Infants with Special Needs

Lactation Support for
Special Needs Infants
Ellen Lechtenberg RD IBCLC CSP
Infants with Special Needs
• Neurological
• Oral-Facial
• Cardiac
• Jaundice
• Gastrointerological
• Late Preterm
• Post Mature
• Misc. Diagnosis
Neurological
• Hypotonic
–
–
–
–
Floppy infant syndrome
Prader-Willi Syndrome, Hydrocephalus
Asphyxia, Trisomy 13, Trisomy 18, Trisomy 21
Medullary lesions, muscular abnormalities
• Hypertonic
– Cerebral Palsy, drug exposed
• Neural tube defects
• Hydrocephalus
• Hypotonic
Neurological
– weak suck, poor suction, ineffective tongue
movement lead to feeding problems
– Need head support, maternal breast support
– Fast flow better! Nipple shield may help
• Hypertonic
– Hypersensitive, sensitive gag
reflux, arch at breast, retract and/or
thrust tongue, clamp/chomping
– Swaddling, sling to bring arms
midline & shoulders forward
Neurological
• Down Syndrome/Trisomy 21
– Characteristic physical features
• Small mouth, macroglossia and hypotonia (90%)
• Congenital heart disease, GI anomalies
– Breastfeeding Implications
• Encourage breastfeeding!
• Weak suck, poor seal, tongue protrusion
• Encourage skin to skin holding
• Close monitoring latch and weight gain
• Pumping may be needed to build/protect
supply
• Empowers mothers!
Neurological
• Breastfeeding Management Trisomy 21
–
–
–
–
Positioning: body supported, horizontal
Stabilize jaw (Dancer hand position)
Nipple shield may be helpful
Developmental Feeding Therapist
Dancer Hand Position
Neurological
• Neural Tube Defects
– Congenital anatomical abnormalities of
the brain and spine
– 1 in 1,365 babies in Utah affected
– Spina bifida (Myelomeningocele) most
common
• Variable neurological deficits/anomalies
– Chiari II malformations: herniation of brain stem
– Hydrocephalus
• Surgical repair within 24-72 hours
• May need additional surgery for shunt (90%)
Neurological
• Neural Tube Defects –Breastfeeding
Management
• Most children are able to breastfeed (unless
significant brainstem involvement-chiari II)
– Watch for stridor, poor suck, apnea,
swallowing problems, arching, absent cry
• Biggest challenge is positioning post operative
• Baby is prone, flat on back/side for several days
• Cannot be burped on back (rock or rub
shoulders)
• Pumping on surgery day and to build supply
(Often mother is still at delivery hospital)
Neurological
• Hydrocephalus – accumulation of fluid in
the cerebral ventricles
– May be congenital, isolated, from IVH or
myelomeningocele
– Infant’s head enlarges
– Irritability, weakness,
neurologic defects vary
but can be severe
– Surgery required
Neurological
• Breastfeeding infant with hydrocephalus
– Positioning key to support large head
– Side lying position works well with pillows
– Be aware of head elevation limitations especially
postoperative
– Infant may have weak suck and tire easily with
increased risk for aspiration
– Infant may not be able to oral eat depending on
neurologic damage
– Pumping to protect & maintain production
Oral-Facial
• Cleft lip and cleft palate
• Pierre Robin Sequence
• Choanal Atresia
• Ankyloglossia
What is Normal?
Craniofacial Defects(cleft lip
and/or cleft palate)
• Incidence of defects (1 in 750 births):
– Orientals: higher incidence than
Caucasians
– African-Americans: lower incidence than
Caucasians
– Native Americans: highest incidence in
the U.S. with 3.5/1000 births.
– Utah rates highest in the United States with
1 in 450 births
Cleft Defects
•
•
•
•
•
•
•
50% involve both lip and palate.
May be unilateral or bilateral.
May involve hard or soft palate or both.
May be overtly visible or submucous.
Consult developmental feeding therapist
Cleft lip alone = Breastfeed.
