NURS 4352 PEDS Test 3 24- The Child with Hematologic or Immunologic Dysfunction

1 NURS 4352 PEDS Test 3 24- The Child with Hematologic or Immunologic Dysfunction Anemia  The most common hematologic disorder of childhood  Decrease in number of red blood cells (RBCs) and/or hemoglobin (Hgb) con ...

1 NURS 4352 PEDS Test 3 24- The Child with Hematologic or Immunologic Dysfunction Anemia  The most common hematologic disorder of childhood  Decrease in number of red blood cells (RBCs) and/or hemoglobin (Hgb) concentration below normal  Decreased oxygen-carrying capacity of blood  Consequences of Anemia: When anemia develops slowly, child adapts  Effects of Anemia on the Circulatory System o Decreased peripheral resistance o Increased cardiac circulation and turbulence:  May have murmur  May lead to cardiac failure o Cyanosis o Growth retardation  Diagnostics o CBC:  Decreased RBCs  Decreased Hgb and hematocrit (Hct) o Other tests for particular type of anemia Management of anemia  Treat underlying cause: o Transfusion after hemorrhage if needed o Nutritional intervention for deficiency anemias  Supportive care: o IV fluids to replace intravascular volume o Oxygen o Bed rest  Nursing Considerations o Prepare child and family for laboratory tests2  Explain significance of each test, particularly why the tests are not all done at one time  Encourage parents or another supportive person to be w/the child during procedure  Allow child to play w/equipment on a doll or participate in actual procedure o Decrease oxygen demands  Continuous assessment of child’s energy levels o Prevent complications  Prone to infections b/c tissue hypoxia causes cellular dysfunction that weakens the body’s defense against infectious agents  Observe for s/s of infection (temp elevation, and leukocytosis) o Support family Iron deficiency anemia  Pathophysiology o Caused by inadequate supply of dietary iron o Generally preventable:  Iron-fortified cereals and formulas for infants  Special needs of premature infants  Adolescents at risk due to rapid growth and poor eating habits  Therapeutic management o Focus is on increasing the amount of supplemental iron  Administration of iron supplements  Dietary interventions  Nursing considerations o Teaching for iron administration o Given in two doses between meals o Taking with citrus of fruit juice aides in absorption. Cow’s milk can interfere with absorption o Ingestion of excessive quantities can be fatal o Stool may turn tarry green or black o If taken in liquid form, may cause discoloration to the teeth  Prognosis: Early recognition is key and prognosis is very good for a child with iron deficiency anemia.  Quality Outcomes: o Early recognition o Appropriate quantity of milk, use of iron fortified formula, and introduction of solid foods. o Adherence to oral iron supplements with the appropriate administration. o Hemoglobin increase within 1 month and anemia resolved in 6 months. Sickle cell anemia  One of a group of diseases in which normal adult hemoglobin (Hbg) is partly or completely replaced by abnormal sickle Hgb. Autosomal recessive disorder meaning that both parents have the sickle cell trait.  Clinical features primarily a result of: o Obstruction caused by the sickled RBCs o Vascular inflammation o Increased RBC destruction  Diagnostic Evaluation o Newborn screening o Sickle-turbidity test (Sickledex) o Hemoglobin electrophoresis  Therapeutic management o Prevent the sickling phenomena o Treat the medical emergencies  Acute chest syndrome (ACS) symptoms include:  Severe chest, back, or abdominal pain  Fever of 38.5 Celsius or 101.3 Fahrenheit or higher3  Cough  Dyspnea, tachypnea  Retractions  Declining oxygen saturation (oximetry)  Cerebrovascular accident (CVA) symptoms include:  Severe unrelieved headaches  Severe vomiting  Jerking or twitching of the face, legs, or arms  Seizures  Abnormal behavior  Inability to move extremities  Unsteady gait or stagger  Slurred speech or stuttering  Weakness  Vision changes  Clinical manifestations o General  Possible growth retardation  Chronic anemia (hemoglobin level of 6 to 9 g/dl)  Possible delayed sexual maturation  Marked susceptibility to sepsis o Vasooclusive Crisis  Pain in the area of involvement  Symptoms related to ischemia in the areas involved  Extremities: painful swelling of hands, feet, or joints.  Abdomen: Severe pain that can resemble an acute surgical condition.  Cerebrum: stroke, visual disturbances.  Chest: symptoms that can resemble pneumonia or a pulmonary disease.  Liver: obstructive jaundice, hepatic coma  Kidney: Hematuria  Genitalia: Priapism  Complications o Sequestrian Crisis: Pooling of large amounts of blood that can result in:  Hepatomegaly  Splenomegaly  Circulatory collapse o Chronic Vasooclusive Phenomena:  Heart: Cardiomegaly, systolic murmurs  Lungs: Altered pulmonary function and increased susceptibility to infection  Kidneys: Enuresis and progressive renal failure  Liver: Hepatomegaly, cirrhosis, intrahepatic cholestasis  Spleen: Splenomegaly and increases susceptibility to infection  Eyes: Visual disturbances and possible progressive retinal detachment and blindness  Extremities: Avascular necrosis of the hip or shoulder, skeletal deformities, chronic leg ulcers, and susceptibility to osteomyelitis  Central nervous system (CNS): Hemiparesis, seizures  Additional complications o Aplastic crisis: diminished red blood cell production (RBC) triggered by a viral infection. o Hyperhemolytic crisis: accelerated rate of RBC destruction that is characterized by jaundice, anemia, and reticulocytosis. o Acute chest syndrome: Clinical manifestations may look similar to pneumonia. The presence of a pulmonary infiltrate that can be associated with fever, chest pain, cough tachypnea, wheezing, and hypoxia (Review nursing alert on page 798 of the textbook). o Cerebrovascular accidents (CVA): stroke related to sickled cells blocking major blood vessels in the brain.4  Medical management o Hydration o Rest to minimize energy expenditure o Electrolyte replacement o Pain control for vasoocclusion o Blood replacement o Antibiotics to treat any existing infection  Nursing Management o Educating the client and family o Health promotion o Symptom identification and management o Providing client and family support  Prognosis o Can vary based on patient response to treatment but most patients will live into their fifties.  Quality Outcomes o Early recognition of signs and symptoms o Tissue deoxygenation minimized o Sickle cell crisis prevent or managed quickly o Pain managed appropriately o Stroke prevented o Prophylactic penicillin regimen followed o Hypoxia prevention when surgery is necessary o Pneumonia, Influenza, and Meningitis vaccines administered Beta-thalassemia  A common genetic disorder characterized by deficiencies in the production of specific globin chains in hemoglobin (Hgb).  Results in defective Hgb formation that are unstable causing it to disintegrate and when it does it damages RBC’s causing severe anemia.  Diagnostic evaluation: Hematology studies  Therapeutic management: o Transfusion administration to maintain a Hgb level above 9.5 g/dl and chelation therapy. o Goal: to maintain sufficient Hgb to support normal growth, physical activity, and to prevent bone marrow expansion and body deformities.  