CCANN’s Spring Conference 2023
On Saturday, April 15th, CCANN held its Spring Conference, welcoming almost 100 members,
students, and clinicians in our community on “A Journey through Genetics.”
We were joined by multiple physicians and staff from Valley Children’s Hospital who provided their insight into the blossoming field of genetics and its implications in their specialties and the NICU.
The morning kicked off with breakfast and an exhibition from our vendors, who provided free samples and a review of exciting innovations and products. Vendors included representatives from Cerave, Mead Johnson Nutrition, Johnson’s Baby, Kentec Medical, Dandelion, Draeger, Pacific Biomedical, and Silentia.
Dr. John-Paul Berauer, an expert in Pediatric Gastroenterology and Pediatric Transplant Hepatology, and a recent addition to our Valley Children’s team, began the presentations with a review of Neonatal Cholestasis. This condition, which differs from the unconjugated hyperbilirubinemia often seen in NICU populations related to liver immaturity, feedings, and fetal blood cell destruction, is instead caused by changes in bile formation and flow. Dr. Bauer discussed current advances in genetic testing used to diagnose defects in these processes early in order to initiate treatment of the underlying cause prior to further deterioration of liver function. Beyond genetic causes, patients in the NICU are predisposed to acquired cholestasis related to PN use and sepsis-induced cholestasis. Indicators of cholestasis that should be monitored in the NICU include pale, clay-colored stools, jaundice extending beyond day 14 to 21 of life, direct bilirubin >1, and an enlarged liver.
Dr. Bindu Sathi, a Pediatric Hematology and Oncology physician and the director of Valley Children’s Sickle Cell Disease and Apheresis Programs, followed to review neonatal anemias and their genetic considerations. Inherited traits can impact both red blood cell destruction, like those seen in Hemolytic Disease of the Fetus and Newborn (HDFN), and production, in which anomalies can lead to inherited bone marrow failure syndromes. Early identification of infants displaying symptoms of anemia or high red blood cell turn over can impact patients long beyond the neonatal period.
After a brief recess, the conference proceeded with a discussion of Childhood Interstitial Lung Disease and Ciliopathies by Dr. Denna Yousif, a Pediatric Pulmonologist at Valley Children’s Hospital whose dedication to research in areas such as bronchopulmonary dysplasia and neonatal bronchoscopy provided great insight into NICU population. Childhood Interstitial Lung Disease (chILD syndrome), Dr. Yousif reports, is the remodeling of the lung tissue and airspaces due to a range of different mutations in genes that code for surfactant proteins or other lung components. The severe tissue injury that the lung can sustain with different types of chILD necessitates the early diagnosis using CT scans and genetic testing. Any infant that exhibits unexplained respiratory distress warrants further work-up and a consultation with pulmonology to rule out these conditions. This is especially true for those born full-term or with commonly associated conditions such as congenital heart disease, dysphagia, reflux, or recurrent aspiration.
The final two presentations were given by Valley Children’s Genetic Counselors. Jason Carmichael, Valley Children’s lead Genetic Counselor, discussed the new California Prenatal Screening Program which aims to identify pregnancies at risk for genetic anomalies and promote appropriate follow-up across all socioeconomic backgrounds.
Lily Radanovick, another Genetic Counselor at Valley Children’s, followed in cooperation with Melissa Martz, a Nurse Practitioner from Valley Children’s Neurology Clinic, to discuss Spinal Muscular Atrophy (SMA), an autosomal recessive trait detected on the Newborn Screen, which can lead to loss of motor function and death without treatment in the first few days of life. With early pharmaceutical intervention many SMA patients can have considerable improvement in neuromuscular development and life expectancy.
A special thank you to all of our sponsors and presenters, as well as to Dr. Chokshi and Kamela Loo for all they did to coordinate this event. Mark your calendars for our next in-person conference on September 16th !
Pharmacology Dinner Event
CCANN together with Mead Johnson sponsored an educational dinner event on NICU Pharmacology last June 29th at Manhattan Steakhouse Bar and Restaurant in Fresno. The conference was attended by several healthcare professionals across central valley along with the invited guest speakers : Renzio Apostol VCH PharmD and Dr. Naisha Choksi VCH Neonatologist.
Pharmacist Renzio discussed the importance of pain assessment and it’s adequate control and sedation management in the NICU - to prevent it’s negative impact to the neurodevelopment status of our babies.
He introduced the multi modal approach to manage pain : First, prevention by grouping lab draws. Second is by institution of non pharmacologic techniques to manage stress. And the last is the use of pharmacotherapy.
