Your Health Coach has prepared this information packet for your family to help explain the medical needs of your newborn as you prepare to leave the hospital. A Health Coach helps families/ caregivers adjust to working directly with the health care providers as well as increasing your ability and confidence to care for your infant.

When infants are born before their due date or with health problems, families often need help managing their baby’s health care after leaving the hospital. Since they spend the first part of their lives in the hospital, the babies and their families may find it helpful to have extra support.

Your Health Coach will work with your family to teach you to care for your infant, connect with the right doctors and specialists for treatment, and find resources in your area. The role of the Health Coach is as an educator, not as a caregiver.

Planning must start before hospital discharge and continue through followup with the primary care provider. After discharge from the hospital, your infant’s care must be well coordinated and clearly communicated to avoid medical errors that could harm the baby.

After your infant is discharged, your Health Coach should follow up with you by phone within a few days to make sure the transition is going well. Your Health Coach will want to know how your baby is doing, whether you have any questions or concerns, if your infant has been to the scheduled appointments, etc.

NICU Needs Assessment

Tips for Finding a Pediatrician

• Your family will need to visit a pediatrician within the first week after leaving the hospital.
• Plan ahead to avoid the pressure and frustration of finding a pediatrician right before your baby is discharged from the hospital.
• Ask your Health Coach or social worker if you need help finding a doctor for your baby.
• Decide whether the pediatrician's office needs to be close to your home, job, or day care.
• Call the pediatrician's office to ask if they accept your current health insurance and if they are accepting new patients.
• Ask the pediatrician's office what your co-payment and responsibilities are.
• Ask the pediatrician's office which doctor your baby will see at every visit.
• Ask the pediatrician's office if a doctor is on-call 24 hours a day for emergencies.

Tips About Health Insurance

Your baby will need to visit the doctor regularly after birth.

You have only 30 days to add your newborn to your insurance plan. Call your current insurance company as soon as possible to add your newborn.

Ask your social worker if you qualify for Medicaid, and apply while your baby is still in the hospital.

Ask about Children's Medicaid (CHIP), Project Medical Home, and other low-cost health care benefits if you do not qualify for Medicaid.

Common Causes

• Hunger.

• Pain.

• Sleepy.

• Clothes too tight.

• Soiled diaper.

• Too hot or too cold.

Call your doctor if:

• Your baby looks or acts sick (is throwing up, appears to be in pain).

• You are exhausted from the crying and fear you might hurt your baby.

• Unexplained crying for 3 or more hours a day.
• Excessive crying.
• Usually begins suddenly and for no clear reason.
• Difficult to comfort.
• Usually goes away as your baby gets older than 3 months of age.

• Hold and soothe your baby.
• Provide gentle rocking motion in a rocking chair, cradle, or by slow dancing with your baby.
• Place your baby in a swing or vibrating chair.
• Give your baby a warm bath.
• Take your baby for a ride in a stroller or car.
• Feed your baby if it has been more than 2 hours since the last feeding.
• If you have held your crying baby for more than 30 minutes, put him on his back to sleep.

Remember that colic is normal in a lot of babies and is simply part of their personality. It has nothing to do with your parenting or any illness.

It is important to remain calm and never shake your baby.

Everyone must wash their hands or use hand sanitizer before and after they touch your baby.

• Avoid large crowds.
• Avoid sick people.
• Have people cover their mouth and nose with a tissue when they cough or sneeze.
• Do not allow anyone to smoke around the baby.

What is Respiratory Syncytial Virus (RSV)?

• A common virus that affects babies and infants.
• Leading cause of two lung infections: pneumonia and bronchitis.

Symptoms of RSV

• Starts out like a cold with fever or runny nose.
• Can also include:
  • Coughing.
  • Problems breathing.
  • Fast breathing.
  • Not eating well.

How is RSV Spread?

• Contact with someone who is coughing or sneezing.
• Enters the body through the eyes, nose, or mouth.

How to Prevent the Spread of RSV?

• Wash your hands before and after handling a baby.
• Avoid exposing your baby to others with cold symptoms.
• Cover coughs/sneezes and throw away used tissues.
• Keep your baby away from crowded areas.
• If your baby is at high risk for RSV, talk to your doctor about a monthly shot that can help lower the risk of a baby getting severe RSV.
• Your baby may be eligible for palivizumab (brand name: Synagis®), a treatment given to prevent and reduce RSV.
• The shot, given monthly during RSV season, reduces the chance of your baby getting pneumonia and bronchitis.
• Babies may still get RSV but will be less sick.

What is an immunization?

Immunizations protect us from getting sick with serious diseases. Often, they are given by a shot, but sometimes they are given by mouth or nose.

Premature babies

Immunizations are given to premature babies at the same age as full-term infants.

At birth

• Hepatitis B Vaccine:
  • First dose is usually given at birth before leaving the hospital.
  • Should complete the doses by 6-18 months of age.

6-8 weeks old

• Diphtheria and tetanus and pertussis vaccine (DTaP).
• Haemophilus influenzae vaccine (Hib).
• Pneumococcal vaccine.
• Poliovirus vaccine (IPV).

Respiratory Syncytial Virus (RSV)

Your child may be eligible for a treatment to prevent RSV, a virus that can cause serious lung infections. Ask your baby's doctor.

Benefits of vaccines

• Vaccines prepare your baby's body to fight illness.
• Vaccines protect you and others around you.
• It cost less to prevent a disease than to treat it.

Are your child's immunizations up to date?

You can find out more about which immunizations babies need on the Centers for Disease Control and Prevention's (CDC's) Web site http://www.cdc.gov/vaccines/.

• Keep a list of your baby's medications with your baby at all times.
• Speak to your baby's health care professional, and ask specific questions about your baby's medicines.
• When picking up refills, check the label carefully for correct medicine, strength, and dose.
• Read over-the-counter labels to make sure the product is appropriate for your child's age.
• Use medicines only as directed.
• Use the measuring devices that come with the products, and use these devices as instructed.
• Keep medicine bottles away from other small children.
• Never allow a child to drink directly from a medicine bottle.