Nursing Management: o Promote compliance o Client and family support o Monitoring for complications o Providing Education o Assist the child with coping Aplastic anemia  A rare life-threatening disorder that refers to bone marrow failure that caused formed elements in the blood to be simultaneously depressed.  Diagnostics: blood smear  Clinical manifestations include pancytopenia  Therapeutic management: o Immunosuppresive therapy o Bone marrow transplantation  Nursing care management: o Education o Procedure preparation o Precautions and care for pancytopenia5 o Symptom management Hemophilia  Hemophilia A o Classic hemophilia o Deficiency of factor VIII o Accounts for 80% of cases of hemophilia o Occurrence: 1 in 5000 males  Hemophilia B o Caused by deficiency of factor IX o Accounts for 15% of cases of hemophilia  Etiology o X-linked recessive trait o Males are affected o Females may be carriers o Degree of bleeding depends on amount of clotting factor and severity of a given injury o Up to a third of cases have no known family history  In these cases disease is caused by a NEW mutation  Clinical manifestations o Prolonged bleeding from or within the body o Symptoms may not occur until 6 months of age:  Mobility leads to injuries from falls and accidents o Hemarthrosis:  Bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility o Hematomas:  Swelling, warmth, redness, pain, and loss of movement o Spontaneous hematuria o Excessive bruising even from a slight injury o Epistaxis (nose bleed) o Bleeding after procedures:  Minor trauma, tooth extraction, minor surgeries  Large subcutaneous and intramuscular hemorrhages may occur  Bleeding into neck, chest, mouth may compromise airway  Diagnostics o Can be diagnosed through amniocentesis o Genetic testing of family members to identify carriers o Diagnosis on basis of history, laboratory studies, and examination  Labs: low levels of factor VIII or IX, prolonged PTT  Normal: platelet count, PT, and fibrinogen  Prognosis: o In the past the prognosis was poor with most patients dying by the age of 5 years, but now mild to moderate hemophilia patients live near normal lives o Gene therapy for future o Infused carrier organisms act on target cells to promote manufacture of deficient clotting factor  Quality outcomes: o Early recognition of signs and symptoms o Bleeding episodes prevented o Bleeding episodes treated early with factor replacement o Adherence to prophylactic factor replacement program when indicated o Hemarthrosis prevented when possible to limit joint damage o Exercise program and physical therapy ongoing.  Management: o DDAVP: synthetic form of vasopressin  IV6  Causes 2-4 times increase in factor VIII activity  Used for mild hemophilia o Replace missing clotting factors (primary)  Transfusions  At home with prompt intervention to reduce complications  Following major or minor hemorrhages o Interventions  Close supervision and safe environment  Dental procedures in controlled situation  Shave only with electric razor  Superficial bleeding: apply pressure for at least 15 minutes and ice to vasoconstriction  If significant bleeding occurs, transfuse for factor replacement  Hemarthrosis Management: o During bleeding episodes, elevate and immobilize joint o Ice o Analgesics o Range-of-motion exercises after bleeding stops to prevent contractures o Physical therapy o Avoid obesity to minimize joint stress Idiopathic thrombocytopenia purpura (ITP)  ITP is an acquired hemorrhagic disorder characterized by: o Thrombocytopenia o Absence or minimal signs of bleeding o Normal bone marrow with normal or an increase in the number of immature platelets and eosinophils.  Can be either acute or chronic  Diagnostics: Based on clinical manifestations and laboratory testing.  Therapeutic Management: o Primarily supportive o In acute cases:  Prednisone, IV immunoglobulin, and anti-D antibody  Nursing Management: o Education: avoidance of activities that increase the risk for serious bleeding o Prevention of bleeding episodes o Treatment administration Epistaxis (nosebleed)  Isolated and transient epistaxis is common in childhood  Recurrent or severe episodes may indicate underlying disease  Epistaxis Management o Remain calm, keep child calm o Have child sit up and lean forward o Pressure to nose o Further evaluation if bleeding continues Immunologic deficiency disorders  Severe Combined Immunodeficiency Disease (SCID): characterized by the absence of both humoral and cell mediated immunity. o Most common manifestation is susceptibility to infection early in life, often within the first month. o Diagnosis: recurrent severe infections in early infancy, family history of the disorder and labs. o Therapeutic management:  Hematopoietic stem cell transplantation (HSCT) o Nursing management:7  Focuses on infection prevention  Symptom management  Education  Family and client support  Wiskott-Aldrich Syndrome o Congenital x-linked recessive disorder characterized by a triad of abnormalities  Thrombocytopenia  Eczema  Immunodeficiency of selective functions of the B lymphocytes and T lymphocytes. o Medical treatments:  Platelet transfusions  IVIG infusions to provide passive immunity  Prophylactic antibiotics  Aggressive local therapy for eczema o Nursing management:  Mainly involves supportive the family and the client HIV/ AIDS  Defined as a virus that affects the T lymphocytes, CD4 and T cells causing them to reach critical levels that leads to a risk of opportunistic illness that is followed by death.  Common clinical manifestations include: o Lymphadenopathy o Hepatosplenomegaly o Oral candidiasis o Chronic or recurrent diarrhea o Failure to thrive o Development delay o Parotitis  Defining conditions o Pneumocystis carinii pneumonia (PCP) o Lymphoid interstitial pneumonitis (LIP) o Recurrent bacterial infections o Wasting syndrome o Candida esophagitis o HIV encephalopathy o Cytomegalovirus disease o Mycobacterium avium-intrecellulare complex infection o Pulmonary candidiasis o Herpes simplex disease o Cryptosporidiosis  Diagnostic evaluation: HIV enzyme-linked immunosorbent assay and Western blot immunoassay.  Therapeutic management: o Slowing down the growth of the virus. o Preventing and treating opportunistic infections. o Providing nutritional support o Symptomatic treatment.  Nursing Management: o Prevention education o Health promotion o Encouraging adherence to therapy. o Supporting the client and family Apheresis and blood transfusion therapy8  Obtain a full set of vital signs o Pretransfusion o 15 minutes after initiation o Hourly while infusing o And on completion  Always use 2 patient identifiers  Administration requires 2 registered nurses  Begin administration slowly and stay with the client for the first 15 minutes and monitor for any reaction (review table 24-3 in the textbook for nursing care of the child receiving blood transfusions).  Have normal saline available  Only administer using the appropriate blood tubing.  Use blood within 30 minutes of arrival.  Infuse the blood within 4 hours.  