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Acquired Hypoxaemia Chronic lung disease Cyanotic congenital heart disease with rightlleft shunt Living at high altitude Chronic alveolar hypoventilation gastritis and bloating order prevacid 30mg on line. Take a detailed history with attention to smoking habits, alcohol consumption, diuretic therapy, dyspepsia, and thrombosis. Hgb >185g/L, > 165g/L or Hgb or Hct >99th percentile of reference range for age, sex, or altitude of residence or Hgb >170g/L. Polycythemia vera and essential thrombocythemia: 2013 update of diagnosis, risk-stratification, and management. In some patients near n counts are maintained without therapy as a result of d proliferative capacity due to early myelofibrosis. Subsequently an advanced phase can develop, which is due to extensive myelofibrosis and associated with progressive hepatosplenomegaly, pancytopenia, and systemic symptoms (fever and weight loss). Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. Early studies showed i risk of thrombosis in first 3 years after treatment with venesection alone thus additional cytoreductive treatment is required in patients with a higher risk of thrombosis. It has been argued that the risks and benefits of aspirin should be carefully considered in very low-risk patients. Most may be managed as for low-risk patients but cytoreductive therapy may be required in some. Poor compliance to venesection or progressive myeloproliferation (splenomegaly, constitutional symptoms, leucocytosis, and thrombocytosis) can be an indication for cytoreductive therapy. Side effects (fever, flu-like symptoms, weakness, myalgia, depression) can reduce compliance. Neither is therefore recommended for patients 75 years who should receive hydroxycarbamide. However, in patients >75 in whom compliance or regular monitoring of hydroxycarbamide dose is a problem, busulfan is likely the best choice of treatment. Splenectomy is often considered due to discomfort, recurrent infarction, or hypersplenism but often followed by massive hepatomegaly due to extramedullary haematopoiesis. Sometimes abates when excess myeloproliferation controlled and Hct reduced but may persist despite adequate control. May be precipitated by antithrombotic therapy: avoid in patients with history of haemorrhagic events, gastric ulcers, or varices. Defer surgery until Hct and platelets normalized for 2 months due to risk of thrombotic and haemorrhagic complications; if emergency surgery necessary perform venesection. Preplan conception when possible with cessation of any teratogenic agents and control of Hct. Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Hydroxyurea in the treatment of patients with essential thrombocythemia at high risk of thrombosis: a prospective randomized trial. Long-term effects of the treatment of polycytemia vera with recombinant interferon alpha. Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: pathogenetic mechanisms and prevention. Response criteria for essential thrombocythemia and polycythemia vera: result of a European LeukemiaNet consensus conference. Symptoms Non-specific and those of the underlying cause (particularly if cardiac or pulmonary) may predominate. Venesection is the treatment of choice and if possible should be continued until a target Hct is achieved. Some cases may represent extreme ends of n ranges for red cell and plasma volumes, but in most obesity, cigarette smoking, and hypertension are present singly or in combination. Guidelines for the diagnosis, investigation and management of polycythemia/erythrocytosis. Incidence True incidence unknown but probably up to 3 per 100,000 annually;1 slight excess in; median age at diagnosis 60 years; frequently occurs <40 years; very rare <20 years. The risk of life-threatening complications or of leukaemic transformation is very low. Low-dose aspirina Low-dose aspirina Age >60 years Not applicable Not applicable of childbearing age A multidisciplinary approach with close maternal and fetal monitoring is recommended. An activating splice donor mutation in the thrombopoietin gene causes hereditary thrombocythemia. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukocytosis is a risk factor for thrombosis in essential thrombocythemia: interaction with treatment, standard risk factors, and Jak2 mutation status. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. A statement from the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. Acute leukemia in polycythaemia vera: an analysis of 1638 patients enrolled in a prospective observational study. A unified definition of clinical resistance/intolerance to hydroxyurea in essential thrombocythemia: results of a consensus process by an international working group. Pregnancy and its management in the Philadelphia-negative myeloproliferative diseases. However, short-term anticoagulant or antiplatelet therapy is advised for marked thrombocytosis occurring in the immediate post-splenectomy period as an i incidence of thrombosis has been described in this situation. Incidence Rare disorder; 75 cases per million per annum; predominantly elderly patients (median 65 years); affects and equally. Cytogenetics: abnormalities in up to 50%: 13q-, 20q-, +8, +9, t(1;7), der(6), t(1;6),(q21723;p21. Megakaryocyte proliferation and atypiaa accompanied by either retuculin and/or collagen fibrosis, or In the absence of reticulin fibrosis, the megakaryocyte changes must be accompanied by i marrow cellularity, granulocytic proliferation, and often i erythropoiesis. Impressive clinical/symptomatic response though no change in disease course documented as yet. Gains on 9p are common genomic aberrations in idiopathic myelofibrosis: a comparative genomic hybridization study.
