→ A benign neoplasm is an abnormal but noncancerous growth that may occur in different parts of the body. → “Neoplasm” came from the Greek word “neo”, which means new, and “plasma”, which means formation or creation (abnormal growth of new tissue) NCM 109 – FINALS Care of Mother and Child at Risk or with Problems Learning Outcome: Describe usual cellular growth & theories. Assess a child with a common malignant process. Formulate nursing diagnosis related to a child with a malignancy. Establish expected outcomes for a child with a malignancy. Implement nursing care for a child with a malignancy. Evaluate expected outcomes for achievement and effectiveness of care. a) b) c) d) e) f) BENIGN slow growing capsulated noninvasive do not metastasize well-differentiated suffix “oma” (ex. fibroma) a) b) c) d) e) f) MALIGNANT fast growing non capsulated invasive & infiltrate metastasize poorly differentiated suffix “carcinoma” or “sarcoma” HYPERTROPHY CELLULAR ABERRATIONS → The cell is the body’s basic building block and the smallest living component of an organism → The cell faces many challenges through its life span. → If the cell’s reserves are insufficient, the cell dies. • • increase in cell size normal organization • • increase in cell number normal organization • disorganized growth • • disorganized growth net increase in number of dividing cells HYPERPLASIA CANCER → “Cancri” “Crab” → A disease of the cell in which the normal mechanisms of the control of growth & proliferation have been altered or there is an uncontrolled proliferation of cells. DYSPLASIA TERMINOLOGIES RELATED TO CANCER 1. Benign Neoplasm 2. Neoplasia 3. Hyperplasia 4. Hypertrophy 5. Metaplasia 6. Dysplasia 7. Anaplasia 8. Metastases 9. Oncology 10. Adenocarcinoma 11. Carcinoma 12. Sarcoma 13. Carcinogens NEOPLASIA Metaplasia • initial changes of normal cells to a different cell type • replacement of one adult cell type by a different adult cell type • noncancerous while dysplasia can be cancerous 1 Carcinoma • a cancer arising in the epithelial tissue of the skin or of the lining of the internal organs Adenocarcinoma • a malignant tumor formed from glandular structures in epithelial tissue ADENOMA develop in glands which secrete fluids • benign • can affect people at any age • CARCINOMA develops in tissues that line the inner or outer surfaces of the body • malignant • rarely affect children • Soft Tissue Sarcomas: Definition • • Sarcomas are malignant tumors that arise from skeletal and extraskeletal connective tissues (mecells) . Including: o Adipose tissue o Bone o Cartilage o Smooth muscle o Skeletal muscle PATHOGENESIS OF CANCER a) Cellular Transformation & Derangement Theory − normal cells may be transformed into cancer cells due to exposure to some etiologic agents. b) Failure of the Immune Response Theory − Cancer cells are recognized by the immune response system. So, the cancer cells undergo destruction. − Failure of the immune response system leads to inability to destroy the cancer cells. Etiologic Factors: 1. Viruses − Prolonged or frequent viral infections may cause breakdown of the immune system or overwhelm the immune system. 2. Chemical Carcinogens − Causes cell mutation or alteration in cell enzymes and proteins causing altered cell replication. − eg. Industrial compounds; drugs , food preservatives 3. Physical Agents − Radiation. from x rays; from sunlight/ultraviolet rays. − Physical irritation/trauma from pipe smoking, multiple deliveries, jagged tooth, irritation of the tongue, “overuse” of any organ/body part. 4. Hormones − Hormone Therapy 5. Genetics − A family history of certain cancers can be a sign of a possible inherited cancer syndrome. − When oncogene(hidden or repressed genetic code of cancer that exists in all Individuals) is exposed to carcinogens, changes in cell structure occurs, malignant tumor develops. Predisposing Factors: 1. Sex 2. Urban Residence 3. Geographic Distribution 4. Occupation 5. Heredity 6. Stress 7. Precancerous Lesions 8. Obesity Causes of Neoplastic Growth − − Exact origin is unknown. cancerous tumor growth is triggered by DNA mutations within the cells. Pathophysiology of Cancer 1. Genetic mutation. The disease process of cancer starts when the genetic mutation in the cellular DNA transforms an abnormal cell. 2. Neoplasia or proliferation. The abnormal cells grow uncontrollably without following any physiologic demand. Cancer cells also do not appear the same as normal cells and are termed "undifferentiated". They tend to increase in number and size at the periphery while attempting to infiltrate the body tissues nearby in order to destroy them. 3. Metastasis. Malignant cells invade other parts of the body by means of using blood and lymphatic channels. They can also "metastasize" or spread to a distant body part or region. a) − − Cell Injury Injury to components of cells can lead to disease as the cells lose their ability to adapt. Cell injury may result from any of several intrinsic or extrinsic causes and may be classified as: 2 • • • • Toxic injury (endogenous & exogenous factors) Infectious injury physical injury (2 major types: thermal & mechanical) deficit injury WARNING SIGNS Change in bowel/ bladder habits A Sore that does not heal Unusual bleeding / discharges. Unexplained sudden weight loss. Unexplained anemia Thickening/lump in the breast/elsewhere Indigestion or difficulty in swallowing Obvious change in wart or mole. Nagging cough/ hoarseness of voice. Signs & symptoms of childhood cancer are nonspecific and include many findings observed in a variety of childhood disorders. These include: a. Fever b. Musculoskeletal symptoms c. Pain, fatigue d. Pallor e. Bruising f. Bleeding g. Headache h. Lymphadenopathy & loss of appetite i. Vomiting & weight loss. Cancer Detection Examination 1. − 2. − 3. − 4. − Cancer screening screening exams are useful in diagnosing cancer early to give the patient the opportunity to be treated as soon as possible. Physical exam the doctor will find lumps, skin changes, and other signs and symptoms related to cancer Imaging tests X-rays, CT scans, PET scans, ultrasounds, and other imaging exams can be used to examine the body in a non-invasive method; imaging tests are useful in knowing the extent of cancer, also known as cancer staging Biopsy the collection of cell samples and the subsequent examination of these cells under a microscope; this can be a definitive diagnostic tool to determine if cancer cells exist Staging of Cancer Three factors: − size and growth of the tumor whether cancer has spread to the lymph nodes whether it has spread to other parts of the body. The TNM Staging System − The TNM system is the most widely used cancer staging system. Most hospitals and medical centers use the TNM system as their main method for cancer reporting. You are likely to see your cancer described by this staging system in your pathology report unless there is a different staging system for your type of cancer. Examples of cancers with different staging systems include brain and spinal cord tumors and blood cancers. In the TNM system − The T refers to the size and extent of the main tumor. The main tumor is usually called the primary tumor. − The N refers to the number of nearby lymph nodes that have cancer. − The M refers to whether the cancer has metastasized. This means that the cancer has spread from the primary tumor to other parts of the body. 3 Cancer Treatment Measures Used Treatment Modalities for Cancer 1) Immunotherapy − a type of cancer treatment that helps your immune system fight cancer. − The immune system helps your body fight infections and other diseases. It is made up of white blood cells and organs and tissues of the lymph system. − a type of biological therapy 2) stem cell transplant − A procedure in which a patient receives healthy stem cells (bloodforming cells) to replace their own stem cells that have been destroyed by treatment with radiation or high doses of chemo How is childhood Cancer different from Adult cancer? − − Therapy for a child w/ cancer focuses on devising ways to kill the growth of abnormal cells while protecting the normal surrounding cells. Treatment may include any one or a combination of cancer treatment 4 − − Children are different from adults in terms of body structure and function, social, emotional and cognitive aspects. − Tumors may grow because normal cell growth has been altered by environmental exposure such as chronic exposure to chemical irritants or cigarette smoke. ADULTS: CHILDREN: − Tumors most frequently occurs in organs unexposed to the environment such as • leukemia of the bone marrow • nephroblastoma of the kidney (Wilm tumor) • tumors of the brain; or • neuroblastoma in the abdomen. CHILDHOOD CANCER The Problem CHILDHOOD CANCER Key Facts: − Each year, approximately 400 000 children & adolescents of 