If any reaction is suspected: o Stop the transfusion o Assess vital signs o Maintain patent IV line with normal saline and new tubing. o Notify the provider  Apheresis: o Removal of blood from a client, separation of the blood into its components, retention of one or more of these components, and reinfusion of the remainder of the blood into the client. Chapter 25- The Child with Cancer Cancer overview  Childhood cancer is rare and the incidence can vary according to age, sex, and race. Although there have been numerous studies completed and many in progress as we speak, there is no known method of prevention at this time.  Cardinal symptoms of cancer in children can include: o An unusual mass or swelling o Unexplained paleness and loss of energy o Persistent localized pain and or limping o Prolonged, unexplained fever or illness o Frequent headaches often accompanied with vomiting o Sudden eye or vision changes o Excessive, rapid weight loss  Diagnostics: o Laboratory tests o Diagnostic procedures o Imaging o Pathologic evaluation  Treatment modalities o Surgery o Chemotherapy o Radiotherapy o Biologic Response Modifiers o Blood or Marrow Transplantation  Nursing Management: Education, treatment administration, pain management, procedure preparation, health promotion, and assisting the patient and family with coping. Cancers of the blood and lymph systems  Leukemia- malignant disease of the bone marrow and lymphatic system. Includes an unrestricted proliferation of immature white blood cells in the blood-forming tissues of the body.9 o Two types  Acute Lymphoblastic Leukemia (ALL)- most common form of childhood cancer  Acute Myelogenous Leukemia (AML) o Bone marrow dysfunction: in all types, bone marrow production of the formed elements of the body is depressed which deprives the normal cells of the essential nutrients needed for metabolism resulting in the following.  Anemia that results from decreased erythrocytes  Infection from neutropenia  Bleeding caused by decreased platelet production o Onset: in most cases there are few symptoms. The parent may be concerned when the child begins to become pale, listless, irritable, febrile, anorexic, joint pain and have unexplained bruising. o Prognosis: factors to determine long-term survival are the initial white blood cell count the patient’s age at diagnosis, cytogenetics, immunologic subtype, and the child’s sex. o Diagnostics:  History and physical manifestations  Peripheral blood smear  Low blood counts  Definitive diagnosis is completed through bone marrow aspiration or biopsy  Leukemia mgmt. o Therapeutic Management: 3 phases: Induction, Intensification, and Maintenance  Remission Induction: induction therapy lasts for 4 to 5 weeks and patients are at high risk of infection and spontaneous hemorrhage during this period.  Intensification or Consolidation Therapy: Consists of pulses of chemotherapy medications that are given during the first 6 months of treatment.  Maintenance: The goal of this therapy is the preserve remission and further reduce the number of leukemic cells. During this therapy weekly or monthly complete blood counts are taken to test the marrow’s response to the drugs.  Central Nervous System (CNS) Prophylactic Therapy: The CNS is at high risk for invasion and this mode of therapy is usually reserved for high-risk patients or those with resistant CNS disease.  Reinduction after Relapse: Additional therapy may be necessary when a relapse occurs. With each relapse it indicates an increasing poor prognosis.  Blood or Marrow Transplantation (BMT): Has been successful in treatment of ALL, but BMT is indicated for those that are high risk of have poor early therapy response.  Consequences of Leukemia: o Anemia from decreased red blood cells o Infection from neutropenia o Bleeding tendencies from decreased platelet production o Spleen, liver, and lymph glands show marked infiltration, enlargement, and fibrosis  Nursing Management o Care is directly related to the regimen of therapy.  Symptom management: myelosuppression, drug toxicity, leukemic infiltration.  Infection control: Visitor restriction, environment.  Procedure preparation:  Will include multiple tests, the most traumatic being bone marrow aspiration of biopsy along with lumbar punctures. Be sure to prepare the child and family as well as educate them.  Providing emotional Support:  Adequately preparing patients for treatment regimen beforehand  Providing reassurance  Providing education and guidance so the parents can recognize symptoms that require immediate medical attention. Lymphomas- hodgkins and non-hodgkins  Lymphomas- neoplastic disease from the lymphoid and hematopoietic systems10  Two types: Hodgkins and Non-hodgkins Lymphoma (NHL)  Hodgkins: o Originates in the lymphoid system and predictably metastasizes to non-nodal sites, tissues and organs. o Clinical manifestations:  Painless enlargement of lymph nodes  Other symptoms will depend on the extent and location of involvement.  Systemic symptoms: low-grade or intermittent fever, anorexia, nausea, weight loss, night sweats, and pruritus. o Diagnostic Evaluation:  History and physical  Labs  Radiographic tests o Therapeutic Management:  Chemotherapy and irradiation o Nursing Care Management:  Preparation for diagnostics/procedures, explanation of side effects, symptom management, and providing the child and family support.  Non-Hodgkins: o Clinical manifestations:  Will vary based on the anatomic site and the extent of involvement. o Diagnostic Evaluation:  History and physical  Surgical biopsy  Bone marrow aspiration  Radiographic tests  Lumbar puncture o Therapeutic Management:  Chemotherapy and irradiation o Nursing Care Management:  Preparation for diagnostics/procedures, explanation of side effects, symptom management, and providing the child and family support. Brain tumors  Most common solid tumor in children  2nd most common childhood cancer  Typically glial or neuronal in origin  Three Main types: o Infratentorial o Supratentorial o Suprasellar region  Clinical manifestations: o Signs and symptoms will be directly related to the anatomic location and size and may also depend on the child’s age. o Some common symptoms of an infratentorial brain tumor includes headache especially on awakening and vomiting not related to feeding.  Diagnostic Evaluation: o Clinical symptoms o Diagnostic imaging  MRI  CT Scan  Angiography  EEG  Lumbar puncture (LP) o Histology11  Nursing Management o Baseline assessment o Assessment: Head circumference, monitor for headache, vomiting, seizure activity, and assess gait daily o Family Support; Procedure and surgery preparation o Prevent post-op complications  Frequent vital signs, attention to temperature  Neurologic assessments  Monitor for increased intracranial pressure, meningitis, respiratory tract infection  Monitor dressings, drainage, drains  Nutrition  Pain management  Comfort management  Bowel regimen  Fluid Regulation  Positioning  Therapeutic Management: o Surgery o Radiotherapy o Chemotherapy o Proton radiation Neuroblastoma  Neuroblastoma : Is a “silent tumor” and diagnosis is usually made after metastasis takes place and symptoms arise at the nonprimary site.  Clinical Manifestations: o Signs and symptoms will depend on the location and the stage of the disease. o Ex: abdominal tumor will have a firm, nontender, irregular mass that crosses the midline.  