Hyperbilirubinemia and Jaundice

Hyperbilirubinemia Definition

• Excessive accumulation of bilirubin in the blood
• Characterized by jaundice, a yellowish discoloration of skin, sclera, and mucous membranes

Physiological Jaundice

• Appears after 24 hours
• Total bilirubin rises less than 5 mg/dL per day
• Maximum intensity occurs by the 4th-5th day in term infants and 7th day in preterm infants
• Serum bilirubin level is 12-15 mg/dL
• Clinically not detectable after 14 days
• Resolves without treatment

Pathological Jaundice

• Appears after 24 hours
• Total bilirubin rises less than 5 mg/dL per day
• Maximum intensity occurs by the 4th-5th day in term infants and 7th day in preterm infants
• Serum bilirubin level is 12-15 mg/dL
• Clinically not detectable after 14 days
• Resolves without treatment

Etiology Risk Factors

• Jaundice within the first 24 hours
• Sibling with a history of jaundice as a neonate
• Unrecognized hemolysis
• Non-optimal sucking/nursing
• G6PD deficiency
• Infection
• Cephalhematoma/bruising
• East Asian/North Indian ethnicity
• Delayed meconium passage
• Inadequate paternal feeding behavior
• Malnourished infants

Etiology Predisposing Factors

• Family history
• East Asian race
• Prematurity
• Neonatal immaturity of liver enzymes (e.g., glucuronyl transferase deficiency)
• Hemolytic disorders (e.g., ABO or Rh incompatibility)
• Genetic conditions (e.g., Gilbert’s syndrome, Crigler-Najjar syndrome)

Etiology Precipitating Factors

• Diabetic mother
• Dehydration
• Delayed breastfeeding and failure
• Bruising or cephalohematoma
• Prematurity or LBW (low birth weight)

Symptomatology

• Yellow discoloration of the skin
• Yellow sclera
• Yellowing of mucous membranes
• Poor feeding
• Lethargy
• Dark urine or dark yellow
• Pale stools
• Irritability or fussiness
• High-pitched crying

General Pathophysiology

• Bilirubin is produced from the breakdown of red blood cells (RBCs)
• Unconjugated bilirubin circulates bound to albumin, but some circulates as 'free' bilirubin, being lipid-soluble and able to cross the blood-brain barrier
• UDP-glucuronosyltransferase (UGT) in the liver converts unconjugated bilirubin by adding amino acid
• Conjugated bilirubin is water soluble but lipid insoluble, so it cannot cross the blood-brain barrier
• Conjugated bilirubin goes to the small intestines where it's converted back to unconjugated bilirubin by ß-glucuronidase
• Unconjugated bilirubin re-enters circulation via the enterohepatic circulation
• Remaining conjugated bilirubin is metabolized by intestinal bacteria into urobilinogen and stercobilinogen
• Urobilinogen is oxidized to urobilin, giving urine its yellow color
• Stercobilinogen is oxidized to stercobilin, giving feces its brown color

Laboratory & Diagnostic Tests

• Serum Bilirubin Levels
• Complete Blood Count
• Blood typing and Coombs test
• Peripheral blood smear
• Liver Function Test

Medical Management: Increased Breastfeeding

• Frequent, effective breastfeeding helps the baby eliminate bilirubin

Medical Management: Phototherapy

• First-line treatment for reducing bilirubin levels
• Uses blue light (430-490 nm wavelength) to convert bilirubin into an excretable, water-soluble form
• A safe treatment for all ages, phototherapy is a common treatment for newborn jaundice
  • Fiber-optic blanket
  • Intensive phototherapy

Recommended Phototherapy Duration

• Mild Jaundice: 12-24 hours
• Moderate Jaundice: 28-48 hours
• Severe Jaundice: 48+ hours (with intensive therapy)

Continuous Phototherapy

• Maintains the jaundiced neonate under phototherapy continuously
• Allows only minimal interruptions for feeding or cleaning
• Maximizes time under radiant energy and minimizes the duration of phototherapy and hospital stay

Intermittent Phototherapy

• Regular cessation of phototherapy at specific times and durations
• Reduces exposure to radiant energy and allows parent-infant interaction time
• There’s no specific time schedule, but a common order is 6 hours on, 6 hours off (e.g., 7 am on, 1 pm off, 7 pm on, 1 am off)

Exchange Transfusion

• Replaces the infant's blood for quick bilirubin level reduction
• Calculated based on the TSB level, its rate of rise, neonatal age, and neurologic complication risk factors
• Rapidly removes bilirubin and hemolysis-causing antibodies

