CA 2 REVIEW: PEDIATRIC NURSING

pathologic pedia day 2

CARDIOVASCULAR DISORDER
2 types:

Congenital Disorders
Congenital Acquired Disorders

Congenital - it has something to do with the flow of blood

CLASSIFICATION OF CONGENITAL HEART DISORDERS

Acyanotic - usually doesn't show any signs of cyanosis.

(blood is coming from the left side of the heart going towards the right side of the heart).

Cyanotic - mostly blood coming from the right side of the heart gets mixed with the left side
of the heart that causes unoxygenated blood being mixed towards with the oxygenated
blood it will go towards the systemic circulation.

ACYANOTIC

the oxygenated blood will be going towards the right side of the heart it will mix in
unoxygenated blood.
there will be no signs of cyanosis because it will eventually go back to the lungs and the
rest will be oxygenated and then going towards the left side of the heart.
the only problem is that the right side of the patient will be filling up.

1. Increased Pulmonary Blood Flow

- Atrial septal defect (ASD) - Septic Wall between the atriums remained open. There is an
absence of the septum. this is congenitally acquired. It only allows the oxygenated blood
returning from the lungs to pass in to the right atrium.

ASSESSMENT:

Harsh systolic murmur in the 2nd or 3rd ICS/ pulmonic area (S2 split)
RA & RV enlargement by 2D ECHO
May develop CHF (Congestive Heart Failure)
Risk for atrial dysrhythmia

Notes: for diagnosing Atrial Septal Defect used the 2D ECHO. it is an ultrasound to see the
movement of blood in the heart.

SURGICAL MANAGEMENT:

Dacron Patch - a kind of implantable consumables in cardiac surgery by putting patch

between the right and left atrium. this is a more invasive procedure. It can also be used
through Cardiac Catheterization which is much lesser invasive.

- Ventricular Septal Defect (VSD) - the hole is in the septum between the right ventricle and
the left ventricle. The pressure is stronger on the left side of the heart the blood will be
passing through this problematic septum going from the right ventricle. (the problem is in the
ventricles)

ASSESSMENT:

Asymptomatic at birth
4–8 weeks old may demonstrate easy fatigue
Loud harsh pansystolic murmur at 3rd ot 4th ICS (intercostal spaces)
Palpable thrill

SURGICAL MANAGEMENT

PALLIATIVE - Pulmonary artery banding

> Placement of band around the main pulmonary artery to decrease pulmonary blood flow
may be done in infants with multiple muscular VSD.

Note: so that the lungs are not filled too much blood.

COMPLETE REPAIR

Small defects - purse string sutures

Large defects - Knilled Dacron patch be sewn over the opening. Cardio Pulmonary Bypass is
used for both procedures. This is done through open heart surgery.
- Patent Ductus Arteriosus (PDA) - It did not close the PDA of patient.

Note: After the patient has been born supposedly within 24 hours the ductus arteriosus
should have been closed. There should be spontaneous closure.

- The symptoms of the PDA is depends on the size of vessels and the age of the child.

- they may have no symptoms at birth

- usually hear a Machinery like murmur. (together with increased dysrhythmia and wild pulse
pressure.)

PROGNOSIS:

Spontaneous closure after infancy stage rarely occurs

Without treatment-life expectancy is short

MANAGEMNT:

Oral or IV indomethacin (prostaglandin inhibitor) to promote ductus closure

Interventional cardiac catheterization with Dacron coated stainless coil insertion by 6
mos to 1 year
Surgical Closure by Ductal ligation (tie or incision to close)

****Atrioventricular Canal - a type of congenital heart defect. A person born with

atrioventricular canal defect has a hole in the wall separating the heart's chambers and
problems with the heart valves. The condition may be partial, involving only the two upper
chambers, or complete, involving all four chambers.

Complete defect - The hole is big enough to provide an opening between all four chambers of
your heart. Instead of the normal two valves between the upper and lower chambers, there’s
just one valve. Its flap might not open and shut correctly.