Diagnostics: aimed at locating the primary site and areas of metastasis o Radiology tests (CT scan, bone scan). o Bone marrow biopsy or aspiration o Urine tests (may have urinary excretion of catecholamines).  Therapeutic management: o Accurate clinical staging is important to determine the initial treatment o Stages 1 and 2 involve complete surgical removal. o Surgery is limited to biopsy in stages 3 and 4 o Chemotherapy may also be used.  Nursing Management: o Procedure preparation o Management of clinical symptoms o Education o Family support Bone tumors  Two types: o Osteosarcoma (most common bone tumor) o Ewing sarcoma  Clinical manifestations: o Localized pain in the affected site o Diagnostics o History and physical o Radiologic studies provide a definitive diagnosis o Needle or surgical biopsy (Ewing sarcoma)  Osteosarcoma12 o Therapeutic management  Surgery and chemotherapy o Nursing management  Will depend on the surgical approach  The child may need education and support regarding  Prosthesis  Chemotherapy  Stump care (phantom lib pain)  Grieving from loss of limb  Ewing Sarcoma: may arise in the marrow spaces rather than from the osseous tissue. o Therapeutic management:  Radiotherapy and chemotherapy  Limb salvage procedures may be feasible in extremity lesions o Nursing Management:  Preparation for various procedures  Encouraging extremity use (physical therapy)  Support for adjustment to changes. Wilms tumor  Etiology: malignant, undifferentiated cluster of primordial cells  Clinical manifestations: o Abdominal mass – firm, non-tender, confined to one side o Abdominal enlargement o Weight loss o Fatigue / malaise o Fever o Possible hematuria and hypertension o Secondary manifestations if metastasis  Diagnostic evaluation: o History and physical o Abdominal ultrasound o Abdominal and chest CT/MRI o Blood work and urinalysis  Therapeutic Management: biopsy, surgery; chemotherapy; possible radiation  Nursing Management: o Preoperative care:  Preparation of child and family for labs and procedures  Monitor vital signs  *Do not palpate abdomen / tumor*  Family education about post op expectations o Postoperative care:  Monitor - GI function, BP, urine output, signs of infection, and vital signs  Provide pain relief  Pulmonary hygiene  Family support Chapter 27- The Child with Cerebral Dysfunction Pediatric differences  A child with open fontanels can compensate for increased volume through widening of sutures and skull expansion, however spatial compensation can be limited. Once all compensation is exhausted, any further volume increase can result in a rapid increase in intracranial pressure (ICP).13  Most information in infants and small children will be obtained through observing spontaneous and elicited reflex responses  Persistence or reappearing primitive reflexes can be an indicator of a pathologic condition.  Obtain a thorough pregnancy and delivery history as there are intrauterine and extrauterine factors that can influence how the central nervous system matures. Altered states of consciousness  Level of Consciousness (LOC) o Full consciousness: Defined as awareness- the ability to respond to sensory stimuli and have subjective experiences  Arousal: waking state and ability to respond to stimuli  Cognitive power: ability to process stimuli and produce a verbal and motor response. o Unconsciousness: is a depressed cerebral function and includes the inability to respond to sensory stimuli. o Coma: no motor or verbal response to noxious stimuli  Glasgow Coma Scale o Three-part assessment:  Eyes  Verbal response  Motor response o Score of 15: unaltered LOC o Score of 3: extremely decreased LOC (worst possible score on the scale) Nursing implications for the child with cerebral dysfunction  Neurologic Assessment o Level of consciousness (LOC) o Vital signs o Skin o Eyes o Motor function o Posturing o Reflexes  Nursing Implications o Positioning o Exercise o Suctioning o Nutrition and hydration o Medications o Thermoregulation o Elimination o Hygienic care o Sensory stimulation o Family support Increased ICP  Compensatory changes o Blood volume decreases o Cerebrospinal fluid (CSF) increases or decreases o Brain mass shrinks o Skull expansion – depending on fontanels and sutures14  Cause o Tumors or lesions o Accumulation of CSF in ventricular system o Bleeding o Cerebral edema  Clinical manifestations in infants: o Tense, bulging fontanel o Separated cranial sutures o Macewen (cracked pot) sign o Irritability / restlessness o Drowsiness o Increased sleeping o High pitched cry o Increased fronto-occipital circumference o Distended scalp veins o Poor feeding o Crying when disturbed o Setting sun sign (eyes rotated downward)  Clinical manifestations in children: o Headache o Nausea / vomiting o Diplopia, blurred vision o Seizures o Indifference, drowsiness o Decline in school performance o Diminished physical activity & motor performance o Increased sleeping o Inability to follow simple commands o Lethargy  Late signs in infants and children o Bradycardia o Decreased motor response to command o Decreased sensory response to painful stimuli o Alterations in pupil size and reactivity o Flexion (decorticate) & extension (decerebrate) posturing o Cheyne-stokes respirations o Papilledema o Decreased consciousness o Coma  a. Flexion (decorticate)  b. Extension (decerebrate ICP monitoring  Guides therapy to reduce ICP  Provides information on intracranial compliance , cerebrovascular status, and cerebral perfusion  Associated risks o Infection, hemorrhage, malfunction, obstruction  Indications o GCS less than 8 o Traumatic brain injury with abnormal CT scan o Deterioration of condition o Subjective judgment15 Head injury  Cause: o Falls o Motor vehicle injuries o Bicycle or sports related injuries  Risk factors: o Physical characteristics of child o Immature motor development o Natural curiosity and liveliness o Unattended child  Pathophysiology: o Physical forces act through acceleration, deceleration, deformation o Brain strikes inside of skull o Distortion and shearing o Increased blood volume or redistribution of cerebral blood volume  Concussion o An alteration in neurologic or cognitive function with or without loss of consciousness which occurs immediately after a head injury o Most common head injury o Hallmark signs: confusion and amnesia o Resolves 7-10 days o Pathology unclear  Shearing forces  Altered nerve fibers  Contusion o Petechial hemorrhages or localized bruising along the superficial aspects of the brain at either the coup (point of impact) or contrecoup (remote site of impact) o Manifestations:  Focal disturbances in strength, sensation, or visual awareness  Symptoms may be clinically indistinguishable from concussion  Laceration o Brain tissue is torn with bleeding into and around the tear o Manifestations:  Unconsciousness  Paralysis  Scarring on brain  Some degree of disability  Types of fractures o Linear o Depressed o Comminuted o Basilar  Battle sign  Raccoon eyes  Hemotympanum  CSF leakage o Open  Complications  Hemorrhage  Infection  Edema  Herniation  Epidural hemorrhage: Bleeding in the space between the dura mater and the skull16 