OXYGENATION in Pediatrics

OXYGENATION in Pediatrics
By Prof. Unn Hidle Updated Spring 2010

Congenital Heart Disease
Incidence 5-8/1000 live births >35 well-recognized defects, but most common is Ventricular Septal Defect (VSD) Etiology in 90% of CHD is unknown Multifactorial: Genetic and environmental

Acyanotic VS Cyanotic Cyanosis may occur as a later sign in “acyanotic” conditions. Cyanotic conditions may appear “pink” or of normal skin color Classification system based on hemodynamic characteristics is better (blood flow patterns): Increased pulmonary blood flow Decreased pulmonary blood flow Obstruction to blood flow out of the heart Mixed blood flow (saturated and desaturated)

“Acyanotic” Increased pulmonary blood flow OR obstruction of blood flow from ventricles LEFT to RIGHT cardiac shunting (except when there is an obstruction) Often leads to CHF because excess blood is going to the pulmonary circulation Murmurs are commonly auscultated

LEFT-TO-RIGHT CARDIAC SHUNTS
Description Blood is shunted to the right side of the heart because the left side is normally functioning under a higher pressure than the right Oxygenated and unoxygenated blood mix, which results in increased pulmonary blood flow because the opening sends more blood to the right side of the heart than normal

Types of L-to-R shunting
Atrial-Septal Defect (ASD) Ventricular Septal Defect (VSD) Patent Ductus Arteriosus (PDA) Atrioventricular Canal Defect (AVCD)

Obstructive/stenotic lesions
Narrowing or constriction of an opening in a valve or vessel that results in obstruction of blood flow through the area TYPES: Aortic Stenosis Pulmonary Stenosis Coarctation of the Aorta

Overall Assessment: L-to-R or Obstructive
May be asymptomatic May show signs and symptoms of CHF May exhibit failure to thrive Growth retardation Diaphoresis Fatigue Tachypnea Poor eating Dyspnea Hypoxemia

Implementation Assess respiratory status for the presence of nasal flaring and use of accessory muscle Auscultate lungs for the presence of crackles and rhonchi Assess for signs of CHF such as fluid retention in the eyes, hands, feet, and chest Assess for diuresis Assess urine output; weighing diapers is necessary Assess calorie intake Plan interventions to allow maximal rest for the child Allow parent or child if appropriate to verbalize feelings and concerns regarding disorder

“Cyanotic” RIGHT to LEFT cardiac shunt Usually does not lead to CHF
Occur when blood is shunted to the left side of the heart because one of the right heart chambers has a higher pressure Oxygenated blood mixes with unoxygenated blood; cyanosis occurs Decreased pulmonary blood flow or mixed blood flow Deoxygenated blood is circulating where oxygenated blood should Usually does not lead to CHF

Types of R- to – L shunting
Tetralogy of Fallot (both) Transposition of the Great Arteries Truncus Arteriosus Pulmonary Atresia Tricuspid Atresia Hypoplastic Left Heart Syndrome

Assessment Symptoms occur in the first week of life
Dyspnea after feeding, crying, or other activities Hypercyanotic or “tet spells” (blue spells) characterized by increased respiratory rate and depth and increased hypoxemia with TOF Squatting episodes with tetralogy of Fallot Signs of CHF Respiratory distress Clubbing of digits Poor growth Tachycardia

Implementation Monitor vital signs
Monitor respiratory status notifying physician if any changes occur Auscultate breath sounds for crackles or rales Keep child as stress free as possible If respiratory effort is increased, place child in reverse Trendelenburg (elevate head and upper body) to decrease the work of breathing Monitor for hypercyanosis and place child in knee-chest position and notify physician Administer humidified oxygen as prescribed

Provide endotracheal tube and ventilator care as prescribed and restrain hands of intubated child
Monitor for signs of CHF Monitor body weight (daily weight) Monitor I&O and notify physician if a decrease in urine output occurs Palpate liver noting enlargement, which is an indication of right-sided heart failure Administer diuretics as prescribed

CHF – What do you think? When children develop congestive heart failure from a congenital heart defect, the failure is usually: Right-sided only Left-sided only Cor pulmonale Both Right- and Left-sided

CONGESTIVE HEART FAILURE
Description Inability of the heart to pump sufficiently to meet the metabolic needs of the body Increased right ventricular workload leading to right sided heart failure In infants and children, inadequate cardiac output is most commonly caused by congenital heart defects that produce an excessive volume or pressure load on the myocardium In children a combination of both left-sided and right-sided heart failure is usually present

Congestive Heart Failure
Common causes Volume overload – especially in L-to-R shunting Pressure overload – obstructive lesion (i.e. coarctation of the aorta) Decreased contractility – cardiac myopathy or myocardial ischemia High cardiac output demands Mostly seen in L-to-R shunting ASD, VSD, PDA

Assessment of Early and Late Symptoms Tachycardia Arrhythmia – gallop (S3 and S4) Poor perfusion Mild cyanosis Tachypnea leading to dyspnea during feeding Poor feeding Poor weight gain due to increased metabolic rate – FIRST SIGN! Activity intolerance Diaphoresis during feeding

Retractions Wheezing Cough/hoarseness (pressure on the laryngeal nerves) Orthopnea Hepatosplenomegaly due to pooling of blood in the portal circulation and accumulation in the hepatic tissue Edema / weight gain from Na and H2O retention Ascitis Pleaural effusion Distended neck and peripheral veins from consistent increased CVP Prolonged CHF leads to developmental delays

Commonly used term for CHF is cor pulmonale resulting from obstructive lung disease (i.e. Cystic fibrosis or bronchopulmonary dysplasia) Diagnosis Based on clinical S/S CXR: cardiomegaly and increased pulmonary vascular marking EKG: ventrcular hypertrophy (arrhythmias) ECHO

Implementation goals: To improve cardiac function To remove fluid and Na To decrease cardiac demands To improve tissue oxygenation and decrease oxygen consumption

Implementation Elevate the head of the bed Administer oxygen as prescribed during stressful periods such as bouts of crying or invasive procedures Feed in a relaxed environment Provide small frequent feedings, which will be less tiring Monitor STRICT I&O and DAILY WEIGHT to assess for fluid retention Weigh diapers

Implementation (cont’d) Monitor for facial or peripheral edema, auscultate lung sounds, and report weight gain to physician Monitor electrolyte levels Treat existing infections Instruct parents regarding diagnosis and administration of medications Instruct parents CPR

Medications used in CHF
DIGOXIN: Know your Dig! Cardiac glycoside = improves function; strengthens heart muscle; slows down heart rate Therapeutic serum level: 0.8 – 2.0 ng/ml Monitor EKG rhythm for desired effect (i.e. if prolonged P-R interval = hold and notify MD) Monitor AHR (i.e. bradycardia = hold dose) – know your child’s normal HR range

Medications cont. Angiotensin-converting enzyme (ACE inhibitors)
Inhibits the normal function of the renin-angiotensin system in the kidneys Blocks angiotensin II so instead of vasoconstriction, vasodilatation occurs and arteries opens (decreased BP; improved systemic circulation; decreased afterload and decreased R-L arterial pressure) Examples Captopril (Capoten); Enalapril (Vasotec) and Lisinopril (Zestil) = most common (QD)

Medications cont. DIURETICS Furosemide (Lasix) = potassium wasting
Spironolactone (Aldactone) = potassium sparing --- Be careful with IV additives!

