Breastfeeding support for infants with special needs

Marsha Walker, RN, IBCLC
Marshalact@gmail.com
Infants with special needs
 Neurological
 Oro-facial
 Cardiac
 Gastrointestinal
 Late Preterm
 Preterm
 Drug exposed
 Inborn errors of
metabolism
Neurological
 Hypotonic
 Floppy infant syndrome
 Prader-Willi Syndrome (use straddle position with mild head
extension
 Hydrocephalus
 Asphyxia
 Trisomy 13, Trisomy 18, Trisomy 21
 Medullary lesions
 Muscular abnormalities
 Hypertonic
 Cerebral Palsy
 Drug exposed
 Neural tube defects
Neurological
 Hypotonic
 weak suck, poor suction,
ineffective tongue
 Need head support, maternal
breast support
 Nipple shield may help
 Breast compressions, small
milk boluses
 Stimulates central sucking
pattern generator
 Tube feeding device at breast
for flow dependent sucking
 Not too fast
 Not too slow
 Finger feeding
 May need to do pre and post-
feed weights
Techniques to initiate sucking,
feed, and supplement
Photos from Breastfeeding an illustrated
guide to diagnosis and treatment Denise
Both and Kerri Frischknecht)
Hypertonic
 Hypersensitive, sensitive
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gag, reflux, arch at breast,
retract and/or thrust
tongue,
Clamp/chomping
Swaddling, sling to bring
arms midline & shoulders
forward
Craniosacral therapy
Avoid direct pressure on
the back of the baby’s head
Placing baby on pillow and
allowing to nurse in
extended position
Trisomy 21 (Down syndrome)
 Small mouth, macroglossia
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and hypotonia (90%),
small mandible
Congenital heart disease,
GI anomalies
Weak suck, poor seal,
tongue protrusion
Lax ligaments of 1st 2
cervical vertebrae that puts
pressure on brain stem or
spinal cord with excessive
head flexion or extension
Encourage skin to skin
holding
Down syndrome and breastfeeding
 Ventral position for breastfeeding
 Dancer hand position for jaw support
 Gentle tapping and massage around the lips
 Cause milk ejection first before placing baby to
breast
 Nipple shield if necessary
 Frequent feedings for inability to sustain sucking
 Close monitoring weight gain
 Pumping may be needed to build/protect milk
supply
 Hindmilk supplements if necessary
Down syndrome
 Some infants may feed better if legs and arms are in
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extension
Alternate massage
Perioral muscles, lips, and muscles of mastication are
hypotonic
 Use of a bottle further weakens these muscles
 Breastfeeding strengthens these muscles
 Bottles contribute to narrowing of the palate to which
these babies are already prone
Avoid pacifiers as they may mask hunger cues
May see tremors of the jaw and tongue at start of
feedings
Trisomy 21 has different growth charts
Neural tube defects
 Congenital anatomical abnormalities of the brain and
spine
 Spina bifida (Myelomeningocele) most common
 Saclike casing with cerebral spinal fluid, spinal cord & nerve
roots that have herniated through a defect in vertebral arches
and dura
 Spina bifida occulta involves the lesion being covered by skin
without herniation and may present as a hairy patch, dermal
sinus tract, dimple, hemangioma in the thoracic, lumbar or
sacral regions
 Infant may be latex sensitive
 Chiari II malformations
 herniation of brain stem below foramen magnum and can be
a surgical emergency
 Hydrocephalus
Neural tube defects
 Most children are able to breastfeed (unless significant
brainstem involvement-Chiari II)
 Watch for stridor, poor suck, apnea, swallowing
problems, arching, absent cry
 Challenge to position at breast post operatively
 Baby may be prone, flat on back/side for several days
with flexion of the spine impossible
 May not be able to be burped on back (rock or rub
shoulders)