0-19 years old are diagnosed with cancer. − The most common types of childhood cancers include leukemias, brain cancers, lymphomas and solid tumors , such as neuroblastoma and Wilms tumors. − Cancer is a leading cause of death for children and adolescents, particularly in high income countries. − The likelihood of surviving a diagnosis of childhood cancer depends on the country in which the child lives: in high-income countries, more than 80% of children with cancer are cured, but in many LMICs only 15-45% are cured. Key Facts: high-income countries > more than 80% of children with cancer are cured. In low- and middle-income countries (LMICs), an estimated 15-45% are cured. Childhood cancer cannot generally be prevented or identified through screening. cancers can be cured with generic medicines and other forms of treatment, including surgery and radiotherapy. Treatment: can be costeffective in all 9income settings. The problem … The reasons for lower survival rates in LMICs include: a. delay in diagnosis and advanced disease b. inability to obtain an accurate diagnosis c. inaccessible therapy d. abandonment of treatment e. death from toxicity (side effects) f. avoidable relapse. Childhood cancer data systems are needed to drive continuous improvements in the quality of care, and to inform policy decisions. The Problem − Improving access to childhood cancer care, including to essential medicines and technologies, is highly cost effective, feasible and can improve survival in all settings What Causes CANCER in children? 1. HIV 2. Epstein-Barr virus 3. Malaria − approximately 10% of all children with cancer have a predisposition because of genetic factors. 5 Improving outcomes of childhood cancer − Prompt, correct diagnosis − Effective, evidence-based therapy with tailored supportive care. Treatment − Chemotherapy, surgery and/or radiotherapy. − Physical, cognitive growth and nutritional status. − Effective diagnosis, essential medicines, pathology, blood products, radiation therapy, technology. − Psychosocial and supportive care. Most Common Cancer in Children 1. Leukemia 2. Brain and spinal cord tumors 3. Neuroblastoma 4. Wilms tumor 5. Lymphoma (including both 6. Hodgkin and non-Hodgkin) 7. Rhabdomyosarcoma 8. Retinoblastoma 9. Bone cancer (including osteosarcoma and Ewing sarcoma) − − − − − 1. LEUKEMIA is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells called "blasts”. Is the distorted and uncontrolled proliferation of WBCs(leukocytes) Classifications: a. Acute Lymphocytic (Lymphoblastic) Leukemia (ALL) → is a type of cancer of the blood and bone marrow — the spongy tissue inside bones where blood cells are made. − → the disease progresses rapidly and creates immature blood cells, rather than mature ones. → Account 75% of leukemias and involve lymphoblasts (immature lymphocytes). → Rapid proliferation of so many immature lymphocytes causes decrease production of RBC and Platelets. Symptoms: • nose bleeds • weight loss • swollen lymph nodes • fever • night sweats • bleeding easily • bone pain • red spots on skin Assessment: 1) Pallor 2) Low-grade fever 3) Lethargy 4) A low thrombocyte count 5) Spleen and liver begin to enlarge, abdominal pain, vomiting & anorexia occur. 6) Bone & joint pain 7) Headache or unsteady gait 8) RBCs are of normal size and color but few in number 9) X-rays of long bones may reveal lesions 10) Lumbar puncture – shows evidence of blast cells in the CSF Therapeutic Management: Up to 95 % of children with ALL will achieve a first remission. If a child experiences relapse, the chances of long term survival are reduced & bone marrow transplantation may be required to achieve long term survival. Chemotherapy 6 Radiation Therapy Biological Therapy Stem Cell Transplant − − 2. LYMPHOMA b. Acute Myeloid Leukemia (AML) → characterized by the rapid growth (fast-growing) of abnormal cells that build up in the bone marrow & blood & interfere with normal blood cells. Symptoms: • shortness of breath • easy bruising • fever • weakness • pale skin • infections Therapeutic Management: bone marrow aspiration and biopsy − − − − − − − Nursing Diagnosis: o Risk for infection r/t non -functioning WBCs and immunosuppressive effects of therapy o RISK for deficient fluid volume r/t increased chance of hemorrhage from poor platelet production o PAIN r/t invasion of leukocytes o Ineffective health maintenance r/t long-term therapy for leukemia − The disease can spread to nearby lymph nodes. Later it may spread to the lungs, liver, or bone marrow. Etiology is unknown but both genetic and environmental factors both play a part. Many researchers have suspected an infectious component (viral). The first sign is often an enlarged lymph node. Symptoms: • Fever • Swelling of the face and neck • Lump in your neck, armpits, or groin • Excessive sweating at night • Unexpected weight loss • Loss of appetite • Feeling of weakness • Breathlessness • Itchiness Classifications: a. Hodgkin Lymphoma → More often localized to a single axial group of nodes (cervical, mediastinal, paraaortic) → Spreads by contiguity → Mesenteric nodes and Waldeyer’s ring rarely involved → Extranodal involvement uncommon 7 − − − b. Non-Hodgkin Lymphoma → More frequent involvement of multiple peripheral nodes → Noncontagious spread → Waldeyer’s ring and mesenteric nodes commonly involved → Extranodal involvement common c. Burkitt’s Lymphoma → 30-50 day incubation → Most cases asymptomatic → Associated with chronic coinfections with malaria → Nasopharyngeal carcinoma in Chinese & African men Causes: o Unknown o It occurs when the body makes too many abnormal lymphocytes, continue to grow and divide. o Enlarges the lymph nodes. Who Is at Risk? o older age o the use of immunosuppressant drugs o an infection (HIV, Epstein-Barr virus, or Helicobacter pylori ) o exposure to certain chemicals Can be detected using Kidney Function Test − Primary Nursing Diagnosis: o Risk for infection related to impaired primary and secondary defenses 3. Neoplasm of the Brain TYPES 1. CEREBELLAR ASTROCYTOMAS → Slow-growing, cystic tumors that arise from glial or supporting tissue surrounding neural cells 2. MEDULLOBLASTOMAS → Fast-growing tumor found most commonly in the cerebellum → Causes 4th ventricle compression and disturbances in the flow of CSF − − − Confirmation Test 1) Bone marrow analysis 2) Liver function test 3) Chest and Abd’l CT SCAN/MRI 4) Lymphangiography (detects size and location of deep nodes involved) 5) 5. Abd’l biopsy Diagnostic Tests: Therapeutic Management: Chemotherapy Radiation A stem cell transplant Medications 3. BRAINSTEM GLIOMAS → A cancerous glioma tumor in the brainstem → Start in the brain or spinal cord tissue and typically spread throughout the nervous system. 8 • • • Form a preventive strategy for pain management around the clock Educate parents and child about analgesics Assist parents to formulate activities that will not trigger or heighten headache pain. 4. OTHER CHILDHOOD NEOPLASM a. Neuroblastoma → Tumors that arise from the cells of the sympathetic nervous system − − − − Diagnostic Tests • Neurologic exam • CT • MRI • Angiogram • Spinal tap • Biopsy Management • Surgery • Radiation therapy • Chemotherapy Nursing Diagnosis • Acute pain • Anxiety • Risk for injury • Fear r/t diagnosis of brain tumor Assessment/Nsg. Mgt. • PAIN, Assess the severity and duration of a headache • Use a pain assessment tool appropriate for age and developmental level to determine the pain intensity • Administer analgesic as prescribed • Instruct the child to refrain from sneezing, coughing, or straining, during defection • Apply a cool compress on the head for low to moderate pain • Provide toys, games for quiet play − Symptoms − b. Rhabdomyosarcoma → A tumor of straited muscle → arises from the embryonic mesenchyme tissue the form muscle, connective, and vascular tissue Symptoms c. Nephroblastoma (Wilms Tumor) → A malignant tumor that arises from the metanephric mesoderm cells of the upper pole of the kidney 9 → Commonly seen in kids from ages 3-4 and tends to occur less frequently after the age of 5 → Most common kidney cancer in children − − − − Symptoms • swelling in the abdomen • pain in the abdomen • mass in the abdomen which can be felt • fever • hematuria or blood in the urine Birth Defects & Risk Factors of Wilms Tumor • Aniridia • Cryptorchidism • Hypospadias Assessment • Tumor is felt as firm, nontender abdominal mass • Child’s abdomen should not be palpated –may ais to metastasis. Place a sign reading “No Abdominal Palpation” over the child’s crib. Therapeutic Management Tumor will be removed by nephrectomy Followed by radiation therapy Chemotherapy –given for as long as 15 months Second surgical procedure may be scheduled after 2 or 3 months to remove any remaining tumor − Complications nephritis, small bowel obstruction, hepatic damage