What do you think? The two main angiotensin-converting enzyme (ACE) inhibitors most commonly used for children with CHF are: Digoxin and captopril Enalapril and Captopril Enalapril and furosemide Spironlolactone and captopril

What do you think? The calories are usually increased for an infant with CHF by: Increasing the frequency of feedings Introducing solids into the diet Increasing the density of the formula Gastrostomy feedings

Treatment Options For Various Conditions
PDA (Patent Ductus Arteriosis) Indomethacin (Indocin) Prostaglandin E1 Coil embolization VATS = Video-assisted throacoscopic surgery ASD (Atrial Septal Defect) Cardiac catheterization Laparoscopic techniques Sternotomy (“open heart”)

Cardiac Catheterization

VATS: Video Assisted Thorascopic Surgery

Sternotomy

VSD (Ventricular Septal Defect)
Some close on their own Diuretics Cadiotonics: Ca Channel Blockers (Nifedipine or Procardia) If surgery, then similar to ASD Aortic and Pulmonic Stenosis Balloon angioplasty

Balloon Angioplasty

What do you think? Coarctation of the aorta should be suspected when:
The BP in the arms is different from the BP in the legs The BP in the R-arm is different from the BP in the L-arm Apical pulse is greater than the radial pulse Point of maximum impulse is shifted to the left

Coarctation of the Aorta
Specific S/S: Increased BP and bounding pulses in upper extremities and decreased BP and weak/absent pulses (femoral) in lower extremities Balloon angioplasty Surgical intervention = end-to-end anastomosis

Resection & End-to-End Anastomosis

Atroventricular Canal (AVC) defect
Complete surgical repair in infancy Palliative if older TOF (Tetrology of Fallot) Palliative surgery (Blalock-Taussig Shunt) Corrective open-heart surgery / sternotomy

Complete Transposition of Great Vessel
NO communication b/t systemic and pulmonary circulation Repair WITHIN HOURS! Not conductive with life Palliative procedure = pulmonary artery banding Corrective surgery = arterial switch procedure Truncus Arteriosus EXTENSIVE surgical treatment

Hypoplastic Left Heart Syndrome (HLHS)
Pulmonary Atresia EXTENSIVE surgical treatment Tricuspid Atresia Palliative and EXTENSIVE surgical treatment Hypoplastic Left Heart Syndrome (HLHS) Multiple stage surgery Heart transplantation Endocardial Cushing Total mixing of blood = EXTENSIVE surgical repair

What do you think? Chronic hypoxemia is clinically manifested by which of the following signs? Squatting Polycythemia Clubbing All of the above

Cardiac Surgery Implementation PRE-OPERATIVELY Similar as with CHF
Remember HOB elevated / high Fowlers with acyanotic conditions and knee-chest position (squatting) with cyanotic conditions – GENERALLY! Prepare for procedure Developmentally appropriate teaching Preparing what is to be expected post-operatively Equipment Pulmonary exercises

What do you think? The MOST painful part of cardiac surgery for the child is usually the Thoracotomy incision site Graft site on the leg Sternotomy incision site Intravenous insertion sites

CARDIAC SURGERY Implementation POST-OPERATIVELY NICU/PICU
Monitor vital signs frequently (as per policy) Monitor temperature – report ANY fevers! ** Usually low-grade fevers are accepted in 1st 24 hours (i.e F) Maintain aseptic technique Monitor for signs of sepsis such as fever, chills, diaphoresis, lethargy, and altered levels of consciousness

CARDIAC SURGERY Postoperatively (cont’d) Monitor equipment:
Cardiac monitor IV lines (CVL, PIVL, CVP, A-lines) ET tube Chest tube **** see X-ray **** Dressings NGT Foley Restraints

Thoracotomy: Chest tube

Postoperatively (cont’d)
Assess for signs of discomfort such as irritability, changes in heart rate, respiratory rate and blood pressure, and inability to sleep Sedation Pain Antibiotics and antipyretics as prescribed Encourage rest periods Facilitate parent-child contact as soon a possible

What do you think? An infant who weighs 7 kg has just returned to the ICU following cardiac surgery. The chest tube has drained 30 ml in the past hour. In this situation, what is the FIRST action for the nurse to take? Notify the surgeon Identify any other signs of hemorrhage Suction the patient Identify any other signs of renal failure

HOME CARE POSTOPERATIVE CARDIAC SURGERY
MOST IMPORTANT: Follow individualized plan! Omit play outside for “several weeks” Avoid activities where the child could fall, such as bike riding, for a period of time Avoid crowds in the immediate time after discharge Follow a no added salt diet if prescribed Do not add any new foods to the infant’s eating schedule

Do not place creams, lotions, or powders on incision until completely healed
Child may return to school 2-3 weeks after discharge starting (depending on type of procedure and “individualized plan”) No physical education until cleared by MD Instruct parents to discipline the child normally Instruct parents about the importance follow-up

Avoid immunizations, invasive procedures, and dental visits for 2 months
Advise parents regarding the importance of a dental visit every 6 months after age 3 and to inform the dentist of the cardiac problem so that antibiotics can be prescribed if necessary (new guidelines regarding prophylactic antibiotics) Inform parents to call the physician when coughing, tachypnea, cyanosis, vomiting, diarrhea, anorexia, pain, fever, or any swelling, redness, or drainage occurs at the site of the incision

Critical Thinking Tommy, a 4-year old with TOF has just returned from the catheterization laboratory. He has vomited, and his mother calls you to the bedside to tell you that he is bleeding. You arrive to find Tommy crying and sitting up in a puddle of blood. What is the FIRST thing you should do? Increase the rate of his IV fluids Give an antiemetic and keep Tommy NPO Call the cardiologist Lie Tommy down and apply direct pressure above the catheterization site

Cardiac Catheterization
Pre-procedure History including allergies Ht / Wt NPO X 8 hours Mark pulse location Prepare for administration of analgesics / sedatives / anxiolytics

Post-procedure Monitor distal pulses and VS (BP)
Monitor perfusion: temperature and color of affected extremity and compare Dressing for bleeding Leg extended straight for 4-6 hours Strict I&O Encourage voiding to remove dye Analgesics

RHEUMATIC FEVER

RHEUMATIC FEVER An inflammatory “autoimmune disease” that affects the connective tissues of the heart, joints, subcutaneous tissues, and/or blood vessels of the CNS Presents 2 to 6 weeks following an untreated or partially treated group A beta-hemolytic streptococcal infection of the upper respiratory tract Serious complication is rheumatic heart disease, which affects the cardiac valves

RHEUMATIC FEVER Clinical Manifestation Starts with URI
Inflammatory hemorrhagic bullous lesions called Aschoff bodies are formed, which causes swelling, fragmentation and alteration in the connective tissues (circulatory system, brain, pleura and joints) Fibrous tissue forms causing valve stenosis and occlusion of blood flow = murmur Carditis is the end result

What do you think? One of the most common findings on physical examination of the child with acute rheumatic heart disease is A Systolic murmur Pleural friction rub An ejection click A split S2