Know the name of the medicine and what it is for

• Carefully read the label of each medicine before giving it to your baby.
• Know why your baby needs to take this medicine.

Know the dose

• It is best to use an oral syringe to give liquid medicine to a baby.
• Avoid using teaspoons because they are not precise.
• Ask the pharmacist to mark the syringe at the dose you need to give.
• Keep the syringe with the corresponding bottle and color code them.

Know how often to give the medicine

Ask your baby's doctor or the pharmacist what to do in case you miss a dose.

Know possible side effects

• Always observe your baby for any bad reactions to the medicine.
• Call your baby's doctor in case of a bad reaction.

Use the following chart to convert teaspoons to milliliters

• Visit the NICU often, and help care for your baby as much as possible in the hospital.
• Learn how to use equipment: pulse oximeter, oxygen tank, nasal canula.
• Know the signs of difficulty breathing.
• Learn CPR.
• Learn how to give your child’s medicines.
• Plan ahead for extra help at home.
• Schedule routine followup doctor visits.
• Wash your hands frequently.
• Avoid crowds.
• Have your child immunized.

Characteristics

• Need for supplemental oxygen at 36 weeks postmenstrual age, with radiographic changes on chest x-ray (bilateral, diffuse hazy lungs; interstitial thickening; increased lung inflation).
• Symptoms: tachypnea, increased work of breathing, chronic wheeze or cough, oxygen dependence, +/- ventilator dependence, hypercarbia with compensated respiratory acidosis.
• Course characterized by acute exacerbations of respiratory distress.
• Overall incidence is unchanged due to increased survival of extremely premature infants.(~7500 new cases/year) but is decreased with surfactant and early extubation to NCPAP.
• Risk factors: premature birth, respiratory failure, O₂supplementation, mechanical ventilation, air leaks, pulmonary edema, PDA ligation, infection causing lung injury, lung hypoplasia.

Treatment

• Oxygen (see home oxygen topic sheet).
• Fluid restriction and diuretics:
  • Use of diuretics is reserved for severe BPD and evidence of diuretic responsive disease.
  • Furosemide, chlorothiazide, spironolactone (see medication sheet for dosing).
  • May need KCl or NaCl supplements to correct electrolyte deficiencies.
  • Chronic furosemide use improves both oxygenation and lung compliance, but has not decreased length of hospital stay or need for oxygen therapy.
• Inhaled bronchodilators or corticosteroids (see medication sheet for dosing).
  • Only for infants that show evidence of reversible airway obstruction.
  • Only patients that have a family history of asthma and have episodic attacks similar to asthma have been shown to have any benefit from inhaled steroids.
• RSV immunoprophylaxis; influenza and pneumococcus vaccinations.
• Higher caloric intake needed to account for increased work of breathing, as well as ongoing tissue repair and tissue deposition; due to fluid restrictions, these patients may need fortified feeds to maintain an adequate growth rate.

Monitoring

• Monitor O₂ saturations, resting respiratory rate and effort, presence of retractions, and color.
• Watch for adequate weight gain (15-30 g/day).
  • If inadequate, may be due to insufficient caloric intake or hypoxemia.
• Watch for excessive fluid retention (tachypnea, retractions, rales, excessive weight gain, enlarged liver, poor feeding, O₂ saturation <92%).
• Watch neurodevelopment closely.

Outcomes

• 50% with CLD require rehospitalization in the first year, and 37% in the second year of life.
• More prone to: frequent lower respiratory tract illnesses, feeding difficulties, growth failure, rehospitalization during infancy.
• Despite treatment, approximately 10% of these children die in the first year of life.
• Increased risk for cognitive, motor, and language impairment, hearing loss, and poor academic performance.
• Lung function generally remains reduced until adolescence, at which point it approaches normal for age; in some, pulmonary function will again deteriorate in adulthood.
• Children with CLD are more likely to develop asthma and require bronchodilator therapy.

What is Bronchopulmonary Dysplasia (BPD)?

• Type of chronic lung disease.
• Common in babies born early.
• Damaged lung tissue causes breathing and health problems.
• Lungs trap air, fill with fluid, and produce extra mucus.

What Causes BPD?

• Being born early.
• Having a virus called RSV (ask your Health Coach for a fact sheet about RSV).
• Having a heart condition.
• Being on a ventilator.
• Lack of nourishment.
• Fluid in the lungs.

What are the Symptoms of BPD?

• Grunting.
• Breathing heavily.
• Flaring nostrils.
• Sucking in air.
• Tiring easily.
• Pale or grey skin.

How is BPD Diagnosed?

• If your baby still needs oxygen at 36 weeks old.
• If your baby has been on a ventilator.

How is BPD Treated?

• BPD is treated with oxygen to control fluid in the body and medicine to relax the airway.
• Treatment does not cure BPD.
• Treatment helps your baby breathe better.
• Lungs will eventually heal.
• Your baby needs nutrients for healthy growth.

Characteristics

• Respiratory pause for at least 20 seconds, or a pause that is accompanied by bradycardia (heart rate <100 bpm), cyanosis, or pallor in an infant <37 weeks postmenstrual age (PMA).
• Types of apnea:
  • Central: the respiratory center in the immature brain stem does not trigger inspiration; frequently responds to methylxanthine treatment.
  • Obstructive: due to impaired tone of larynx/pharynx.
  • Mixed: combination of the two; most common type found in neonates.
• Can persist for many weeks in very preterm infants, although resolution typically occurs by 37-43 weeks PMA (resolution tends to be later for extremely low-birthweight infants).
• Periodic breathing: recurrent sequences of pauses in respiration lasting 5-10 seconds, followed by 10-15 seconds of rapid breathing without bradycardia or other symptoms.
• Benign apnea: isolated apneas of 5-10 seconds, without bradycardia or color change; resolve spontaneously.

Home Treatment

• Most infants still having apnea are kept hospitalized; few may be discharged home.
• Methylxanthines: act primarily on the brainstem respiratory structures, producing a central stimulatory effect.
  • Caffeine is most commonly used due to its broader therapeutic index and slower excretion rate than theophylline.

