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304 Cards in this Set
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Circulation and changes after birth.
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A. Three Fetal Circulation Shunts
-- Ductus venosus – a structure that shunts blood past the portal circulation -- Foramen ovale – an opening between the right and left atria of the heart -- Ductus arteriosus – a vessel between the aorta and the pulmonary artery that shunts blood from the PA to aorta * Shunts should close in several days after delivery. B. Fetal Circulation: 1. Main blood flow: Placenta --> Umbilical Vein --> Liver --> Ductus Venosus --> Inferior Vena Cava --> Right Atrium --> Foramen Ovale (by pass going to lungs for oxygenation) -->Left Atrium --> Left Ventricle --> Aorta --> Body 2. Secondary blood flow: Right Atrium --> Right Ventricle --> Pulmonary Artery --> Ductus Arteriosus (bypass going to the lungs because the blood is already oxygenated) --> Aorta --> Body 3. Third route of blood flow – very minimal amount Right Atrium --> Right Ventricle --> Pulmonary Artery --> Lungs (needs to perfuse the lungs and upper body with oxygen) -->Left Atrium --> Left Ventricle --> Aorta --> Body C. Changes in Circulation at Birth -- The umbilical arteries and vein and the ductus venosus become non-functional -- Decreased pulmonary vascular resistance and increased pulmonary blood flow -- Increase in pressure of the left atrium, decrease pressure in right atrium, causing closure of foramen ovale. -- Pulmonary resistance is less than systematic resistance so there is left-to-right shunting resulting in closure of the ductus arteriosus. |
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What is normal newborn circulation? **What happens after birth?
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1. Ductus venosus
a. Umbilical vein carries oxygenated blood from placenta to infant b. Blood bypasses liver through ductus venosus c. When umbilical cord is clamped and cut, blood flow ceases and ductus venosus closes d. Blood flows into liver 2. Foramen ovale a. Systemic blood enters right atrium b. Oxygenated blood flows from right to left atria through foramen ovale c. Blood bypasses lungs which are nonfunctional d. Blood flows from left atria to left ventricle and out to aorta e. Foramen ovale closes after birth with change in pressure in cardiac chambers 3. Ductus arteriosus a. A fistula between aorta and pulmonary artery allows for mixing of blood b. Blood flowing through pulmonary artery may enter aorta through a patent ductus arteriosus c. **Ductus arteriosus closes after birth, sometimes with first few breaths but may take up to 3 days |
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What problems can occur from newborn circulation and improper closing of ducts?
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Several congenital (present at birth) heart defects can occur if one of the blood shunts in the fetal circulation system does not close properly after birth (a shunt that remains open is said to be patent).
Patent Ductus Venosus (PDV)- A patent ductus venosus is uncommon, being very rare in children and even more so in adults. It can contribute to portosystemic encephalopathy, a central nervous system disorder believed to be the result of liver problems and/or liver failure. Once diagnosed, PDV can be treated using a catheter, surgery, or other more conservative methods. Patent Ductus Arteriosus (PDA)- If the ductus arteriosus remains open (patent) after birth, it results in abnormal blood flow between the aorta and the pulmonary artery. This allows oxygenated blood from the aorta to mix with oxygen-poor blood in the pulmonary arteries. A small PDA may not have any damaging effects on the baby other than a heart murmur. However, large PDAs are more serious and put extra strain on the heart and lungs. The lungs may become congested and the child may experience difficulty breathing. In addition, the extra blood flowing to the lungs increases pressure in the lung's blood vessels, which may lead to permanent damage if not corrected. If the PDA is small, it may close on its own and not require treatment. Medication has been effective in treating some premature babies with PDAs. However, for larger PDAs, catheterization or surgery can be used to close off the PDA. Doctors can use a catheter (long thin tube) to insert a device that will block the PDA, or they may surgically close the PDA with a clamp or suture. Patent Foramen Ovale (PFO)- If the foramen ovale does not properly close after birth, the valve may continue to open when the chest is subjected to pressure, such as while coughing or sneezing. This means that blood can flow between the right and left atrium. People with PFO may have an increased risk of stroke and migraines. In fact, 40 percent of patients who have a stroke with no known cause are diagnosed for PFO. A patent foramen ovale may also be linked to an atrial septal aneurysm, in which the atrial septum is more mobile than normal. Many people with PFO have no symptoms, but it can be detected by an echocardiogram or a transesophageal echo. If there are no problems resulting from the PFO, treatment may not be needed. However, if the PFO leads to serious problems such as a stroke, a doctor can close it through catheterization or through surgery. Medication may also be prescribed to treat the PFO. |
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What are post cardiac catherization priorities for you as a nurse?
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1. Monitor findings on the cardiac monitor and oxygen saturation for 4 hours after procedure.
2. Assess pulses below the catheter site for equality and symmetry (pulse distal to the site may be weaker for the first few hrs but should gradually increase in strength). 3. Assess the temperature and color of the affected extremity and report coolness, which may indicate arterial obstruction. 4. Monitor vital signs frequently, usually every 15 minutes four times, every half-hour four times, and then every hour four times. (Watch HR, count for 1 full min). (Watch BP for hypo-tension--may indicate hemorrhage.) 5. Assess the pressure dressing for intactness and signs of hemorrhage. 6. Check the bed sheets under the extremity for blood, which may indicate bleeding from the entry site. 7. If bleeding is present, apply continuous, direct pressure at the cardiac catheter entry site and report it immediately. 8. Immobilize the affected extremity in aflat position for at least 4 to 6 hours for venous entry site and 6 to 8 hours for arterial entry site as prescribed. 9. Hydrate the child via the oral or intravenous route or both routes as prescribed (Bloss loss in cath lab, the child's NPO status, and diuretic actions of dyes = hypovolemia & dehydration). 10. Administer acetaminophen (Tylenol) or ibuprofen (Motrin) for pain or discomfort as prescribed. 11. Prepare the parents and child, if appropriate, for surgery. 12. Blood glucose levels for hypoglycemia, esp in infants who should receive dextrose containing IV fluids. For teaching for parents---- 1. Limit activity for 24 hrs post surgery to avoid disturbing the insertion site. 2. Child is instructed on the intake of fluids. Maintaining hydration is important b/c the contrast medium used during the procedure has a diuretic affect. 3. Monitor for temperature elevation, which is an early sign of infection. |
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What common procedures are done in the cath lab to correct congenital heart defects?
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--Valvuloplasty (widens a narrowed valve)
--Angioplasty (widens a narrowed blood vessel) --stent implanation (to keep narrowed vessel open!) |
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Patient education for after catherization?
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1. Remove the dressing on the day after the procedure and cover it with a Band-Aid for 2 or 3 days as prescribed.
2. Keep the site clean and dry. 3. Avoid tub baths for 2 to 3 days. 4. Observe for redness, edema, drainage, bleeding, and fever, and report any of these signs immediately. 5. Avoid strenuous activity, if applicable. 6. The child may return to school, if appropriate. 7. Resume regular diet w/o restrictions. 8. Administer acetaminophen or ibuprofen for pain, discomfort, or fever. 9. Keep follow-up appointment with primary care provider. |
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How do many congenital heart defects present?
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The most common heart anomaly is ventricular septal defect (VSD)
They typically present with A. Acyanotic defects & B. Cyanotic defects. Acyanotic (but may clinically have cyanosis)--- Left to right shunt Mainly oxygenated blood enters systemic circulation Result in overcirculation to the lungs CHF! Cyanotic (may not always actually have cyanosis) Right to left shunt Unoxygenated blood enters the systemic circulation May result in polycythemia Hypoxemia!! Problematic classification!! 1. Increased pulmonary blood flow-- Typically represented as left to right shunt (blood flows from higher pressure to lower) = CHF, Atrial septal defect, ventircular septal defect, patent ductus arteriosus (Septal defect occurs when there is a hole between two atriums!) Obstruction defects- Impede blood flow out of the ventricles; obstruction on left side= CHF, right side= cyanosis .... Coaractation of aorta, aortic stenosis, pulmonic stenosis (Stenosis is a narrowed valve that has trouble opening, Atresia is when there is NO opening!) Decreased pulmonary blood flow= cyanosis.... tetralogy of fallot, tricuspid atresia. Mixed blood flow-- transposition of great arteries, etc. **Most defects present with hypoxemia (w/ or w/o cyanosis) and CHF together. ***Explanation for shunting: Cyanotic: Deoxygenated blood bypasses pulmonary circulation and goes right to the left side of the heart to be pumped into the systemic circulation. Since it never got oxygenated in the lungs, the person will get cyanosis. Does that make sense? |
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What is persistent pulmonary hypertension (PPH)?
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When a baby is in the womb, the oxygen is supplied through the umbilical cord. After birth, the baby's system should switch to receive oxygen from the lungs. In babies born with PPHN, the heart, blood vessels, and lungs (circulatory system) do not make the adjustment. When babies with PPHN are born, the blood does not interact with the lungs, and instead circulates as it did in the womb.
