1. When will the California Newborn Screening Program Expand? On or before August 2005, the California Department of Health Services will be expanding the Newborn Screening Program to include:

- multiple metabolic conditions detectable via Tandem Mass Spectrometry (MS/MS)

amino acid disorders

organic acid disorders

fatty acid disorders

- classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency

salt washing

simple virilizing

Prior to implementation of the expansion the state will be conducting a three-week pilot project (May 2-20, 2005) to test the new computer system, and make any necessary modifications. The Newborn and Prenatal Screening (NAPS) Laboratory at Memorial Medial Center of Long Beach has been selected as the testing laboratory for this pilot. Newborn specimens submitted to Memorial Medical Center of Long Beach will be part of this pilot. These specimens will be tested for both disorders included in the current program as well as for additional metabolic disorders and congenital adrenal hyperplasia.

2. Which Inborn Errors of Metabolism will the expanded newborn screening identify? Tandem Mass Spectrometry allows for reporting patterns associated with 46 disorders using a single analytical run. Forty-two analytes are measured. Evaluations in analytes and/or ratios of certain analytes consistent with a known disorder are reported as positive for one or more condition.

Congenital adrenal hyperplasia will also be added to the expanded newborn screening panel of tests. The current newborn screening for galactosemia, primary congenital hypothyroidism and hemoglobin disorders will continue. PKU testing will also continue but the method will be changed to MS/MD. For detailed information click here.

3. What is the cost of the Expanded Newborn Screening? The cost is $78 dollars. In addition hospitals can charge up to $6 for blood collection.

4. At what age should the screening be done? The guidelines for obtaining the specimen remain the same as they are now. The blood spot should be collected after 12 hours of age, but before the end of the sixth day of life. For exceptions related to transfusion, partial transfusions, early discharge, etc.

5. What is the chance that an infant will have a positive newborn screening test for one of the disorders detectable via MS/MS? Each infant has approximately a 1 in 885 chance of having a positive MS/MS screening test, but exact positive rates will not be known until closer to the start date.

6. What is the chance that an infant will have a disorder identified via newborn screening? The current newborn screening program identifies approximately 1/1,200 newborns with phenylketonuria (PKU), primary congenital hypothyroidism, galactosemia, or a hemoglobin disorder. With expanded screening to include multiple metabolic disorders and congenital adrenal hyperplasia, combined with current testing, it is estimated that 1/1,000 newborns will be diagnosed with a specific detectable disorder.

7. Can the Expanded Newborn Screening test be used to evaluate an older child with developmental delay or mental retardation? NO. However, the same instrumentation, MS/MS, may be used by commercial labs for determining an acylcanitine profile on a blood specimen from a patient with developmental delay or mental retardation. An abnormal acylcamitine profile often leads to the diagnosis of an inborn error of metabolism.

8. Will the California Newborn Screening test identify all inborn errors of metabolism? No. There are many inborn errors of metabolism which are not identified by the Expanded Newborn Screening test, MS/MS identifies an abnormal pattern of metabolites that is associated with a specific disorder. The screening is designed to minimize the number of false negative tests. However, consider evaluating any child who has clinical features of a metabolic disorder even though the newborn screening test was negative.

Multichannel cochlear implants (CIs) have been commercially available to the pediatric population for almost 20 years. Changes in technology and criteria, as well as newborn hearing screening legislation, have made CIs available to more children at increasingly younger ages. Although results for individuals vary, most early-implanted children tend to develop speech and language skills at a rate similar to normal-hearing children. *1,2,3,4 The purpose of this article is to provide pediatricians with information regarding candidacy criteria and guidelines for referring potential candidates for CI evaluation.

Current Criteria for Pediatric Cochlear Implantation: Today, criteria for pediatric cochlear implantation includes:

• Severe to profound bilateral sensorineural hearing loss (age 18 months and up; profound loss for ages 12-18 months)
• Minimum of 12 months of age (younger if medically warranted, such as deafness due to meningitis)
• Lack of benefit from appropriately fit hearing aids; lack of progress in auditory skill development
• Medically able to undergo surgery
• Family and educational support network
• Realistic expectations of parent(s)/family

There is no way to predict how much benefit an individual will receive from a CI. However, the following factors can influence potential benefit:

• Age at implant
• Duration of hearing loss and/or duration of use of amplification (hearing aids)
• Presence of additional disabilities (e.g., learning disabilities, developmental/cognitive delays, etc.)
• Status of cochlea and/or auditory neural pathways
• Individual motivation/realistic expectations

Guidelines for Referring Patients with Hearing Loss: When referring children who may be cochlear implant candidates, it is important to consider centers that offer comprehensive services.