Rheumatic Fever Assessment Edema, inflammation of large joints
Joint pain Fever Pallor and weakness Anorexia, weight loss Erythematous macular rash on trunk and extremities Chorea Subcutaneous nodules in the joints, scalp, and spine Arthritis Epistaxis Abdominal pain Systolic murmur, tachycardia Pericardial rub c/o chest pain (pericarditis) SOB

Diagnostic Evaluation
No single S/S or lab test: Based on diagnostic guidelines set by the American Heart Association (Jones criteria): Increased Anti-Streptolysin-O titers (ASLO) Measure of antibodies formed against streptolysin-O (streptococcal extracellular product which produces lysis of RBC). Formed within 7 days and peaks at 4-6 weeks. Reliable test in evidence of recent streptococcal infection Normal value is TODD units. Value >333 = RF 2 tests are required to confirm diagnosis Increased C-Reactive Protein (CRP) Leukocytosis Anemia Increased Erythrocyte Sedimentation Rate (ESR) Prolonged P-R interval

RHEUMATIC FEVER Implementation PREVENTION!!!!!!!!!!!
Antibiotics for Strep (PCN or Erythromycin) Antibiotics prophylactically (teaching) Bedrest until afebrile Salicylates (Aspirin) is used for the INFLAMMATORY PROCESS to decrease fever and comfort (** Pediatrics + Aspirin = Reye’s syndrome – toxic encephalopathy) – This is an exception!!! Steroids (i.e. prednisone) to decrease inflammation Administer anti-inflammatory agents as prescribed and if aspirin is prescribed, it should not be given to a child who has chicken pox or other viral infections Initiate seizure precautions if the child is experiencing chorea

Complications of Rheumatic Fever
Polyarthritis Edema, inflammation and effusion of joint tissue Lat approx. 2 days after fever Erythema marginatum Erythematous macules (clear center) Subcutaneous nodules 0.5-1cm non-tender swelling nodules that resolves Carditis Endocardium, pericardium and myocardium CNS involvement CNS irritability = Sydenham’s Chorea (St Vitus Dance): Sudden, aimless movements of extremities, facial grimacing and contortions.

KAWASAKI DISEASE An acquired heart disease with progressive inflammation of the vessels and damage to their walls Believed to be caused by a noncontagious infection Without treatment, 20-25% develop cardiac sequela The leading cause of heart disease in the US and Japan

What do you think? The peak age for the incidence of Kawasaki disease isin the Infant age group Toddler age Group School age group Adolescent age group

Diagnosis of Kawasaki Disease
No specific diagnostic test Dx is based on clinical signs & symptoms Fever for > 5 days Bilateral conjunctival injection (inflammation) without exudation “Strawberry tongue” Change in extremities: peripheral edema, erythema of palms and soles; desquamation of hands Polymorphous rash Cervical lymphadenopathy (one lymph node >1.5cm)

3 Phases of Kawasaki ACUTE PHASE (1-2 weeks) Complications
Abrupt onset of high fever >5 days No response to antipyretics or antibiotics Bulbar conjunctiva with erythema and tearing Inflamed pharynx Red, cracked lips “Strawberry tongue” Desquamation of hands and feet Cervical lymphadenopathy VERY irritable and inconsolable Complications Myocarditis, CHF and temporary arthritis EKG changes (ischemia)

Kawasaki Disease SUBACUTE PHASE (2 to 6-8 weeks) Lab results
Fever resolves and s/s begins to disappear Still irritable Risk of developing coronary artery aneurysm Thrombocytosis and hypercoagulability = coronary thrombosis Arthritis continues Lab results CBC: anemia and leukocytosis Increased ESR Transient elevated LFTs

Kawasaki Disease CONVALESCENT PHASE All clinical s/s are gone
This phase is completed when blood values return to normal; usually 6-8 weeks after onset There is always a high risk of MI that remains

Treatment of Kawasaki High dose IV immune globulin (IVIG) given within 10 days of infection. One infusion of 2 Gm/Kg over 10 hours. High dose aspirin for the first 2 weeks Decreases fever, edema and inflammation This combination tends to show good results within 24 hours After 2 weeks of high dose aspirin, change to low dose for the next 4-6 weeks (prevent clots) Anticoagulation therapy (Coumadine) may be indicated

What do you think? Because of the drug used for long-term therapy, children with Kawasaki disease are at risk for Chicken pox Influenza Reye syndrome Myocardial infarction

Kawasaki - Nursing Teach to restrict physical activity due to increased bleeding Monitor cardiac status (CHF) Symptom relief Fever reduction Mouth care Discharge teaching Irritability may last up to 2 months No live immunizations until about 5 months (11 months for Varicella and MMR) PROM exercises Assess for aspirin toxicity (tinnitus, H/A, dizziness and confusion) CPR Prognosis Usually full recovery

Hematologic Disorders

Sickle Cell Anemia

Sickle Cell Anemia Normal adult hemoglobin (HgbA) is partly or completely replaced by abnormal sickl hemoglobin (HgbS) Sickle cell trait is the heterozygous form Primarily seen in the African American group The RBCs are “sickled” and cannot carry enough Hgb They are destroyed earlier than normal

Sickle Cell Anemia Two main occurrences:
OBSTRUCTION from sickled RBCs DESTRUCTION of RBC Sickled cells causes vaso-occlusion = vaso-occlusive crisis (“sickle cell crisis”) Results in local hypoxia leading to tissue ischemia and infarction (cellular death) Can be life threatening

S/S of Sickle Cell Anemia
SPLEEN Splenic sequestial crisis from engorgement with sickled cells (enlarged and no function) By age 5 years, functional cells are replaced by fibrotic tissue = functional ASPLENIA Prone to infection HEPATIC Hepatomegaly from extensive lysis of RBC Development of gallstones (obstruction) RENAL Kidney ischemia leads to hematuria and decreased renal function Nephrotic syndrome

BONES Bone changes due to bone marrow’s hyperplastic production of RBC to compensate for destruction of sickled cells Bone marrow crisis (aplastic crisis) – may lead to osteoporosis Lordosis and kyphosis Osteomylitis secondary to chronic hypoxiap CNS CVA due to occlusion, ischemia and infarction HEART Cardiomegaly – from chronic anemia Systolic flow murmur May lead to MI

Sickle Cell Anemia

4 Types of Sickle Cell Crisis
Vaso-occlusive crisis Most common and non-life threatening PAIN (joints; abdomen) Priapism Spenic Sequestration crisis More severe Blood pools in spleen and causes quick drop in blood volume = hypotension and hypovolemic shock

Other manifestations Exercise intolerance Anorexia
Jaundice / icteric sclera Gallstones Leg ulcers Growth retardations (height and weight) Delayed sexual maturation Decreased fertility

Hyperhemolytic crisis
Aplastic crisis BM crisis: decreased RBC production Triggered by viral infection: human parvovirus Increased destruction of RBCs leads to anemia (megaloblastic anemia resulting from folic acid and Vit. B deficiency) PRBC transfusion usually required Hyperhemolytic crisis Very rare Decreased production of non-sickled RBC & overall hyperplastic production Accelerated rate of RBC destruction and BM tries to replace dead RBC

Diagnosis of SCA Blood smear CBC Genetics Signs and symptoms
View sickled cells Hgb electrophoresis = “fingerprints” which detects different types of Hgb (A=Adult; F=Fetal; S=sickled) CBC Increased WBC, Platelets and Iron Genetics Perinatally through amniocentesis and CVS Signs and symptoms Pain: joints, back and abdomen

