Pediatric Cardiac Disorders

Cardiac Disorders in Pediatrics

Two Major groups of Disorders:
• Congenital
- AKA “born with”
- Most structural defects
• Acquired
- Develop later in life
- Bacterial endocarditis
- Rheumatic fever
- Kawasaki disease
- Systemic HTN
Incidence and Causes
• 5-8 in 1000 live births Background info/Hemodynamics
• Cause unknown
- Multiple factors • Review fetal to neonatal circulation
- Genetic/family history • Blood flows from area of high pressure to one
- Environment of low pressure
-Toxins • The greater the pressure gradient, the greater
- Viruses the rate of flow.
- Maternal Chronic Illness (diabetes, • The greater the resistance, the lower the rate
seizure meds) of flow
-Chromosomal abnormalities • In the NORMAL HESRT, pressure on the R side
- Down Syndrome are less than the L side, and the resistance in
- DiGeorge syndrome the pulmonary circulation is less than that in
- Noonan or William Syndrome the systemic circulation.
- Trisomy 13 or 18
Older Classifications of Congenital Heart Disease
• Acyanotic
- “Pink”
- No unoxygenated blood goes to the
periphery
• Cyanotic
- “Blue”
- Unoxygenated blood is shunted to be
periphery
- May be pink
Newer Classifications of CHD
• Hemodynamic Characteristics
- Increased Pulmonary blood flow- meaning
too much to lungs; “pink”; pulmonary
edema
- Decreased pulmonary blood flow-
meaning too little to lungs; “blue”;
cyanotic
- Obstruction of blood flow out of the heart-
meaning can’t get to lungs or body.
- Mixed blood flow- most common
 Nursing Interventions
• Pre-procedure:
- Complete a thorough hx and physical exam
- Check for allergies to iodine and shellfish
- Age-appropriate teaching and preparation
- NPO 4-6 hrs before procedure; sedation-IV
or PO
- Monitor VS, SaO2, Hgb, Hct, coags, BMP
- Mark pedal pulses- before procedure to
ensure correct palpation afterwards.
- Determine the amount of sedation based
on the child’s age, condition and type of
procedure.
• Post-procedure
- For bleeding at site of insertion of catheter
in groin
- Pulses esp. distal to site of insertion, temp
and color of extremities, VS q 15
- Remember the 5 P’s (pain, pallor, pulse,
paresthesia, paralysis) OR CMTS-
circulation, mobility, temp, sensation
- Heart rate for one full minute, for signs of
dysrhythmias or bradycardia.
- Prevent bleeding by keeping immobilized
for 4-8 hrs.
- I & O, especially O. fluids may be offered
po staring with clear liquids.
- Labs: infants are at risk for hypoglycemia-
monitor blood glucose as child may need
Test of Cardiac Function IV with dextrose
• Prenatal ultrasound - Encourage the child to void to promote
• Chest x-ray excretion of contrast medium.
• Electrocardiogram (ECG) Potential Cardiac Catheterization Complications:
• Echocardiogram • Potential cardia catheterization complications:
• Cardiac Catheterization - Nausea &/or vomiting
• Stress Test (dobutamine or exercise) - Low-grade fever
• Cardiac MRI - Loss of pulse in catheterized extremity
- Transient dysrhythmias
Cardiac Catheterization - Acute hemorrhage from entry site
• Invasive routine diagnostic procedure - Apply direct continuous pressure at
• Benefits 2.5 cm above the catheter entry site to
- Better visualization localize pressure over the location of
- Actual pressures, saturations, the vessel puncture.
hemodynamic values. - Keep the child flat and notify the
• Risks: physician
- Hemorrhage - prepare for possible administration
- Fever of additional fluids prn
- N/V
- Loss of a pulse
- Transient dysrhythmias
 - Increased pulmonary blood flow
- Decreased systemic blood flow
PDS, ASD, VSD
Symptoms
- Increased work of breathing
- Rales/rhonchi and/ or wheezing
- Failure to thrive

Patent Ductus Arteriosus

• Ductus (connects the pulmonary artery to the
aorta) doesn’t close
• Common in preemies
• “machinery” murmur at the upper left sternal
border
• Treatment
Symptoms of Congestive Heart Failure - Indomethacin
• Increased work of breathing -Ibuprofen- drug of choice
• Tachycardia - Cath lab
• Decreased pulses - Ligation
• Decreased urinary output
• Poot weight gain Arterial Septal Defect
• Diaphoresis with activity • Hole between two atria of heart
• Hepatomegaly • Usually, asymptomatic
• Cold, cool extremities, especially with stress or • Harsh systolic murmur over the 2 nd and 3rd
activity. intercostal space
• Jugular vein distention • Split heart sound (lub-dub-dub)
• Decreased BP is LATE sign • If not treated, increased risk of atrial
dysrhythmia or stroke
Continued management of CHF • Usually close on own

