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Conditions in Pediatric Nursing

Submitted by:

Mercado, Colleen P.

BSN/NUR04A/2ND YEAR

In Partial Fulfillment of the Requirements for NCM 102 LEC

Submitted to:

Arnel Jay Sali, RM,RN, MSN

February 08, 2012

Conditions in Pediatric Conditions in Pediatric NursingNursing

CARDIOVASCULAR SYSTEM DISORDER

ANATOMY AND PHYSIOLOGY OF HEART

Structure and function of the human heart

The size of a fist, the human heart is made of a unique muscle tissue. Protected beneath the rib cage, it pumps life throughout the body, driven by electrical impulses controlled by the brain and hormones. Understanding the heart's basic anatomy and function is important to understanding diseases that involve not only the heart, but also the lungs, kidneys, blood, and brain.

Anatomical Terminology

The heart is a unique organ, and as such it is discussed using unique terminology. It also includes a number of parts that may be recognized from other anatomy.

Cardiac: dealing with the heart Myocardium: the heart tissue, it is made up of cells called myocytes Endocardium: interior heart muscle Pericardium: around the heart, such as the pericardial sac, a membrane that encloses the heart Coronary arteries: blood vessels serving the heart muscle Septum: a wall of tissue dividing the left and right sides of the heart Valve: a one-way opening that closes to prevent blood backflow, it is present at the exits of the

heart and between atria and ventricles Atrium: an upper heart chamber (plural, atria), they receive blood from the circulation (systemic

or pulmonary) Ventricle: a lower heart chamber, they force blood out into the circulation (systemic or

pulmonary) Apex: bottom of the heart Artery: blood vessel carrying blood away from the heart Vein: blood vessel carrying blood to the heart

Heart Structure

The heart is made of four chambers, and blood flows through them in order: right atria, right ventricle, left atria, left ventricle. Between the right and left sides, though, the blood goes through the lungs to exchange carbon dioxide it collected in the body for oxygen it can carry to the body.

Blood enters the right atria from the venous system, and exits the left ventricle through the aorta into the arterial system. Blood flows from the atrium to the ventricle during a heart beat based on the

synchronous contraction of the heart muscle (see below). Between each atrium and ventricle, there is a valve. Valves are also found where the heart and aorta, and heart and pulmonary artery meet.

Factors of Congenital Heart Disorder:

1) Familial- family history of heart disease that 30% the fetus will get this disease.

2) Mother is exposed to rubella virus.- if the mother is exposed to rubella virus, the fetus is at risk of getting these following

conditions:- CNS damage- Congenital cataract- Congenital heart disease

3) Mother is < 18 years old or > 40 years old.- < 18 years old is in high risk because the uterus is immature and in > 40 years old there

will be degeneration of uterus and imbalances of hormones that will cause lost of elasticity. 4) Failure of the heart structure to progress

CLASSIFICATIONS:1) Acyanotic

- characterized by the absence of cyanosis. - It involves heart or circulatory anomalies involve either a stricture to the flow of blood

or shunt that move from arterial to venous system (oxygenated blood to deoxygenated blood).- It tends to be left to right side heart shunts, whereas cyanotic heart disease is right to

left shunt which lowers oxygen saturation level.-There are different types of acyanotic heart diseases, such as Patent Ductus Arteriosus,

Ventricular Septal Defects, Arterial Septal Defects, Coarctation of the Aorta, Aortic Stenosis.

2) Cyanotic

-is a heart defect, present at birth (congenital) that results in low blood oxygen levels where it causes a bluish discoloration of the skin and mucous membranes

- Occurs when blood is shunted from the venous to arterial as result of abnormal communication between two systems.(deoxygenated blood to oxygenated blood)

- Right-to-left cardiac shunt-There are different types of cyanotic heart diseases, such as Transposition of the Great

Arteries, and Tetralogy of Fallot.