Intervention for Sickle Cell Crisis
Hydration to prevent sickling of cells Prevent acidosis: O2 supplementation Supplement with Folic acid Bedrest to decrease energy expenditure PROM PRBC transfusion prn (maintain Hgb >10mg/dl) Analgesics / Opioids Heat for relief of joint pain Strict I&O and monitor weight Regular eye exam: damage to the retina

What do you think? Infants are often not diagnosed with sickle cell anemia until they are 1 year of age. Why? Usually there are no symptoms until after age 1 year High intake of fluids from formulas prevents sickle cell crises during this age. Fetal Hemoglobin is present during the first year of life Increased hemoglobin and hematocrit amounts compensate during this period

“Treatment” Splenectomy Prophylactic PO Penicillin Vaccines
In children < 5 years old (remember, they have functional asplenia > 5 years of age) May be life saving! Spleen is the major site of sickling and destruction of RBC Prophylactic PO Penicillin Reduce the chance of pneumococcal sepsis Vaccines HIB (Haemophilus influenza type B) Meningococcal Bone Marrow Transplant

Complications of SCA STROKE CHEST SYNDROME OVERWHELMING INFECTIONS
Sickled cells block major vessels to the brain CHEST SYNDROME Resembles pneumonia May lead to restrictive lung disease and pulmonary hypertension OVERWHELMING INFECTIONS Due to defective splenic function May cause death in children <5 years

What do you think? Pat is a 4 year old being admitted because of diminished RBC production triggered by a viral infection. What type of sickle cell crisis is she most likely experiencing? Vasoocclusive crisis Splenic sequestration crisis Aplastic Crisis Hyperhemolytic crisis

What do you think? Therapeutic management of sickle cell crisis generally includes which one of the following? Long-term oxygen use to enable the oxygen to reach the sickled RBCs Decrease in fluids to increase hemoconcentration Diet high in iron to decrease anemia Bed rest to minimize energy Expenditure

HEMOPHILIA A group of bleeding disorders resulting from a congenital deficiency of specific coagulation proteins Excessive uncontrollable bleeding! X-linked recessive trait: MOTHER passes it on to SON Boys (XY): Hemophilia is transmitted on X chromosome Girls (XX): Inherits the carrier status ONLY

Two types of Hemophilia
Hemophilia A Classic hemophilia Most common – 75% Deficiency in clotting Factor VIII Hemophilia B Also called “Christmas disease” Deficiency of clotting Factor IX

Diagnosis of Hemophilia
Based on history of bleeding Abnormal PTT Prolonged > 40 seconds -- (Normal = seconds) Platelet function Normal Specific tests for factor VIII and IX assay DNA testing

Signs and symptoms Infant Child
Excessive bleeding at umbilical or circumcision site Child Excessive bleeding anywhere in the body Hematuria Melena (black, tarry feces) Epistaxis Hemarthrosis = joint bleeding Very painful Dangerous = blood loss Bone changes and cripling deformities over years

Hemophilia

Treatment of Hemophilia
Main focus: replacement of missing clotting factors Blood transfusion for Factors VIII or IX Cryoprecipitate (plasma derivative with factor VIII): NO LONGER RECOMMENDED! – cannot guarantee safe elimination of Hepatitis or HIV Corticosteroids DDAVP (I-deamino-8-D-arginine vasopressin): IV or intranasal Causes vasoconstriction Increases plasma factor VIII Plasminogen activator Analgesics, but NO aspirin

TEACHING R = Rest I = Ice C = Compression E = Elevation
Goal is to prevent bleeding! Dental care: soft toothbrush No contact sports Avoid aspirin Diet: avoid obesity (stress on joints); iron rich foods Genetic counseling Support groups Teach s/s of bleeding If bleeding occurs: RICE R = Rest I = Ice C = Compression E = Elevation

Complications from Hemophilia
Bone changes = crippling deformities GI hemorrhage Intracranial bleeding Bruising over spinal cord = paralysis Airway obstruction if bleeding in throat/pharynx

What do you think? Which one of the following is the most frequent form of internal bleeding in the child with hemophilia? Hemarthrosis Epistaxis Intracranial hemorrhage Gastrointestinal tract hemorrhage

Beta (B) Thalassemia Major
Also called Cooley’s anemia Group of disorders characterized by reduced production of the globin chains in the synthesis of hemoglobin (B-chain affected) Cultures such as Italians, Greeks and Syrian living near the Mediterranean Sea are affected Another group is the Alpha, affecting groups such as Chinese, Thai and Africans Autosomal recessive trait: both males and females must be carriers in order to pass it on

Types of Thalassemia Thalassemia major Thalassemia minor
Cooley’s anemia Homozygous Those who have the diseases Severe anemia and not compatible with life without transfusion support Thalassemia minor Heterozygous Have trait only Asymptomatic or mild microcytic anemia Thalassemia intermedia Moderate to sever anemia and spenomegaly

Diagnosis Hemoglobin electrophoresis (analysis of protein mixtures) confirms the diagnosis and distinguishes the type and severity There is no cure! Children usually die in late adolescent Must cope with frequent blood transfusions in the late stages Genetic counseling in all types

Signs and symptoms Pallor Fatigue r/t hypoxia Poor feeding
Hepatosplenomegaly Neurological impairment r/t hypoxia Bone and joint pain Anorexia Exercise intolerance Growth retardation

Long term complications
Splenomegaly (splenectomy) Hepatomegaly: cirrhosis with jaundice (yellow from bilirubin mixed with retained iron) = Bronze skin color Weak bones due to osteoporosis = spontaneous fractures Skeletal changes Thick cranial bones Enlarged maxilla Prominent facial bones Bossing (but not from hydrocephalus, from bone changes) “Frankenstein appearance” “Mongoloid” appearance Teeth: Malocclusion (malposition of mandibular and maxillary teeth) “Buck teeth” = crowded and crooked teath

Cardiac abnormalities
Dysrhythmias CHF Fibrotic cardiac muscle Gall bladder disease cholecystectomy Growth retardation Endocrine problem (DM) due to iron deposit’s effect on pancrease Delayed sexual maturation Small genitalia Decreased pubic hair

Treatment SUPPORTIVE! Transfusions are the foundation of medical management (keep Hgb >9.5) Iron Chelation theraphy = Deferoxamine Due to multiple transfusion, iron stores increases Helps pull iron from tissues and eliminate Fe Give with oral Vitamin C (to draw iron out of tissues) It is given either of 2 ways: SQ – over 8-10 hours 5-7 days/week (during sleep) IV – over 4 hours Can also be given as deep IM injection Prophylactic Antibiotics

Folic acid supplements (Vitamin B9) – stimulates production of RBCs
Avoid injury, rest, good hygiene and nutrition Avoid infectious persons Avoid contact sports Support groups Genetic counseling

What do you think? Norma, age 2 years, is to begin therapy for B-thalassemia. Which one of the following would be appropriate for the nurse to include in the educational session held with the parents? Norma will need frequent blood transfusions to keep her Hgb level above 12g/dl Large doses of vitamin C will be needed throughout the disease Chelation therapy is delayed until after 6 years of age to promote normal physical development To minimize the effect of iron overload, deferoxamine (Desferal), an Iron-Chelating agent, will be given IV or SQ

DIC = Disseminated Intravascular Coagulation
ANY QUESTIONS?????