caused by fibrotic scarring from radiation can occur sterility in girls –radiation related damage to the ovaries radiation to the lungs –interstitial pneumonia spine radiation-scoliosis d. Retinoblastoma → An eye cancer that begins in the retina –the sensitive lining on the inside of your eye − Symptoms • A pupil that looks white or red, instead of the normal black • A crossed eye, which is an eye looking either toward the ear or toward the nose. • Poor vision. • A red, painful-looking eve • An enlarged pupil. • Different-colored irises − − − Diagnostic Tests 1. MRI 2. CT scan 3. Ultrasound (sonogram) 4. PET scan 5. X-ray 6. Biopsy Management a. Surgery b. Radiation c. Chemotherapy Nursing Interventions Protect the skin receiving radiation Protect oral and gastro-intestinal tract mucous membranes For diarrhea, switch to low-residue diet and administer anti-diarrheas as ordered Teach patient about risk infection 10 TOPIC 2 ALTERATION IN NUTRITION I. Objectives: Identify factors influencing nutrition. Discuss essential components and purposes of nutritional assessment and nutritional screening. Present nursing interventions to promote optimal nutrition and to treat clients with nutritional problems. Plan, implement ,and evaluate nursing care associated with nursing diagnoses related to nutritional problems. Nutrition → the sum of all the interactions between an organism and the food it consume. Nutrients → are organic and inorganic substance s found in foods that are required for body functioning. Factors Affecting Nutrition 1. Development 2. Gender 3. Ethnicity and Culture 4. Beliefs about Food 5. Personal Preferences 6. Religious Practices 7. Lifestyle 8. Economics 9. Medications and therapy 10. Health 11. Alcohol Consumption 12. Advertising 13. Psychological factors Daily Food Guide → Both inadequate and excessive intakes of nutrients result in malnutrition. → Malnutrition is commonly defined as the lack of necessary or appropriate food substances, but in practice includes both undernutrition and overnutrition. Overnutrition → a caloric intake in excess of daily energy requirements, resulting in storage of energy in the form of adipose tissue. National Heart, Lung, and Blood Institute → overweight = BMI is between 25 & 29.9 kg/m2 and obese when the BMI is >30 kg/m2. II. Undernutrition → intake of nutrients insufficient to meet daily energy requirements because of inadequate food intake or improper digestion and absorption of food. Inadequate food intake may be caused by: inability to acquire and prepare food inadequate knowledge about essential nutrients and a balanced diet discomfort during or after eating dysphagia anorexia nausea vomiting Improper digestion and absorption of nutrients may be caused by: an inadequate production of hormones or enzymes medical conditions Inadequate nutrition can be associated with: marked weight loss generalized weakness altered functional abilities delayed wound healing increased susceptibility to infection decreased immunocompetence impaired pulmonary function prolonged length of hospitalization. III. Protein-calorie malnutrition (PCM) significant problem of clients with long-term deficiencies in caloric intake − Characteristics 1) depressed visceral proteins (e.g., albumin) 2) weight loss 3) visible muscle and fat wasting. 11 − − − − − Nursing Management → A comprehensive nutritional assessment. • Components: Anthropometric, Biochemical, Clinical, Dietary Nutritional Screening → an assessment performed to identify clients at risk for malnutrition or those who are malnourished. Nursing History 1) Age, sex, and activity level 2) Difficulty eating 3) Condition of the mouth, teeth, and presence of dentures 4) Changes in appetite 5) Changes in weight 6) Physical disabilities that affect purchasing , preparing, and eating 7) Cultural and religious beliefs that affect food choices 8) Living arrangements (e.g., living alone) and economic status 9) General health status and medical condition 10) Medication history. Diagnosis a) Imbalanced Nutrition: Less Than Body Requirements b) Obesity c) Overweight d) Readiness for Enhanced Nutrition e) Activity Intolerance related to inadequate intake of iron-rich foods resulting in iron deficiency anemia f) Constipation related to inadequate fluid intake and fiber intake g) Chronic Low Self-Esteem related to obesity h) Risk for Infection related to immunosuppression secondary to insufficient protein intake. Planning → Maintain or restore optimal nutritional status. → Promote healthy nutritional practices. → Prevent complications associated with malnutrition. → Decrease weight. → Regain specified weight. ALTERATION IN GASTROINTESTINAL SYSTEM Learning Objectives At the end of the lecture discussion, students will be able to: Reviewed the anatomy and physiology of Gl system. Assess child with altered gastrointestinal function. Describe common gastrointestinal disorders seen in children. Utilize nursing process in the care of children with gastrointestinal disorder. 12 − − − − − Disorders Caused by Food, Vitamin,& Mineral Deficiencies 1) Kwashiorkor 2) Nutritional Marasmus 3) Vitamins & Mineral Deficiencies Gastro Intestinal Tract Disorder (Nursing Process) → Assessment assessed for signs of fluid loss compare the child's current weight with past weight measurements Refer → Nursing Diagnosis 1) Impaired parenting related to interference with establishing the parent-infant bond. 2) Interrupted family processes related to a chronic illness in child. 3) Risk for deficient fluid volume related to chronic diarrhea. 4) Imbalanced nutrition, less than body requirements, related to malabsorption of necessary nutrients 5) Situational low self-esteem related to feelings of being different resulting from special dietary restrictions Outcome Identification and Planning → Include the person who prepares or supervises the child's nutrition when helping plan a new nutritional pattern for a child. → If feedings will be given by nasogastric or gastrostomy tube, parents need enough practice to be proficient with the equipment and the technique before they are given the responsibility of doing it alone at home. Implementation → Parents need a great deal of support to adapt their busy life to these alternative methods of feeding or care. → Be certain to give clear, simple explanations and praise both parents and child after they demonstrate these procedures Outcome Evaluation → Recording children's height and weight. → making certain children gradually learn more about their specific nutritional measures. Common Gastrointestinal Symptoms of Illness in Children 1) Vomiting 2) Diarrhea (Mild, Severe) 3) Bacterial Infectious Diseases that Cause Diarrhea & Vomiting 4) Protozoan or Viral Diarrhea I. VOMITING → Or throwing up is a forceful discharge of stomach content → Recurrent vomiting may be caused by underlying medical conditions → Is Vomiting Harmful? Some examples of serious conditions that may result in nausea or vomiting include: Concussions Meningitis Intestinal blockage Appendicitis Brain tumors Dehydration → A concussion is a traumatic brain injury that affects your brain function. → Effects are usually temporary but can include headaches and problems with concentration, memory, balance and coordination. → Concussions are usually caused by a blow to the head → Assessment: Differentiation Between Regurgitation & Vomiting • Vomiting is the ejection of contents of the stomach and upper intestine • Regurgitation is the ejection of small amounts of chyme or gastric juice from the mouth and antecedent nausea. 