 Mother may still be in hospital
 Express milk prior to surgery if possible
Positioning post surgery
 Mother can lie on her side next to the infant to feed if
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baby is prone or on his side
If baby is supine, mother can lean over the infant
bringing to breast to the baby rather than the infant to
breast
Slide the infant on pillows into the mother’s lap
Baby may gag easily
Be on alert for a Chiari crisis (weak or absent cry,
stridor, apnea & color changes, swallowing problems,
arching of the neck, reflux, failure to thrive)
If significant brain stem involvement, feeding at breast
may be difficult or impossible
Hydrocephalus
 Accumulation of fluid in the cerebral ventricles
 May be congenital, isolated, from IVH or associated
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with myelomeningocele
Infant’s head enlarges
Irritability, weakness,
Neurologic defects vary but can be severe
Surgery to place shunt diverts cerebral spinal fluid to
peritoneal cavity
White of the eye showing above the iris (“setting sun
sign”)
Hydrocephalus
 Positioning key to support
 Upright positioning with
pressure off of the shunt
 Be aware of head elevation
limitations postoperatively
 Mother can lean over
supine infant bringing
breast to baby
 Infant may have weak suck
and tire easily with
increased risk for
aspiration
Pierre Robin Sequence
 Micrognathia or retrognathia (small jaw; receding
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chin)
Glossoptosis-tongue may have tendency to fall back
and block airway
Wide U-shaped cleft palate
Occurs in 1 out of 8850 births
80% of infants with PRS have another syndrome:
 Stickler syndrome (40%)
 Velo-cardio-facial syndrome (15%)
Pierre Robin Sequence
 Primary problem is airway
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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
O2 saturation monitored
while feeding
Choanal atresia
 Congenital anomaly of the
anterior skull with blockage
or narrowing of the nasal
airway
 Occurs in 1 out of 7000-8000
births
 Unilateral choanal atresia
may be asymptomatic until
respiratory illness
 Symptoms:
 difficulty coordinating
breathing while suckling
(sputtering, choking,
coughing)
 mouth breathing
 chest retractions
 Circumoral cyanosis
Choanal atresia
 Surgery for placement of airway followed by stents
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placed to maintain integrity of airway
If both airways are blocked very early surgery
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
May be associated as a component of CHARGE
syndrome (group of anomalies may be seen as weak
sucking, swallowing difficulty, reflux, aspiration)
May see oral defensiveness or aversion
Congenital heart disease
 Most common structural birth defect with overall
incidence of 1%
 May be part of many congenital syndromes
 CHARGE, Down Syndrome
 Watch for infant during feeding for cyanosis/hypoxia,
fatigue, poor suck, tachypnea, & uncoordinated sucking
patterns
 Congestive heart failure could be noted shortly after birth
or could be weeks after discharge
 AAP recommends screening/detection for CHD by pulse
oximetry after 24 hours of age and prior to hospital
discharge
 Some infants with cardiac disease may still be missed
Congenital heart disease
 Breastfeeding Management depends on severity of heart
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disease and failure
Baby may tire quickly
May become tachypneic while feeding
Watch for color changes
May see frequent pauses to rest with lengthy feedings
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
Monitor weight
Congenital heart disease
 The “work” of breast-feeding is actually less than the
work of bottle-feeding.
