What happens in the NICU?

The Neonatal Intensive Care Unit (NICU), in contrast to the newborn nursery, is a highly specialized unit with skilled staff prepared to handle the most dangerous postnatal complications of sick or premature infants. Babies can be found in incubators known as isolettes, which control their temperatures. The  average gestational age (GA) at birth is between 38 and 42 weeks, and an infant delivered before 37 weeks is considered to be "premature". The last trimester (12 weeks) in the womb is an important time for the baby's growth, and underdeveloped organs are vulnerable. The NICU staff, led by a neonatologist, monitors around the clock to identify and manage any problems. Here are a few of the most common:

Apnea: when a baby, typically less than 35 weeks GA, stops breathing momentarily for OVER 20 seconds. This can be central (the brain needs help reminding the baby to breathe, which is treated with caffeine) or obstructive (managed with suctioning and repositioning).

Bronchopulmonary Dysplasia (BPD): when the baby's lungs do not have a change to fully develop, they will need help to breathe until their lungs mature. This may include supplemental oxygen using either a nasal cannula, CPAP, or mechanical ventilation. Typically, OB/GYN doctors help prevent this by giving steroids (betamethasone) to mothers within 24 hours of their delivery if between 24-34 weeks GA. After delivery of a preterm infant, the neonatologist may provide further protection by coating the newborn's lungs in a material called surfactant. BPD is diagnosed if any newborn requires 28 or more days of oxygen support, and is further classified as mild, moderate, or severe depending on how much oxygen he or she is on at 36 weeks GA.

Polycythemia or Anemia: when the baby's hematocrit (ratio of red cells in the blood) is greater than 65% or below 30%, respectively. Anemia of prematurity occurs in the second month of life.

Patent Ductus Arteriosis: persistence of a connection between the right sided chamber of the heart and the aorta, which normally closes at birth. Characterized by tachypnea (fast breathing), cardiac murmur, and a low mean arterial pressure (MAP). Diagnosed with an echocardiogram.

Retinopathy of Prematurity: poor formation of the blood vessels of the eyes, occurs in most infants less than 28 weeks GA, screened for by an ophthalmologist if the baby is <30 weeks GA.

Necrotizing Enterocolitis: when the baby's bowel becomes overwhelmed with bacteria, leading to air in the walls of the intestines and can lead to perforation. Usually occurs in very preterm infants and is characterized by poor feeding, abdominal distension, and bloody stools.

Intraventricular Hemorrhage: when there is bleeding into spaces within the baby's brain known as the ventricles. Usually occurring within the first 3 days of birth and in very premature infants (<1500 grams) who have not fully developed their brains yet.


Problems in full term newborns (>37 weeks) that may require a NICU stay include hypoglycemia, poor feeding, poor tone, meconium aspiration syndrome, transient tachypnea of the newborn, infection, hyperbilirubinemia, and anatomical/chromosomal abnormalities.

Heat Illness in Children

Pediatricians often caution their patients to "drink more water" on hot days or while exercising, but what is the reasoning behind this? 

Children and adolescents have two disadvantages when it comes to heat illness, which makes them more susceptible. Both their decreased sweat response (we cool off by sweating) and their poor thirst response (the feeling of being thirsty) when compared to adults. It is for this reason that heat illness is the 3rd leading cause of death in athletes in the U.S. The illness is categorized by the degree of temperature elevation:

Heat Cramps

Mild dehydration leads to cramps in the hamstrings or calves, and responds well to electrolyte fluid replacement. 

Heat Exhaustion

Characterized by a 100-103 degree body temperature, along with headache, weakness, nausea and vomiting. The treatment is to remove the clothes, apply ice packs to the body and to place the patient under cooling fans. Fluids are given for rehydration, whether by mouth or via an IV. 

Heat Stroke

A medical emergency characterized by a body temperature of >104 degrees paired with intense sweating caused by overexertion. The patient is immediately cooled to prevent organ damage, and IV fluids are administered. Unfortunately, the result is death in 50% of cases,

Athletes are encouraged to drink water every 20 minutes, and if exercise exceeds 1 hour then an electrolyte-rich sports drink is indicated.