Babies with PPHN do not receive the necessary oxygen from the lungs that is normally supplied. Symptoms of PPHN typically appear within 12 hours after birth. |
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How is PPH treated? (pg. 292 class book)
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1. Regulate IV fluids
2. Provide supplemental O2 and assisted ventilation 3. Administer vasodilators to widen pulmonary artery. 4. Maintain acid-base balance. 5. Prevent hypoxemia and hypercarbia. 6. Adminster inhaled nitric oxide (NO) or extracorporeal membrane oxygenation (ECMO) 7. Viagra (from PPT) |
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How do PPH babies look like after birth?
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Hypoxia
Marked cyanosis Tachypnea with grunting and retractions Decreased peripheral pulses and prolonged cap refill (poor perfusion) shock |
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What happens if PPH isn't treated?
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Even with quick treatment, things resulting in shock, heart failure, brain hemorrhage, seizures, kidney failure, multiple organ damage, and possibly even death can occur.
Periods of inadequate oxygenation can have long-term effects on infants who survive PPHN, such as bronchopulmonary dysplasia (a chronic lung disease associated with scarred, stiffened lungs) and breathing difficulties. Seizure disorders, developmental delay, and neurological deficits may also be seen. *Weeks following treatment, infants with PPHN may not be able to feed by mouth. *Hearing problems are a common condition associated with PPHN. |
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What are signs of CHF ?
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Left Sided Failure
Crackles and wheezes Cough Dyspnea Grunting (infants) Head bobbing (infants) Nasal flaring Orthopnea Periods of Cyanosis Retractions Tachypnea Right Sided Failure Ascites Hepatosplenomegaly Jugular vein distention Oliguria Peripheral edema, esp. dependent edema, and periorbital edema. Weight Gain |
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What are S/S of decreased cardiac output?
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Decreased peripheral pulses
Exercise intolerance Feeding difficulties Hypotension Irritability Oliguria Pale, cool extremities Tachycardia |
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Atrial Septal Defect
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1. Hole in the atrial septum
--Patent Foramen Ovale—type of ASD 2. Causes RV overload (ventricular hypertrophy) with pulmonary congestion; can lead to right sided failure 3. Usually acyanotic/increased pulmonary blood flow Signs and symptoms 1. Often asymptomatic 2. May develop CHF if large and left untreated 3. Characteristic murmur 4. At risk for atrial dysrhythmias 5. May have exercise intolerance Treatment 1. Nothing--May close on its own if small 2. Patch closure 3. Open heart 4. Via cardiac cath |
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Ventricular Septal Defect
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1. Hole (s) in the ventricular septum
a. Swiss cheese VSD 2. Blood shunts from the left ventricle to the right ventricle and results in increased pulmonary circulation; can lead to right sided failure 3. Usually acyanotic/increased pulmonary blood flow Signs and sypmtoms 1. CHF common, pulmonary edema 2. Murmur 3. May have dysrythmias 4. Frequent pulmonary infections Treatment 1. Nothing--Small defect may close spontaneously 2. Patch closure 3. Open heart 4. Via cardiac cath |
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Patent ductus arteriosus
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1. Failure of ductus to close after birth
a. Blood shunts from aorta to pulmonary artery which results in overcirculation to the lungs b. Usually closes with first breath c. Can take up to 3 months d. Usually acyanotic/increased pulmonary blood flow Signs and symptoms 1. Murmur 2. Widened pulse pressure 3. Possible CHF 4. Pulmonary congestion 5. Chronic pulmonary disease Treatment 1. Medical (Indomethacin) 2. Via Cardiac Cath (Plug/Coil placement) 3. Surgical (Ligation or Clip placement ) |
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Coarctation of the aorta
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1. Narrowing of the aortic lumen results in > pressure proximal to the defect, < pressure distal to defect
2. Acyanotic/obstructive blood flow Signs and symptoms 1. Upper extremities--High B/P, bounding pulses 2. Lower extremities--Low B/P, poor or absent pulses, cool 3. Older children--H/A, vertigo, nosebleeds 4. Signs of CHF 5. Severity depends on degree of narrowing 6. History of Coarc—can recur Treatment 1.Balloon angioplasty via cardiac cath 2. Surgical resection |
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Tetralogy of fallot
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1. Obstruction of pulmonary blood flow
2. R to L shunt a. Bood has problem exiting the right side of heart, pressure increases, exceeding left side pressure 3. Includes four defectsVSD a. Pulmonic stenosis b. Overriding aorta c. RV hypertrophy Usually cyanotic/decreased pulmonary blood flow Signs and Symptoms 1. Cyanosis that increases with crying 2. CHF symptoms 3. Chronic hypoxemia manifestations Treatment 1. Initial BT shunt 2. Complete repair via open heart a. First year of life b. Close VSD c. Resect stenosis d. Pericardial patch to enlarge RV outflow |
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Transposition of the Great Arteries (TGA)
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1. Aorta arises from the RV
2. PA arises from LV 3. No communication between circulation—Two separate circuits a. Body-heart-body b. Lungs-heart-lungs 4. MUST have other defects to survive a. ASD, PDA, VSD b. The bigger the defect the less the cyanosis 5. Cyanotic/Mixed blood flow Signs and Symptoms 1. Cyanosis a. Depends on severity of defect b. If large septal defects < cyanosis 2. CHF and hypoxemia manifestations 3. Murmurs – vary with type of defect 4. Emergency if no septal defects!!! Treatment 1. IV PGE to keep ductus patent 2. Cath Lab a. Balloon Atrial Septostomy http://www.youtube.com/watch?v=0Ka9OJAGgJo 3. Surgery a. Arterial Switch Procedure |
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Patent Foramen Ovale
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Treatment needed...etc.
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In general, what nursing management/considerations do you have for anyone who has any cardiac shunt as a result of a congenital defect? What about as a result of surgical re-routing of blood flow?
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1. LISTEN FOR MURMUR!
2. No murmur is a REALLY BAD THING! 3. Must be on some anti-platelet meds 4. O2 Sats in 70s-80s is a good thing 5. Help parents adjust 6. Keep patients as calm as possible 7. Prevent respiratory infections |
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S/S of cyanotic heart defects?
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One symptom is cyanosis, which usually seen as a bluish discoloration of the lips, fingers, and toes.
--Dyspnea-- tripod positioning --Anxiety --Overbreathing (hyperventilation) Sudden increase in cyanosis --may get tired & sweat while feeding -Fainting (syncope) and chest pain may occur. Bluish or grayish skin Feeding problems or reduced appetite Puffy eyes or face Tiredness all the time |
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Why do cyanotic heart defects present the way they do?
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This abnormal blood flow (called right-to-left shunt) (because blood has difficulty exiting right side of heart
via the pulmonary artery B/C of obstruction of pulmonary blood flow or an ASD or VSD, pressure increases on right side and flows to LEFT!) can result in too little oxygen in the blood moving through the rest of the body, and therefore cyanosis. |
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What are long term consequences of cyanotic heart defects?
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Complications of cyanotic heart disease include:
Arrhythmias Brain abscess Heart failure Hemoptysis (coughing up blood) Impaired growth Infectious endocarditis Polycythemia Pulmonary hypertension Stroke Cyanosis is a sign of Eisenmenger syndrome, a condition that occurs in patients with congenital heart disease. Eisenmenger syndrome occurs as a complication of increased blood flow from the left side of the heart directly to the lungs. This results in severe lung diseases and increased pressure on the right side of the heart. |
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S/S of acyanotic heart disease?
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CHF presenting symptom!
difficulty in feeding (over 30 min) tachypnea sweating subcostal retraction S3 gallop and pulmonary rales |
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Why do these s/s occur with acyanotic heart disease?
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CHF presents because blood flows from the higher pressure left side of the heart to the lower pressure right side. Increased blood volume on the right side of the heart increases pulmonary blood flow and therefore show CHF.
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What is polycythemia and why/when does it occur? Why is this a problem?
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Polycythemia is an excess number of red blood cells.
Polycythemia vera occurs when a mutation in a bone marrow cell causes a problem with blood cell production. Normally, your body carefully regulates the number of each of the three types of blood cells you have. But in polycythemia vera, the mechanism your body uses to control the production of blood cells becomes impaired, and your bone marrow makes too many of some blood cells. It is an effort to increase oxygen carrying capacity of your blood. This is a problem b/c polycythemia vera causes your blood to thicken and slows blood flow, it increases your risk of developing blood clots. If a blood clot occurs in your head, it can cause a stroke. |
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Why is polycythemia a problem?
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---Blood clots. Polycythemia vera causes your blood to be thicker than normal, which can slow the rate of blood flow through your veins and arteries. Increased blood thickness and decreased blood flow, as well as abnormalities in your platelets, increase
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Why do we see CHF in children?
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It is the result of the underlying heart defect
1. Excessive workload on the heart 2. Volume overload 3. Pressure overload (resistance) 4. Decreased contractility 5. Excessive demand for cardiac ouput |
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How is CHF managed?
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1. Improve cardiac function
Digoxin, ACE inhibitors (Captopril) 2. Remove accumulated fluid and sodium 3. Diuretics, fluid and sodium restrictions 4. Decrease cardiac demands 5. Neutral thermal environment, treat infections 6. Reduce effort of breathing with semi-fowlers 7. Sedate if needed 8. Provide rest, decrease environmental stimuli 9. Improve tissue oxygenation |
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Nursing considerations for CHF?