13 → Differentiation between normal stool and diarrheal stool in an infant → When to seek immediate medical care? • blood in the vomit (bright red or "coffee grounds" in appearance) • Severe headache or stiff neck • Lethargy, confusion, or a decreased alertness • Severe abdominal pain • Diarrhea • Rapid breathing or pulse → Therapeutic Management II. DIARRHEA → When stools (bowel movements) are loose and watery. → Mild → Severe → Causes • Bacterial infection. • Viral infection. • Trouble digesting certain things (food intolerance) • An immune system response to certain foods (food allergy). • Parasites that enter the body through food or water • Reaction to medicines. • An intestinal disease, such as inflammatory bowel disease. → Diarrhea may be either: a. Short-term (acute) • Diarrhea that lasts 1 or 2 days and goes away. • may be caused by food or water that was contaminated by bacteria (bacterial infection). Or it may happen if your child gets sick from a virus. b. Long-term (chronic) • Diarrhea that lasts for a few weeks. • May be caused by another health problem such as irritable bowel syndrome. Can also be caused by an Intestinal disease. (ulcerative colitis, Crohn's disease, or celiac disease) → Symptoms: Cramping Belly (abdominal) pain Swelling (bloating) Upset stomach Urgent need to use the bathroom Fever Bloody stools Loss of body fluid Incontinence → Diagnostic Test Stool evaluation Blood tests Imaging tests Stool culture Sigmoidoscopy 14 → Sample Nursing Sample Nursing Diagnoses and Related Interventions a. Nursing Diagnosis: • Deficient fluid volume related to loss of fluid through diarrhea. b. Related Interventions: • Promote Hydration and Comfort. • Record Fluid Intake and Output. I. GASTROESOPHAGEAL REFLUX DISEASE (GERD) → A digestive disorder that affects the ring of muscle between the esophagus and stomach. → This ring is called the lower esophageal sphincter (LES). → GERD occurs when stomach acid frequently flows back into the tube connecting the mouth and stomach (esophagus). → This backwash (acid reflux) can irritate the lining of the esophagus. III. BACTERIAL INFECTIOUS DISEASES THAT CAUSE DIARRHEA & VOMITING a. Salmonellosis b. Listeriosis c. Shigellosis (Dysentery) d. Staphylococcal Food Poisoning IV. PROTOZOAN OR VIRAL DIARRHEA Common Disorder of the Stomach and Duodenum 1) Gastroesophageal Reflux Disease 2) Pyloric Stenosis 3) Peptic Ulcer Disease 4) Hepatic Disorders: → Hepatitis a. Нера А, B, C, D, E b. Acute Hepatis c. Chronic Hepatitis d. Fulminant Hepatic Failure 5) Obstruction Of The Bile Ducts 6) Nonalcoholic Fatty Liver Disease & Cirrhosis a. Esophageal Varices 7) Liver Transplantation → (stomach acid) Hydrochloric acid in the gastric juice breaks down the food and the digestive enzymes split up the proteins. → Diagnostic workup may include the following: a) Upper Gl series b) pH probe c) Esophageal manometry d) Endoscopy 15 − − − − − Therapeutic Management conservative treatment medication surgery In infants, it is treated by feeding a thickened formula and keeping the infant upright after feedings. • Adolescents are prescribed a proton pump inhibitor and advised to sleep with two pillows. → avoid lying down until 3 hours after a meal → sleep at night with their upper body elevated on a foam wedge or extra pillow. avoid acidic foods → Avoiding foods that delay gastric emptying such as fatty foods, chocolate, or alcohol losing some weight if overweight → avoid bending over after meals → Remove tight belts Risk factors • cerebral palsy • Down syndrome cystic fibrosis • Obesity • Typical symptoms heartburn that occurs 30 to 60 minutes after a meal and regurgitation. Common symptoms of GERD include 1. Burping or belching 2. Not eating 3. Having stomach pain 4. Being fussy around mealtimes 5. Vomiting often 6. Having hiccups 7. Gagging 8. Choking 9. Coughing often 10. Having coughing fits at night Therapy: → In many cases, GERD can be eased by diet and lifestyle changes. → Sometimes medicines, tube feedings, or surgery may be needed. For babies: → After feedings, hold the baby in an upright position for 30 minutes. → If bottle-feeding, keep the nipple filled with milk. This way the baby won't swallow too much air while eating. Try different nipples. Find one that lets the baby's mouth make a good seal with the nipple during feeding. II. PYLORIC STENOSIS → an uncommon condition in infants that blocks food from entering the small intestine. → Thickening and abnormal enlargement of the pylorus muscles, blocking food from reaching the small intestine. − − − → Pyloric stenosis can lead to forceful vomiting, dehydration and weight loss. → Babies with pyloric stenosis may seem to be hungry all the time. Surgery cures pyloric stenosis. → Cause: • Unknown, but genetic and environmental factors might play a role Risk factors • Sex • Race • Premature birth • Family history • Smoking during pregnancy • Early antibiotic use • Bottle-feeding → Infantile hypertrophic pyloric stenosis is 5 times more common in male infants Complications: 1. Failure to grow and 2. develop 3. Dehydration 4. Stomach irritation 5. Jaundice When to see a doctor Projectile vomits after feeding Seems less active or unusually irritable Urinates much less frequently or has noticeably fewer bowel movements • Isn't gaining weight or is losing weight 16 − − − III. PEPTIC ULCER DISEASE → a shallow excavation formed in the mucosal wall of the stomach, the pylorus, or the duodenum. → Includes gastritis (irritation of the lining of the stomach or duodenum) − Cause: Helicobacter Pylori infection NSAIDs (Diclofenac, Indomethacin, Ibuprofen) Alcohol, Caffeine Common Signs of a Stomach Ulcer: A dull burning or gnawing ache in the stomach Gas and bloating Nausea or vomiting Loss of appetite Fatigue Symptoms: Burning Fatigue Heartburn Loss of appetite Vomiting Nausea Bloating Burping Weight loss Dull pain Diagnostic → History • Epigastric abdominal pain, postprandial • For patients <60 without alarm features, in regions with I. • Pylori prevalence > 20% • Other patients, patients 260, patients with alarm features → Test and treat for H. Pylori → Endoscopy with ulcer biopsy 17 − − − Drug Therapy → Antacids • Used as adjunct therapy for peptic ulcer disease • ↑ gastric pH by neutralizing acid → Anticholinergic drugs • Occasionally ordered for treatment • ↓ cholinergic stimulation of HCI acid Management The objectives of management are to: relieve pain and discomfort accelerate healing prevent recurrence and complications Treatment If the ulcer is related to H. pylori, the doctor will prescribe a combination of drugs to eradicate the infection and normalize gastric acid levels so that the stomach can heal. Diet > foods that are easily digested and place little stress on the stomach → Vaccines to hepatitis A and B are available and are routinely given to children. IV. HEPATITIS → "Hepatitis" means inflammation of the liver → Can be caused by: a) Genetic diseases b) Medications (including overthe-counter) c) Alcohol → Hepatitis viruses (A,B,C, D, E) − Signs and Symptoms of HCV • When patients develop symptomatic acute HCV infection, they most often present with dark urine and light colored stools, followed by jaundice in which the skin and whites of the eyes appear yellow • Itching of the skin may be present. • On average, symptoms appear 6 to 7 weeks after infection 18 − − Chronic Hepatitis → Chronic hepatitis is defined as continuing or relapsing hepatic disease for more than 6 months, with presence of symptoms and other diagnostic parametersserology, biochemical and histopathology → Fulminant hepatic failure is a clinical syndrome resulting from massive necrosis of hepatocytes or from severe functional impairment of hepatocytes → Synthetic, excretory, and detoxifying functions of the liver are all severely impaired. Patterns of Hepatitis: 1) Acute hepatitis: if its manifestation persist for period less than six months 2) Chronic hepatitis: if there is clinical or serological evidence of liver pathology persistent for more than six consequent months. 3) Fulminant hepatitis: if massive hepatic cell necrosis happened within few weeks leading to acute hepatic failure and hepatic encephalopathy 4) Carrier state: is an individual who harbor and can transmit the virus but has no manifest symptoms. → the blockage of any duct that carries bile from the liver to the gallbladder or from the gallbladder to the small intestine → this can occur at various levels within the biliary system. → the major signs and symptoms of biliary obstruction result directly from the failure of bile to reach its proper destination. − Possible causes of a blocked bile duct include: • Cysts of the common bile duct. • Enlarged lymph nodes in the portal hepatis V. OBSTRUCTION OF THE BILE DUCTS → A biliary obstruction is a blockage of the bile ducts 19 VII. LIVER TRANSPLANTATION VI. Non Alcoholic Fatty Liver Disorder (Esophageal Varices) → abnormally enlarged veins in the lower part of esophageal tube − − − The main causes of portal hypertension in children: a. portal vein thrombosis or cirrhosis Treatment options: • Endoscopic • Radiographic • surgical strategies − − − Cirrhosis → General Signs associated with liver disease: 1. Jaundice 2. Clubbing 3. Palmar erythema 4. Spider nevi 5. Hepatomegaly 6. Gynaecomastia 7. Testicular atrophy 8. Caput medusae Preoperative Care • nursing history and physical examination. • Provide routine preoperative care. • Discuss preoperative and postoperative expectations with the client and family • Once a donor liver is located, check for evidence of infection; if no infection is present. begin preoperative antibiotics as ordered. Postoperative Care • Routine postoperative care • Maintain airway and ventilatory support until awake and alert. • Frequently monitor hemodynamic pressures, including arterial BP, central venous pressure, and pulmonary artery pressures. Monitor for signs of active bleeding. Provide discharge teaching: a. Teach how to reduce risk of infection, and signs of infection to report. b. Instruct to recognize and report signs of organ rejection. c. Discuss all medications. d. Discuss possible changes in body image and psychologic responses to receiving a transplanted organ. Refer to a counselor or support group as indicated. e. Refer for home health services for continued assessment and teaching. f. Stress importance of continued follow-up with transplant team and primary care provider. 