Blue or gray skin color due to low oxygen levels

Difficulty breathing or rapid breathing
Excessive sweating
Fatigue
Irregular or rapid heartbeat
Lack of appetite
Poor weight gain
Swelling in the legs, ankles and feet
Wheezing
Partial Defect - The hole is only between the wall of the two upper chambers (most typical) or
the two lower chambers, not all four. There are two valves between the upper and lower
chambers, but one of them (usually the mitral valve between the upper left atrium and lower
left ventricle) doesn’t work correctly.

Fatigue and weakness

Nausea and lack of appetite
Persistent cough or wheezing
Rapid or irregular heartbeat, also called an arrhythmia
Reduced ability to exercise
Shortness of breath
Swelling in the legs, ankles and feet
Chest pressure or pain

Possible complications of atrioventricular canal defect include:

Enlargement of the heart. Increased blood flow through the heart forces it to work
harder than normal, causing it to become larger.
High blood pressure in the lungs. This condition is also called pulmonary hypertension. A
hole in the heart lets oxygen-rich blood mix with oxygen-poor blood. The lungs get too
much blood. Pressure builds up in the lungs.
Respiratory tract infections. A hole in the heart can lead to repeated lung infections.
Heart failure. If atrioventricular canal defect is not treated, the heart can't pump enough
blood to meet the body's needs.

1. Obstruction to blood flow from the ventricles

COARTATION OF AORTA - narrowing of aorta usually this happens in aorta going towards the
systemic circulation downwards.

> There is narrowing of the lumen of the aorta.

> This may be an Isolated Defect or Associated with other cardiac malformation.

ASSESSMENT:

Absent, weak or diminished femoral pulse

Measure BP in both arms and a leg to assess the pulse in both upper and lower
extremities.

MANAGEMENT:

Surgical Repair - only permanent treatment; usually deferred until 3 years of age by
interventional angiography.

> Subclavian Flop - type of surgery - they are going to cut the remaining flop and put it
over the narrowed area to enlarge the aorta

> Resection and End to end anastomosis - they are going to cut and cohere to repair
 Balloon Angioplasty - using cardiac catheterization. It could return. If the balloon
angioplasty dislocate or the ballooning disappear. It is sometimes temporary for the
child.

PULMONIC VALVE STENOSIS OR PULMONARY STENOSIS - Narrowing of the valve or the

pulmonary artery distal to the valve.

Note: sometimes the problem is not the valve but the artery itself.

ASSESSMENT:

Asymptomatic
Signs of mild right sided heart failure (one of the complications; Pulmonary Atresia or
Ventricular Septal Defect.)
Typical systolic ejection murmur can be heard at the upper left sternal border.

MANAGEMENT:

Balloon angioplasty to dilate the valve

Surgical treatment - Brock procedure (valvotomy) a procedure in which they replace the
pulmonary valve.

> it can be the chemical or the valves that are obtained through the cows. they are mostly
compatible with humans.

AORTIC VALVE STENOSIS OR AORTIC STENOSIS - Stricture of the aortic valve that prevent
blood from passing freely.

Note: the problem here is the aorta going towards the systemic circulation.

ASSESSMENT:

mild to moderate asymptomatic

typical murmur, a rough systolic sound heard loudest at the right 2nd ICS
thrill at suprasternal notch
(severe): faint pulse, hypotension, tachycardia, inability to suck for long period.

MANAGEMENT:

Beta blockers and calcium channel blockers to reduce cardiac hypertrophy.

Balloon valvuplasty - they are going to place balloon using cardiac catheterization or
changing of aortic valves it can be mechanical or tissue valves. (usually used valves
coming from the cows, but it cannot grow along with the child's growth, so they have to
do the procedure again)

CYANOTIC

- the unoxygenated blood will be mix with the oxygenated blood.

1. Decreased Pulmonary Blood Flow

- Tetralogy of Fallot - the most common congenital heart defect. There are four defects;

> V - VSD

> O - Overiding aorta

> P - Pulmo stenosis

> E - Enlarged RV wall or RV hypertrophy

Note (EXPLANATION) :
VSD

> it's either there is a hole between the right and left ventricle.

> the septum can be absent.