 Sucking, swallowing and breathing are easier for a
baby to coordinate, and the amount of oxygen
available to the baby is greater while breast-feeding
than when bottle-feeding
 In general, when compared to bottle-fed babies,
breast-fed babies with congenital heart defects have
more consistent weight gain because it is not as
physiologically taxing
Problems with weight gain
 Could stem from
 Congestive heart failure with associated congestion in the gut
(anorexic or nauseated due to effects on gut motility or from
medications)
 Reflux is common
 Energy needs may exceed 110-110kcal/kg/d and may actually
need more like 140-160 kcal/kg/d due to increased respiratory
effort and circulating stress hormones
 Fluid restriction
 Infants with hypoplastic left heart syndrome post surgery may
have difficulty swallowing and be at increased risk for NEC
Breastfeeding and CHD
 More extended or upright positioning rather than
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flexion to avoid placing pressure on a distended or
sensitive liver
Hindmilk supplements with nursing supplementer
Use alternate massage (breast compressions) to
sustain sucking
Nipple shield
Skin-to-skin care pre and post operatively
Shorter more frequent feeds may help increase the fat
content of the milk
Congenital heart disease
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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
Gastrointestinal
 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
 Keep baby in upright position after feedings
 May benefit from small frequent feedings
Pyloric stenosis
 Narrowing of pyloric orifice connecting stomach to
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intestines
Exposure of infants in the first 2 weeks of life to oral
azithromycin (or erythromycin) increases risk for
pyloric stenosis, Such infants should be monitored
Usually diagnosed at 2-6 weeks of age
Vomiting after each feeding often projectile
Frequent feeding followed by more emesis
Dehydration and weight loss are possible
If severe–surgery may be required with hospitalization
Mom to pump while baby NPO/advancing feeds
Breastfeed after recovery from anesthesia
Esophageal Atresia & TE Fistula
 Anomalies occur early in
fetal development
 Occur 1 in 1500-4500 live
births
 30-40% infants have
additional congenital
anomalies or syndromes
 5 Classifications based
on esophageal
configuration and
presence/absence of a
fistula
Esophageal Atresia & TE Fistula
 Classic symptoms are evident shortly after birth
 Copious white frothy bubbles of mucous at the mouth
 Noisy respirations
 Coughing, choking, cyanosis which worsen when fed
 If fed at birth choke & gag with drooling and regurgitation
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from mouth and nose
Early surgical repair
Pumping required for weeks to months
May demonstrate aversive behavior when finally put to the
breast
Sham breastfeedings may be done with milk draining out
of a stoma to experience pleasurable feeds at the breast
GER is common so best feeding position may be
completely upright with infant straddling mother’s thigh
Chylothorax
 Obstruction in lymphatic system due to congenital
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anomalies or secondary to injury in post thoracic
surgery
Extravasation of chylous lymph of the lymphatic
system into the pleural space
Chylus (lymph and emulsified fat secreted from
intestine) fluid accumulates in chest cavity
Chest tubes
Octreotide IV medication to reduce lymph production
Dietary management low fat (medium chain
triglycerides)/high protein diet for several weeks
Chylothorax
 Average time to introduction
of normal diet is 9 days
 Mom to pump to protect
supply
 Use of modified breastmilk
during that time
 Remove fat from
breastmilk
 Use modified milk until the
thoracic duct is healed and
chylous effusion ceases
 Milk was centrifuged at 3000
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r.p.m. for 15 min at 2 degrees C
After centrifugation, the milk
separated into a solidified-fat
top layer and a lower liquid
portion
The fat-free liquid portion was
then poured into collection cups
and frozen for the patient's use
at a later date
A sample of the mother's milk
before and after processing was
stored and analyzed for fat,
sodium, potassium, calcium and
zinc
Chan & Lechtenberg. J Perinatol
2007; 27:434-436
A population at risk
(Adamkin, 2006; Engle et al, 2007).
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airway instability
apnea
bradycardia
excessive sleepiness
large weight loss
dehydration
feeding difficulties
weak sucking
jaundice
hypoglycemia
hypothermia
immature self
regulation
 respiratory distress,
 sepsis,
 prolonged formula
supplementation,
 hospital readmission,
 breastfeeding failure
 Newborn morbidity
rate doubles in infants
for each gestational
week earlier than 38
weeks
Breastmilk protection
• Provision of human milk is important to
infants born preterm as these babies have a
lower antioxidant capacity.
• May be why they are so vulnerable to
diseases and conditions associated with
oxidative stress such as necrotizing
enterocolitis, chronic lung disease,
retinopathy of prematurity, periventricular
leukomalacia, and intraventricular
hemorrhage.
• Breastmilk is much higher in antioxidant
capacity than infant formula and helps
neutralize oxidative stress on young babies
(Ezaki et al, 2008).
Importance of the last 6 weeks
 Brain weights at 34 and 36 weeks are 65% and 80%
of brain weights at term affecting such functions as
arousal, sleep-wake behavior, and the coordination
of feeding with breathing.
 1/3 of brain growth occurs in the last 6-8 weeks of
gestation
 The immature brainstem adversely impacts upper
airway and lung volume control, laryngeal reflexes,
and the chemical control of breathing and sleep
mechanisms, with 10% of these infants
experiencing significant apnea of prematurity
(Darnall et al, 2006).