DIFFERENT TYPES OF ACYANOTIC HEART DEFECTS

VENTRICULAR SEPTAL DEFECT (VSD)

- It is the opening between the two ventricles

Pathophysiology:

- Ventricular septal defect (VSD) is the most common type congenital cardiac disorder, accounts for 30% of all instances of congenital heart disease or about 2 in every 1000 live births. With this defect, an opening is present in the septum between two ventricles. Because pressure in the left ventricle is greater than that in the right ventricle, blood shunts from left to right across septum. This impairs the effort of the heart because blood that should go into the aorta and to the body is shunted back into pulmonary circulation, resulting right ventricular hypertrophy and increased pressure in the pulmonary artery.

Signs and Symptoms

- Systolic murmur at the lower boarder of the sternum which is the outstanding sign of VSD

- There is an elevation of oxygen saturation at the right side of the heart

- Hypertrophy at the right side of the heart

Diagnostic Procedure

Cardiac Catheterization

-is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the

heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.

Nursing Intervention:

NPO 6 – 8 hours before the procedure CBR – Complete Bed rest without bathroom privileges Protect the site Assess for complication

Chest X-ray

- To see the heart's size

- Diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. With a VSD, the heart may be enlarged because the right ventricle handles larger amounts of blood flow than normal. Also, there may be changes that take place in the lungs due to extra blood flow that can be seen on an x-ray.

Echocardiogram

- A test that uses sound waves to create a moving picture of the heart

-a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the septal opening, and determine how large the opening is, as well as much blood is passing through it.

Electrocardiogram (ECG or EKG)

-a test that records the electrical activity of the heart

-a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the septal opening, and determine how large the opening is, as well as much blood is passing through it.

Magnetic resonance imagining (MRI) or computed tomography (CT)

-scan to better see the restricted area and surrounding blood vessels

Management

1) Long - term antibiotic- To prevent sub – acute bacterial endocarditis which is the inflammation of

endcardium (inner lining of the heart).

2) Open heart surgery – a surgery to fix large VSD. This usually scheduled before 2 years of age to prevent pulmonary artery hypertension.

ATRIAL SEPTAL DEFECT (ASD)

- Failure of the foramen ovale to close.

- Normally foramen ovale should close within 24 hour after birth up 3 months going 1 year.

Pathophysiology:

In fetal circulation, normally foramen ovale or an opening between the two atria (the upper chambers of the heart) allows blood to bypass the lungs. This opening usually closes around the time the baby is born.

If the ASD is persistent, blood continues to flow from the left to the right atria. This is called a shunt. If too much blood moves to the right side of the heart, pressures in the lungs build up. The shunt can be reversed so that blood flows from right to left. Small atrial septal defects often cause very few problems and may be found much later in life. Many problems can occur if the shunt is large, however. In advanced and severe cases with large shunts the increased pressure on the right side of the heart would result in reversal of blood flow (now from right to left). This usually results in significant shortness of breath.

BEST POSITION for patient with ASD: Right side- lying position because of increasing pressure at left side of the heart. Normally the pressure at right of the heart but when the baby is outside there is shifting of pressure in left side.

Signs and Symptoms:

- Systolic murmur at upper boarder of sternum which is the outstanding sign for ASD

- There is an elevation of oxygen saturation at the right side of the heart

- Hypertrophy at the right side of the heart

Diagnostic Procedure:

Cardiac catherization – in order determine location of the defect

Management:

Open heart surgery – to close the defect which is done electively 1 and 3 years of age. Closure is important because without it, a child is at risk for infectious endocarditis and even heart failure. It is particularly important ASD be repaired in girls, because they can cause emboli during pregnancy.

PATENT DUCTUS ARTERIOSUS

- Failure of the Ductus Arteriosus to close which normally, the closes within 24 hours after birth up to 1 month

- The ductus arteriosus is a blood vessel that allows blood to go around the baby's lungs before birth. Soon after the infant is born and the lungs fill with air, the ductus arteriosus is no longer needed. It usually closes in a couple of days after birth.