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1. Administer digoxin correctly
a. Check dose with another nurse b. Hold for HR<100 in infant and <60 in a child 2. Monitor for toxicity of digoxin a. Anorexia, N&V, halos, drowsiness, H/A 3. Watch for hypotension 4. Monitor electrolytes, esp. potassium 5. Restrict sodium and fluids if needed 6. Allow rest for feeding, hold upright 7. Calorie dense formulas 8. May need NG tube feedings 9. Feed before allowing to cry 10. Teach family care needs |
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How is Lasix managed in children?
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1. Begin to record output as soon as drug is given.
2. Observe for dehydrration caused by profound diuresis. 3. OBserve for S/S (N/S, diarrhea, ototoxicity, hypokalemia, dermatitis, postural hypotension) 4. Encourage foods high in potassium and/or give potassium supplements. 5. Monitor chloridge and acid-base balance with long-term therapy. 6. Observe for signs of Digoxin toxicity. |
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What is a hypercyanotic spell? What is usually happening to the child when this occurs? What should the nurse do? What if it happens at home with the parents, what should the nurse teach them before discharge? What position/posture is associated with shunting?
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Hypercyanotic spells are also known as tet spells or blue spells and occur in infants or children w/ certain heart defects. The infant or child becomes acutely cyanotic and hyperpenic because of the sudden infundibular spasm. May occur from result of stressful procedures or from feeding, crying or defecation. If spell occurs the nurse should:
1. provide a calm and comforting approach while immediately placing the infant in a knee-chest position (assists breathing and increases oxygenation to body tissues) 2. Administer oxygen by facebook or blowby. 3. Administer morphine (reduces infundibular spasm) 4. Administer fluids (replace fluids keep well hydrated and keep hematocrit and blood viscosity within acceptable limits) 5. Document occurrence, actions taken, and infant's response. |
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What is bacterial endocarditis? What are some of the clinical manifestations? How can it be prevented?
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Infection of valves and lining of heart, occurs with bacteremia w/ children who have cardiac abnormalities, especially valvular.
Fever, low grade, intermittent Anorexia, malaise H/A, diaphoresis, weight loss Splinter hemorrhages under nails **BLACK LINES UNDER NAILS Red painful intradermal nodes on pads of the fingers (Osler nodes) Painless hemorrhagic areas on palms and soles (Janeway spots) Oral mucosa petechiae Splenomegaly Treatment 1. Prevention if susceptible is best 2. Prophylactic antibiotics pre procedure for high and moderate risk children 3. If infection present High dose antibiotics IV for 2-8 wks 4. CHF treatment Education 1. Counsel parent’s for prophylactic antibiotics 2. Instruct on oral health 3. Teach how to monitor for infections 4. Unexplained fever, weight loss, malaise 5. Assess for complications of CHF |
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What are long term effects of chronic hypoxemia?
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1. Polycythemia
a. An effort to increase oxygen carrying capacity b. Must be on iron supplementation to have enough available for additional RBCs c. Must maintain hydration to decrease blood viscosity 2. Clubbing 3. Hypercyanotic spells or "tet spells" a. Spasms that decrease pulmonary blood flow b. Often preceded by agitation, exertion, or bearing down c. Knee chest/squatting position to increase systemic vascular resistance and increase pulmonary blood flow d. Sedation, if appropriate 4. Poor weight gain/small stature 5. Developmental delay 6. Activity intolerance |
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Pallative treatment for hypoxemia?
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1. IV Prostaglandin
a. Works well in newborns <14 days b. Keep Ductus Arteriosis open 2. Modified Blalock-Taussig shunt a. Surgically make a “permanent†PDA b. Subclavian Artery to PA 3. Used for a variety of reasons a. Until child is old enough/big enough for surgical correction b. In lieu of surgical correction c. As surgical correction |
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What is rheumatic fever? Why does it happen? What are some long term care considerations?
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1. inflammatory autoimmune disease that affects the connective tissues of the heart, joints, subCut tissues, and blood vessles of the CNS.
2. Manifests 2-6 weeks after an untreated or partially treated w/ group A-beta, strep, affects the heart, CNS, subcut tissue 3. Prevent permanent cardiac damage, prevent recurrences, prophylactic IM/oral penicillin or sulfa drug monthly, all the time, prevent strep infection |
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What are the signs and symptoms of Kawasaki Disease?
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1. Acute stage
1. fever 2. conjuctival hyperemia 3. Red throat 4. Swollen hands, rash and enlargement of cervical lymph nodes. 5. Unresponsive to antibiotics and antipyretics. 6. strawberry tongue (slough first layer). Sub-acute stage 1. Cracking lips and fissures 2. Desquamation of the skin on the tips of the fingers and toes. 3. Joint pain 4. Cardiac manifestations 5. Thrombocytosis |
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What is the management of Kawasaki disease?
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1. High dose IV immune globulin (reduce duration of fever and the incidence of coronary artery lesions and aneurysms; this is a blood product, so blood precautions when administering it are warranted.)
2. aspirin **give for its anti-pyretic and anti-platelets effects. 3. Coumadin therapy if at high risk |
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What is the major complication of Kawasaki disease?
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**Heart attack. Read below.
Heart complications include: Inflammation of the heart muscle (myocarditis) Heart valve problems (mitral regurgitation) Abnormal heart rhythm (dysrhythmia) Inflammation of blood vessels (vasculitis), usually the coronary arteries, that supply blood to the heart Any of these complications can damage your child's heart. Inflammation of the coronary arteries can lead to weakening and bulging of the artery wall (aneurysm). Aneurysms increase the risk of blood clots forming and blocking the artery, which could lead to a heart attack or cause life-threatening internal bleeding. |
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What teaching must you provide as the nurse concerning the treatment for Kawasaki disease?
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1. Follow up care is essential to recovery.
2. S/S include: irrability that may last 2 months after onset of symptoms, peeling of hands and feet may occur. Pain in joints may occur over several weeks, and there is stiffness in the morning, after naps, and in cold temps. 3. Record temp (high fever expected) until fever subsides for several days. 4. Notify dr. if fever is 101F or higher. 5. Aspirin toxicity: tinnitus, headache, vertigo, and brusing; do not give to children with chicken pox or flu. 6. S/S of bleeding include: epitaxis, hemoptysis (coughing up blood), hematemsis, hematuria, melena (blood in stool), and bruises on body. 7. S/S of cardiac complications: chest pain or tightness, cool and pale extremities, abd pain, N/V, irritability, restlessness, and uncontrollable crying. 8. Avoid contact sports if taking aspirin or anticoagulants. 9. Avoid administer of MMR or varicella vaccine to child 11 months after IV immunoglob therapy, if possible. |
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How are children different in regards to hematology?
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1. Kids will hold maternal iron stores up until 9 month of age, therefore any anemic issues may not arise until this time. 2. Clotting disorders are particularly challenging given the child’s natural tendency to explore/play/be injured
3. Immunologic dysfunction is challenging in children due to the fact kids naturally get sick more often |
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What are common causes of iron deficiency anemia? How is it treated?
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Results from blood loss, increased metabolic demands, syndromes of GI malabsorption, and dietary inadequacy such as fortified formula, excessive milk intake (milk baby), poor diet, eat cereal, organ meats, spinach.
It is treated with iron supplements. |
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What are some important nursing/teaching considerations when administering iron supplementation?
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Ascorbic acid enhances absorption! – give with orange juice,
drink with straw, stains teeth, dairy products can interfere with absorption, cause tarry (black) green stools, cause nausea and constipation, and has a foul aftertaste, don't take on empty stomach |
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How is sickle cell disease acquired?
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Inheritance of a gene for a structurally abnormal portion of the hemoglobin chain.
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What is used for sickle cell screening?
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Sickledex
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What provides a fingerprint of the Hgb protein for sickle cell?
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Hemoglobin electrophoresis
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Sickle cell clinical manifestations?
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Often not present until 6 mos old
Chronic anemia Poor growth Enlarged spleen Enlarged liver Hematuria Delayed sexual maturity |
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What are common causes for sickle cell disease?
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1. Illness
--Avoid as much as possible --Oral penicillin prophylaxis 2. Dehydration --Keep well hydrated/increase fluid intake 3. Stress --Avoid as much as possible 4. Hypoxia 5. Avoid respiratory illness/treat chronic respiratory problems/avoide situations that increase need for oxygen 6. Strenuous physical activity --Avoid these activities |
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What are prevention strategies for sickle cell?
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Keep well hydrated,
avoid illness, oral penicillin prophylaxis all the time, avoid stress, avoid resp ill, avoid strenuous physical activity |
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How will you care for someone in crisis?