Fetal and Neonatal
Brain Development
Volpe, Neurology of the
Newborn, 3rd Ed, 1995
Inadequate Milk Intake
 Depressed sucking pressures
 Baby uses suction to draw nipple into mouth
 Needs -50 to -60 mm Hg during pauses to keep
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nipple in mouth; can explain why baby keeps
slipping off breast
 Depends on expression to extract milk
Tire easily at breast/reduced endurance
Reduced intake per feed
Insufficient feeds per 24 hours
Reduced maternal milk supply
Disorganized suck
Long periods of sleep
Maternal health problems/separation
Babies do not consume milk from the breast simply
because it is there
Immediate Postpartum Care
 Skin-to-skin contact
Physiologic stability
Provides warmth
Proximity to breasts
Improves oxygenation
Decreases crying
Does not interrupt
initial breast-seeking
behaviors
 Avoids hypoglycemia
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Positioning Late Preterm Infants
 Position infant for
maximal lung
expansion, head
slightly extended for
open airway
 Assure that the head is
stable, in straight
alignment with neck
and hips
In-hospital feeding plan
Place baby skin to skin on your chest
Watch for rapid eye movements under the eyelids
Feed your baby frequently
• within 1 hour after birth
• once every hour for the next 3 to 4 hours
• every 2 to 3 hours until 12 hours of age
• at least 8 times each 24 hours during the hospital stay
Move baby to breast when baby shows feeding cues
􀂄􀂄 Sucking movements of the mouth and tongue
􀂄􀂄 Rapid eye movements under the eyelids
􀂄􀂄 Hand-to-mouth movements
􀂄􀂄 Body movements
􀂄􀂄 Small sounds
Make sure you know how to tell when your baby is swallowing
• baby’s jaw drops and holds for a second
• you hear a “ca” sound
• you feel a drawing action on the areola and see it move towards
your baby’s mouth
• you hear the baby swallow
•you feel the swallow when you place a finger on the baby’s throat
•your nurse hears the swallow when a stethoscope is placed on the
baby’s throat
Use alternate massage if your baby doesn’t swallow after every 1 to 3
sucks.
Massage and squeeze the breast each time she stops between sucks. This
helps get more colostrum into her and keeps her sucking longer.
If your baby does not swallow when at the breast, hand
express colostrum into a teaspoon and spoon feed 2
teaspoons to your baby using the above guidelines
Alternative feeding methods
Morton et al. Five steps to improve bedside breastfeeding care.
Nursing for Women’s Health 2014; 17:478-488
Cup feeding helps increase
breastfeeding likelihood
 32-35 weeks
 268 bottle supplemented, 254 cup
supplemented
 Cup feeding increased exclusive
breastfeeding on discharge and at 3 and 6
months
 Did not increase length of hospital stay
 Yilmaz et al. J Hum Lact 2014; 30:174-179.
Importance of human milk for the
preterm infant
 Reduces risk of: enteral
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feeding intolerance
Nosocomial infections
Necrotizing enterocolitis
Chronic lung disease
Retinopathy of
prematurity
Developmental and
neurocognitive delay
Re-hospitalization after
NICU discharge
Reduces risk of oxidative
stress which contributes to
many of these conditions
Preterm babies 11.39% in 2013
Supportive care in the NICU
 Provide evidence-based information about
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breastfeeding, breastmilk, and infant formula for
informed decision making
Communicate staff’s value of breastfeeding
Have a written breastfeeding policy communicated to
and followed by all staff
Provide current and consistent
breastfeeding/pumping guidelines
Involve the mother in all feeding plans
Encourage mothers to assume responsibility for
feeding tasks such as performing pre- and postfeed
weights and fractionating their milk
Teach, assess, and monitor milk expression, storage,
and transport
Supportive care in the NICU
 Initiate skin-to-skin care (kangaroo care)
 Introduce the breast early with frequent learning
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opportunities
Use a demand or semi-demand breastfeeding strategy
Teach positioning, assess latch, sucking, and swallowing
Use assistive devices as needed
Measure milk transfer
Supplement without bottles if possible
Support the father’s presence and provide guidelines for his
help with breastfeeding