Overiding Aorta

> the aorta is going mediate between the two ventricles

Pulmo Stenosis

> narrowing of pulmonary artery

Enlarged RV wall or RV Hypertrophy

> the amount doubles and the blood from the left mixes. What will happen now is that the
right ventricle will work harder because it will work quickly, the pressure from the right
ventricle will now increase. when the pressure going towards the right ventricle increases, the
blood will go towards the left side of the heart. It will now mix and because of the aorta is in
the middle overriding, the unoxygenated blood going towards the systemic circulation will
enter.

ASSESSMENT:

Hypoxic / "tet" spells (a child suddenly becomes cyanotic)

Fainting
stunted growth, clubbed fingers and toes
squatting position (characteristics position to relieve dyspnea)
clubbing of fingers
polycythemia (red blood cells decrease)

MANAGEMENT:

Decrease hypoxic spells - minimize or avoid crying (less stress, do not fill the child too
much food, so they don't have difficulty of breathing, give food that easy to digest)
Place in knee-chest / squatting position

 O2 therapy
Morphine sulfate to reduce symptoms
Propanolol (Inderal) for vessel dilatation
"Blalock-Taussig shunt" - temporary or palliative surgical repair creating shunt between
aorta and pulmonary artery.

TRICUSPID ATRESIA

- Condition in which tricuspid valve did not form an opening between the right atrium and
right ventricle

- 2% of CHD

ASSESSMENT:

Profound cyanosis and dyspnea at birth

emergency management needed ASAP

MANAGEMENT:

IV infusion of prostaglandin ( so that it can continuously open the ductus arteriosis,

temporarily)
Surgery: Fontan procedure (Glenn shunt Baffle) making of venacave-to-pulmonary
artery shunt. (patch is removed from right atrium, small hole between right atrium and
right ventricle to patch the septum so that it cannot pass through)

1. Mixed Blood Flow

Transposition of great arteries

- a rare type of congenital heart defect.

- Aorta arises from the right ventricle; pulmonary artery arises from the left ventricle

- Degree of cyanosis depends on the abnormal connections.

ASSESSMENT:

Cyanotic at birth (most important clinical sign)

Develop polycythemia thus at risk of emboli and thromboses
May develop CHF or pulmonary vascular obstruction
INTERVENTIONS:

Require emergency medical treatment

Cardiac catheterization
Balloon atrial septostomy
Surgery: Arterial switch

Total anomalous pulmonary venous return (TAPVR)

- Condition in which all the pulmonary venous blood returns to the heart

- Absent spleen is often associated

ASSESSMENT:

Cyanosis
Severe respiratory distress (because the blood does not become oxygenated)

MANAGEMENT:

Prostaglandin IV to keep ductus arteriosus open

Surgical intervention ASAP
Surgery of choice: Atrial septal pull-through (to have a supply of oxygenated blood)

TRUNCUS ATERIOSUS

- Single vessel arising from the ventricles just above a large VSD

ASSESSMENT:

Retarded growth; enlarged liver and heart

Usually infants die within the 1st year

*****HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

- a birth defect that affects normal blood flow through the heart. As the baby develops during
pregnancy, the left side of the heart does not form correctly. Hypoplastic left heart syndrome
is one type of congenital heart defect

Symptoms

Babies born with hypoplastic left heart syndrome usually are seriously ill soon after
birth. Symptoms include:

Grayish-blue color of the lips and gums (cyanosis)

Rapid, difficult breathing
 Poor feeding
Cold hands and feet
Weak pulse
Being unusually drowsy or inactive (Lethargy)

Complications

With proper treatment, many babies with hypoplastic left heart syndrome survive. But
most have complications later, which may include:

Tiring easily during sports or other exercise

Heart rhythm problems (arrhythmias)
Fluid buildup in the lungs, abdomen, legs and feet (edema)
Not growing well
Developing blood clots that may lead to a pulmonary embolism or stroke
Developmental problems related to the brain and nervous system
Need for additional heart surgery or a heart transplant

Surgical Treatment:

- Norwood Procedure (performed shortly after birth)

- Bi-directional Glenn Operation (Performed about 6 months after the norwood)

- Fontan Operation (the third stage, usually performed about 18 to 36 months after the
Glenn.)

