UNIT 5 NURSING CARE OF CHILDREN WITH ENDOCRINE AND METABOLIC DISORDERS Structure 5.0 Objectives 5.1 Introduction 5.2 Classification of Endocrine Disorders 5.2.1 Disorders of Pituitary Function 522 Disorders of Thyroid Function 52.3 Disorder of Parathyroid Function 52.4 Disorder of Adrenal Function 52.5 Disorders of Pancreatic Hormone Function 5.3 Common Endocrine Disorders 5.3.1 Diabetes Mellitus 5.32 Diabetes Insipidus 5.4 Inborn Errors of Metabolism 5.5 Let Us Sum Up 5.6 Answers to Check Your Progress 5.0 OBJECTIVES After completing this unit, you should be able to: classify various endocrine disorders; explain the diabetes insipidus; differential between the various categories of diabetes mellitus; e describe the management and nursing care of child with diabetes mellitus; formulate a teaching plan for educating the family and child with diabetes mellitus; describe inborn errors of metabolism; and discuss nursing care of a child with inborn errors of metabolism. 5.1 INTRODUCTION In the previous unit we have discussed about pediatric emergencies. Now we shall focus on endocrine and metabolic disorders. Endocrine dysfunction in children fiequently leads to altered growth and development. The accurate identification and assessment of children is important to detect any deviation in growth and developmental patterns and identify the factors that cause any alteration in normal functioning of the body. In this unit we shall focus on general classification of endocrine disorders. We shall also discuss about common conditions such as diabetes insipidus and diabetes mellitus in detail. At edwe \ha\\ bon inborn enorsorsofmetabolism. These BNS-106. also been discussed in medical suigical nursing wit kindly refer BNS-106. problems have Before reading this Nursing Care of Children with Medical and Surgical Problems-I1 5.2 CLASSIFICATION OF END~CRINE DISORDERS -- The endocrine disorders can be classified as given below: 5.2.1 Disorders of Pituitary Function The disorders of pituitary function result in following conditions. Hypopituitarism: Growth Hormone (GH) Deficiency Hypopituitarism is primarily a disorder associated with deficient secretion of GH (somatropin). It may be caused by a variety of conditions which may include developmental defects, destructive lesions such as tumors, trauma, vascular abnormalities, or surgery, certain hereditary disorders, or functional disorders such as anorexia nervosa or psychosocial dwarfism. In more than half of children with hypopituitarism, no lesion is evident and the cause is unknownidiopathic hypopituitarism or idiopathic pituitary growth failure. The children with hypopituitarism may be normal at birth but later during infancy there is deviation in growth from normal growth rate. Treatment consists of replacement of growth hormone and in cases where cause is due to organic lesion then surgical removal of lesion is done. Fig. 5.1: Child with Hypopituitarism Pituitary Hyperfunction If the excess of Growth Hormone (GH) occurs prior to closure of the epiphysieal shafts it results in proportional overgrowth of long bones until the individual reaches a height of 8 feet or more. There is rapid and increased development of muscles and viscera along with vertical growth. Weight is increased but is usually in proportion to height. Also there is proportional enlargement of head circumference which may result in delayed closure of the fontanels. Children with a pituitary-secreting tumor may also demonstrate signs of increased intracranial pressure, especially headache. If hypersecretion of GH occurs after epiphyseal closure, growth takes place in the transverse direction, producing a condition known as acromegaly. Typical facial features include sinuses, separation and malocculsion of the teeth in enlarged jaw, disproportion of the face to the cerebral division of the skull, increased facial hair, and thickened and deeply creased skin. Therapeutic treatment includes cryosurgery or hypophysectomy to remove tumor whenever feasible followed by other therapies such as external irradiation and radio active implants. Depending upon the insufficiency hormone replacement is also done. Diabetes Insipidus It is a principal disorder of posterior pituitary hypofunction. It is also known as Neurogenic Diabetes Insipidus (DI). It occurs as a result of hyposecretion of antidiuretic hormone (ADH), or vasopression, which produces a state of uncontrolled diuresis. The causes treatment and nursing care is discussed in detail in section 5.4.2. Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIAH) This is a disorder caused by hypersecretion of the posterior pituitary antidiuretic hormone (ADH, vasopressin). The syndrome of inappropriate Antiduretic. Hormone secretion occurs with increased frequency in various conditions such as tumour, infections and trauma of central nervous system. Increased secretion of ADH causes the kidneys to reabsorb water, which increases the fluid volume and decreases serum osmolality. When serum sodium levels are lowered to 120 mEgL, the child presents the symptoms of anorexia, nausea (sometimes vomiting), stomach cramps, irritability, and personality changes. With progressive reduction in sodium, child may present other neurologic signs such as stupor and seizures, etc. The symptoms disappear when the underlying disorder is corrected. Immediate management consists of restricting fluids. 