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Once the patient can eat gastritis diet prevacid 15mg, the value of oral nutritional support programmes for advanced cancer patients is unclear. In advanced head and neck cancer patients undergoing radiation, the group randomized to the nutritional support programme lost less weight but had an overall poorer outcome (Rabinovitch et al. Those with multilevel obstruction are almost never surgical candidates and should be managed with changes in oral intake and medications. Role of octreotide, scopolamine butylbromide and hydration in symptom control of patients with inoperable bowel obstruction having a nasogastric tube. Clinical-practice recommendations for the management of bowel obstruction in patients with end-stage cancer. Conclusions Bowel obstruction secondary to cancer or its treatments is encountered relatively frequently in supportive care as well as in hospice/ palliative care practice, carries a poor prognosis, and is associated with significant symptoms. In someone with a single-level obstruction and good functional status, surgery 14. It can be a passive participant in states of disease or be directly involved by disease or by its treatment. In contrast to disease progression, which can be occult or more of an abstract notion, skin manifestations are easily perceived, measured, and followed. They focus attention and cause distress that often outsize their real risk, being a constant reminder of the harboured disease. In those with cancer particularly, the most alarming aspect of skin involvement may be the threat of disfigurement. When a distressing skin symptom is addressed and treated successfully, the patient may derive a renewed sense of hope that reflects on the motivation to participate actively in therapy. Cellular adhesion is lost at the surface of the skin, where keratinocytes detach as flakes and scales, which are usually unnoticeable. The regenerative potential of the skin is harboured in colony-forming cells that reside in the hair follicles (Oshima et al. In states of damage to the skin, it is these cells that generate a new epidermal covering. This highly specialized basal lamina acts as a highly selective pathway for the migration of cells and transport of macromolecules. The basal cells are anchored to the basal lamina through an array of tonofilaments and hemidesmosomes. Epidermal integrity may be disrupted by any pathology that weakens keratinocyte adhesion or damages the basal lamina. Depositions of antibodies against proteins or collagen subtypes that build these structures can split the epidermis and lead to the formation of blisters. Excessive accumulation of fluids in the dermis can generate similar forces and damage skin integrity by distending the intercellular matrix, causing cells to detach from each other. The skin appendages are specialized differentiated subunits with unique functions. They include the hair follicle, sebaceous gland, and the eccrine and apocrine sweat glands. Skin structure and function the basic function of the skin is to serve as a barrier, separating the human body from the external environment. The skin is made up of three distinct layers: Epidermis the epidermis comprises mainly keratinocytes. Intercalated among them are the immigrant cells: Dermis the dermis is a network of collagen and elastin fibres embedded in an amorphic extracellular matrix of mucopolysaccharides, which nests below the epidermis. The fibres and extracellular matrix of the dermis are synthesized by fibroblasts-cells that are dispersed throughout the dermis. The dermis also contains the blood supply for the non-vascularized epidermis tissue. Two webs of capillary blood vessels, deep and superficial, stretch along the dermis to oxygenate and nourish both layers. Parallel lymphatic vessels remove fluids from the skin back into the intravascular compartment. Trafficking immune cells traverse the lymphatic channels to and from regional lymph nodes as part of a screening and defence process against invasion. Inflammation of the skin-dermatitis-results in oedema of the dermis, vasodilatation, and accumulation of leucocytes and lymphocytes in the dermis. Columnar keratinocytes at the base of the epidermis form the germinative layer of the skin, which is known as the basal layer. These keratinocytes undergo a process of differentiation while they move upwards towards the surface (Chu et al. A small proportion of keratinocytes is constantly dividing to replace cells that are injured and removed. A pathological state associated with inflammation results in an increased turnover of keratinocytes. Differentiating keratinocytes produce and contain keratin filaments arranged in bundles that anchor each keratinocyte 14. In states of starvation and cachexia, the subcutaneous fat is severely depleted and the skin is exposed to pressure without the protective effect of fat. Discoloration the colour of the skin is based on the reflection of chromophores, which are normal constituents of skin that may form abnormal deposits as part of disease. Mild darkening of the skin may be seen with adrenal insufficiency, with adrenocorticotropic hormone-producing tumours such as primary tumours of the pituitary gland, or with other malignant tumours metastatic to the pituitary gland. Increased melanization of the basal layer, leading to hyperpigmentation, may be a side effect of chemotherapeutic agents (Alley et al. Bleomycin, busulphan, capecitabine and hydroxyurea can cause generalized persistent hyperpigmentation (Singal et al. Drug-induced hyperpigmentation may be limited to mucosae, nails, palms, and soles. Widespread inflammation of the skin from any cause results in post-inflammatory hyperpigmentation and persistent brown tint. Skin manifestations of neoplastic disease the skin may be adversely affected by any serious medical illness, often as a secondary process related to infection, trauma, nutritional deficiencies, and other factors (see below). Disease-specific skin involvement occurs commonly in some conditions, and is best characterized in cancer. Skin of the patient with advanced cancer is unique compared to other organs of the body. While the sequela of metastatic spread to internal organs often is replacement of normal tissue and resultant organ failure, widespread replacement of skin with a neoplasm is uncommon. Yet, even a local disruption of skin integrity can cause deterioration in the quality of life, debilitation, and even mortality. Other aspects of neoplastic disorders affecting the skin include accumulation of abnormally produced metabolites, adverse effects to treatment, and paraneoplastic syndromes. Direct invasion of skin by tumour Metastases to the skin are not an infrequent occurrence in cancer patients. In a retrospective series of 4020 metastatic cancer patients, 10% had skin involvement (Lookingbill et al. The skin may be involved with the primary tumour or may be the target of systemic metastatic spread (Brenner et al. Direct invasion of the skin results from an uncontrolled local-regional disease, as in melanoma and breast cancer, where the tumour invades the superficial lymphatic vessels and infiltrates the skin. This commonly appears as discrete nodules, but can also form a diffuse pattern similar to oedema. An inflammatory plaque also may occur, which is often mistaken for erysipelas until diagnosed correctly. Any aggressive tumour that arises in an adjacent organ may directly extend to the overlying skin. The pressure of a tumour mass interferes with cutaneous blood supply and may lead to ulcerating wounds, which discharge foul smelling necrotic material and may require daily washing and the use of hydrocolloids or absorbent dressings. Incisional metastases are also a common cause of abdominal skin involvement (Lookingbill et al.