Pharmacological Management: Phenobarbitone

• Reduces peak serum bilirubin
• A loading dose of 20 mg/kg may enhance the therapeutic benefit
• Does not cause the respiratory depression seen in higher doses

Intravenous Immunoglobulin (IVG)

• Considered if bilirubin keeps rising after phototherapy and exchange transfusion isn't possible
• IVIG can lower hemolysis with doses of 0.5 to 1 g/kg over 2 hours in hyperbilirubinemia with high total serum bilirubin

Nursing Interventions

• Clinically assess initial jaundice level
• Monitor daily patient weight
• Encourage frequent breastfeeding
• Supervise phototherapy system setup
• Explain phototherapy to parents
• Assess for neurologic changes
• Note stool and urine color, frequency
• Keep body warm and dry
• Check skin and core temperature often
• Assess parent knowledge about condition

Nursing Management

• Assess neonatal bilirubin using transcutaneous bilirubin (TcB) or total serum bilirubin (TSB) tests
• Determine jaundice severity to guide treatments
• Start phototherapy with maximum skin exposure, eye and genital covering
• Phototherapy converts unconjugated bilirubin into an excretable, water-soluble form, reducing serum bilirubin
• Check for overheating/hypothermia and adjust temperature settings, as phototherapy increases insensible water loss, causing complications
• Reposition the infant every 2 hours for even phototherapy exposure, preventing pressure sores and improving light exposure
• Ensure hydration/nutrition, encourage breastfeeding/formula, and assess for dehydration
• Proper hydration promotes bilirubin excretion through urine and stool
• Monitor stool/urine output and temperature, adjust phototherapy, as bilirubin excretion occurs via the stool

Nursing Management: Bilirubin Excretion

• Bilirubin is primarily excreted through stool, indicating effective bowel movements

Nursing Management: Parent Education

• Educate on jaundice, treatment importance, and signs of worsening (lethargy, poor feeding, arching)
• Parental understanding ensures treatment compliance and timely reporting

Nursing Management: Kangaroo Care

• Promote skin-to-skin contact and encourage parents to hold the baby on their chest for at least an hour daily
• Kangaroo Care improves thermoregulation, stabilizes vitals, enhances breastfeeding and bilirubin excretion in stool, reduces stress, and improves parent-infant bonding

Nursing Management: Skin/Eye Protection

• Protect skin and eyes by shielding the eyes with phototherapy goggles and repositioning every 2 hours to prevent pressure sores
• Eye protection prevents retinal damage and repositioning prevents skin breakdown. Light exposure is also enhanced via the goggles.

Nursing Management: Exchange Transfusion

• Prepare for and assist with exchange transfusion if bilirubin approaches dangerous levels via IV access and stabilizing vital signs
• Reduces bilirubin, preventing kernicterus

Nursing Diagnosis

• Impaired skin integrity related to jaundice
• Deficient knowledge related to hyperbilirubinemia diagnosis
• Hyperthermia related to phototherapy
• Ineffective newborn feeding pattern related to lethargy
• Risk for paired parent-infant attachment during phototherapy

Prognosis: Treated

• Physiological: Infants fully recover without complications
• Pathophysiological: Improves with treatment like medication/surgery, or treating underlying conditions

Prognosis: Untreated

• Acute bilirubin encephalopathy or kernicterus
• Bilirubin interferes with brain cell formation, causes permanent damage and neurologic problems

Spina Bifida Definition

• Congenital neural tube defect where the spine and spinal cord do not develop properly
• Causes varying degrees of disability
• Occurs when the neural tube doesn’t completely close during development, causing spinal column and nerve defects

Types of Spina Bifida

• Spina Bifida Occulta: small splits in vertebrae, the spinal cord doesn't protrude
• Meningocele: A sac of spinal fluid bulges via spine opening
• Myelomeningocele: Part of spinal cord, protective covering, and spinal nerves push via opening a birth forming a sac
• Myeloschisis: an open neural tube defect where neural tissue is exposed without meninges or sac

Anatomy and Physiology of the Brain

• The brain is one of the most complex organs that sends/receives chemical and electrical signals throughout the body

Anatomy and Physiology of Cerebrospinal Fluid

• Assists with brain protection/nourishment/waste removal via Meninges
• Meninges are a layered connective tissue covering brain/spinal cord

Anatomy and Physiology of the Spine

• The spine’s central support structure connects parts of the musculoskeletal system

Anatomy and Physiology of Vertebrae

• Has 33 stacked vertebrae forming the spinal canal that protects the spinal cord/nerves