5.2.2 Disorders of Thyroid Function The disorders of thyroid function may lead to following problems: Juvenile Hypothyroidism Hypothyroidism is one of the most common endocrine problems of childhood. It may be either congenital or acquired. In this there is a deficiency in secretion of thyroid hormone (TH). It may present as decelerated growth, dry skin, puffiness around eyes, sparse hair, constipation, sleepiness and mental decline Therapeutic treatment consist of replacement of thyroid hormone. Goiter A goiter is an enlargement or hypertrophy of the thyroid gland. It can be congenital or acquired. Congenital disease usually occurs as a result of antithyroid drugs andlor iodides administered to the mother during pregnancy. The acquired disease can result from increased secretion of pituitary thyrotropic hormone in response to decreased circulating levels of TH, neoplastic or inflammatory processes, or dietary iodine deficiency. The enlargement of thyroid at birth can cause respiratory distress so immediate surgery is needed to remove the enlarged part of the gland. The replacement of thyroid hormone is necessary to treat hypothyrodism. Lymphocytic Thyroiditis Lymphocytic throiditis is the most common cause of thyroid disease in children and adolescents, and it accounts for the largest percentage of juvenile hypothyroidism. It also accounts for many of the enlarged thyroid glands formerly designated as thyroid hyperplasia of adolescence, or "adolesecent goiter". The disease is more common in girls than in boys and in white persons thgn in black persons. It occurs morr. frequently after age 6, reaching a peak Nursing Care of Children with Endocrine and Metabolic Disorders Nursing Care of Children with Medical and Surgical Problems-I1 incideqce at adolescence there is evidence that the disease is self-limited. A child with this condition may present various signs and symptoms such as enlarged thyroid gland, sense of fullness, hoarseness dysphagia, nervousness, irritability, increased sweating and hyperactivity. Treatment consists of oral administration of thyroid hormone. Hyperthyroidism (Graves Disease) Graves diseases is the most common cause of hyperthyroidism in children and is usually associated with an enlarged thyroid gland and exophthalmus. The peak incidence of the disease occurs between 12 and 14 years of age, but it may be present at birth in children of thyrotoxic mothers, The incidence is five times higher in girls than in boys. There is no specific cause of this disease but it is apparently caused by a serum thyroid stimulating immunoglobulin and has familial association; a large number of persons with the diseAse possess the histocompatibility antigenc (HLA-B8). The child presents the signs and symptoms such as; emotional liability, restlessness, low school performance, fatigue, tachycardia, dyspnea on exertion, exophthalmos tremor, goiter, warm moist skin, heat intolerance, systolic murmur, thyroid storm such as severe irritability, anorexia and weightloss vomiting, diarrhoea, hyperthyroidism, hypertension, severe tachycardia, prostration. This may lead to delirium coma and death. Therapeutic management consists of anti thyroid drugs, subtotal thyroidectomy and ablation with radio iodine (13 1 - lodide). The children should be advised to restrict vigorous exersion until1 thyroid levels are decreased to normal. 5.2.3 Disorder of Parathyroid Function The disorders of parathyroid function may either lead to increased production or decreased production of parathormone. Hypoparathyroidism There are two classic forms of hypoparathyroidism which may occur during childhood as given below. a) Autoimmune hypoparathyroidism, in which there is decreased production of parathormone (PTH), usually in relation to auto-immune phenomena. b) Familial hypoparathyroidism is inherited as an autosomal recessive trait. It has early onset, usually in the first month of life. In pseudohypoparathyroidism there is increased production of PTH but end-organ responsiveness to the hormone is deficient. Pseudohypoparathyroidism is also thought to be inherited as an X-linked dominant trait with variable expressivity. Transient hypoparathyroidism may also be observed in infants born to mothers with the hypothyrodism or in infants fed a milk formula with a high phosphate/calcium ratio. The hypoparathroidism in children may present with dry, scaly, coarse skin with eruptions, hair often brittle, thin nails with grooves, Tetany, dental and enamel hypoplasia, muscle crumps or twitching, paresthesia tingling, diarrhoea, vomiting, headache depression, loss of memory etc. Therapeutic treatment consists of oral administration of calcium glucosnate and vitamin D therapy. Hyperparathyroidism Hyperparathyroidism is rare in childhood but can be primary or sencondary. The most common cause of primary hyperparathyroidism