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Edward Buckley, M.D.

  • Department of Neurology
  • Duke University Medical Center
  • Durham, NC

With a combination of chemotherapy blood pressure 140 80 cheap 1.25 mg ramipril fast delivery, radiation you buy ramipril 5mg line, and surgery arteria yugular cheap ramipril 10mg mastercard, the 5-year survival rate is now 75% arrhythmia xanax buy 2.5mg ramipril. This loss of cartilage results in formations of new bone, called osteophytes, at the edges of the bone. Fragments of cartilage may also break free into affected joint spaces, producing loose bodies called "joint mice. A characteristic clinical appearance is the presence of crepitus, a grating sound produced by friction between adjacent areas of exposed subchondral bone. In contrast, anti-IgG autoantibodies (rheumatoid factor) are seen with rheumatoid arthritis, deficient enzyme in the metabolic pathway involving tyrosine (homogentisic acid oxidase) is seen with alkaptonuria, deposition Musculoskeletal System Answers 489 of needle-shaped negatively birefringent crystals (uric acid) is seen with gout, and deposition of short, stubby, rhomboid-shaped positively birefringent crystals (calcium pyrophosphate) is seen with pseudogout. Subcutaneous nodules with a necrotic focus surrounded by palisades of proliferating cells are seen in some cases. In the joints, the synovial membrane is thickened by a granulation tissue pannus that is infiltrated by many inflammatory cells. Nodular collections of lymphocytes resembling follicles are characteristically seen. The thickened synovial membrane may develop villous projections, and the joint cartilage is attacked and destroyed. In contrast, extensive gumma formation is seen with syphilis, tophus formation is seen with gout, and caseous necrosis of bone is seen with tuberculosis. Secondary gout may result from increased production of uric acid or from decreased excretion of uric acid. Primary (idiopathic) gout usually results from impaired excretion of uric acid by the kidneys. Most patients present with pain and redness of the first metatarsophalangeal joint (the great toe). Needle-shaped, negatively birefringent crystals of sodium urate precipitate to form chalky white deposits. Urate crystals may precipitate in extracellular soft tissue, such as the helix of the ear, forming masses called tophi. The degenerative joint disease osteoarthritis is the single most common form of joint disease. It is a "wear and tear" disorder that destroys the articular cartilage, resulting in smooth (eburnated, "ivorylike") subchondral bone. Rheumatoid arthritis, a systemic disease frequently affecting the small joints of the hands and feet, is associated with rheumatoid factor. Rheumatoid factors are antibodies-usually IgM-that are directed against the Fc fragment of IgG. In the joints, the synovial membrane is thickened by a granulation tissue (a pannus) that consists of many inflammatory cells, mainly lymphocytes and plasma cells. Ochronosis, 490 Pathology caused by a defect in homogentisic acid oxidase, is associated with deposition of dark pigment in the cartilage of joints and degeneration of the joints. Another change seen in denervated muscle is the presence of distinctive three-zoned fibers called target fibers. Reinnervation is characterized by type-specific grouping of fibers, which is in contrast to the mixed "checkerboard" pattern of type 1 and type 2 fibers seen in normal skeletal muscle. Variation in size and shape along with degenerative changes and intrafascicular fibrosis are features of muscular dystrophy. Eosinophils within muscle are found in association with parasitic infections, the most common of which is trichinosis. Transection of a peripheral nerve may result in the formation of a traumatic neuroma if the axonal sprouts grow into scar tissue at the end of the proximal stump. Sometimes peripheral nerves may be compressed (entrapment neuropathy) due to repeated trauma. Carpal tunnel syndrome is the most common entrapment neuropathy and results from compression of the medial nerve within the wrist by the transverse carpal ligament. Symptoms include numbness and paresthesias of the tips of the thumb and second and third digits. Another type of compression neuropathy is associated with a painful swelling of the plantar digital nerve between the second and third or the third and fourth metatarsal bones. The defective gene is located on the X chromosome and codes for dystrophin, a protein found on the inner surface of the sarcolemma. The weak muscles are replaced by fibrofatty tissue, which results in pseudohypertrophy. The classification of the muscular dystrophies is based on the mode of inheritance and clinical features. Sustained muscle contractions and rigidity (myotonia) are seen in myotonic dystrophy, the most common form of adult muscular dystrophy. In contrast, dermatomyositis is an autoimmune disease that is one of a group of idiopathic inflammatory myopathies. The inflammatory myopathies are characterized by