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1. Maintain adequate hydration and blood flow through oral and IV fluids; replace electros
2. Give O2 and blood transfusions/Exchange transfusions 3. Administer analgesics (around the clock). 4. Bed rest. 5. Keep extremities extended to promote venous return; Elevate HOB 30 deg. 6. Consume high cal, high protein diet with folic acid supplementation. 7. Give antibiotics to prevent infection. 8. Watch for complications (increasing anemia, decreased perfusion, and shock (mental status changes, pallor and vital sign changes.) 9. bone marrow transplant can cure SCD, but carries high mortality rate. |
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How is Beta-thalessemia major treated?
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Treatment is supportive; the goal of therapy is to maintain normal Hgb levels by the administration of blood transfusions q 3 weeks or so.
--May be overloaded by iron from the transfusions. If so, use chelation therapy or Desferal to treat iron overload. --CHF prevention |
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What lifestyle impact can this have on the patient/family
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Anxiety, chronic illness.
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What will be required as a result of this treatment?
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---
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Nursing care of child with transfusions
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Hemolytic Reactions
1. tranfuse slowly and/or inital 20% of blood volume 2. stop tranfusion when S/S occur, maintain intact IV line, notify Dr 3. Save donor blood to recross match wit pt's blood 4. insert catheter and monitor hourly outputs. 5. get sample and blood and urine and send to lab for Hgb (hemolysis?) Febrile Reactions 1. Give acteaminophen for prophaxlis 2. Stop transfusio immediately; call dr. allergic reactions 1. Give antihistmines for prophylaxis. 2. stop transfusion 3. give epinephrine. Circulatory overload 1. transfuse blood slowly 2. prevent overload by using packed RBs and divide amounts of blood to be given. 3. Stop transfusion. 4. PLACE CHILD UPRIGHT WITH FEET IN DEPENDENT POSITION to increase venous resistance. Air Emboli 1. normalize pressure before container is empty when infusing blood under pressure 2. clear tubing of air by aspirating air with syringe; disconnect tubing and allow blood to flow until air as escaped only if a Y connector is not available. Hypothermia 1. allow blood to warm to room temp. 2. take temp if pt complains of chills; if subnormal, stop the transfusion. Electrolyte disturbances (hyperkalemia) 1. use rashed RBCs or fresh blood if pt is at risk Transmission of infection (delayed reaction) 1. test blood for presence of HIv, Hep C & B antibodies. 2. Report S/S of infection, if it occurs, stop transfusion! send sample for C&S, notify dr. Alloimmunization 1. Use limited number of donors |
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General guidelines for transfusions?
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1. Take vitals, esp BP, before and after q 15 min for 1 hr and then after it is done transfusing.
2. Check identication of the recipient with the odnor's blood group and type. 3. Administer first 50 ml of blood or 20% of volume (whichever is smaller) slowly and stay with the child. 2. administer with NS 3. administer blood through a filter to elimniniate particles and prevent precipitation; gently shake freq. 4. use blood within 30 min of arrival 5. infuse a unit of blood within 4 hrs. If infusion will exceed this, divide the blood and refrigerate approp. 6. If reaction occurs, take vitals, stop it, maintain a patent IV line with NS and new tubing, notify the dr. |
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What does hemophilia cause?
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1. Abnormal bleeding in response to trauma or surgery (sometimes is detected after circumcision)
2. Epistaxis (nosebleeds) 3. Joint bleeding causing pain, tenderness, swelling, and limited range of motion 4. Tendency to bruise easily 5. Results of tests that measure platelet function are normaL Results of tests that measure clotting factor function may be abnormal. |
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What are nursing hemophilia concerns?
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1. Monitor for bleeding and maintain bleeding precautions.
2. Prepare to administer replacement factors as prescribed. 3. DDAVP (1 -deamino-8-D-arginine vasopressin), a synthetic form of vasopressin, increases plasma factor VIII and may be prescribed to treat mild hemophilia. 4. Monitor for joint pain; immobilize the affected extremity if joint pain occurs. 5. Assess neurological status (child is at risk for intracranial hemorrhage). 6. Monitor urine for hematuria. 7. Control joint bleeding by immobilization, elevation, and application of ice; apply pressure (15 minutes) for superficial bleeding. 8. Instruct the child and parents about the signs of internal bleeding. 9. Instruct parents in how to control the bleeding. 10. Instruct parents regarding activities for the child, emphasizing the avoidance of contact sports and the need for protective devices while learning to walk; assist in developing an appropriate exercise plan. 11. Instruct the child to wear protective devices such as helmets and knee and elbow pads when participating in sports such as bicycling and skating. |
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How is hemophilia treatred?
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Factor replacement
Injury prevention and prompt treatment Avoid all anti-clotting medications (No ibuprofen, Tylenol only)!! |
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How is demophilia diagonosed?
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Family/patient history
Labs Factor Assays Coags |
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What concerns do you have for the pediatric patient with this condition?
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Hemorrhaging, dying
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How is HIV/Aids diagonosed?
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Diagnosis
<18 months age-HIV PCR test >18 months age-ELISA and Western Blot |
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How is HIV/Aids treated?
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Antiretroviral drugs
IVIG for serious infections Immunizations Nutritional interventions, if necessary |
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What are considerations for HIV/aids for peds?
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Elisa western blot are more common.
IVIG– giving actual antibodies for an infection. If have active AIDS– do not give vaccines. If T cell count OK- can get the vaccines. |
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Pedatric cancers?
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1. Second leading cause of death in children
2. Diagnosis ---Labs --Radiologic Imaging --Biopsy --Spinal tap ---Bone marrow aspirate 3. Treatment ---Surgery ---Chemotherapy ---Bone Marrow Transplant ---Immunotherpay |
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What is the difference between AML and ALL?
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1. Most common pediatric cancer
2. Malignant disease ---Uncontrolled proliferation of leukocytes 3. Two types ---ALL (Acute Lymphoid Leukemia) Most frequent type Involves lymphatic system Stem cell/Blast cell Higher incidence in children with trisomy 21 Good prognosis -----AML (Acute Mylogenous Leukemia) Non-lymphoid Bone marrow origin Poorer prognosis***** |
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What is the difference between Hodgkin's and Non-Hodgkin's?
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1. Hodgkin—primarily lymph nodes
--Better prognosis --4 stage classification system --Enlarged, firm, nontender, movable lymph nodes (usually supraclavicular or cervical) 2. Non-Hodgkin—more diffuse in stem cells and lymphocytes rather than nodular Poorer prognosis Usually widespread disease at diagnosis Lymphoid tumors with frequent mets to meninges; can cause dysfunction my compressing nearby organs |
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What symptoms do you see as a result of leukemic cells competing for space?
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1. Enlarged lymph nodes
2. Hepatosplenomegaly 3. Bone pain 4.Headache (if CNS involvement) 5. Leukemic cells compete and crowd out other cells causing: --Anemia (low grade fever, pallor, fatigue) ---Decreased RBCs --Bleeding/Bruising/Petechiae --Decreased platelets --Infection --Neutropenia |
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What is the treatment for leukemia?
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Chemotherapy in 4 phases
1. Induction --High remission rate in ALL 2. Intensification --May require several months to eliminate all leukemic cells 3. Intrathecal Chemo (give through spinal tap). --To prevent leukemic cells from invading CNS 4. Maintenance --Preserve remission. May take up to two years. --Cured when free from disease for 4 years! ---Bone Marrow transplant may be needed for children with AML. |
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Nursing Care of the Pediatric Cancer Patient?
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1. Focus on the positives
2. Accommodate the family when possible 3. Practice/teach infection control 4. Promote normal childhood activities as much as possible 5. Refer to Make-A-Wish Nursing care is directed toward managing symptoms of leukemia as well as preventing/treating side effects of chemo. Supportive care includes: 1. protection of body from injury to prevent trauma to RBC's present 2. rest periods to combat fatigued RT anemia 3. Activity intolerance will require organization of nursing care to allow for adeuqate rest 4. Adequate nutrient intake is necessary for production of new RBC's. |
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What are some nursing diagonoses for the ped. Cancer patient?
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Chemo nursing DX
1. Risk for infection 2. Risk for injury 3. Altered nutrition: less than body requirements 4. Risk for altered oral mucous membranes 5. Disturbed body image RT hair loss. Cancer Nursing DX 1. risk for infection 2. risk for injury 3. activity intolance 4. anxiety 5. risk for ineffective family coping 6. Pain |
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What are your general goals for a ny child with neuromuscular dysfunction?
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1. Prevent injury/further injury
2. Return to baseline or maximum functioning as soon as possible 3. MAXIMIZE POTENTIAL 4. Minimize/manage associated problems 5. Control pain 6. Consider developmental concerns/issues regarding therapy, interventions, and nature of condition |
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What is torticollis?
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Torticollis, or wryneck, is a stiff neck associated with muscle spasm, classically causing lateral flexion contracture of the cervical spine musculature (a condition in which the head is tilted to one side). The muscles affected are principally those supplied by the spinal accessory nerve.
1. Congenital or acquired 2. Head tilted to one side: neck flexed 3. limited neck movement 4. Mass in neck on affected side 5. Fibrous tissue growth on affected side 6. extreme cases: surgery (12-18 mos) |
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How is torticollis treated?
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With stretching exercises.
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What could you teach parents about torticollis?
