CHOC Becomes SCID Referral Center

CHOC Children’s is pleased to have recently become a referral center for severe combined immune deficiency (SCID), filling a regional gap that once required Orange County infants to go outside the county for care.

Led by Drs. David Buchbinder, Wan-yin Chan, Diane Nugent and Jasjit Singh, the immunodeficiency program is a multidisciplinary effort crossing multiple specialties at CHOC including allergy and immunology, hematology and infectious disease

Though they appear healthy at birth, infants with this primary immunodeficiency disease lack T lymphocytes, one of the white blood cells that help fight infections. 

Babies with SCID cannot fight even the most innocuous infections and often die. The condition is considered by the medical community as a pediatric emergency.

“Prior to development of SCID newborn screening, the diagnosis would be delayed,” Dr. Chan says. “Often times these patients would not get sick until after 6 months of age. No one would know they were affected until the antibodies from their mother would wane. They end up with life threatening infections with serious complications often resulting in death.”

However, studies show that early bone marrow or stem cell transplants can improve outcomes significantly, Dr. Chan says.

Survival rates increase to 94 percent if administered to an affected infant by age 3 ½ months. However, if transplants occur after that age, survival rates increase to only 70 percent, underscoring the importance of early detection and intervention. 

To that end, California became one of the first states to add SCID to its list of recommended newborn screenings in 2010. In the years since, all states have followed suit. 

Under CHOC’s program, immunodeficiency team physicians review each case of Orange County babies who test positive in newborn screenings for SCID and ask parents to immediately seek a confirmatory blood test for the infant, Dr. Chan says.

If the additional tests confirm the diagnosis, patients are urgently admitted to CHOC for workup and treatment, Dr. Chan says.

Since CHOC’s center was formed in August, more than 20 patients have been flagged in the surrounding communities and each individual case has been reviewed by the immunodeficiency team in collaboration with local pediatricians.

Those urgent blood tests confirmed the presence of SCID or a SCID-like disorder in more than 25 percent of cases thus far. 

Protect Infants from Next Wave of Pertussis

The California Immunization Coalition, along with Dr. Jasjit Singh, director, infection prevention and epidemiology at CHOC Children’s, recommend the following pertussis guidelines for pediatricians and their patients.

As pertussis increases to peak levels every 3- 5 years, California is due for its next epidemic by 2019. Young infants remain at highest risk of hospitalization and death from pertussis. Since 2010, at least 2,800 California infants younger than four months of age have contracted pertussis. Most of these infants have been hospitalized, and at least 18 have died.

Please consider the following measures to protect infants against pertussis:

Prevention

  • Prenatal Interview – Promote Prenatal Immunization! Remind parents and prenatal providers to give prenatal Tdap vaccine to women between 27 and 36 weeks of gestation of each pregnancy, regardless of vaccination history. Encourage vaccination of household and caregivers.
  • Administer the first dose of DTaP vaccine to infants promptly at 6-8 weeks of age. A dose as early as 6 weeks will help protect infants sooner if their mothers did not receive Tdap during pregnancy. Complete the DTaP series without delay.
  • Maternal immunization is associated with infant survival. Receipt of Tdap by mothers between weeks 27 and 36 of pregnancy and receipt of DTaP by infants prior to illness greatly reduce the risk of death from pertussis.

Presentation

Pertussis should be considered in any infant without a documented fever who presents with coryza, cough (especially paroxysmal), apnea, gagging, or post-tussive emesis.

Suspect pertussis in adolescents and adults with prolonged cough, and test and treat promptly to prevent transmission to infants.

Testing

Obtain nasopharyngeal swabs for pertussis PCR testing to confirm the diagnosis. Additional guidance is at www.cdc.gov/pertussis/clinical/diagnostic-testing/specimen-collection.html.

  • In infants, check white blood cell counts with differential – a leukemoid reaction is associated with life-threatening pertussis. A WBC count greater than 10,000 cells/mm3 with ˃50% lymphocytes should be repeated 24 hours later; increasing lymphocytosis should prompt additional monitoring and treatment. A WBC of >20,000 cells/mm3 with >50% lymphocytosis should be considered as a very strong indication that the infant has pertussis.

Treatment

Azithromycin (10mg/kg/day in a single dose for five days) for infants less than 6 months of age.

Empiric treatment is appropriate while awaiting the results of PCR testing.

Additional clinical guidance and access to consultation can be found at eziz.org/assets/docs/Pertussis-YoungInfants2011.pdf.

Further information, California case counts, and clinical guidance for pertussis can be found here.

 

Nasal Flu Vaccine Not Recommended This Season – What Patients and Families Should Know

An advisory committee of the Centers for Disease Control and Prevention (CDC) recently recommended that the nasal spray influenza vaccine not be used this upcoming flu season. In this Q&A, Dr. Jasjit Singh, medical director of infection prevention and control at CHOC Children’s, offers an explanation for your patients and their families.

Q: What does this mean for influenza vaccine recommendations for the upcoming flu season?

A: All individuals over the age of 6 months are recommended to get the influenza vaccine, and that will continue to be the case.  However, for this season at least, the nasal flu vaccine is not an option. Therefore, parents will need to plan for their children to get the flu shot this upcoming season.

Jasjit Singh, M.D.
Jasjit Singh, M.D.