immune-mediated inflammation and injury of skeletal muscle and include polymyositis, dermatomyositis, and inclusion-body myositis. These diseases are associated with numerous types of autoantibodies, one of which is the anti-Jo-1 antibody. Damage is by complement-mediated cytotoxic antibodies against the microvasculature of skeletal muscle. Histologically, examination of muscles from patients with dermatomyositis reveals perivascular inflammation within the tissue around muscle fascicles. This is in contrast to the other types of inflammatory myopathies, where the inflammation is within the muscle fascicles (endomysial inflammation). Werdnig-Hoffmann disease is a severe lower motor neuron disease that presents in the neonatal period with marked proximal muscle weakness ("floppy infant"). These antibodies cause abnormal muscle fatigability, which typically involves the extraocular muscles and leads to ptosis and diplopia. Other muscles may also be involved, and this may cause many different symptoms, such as problems with swallowing. Two-thirds of patients with myasthenia gravis have thymic abnormalities; the most common is thymic hyperplasia. Lack of lactate production during ischemic exercise is seen in metabolic diseases of muscle caused by a deficiency of myophosphorylase. Dermatomyositis is an autoimmune disease produced by complement-mediated cytotoxic antibodies against the microvasculature of skeletal muscle. Rhabdomyolysis is destruction of skeletal muscle that releases myoglobin into the blood. Rhabdomyolysis may follow an influenza infection, heat stroke, or malignant hyperthermia. The inflammatory myopathies are characterized by immunemediated inflammation and injury of skeletal muscle and include polymyositis, dermatomyositis, and inclusion-body myositis (the most common type of myositis in the elderly). These disorders are associated with numerous types of autoantibodies, one of which is the anti-Jo-1 antibody. In addition to proximal muscle weakness, patients typically develop a lilac discoloration around the eyelids with edema. Histologically, examination of muscles from patients with dermatomyositis reveals perivascular inflammation within the tissue that surrounds muscle fascicles. In particular, inclusion-body myositis is characterized by basophilic granular inclusions around vacuoles ("rimmed" vacuoles). A 50-year-old male presents with headaches, vomiting, and weakness of his left side. Physical examination reveals his right eye to be pointing "down and out" along with ptosis of his right eyelid. Swelling of the optic disk (papilledema) is found during examination of his retina. Aneurysm of the vertebrobasilar artery Arteriovenous malformation involving the anterior cerebral artery Subfalcine herniation Tonsillar herniation Uncal herniation 470. A newborn infant is being evaluated for a cystic mass found in his lower back at the time of delivery.

Ependymomas are distinguished by 514 Pathology ependymal rosettes blood pressure 60 over 30 discount 1.25mg ramipril with amex, which are ductlike structures with a central lumen around which columnar tumor cells are arranged in a concentric fashion blood pressure medication how long to take effect ramipril 2.5 mg with amex. Although they are usually tumors of middle or later life arteria fibrillation cheap 10mg ramipril with mastercard, a small number occur in persons 20 to 40 years of age blood pressure chart evening buy cheap ramipril 10 mg online. They commonly arise along the venous sinuses (parasagittal, sphenoid wings, and olfactory groove). Although meningiomas are benign and usually slowgrowing, some have progesterone receptors and rapid growth in pregnancy occurs occasionally. The typical case, however, does not invade the brain, but displaces it, causing headaches and seizures. Histologically, many different patterns can be seen, but psammoma bodies and a whorled pattern of tumor cells are somewhat characteristic. In contrast, Antoni A areas with Verocay bodies are seen in schwannomas, endothelial proliferation and serpentine areas of necrosis are seen in glioblastoma multiformes, a "fried-egg" appearance of tumor cells is characteristic of oligodendrogliomas, and true rosettes and pseudorosettes can be seen in medulloblastomas. Patchy intracerebral calcification may develop in tuberous sclerosis, an autosomal dominant disease characterized by the triad of epilepsy, mental retardation, and facial skin lesions (multiple angiofibromas). In addition, subependymal gliosis, cardiac rhabdomyoma, renal angiomyolipoma, and periungual fibroma occur. Calcification of the basal ganglia occurs in about 20% of patients with chronic hypoparathyroidism, which sometimes leads to a Parkinsonian syndrome. Astrocytomas occur predominately in the cerebral hemispheres in adult life and old age, in the cerebellum and pons in childhood, and in the spinal cord in young adults. The pilocytic astrocytoma is a subtype that is the most com- Nervous System Answers 515 mon brain tumor in children, and therefore it is also called