o Cause: damage to dural blood vessels, particularly the middle meningeal artery o Clinical manifestations:  Momentary loss of consciousness after a head injury followed by a normal period of alertness, then deterioration to lethargy and unconsciousness  Confusion  Dizziness  Drowsiness  Enlarged pupil in one eye  Severe headache  Nausea / vomiting  Weakness in part of the body o Treatment:  Surgical evacuation  Medications: anticonvulsants, corticosteroids, hyperosmotic agents  Subdural hemorrhage: Bleeding between the dura mater and arachnoid membrane o Cause: tearing of blood vessels along the surface of the brain  Associated with severe head injury o Clinical manifestations:  Irritability  Increased sleepiness or lethargy  Feeding difficulties  Vomiting  Increased head circumference  Bulging fontanel and or separated sutures  Seizures o Treatment:  Subdural tap / surgical evacuation  Medications – anticonvulsants, diuretics, corticosteroids  Cerebral edema o Evident within 24 – 72 hours of head injury o Results from increased intracranial volume and changes in cerebral blood flow o Observe for signs of increased ICP Craniocerebral Trauma Diagnostic Evaluation  History o Description of event o Alterations in consciousness o Other signs or behaviors  Physical exam o CAB (Circulation, Airway, Breathing) o Stabilization of neck and spinal cord o Neurological exam: focused on mental status o Pupillary responses o Motor responses  Physical Assessment o Vital signs:  Indications of brainstem involvement  Deep, rapid, periodic, or intermittent & gasping respirations  Wide fluctuations or slowing of heart rate  Widening pulse pressure or extreme fluctuations in blood pressure o Eyes:  Indications of increased ICP and/or brainstem involvement  Fixed, dilated, & unequal pupils  Fixed and constricted pupils17  Poorly reactive or non-reactive pupils  Early sign of increased ICP  Dilated, non-pulsating vessels on funduscopic exam  Retinal hemorrhages – frequent finding in shaken baby syndrome o Scalp:  Lacerations  Abnormalities o Nose and ears:  Bleeding  Watery discharge (rhinorrhea that is glucose positive is suggestive of leasing CSF from a skull fracture)  Diagnostic Tests o CT Scan  Alteration in consciousness, headache, vomiting, skull fracture, seizure, predisposing medical condition o MRI  Cerebral edema, structural brain abnormalities o X-Ray  Not beneficial in identifying skull fractures o EEG  Useful in defining seizure activity Post-traumatic syndromes  Post-concussion syndrome: o Common to brain injury o Develops hours to days after injury, lasts days to months o Nausea, dizziness, headache, diplopia, disorientation  Post traumatic seizures: o More common in children than adults o More likely to occur within first few days of severe head injury  Structural complications: o Depends on location and nature of trauma o Cognitive deterioration, motor deficits, optic atrophy, cranial nerve palsies, aphasia Traumatic brain injury mgmt.  Therapeutic Management o Mild head injury  Cared for and observed at home  Provide verbal and written instructions  Signs and symptoms to report  Check child every 2 hours for changes in responsiveness  Follow up with PCP in 1-2 days o Severe head injury  Hospitalization until stable  NPO, IV fluids  Close monitoring of fluid balance  Medications o Sedatives – not in acute phase o Acetaminophen, opioids o Anticonvulsants o Antibiotics o Corticosteroids  Surgery18 o Suture of lacerations or torn dura o Reduction of fracture / removal of bone fragments  Nursing Care Management o Frequent VS, neurological assessments o Bed rest, HOB elevated, head position, side rails up o Seizure precautions o Quiet environment o Sedation and analgesia o Observing movement o Observe for drainage from body orifices o Assess for and report additional body markings o Observe behavior o Family support o Rehabilitation o Prevention education Meningitis  Causative organisms: o Bacterial (septic, contagious) o Viral (aseptic) o Tuberculin  Review box 27-4 pg. 891 for clinical manifestations  Diagnostic Procedures (review preparation in ATI textbook chapter 12 and remember to consider developmental age and atraumatic care for procedures) o Lumbar puncture o CT Scan or MRI  Nursing interventions: o Place the patient on droplet precautions o Monitor: vital signs, monitor intake and output, and neurologic status o Monitor for signs of increased intracranial pressure and treat appropriately. o Decrease environmental stimuli o Provide comfort o Medications: antibiotics (bacterial infections), corticosteroids, and analgesics Reye syndrome  Cause: abnormal mitochondrial function induced by a common viral illness, typically influenza or varicella; association with aspirin administration in children  Clinical manifestations: o Onset: profuse vomiting, varying degrees of neurological impairment o With progression: seizures, coma, increased ICP, herniation, death  Diagnostic evaluation: liver biopsy  Nursing management: care as per any unconscious child and increased ICP o Accurate and frequent intake and output, monitor lab values (coagulation labs, ammonia, and liver function tests), and parent education and support Febrile seizures  1 month to 5 years of age  No previous neurologic abnormality  Risk factors: viral infection; family history  Causes: environmental and genetic  Antipyretic therapy will not prevent a febrile seizure and are ineffective at lowering the temperature associated with a fever that leads to a febrile seizure  Management & Treatment19 o Plan of care will include standard seizure management and can include things such as safety and airway management o Most febrile seizures will stop by the time the child is taken to a medical facility and do not require treatment. If prolonged greater than 5 minutes administration can include benzodiazepines IV, lorazepam IV, or rectal diazepam is administered. o Children with febrile status epilepticus require multiple antiepileptic medications for seizure control o Parent education (when to seek medical attention) and reassurance Hydrocephalus  Imbalance in the production and absorption of CSF in the ventricular system. o Impaired absorption of CSF (communicating hydrocephalus) o Obstruction to the flow of CSF (non-communicating hydrocephalus)  With accumulation of CSF, ventricles dilate and compress brain tissue  Causes include developmental defects, neoplasms, infections, trauma, or hemorrhage  Early infancy o Abnormally rapid head growth o Bulging fontanels (tense and non-pulsatile) o Dilated scalp veins o Separated sutures o Macewen sign (cracked pot) o Thinning of skull bones o Severe cases in infants  Frontal enlargement/bossing  Depressed eyes  Setting sun sign (downward rotation of eyes)  Sluggish pupils with unequal response to light  Clinical manifestations o Infancy (general)  Irritability  Lethargy  Seizure activity  Infant cries when held or rocked, quiets when allowed to lay still  Not meeting developmental milestones  Changes in LOC  Spasticity in lower extremities  Vomiting  Difficulty sucking and feeding  Shrill, high pitch cry  Cardiopulmonary arrest o Childhood  Headache on awakening, improves after emesis or taking a position of upright posture  