Hemolytic Disorders and Blood Incompatibility

Hemolytic Disorders

• Pathologic jaundice arises within the first 24 hours of life, stemming from hemolytic disease in newborns.
• Treatment involves phototherapy (bilirubin lights) to facilitate bilirubin breakdown and excretion through stool, or exchange transfusion.
• Untreated pathologic jaundice can escalate, causing brain damage known as Kernicterus, which is an increased bilirubin in brain tissue.
• Jaundice management includes routine assessments using a bilimeter (done routinely at 8 hours of age) and serum bilirubin levels for at-risk infants.
• Phototherapy employs light waves to treat skin conditions by exposing the skin to ultraviolet (UV) light for a specified duration.

Blood Incompatibility

• Rh incompatibility occurs when an Rh-negative mother carries an Rh-positive fetus.
• Maternal antibodies develop and attack the fetus if their blood mixes.
• Rhogam is administered at 28 weeks of gestation and within 72 hours after birth to prevent antibody formation.
• The indirect Coombs test screens maternal blood; a positive result indicates a risk of pathologic jaundice in the baby.
• ABO incompatibility arises when the fetus's major blood group antigen differs from the mother's.
• RhoGAM is administered within 72 hours of any event causing blood mixing, and blood transfusions may be required.
• Diagnosis involves recognizing jaundice and performing a direct Coombs test on the baby's umbilical cord blood; a positive result indicates a risk of hemolytic disorders.
• Intrauterine transfusions infuse blood through umbilical vein into the fetus.
• Exchange transfusions involve removing small amounts of blood and replacing it with compatible blood if the indirect Coombs test is positive.

Immune System

• Newborns receive passive immunity from the mother through immunoglobulin G (IgG).
• Breast milk provides immunity, which lessens the risk of allergies and food intolerance.
• Babies are capable of generating an immune response to vaccines.
• Inflammatory and immune mechanisms are hypo-functioning in newborns.

Neonatal Infections

• Sepsis arises due to the presence of toxins or infection.
• Newborns are vulnerable to sepsis because of immature immune defenses.
• Maternal fever/infection, prolonged rupture of membranes (ROM) exceeding 18 hours, premature rupture of membranes (PROM), preterm birth, asphyxia during birth, invasive procedures, stress, and congenital anomalies elevate risk.
• Group B Streptococcus (GBS) is usually asymptomatic in women, but testing occurs at 36 weeks of gestation. It is extremely virulent for newborns.
• Babies of GBS-positive mothers remain hospitalized for 48 hours post-birth even if they do not acquire GBS
• GBS is acquired during birth or in utero if membranes are ruptured. Intrapartum intervention decreases early-onset but not late-onset infections
• Treat with Ampicillin and Gentamicin IV, and PCN G.
• If symptomatic, culture and begin antibiotics (abx) immediately

TORCH Complex

• TORCH complex includes infections contracted during pregnancy.
• Toxoplasmosis is contracted from cat feces or uncooked meat.
• Hepatitis B vaccine is offered to all newborns; babies of infected mothers receive HBV immunoglobulin.
• Parovirus exposure can cause aplastic crises in the baby (reduced count of reticulocytes – immature RBCs).
• Varicella can cause birth defects.
• Syphilis can be transferred without maternal knowledge.
• Listeriosis can cause birth defects depending on timing of exposure.
• Congenital Rubella Syndrome can cause severe birth defects if contracted during pregnancy, vaccine is contraindicated during pregnancy and for 28 days after MMR.
• Cytomegalovirus is contracted during pregnancy.
• Herpes Simplex Virus can be transmitted during birth and requires cesarean delivery (c/s).
• Antiviral medications are given at 36 weeks prophylactically.

Integumentary System (Normal)

• Vernix caseosa is a cheese-like substance that protects the skin.
• Acrocyanosis is normal up to 24 hours of age.
• Lanugo is fine, downy hair covering the body.
• Note any bruising, edema, petechiae, or signs of birth injury.
• Term infants have more creases on palms and soles, assessed at birth. Premature infants have fewer creases.