20 Intestinal Disorders 1) Intussusception 2) Volvulus with malrotation 3) Necrotizing enterocolitis 4) Short Bowel/Short -Gut Syndrome 5) Appendicitis a. Ruptured Appendicitis 6) Meckel's Diverticulum 7) Celiac Disease (Malabsorption syndrome, Gluten-Induced Enteropathy, Celiac Sprue) I. INTUSSUSCEPTION → Intussusception of the bowel is defined as the telescoping of a proximal segment of the gastrointestinal tract within the lumen of the adjacent segment II. VOLVULUS WITH MALROTATION → Malrotation occurs when the intestine does not make the turns as it should. → Volvulus is a problem that can occur after birth as a result of intestinal malrotation. − Etiology: 21 − − Ladd Procedure Types: 1. Midgut Volvulus 2. Cecal Volvulus 3. Sigmoid Volvuluis − Midgut volvulus Malrotation − Symptoms: • Vomiting bile †greenish-yellow digestive fluid) • Drawing up the legs • Pain in the abdomen (belly) • Abdominal distention (swelling) • Rapid heart rate • Rapid breathing • Bloody stools • Malnutrition • Slowed growth Diagnostic Test (Adbml UTZ) − III. NECROTIZING ENTERO COLITIS (NEC) → is a devastating disease that affects mostly the intestine of premature infants. − − Symptoms: STAGE 1 suspected NEC bloody stools diminished activity (lethargy) slow heart rate an unstable temperature mild abdominal bloating vomiting. Symptoms: STAGE 2 definite NEC symptoms of stage 1 slightly reduced blood platelet levels slight excess of lactic acid no bowel sounds pain when the abdomen is touched reduced or no intestinal movement growth of gas-filled spaces in the walls of the intestine. 22 − − Treatment: a) Primarily supportive care. Enteral feeds, gastric decompression with intermittent suction fluid repletion to correct electrolyte abnormalities, parenteral nutrition and prompt antibiotic therapy. b) Monitoring is clinical. Abdominal roentgenograms should be performed every 6 hours. c) Bowel perforation. Emergency surgery to resect the dead bowel. Colostomy - which may be able to be reserved at a later time Short bowel syndrome malabsorption caused by the surgical removal of small intestines. Management, Surgical → Early Surgical Consultation → Indications for surgery: Perforation: 20-30 % of cases 12-48 hrs after onset Full-thickness necrosis Deterioration despite aggressive medical treatment V. APPENDICITIS → where the appendix becomes swollen, inflamed, and filled with pus. 1) McBurney’s Point: The junction of the lateral and middle third of the line joining the right anterior superior iliac spine and the umbilicus. typically, this area is of greatest discomfort in acute appendicitis. IV. SHORT BOWEL -GUT SYNDROME − Treatment: Mild Short Bowel Syndrome: 1. eating small, frequent meals 2. drinking fluid 3. taking nutritional supplements 4. using medications to treat diarrhea Moderate Short Bowel Syndrome: 1. similar to that for mild short bowel syndrome, with the addition of parenteral nutrition as needed. 2) Psoas Sign: Irritation of the psoas muscle caused by active right thigh flexion or passive right hip extension in patients with appendicitis. The psoas sign. Pain on passive extension of the right thigh. Patient lies on left side. Examiner extends patient's right thigh while applying counter resistance to the right hip (asterisk). 23 3) Obturator Sign: Irritation of the obturation muscle caused by passive internal rotation of the right thigh in patients with appendicitis. Inflamed appx is in pelvis, in contact with obturator muscle. 4) Rovsing's Sign: Palpation of the left lower quadrant causes pain at the right lower quadrant in patients with appendicitis. − − Symptoms: Sudden pain on the right side of abdomen Vomiting Fever Abdominal bloating Constipation or diarrhea Loss of appetite Management: Surgery is indicated if appendicitis is diagnosed. To correct or prevent fluid and electrolyte imbalance and dehydration, antibiotics and intravenous fluids are administered until surgery is performed. Analgesics can be administered after the diagnosis is made. (Morphine sulphate 10 mg/ml) Antibiotics o Cefotaxime 250mg, 500mg o Levofloxacin 500 mg 24 o Metronidazole 500mg/100ml, 400 mg tablet − − − − − Ruptured Appendicitis → A rupture spreads infection throughout the abdomen (peritonitis). → requires immediate surgery to remove the appendix. → Symptoms: Nausea Vomiting Diarrhea Constipation Appetite loss Swelling in the abdomen Fever Pain in the lower right abdomen Acute Appendicitis Signs 1. RIF Tenderness in McBurney’s point 2. RIF Rebound Tenderness, Release tenderness or Blumberg's sign 3. Guarding/Rigidity 4. Cope's Psoas Test 5. Cope's Obturator Test 6. Rovsing's Sien 7. Hyperesthesia in Sherren's Triangle − Clinical Presentation → Majority of Meckel's diverticuli are clinically silent (Asymptomatic) Symptoms a) Severe hemorrhage b) Intussusception c) Meckel's diverticulitis d) Chronic peptic ulceration e) Intestinal obstruction Nursing Management: (newborn) Symptoms: Bleeding, anemia, severe colicky abdominal pain Abdominal distention, hypoactive bowel sounds, guarding, abdominal mass, rebound tenderness Administer ordered blood products and IV fluids Maintain NPO status Perform postoperative care and family education Surgery: VII. CELIAC DISEASE → a digestive disorder that damages the small intestine, triggered by eating foods containing gluten VI. MECKEL'S DIVERTICULUM → most common congenital defect of the GIT → an abnormal pouch of tissue on the small intestine. 25 → Skin Brittle nails Ache or eczema → Stomach Pain and nausea → Intestinal Diarrhea Bloating Constipation → In female Infertility Miscarriage Early menopause → Mouth Ulcer and tooth enamel erosion → Joint and muscle Pain and swelling Lactose intolerance Anemia Dizziness Migraines Depression Low vitamin D Chronic fatigue − − → inherited condition that prevents the small intestine from absorbing nutrients, causing malnutrition. → Gluten, a protein found in some grains. stimulates immune system antibodies. Antibodies attack. damage lining of small intestine. Celiac disease, Endothelial cells, Villi, microvilli are damaged by body's reaction to gluten. Symptoms: Diarrhea Malnutrition weight loss skin rash some people have no symptoms − Management Dietary Complete elimination of glutencontaining grain products (including wheat, rye, and barley) Children must follow a gluten-free diet for life. Benefits of a Gluten-Free Diet Lowers the risk of intestinal distress Controls the itching connected to dermatitis herpetiformis Can possibly lower death risk when followed from under the age of two 26 BRISTOL STOOL CHART − Gluten Free Substitutes 1) Grains (avoid wheat barley and rye) 2) Dairy (plain milk, cheese, yogurt) 3) Beans 4) Fish 5) Meat 6) Fruits 7) Vegetables − CONSTIPATION → refers to bowel movements that are infrequent or hard pass. The stool is often hard and dry. Etiology → Congenital: a) Anorectal defects b) Neurogenic c) Colonic neuropathies d) Colonic defects → Acquired: a) Functional b) Anal lesions c) Neurologic conditions d) Metabolic e) Endocrine f) Drug induced g) Low fiber diet h) Psychiatric problems SEVERE CONSTIPATION TYPE 2 Lumpy and sausage like MILD CONSTIPATION A sausage shape with cracks in the surface NORMAL like a smooth, soft sausage or snake NORMAL Soft blobs with clear-out edges LACKING FIBRE TYPE 3 Disorders of the Lower Bowel 1) Constipation 2) Inguinal Hernia 3) Hirschsprung Disease (Aganglionic Megacolon) 4) Inflammatory Bowel Disease a. Ulcerative Colitis b. Crohn Disease 5) Irritable Bowel Syndrome 6) Chronic Recurrent Abdominal Pain I. TYPE 1 Separate hard lumps TYPE 4 TYPE 5 − TYPE 6 Mushy consistency with ragged edges MILD DIARRHEA TYPE 7 Liquid consistency with no solid pieces SEVERE DIARRHEA Management foods high in fiber non-stimulant laxatives stimulant laxatives enemas suppositories biofeedback training prescription medications surgery 27 − − Nursing Intervention 1. Increase fluid intake. 2. Laxative fluids (e.g., orange juice relives mild constipation) 3. Small soapy enema may be given. 4. Milk of magnesia may be used as a temporary measure. 5. Establish or maintain regular bowel action by nature means rather than using purgatives. 