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In the porphyrias enzymatic defects lead to an insufficient production of haem and deposition of toxic porphyrin gastritis neck pain buy generic prevacid 15mg on line. Each particular enzymatic defect leads to different porphyrins, which depending on their chemical properties deposit in different locations. Pathophysiologically porphyrias are divided into hepatic or erythropoietic, based on the site of haem deposition. Signs and symptoms Symptomatically the porphyrias are divided into acute or cutaneous prophyrias. Precipitating factors for acute attacks are stress, infections, barbiturates, some antibiotics (sulfonamides), surgery, and low caloric intake. Should be given as soon as an attack starts; proven to reduce duration and intensity of attacks. Best practice guidelines on clinical management of acute attacks of porphyria and their complications. This condition is a descriptive term; a proliferation of fibrous tissue is seen in the retroperitoneum, mainly around the aorta. There is an association with autoimmune conditions, previous radiotherapy, malignancy, certain drugs (hydralazine, beta-blockers) and infections. Diagnosis Imaging can be diagnostic on its own (confluent mass surrounding the aorta). Clonal proliferation of Langerhans cells (myeloid lineage-derived dendritic cells). Rare inherited disorder whereby the osteoclast function is impaired (number of osteoclasts variable). This leads to impaired bone resorption with ongoing bone formation leading to hardened bones. Despite this, patients with osteopetrosis have an i risk of fractures and other bone problems. The main difficulty with Internet resources is that they change so frequently and they are constantly being updated and outdated. Plate 1 Radiograph of pelvis in a patient with multiple myeloma showing abnormal low density bone texture in the left superior pubic ramus and ischium (E see p. From this pair of diverticula from the sides of the forebrain and the mesodermal and ectodermal structures in contact with it, the two eyes develop. The inner layer of the cup forms the main structure of the retina, the nerve fibres from which eventually grow backwards towards the brain. The hyaloid artery enters the optic cup through the embryonic fissure and grows forward to meet the lens, bringing temporary nourishment to the developing structures before it eventually atrophies and disappears; as it does so, its place is taken by a clear jelly (the vitreous) largely secreted by the surrounding neural ectoderm. While these ectodermal events are taking place, the mesoderm surrounding the optic cup differentiates to form the coats of the eye and the orbital structures; that between the lens and the surface ectoderm becomes hollowed to form the anterior chamber, lined by mesodermal condensations which form the anterior layers of the iris, the angle of the anterior chamber and the main structures of the cornea; while the surface ectoderm remains as the corneal and conjunctival epithelium. The ectoderm is of two types: (i) the neural ectoderm derived from the neural tube and (ii) the surface ectoderm on the side of the head Table 1. The anterior part of the sclera is covered by a mucous membrane, the conjunctiva, which is reflected from its surface onto the lids. Inside the eye, posteriorly the sclera is lined by the uveal tract and retina and the globe are broadly divided into the anterior segment and posterior segment by the lens. The stromal collagen fibrils are of regular diameter, arranged as a lattice with an interfibrillar spacing of less than a wavelength of light so that tangential rows of fibres act as a diffraction grating resulting in destructive interference of scattered rays. The primary mechanism controlling stromal hydration is a function of the corneal endothelium which actively pumps out the electrolytes and water flows out passively. Endothelial cells become less in number with age and the residual individual cells may enlarge to compensate. It is dependent for its nourishment upon diffusion of tissue fluid from the vessels at its periphery and the aqueous humour. The cornea is very richly supplied with unmyelinated nerve fibres derived from the trigeminal nerve. In each case the solid black is the neural ectoderm, the hatched layer is the surface ectoderm and its derivatives, the dotted area is the mesoderm: a, cavity of the forebrain; b, cavity of the optic vesicle; c, cavity of the optic cup (or secondary optic vesicle) formed by invagination. The outer surface of the sclera is covered by the conjunctiva, beneath which is a layer of loose connective tissue called episclera and the innermost layer of the sclera consists of elastic fibres called the lamina fusca. Lining the inner aspect of the sclera are two structures-the highly vascular uveal tract concerned chiefly with the nutrition of the eye, and within this a nervous layer, the true visual nerve endings concerned with the reception and transformation of light stimuli, called the retina. In the posterior segment, the posterior chamber lies behind the lens, has a cavity filled with a transparent gel-like substance called the vitreous humour and is bounded by the ciliary body and retina. Anterior Chamber the anterior chamber is a space filled with fluid, the aqueous humour; it is bounded in front by the cornea, behind by the iris and the part of the anterior surface of the lens which is exposed in the pupil. The extracellular spaces contain both a coarse framework (collagen and elastic components) and a fine framework (mucopolysaccharides) of extracellular materials, which form the probable site of greatest resistance to the flow of aqueous. It has three main parts the outer capsule lined by the epithelium and the lens fibres and is developed from an invagination of the surface ectoderm of the fetus, so that what was originally the surface of the epithelium comes to lie in the centre of the lens, the peripheral cells corresponding to the basal cells of the epidermis. Just as the epidermis grows by the proliferation of the basal cells, the old superficial cells being cast off, so the lens grows by the proliferation of the peripheral cells. The old cells, however, cannot be cast off, but undergo changes (sclerosis) analogous to that of the stratum granulosum of the epidermis, and become massed together in the centre or nucleus. The lens fibres have a complicated architectural form, being arranged in zones in which the fibres growing from opposite directions meet in sutures. In Neural crest* Corneal stroma, keratocytes and endothelium Sclera Trabecular meshwork endothelium Iris stroma Ciliary muscles Choroidal stroma Part of the vitreous Uveal and conjunctival melanocytes Meningeal sheaths of the optic nerve Ciliary ganglion Schwann cells of the nerve sheaths Orbital bones Orbital connective tissue Connective tissue sheath and muscular layer of the ocular and orbital blood vessels *During the folding of the neural tube, a ridge of cells comprising the neural crest develops from the tips of the converging edges and migrates to the dorsolateral aspect of the tube. Neural crest cells from this region subsequently migrate and give rise to various structures within the eye and the orbit. At the periphery of the angle between the canal of Schlemm and the recess of the anterior chamber there lies a loosely constructed meshwork of tissues, the trabecular meshwork. The mass of epithelium which constitutes the lens is surrounded by a hyaline membrane, the lens capsule, which is thicker over the anterior than over the posterior surface and is thinnest at the posterior pole; the thickest basement membrane in the body it is a cuticular deposit secreted by the epithelial cells having on the outside a thin membrane, the zonular lamella. The lens in fetal life is almost spherical; it gradually becomes flattened so as to assume a biconvex shape. This is not a complete membrane, but consists of bundles of strands which pass from the surface of the ciliary body to the capsule where they join with the zonular lamella. The strands pass in various directions so that the bundles often cross one another. The anterior layer consists of flattened cells and the posterior of cuboidal cells. From the epithelial cells of the former, two unstriped muscles are developed which control the movements of the pupil, the sphincter pupillae, a circular bundle running round the pupillary margin, and the dilator pupillae, arranged radially near the root of the iris. The anterior surface of the iris is covered with a single layer of endothelium, except at some minute depressions or crypts which are found mainly at the ciliary border; it usually atrophies in adult life. The iris is richly supplied by sensory nerve fibres derived from the trigeminal nerve. The sphincter pupillae is supplied by parasympathetic autonomous secretomotor nerve fibres derived from the oculomotor nerve, while the motor fibres of the dilator muscle are derived from the cervical sympathetic chain. Ciliary Body the ciliary body in anteroposterior section is shaped roughly like an isosceles triangle, with the base forwards. The chief mass of the ciliary body is composed of unstriped muscle fibres, the ciliary muscle. The greater part of the muscle is composed of meridional fibres running anteroposteriorly on the inner aspect of the sclera to find a diffuse insertion into the suprachoroid. Most of the remaining fibres run obliquely in interdigitating V-shaped bundles so as to give the impression of running in a circle round the ciliary body, concentrically with the base of the iris. The third portion of the muscle is composed of a few tenuous iridic fibres arising most internally from the common origin and finding insertion in the root of the iris just anterior to the pigmentary epithelium in close relation to the dilator muscle. The inner surface of the ciliary body is divided into two regions; the anterior part is corrugated with a number of folds running in an anteroposterior direction while the posterior part is smooth. The anterior part is therefore, called the pars plicata; the posterior, the pars plana. About 70 plications are visible around the circumference macroscopically, but if microscopic sections are examined, many smaller folds, the ciliary processes, will be seen between them. These contain no part of the ciliary muscle, but consist essentially of tufts of blood vessels, not unlike the glomeruli of the kidney.