Nutrition Ventricular Septal Defect

- Smaller, more frequent feeds
- High calorie formula
Decrease Respiratory Effort
- Rest
- Avoid colds, RSV
- Position with HOB
- Avoid crying and distress
Family Support/ Education
- Keep them present, holding, rocking, AMAP
Improve Tissue Oxygenation
- Meds assist with this by increasing efficiency of
the heart
- Oxygen may be added with appropriate order,
especially if there is pulmonary edema or
lower respiratory infection
Defects with Increase Pulmonary Blood Flow
Abnormal connection between two sides of heart leads
to:
- Increased blood volume on right side of heart
• Hole between two ventricles of heart
• Symptoms related to size and location of VSD
and amount of pulmonary blood flow (easy
fatigue, loud harsh systolic murmur, thrill is
palpable)
• Fix by patching with Goretex
• Diuretic (digoxin) to prevent pulmonary edema
Atrioventricular Canal
• Endocardial cushion defects results from
incomplete fusion of the endocardial cushion
or septum
• ASD, VSD, and affected mitral and tricuspid
valves
• Associated with Down syndrome
• Symptoms related to size of hole, degree, and
size of ventricles
• Often accompanied with pulmonary
hypertension
Nursing Management
• Avoid oxygen, especially pre-op
• Diuretics- furosemide, chlorothiazide,
spironolactone
• Monitor Vs, I&O, daily wt.
• Encourage rest periods to conserve energy.
• Monitor lab: Hgb, Hct, electrolytes
• Closely monitor feedings
-may need higher calorie foods

Obstructive Defects
• Coarctation of the aorta, artic, stenosis,
pulmonic stenosis. Defects with Decreased Pulmonary Blood Flow and
• Symptoms dependent upon area of Mixed Defects
obstruction
• May or may not be cyanotic (usually are)
Coarctation of Aorta • Tetralogy of Fallot
• Narrowed aorta leads to decreased systemic • Transposition of Great Arteries
blood flow • Truncus Arteriosus
• May not present until early childhood • Hypoplastic Left Heart Syndrome (HLHS)
• Bounding upper extremity pulses, weak to • LOTS of other defects that are uncommon,
absent lower extremity pulses. book discusses them
• Hypertension
Post-op Coarctation Care Effects of Hypoxemia
• Nuero checks • Main clinical manifestations:
• Urine output - Cyanosis
• Blood pressure. -Polycythemia
• Pain - Thicker blood
- Clubbing
Aortic Stenosis - Clotting abnormalities
- Delayed growth and development- can be
• Narrowing of the aortic annulus due to
associated with any heart defect.
degeneration and calcification of the valve
leaflets
Hypoxemia Management
• Obstructs blood flow to body
• Prostagladin E1 given if cyanosis shown as
• Leads to left ventricle hypertrophy
newborn
• Asymptomatic often
• Assess for and treat tet spell
• Chest pain with exercise
• Surgery
• Sometimes see sudden death
- Corrective or palliative- often stage
• Repair with ballooning, repair, or replacement
• Prevent dehydration
of valve
• Avoid oxygen
Hypercyanotic “tet spells”
• Acutely cyanotic
• Decreased pulmonary blood flow & increased
right to left shunting
• Prompt tx to prevent brain damage &/or death
-Calm infant/child
- place in knee chest position
- toddler will get in “squatting” position to
compensate for hypoxia
- Give oxygen
- morphine/fentanyl/versed given
Tet Repair
• Complicated
• Dependent on how big RV is, how stenotic
pulmonic valve is and how big the VSD is
• Either fly or die
• Palliative Shunt: modified Blalock- Taussig
shunt
• Complete repair – operative mortality less than
3%
Transposition of Great Arteries
• Not good
• Cath lab initially
• Prostaglandins
• Surgery at 6-7 days old- arterial switch of
pulmonary artery and aorta, but also coronary
arteries are switched and re-anastomosed
• Long term prognosis very good
Hypoplastic Left Heart Syndrome
• Very Bad. However, survival rates have
changed dramatically in the last 15 years. Can
be as high as 95%
• Can not correct easily- parents must choose
• 3 staged surgeries: Norwood, Mod Blalock
Taussig, & Glenn procedure vs transplant
• Long term data not in yet, probably need
transplant
Management of Children with mixed Defects
Medications
• Digoxin
- Improves contractility of heart
- Review dig toxicity- pulse rate in infants and
children
• Diuretics- furosemide
 • Ace-inhibitors (angiotensin converting - Congenital Heart Information Network: Lots
inhibitors- the PRIL’s) of links for families and persons with CHD
-Reduce afterload on the heart make heart - Website: from Cincinnati children hospital
pump more efficiently:
• Beta-blockers- cause decreased heart rate, BP Kawasaki Disease
vasodilation • Multi disorder involving vasculitis & may
• Decreased cardiac workload progress to coronary arteries causing
- Med as stated aneurysm formation
-Decrease stimulation • Leading cause of acquired heart dz in US
- Cluster care • Etiology still unknown
- Maintain neutral thermal environment • 3 phases: acute, subacute, convalescent
-Sedation for irritable child
• Remove accumulated fluid and sodium Criteria for KD (must meet 5 out of 6)
- Closely monitor I and O • Fever greater than 5 days
Restrict fluid in acute phase • Conjunctival infection without exudate
Weight daily if stable. • Oral changes: erythema, “strawberry tongue,
fissure lips
Post-Operative Care • Extremities changes: peripheral edema,
• Pain erythema of palms and soles, peeling of hands
• Cardiac monitoring and feet
-heart rate • Erythematous rash
- Blood pressure • Cervical lymphadenopathy
-Intracardiac pressures
• Chest tube care Other Manifestations
- Quantity and quality of output • Symptoms of inflammation
• Urine output - Increased C reactive protein level
- minimum 1 ml/kg/ hour - Increased ESR
• Neurological checks • Cardia symptoms
- move all extremities - low L ventricular function as seen on
- Back to baseline Echocardiogram
• Respiratory care -Children do not generally have sx of CHF
- deep breathing • Other lab changes
- IS - anemia
• Rest and activity - Leukocytosis with “L shift”
- Up next day • Tx best within first 7-10 days:
- Ambulate -ASA 80-100 mg/kg/day initially- this is one dx
• GI distress that requires use of high doses of aspirin even
-avoid vomiting in children. Dose is decreased to 3-5
mg/kg/day once afebrile 48-72 hrs.
Care of the Family and Child with Congenital Heart - IVIG 2g/kg over 8-12 hrs
Disease
• Help gamily adjust to the disorder
- May be grieving loss of normal child
• Educate family
• Help family cope with effects of the disorder
• Prepare child with effects of the disorder
- Remember developmental level of child
- Pain, scars, IS, activity
• Refer to support group with families who have
already been through the experience.
- Touch is the IL Assoc. The link opens a broad
site, then click on IL