6. Psychological support to express his fear and his own emotional reactions. Expected outcome infant/child will show no evidence of discomfort and passes stool according to his habit. II. INGUINAL HERNIA → a protrusion of abdominal-cavity contents through the inguinal canal. → symptoms are present in about 66% of affected people. − − Signs and Symptoms → Many hernias present no problem, showing a painless swelling that gives no symptoms, which may be worse when standing, straining, or lifting heavy items. → Immediate medical attention should be sought if an inguinal hernia produces acute abdominal complaints such as: a) Pain b) Nausea c) Vomiting. d) The swelling in these cases is typically firm and tender and cannot usually be reduced e) Hiatal hernia can produce symptoms of acid reflux - producing heartburn when stomach acid gets into the esophagus. Management → Inguinal hernias should always be repaired (herniotomy, herniorrhaphy) unless there are specific contraindications. → Types of operations: a) a permanent suture, as in Shouldice repair (layered suture). b) a permanent mesh -greater frequency to decrease tension. → Inguinal hernia can occur in children. It is surgically corrected when recognized. III. HIRSCHSPRUNG DISEASE → Also known as congenital aganglionic megacolon. → Is an absence of ganglionic innervation to the muscle of a section of the bowel—in most instances, the lower portion of the sigmoid colon just above the anus. → This results in chronic constipation or ribbonlike stools. − → Congenital agenesis of ganglion cells of distal colon Clinical • Failure to pass meconium • Constipation/Intermittent loose stool • Bilious vomiting, poor PO intake, abdominal distension • Enterocolitis (toxic megacolon) complication 28 − − − − − Diagnosis • Rectal biopsy (gold standard) • Contrast enema (presence of transition zone) Management • Surgical excision of aganglionic segment • Colostomy with subsequent end-to-end anastomosis Clinical Manifestations 1. Failure to pass meconium within 24 hours 2. Constipation during first month of life 3. Bile-stained emesis 4. Abdominal distension 5. Distended abdomen 6. Reluctance to eat 7. Failure to thrive 8. V/D; stool w/ ribbon-like appearance Diagnostic evaluation 1. Rectal exam 2. Barium enema or ultrasound 3. Biopsy of the affected segment or 4. Anorectal manometry Therapy → may involve a temporary colostomy followed by surgery in 6 to 12 months to remove the affected bowel portion − − − − − IV. INFLAMMATORY BOWEL DISEASE (IBD) → is an umbrella term used to describe disorders that involve chronic inflammation of the digestive tract. − Crohn's Disease → may affect any part of the digestive system from mouth to anus. All layers of the lining of the bowel may be inflamed. Ulcerative Colitis → affects the large intestine, which is made up of the rectum and colon. Only the inner lining of the bowel is inflamed. Signs & Symptoms → Ulcerative colitis • Attack of diarrhea • Rectal bleeding • Abdominal cramps → Crohn's disease • Recurrent diarrhea • Abdominal pain • Anorexia • Unexplained fever • Malaise • Arthritis • Weight loss Management Mild to moderate symptoms oral medication, vitamins and mineral deficiencies should be corrected. Severe cases bowel rest, enteral or total parenteral nutrition Non-pharmacological therapy: Avoid anti-diarrheal meds, anticholinergics, opiates and NSAIDs. Address diet case-by-case. Vit D and Ca supplementations in all pts on steroids. Surgery case by case Therapy • Long term • If medical therapy is unsuccessful, portions of the bowel may be surgically removed. 29 V. IRRITABLE BOWEL SYNDROME (IBS) → a common disorder that affects the large intestine. → a chronic condition that need to manage long term. − Signs & Symptoms • Cramping • abdominal pain • bloating • gas • diarrhea or constipation, or both − Causes 1. Diet 2. Intolerance to Food Items 3. Mental Ailments 4. Infections 5. Emotional Stress 6. GI Motor Problems − − Inflammatory Bowel Disease • Classified as a disease • Inflammatory condition • Can cause hospitalization and/or need for surgery • Can cause permanent damage to intestines • Doctors can diagnose with exam and imaging • Risk factor for colon cancer • Treated with anti-inflammatory drugs or surgery Irritable Bowel Syndrome • Classified as a syndrome set of symptoms) • Non-inflammatory condition • Rarely requires hospitalization or surgery • Doesn't cause permanent damage to intestines • Exam and imaging don't show clinical signs of disease • Not a risk factor for colon cancer • Diet is the primary treatment VI. CHRONIC RECURRENT ABDOMINAL PAIN → Chronic abdominal pain - pain of at least three months duration, although some clinicians consider pain of more than one to two months' duration to be chronic. → Recurrent abdominal pain - one of the most common recurrent pain syndromes in childhood. The classic definition is based upon four criteria: 1. History of at least three episodes of pain 2. Pain sufficiently severe to affect activities 3. Episodes occur over a period of three months 4. No known organic cause → Recurrent (Chronic) Abdominal Pain - Recurrent abdominal pain is defined as the occurrence of multiple episodes of abdominal pain over at least 3 months that are severe enough to cause some limitation of activity. - Recurrent abdominal pain is a common problem in children, affecting more than 10% of children at some time during childhood. - The peak incidence occurs between ages 7 and 12 years. - Although the differential diagnosis of recurrent abdominal pain is fairly extensive, most children with this condition are not found to have a serious (or even identifiable) underlying illness causing the pain. Disorder Caused by Food, Vitamin and Mineral Deficiencies 1) Kwashiorkor 2) Nutritional Marasmus 3) Vitamins & Mineral Deficiencies I. PROTEIN ENERGY MALNUTRITION (PEM) → Marasmus was thought to result primarily from inadequate energy intake, whereas kwashiorkor was thought to result primarily from inadequate protein intake. → Marasmic kwashiorkor, has features of both disorders (wasting and edema). 30 − Types of Malnutrition − Kwashiorkor → a form of severe protein malnutrition characterized by edema and an enlarged liver with fatty infiltrates. → It is caused by sufficient calorie intake. Symptoms failure to grow or gain weight. edema (swelling) of the ankles, feet, and belly. damaged immune system, which can lead to more frequent and severe infections. irritability. − − II. NURITIONAL MARASMUS → a severe form of protein-energy malnutrition that results when a person does not consume enough protein and calories. Signs & Symptoms 31 TOPIC 3 ALTERATION IN ENDOCRINE OR METABOLIC FUNCTION III. VITAMIN E MINERAL DEFICIENCES Vitamin A Lack of yellow vegetables in diet Tender tongue, cracks at corners of mouth, night blindness Xerophthalmia (dry and lusterless conjunctivae) Keratomalacia (necrosis of the cornea with perforation, loss of ocular fluid, and blindness) Vitamin B1 Most common in children who cat polished rice as dietary staple because B, is contained in hull of rice Beriberi (tingling and numbness of extremities, heart palpitations, exhaustion) Diarrhea and vomiting Aphonia (crying without sound) Anesthesia of feet Niacin Common in children who cat corn as dietary staple because corn is low in niacin Pellagra (dermatitis, resembles a sunburn), diarrhea, mental confusion (dementia) Vitamin C Lack of fresh fruits in diet Scurvy (muscle tenderness, petechiae) Vitamin D Lack of sunlight Poor muscle tone, delayed tooth formation Rickets (poor bone formation) Craniotabes (softening of the skull) Swelling at joints, particularly of wrists and cartilage of ribs Bowed legs, tetany (muscle spasms) Learning Outcomes : Review the structure and function of the endocrine glands and why metabolic illnesses occur. Identify metabolic & endocrine disorders Assess a child with a disorder of endocrine or metabolic dysfunction. Formulate nursing diagnoses for a child with altered endocrine or metabolic dysfunction. Establish expected outcomes for a child with endocrine or metabolic dysfunction. Implement nursing care for a child with endocrine or metabolic dysfunction. Evaluate expected outcomes for achievement and effectiveness of care. The word hormone is from the Greek hormaein ,which means “to set in motion.” 