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Several attempts have been made to develop observation-based instruments to measure pain in the cognitively impaired (Zwakhalen et al gastritis hemorrhage cheap prevacid 15mg overnight delivery. In summary one may say that the patient-focused assessment recommended is very similar to the strategy developed earlier in life, focusing on symptom control and how to relieve the patient burden. However, during end-of-life care, spiritual and existential Cognitive impairment Cognitive impairment as part of dementia, amnestic disorder or delirium is prevalent in palliative care (Robinson, 1999; Casarett and Inouye, 2001). Interviews conducted by health-care providers by means of specific interview guides and observation of behaviour are the appropriate methods for the detection of cognitive impairment (Hjermstad et al. The subjective experience of cognitive impairment is weakly correlated to neuropsychiatric disturbances but much stronger to psychological distress (Cull et al. Physicians or other health professionals can use these measures without specific preparation, although it is wise to compare first time performances with more experienced personnel as part of the introduction routines into a research project or clinical practice. However, more research is needed before such an abbreviated version can be recommended for general use. Multidimensionality: generic Content of the measure: generic No common metric: generic Heterogenic intervention: palliative care specific Multiple problems (symptoms): palliative care specific Progressive disease: palliative care specific. A variety of instruments have been used in the published studies to examine different aspects and models of care (Rinck et al. New, shorter, and more comprehensive instruments are therefore needed, which ideally could be completed both by the patient and proxy raters in sequence. Interpretation of data What is the clinical relevance of a summary score on a single item when comparing groups of patients or individuals This is one basic question to ask both in daily clinical practice, in interpreting clinical research, and in sample size calculation in the planning process of a clinical trial. The clinical significance is related to the importance of the symptoms or the signs. When discussing the clinical significance of a pain score, two important questions need to be answered. What is a relevant cut-off point in order to classify the score of those in need of intervention Change in any clinical variable, independent of the nature of the variable, that is, physiological (blood pressure), psychological (anxiety), and performance (physical function) needs to be interpreted in a clinical framework. It is not a methodological or statistical question whether a change of 20 on a scale from 0 to 100 is of clinical significance. Similar discussions arise, for example, in interpreting the clinical significance of blood pressure medication, interpreting the reduction of tumour size caused by chemotherapy, and the importance of change in median survival in patients with non-small cell lung cancer admitted to a randomized chemotherapy trial. The complexity of human biology may cause an increase in intensity in one symptom when another symptom is relieved. Furthermore, most patients have a mosaic of symptoms, which often needs broad interventions, and consequently one specific outcome may be difficult to identify. In palliative care these measures have been criticized for not covering the existential and spiritual issues sufficiently. A series of measures for use in health care in general (generic instruments) and for use in specific diagnostic groups, such as cancer (cancer specific) and palliative care (palliative care specific), have been developed. The latter is developed for assessing specific symptoms or signs, such as pain, fatigue, depression, anxiety, physical function, spirituality, etc. The plethora of instruments is a challenge for the users of the instruments, the readers of scientific reports, and for the performance of meta-analyses. Most instruments have been developed for use in research and may not be suited for use in daily clinical practice. Many instruments for measurement of the same constructs are available and validation has often been insufficient both in relation to the palliative population and on a more general level. Still, the content of a questionnaire always needs to be cautionary investigated to assure that it fits the purpose for the assessment in clinical research, in quality assurance, or in clinical practice. Quality of life in palliative cancer care: results from a cluster randomized trial. Methodologic issues in effectiveness research on palliative cancer care: a systematic review. A broader definition was offered in 2002 by the American Academy for Health Services Research and Health Policy: Health services research is the multidisciplinary field of scientific investigation that studies how social factors, financing systems, organisational structures and processes, health technologies, and personal behaviors affect access to health care, the quality and cost of health care, and ultimately our health and well-being. Its research domains are individuals, families, organizations, institutions, communities, and populations. It involves the systematic search for knowledge that will lead to improvements in the delivery of health care (Crombie and Davies, 1996). Health services research in palliative care and end-of-life care During the last century, achievements in public health and improved living conditions in developed countries have led to an increase in life expectancy and a significant rise in the proportion of the elderly in the population (Seale, 2000). Death usually occurs from chronic and degenerative diseases, and typically is preceded by a prolonged terminal illness (United Nations Population Division, 2002; Lunney et al. High illness burden prior to death may be ameliorated by interventions identified as palliative care or delivered by services that provide specialist palliative care. Palliative care could potentially benefit between 50% and 89% of the 50 million people worldwide who die annually (McNamara et al. Ensuring equal and adequate access to quality palliative care presents a major public health challenge (Davies and Higginson, 2004). Public health policy is ideally informed by an up-to-date and unbiased evidence base, which can be provided only by health services research. Investigating and interpreting the use of palliative care, including its costs, quality, accessibility, delivery, organization, and outcomes, is important in determining the most effective public health strategies. During the past two decades, clinical research relevant to the domains of palliative care has increased rapidly. At the same time, rapid developments in health services research have generated complementary knowledge about the organizational aspects of palliative care and the experiences of patients and professionals involved in the services. Health services studies have evaluated the caregiving process and the feasibility and effectiveness of one or more particular service or intervention (Steinwachs and Hughes, 2008). Researchers in palliative and end-of-life care have examined topics such as need, access, and quality, and the feasibility, effectiveness and cost of palliative and end-of-life care services and interventions. The principle of equitable distribution of health care and the commitment to ensuring equitable access is widely acknowledged (European Union, 2000; Van Doorslaer et al. Equitable access is often defined as horizontal equity, which means that people with equal needs are treated equally (Wagstaff et al. Health services research has attempted to identify situations in which patients with specific palliative care needs are denied care on the basis of ethnicity, gender, age, socioeconomic status, educational level, or geographical isolation, or because of inadequate service levels (Scott and Campbell, 2000). These type of data can be used to redress disparities in care through system-level changes. Evaluation of palliative and end-of-life care services and interventions raises the question of whether the service or 19. Contamination, that is, the risk that the demonstrated effect of the intervention will be weakened because control group patients unintentionally receive some or all of the intervention, is a significant concern when the intervention is complex (Elley et al. For example, if the intervention is the introduction of a palliative care service in a hospital or in a specific ward, all patients in the hospital or ward will potentially have contact with the intervention. Newly diagnosed patients were randomly assigned to receive either early palliative care integrated into standard oncological care or standard oncological care alone. The study found that those receiving early palliative care had a longer median survival, better quality of life, and fewer depressive symptoms than those receiving standard care. The study concluded that introducing early palliative care for this patient population led to important improvements in quality of life and mood. Although the development of palliative care services and interventions should be based on evidence that they will increase the likelihood of desired outcomes, clear information on feasibility and effectiveness is often lacking (Kaasa et al.