- Heart Transplant (Suitable donor heart for babies are often in short supply)

Diagnosis: Echocardiogram,  a test that uses sound waves to create a moving picture of the
heart.

CARDIOVASCULAR DEFECTS MANAGEMENT:

Monitor VS closely
Monitor respiratory status
Auscultate breath sounds for crackles, rhonchi or rales
If respiratory effort is increased, place child in reverse trendelenburg position
Administer humidified O2
Monitor for hypercyanotic spells:

- Place infant in knee-chest or squatting position

- Administer O2 via mask

- Administer morphine as ordered

- IVF as prescribed
Assess for signs of CHF (also signs of edema)
Assess peripheral pulses
Keep child stress-free as possible; allow maximal rest

CARDIAC SURGERY: POSTOP

 Monitor for signs of discomfort
Monitor for signs of sepsis (fever, lethargy, diaphoresis, altered LOC)
Monitor lines, tubes or catheters; remove promptly
Administer pain medications; note effectiveness
Encourage rest periods (early ambulation, it would help for early recovery)
Facilitate parent-child contact ASAP

CARDIAC SURGERY: HOME CARE

Omit activities in which child could fall for 2-4 weeks post surgery
Avoid crowds for 2 weeks after discharge
No added salt diet
Do not put creams, lotions or powders on incision site
Child may return to school 3rd week after discharge
No physical education for 2 months
Followup check up 2 weeks after discharge
Avoid immunizations; invasive procedure and dental visits for 2 months
Advise parents regarding importance of dental visit every 6 months

Notes: Endocarditis, one of the complications. At the same time their immune system
decreases.

ACQUIRED HEART DISEASE:

CONGESTIVE HEART FAILURE in Children

Impaired myocardial function

- Tachycardia, fatigue, weakness, restless, pale, cool extremeties, decreased BP,

decreased urine output

- Usually happens in left sided

Pulmonary Congestion

- Tachypnea, dyspnea, respiratory distress, exercise intolerance cyanosis, wheezes

- Usually happens in right sided

Systemic Venous Congestion

- Periphreral and periorbital edema, weight gain, ascites, hepatomegaly, neck vein
distention.

CHF Treatment:

Goals:

Improved Cardiac Function

- Digitalize - Digoxin

 - increased CO, decreased size and venous pressure, relieve edema

> Lanoxin (Pedia)

- more rapid in onset

- Oral / IV doses x 24 hours followed by maintenance dose (BID) to maintain blood levels
(Digitalizing Dose)

- ACE Inhibitors (Capoten / Enalapril)

> (-) normal function of R-A system in kidney

> Blocks conversion of AI to All (Vasodilator)

> (emergency) Captopril - can be given smaller doses

Note: R-A system >>> Renin-Angiotensin System or RAS

- It blocks the conversion of angiotensin 1 into angiotensin 2 there will be dilation of the
blood vessels. the patient's blood flow will be good.

Remove accumulated Fluid and Sodium

- Diuretics - mainstay of treatment to eliminate excess H2O and salt

- Bidirectional glenn
Keep hct and blood viscosity within acceptable limits (hydrate)
Monitor for anemia, Fe supplementation, blood transfusion
Respiratory infection or reduce pulmonary function can worsen hypoxemia

- Aggresive pulmonary hygiene

- CPT, antibiotics

- O2

RHEUMATIC FEVER

Autoimmune GABHS
group A beta-hemolytic Streptococcus (GABHS) is the most common bacterium
associated with pharyngitis in children.

JONES Criteria:

- In order to diagnosis rheumatic fever by doing jones criteria.

- To clinically diagnose Rheumatic Fever;

2 Major

E.g. If the patient being positive in ASO titer + 2 major sympstoms shows, it could now
diagnose the client to have rheumatic fever.

1 Major and 2 Minor

E.g. If the patient has no 2 major symptoms but it does have 1 major and 2 minor symptoms +
a positive ASO titer, it could now declare the patient to have rheumatic fever.