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B/c the problem is a contracted, stiff muscle, the treatment is gradually stretch the muscle. Do the following:
1. Approach baby from unaffected side (baby will turn head) 2. Do shoulder-to-shoulder stretching. At each diaper change, hold the baby's head and rotate the neck so the chin approaches the shoulder. 3. Teach tummy time! 15-30 min couple X a day. Improves neck strength and flexibility. 4. Carry baby in football hold. Turn her head outward, away from affected side. |
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What are the three types of spinal curvatures?
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1. Kyphosis- posterior accentuation of the thoracic curvature (hunchback)
2. Lordosis- anterior accentuation of the lumbar curvature 3. Scoliosis- lateral curvature of the spine and vertebral rotation |
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Why do these spinal curvatures occur?
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Kyposis: Happens due to poor posture (hiding boobs & big breasts), short height.
Lordosis: Due to pregnancy and obesity in children. |
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What are treatments for these spinal curvatures?
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Kyposis: Exercises to strengthen muscles, correct posture
Lordosis: Weight loss. |
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How do you screen for scoliosis?
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X-ray – radiologist – true grading measurement taken;
< 20 degrees, exercise, firm mattress, correct posture, >20, but < 40, brace therapy to stretch spine and relax pressure on vertebrae; > 40, spinal fusion to stabilize spine and stop progression of curve |
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Scoliosis INFO
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1. Scoliosis—Can be idiopathic* or secondary to disease/trauma
2. Most common in adolescent females 3. Symptoms ---Uneven shoulder and/or hip height ---Patients may notice ill fitting bra, uneven hemline |
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What is congenital hip dysplasia?
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1. One or both femoral heads displaced from the hip socket
2. Usually unilateral 3. Can be caused by fetal positioning, breach birth, inherited |
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How do you test an infant for congenital hip dysplasia?
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Diagnosis-X-ray, pelvic ultrasound
Treatment - Abduction 1. Splint 2. Harness: Pavlik harness 3. Spica cast (severe cases) |
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What signs and symptoms would lead you to suspect congenital hip dysplasia?
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1. Shortened leg
2. Asymmetry of gluteal skin folds 3. Limited abduction 4. Waddling gait 5. Abduction exam - (birth – 3 months) --(Positive)+Ortolani’s sign ------Feel it, hear a clunk, slip back in with pressure from behind --+Barlow’s sign -------hip pop when popping hip out, slip back in with pressure from the front |
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What is slipped capital femoral epiphysis? Who likely gets it?
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1. Spontaneous displacement of femoral head
2. Usually occurs in obese children, tall/lanky children, and at onset of puberty 3. Usually idiopathic, multifactorial ---puberty hormone changes, bone changes 4. Symptoms-hip pain, groin pain 5.Diagnosis-X-ray 6. Treatment ---Prevent further slippage and restore use ---Complete non weight bearing ---Surgery |
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What is clubfoot?
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1. “talipes equinovarus”-- plantar flexion in which the toes are lower than the heel.
2. Complex deformity of ankle and foot 3. Can be unilateral or bilateral 4. Varies in severity |
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How would you teach parents about treatment/correction?
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1. Positional (pseudo talipes)-caused by intrauterine crowding
----Stretching exercises, shoes, standing 2. Idiopathic (true clubfoot) ---Serial casting ---Surgery, if necessary 3. Treatment begins soon after birth as possible. 4. Manipulation and casting are perfromed weekly for about 8-12 wks; a splint is applied if casting and manipulation are successful. 5. Surgical intervention may be necessary if normal alignment is not achieved by 6-12 wks. 6. Monitor for pain, and monitor the neurovascular status of the toes. |
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Why do kids get the kinds of fractures they do?
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1. Because bones can bend 45 degrees before breaking
2. Fractures occur in children RT increased mobility and inadequate or immature motor and cognitive skills; result from trauma (jumping, falling, rollerblading, etc) or bone diseases such as congenital bone disease or bone tumors. 3. Fractures in infants are rare and warrant investigation to rule out abuse!!!! |
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Fracture Nursing care & Assessment guidelines
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1. Assess extent of injury and immbolize the affected extremity.
a. Assess Five “P’s”—Pain & tenderness, pulses (distal to fracture site), pallor (color), paresthesia (sensation), and paralysis (mvt). 2. If a compound fracture exists, cover the wound with a sterile dressing or a clean dressing. 3. Elevate the injured extremity. 4. Apply cold to the injured area. 5. Monitor neuro staus |
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What are common types of traction used in children?
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1. Russell skin traction (see pg. 986 Saunders book for pics).
a. Used to stabilize a fractured femur before surgery b. Similar to Buck’s traction, but provides double pull using a knee sling that pulls the knee and foot 2. Balanced suspension a. Used with skin or skeletal traction to approximate fractures of the femur, tibia, or fibula b. Balanced suspension is produced by a counterforce other than the child. c. Provide pin care if pins are used with the skeletal traction. d. Position client ina low Fowler's posiiton on either side or back and maintain a 20 deg. angle from thigh to the bed. 3. 90-degree—90-degree traction a. The lower leg is supported by a boot cast or calf sling. b. A skeletal Steinmann pin or Kirschner wire placed in the distal fragment of the femur, allowing 90-degree flexion at the hip and the knee. |
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What are nursing considerations for the various types?
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a. Maintain correct amount of weight
prescribed. b. Ensure that weights hang freely. c. Check all ropes for fraying and all knots tighmess; be sure that the ropes are appropriately tracking in the grooves of the pulley wheels. d. Monitor neurovascular status of the involved extremity. e. Protect the skin from breakdown. f. Monitor for signs and symptoms of complica- tions of immobilization, such as constipation. skin breakdown, lung congestion, renal complications, and disuse syndrome of unaffected extremities. g. Provide therapeutic and diversional play. h. monitor Color, motion and sensation of the affected extremity. i. monitor insertion sites for redness, swelling, drainage, or increased pain. j. provide insertion site care as prescribed. |
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What is JIA?
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Juvenile Idiopathic Arthritis
1. Chronic inflammation of the joints and fibrosis of cartilage 2. Types (categorized by onset) ---Systemic onset ---Pauciarticular (<5 joints) ---Polyarticular (simultaneous involvement >4 joints) 3. Symptoms ---Stiffness, swelling, loss of motion ---Morning stiffness |
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As a nurse, what important management would you focus on for a child with JIA and their family?
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1. Diagnosis-of exclusion, no definitive tests
---May have increased sed rate 2, Treatment ---Medication ---NSAIDs, Anti-rheumatic drugs, corticosteroids 3. Physical therapy ---heat, exercise, pool therapy, splinting/positioning when sleeping 4. Health promotion infection, injury can exacerbate symptoms |
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What are osteosarcomas?
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Malignant tumor of the long bones, esp in the lower extremities, with msot tumors occuring in the femur. (pg. 532-33 Saunders)
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How are they treated?
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1. Treated with radiation & chemo,
2. Treatment may include surgical resection (limb salvage procedure) to save a limb or remove affected tissue 3. amputation |
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What is the potential psychosocial impact of this?
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may be problems with self-image afterwards.
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What is rhabdomyosarcoma?
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Malignant tumors of the soft tissue, usually muscles, as a lump found incidentally, symptoms depend on site of origin, CAN RECUR, if so poor survival. Mets are frequent in lungs, chest, abdomen
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What is CP?
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1. Non-progressive motor function disorder characterized by impaired movement and posture; usually associated with abnormal or premature birth and intrapartal asphyxia/anoxia
2. 1.5 to 3 per 1000 live births 3. Perceptual, language, intellectual problems 4. Most common permanent physical disability of childhood 4. Prevalence has increased due to the improved survival rates of extremely-low-birth weight and very low birth weight infants |
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What are some CP causes?
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1. Cerebral anoxia (pre-natal, post-natal) from trauma
2. Neonatal distress 3. Prematurity – causes insult to immature brain 4. Spastic CP is the most common! |
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How does it usually present?
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1. Abnormal muscle tone and coordination
2. Clinical manifestations may not present until after 6 months 3. Delayed gross motor development ---Poor head control ---Delayed sitting 4. Abnormal motor performances --Persistent tongue thrust --Clenched fists --Using only hands/one side of body to crawl 5. Alterations in muscle tone --Abnormal posture -----Rigid extremities -----Floppy -----Arching --Reflex abnormalities -----Hyperreactive -----Persistent |
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What are important schooling/developmental considerations for someone with CP?
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1. Physical therapy, occupational therapy, speech therapy, education, and recreation.
2. Assess the child's developmental level and intelligence. 3. Encourage early intervention and participation in school programs. 4. Encourage communication and interaction with child based on his devleopmental level. |
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What is the goal of CP management?
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The goal of management is early recongition and interventions to maximize the child's abilities.
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Who and what is involved in the management of CP?
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1. A multidisciplinary team approach is implemented to meet the many needs of the child.