Education of Parents
• Teach parents common sign of aspirin toxicity
while on high doses of ASA
- Tinnitus
- Headache
- Dizziness
- Confusion
• Teach parents to report recurrence of fever
• Teach parents CPR
• Inform parents that final cardiac sequelae may
not be known for some time.
NURSING CARE OF CHILDREN WITH HEMATOLOGIC
ALTERATIONS
• Disorders related to the blood and blood
forming organs in childhood encompass a wide
range of diseases and pathologic states.
• Since the blood is a multipurpose fluid involved
in the functions of so many tissues and organs,
either primary or secondary changes in the
blood are reflected in the essential functions of
these structures.
• Disorders of blood and blood forming organs in
childhood include the anemias, defects in
hemostasis, and the immunologic deficiency
diseases. Neoplastic disorders of the
hematopoietic system are also included.
ANATOMY
The hematologic system consists of:
• The blood
• And blood forming (hemopoietic) tissues:
o Red bone marrow (myeloid tissue)
o Lymph nodes
o Spleen
Blood is composed of two components:
• A fluid portion called plasma
• A cellular portion known as the formed
elements of the blood
The two components are approximately equal in
volume.
• Plasma is about 90% water and 10% solutes.
• The principal solutes are:
o The proteins: albumin, globulin, and
fibrinogen.
• The cellular elements are:
o Red blood cells (erythrocytes),
o White blood cells (leukocytes),
o Platelets (thrombocytes).
Hematopoiesis (blood-formation)
• All of the formed elements of the blood, except
to some extent the agranulocytes, are believed
to be formed in myeloid tissue during
postnatal life.
• During embryonic development the
mesenchyme, spleen, liver, thymus, and yolk
sac serve as additional sites of blood cell
formation.
• In certain blood disorders these sites,
particularly the spleen, can be stimulated to
produce blood cells, and constitute
extramedullary hematopoiesis.
• Each blood cell originates from a primordial
(primitive) cell called a blast, or stem, cell in the
bone marrow. This hemocytoblast in turn gives
rise to the erythroblast, myeloblast,
monoblast, lymphoblast, and megakaryoblast.
• Reticuloendothelial system consists of widely
dispersed cells of mesodermal origin that line
the vascular and lymph channels. These cells,
called reticular cells, are capable of:
o phagocytosis (ingestion and digestion
of foreign substances),
o formation of immune bodies,
o differentiation into other cells, such as
hemocytoblasts, myeloblasts, or
lymphoblasts.
Blood functions:
• Transporting substances needed for cellular
metabolism in the tissues;
• Regulating acid base balance;
 • Protecting against infection and injury.
Differences between child and adult hematological
systems
• In infants and young children all of the bone
contains red marrow (so called because of its
color from formation of erythrocytes).
• At the end of adolescence, only the ribs,
sternum, vertebrae, and pelvis continue to
produce blood cells. The remainder of the
bone marrow becomes yellow from deposition
of fat.
Regulation of erythrocyte production
• The usual life span of the mature erythrocyte is
120 days.
• They are removed from circulation, mainly by
the spleen.
• Because RBCs are not capable of replication,
the bone marrow releases new, immature
erythrocytes called reticulocytes to replace the
RBCs removed from circulation.
• The hemoglobin is broken down into the iron
containing pigment hemosiderin and the bile
pigments biliverdin and bilirubin.
• The basic regulator of erythrocyte production
is believed to be tissue oxygenation.
• In states of tissue hypoxia, erythropoietin (also
called erythropoietic stimulating factor or
hemopoietin) is released by the kidneys into
the blood stream.
1 week – 1 year 2.7-4.5 x 10^12 cells/L
2 – 12 year 3.9-5.3 x 10^12 cells/L
12 – 18 year: Male 4.5-5.5 x 10^12 cells/L
Female 4-5 x 10^12 cells/L
Age peculiarities of blood newborn:
• Short life span of erythrocytes 12 40 days
versus 120 days
• Erythrocytosis 6 7 × 10^12 cells/L
• Anisocytosis (presence of different forms of
RBCs inside vessels)
• Hemoglobin level 180 240 g/L
• Hb F – 63 92 % versus 5 % adults
• Color index (CI) 1. 1 1. 3 (degree of saturation
of Hb in one erythrocyte)
• Erythrocyte sedimentation rate 0 2 mm/hour
• Leucocytosis 11 33 × 10^9 cells/Liter
• Presence of extramedullar sources of
hemopoiesis.
Age peculiarities of blood – child 1 mo – 1 yr:
• Decreasing Hb level to 120 110 g/L
• Erythrocyte level decreases, as well, to 4 3. 5 ×
10^12 cells/L
• Reticulocyte count is up to 10 %
• Colour index is low: 0. 8 0. 7
• Leucocytosis 10-12 × 109 cells/L appears after:
– adding new food in menu – during crying –
emotions
• 1st cross of WBC count – 5 mo
• Platelets have gigantic forms