Cardiorespiratory Home Monitors

• Infants are generally monitored for a 5- to 7-day apnea-free period after discontinuing methylxanthine therapy before sending an infant home without a monitor.
• Home monitors are rarely indicated for the detection of apnea, because infants with immature respiratory systems are generally still hospitalized until they are no longer at risk for apnea of prematurity.
• Any home cardiorespiratory monitor used must have download capability.
• Monitoring may be prescribed for some preterm infants with an unusually prolonged course of recurrent, extreme apnea.
• Monitoring should be limited to approximately 43 weeks' PMA or after the cessation of extreme episodes, whichever comes last.

Additional Factors

• Preterm infants are known to be at higher risk for SIDS; high-risk period lasts up to 10 months.
• As "a causal relationship between prolonged apnea and SIDS has not been established," home cardiorespiratory monitoring should not be used to prevent SIDS (AAP Task Force on Prolonged Infantile Apnea, 2003).
• Premature infants beyond the immediate neonatal period can experience apnea following ketamine sedation or general anesthesia; they should have cardiorespiratory monitoring in hospital for 24 hours after surgery.

SIDS

• Preterm infants are known to be at higher risk for SIDS; high-risk period lasts up to 10 months.
• To reduce risk:
  • Use supine positioning on a firm mattress with no fluffy or loose bedding.
  • Be sure the infant's face remains uncovered.
  • Do not allow smoking in the household.
  • Support breastfeeding through the first year of life.
  • Consider offering a pacifier to infants >1 month of age at naptime.

Sleep States

• Active sleep:
  • Respiration is uneven and primarily costal in nature; REM occurs intermittently.
  • Sporadic motor movements may occur with low muscle tone between these movements.
  • May have a functional role in stimulating the nervous system, encouraging growth and maturation.
• Quiet sleep:
  • Respiration is regular and abdominal in nature.
  • Maintains tonic level of muscle tone.
  • Motor activity is limited to occasional startles, sighs, or other brief movements.

Development

• As development progresses:
  • Awake time and quiet sleep time increases, and active sleep without REM decreases.
• Newborn infant spends 2/3 of each day asleep; by 6 months only half of each day is spent asleep.
  • Sleep consolidation is an important aspect of the infant's sleep development.
• Infants after 28 weeks corrected postmenstrual age may benefit from circadian lighting cycle.
  • Studies have shown that these benefits are most pronounced after discharge and persist for several months:
    • More organized and longer sleep cycles.
    • Improvements in weight gain.

Sleep Patterns and Cognitive Development

• Development of sleep-wake cycles likely has major impact on development of most infant behaviors.
• Term infants who sleep for long, quiet periods early in the night and infants who sleep for longer periods uninterruptedly are more likely to obtain high scores on the Bayley mental scale at 6 months and 1 year of age.
• Sleep problems are more common in preterm infants, especially in the early weeks at home.
  • Caused by: immature sleep-wake cycles, disruptions in sleep patterns caused by the need to give medications, and heightened parental concerns about infant's vulnerability.
  • If severe, may benefit from sleep training or referral to sleep specialist.
• To help the infant's state regulation, swaddle the infant, keeping the infant's arms and legs close to the body, and avoid sudden movements.

What is SIDS?

• "SIDS" stands for Sudden Infant Death Syndrome.
• Sudden, unexplained death of an infant less than 1 year old.

What should I know about SIDS?

• Babies sleep safer on their backs.
• Babies should be placed on a firm sleep surface.

What can I do to lower my baby's risk of SIDS?

• Babies should be put on their backs to sleep for nap and at night.
• Keep toys, objects, and loose bedding out of your baby's sleep area.
• Do not allow smoking around your baby.
• Do not allow your baby to overheat during sleeping.

What about "tummy time"?

• Daily tummy time is necessary for normal development.
• Make sure your baby spends several hours on their tummy when they are awake and someone is watching.

Bottle Feeding

• Feed your baby ONLY infant formula and breast milk for their first 4 to 6 months.
• Feed your baby at least every 3 hours, day and night.
• Before each feeding, warm the breast milk or formula to room temperature by placing the bottle in warm water; do not leave cold bottles on the counter to warm up.
• Never heat breast milk or formula in a microwave oven.
• Throw away any remaining breast milk or formula after each feeding.
• When traveling, keep the breast milk or formula cold in a cooler

Formula Preparation

Infant formulas are available in 3 ways:

• Ready to Feed
  • Do not add water.
• Liquid Concentrate
  • Add sterile water.
• Powder
  • Add sterile water.

To make Sterile Water:

• Boil water for 2 minutes.
• Cover the pot.
• Let water cool to room temperature.

Formula Storage and Use

• Store prepared formula in a refrigerator.
• Use formula in 24 to 48 hours.

Bottle Cleaning

• Clean bottles and nipples by washing with hot, soapy water or on top rack of dishwasher.
• Allow bottles and nipples to air dry.

Breastfed babies have:

• Fewer ear infections.
• Lower chance of asthma, food allergies, and dental cavities.
• Protection against diarrhea, stomach, and lung infections.
• Better nervous system development and higher IQ levels.
• Lower risk of some childhood cancers.
• Lower chance of becoming over weight.

Mothers who breastfeed have:

• Lower risk of pre-menopausal breast cancer.
• Lower chance of osteoporosis later in life.
• Quicker return to pre-pregnancy weight.
• Food source for their babies even during emergencies.
• Lower chance of becoming pregnant before menstruation returns.

Benefits

Optimal food for almost all infants regardless of gestational age.

Has been shown to improve subsequent mental development in term/preterm infants as well as provide enhanced protection against infection.

Premature infants with BW<1800-2000g are supplemented with human milk fortifier while hospitalized to provide adequate calories, protein, and nutrients for growth and development.

Human milk fortifier is not recommended after discharge.

