Peds Quiz 2
Anemia: What is it:
It is a condition that develops when your blood lacks healthy red blood cells or hemoglobin. Hemoglobin : the main part of red blood cells that binds oxygen. Having inadequate amount of hemoglobin will cause the cells in your body to suffocate (oxygen deprived) . “WebMD”
The most common form of Anemia:
is Iron Deficiency Anemia
what is it:
Decrease in Oxygen-carrying capacity of blood, and a reduction in the amount of oxygen available to the cells.
CBC: RBC, Hgb, Hct. Will be below normal.
Iron studies: total iron-binding capacity (TIBC). Will be increased above normal (compensatory mechanism). Serum Iron level will be low.
- Hemoglobin measured at 1 and 2 years old. CBC, between 5-12 years old, and in adolescence.
- All formula fed infants will need iron-fortified formula, cereals. At 1 years old, cow milk intake is limited to 16-24oz/day. Also, common with increased lead levels.
- Oral iron supplementation is possible. Too much iron causes constipation.
- Blood transfusion with severe anemia.
- If there is a problem with iron absorption (GI). Give Intramuscular injection: For IM iron supplementation use the Z-track injection method: Move the fatty tissue away, get to the muscle.
Anemic Individual will appears Pale : Pallor.
Tachycardia: increased heart rate.
Will get tired very quickly or easily: activity intolerance. Decrease attention span.
This disease can lead to heart failure, retarded growth, failure to thrive and more.
Sickle Cell Anemia:
What is it: Think of a sickle if you are not sure what a sickle is think of the weapon that death carries. That’s a sickle.
Normal Hgb A is replaced by abnormal sickle hemoglobin Hgb S.
RBC will change the shape to crescent/sickle-shape.
When does it happen:
Under Stress. Too much stress is never good.
Abnormally shaped RBCs, act to increase viscosity of blood, physically block arteries.
Numerous complications: heart attack, stroke, PVD.
Manifested by inadequate oxygen supply and decrease in RBCs.
Present in people of African or Mediterranean decent.
Vasooclusive crisis is most common. Child complains of pain localized or generalized. This is due to ischemia (death of tissues) brought on by vessel occlusion from sickled RBCs.
Aplastic crisis is diminished RBC production due to infection. Place on reverse isolation. Protect from us.
Hyperhemolytic crisis is an accelerated rate of RBC destruction. Child has jaundice, anemia, reticolytosis.
Can have CVA or MI, restrictive lung disease.
Sepsis from S.pneumonae or H.influenza is the major cause of death for children with SCD under 5 years old.
First discovered in 1981.
HIV is a global pandemic. Sub-Saharan Africa, Caribbean, Asia, Eastern Europe, South America have growing epidemics.
Most risk poses sexual transmission between MSM (men have sex with men), receiving partner, high viral load in the infected partner.
Can be transmitted with blood exposure in health care providers (rare). Blood transfusion prior to 1985.
HIV infects human cells with CD4 receptors on their surfaces (T-helpers).
Initially immune response is normal, and B-cells develop HIV-specific antibodies. Then, T cells are destroyed by HIV.
CD4 number reflects the stage of the disease: 800 – 1200 CD4 T cells is normal.
Immune system will remain healthy with CD4 count of 500 and above.
AIDS diagnosed as an end stage of the disease with CD4 count of below 200.
Opportunistic diseases will develop.
Prevention: teach the general public. HIV screening to be offered. It is a law in NYS, to offer HIV testing to EVERY patient. Children over 13 years old can be tested without parental consent.
Teach: No needle sharing for IV drug users. HIV testing of every pregnant women. AZT started if positive as soon as possible, to prevent vertical transmission.
Vertical transmission: 30-50% of babies born to HIV+ mothers, will be HIV+.
If mother is treated with AZT prenatally, the risk drops to 4-8%. All of the children will test positive initially then, should test negative by 18 mos.
Manifestation and Care.
- Will receive same drugs. May have monthly gama globulin infusion.
- No live vaccine immunization.
- Patient will have failure to thrive, weight loss.
- Diapering using standard precautions.
- Clean surfaces with Bleach to prevent contamination.
- High risk for pneumonia, and other diseases.
- Child should attend school. Risk of transmission is low.
VENTRICULAR SEPTAL DEFECT (VSD)
Is the Opening between ventricles. Blood from left ventricle (LV) gets mixed with blood in Right Ventricle (RV). Resulting in Increased pulmonary blood flow/pressure. Murmur present on auscultation. HF is common.
Small defects may close on their own. Larger defects repaired with placing Dacron patch over the opening. Approach is through right atrium.