Age peculiarities of blood:

WBC differential count during childhood
• Smaller than in adults general volume of blood.
3d 5d 3 yr 5 yr 12 yr
• Higher than in adults relative blood volume:
Eosinophils 1-4 % 1-4 % 3% 1-4 % 1-4 %
versus 50 80 mL/kg in adults.
Basophils 0-0.5 0-0.5 0-0.5 0-0.5 0-0.5
• RBC volume varies with age.
% % % % %
• Immediately after birth there is a period of
Neutrophils 3-5% 3-5% 3-5% 3-5% 3-5%
inactive erythropoiesis during which the iron
“bands”
obtained from catabolized RBCs is stored as
Neutrophils 54- 39- 24- 39- 54-
hemosiderin in the bone marrow and the liver
“segs” 62% 42% 30% 42% 62%
tissue.
Lymphocytes 11- 45% 63% 45% 26-
• These stores are greatest at 4 to 8 weeks of age
25% 33%
and function to protect the infant from
Monocytes 6-10% 6- 6- 6- 6-
anemia.
10% 10% 10% 10%
• Premature infants use up these stores within 6
to 12 weeks, whereas the iron stores of full
Age peculiarities of blood – child 1 mo – 1 yr:
term infants last up to 20 weeks.
• Increasing of Hb level to 130 140 d/L till 15
years
Erythrocyte (RBC) count – age reference
• Erythrocyte level 4. 5 5. 0 × 10^12 cells/L
Cord 5-7 x 10^12 cells/L
• Reticulocytes are about 0. 5 5 %
1-3 days 6-7 x 10^12 cells/L
 • Leukocyte level 7 9 × 10^9 cells/L o Moderate: Hb 89 70 g/L
• Second WBC count cross in 5 years of age o Severe: Hb < 70 g/L
• Morphologic (describe the size of RBCs):
Examination: subjective data o (1) normocytic,
There are some weighty clinical signs that directly show o (2) microcytic,
on blood disorders. They are: o (3) macrocytic.
• Hemorrage (bleeding); • According to the amount of hemoglobin in the
• Hematoma; cell (describe the color and Hb content of the
• Enlargement of lymph nodes; cells):
• Pallor; o (1) normochromic
• Ossalgia (pain in bones). o (2) microchromic.

