Caring for children with endocrine dysfunction

Caring for children with endocrine dysfunction 2005/3/2 1 Course objectives • Describe the pediatric differences in the endocrine system • Describe the disorders of pituitary function; which includes growth hormone deficiency, gigantism, acromegaly, and precocious puberty • Describe the disorders of thyroid function • Discuss the signs and symptoms and the nursing care of diabetes mellitus 2 THE ENDOCRINE SYSTEM Three major components (p. 1703 ~ 06) 3 Cell • Sends a chemical message by means of hormone – Hormone • a complex chemical substance produced by one gland or tissue and secreted into body fluids by a cell or a group of cells that exerts a physiologic controlling effect on other cells (Table 38-1) 4 • Target cells • Environment – The chemical is transported form the site of synthesis to the sites of cellular action (end organs, 標的 細胞/器官) – Receive the chemical message (Fig. 38-2) 5 • Major hormones of the anterior pituitary – Prolactin 泌乳激素 – Growth hormone (GH) 生長激 素 – Adrenocorticotropic hormone (ACTH) 促腎上腺皮質素 – Thyroid-stimulating hormone (TSH) 甲狀腺刺激素 – Follicle-stimulating hormone (FSH) 濾泡刺激素 – Luteinizing hormone (LH) 黃 體素 • Posterior pituitary – Oxytocin (OT) 催產激素 – Antidiuretic hormone (ADH) 抗利尿激素 6 Summary of the endocrine system (Gland/hormone, Effect, Hypofunction, Hyperfunction) (Table 38-1, 1707 ~ 08) 7 Control of hormone secretion “Negative Feedback (負回饋機制)” • 每種內分泌腺體都有持續分泌的傾向, 一但 達到它的生理機能, 便有訊息經由某種方式 傳到該腺體, 阻止它作更進一步的分泌  即當體內標的器官內激素的濃度升高時, 會 導致腦下垂體激素的分泌減少 • Hypothalamic-pituitary axis – hypothalamic-pituitary-target cell 8 Pediatric differences in the endocrine system • Less developed at birth than any other body system • Hormonal control of many body function is lacking until age 12 ~ 18 months 9 Disorders of pituitary function 10 Growth hormone deficiency 生長激素缺乏 “short stature” (Dwarfism, 侏儒症) 11 Etiology (Box 38-2, p. 1706) • Hypopituitarism (腦下垂體功能低下) (p. 1706 – 1713) – Aplasia or hypoplasia 發育不良/不全 • Developmental defects 生長缺陷 • Idiopathic 自發性 – Destructive lesions • Trauma • Infiltrative lesions – tumors (craniopharyngiomas 顱咽管瘤) (most common) – – – – Irradiation Autoimmune hypophysitis (自體免疫之腦下垂體炎) Surgery Functional deficiency • Anorexia nervosa 厭食症 12 Definition • An absolute height < -2 SD for age • A linear growth velocity < -1 SD for age, consistently • ♂:♀= 3:1 • Usually normal at birth, growth patterns deviate from the normal growth rate beginning in infancy 13 Causes of short stature (Fig. 38-3, p. 1709) • Normal variant – Constitutional delay of growth and adolescence 體質構造性生長緩慢與青春期遲延 – Familial or genetic short stature 遺傳性身材矮小 • Pathologic – Proportionate 對稱 • Increased Wt/Ht (身高比體重有明顯的發展遲緩) – GH deficiency 14 Clinical manifestations (Box 38-3, p. 1706) • Short stature but proportional height and weight – Increased Wt/Ht (身高比體重有明顯的發展遲緩)  Overweight • Delayed epiphyseal plate (骨骺板, 軟骨板, 骨成長之處) closure • Retarded bone age proportional to height • Premature aging common in later life – Looks younger than their age • Eruption of the permanent teeth is delayed • Increased insulin sensitivity (easy to have hypoglycemia) • Emotional adjustment problems 15 Diagnostic evaluation • Family history – Parental height: mid-parental height • Child’s past health history • PE – Evaluating the growth curve (Box 38-4) • Psychological evaluation • Radiographic surveys – Ossification 骨化程度 (bone age 骨齡, 左手脕掌) • GH studies  stimulate the release GH – Clonidine Stimulation Test – Insulin Tolerance Test – L-Dopa Test 16 Therapeutic management • Correction of the underlying diseases • GH replacement therapy (皮下注射/口服舌下 吸收) – Successful rate: 80% – Growth velocity: 5cm/yr  10-12cm/yr in the first year  7-9cm/yr during the next several years 17 Nursing considerations • Identifying children with growth problems • Education for patient self-management – GH replacement • Administered at bedtime • Encouraging exercise 18 • Child & family support – Enhancing the child’s self-esteem, body image, & achievement of developmental tasks – Human Growth Foundation (http://www.hgfound.org/) – 中華民國兒童生長協會 (http://www.childgrowth.org.tw) 