Exam 3 Study Guide PDF

Exam 3 will consist of 40 questions, and you will have 60 minutes to complete the exam. It will cover content from week 8-10. There will be 4 math questions, and then 6-10 questions from each of the modules. These questions will largely be multiple choice, potentially including select-all-that-apply...

Exam 3 will consist of 40 questions, and you will have 60 minutes to complete the exam. It will cover content from week 8-10. There will be 4 math questions, and then 6-10 questions from each of the modules. These questions will largely be multiple choice, potentially including select-all-that-apply. You are allowed to bring a calculator (not your phone) and/or use scrap paper. Within the following conditions, you should understand the pathophysiology, clinical manifestations, nursing assessment and interventions, education and management. Week 8: Musculoskeletal Impact of immobility in children o Loss of innervation o Sensory and perceptual deprivation🡪 ▪ Decreased exposure to normal play opportunities🡪 Impaired growth and development NURSING CARE OF THE IMMOBILIZED CHILD Physical assessment ○ Repositioning- Position changes and moving child around the room as tolerated ○ Pressure reduction mattresses ○ ROM exercises ○ Braden scale Encourage activity and mobility devices Allow autonomy when possible Fractures & complications ○ Fractures Toddler’s fracture 9-36 months Trivial trauma Spiral fx of the tibia Stress fractures: repetitive sports activities May need MRI for diagnosis, not seen on plain films ○ Child abuse- make sure the history matches the injury! See multiple fractures that you're not looking for ○ Muscle strains/sprains Always remember about Salter 1 injuries Bones will not grow if epiphyseal plate fracture + plates are open Docs will cast even if there's no confirmed fracture Fractures in children ○ Growth plate absorbs the shock ○ The periosteum of a child's bone is thicker and stronger ○ Bone are more likely to bend, buckle, and break ○ Younger the child, the quicker the healing ○ Children only complain when something is wrong. ○ Clinical manifestations Generalized swelling/edema Pain or tenderness Decreased use of affected area or physical deformity Ecchymosis Warmth or redness Diagnostic Evaluation Therapeutic Management ○ Nursing care management: child with emergent fracture Stabilize the injured site Cover open wounds with a sterile dressing Apply manual traction if circulatory compromise is present* R ICE ○ Complications of fractures Pain: Assess for the presence of severe pain Pulse: Assess pulse distal to the fracture site- late and ominous sign Pallor: Assess for decreased blood flow Paresthesia: Assess for sensation distal to the fracture site Paralysis: Assess movement distal to the fracture site ○ Toddlers fracture → ○ Common pediatric fractures → Cast care in children Clubfoot Clubfoot (congenital talipes equinovarus) Clubfoot is a complex deformity of the ankle and foot, involving bone deformity and malposition with soft tissue contracture genetic disorder causing deformity ○ risks: maternal smoking/ETOH/diabetes, family history s/s: calf muscle may be smaller dx: visual/ultrasound  Treatment ○ Goal is to straighten foot ○ Serial casting ○ Surgery LEGG CALVE PERTHES DISEASE disruption of blood flow to the femoral head- decreased perfusion idiopathic manifestations: painless limp, help pain/ stiffness, decreased hip ROM short affected led tx: activity restrict, NSAID, PT, osteotomy (femur cut and reposition) Scoliosis Idiopathic scoliosis ○ Complex but most COMMON spinal deformity in three planes ○ Classification based on age of onset ○ Infantile - birth to 3yo ○ Juvenile - 3-10yo ○ Adolescent - 10+ (most common due to growth spurts) ○ School screening controversial – but screening necessary ages 10-12 girls; ages 12-13 boys ○ Nursing care crucial for education + support ○ S/S: asymmetry of shoulder height, scapular shape, hip height ○ dx: adams forward bend test- scoliometer confirmed using XR SCOLIOSIS: TREATMENT OPTIONS Treatment options depend on severity: ○ Bracing + exercises ○ OR ○ Surgical intervention (Important to set expectations pre-op) ○ Post-op Care: Vital signs Skin, circulation and NEURO status Pain control  when turning patient- LOG ROLL opioid analgesics for pain Developmental dysplasia of