Blood Transfusion in Prague, where I was employed. I examined in total 76 patients, hemophiliacs A, who were at least 8 days without any treatment or substitution. I assigned numbers to patient's samples to ensure anonymity of patients. I worked with the automatic coagulation analyzer STA- R Evolution? from Diagnostica Stago, which works on the principle of photometry and chronometry. I determined the factor VIII by one-phase method and two-phase method and I compared the results. The genetic part of the work was analyzed in the genetic laboratory, which is part of our department. I worked up the results obtained from both methods in 76 patients to the table and the graph. The group included 14 (18 %) moderate and 56 (74 %) of mild hemophiliacs, then 6 (8%) hemophiliacs who did not meet the criterion of a mild hemophilia A, but clinically they belonged into mild hemophiliacs. Based on the stated criteria, we found out that 15 (20 %) patients had a ratio of FVIII: C1st/FVIII: Chr or FVIII: Chr / FVIII: C1st 0.6, they differed significantly in their values set by one-stage clotting FVIII and FVIII set by the chromogenic method. A total of 11 patients with FVIII activity were higher in the single-phase method. At three patients FVIII: C1st was even on the upper limit of the normal value, while FVIII chromogenic method gave on average 16%. We managed to find a causal mutation in the FVIII in 14 patients with "the different results", we could not investigate 1 patient genetically because of the missing genetic material. Mutations in patients with lower activity of FVIII set up by the chromogenic two-phase method were concentrated predominantly into the A3 domain; mutations in patients with FVIII a lower activity set up by one-phase clotting assay were concentrated in the A2 domain. The results presented show, that diagnostic of any patient with mild or moderate hemophilia A should include determination of FVIII by both methods; FVIII: C1st and FVIII: Chr.
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Nursing care of patient with coagulopathy.
RŮŽIČKOVÁ, Ivana
Abstract This thesis deals with the nursing care for patients with coagulopathy. Its aim is to monitor the issue and create a summary of information about the disease, its distribution, symptoms, possible treatments used in the diagnosis and subsequent treatment options for patients suffering from this disease. The main point of this work is of course the part about nursing care, where the emphasis is laid on the patient and the problems associated with the disease. After that, dealing with a deficit of needs is described where the nurse plays major role, in removing the deficit. Coagulopathy is a general name for diseases that are characterized by increased bleeding disorder or a lack of clotting factors or their inhibitors. The disease can be divided into two basic groups. The first group refers to the coagulopathy, which have originated based on heredity. Conversely, the second group is called up during the life of a patient and is due to the primary disease; that is most commonly impaired liver function. Congenital coagulopathy is often found in the literature under the name of hemophilia. This disease can be further subdivided according to which coagulation factor is violated. If the disorder relates to coagulation factor VIII, we're talking about the type of hemophilia A. Hemophilia B is then characterized by a lack of factor IX. Hemophilia C, which in our geographical conditions doesn't occur, is reported as a deficiency of coagulation factor number XI. Von Willebrand's disease, which is specific deficiency of von Willebrand factor in the coagulation cascade, can also be included among hemophilic disorders. Acquired coagulopathy arises as a secondary disease. Its cause is the liver diseases, which are mostly liver cirrhosis, hepatitis, obstructive jaundice. Coagulopathy, however, can be caused by poisoning by pharmacological drugs. Most are coumarin-type drugs, among which belong Paralen, Warfarin, Macumar. The difference in the treatment of patients with haemophilia and congenital coagulopathies lies in the realization that congenital coagulopathies are the subsequent diseases resulting from complications of the underlying disease. In this work we will also learn about investigative methods that lead to a subsequent diagnosis. Emphasis is placed on medical history, physical examination and subsequent laboratory methods that are most important for us in this disease. Among the basic laboratory tests there belong a blood count and coagulation tests. After that, imaging methods are important in patients with coagulopathy, because they are considered preventive care, preventing patients suffering from this disease from the occurrence of complications. The main point of this work is nursing, in which it is important for us to know how to manage care for patients with this disease and to deprive them of their current deficits of needs. Most often are these deficits associated with increased risk of bleeding and consequent violation of the integrity of the skin or soft tissues. A frequent problem is also the risk of infection. A major problem in the treatment of such patients are disturbances of mental state, where there is mostly disturbed body image due to hematoma forming on the patient's body or frequent bleeding. Patients with coagulopathy should also be aware of the possibilities of the refilling of vitamin K in the diet. Vitamin K is an integral part of the treatment of patients with acquired coagulopathy. A major problem for patients and for us as a medical staff is, after that, deficit of knowledge about the disease and its subsequent treatment by application of intravenous coagulation factor concentrates. In the Czech Republic there are two associations for this kind of patients. We're talking about Hemojunior association, which is intended for children suffering from hemophilia. Patient with this disease should bbe taken as an individual, and thus we should approach him.