Mothers of premature babies produce milk with significantly higher concentrations of lipids, proteins, sodium chloride, iron, anti-infective properties (e.g., IgA), and neuroprotective properties for approximately 4-6 weeks following birth.

Nutritional composition of breast milk varies greatly among mothers.

Recommendations

Slow feeding and easy fatigue are common problems after discharge.

Feed on demand every 1.5-3 hours; preterm infants may require a schedule for feedings (e.g., every 3 hours).

If supplementation is required at discharge due to prematurity, poor growth, inadequate volume intake, or fluid restriction: – 2-4 feedings per day with premature transitional formula (22kcal/oz), and remainder as breastfeeding (preferred method); OR – Add premature transitional formula powder (Enfamil EnfaCare Lipil 22 or Similac NeoSure 22) to expressed breast milk to make 24-30 kcal/oz (call dietitian for recommendations and recipe).

Infants <34 weeks or BW<1800g should receive a multivitamin (1mL/day) and an iron supplement (2 mg/kg/day) for the first year of life (can be given as 1mL of MVI with Fe).

All term breastfeeding infants should receive Vitamin D (400 IU/day) as 1mL of MVI.

Infants who have birthweight <1500g and are discharged on unfortified human milk may be at risk for nutritional insufficiency (growth failure and metabolic bone disease). – Evaluate at 2-4 weeks post-discharge and as needed thereafter: weight, length, FOC, serum phosphorous and alkaline phosphatase activity. – If poor growth or abnormal laboratory results, recommend neonatal dietitian consult to assess need for supplementation or further evaluation.

Growth Outcomes

Very low-birthweight infants fed human milk initially have slower early growth, but have improved Bayley mental developmental index scores.

Early catch-up growth for length (<9 months) and head circumference (<4 months).

We recommend breastfeeding for all infants when possible. However, when breastfeeding is unavailable or undesired, the following formula recommendations apply.

Benefits

• Premature transitional formulas have higher contents of protein, minerals, trace elements, and long-chain polyunsaturated fats, as well as providing higher caloric intake (e.g., 22kcal/oz), compared to term formula.
• Increased energy density may be required to optimize growth in the presence of fluid restriction.
• Soy formulas are not recommended for preterm infants due to an increased incidence of osteopenia and poorer protein bioavailability.

Recommendations

• Infants with birth weight 1800g or poor growth history, fluid restriction, or abnormal laboratory indices may be discharged on premature transitional formula until 9 months corrected age.
• Healthy, larger preterm infants may demonstrate catch-up growth more quickly and uncommonly require the use of transitional formula.
• Former very low-birthweight infants should receive formula with arachidonic acid (ARA) and docosahexaenoic acid (DHA).
• Preterm infants on iron fortified premature transitional formula should be given multivitamin (0.5 mL/day) until weight is >5 kg.
• Preterm infants receiving an iron fortified term formula should be given multivitamin (0.5mL/ day) until weight is >3 kg.

Growth Outcomes

• Premature infants fed formula supplemented with long-chain polyunsaturated fats (ARA and DHA) have shown improved growth and psychomotor development.
• Transitional formula may result in greater weight gain, linear growth, and bone mineralization than term infant formula.

Description

• The introduction of solid food feedings and the gradual replacement of milk by solid food as the main source of energy and nutrients.
• Shared pleasure in eating and feeding enhances the relationship between infants and their caregivers.
• Signs of readiness for solid foods:
  • Adequate head control.
  • Interest in feedings.
  • Decrease in tongue thrust.
  • Ability to sit with support at 60-90 degrees.

Considerations

• Very low-birthweight infants are at risk for developing aversions to feeding.
  • May have more gagging when initiating solid feeding.
• Water and juices should not be offered prior to 6 months of age.
• Whole milk should be offered at ³12 months of age.
• Weight gain may be jeopardized by adding poor caloric solid foods in place of breast milk or formula.
• Premature infants may feed more slowly due to weaker global tone, limited energy, and a lack of fully coordinated suck and swallow reflexes and oromotor tone.

Recommendations

• AAP recommends solid foods be introduced at 4-6 months corrected age.
• Use preterm formula milk to liquefy dried cereal foods until 9 months corrected age.
• Introduce rice cereal first, then vegetables or fruits.
• Introduce single-ingredient baby foods one at a time, and continue for 5-7 days before introducing an additional new food.

Types of Devices

• Nasogastric (NG) tube: non-weighted, polyurethane tube for use more than 10 days.
• Gastrostomy tube: tube protrudes from anterior abdominal wall; most common initial gastrostomy device.
• Button/skin-level gastrostomy: access device that is flush to the skin.
  • Mushroom tip: mushroom or wing-tip portion to secure the tube to the stomach wall.
  • Balloon tip: port on external portion of catheter to inflate or deflate the balloon securing the tube to the stomach wall.

Care

• Recommend management with consultation from GI and pediatric surgery.
• Check NG tube position by aspiration of gastric fluid before each feeding, or if child has been retching/vomiting/coughing.
• NG or G-tube/button care:
• Assess skin around tube site for redness and skin breakdown with every feeding.
• Use ordinary soap and water to clean around the tube site to prevent build-up of debris.
• Keep the tube open for a short time after feedings, to allow infant to “burp.”
• Flush tube with water before and after feedings or medications to prevent occlusion.
• Check external tube length daily to make sure tube has not migrated, and adjust as needed.
  • Check G-button balloon volume 1-2 times per week, and re-inflate as needed to initial volume.
  • Button devices typically last several months; replace when the valve fails and/or the tube leaks.

Complications

NG tubes: – Easily displaced. – Risk of malplacement/pulmonary aspiration. – Gastric or esophageal trauma during placement.

Gastrostomies:

• Granulation tissue (67%).
  • Bleeding (28%).
  • Pain (65%).
  • Requiring silver nitrate (56%).
• Broken or leaky tube (56%).
  • Dressing needed on a regular basis to control drainage (60%).
  • Stomal infection requiring antibiotics (45%).
  • Drainage of pus or clear fluid from the stoma (82%).
  • Leakage of tube feed at least 1-2 times per month (49%), daily (25%).