• Hearing Aid Services: Because most cochlear implant centers require an adequate hearing aid trial prior to final determination of candidacy, it is beneficial to refer potential implant candidates to a center that offers knowledgeable, skilled pediatric hearing aid fitting services. By choosing a center with both hearing aid and implant services, patients can benefit from continuity of care as they bridge from hearing aid use to cochlear implant use. Additionally, for children who continue to wear a hearing aid on the unimplanted ear, both hearing aid and CI services can be obtained during the same visit to a single center.
• Medical Services: When referring pediatric patients, particularly those under age 2, it is important to choose a center whose implant surgeon has experience with implanting very young children. It is also important to ensure tht the implant center has highly trained pediatric anesthesiologists.
• Audiologic Services: The process of programming the speech processor for a CI is very different for young children than for adults. It is very important that the implant center has audiologists on staff that are experienced in working with very young children. In addition, objective measures are being used more routinely in the clinical setting to assist in programming the speech processor for young children. Most newer CIs have the ability to directly measure auditory nerve responses through the implant, which can significantly aid the programming process. Thus it is important to choose a center that can offer these types of comprehensive audiologic services.
• Speech-Language Services: Achieving full benefit with a CI cannot happen simply with surgery and a single speech processor programming session. Numerous visits are necessary, particularly within the first year of implant use. In addition, it is important to choose a center that offers or coordinates speech and language therapy services to facilitate and optimize development of oral communication skills. The speech-language pathologist also conducts periodic assessments to measure a child's progress with the implant over time.
• Aural RE(Habilitation) Services: Learning to listen with a CI is equally as important as learning how to talk. A comprehensive cochlear implant center should offer services targeted at teaching recipients how to create optimal listening environments and practicing auditory skills (detection, discrimination, identification) with the implant.
• Educational Coordination: It is important that the cochlear implant center supports options for communication modality choices that are consistent with the child's educational placement. For example, young implanted children who communicate using both sign and auditory/verbal means often transition to an auditory-only mode after some time of implant use. It is important for the implant center to work closely with the child's school system to ensure that the child's educational placement is appropriate for the child's communication abilities and goals.

References

1. Manruqie, M., Cervera-Paz, F., Hurate, A. & Molina, M. (2004). Advantages of cochlear implantation in prelingual deaf children before 2 years of age when compared with later implantation. Laryngoscope. 114 (8), 1462-1469
2. Uchanski, R. & Geers, A. (2003). Acoustic characteristics of the speech of young cochlear implant users: A comparisons with normal-hearing age-mates. Ear & Hearing. 24 (1) Supplement: 90S- 105S.
3. Robbins, A., Green, J. & Waltzman, S. (2004). Bilingual oral language proficiency in children with cochlear implants. Archives of Otolaryngology Head and Neck Surgery. 130, 644-647.
4. Robbins, A., Koch, S. Osberger, M. & Zimmerman- Phillips, S. (2004). Effect of age at cochlear implant on auditory skill development in infants and toddlers. Archives of Otolaryngology Head and Neck Surgery. 130, 570-574

The Orange County Chapter of the American Academy of Pediatrics has been awarded a $295,000 grant to improve the quality of care provided by a group of local school nurses. An additional purpose of this grant is to create a linkage between county pediatricians, medical homes and the School Readiness Nurses. Project staff will support the professional development of this cadre of newly hired nurses. Early topics have included Developmental Screening, Otoscopy and the new Otitis Media guidelines. AAP Area Representative Chris Koutures presented to the nurses on activity screening for children aged 0 - 5 years. During the summer, AAP physicians will provide training for the nurses to do both level one developmental screens (such as the PEDS or Ages and Stages) and then additional testing as appropriate (Brigance Screening).

WE NEED YOUR HELP! The project team wants to IMPROVE COMMUNICATION between your office and your local schools. We hope to:

• Help children in need find medical homes.
• Let your office know the results of vision, hearing and related assessments done for 3 and 4 year olds at school.
• Improve the efficiency of communication between your office and your schools.