Integumentary Variations

• Single palmar crease is common in babies with Down syndrome, or Asian descent.
• Milia are tiny white sebaceous glands on the nose or forehead.
• Desquamation is skin peeling, usually seen in post-term babies.
• Mongolian spots are dark pigmentation on the back and buttocks, fading over years and usually seen in darker skin.
• Nevi ("stork bite or angel kisses") are flat pink capillary angiomas that blanch white.
• Port-wine stains are capillary malformations that do not blanch and darken over time.
• Erythema toxicum is a newborn rash in the first 24-72 hours; no treatment is needed.
• Pallor/plethora, petechiae, central cyanosis, or jaundice require investigation.

Reproductive System

• Swollen breasts (especially on females) and labia changes are normal due to maternal estrogen.
• Witch's milk is a thin discharge from the breasts, seen in both sexes.
• Swollen, darker labia with hymenal tag, slight bloody spotting occur.
• In preterm infants, the clitoris is prominent.
• In term babies, labia cover the clitoris.
• Palpable descended testes are normal.
• Teach parents not to wash off vernix from labial folds.

Signs of Reproductive Problems

• Cryptorchidism is undescended testicle.
• Epispadias and hypospadias require no circumcision.
• Hydrocele is fluid accumulation around the testes, worse with crying.
• Ambiguous genitalia require ultrasound (UTZ).
• Inguinal hernia occurs when the intestines bulge through abdominal wall.
• Rectovaginal fistula is stool coming from the vagina.

Skeletal System (Normal)

• Molding occurs to allow movement through birth canal.
• Suture lines can be palpated.
• Anterior fontanel is diamond-shaped and closes at 18 months.
• Posterior fontanel is triangle-shaped and closes at 6-8 weeks/1-2months after birth.
• Examine the fontanels when baby is lying flat and not crying:.
• Bulging = Too much fluid, crying. Sunken = Dehydration.
• Spine should be straight with no pilonidal dimple.

Skeletal Variations and Problems

• Caput succedaneum is generalized edema of the scalp that crosses suture lines and is commonly found on the occiput. Pressure of baby sitting in the birth canal can cause it.
• Cephalohematoma is a collection of blood, does not cross suture lines.
• Subgaleal hemorrhage is bleeding in the skull.
• Oligodactyly is not having enough fingers/toes.
• Polydactyly is having too many fingers/toes. Use mitten to tie off suture to remove finger or toe.
• Syndactyly is having webbed fingers/toes.
• Developmental dysplasia of the hip (DDH) occurs when the hips are not even.
• Asymmetric Moro reflex indicates a possible broken clavicle.

Birth Injuries

• Birth injuries occur during labor and birth.
• Few birth injuries are fatal, but some cause lasting injury.
• The clavicle bone is most common fracture.
• Treatment: immobilization with slings, splints, swaddling.

Neuromuscular System

• Transient tremors of the mouth and chin when crying.
• Flexion of arms at elbows and legs at knees.
• Babies respond to pain.
• Persistent tremors, seizures, hypotonia, hypertonia, and lack of response to painful stimuli are problems.

Peripheral Nervous System Injuries

• Brachial plexus injuries occur when nerves from the spine through the neck into the arms are injured.
• Moro reflex is absent on affected side in all plexus injuries.
• Erb's palsy is an upper plexus injury, causing paralysis of affected extremity and muscles; arm hangs limp rotated internally.
• Treatment includes preventing contractures, positioning, passive range of motion (ROM). Complete recovery from stretched nerves takes 3-6 months. If nerves are completely damaged or pulled out, permanent damage occurs.
• Facial nerve injuries manifest with affected side.
• Nursing care includes helping with sucking/feeding techniques, potentially requiring gavage feedings, and protecting the affected eye (often tape shut and administer eye drops daily).
• Phrenic nerve paralysis causes diaphragmatic paralysis.
• Treatment includes positioning on the affected side so that expansion of the unaffected side can be maximized. Provide same nursing care of any infant with respiratory distress.

