Congenital rubella syndrome surveillance Dr Esteghamati national EPI

Congenital rubella syndrome surveillance Dr Esteghamati, national EPI Manager Pediatrician

Number of Vaccines in the Routine Childhood and Adolescent Immunization Schedule 1985 1995 2006 Measles Rubella Mumps Diphtheria Tetanus Pertussis Polio Hib (infant) Hep. B Varicella Measles Rubella Mumps Diphtheria Tetanus Pertussis Polio Hib (infant) Hep B Varicella Pneumococcal disease Influenza Meningococcal disease Hep A Rotavirus HPV 7 10 16

Congenital Rubella Syndrome • severe bilateral deafness • severe bilateral visual defects • cataract • corneal opacity

WHO/UNICEF Global Strategic Plan for Measles Mortality Reduction, 2001 -2005 Each country should assess its rubella situation Countries undertaking measles elimination may consider the opportunity to eliminate rubella as well by using MR or MMR vaccine. n n

Percentage of Member States, by WHO region, with routine rubella vaccinationwide, 1996 versus 2004 Source: WHO/IVB database, August 2005

WHO Regions by Rubella/CRS Control Target Rubella Elimination/CRI Prevention - 2 CRS Elimination - 2010 Rubella Elimination 2010

WHO/UNICEF Global Strategic Plan for Measles Mortality Reduction, 2001 -2005 n n Each country should assess its rubella situation Countries undertaking measles elimination should take the opportunity to eliminate rubella as well by using MR or MMR vaccine in their childhood immunization program and also in measles campaigns.

n Rubella is a viral illness caused by a togavirus of the genus Rubivirus and is characterized by a mild, maculopapular rash. n When rubella infection occurs during pregnancy, especially during the first trimester, the risk of fetal infection may be as high as 90%.

Rubella Complications Arthralgia or arthritis children adult female rare up to 70% Thrombocytopenic purpura Encephalitis Neuritis Orchitis 1/3000 cases 1/6, 000 cases rare

n Consequences of congenital rubella infection include abortions, miscarriages, stillbirths, and a constellation of severe birth defects known as CRS. n The most common congenital defects are cataracts, heart defects, hearing impairment, and developmental delay.

Congenital rubella syndrome (CRS) a) infection of fetus during first trimester of pregnancy b) at least 20% of infants have severe birth defects i. neurosensory deafness ii. blindness (total or partial; cataracts are especially common) iii. congenital heart disease iv. microcephaly with mental retardation c) other symptoms associated with CRS i. bone translucency and retarded growth ii. hepatosplenomegaly iii. Intrauterine growth retardation d) 10 -20% of babies with CRS die within 1 year e) 20 % will develop insulin dependent diabetes mellitus as young adults f) CRS babies continue to shed Rubella virus from their throats for several months up to a year after birth and pose a serious risk to pregnant women.

Congenital rubella syndrome (CRS)

Epidemic Rubella – United States, 19641965 n n n 12. 5 million rubella cases 2, 000 encephalitis cases 11, 250 abortions (surgical/spontaneous) 2, 100 neonatal deaths 20, 000 CRS cases q q q Deaf - 11, 600 Blind - 3, 580 Mentally retarded - 1, 800

n Rubella and CRS continue to be global burdens. n With the increased use of rubella vaccine; however, the burden of rubella infection should decrease in the future.

n Despite routine rubella vaccination among children, some rubella outbreaks continue in the U. S. n These outbreaks are primarily confined to groups who traditionally refuse vaccinations and to adults from countries without a history of rubella vaccine. n Throughout the 1990 s, the majority of infants with CRS were infants of mothers who fall into these categories.

n Worldwide, it is estimated that there are more than 100 000 infants born with congenital rubella syndrome (CRS) each year. n In 2001, 123 countries/territories reported a total of 836 356 rubella cases. In the future more countries are expected to report on rubella as a global measles/rubella laboratory network is further developed under the coordination of the WHO.

Rubella vaccine use varies by stage of economic development: 100% for industrialized countries, 71% for countries with economies in transition, and 48% for developing countries.

n Congenital rubella syndrome (CRS) can lead to deafness, heart disease, and cataracts, and a variety of other permanent manifestations.

n n Hearing loss occurs in 70 -90% of CRS cases and in 50% is the only sign of CRS, although it is often not detected initially. Evidence for underestimation.

In developing countries, the burden of CRS can be assessed by: surveillance of CRS; surveillance of acquired rubella; age-stratified serosurveys; serosurveys documenting the rubella susceptibility of women of childbearing age.

Rates of CRS per 1000 live births Jamaica 1. 7 Israel 1. 7 Oman 0. 7 Panama 2. 2 Singapore 1. 5 Sri Lanka 0. 9 Trinidad and Tobago 0. 6 *These rates are similar to those reported from industrialized countries during the pre-vaccine era.

Importance of rapid case identification n As infants with CRS may shed virus for prolonged periods, they should be identified as early in life as possible in order to prevent further spread of the virus. n Infants with CRS may shed virus up to 1 year of age or longer and should be considered infectious until they are at least 1 year old or until two cultures of clinical specimens obtained 1 month apart are negative for rubella virus after age 3 months.

n Early diagnosis of CRS facilitates early intervention for specific disabilities. n Significant enhancement of speech and language development, and eventual success in school, for children with hearing impairment if they are identified early and intervention begins immediately.

Importance of surveillance n The goal of rubella vaccination is to prevent congenital rubella infection. n Surveillance data are used to identify groups of persons or areas in which disease control efforts can reduce disease incidence To evaluate the effectiveness of disease prevention programs and policies. n

Promoting awareness that rubella and CRS still exist. n Efforts should continue to promote physicians’ awareness of the possibility of rubella and CRS, especially when evaluating patients with suspected measles who have negative serologic tests for acute measles infection (negative serum measles Ig. M).