Idopathic Thrombocytopenic Purpura (ITP)
Aquired hemorrhagic disorder: Excessive destruction of platelets (thrombocytopenia) Purpura (petechia), mucocutaneous bleeding Occasional bleeding into tissues Normal bone marrow with unusual increase in large, young platelets Acute or transient Acute self limiting: “comes and goes” Chronic: “comes and goes” into remission (>6 month duration) Acute following viral illnesses such as measles, varicella, mumps and URI (onset usually 1-4 weeks after the viral illness) Suspected that an immune mechanism is the basis for the thrombocytopenia

Signs and symptoms Easy bruising especially over bony areas
Bleeding from gums / mucous membranes Epistaxis Menorrhalgia (painful menstruation) Hematemesis Hematomas Hemarthrosis Intracranial hemorrhage is a rare (<1% of cases), but serious outcome

Diagnosis Often diagnosed with clinical symptoms Thrombocytopenia
Platelet count <20,000 m3/dl On blood smear, the platelets are large (megathrombocytes) = increased marrow production Usually occurs around time of puberty Danger of bleeding with menses

Treatment Excellent prognosis (75% recover)
Sometimes no medical interventions, self-limiting Restrict activity! Prevention from trauma and bleeding Corticosteroids: Prednisone; Decadron (Dexamethasone) Increases platelet count temporarily Avoid long term therapy (usually <3 weeks) in order to decrease side effects: Bone marrow suppression Cushingoid changes Growth failure Blood transfusion = Only transient benefits Given in life threatening situations Platelet transfusion = Only transient benefits Platelets has a short survival Splenectomy If unresponsive to treatment Usually only with chronic ITP Decreased risk for hemorrhage

Treatment Chemotherapy agents = Not commonly used Danazol (danocrine)
Vincristine Severe side effects Danazol (danocrine) Decreases estrogen and halt menses IVIG (Gamma Globulin) IV administration of antibodies to spare the removal of antibody coated platelets in the spleen Sustained rises of platelet count Large doses may induce remission Rituxan Monoclonal antibody New and investigational Sandimmune (cyclosporin) Used to prevent organ rejection and inhibit WBC growth factors

Excorim System (Protein A Immunoabsorption)
Antibody removal system, used mostly in Europe Plasmapharesis: Plasma is removed and filtered until desired lowered immunoglobulin level is achieved: Plasma is removed and “rinsed” before being re-mixed with blood in the cell separator and returned to the patient. The process is performed on-line and continuous until the desired amount of plasma is processed. Very noisy, LIJ has it Anti-D antibody Temporary and sometimes long-term elevation of the platelet counts Works by coating the RBC and blocking the spleen’s destruction of certain platelets. By doing so, there is an increase in platelet count within 1-3 days with a peak in counts 8 days after infusion The elevation in platelets last approximately 1 month – longer than IVIG Advantage over IVIG in that it is given IV push and IT IS CHEAPER! Pre-medicate: Decrease risk of reaction Decrease pain

What do you think? Which one of the following does the nurse recognize as true when administering anti-D antibody for ITP? The platelet count will increase immediately after administration Eligible patients include those with lupus Bone marrow examination to first rule out leukemia is necessary before administration Pre-medicate the patient with Acetaminophen before medication is infused.

LEUKEMIA

LEUKEMIA Description Malignant exacerbation in the number of leukocytes, usually at an immature stage, in the bone marrow Affects the bone marrow causing anemia, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunity The cause is unknown and appears to involve gene damage of cells leading to the transformation of cells from a normal state to a malignant state

LEUKEMIA Description (cont’d) The most common form of childhood cancer
Peak incidence is age 2-6 years for acute lymphocytic leukemia (ALL) Dramatic improvements in survival rates, 80% (>5 years) due to the extensive research Risk factors include genetic, viral, immunologic, and environmental factors and exposure to radiation, chemicals, and medications Risk factors in children include those with Down’s syndrome or a twin of a child who has had leukemia

What do you think? Of the following assessment findings, the one that would most likely be seen in a child with leukemia is: Weakness of the eye muscle. Bruising, nosebleeds, paleness, and fatigue. Wheezing and shortness of breath. Abdominal swelling

Affects bone marrow = anemia + bleeding
Diagnosis is based on % of blasts (immature WBC) in the blood CBC will show May have an increased WBC, BUT they are really leukopenic because the WBC are IMMATURE Decreased Hgb/Hct = ANEMIA Decreased platelets = thrombocytopenia

LEUKEMIA Classification of Leukemia Acute Lymphocytic Leukemia (ALL)
Mostly lymphoblasts present in bone marrow Age of onset is less than 15 years Usually higher success in treating 3 subtypes (“markers” on cell surface antigens) 84% of the leukemias Acute Myelogenous Leukemia (AML) Mostly myeloblasts present in bone marrow Age of onset is between 15 and 39 years Survival rate is not as positive 8 subtypes Approximately 20% of the leukemias

LEUKEMIA Assessment: COMMON PRESENTATION!!!
Most of the signs/symptoms are a result of bone marrow infiltrate Anorexia, fatigue, weakness, weight loss Pallor and anemia Bruising and bleeding from the nose or gums, rectal bleeding, hematuria Prolonged bleeding after minor abrasions or lacerations Hemorrhage and Petechiae Elevated temperature

Lymphadenopathy, splenomegaly, hepatomegaly from marked infiltration
Lymphadenopathy, splenomegaly, hepatomegaly from marked infiltration. May lead to fibrosis Palpitations and tachycardia Dyspnea on exertion Vague abdominal pain caused by inflammation from normal flora invading intestinal tract Wasting of major organs due to infiltration of leukemic cells Increased ICP if meninges are infiltrated CNS: severe headache, vomiting, papilledema (edema & inflammation of the optic nerve which may result in blindness), irritability, lethargy and eventually coma Muscle wasting, weight loss, anorexia and fatigue

LEUKEMIA

LEUKEMIA Infection A major cause of death in the immunosuppressed child Can occur through autocontamination (i.e. Staph aureus on the skin) or cross contamination Most common sites of infection are the skin, respiratory tract, and GI tract

Neutropenic Diet Reverse Isolation
Infection: Neutropenic Diet Reverse Isolation

LEUKEMIA Protecting the Child from Infection
Initiate protective isolation procedures Maintain child in a private room Frequent and thorough handwashing Strict aseptic technique for all nursing procedures Limit the number of caregivers entering the child’s room and ensure that anyone entering the child’s room is wearing a mask = REVERSE ISOLOATION Keep supplies for child separate from supplies for other children Reduce exposure to environmental organisms by eliminating raw fruits and vegetables from the diet, and keeping fresh flowers and standing water out of the child’s room * Neutropenic diet

What do you think? The severe cellular damage that is caused by chemotherapy drugs infiltrating into surrounding tissue occurs when the chemotherapeutic agent is a(n) Hormone Steroid Vesicant antimetabolite

LEUKEMIA Bleeding During the period of greatest bone marrow suppression (the nadir), the platelet count may be extremely low Children with platelet counts below 20,000/mm3 may need a platelet transfusion For children with severe blood loss, packed red blood cells may be prescribed (for Hgb <8.0)

LEUKEMIA Fatigue and Nutrition
Assist the child in selecting a well-balanced diet Provide small meals that require little chewing Assist the child in self-care and mobility activities NORMALIZE their life as much as possible!