Troubleshooting

Giving medicines and feeding if your baby has a gastrostomy tube

• Clear the G tube or button as your health care provider showed you.
• Check for placement of the G tube or button.
• Slowly push in liquid medicine or feeding with a syringe.
• If the pharmacist says it is ok, pills and capsules may be dissolved in 10 to 20 cc of warm tap water.
• All medicines and feedings should be flushed in with 5 to 10 cc of warm tap water.
• Ask your baby’s doctor, nurse, or pharmacist how to measure the tap water.

It is important to use the specific tube adapter made by the manufacturer of your button.

• In fluid restricted babies flush medicines with ONLY 1 to 5 cc of warm tap water.
• Vent the tube after feeding to remove excess air or fluid and reduce leaking.

Protecting the G Tube or Button

• Snap t-shirts and onsies work best to prevent babies from pulling on the tube or button.
• You may also use a sticky wrap or stretchy dressing.

Tracheostomy Basics

• Indications: upper airway obstruction, long term ventilation, ease of pulmonary toilet, to protect the airway.
• Tube selection based on:
  • Indication for tracheostomy.
  • Tracheal width/length/shape.
  • Upper airway resistance.
  • Lung mechanics.
  • Needs of the child for speech/ventilation/airway clearance.
• Tube diameter:
  • Outer diameter of the tube should not exceed 2/3 of the tracheal diameter.
  • Use the largest size possible to reduce airway resistance, keep work of breathing low, reduce the risk of aspiration, and allow efficient pulmonary toilet.
• Length of tube:
  • Should extend 2 cm distal to the stoma, but be no closer than 1-2 cm from the carina.
  • Should be 3-6 cm (neonatal length tube) for children under 1 year.
• Curvature of tube:
  • Distal end of tube should be concentric and co-linear with the trachea.
• Tracheostomy tube connector:
  • Needs standard 15 mm connector at upper end to allow connection to a bag or ventilator.

Types of Tubes

• PVC: Softens at body temperature but becomes more rigid with use and may develop cracks.
  • Shiley, Portex.
• Silicone: Less rigid, naturally soft, does not stiffen with repeated use, may be an option in children in whom standard PVC tube does not provide a satisfactory fit.
  • Bivona.
• Cuffed: Indicated to prevent chronic aspiration, or when ventilating with high pressures.
  • May cause tracheal erosion unless the cuff is regularly deflated.
• Fenestrated: Allows the patient to breathe around and through the tube; may promote translaryngeal airflow and enhance secretion clearance.
• Speaking valves: Have thin silicone membrane that allows inspiration via the tracheostomy tube and direct expiratory flow around the tube up to the vocal cords.
  • If expiratory flow is obstructed, this may cause overinflation of the lungs.
  • These should not be used in very small or very sick infants due to the superimposed increased work of breathing.

Care

• Stomal care:
  • Clean skin around the neck with a damp cloth.
  • Encrusted secretions under neck plate can be removed with a cotton wool swab and normal saline twice a day and as needed.
    • Always remove secretions by rolling from the stoma outward.
  • Skin around stoma should be kept dry; a partially slit dressing made of non-fluffy, water-permeable material may help.
• Humidity: Lack of humidity in inhaled air will cause pathological changes in structure and function of airway, as well as thickening of secretions and increased risk of mucous plugging.
  • Always add warm humidified oxygen to ventilator or tubing.
  • Passive humidifier (artificial nose, Heat-Moisture-Exchanger): picks up heat and moisture during exhalation and partially returns it during inspiration.
    • Cannot be used with speaking valves, and creates extra resistance.
• Suctioning:
  • Best performed on as-needed basis.
  • Indications: Oxygen desaturation, patient anxiety or restlessness, visible secretions in tube, or increasing respiratory distress with noisy respirations or tachypnea.
  • Set suction pressure between 80-100 mmHg.
  • Shallow suctioning: Only insert catheter to depth where side-holes reach tip of tube.
  • Deep suctioning: Insert catheter until resistance met, apply suction on withdrawal:
    • This technique should not routinely be useddue to risk of mucosal damage.
  • Suction rapidly (<5 sec) to prevent development of atelectasis.
  • On withdrawal of catheter, twist between fingers to clear secretions as much as possible.
  • American Thoracic Society does not recommend any use of saline during suctioning.
  • Use clean technique (reuse catheters cleaned with alcohol and soapy water) at home.
• Changing out tracheostomy:
  • Change the tube every few weeks; more frequently if develops inspissated secretions.
  • Replacement tube, smaller tube, and self-inflating bag should all be in easy reach.
  • Position infant with blanket under shoulders to extend neck slightly for visualization.
  • Use an obturator (the curved plastic device that fits inside trach tube) to place tube, with a downward curving motion, then immediately remove obturator once tube is in place.
  • Secure trach with twill tapes tied by triple square knots to both sides of neck flange.
  • Tapes should be tight enough to prevent accidental decannulation, but loose enough to allow for change in neck size during activity.
  • Correct tension is when one finger can be slipped beneath tape at back of flexed neck.
• Speech development:
  • Crucial factors affecting speech and language are the age at and duration of cannulation.
  • Use speaking valve (e.g., Passy-Muir valve), if tolerated, in infants as young as 2 weeks.
  • If not tolerated, alternative methods of communication should be introduced.
  • All patients with a tracheostomy should be followed by speech therapist.
• Additional concerns:
  • When continuous presence of a competent caregiver is not available, child should always be connected to a reliable monitoring device.
  • Oral feedings should always be supervised to prevent aspiration through tracheostomy.
  • Bath water level should not be higher than the abdomen; constant supervision and care are necessary to prevent aspiration of splash water.
  • Clothing should not have furry or fluffy materials; high necklines should be avoided.
  • Avoid exposure to feathery or furry pets, garden sprinklers, fountains, swimming pools, sand pits, powdery building or cleaning materials, smoke, household sprays.
  • Smoking should be forbidden around a patient who has a tracheostomy.
• Emergency package should be with child at all times and contain:
  • Spare tracheostomy tube of same size and one size smaller, scissors, ties, suction catheter, normal saline, gloves, self-inflating bag with appropriate sized mask.
  • Information card with details on brand, size, and length of tube; catheter insertion depth; reason for tracheostomy; potential individual risks; names and telephone numbers of physicians, therapists, nurses, and service and maintenance companies.