Cleft palate= Breastfeed 5-10 minutes than
supplement with expressed human milk via cleft
feeding device
• Pumping to build and protect supply
Cleft Defects
Every nurse who cares for mother’s and
babies should have a working knowledge of
feeding methods for cleft-affected infants
Pierre Robin Sequence
• Oral facial abnormalities
– Micrognathia or retrognathia
– Glossoptosis
– And wide U-shaped cleft palate
• Occurs in 1 out of 8850 births
• 80% of infants with PRS have another
syndrome: Stickler syndrome (40%) or
velo-cardio-facial syndrome (15%)
Pierre Robin Sequence
•
•
•
•
Primary problem is airway obstruction
Prone position preferred
May need tracheostomy
Feeding method determined by degree of
and type of medical management for
airway management
• Breastfeeding & PO feeds in mild cases
• Most require tube feedings
• Pumping essential to build and protect milk
supply
Pierre Robin Sequence
Choanal Atresia
• Congenital anomaly of the anterior
skull with blockage or narrowing of the
nasal airway
• Occurs in 1 out of 7000-8000 births
• May be asymptomatic until respiratory
illness
• Symptoms: difficulty coordinating
breathing while suckling, mouth
breathing, chest retractions
Choanal Atresia
• Surgery for placement of airway
followed by stents placed to maintain
integrity of airway
• Breastfeeding implications
– Creative positioning
– Length of stents may need to be adjusted
– Pump prior to surgery (baby is usually OG
fed) and until baby is established
completely at breast
Ankloglossia (tongue-tie)
• Congenital anomaly
– Short lingual frenulum
– Highly attached genioglossus muscle
restricts tongue movement
• Difficulty lifting tongue
• Impaired side to side movement of tongue
• Inability to bring tongue beyond gums
– Wide variety of opinions regarding clinical
significance and management
Ankloglossia (tongue-tie)
• Breastfeeding Implications
–
–
–
–
–
–
Majority of infants have no difficulty
Poor latch
Maternal breast pain/trauma
Poor milk transfer
Dehydration
Failure to thrive
• Frenotomy may be necessary
Congenital Heart Disease
• Most common structural birth defect
• Part of many congenital syndromes
– CHARGE, Down Syndrome,
• Watch for infant during feeding for
cyanosis/hypoxia, fatigue, poor suck,
tachypnea, & uncoordinated patterns
• Congestive heart failure noted shortly
after birth or could be weeks after
discharge
Congenital Heart Disease
• Breastfeeding Management depends
on severity of heart disease and failure
– Maintain adequate oxygen levels
– May need supplementation due to fluid
restrictions and increased calorie needs
• Add supplements to expressed milk
• Use supplemental nursing system
• Hind milk feedings from bottle or gavage after
breastfeeding
– Monitor weight
Congenital Heart Disease
• Breastfeeding
–
–
–
–
–
–
–
Individualized to both infant and mother
Weak suck common: breast massage
Fatigue easily: breastfeed on one side/feeding
Maternal breast support C-hold, Dancer
Nipple shield may be beneficial
Short feeding time – more frequent tolerated
Stop feeding if tachypnic, fatigued, coordination
changes
– May need to gavage after feedings or if baby too
tired to awaken for feeding
– Mom must pump to protect & maintain supply
Jaundice
• Distinguish between pathologic
jaundice and physiologic jaundice
• Distinguish between early jaundice
and late jaundice
• Distinguish between breast milk
jaundice and breastfeeding jaundice
• Distinguish between healthy, full-term
infant and preterm or ill infant.
Jaundice
• Pathological
– Heamolysis of baby’s blood
• ABO incompatibility
• Drug therapies
• Maternal disease states
– Peaks early ~24 hours after birth
– May require photo therapy or exchange
transfusion
– Breastfeeding &Human Milk preferred
feeding
Jaundice
• Physiological or idiopathic
– Common condition
• Preterm
• Infections
• Bruising (forceps or vacuum delivery)
–
–
–
–
–
More common in breastfed infants
Peaks 3 to 4 days after delivery
Breastfeed after birth as soon as possible
Demand feeding
Check positioning and latch
Early Jaundice
•
•
•
•
•
•
•
•
•
Physiologic
Day 2 – 5 of age
Related to feeding practices (infrequent)
Transient (10 days)
More common in primiparas
Infrequent or delayed stooling
Receiving H20 or D5W
May or may not need phototherapy
Bili peaks <15 mg/dl
Breastmilk (Late) Jaundice
• Prolonged jaundice in newborn may
persistent >1 month.
• Peaks usually day 5-10
• Etiology unknown: All children of given
mother
• Occasionally stop breastfeeding for 12-24
hours – make sure mom pumps during this time
• Milk volume is not the problem.
• Normal stooling.