Tay Sachs Disease

Tay Sachs disease is a type of degenerating neurological disease caused by a deficiency of the enzyme protein hexosaminidase A. Because the enzyme is deficient, a lipid molecule called GM2 ganglioside builds up inside the lysosomes of cells. It's for this reason that doctors categorize it as a type of lysosomal storage disease.

The disease occurs when parents that are BOTH carriers of the genetic mutation have a baby. The most common carriers are Ashkenazi Jews, French Canadians and Louisiana Cajuns (1 in every 27), though carrier frequencies are also high in Irish Americans (1 in 50) and the general population (1 in 250). 

The disease typically occurs acutely in infancy, but there are three documented presentations: acute, subacute and late onset. 

The classic presentation, the acute (infantile) form, involves progressive motor weakness with loss of milestones and myoclonic jerking beginning at 3 months of age. 98% of patients develop seizures, and death typically occurs within the first few years of life. The severity is due a complete absence of the hexosaminidase A enzyme.

The subacute (juvenile) form will present as late as 9 years old, and is characterized by seizures, choreiform movements, speech problems and incontinence.  These children will typically develop dementia and won't live past the teenage years. 

Late onset Tay Sachs (LOTS, adult) cases have been reported in older patients, and usually involve subtle disturbances in speech, mood and psychological state. 

Pediatricians should suspect Tay Sachs Disease in any Jewish patient or patient of Jewish descent with delayed milestones and myoclonic jerks before the age of 1. Close inspection of the eye will typically reveal a cherry-red colored spot on the macula of the retina, which is only present in a few childhood diseases (Krabbe, Canavan, Alexander, and Gaucher diseases). A blood test will officially confirm the diagnosis. 

The differential diagnosis of neurologic progression in pediatric patients involves neurodegenerative disorders (spinal muscular atrophy/SMA, amyotrophic lateral sclerosis/ALS, Friedreich ataxia/FA and leukodystrophy)

Management of the disease involves maintaining nutrition, respiratory function and motor function while minimizing infectious diseases and seizures. Later forms benefit from both speech and physical therapies. All patients and their families should be referred to a certified genetic counselor for counseling. 

The prognosis for Tay Sachs disease is very poor. One retrospective study followed 97 infants diagnosed with Tay Sachs disease and noted that death occurs before the age of 5 in nearly all patients. Milestones such as head control, transferring objects from hand to hand and sitting without suport were acheived by the babies in over half the cases, but all lost their abilities by 1 year of age. The median lifespan of the infants was only 47 months.

Reference

Tay Sachs Disease. Dynamed Database. Updated May 12 2014. Accessed July 10, 2014. 

Your Child's Development: Pediatric Milestones

"Is my child developing normally?" 

This question is almost guaranteed during nearly all well child encounters in the pediatric office setting. Parents, rightfully so, are commonly concerned about their child's development and need a small amount of  reassurance from their professional caregiver. What are the guidelines that all pediatricians follow? 

The pediatrician references a set list of known guidelines when evaluating the development of a child. He or she often does this in three ways: using an Ages and Stages Questionnaire (sensitivity 75%, specificity 81%) filled out by the parent, asking the parent specific questions about the child and physically examining or interviewing the child. 

Below is the list of developmental milestones. It's important to remember two things:

1) The milestones are simply a guide: physicians use it to help determine if a child has fallen below normal when compared to other age-matched children. It is the job of the pediatrician to recognize a child that has fallen behind in one area and to offer support or resources to help him or her catch up (i.e, the Early Steps program).

2) All children develop differently. One child may meet all milestones on time while another may be persistently behind in one area. It's important to always be patient with (and supportive of) your child.