Other complaints: Anemia Assessment

• Often headaches, • Take health history:
• Dizziness, light headednass, o Careful diet history to identify any
• Slowed thought processes, dicreases attention deficiencies,
span, o Evidence of pica – eating clay, ice,
• hyperthermia, paste.
• Apathy, • Observe for manifestations of anemia:
• Shortness of breath, o Muscle weakness
o Easy fatigability:
• Easy fatigability.
▪ frequent resting,
- Pica – character sign of iron deficient anemia.
▪ shortness of breath,
Eating clay, ice, paste.
▪ poor sucking (infants)
Anemias
Manifestations of anemia
• A condition in which the number of RBCs
• Pale skin, mucous membranes, lips, nail beds,
and/or the hemoglobin concentration is
and conjunctiva
reduced below normal:
o Waxy pallor seen in severe anemia
o 0 14 days Hb<145 g/L
o Capillary refill
o 15 28 days Hb<120 g/L
o no change color
o 1 mo 1 yr Hb<110 g/L
The basic cause of anemia is either • Rapid, pounding heart beat
(1) An increased loss or destruction of red blood • CNS manifestations:
cells. o Headache
(2) An impaired or decreased rate of production. o Dizziness
o Light headedness
An etiologic classification is based on the various o Irritability
conditions that can lead to either of these results. o Slowed thought processes, dicreases
attention span
Classification of anemias: o Apathy
• Decreased Production of RBCs: o Depression
o Iron Deficiency Anemia
o Aplastic Anemia Severe anemia
• Increased Destruction of RBCs: • Impaired healing and loss of skin elasticity
o Hemolytic Anemia • Thinning and early greying of the hair
o Sickle Cell Anemia • Abdominal pain, nausea, vomiting, anorexia
o Thalassemia • Low grade fever
• Blood loss:
o Iron Deficiency Anemia Aplastic anemia
o Hemorrhagic Anemia • Is a condition wherein injury to or abnormal
• Severity: expression of the stem cells in the bone
o Mild: Hb 110 90 g/L marrow results in the production of
 inadequate numbers of erythrocytes,
leukocytes, and platelets.
• Can be hereditary or acquired.
• The hereditary form of aplastic anemia,
Fanconi's anemia (FA), is a rare autosomal
recessive disorder that develops early in life
and is accompanied by multiple congenital
abnormalities.
• The age of onset of varies.
• Typically, the first sign noted is increased
bruising caused by the decreased number of
platelets.
• Vulnerability to infection because of the
decreased number of WBCs.
• When the RBCs have decreased in number, the
signs and symptoms of anemia begin to
manifest:
o pallor, weakness, and difficulty
breathing
• Impaired growth and development
Criteria for severe aplastic anemia
Severe Aplastic Anemia
• Neutrophils < 2 × 10^9 cells/L
• Platelets < 50 × 10^9 cells/L
• Reticulocytes < 0. 5 %
• Bone Marrow Cellularity < 20 %
Nursing Diagnosis
Iron Supplements
• Ineffective tissue perfusion related to anemia
• Imbalanced nutrition: less than body
requirements related to inadequate iron
intake
• Deficient earegiver knowledge related to age
appropriate iron intake
• Activity intolerance related to decreased
oxygen delivery to the tissues.
Goals:
1. Minimize physical exertion and emotional
stress:
o Anticipate and assist in those activities
of daily living that may be beyond
child’s tolerance.
Blood Transfusion Nursing responsibilities
o Provide play activities that promote
rest and quiet but prevent boredom
and withdrawal.
o Encourage parents to remain with
child, etc.
2. Increase oxygen to tissues:
o Position for optimim air exchange
o Administer supplemental oxygen if
needed
3. Help replace blood elements:
o Administer blood, packed cells,
platelets as prescribed.
4. Promote adequate intake of iron rich foods:
o Encourage exclusive breastfeeding of
infants for 4 6 months after birth.
o Diet rich with food sources of iron:
meat, liver, fish, egg yolks, spinach,
oysters, peas, green leafy vegetables,
nuts, whole grains, iron fortified infant
cereal and dry cereal.
o Feed milk as supplemental food in
infant’s diet after solids are begun.
o Administer iron preparations as
prescribed:
▪ Give between meals
▪ Administer with fruit juice or
multivitamin preparation
▪ Do not give with milk or
antacids
▪ Give fluid preparations with
dropper, syringe, or straw to
avoid contact with teeth
▪ If the child is getting enough
iron, stools should be a tarry
green color.
• Although oral iron supplementation is
generally perceived as a safe treatment
alternative, an overdose can be lethal for
children.
• Initial signs and symptoms of overdose: –
vomiting, – abdominal pain, – bloody diarrhea.
• These manifestations are typically followed by
shock, lethargy, and dyspnea. Often the child
will appear to improve before the onset of
severe metabolic acidosis, coma, and death.
• Urgent treatment is essential and includes
flushing out unabsorbed pills, administration of
Desferal, and supportive therapy.
• Positively identify donor and recipient blood
types and groups before transfusion is begun;
verify with one other nurse or physician.
• Transfuse blood slowly for first 15 20 min;
remain with patient.
 • In event of signs or symptoms, stop transfusion
immediately, maintain patent IV Line, notify
physician.
• Insert urinary catheter to monitor hourly
outputs
• Monitor for evidence of shock.
• Save donor blood to re crossmatch with
patient’s blood.
BLOOD TRANSFUSION COMPLICATIONS
• Hemolytic reactions
• Febrile reactions
• Allergic reactions
• Circulatory overload
o too rapid transfusion or excessive
quantity of blood transfused
• Air emboli
o May occur when blood is transfused
under pressure
• Hypothermia:
o Allow blood to warm at room
temperature (1 hr)
• Hyperkalemia
o In massive transfusion or patient with
renal problems.
SICKLE CELL ANEMIA
• A hereditary disorder in which normal adult
hemoglobin (Hb. A) is partly or completely
replaced by an abnormal hemoglobin (Hb. S)
causing distorsion and rigidity of red blood
cells under condition of reduced oxygen
tension.
• Sickle cell anemia is part of a group of diseases
called hemoglobinopathies.
• Sickle cell anemia is an autosomal recessive
disorder. The inheritance is described as
intermediate because the gene is partially
expressed in the heterozygous state and
completely expressed in the homozygous