Normal Newborn Behavioral Characteristics

• Babies should exhibit sucking and rooting reflexes, tonic neck or fencing reflexes, Moro or startle reflexes, and Babinski or plantar reflexes.
• Preterm babies will sleep more, fatigue easier, show physiologic stress, and need quiet time. Newborn eyes are structurally incomplete with immature muscles
• They Follow bright light and track eyes, prefer patterns, recognize smell of mom’s breast and milk, and distinguish sweet vs sour
• Newborn hearing screen is performed before discharge, & recognize moms/familiar voice
• Touch is essential to normal growth and development.
• Crying may signal hunger. Infants respond to cold, overstimulation, & uncomfortable stimuli with crying.
• High pitched, inconsolable cry with hyperirritability and tremors indicate baby may be withdrawing from medication/drugs in utero

Drug-Exposed Infant

• We want baby to “withdraw” in the hospital.
• Prognosis depends on type of drug and stage of fetal development when drug was taken.
• They are at risk for failure to thrive if signs/symptoms of withdrawal not recognized prior to discharge.
• Decrease stimuli, swaddle snugly, and provide adequate nutrition and promote mother-infant bonding.
• Touch times should be limited to Q3 hours due to over-stimulation.
• Methamphetamine exposure is associated with placental abruption, fetal growth restriction, preterm labor, smaller head, and lower birth weights.
• Withdrawal signs/symptoms include agitation, vomiting, tachypnea, poor feeding
• Marijuana exposure is associated with decreased fetal growth, low birth weight, and length.
• Selective serotonin reuptake inhibitor exposure results in withdrawal signs/symptoms such as hypertonia, tremulousness, wakefulness, high-pitched crying, and feeding problems. Passes in breast milk.

Narcotics Exposure

• Narcotics cross the placenta and cause neonatal abstinence syndrome (NAS), which is when baby has withdrawal symptoms.
• Signs and symptoms include diaphoresis, irritability, poor feeding, frantic uncoordinated suck, and high-pitched cry.
• Heroin withdrawal usually manifests in 12 hours.
• Methadone withdrawal usually manifests in 1-2 days.
• Breastfeeding is contraindicated if the mother is still using drugs.
• Fetal alcohol withdrawal is indicated by jitteriness, increased tone and reflex responses, irritability, and seizures can have obvious facial features, learning disabilities, and growth abnormalities.
• Quantity of alcohol required to produce fetal effects is unclear
• Withdrawal is seen with abuse of tranquilizers, sedatives, or other drugs

Newborn’s Standard Precautions

• Standard precautions: (1) wear gloves until baby has a bath, (2) maintain airway and adequate oxygenation*, (3) maintain body temp: remove wet towels, wrap in a warm blanket (4) Eye prophylaxis: azithromycin to each eye (5) Vitamin K prophylaxis, (6) Promote bonding: skin to skin (7) Maintain protective environment, (8) Listen to HR, (9) Assess umbilical cord=2 arteries & 1 vein (AVA) (10) APGAR at 1 min and 5 min **regardless of 1 min score
• Physical assessment includes general appearance, vitals, baseline measurements of physical growth, and neuro assessment (reflexes)
• Gestational age assessment is assessed with the Dubowitz or Ballard chart. and blood sugar checks if SGA or LGA

Dubowitz or Ballard Scoring

• Use chart to determine neuromuscular and physical maturity score.
• Classification newborn by gestational age and birth weight with the Dubowitz or Ballard scoring SGA, AGA, LGA

Prematurity

• Preterm or premature: baby born before 37 weeks
• Late preterm: born between 34-37 wks
• Early term: born between 37 to 38 wks
• Term: 37 wks to 42 6/7 wks
• Post-term: after 42 weeks – Placental dysfunction (placenta starts to die, increased mortality – Absence of lanugo, very little vernix, abundant scalp hair, long fingernails, cracked desquamating skin, Wasted physical appearance, at risk for meconium aspiration.

NICU Care

1. Vitals determined by infant’s acuity
2. Accurate I&O, ongoing labs, temp control, infection prevention, keep hydrated, maintain nutrition, skin care, developmental care, touch times
3. Q3 hours for feedings or vitals, promote skin to skin if possible Infants of diabetic mothers

• Glycemic control before conception and in first trimester can prevent malformation
• Macrosomia but sometimes you will see SGA due to maternal diabetes
• Risk of shoulder dystocia, congenital anomalies, hypocalcemia, hypomagnesemia, polycythemia, hyperbilirubinemia which leads to Respiratory Distress Syndrome (RDS)
• Hypoglycemia: they have to eat early, and we have to check blood sugars If Symptomatic: feed, if unable to feed give IV dextrose continuously

Newborn Screenings

• Newborn Screening Test (NBS) involves blood collected via heel stick after 24 hours of birth until 2 weeks to check for phenylketonuria.
• Newborn Hearing Screening Test (NBHST)
• Heart Defect Screening involves pulse oximetry on the hand and one on the feet to see if it is within 3 points of each other check for preductal to see if it is within 3 points of each other
• If yes, baby can go home If no, repeat in one hour If still not within 3 points further testing because that could mean a cardiac problem.