- PDA leads to abnormal blood flow between the aorta and pulmonary artery, two major blood vessels that carry blood from the heart.

Pathophysiology:

The Ductus Arteriosus is an accessory to fetal structure that connects the pulmonary artery to the aorta. If it fails to close at birth (closure begins at first breath, and usually complete to between 7 – 14 days of age, athough full closure may not occur until 3 months), blood will shunt from aorta (oxygenated blood) to pulmonary artery (deoxygenated blood) because of increased pressure in the aorta.The shunted

blood returns to left atrium of the heart, passes to the left ventricle, out of the aorta, and shunts back to pulmonary artery. This causes increased pressure in the pulmonary circulation from extra shunted blood: this leads to right ventricular hypertrophy and ineffective heart action.

Signs and symptoms:

- Continuous machinery murmur which is the outstanding sign of PDA

- Prominent radial pulse

- Hypertrophy at the right ventricle

Drug of Choice:

INDOMETHACIN

Brand name: INDOCIN

Action: Prostaglandin inhibitor that facilitates closure of the ductus arteriusus.

Indication/Routes/Dosage: Alert! May give up to 3 doses at 12 to 24 hr- interval

IV: NEONATES: Initially, 0.2 mg/kg

>7 year old: 0.25 mg/kg for 2nd and 3rd dosage.

2-7 years old: 0.2 mg/kg for 2nd and 3rd dosage.

48 hours old: 0.1 mg/kg for 2nd and 3rd dosage.

Contraindication: Pregnancy Category B( D in third trimester)

Side Effects: Bleeding Disturbances

Adverse Effect: Acidosis,headache,dizziness, nausea,gastrointestinal bleeding, constipation,rash apnea, bradycardia, alkalosis occur rarely.

Nursing Implication:

Surgery:

Ligation of PDA - Is a corrective surgical procedure for children whose Ductus Arteriosus is still open after birth. This defect can lead to congestive heart failure in children who are older and adults if not cured at the earliest.

The procedure:

The pediatric cardiologist makes a small incision on the left side of the chest through the ribs with the patient under general anesthesia. The surgeon either ties it off or has it divided and cut upon once he reaches the ductus arteriosus, the wound is closed and the patient is moved once the patent ductus arterious litigateion is successfully complete, the patient is eventually moved to the intensive care unit. The patient’s progress is monitored for several days. Similarly, the child is kept sedated if he/she is on a respirator. Fluids and medication are provided through the IV lines and nasogastric tubes during the recovery period in the ICU. To drain excess fluid, blood and air from the chest, Chest tubes may be used for this purpose. Urine is drained from the body with the help of a catheter in the bladder.

COARCTATION OF AORTA (COA)

- narrowing/ stenosis of aorta

Pathophysiology:

Coarctation of the aorta, a narrowing of the lumen of the aorta due to a constricting band, accounts for 6 % of congenital heart disease. It occurs more frequently in boys than girls and is leading cause of congestive heart in the first few months of life. There are two locations which commonly occur. The first location is preductal which is the constriction occurs between subclavian artery and the ductus arteriosus. The second location is postductal, constriction is distal to the ductus arteriosus.

Because it is difficult for the blood to pass through the narrowed lumen of the aorta, blood pressure increases proximal to the coarctation and decreases distal to it. This results in increased pressure in the heart and upper portions of the body as pressure of subclivian artery increases.

Sign and Symptoms:

- Absents of Femoral pulse

Nursing Intervention:

Monitor the BP of four extremitiesABNORMALITIES:

Blood pressure in the upper extremities is high which there will be pounding pulseThe Blood pressure in the lower extremities is low which there will be weak thread pulse.

Management:

Angiography (balloon catheter)

- is surgery where the narrowed portion of aorta is removed and the new end of the aorta is anastomosed.

DIFFERENT TYPES OF CYANOTIC HEART DEFECTS

TRANSPOSITION OF THE GREAT ARTERIES (TOGA)

- Aorta rises from the right ventricle instead of the left, and pulmonary artery arise from the left instead of the right.