Several procedures available. OR operation with placement on cardiopulmonary bypass machine. Another approach is during cardiac cath. Child is sedated. Septal occluder introduced through femoral vein, the IVC and through the right atrium to the defect.
ATRIAL SEPTAL DEFECT (ASD)
Is an Opening between atria. Oxygenated blood flow from left atria gets mixed with de-oxygenated blood.Increased pulmonary blood flow. Usually patient asymptomatic. May have murmur, May develop HF when 30-40 y.o. May develop atrial dysrhythmias. Surgery before school age : surgical patch closure.
Surgery with cardiopulmonary bypass. Other approach same as VSD, during cardiac cath. Patient will need 6 month low dose aspirin therapy.
TETRALOGY OF FALLOT
Four defects: VSD, pulmonary stenosis (PS), overriding aorta, right ventricular hypertrophy. Could be right to left, or left to right shunt. Also, pulmonary stenosis causes backflow to RV, due to Aorta position mixed blood could be distributed systemically.
Infants could be pale/cyanotic, especially during feeding/crying. Murmur present. Older children may have “blue spells”. Squatting relieves cyanosis.
Surgical treatment: Palliative shunt- created to move blood from subclavian artery to pulmonary arteries. Complete repair- first year of life: closure of VSD, resection of stenosis, pericardial patch to increase RV outflow. Open heart surgery with by-pass machine
Tonsillectomy – Surgical removal of the palantine tonsils:
- Not be done before: Age 3 years
- not for children with Cleft Palate
- No Acute infection at the time of surgery
- Blood dyscrasias
Caused by the Epstein-Barr virus Mildly contagious, Transmitted by direct contact, blood transfusions, transplantations, sexual contact. Known contact incubation 30-50 days
Symptoms include general malaise, lymphadenopathy, severe sore throat, fever Macular rash on the trunk Enlarged tonsils, Compromised airway, Enlarged spleen.
Acute symptoms last 7-10 days. Fatigue 2-4 weeks. COMPLICATIONS includes: Seizures, Ataxia, Anemia, Myocarditis, Ruptured Spleen, Rare is Guillian-barre’
Nursing Care provide comfort. Fluid intake Medications Tylenol, Opiods, Tylenol with codeine elixir form Bed Rest
No contact sports for 1 mos (risk of ruptured spleen).
The Occurrence is rare it is a MEDICAL EMERGENCY ! it involves Inflammation of the epiglottis. Swollen epiglottis cannot rise and allow the airway to open Incidence mostly in children 2-5 years of age and adults.
It Begins with mild upper respiratory infection. The complain: severe sore throat, problem swallowing High fever.
“Hot potato” voice ( I do not know how they determine that). Increasing respiratory distress: drooling, noisy breathing, unable to lie down. Tripod position: sitting upright leaning on hands, rapid breathing, open mouth, drooling again.
Child may protrude tongue to increase movement in the pharynx. If gag reflex is stimulated, swollen, inflamed epiglottis can be seen to rise in the back of the throat; appears cherry red; however, this should a be done if the child has the classic symptoms of epiglottitis. NEVER attempt to visualize the epiglottis directly with a tongue blade or obtain a throat culture.
To Diagnos: Lateral neck X-ray Treatment includes: IV antibiotics 7-10 days, IV therapy to maintain hydration, Possible prophylactic intubation or tracheostomy, Corticosteroids, Humidified oxygen, Pulse oximetry. Remember to provide education and support to patients.
lastly: for Now:
One un affected Carrier Mom, One unaffected carrier Dad: One in 4 Children will be affected. 2 in 4 children will be affected. 1 in 4 children will be a carrier.
Genetic disorder in people of Mediterranean descent. Thalassemia major is also known as Cooley anemia. If untreated Cooley anemia is fatal in first decade of life.
How it Works
Normal Hgb composed of 2 alpha and 2 beta chains. In thalassemia there is a defect in synthesis of beta chains. This results in defective, unstable hemoglobin. This causes severe anemia. As a compensatory mechanism, body produces extra RBCs. Due to defective Hgb, RBCs have shortened lifespan.
Due to treatment (blood transfusions) and RBC overproduction, excessive amounts of iron are stored in the various body organs. This may bring organ damage.
Sign and Symptoms:
Chronic hypoxia. Severe anemia. Retarded growth, delayed sexual development. End organ damage: DM (iron destruction of pancreas), Splenomegaly, hepatomegaly, cardiomegaly, bone marrow hyperplasia, bone deformity.
How is it treated
Blood transfusions to maintain Hgb level above 9.5g/dl. Every 2-3 weeks. Iron-chelating agents given IV or SQ (with pump) or PO. Vitamin C to increase iron excretion. Splenectomy maybe required. Reminder, patient is at high Risk for infections after splenectomy.