Newborn screening (NBS) for Cystic Fibrosis (CF) has revolutionized the diagnosis of this inherited disease. CF NBS goals are to identify, diagnose, and initiate early CF treatment to attain better health outcomes. Abnormal CF NBS infants require diagnostic analysis via sweat chloride testing (ST). During ST, insufficient sweat volume collection causes a “quantity not sufficient” (QNS) test result and may delay CF diagnosis. The CF Foundation recommends QNS rates <10% for infants <3 months, but many CF Centers experience difficulties meeting this standard. Our quality improvement (QI) study assessed infant and laboratory factors contributing to ST success and QNS rates from 2017–2019. Infants’ day of life (DOL) at successful ST completion was analyzed according to infant factors (birth weight (BW), gestational age, ethnicity, and sex). Laboratory factors and procedures affecting ST outcomes were also reviewed. At our institution, BW and gestational age were the infant factors found to significantly affect DOL at ST completion. ST education, reduced number of laboratory technicians, and direct observation during ST completion also improved ST success rates. This study supports QI measures and partnerships between CF centers and laboratory staff to identify and improve ST QNS rates while sustaining practices to ensure timely CF diagnostic testing.

Because side sleeping position was observed in the majority of infants, and one-third of the nurses queried disagreed with the AAP recommendations, education of nurses about Sudden Infant Death Syndrome prevention through "Back to Sleep" is still necessary.

.Sch.of ~ez.','~hila., PA. 'Management of t h e infant with severe infantile apnea remains controversial. Studies have suggested t h a t home cardiorespiratory monitoring failed t o prevent subsequent deaths from SIDS in a s many a s 10% of patients. In order t o assess the effectiveness of home cardiorespiratory monitoring upon subsequent outcome in severe infantile apnea, 32 infants were evaluated and prospectively followed. These infants represented 8%(32/396) of t h e children seen a t t h e Children's Hospital of Philadelphia during a two year period. All 32 infants had life-threatening apnea requiring cardiopulmonary resuscitation by parents, physicians, or paramedical personnel. Twenty-five (78.1%) were term infants, 7 (21.9%) were preterm babies. Mean + SEM G.A. a t birth was 37.9 2 0.3 wks. Age a t t h e t i m e of initial apneic episode was 8.6 2 1.3 wks. Initial thermistorpneumocardiogram evaluation revealed respiratory pattern abnormalities in 16 (50%) infants: central apnea -5 (15.6%); obstructive apnea -1 (3.2%); periodic breathing -6 (18.7%); gastroesophageal reflux associated apnea -4 (12.5%). All infants were treated with home cardiorespiratory monitoring. Mean + SEM duration of monitoring was 4.7 2 0.5 months.Ten infants (31.3%) had apnea while monitored which required vigorous stimulation. Two infants (6.25%) required vigorous stimulation including CPR. Infants have been followed for a mean of 21.3 + 6.3 SEM months.No deaths have occurred. These results suggest that one-third of infants with severe infantile apnea will have subsequent respiratory episodes a t home. Home monitoring appears t o be a n effective therapy in this group of babies a t highest risk f o r continuing apnea. Mixed apnea of infancy is a respiratory pattern characterized by both central and obstructive apnea. In order t o characterize this form of apnea more completely, 67 patients were evaluated in t h e Apnea Screening Program of The Children's Hospital of Philadelphia. These infants had predominantly mixed apnea and represented 8.1% (671838) of t h e children evaluated for apnea during a two year period. Mean BW was 1700 + 88.8 SEM gms, mean GA was 31.6 + 0.4 SEM wks. The average age a t t h e time of study was 5.8 2 0.5 SEM wks. Fifty-nine (88%) infants were premature, eight (12%) were terln babies with infantile apnea or siblings of SlDS victims. Infants were evaluated for a minimum of 6 hours by thermistorpneumocardiogram study. 279 episodes of mixed apnea were detected. 217 (77.8%) episodes began a s central apnea and progressed t o obstructive apnea. 62 episodes (22.2%) initially demonstrated obstructive apnea t h a t subsequently became central. 155 (55.6%) episodes of mixed apnea were accompanied by bradycardia (decrease in heart r a t e >40 bpm below baseline level). The duration of mixed apneic episodes tended t o be prolonged with 85 (30.5%) 10-15 seconds duration, 120 (43%) 15-20 seconds duration, and 74 (26.6%) D 20 seconds duration. Children with predominantly mixed apnea had associated other apnea: 33 (49....

Clinicians need to be cautious when recommending the side or prone position in this group of high-risk infants. The results in this investigation provide support for the Back to Sleep Campaign recommendations to be applied, not only to healthy term infants, but higher risk infants as well. Studies of the high-risk infant in the supine position are warranted.