MAJOR SYMPTOMS: that need to look out for

S - Subcutaneous nodules (kulani)

P - Poly arthritis (mostly in the joints, the swelling of the joints shifts)
E - erythema marginatum (skin patches)
C - Carditis (mitral) (swelling in cardiac muscles)
C - Chorea (involuntary movements)

MINOR SYMPTOMS:

Fever
Arthralgia (joint pains)

DIAGNOSTIC RESULT:

ASO titer - ASO titer means Antistreptolysin O (ASO) a blood test to measure antibodies
against streptolysin O, a substance produced by group A streptococcus bacteria.
ESR - erythrocyte sedimentation rate (ESR), is a blood test that can reveal inflammatory
activity in your body.

NURSING MANAGEMENT:

P - Pain control
A - Antibiotics
R - Rest
A - Aspirin
F - Follow up
I - Inform about respiratory infection

KAWASAKI DISEASE

Mucocutaneous LymphNodes (LN) syndrome

Acute systemic vasculitis
< 5 y/o (Peak: toddlers) most commonly seen
Etiology: unknown
Most serious complication (if not treated immediately)
Coronary artery aneurysm

SYMPTOMS:

C - Conjunctivitis (Pink eye)

 R - Rash
A - Arthritis (Joint pain)
S - Strawberry tongue
H - Hands (peeling skin)

Landmark: The Px is irritable, fever, and the CRASH symptom.

No specific diagnostic test
IRRITABILITY - hallmark of kawasaki disease
Persists in 2 weeks

INTERVENTIONS:

Monitor temp. frequently

Assess heart sounds and rhythm
Assess for edema (extremeties), redness, desquamation
Monitor mucous membrane for inflammation
Weigh daily
Administer IV immune globulin (to increase the immune system and given only once to
the patient)
Instruct parents in administration of prescribed meds (Aspirin)

Notes: Initially the px had fever and it will die down after a week or 2 weeks. kawasaki disease
is a long treatment. It could be 1 month to 2 months. If the px has given IV IG, usually the
fever is gone, but if there is still a fever, another dose of IV IG will be given to the px.

GENITOURINARY DISORDER

GLOMERULONEPHRITIS

- which is another complication of pharyngitis or tonsillitis.

- especially if its post streptococcal glomerulonephritis

- it could be ACUTE or AFTERMATH of streptococcal infections.

- There is already the Inflammation and Infection of the nephrons

- Glomerulus and the Nephrons of the kidney

Destruction
Inflammation
Sclerosis

CAUSES:

B - Boys ( commonly seen )

A - Autoimmune
G - GABHS
 U - Upper respiratory infection

ACUTE:

-Once there is already this infection it will began in:

Filtration decrease

- decreasing oxygen supply resulting in poor function of the kidneys. Filtration rate
decreased. >
Na retention

- Sodium retention may lead to increase BP >>

Increase BP, Heart fx, renal fx

- Hypertension, it can also cause cardiac output problems. Lessen blood supply
oxygen. The heart will work hard to increase cardiac output. If there is Na retention it
can cause to Water retention.

SIGNS AND SYMPTOMS:

D - Decreased UO (oliguric/oliguria)
A - Anorexia
T - Tea colored urine (hematuria)
E - Edema

L - Lethargy
I - Irritability
P - Pale

MANAGEMENT:

V - VS Monitor (especially the BP)

I - Intake and Output monitor (specifically the output)
D- Daily weight
U - Urine Characteristics
R - Rest

DIET :

- low salt and low fat diet

- restriction of fluids

NEPHROTIC SYNDROME

- no infection, its more on autoimmune.

- which may lead to severe loss or Abnormal loss of protein in the body.