Papilledema  Strabismus  Ataxia  Irritability  Lethargy  Apathy  Confusion  Incoherence  Vomiting  Nursing care management: o Observe for signs of increasing ICP o Measure head circumference o Palpate fontanels and suture lines20 o VS o Change in feeding behavior o Prepare the child and family for tests o Assist with ventricular tap  Post operatively: o Routine post op care and observation o Positioning o Pain management o Monitor for signs of increased ICP, and infection o Family support and education  Prognosis o Rate at which hydrocephalus develops o Duration of the increased ICP o Frequency of complications o Underlying cause of the hydrocephalus  Diagnostic evaluation o Infants – head circumference which crosses at least one percentile line on the head measurement chart within 2-4 weeks o Older children – CT Scan, MRI  Therapeutic management – surgery to relieve obstruction or place a shunt that provides drainage of the CSF from the ventricles to another body compartment, usually the peritoneum Chapter 29- The Child with Musculoskeletal or Articular Dysfunction Soft tissue injury  Contusions (bruise): includes damage to the soft tissue subcutaneous tissue, and muscle. May have ecchymosis an deep contusions can produce myositis ossifications.  Crush injury: when an extremity or digit is crushed. A severe crushing injury may include bone with swelling and bleeding underneath.  Dislocation: occurs when the force of stress on a ligament is so great that it displaces the normal position of the opposing bones in its socket. A common injury seen in children is the subluxation or partial displacement of the radial head (nursemaid’s elbow).  Sprain: occurs when a ligament is torn or stretched when a joint is twisted.  Strains: occurs when there is a tear to the musculotendinous unit. The area is painful to touch and swollen.  Soft tissue injury mgmt. o The first 12 to 24 hours are crucial for soft-tissue injuries. Management includes using care described by the acronyms RICE and ICES:  Rest  Ice  Compression  Elevation  Ice  Compression  Elevation  Support Fractures  Common injury in children  Methods of treatment are different in pediatrics than in older adult population21  Rare in infants, except with motor vehicle crashes  Clavicle is the most frequently broken bone in childhood, especially in those less than10 years old  School age: bike, sports injuries  Types of fractures: o Plastic deformation: Occurs when the bone is bent but not broken. Can bend 45 degrees or more before breaking. o Buckle: Produced by compression of the pourous bone and appears like a raised or bulging projection at the site o Compound or open: fractured bone protrudes through the skin o Complicated: bone fragments have damaged other organs or tissues o Comminuted: small fragments of bone are broken from fractured shaft and lie in surrounding tissue o Greenstick: compressed side of bone bends, but tension side of bone breaks, causing incomplete fracture  Epiphyseal injuries: o Weakest point of long bones is the cartilage growth plate (epiphyseal plate) o Frequent site of damage during trauma o May affect future bone growth o Treatment may include open reduction and internal fixation to prevent growth disturbances  Clinical manifestations o Generalized swelling o Pain or tenderness o Diminished functional use o May have bruising, severe muscular rigidity, crepitus  Assessment of Fractures: The 6 P’s o Pain and point of tenderness o Pulse: distal to the fracture site o Pallor: pale appearance, signs or poor perfusion o Paresthesia: sensation distal to the fracture site o Paralysis: movement distal to the fracture site o Pressure: Involved limb or digit may feel tense or warm, skin is tight and shiny  Bone healing and remodeling o Typically rapid healing in children o Neonatal period: 2-3 weeks o Early childhood: 4 weeks o Later childhood: 6-8 weeks o Adolescence: 8-12 weeks o Diagnostic evaluation: x-ray is most useful diagnostic tool. X-ray may reveal evidence of fractures at various stages of healing that warrants further assessment to rule out child abuse. The child in a cast  Cast application techniques: consider development age when explaining procedure.  Nursing considerations: o Skin integrity (assess for wounds and cuts prior to application and avoid denting plaster with fingertips as this can create unwanted pressure). o Assessment: Assess 6 p’s prior to application and frequently after o Health Teaching and Promotion  Keep the affected extremity elevated and the client should avoid strenuous activities for the first few days.  Teach safe crutch used and be sure to teach the parents to remove clutter  Do not allow the child to place anything in the cast and no use of fans or dryers on the cast.  Cast removal: accomplished through using a cast cutter which can be loud and scary. Prepare the child for this procedure as it can be frightening based on their developmental age.22 The child in traction  Traction: extended pulling force may be used o Purposes of traction:  To fatigue the involved muscles and reduce muscle spasm so bones can be realigned.  To position in desire realignment to promote satisfactory bone healing.  To immobilize fracture site until realignment has been achieved to allow casting or splinting.  To prevent or improve contracture deformity.  To provide immobilization for specific areas of the body.  To reduce muscle spasms (rare in children) o Traction: forward force produced by attaching weight to distal bone fragment  Adjust by adding or subtracting weights o Countertraction: backward force provided by body weight  Increase by elevating foot of bed o Frictional force: provided by patient’s contact with the bed  Types of traction o Type of traction depends on the child’s age, condition of the soft tissue, and the type and degree of displacement of the fracture. o Manual traction: applied to body part by the hand placed distally to fracture site o Skin traction: pulling mechanisms are attached to skin with adhesive material or elastic bandage o Skeletal traction: applied directly to skeletal structure by pin, wire, or tongs inserted into or through diameter of bone distal to the fracture o Subtypes of Traction:  Bryant traction: traction in which the pull is only in one direction.  Buck’s extension: traction with the legs in an extend position  Russell traction: used skin traction on the lower leg with a padded sling under the knee.  90 to 90 traction: a common skeletal traction  Balanced suspension: can be used with our without skin or skeletal traction. Suspends the leg in a desire flexed position to relax the hip and hamstring muscles.  Cervical traction: Halo brace or halo vest is used.  