2. Therapeutic management includes physical therapy, occupational therapy, speech therapy, education, and recreation. 3. Prepare for using mobilizing devices to help prevent or reduce deformities. 4. Provide a safe environment by removing sharp objects, using a protective helmet if the child falls frequently, and implementing seizure precautions if necessary. 5. Provide safe, appropriate toys for the child’s age and developmental level. 6. Position the child upright after meals. 7. Medications may be prescribed to relieve muscle spasms, which cause intense pain; antiseizure medications may also be prescribed. 8. Provide the parents with information about the disorder and treatment plan; encourage support groups for parents. |
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What is the prognosis?
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Cerebral palsy doesn’t always cause profound disabilities. While one child with severe cerebral palsy might be unable to walk and need extensive, lifelong care, another with mild cerebral palsy might be only slightly awkward and require no special assistance. Supportive treatments, medications, and surgery can help many individuals improve their motor skills and ability to communicate with the world.
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What are neural tube defects?
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1. Failure of midline of neural plate to close as it forms the neural tube during 3rd and 4th week of gestation
2. Defects vary in size 3. Rate is down 0.32 per 1000 births 4. Splitting of closed neural tube r/t an increase in CSF pressure in first trimester |
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What are causes of neural tube defects?
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1. Multi-factorial
2. Increased incidence within family 3. Drugs- Valporic Acid (seizures) 4. Maternal heat exposure- hot tub, saunas |
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What is the best way to prevent neural tube defects?
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increase folic acid intake (BEFORE Conception- 1mo before 1st trimester)
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What types of spina bifida are there?
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1. Spina Bifida Occulta (no herniation of mengines; no loss of function).
2. Spina Bifida Cystica (cystic lesion in midline of vertebral column. More serious defect). A. Meningocele -------Defect in meninges + CSF protrude thru unfused vertebral arches, soft saclike appearance B. Mylelomeningocele -------Meninges & CSF & Spinal Cord. -------Cyst like protrusion, contains portion of spinal cord, meinges, + CSF C. Encephalocele -------Meningocele or myelomingocele occipital area of skull |
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Consequences of spina bifida?
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1. Results in ROM, sensory, and reflex deficits
2. Abnormalities in brain development 3. Chiari Type II malformations 4. Hydrocephalus usually develops 5. 2/3 have normal intelligence 6. Potential for seizures 7. Involvement depends on location |
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How is spina bifida diagnosised ?
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1. Intrauterine – sonogram, amniocentesis, AFP in Maternal blood
2. Birth – visible 3. Neuro exam 4. CT scan, ultrasound, MRI 5. Management: Multidisciplinary A. Ultrasound may show abnormality B. Alpha feta protein done 16-18 weeks gestation; chorionic vallus sampling (CVS) C. Keep in mind that an elective c-section may result in less motor dysfunction D. Neuro exam to find level affected E. Management: neuro, urology, ortho, rehab, PT, OT, nursing |
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How is it treated?
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Treated with surgery.
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How soon is it treated?
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Within 24-72 hrs after birth. Some within 12-18 hrs to prevent stretching of other nerve roots.
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Surgery pre-op care?
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Focus pre-op care on maintaining skin integrity of the sac and keep it free of infection' position infant on side or abd. to attain this!! Keep sac moist with sterile, soaked saline dressings' avoid contamination of sac area by urine or feces
1. Assess neuro: Neuro s/s of meingits: increased temp, irritability, pallor, vomiting, nuchal rigidity 2. Check head circumference q shift, mark area 3. Isolette warmer: keep warm 4. Sterile moist drsg NaCl change every 2-4hr; PREVENT INFECTION 5. Stict aspetic technique! Sac care! --- 5. Minimize tension with position/ trauma to sac; place rolls; pt in prone/side position 6. Bowel/Bladder considerations-- Neuropathic bladder dysfunction, crede bladder every 2-3 hr; clean intermittent cath, ditropan-improve bladder storage 6. 18-60% may have latex allergy due to exposure to latex in surgery, freq caths: urticaria, wheezing, anaphylaxis 7. PREVENT: identify and use of anti histamines and steroids prior to surgeries |
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Surgery post-op care?
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1. Continue with care similar to pre-op till healed
2. Check neuro status, head circ 3. Check sphincter control 4. Check movement of extremities 5. Further care will depend on level of defect 6. Family support 7. Home care Considerations--------- 1. Hydocephalus 90-95% High incidence of hydrocephalus : check fantanels 2. Check anal reflex 3. Orthopedic: prevent joint contractures, correct deformities, prevent skin breakdown, obtain best motor function 4. Home: spina bifida assoc; drsg dry and intact; prone position 5. Complications: meningitis – irritability, lethary, nuchal rigidity (increased ICP); infection, UTI, hydronephrosis, 6. Urinary: variety of methods available: self cath, drugs, surgery 7. Stool: continence achieved with training, diet; prevent constipation and impaction |
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Patient teaching for spinia bifida?
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1. Teach family about possiblity of developing hydrocephalus and s/s of increased ICP
2. Since most children with tube defects (except those with spinia bifida occulta) have some neurogenic bladder, teach about clean intermittent straight cath is important; work with fam to dveelop bowel management program also. |
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What are your nursing considerations for a patient with unrepaired spina bifida cystica?
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Keep the sac area clean and free from S/S of infection.
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What is Guillain Barre syndrome?
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1. Post-infectious polyneuritis
---Post-viral illness 3. Acute neuromuscular disease (acute demyelinating disease of the nervous system!!) ---Progressive weakness + paralysis 4. Peripheral nerve inflammation with progressive myelin breakdown 5. Inflammation and edema occur in spinal and cranial nerves; Decreased speed + intensity of peripheral nerve conduction 1. Follows viral illness – measles, mumps, mono, viral vaccine immunization, H.pylori, myoplasma, pneumocystis infections usually by about 3 weeks post illness 2. Cause – any age, but 4-9 years old common if in children 3. Decreased speed – self limiting, slow return of neuro function; death if resp and cardiac complications |
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how soon does paralysis occur? What is the prognosis?
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1-2 weeks. Majority of clients recover in 2-15 months with no residual disabilities.
1. Better with younger children 2. Better with little respiratory involvement 3. Sometimes improvement in a few weeks 4. Children do recover usually within one year (may take up to two) 5. Reassure family |
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What causes it?
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toxic autoimmune reaction of viral agent
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How does it present?
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1. Sore throat ~ then several days to weeks later (or with in 24 hours)
2. Sudden pain / weakness feet and legs, cramps 3. Progressive upward to trunk and extremities 4. Respiratory insufficiency (most serious complication) 5. Cranial nerve paralysis: facial weakness, impaired swallowing & gag reflex (7th cranial) 6. Autoimmune function impaired: hypotension, cardiac arrhythmias, can lead to shock 7. Urinary retention |
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What nursing considerations/concerns do you have for someone with GB?
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Diagnosis
1. EMG-- abnormal pattern of nerve conduction. 2. Paralysis symptoms 3. CSF analysis-- LP reveals incrased portein and few WBCs Treatment 1. Maintain vital function 2. Ventilator 3. Watch for cardiac complications 4. IVIG, Plasmaphoresis, Steroids 5. Pain control 6. Orthopedic care: gentle ROM, splints |
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Continued from above.
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a. Medical treatment includes administration of IVIG and immunosuppressive drugs and plasmapheresis
b. Care is supportive and similar to that provided to a quadriplegic client; during initial phase, assess closely for advancement of paralysis to include respiratory area; observe closely for difficulty with breathing or swallowing; place on a cardiac monitor and have all emergency equipment accessible to bedside; children with respiratory distress usually require mechanical ventilation c. Prevent complications using good postural alignment, frequent change of position, and passive ROM exercises; recovery period can last from two weeks to several months depending on degree of paralysis d. After the recovery period an extensive rehabilitation process begins; while complete recovery is possible, there is possibly some permanent disability e. Providing emotional support for child and family is an important nursing function; loss of motor functions and control can be very frightening for a child; parents need to be encouraged to bring favorite toys from home and provide other comforting activities to help child feel secure |
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What would you teach a family With GB?
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a. Family needs to understand mechanism of disease and treatment strategies being used
b. Parents need to learn how to provide basic care for child, including need for good skin care, and turning and positioning |
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What is SMA?
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Spinal Muscle Atrophy
1. Progressive loss of skeletal muscle function 2. Varies in severity depending on type 3. Intact intellectually 4. Lifelong illness 5. Goal of therapy-maximize function 6. Family coping |
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What types are there?
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1. Type 1 (Infantile)
Most severe form Diagnosed within first six months Weakness (shoulder/arm first) No normal gross motor skills Life expectancy-Preschool (usually) Trach/Vent/G-tube 2. Type 2 (Intermediate) Diagnosed before 18 months Weakness predominantly in lower extremities May have achieved some milestones Life expectancy-School age to early adulthood Type 3 (Juvenile) Diagnosed after 18 months May achieve normal milestones, then gradual loss of function Normal life expectancy |
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How are they managed?
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Treatment is symptomatic and preventive, primarily preventing joint contactures and treating orthopedic problems, primarily scolosis. Also, to retain mobility as long as possible, preventing skin breakdown, optimize resp. function, and providing support to pt. and family.
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What concerns do you have as the nursing caring for someone with SMA?
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1. Underlying goal of treatment should be to assist the child and family in dealing with the illness & progressing toward a normal life.