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A short gastritis diet purchase prevacid uk, curved needle with its bevel towards the globe is passed through a conjunctival incision and medication injected close to the inflamed tissue. These injections are used in the treatment of intermediate and posterior inflammations. Other antimicrobial agents are effective against spirochaetes, rickettsiae, fungi and viruses. In general, effectivity against gram-positive organisms is seen with penicillin G, erythromycin, oxacillin and vancomycin, while neomycin, polymyxin B, azlocillin and streptomycin are largely effective against gram-negative organisms. If a fluoroquinolone or aminoglycoside drug does not show clinical efficacy, the drug should be switched or a drug from another group added. Carbenicillin sodium, ticarcillin and azlocillin are given parenterally and act against Pseudomonas aeruginosa. Penicillins show a synergistic action with antibiotics of the aminoglycoside group. In deep-seated inflammations of the orbit or lids, they are administered parenterally. In superficial inflammations of the conjunctiva and cornea drops or ointment are administered locally. Cloxacillin and flucloxacillin: these penicillins are not affected by staphylococcal penicillinase and are therefore used for treating staphylococcal infections which are resistant to other penicillins. Flucloxacillin has the same activity against penicillin-resistant staphylococci as cloxacillin, but levels of flucloxacillin in the blood after oral administration are nearly twice as high as after an equivalent dose of cloxacillin. This is because flucloxacillin is better absorbed from the gastrointestinal tract and is also excreted more slowly from the body. Serum levels following intramuscular or intravenous injections of flucloxacillin are also higher than those of cloxacillin. Carbenicillin is resistant to the penicillinase produced by some strains of Proteus, Pseudomonas and coliform organisms. Ampicillin is a broad-spectrum penicillinase sensitive penicillin which is acid-resistant and is usually administered orally. It is not as effective as benzyl penicillin and should be used for organisms which are resistant to benzyl penicillin but do not produce penicillinase. Amoxycillin: this penicillin has an antibacterial activity identical to that of ampicillin but its main advantage is that it is well absorbed after oral administration, producing serum levels about twice as high as those after an equivalent dose of oral ampicillin. Food in the stomach has little effect on amoxycillin absorption from the small bowel. Penicillins All penicillins have a bactericidal effect, but have a short half-life. Differences in antibacterial activity, absorption and resistance to penicillinase depend on alteration of the side-chains attached to the amino group. They are excreted mainly via the kidney and appear in the urine in active forms; a small fraction is excreted via the biliary tract. Most of them have a rather narrow antibacterial spectrum, being chiefly confined to cocci and gram-positive organisms. When given systemically some have to be injected intramuscularly because they are destroyed by the acidic gastric juice, others can be given by mouth. As patients are liable to develop hypersensitivity to penicillin, it is wise to enquire about this before starting a course of treatment. Immediate reactions such as urticaria and anaphylactic shock are probably associated with hypersensitivity to the 6-aminopenicillanic acid nucleus. Delayed reactions may be due to hypersensitivity to protein residues occasionally present in penicillin preparations derived from the fermentation process. Penicillins effective against coccal infections and gram-positive bacilli: Benzyl penicillin is not acidstable and can therefore only be given parenterally. Penicillinase-resistant penicillins consist of cloxacillin sodium and flucloxacillin sodium. Their advantage lies in their activity against penicillin-resistant staphylococci. Broad-spectrum penicillins such as ampicillin and amoxycillin are absorbed well orally and can also be administered parenterally. They are effective against Cephalosporins these drugs have a structure and mode of action similar to penicillin and are bactericidal. When cephalosporins are used extensively, strains of staphylococci resistant to cloxacillin as well as the cephalosporins emerge. The first generation of cephalosporins, such as cephazolin and cephalexin, were highly effective against gram-positive cocci, but moderately so for some gramnegative enterobacilli. The second generation of cephalosporins, such as cefuroxime and cefaclor, had a wider gram-negative spectrum. It is more stable than cephazolin to the beta-lactamase produced by Staphylococcus aureus and to the betalactamases of some gram-negative bacilli. Ceftazidime acts against many gram-positive and gramnegative organisms, especially Pseudomonas. Aminoglycosides this group includes streptomycin, soframycin, neomycin, gentamicin, sisomycin, netilmycin, tobramycin and amikacin, which are all bactericidal. Though they have a broad spectrum of activity against many gram-negative organisms and grampositive staphylococci, they provoke allergy and bacterial resistance. All these agents are toxic to the eighth nerve and the kidney, and interfere with neuromuscular conduction, causing serious paralysis in patients with myasthenia gravis or those receiving neuromuscular blocking agents. Streptomycin: this bactericidal drug is used in the treatment of Mycobacterium tuberculosis infections but the organism rapidly develops resistance. Thus, the drug should be used only after confirmation of in vitro susceptibility and in combination with a second drug to prevent resistance developing during treatment. Soframycin is highly effective against gram-positive cocci and gram-negative bacilli including Ps. It is not available for systemic use and can only be administered topically as drops or ointments. Gentamicin: this drug may be used parenterally for the treatment of serious infections by gram-positive and gramnegative organisms. As the margin between toxicity and efficacy is narrow, it should be reserved for infections resistant to other antibiotics. Because it is nephrotoxic and ototoxic and secreted through the kidneys, the dose must be decreased in patients with renal disease. Gentamicin is effective against an exceptionally wide range of bacteria which includes penicillin-resistant strains of staphylococci and Ps. Bactericidal concentrations are found in the aqueous following topical administration of 0. As it may occasionally cause conjunctival necrosis, the sub-Tenon route is recommended. Neomycin: this has similar properties to gentamicin but is too toxic for parenteral use. It can be used topically, especially in combination with another antibiotic as eye drops or ointment, but often causes contact allergy. Sisomycin and netilmycin are similar to gentamicin, but are more effective against gram-negative organisms. Tobramycin is more effective than gentamicin against Pseudomonas, but less for other gram-negative bacteria such as the Enterobacteriaceae. Fortified drops enhance bioavailability and it can also be given sub-conjunctivally or intravitreally. It is the drug of choice in endophthalmitis together with amikacin or ceftazidime. Fluoroquinolones these bactericidal drugs are derivatives of nalidixic acid and have a broad spectrum of activity. Ciprofloxacin, norfloxacin, ofloxacin,lomefloxacin, gatifloxacin, levofloxacin and moxifloxacin are used topically and have prolonged bactericidal concentrations in the tear film.