Nursing care o Assessment 6 P’s o Maintain traction, alignment o Prevent skin breakdown o Prevent complication o Pain management and comfort measures Distraction  Process of separating opposing bone to encourage regeneration of new bone in created space  Can be used when limbs are unequal in length and new bone is needed to elongate shorter limb Developmental dysplasia of the hip (DDH)  Formerly called congenital hip dysplasia or congenital dislocation of the hip  DDH reflects variety of hip abnormalities: o Shallow acetabulum o Subluxation o Dislocations  Three Degrees of DDH o Acetabular dysplasia (preluxation)  Mildest form; osseous hypoplasia of acetabular roof  Femoral head remains in the acetabulum o Subluxation: incomplete dislocation of hip o Dislocation: femoral head loses contact with acetabulum and is displaced posteriorly and superiorly; ligaments elongated and taut23  Clinical manifestations of DDH o Infant:  Shortened limb on affected side  Restricted abduction of hip on affected side  Unequal gluteal folds when infant prone  Positive Ortolani test  Positive Barlow test o Older infant and children  Affected leg shorter than the other  Telescoping or piston mobility of joint  Trendelenburg sign  Greater trochanter is prominent and appears above line from anterosuperior iliac spine to tuberosity of ischium  Marked lordosis if bilateral dislocations  Waddling gait if bilateral dislocations  Therapeutic mgmt. o Importance of early intervention o Newborn to age 6 months: Pavlik harness for abduction of hip o Age 6-18 months: dislocation unrecognized until child begins to stand and walk; use traction and cast immobilization (spica) o Older child: operative reduction, tenotomy, osteotomy; difficult after 4 years Congenital clubfoot  Types of Clubfoot: o Talipes varus: inversion, or bending inward o Talipes valgus: eversion, or bending out o Talipes equinus: plantar flexion with toes lower than the heel o Talipes calcaneus: dorsiflexion with toes higher than the heel  Classification: o Mild or postural  May correct spontaneously or require passive exercise or serial casting o Tetralogic  Associated with other congenital anomalies  Usually requires surgical correction with high incidence of recurrence o Idiopathic  Bony abnormality almost always requiring surgical intervention  Diagnostic evaluation: deformity is apparent at birth if not seen in ultrasound.  Therapeutic management: goal is to achieve painless, plantigrade, functional foot.  Nursing considerations: o Short term and long term goals that include cast care, educating the family, and encouraging the family to facilitate a normal development for the child. Other developmental defects  Metatarsus Adductus o One of the most common congenital foot deformities. o Medial adduction of the toes and forefoot. o Differs from clubfoot because the heel and the ankle remain in a neutral position o Management will depend on the rigidity and type of deformity. o Treatment make include stretching, manipulation and casting, or corrective shoes.  Osteogenesis Imperfecta o Rare genetic disorder characterized by bones that fracture easily. o Management:  Primarily supportive24  Use of bisphosphonate therapy with IV pamidronate to promote increased bone density and prevent fractures. o Nursing management:  Handling the client carefully  Educating the parents about home care  Supportive treatment Kyphosis and lordosis  Kyphosis: lateral convex angulation of the thoracic spine o Exercises o Brace o Physical therapy o Surgical fusion if indicated.  Lordosis: Lateral inward curve of the cervical or lumbar spine. o Treatment include:  Weight loss  Deformity correction  Special exercises Scoliosis  Most common spinal deformity in childhood  Complex spinal deformity in three planes: o Lateral curvature o Spinal rotation causing rib asymmetry o Thoracic hypokyphosis  May be congenital or develop during childhood  Multiple potential causes; most cases idiopathic  Generally becomes noticeable after preadolescent growth spurt  May have complaints of ill-fitting clothes  Diagnostic eval o Standing radiographs to determine degree of curvature o Asymmetry of shoulder height, scapular or flank shape, or hip height o Often have primary curve and compensatory curve to align head with gluteal cleft  Therapeutic mgmt. o Team approach to treatment o Bracing o Exercise o Surgical intervention for severe curvature (instrumentation and fusion):  Harrington rods  L-rods Osteomyelitis  Defined as an infectious process in the bone that can occur at any age but commonly seen in children 10 years and younger.  Causes o Infectious organism  NB: S. aureus, group b streptococcus, gram-neg. enteric rods  Infants: S. aureus (MRSA), haemophilus influenza  Older children: S. aureus, pseudomonas organisms, salmonella organisms, Neisseria gonorrhoeae  Adolescents and adults: pseudomonas organisms, mycobacterium TB o Direct inoculation o Abscess o Dead bone25  Therapeutic Management: o Culture specimens obtained o IV antibiotic therapy o Surgery if indicated  Nursing Care Management: o Position client comfortably with the affected limb supported o Temporary splint or cast may be applied o Weight bearing should be avoided o Pain management Septic arthritis  Bacterial infection of the joint (staph aureus is the most common).  Most common affected joints: knees, hips, ankles, and elbows.  Therapeutic and Nursing Management: o Aspiration of a specimen from the affected joint o Radiology studies o IV antibiotic therapy o Pain management o Surgical intervention if indicated o Physical therapy Skeletal TB  Infection of the bones and joints from lymphohematogenous spread at the time of primary infection.  Not commonly seen in the United States.  Diagnosis requires isolation of Mycobaterioum tuberculosis.  Managed with tuberculosis chemotherapy.  Nursing care will depend on the site and extent of the infection.  May include casting, surgical fusion, or immobilization. Juvenile idiopathic arthritis  Chronic childhood arthritics that caused inflammation in the joint synovium and the surrounding tissue.  Girl are twice as likely to be affected.  Clinical manifestations: o Swelling and loss of motion in a single or multiple joints that are affected o Functional changes: development of a limp or limitations of joint movement. o Growth disturbances  Diagnostics: o No definitive tests to determine. o Labs and radiology tests may serve as supporting evidence  Therapeutic Management: o Goals  Pain control  Minimize effects of inflammation  Preserve range of motion and function.  Promote normal growth and development. o Medications used:  Nonsteroidal anti-inflammatory drugs (NSAIDS)  Disease-modifying Antirehumatics  Glucocorticoids o Physical and Occupational therapy  Nursing Management: o Pain management26 o Health promotion o Facilitate Adherence o Comfort measures and exercise o Support the child and family Systemic lupus erythematosus  Systemic lupus erythematosus (SLE) is sever chronic autoimmune disease that results inflammation and multi-organ system damage.  Clinical Manifestations: Multiple system involvement (Box 29-10 pg. 976) o Constitutional o Cutaneous o Musculoskeletal o Neurologic o Pulmonary and cardiac o Renal o GI o Hepatic, splenic, and nodal o Hematologic o Ophthalmologic o Vascular  Supportive Care: o Nutrition o Sleep and rest o Exercise o Limited sun exposure  Therapeutic management: o Medications:  Corticosteroids  NSAIDS  Antimalarials  Immunosupressive agents  Nursing Management: o Main goal is to help the client and the family adjust to the disease and the associated therapy. Chapter 30- The Child with Neuromuscular or Muscular Dysfunction Cerebral palsy (CP)  Characterized by early onset and impaired movement and posture  Most common permanent physical disability in childhood  70% of CP patients have normal IQ  Etiology: o Intrauterine hypoxia or asphyxia  Intrapartum asphyxia  12%-23% of CP occurs in term infants with intrapartum asphyxia  Postnatal  Often no identifiable immediate cause o Preterm birth with very low birthweight and extremely low birthweight is single most important determinant of CP o Anoxia is most common cause of brain damage whenever it occurs  Classification of CP27 o Spastic (pyramidal): most common clinical type. Characterized by persistent primitive reflexes.  