Complications

• Mortality directly associated with tracheostomy ranges between 0.5% and 3%.
  • Most result from accidental decannulation or blockage of tracheostomy tube.
• Granuloma formation is the most frequent complication.
  • Usually occurs just superior to the internal stoma site on the anterior tracheal wall.
  • Large granulomas may cause bleeding, aphonia, and accidental decannulation.
• Accidental decannulation: Emergency kit described above should always be at hand.
• Blockage of the tracheostomy tube will cause sudden deterioration in a child with a tracheostomy.
  • Suction, and give positive pressure breaths with the self-inflating bag.
  • If no response, change the tracheostomy tube.
  • If tube cannot be replaced, bag-mask ventilate while occluding stoma.
• Bleeding:
  • Tip of tube may irritate tracheal wall, causing irritation, inflammation, and ulceration.
  • Anterior wall erosion can invade innominate artery, causing catastrophic hemorrhage.
• Infection:
  • Bronchitis/tracheitis may cause increase in purulent secretions with an increased risk of plugging.
  • Periostomal infection may cause erythema or drainage around stomal site; treat with topical antimicrobials, more frequent tube changes and cleanings.
• Tracheo-esophageal fistula may occur as a result of posterior wall erosion.
• Aspiration is a risk associated with fractured tracheostomy tubes or other foreign objects.
• Granulation tissue that grows around the external stoma causing partial occlusion may be treated with silver nitrate application.
• Suprastomal collapse:
  • Pressure on tracheal cartilage just superior to the stoma may cause chondritis and weakening of the cartilage.
• Subglottic stenosis may be caused by intraoperative damage to cricoid, tracheostomy placed too high, mucosal trauma, or low-grade chronic inflammation.

Followup and Decannulation

• Once stable, patients should be seen every 1-3 months by ENT; initially they should be seen more frequently.
• Imaging is not routinely required, only when complications develop.
• Routine endoscopic evaluation by flexible bronchoscopy on a 6- to 12-month basis to assess airway pathology and readiness for decannulation.
• Decannulation:
  • Most children are able to be decannulated within 2 years.
  • Tracheostomy tube is serially downsized, and when the child tolerates it, capped.
  • Stoma usually closes within few hours of tracheostomy tube's removal.
  • Removal creates an increase in dead space, resulting in an increased work of breathing.
• Persistent tracheocutaneous fistula:
  • Occurs in up to 40% of children post-decannulation.
  • May signal an underlying airway obstruction; assess airway patency prior to closure.

Ostomy Basics

• An ostomy is usually temporary and created as part of a staged surgical approach to divert fecal matter, allow for resolution of inflammation, and to provide time for infant to grow and stabilize.
• Often has 2 parts: proximal ostomy and distal mucous fistula for distal bowel decompression.
• Premature infants have specific issues:
  • Epidermal barrier is far less well-developed, with only 2-3 layers of stratum corneum, compared to term infant's 10-20 layers.
  • Dermal-epidermal junction has fewer anchoring elements to bond the epidermis to the underlying dermis, so skin is more susceptible to friction injury and blistering.

Care

• Skin must be protected from effluent (i.e., low pH, liquid, and contains enzymes).
• Peristomal skin must be protected from mechanical trauma due to use of aggressive adhesives, improper adhesive removal, or inappropriate cleansing.
• Moldable barrier or caulking strips can be used to fill in skin creases or scars.
• After applying barrier, warm with hand to mold to contours of abdomen and increase adhesion.
• Attach pouch. It should be clear to allow inspection of stoma and effluent, and the pouch opening should be no more than 1/8 inch in diameter larger than stoma.
• Vent frequently to prevent gas pressure from compromising the seal.
• To remove pouch, use a soft cloth dampened with warm water to loosen barrier adhesive.
• Soap may be helpful for particularly adherent barriers, but be sure to completely rinse it away.
• The soiled pouch should be removed by gently pushing down on skin to separate it from the wafer while lifting up on the pouch.
• Commercial adhesive removers should not be used as they contain alcohol, which may be absorbed and cause neurologic toxicity.
• Use only alcohol-free skin sealant, a plasticizing agent applied to the skin to create a barrier.
• Gently clean peristomal skin with water between pouch applications.

Troubleshooting

• Peristomal skin damage: loss of epidermis results in "weeping" of serous exudate, and fluid accumulation can decrease adhesion of pouch.
  • Apply skin barrier powder to "weepy" areas prior to pouching.
• Denuded peristomal skin:
  • May need period of non-pouching.
  • Protect peristomal skin from effluent with an occlusive barrier ointment. (Do not use a cream, as they are water soluble).
  • Place fluffed gauze or diaper around stoma to absorb effluent.
• Fungal infections: can occur in the warm, moist environment beneath the barrier and erode skin.
  • Apply nystatin powder to involved area; gently brush away excess powder before applying the barrier and pouch.
  • May "seal" nystatin powder by patting it with a damp finger after application and allow it to dry before reattempting barrier placement.
• Liquid stool pooling around stoma and degrading barrier is common in ileostomies and jejunostomies.
  • Use cotton or gauze to wick the fluid away from the stoma toward the bag.

Call the doctor if your baby:

• Has a fever more than 100 degrees Fahrenheit.
• Has diarrhea for more than one day.
• Is throwing up forcefully.
• Is not eating well.
• Has not had at least six to eight wet diapers per day.
• Has very yellow eye color or skin color.
• Is hard to wake up.
• Has fast or difficult breathing.
• Makes jerking movements.
• Is not looking well or acting well.