Phototherapy & Jaundice
• Stressful especially with hospitalization
• Additional fluids may be needed (IVF)
• Determine cause
– Late preterm
– Inefficient milk transfer
– Too sleepy
•
•
•
•
Frequent feeding
Supplementation – SNS, cup, gavage
May need scheduled feedings every 2-3 hours
Mom to pump with hospital grade pump
Gastrointerological
• Reflux
• Pyloric Stenosis
• TE Fistula
• Esophageal atresia
• Gastroschesis
• Chylothorax
• Imperforate anus
Gastrointerological
• Vomit /Reflux
• Persistent vomit after feeds with no pain
• Usually normal growth patterns
• Outgrows within several months
• Reassure mom her milk is perfect
– Breastfeeding implications
• Breastfeed in upright position – modified
football hold
• Keep baby in upright position after feedings
• May benefit from small frequent feedings
Gastrointerological
• Vomit/pyloric stenosis
– Narrowing of pyloric orifice connecting
stomach to intestines
– Usually diagnosed at 2-6 weeks of age
– Vomiting after each feeding often projectile
– Frequent feeding followed by more emesis
– Dehydration and weight loss common
– Surgery required with hospitalization
• Mom to pump while baby NPO/advancing feeds
• Breastfeed after feeding volumes advanced
Gastrointerological Tract
Esophageal Atresia & TE Fistula
• Anomalies occur early in fetal development
• Occur 1 in 1500-4500 live births
• 30-40% infants have additional congenital anomalies
– syndromes
• 5 Classifications based on esophageal configuration
and presence/absence of a fistula
• If fed at birth choke & gag with drooling and
regurgitation from mouth and nose
• Early surgical repair – NPO
• Enteral feedings
• Pumping required for weeks to months
Chylothorax
• Obstruction in lymphatic system
– Congenital
– Surgical
– Chylus fluid accumulates in chest cavity
• Chest tubes
• Dietary management low fat/high protein diet
for several weeks
– Breastfeeding implications
• No breastfeeding for designated time
• Mom to pump to protect supply
• Use of fat free milk
Imperforate Anus
• Large range of congenital defect
– No opening
– Blind rectal pouch just above opening
•
•
•
•
Often associated with other anomalies
Surgical repair with colostomy
NPO until bowel sounds return
Pumping until baby able to tolerate full
volume breastfeeding
• Second surgery and hospitalization to
reconnect/reverse colostomy
Late Preterm Incidence
• Late Preterm defined as 34-36.6 completed weeks gestation
• More than 70% of all pre-term births in the U.S. are LATE PRETERMS. (Martin et al. 2008)
• Late pre-term births have decreased over the past 3 years
• National Statistics for Induction of late-preterms increased
approximately 5% from 1992 to 2002 (NCHS, Final Natality
Data; March of Dimes, April 2006)
• National campaign to decrease elective deliveries before 39
weeks gestation – NICHD
• Intermountain Experience
• 1990 = 10.6% of all live births
• 2006 = 12.8% of all live births
• 2009 = 12.2% of all live births
Natl. Vital Stat Rep 2011
Problems with the Late Preterm
•
•
•
•
•
•
•
•
Thermoregulation
Hypoglycemia
Nutrition
Jaundice
Neurodevelopmental Outcomes
Increased sepsis risk
Hospitalization Longer
Readmissions 5-10 times increased risk!
Readmissions Risk
5 – 10 times increased
risk for readmission due to
– Excessive weight loss
– Jaundice
– Dehydration (hypernatremia)
– Hypoglycemia
– Breastfeeding Failure
(Wight, 2004)
Potential for Lactation Failure
“The first two weeks post
birth pose the greatest
risk for early
breastfeeding failure and
lactation associated
morbidities in the late
pre-term infant and
mother.” (Meier, 2007)
Potential for Lactation Failure
• Immaturity
–
–
–
–
Sleepier
Have less stamina
Increased difficulty with latch
Suck, swallow, breath coordination
• Great Pretenders
– Appear deceptively vigorous
– Receive less observation than should
have
Breastfeeding Management
•Feed the baby!