32 Endocrine & Metabolic Disorder I. PITUITARY GLAND DISORDERS 1. Growth hormone deficiency 2. Growth hormone excess 3. Diabetes insipidus Function of the pituitary gland: • Anterior Pituitary Gland a) GH b) TSH c) ACTH d) FSH e) LH f) PROLACTIN • Posterior Pituitary Gland a) Oxytocin b) ADH − − GH Deficiency → The cause of the defect is unknown; it may have a genetic origin. → A pituitary tumor must be ruled out as the cause of decreased GH production → Results in extremely short stature if left untreated • Therapeutic Management Administration of intramuscular recombinant human growth hormone (rhGH) Usually given daily at bedtime, the time of day at which GH normally peaks GH Excess → There is an overgrowth of body tissues → Usually is caused by a benign tumor of the anterior pituitary (an adenoma) • Diagnostic Test: CBC → erythrocyte sedimentation rate (ESR); → electrolytes and general health chemistry panel → urinalysis → serum for thyroid function studies, ESR → is a type of blood test that → measures how quickly erythrocytes (red blood cells) settle at the bottom of a test tube that contains a blood sample. − Diabetes Inspidus → Extreme thirst or appetite may occur → Therapy is the administration of desmopressin, an arginine vasopressin. (Pituitary gland produces insufficient ADH, hence the kidneys make a lot of urine) • Assessment Frequent voiding in children most often reflects a urinary tract infection, but it may be evidence of excessive urine excretion (polyuria), possibly from pituitary dysfunction or diabetes mellitus. A child’s general appearance may reveal early or late puberty changes, scaling or dry or darkening skin, drooping eyelids, protrusion of the eyeballs. II. THYROID GLAND DISORDERS 1. Congenital Hypothyroidism 2. Acquired Hypothyroidism (Hashimoto Thyroiditis) 3. Hyperthyroidism (Graves Disease) Function of the thyroid gland: a) It plays a major role in the metabolism, growth and development of the human body. b) Thyroxine - controls metabolic rate c) Calcitonin - lowers blood calcium levels Functions of Thyroid Hormone: 1) Metabolic: increases cellular oxygen consumption and heat production in part by stimulating Na+/K+ ATPase 2) Cardiovascular: marked positive inotropic and chronotropic effects on heart 3) Sympathetic: increases number of alpha and beta-adrenergic receptors in heart muscle and beta receptors in skeletal muscle, adipose tissue, and lymphocytes 4) Respiratory: maintains normal hypoxic and hypercapneic drive in medullary respiratory center 5) Hematopoietic effects: stimulates secretion of Epo to increase BC synthesis 33 − • Congenital Hypothyroidism • (Cretinism) occurs as a result of an absent or nonfunctioning thyroid gland. • The condition is discovered by a blood spot test at birth • Therapy: oral administration of synthetic thyroid hormone • The symptoms of the disorder become apparent during the first 3 months of life in a formula-fed infant and at about 6 months in a breastfed infant. − • − Almost all cases identified through Neonatal Screening Clinical - Constipation - Hypotonia - Hoarse cry - Macroglossia Delayed treatment can lead to: - Learning disabilities - Cognitive deficits - Clumsiness - Diminished fine motor skills • Therapeutic Management Oral administration of synthetic thyroid hormone (sodium levothyroxine) a small dose is given at first, and then the dose is gradually increased to therapeutic levels. Acquired Hypothyroidism • (Hashimoto disease) is an autoimmune phenomenon that interferes with thyroid gland function. • Therapy: administration of synthetic thyroid hormone. Symptoms of hypothyroidism in children Short stature or slow growth Rough, dry skin Cold intolerance Fatigue Bruising easily Delayed puberty → The excretion of thyroid-stimulating hormone (TSH) from the pituitary increases when thyroid hormone → production decreases in an attempt by the pituitary gland to increase thyroid function. → in response to the increased level of TSH, hypertrophy of the thyroid gland (goiter) can occur, → and body growth is impaired by a lack of T4, with prominent symptoms of obesity, lethargy, → and delayed sexual development • Therapeutic Management Administration of synthetic thyroid hormone (sodium levothyroxine) Hyperthyroidism • (Graves Disease) caused by overproduction of thyroid hormones. It leads to jitteriness and tachycardia. Treated by medication to suppress thyroxine release. • in children is caused by an autoimmune reaction that results in overproduction of immunoglobin G (IgG) III. ADRENAL GLAND DISORDER 1. Acute Adrenocortical insufficiency 2. Congenital Adrenal Hyperplasia 3. Cushing Syndrome → also known as suprarenal glands → Adrenal glands secrete hormones which help regulate stress and metabolism and supplement other glands → Fight or Flight Response - A reflex response which is designed to help individual manage physically when under stress. - Activated in times of stress as the body perceive stress as a threat. 34 secreted by the pituitary increases in an attempt to stimulate the gland to increase function. → Although the adrenals enlarge (hyperplasia) under the effect of ACTH, they still cannot produce cortisol; instead, they overproduce androgen. - − Physical Indications of Fight or Flight Response dilated pupils trembling pale or flushed skin rapid heart beat and breathing Acute Adrenocortical Insufficiency → an emergency situation in which there is abrupt nonfunction of the adrenal glands. → Usually, this occurs following a severe overwhelming body infection such as meningococcemia. → can occur in either an acute or chronic form. • − Assessment BP drops to extremely low levels May appears ashen gray, and the pulse will be weak. Temp. gradually becomes elevated dehydration and hypoglycemia seizures may occur. • Therapeutic Management Immediate replacement of cortisol Administration of deoxycorticosterone acetate (DOCA) IV 5% glucose in normal saline solution Congenital Adrenogenital Hyperplasia → Abnormalities in specific enzyme of the adrenal gland that causes severe salt lose, dehydration and abnormally high levels of male hormones in both boys and girls. → Therapy: administration of hydrocortisone and also aldosterone if sodium loss is present. → a syndrome that is inherited as an autosomal recessive trait and which causes the adrenal glands is not able to synthesize/produce cortisol. → Because the adrenal gland is unable to produce cortisol , the level of adrenocorticotropic hormone (ACTH) − → Salt-wasting Congenital adrenogenital hyperplasia - adrenal glands make too little aldosterone, causing the body to be unable to retain enough sodium (salt). - Too much sodium is lost in urine (thus the name, "salt-wasting"). • Assessment genital organs in a male fetus to "overgrow," or increase in size masculinizes a female fetus if the labia are fused, she appears to be a boy with undescended testes and hypospadias • Therapeutic Management Replace the cortisol that is missing corticosteroid agent Cushing Syndrome → caused by the overproduction of cortisol by the adrenal gland, which is usually caused by a tumor in the gland. → Children appear abnormally obese. → Therapy is the surgical removal of the tumor 35 − • IV. Type 1 Diabetes Mellitus → an autoimmune process that destroys insulin production islet cells. → An acute loss in weight is often the first symptom → Therapy is a combination of insulin, diet, and exercise. Therapeutic Management a) Treatment: surgical removal of the causative tumor b) Prognosis depends on whether the tumor is benign or malignant because a carcinoma of this type trends to metastasize rapidly. c) If a major part of the adrenal glands are surgically removed, the child will need replacement cortisol therapy indefinitely. PANCREATIC DYSFUNCTION 1. Type 1 diabetes mellitus 2. Type 2 diabetes mellitus 3. Cystic fibrosis 36 • − Therapeutic Management Insulin administration Regulation of nutrition and exercises Stress management Blood glucose and urine ketone monitoring. Type 2 Diabetes → Sometimes called a “lifestyle” disease. → May be revealed by being overweight → Therapy: diet, exercises, and an oral anti glycemic agent. • Two diagnostic tests used to confirm diabetes: Fasting blood glucose test Random blood glucose test → Role of insulin in the body 1. Insulin helps your body turn blood sugar (glucose) into energy. 2. It also helps your body store it in your muscles, fat cells, and liver to use later, when your body needs it. • Laboratory Studies Laboratory studies usually show a random plasma glucose level greater than 200 mg/dl Normal range, 70 to 110mg/dl fasting; 90 to 180mg/dl not fasting and significant glycosuria. 