Characteristics

• Occurs during the normal developmental switch from fetal to adult hemoglobin synthesis.
• Immediately after birth, the increase in blood oxygen content results in the downregulation of erythropoietin.
• Once hemoglobin level decreases enough, tissue oxygen needs are greater than oxygen delivery, and erythropoietin production increases.
• Anemia is more profound and occurs earlier in premature infants due to:
  • Blood loss from sampling.
  • Short survival time of RBC's (40-60 days compared to adult 120 days, and term infant 60-80 days).
  • Suboptimal erythropoietic response.
  • Relatively more rapid rate of growth than in term infants.

• Usually reach nadir of 7-10 g/dL at 4-8 weeks of life.
• Normocytic, normochromic, hypoproliferative anemia.
• Premature infants may have lower iron stores despite iron supplementation; consider iron deficiency anemia as an etiology for persistent or progressive anemias.

Monitoring

• May check hematocrit and reticulocyte count periodically, but most babies can be managed by watching for symptoms (tachycardia, tachypnea, apnea and bradycardia, poor weight gain, oxygen requirement, diminished activity, pallor, poor feeding).

Treatment

• Healthy asymptomatic newborns will self correct, provided their iron intake is adequate.
• Transitional formulas and fortified breast milk provide approximately 2 mg/kg/day of iron.
• Iron administration before 10-14 weeks of age does not reduce the nadir, but iron is stored for later use.
• Full-term infants should receive 1 mg/kg/day of iron supplementation from age 4 months to 1 year.
• Premature or low-birthweight infants (<2500g) should receive 2 mg/kg/day from 2 months to 1 year (from iron enriched formula or 1 mL multi-vitamin with iron).
• No strong evidence to favor use of erythropoietin.
• Infants with significant respiratory disease or congenital heart disease may need a hematocrit maintained >40 gm%.
• Infants with a hematocrit <25 gm% and symptoms may require red blood cell transfusion.
• For infants requiring blood transfusion:
  • [Insert contact information for appropriate specialist or department.]
  • Order: PRBC, leukocyte poor, irradiated, CMV negative, 15 ml/kg.
  • If the baby is very fluid sensitive (e.g., chronic lung disease), IV lasix 1 mg/kg immediately after transfusion may be indicated.

Characteristics

• A persistent open connection beyond 3 months of age between the pulmonary artery and the aorta with blood flow from the aorta to the pulmonary artery.
• An open ductus may lead to:
  • Congestive heart failure.
  • Pulmonary hypertension.
  • Increased risk of bacterial endocarditis.

Followup

• A high rate of spontaneous closure occurs during the first 2 years of life.
• If the ductus is open at the time of hospital discharge, followup with a cardiologist should occur within 2 months of discharge to assess well-being and the presence/absence of congestive heart failure.
  • Earlier followup should occur if poor weight gain, difficulty feeding, and tachypnea develop.
  • Generally do not need a followup echocardiogram, as anatomy is already known.

Management

• If spontaneous closure does not occur, closure should be performed to:
  • Eliminate pulmonary overcirculation.
  • Eliminate risk of endocarditis.
• If symptomatic:
  • Initially, try diuretics and maximize caloric intake to 140 kcal/kg/day.
  • If still symptomatic despite optimal medical management and:
    • >2.4 kg, surgical ligation or percutaneous closure should be considered.
    • <2.4 kg, surgical ligation should be considered.
• If asymptomatic:
  • Wait until patient weighs between 10-12 kg, or is close to 2 years of age, then attempt percutaneous closure with Amplatzer occlude or coils.
  • Surgery if percutaneous attempt is unsuccessful.

Pathophysiology

• Functional disorder caused by alterations of normal intestinal anatomy and physiology.
• Shortened bowel combined with malabsorption; dependent on parenteral nutrition >3 months.
• May result from: necrotizing enterocolitis, congenital bowel atresia, volvulus, gastroschisis, Hirschsprung's disease.
• After resection, the residual small bowel undergoes intestinal adaptation, stimulated by hormones and oral nutrients:
  • Mucosal hyperplasia.
  • Villus lengthening and increased crypt depth.
  • Bowel dilatation.
• Jejunum: long villi and large absorptive surface with high concentration of enzymes; site of greatest nutrient absorption.
  • If resected, will have transient or permanent nutrient losses.
  • Ileum can develop the absorptive capacity of the jejunum for various nutrients.
• Ileum: shorter villi, more lymphoid tissue, tighter epithelium; effective absorption of fluid and electrolytes; responsible for absorption of vitamin B12 and bile salts through receptors.
  • Other bowel will never develop ability to absorb B12 and bile salts.
  • Resection may impair bowel motility (many GI hormones produced in ileum).
  • Intestinal adaptation after massive ileal resection is more difficult than after jejunal.
• Malabsorption of rapidly digested carbohydrates produces osmotic diarrhea.
• Fat soluble vitamins may also be inadequately absorbed.
• No absolute number can be placed on the length of remaining bowel necessary for successful enteral nutrition; remaining bowel may be damaged and act dysfunctional.
• Best prognosis over time is for infants in whom the duodenum, distal ileum, and ileocecal valve can be preserved.

Complications

• Long term TPN use:
  • Hepatobiliary disease.
  • Catheter-associated sepsis.
  • Fluid and electrolyte imbalance.
  • Bacterial intestinal overgrowth.
• Malnutrition.
• Failure to thrive.
• Dumping syndrome: post-prandial tachycardia, diaphoresis, lethargy, watery diarrhea.
  • Marked increase in stools may indicate poor absorption, and enteral feedings should not be advanced.
  • If significant volume loss, rehydration therapy and electrolyte replacement will be needed.
• Mortality of 30-40% from sepsis or liver failure.