LEUKEMIA Chemotherapy Monitor for severe bone marrow suppression
Monitor for infection and bleeding Protect child from life-threatening infections for at least 2 to 3 weeks following chemotherapy Monitor for nausea, vomiting, and diarrhea Assess oral mucous membranes for stomatitis Monitor for renal, hepatic, and cardiac toxicity

LEUKEMIA Chemotherapy
Inform parents that hair loss may occur from chemotherapy Instruct parents about the care of central venous access devices as necessary Listen and encourage child and family to verbalize their feelings and express their concerns Introduce family to other families of children with cancer

Treatment (i.e. NYII protocol)
INDUCTION THERAPY 4-6 weeks in length Goal is to achieve complete remission Corticosteroid = decrease inflammation Vincristine and L-asparaginase with or without Doxorubicin CNS prophylactic therapy: IT (not IV due to the blood-brain barrier) Methotrexate – given directly into the CSF via LP. It protects against CNS invasion of leukemic cells. Radiation used to be done but changed due to long term side effects. Goal is met if <5% blasts in BM If CNS disease is present, give Sanctuary therapy = combination of chemotherapy agents, more intense and therefore need to watch for neurotoxicity

Always concerned about tumor lysis syndrome during induction therapy
Caused by sudden, rapid death of cells in response to treatment and that in terms causes electrolyte and metabolic disturbances To prevent tumor lysis syndrome: Alkalanization = 2X IV maintenance therapy with NaHCO3 Allopurinol to decrease uric acid Amphojel to decrease phosphate Strict I&O maintaining urine pH >7.5 Tumor lysis syndrome would be a concern with: Increased Phosphate Increased Uric Acid Increased Potassium Decreased Calcium

MAINTENANCE Also called consolidation
Serves to maintain the remission phase Duration may be 2 1/2 to 3 years Continue corticosteroids, methotrexate and vincristine

Side effects to treatment
Chemotherapy Nausea and vomiting Alopescia (temporary) Certain agents, i.e cytarabine (cytosin) will cause peripheral neuropathy with decreased sensation and reflexes Interthecal chemotherapy – Ommaya resorvoir or LP Spinal headache Keep flat or Trendelenberg for at least 1 hour after administration to prevent headache and distribute drug throughout the body

Bone Marrow Transplant
Not recommended for children with ALL during first remission because of the excellent result with chemotherapy during second remission Complete reverse isolation Pt is typed and crossed with donor marrow Sibling is the #1 choice for HLA match BM donor banks – difficult for certain ethnicities BM aspirate – local anesthesia

Outcome “Cured” if 5 years in remission after treatment
Adjunct goal: Pt has no assessment of disease but cancer cells are evident through tests Palliative goal: Make them “comfortable” and control s/s until death occurs (i.e. Hospice or home) “To Live Among Lions”

&/OR INEFFECTIVE BREATHING PATTERNS
IMPAIRED GAS EXCHANGE &/OR INEFFECTIVE BREATHING PATTERNS

Infections of the upper and middle airways

TONSILLITIS An infection or inflammation (hypertrophy) of the palatine tonsils Most children with pharyngitis have infected tonsils, but NOT necessarily tonsillitis (may “just” have phyaryngitis) Viral or bacterial Tonsils are lymphoid tissues: filter and protect respiration tract from invasion of microorganisms form antibodies Children usually have larger tonsils than adolescents or adults Adenoids are located above the tonsils Tonsillitis usually occurs with pharyngitis

TONSILLITIS ASSESSMENT: DIAGNOSIS: Enlarged and edematous tonsils
“Kissing tonsils” = they may meet at midline and obstruct passage of food or air Frequent throat infections Cervical lymphadenopathy Difficulty swallowing or breathing Adenoids enlarge and cause difficulty for air to pass from nose to pharynx. Usually snoring when adenoids are affected Mouth breathing Drying of mucous membranes secondary to mouth breathing Otitis media and possibly decreased hearing may result (adenoids close to eusthasian tubes) DIAGNOSIS: Based of visual inspection and clinical manifestation Throat culture

TONSILLITIS MANAGEMENT: Symptomatic treatment: Provide comfort
Acetaminophen (may be given PR) or ibuprofen to decrease inflammation and throat pain Cool, non-acidic fluids and soft foods; throat lozenges Ice chips or frozen juice pops (increase hydration) Humidification / vaporiziation (cool mist) Gargle with warm salt water (soothing) Antibiotics based on C/S (Throat culture: pharyngeal – tonsil swab). First line antibiotic is usually penicillin base (i.e. amoxicillin) Surgery: Tonsillectomy &/or adenoidectomy (T&A) Recommended if recurrent throat infections (documented Strep throat: >3 in 6 months or >5/ in one year) Chronic tonsillitis Obstructive sleep apnea Nasal speech After the age of 3 years (it can stimulate growth of other lymphoid tissue in the nasopharynx)

TONSILLITIS NURSING PRE-OP and POST-OP: Teaching is very important
Baseline VS History of bleeding pattern Side-lying following surgery to drain secretions No coughing frequently, clearing throat and blowing nose - may aggravate operative site Assess for hemorrhage: Most common within the first 24 hours or 7-10 days after tonsillectomy (scar is forming during that time) Use penlight Inspect secretion and vomit for evidence of fresh blood Pallor Increased pulse (>120, but depends on age-group – refer to G&D chart) If bleeding is suspected, call MD immediately Diet: Cool water, crushed ice, ice pops, dilute fruit juice (avoid citrus - acidic - irritating to site), soft foods - cooked fruits, jello, soup, etc

TONSILLITIS Airway obstruction may occur due to edema or accumulated secretions Assessment of respiration distress: Stridor Drooling Restlessness Increased respiration rate Suction & 02 should be available Assess for infection (most common first 7-8 days): White coating in back of throat Odor Low grade fever (report temp >102) (38.8C) Discharge teaching: Avoid highly seasoned food Avoid gargling Avoid vigorous tooth-brushing Avoid Ibuprofen the first post-op week. Use acetaminophen Discourage coughing and throat clearing Use mild analgesics Limit activity to decrease potential for hemorrhage Persistent sever earache, fever or cough required MD evaluation.

What do you think? Adenoidectomy would be contraindicated in a child
with recurrent otitis media. with malignancy. with Thrombocytopenia. over the age of 3 years.

What do you think? Which of the following food(s) is/are the MOST appropriate to offer first to an alert child who is in the post-operative period following a tonsillectomy? Ice cream Red gelatin Flavored Ice Pops All of the above

CROUP SYNDROME Term applied to a broad classification of upper airway illnesses that result from swelling of the epiglottis and larynx, often extending into the trachea and bronchi Boys > girls Seasonal: Late autumn and early winter = more frequent episodes Classification system of croup includes viral syndromes such as (the “big three” of pediatric respiratory illness = affects the greatest number of children across all age groups in both sexes): 1) Spasmodic laryngitis (spasmodic croup)

CROUP SYNDROME 2) Laryngotracheobronchitis (LBT) = most common!
3 months - 8 years Viral invasion Slow progression. Specific symptoms: URL Stridor Degree of inspiratory stridor and may lead to respiration distress due to edema or obstruction of airway Seal-like “barking” cough Resonant cough: “brassy” like in sound Hoarseness Dyspnea Restlessness Irritability Low grade fever Nontoxic Treatment: Humidity Racemic epinephrine.