is adenoma of the gland and that of secondary hyperparathyroidism is chronic renal disease, renal o~teodystrophy,and congenital anomalies of the urinary tract. Hypercalcernia present in both primary and secondary cases of hyper parathyroidism. is 5.2.4 Disorder of Adrenal Function The disorders of adrenal function may lead to acute and chronic .adrenocortical insufficiency. Let us briefly describe each one as follows. Acute Adrenocortical Insufficiency This is a rare disorder and may result from variety of causes such as haemorrhage in glands from trauma due to difficult labour, meningococcal infections and or abrupt withdrawal of exogenous sources of cortisone or failure to increase exogenous supplies during stress or congenital adreno genital hyperplasia. Management includes replacement of cortisol, body fluids to correct dehydration and hypovolemia administration of glucose to correct hypoglycemia and specific antibiotic therapy. Blood transfusion is given if haemorrhage is severe. Chronic Adrenocortical Insufficiency (Addison's Disease) This disorder rarely occurs in children. It is caused by destructive lesion of the adrenal glands or a neoplasm, or it is idiopathic. The child may present with neurologic symptoms such as muscular weakness, mental fatigue, irritability pigmentry changes, palinar creases, hyperpigmentation over pressure points, dehydration, anorexia weight loss, headache, hunger, sweating and weakness etc. Therapeutic treatment includes replacement of cotisol and aldosterone. Cushing Syndrome Cushings syndrome is uncommon in children. It is a characteristic group of manifestations caused by excessive circulating free cortisol. (Fig. 5.2). It may occur due to one or more of the following causes: Pituitary with adrenal hyperplasia, usually attributed to an excess of ACTH Excessive growth Swollen face Red abdominal striae Poor wound healing Nursing Care of Children with Endocrine and Metabolic Disorders I Nursing Care of Children with Medical and Surgical Problems-I1 Adrenal with hypersecretion of glucocorticoids, generally the result of adrenocortical neoplasms Ectopic with autonomous secretion of ACTH, most often caused by extrapituitary neoplasms Iatrogenic, frequently the result of administration of large amounts of exogenous coricosterioids Food dependent, inappropriate sensitivity of adrenal glands to normal postprandial increases in secretion of gastric inhibitory polypeptide Therapeutic management involves bilateral adrenalectomy and post operative replacement of the cortical hormgnes. Congential Adrenogenital Hyperplasia (CAH) In the congential adrenogenital hyperplasia there is excessive secretion of andogens by the adrenal cortex. These conditions are variously known as congenital adrenogenital hyperplasia (CAH), adrenocortical hyperplasia (AH), adrenogential syndrome (AGS), and congenital adrenocortical hyperplasia (CAH). In children most common cause of congenital adrenogenital hyperplasia (CAH) is hyper function of adrenal gland, an inborn deficiency of various enzymes necessary for the biosynthesis of cortisol. CAH is inherited as an autosomal-recessive disorder or may result from a tumor or maternal ingestion of steroids. The child may present with enlarged clitoris, fusion of labia vaginal orifice. in females and precocious of genital development, frequent eruptions and genital enlargement, etc. in male. Therapeutic management includes cortisone administration to suppress the abnormally high secretion of ACTH. Reconstructive surgery may be done in females. Since, these children are not diagnosed at early stages therefore due to masculanisation of the external genitalia in female may, lead to sex assignment as male so the child should be reared as male. 5.2.5 Disorders of Pancreatic Hormone Function The most common disorder of pancreatic hormone secretion results in Diabetes Mellitus (DM). DM is a most frequent endocrine disorder of childhood, with the peak incidence during early adolescence. It is a disease of metabolism characterized by a total or partial deficiency of the hormone insulin, resulting in a metabolic adjustment or physiologic change in almost all areas of the body. The details of this disorder are described in subsection 5.4.2 5.3 COMMON ENDOCRINE DISORDERS We shall focus on two conditions Diabetes mellitus and Diabetes inspidus. 5.3.1 Diabetes Mellitus Diabetes mellitus (DM) is a disorder of glucose intolerance caused by deficiency in insulin production and action, resulting in hyperglycemia and abnormal carbohydrate, protein, and fat metabolism. It can be classified into three major groups Insulin Dependent (IDDM), or type I: This is characterized by catabolism and the development of ketosis in the absence of insulin replacement therapy. Its onset is typcially in childhood and adolescence but can be at any age Non-insulin-dependent (NIDDM), or type 11: It involves resistance to insulin action and defective glucose-mediated insulin secretion. Its onset is usually after the age 40. The persons with this type of DM may or may not require daily insulin injections. Maturity-onset Diabetes of Youth (MODY): It is transmitted as an autosomal-dominant disorder in which there is formation of structurally abnormal insulin that has decreased biologic activity Type I, formerly called juvenile onset or insulin dependent diabetes mellitus (IDDM) most commonly occurs in younger children or school aged children. Type I DM affects as many as 1 in 500 children. Type 11diabetes mellitus (formerly called adult onset or non-insulin dependent diabetes mellitus (NIDDM) was formerly found in only about 2 per cent of cases of diabetes in children and adolescents. This is rapidly changing, and occurrence of Type I1 diabetes is increasing among adolscents. Etiology The factors responsible for the diabetes mellitus are genetic, environmental or acquired factors abnormal immune responses, including autoimmune reactions. 0 Morbid obesity, sedentary life style, high calorie intake and family history of diabetes. Pathophysiology As you'know insulin is needed to support the metabolism of carbohydrates, fats, and proteins, primarily by facilitating the entry of these substances'into the cell, with the exception of nerve cells and vascular tissue. Due to deficiency of insulin, glucose is unable to enter the cell, and its concentration in the blood stream increases. The increased concentration of glucose produces an osmotic gradient that causes the movement of body fluid from the intracellular space to the extravcelluar space, from there the body fluid is excreted by the kidneys. When the serum glucose level exceeds the renal threshold (+ 180 mgldl), glucose "spills" into the urine leading to glycosuria, and there is an osmotic diversion of water called Polyuria which is a cardinal sign of diabetes. The urinary fluid losses cause the excessive thirst called Pblydipsia which is present Nursing Care'of Children with Endocrine and Metabolic Disorders Nursing Care or Children with Medical and Surgical Problems-I1 in all cases of diabetes. he excessive urination (water loss) results in a depletion of other essential chemicals in the body. Clinical Manifestations A child with diabetes myellitus may present with following symptoms: 1) Rapid onset (usually over a period of a few weeks) 2) Major symptoms: - Increased thirst - Increased urination, enuresis - Increased food ingestion - Weight loss - Fatigue Minor symptoms: - Skin infections - Dry skin, poor wound healing - Monlial vaginitis in adolescent girls If there is diabetic ketoacidosis then following symptoms may occur because the patient may go into shock. 3) Diabetic ketoacidosis (DKA) The symptoms at precomatose state and comatose state are given below: Precomatose state: - Drowsiness - Dryness of skin - Cherry red lips - Increased respirations - Nausea - Vomiting Abdominal pain Comatose state: - Extreme hyperpnea (kussmaul breathing) - - Acetone breath - So% sunken eyeballs - Rigid, weak pulse - Decreased temperature - Decreased blood pressure Circulatory collapse and renal failure may follow, resulting from the combination of lowered PH, electrolyte deficiency and dehydration Diagnostic Evaluation The diabetes myellilus may be diagnosed by following: - Presence of symptoms as already discussed * - Glycosuria on routine examination of urine - Random blood glucose which shows blood glucose level higher than the 200 mg/dl - Ketonuria - Metabolic acidosis (pH less than 7.3 and bicarbonate less than 14 mEq/L) Treatment If the child presents the symptoms of ketocidosis then folloking measures are taken: Nursing Care of Children with Endocrine and Metabolic Disorders Insulin therapy-to reduce hyperglycemia and inhibit lipolysis and ketogenesis Fluid therapy to-treat dehydration, increase peripheral perfusion, and replace sodium and potassium loss due to osmotic diuresis. Subcutaneous insulin may be given twice a day at a dose usually of a 2:l ratio of NPH to regular (or lispro) and a 2:l ratio for morning to evening dose. Initially short-acting insulin is often given four times a day. Dosage is based on the child's size, diet, and level of activity. Dosages should be adjusted through daily monitoring of blood glucose levels DKA treatment - low dose continuous IV infusion of regular insulin only In many settings insulin pump therapy, consisting of a continuous subcutaneous infusion of insulin that can be programmed to give bolus and basal rates, may be used in some children. Balanced diet should be given which should contain controlled carbohydrates and adequate protein and fat to meet energy and growth requirement. Complications There can be acute, subacute or chronic complications Acute Usually reversible Dabetic ketoacidosis (DKA) accounts for 70 per cent of diabetes-related deaths in 'children under 10 years of age Cerebral edema - this may be related to treatment correction and is due to a rapid decline in blood glucose causing a fluid shift. Hyperglycemia as a result of under of untreated cases Hypoglycemia as a result of (insulin reaction) Subacute Develops over short period of time Lipohypertrophy (localized tissue build-up from giving injections in the same site) - repeated injections in the same area; can cause abnormal