a juvenile pilocytic astrocytoma. Meningiomas, found within the meninges, have their peak incidence in the fourth and fifth decades. Ependymomas are found most frequently in the fourth ventricle, while the choroid plexus papilloma, a variant of the ependymoma, is found most commonly in the lateral ventricles of young boys. The medulloblastoma is a tumor that arises exclusively in the cerebellum and has its highest incidence toward the end of the first decade. In children medulloblastomas are located in the midline, while in adults they are found in more lateral locations. These tumors are typically located at the cerebellopontine angle or in the internal acoustic meatus. Involvement of the facial nerve produces facial weakness and loss of corneal reflex. Histologically, an acoustic neuroma consists of cellular areas (Antoni A) and loose edematous areas (Antoni B). Verocay bodies (foci of palisaded nuclei) may be found in the more cellular areas. These include hemangioblastomas of retina and brain (cerebellum and medulla oblongata), angiomas of kidney and liver, and renal cell carcinomas (multiple and bilateral) in 25 to 50% of cases. Only the central, or acoustic, form produces bilateral acoustic neuro- 516 Pathology mas; the classic form may produce unilateral acoustic neuroma. It encodes for neurofibromin, a protein that regulates the function of p21 ras oncoprotein. Primary tumors of the pineal gland are very uncommon but are of interest, especially in view of the mysterious and relatively unknown functions of the pineal gland itself. The gland secretes neurotransmitter substances such as serotonin and dopamine, with the major product being melatonin. Tumors of the pineal gland include germ cell tumors of all types, including embryonal carcinoma, choriocarcinoma, teratoma, and various combinations of germinomas. Germ cell tumors may arise extragonadally within the retroperitoneal space and the pineal gland, with the only commonality being that these structures are in the midline. Primary tumors of the pineal gland occur in two forms: the pineoblastoma and the pineocytoma. Pineoblastomas occur in young patients and consist of small tumors having areas of hemorrhage and necrosis with pleomorphic nuclei and frequent mitoses. Pineocytomas occur in older adults and are slow-growing; they are better differentiated and have large rosettes. The signs and symptoms produced are related to the structures of the caudal medulla normally supplied by this vessel. Interruption of the lateral spinothalamic tracts results in segmental sensory dissociation with loss of pain and temperature sense, but preservation of the sense of touch and pressure or vibration, usually over the neck, shoulders, and arms. Since the most common location of a syrinx is the cervicothoracic region, the loss of pain and temperature sensation affects both arms. Characteristic features also include wasting of the small intrinsic hand muscles (claw hand) and thoracic scoliosis. The cause of syringomyelia is unknown, although one type is associated with a Chiari malformation with obstruction at the foramen magnum. The facial nucleus, which is located within the pons, is divided in half; the upper neurons innervate the upper muscles of the face, while the lower neurons innervate the lower portion of the face. It is important to realize that each half receives input from the contralateral motor cortex, while only the upper half receives input from the ipsilateral motor cortex. Patients present with facial asymmetry involving the ipsilateral upper and lower quadrants. Because the lacrimal punctum in the lower eyelid moves away from the surface of the eye, lacrimal fluid does not drain into the nasolacrimal duct. Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barrй syndrome) is a life-threatening disease of the peripheral nervous system. The disease usually follows recovery from an influenza-like upper respiratory tract infection and is characterized by a motor neuropathy that leads to an ascending paralysis that begins with weakness in the distal extremities and rapidly involves proximal muscles. In rare patients, instead of an acute course, Guillain-Barrй syndrome takes a chronic course with remissions and relapses. Focal peripheral neuropathies may involve one nerve (mononeuropathy) or multiple nerves (multiple mononeuropathy or monoradiculopathy). An example of a mononeuropathy is compression of the median nerve, which produces carpal tunnel syndrome. The median nerve provides sensory information from the palmar surface of the lateral three and one-half digits and the lateral portion of the palm. Also innervated by the median nerve are the major pronators (pronator teres and pronator quadratus), the thumb flexors (flexor pollicus longus and flexor pollicus brevus), and the opponens pollicis. Damage to the median nerve at the wrist as it lies deep to the flexor retinaculum results in burning sensations in the thumb, index and middle fingers, and lateral half of the ring finger (carpal tunnel syndrome). This