37 − Gestational Diabetes → condition in which blood sugar (glucose) levels become high during pregnancy and usually disappears after giving birth • • − Complications - Fetal risks Excessive birth weight Jaundice: baby with yellow skin Hypoglycemia (Their own insulin production is high as mother gave them lots of glucose) Higher risk of type I diabetes later in life (Breastfeeding may lower the risk) Cystic fibrosis → is a hereditary disease that affects the lungs and digestive system. → The body produces thick and sticky mucus that can clog the lungs and obstruct the pancreas. → Cystic fibrosis (CF) can be life-threatening, and people with the condition tend to have a shorter-than-normal life span. Cystic fibrosis and your child's body a) Children with CF have a high ratr of sinus infection. b) In 90% of children with CF, the pancreatic duct is blocked. c) The lack of sufficient pancreatic enzymes leads to the malabsorption of nutrients. d) Cross section thru bronchus (Normal airway); thin layer of mucus e) (Airway with CF) bacterial infection, thick layer of mucus, blocks airway → causes thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. → This may cause malnutrition, poor growth, frequent respiratory infections, breathing problems, and chronic lung disease. V. PARATHYROID GLAND DISORDER 1. Hypocalcemia 2. Hypercalcemia 38 − Hypocalcemia → LOWERED blood calcium level, causing tetany to develop. → Therapy: administration of calcium. • Signs • Causes 1. Supplements 2. Overactive parathyroid glands (hyperparathyroidism) 3. Cancer 4. Other diseases 5. Hereditary factors 6. Immobility 7. Severe dehydration 8. Medications Treatment - If hypercalcemia is mild: monitoring of bones and kidneys over time to be sure they remain healthy. - severe hypercalcemia: medications or treatment of the underlying disease Surgery Medications: Calcitonin - controls calcium levels in the blood Calcimimetics - help control overactive parathyroid glands. Bisphoshonates - Intravenous osteoporosis drugs, which can levels, are often used to treat hypercalcemia due to cancer Denosumab - used to treat people with cancer-caused hypercalcemia who don't respond well to bisphosphonates. Prednisone - If hypercalcemia is caused by high levels of vit. D, short term use of steroid pills such as prednisone are usually helpful. IV fluids and diuretics - to promptly lower the calcium level to prevent heart rhythm problems or damage to the nervous system. • • 1) 2) • − Signs and Symptoms C – onvulsions A – arrhythimus T – etany S – tridor and spasms • Causes Vitamin D inadequacy or vitamin D resistance Hypoparathyroidism following surgery Hypoparathyroidism owing to autoimmune disease or genetic causes Renal disease or end-stage liver disease causing vit. D inadequacy Pseudohypoparathyroidism Hypercalcemia → Calcium level in the blood is above normal 3) 4) 5) 6) VI. DISORDERS OF METABOLISM 1. PKU 2. Galactosemia 3. Tay – Sachs disease → a term that is used to describe all chemical reactions involved in maintaining the living state of the cells and the organism. 39 → Metabolism two categories: a) Catabolism b) Anabolism • • 1) PKU → A disorder of amino acid metabolism wherein the body is not able to use phenylalanine leading to its accumulation. → Phenylalanine is an amino acid found in many foods and used by your body to produce proteins and other important molecules. 2) Galactosemia → A disorder of carbohydrate metabolism wherein the body is not able to use galactose leading to its accumulation. 3) Tay-Sachs disease → a rare disorder passed from parents to child → caused by the absence of an enzyme (gangliosides) that helps break down fatty substances. → Leading to build up to toxic levels in the child's brain and affect the function of the nerve cells − Classical Phenylketonuria (Phenylalanine hydroxylase deficiency) - Most common outcome is severe mental retardation (IQ <50) - Cerebral palsy (33 %), behavioral problems - Often associated with a mousy odor - Vomiting - prominent early symptoms - Eczema (20-40%) – mild - Reduced hair, skin and iris pigmentation - Reduced growth and microcephaly - Neurologic impairments (25% epilepsy, 30% tremors, 5% spasticity, 80% EEG abnormalities) Management Dietary Restriction of PHE - Special formulas - Limited protein diets/food - Vitamin supplement Periodic assessment of plasma PHE levels - Weekly for 2-4 months - Every 2 weeks until 6-12 months - Monthly thereafter Neurodevelopmental assessment Early intervention (speech therapy, PT, OT) Genetic counseling Phenylketonuria → (PKU) A disorder of amino acid metabolism wherein the body is not able to use phenylalanine leading to its accumulation 40 • − • TREATMENT: Dietary restriction of phenylalanine Goal: limit dietary intake of phenylalanine in amount required for normal growth and development but below toxic level. For babies: special milk formula + controlled amount of breast milk and/or regular milk formula Typical diet consist of low protein foods, meaning limited intake of meat, fish, poultry, milk, eggs, cheese, legumes, and regular flour. Monitoring of blood phenylalanine level at least quarterly. • Galactosemia → A disorder of carbohydrate metabolism wherein the body is not able to use galactose leading to its accumulation. → Galactosemia, which means "galactose in the blood," refers to a group of inherited disorders that impair the body's ability to process and produce energy from a sugar called galactose. • − • Clinical Manifestations: 1. It appear within days of birth or initiation of milk feedings 2. Signs and symptoms depend on the level of enzymes deficiency. 3. Vomiting & jaundice - early signs 4. Sepsis - neonatal period 5. Failure to thrive 6. Hepatomegaly 7. Cataract - after 3-4 weeks of milk feedings 8. Mental Retardation 9. Fatal - if milk feedings are continued Diagnosis Newborn Screening can detect virtually 100% of affected infants Requirements: 1. Lactose-containing milk/foods 2. No blood transfusions Confirmatory Test FREE Repeat DBS (filtercard) — Beutler test Long term treatment and follow-up a. Diet - remove galactose from the diet b. Monitor Gal/Gal-1-P level c. Early intervention for problems of motor, speech and cognitive development d. All girls should be evaluated for premature ovarian failure at 10-12 years of age e. Regular ophthalmologic evaluation for those with cataracts f. Regular evaluation of liver profile Prognosis: With strict adherence to a galactosefree diet, the prognosis is good. Cataracts are reversible if therapy is started before 3 months. There is recovery of the liver abnormalities with dietary restrictions. Tay-Sachs Disease → is a rare, inherited disorder. It causes too much of a fatty substance to build up in tissues and nerve cells of the brain. → A rare disorder passed from parents to child. → Caused by the absence of an enzyme (gangliosides) that helps break down fatty substances. Leading to build up toxic levels in the child’s brain and affect the function of the nerve cells. • Signs and Symptoms Is classified in variant forms, based on the time of onset of neurological symptoms. 1. Infantile (3 to 10 months) 2. Juvenile (Two and 10 years) (extremely rare) 3. Adult/Late Onset (20 and 30 years) (usually non-fatal) 41 2. Effective treatment plans and medication administration techniques can facilitate successful interventions in long-term care. 3. Increase compliance Nursing Process Assessment 1. Measure the child’s height and weight to find out if above or below a typical measurement for that age. 2. To obtain information on activity in the child, take a day history by asking a parent or child to describe all the child’s actions on a typical day. 3. Assess dietary and elimination habits. Nursing Diagnosis 1. Deficient fluid volume related to constant excessive loss of fluid through urination. 2. Risk for imbalanced nutrition, less than body requirements, related to an inability to use glucose because of diabetes mellitus 3. Disturbed body image related to abnormal height (Health-seeking behaviors related to the self-administration of insulin). 4. Deficient knowledge related to long-term treatment needs. 5. Fear related to the potential and unknown illness outcome. 6. Anticipatory grieving related to presumed losses associated with diagnosis of longterm illness. 7. Interrupted family processes related to the child's chronic illness. 8. Anxiety related to financial resources required to maintain optimum family health Outcome Evaluation 1. Periodic evaluations throughout childhood because growth and changing activities necessitate changes in medication dosages or schedules. 2. Adherence to a medication program may become erratic. Only by periodic reevaluation can these problems be identified so that healthcare plans can be modified and adapted to the child's s needs, enabling the child and family to continue coping with a long-term illness. The following examples suggest desired outcomes have been achieved: 1. The child brings to clinic a written record showing that she took her medication as recommended. Parents list developmentally appropriate, not size-appropriate, activities for their child with a short stature. 2. Parents demonstrate correct insulin injection technique and state they are comfortable administering an injection to their child. 3. Education and genetic counseling 4. School nurses can be instrumental not only in counseling and helping students manage this disorder during school hours but also in helping prevent T2D by serving as an advisor to what foods and snacks ought to be available in school settings. Outcome Identification and Planning 1. Helping parents create reminder charts or set alerts on their smartphones are effective measures to increase compliance. 2. Evaluate both the school and home situation for any child with a chronic illness. Implementation 1. Referrals to age-appropriate support groups within their communities that will serve to build lifelong bonding and relationships. 42