CROUP SYNDROME 3) Bacterial syndromes (includes bacterial trachitis and epiglottitis) 1-8 years old Bacteria Invasion (Haemophilus influenzae) Almost non-existent due to vaccination: Hib = PREVENTION! Rapidly progresses Specific symptoms Dysphagia Stridor aggravated when supine Drooling High fever Toxic Tachycardia and tachypnea Treatment: Antibiotic therapy Airway protection (intubate in the OR) Corticosteroids for edema

What do you think? In the child who is suspected to have epiglottitis the nurse should: Have Intubation equipment available Prepare to immunize the child for Haemophilus influenzae Obtain a throat culture All of the above

CYSTIC FIBROSIS Inherited autosomal recessive disorder (both parents) of the exocrine glands that results in physiologic alterations in: Respiratory system: Abnormal accumulation of viscous, dehydrated mucus Inflammation and lung changes Gastrointestinal system Integumentary system Musculoskeletal system Reproductive system All body organs with mucous ducts become obstructed and damaged Predominantly in white children Gender is not a factor Average life span is 30 years (may vary)

CYSTIC FIBROSIS Blocked pancreatic ducts and resulting pancreatic damage causes a STOP in the secretion of natural enzymes necessary to digest fats and protein. As a result, essential nutrients are excreted in the stool ESSENTIAL CHANGES IN PATHOPHYSIOLOGY: Respiratory system: Bronchi, bronchio-pneumonia & bronchial emphysema (barrel chest) Increased secretion requiring intervention Classic cough secondary to increased mucus Atelectasis Secondary respiratory infections = increased morbidity and mortality Small intestine: Obstruction from mucous If newborn does not pass meconium you must R/O cystic fibrosis

CYSTIC FIBROSIS Pancrease: Ducts blocked Unable to absorb & digest fat
Missing pancreatic enzymes that break down fat Steathorrhea Frothy (bulky and large in quantity) Foul smelly Contain fat (greasy) Bile ducts: Obstructed May lead to cirrhosis Jaundice, portal HTN Sweat glands and salivary glands: Lose a lot of salt – electrolyte imbalances due to loss of Na and Cl SALTY KISSES!!!!!! – initial S/S Reproductive system: Male sterility due to blockage or absence of the vas deferens Females difficulty conceiving due to increased mucus secretions in the reproductive tract interfering with sperm passage

CYSTIC FIBROSIS DIAGNOSIS: ASSESSMENT: Family history - genetic
SWEAT TEST: patch on skin with measure of amount of Na absorbed Chloride concentration of mEq/L is suspicious Chloride concentration >60mEq/L is diagnostic If positive: Pancreatic enzyme fast (eliminate) and check for steatorrhea CXR: to check for mucus CNS: Possibly hyperactive children (evident early in childhood) ASSESSMENT: INTESTINAL & PANCREAS: Steatorrhea (fat, frothy, foul smelling) Eats large amount, but does not gain weights because can not absorb fat Missing pancreatic enzymes - Trypsin, Lipase and Amylase. Need vitamins because fat-soluble vitamins will not be absorbed through food RESPIRATORY: Wheezing, rales, dyspnea, non-productive dry cough May develop atelectasis Clubbing May have ear, nose and throat problem more than average kids.

CYSTIC FIBROSIS Long-term complications
HEART: Corpulmonal - Right sided enlargement CHF = Pulmonary blood flow is obstructed by the mucous ESOPHOGEAL: Esophageal varisies = enlarged vein - may bleed LIVER: Jaundice SPLEEN: Spleenomegaly REPRODUCTIVE SYSTEM: ducts blocked, tubes blocked, mucous in vaginal tract vas deferens blocked

CYSTIC FIBROSIS TREATMENT: Respiratory:
Cupping, postural drainage, deep breathing, bronchodilators (Q4H before meals) – percussion/vibriation vest Aerosol treatments (bronchodilators) Nutrition: Increase SALT, especially with exercise HIGH CALORIES AND PROTEINS Salt tablets in warm weather Pancreatic enzymes pills taken with meals (non-fat & non-protein such as fruit, vegetables or carbohydrates). Sprinkle on food, “mix” in well AS A “SANDWICH”. Don't get on lips or skin = breakdown. NURSING: Teach about disease and how to explain the condition to the child Becomes progressively worse Avoid infection Encourage activity, rest, ventilate feelings, support groups Genetic counseling

Percussion Vest

Postural Drainage

ASTHMA Chronic inflammatory disorder of the airway with airway obstruction that can be partially or completely reversed Chronic condition with acute exacerbations or persistent symptoms Approximately 5 million children are affected in the United States (school absenteeism) Boys > girls until the age of 10 years and then it equals out Most are diagnosed before the age of 5 years Called Reactive Airway Disease (RAD) in infants….. Discussion: RAD vs ASTHMA ETIOLOGY: Bronchospasm Increased mucous secretion Usually results from allergic hyper response. Before puberty – boys Air gets trapped in the lungs and cannot get out (wheeze) Major allergic component: Seasonal allergies Irritants: smoke Roach allergy Food allergies Skin sensitivity tests to determine causative agents

ASTHMA ASSESSMENT: Respiratory difficulties of an asthma attack result from inflammation that contributes to airway obstruction (narrowing) Mucus formation , mucosal swelling and airway muscle contraction A stimulus or TRIGGER initiates an asthmatic episode S/S visible includes: Air hunger Dyspnea Anxiety Coughing (productive vs non-productive) Fatigue Wheezing - inspiratory and/or expiratory Tachypnea Retractions Cyanosis, and diaphoresis --- late signs Barrel chest (chronic)

ASTHMA

TREATMENT OPTIONS Long-term control medications
Leukotriene modifiers Long-acting beta-2 agonists (LABAs) Theophylline Quick-relief medications Short-acting beta-2 agonists Ipratropium (Atrovent) Oral and intravenous corticosteroids Medications for allergy-induced asthma Immunotherapy Anti-IgE monoclonal antibodies

Long-term control medications (Usually taken on a daily basis)
Inhaled corticosteroids: fluticasone (Flovent Diskus), budesonide (Pulmicort), triamcinolone (Azmacort), flunisolide (Aerobid), beclomethasone (Qvar) and others. Reduce airway inflammation and are the Most commonly used long-term asthma medication Low-risk for side effects as compared to systemic corticosteroids These inhaled medications work by opening airways, reducing inflammation and decreasing mucus production. Leukotriene modifiers: montelukast (Singulair), zafirlukast (Accolate) and zileuton (Zyflo CR) Inhaled medications; work by opening airways, reducing inflammation and mucus production

Long-term control medications (Usually taken on a daily basis)
Cromolyn and nedocromil (Tilade) Inhaled medications reduce asthma signs and symptoms by decreasing allergic reactions Considered a second choice to inhaled corticosteroids Given TID to QID Long-acting beta-2 agonists (LABAs): salmeterol (Serevent Diskus) and formoterol (Foradil Aerolizer). Inhaled, long-acting bronchodilators, open the airways and reduce inflammation Used in combination with inhaled corticosteroids with persistent asthma Long-acting bronchodilators should not be used for quick relief of asthma symptoms Theophylline Daily tablet that opens your airways (bronchodilator) Relaxes the muscles around the airways – VERY RARELY USED