syndrome is found in people who use their hands a lot, such as jackhammer operators, typists, and tailors. Treatment may involve cutting the transverse carpal ligament to decompress the nerve. Bibliography Abenhaim L, Moride Y, Brenot F et al: Appetite-suppressant drugs and the, risk of primary pulmonary hypertension. The Ohio State University College of Medicine and Public Health [12 April 2004] 15. The members of the committee responsible for the report were chosen for their special competences and with regard for appropriate balance. This study was supported by a contract between the National Academy of Sciences and the U. Contributors to the Fund have included Roche Vitamins, M&M/Mars, Mead Johnson Nutritionals, and the Nabisco Foods Group. Any opinions, findings, conclusions, or recommendations expressed in this publication are those of the author(s) and do not necessarily reflect the views of the organizations or agencies that provided support for the project. Dietary reference intakes for water, potassium, sodium, chloride, and sulfate / Panel on Dietary Reference Intakes for Electrolytes and Water, Standing Committee on the Scientific Evaluation of Dietary Reference Intakes, Food and Nutrition Board. The serpent has been a symbol of long life, healing, and knowledge among almost all cultures and religions since the beginning of recorded history. The serpent adopted as a logotype by the Institute of Medicine is a relief carving from ancient Greece, now held by the Staatliche Museen in Berlin. Upon the authority of the charter granted to it by the Congress in 1863, the Academy has a mandate that requires it to advise the federal government on scientific and technical matters. The National Academy of Engineering was established in 1964, under the charter of the National Academy of Sciences, as a parallel organization of outstanding engineers. It is autonomous in its administration and in the selection of its members, sharing with the National Academy of Sciences the responsibility for advising the federal government.

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As previously discussed blood pressure medication images discount 1.25 mg ramipril free shipping, a variety of methodological issues complicate the interpretation of observational studies arteriosclerosis obliterans buy ramipril 1.25mg without prescription. In this setting blood pressure of 100/70 ramipril 1.25 mg amex, clinical trials are the most appropriate study design to assess the relationship between sodium intake and blood pressure ocular hypertension buy discount ramipril 10 mg, and numerous trials have evaluated this relationship in nonhypertensive and hypertensive individuals (see Tables 6-12 and 6-13). The studies differ in size (< 10 to > 500 persons), duration (range: 3 days to 3 years), extent of sodium reduction, background diet. Only 10 trials tested three or more levels of dietary sodium intake (see Appendix I). Study populations also differed in age, race-ethnicity, and other dimensions that might affect the blood pressure response to changes in sodium intake. Notwithstanding these differences, available trials have provided relatively consistent evidence that a reduced intake of sodium lowers blood pressure in nonhypertensive adults (see Table 6-12). Some trials did not detect any effect on blood pressure from changes in sodium intake, while other trials recorded substantial reductions in blood pressure. Potential reasons for this heterogeneity include differences in study populations, inadequate statistical power, limited contrast in sodium intake, and other methodological issues. In trials with hypertensive participants (Table 6-13), the extent of blood pressure reduction from a lower intake of sodium was more pronounced than that observed in nonhypertensive participants. Individual trials that tested three or more levels of sodium intake provide the best evidence to assess dose-response relationships between dietary sodium intake and blood pressure. No trial lasted for more than one month, and several lasted only a few days (see Appendix Table I-2). The trial by MacGregor and coworkers (1989) is a well-controlled trial that documented a direct, progressive relationship between sodium intake and blood pressure, but the trial enrolled only 20 individuals, all of whom were hypertensive. The trial by Johnson and colleagues (2001) tested increasing levels of sodium intake from baseline by giving four different levels of sodium chloride (range of total intake: 0. A detailed overview of the trial by Sacks and colleagues (2001) is warranted in view of its size, duration, and other design features. In contrast, the potassium, magnesium, and calcium levels of the control diet corresponded to the 25th percentile of U. A total of 412 participants enrolled; of these, 41 percent were hypertensive, 40 percent were white, and 57 percent were African American (Sacks et al. The average achieved levels of sodium intake, as reflected by 24-hour urinary sodium excretion, were 142, 107, and 