Working advocacy teams will be established between AAP physicians, school nurses at 12 or more Orange County school districts. These teams will look for additional opportunities to make a linkage between the community services and learning programs of your neighborhood school and your offices.

Program Staff:

• Dian Milton, RN, was previously Director of Pediatric Intensive Care at CHOC. She is the SRN Manager
• Marc Lerner, M.D., Immediate Past President of CA-4 and Developmental Pediatrician
• Maria Tupas, M.D., Current CA-4 President and General Pediatrician (involvement until 6/30/05)
• Starting 7/1/05, new members of the Program Staff are Teresa Hollander, M.D., General Pediatrician and Beverly Myers, M.D., General Pediatrician
• Through the support of the Orange County Children and Families Commission, a Volunteer in Service to America (VISTA) project staffer is being recruited.

The local school nurses are noting an increase in the incidence of lesions that are fluid- filled but do not appear to be vesicular in nature. These lesions are appearing in previously immunized children, and are being treated as recurring chicken pox by the nurses. The school nurses are requiring these children to be isolated per the school policy until the "vesicles" are crusted. Some of our local school nurses have expressed concern, when an occasional community physician diagnoses these lesions as "flea bites" because the vesicles are not typical for chicken pox, and then does not recommend isolation measures.

Dr. Phillip Brunell, Editor of The Infectious Disease Journal, was contacted for his input regarding these vesicles and writes " previously vaccinated children who get breakthrough chicken pox can be infected with the wild virus, which can be spread to contacts. These children should be treated as any other child with chicken pox and isolated per school policy until the lesions are crusted."

We recommend that these lesions be treated as recurring chicken pox in a previously immunized child and the children isolated until these lesions are crusted over. The risk to other children exposed to these lesions, especially immunocompressed children, is high and therefore isolation measures are essential to their welfare.

More than 900 children under the age of 15 drowned in swimming pools in 2002. In addition, 2,700 children nearly drowned that year, many of which resulted in permanent brain damage. Drowning is the leading cause of injury-related death among Orange County children under the age of 5 years. Most drowning incidents occur in residential pools or spas. So far this year, four children under the age of 5 have drowned and 7 have nearly drowned in Orange County. The majority of these incidents occurred in residential or community pools.

The California Chapter 4, American Academy of Pediatrics Injury Prevention Program has developed this new, full color pool safety poster. Please hang this poster in your office part of our public education campaign.

Funded by the Children and Families Commission of Orange County, the Injury Prevention Program provides expertise, evidence-based guidelines and public policy advocacy for childhood injury prevention. Local pediatricians participate in the Chapters' programs and advocacy efforts aimed at reducing childhood injuries. Established in 2001, in its short history, the Injury Prevention Program has achieved local, state and national recognition.

Community-acquired MRSA: Anecdotal reports of community-acquired MRSA continue to increase in Orange County and throughout the U.S. Consider MRSA in your patients with skin and soft tissue infections. For more information, see http://www.ochealthinfo.com/epi/mrsa/providers.htm for physicians and http://www.ochealthinfo.com/epi/mrsa/index.htm for patients.

West Nile Virus: West Nile Virus has been detected in birds and mosquitoes in Orange County for months and as the warm weather approaches, human cases are expected at any time. For more information, see our WNV Newsletter or email epi@ochca.com to be placed on the distribution list. Additional WNV information is available at http://www.west nile.ca.gov/ or http://www.cdc.gov/ncidod/dvbid/westnile/.

Meningococcal Disease: With the licensure and ACIP/AAP approval of the new conjugate meningococcal vaccine, surveillance of N. meningitidis serogroups is especially important and we ask that all isolates from confirmed cases and blood/CSF from culture-negative but highly suspicious cases be forwarded to the Orange County Public Health Laboratory.

Pertussis: Pertussis cases continue to increase in Orange County and California. A new pertussis vaccine for adolescents and adults has been approved by the FDA but ACIP and AAP recommendations are still pending. Stay tuned.

For more information on any of the above topics, please call Epidemiology at 714-834-8180.

email: ca4aap@sbcglobal.net

phone: 714/971-0695

web: http://www.aapca4.org