Pathophysiology:

- In this disorder, blood enters the heart from vena cava to the right atrium, then flows to the right ventricle, and goes out into the aorta to the body completely deoxygenated: it returns again to the vena cava. A secondary source of blood enters the heart from pulmonary veins, goes to left atrium, left ventricle and out the pulmonary artery to the lungs to be oxygenated, and returns to left atrium, a second closed circulatory systems. This severe a defect is incompatible with life. In most instances, atrial and ventricular septal defects occur in connection with this transposition, making the entire heart one mixed circulatory system.

Signs and Symptoms

1) CyanosisNormal Color: Pinkish - good oxygenAbnormal Color: Bluish - indicates decrease O2

- indicates Cyanotic Heart Defect- indicates Respiratory distress syndrome

1st Cry of BabyNormal: Strong, vigorous, rusty cryAbnormal: Cat – like cru

Cri-du-chat syndrome – deficiency of chromosome no. 5 High pitch shall cry because of increase intracranial pressure

2) Polycythermia Vera- increase no. of RBC due to consistent a decrease of oxygen- Plethora (redness or ruddy discoloration of skin due to polycythermia vera)

3) Cardiomegaly

TETRALOGY OF FALLOT

- has four anomalies present

4 MAIN CONDITIONS

1) Pulmonary artery stenosis2) Ventricular Septal Defect3) Overriding of aorta4) Hypertrophy of right

ventricular

Pathophysiology:

Tetralogy of fallot has four anomalies are present: pulmonary artery stenosis, ventricular septal defect, overriding of aorta and, hypertrophy of right ventricular. Because of the pulmonary stenosis, pressure builds up in the right side of the heart. Blood shunts from this area of increased pressure into the left ventricle and the overriding of aorta. The extra effort involved to force blood through stenosed pulmonary artery causes the fourth deformity which is hypertrophy of right ventricular.

Signs and Symptoms:

High level of cyanosis Polycythermia vera Severe dyspnea Tet – spell or Blue spells

- bluish discoloration due to short episodes of hypoxia. Mild mental Retardation due to decrease oxygen in the brain Syncyse Growth Retardation Clubbing of fingers due decrease perfusion X ray film that shows boot-shape-heart

Management:

Position patient in squatting or knee – chest position because it promotes lung expansion, inhibits venous return and relief DOB

Administer oxygen as ordered

Boot-shaped heart

No valsalva maneuver because it can cause vasoconstriction that will increase stimulation of venous nerve which can cause bradycardia that will lead to increase workload of the heart that causes DOB then that will lead to cyanosis.

Increase fiber in diet Administer propanol that lowers vasospasm.

SURGERY: Blalock Tausig procedure(BLT): - is an anastomosis of pulmonary artery and sublingual artery which

does increase peripheral blood flow.- Patient with success or post Blalock Tausig procedure has no sign of

cyanosis

ACQUIRED HEART DISEASE

RHEUMATIC HEART FEVER

- 3 – 5 x of sore throat

- Systemic inflammation disease that affects heart, joint and subcutaneous tissues.

- Causative microorganism: Group A Beta Hemolytic Streptococcus.

Complication: Rheumatic heart disease which is the destruction of bicuspid valve.

Diagnostic Procedure:

Jones Criteria – is a tool to diagnosed rheumatic heart fever and rheumatic heart disease.

MAJOR SYMPTOMS MINOR SYMPTOMS

- Polyarthritis - arthalgia(simple joint pain)

- Subcutaneous nodules - low grade fever

- carditis - increase ASOtiter or antistreplysin O

- erythema margina which is lab test to confirm if patient

- chorea (purposeless movement of manifest tonsillitis and Rhematic of body and hand) aka: st. vitous dance heart fever