the hip Developmental dysplasia of the hip ○ Hip instability after birth caused by abnormal development of the femur head and acetabulum ○ Pathophysiology: Cause is unknown ○ Clinical signs and symptoms: Asymmetrical skin folds Galeazzi’s sign (Knee on affected side is lower than the other knee) Limited hip abduction Hip instability Infant: Asymmetry of gluteal and thigh folds (A) Limited hip abduction (B) Galeazzi’s sign © Positive Ortolani test (d) Positive Barlow test (d) Child: One leg is shorter than the other Positive Trendelenburg's sign (E) Walking on toes on one foot Walking with a limp Diagnosis: Ultrasound, x-ray (if > 4 months) ○ Degrees of DDH ○ Therapeutic mgmt Pavlik harness Traction and then surgical closed reduction and immobilization using a spica cast  ○ Nursing care management Assessment of skin integrity Education and support of family no triple diapering clothes under harness, diaper under straps Osteogenesis imperfecta Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by bones that fracture easily. faulty bone mineralization, abnormal bone architecture, and increased susceptibility to fracture. Osteomyelitis Bone infection: Often local trauma and bacteremia lead to bacterial seeding of the metaphysis. Accounts for only 2% of all limping children who present to ED Infection most common in long bones of the body Symptoms include swelling, redness ,warmth and tenderness ○ Initial symptoms can be very mild, not always Neuro ICP Recognition of/care of increased ICP ○ Increased ICP (intracranial pressure) is excessive pressure on the rigid cranial vault that disrupts neurological function ○ Etiology: Can result from any condition that increases tissue or fluid volume in the skull Manifestations of increased ICP Early: Irritability, fatigue, personality changes ○ In infants and young children: Tense, bulging anterior fontanelle High pitched cry Increased head circumference “Setting-sun” sign eyes bulging and pointing down Irritability, restlessness Feeding changes Late Manifestations of Increased ICP ○ Bradycardia ○ Decreased motor response to command ○ Decreased sensory response to painful stimuli ○ Alterations in pupil size and reactivity  ○ Extension or flexion posturing ○ Cheyne-Stokes respirations ○ Papilledema ○ ↓ Motor response ○ ↓ Sensory response to painful stimuli ○ ↓ level of consciousness (ranging from lethargy to coma) ○ Decorticate and decerebrate posturing NURSING CARE AND MANAGEMENT OF INCREASED ICP Assess for early changes in ICP Administer prescribed medications Keep head and neck at neutral Monitor for SIADH and DI related to hypothalamic dysfunction Hydrocephalus definition: Increased amount of CSF in the ventricles and subarachnoid spaces of the brain Causes: Impaired absorption (most common), excessive production, or obstructed circulation of CSF Communicating vs non-communicating traditionally refers to obstructive or non-obstructive Clinical manifestations ○ Newborns and infants Bulging fontanelles Inc head circ Sunset eyes Irritability High pitched catlike cry Visible scalp veins ○ Children Headache Visual disturbances n/v Sluggish pupils Decrease in consciousness  Seizures Cushing's triad Management and complications ○ Treatment of excessive CSF is usually ventriculoperitoneal shunt (VP shunt) ○ Post-op Care: Monitor for signs of increased ICP that indicate obstruction of the shunt. Neuro Assessment includes pupil dilation (pressure causes compression or stretching of the oculomotor nerve, producing dilation on the same side as the pressure) Other assessments Blood pressure (hypoxia to the brainstem causes variability in these vital signs). The nurse also observes for abdominal distention and constipation because CSF may cause peritonitis or a postoperative ileus as a complication of distal catheter placement ○ VP shunts VP shunt Malfunction: most often caused by mechanical obstruction either within the ventricles from particulate matter (tissue or exudate) or at the distal end from thrombosis or displacement as a result of growth Vp shunt infection: can occur at any time, but the period of greatest risk is within the first month after placement Nursing ed Parent education: ○ Watch for s/s of increased CSF at home ○ Can be subtle Changes in school performance, intermittent headache, mild behavior changes ○ Participate