absorption Skeletal and joint abnormalities - limited joint mobility Growth failure and delayed sexual maturation due to underinsulinization Chronic These are Very rarely seen in children which include: Retinopathylcataracts - may cause blindness Neuropathy-peripheral and autonomic Nephropathy-proteinuriahenal failure Cardiooathv-connestive cardiac failure I Surgical Problems-I1 Nursing Assessment You have to make assessment during the onset of symptoms after the diagnosis and during the treatment. a Obtain history of onset of signs and symptoms Assess for levels of dehydration and weight loss and level of appetite a Check for sores that slowly heal Identify any fruity smell to breath-acetone breath due to ketosis If the child is diagnosed as a case of diabetes and is getting treatment for ketoacidosis then you have to: a I Assess for potential cerebral edema (diminished level of consciousness) when fluid replacement is initiated Assess cardiac function-There may be tachycardia due to dehydration, arrhythmias related to potassium imbalances Assess renal function by checking - urinary output with intake and output, presence of ketonuria and glucosuria a I Watch for hypoglycemia-over treatment of insulin may lower glucose level. IV replacements frequently contain glucose to prevent large osmotic fluid shifts leading to cerebral edema. During treatmentlroutine follow-up: Assess weight-excessive weight gain may indicate overtreatment of insulin (child eats due to constant hunger). Loss or lack of weight gain may indicate underinsulinization (losing calories that are not metabolized). Review blood glucose daily for level of control and need for insulin adjustments (check for appropriate adjustments of insulin made by parents). Obtain history of any hypoglycemic reactions dietary record and exerciselactivity. I - be specific to time of day, Assess injection sites-look for sign of lipchypertrophy e I Assess for signs of hyperglycemia-polyuria, polydipsia. Does child need to get up in the night to go to the bathroom Nursing Diagnoses I These may be Deficit of fluid volume which is related to osmotic diuresis and vomiting Altered nutrition: Less than body requirements due to metabolic catabolism due to lack of insulin a Lack of Knowledge related to insulin management and to blood glucose monitoring Risk for injury related to hypoglycemia Fearlanxiety of child and family related to diagnosis, treatment and management procedures Nursing Interventions 110 Maintain fluid balance Administer IV fluids as ordered Monitor intake and output, blood pressure, serum electrolyte results and daily weight Report any abnormality Assess for signs of dehydration-dry skin and mucous membranes, constipation Encourage to take oral fluids when the child is able to take orally Maintain Adequate Nutritional Status 1) Provide an adequate diet for the child and teach the family about the diet i) Explain that the diet should contain 55 per cent carbohydrate, 30 per cent fat, and 15 per cent protein ii) Approximately 70 per cent of the carbohydrate content should be derived from complex carbohydrates such as starch iii) Foods with high fiber content should be encouraged iv) All diets must supply sufficient caloric intake for activity and growth, sufficient protein for growth, and the required vitamins and minerals - Provide and distribute Foods throughout the day to accommodate varying peak action of insulin and adjust the foods according to increased or decreased amounts of exercise - Include fluids containing sugar (sodas, juices, milk) in carbohydrate count. 2) Include the child and parents in meal planning as soon as possible 3) During hospitalization allow the child normal activity so that the observed result of the dietary control will be valid. 4) Allow the child to eat with other children 5) Make certain that the child adheres to the prescribed diet and understands the rationale for it 6) Refer family to a dietitian for additional planning and education Teach About Administration of Insulin a Insulin should be given as directed. Lispro (Humalog) insulin begins to work immediately and should be given right before the meal. If taking regular insulin, the child should wait 20-30 minutes before eating. a Be aware of the major types of insulin and their effects . Develop a systematic plan for injections that emphasizes rotation of sites to avoid ulceretion or wound Fig. 5.3. a Guidelines for site location: Nursing Care of Ct~ildren with Endocrine and Metaholic Disorders Nursing Care of Children with Medical and Surgical problems-11 Be certain that the measuring scale of the syringe matches the unit strength on the bottle of insulin. U-100 insulin is preferred because it allows the smallest possible amount to be given. Other concentrations of insulin are rarely used. Use insulin that is at room temperature - Keep the bottle of insulin in use at room temperature for approximately 1 month without losing appreciable strength - Extra bottles should be stored in the refrigerator Before giving insulin mix the solution thoroughly by shaking the bottle (rolling may not be effective) Administer