65 mmol/day, respectively, corresponding to approximate intakes of 3. On the control diet (Figure I-14 and Tables I-1a and 1c), reducing sodium intake from the higher (3. The trial by Sacks and colleagues (2001) also provided an opportunity to assess the impact of sodium reduction in relevant subgroups (Vollmer et al. On the control diet, significant blood pressure reduction was evident in each subgroup. Reduced sodium intake led to greater systolic blood pressure reduction in individuals with hypertension compared with those classified as nonhypertensive, African Americans compared with non-African Americans, and older individuals (> 45 years old compared with those 45 years old). First, the blood pressure response to sodium reduction was nonlinear, that is, there was a steeper decline in blood pressure when sodium was reduced from 2. In addition to the 10 trials that directly tested three or more levels of sodium intake, the Trials of Hypertension Prevention­Phase 1 (Kumanyika et al. In this 18-month randomized trial in which 327 nonhypertensive individuals were assigned to a reduced sodium behavioral intervention and 417 individuals were assigned to a control group, there was a mean net reduction in urinary sodium excretion of 44 mmol (1. In analyses that corrected for intraperson variability in sodium excretion and blood pressure, the estimated average systolic and diastolic blood pressure reductions per 100 mmol (2. Overall, available dose-response trials are consistent with a direct, progressive, dose-response relationship between sodium intake and blood pressure across a broad range of intake. A progressive relationship was also apparent in two smaller studies that tested four or more sodium levels across a broader range of sodium intake (range: 0. However, observational analyses of the four isolated populations in the Intersalt study suggest a progressive relationship for systolic blood pressure at urinary sodium levels between less than 0. Effects of Sodium Intake on Blood Pressure: Evidence from MetaAnalyses of Intervention Studies. Several meta-analyses of clinical trials have been conducted to assess the effects of sodium intake on blood pressure (Table 6-15). Typically, these studies estimate the ratio of the average change in blood pressure to observed average change in sodium intake. However, such ratios cannot be used to assess dose response unless the relationship is linear. The earliest meta-analyses aggregated data across a wide range of study designs, from very brief feeding studies lasting a few days to long-term behavioral intervention studies lasting a year or more. These meta-analyses have provided consistent evidence that a reduced sodium intake lowers systolic and diastolic blood pressure in hypertensive individuals. However, the extent of blood pressure reduction in nonhypertensive individuals is less consistent. The corresponding reductions in systolic/diastolic blood pressures in nonhypertensive persons were 2. One meta-analysis focused on trials conducted in older-aged persons (mean age close to 60 years) (Alam and Johnson, 1999). In this meta-analysis, which included both nonhypertensive and hypertensive persons, sodium reduction significantly lowered systolic and diastolic blood pressure by 5. The effect was more pronounced in trials that exclusively enrolled individuals older than age 60. A meta-analysis was conducted to assess the effect of modest sodium reduction to levels that would be relevant to public health decision-making (He and MacGregor, 2002). Trials of brief duration and those with extremely low sodium intakes were excluded. All of the included trials lasted 4 or more weeks, and many were controlled feedings studies. Another meta-analysis assessed the long-term effects of advice to reduce sodium intake (Hooper et al. Most included trials used intensive behavioral interventions in freeliving individuals. Net reduction in urinary sodium excretion as the result of the behavioral interventions was 35. This meta-analysis documents the difficulties of sustaining a reduced sodium intake in free-living persons over the long-term. Because of the limited net reduction in sodium intake as evidenced by attained urinary sodium excretion, the efficacy of sodium reduction as a means to lower blood pressure cannot be assessed from this analysis. In Canada, approximately 27 percent of adults 35 to 64 years old have hypertension (Wolf-Maier et al. It has been estimated that almost one-third of blood pressure-related deaths from coronary heart disease are estimated to occur in individuals with blood pressure in this range (Stamler et al. The prevalence of hypertension rises progressively with age, such that more than half of all Americans 60 years of age or older have hypertension (Hajjar and Kotchen, 2003). Among nonhypertensive adults, the estimated lifetime risk of developing hypertension is 0. The rise in blood pressure with age, while commonplace in Western countries, is not universal, as there are non-Western populations, as well as some