in normal activities and lifestyle ○ Contact sports are prohibited ○ Frequent developmental screenings and routine medical checkups Spina bifida conditions ○ Part of group of neural tube defects ○ insufficient folic acid intake during pregnancy think- INSUFFICIENT FUNDS SSSSSS you ain't got no folic i think i am going insane i've been in snell since 5:30 pm it is 11:30 ○ Failure of osseous spine to close Spina bifida occulta  Not visible externally Does not affect the spinal cord but may have external signs such as dimpling of the skin, nevi or hair tufts May go undetected most have no neurologic signs If they do have neuro signs, usually motor or sensory deficit of lower extremities and urinary impairment tx: surgically close the tube defect baby in prone position, hips flexed Spina bifida cystica (meningocele and myelomeningocele) meningocele and myelomeningocele – visible external sac like protrusion protect sac with seal non adherent dressing complications: hydrocephalus, latex allergy, increased risk for pressure injury and burn, bladder dysfxn Epilepsy vs febrile seizures Epilepsy: ○ 2 or more seizures unprovoked at least 24 hours apart OR ○ One unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures occurring over the next 10 years Epilepsy is considered a disease of the brain (not a disorder) Most common pediatric neuro disorder Manifestations of Seizures Depends on region of the brain that they originate from May include unconsciousness or altered consciousness, involuntary movements, and changes in perception, behaviors, sensations, and/or posture. Potential causes include infections, intracranial lesions or hemorrhage, metabolic disorders, trauma, brain malformations, genetic disorders, or toxic ingestion. Types of seizures Focal seizures – most frequent seizures - formally known as partial Without impaired awareness – simple - with motor signs is clonus, the rhythmic alternating contraction and relaxation of muscle groups With impaired awareness – complex – most common ○ may begin with aura—sensation or sensory phenomenon precedes seizure activity. eg taste, fear/anxiety, hallucination etc, part of seizure event + associated with EEG changes  ○ Small children may emit a cry as a manifestation of an aura. Generalized: Involving both hemispheres (usually no aura), Tonic-clonic Absence seizures: brief LOC for 5-10 seconds; may be unrecognized → "daydreaming" Status epilepticus: emergency – multiple seizures at brief intervals that don't allow child to regain consciousness OR > 30 mins Febrile seizures Febrile seizures: 4% of pediatric population (common ages 6mos- 5 years) ○ Seizure associated with febrile illness in the absence of CNS infection in children older than 1 month without prior seizure activity ○ Simple partial: Most common Cause: Uncertain Risk factors: Family hx; temperature >101.8 F (38.8 C), preceded by URI or GI illness Treatment: Usually over by the time they seek care, give IV or rectal diazepam and reduce temperature with Tylenol or Motrin Parental education is vital! ○ Antipyretics control symptoms of fever but do not prevent ○ Attempts to lower temp will not help (tepid baths, antipyretics) ○ Long term anti-epileptic therapy not needed ○ Not a high risk to develop epilepsy (1%) unless family history (10%) ○ Give rectal diastat (diazepam) for seizure lasting longer than 5 minutes Meningitis Acute infection of meninges or CSF Bacterial or viral Droplet infection through nasopharyngeal secretions Role of Hib and Pneumococcal vaccine in incidence Manifestations of Meningitis Usually abrupt onset ○ Fever ○ Chills ○ Headache ○ Vomiting ○ Alterations in sensorium ○ Seizures (often the initial sign) ○ Irritability ○ Agitation ○ Nuchal rigidity ○ Positive Kernig and Brudzinski signs ○ Hyperactivity but variable reflex responses Kernig’s sign - - lying on back – pain with attempt to extend knee past 135 degrees Brudzinski - lying on back - - passive forward neck flexion causes pt to involuntarily lift hips or knees  Symptoms may progress: May develop the following: ○ Photophobia ○ Delirium ○ Hallucinations ○ Aggressive behavior ○ Drowsiness ○ Stupor ○ Coma TREATMENT & MANAGEMENT ○ IV Abx ○ Dx made by LP +/- Labs + cultures ○ Maintain resp/droplet precautions for at least 24 hrs on abx Head trauma; s/s of head injury; concussion care