Create a feeding plan for the post-discharge period
Refer parents to community sources for breastfeeding
support
Milk expression in the NICU
 Secure effective breast pump
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with double pumping and
correctly fitted flange
Begin milk expression within
1 hour of birth
Hand express x5/d + pump
x5/d during first 3 days
Use breast compressions
while pumping
Encourage skin-to-skin care
Oxytocin nasal spray for
impaired let down
Power pumping
Laughter while pumping
 Use a pumping log
 Aim for high milk production
by 14 days
 800-1,000 mL per day
 Adequate milk production
may be achieved if 3,500 mL
per week is achieved by week
2
 Address low milk supply
immediately
 Recombinant human
prolactin
 Music while pumping
 Warmed pump flanges or
warm compresses to breasts
Oropharyngeal colostrum (mouth
care) application
 Colostrum placed or
swabbed around infant’s
mouth (.2 mL q3h for 72
hours)
 Immune factors absorbed
through oral mucosa
 Decrease clinical sepsis,
inhibit secretion of proinflammatory cytokines,
and increase levels of
circulating immuneprotective factors
 Lee et al. Pediatrics 2015;
135:e357-e366
Transitioning to feeding at breast
 Practice sessions during skin
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to-skin
Cross cradle or clutch
positioning
Dancer hand position
Weak vacuum may be helped
with nipple shield
Supplemental feedings
 Nasogastric tube with
pacifier
 Cue based feedings when
consuming more than 50% at
breast and/or with
supplementation
 Pre and post feed weights
 Individualized milk
fortification
 Targeted-milk is analyzed
and fortified up to a target
nutrient intake which is
predefined requirements of
preterm infants
 Adjustable fortificationprotein intake adjusted
according to infant’s
metabolic response
 Increasing volume per
feeding at breast
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Alternate massage
Initiate MER 1st
Nipple shield
Supplementing at breast
with tube feeding device
Drug exposed infants
 Neonatal Abstinence Syndrome (NAS)
 Neurological
 Cardiovascular
 Gastrointestinal
 Musculoskeletal
 May be hypertonic, irritable, show abnormal
movements, be hypersensitive, thrash at the breast,
clamp down on the nipple, unable to modulate their
state very well, be difficult to position at breast, pull
back from the breast if experiencing nasal stuffiness
 May ingest lower volume of milk per suck
 May demonstrate less rhythmical swallowing
Feeding interventions
 Feed when in a drowsy
 May need alternative
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state
Swaddle with vertical
rocking
Tame the environment
Gradual oral stimulation
Wrap baby in soft blanket
to restrain thrashing
movements
Use ventral positioning
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feeding devices
Mother may need to pump
milk
Feed in a dim, quiet room
For nasal stuffiness- gentle
nasal suctioning with
saline drops
Small frequent feedings
Soft talking, gentle
handling, skin-to-skin
contact
Inborn errors of metabolism
PKU
 Over 100 metabolic diseases
 Newborn screen
 PKU 1 in 10,000 to 15,000
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births
Breastfeeding combined with
special metabolic formula
Frequent monitoring clinical
parameters
Babies breastfed prior to
diagnosis show improved
neurodevelopment
362 mL/d (1st month) to 464
mL/d (4th month) of
breastmilk in addition to low
PHE formula grow and
develop well
 BF can alternate with PHE-
free formula feeds with
number of feedings at breast
adapted to plasma PHE
concentrations
 Mothers can do pre and post
feed weight following BF to
determine amount of milk
consumed and calculate how
much formula for next
feeding
 PKU infants may be more
susceptible to thrush
Galactosemia
 Galactosemia 1 in 40,000 to
60,000 births
 Symptoms may start
around 3rd day of life
 Jaundice, enlarged liver,
vomiting, poor feeding,
poor weight gain, lethargy,
irritability
 When testing indicates
possible galactosemia BF is
stopped
 Mothers should continue
to pump until confirming
test determines classic or
Duarte variation
 Duarte variation may show
varying levels of enzyme
activity
 Depending on levels of
galactose-1-phosphate in
the blood some babies may
be able to partially or
totally breastfeed
 Mothers may be able to
alternate breastfeeds with
soy based formula