Types of Spastic CP:  Quadriparesis (tetraparesis): o Four extremities involved, severe disability o Speech and swallowing difficulties o Tongue protrusion (incomplete) o Labile emotions in some patients  Diplegia  Monoplegia  Triplegia  Paraplegia  Dyskinetic (nonspastic, extrapyramidal):  Athetoid: Chorea(involuntary, irregular jerking movements)  Dystonic: Slow twisting movements of the trunk or extremities.  Ataxic (nonspastic, extrapyramidal). Wide-based gait, rapid repetitive movements performed poorly.  Mixed type: combination of spastic and dyskinetic  Signs of CP o Motor Signs:  Failure to meet developmental milestones (rolling over, raising head).  Poor head control after age 3 months  Stiff or rigid limbs  Arching back, pushing away  Floppy tone  Abnormal posture  Inability to sit without support at age 8 months  Clenched fists after age 3 months  Persistent infantile reflexes  Review boxes 30-2 and 30-3 in textbook pg. 980 o Behavioral Signs:  Excessive irritability  No smiling by age 3 months  Lack of interest in surroundings  Feeding difficulties:  Poor sucking  Persistent tongue thrusting  Frequent gagging or choking with feedings  Therapeutic Management 5 goals: o Establish locomotion, communication, and self-help skills o Gain optimal appearance and integration of motor function o Early diagnosis and correcting associated defects as early and effectively as possible o Provide education that is adapted to the child’s needs and abilities o Promote socialization among peers  Nursing Management: o Encourage rest periods to avoid fatigue o Feeding management;  Child may have gastroesophageal reflux (GERD)  Feeding and swallowing difficulties  Gastrostomy care o Safety o Physical therapy o Family support and education Neutral tube defects (NTDs)28  Failed closure of neural tube  May involve entire length of the neural tube or small portion  Incidence: o Affects more girls than boys o Occurs three times more often in Caucasians than in African Americans  Cause: o 50% or more: folic acid deficiency o Other cases: multifactorial  Two most common types: o Anencephaly o Spina bifida (myelomeningocele)  Treatment = prevention: o Supplementation: folic acid 0.4 mg/day o If history of NTD, 4.0 mg/day o Begin preconception  Antenatal Diagnosis of Neural Tube Defects: o Amniocentesis will show elevated α-fetoprotein in amniotic fluid—16-18 weeks of gestation o Uterine ultrasound to show extent of the lesion o Schedule cesarean birth to manage sac without labor or stress Anencephaly  Absence of cerebral hemispheres  Brainstem function may be intact  Incompatible with life o Few hours to few days o Death due to respiratory failure Spina bifida  Two types: o Spina bifida occulta  Not visible externally o Spina bifida cystica  Visible defect  Saclike protrusion  Usually lumbosacral, L5-S1  Skin indicators (absent, singly or combination): o Sacral dimple o Sacral angioma or port wine nevus o Sacral tufts of dark hair o Sacral lipoma  Diagnostics o X-ray o MRI o CT o Ultrasonography Spina bifida cystica  Visible defect with external saclike protrusion  Two types: o Meningocele  Sac contains meninges and spinal fluid but no neural elements  No neurologic deficits o Myelomeningocele29  Neural tube fails to close  May be anywhere along the spinal column  Lumbar and lumbosacral areas most common  May be diagnosed prenatally or at birth  Sac contains meninges, spinal fluid, and nerves  Varying and serious degrees of neurologic deficit  Clinically, myelomeningocele term is interchangeable with phrase spina bifida  Myelomeningocele: the sac  May be fine membrane o Prone to leakage of cerebrospinal fluid (CSF); easily ruptured  May be covered with dura, meninges, or skin o Rapid epithelialization  Location and magnitude of defect determine nature and extent of impairment  If defect below 2nd lumbar vertebra: o Flaccid paralysis of lower extremities o Sensory deficit  Not necessarily uniform on both sides of defect  Mgmt. of myelomeningocele  Prevent infection  Assess neurologic and associated anomalies  Management of genitourinary function, bowel control, and orthopedic considerations, especially considering locomotion.  Early closure in 12-72 hours after birth o Prevent stretching of other nerve roots and further damage Latex allergy  Identified as serious health hazard when a child with spina bifida experiences anaphylaxis due to latex allergy  Spina bifida patients are at high risk for latex allergy due to repeated exposure to latex products from multiple surgeries and repeated urinary catheterizations  Allergic Reactions to Latex: o Range from urticaria, wheezing, rash, to anaphylaxis o Reactions tend to increase in severity when latex comes in contact with mucous membranes, wet skin, bloodstream, or airway o Cross-reactions with foods: banana, avocado, kiwi, chestnuts  Nursing assessment should include monitoring the child for signs and symptoms of an allergic reaction and performing a good health history with the caregivers regarding previous experiences with latex that may indicate an allergy. Muscular dystrophies (MDs)  Largest group of muscular diseases in children  All have genetic origin with gradual degeneration of muscle fibers, progressive weakness, and wasting of skeletal muscles  All have increasing disability and deformity with loss of strength  3 types: o Duchenne o Facioscapulohumeral o Limb-girdle Duchenne muscular dystrophy (DMD)  Also known as pseudohypertrophic muscular dystrophy  Most severe and most common of muscular dystrophies in childhood  X-linked inheritance pattern; 1/3 are fresh mutations30  Characteristics: o Onset between age 3 and 5 years o Progressive muscle weakness, wasting, and contractures o Calf muscles hypertrophy in most patients o Progressive generalized weakness in adolescence o Depressed or absent reflexes o Death from respiratory or cardiac failure  Clinical manifestations: o Waddling gait, frequent falls, Gower sign o Lordosis o Enlarged muscles, especially thighs and upper arms o Profound muscular atrophy in later stages o Mental deficiency common  Diagnostic eval o Suspected based on clinical appearance o Confirmation by EMG, muscle biopsy, and serum enzyme measurement o Serum CPK and AST levels high in first 2 years of life, before onset of weakness; levels diminish as muscle deterioration continues  Therapeutic mgmt. o No effective treatment established o Primary goal: maintain function in unaffected muscles as long as possible o Keep child as active as possible o Range of motion, bracing, performance of activities of daily living, surgical release of contractures as needed o Genetic counseling for family and family support and education.