state.
• Sickle cell anemia is found primarily in the
black race, although infrequently it affects
whites, especially those of Mediterranean
descent.
SICKLE CELL ANEMIA – Manifestations:
• Take health history, especially regarding any
evidence of sickling crisis and history of the
disease in family members.
• General:
o Growth retardation
o Chronic anemia
o Delayed sexual maturation
o Marked susceptibility to sepsis
• Other signs and symptoms.
o weakness; anorexia; joint, back, and
abdominal pain; fever; and vomiting.
o Chronic leg ulcers are common in
adolescents and adults and are
thought to be the result of thrombosis
and decreased peripheral circulation.
SICKLE CELL ANEMIA – Vaso-occlusive (sickling) crisis
• Pain in area(s) of involvement
• Signs of ischemia:
o Extremities: painful swelling (“hand
foot” syndrome), painful joints
o Abdomen: severe pain resembling
acute surgical condition
o Cerebrum: stroke, visual disturbances
o Chest: pseudopneumonia, propracted
episodes of pulmonary disease
o Liver: obstructive jaundice, hepatic
coma
o Kidney: hematuria
Diagnostic evaluation
• For screening purposes:
o the Sickledex is commonly used. If the
test is positive, hemoglobin
electrophoresis is necessary to
distinguish between those children
with the trait and those with the
disease.
• Sickling test (sickle cell slide preparation):
place a drop of blood on a slide and cover it
with a sealed cover slip to produce deox
ygenation. Eventually sickling of the red blood
cell occurs.
• Hemoglobin electrophoresis
("fingerprinting"). This test is accurate, rapid,
and specific.
Child with Fluids and Electrolytes Problem
Causes and Risk Factors
Electrolytes are found in fluids in the body.
Dehydration can upset the delicate balance of
electrolytes in an infant or child. Children are especially
vulnerable to dehydration due to their small size and
fast metabolism, which causes them to replace water
and electrolytes at a faster rate than adults.
An illness that causes severe vomiting, diarrhea, and a
high fever increases the risk of a fluid and electrolyte
disturbance, as does taking medication that causes
excessive urination. Profuse sweating from physical
exertion can also increase the risk of dehydration.
Fluid and electrolyte disturbances are common in
children with a serious underlying medical condition.
For instance, children with chronic kidney disease, a
condition that affects the ability of these organs to
maintain proper fluid and electrolyte levels, are at risk.
Conditions that affect the production of thyroid
hormones and parathyroid hormones, which help
regulate calcium and other electrolytes, can also make
a child susceptible to a fluid and electrolyte imbalance.
Children with heart disease may retain sodium and
water, and develop abnormal electrolyte levels.
Those who have cancer and receive a bone marrow
transplant—which increases the risk of infections and
fever—or take chemotherapy medications that cause
kidney problems are also at higher risk of developing a
fluid and electrolyte imbalance.
Symptoms
Signs of a fluid or electrolyte disorder vary widely. Mild
electrolyte disorders often cause no symptoms.
Symptoms of a more severe imbalance depend on
the type of disorder.
Dehydration may make your child’s urine appear
darker than usual. Other electrolyte disorders cause
confusion, weakness, cramping, and muscle spasms.
Some can cause difficulty breathing, dizziness, and a
rapid heart rate. Parents who notice any of these
symptoms, especially if a child has an underlying
medical condition or a fever, should have their child
evaluated by a doctor immediately.
Rapid diagnosis and treatment are important. Severe
dehydration and the accompanying electrolyte
disturbances can reduce blood and mineral flow to vital
organs, including the brain, heart, and liver. In rare
instances, this can make brain tissue swell or shrink,
causing seizures, or life-threatening disturbances in
heart rhythm, known as arrhythmia.
Our doctors use the results of a physical exam and
laboratory tests to diagnose fluid and electrolyte
disorders in children.
Physical Exam
During a physical exam, your child’s doctor assesses the
severity of a fluid and electrolyte disturbance and looks
for clues to identify the cause. The doctor asks if your
child has been ill recently and whether he or she has
had any unusual symptoms, such as muscle cramps,
dizziness, rapid heart rate, or confusion.
The doctor measures your child’s blood pressure to see
if it is low, which may suggest that the child is
dehydrated. In addition, he or she may examine your
child’s skin to check its elasticity. The doctor may also
test your child’s reflexes, which can be affected by
dehydration.
Laboratory Tests
Blood and urine tests are used to confirm an electrolyte
imbalance and determine its severity. Depending on
how ill your child is, these tests can be performed using
blood and urine samples taken in the doctor’s office or
the hospital. Laboratory results are usually available
quickly.
Based on the results of these tests and any underlying
medical conditions your child may have, our doctors
develop an appropriate treatment plan to correct a
fluid and electrolyte imbalance and prevent
complications.
Alterations in Fluid, Electrolyte and Acid-Base Balance
in Children
• Fluid is dynamic state
• Body fluid: is body water that has solutes
dissolve on it. Some solutes are electrolyte.
• Electrolyte such as Na, K, Ca, CL and Mg.
Water may serve as:
1. Medium of metabolic reaction with cells.
2. Transporter for nutrients, waste products, and
other substance.
3. A lubricant
4. A shock absorber
5. Regulate and maintain body temperature.
General concepts:
• Intake= output = fluid balance
• Sensible losses
- Urination
- Defecation
- Wound drainage
• Insensible losses
- Evaporation from skin
- Respiratory loss from lungs
Fluid compartments
• Intracellular
 - 40% of body weight
• Extracellular
- 20% of body weight
2 types:
- Interstitial (between)
- Intravascular (inside)
- Transcellular: includes cerebrospinal fluid,
pleural, peritoneal and synovial fluid.
Function of ICF & ECF:
• ICF: is vital organ to normal cell function, its
contain solutes such as oxygen, electrolytes