Q: How does the standard flu shot differ from the nasal spray version in terms of composition and effectiveness?

A: Both the flu shot and the nasal spray contain the anticipated predominant circulating strains of influenza every year, but the nasal spray strains are made of weakened live virus while the flu shot is made of inactivated viral components. The CDC reviewed data from this past season that suggested that the nasal spray did not perform as well as it had in the past.

Q: Besides ensuring their children get a flu shot, what else can parents do to help prevent the flu?

A: In addition to ensuring their child is vaccinated against the flu every year, there are many things parents and other caregivers can do to help prevent the flu. Use proper hand-washing techniques, use respiratory etiquette, and stay home from work or school if you are sick with the flu, to prevent spreading it to others.

Q: What do you anticipate will be parents’ reactions to this recommendation? Is there anything else you’d like them to know? Is there anything else you’d like to share with parents about the importance of vaccinations?

A:  Vaccinations are one of the most effective public health measures that have been developed and they save thousands of lives each year. Influenza vaccinations are important because young children can get quite sick from the flu, and some even require hospitalization. Every year there are pediatric deaths in the U.S. due to influenza, about half of which occur in normal healthy children. Children can pass influenza on to the elderly or other fragile members of our community. It’s important for adults to get the flu shot too, particularly those who are caring for young children. Even though the nasal spray is not an option for vaccinating this particular season, it is still important to have your child vaccinated for this year.

 

Dr. Katherine Andreeff Discusses Bacterial, Viral Infections

Patient history and physical examinations are sometimes not enough to determine whether an infection is viral or bacterial, Dr. Katherine Andreeff, a hospitalist at CHOC Children’s, tells “American Health Journal.”

In these cases, blood work and imaging may be required to make an accurate diagnosis, says Dr. Andreeff. Hand-washing and vaccination can help prevent infections, whether bacterial or viral.

Learn more about infections and key questions parents should ask physicians in “American Health Journal,” a television program that airs on PBS and other national network affiliates that reach more than 30 million households.

Each 30-minute episode features six segments with a diverse range of medical specialists discussing a full spectrum of health topics. For more information, visit www.discoverhealth.tv.

Katherine Andreeff, M.D., attended medical school at Wayne State University in Detroit. She completed her pediatric training including a chief residency at CHOC Children’s. She is active in meningitis research and has published a chapter on renal tubular acidosis.

Get more information about referring patients to CHOC, including a referral information directory, services directory and referral guidelines.


Curiosity Drives Infectious Diseases Research

A CHOC Children’s investigator-initiated trial into neonates with necrotizing enterocolitis (NEC) has discovered key microbial and survival differences in patients who develop blood stream infections. These findings may help explain why premature infants with intestinal pathology experience more complications and higher mortality rates. CHOC Infectious Diseases Medical Director Antonio Arrieta, M.D., and his team have also described better outcome of bacteremia in full-term neonates when it is associated with urinary tract infections (UTI). This, they hope, will lead to changes in how both populations are managed.

Dr. Arrieta and CHOC Resident Jordan Fisher, M.D., presented their UTI data in November 2011 at the World Society of Pediatric Diseases in Melbourne. In May 2012, the data on NEC was presented to the European Society for Pediatric Infectious Diseases in Greece.

These are two of the many investigatorinitiated research trials Dr. Arrieta and his team conduct with CHOC residents to answer questions they hope will ultimately impact children everywhere.

“Our investigator-initiated trials are a small portion of the total research we do, but these are our ideas. We develop the concepts, write the protocols, seek funding, and hope to change the lives of children throughout the world.” — CHOC Infectious Diseases Medical Director Antonio Arrieta, M.D.

CHOC Infectious Diseases also participates in several collaborative pharmacokinetic (PK) trials to reassess the efficacy, safety and dosing of new antimicrobial agents. Many of these trials are industry-sponsored and focus on neonatal, oncology and cystic fibrosis patients. CHOC Infectious Diseases is also collaborating with Duke University in a Eunice Kennedy Shriver National Institute of Child Health and Human Development-funded grant to conduct PK trials for neonates, and with the University of California, Los Angeles on a NIH grant for pediatric HIV.

Training Tomorrow’s “Clinician Scientists”

During his medical training, Dr. Arrieta was taught to “always stay curious and answer questions with research.” He passes that philosophy on to CHOC residents by encouraging them to also participate in research and submit their results. As a bonus, the resident gets to present any accepted poster or paper. In recent years, CHOC residents have presented at meetings in Australia, Switzerland and France.

“We believe emphatically in training scientific doctors because answering questions through research is integral to being a clinician,” Dr. Arrieta said. “Without that, they will not be complete. Many CHOC residents have gone on to become scientists and teachers, and that makes us very proud of what we do.”


Preliminary Pneumococcal Vaccine Data Soon Available

CHOC Children’s Infectious Diseases is assessing the impact of a new vaccine in reducing invasive pneumococcal disease and pneumonia. Similar to previous work conducted after the introduction of the first pneumococcal conjugate vaccine, and published in the Journal of Pediatric Infectious Diseases in 2011, CHOC is conducting an annual three-year interim analysis and then a final five-year study. Initial results will be available soon to share with parents about the importance of immunization. To learn more about Research at CHOC, please visit www.choc.org/research.