Head Injuries Head trauma encompasses everything from a mild bump to severe brain damage PATHOPHYSIOLOGY OF HEAD INJURY: ○ Intracranial contents of the head (brain, blood, CSF) are damaged because the force is too great to be absorbed by the skull and musculo-ligamentous support of the head ○ Signs and symptoms depend on the area affected ○ Most common (in order) reasons for Head injury in children: Falls being struck by or striking an object with one's head motor vehicle accidents ○ Assault #1 cause of death from TBI in kids >4 Concussion  Transient and reversible Alteration in mental status with or without loss of consciousness immediately after the injury Generally followed by amnesia and confusion Skull Fracture Often result from a direct blow or injury to the skull Occur often even with minor injuries Common up to age 2 years old, infants at increased risk even with minor trauma Usually due to falls within 3 feet (fall from arms, bed) Types: ○ Linear fractures ○ Depressed fractures ○ Basilar fractures; Battle's sign (bruising over the mastoid process) and Raccoon eyes (bruising around the eyes) ○ Diastatic fractures Complications of Head Trauma Epidural hemorrhage  Subdural hemorrhage Herniation through the brainstem Cerebral edema Post-traumatic syndromes Head Trauma Post Injury Immediately and long term: 1st sign of worsening head trauma is change in level of consciousness Post injury Nursing Considerations: Frequent assessment—vital sign and neurologic checks Assessing LOC: neuro checks are necessary. Need to know baseline alertness prior to injury, immediately after injury, and then long term to assess progression. OUTPATIENT CARE: If mild, no complications INPATIENT: Care in hospital if severe injuries, loss of consciousness for several minutes, prolonged or continued seizures ○ NPO initially ○ Possible surgical interventions ○ Prognosis Cerebral palsy Non-progressive, neuromuscular disorder of varying degrees resulting from damage/dev defects in the part of the brain that controls motor function. manifests diff w each child Most common permanent physical disability in childhood In addition to motor/musculoskeletal problems the condition often involves ○ disturbances of sensation, perception, communication, cognition, and behavior; secondary musculoskeletal problems; and epilepsy Developmental outcomes vary and are dependent on the severity of the injury. Etiology ○ 70-80% of CP cases are due to some UNKNOWN PRENATAL factor ○ Prenatal risk factors: Maternal infections, medications, malnutrition, toxins, unknown factors ○ Perinatal (Period immediately before and after birth) risk factors : Perinatal infections Birth weight Long labor, preeclampsia Asphyxia/Anoxic injury Intraventricular hemorrhage, perinatal stroke, meconium aspiration  ○ Childhood or postnatal risk factors: Brain injury, infections, strokes, shaken baby syndrome ○ Biggest risk factor for development of CP is preterm birth of low birth weight and extremely low birth weight infants Clinical manifestations at time of dx ○ Delayed gross motor development* ○ Abnormal Motor Performance ○ Alterations of Muscle Tone ○ Abnormal Postures ○ Reflex abnormalities ○ Associated Disabilities Other associated disabilities ○ Altered learning and reasoning ○ Wide range of intelligence (50-60% have normal intelligence) ○ Seizures ○ Behavioral problems ○ Sensory impairment (vision, hearing) ○ Speech and language disorders ○ Bowel and bladder problems ○ Dental concerns ○ Skin breakdown Types of cp ○ Spastic: most common type (80%) Area of brain affected: Cortex Can be hemiplegia, diplegia, quadriplegia, etc. depending on limbs affected Characterized by stiffness & difficulty moving Other signs: Persistence of primitive reflexes Hypertonicity with poor control of posture, balance and coordinated movement Impairment of fine and gross motor skills Tendency to have contractures Weakness of affect limbs Characterized by stiffness & difficulty moving Child has characteristic scissor gait, crossing one foot over the other Other signs: Tendency to have contractures  Weakness of affect limbs ○ Dyskinetic Area of the brain affected: Basal ganglia Characterized by involuntary and uncontrolled movement (dystonia) Other signs: Abnormally slow, writhing movements Chorea: Irregular jerking movements Abnormal postures Poor