Drugs like Opioids, Benzodiazepines, and others.Fentanyl and Morphine are the most commonly used opioids in the NICU. Despite Morphine to have a slower onset of action compared to Fentanyl but they both have a higher half lives in the babies. So, monitoring their side effects and clearances is a must. Tolerance and withdrawals likely to develop on prolonged exposure to these medications. Other agents like Propofol, Ketamine and Dexmedetomidine have limited published studies on the safety, drug pharmacodynamics and kinetics to the neonatal population.
Ross Nichols ,the MJ representative of central valley showed the video as to how their pharmaceutical company navigate the crisis of formula production during the time of pandemic. He also showed a video clip on the importance of checking osmolality of the milk once fortifiers are added. Once it exceeded the limit of
normal body osmolality it diminishes it’s absorptive capacity in the gastrointestinal tract of the baby.
Dr. Choksi discussed about Brochopulmonary Dysplasia (BPD)and the medications commonly associated with this diagnosis. She started the definition of BPD, the grading system and its complications of cardiac impairment, growth failure and even death.
Medical treatment includes non corticosteroid agents like Azithromycin which has antimicrobial and
anti-inflammatory property. Introduction of Caffeine that is given within the first 72 hours of life which enhances baby’s motor development and reduces risk of BPD by increasing lung compliance and minute
ventilation, decrease apnea, airway resistance and inflammation yet augment airway remodelling and
improve diaphragm contractility.
Vitamin A is needed as a key regulator of the normal respiratory tract epithelium growth that is stored in
septal cells of the alveoli that is involved in alveolar septation.
Antenatal steroids indirectly reduced BPD risk by mitigating the neonate’s risk to develop RDS, IVH,
NEC and other early onset sepsis that will trigger infant to be hooked to ventilator and be at risk of BPD. There is also the strategy of fluid restriction, use of diuretics and inhaled bronchodilators. It is also
pertinent to start infant on early parenteral support.
The lecture ended with an interactive play of Q and A portion that gave out several gift cards to audience
who gave the right answers.
Dermatology Dinner Conference at Ruth Chris Restaurant
Central California Association of Neonatal Nurses in coordination with Cerave presented a mini lecture on Pediatric Skincare Conditions to a group of nurses in the Central Valley on September 21, 2023.
In the above topic Dr. Jennifer Sorell talked about the structural and functional maturation of the neonatal skin at various gestational age.
Around 34 weeks the epidermis is well developed, at 37 weeks the stratum corneum is fully mature. Consequently, around 2-4 years of life is when it is fully completed. She also differentiated the adult skin from the infant’s skin.Total epidermal water loss is significantly higher in infants compared to the adults. The stratum corneum is significantly higher in the infants around 3-4 months to 24 months. The skin’s enzymatic activity is also higher than the adult.All these result in increased desquamation and thinner stratum corneum and challenges for the barrier formation.
During the infant’s first year of life the statum corneum function and thickness develops. And the skin ph is elevated to plus minus 6.0. The ph of the neonatal skin ranges from 6.34-7.5. Several factors play a role in the elevated ph. Slightly acidic surface defends the skin against infection.It also acts as a barrier in the activation of enzymes involved in the extracellular processing of the stratum corneum lipid.
The vernix caseosa is a rich ceramide coating formed during the last trimester. It consist of water containing comeocytes embedded in a lipid matrix. This protects the fetus while submerged in the amniotic fluid. Back in the day the vernix caseosa is entirely removed after birth, but now it is allowed to separate over 6 hours to 5 days after birth.The protective layer deceases skin surface ph , inhibiting pathological bacterial growth.
Skin conditions arise when the ceramide is decreased. Without proper ratio of ceramides the stratum
corneum becomes incompetent. Impaired synthesis of cholesterol, ceramides and fatty acids adversely affects lamellar layer formation, thereby impairing barrier homeostasis ,leading to
dryness, irritation, erythema and itching.
A study was conducted and it was found out that at 6 months of life 18.7% of the infants in the study group presented with atopic dermatitis.This concludes that impairment of skin function at birth ( 2 days of life) was shown to precede clinical atopic dermatitis.
The use of moisturizer for skin hydration is the primary treatment for mild disease in conjunction with other agents for moderate and severe disease. The care of the newborn and infant’s skin should optimize stratum corneum’s lipid and water content. The use of ceramides support this structure. The use of mild liquid cleansers containin emolients further protects the skin.
Neonates and infants greatly benefits from frequent moisturizer use containing ceramides which mimics physiological lipids supporting homeostasis and
improving skin conditions.