- Increase Erythrocytes Sedimentation

Rate due to systemic inflammation

- Increase C- reactive protein

Management

Increase oral fluid intake Complete Bed rest Throat swab exam to culture and sensitivity Administer antibiotics like ampicillin, if allergic to ampicillin administer erythromycin Oral ibuprofen is prescribed to reduce inflammation and joint pain Corticosteroids may administered to reduce inflammation in children who are not responding to

ibuprofen therapy alone Phenobarbital and diazepam are both effective to reduce chorea . ASA therapy or acetyl salicylic acid or aspirin

5 actions of Aspirin- Analgesic- Anti-pyretic- Anti-inflammatory- Anti-platelet- Anti-thrombotic

Aspirin are given after meal because it is gastrointestinal irritant Over dosage of aspirin causes tinnitus

MULTI-ORGAN SYSTEM DISORDER

KAWASAKI DISEASE (Mucocutaneous lympnode syndrome)

- Kawasaki Disease is a febrile, multisystem disorder that occurs almost exclusively in children before age of puberty. It has replaced rheumatic heart fever as most likely cause of acquired disease in children. The peak of incidence is in boys under 4 years of age. Vasculitis is principal and life-threatening finding because it can lead to aneurysm and myocardial infarction. The cause of Kawasaki disease is unknown, but it apparently develops in genetically predisposed individuals after-yet –unidentified infectious agent. After the infection (perhaps an upper respiratory infection), altered immune function occurs. An increase in antibody production creates immune (antibody-antigen) complexes that bind to vascular endothelium and cause inflammation. The inflammation of blood vessels leads to aneurysm, platelet accumulation, and formation of thrombi or obstruction in the heart and blood vessels.

Signs and symptoms:

1) Fever- constant2) Bilateral conjunctivitis3) Lymphadenopathy > 1.5 in diameter4) Polymorphous rash5) Desquation of the skin usually palms and soles6) Vasculitis(inflammation of blood vessels)7) Strawberry tounge is the most outstanding sign of Kawasaki disease

Management:

Reverse Isolation because patient is immunocompromise Aspirin Therapy DRUG OF CHOICE: Immuno – Globulin

- it has anti-inflammatory

ACTION: Lowers risk of CAD

RESPIRATORY SYSTEM DISORDER

Normal RR of Newborn:

32- 60 bpm Shallow Irregular respiration

Periods of apnea- < 15 seconds-> 15 – Respiratory Distress Syndrome

ASTHMA

- is an immediate hypersensitivitivity (type I) response.

Types of Asthma

Intrinsic Asthma – internal stressors

- Stress, type - A personality, emotion (exaggerated emotion)

Extrinsic Asthma – external factors

- Environmental factors, allergen (dust), pollens, perfume, drugs (antibiotic), food( seafood, chocolate), change of weather

Signs and Symptom:

- Hacky, non-productive cough

- Shortness of Breaths / Difficulty of Breathing

- Prolong expiratory period

- Expiratory wheezes which the most outstanding sign of asthma

- Itchiness at the base of neck

Drugs:

1) Albuterol2) Salbuterol3) Aminophylline4) Lagundi or vitex negundo

Status asthmaticus- Recurrent episodes of asthma attackDOC: epinephrine

Absence of wheezes: Should report to the physician because it is sign of airway obstruction

Management: Position the client head elevated/semi fowler/ sitting position Administer oxygen Monitor the Vital sign = RR Monitor skin color

RESPIRATORY DISTRESS SYNDROME

- Deficiency of surfactant which is secreted and produced by Type II alveoli cells

Surfactant is important for:

1) Decrease surface tension2) Promotes lung expansion3) Prevents lung collapse

Diagnostic Procedure Amniocentesis

- a test that indicates lung maturity

-L/s ratio or lecithin/sphingomyelin ratio: Normal Value: 2:1

Shake’s test

-After shaking a mixture of amniotic fluid and ethanol, the stability of the bubbles formed is estimated qualitatively. It has 100% positive predictive value.