swallowing and drooling ○ Ataxic Area of the brain affected: Cerebellum Characterized by balance and coordination difficulty Other signs: Poor balance and muscle coordination Wide based gait Intention tremor Therapeutic management ○ Mobilizing devices ○ Surgical interventions ○ Early recognition + promotion of optimum developmental course ○ Pharmacologic treatments: goal is to control pain, decrease spasticity and control secondary conditions like seizure disorders Baclofen: Centrally acting skeletal muscle relaxant, decreases muscle spasm/severe spasticity (oral or intrathecally via pump) Diazepam (Valium): Skeletal muscle relaxant used to decrease muscle spasms and severe spasticity Botox: Reduced spasticity in certain muscle groups AEDs (if seizures) to control seizure activity ○ Technical aids ○ Manage associated problems ○ Therapies, Education and Recreation and Nursing Care Management Physical therapy Occupational therapy Speech therapy and hearing aids Education Recreation Duschenne’s MD Muscular dystrophy  Muscular dystrophy (MD) is a group of inherited disorders with progressive degeneration of symmetric skeletal muscle groups causing progressive muscle weakness and wasting All have increasing disability and deformity with loss of strength Onset of disease, pace of progression and muscle group affected depend on the type of MD Various types: Duchenne, Becker, Myotonic, etc. Duchenne Muscular Dystrophy (DMD) - Most severe and most common type ○ Muscular dystrophy is a group of inherited, progressive degenerative diseases that cause muscle fiber degeneration and muscle weakness and atrophy. ○ Risk factors: X-linked recessive inheritance pattern ○ Mutation present in a gene that codes of dystrophin Dystrophin is a protein that strengthens muscle fibers and prevents them from injury as muscles contract Found primarily in skeletal/ voluntary muscles that control movement Fat and connective tissue replace the degenerated nerve fibers ○ Onset between 3y and 5y- Preschool age ○ Clinical manifestations Normal development, early in life First seen in extremities and trunk Progressive generalized weakness starting around 3-5 years old Progressive muscular weakness, wasting and contractures occur Gower’s sign First signs are often difficulty in running, riding a bike and climbing stairs Calf muscle hypertrophy Possible cognitive disabilities due to dystrophin in brain tissue Eventual death from respiratory or cardiac failure ○ Complications Disease progresses rapidly Contractures and joint deformity Scoliosis Atrophy of disuse Infections Obesity Cardiac complications By age 12, children usually cannot walk. ○ Diagnosis  Dx based on clinical manifestations, and serum levels of dystrophin, muscle biopsy. CK levels rise before muscle weakness becomes severe. Prenatal diagnosis Ethical considerations ○ Interventions No effective cure or treatment Research Treatment is symptomatic Very multidisciplinary approach involving multiple disciplines Glucocorticoids (Prednisone or deflazacort) may be used to help with muscle strength, motor function, pulmonary function, scoliosis and loss of ambulation Maintain optimum function for as long as possible and prevent contractures May have surgery for muscle lengthening for contractures. Respiratory devices Week 10: Oncology & Hematology Oncology: Complications of oncologic therapy/treatment and their management ○ Infection: Chemotherapy decreases the ability to fight infection; significantly increased risk of infective illness, with decreased ability to fight infection ○ Tumor Lysis Syndrome : Rapid release of intracellular contents that occurs with malignancies with large tumor burdens ○ Hyperleukocytosis: Peripheral WBC count >100,000 which can lead to capillary obstruction, microinfarction, or organ dysfunction Often found on diagnosis ○ Superior Vena Cava Syndrome: Space-occupying lesions can cause compression of the SVC ○ Spinal Cord Compression: Malignancies impinge on the spinal cord causing cord compression ○ DIC: Sepsis and overwhelming infection can cause excessive microthrombi and hyperactivation of the clotting cascade, downregulation of anticoagulants and impaired fibrinolysis ○ mgmt Pain- Either from cancer or treatment Nausea and vomiting Altered nutrition Alopecia: hair loss  Mucosal irritation: Ulcers occurring