and glucose. It provides a medium in which
metabolic process.
• ECF: it is the transport system that carriers
nutrients and waste product from the cell.
Pediatric Differences
• ECF/ ICF ratio varies with age
• Neonates and infants have proportionately
larger ECF volume
• Infants: high daily fluid requirement with little
fluid reserve; this makes the infant vulnerable
to dehydration.
Fluid loss; infants and <2yr.
• Excretion is via the urine, feces, lungs and skin
• Have greater daily fluid loss than older child
• More dependent upon adequate intake
• Greater about of skin surface (BSA), therefore
greater insensible loss
• Respiratory and metabolic rates are higher
therefore dehydrate more rapidly.
Pediatric differences
• <2yr kidneys immature
• Less able to conserve or excrete water and
solutes effectively
• Infants have weaker transport system (ion,
bicarbonate) greater risk for acid/base
imbalances
• Difficulty regulating electrolyte such as Na, Ca
Fluid volume imbalances
• Dehydration: loss of ECF fluid and sodium
- Caused by vomiting, diarrhea, hemorrhage,
burns, NG suction.
- Manifested by weight loss, poor skin turgor,
dry mucous membrane, VS changes, sunken
fontanel
• Fluid overload: excess ECF fluid and excess
interstitial fluid volume with edema
- Causes: fluid overload, CHF
- Manifested by weight gain, puffy face and
extremities, enlarged liver
Mild dehydration: by history
• Hard to detect because the child may be alert,
have moist mucous membranes and normal
skin turgor
• Weight loss may be up to 5 % of body weight
• The infant might be irritable; the older child
might be thirsty
• Vital signs will probably be normal
• Capillary refill will most likely be normal
• Urine output may be normal or less
Moderate dehydration
• dry mucous membranes; delayed cap refill >2
sec; weight loss 6-9% of body weight
• irritable, lethargic, unable to play, restless
 • decreased urinary output: <1ml/kg/hr; dark loss (dehydration) delaying sign and symptoms
urine with SG > 1.015 (in child >2 yr) until condition is quite serious.
• sunken fontanel • Caused by concentrated IV fluids or tube
• HR increased, BP decreased. Postural vital feedings.
signs
Etiology of Dehydration
Severe dehydration 1. Vomiting and diarrhea, nasogastric suction and
• Weight loss> 10% BW burn
• Lethargic/comatose 2. Water loss=under the warmer environment
• Rapid weak pulse with BP low or undetectable; 3. Accumulation of fluid in third space.
RR variable and labored 4. Over use diuretics.
• Dry mucous membranes? Parched; sunken 5. Excessive exercise
fontanel
Rotavirus
• Decreased or absent urinary output
• Common viral from diarrhea
• Cap refill >4 sec
• All ages but 3mo-2yrs most common
Types of dehydration and sodium loss • Fecal/oral route
Sodium may be: • Virus remains active;
-10 days on hard, dry surfaces
• Low -4 hours on human hands
• High -1 week on wet areas
• Normal • Incubation period 1-3 days
• Symptoms: mild/ mod fever, stomach ache,
frequent watery stool (20/day)
1. Isotonic dehydration or Isonatremic • Treatment: prevention, hand washing and
dehydration isolation of the infected child.
• Loss of sodium and water are in proportion • Fluid rehydration for diarrhea, advanced to
• Most of fluid lost is from extracellular bland diet for older children
component • Breastmilk for the infant who BF
• Serum sodium is normal (130-150 mEq/L)
- Most practitioners consider below 135 and Clinical Management for Dehydration
above 148 a more conservative parameter • Blood may be drawn to assess electrolytes,
(138-148) BUN and creatinine levels
- Most common form of dehydration in young • An IV may be placed the same time
children from vomiting and diarrhea. • Oral rehydration solution is the treatment of
choice for mild-moderate dehydration
2. Hypotonic or Hyponatremic dehydration - 1-3 tsp of ORS every 10-15 mins to start (even
• Greater loss of sodium than water if vomits some)
• Serum sodium below normal - 50ml/kg/Hr is the goal for rehydration
• Compensatory shift of fluids from extracellular
to intracellular makes extracellular Why are drinks high in glucose avoided during
dehydration worse rehydration?
• Caused by severe and prolonged vomiting and • Simple sugars increase the osmotic effect in
diarrhea, burns, renal disease. Also by the intestine by pulling water into the colon,
treatment of dehydration with IV fluids thereby increasing diarrhea and subsequent
without electrolytes. fluid/ electrolyte loss
• Drinks high in glucose: apple juice, sodas, jello
3. Hypertonic or Hypernatremic Dehydration water.
• Greater loss of water than sodium
• Serum sodium is elevated Recommended foods during rehydration progression:
• Compensatory shift from intracellular to • Starches, cooked fruits and vegetables, soups,
extracellular which masks the severity of water yogurt, formula, breast milk.
 • Recent research has shown no difference that
return to normal diet with some attention to
lactose containing foods, depending upon the
child’s response.
IV Therapy
• Used for severe dehydration or in the child
who will not/ cannot tolerate ORS
• 24hr maintenance plus replacement given
within first 6-8hr (in ER) to rapidly expand the
intravascular space. Usually a normal saline
bolus.
• Slower IV rate for the remainder of the first
24hrs
• Nurse records IV volume infused hourly
Fluid Overload: Edema
• Increase capillary blood flow: Inflammation,
infection
• Venous congestion: ECF excess, Right sided
heart failure, muscle paralysis
• Increase Albumin excess: Nephrotic Syndrome
• Decrease albumin synthesis: Kwashiorkor, liver
cirrhosis
• Increase capillary permeability:
Inflammation/burns
• Blocked lymphatic drainage: tumors/surgery
Assessment/Management of Edema
• Ascites; periorbital edema
• Pitting edema for degree of swelling
• Daily weight and strict I and O monitoring
• Elevation/ change position Q2hr/ protect skin
against breakdown
• Distraction to deal with discomfort and
limitations of edema
Electrolyte Imbalance
• Electrolytes usually gained and lost in relatively
equal amounts to maintain balance
• Imbalance caused by:
- Abnormal route of loss (vomiting/diarrhea)
can disturb electrolyte balance
- Disproportionate IV supplementation