insulin subcutaneously e Observe the skin closely for signs of irritation and allergic reaction (rash). Don't give insulin injection on a site if there are signs of irritation Fig. 5.3: a) b) Insulin injection sites are the upper outer portions of the arms, the thighs, and the abdominal area. Injection sites are ortated, with subsequent injections given about 2.5 cm (1 inch) apart. Fig. 5.3: (C) Inssiin injection by a child. Teach the importance of normal activity Explain that exercises and infection can increase the need for insulin Explain the patient and be alert for signs of infection and dehydration Encourage the child to express feelings about the injections. Nursing Care of Children with Endocrine :nd Metabolic Disorders Providing Information about Blood Glucose Monitoring Teach the child and parents about monitoring the blood glucose. Explain that the procedure requires a drop of blood (obtained by finger stick a health care personnel or parents. Identify and Control Hypoglycemia Explain family about the causes, of hypoglycemia. Which include. Overdose of insulin Reduction in diet or increased exercise without sufficient caloric coverve Teach Symptoms of hypoglyceiilia which include: Trembling, shaking, dizziness Sweating, apprehension Tachycardia Hunger, weakness Drowsitress, unusual behaviour Seizures, coma Be prepared to give orange, juice, sugar cubes, or another food containing readily available simple sugars. Watch for a patte% of activity or time of day that precedes hypoglycemic reactions and work with family to alter behaviour to prevent reactions If glucagons cannot be given and the child is unresponsive, honey orcorn syrup can be rubbed inside a cheek while positioning the child to prevent aspiration Reduce Fear and Anxiety I procedures on yourself first (e.g. finger, sticks for glucose testing, allow parent to give saline injection for practice of insulin injection). Allow parents to verbalize feelings related to the expectations of their performance. Assist the parents in performing the needed tasks (finger sticks, insulin injections) to build their confidence. Instruct home management Caution the parents that the focus on the diabetic child may cause sibling rivalry.& ) , E n c them ~ to~involveall ~ ~ family ~ members in care and give K,ttentian to other children Prevent the child fmm developing pilty feeling for oc,~unenclof disease. - Explain about care and managementdm health living - pt'A 11' Nursing Care of Children with Medical and Surgical Problems-I1 Community and Home Care Considerations Perform home assessment for adequate nutritional resources Review and reteach family 's adherence to insulin administration blood glucose monitoring and ability to respond to hypoglycemia Ensure that school is able to follow through with management plan for insulin administration, planned exercise and meal times, and responding to hypoglycemia Teach family how to monitor condition, maintain insulin coverage, and notify the health care provider when child is ill, evaluate for dehydration, hyperglycemia, and ketonuria. Family Education and Health Maintenance Educate the family about: Influence of exercise, emotional stress, and other illness on both insulin and diet needs how to recognise the symptoms of insulin shock and diabetic acidosis and related emergency management Prevention of infection: - Attend to regular body hygiene, with special atte:lc;on to foot care - Report any breaks in the skin. Treat them promptly. - Use only properly fitted shoes; do not wear vinyl or plastic, which do not permit ventilation. Avoid calluses and blisters - Dress the child appropriately for the weather - See that the child receives regular dental checkups and maintenance every 6 months - Follow routine immunizations according to the recommended schedule Precautionary measures: a) Instruct the child to carry an identification card that states that he or she has diabetes and includes name, address, telephone number, and health care provider's name and telephone number b) Suggest a simple, convenient source of sugar that can be easily carried by the child or parents in a pocket, purse, or backpack to have available for hypoglycemic symptoms. A good example is five sugar cubes or cake decorating gel that comes as a tube, such as Cake Mate. Follow up with care provider or pediatrician for immunizations, at regular health check-ups, and growth and development evaluation. Outcome B p e d Evaluation Intake equals output, blood pressure is stable, sodium and potassium within normal limits Parents and child describe a meal plan that is followed consistently Child and parents demonstrate correct insulin administration technique and correct glucose monitoring technique 8 8 Child and parents describe causes, signs and symptoms, and treatment for hypoglycemia Child and parents talk freely about diabetes, ask appropriate questions and display no cryinglfear I 5.3.2 Diabetes Insipidcs Diabetes insipidus (DI) is failure of the body to conserve water due to a ' deficiency of Antidiuretic Hormone (ADH), decreased renal sensitivity to ADH, or suppression of ADH secondary