Signs and symptoms:

1) Inspiratory grunting which is the outstanding sign of RDS2) Tachypnea3) Nasal flaring4) Sternal retraction

Management:

Position the patient in head elevated position Proper suctioning

- 5 – 10 seconds or 5 – 15 seconds Corticosteroid

BETHAMETHASONE

- prevents RDS- promotes surfactant maturation

Bronchodilators – to give artificial airway

LARYNGOTRACHEABRONCHITIS (LTB)

- Inflammation of larynges, trachea and bronchus

- Viral infection

CAUSATIVE AGENTS: Parainfluenza virus 1, 2, 3

RSV(respiratory Sincytial Virus)

Signs/Symptoms

1) Inspiratory stridor is the outstanding sign of LTB

- Rooster-like sound due to laryngoconstriction

2) Barking /croupy cough due to inflammation of larynges

- because of the inflammation of larynges

- “seal-barking”3) Labored respiration4) Respiratory Acidosis

Diagnostic Test:

Throat swab – to determine culture and sensitivity

Management:

Position in high fowlers position Administered oxygen as ordered Bronchodilator Corticosteroids

Predisone- anti-inflammatory

Betamethasone

HEMATOLOGICAL SYSTEM DISORDER

HEMOPHILIA

- Genetic acquired

- Deficiency / lacking of clotting factor

- Pattern of inheritance:

X- linlad recessive gene

- Usually carrier: mother

- Usually affected: son

- Rare 1%: father to be carrier and daughter to be affected

3 TYPES

1) Hemophilia A (80%)- most common- other name: “Classic Hemophilia”- lacking of clotting factor no.8

2) Hemophilia B (18%)- other name:” Christmas Disease”- deficiency of clotting no. 9

3) Hemophilia c (2%)- rarest- deficiency of clotting no. 11

Signs/Symptoms:

1) Hemarthroses which is the outstanding sign of hemophilia

- Bleeding of the joints

- Repeated damage of syuvial membrane

2) Hematuria3) Melena – (black tarry stool)4) Epistaxis5) Hemmorhage

Diagnostic Procedure:Platelet count

Normal value: 150,000 – 400,000 cubic millimeter

Prothombin time

Partial Thromboplastin time (PTT)

- Normal PTT of infants: < 90 secs

- Abnormal: prolonged

Management:

Provide safety precautionary measures Control bleeding

- closed supervision- avoid directed /contact sports like ball games, fencing, tag of war & etc.

FallsBEFORE NOW

- Splint affected area - Rest- immobilized - Ice - elevate at level of the heart - compress

- elevate Blood transfusion No aspirin

SICKLE CELL ANEMIA

- Presence of crescent-shaped RBC

Normal shape of RBC should be biconcave disk

- It is an autosomal recessive gene disorder which both parent are the carrier.

- It’s RBC is replaced by Hemoglobin S

Normal RBC should be replace hemoglobin A

- Has a short lifespan of 10- 12 days; maximum 20 days

Normal RBC should have long lifespan of 120 days.

Pathphysiology:

The erythrocytes become characteristically crescent - shaped (sickled) when they are submitted to low oxygen, a low blood ph (acidosis) or increased viscosity such as dehydration or hypoxia. When RBCs sickle, they cannot move freely through vessels. Stasis and further sickling or sickle-cell crisis occur. Blood flow halts and tissue to distal to the blockage becomes ischemic, resulting to acute pain and cell destruction.

Signs and symptoms:

- decrease of oxygen capacity of blood.

- Short lifespan of RBC’s which 10-12 day and maximum is 20 days.

- increase viscosity of blood

- vaso-occlussive cris which is the oustandibg sign of SCA

-Organ damage

- Pain

Other Complications:

1) Liver Damage -which there will destruction of hepatocytes

2) Eyes-Retinal Detachments where there is decrease of curded light vision

3) Genital

- Priapism(painful continous erection) which can lead sterility4) Heart

- Myocardial infarction 5) Extremities

-leg ulceration6) Kidney

- renal failure

Management:

Hydration Oxygenation – to prevent further sickling Pain management

Narcotic Analgesia: Morphin which target severe pain

Blood transfusion – Pack RBC’s for 4 hours Encourage patient to increase iron intake and folic acid to promote maturation of RBC’s and

lengthening lifespan of RBC’s.