Pediatric milestones by age:

1 month

Vision: regards face, fixes transiently

Social: smiles spontaneously

6 weeks

Vision: follows to midline

Language: vocalizes (other than crying)

2 months

Hearing: alert to sound- localize by turning head and eyes

Social: cuddle when held, smiles responsively

10 weeks

Gross motor skills: lifts head prone 45-90 degrees

3 months

Language: coos (ooh, ah), laughs

Gross motor skills: head control when pulled to sitting position 

Fine motor skills: hands together

4 months

Vision: follows 180 degrees (visual tracking)

Language: squeals, makes raspberry sound, babbles d,t,z then b,m,p

Gross motor skills: may sit with support, raise head when lying prone

Fine motor skills: grasps rattle

5 months

Gross motor skills: rolls over (prone to supine)

6 months

Gross motor skills: pulls to sit without head lag, reach for toy, rolls both ways, sits with support and alone

Social: stretches arms to be picked up, shows likes

Warning sign: significant head lag, persistent fisting, persistent squint, persistent asymmetric tonic neck reflex, inability to roll over

8 months

Hearing: turns to voice

Gross motor skills: sits without support

Fine motor skills: transfers objects hand to hand, feeds self cracker

9 months

Hearing: turns directly to sound

Gross motor skills: crawls

Social: plays games, finger feeds, holds own bottle, object permanence

10 months

Gross motor skills: pulls to stand

Language: mama, dada, nonspecific, imitate speech sounds, repetitive consonants

1 year

Language: mama, dada specific one word other than mama and dada

Gross motor skills: pivots while sitting

Fine motor skills: bangs objects with hands, thumb/finger grasp, grasps by radial raking, "pincers" grasp, drops and throws toys

Social: indicates wants other than crying, comes when called

Warning sign: failure to sit without support, persistent squint

13 months

Gross motor skills: stands momentarily, cruises (stands and moves between large objects in room by hanging on)

14 months

Gross motor skills: walks well 

15 months

Language: immature jargonizing, understands "no", one-step commands, can point to body parts

Gross motor skills: climbs

18 months

Gross motor skills: kicks or throws ball

Fine motor skills: turns pages, feeds self with spoon

Social: follow simple commands, cuddles toys, enjoy imitative play, copies household tasks, plays some with others, symbolic play (thought)

Warning sign: inability to stand, no pincer grasp, no spontaneous vocalizations, abnormal hand posture

20 months

Language: 3 words other than mama/dada

2 years

Language: 50 words in vocabulary, "NO", can follow 2-part commands, points to named part of body, combines 2 words

Gross motor skills: run, walks up and down stairs with help, climb stairs alternating steps

Fine motor skills: spills little, uses cup, washes/dries hands, may help with dressing, eats by self, scribble spontaneously builds 6-cube tower, uses spoon, removes garment

Social: asks to go to toilet, plays with others (imitation), solitary parallel play (play side-by-side)

Warning sign: absence of recognizable words, lack of walking, tremor or ataxia

3 years

Language: gives first and last name, age, address, name parts of body, speech understandable to family, 3-4 word sentence, 250 word vocabulary, uses plurals, prepositions, pronouns

Gross motor skills: jumps in place, stand on 1 foot, kick ball, rides tricycle

Social: mostly toilet trained, participates in family life, plays interactive games (for example, tag)

Warning sign: speech not understood 75% of time

4 years

Gross motor skills: can heel walk, hops on 2 feet, climb ladder, tiptoe, hop, jump, balances on 1 foot for 5 seconds

Fine motor skills: dresses without supervision, copies, buttons clothes, uses pencil, glue, scissors, copies line and circle

Social: brush teeth, wash hands, toilet, separates easily from mother, enjoys jokes with parents, games with rules

5 years

Vision: recognizes 3 of 4 colors 

Language: counts 10 objects, names 4 colors; can define spoon and cat, can narrate story

Gross motor skills: skips

Fine motor skills: draws 6-part man (head, body, arms, legs), dresses/undresses without supervision, copy triangle, cut and paste

Social: understand good/bad, right/wrong

6 years 

Language: copies sound, composition of door/shoe/spoon

Gross motor skills: hop on 1 foot, walks backward heel-toe

• If you have any questions regarding your child's development, it's important to ask your pediatrician

• Research suggests that faster attainment of developmental milestones in first year of life associated with better educational outcomes at ages 16 and 31 years

• Studies have shown that bed-wetting at age 53 months is associated with a delay in developmental milestones 

• Speech should be intelligible by 3 years of age. If it isn't, the pediatrician will order a hearing evaluation

Reference

Developmental Milestones. Dynamed Database. Updated March 27, 2014. Accessed July 7, 2014.