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Palliative therapeutics generally should only be implemented once the underlying causative mechanisms have been established gastritis diet purchase line prevacid, since therapies directed at the primary cause may ultimately have a more effective outcome for symptom management. Measurement refers to the application of a metric to a specific dimension of symptom experience. The measurement of symptoms in children often utilizes formal scales that assess symptom intensity or frequency. Case study: volunteering and practical support An 8-year-old girl with neurological disease was receiving palliative care. A 22-year-old female volunteer from an Australian/Asian background was placed with the family, initially to play with the unwell daughter and offer light practical help. As placement progressed, routines formed and as the home tasks were completed the mother, volunteer, and daughter started taking walks in their local area and visiting cafes. This is not necessarily true as quality of life is a complex, multifactorial, and dynamic process. Symptom management in children with advanced illness the adequate, proficient, and timely management of symptoms in the dying child is of critical importance. Not only is it important from a humanitarian viewpoint, but also it is apparent that the memory of unrelieved symptoms in dying children may be retained by parents many years after their child has died (Wolfe et al. It will be impossible for children and their families to negotiate the domains of psychological and spiritual care if physical symptomatology has not been treated adequately. As few controlled studies of symptom management have been performed in childhood, many of the therapies used in children have been devised utilizing best practice for adults. The reader is referred to the adult section of this volume for the principles of management of unusual symptoms. An emphasis has been given in this chapter to the palliative care emergencies of childhood. Symptom measurement and symptom management studies in children with life-threatening illness the progress in symptom control for adults receiving palliative care does not have a parallel experience in children. This is due, in part, to the paucity of symptom control research in children and explains the reliance of best practice in paediatric palliative care on the best evidence in adult palliative care. In addition, most of the validated symptom assessment tools in paediatrics have focused on three common symptoms, pain, nausea (Zeltzer et al. In contrast to instruments measuring nausea and vomiting, instruments measuring pain in children largely have been validated predominantly in the noncancer setting. Systematic symptom assessment may be useful in assessing symptom burden as part of decision-making towards palliative care and in future epidemiological studies of symptoms in dying children. One major difficulty in performing symptom management studies in dying children pertains to the heterogeneous nature of symptoms in this population. Children with cancer, for example, tend to receive therapies directed at control of their tumours until very late in the course of their illnesses and are frequently very ill and highly symptomatic. These epidemiological and treatment variables make it less likely that a subpopulation of children receiving palliative care exists who have a stable, chronic pattern of symptoms amenable to evaluation in a trial. The palliative care emergencies of childhood Seizure control Seizures in paediatric palliative care patients may be either recent in onset or part of a long-standing, underlying seizure disorder. In the former situation, the onset will usually be frightening to patients and families and may be due to many possible causes. Worsening seizure control in a patient with an underlying seizure disorder may indicate either disease progression or factors related to anticonvulsant dose, class, or administration which should be reviewed. Buccal midazolam has been shown to be at least as effective as rectal diazepam in the acute treatment of seizures (Scott et al. Administration via the mouth is more socially acceptable and convenient and may become the preferred treatment for long seizures that occur outside hospital. Traditionally, refractory status epilepticus is treated with barbiturate-induced coma or general anaesthetics, both of which require invasive cardiorespiratory and haemodynamic monitoring and are associated with significant complications. Midazolam has been effective in terminating seizures refractory to diazepam, lorazepam, phenytoin, and phenobarbitone in paediatric patients (Scott et al. Validity was evaluated by comparison with the medical record, parental report, and concurrent assessment on visual analogue scales for selected symptoms. Back pain, more often than abnormal neurologic signs or symptoms, is the usual initial presenting sign of spinal cord compression in children (Lewis et al. As terminal delirium cannot be predicted, a therapeutic plan for its management should be considered in every dying child. The usual therapies consist of haloperidol for delirium per se with consideration of adding a benzodiazepine if there is agitation as well. Bleeding Although the fear of external bleeding is paramount in the minds of families and caregivers of children dying of either haematological malignancy or liver failure, massive external bleeding as a mode of death in childhood is uncommon. While some children with malignancy receive blood products indefinitely, this is not always feasible or appropriate. Many families negotiate the use of blood products only if minor bleeding or anaemia becomes problematic. The palliation of other symptoms in childhood advanced illness Constipation the aetiology of constipation is often multifactorial and may include reduced physical activity, mechanical obstruction, metabolic derangement, poor diet and low fluid intake, bowel atony due to opioids, and so on. Although unusual, bowel obstruction and faecal impaction must be excluded and treated urgently in any child presenting with constipation. Generally, dietary changes are recommended in the first instance (increased vegetables and fruity, bulk, prune juice, etc. In addition, attention should be given to hydration, mobility, and other activities of daily living. Whilst there are emerging adult data to suggest oral naloxone (Culpepper-Morgan et al. The osmotic laxative, Movicol-Half is being used increasingly to treat constipation in children. Terminal dyspnoea Dyspnoea is an uncomfortable awareness of breathing (Bruera et al. In the terminal phase it is often highly distressing to patients and for families to watch. These include pulmonary metastases, intrinsic lung disease or infection, cardiac failure, acidosis, muscle weakness, and so on. Non-invasive ventilation may be a viable choice for symptom management of dyspnoea related to muscle weakness, for example, and bronchospasm could be reversed easily with bronchodilators. Most of the data on the management of terminal dyspnoea are from studies of adults with terminal malignancy. There are no data on the appropriate management of dyspnoea related to muscle weakness or complications of cystic fibrosis, for example. The subjective sensation of dyspnoea is improved in patients receiving supplemental oxygen (Bruera et al. As anxiety is often a component of terminal dyspnoea, judicious prescription of a benzodiazepine may be warranted. Fatigue Fatigue is a common symptom of children with cancer (Hockenberry-Eaton et al. The aetiology of fatigue in children dying of cancer may be due to a combination of factors including anaemia, poor nutrition, insomnia, metabolic derangement, the increased effort of breathing in patients with dyspnoea, side effects of medication, and psychological factors. In the assessment of fatigue in a child, and the matrix of its potential causes, it is important to establish if this symptom is distressing to the child and/or their family. There are adult and limited paediatric data on the use of stimulant medication for the treatment of opioid-induced somnolence (Bruera et al. In children, it has become more common practice to switch opioids, for somnolence as a dose-limiting side effect of opioid therapy (Drake et al. Secretions the management of noisy secretions in an unconscious patient is aimed at reducing the distress of the family, other patients, and staff. The sound of noisy secretions can be haunting to all concerned and should be given some priority by the attending clinician. Accepted management strategies include explanation to relatives, positioning, suction, and anticholinergics.