19 Pituitary Hyperpituitarism (腦下垂體機能亢進) (p. 1713 ~ ) • Gigantism (巨人症) – Hypersecretion of GH before the epiphseal closure • Acromegaly (肢端肥大症) – Hypersecretion of GH after epiphyseal closure 20 Clinical manifestations • Gigantism (巨人症) – 長骨 (四肢) 過度生長, 身高可能達 200cm 以 上 – 肌肉及內臟亦快速增長 • Acromegaly (肢端肥 大症) – 生長往橫的方向 – 常發生在成人 – 骨頭變大 – 關節疼痛 – 手腳肥大粗糙 21 • Diagnostic evaluation – Same as Growth hormone deficiency • Therapeutic management • Nursing considerations – Early identification of children with excessive growth rates – Child & family support – Information providing (diseases, treatment, & intervention) 22 Precocious puberty (性早熟) excessive gonadotropins (促性腺素分泌過多) p. 1714 ~ 15 • Definition – Manifestation of sexual development before age 9 in boys or age 8 in girls – Premature appearance of secondary sexual characteristics, advanced growth rate, & bone maturation 23 Etiology/incidence (Box 38-6, p. 1714) • Disorders of the following – The hypothalamic-pituitary-gonadal axis – The gonads 性腺 – An adrenal gland 腎上腺 • Girls > Boys 24 Classification (Box 38-6, p. 1714) • Central precocious puberty (CPP, 中樞性早熟) – Premature activation of the hypothalamic-pituitarygonadal axis – Activated by Gn-RH (gonadotropin-releasing hormone 性腺刺激素促泌素) – May be the result of congenital anomalies, infectious, or neoplastic to the CNS – 50% of children with precocious puberty • ♀>♂ • 95% idiopathin in girls • 90% organic problems in boys 25 • Peripheral precocious puberty (PPP, 週邊性 早熟) – no early secretion of Gn-RH – Other hormone stimulation • Adrenal gland 腎上腺 26 Clinical manifestations • Premature thelarche – Development of breasts in prepubertal females 乳房發育提早出現 • Premature pubarche (adrenarche) – Early development of sexual hair 出現陰毛、 腋毛 • Premature menarche – Isolated menses without other evidence of sexual development 出現月經 27 Diagnostic evaluation • History taking & PE • Blood tests – LH, FSH, testosterone, estrogen • Radiographic studies – Bone age, X-rays – CT & MRI 28 Therapeutic management • To stop or reverse the development of secondary sexual characteristics and to slow down the rapid growth rate and one age advancement – CPP [每月皮下注射類黃體化荷爾蒙釋放激素 (LH-RH)， 調節腦下垂體之分泌，期青春期之發育正常] • Treatment is discontinued at a chronologically appropriate time, allowing pubertal changes to resume 29 Nursing considerations • Psychologic support and guidance – Providing information to support home care – Monitoring G & D – Enhancing the child’s self-concept, body image, & promoting effective family coping 30 Disorders of thyroid function 31 Juvenile hypothyroidism (幼年型甲狀腺功能低下) • Etiology – A deficiency in secretion of TH – Aplasia or hypoplasia 發育不良/不全 – Dietary insufficiency of iodine (rare) 32 Clinical manifestations • Decelerated growth • Myxedematous skin changes (黏液水腫皮膚 變化) • Constipation, sleepiness, mental decline (aggressive behavioral reactions, learning problems, poor school achievement) • Mental retardation & neurological sequelae are not associated with it 33 • Diagnostic evaluation – Blood tests (甲狀腺刺激素促泌素試驗) • Therapeutic management – TH replacement (L-thyroxin) 34 Nursing considerations • Early recognition • Monitoring G & D • Providing information to support home care – Adherence & monitoring of response of therapy 35 Hyperthyroidism (甲狀腺機能亢進, Graves’ disease ) • Etiology – Autoimmune – Enlarged thyroid gland & Exophthalmos (眼球 突出) – 6 ~ 15 y/o, peak 12 ~ 14 y/o – 5 times higher in girls than boys – Familial association, high concordance incidence in twins 36 Clinical manifestations • Excess motion • Weight loss; appetite↑ • Muscle weakness • Hyperactivity of GI tract → diarrhea & vomiting • Cardiac manifestations • Skin warm, flushed, & moist; heat intolerance • Hair fine • Exophthalmos (眼球突出) – protruding eyeballs 37 Diagnostic evaluation • Blood tests – T3 & T4↑ – Thyrotropin (TSH) ↓ 38 Therapeutic management • Retarding the rate of hormone secretion – Antithyroid drugs – interfere with the biosynthesis of TH – Subtotal thyroidectomy 局部切除甲狀腺 – Ablation with radioiodine (131I-iodide) 39 • Thyrotoxicosis (甲狀腺毒症) – thyroid crisis / thyroid storm – Sudden release of the thyroid hormone – Acute