Patient with fever and rash Sampling Test for M. Ig. M Negative Positive Check for Rubella Confirmed Measles case Positive Negative Confirmed Rubella Check for parvo V. B 19

Promoting awareness of high-risks groups for rubella infection and CRS births n Rubella vaccine is not administered routinely in many countries, Hence Health-care providers should have a heightened index of suspicion of rubella and CRS births in individuals from countries without a history of routine rubella vaccination programs.

Conducting active surveillance n n n Surveillance for CRS should be implemented when confirmed or probable rubella cases are documented in a setting where pregnant women might have been exposed. Women who contract rubella while pregnant should be monitored for birth outcome, and a rubella-specific Ig. M antibody test should be performed on the infant after birth. Health-care providers should be advised to evaluate infants born with conditions consistent with CRS and to perform a rubella-specific Ig. M antibody test on infants suspected of having CRS.

In order to identify CRS cases in infants, it is important to investigate rash illness in pregnant women. will be most practical in countries where women attend antenatal clinics during the first 16 weeks of pregnancy, as the risk of CRS is low in women infected after the first trimester.

Searching laboratory records n n n laboratory records may provide reliable evidence of previously unreported serologically confirmed or culture-confirmed cases of congenital rubella syndrome. Infants with CRS have been identified by including the serological results for toxoplasmosis, rubella, cytomegalovirus, and herpes (TORCH) agents in laboratory records. This may be particularly useful in hospitals.

Comparing other data sets n n Birth defects registries may reveal unreported CRS cases. In addition, children with CRS whose cases were never reported may be enrolled in schools for the deaf or blind. Pediatric specialty clinics caring for children with mental retardation, congenital heart defects, congenital deafness and hearing impairment, congenital cataracts, and growth retardation may be a source of unreported CRS patients. These activities should be undertaken following rubella outbreaks as part of enhancing surveillance for CRS.

Reviewing hospital discharge data and linkages with newborn hearing screening programs n n n Reviewing hospital discharge data in high-risk areas has proven useful in identifying undiagnosed cases of CRS. Infants with discharge codes consistent with CRS may then be categorized according to the CRS case definition, allowing for greater insight into the rates of CRS in high-risk populations. Furthermore, if newborn hearing screening is routinely performed, infants identified through screening with hearing deficiencies or progressive hearing loss may also be tested for CRS, as hearing impairment is the most common single defect associated with CRS.

n Cases of indigenous rubella have occurred among susceptible persons providing care for infants with CRS. n Persons having contact with infants with CRS should be immune to rubella. n Infants with CRS should be placed in contact isolation.

Comparison of 20 th Century Annual Morbidity and Current Morbidity: Vaccine-Preventable Diseases 20 th Century Annual Morbidity† Disease 2006 (final)†† Percent Decrease Smallpox 48, 164 0 100% Diphtheria 175, 885 0 100% Measles 503, 282 55 > 99% Mumps 152, 209 6, 584 96% Pertussis 147, 271 15, 632 89% Polio (paralytic) 16, 316 0 100% Rubella 47, 745 11 > 99% Congenital Rubella Syndrome 823 1 > 99% Tetanus 1, 314 41 97% Haemophilus influenzae 20, 000 208* 99%

Surveillance of congenital rubella syndrome (CRS) requires a comprehensive system to detect suspected CRS cases in infants who present to a range of different health services. Most industrialized countries have established surveillance of CRS with national disease notification programs and/or birth defects monitoring programs in developing countries CRS cases are likely to be underreported in areas and among populations where a high proportion of births occur at home and Where neonatal and childhood deaths are often unreported

The following sites and specialists should be provided with written guidelines and training: n n n n Sites that routinely participate in surveillance for EPI diseases Neonatal wards and neonatal intensive care units, Obstetrics services, including obstetricians and midwives, General hospitals, including the pediatric ward, Referral hospitals, Ophthalmologists, optometrists Otologists and audiologists, Cardiologists and cardiac surgeons.

Surveillance of rubella is case-based rubella surveillance likely to be most practical when countries have reached is only recommended in countries the stage of measles/rubella elimination, that have established a rubella elimination goal

Routine CRS surveillance focuses on identifying infants 0 -11 months of age with CRS, although some defects associated with CRS may not be detectable until older ages In children older than one year it is very difficult to confirm rubella as the specific etiology of congenital malformations or birth defects.

CRS case definitions (1) Suspected CRS: A child <1 year with maternal history of rubella in pregnancy and/or heart disease eye signs Cataract Diminished vision Pendular eye movement (nystagmus) Squint Smaller eye ball (micropthalmos) Larger eye ball (congenital glaucoma) deafness

CRS case definitions (2) Clinically confirmed CRS: A child <1 year with two complications in group (a) or one from (a) and one from (b) (a): cataract, congenital glaucoma, congenital heart disease, loss of hearing, pigmentary retinopathy (b): purpura, splenomegaly, microcephaly, mental retardation, meningoencephalitis, radiolucent bone disease, jaundice with onset within 24 hours after birth.

CRS case definitions (3) Laboratory-confirmed CRS: An infant with a positive blood test for rubellaspecific Ig. M and clinically-confirmed CRS. Congenital rubella infection (CRI): An infant with a positive blood test for rubellaspecific Ig. M who does not have clinicallyconfirmed CRS.

Suspected CRS Refer suspected CRS case to qualified physician Blood sample not obtained Examination by qualified physician Discard Clinically-confirmed CRS Blood sample(1 ml ) obtained Rubella Ig. M negative Rubella Ig. M positive + Discard Not clinically- confirmed Congenital Rubella Infection (CRI) Clinically-confirmed CRS Laboratory-confirmed CRS