Western populations. In ecologic observational studies, a reduced intake of sodium and an increased intake of potassium have been associated with a blunted age-related rise in blood pressure (Rose et al. Hypertension can be prevented by complementary application of strategies aimed at achieving a downward shift in the distribution of blood pressure in the general population (population-based strategy) and more intensive targeted strategies aimed at achieving a greater reduction in blood pressure in individuals and groups at greater risk for high blood pressure (intensive targeted strategy) (Whelton et al. Because the health benefits of a population strategy are applied to large numbers, even small downward shifts in the distribution of blood pressure can be expected to result in a substantial reduction in the burden of illness in the population being targeted (Rose, 1985; Whelton et al. For example, a downward shift in the population distribution of systolic blood pressure by 2 mm Hg would be expected to result in an annual reduction of 6 percent in mortality from stroke and 4 percent from coronary heart disease (Stamler, 1991). The corresponding estimates would be 8 and 5 percent for a 3-mm Hg downward shift in the population distribution of systolic blood pressure, and 14 and 9 percent for a 5 mm Hg shift (Stamler, 1991). Sodium reduction is one of several nutritional therapies that have been proposed as a means to reduce blood pressure and thereby affect a downward shift of blood pressure in the population (Chobanian et al.

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Thus blood pressure which arm order ramipril 1.25 mg line, the counselor can provide services as group counseling to help the family adjust to the special need(s) and aid in Intervention of the family member blood pressure medication hctz buy discount ramipril 10mg on-line. If the family is to play a role in the Intervention of your child heart attack the alias radio remix demi lovato heart attack remixes 20 buy ramipril 1.25 mg online, then the family must be prepared for that role blood pressure medication chart buy 5 mg ramipril. Selecting the best options for your child ­ the following five very good sources of information can help you make choices about Early Intervention 1. Other parents ­ particularly those who are active leaders in local parent organizations. They see the big picture and can provide very useful tips about how to proceed and where the best supports are in your community 4. Professionals who are on the front line are good sources of information by working directly with families and 43 children in Early Intervention programs. They are aware of innovative methods, attend professional meetings, receive continuing education, and have a reservoir of information from their own first hand experiences 9. What kind of standing does it have in the community for the quality of the program? Does it encourage family members to participate in some Intervention sessions and practice with your child? How well are its costs covered by insurance or Medicaid and what are your financial liabilities? If it is an outpatient or home program, is there someone living at home who can provide care? Services that may be included in Early Intervention programs but not limited to , are listed as follows: a. Four types of Intervention strategies that can be used with children demonstrating deficits in home management or care giving are: a. Remediating performance areas and performance component deficits ­ this phase focuses to improve or restore performance to pretreatment levels. Certain parameters need to be considered when measuring the effectiveness in this phase: physical assistance, supervision, task demands, amount of task, type of task, and the environment b. Teaching new methods of task performance to compensate for performance area or performance component deficits ­ this phase focuses on using remaining abilities to achieve the highest level of functioning possible in the areas of home and family management. These techniques would require that the Physical Therapist and the child/parent address the following points: 1. Determine what tasks need to be improved (what tasks take too long, cause fatigue, or take too much energy) 45 2. Develop a new method for performing the task ­ consider eliminating the unnecessary steps, combining motions and activities, rearranging the sequence of the steps, and simplifying the details of the task by taking the following steps: A. In sitting, your child should avoid positions that require lifting the shoulders or "winging out" the elbows B. Having necessary supplies and equipment increases productivity with less effort D. Use labor saving devices ­ includes wheels for transport and electrical devices E. Use proper body mechanics using a wide base of support, using both sides of the body and keeping objects close to the body, facing objects when reaching or lifting to avoid twisting, pushing rather than pulling objects, and alternating positions and motions