LEUKEMIA

- cancer of WBC

- group of malignant disease characterized a rapid proliferation of immature RBC’s.

2 TYPES

Lympho – it affects the lymphatic system or the immune systemMyelo – if affects the bone marrow.

Acute lymphocytic Leukemia

- targets children 2-10 years old

- most common

- good prognosis

Diagnostic Procedure:

-Peripheral Blood Smear – reveals immature WBC’s

-Complete Blood Count

-Bone Marrow Aspiration

ADULT: post iliac crest

INFANT/CHILD: sternum, posterior –tibia

Management:

Provide neutropenic precaution

- reverse isolation

- No freshfruit and flower because client is immunocompromise

Administer Chemotherapy.

GASTROINTESTINAL SYSTEM DISORDER

FUNCTIONS:

Maintain fluid and electrolyte balance Process and absorbed nutrients from metabolism. To excrete waste products from digestive process

CLEFT LIP AND CLEFT PALATE

CLEFT LIP

- Failure of the medium maxillary of the nasal process to fuse by 5-8 week of pregnancy.

- Common in boys

CLEFT PALATE

- Failure of the palate to fuse by 9-12 weeks of pregnancy.

- Common in girls

Signs/Symptoms:

- Evident at birth

- During feeding the milk escapes to the nostrils

- Frequent colic otitis media, Upper Respiratory tract Infection

Surgery:

CLEFT LIP

- Cheiloplasty, is a surgery for cleft lip which is done1-3 months to save the sucking reflex

- Rule of Ten, baby can have surgery if he is:

- 10 weeks old

- 10 grams of hemoglobin

- 10 lbs of weight

Nursing Care:

Provide a ngt to large baby Burp the patient as frequent as possible.

CLEFT PALATE

- Palatoplasty, is a surgery for cleft palate which is done to 6-8 months to save the speech or prior to development of speech.

- Nursing Care

1) Pre-op care Provide an emotional support to mother Position patient in upright position

Burp the patient as frequent as possible. Feeding method: used rubber tipped syringe

2) Post-operative care Positioning the patient in side-lying or supine position because it

facilitates the drainage of secretion Feeding method :soup spoon, paper cup not straws Apply elbow restrain Apply logan bar to maintain suture line

HIRSCHSPRUNG’S DISEASE

- congenital anganglionic megacolon

Signs and symptoms:

- Palpable fecal matter

- Abdominal distention

- Vomiting

- bilous

- contains fecal matter

- Halitosis

- Anorexia

- Loss of weight

- chronic constipation

- Ribbon-like stool is outstanding sign of hirschsprung’s disease

Diagnostic Procedure:

Barium enema

Ultrasound

Rectal biopsy which is the conclusive test of hirschsprung’s disease that reveals absence of

ganglion cells

Management:

Monitor Fluid output Administer enema as ordered

INTUSSUSCEPTION

- invagination in telescoping of one bowel to another portion

Signs and symptoms:

-Palpable sausage –shaped mass in abdomen

- Vomiting

- bilous and mucoid

- presence of currant jelly stool

- persistent parosymal abdominal pain

Complication:

Peritonitis

Management

Monitor vital sign especially temperature Monitor stool passage

HYPERTROPIC PYLORIC STENOSIS

- Progressive thickening of the normal layer of pylorus that causes narrowing and obstruction

Signs and Symptoms:

-Palpable olive mass

-Upper abdominal distention

-Projectile vomiting

- non bilous

- mucoid

- Visible peristaltic waves – left to right

Diagnostic Procedure

Ultrasound

Abdominal x-ray x-ray (upper area with Barium swallow

Surgery: Plyoromyotomy (Fredict Ramstedt)

– to loosen tight of pylorus

Nursing Care:

Monitor Vital sign

Monitor the dehydration and metabolic alkalosis Diet: Thickened Formula

CELIAC DISEASE

Also called Gluten-Sensitive Enteropathy.