2. Prevent muscle and joint contractures 3. Promote independence in ADLs 4. Become corporated into the mainstream of school. 5. Assess oxygenation during sleep (breathing problems). Later URI's may occur that are treated with antibiotics. 6. Verbal, tactile, and audiotory stimulation are important. 7. Provide proper alignment when sitting upright. 8. May need gastro feedings (poor nutrition) |
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What considerations do you have for the child’s family?
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Parents need support and resources to be able to provide for the child and
remain an intact family. Parents need support & encouragement. Genetic testing is encouraged. |
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What happens in Duchenne’s Muscular Dystrophy?
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1. Pseudohypertropic muscular dystrophy….Progressive muscular weakness, wasting, and contractures, calf muscle hypertrophy , loss of independent ambulation by 9-12 yo
2. Muscle fibers replaced with fibrous tissue & fat deposits 3. X-linked inherited condition 4. Usually affects males (1:3600) 5. Symptoms appear 3-5 years old |
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What is the hereditary charact of sickle cell anemia?
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autosomal recessive
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Who is likely to have it?
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Usually affects males (1:3600)
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What is the name of the pathol Hb in sickle cell anemia?
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Hb S
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What is the prognosis?
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Death before age 30 – due to resp and cardiac progression
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When does Hb S morph in sickle cell?
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when it's deoxygenated
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What's the name of most freq and painful sickle cell crisis?
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vaso-occlusive sickle cell crisis
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What 2 things can happen during a vaso-occlusive sickle cell crisis?
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tissue necrosis,
pain |
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What blood condition does pt w/ sickle cell often have?
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chronic hemolytic anemia
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Why is sickle cell called a hemolytic anemia?
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sickled RBCs have shorter life span
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What are sym of sickle cell just after birth for neonate?
Why? |
asymptomic b/c
^ levels of mom's Hb F prevent excessive sickling |
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At what age do problems often start for kids w/ sickle cell?
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4-6 mos old
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What is the life cycle of spleen r/t sickle cell?
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1st - enlarged w/ sickled cells & has v funct
*spleen may rupture 2nd - small and fibrotic r/t multi infarc & can't filter bacteria |
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Why are sickle cell pts more susceptible to infection?
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fibrotic spleen can't filter bacteria
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To what infection is pt w/ sickle cell prone?
Why? |
meningitis r/t dysfun'l spleen
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What should you suspect if infant has swollen hands and feet?
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sickle cell
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What is hand-foot syndrome?
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swollen hands and feet
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What is often the first sym in infant w/ sickle cell?
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hand-foot syndrome
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If infant has priapism, what could you suspect?
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sickle cell
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What often precipitates vaso-occlusive crisis?
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infection
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What can infection precipitate r/t sickle cell anemia?
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vaso-occlusive crisis
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What two effects can vaso-occlusive crisis cause (besides pain & necrosis)?
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slowed circulation,
renal failure |
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What is a sequestration crisis?
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r/t sickle cell,
large amt of blood pooled in liver and spleen |
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What is it called when viral or other infection causes v prod of RBC in sickle cell pt causing profound anemia?
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aplastic anemia
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What is it called when lrg amt of blood pools in liver and spleen r/t sickle cell?
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sequestration crisis
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What are the charac of the anemia caused by sickle cell?
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normocytic, normochromic
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What are the 2 tests you give in succession to dx sickle cell?
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sickledex then
Hgb electrophoresis |
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What is the procedure for sickledex test?
How long do results take? |
finger stick,
3 min |
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What tests tells if kid has sickle cell disease or trait?
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Hbg electrophoresis
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What are 3 primary NI to teach parents for between sickle cell crises?
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1. promote tissue oxygenation
2. promote hydration 3. prevent infection |
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How do you prevent sickling?
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promote tissue oxygenation
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What are 3 things that ^ need for O2 that you should try to prevent r/t sickle cell?
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strenuous activity,
cold, stress |
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What's the next most imp thing to provide for kid w/ sickle cell besides tissue oxygenation?
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hydration,
give adequate fluid intake |
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What are 4 primary NI during sickle cell crisis?
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1. minimize O2 use (bedrest)
2. promote hydration 3. relieve pain 4. maybe Trental |
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What does Trental do?
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makes RBCs slippery in sickle cell
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When can kid in sickle crisis do more than bedrest for activity?
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when pain gone
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Describe O2 admin during sickle crisis?
What do you assess? |
no prolonged O2,
observe response r/t pain |
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What is the most helpful during sickle crisis?
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give oral & IV fluids,
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How much fluid do you give during sickle crisis?
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2x normal
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What is the best way to evaluate hydration during sickle crisis?
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check mucus membrane
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What is another way to evaluate hydration r/t sickle crisis besides wt, I&O, mucus membranes?
What are 2 methods you can't use? |
serum electrolytes,
can't use amt or spec grav |
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What is pain med tx during sickle crisis? (when, what route, and what?)
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around the clock
IV morphine |
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What's a non-medical Rx for pain during sickle crisis?
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heat to painful areas
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What do you want to know about condition of hip dislocation r/t dysplasia?
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if dislocates
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What might you feel if infant hip dislocates?
What's it called? How long is test affective? |
clunky feeling or clicking.
Ortolani's maneuver, most effective up to 3 mos old |
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If kid has hip dysplasia, what will unaffected hip do if he stands on affected leg?
What's it called? |
tilt downward instead of upward,
Trendelenberg's sign |
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How intense is the rx for congenital hip dysplasia if caught in first 6 mos?
When and what results are expected? |
conservative Rx results in
normal hip by the time walking |
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What Rx for congenital hip dysplasia for birth - 6 mos? x3
How long |
legs are gently flexed,
externally rotated, held in abduction. ~ 6 wks |
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What are 3 options for devices for congenital hip dysplasia for birth - 6 mos?
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splint,
brace, Pavlik harness |
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What's a no-no old school trick for congenital hip dysplasia for birth - 6 mos that causes hip extension?
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double or triple diapers
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What are the 3 steps to Rx for newly dx unrelocatable hip in 6-18 mos old?
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1. skin traction
2. hip reduced 3. spica cast |
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How often do you change spica cast for 6 - 18 mos old?
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q 6 wks
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What's priority care if skeletal traction used r/t hip dysplasia?
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pin care aimed at preventing osteomyelitis
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What do you use and what's a no-no when trying to prevent irritation under spica cast?
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yes: clothing
no: lotion or powder |
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What's imp re: position when hip device removed to teach parents r/t cong hip dysp?
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keep hips abducted
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What can you use to relieve itching under cast?
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blow-dryer on cool
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What's another name for talipes equinovarus?
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congenital clubfoot
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clubfoot - another name?
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talipes equinovarus
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What's the imp assessment re: dx of clubfoot?
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differentiate from IU position deformity
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How is IU position deformity different from clubfoot?
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IU position def = only muscular and can be passively manipulated past midline
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What do you start Rx for clubfoot?
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ASAP after birth
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How often casts replaced for club foot?
For how long? |
q 1-2 wks
for ~6wks |
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What's goal of casting clubfoot?
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over correction
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What might be used instead of or after overcorrection of cast r/t clubfoot?
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Denis Browne splint
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What should you do r/t angle of shoes on Denis Browne splint bar? x2
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should not be repositioned,
only tightened against bar if loose |
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What are the body's px responses to CHD?
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Babbling heart (murmurs)
Additional RBCs (polycythemia vera) Decreased exercise tolerance & dyspnea Hypoxia of tissues Elevated P & RR Azure (cyanotic) Recurrent respiratory infections Taken out heart (CHF) Slow growth |
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What kind of ABG condition occurs r/t CHD?
Why? |
metabolic acidosis r/t tissue hypoxia
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If you see clubbing of fingers, what might it point to?
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CHD
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What pathology should you suspect if in knee chest position, squatting?
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CHD
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What position is common to get into r/t CHD?
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knee-chest position, squatting
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What's most common drug for CHF?
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Digoxin
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What's the most common type of cardiac cath for children?
What's the path? |
R-heart cath,
thru femoral vein into R atrium |
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What should you expect from inserting cardiac cath in leg?
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pressure or pushing feeling
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What do you feel when dye injected into cardiac cath?
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warmth
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How long does cardiac cath take once dye injected?
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2 to several hours
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How often do you check color and VS after cardiac cath?
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q 15 min
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What is a critical ass post cardiac cath?
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check incision & dsg for bleeding or hematoma
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What do you do if bleeding at cardiac cath site?
x2 |
direct pressure for several min and
report stat |
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What are two things to try to implement for kid post cardiac cath for 6-8 hours?
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keep quiet in bed,
keep arm or leg straight |
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How should dsg appear post cardiac cath?
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clean and dry
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What are 3 things to tell parents post cardiac cath?
How long? |
no tub baths,
no strenuous activity, no school for at least 3 days |
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What are 3 actions to teach kids w/ CHD during episodes of cyanosis and dyspnea ?
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place on abdomen;
place on side, knee-chest pos (squatting); loosen tight clothing |
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Prevent what from kid if CHD?