- Protenuria

- Edema

- Hypo-albuminemia

SYMPTOMS:

S - Shiny Skin (cause of generalized edema)

A - Anasarca
W - Weight Gain

D - Decrease UO
I - Increase BP (hypertension due to increase salt)
D - Dark Urine

UNCOMPLICATED CASES:

- Regular diet; no added salt diet

(+) HYPERTENSION OR EDEMA:

- Moderate Na restriction

AZOTEMIA (Increased BUN)

- Protein free diet

GOAL OF TREATMENT:

Promote Diuretics (decrease of excess water)

Adequate Nutrition
Prevent Infection (prednisolone or corticosteroids)

LAB & DIAGNOSIS

BUN (Always check if this BUN increase)

CREA (check for kidney function)
URINE DIP STICK (check for protenuria, hematuria)
Serum CHOLESTEROL (increase lipids)

MEDICATIONS: Main Treatment

D- Diuretics
O - Oral restriction
C - Corticosteroids
No antibiotics unless the px has infection.

NURSING MANAGEMENT:

Antibiotics
Na Restriction
VS, I & O
Fluid Restriction
Weigh Daily
 HEMATOLOGIC DISORDERS

IRON DEFICIENCY ANEMIA

Iron stores are depleted, resulting in a decreased supply of iron for the manufacture of
hemoglobing in RBCs.
Results from blood loss, increased metabolic demands, syndromes of GI malabsorption,
dietary inadequacy

ASSESSMENT:

Pallor
Weakness and fatigue
Irritability

MANAGEMENT:

Increase oral intake of iron

Diet (high in iron)
Iron supplements as prescribed
Liquid iron prep stains
Side effects of iron therapy (side effects; constipation, black stools, taking iron on empty
stomach can lead to stomach ache)

APLASTIC ANEMIA

A deficiency of circulating erythrocytes resulting from the arrested development of RBCs

within the blood marrow
Causes: Exposure to toxic agents (e.g. household chemicals), viruses, infection,
autoimmune disorders, allergic states
Definitive diagnosis: BMA or Bone Marrow Aspiration

ASSESSMENT:

Pancytopenia (decrease RBC, WBC, platelet count)

Petechiae, purpura, bleeding, pallor, weakness, tachycardia, and fatigue

MANAGEMENT:

Blood transfusion
Splenectomy
Corticosteroids (for the inflammation)
Immunosupressive therapy
 Bone marrow transplantation (if severe aplastic anemia)
Medic-alert bracelet

Note: People with aplastic anemia are prone for bleeding because of their low platelet count.

SICKLE CELL DISEASE

Hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin (HgbS)

HgbS - Sensitive to changes in oxygen content of RBC
Sickling response - reversible under adequate oxygenation
Sickle cell crises - vaso-occlusive crisis, splenic sequestration, plastic crisis. (painful
because of the blockage)

ASSESSMENT:

Vaso-Occlusive Crisis

- Most common type

- Caused by blood stasis with dumping of cells in microcirculation, ischemia and

infarction

- Fever, pain and tissue engorgement

Note: Obstruction causing less oxygen...

Splenic Sequestration

- Pooling of blood in the spleen (swelling of spleen)

- Profound anemia, hypovolemia and shock (hypovolemic shock)

Aplastic Crisis

- Causes: Decrease production and Increase destruction of RBCs, viral infection,

depletion of folic acid

- Anemia and pallorc

MANAGEMENT:

O2 and BT
Analgesics
Adequate hydration (oral fluids / IVF)
Comfortable position (extension of extremities no more than 30 degrees)
Avoid strain on painful joints
High calorie, high protein diet with folic acid supplementation
Antibiotics
Monitor signs of increasing anemia and shock

BETA THALASSEMIA MAJOR

 Also called Cooley's anemia
Autosomal recessive disorder (genetic)
Reduced production of one of the globin chains in the synthesis of Hgb
Mediterranean descent - Highest incidence

ASSESSMENT:

severe anemia
pallor
failure to thrive
hepatosplenomegaly (enlargement of liver and spleen)
microcytic, hypochromic RBCs

MANAGEMENT:

Folic acid administration (Vit. B9)

BT or Blood Transfusion
Monitor iron overload
Chelation therapy with deferoxamine (for iron overload)
Genetic counseling

LEUKEMIA

Most frequent type of childhood cancer

Peak: 3-5 years
Proliferation of abnormal WBCs that do not mature beyond the blast phase
Blast cells - infiltrate other organs (liver,spleen, lymph tissue)
Causes: environmental (virus), familial/genetic, host factors

Acute Lymphocytic Leukemia (ALL)

80 - 85%
Acute
75 % chance of surviving

Acute Myelogenous Leukemia (AML)

Includes granulocytic and monocytic types

60 - 80% will obtain remission
30-40% cure rate

ASSESSMENT:

Anemia, weakness, pallor, dyspnea

Bleeding, petechiae, spontaneous bleeding, ecchymoses (bruise)
 Infection, fever, malaise
Enlarged lymph node
Enlarge spleen and liver

Diagnostics: Blood Studies, BMA or Bone Marrow Aspirations

Note: the other anemia will be ruled out first before doing the BMA.