anywhere along the GI alimentary tract due to radiation or chemo Anemia and bleeding: avoid aspirin (can increase bleeding), avoid contact sports with low PLT counts Neurotoxicity: Numbness, weakness, gait disturbances Infection- due to myelosuppression; monitoring for fever, avoiding sick contacts Leukemia ○ In a healthy child, bone marrow makes blood stem cells that mature and become lymphoid or myeloid stem cells, In leukemia, there is unrestricted proliferation of immature white blood cells in the blood-forming tissues of the body (myeloblasts or lymphoblasts) ○ Classified by the type of WBCs that become neoplastic and commonly divided into two groups: Acute lymphoblastic leukemia [ALL] acute myelogenous leukemia [AML] ○ Most common form of pediatric cancer, peak onset 3-5 years old ○ manifest Early: Anorexia, irritability, lethargy, chronic infections Late: Fever, shortness of breath, bleeding, pallor, petechiae Bone pain, limp or refusal to walk due to neoplastic tissue in medullary spaces Lymphadenopathy, hepatosplenomegaly, CNS infiltration ○ dx Labs: CBC Bone marrow biopsy, LP to determine CNS involvement ○ tx Treatment: Chemotherapy, Intrathecal chemotherapy, radiation, bone marrow transplant Hodgkins/Non-Hodgkin’s lymphoma ○ Cancer of the lymphoid system, cellular proliferation of the lymphoid tissue. ○ difference = type of abnormal lymphocyte cell called Reed- Sternberg cells. ○ Hodgkin’s Lymphoma: Develops in a single lymph node (often cervical or supraclavicular) Contains Reed-Sternberg cells in lymph node biopsy Readily curable (95%) Prolonged onset Common in adolescents ○ Non-Hodgkin’s Lymphoma:  Occurs in the peripheral lymph nodes and spreads throughout the body Chest and mediastinum may be involved Quick onset Thought to be potentially caused by Epstein Barr virus, HIV (immunosuppression increases risk) More common in children under 15 ○ Signs and symptoms: Firm, non-tender lymph nodes, often in cervical or supraclavicular region (HL) May have pressure due to enlargement of adjacent lymph nodes, including airway obstruction, intestinal obstruction, cranial nerve palsies and spinal paralysis Fever, weight loss, night sweats, cough, abdominal discomfort, anorexia, nausea, pruritus. ○ Diagnosis History and Physical, Imaging (lymphangiogram), lymph node biopsy ○ Treatment: Chemotherapy, Radiation Osteosarcoma ○ Cancer in bone tissue ○ Starts in immature bone cells that normally form new bone tissue (osteoblasts) ○ Commonly in the ends of long bones: arms and legs ○ Most common in 2nd decade of life during growth spurt (Late adolescence is peak, rare in childhood) ○ Signs and symptoms Pain localized to the site Mass (not early on), limping, decreased range of motion Broken bone that occurs without injury or minimal injury ○ Diagnosis Imaging- X-ray, CT, MRI Biopsy for definitive diagnosis ○ Treatment: Chemotherapy + Surgery Chemotherapy- pre and post surgery Surgery -Limb salvage vs. Amputation Brain tumors ○ Signs and symptom-related to the size and location of the tumor May cause s/s of increased ICP (headache on awakening, nausea, vomiting, Increased head circumference in infants) ○ Diagnosis  Symptoms- History from parents Imaging- MRI Gold standard, CT ○ Treatment: Surgery, radiation and/or chemotherapy Neuroblastoma vs nephroblastoma (Wilm’s) ○ nephroblastoma (wilms) Malignancy that occurs in the kidneys or abdomen- originated during fetal development, typically found around age 3-4 years Usually is unilateral but can be bilateral (10% of cases) Signs and symptoms: Most common is a firm, non-tender abnormal mass Hypertension (due to changes in renin-angiotensin aldosterone system) Hematuria Fever (unknown reason) Specific RN Considerations: Do not palpate the abdomen (pearl) ○ Could burst it/spread Caution with handling Ensure that the blood pressure is obtained as ordered; manual pressures indicated Strict I/Os to ensure they are urinating. Monitor nutritional intake. Loose clothing around waistband diagnosis Imaging- Abdominal US, CT scan, MRI tx Surgery→ Remove the affected kidney May use radiation and chemotherapy pre- op if both kidneys are involved to decrease the size of the tumor and preserve 1 kidney May use radiation/chemotherapy after surgery for large tumors, metastasis Favorable prognosis above 90% ○ neuroblastoma Malignancy