Developmental Stages and Ages

• Infant (0-1)
• Toddler (1-3)
• Preschooler (3-5)
• School age (5-12)
• Adolescent (13-18)

Psychosexual Development

• Admission assessment includes ADLs, meds, baseline physical, neuro assessment to get a baselin
• Preparing the child for admission by Prehospital counseling, proper room assignment based on the kids age, situation, and gender
• Do your best to adapt to the child’s normal habits

Effects of Hospitalization

• Effects may be seen before admission, during hospitalization, or after discharge.
• A child’s concept of illness is more important than intellectual maturity in predicting anxiety.
• Child may or may not be affected by previous hospitalizations.
• Kids b/w 6 mo and 5 yrs struggle with stress more
• Males are more affected Continued repeating stressors increase stress

Nursing Interventions

1. Preventing or minimizing separation- Important in kids less than 5
2. Preventing or minimizing parental absence
3. Minimizing loss of control: give them choices and promote freedom of movement. More control = less stress
4. Preventing or minimizing fear of bodily injury
5. Providing developmentally appropriate activities

• Provide opportunities for play and expression
• Diversional activities o Expressive activities such as creative expression and dramatic play
• Make things into a game if possible eg. Toys, utilize a child life specialist: they are trained in education, play therapy, and helping children through procedures
• Tell parents to not go and buy new toys, the child needs familiarity

Nursing Care

Warn parents and siblings about what the kid may look like when they see them

• Provide information: teach about disease, what is normal and abnormal, what to expect when you go home
• Encourage parent participation: incorporate them in care of the child
• Preparing for discharge and home care: (begins at admission) Maximizing potential benefits of hospitalizations
• Foster parent child relationships
• Provide educational opportunities
• Promote self-mastery: having a challenge, coping, and overcoming that challenge
• Provide socialization Special hospital situations
• Ambulatory or outpatient setting: decrease stress of hospitalization, decrease risk of infection, cheaper, teaching is important
• Isolation: a big stress on families, talk to kid like they are special because they are on isolation
• Emergency admission: most traumatic, lots of education, let the parent be with the kid as much as possible while in the ER
• ICU: explain every tube and line, help the parents cope, be honest with the parents, ask the parents what the child likes Pediatric variations in nursing interventions

General Concepts

1. Informed consent: capable of giving consent at 18 w/o a parent, if younger than 18 get consent from guardian, child must give assent (agree), must act voluntarily
2. Prepping for procedures: education, be honest with kid, be trustworthy
3. Safety in Environmental factors by paying attention to little things kids can swallow, make sure furniture is not where a child can pull it on top of them, high fall risk
4. Infection control with use of standard precautions
5. When transporting infants and children: (transport kids in their crib, wagon, bassinets, or the bed (do not carry kids around the hallway), protect the head and neck of younger kids. When transporting ICU kids have 2 staff and resuscitation equipment with you
6. Restraints consist of Behavioral restraints: require an order, doctor has to assess pt Q hour then Q 4 hours, assessed Q 15 by nurse Medical surgical restraints: authorized Q day, protocol for cleft palate surgery (elbow immobilizers, IV); monitor Q2

Collection of Specimens

For urine specimens or Clean catch: can only be used on a kid who is mature/old enough

• Use urine bag (wee bag) for pedia patients which sticks over the urethra then empty the bladder and discard the first one. Urine must be kept on ice
• Catherization: prevent this if possible
• Collect a nonsterile specimen 1st then decide (based on the results of that specimen) if a catheter is needed. If a kid is under 3 months old with unknown cause of fever, they must have a catheter
• Stool specimens: get from diaper, may be hard if watery stool (lay saran wrap down first)
• Blood specimens: try not to stick the kid a lot of times so use 2 nurses to help hold the child down, let the parent be the hero