Quick-Relief Medications (also called rescue medications)
Short-acting beta-2 agonists: Albuterol and Xopenex Inhaled bronchodilators, relax airway muscles Act within minutes, and effects last four to six hours Ipratropium (Atrovent) Inhaled anticholinergic for the immediate relief of your symptoms; relaxes the airway Mostly used for emphysema and chronic bronchitis Oral and intravenous corticosteroids Prednisone and methylprednisolone In treatment of acute asthma attacks and severe asthma May cause serious side effects when used long term. Used short-term for asthma

Other Asthma Medications
Medications for allergy-induced asthma: To decrease the body's sensitivity to a particular allergen or prevent the immune system from reacting to allergens. Allergy treatments for asthma include: Immunotherapy: Allergy-desensitization shots (immunotherapy) are generally given once a week for a few months, then once a month for a period of three to five years. Over time, they gradually reduce your immune system reaction to specific allergens. Anti-IgE monoclonal antibodies: omalizumab (Xolair) Reduces the immune system's reaction to allergens Xolair is delivered by injection every two to four weeks.

ASTHMA TREATMENT Most commonly what we see:
Bronchodilators: Beta2-agonists (short-acting: Proventil / Albuterol) for ACUTE ATTACK. Proventil: teach about SE including tachycardia, nervousness, tremors Inhaled steroids: Used as a prophylactic (i.e. before exercise), during and after exacerbation of asthma for more effective treatment (i.e. fluticasone (Flovent Diskus), budesonide (Pulmicort), flunisolide (Aerobid), combination drug such as Advair [Fluticasone and Salmeterol]) Now considered MOST EFFECTVE treatment in asthma: Reduces asthma symptoms and flare-ups Improves lung function Reduces bronchial reactivity NOT ADDICTIVE! Increased risk of thrush Rinse mouth and/or brush teeth after treatment

Treatment (cont.) GIVE ALL MDI MEDICATIONS WITH SPACER
Oral corticosteroids: If inhaled steroids are not used, systemic steroids with a 5 day course of Prednisone is usually given Due to the short course of treatment, it is very rare to see any of the common side effects to corticosteroids: HTN, muscle wasting, adrenal suppression, Cushing's, impaired immune system, hirshuism, anorexia Anti-inflammatory: Used for prophylaxis such as Cromolyn NOT USED during exacerbation of asthma

ASTHMA Epinephrine: Fast acting (arrhythmias, nervous, restless, tremor, headache, insomnia). Used more if asthma is caused by allergic reaction Aminophylline: VERY RARELY SEEN USED Must be administered through IV Pump! (tachycardia, nervous, n & v, anxiety, seizures) Theophyllin: VERY RARELY SEEN USED! Intoxication can occur-monitor serum concentrations (seizures, n/v, tachycardia, anxiety)

ASTHMA NURSING: Know baseline PEAK FLOW METER:
Best attempt out of three rapid expirations Consider the zone they are in (red, yellow, green) High Fowler's position and bend forward in mild exacerbation CPT: Always do aerosol therapy before CPT Oxygen Suctioning as needed VS very important Monitor PC02 (35-48) & P02 (80-100) pH ( ) if possible (ABG or VBG) Pulse oximetry (keep PO2 >95%) Extensive IV fluids to hydrate them and liquify secretions Antibiotics prophylactically or specific for present infection Promote normal activities Prevent further episodes, support.

STATUS ASTHMATICUS: Acute, severe prolonged asthma attack.
Does not respond to normal treatment. Person can die. Teach patient not to wait too long before initiating treatment or seeing MD/NP

ASTHMA

BRONCHIOLITIS Lower respiratory tract illness caused by virus or bacteria which causes inflammation and obstruction of the bronchioles Occurs mostly in in toddlers and preschoolers Most severe in infants under 6 months (if <2 months, very prone and hospitalized) Most common cause: Respiratory Syncytial Virus (RSV): Viral infection mostly of bronchiolar level Primarily occurs in winter & spring The disease usually begins in the fall, reaches a peak in the winter and decreases in the spring Bronchiole mucosa swells and fill with mucous and exudate Frequently sloughed epithelial cells obstruct the lumen, mostly on expiration Hyperinflation could occur and cause air trapping The trapped air distal to the obstruction causes progressive overinflation (emphysema) Transmitted via respiratory secretions, hand to eye, nose or other mucous membrane.

BRONCHIOLITIS ASSESSMENT: Rhinorrhea Pharyngitis Coughing Sneezing
Breath sounds: wheezing, diffuse crackles/rhonchi, rales Intermittent fever. With progression of illness: Increase coughing and wheezing Air hunger, tachypnea, retractions, cyanosis

INFANTS' ASSESSMENTS: DIAGNOSIS: Severe illness:
Tachypnea >70/min., listlessness, apneic spells, poor air exchange, poor breath sounds. OM and conjunctivitis may also be present. Once the lower airway is involved, classic manifestations include: Signs of altered air exchange, such as wheezing, retractions, crackles, dyspnea, tachypnea and diminished breath sounds. INFANTS' ASSESSMENTS: Poor feeding, slight lethargy, irritability, possibly low grade fever DIAGNOSIS: Tests done on nasal or nasopharyngeal secretions (swab or aspirate) using IFA or ELISA techniques for RSV antigen detection.

BRONCHIOLITIS TREATMENT: Based on the symptoms
PREVENTION: RSV Medication: SYNAGIS (palivizumab) Monthly injection in the winter-months which provides antibodies Used for premis and other high-risk groups High humidity Adequate fluid intake Rest Medications depending on symptoms and cause Hospitalization: Usually recommended for children with complicating conditions, such as underlying lung or heart disease. The child who is tachypneic, has marked retractions, seems listless, history of poor fluid Intake should also be admitted. Any infant born premature or SGA Mist therapy combined with oxygen to maintain adequate O2 saturations IV fluids are preferred until crisis of the disease has passed Blood gas values guide the therapy (if available)

BRONCHIOLITIS MEDICATIONS:
Bronchodilators, corticosteroids, cough suppressants and antibiotics have not proved to be effective. Bronchodilators may be used for symptomatic wheezing For intubated and vetilated patients: FUROSEMIDE, THEOPHYLLINE AND CORTICOSTEROIDS RIBAVIRIN An anti-viral agent is the only specific therapy available for RSV. HOWEVER, studies have NOT confirmed its effectiveness and it is therefore reserved for life-threatening cases (i.e. premature infants, underlying conditions, etc) VERY RARELY SEEN USED! Ribavirin has many precautions: Visitors and nurses must know about the potential harm to themselves if exposed to the drug Teratogenic: Pregnant women should not be exposed to medication Cannot use contact lenses with administration as it calcifies Administered via mist in hood or tent

BRONCHIOLITIS High risk infants and children: NURSING:
RespiGam (RSV immune globulin) can be used as preventative treatment for RSV as well as Synagis NURSING: Hand washing and not touching nasal mucosa or conjunctiva Diminish the number of personnel, visitors and uninfected patients in contact with the child Nurses try to avoid taking other patients who are considered high risk for catching RSV.

THE END!

OXYGENATION in Pediatrics

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OXYGENATION in Pediatrics

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