Management/Feedings

• Recommend managing with consulting services in pediatric surgery, GI (and possibly the liver team), and neonatal dietitian.
• Attempt to wean off TPN as soon as possible.
• Enteral nutrition should be started promptly to promote intestinal adaptation.
• Usually started on elemental/semi-elemental diets containing free amino acids or small peptides, or breast milk.
  • There are no elemental preterm formulas.
  • Once they tolerate feeds, can gradually introduce a portion of preterm formula, fortifier, or supplements to improve the mineral and protein content of a term formula.
• These infants may have "leaky gut" with high rates of sensitization to cow's milk or soy protein.
• High proportion of fat in long-chain fatty acids promotes more mucosal adaptation; but those with ileal resection may not be able to absorb these and may need more medium-chain fats.
• Feedings are started slowly and continuously to maximally saturate carrier proteins.
• Higher concentrated formulas may cause osmotic diarrhea.
• Oral feedings should be initiated promptly; may have solids at 4 months CGA (high in protein and fat and low in carbohydrate is preferred).
• For high stomas, may be beneficial to re-feed the proximal stoma effluent through the mucous fistula for additional nutrient and fluid absorption and to stimulate distal gut.
• For bacterial overgrowth, may need intermittent dosing of antibiotics that affect anaerobic bacteria.
• Infants with ileal resection are at risk for B12 deficiency; may need parenteral B12 every 1-3 months.
• Children with enterostomies or diarrhea are at risk for zinc deficiency and may need supplementation:
  • Poor growth, diarrhea, impaired wound healing, perianal and perioral skin rash, alopecia.
• Gastric acid hypersecretion:
  • More common in larger bowel resections and initiation of enteral feedings.
  • Can cause secretory diarrhea.
  • Ranitidine may help.

Surgical Treatments

• Goals of surgery:
  • Slow intestinal transit.
  • Increase mucosal surface area.
  • Improve peristaltic function.
  • Increase intestinal length.
• May eventually require intestinal +/- liver transplant.

Characteristics

• Renal lithiasis in which calcium deposits form in the renal parenchyma and result in reduced
  kidney function and hematuria.
• Seen with renal ultrasound, or occasionally on plain radiographs of the kidneys.
• Results from an imbalance of stone-promoting and stone-inhibiting factors.
• Incidence in very low-birthweight infants (BW1500g) ranges from 16-64%.
• Risk factors in at risk infants:
• Medications with hypercalciuric effects (furosemide, corticosteroids, aminoglycosides).
• Metabolic acidosis.
• Hypocitraturia.
• Hypercalciuria.
• High urinary oxalate:creatinine ratios.
• High urate:creatinine ratios.

Effects

• Glomerular function: Mean GFR and microalbuminuria in preterm infants is slightly worse
  than in healthy infants.
• Proximal tubule function: Phosphate reabsorption is significantly lower in patients with
  nephrocalcinosis, but plasma phosphate levels are within reference limits; no firm evidence for
  proximal tubular dysfunction.
• Distal tubule function: Urine anion gap of infants with nephrocalcinosis is high, and serum
  bicarbonate levels are low, indicating distal tubular dysfunction.
• Blood pressure: Not higher in infants with nephrocalcinosis, but overall higher in preterm
  infants than in healthy term infants.
• Hypercalciuria: Significantly more hypercalciuria in infants with nephrocalcinosis.

Monitoring/Treatment

• If associated with a medication that causes hypercalciuria, consider trying to stop the
  medication and monitor for resolution.
• Resolves in 75% by 7 years of age.
• Does not appear to have any long-lasting significant health effects.
• Should have:
  • Blood pressure checked at every visit.
  • Yearly renal ultrasounds until resolution occurs.
  • Electrolytes and BUN/Cr checked every 2 years.
• Patients do not need to be followed by renal team unless other issues arise.
• UTI and urolithiasis do not occur any more frequently than in general population.
• Do NOT stop the use of high-calcium containing infant formulas.

Visual Defects Seen in Preterm Infants

• High-risk infants are more likely to have permanent visual deficits and/or show a delay in visual development that persists until adolescence.
• Deficits in the ability to perform visual discrimination tasks is one of the most striking, persistent deficits.
• Reduced visual fields, amblyopia, myopia (associated with BPD, seizures and asphyxia), and strabismus (associated with intraventricular hemorrhage (IVH), bronchopulmonary dysplasia (BPD), necrotizing enterocolitis (NEC), periventricular leukomalacia (PVL)).

Retinopathy of Prematurity

• Abnormal blood vessel growth in the incompletely vascularized retinas of premature infants.
• Incidence increases with lower gestational age and birthweight.
• Classification by:
  • Zones 1-3: locates the disease from most posterior (Zone 1) to most anterior (Zone 3).
  • Stages 1-5: degree of vasculopathy at the vascular-avascular transition (Stage 5 is most severe and involves complete retinal detachment).
  • Plus disease: occurs when arrested blood vessel growth resumes abnormally, with tortuous vessels piling up within the retina, forming a thick ridge of tissue.
• Neovascular tissue (i.e., plus disease) may contract and form a scar, which then pulls and distorts the retina, resulting in retinal detachment, especially if ROP is in zone 1.

Screening/Monitoring

• All infants <1500 grams or <30 weeks estimated gestational age (EGA) should be screened prior to discharge.
• If retinal vasculature is completely mature, no further exams are needed.
• Followup is dictated by degree of maturation, severity of ROP, and ophthalmologist.
• Patients with ROP requiring laser therapy, and grade III/IV IVH, PVL, HIE, or hydrocephalus requiring shunting should be seen again at 9-12 months postnatal age by ophthalmology.
• A pediatric ophthalmologic assessment for glasses and possible strabismus or amblyopia therapy should be obtained in the first year of life.

Treatment/Outcomes

• Mild ROP (stage I or II without plus disease) have a somewhat higher incidence of myopia, strabismus, and amblyopia.
• Threshold ROP (residual scar without retinal detachment):
  • Associated with severe myopia, glaucoma.
  • At risk for slowly progressive retinal degenerations that can lead to retinal detachments and acuity loss in later decades.
• Total retinal detachment equates to no useful vision in that eye, even when vitrectomy is performed to reattach the retina.
• Criteria for laser therapy or cryotherapy:
  • Zone 2: plus disease with stage 2 or 3 ROP.
  • Zone 1: plus disease with any stage ROP.
  • Zone 1: stage 3 ROP with no plus disease.