to excessive ingestion of fluids (primary polydipsia). Signs and Symptoms Sudden onset of excessive thirst and polyuria The infants may present with following symptoms: Excessive cryingquieted with water more than milk feeding Rapid weight loss-caloric loss due to water preference over feedings Constipation Growth failure-failure to thrive Sunken fontanel with dehydration Children may have following symptoms: a) Excessive thirst and drinking b) Polyuria with nocturia and enuresis c) Pale, dry skin with reduced sweating Diagnostic Evaludtion Urine specific gravity, sodium and osmolatity are decreased Serum osmolality and sodium are elevated Serum measurement of ADH is low in conjuction with high plasma osmolality Water deprivation test (potentially dangerious) - Fluids are restricted, and the urinary volumes and concentrations are monitored hourly along with the child's weight - Test is terminated if child loses more than 3 per cent to 5 per cent of body weight. Serum sodium and osmolality are measured at completion of test and are high; urine osmolality remains lower 11< I I - Test is completed by giving the child a dose of ADH, which should Nursing Care of Children -th Medical and , ~ r g i c a I problems-II stop the abnormal diuresis. If it does not, child may have nephrogenic Dl (renal unresponsiveness). Assess MRI or CT scan of hypothalamic pituitary region High incidence of associated anterior pituitary disorders Treatment Daily replacement of ADH using desmopression (DDAVP) a synthetic analogue In children with cleft lip and palate, sublingual administration has been shown to be effective Thiazide diuretics in nephrogenic Dl - Complications Dehydration Hypernatremia I Nursing Assessment Assess hydration status and assess the appropriate intake of medicine and accurate dosage Assess children with complaints of polyuria and polydipsia and dehydration Obtain a detailed history of symptoms and behavic~ss- specific attention to changes in sleep patterns (may be caused by enuresis) and choices (drinking of fluid, fiom toilet bowls or dog dishes) Evaluate height and weight as weight boss may be caused by excessive drinking Y Nursing Diagnosis The child with Dl has, Deficit of fluid volume due to disease process Altered nutrition: low intake of food less than body requirhents due to fluid preference over food Disturbance in sleep pattern due to nocturia and enuresis Nursing Interventions Maintain fluid intake Assess for and teach Darents about the assessment of dehvdration - which Administer intravenous (N) fluid as ordered if acutely dehydrated Monitor intake and output and teach parents to maintain record of fluid intake and output in child. Reduced output may require restriction of fluids if overdosage of DDAVP is suspected Keep record of daily weight. Administer and teach proper administration of DDAVP. Proper management should eliminate symptoms Teach parents to provide free dccess to fluid (water) sources at all times. However, caution parents that child should be protected fiom water excess Calculate rough estimated total daily fluid requirements based on body size to assess fluid replacement versus excess: 100 mL/kg for first 10 kg of Maintaining Adequate Nutrition a Ensure that adequate feeding is given between plain water bottles a For older child, provide liquid nutritional supplements. a Emphasis on the the importance of providing nutritional requirements with fluids to ensure adequate calorie intake for growth a Help the parents to discuss with dietitian for dietary advise a Monitor length and weight and developmental milestones at regular intervals Nursing Care of Children with Endocrine and Metabolic Disorders I I Normalizing Sleep Pattern . a Ensure adequate evening administration of DDAVP to prevent nighttime water craving and enuresis a Suggest the use of diapers at night and plastic padding on bed to make bedwetting easier until optimum management of condition is attained a Encourage ea'sy access to fluids and toilet or commode for older child during night ~ a ' m iEducation l~ and Health Maintenance Teach to administer correct dosage in a correct way as follows a a Correct dose is measured and drawn up into catheter is inserted into patient's nostril then other end of cathetric is put ' Catheter in mouth and then it is blown generally a Older children inhale the solution a Explain the parents that nostrils should be as clear as possible before administration of dose a Explain the parents that, if dose is thought to be swallowed do not readminister due to potential overdosage. Split the dose into both nares if there is swallowing of drug a Tell family members to store drug away from heat and direct light and moisture (not in bathroom) a Advise parents that children should wear some identification object for DI a Advise the Parents that school personnel should be informed. about the condition and symptoms needing attention (water intoxication) a Advise routine follow-up; treatment may be temporary or life-long, depending on cause Outcome Based valuation There are a No signs of dehydration and intake equals to output a No weight