Anatomy and Physiology of Spinal Cord

• A bundle of nerves extending from the lower brain to the lower back

Etiology Predisposing Factors for Spina Bifida

• Family History
• Race
• Sex

Etiology Precipitating Factors for Spina Bifida

• Folic Acid Deficiency
• Anti-Seizure Medications
• Obesity
• Poorly Managed Diabetes
• Alcohol Abuse

Physical Signs and Symptomatology for Spina Bifida

• Leg Weakness and Paralysis
• Orthopedic Abnormalities
• Bladder/Bowel Control Problems
• Abnormal Eye Movement
• Pressure Sores/Skin Irritations

Neurological Problems and Symptomatology for Spina Bifida

• Arnold Chiari Il Malformation
• Back portion of the Brain is displaced into the upper neck
• Hydrocephalus
• Corpus Callosum
• Less Organized or Impaired White Matter
• Cortex Abnormalities

Pathophysiology for Spina Bifida

• A neural groove develops into neural tube around day 20 after conception
• At normal development, upper end is to close at day 25 and the lower to close at day 27
• 3 possibilities to cause abnormal closure: 1) Abnormal hyaluronic acid matrix or actin microfilaments won't close neural tube. 2) Overgrowth at the caudal end causes failure. 3) Glycoproteins can't hold cells together during closure
• Incomplete closing = Spina Bifida

Laboratory and Diagnostic Tests (Antenatal Testing)

• Maternal Serum Alpha-Fetoprotein Test
• Ultrasound
• Amniocentesis

Laboratory and Diagnostic Tests (Diagnostic Testing)

• Maternal Serum Alpha-Fetoprotein Test
• Ultrasound
• Amniocentesis

Spina Bifida Medical Management

• PHYSICAL THERAPY
• ASSISSTIVE TECHNOLOGIES
• OCCUPATIONAL THERAPY

Spina Bifida Pharmacologic Management: Anticholinergics (Oxybutynin)

• Route: Oral or transdermal
• Action: Relaxes bladder, reduces contractions
• Use: treats neurogenic bladder dysfunction

Spina Bifida Pharmacologic Management: Antibiotics (Trimethoprim-Sulfamethoxazole)

• Antibiotics (Trimethoprim-Sulfamethoxazole)
• Route: Oral
• Prevents bacterial growth and treats UTIs
• Use: High risk for UTIs due to neurogenic bladder

Spina Bifida Pharmacologic Management: Antiepileptics (Levetiracetam)

• Route: Oral or intravenous
• Action: Stabilizes neuronal membranes, prevents seizures
• Use: Treats patients who develop epilepsy due to abnormalities

Spina Bifida Pharmacologic Management: Pain Management (Acetaminophen or NSAIDs)

• Route: Oral
• Action: Reduces pain and inflammation
• Used: Patients with chronic pain from abnormalities

Spina Bifida Pharmacologic Management: Baclofen (For Spasticity)

• Route: Oral or intrathecal
• Action: Muscle relaxant, inhibits reflexes
• Used: Spina bifida patients develop spasticity

Spina Bifida Pharmacologic Management: Vitamin D and Calcium Supplements

• Route: Oral
• Action: Supports bone health/mineralization
• Used: High risk for osteoporosis/fractures

Spina Bifida Surgical Management

• Postnatal Surgery: Meningocele and myelomeningocele require surgical intervention to reposition the meninges and close the vertebral opening. Surgery within 24–48 hours after birth reduces infection risks and prevents further spinal cord damage.

• Fetal Surgery: A high-risk procedure performed at 21–25 weeks of pregnancy, where surgeons open the uterus to repair the baby’s spinal cord before birth.

• Cesarean Birth: may be part of the treatment for spina bifida; many babies with myelomeningocele tend to be in a feet-first (breech) position.

Nursing Interventions

• neurological to regularly monitor for spinal cord dysfunction or injury
• Provide education and support to parents on proper skin care to prevent pressure ulcers due to immobility.
• Assist with positioning and range of motion exercises to maintain joint mobility and prevent contractures. Teach clean intermittent catheterization techniques to promote bladder emptying and prevent urinary retention.
• Monitor bowel function and implement bowel management strategies, such as dietary modifications or stool softeners, to prevent constipation and bowel accidents. Collaborate with the healthcare team to coordinate surgical Interventions for closure of spinal defects and management of associated conditions. Facilitate access to community resources and support groups for individuals and families living with spina bifida. Provide emotional support and counseling to address anxiety, depression, or adjustment issues related to living with a chronic condition.