onset of severe irritability, vomiting, diarrhea, hyperthermia, hypertension, & tachycardia → 昏迷、死亡 – Antithyroid drugs or ß-adrenergic blocking agents (propranolol) 40 Nursing considerations • Identifying of hyperthyroidism • Treating physical symptoms • Emotional support – Supporting a healthy body image • Providing information to support home care – Nutritional issues – metabolic rate  – Adherence • Surgical care – Monitoring for postoperative complications 41 Disorders of pancreatic hormone secretion (p. 1732 ~ 55) 42 Diabetes mellitus (DM) • Partial or complete deficiency of the hormone insulin • Incidence – Can occur at any age – Peak incidence 10 ~ 15 years of age – 75% diagnosed before 18 – Boys:Girls = 1:1 ~ 1.2:1 43 Classification & Etiology (ADA, 2001) • Type 1 (insulin-dependent diabetes mellitus, IDDM) – Predominant form in pediatrics – Autoimmune disease, the destruction of the insulin-secreting cells of the pancrease – Genetic factors – Diet – Viruses – Two forms 44 • Type 2 (non-insulin-dependent diabetes mellitus, NIDDM) – Insulin resistance • body fails to use insulin properly – Age over 45, overweight, family history – Type 2 in pediatrics 45 • Comparison of characteristics of • Pathophysiology (P. 1734 ~ 1735; Fig. Type 1 vs. Type 2 DM (Table 38-4, p. 1733) 38-6, p. 1735) 46 47 Long-term complication • Nephropathy • Retinopathy • Neuropathy • Thyroid dysfunction (hyperglycemia) • Small joint limited mobility (glycosylation 蛋白質醣化) 48 Clinical manifestations • Weight loss • Abdominal discomfort • 3 Ps – Polyuria – Polydipsia – Polyphagia • Hyperglycemia, Hypoglycemia, & Ketoacidosis (Table 38-5, p. 1740) 49 Diagnostic evaluation • 3Ps • Urine sugar (+) • Weight loss or failure to gain; appetite ↑ • Blood sugar (空腹 > 120 or 飯後 2hrs > 140) • Manifestations of metabolic acidosis, with or without stupor or coma • Glycosylated hemoglobin (糖化血色素, HbA1c) (↑) – in response to prolonged blood glucose elevated 50 Therapeutic management • Goals – Appropriate growth – Age-appropriate lifestyle – Near normal HbA1c (< 7%) – Absence of acute complications (hypoglycemia & hyperglycemia) 51 • Insulin therapy • Islet cell or whole pancreas transplantation (future therapies) • Monitoring – Blood sugar (80 ~ 120 mg/dl) – HbA1c – Urine • Nutrition (Box 38-13, p. 1739) • Exercise • Hypoglycemia • Illness management 52 Nursing considerations (p. 1742 ~ 55) • Acute care  hospital management • General care – Goals • Child & family will be educated about the disease, assessment, techniques, & therapy • Child will experience a minimum of ill effects from complications of diabetes • Child will develop a positive self-image • Child & family will receive adequate support • Developmental issues 53 Nursing implementation -- EDUCATION • Concepts of child and family education – Simple to complex – Good interpersonal skills – Teach about illness and regimen – Overcome obstacles to behavior change 54 • Identification – Medic-Alert identification • Insulin – Injection procedure – Continuous subcutaneous insulin infusion – Monitoring • • • • • Blood sugar HbA1c Urine Exercise Hypoglycemia • Hygiene • Exercise • Record keeping • Complications – Shopping – Travel 55 • Hyperglycemia – Illness – Growth – Emotional upset • Hypoglycemia – Caused by – Early signs – adrenergic – Second set of symptoms – neuroglycopenic (brain hypoglycemia) – Third set of symptoms – severe 56 • Self-management  Shared management – Delegating diabetes responsibilities (appendix IV) • Child and family support – 兒童與糖尿病 http://www.childrenwithdiabetes.com/index_cwd.htm – 榮總 http://www.vghtpe.gov.tw/~meta/iddm.htm – 國泰 http://www.cgh.org.tw/Endoclass.htm – 糖尿病相關網站 http://www.med.org.tw/mi97_1/hh1.htm – 康泰醫療教育基金會 – 糖尿病童服務組 http://www.kungtai.org.tw/aboutiddm.php 57 Nursing care plan • Nursing diagnosis – Hospital care • Risk for injury related to insulin deficiency • Risk for injury related to hypoglycemia – Preparation for home care • Knowledge deficit (diabetes management) related to care of a child with newly diagnosed diabetes mellitus • Altered family processes related to situational crisis (child with a chronic disorder) 58