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crying
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Prevent what and Rx immediately r/t CHD?
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resp infection
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What do you give CHD pt prior to even minor surgery?
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prophylactic antibiotics
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How do parents feel when caring for kid w/ CHD?
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question parenting ability b/c kid never satisfied
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What does kid w/ CHD have difficulty dealing with?
Why? |
difficult situations b/c
few opportunities to do so. |
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What is the most prevalent nutritional disorder in the USA?
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iron deficiency anemia
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What age is iron deficiency anemia esp prevalent?
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6-25 month olds,
adolescents |
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What are charac of RBCs w/ iron deficiency anemia?
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microcytic,
hypochromic |
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What do the following sym indicate?:
waxy pallor, irritable, listless, anorexic, constipation |
iron deficiency anemia
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What are 2 poss issues w/ heart r/t iron deficiency anemia?
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megaly,
systolic murmur |
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What are kids w/ iron deficiency anemia prone to?
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infection
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Why might kid w/ iron deficiency anemia be delayed in G&D?
x2 |
poor muscle dev,
reluctant to explore |
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What are 3 poss conditions r/t eating if kid has iron deficiency anemia?
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milk baby,
anorexic, pica |
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What are sym of iron deficiency anemia?
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Pallor waxy
Listless Anorexic Systolic murmur Tired Irritable Constipation, maybe "PLASTIC" |
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What's diet hx usu have r/t IDA?
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lrg milk intake
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What's a "milk baby" look like? x3
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obese,
pale, poor muscle dev |
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What will blood levels be r/t IDA?:
Hgb? Bct? |
Hgb: <11
Hct: <33 |
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What 6 lab values will be down in IDA?
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Hgb, Hct, MCV, MCH, MCHC, serum iron
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How often and how long do you screen kids for IDA?
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yearly until end of adolescents
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What do you report immediately r/t IDA? x2
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tachycardia,
tachypnea |
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What do you do if tachycardia, tachypnea r/t IDA occurs?
(not call MD) |
O2 prn
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What can cause CNS signs of tissue hypoxia (irritability, fussiness, short attention span, low frustration tolerance, h/a, fatigue)?
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IDA
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Who can be roomate for kid w/ IDA?
Why? |
unaffected kid, b/c of lowered resistance
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Give iron w/ what?
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vit C
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What poop charac show iron supplements working?
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green tarry
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What do you do if kid vomits from iron supplements?
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call MD
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What will MD do if vomiting from iron sulfate?
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^ dose and
give w/ meals |
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When and how can you tell if iron supplements correcting anemia? (specifically what lab results?)
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7 days after iron started,
^ reticulocyte count indicates RBC proliferation |
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boys or girls more prone to scoliosis?
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girls
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When is scoliosis usu dx?
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13 - 15 y/o
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Suspect what if you see forward thrusting of head, asymmetrical rib cage, nipples shoulders hips not horizontal?
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scoliosis
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What position is best for detecting scoliosis?
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bent forward at waist,
head and arms hanging |
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What spine curvature can be held in place by brace?
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20-45°
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How long every day do you wear brace for scoliosis?
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23 hours
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Until when do you wear scoliosis brace?
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6 mos after px maturity
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What's worn under scoliosis brace?
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shirt or stockinette
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How do you prevent atrophy & strengthen muscles of the torso r/t scoliosis?
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exercises
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What's a Harrington rod?
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metal rod installed near spine for scoliosis
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How do you fuse Harrington rod to spine?
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use bone from iliac crest
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When is only time you should remove brace r/t scoliosis?
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bath and exercise
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What's most therapeutic sport r/t scoliosis?
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swimming
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What's priority ass in ICU r/t scoliosis?
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resp function
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How do you check blood loss after scoliosis surgery?
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UO
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How do you turn kid w/ scoliosis post op?
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log roll
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What does the leukemic process in bone marrow interfere with?
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prod of RBCs, WBCs, & platelets
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What is there a proliferation of r/t leukemia?
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immature WBCs
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What is the most common form of childhood leukemia?
What percent? |
ALL,
80% |
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Leukemia causes what 3 blood dyscrasias?
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anemia,
thrombocytopenia, neutropenia |
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What are 5 sym of anemia r/t ALL?
(strength, color, LOC, breathing, heart) |
weakness, pallor, fatigue, dyspnea, cardiac sym
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What do the follow symptoms indicate?:
weakness, pallor, fatigue, dyspnea, cardiac sym |
anemia (r/t ALL)
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What are normal RBC levels?
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4.31
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What are normal platelet levels?
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150k-400k
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What level are platelets if you see epistaxis and oral bleeding?
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20k
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Fever in ALL due to what (not just infection)?
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hypermetabolic state
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What are normal WBC levels?
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4,500-13,500
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How is dx confirmed for leukemia?
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bone marrow analysis
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How long do you apply pressure after bone marrow aspiration?
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5 min
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What disease might you detect from lumbar puncture?
|
leukemia
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What must pt attain before medical rx for leukemia?
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normal F&E balance prior to starting Rx
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What can cause renal failure during rx for leukemia?
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^ uric acid levels
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What 3 ways can you prevent elevation of uric acid levels r/t leukemia rx?
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1. IV hydration
2. alkalinize the urine 3. give allopurinol |
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What allopurinol for?
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inhibiting uric acid production
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What med inhibits uric acid production?
Encourage what when taking it? |
allopurinol,
encourage fluids |
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What's the leading cause of death r/t leukemia?
|
infection
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When do you start antibiotics if pt has leukemia?
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as soon as fever starts
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What often happens with prolonged use of antibiotics?
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candida, fungal infection
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What disease is pt w/ leukemia 10 times more likely to contract?
|
herpes zoster
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What is pain r/t in leukemia?
|
disease and invasive procedures
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What are drug combos of Vincristine, L-asparaginase, and Prednisone used for?
|
remission induction in ALL
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What combo of drugs are used to induce remission in ALL?
|
Vincristine,
L-asparaginase, Prednisone |
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What are 4 non-life-threatening s/e of Vincristine?
|
abd. pain,
n/v, alopecia, constipation |
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What's a toxic effect of Vincristine?
|
peripheral neuropathy
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What could be the source of the following s/e if pt has ALL is doing drug tx?:
abd. pain, n/v, alopecia, constipation |
Vincristine
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Why give methotrexate to pt w/ ALL (if not for maintenance tx)?
h |
prophylaxis for CNS disease
|
meth heads ALL need prophylaxis or they can get syphilis (CNS disease)
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What do you give pt w/ ALL to prevent CNS disease?
|
methotrexate
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What drug is often used for maintenance tx r/t ALL?
|
methotrexate
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What meds are contraindicate w/ methotrexate?
x3 |
ASA,
sulfonamides, folic acid |
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Why no salicylates & sulfonamides w/ methotrexate?
|
v action of drug
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Why no folic acid w/ methotrexate?
|
interferes w/ cyctoxic action of med b/c it's a folic acid antagonist
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What happens re: signs of fever in kid w/ ALL?
|
may not show b/c unable to generate big enough WBC response
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What are 3 ways to reduce fever if kid w/ ALL?
|
tepid sponge,
cooling blankets, Tylenol |
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To prevent infection pt w/ ALL, how do you take temp?
|
no rectal
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What 2 parts of the body do you assess in pt w/ ALL r/t infection?
|
mucus membranes & skin
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What do you do if nosebleed in pt w/ ALL? (3 steps)
|
1. high fowler's, lean forward,
2. apply pressure for 10 min, 3. if still bleeding, cold cloth into nostrils and on bridge of nose |
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What do you do if bleeding in mouth occurs in pt w/ ALL?
|
put a dry tea bag on bleed (tannic acid v bleeding)
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Pt has ALL, what do you do if reaction to transfusion? x2 steps
|
1. stop transfusion stat
2. KVO w/ NS |
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Is analgesic Tx given to Rx pain r/t ALL?
When do you admin it? |
No, it's given to prevent it.
Give on regular sched around the clock |
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What are 3 s/e of chemo?
|
1. stomatitis
2. hemorrhagic cystitis 3. alopecia |
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What are two meds if stomatitis r/t ALL chemo?
|
1. topical (ex) viscous lidocaine
2. systemic analgesics |
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What's diet if presents stomatitis?
|
bland, soft, moist diet
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What are 3 ways to prevent hemorrhagic cystitis in pt on chemo for ALL?
|
1. liberal fluid intake
2. freq voiding 3. admin chemo in AM (allows fluid intake and voiding) |
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When can you give live virus vaccine after finishing chemo?
|
3 mos. after
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Bone marrow transplant, what are first two steps before treatment?
h |
1. protective isolation
2. immunosupression |
all about protecting, protect on the outside from other people germs,
protect on the inside from own body guards you want him alone |
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What makes it extra difficult for parents when one kid gets bone marrow transplant?
|
often end up with two kids (sick kid, and donor kid) in hospital
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1. How many days are req for donor marrow to engraft and begin to proliferate mature cells?
2. What condition is kid in during this period? 3. What should family do? |
1. 10-20 days,
2. child has no ability to fight infection, 3. strictly follow protective isolation |
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