Is a disease of the small intestine marked by atrophy of the villi and microvilli caused by an immune-mediated inflammatory response to gluten, a protein found in common grains such as wheat, rye, oats, and barley.

The cause is unknown, but genetic, environmental, and immunologic elements maybe involved.

The disease is triggered by surgery, pregnancy, viral infection, or severe emotional distress.

It is most common in young children ages 6 to 24 months but can occur at any age.

Symptoms typically diminish or disappear in adolescence and reappear in early adulthood.

Complications include impaired growth, inability to fight infections, electrolyte imbalance, clotting disturbance, and possible predisposition to malignant lymphoma of the small intestine.

Assessment:

Ages 3 to 9 months

1. Acutely ill; severe diarrhea and vomiting2. Irritability3. Possible failure to thrive

Ages 9 to 18 months

1. Slackening of weight followed by weight loss2. Abnormal stools

o Pale, soft, bulkyo Offensive odoro Greasy (steatorrhea)

o May increase in number3. Abdominal distention4. Anorexia, discoloration of teeth5. Muscle wasting: most obvious in buttocks and proximal

parts of extremities6. Hypotonia, seizures7. Mood changes: ill humor, irritability, temper tantrums,

shyness8. Mild clubbing of fingers9. Vomiting: usually occurs in the evening10. Aphthous ulcers, dermatitis

Older Child and Adult

1. Signs and symptoms are commonly related to nutritional or secondary deficiencies resulting from disease.

o Anemia, vitamin deficiency (A, D, E, K)o Hypoproteinemia with edemao Hypocalcemia, hypokalemia, hypomagnesemiao Hypoprothrombinemia from vitamin K deficiencyo Disaccharide (sugar) intoleranceo Osteoporosis due to calcium deficiency

2. Anorexia, fatigue, weight loss.3. May have colicky abdominal pain, distention, flatulence, constipation, and steatorrhea.

Diagnostic Evaluation:

1. Small bowel biopsy, which demonstrates characteristic abnormal mucosa.2. Hemoglobin, folic acid, and Vitamin K levels may be reduced.3. Prothrombin time may be prolonged.4. Elevated immunoglobulin (Ig) A endomysium antibodies and IgA anti-tissue transglutaminase

antibodies.5. Total protein and albumin may be decreased.6. 72 hour stool collection for fecal fat is increased.7. D-xylose absorption test – decreased blood and urine levels.8. Sweat test and pancreatic function studies may be done to rule out cystic fibrosis in child.

Therapeutic Interventions:

1. Dietary modifications includes a lifelong gluten-free diet, avoiding all foods containing wheat, rye, barley, and possibly, oats.

2. In some cases, fats maybe reduced.3. Lactose and sucrose may be eliminated from diet for 6 to 8 weeks, based on reduced

disaccharidase activity.

Nursing Interventions:

1. Monitor dietary intake, fluid intake and output, weight, serum electrolytes, and hydration status.

2. Make sure that the diet is free from causative agent, but inclusive of essential nutrients, such as protein, fats, vitamins, and minerals.

3. Maintain NPO status during initial treatment of celiac crisis or during diagnostic testing.4. Provide parenteral nutrition as prescribed.5. Provide meticulous skin care after each loose stool and apply lubricant to prevent skin

breakdown.6. Encourage small frequent meals, but do not force eating if the child has anorexia.7. Use meticulous hand washing technique and other procedures to prevent transmission of

infection.8. Assess for fever, cough, irritability, or other signs of infection.9. Teach the parents to develop awareness of the child’s condition and behavior; recognize

changes and care for child accordingly.10. Explain that the toddler may cling to infantile habits for security. Allow this behavior, it may

disappear as physical condition improves.

11. Stress that the disorder is lifelong; however, changes in the mucosal lining of the intestine and in general clinical conditions are reversible when dietary gluten is avoided