- Disease states: renal disease
Hypernatremia
• Excess serum sodium in relation to water
• Greater than 146 mmol/l
• Causes:
- Too concentrated infant formula
-Not enough water intake
• Clinical manifestation: Thirst, lethargy,
confusion
- Seizures occur when rapid or is severe
increase.
- Lab test: serum sodium increases
-Treatment: hypotonic IV solution
Hyponatremia
• Excess water in relation to serum sodium
• Less than 135 mmol/l
• Most common sodium imbalance in children
• Causes:
- Infants vulnerable to water intoxication:
dilute form, poorly developed thirst
mechanism so con to drink and cant excrete
excess water or IV fluid
• Clinical Manifestation: decreased level of
consciousness, swelling of brain cells
- Anorexia, headache, muscle weakness,
lethargy, confusion or coma
- Seizures occur when rapid or severe
- Lab tests: serum sodium
- Treatment: hypertonic solution
Hyperkalemia
• Excess serum potassium greater that 5.3 meq/l
• Causes:
- Excess K intake from IV overload, blood
transfusion, rapid cell death (hemolytic crisis,
large tumor destruction form chemo, massive
trauma, metabolic acidosis form prolonged
diarrhea and in DM when insulin levels are low
- Insulin drives K back into the cells.
- Decreased K loss from renal insufficiency.
• Clinical manifestation: all are related muscle
dysfunction: hyperactivity of GI smooth
muscle: intestinal cramping and diarrhea.
- Weak skeletal muscles
- Lethargy
- Cardiac arrhythmias (tachycardia, prolonged
QRS, peaked T waves: also AV block and
ventricular tachycardia)
- Lab test: serum potassium
- Treatment: correct underlying condition
- Dialysis (peritoneal or hemo), kayexalate (PO
or enema), K wasting diuretics, IV calcium,
bicarbonate, insulin and glucose.
- Low potassium diet.
Hypokalemia
• Decreased serum potassium less than 3.5
meq/l
 • Causes: diarrhea and vomiting, diuretics,
osmotic diuresis (glucose in urine as in DM),
NPO without K replacement in IV, NG, Suction
- Also in nephrotic syndrome, cirrhosis,
Cushing syndrome, CHF
• Clinical manifestation: Muscle dysfunction
• Slowed GI smooth muscle resulting in
abdominal distention, constipation and
paralytic ileus
• Skeletal muscles are weak, may affect
respiratory muscles.
• Cardiac arrythmias: hypokalemia potentiates
Digitoxin Toxicity.
• Lab test: serum potassium
• Treatment: oral and/or IV potassium, diet rich
in K.
Hypercalcemia
• Excess calcium 5 meq/l
• Needs vitamin D for efficient absorption; most
of Ca is stored in the bones
• Causes: bone tumors that cause bone
destruction, chemo therapy release Ca from
the bones; immobilization causes loss from the
bones (usually excreted) but if kidneys can’t
clear it, hypercalcemia results, increased
intake.
• Clinical manifestation: Calcium imbalances
alter neuromuscular irritability with non-
specific symsptoms
o Constipation, anorexia, N/V, fatigue,
skeletal muscle weakness, confusion,
lethargy.
o Renal calculi, cardiac arrhythmias
o HyperCa increases Na and K excretion
leading to polyuria and polydipsia.
o Rx: serum Ca, Ionized Ca, fluids, Lasix,
steroids, dialysis
Hypocalcemia
• Decreased serum calcium <4 meq/l
• Causes: decreases intake of Ca and/or Vit D
o Limited exposure to sunlight,
premature infants and dark-skinned
people at increased risk to inadequate.
Vit D and therefore decreased Ca
absorption.
o Parathyroid dysfunction, multiple
transfusion (Citrate binds Calcium),
steatorrhea (as in pancreatitis) binds
Calcium in the stool.
• Clinical Manifestation: acute situation related
to increased muscular excitability: tetany.
• In children: cramping, tingling around the
mouth or fingers.
• In infants: tremors, muscle twitches, brief
tonic-clonic seizures, CHF.
• Laryngospasm, seizures and cardiac
arrhythmias in severe situations.
• In children and adolescents, chronic
hypocalcemia more common, manif. By
spontaneous fractures.
• Lab tests: serum Ca, bone density study
• Rx: oral and/or IV Ca, Ca rich diet.
Acid Base Balance
• Normal arterial blood Ph: 7.35 – 7.43 (in
general)
• Acidosis <7.35: too much acid
• Alkalosis >7.45: too little acid
• pCo2 – reflects carbonic acid status: 40+-5
• HCO3 – reflects metabolic acid status: 24 +-4
Respiratory Acidosis
• Caused by decrease respiratory effort
• Build-up of CO2 in the blood
• pH decreases or normal: pCo2 increase.
• Symptoms manifested: confusion, lethargy,
increase ICP, coma, tachycardia, arrhythmias.
Management of Respiratory Acidosis
• Increase ventilator rate
• Give O2
• Intubate
• adm NaHCO3
Respiratory Alkalosis
• Caused by hyperventilation
• CO2 is being blown off
• pH increase: pCo2 decrease
• Symptoms: dizziness, confusion,
neuromuscular irritability, paresthesias in
extremities and circumoral, muscle cramping.
Management of Respiratory Alkalosis
• First determine if oxygenation is adequate, if
not, you don’t want to slow the RR.
• Determine the cause and correct it:
o Causes of hypervent: hypoxemia,
anxiety, pain, fever, ASA toxicity,
meningitis/encephalitis, Gram
negative sepsis, mechanical
overventilation.
Metabolic Acidosis
• Caused by loss of bicarbonate (HCO3)
• Therefore, is an increase of acids in the blood
• pH decreases or moving towards normal
• pCo2 decrease: HCO3 decrease
 • Symptoms: Kussmaul respirations = increase
rate and depth as compensation
(hyperventilation/acetone breath), confusion,
hypotension, tissue hypoxia, cardiac
arrhythmias, pulmonary edema.

Management of Metabolic Acidosis

• Identify and treat underlying cause
• In severe case may give IV NaHCO3 to increase
pH, or insulin/glucose
• Causes: ingestion of ASA, oliguria, lactic
acidosis (tissue hypoxia).
• Loss of HCO3: urine disease, diarrhea, Renal
failure.

Metabolic Alkalosis
• Caused by loss of H+ or HCO3 retention
• HCO3 increase with probable increase in pH,
increase pCO2.
• Symptoms: weak, dizzy, muscle cramps,
twitching, tremors, slow shallow resp.,
disorientation, seizures.

Management of Metabolic Alkalosis

• Correct underlying cause; facilitate renal
excretion of HCO3
• Admins NS, K+ if hypokalemic, replace loss of
fluids, monitor I and O and electrolytes.
• Causes: prolonged vomiting, antacids, loss of
NG fluids, hypokalemia form prolonged
diuretic use, multiple blood transfusion with
citrate.