Sleepwalking and Night Terrors in Children

Sleepwalking and night terrors are not uncommon events in developing children. Though they can be quite frightening and worrisome for parents, there is little danger to the child. Both conditions are known as parasomnias, or events that occur during sleep. Knowing what do during your child's episode will put you at ease while reducing the amount of danger to your loved one.

Sleepwalking occurs most commonly in children 11-12 years old. The disorder is relatively common: a 2007 Canadian study estimated that 14% of children aged 3-13 years have had at least one episode. Similarly, night terrors occur between ages 2-10 years old, though it is estimated that 39.8% of children aged 2.5-6 years old have had at least one episode. 

A sleepwalking episode typically lasts 30 seconds to 30 minutes, and is characterized by clumsy and purposeless movements while the eyes remain open. Occasionally, additional parasomnias will occur such as enuresis, bruxism (grinding of teeth) and body rocking. The child will never remembers the event in the morning.

Night terrors are commonly misdiagnosed as nightmares, though there are distinct differences between the two. While nightmares occur in the second half of the night and can be remembered the next morning, night terrors occur about 15-90 minutes after falling asleep, cannot be recalled the next morning, are combined with sleepwalking in a third of cases, and involve autonomic nervous system activation such as high heart rate (tachycardia), profuse sweating (diaphoresis) and fast breathing (tachypnea). Additionally, reports of 390 pairs of twins suggest that night terrors may be genetic in origin.

What can you do?

If you're concerned about your child's sleep walking or night terrors, it may help to know that most cases do not have an underlying disorder. Medications such as lithium, valproate and zolpidem have been associated with sleepwalking, but most cases are likely caused by stress or sleep deprivation. A major danger to children suffering from a sleepwalking episode lies in their surroundings- if you're witnessing the event or noticing recurring events, be sure to minimize any potential hazards by reducing any access to stairs or sharp objects. 

If your child is having multiple episodes of sleepwalking, be sure to mention it to your pediatrician. An important cause to rule out is seizure disorder with post-ictal activity. There are significant differences between seizure and sleepwalking, however, in cases of seizure the patient is unable to find his or her way back to their bed. Other medical illnesses, such as overactive thyroid, migraine, anxiety and Tourette syndrome may be to blame.

The treatment for sleepwalking may lie in hypnosis or scheduled nightly awakenings prior to each episode, though these methods have not been completely validated in the medical community. Medications such as benzodiazepines (clonzaepam) for sleepwalking and L-5 hydroxytryptophan for night terrors may help reduce the number of episodes. As a parent, it is important to remember to stay calm during the event and not to confront the child about the episode in the morning. Additionally, be sure that your child is getting quality sleep throughout the night. The following pediatric sleep requirements may be useful to you:

Newborn: 16-18 hours

6 months old: 14.5 hours

12 months old: 13.5 hours

2 years old: 13 hours

4 years old: 11.5 hours

7 years old: 10.5 hours

10-12 years old: 9 hours

Teenagers: 8-9 hours

References

Night Terrors. Dynamed Database. Updated May 13, 2014. Accessed June 23, 2014.

Sleepwalking. Dynamed Database. Updated May 13, 2014. Accessed June 23, 2014.

Preterm infant mortality falling, prevalence of cerebral palsy rising

A recent study by Vincer et al published in the Journal of Pediatrics and Child Health reported on the rates of preterm infant mortality and cerebral palsy (CP) over the last 20 years. They found that the birth prevalence of CP among very preterm infants (less than 31 weeks) is on the rise, but the reasons are unclear.

CP is a permanent condition caused by damage to motor control centers in the brain before or shortly after birth, resulting in non-progressive impaired movement and permanent physical disability. The affected child typically has problems with uncontrolled spastic movements and/or impaired coordination of movement. Some cases are associated with epilepsy, poor cognition and/or difficulty with communication.

Between 1988 and 2007, very preterm infant mortality steadily declined while CP has been on the upswing. Vincer et al notes that maternal risk factors, anemia and tocolytic use have all been on the rise as well. Whether or not these represent a correlation has yet to be determined.

Reference

Vincer MJ, Allen AC, Allen VM, Baskett TF, O’Connell CM. Trends in the prevalence of cerebral palsy among very preterm infants (<31 weeks’ gestational age). Paediatr Child Health. 2014; 19(4):185-9.