loss, proper growth a Child sleeping through out night I I Diagnostic Evaluation Neonatal Screening consists of an initial filter paper blood spot thyroxine (T,) measurement followed by measurement of thyroid stimulating hormone (TSH) in specimens with low T4 values. Screening results that show a low level of T4 and a high level of TSH indicate presence of CH. Therapeutic Management Treatment involves lifelong thryroid hormone replacement therapy as soon as possible after diagnosis to subside all signs of hypothyroidism and reestablish normal physical and mental development. The drug of choice is synthetic levothyroxine sodium (Synthroid or Levothroid). Regular measurement of thyroxine levels is important in ensuring optimum treatment. Home based surveys are also performed to ensure optimum growth. Nursing Considerations The most important nursing objective is Ensure early identification and screening of the disorder Nurses need to be aware of the earliest signs of the disorder Be aware about the parental remarks about the child being an unusually "quite and good" baby coupled with any of the early physical manifestations which helps to suspect hypothyroidism a,ld refer the child for specific tests. Unfortunately, many parents harbor guilt about their impressions of the infant before the diagnosis because the child's inactivity may not have alerted them to a problem with the result that treatment is delayed but you should resure the parents. Explain necessity of lifelong treatment. The importance of compliance with the drug regimen must be emphasized for the child to achieve normal growth and development. Since the drug is tasteless, it can be crushed and added to milk feed, water, or food. If a dose is missed, twice the dose Check the signs of overdose, such as rapid pulse, dyspnea, irritability, insomnia, fever, sweating, and weight loss. Ideally they should know how to count the pulse and be instructed to withhold a dose and consult their doctor if the pulse rate is increased to a certain value. Signs of inadequate treatment are fatigue, sleepiness, decreased appetitite and constipation. Phenyle Ketoneuria It is a disorder of amino acid metabolism. It is either due to deficiency of the enzyme Phenylalaninehydroxylase or in the synthesis or recyling of the biopterm in cofactor for this enzyme. It presents with infantile spasm and developmental dealy at 6-12 months of age. There may be a musty odour due to the metabolitic phenylacetic acid. Some children may develop eczema and most of the children have blue eyes and jelly hair. The condition can be detected by raised level of phenylalanine levels in blood which is detected at 5-7 days when milk feeding has been established. Restriction of dietary phenylananine but you have to ensure that the restriction does not effect normal growth and development (both physical and neurological) of the child. The dietary management is done for at least 10 years. Educate the parents regarding the diseases and role of diet in the disease. Teach the family regarding dietary restrictions. Advise the family to give low Nursing Care o f Children with Endocrine and Metabolic Disorders Check Your Progress 1 1. i) Disorders of pituitary function ii) Disorders of thyroid function iii) Disorder of parathyroid function iv) Disorder of adrenal{unction v) Disorders of pancreatic hormone function Check Your Progress 2 1. i) Insulin dependent (IDDM) or type I iii Non-insulin-dependent (NIDDM), or type I1 iiii Maturity-onset diabetes of youth (MODY) 2. Major symptoms Increased thirst Increased urination, enuresis 1ncreas:d food ingestion Weight loss Fatigue Minor symptoms Skin infections Dry skin, poor wound healing Montial vaginitis in adolescent girls Check Your Progress 3 1. i) Administer IV fluids as ordered i i Monitor intake and output, blood pressure, serum electrolyte results and daily weight report any abnormality iiii Assess for signs of dehydration - dry skin and mucous membranes, constipation iv) Encourage to take oral fluids when the child is able to take orally 2. i) Arms ii) Thighs iiii Abdomen iv) Buttocks 3. Precomatose state: Drowsiness Dryness of skin Cherry red lips Increased respirations Nausea Vomiting Abdominal pain Comatose state: Extreme hyperpnea (kussmaul breathing) Acetone breath 121 • Soft, sunken eyeballs Nursing Care of Children wlth Medical and Surgical problems-11 • Rigid weak pulse Decreased temperature Decreased blood pressure Check Your Progress 4 1. Signs and Symptoms a Sudden onset of excessive thirst and polyuria a The infants may present with following symptoms: a Excessive crying - quieted with water more than milk feeding a Rapid weight loss - caloric loss due to water preference over feedings Constipation a Growth failure - failure to thrive a Sunken fontanel with dehydration Children may have following symptoms: a Excessive thirst and drinking Polyuria with nocturia and enuresis a Pale, dry skin with reduced sweating 2. Complications a Dehydration a Hypernatremia Check Your Progress 5 1. 9 ii) iii) iv) v) Congenital Hypothydoidism (CH) Phenyle Ketoneuria Galactosemia Hypoglycemia Hypocalcemia