STAGES OF TREATMENT:

1. Induction

- Goal: To remove bulk of tumor

- Methods: Surgery, radiotherapy, chemotherapy, BM transplant

- Effects: Often the most intensive phase

- Side effects: potentially life threatening

2. Consolidation

- Goal: To eliminate any remaining malignant cells

- Methods: Chemotherapy / radiotherapy

- Side effects: will still be evident (hair loss, darken skin, skinny, weakness)

3. Maintenance

- Goal: To keep the child disease free

- Methods: Chemotherapy

- Phase may last several years

4. Observation

- Goal: To monitor the child at intervals for evidence of recurrent disease and
complications of treatment

- Methods: Treatment is complete

- Child may continue in his stage indefinitely

LATE EFFECTS OF TREATMENT:

Impaired growth and development and CNS damage

Impaired pubertal development
Development of secondary malignancy
Psychologic problems related to living with a life-threatening disease and complex
treatment regimen

NURSING INTERVENTIONS:

Help child cope with intrusive procedures (especially the BMA upon diagnosis)
Provide information (developmental level and emotional readiness)
Explain procedures, purpose, and how it will feel.
Allow child to manipulate equipment
 Allow child some control in situations
Positioning, selecting injection site
Support child and parents
Acknowledge feelings and allow them to express it
Frequent clinical conference and always tell the truth
Refer to support groups
Support child during painful procedures
Administer sedation as ordered.
Minimize side effects of treatment
Watch out for the side effects :

- Skin breakdown

- Bone marrow suppression

- Nausea and vomiting

- Alopecia

- Nutrition deficits

- Developmental delay

HEMOPHILLIA

X-linked recessive trait (Genetic type disease)

Hemophillia A - Deficiency of factor VIII
Hemophillia B - Deficiency of factor IX
Males inherit hemophillia from their mothers while females inherit the carrier status from
their fathers

ASSESSMENT:

Prolonged bleeding after minor injury

At birth after cutting cord
Following circumcision
Following IM Immunization
Increased bruising as child learns to crawl and walk
Abnormal bleeding in response to trauma
Joint bleeding (hemarthrosis) - pain, tenderness, swelling, limited ROM
Tendency to bruise easily
Prolonged PTT (partial thromboplastin time)
Normal BP, PT or Prothrombin time, Platelet count

MANAGEMENT:

Administration of Fact VIII concentrate/cryoprecipitate

Thaw slowly; gently rotate bottle; infuse immediately (deteriorates at room
temperature)
Monitor for bleeding and joint pains (immobilize extremity)
 Assess neurologic status (risk of intracranial hemorrhage)
Monitor urine (hematuria)
Control bleeding (immobilization, elevation, application of ice; apply pressure for 15
minutes for superficial bleeding)
Instruct parents (activities; avoid aggresive games)
Avoid contact sports

IDIOPATHIC THROMBOCYTOPENIC PURPURA

Increased destruction of platelets with resultant platelet count less than 100,00/mm3
Petechiae and ecchymoses of skin
Exact mechanicsm unknown
Often preceeded by a viral illness
Spleen not enlarged

ASSESSMENT:

Petechiae
Ecchymosis
Blood in body secretions, mucus membrane bleeding, nosebleeds

DIAGNOSTIC TESTS: Platelet count decreases, low hematocrit

MANAGEMENT:

Steroids and immunosuppressants

Platelet transfusion
Surgery: Splenectomy
Control Bleeding
Prevent bruising
Protect from infection
Measure normal circumference of extremeties for baseline