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Building up collections of case reports points to the need for cohort and longitudinal studies as observational evidence is mounted gastritis diet 4 rewards generic 15mg prevacid with visa. The corresponding narrative inquiry of the qualitative approach brings an equally worthwhile persepective. Ethnographic case studies Longitudinal ethnographic case studies draw on many of the techniques of ethnography to develop an in-depth and detailed case account to explore issues for a cultural or ethnic group. An innovative study exploring the experiences of men with prostate cancer used the technique of photo-novella where the men were asked to provide photos to express their experiences along with the interview texts. Some men chose to photograph themselves, others contributed photos of places or symbols that helped them make meaning from their experiences. These include the analysis of public domain texts such as published stories, media texts, and diaries or stories from the Internet. Interviews and supportive therapy interventions conducted by telephone (Sandgren et al. The place of controlled trials has been much debated in palliative medicine, yet they are vital when outcome is examined as the end point. Methodological difficulties experienced in controlled trials include patient refusers and withdrawals, undeclared confounding treatments, dose variations and fidelity of the treatment applied, defaults in interim assessments, and deaths prior to outcome measurements. Despite such inherent challenges, the quality of evidence achieved necessitates greater utilization of these designs in future research in palliative medicine. Mixed methods Increasingly, psychosocial research is being designed utilizing a combined methods approach. Mixed methods can be conducted sequentially where one method leads to another to further explore a construct or phenomena. Triangulation of different data sets can confirm and explain findings more comprehensively. Similarly a study can be designed so that two or more methods complement and inform each other. Bimonthly assessment occurred over 2 years, during which two-thirds became bereaved. Further analysis of 106 narratives revealed that meaning-centred coping increased self-worth, sense of resilience, wisdom, and a perspective that no longer feared death. The importance of meaning-based coping emerged to expand the earlier theory of coping (problem- or emotion-focused processes that regulate distress). A problem with many mixed method approaches is that one or more methods may not be developed with sufficient rigor to do justice to the method or topic. Development of expertise is equally important for both qualitative and quantitative research and collaboration between such researchers generates a powerful armamentarium. Quantitative studies mostly emphasize the content through measurement of specific dimensions, which can be compared to controls over time, but usually at specific points. Qualitative studies add insight into form, with recognition of the pattern of change in outcome over time. Then, repeated measures analysis, with attention to the slope and variability of change 19. Computer-assisted qualitative data analysis software Computers have become an integral part of the repertoire of tools for qualitative analysis (Fielding, 2001). There are a number of packages available from the relative simple text retrievers that are useful to sort and manage data into categories of information, to the highly sophisticated new-generation multimedia capacities of interpretive theory building and mapping packages. Some products are free and may be all that is needed for small, straightforward projects. Complex project usually need the facilities of commercial software, preferably with user assistance provided as part of the package. It is important to understand what capabilities are needed for the research process before deciding on a software package (see Table 19. The recruitment of patients, attainment of an adequate sample size (Jordhoy et al. Employment of trained data managers, adoption of several recruitment methods, and use of well-thought out inclusion criteria does assist sampling problems (Jordhoy et al. Moreover, ethical debates exist regarding the impact of research on patients at the end of their lives (de Raeve, 1994), the withholding of treatment in controlled trials (Corner, 1996), and the informed consent process when delirium is present (Karim, 2000). Extensive interviews and use of batteries of questionnaires can be stressful and intrusive at a vulnerable and potentially poignant time in the life cycle (Atkinson and Silverman, 1997). Despite the challenges involved in such psychosocial research, its value is beyond question and critical to our endeavours to improve the care of the dying. Outcomes in psychosocial research and future directions the clinical significance or utility of each study merits careful reflection so that standards of care are steadily improved. Quality assurance programmes have highlighted the importance of the feedback loop, which leads to altered practice, innovation, and change (Lawton, 1971). For palliative medicine to grow as a discipline, its research activity must be scholarly and generate the needed evidence that informs purposeful clinical activity. It needs to grow through well thought out national and international collaborations, utilizing integrated methodologies of the highest standards. For too long, clinicians have defensively avoided good science through flawed claims about the unsuitability of palliative patients for research. Fortunately, the specialty is maturing and recognizes the imperative for its future of excellence in research studies. Interpersonal psychotherapy is one manualized and standardized intervention waiting to be applied in palliative care-its emphasis on grief, transitions, roles, and relationships makes it particularly suitable with this population. Observational studies using mixed methodologies are needed to explore demoralization, dignity, shame, and unworthy dying, incorporating the experience of patients, families and carers, doctors and nurses, and all related support staff to build a complete gestalt of the many intersecting influences on outcome. Over the next decade, such a body of work should guide future intervention studies aimed at further improving the standard of psychosocial care. Communication studies have developed in oncology over the past decade but remain a vital research domain for palliative medicine. Promotion of adaptive coping and maintenance of hope alongside acceptance of the reality of impending death constitute a fundamental feature of the effective communication needed in palliative medicine. More systematic research into decision-making and informed consent in end-of-life care is also needed that provides patientand family-centred outcomes in terms of quality of care and satisfaction with the process, whilst outcomes that evaluate staff compliance with advance care planning and other decisions are required to provide empirical evidence to inform the otherwise theoretical ethical debate that abounds globally. Finally, an urgent requirement in many countries of the world is the training of dedicated researchers who can then address many of these problems. Without solid education and experience, the pitfalls appear formidable; skill development in research methodology is crucial to respond to the many challenges emerging in this young discipline. Palliative care program effectiveness research: developing rigor in sampling design, conduct, and reporting. The McGill Quality of Life Questionnaire: a measure of quality of life appropriate for people with advanced disease. The validity of the family relationships index as a screening tool for psychological risk in families of cancer patients. Despite the valuable knowledge that has been produced by this research, and the promise of future important advances, its progress has been impeded by a persistent uncertainty about the ethics of these studies (Casarett et al. For instance, there have been concerns raised about whether patients near the end of life should ever be asked to participate in research (de Raeve, 1994; Annas, 1998) although others have objected to this extreme position (Mount et al. Nevertheless, the combination of ethical and practical issues can create substantial barriers to palliative care research (Aktas and Walsh, 2011). This chapter discusses five ethical aspects of palliative care research that investigators and clinicians should consider in designing and conducting palliative care research. Although none of these aspects is unique to palliative care research, palliative care investigators can use these overarching principles to enhance the ethics of palliative care research. Indeed, it is unethical to expose human subjects to risks in studies that peer reviewers agree cannot adequately answer a research question (Rutstein, 1970). In fact, one reason that subjects participate in clinical research is to produce knowledge that will benefit others (Advisory Committee on Human Radiation Experiments, 1995; Casarett et al. Because subjects are willing to accept risks and burdens of research at least in part in order to benefit others, investigators have an ethical responsibility to maximize the probability that a study will be able to do so. Maximizing validity and value in palliative care research There are several ways in which investigators can enhance the validity and value of palliative care research. Problems of underpowered studies, and particularly clinical trials, are both widespread and well described (Meinert, 1986). But they are particularly relevant to palliative care research, in which random variation can be quite large (Moore et al.