Medication Administration

• Safety is very important due to immature (liver/kidney) function
• Meds are weight-based and use accurate measuring devices & identifying the child
• Parenteral fluid therapy involves picking the site based on the kid, gathering all equipment before you go in (take extra supplies with you), use infusion pumps, securing lines well, and checking IV Q 1-2 hours

Pain Assessment and Management

1. Behavioral
2. Physiologic
3. Measures of self-report
4. Test sequential understanding before using a self-report scale

• Children with communication and cognitive impairment
• Trust the parents when they say their kid is in pain

Nonpharmacology Pain Management

1.Distraction 2.Relaxation - Guided imagery 3.Cutaneous stimulation: rubbing/massage 4.Containment and swaddling and Nonnutritive sucking: pacifier, Kangaroo care: skin to skin

Sucrose Pharmacologic Pain Management

1.Acetaminophen 2.NSAIDs and Opioids 3.Work on the CNS, Need to be weaned off 4.Co-analgesia or adjuvant analgesia: giving a med to help with the s/e of another 5.Patient-controlled analgesia- Respiratory status and end tidal CO2 monitor, Analgesia 6.Side Effects -kids metabolize drugs quicker and may need to be dosed more frequently

Consequences of Untreated Pain

• Infant pain often inadequately managed
• Mismanagement of infant pain partially because of misconceptions regarding effects of pain
• Infant pain Chemical and hormonal responses increase Greater morbidity for neonates in NICU
• Managed pain reduces stay in the hospital, lower cost of stay, decreased intubation time
• Cognitive impairment is a general term that encompasses any type of mental difficulty or deficiency classified as mild, moderate, severe, or profound; determined by IQ

Causes of Cognitive Impairment

• Intrauterine infection and intoxication
• Trauma (prenatal, perinatal, postnatal)
• Metabolic or endocrine disorders
• Inadequate nutrition
• Postnatal brain disease
• Unknown prenatal influences

Nursing Care for Children

• Communicate with kid
• Educate child and family
• Teach child self-care skills
• Promote child's optimal development
• Encourage play and exercise
• Assess abilities and deficits
• Positive reinforcement and motivation are important to promote self esteem
• Provide means of communication
• Establish discipline and teaching social norms
• Provide information on sexuality and boundaries
• Help families adjust to future care Care for the child during hospitalization Praise good behavior

Hearing Impairment

• Promote Communication by supplementing with visual and tactile media and including additional aids Hospitalized care for child with hearing impairment include picture board ,lip reading, and sign language

Visual Impairment

• Hospitalized care for child with visual impairment includes providing a safe, consistent environment and encouragement of independence

Nursing care of the family and child

• Support coping methods and educate about disorder General Health Establish realistic future goals and teaching in case of emergencies
• Principles of palliative care addresses Pain and symptom management-focuses on quality of life, ethical considerations between doctors, health care team, parents, child
• Treatment options: Hospital, Home care, Hospice- life expentancy under 6 months
• Talk openly and honestly with family and make sure the kid has a say
• Child and family experience fears of pain, not being there for death Grief and mourning is a process, not an event
• Nurse’s reactions to caring for dying children

Childs Respiratory System

• Children less than 6 y/o use their diaphragm to breathe
• Children less than 6 y/o use their diaphragm to breathe
• Retractions indicate severe airway obstruction or severe distress

Oxygen Delivery

• Pedia Protocol: parameters on what the RR and O2 levels should be
• Mist Tent: provide humidified O2, common in kids
• Treat the patient, not the monitor

Apnea

• Diagnostic evaluation: based ondescription of what is happening, Potential causes: reflux, seizures, neuromuscular disorder, respiratory issues
• Croup syndromes: Swelling of the upper epiglottis all the way down to the trachea/bronchi
• Acute epiglottitis: caused by Haemophilus influenza that leads to complete airway blockage Do NOT visually inspect ,assess the child and monitor their O2- Treatment: IV antibiotics, steroids, fluids
• Laryngotracheobronchitis usually caused by viruses, Nebulized Racemic, Tell parents they can put the child in the car with the windows down if it’s cool outside