that occurs in the developing cells of the sympathetic nervous system Most tumors in the abdomen bc they arise from the adrenal gland Can be in the chest, neck, or pelvis Often spreads to bone marrow, bones, lymph nodes Disease of infancy and early childhood- common under age 2  Signs and symptoms Palpable abdominal mass that crosses the midline, firm-non tender Fever, fatigue 75% of patients present with metastases at diagnosis Diagnosis: CT scan Treatment: Surgical removal, chemo and/or radiation Heme: Anemia: screening; treatments and considerations ○ Reduction of RBC volume and/or Hgb concentration to levels below normal for age ○ Basic causes 1. Excessive red blood cell loss 2. Increased destruction of RBCs 3. Impaired or decreased rate of RBC production Bone marrow failure Deficiency of essential nutrients ○ Manifestations All symptoms are related to a decrease in O2 carrying capacity of blood Muscle weakness Fatigue, inability to concentrate Palpitations, tachycardia Dyspnea on exertion Pallor Craving for non-nutritive substances: PICA Dry brittle nails Concave or spoon-shaped nails CNS manifestations: Headache, dizziness, lightheadedness, irritability slowed thought processes, decreased attention span, apathy, depression Growth retardation ○ Diagnosis Pale conjunctiva Screening, check hgb every few yrs Labs: CBC and iron studies Reticulocytes Iron studies Bone Marrow aspiration  Normal Hgb by age: 1-3 days – 14.5-22.5 g/dl 2 mos – 9-14 g/dl 6-12 yrs – 11.5-15.5 g/dl 12-18 yrs male – 13-16 g/dl 12-18 yrs female – 12-16 g/dl o Micro-hemorrhage can cause GI bleed in children who drink cows milk at 6 mos o Therapeutic management ▪ Prevention Screening only breast milk or formula first 12 months, Iron supplements as prescribed, Limit milk intake (cow's milk low in effective iron) Iron-rich foods ▪ Administer prescribed therapy - Ferrous sulfate 2 or 3 divided doses daily Between meals if tolerated ▪ Parent education Take with Vitamin C to enhance absorption Brush teeth to minimize staining Watch for side effects vomiting, constipation, dark green or black stools, tooth discoloration Dietary counseling - Blood transfusion administration/management o take vs inc bp, 15 mins after initiation, houring w/infusion, done w infusion o Admin first 50ml of blood or initial 20% of the volume (whichever is smaller) slowly and stay with the child. o Administer with NS on a piggyback setup/ have available o Administer blood through appropriate filter o Use blood w/in 30 mins arrival; if not used, return to blood bank o Infuse a unit of blood (or the specified amount) w/in 4 hours. If infusion exceeds this time, blood should be divided into appropriate-size quantities by blood bank and unused portion refrigerated under controlled conditions. o If reaction suspected, stop transfusion, take vs, maintain IV line w ns + new tubing, tell doc, dont start transfusion until child’s condition has been med evaluated. o Signs of a transfusion reaction ▪ Sudden, severe headache ▪ Chills ▪ Shaking  ▪ Fever ▪ Pain at needle site and along ▪ venous tract ▪ Nausea and vomiting ▪ Sensation of tightness in chest ▪ Red or black urine ▪ Flank pain ▪ Hives - Iron deficiency anemia o Anemias caused by nutritional deficiencies: iron deficiency anemia (IDA) ▪ Anemia caused by inadequate supply of iron essential for normal red blood cell production ▪ Patho: Iron req to produce hgb, ↓ fe = ↓ hemoglobin ▪ Etiology: Can be caused by numerous factors including: Low fe stores at birth, maternal fe stores in newborn become depleted inadequate dietary intake of iron ▪ Prominent in age groups with rapid growth (toddlers, adolescents) ▪ Typically occurs between 9 months and 2 years ▪ Clinical manifestations of IDA in infants: “Milk baby” appearance, glossitis (inflammation of tongue), angular stomatitis (inflammation and small cracks in one or both corners of the mouth), koilonychia (soft concave or “spoon” fingernails), impaired neurocognitive function - Enteral Iron administration o s/s: constipation - Sickle cell disease & crisis o Group of hereditary disorders in which there is the presence of abnormal hemoglobin S, Autosomal recessive genetic disorder o Pathology: In states of dehydration, acidosis, hypoxia or extreme temperature, abnormal hemoglobin sickles RBC and causes obstruction and increased