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Usually the result of serious underlying disease but may itself become life-threatening (through haemorrhage or thrombosis) gastritis natural cures prevacid 30mg fast delivery. Immunological (complement activation, release of tissue factor): anaphylaxis, acute haemolytic transfusion reaction, heparin-associated thrombocytopenia, renal allograft rejection, acute vasculitis, drug reactions (quinine). If strongly indicated and bleeding minimal or stopped the infusion may be restarted at 50% the initial dose when the thrombin time has returned to the lower end of the therapeutic range (1. Thrombin activation causes vascular thrombosis and microthrombi cause microvascular occlusion. Doses >2 mg cause unpredictable and prolonged resistance to oral anticoagulants and should be avoided in most circumstances where prolonged warfarin therapy is necessary. Particular care must be taken in patients with prosthetic cardiac valves who may require heparin therapy for several weeks to achieve adequate anticoagulation if a large dose of vitamin K has been administered. All medical, nursing, laboratory, and support staff should know where to find the major blood loss protocol in relevant areas and be familiar with the contents, supported by training and regular drills. There is much focus on practice recommendations for transfusion support of bleeding in trauma patients but specific attention is also needed on the management of patients with bleeding in other clinical settings such as gastrointestinal, obstetrical, or surgical haemorrhage. Good communication is essential between all teams involved in the management of patients. There should be designated Team Leader co-ordinating management who should also nominate a specific member of the team to communicate with the laboratory staff throughout the incident. Hospitals must have a strategy to ensure that red cells and components are readily available in life-threatening bleeding. The patient must have a correctly labelled blood sample for pre transfusion testing before Group O emergency blood is administered. Group-specific blood should be made as soon as possible to conserve Group O RhD negative stocks. Adult trauma patients with or at risk of major haemorrhage, in whom antifibrinolytics are not contraindicated, should be given tranexamic acid as soon as possible after injury, in a dose of 1g over 10min followed by a maintenance infusion of 1g over 8h. The use of tranexamic acid should be considered in non-traumatic major bleeding where there is no contraindication. Onset of paraplegia may be preceded for days or weeks by paraesthesia but in some patients the onset of paraplegia may follow initial symptoms by only a few hours. Where this is not possible, early radiotherapy may provide symptomatic improvement. However, if treatment is delayed until paraparesis has developed, this often proves to be irreversible despite surgery and/or radiotherapy. More common with monocytic leukaemias and the microgranular variant of acute promyelocytic leukaemia. Abdominal pain severe enough to mimic an acute abdomen is sometimes seen due to mesenteric ischaemia. Treatment is a haematological emergency-seek expert help immediately 1 Unless antecubital venous access is excellent, insert a large-bore central apheresis catheter (may need blood product support). Most respond again to further plasma exchange but leaves 15% who become chronic relapsers. Transfusion and splenectomy may be lifesaving in children with splenic sequestration. Part of the clinical decisionmaking process takes into account QoL in judging the most appropriate treatment. QoL is also evaluated as an outcome in clinical trials parallel to conventional measures such as survival. Patient QoL as assessed by the treating physician has been shown to be unreliable in an oncological setting. A number of qualities which go to make up QoL are capable of assessment; these include: ability to carry on normal physical activities, ability to work, to engage in normal social activities, a sense of general well-being, and a perception of health. Several validated instruments now exist to measure QoL; these mainly involve questionnaires completed by the patient. Where survivals are minimally affected it is essential to focus on treatments which will offer the best QoL. Acknowledgement of the need to manage pain effectively is an essential part of successful patient care and management in clinical haematology. It is most important to listen to the patient and give them the chance to talk about their pain(s). Not only will this help determine an appropriate therapeutic strategy, the act of listening and allowing the patient to talk about their pains and associated anxieties is part of the pain management process. Basic to the control of pain is to manage and remove the pathological process causing pain, wherever this is possible. Reduction in requirements, for example, is an indicator that attempts to remove or control the underlying cause are succeeding. Managing pain successfully involves patient and family/carer participation, a collaborative multidisciplinary approach in most categories of haematological disorder-related pain; medication should aim to provide continuous pain relief wherever possible with a minimum of drug-related side effects. Highly soluble and suitable for use in a syringe driver for continuous administration or as a 4-hourly injection. Accumulation can occur with renal impairment Alternatives to opioids Tramadol may be given orally Fentanyl given as slow release transdermal patches may be a valuable alternative to slow release morphine for moderate-to-severe chronic pain. Many hospitals also run specific pain clinics and have palliative care or pain control teams who can review in-patients and offer advice. The support and expertise available should be enlisted particularly for difficult problems with persistent localized pain. For longterm painful conditions it is essential to work with medical and nursing colleagues in Primary Care and in Palliative Care so that the patient receives appropriate support in hospital as well as in the community setting. Physiotherapy care can also be appropriate, particularly with chronic joint damage. The severe acute pain in sickle crises often requires high and frequent doses of strong opiates. Health professionals unused to caring for patients with these conditions are sometimes cautious about use of such high doses and fear that patients may become addicted. Situations can arise whereby the patients are not receiving appropriate care because of such fears. Psychological dependence on opiates is rare, and may be associated with difficult psychosocial circumstances which makes care for such patients challenging. Patients (and their families) experience and demonstrate a number of reactions to their diagnosis, the clinical haematologist needs to have an awareness of this and respond accordingly. Ultimately most patients come to acceptance of their condition; carers/ partners will also go through a similar range of reactions. The clinician needs to be aware of the way in which news of a diagnosis is likely to affect a patient and their family/carers and respond appropriately. In the first instance this will often involve the need to impart the diagnosis, what it means, and what needs to be done clinically. Numbness at learning of a serious diagnosis often means that very little is taken in initially other than the diagnostic label. The various reactions just listed may subsequently emerge during the time the patient comes to accept the diagnosis, what it means, and what is to be done clinically. Within the haematological team there should be support available to the patient and family/carers which can provide them with practical information about the disease and its management. Simply knowing there is a sympathetic ear may be all that is required in the way of support; however, for some patients and families/carers more specialized support may be needed. Children may experience a variety of haematological diseases requiring admission to hospital. Specialist professionals such as play therapists can help families through frightening and confusing experiences. Use can be made of local or national patient support groups; knowledge of others in similar predicaments can help diffuse anger and loneliness.