to avoid fatigue c. Educating your child and family to support the above approaches or as a means of minimizing future problems ­ 46 these are important because they involve learning new strategies. Promote the highest level of learning possible, preferably the problem-solving level 7. State your wishes and opinions on matters that affect your child or yourselves as parents 4. Encouraging appropriate child behavior ­ effective parenting is based on an understanding of your child and his or her current developmental level and needs. You will need to assist your child to develop new skills while providing love and other supports. Some problematic behaviors and challenging skill deficits that are common among children with Batten Disease include: a. When behavior puts your child or others in danger of injury, it is definitely time to get help. When faced with a less severe behavior problem, you may wait, hoping it will get better. If common-sense approaches have not worked and you feel concerned, you may wish to seek the help of an expert. Be sure that the professional you choose has experience dealing with children with special needs and their behaviors, and relies on welldocumented, scientifically sound procedures. A competent specialist will be able to offer you practical advice on what to do and what not to do. You should first ask your Pediatrician or family Doctor for his advice on a behavior specialist. If there is a child development center near you that serves children with special needs, they may have a qualified person, or you may check the hospital. Provide positive reinforcement ­ emphasizing what your child enjoys and does well b. Punishment If under the guidance of a Therapist, you decide to use a punishment procedure, use the following guidelines: Be sure that you can deliver the indicated brief, mild punishment to your child safely and consistently. The punishment is not likely to be effective if your child can ignore verbal reprimands, play with a favorite toy during time-out, or get out of losing a privilege Apply the punishment confidently and not in graded steps. A firm but matter of fact "no" is better than an apologetic request to stop, followed by loud reprimands 49 when your child does not obey. Avoid lectures, nagging, scolding, threatening or sarcastic remarks, extended time-outs, or any physical aggression that may frighten or hurt your child In the beginning, apply punishment immediately each time the targeted inappropriate behavior occurs. When your child misbehaves during a work activity, ensure that the task is completed. Then, simply state the cause or reason for the punishment, describe the consequence and enforce it. If your child has to be removed before the task is complete, return to the task following the punishment Be careful that the delivery of punishment is not associated with the later delivery of reinforcement. Expressing your love in the context of apologizing for having disciplined your child may send a confusing message Avoid prolonged or extensive use of punishment. Review the plan and make adjustments if the punishment does not reduce or stop the problem behavior B. Parent Reactions and Adaptations to Having A Child With A Terminal Illness Every parent of a child with an illness may ask at one time or another, "Why my child? In such instances, parents may expend much emotional and physical energy and money searching for a diagnosis. Sometimes the search for a cause can take precious time from the development of a treatment plan. After the initial period of shock and denial, some family members experience depression. This can result from emotional stress combined with the physical strain of following through on the many appointments, procedures, recommendations, and care required by your child. Other factors contributing to depression may be spousal disagreement over acceptance of the diagnosis, assignment of blame, choice of treatment options, and/or responsibility in caring for your child. Symptoms of depression include extreme fatigue, restlessness or irritability, insomnia, eating disorder, or loss of sex drive. In this state, the parent(s) may not be able to ask for help, know what they need, or stay connected to significant support systems. Even under these circumstances, however, it may be difficult to hear words spoken aloud that confirm your fears. You may go through a period of denial, either before or after receiving the diagnosis. On a deeper level, denial over a short-term functions as a protective mechanism that we use to conserve our emotional energy for the critical tasks that we must accomplish, such as child rearing. When receiving bad news, people either internalize or externalize their reactions. The two most common emotions experienced by people who internalize are guilt and shame. Externalization refers to the process of placing responsibility for events on others. The most common feelings experienced by people who externalize are blame and anger.

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