Brachial Tracheitis and Bronchiolitis

Starts as URI, croupy cough, retractions then Oxygen, intubation, antipyretics, and antibiotics

• Bronchiolitis –treated at home with steroids and breathing treatments, Hospital admission if O2 is compromised

Pneumonia

• Pneumonia – inflammation/infection of the bronchioles and alveolar space Can occur primarily or as a secondary complication
• Viral associated with URI is more common, Mycoplasma pneumonia is most common community acquired with a faster onset, grunting, nasal flaring, irritability, lethargy, diminished appetite Treatment of pneumonia includes supportive care, antibiotics, O2 support, IV fluids

Foreign body aspiration

Can be from food or objects. Treatment is done via the BLS protocol for choking by providing 5 back blows and 5 chest thrusts and heimlich if applicable

Pulmonary Edema

Medical Management: if cardiogenic diuretics, digoxin, and vasodilators and monitoring O2 levels to promote, comfort, and provide support and education for family and child

Smoke inhalation injury

Plastic is toxic to the respiratory system from reflex closure of the epiglottis -Place child on 100% humidified O2, ABGs

Obstructive Sleep Disorder Behavior

• Ranges from mild partial obstruction to Obstructive sleep apnea syndrome (OSAS) Enuresis, and daytime sleepiness ,Done via sleep study Early intervention and teaching is important

Respiratory failure

Treatment includes administer O2, positioning (towel under infants' neck), stimulation, suctioning, early intubation, family can be present if they want

Appendicitis

Symptoms: McBurney’s point rebound tenderness

• if rupture then surgery (appendectomy) that includes antibiotics, NG tube until bowel sounds. Management for surgery or for no rupture includes, maintain hydration, pain support and watching for s/s of infection

Meckel diverticulum

Out-pouching of ileum containing gastric and acid that cause irritation

• Painful Rectal Bleeding,red “currant jelly” stools that include: reassurance child, preop care which consist of Preop: IV therapy, I&O, test stool for blood Pain control, watch for s/s of infection, NG tube, monitor bowel function Test stool for blood

Peptic Ulcer Disease

Give H2 blockers prophylactically to prevent ulcers from the stress of hospitalization, relieve discomfort, upper GI endoscopy, biopsy,

• triple drug therapy, 2 antibiotics + PPI/mucosal protective agents Surgery if severe.

Hepatitis

A or E=fecal/oral and doesn't cause liver damage Most children cared for at home and explain infectious control procedures that include Well Balanced diet and rest, caution with meds Cirrhosis = irreversible damage= monitor liver function and children die waiting on transplant.

Biliary Atresia

• Mild jaundice in Newborn causing cirrhosis if untreated and is unknown but may be viral
• A piece of the intestine gets connected to the liver and works as a port Kasai procedure and baby will still need liver transplant Vitamin K and can be breast-fed.

Cleft Lip and Palate

• Plastic surgeon, speech therapy, hearing specialist that is Common in Native American and Asian
• Look for other congenital anomalies and do Assessment at birth for feeding
• NG tube feeding with Pigeon bottle and keep syringe and suction close by
• Promotes Bonding with No proning, elbow and 7-10 post op with No suction

Esophageal Atresia and Tracheoesophageal Fistula

NG tube to suction that leads to apnea, drooling Esophagus ends in pouch connects to trachea that includes NG tube to suction and monitor airways

Hypertrophic Pyloric Stenosis

• Hypertrophy with Projectile Vomiting/ Palpable Vomiting
• Start with clear fluids and progress as tolerated, with monitor infections

Diaphragmatic Hernia

• Protrude contents into Diaphragm Elevate the head, stimulation with cluster care Omphalocele and Gastroschisis Cover organs but monitor temp-mom can deliver vaginally Intussusception
• telescoping/twisting stool that includes abdominal pain that consists of: Red Currant shaped stool that is corrected with Enemas

Volvulus

Twisting/decreased blood flow from the intestine that leads to Necrosis requires emergency surgery

Ostomies

Ostomies areOpening in intestine that diverts fecal matter where teaching is a priority for development and include family

Poisoning

Call poison control before Intervening assess victims is CPR is needed. Empty mouth flush eyes with saline and identify poison Lead poisoning Monitor for side effects with Chelation-Monitor labs