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Unemployment of people with disabilities in the European Union
Šebest, Ivan ; Palíšková, Marcela (advisor) ; Stříteský, Marek (referee)
Unemployment is one of the major economic and social problems of all the countries of the European Union. Bachelor thesis analyzes the unemployment among people with disabilities in the EU. Structurally the bachelor thesis is divided into two parts, theoretical and practical part. The first part provides a theoretical basis for the issue of unemployment. The second part contains an analysis of unemployment among people with disabilities based on a wide range of criteria as well as analysis of the effectiveness of hemophilia treatment with clotting factor concentrates. Furthermore it shows its impact on unemployment and social status of people with inherited bleeding disorder - hemophilia.
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Specifics of nursing care of patients with hemophilia
HOUSKOVÁ, Tereza
This bachelor thesis is divided into two parts. The topic is the specificities of nursing care of patients with hemophilia. The aim of theoretical part is to sum up in detail the issues connected to inherited coagulopathy, in this case hemophilia. It provides and discusses the definition, severity, causes, manifestations, diagnosis, complications, and therapy of the given disorder. Nursing care is an area not to be omitted when discussing hemophilia, especially due to the character of the disorder, which is highly specific and includes complex care. The empirical part of this bachelor thesis is based on the results of conducted qualitative research. The main goal of this research is to identify the specificities of nursing care of patients with hemophilia, including their specific needs. Further, this research sets to identify in what way are people diagnosed with hemophilia affected by this disorder. For this purpose, three targets were selected. First target is connected to the specific needs of the hemophiliacs. Second target is to map which parts of daily life of the patients are most influenced by hemophilia. Third and the last target cover the specificities connected directly to the nursing care of patients with hemophilia. The research questions were devised according to the aforementioned targets. Semi-structured interviews were used as the qualitative research method in order to fulfill the set targets. Semi-structured interviews were conducted with two peer groups, first group chosen for the purpose of this research were five nurses with experience in care of patients with hemophilia. Second group interviewed comprised of five hemophiliacs. The semi-structured interviews with the nurses aimed to identify the specificities of nursing care of patients with hemophilia, including their needs. The interviewees with hemophilia were asked mostly about their activity in daily life. The interviews were recorded and afterwards manually transcribed. Interviews were properly coded and categorized into specially devised categories. The results of the conducted research show that all the nurses identified as the most important specificity in nursing care of patients with hemophilia regular application of coagulation factor, which is usually done twice a week. However, they further note that during hospitalization, in most cases the time interval is shorter between individual applications. This is due to the planned procedures. The research concludes that due to the character of the disorder such as hemophilia, it is necessary to check on the patient more frequently in his room and monitor bleedingand last but not least the pain. All the nurses further stated that pain is one of the most important factors connected to fatigue. Therefore, heighten need for sleep can be observed in patients with hemophilia. A significant specificity in nursing care of patients with hemophilia, pointed out by the nurses, is that no intermuscular injection can be applied to such patients due to the high risk of bleeding into the muscle. Another contradiction is administration of drugs containing acetylsalicylic acid, which has a negative impact on aggregation of blood cells. All the nurses agreed that in terms of hygiene it is crucial to consider joint stiffness and their swelling, which are often accompanied by strong pain. Pain was also noted by many respondents as a factor that most often have a negative influence on their daily life activities. Contact sports or extreme sports are unsuitable for such patients, as they involve high risk of injury and consequent open or inner bleeding. Further, the respondents felt limited in terms of possible career choices, affected by their bleeding disorders. Based on this research an informational pamphlet was created. This pamphlet can be used as a source of basic information for patients with hemophilia and people close to them as well as for students of medical courses.
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