RBC destruction o RBCs become sickle shaped when triggered and cannot slide through vessels → Clumping, thrombosis, arterial obstruction, increased blood viscosity o Sickle shape leads to entanglement of cells→ hemolysis, intermittent vascular occlusion→ tissue ischemia → organ dysfunction o Abnormal shape of cells cant carry oxygen o Clinical manifestations ▪ General:  Possible growth retardation and delayed sexual maturation Chronic anemia (Hgb 6-9 g/dl) Susceptibility to infection/sepsis ▪ Spleen: Enlargement due to congestion ▪ Liver: Failure and necrosis due to impairment of blood flow ▪ Kidney: Impairment due to congestion hematuria, inability to concentrate urine, enuresis and occasionally nephrotic syndrome ▪ Eyes: Retinopathy ▪ Extremities: Bone changes due to hyperplasia of bone marrow, susceptible to osteomyelitis ▪ CNS: Stroke or CVA ▪ Heart: Chronic stress from anemia, cardiomegaly, murmur o Sickle cell disease crises ▪ Types of crises: Vaso-occlusive Crisis (VOC): Ischemia causes pain o Significant pain in areas involved o Manifestations depend on area involved o Extremities: Dactylitis (primarily 6mon- 2yr) o Abdomen: Severe pain resembling surgical conditions o Acute splenic sequestration ▪ Pooling of blood; can cause decreased blood volume, ultimately shock ▪ Patients may require a splenectomy to avoid splenic sequestration o Treatment and nursing considerations ▪ Aims of treatment: Prevent sickling phenomenon ▪ Treat medical emergency of sickle cell crisis Pain relief Adequate hydration Improve oxygen utilization → limit activity Transfusions for anemia Antibiotics to treat infections Oxygen therapy ▪ Prevent infections and educate parents Prophylactic PCN (before 3 months old and through 5 years) to reduce risk of pneumococcal infection[; - Hemophilia A & B ▪ Definition: Hemophilia refers to a group of inherited bleeding disorders caused by a deficiency of certain coagulation factors ▪ Hemophilia A (Classic Hemophilia): Deficiency of factor VIII (8) Occurrence- 1:5,000 Accounts for 80% of cases of hemophilia ▪ Hemophilia B (Christmas Disease): Deficiency of factor IX (9) Occurrence- 1: 20,000-30,000 ▪ Genetics Inheritance pattern: o X linked recessive o Only about 60% have a family history of the disease o The rest may be caused by a spontaneous genetic mutation ▪ Clinical manifestations Major effect: Prolonged bleeding anywhere from or in the body Symptoms can range from mild to severe (table below) Hemarthrosis: Bleeding into the joint cavities Ecchymosis Spontaneous hematuria Epistaxis Hemorrhages anywhere in the body Severe hemophilia will likely need factor VIII intravenous administration (sometimes able to do at home pending case) ▪ Diagnostic testing Generally diagnosed based on history of bleeding episodes, X-linked inheritance pattern and laboratory findings Labs: o Normal: Platelet count, PT, and fibrinogen o Abnormal: Low Factor VIII or IX levels, prolonged PTT Genetic testing and carrier testing indicated for family members ▪ Therapeutic management Replace missing clotting factors Transfusions of factor XIII or IV given intravenously at home with first signs of bleeding o Prophylactic factor given to severe hemophiliacs  Synthetic form of vasopressin (DDAVP) given intravenously at home for mild Hemophilia A o DDAVP causes 2 to 4 times increase in factor VIII activity Pain Management o Acetaminophen as needed for pain o Aspirin and ibuprofen usually avoided as they can inhibit platelet function Regular exercise and play; Encourage full ROM ▪ Nursing interventions Family support Prevent bleeding Recognize and control bleeding immediately Prevent crippling effects of bleeding o Q: Which of the following should the nurse teach the parent of a child with severe hemophilia to do first if the child sustains an injury to a joint causing bleeding? ▪ a. Apply heat ▪ b. Administer factor per the home-care protocol ▪ c. Give ibuprofen ▪ d. Immobilize the area o Q: A child with hemophilia fell while riding his bicycle. He was wearing a helmet and did not lose consciousness. He has a mild abrasion on his knee that is not oozing. He is complaining of abdominal pain. What is the priority nursing assessment? ▪ a. Perform neurologic checks ▪ b. Assess the ability to void frequently ▪ c. Carefully assess his abdomen ▪ d. Examine his knees frequently