•Establish and
maintain mother’s
milk supply
Breastfeeding Management
•
•
•
•
•
•
•
•
•
Skin to Skin in delivery room
Avoid Separation
Early unrestricted breastfeeding
Nipple Shield
Formal evaluation
Individualized care plan
Supplementation
Pump to build and protect milk supply
Follow-up
Key Points
• Human milk is the optimum feeding for late preterm
infants
• Late preterm infants may look like term infants, but
physiologically & developmentally they are preterm
infants
• Establishing & maintaining a full milk supply is essential
• Supplementation is usually necessary
• Mother and infant should be kept together as much
as possible
• Anticipatory guidance & close follow up are the keys
to establishing full breastfeeding in late preterms
• Any human milk is better than none! Wight 2008
Post Mature
• Fetal Distress
• Birth trauma
• Birth depression
Post Mature
• Post term = 42 weeks or more
– Birth injury or birth trauma
– May have experienced hypoxia
– May be lethargic with weak suck or
endurance issues
– Prone to hypoglycemia
– Encourage breastfeeding and skin to skin
holding
– Encourage pumping to bring in full milk
supply
Birth Trauma
• Conditions or events that happen at birth
–
–
–
–
Fractured clavicle
Vacuum extraction
Deep aggressive suctioning
Hypoxia
• Encourage breastfeeding
• Creative positioning to reduce pressure
• Pumping after feedings
Birth Depression
• Compromised in utero and/or during
delivery with very low apgar scores
• Hypoxia decreases motility of the gut
• Hold feedings for 48-96 hours
• Pumping until breastfeeding established
• Consult developmental feeding therapy
• Poor suck & uncoordinated feeding –
often feed worse with bottles
Other Diagnosis
• Drug exposed
• IUGR
• Inborn errors of Metabolism
• Chronic grief & loss
Drug Exposed
• Neonatal Abstinence Syndrome (NAS)
–
–
–
–
–
Neurological
Cardiovascular
Gastrointestinal
Musculoskeletal
Other
• Substance abuse rate ~10%+
• Infant with increased risk of IUGR and
premature delivery
• Scoring systems (NNNS)
Drug Exposed
• Swaddle infant, minimize stimulation
• Breastfeeding implications
– Depends on drug: Illicit drugs L5
– Methodone: continue breastfeeding as
concentrations low in human milk and are not
therapeutic
– Opioid: often require pharmacologic therapy
– Hypertonic infant: May thrash at breast, struggle
with latch & clamp down on breast
– Monitor weight & feeding tolerance & sleep
patterns
IUGR (low birthweight)
• Infants at risk for infection, jaundice
and hypoglycemia
• Advantages of human milk include the
same as for preemies
• Much easier to get baby to breast
because of maturity.
Inborn Errors Metabolism
• Over 100 metabolic diseases
• Newborn screen – Utah tests 37 disorders
• PKU 1 in 10,000 to 15,000 births
– Breastfeeding with special metabolic formula
– Frequent monitoring clinical paramaters
• Galactosemia 1 in 40,000 to 60,000 births
– Abrupt weaning necessary due to galactose
in milk
Chronic Grief & Loss
• Every person responds differently to
loss
• Special needs infants may never
exclusively breastfeed which
represents a loss
• Special needs infants often have
complex illness and increased
mortality rates
Chronic Grief & Loss
• Provide education on stopping milk
production when discharging terminal infant
and/or when death has occurred
–
–
–
–
Anticipate needs of mother
Prevention of engorgement
Mother may have frozen milk to donate
Mother may continue pumping and donate to
milk bank
Congenital Conditions Incidence
• Heart & Circulation
• Muscles & skeleton
– Club foot
– Cleft lip/palate
• Genital & Urinary
• Nervous system/eye
– Anecephaly
– Spina Bifida
• Chromosonal syndromes
– Down Syndrome
• Respiratory Tract
• Metabolic Disorders
– PKU
1 in 115 births
1 in 130 births
1 in 735 births
1 in 930 births
1 in 135 births
1 in 235 births
1 in 8000 births
1 in 2000 births
1 in 600 births
1 in 900 births
1 in 900 births
1 in 3500 births
1 in 12,000 births
March of Dimes Perinatal Data Center 2000 (Riordan 2010)
Key Points
• Human milk protects baby - especially
important for special needs infants
• Skin to skin holding helps baby & mom
• Breastfeeding often helps improve
coordination
• Support of baby mouth/jaw helpful
• Try different positions
Key Points cont.
• Breastfeed as often as able
• Pumping essential following
delivery
• Ongoing pumping with hospital grade
electric pump to establish and protect
supply
• These special needs infants require initial
feeding assessment/evaluation and
ongoing feeding support
• Any human milk is better than none!!
Thank you
• To Barbara Wilson-Clay and Kay
Hoover
Slides #4, 6, 7, 10, 15, 17, 21 are from The
Breastfeeding Atlas
Used with permission.