Congenital Malformations Registry - Summary Report

Section VI - Current Topics

Conducted by the Congenital Malformations Registry, 2007-2008

Background

The Congenital Malformations Registry (CMR) of the New York State Department of Health (NYSDOH) has been performing surveillance for all major birth defects in children born in New York since 1983. Bound by strict confidentiality laws, the CMR is unable to disclose names of children identified with birth defects. Therefore, when children are reported with birth defects, the CMR staff send the parents of affected newborns informational mailings about potential services that may assist them. In 2004, in response to the Healthy People 2010 goal 1, the CMR enhanced the mailing for families of children born with oral clefts. Families now receive an introductory letter, a brochure published by the Cleft Palate Foundation titled "Cleft Lip and Cleft Palate – The First Four Years," and a list of craniofacial or oral cleft teams located across New York State (NYS). The list of teams is reviewed periodically for accuracy and completeness and it is not the intention of the CMR to endorse any particular team, but to provide the families with information.

Interdisciplinary approach of caring for children with craniofacial anomalies is recommended by the American Cleft Palate-Craniofacial Association (ACPA). Two national surveys of cleft palate and craniofacial teams were conducted and published in the 1990s. The 1992 report described questionnaire results of responding teams listed in the 1988 ACPA directory and the 1998 paper discussed results of the ACPA self-assessments performed by teams in 1996. The reports provided an overview of the status and makeup of interdisciplinary teams in the United States. Our interest was to obtain information about the cleft palate and craniofacial teams located in New York, a large state with geographically diverse areas and approximately 300-350 cases of orofacial clefts among all births each year. This paper describes an overview of the responses to a written questionnaire sent to New York team leaders in an effort to obtain more information about the teams, their patient populations, medical care they were able to dispense, and barriers they faced.

Methods

A written questionnaire was developed that inquired as to the team designation, patient population in terms of numbers and ages, referral activities, specialists serving on the team, barriers to care the children received, and support provided to parents, families, and other health care professionals. The survey was sent to the leaders of the teams located in New York. If no response was received, contact was made by email; otherwise, a second contact was made by mail. A third attempt to encourage participation of non-responders was attempted by mail that was addressed to both the team leader and the team coordinator. Descriptive analysis was performed to provide an overview of the responses.

Results

All but one team indicated that they had a designated team coordinator on staff. Team coordinators included the following: nurses, nurse practitioners, speech pathologists, physicians, surgeons, or individuals with college degrees (B.S. or A.S.). Eleven teams offered continuing education opportunities to the team members, and 11 provided continuing education to non-team members. The teams were asked how often they usually meet face-to-face each year. The range of responses was from 6 to 40 times with a median number of 12 meetings per year. All teams said that they routinely formed treatment plans for each patient.

Table 1. Number who responded that the discipline served on their team and number who would add the discipline if possible
Discipline/specialist Served on team Would add to team
Otolaryngolist 14 0
Orthodontist 14 0
Speech Pathologist 14 0
Plastic surgeon 13 1
Pediatric dentist 13 1
Geneticist 11 2
Neurosurgeon 11 0
Audiologist 10 1
Prosthodontist 9 2
Clinical social worker 8 2
Ophthalmologist 8 1
Radiologist 7 0
Nurse practitioner/Nurse specialist 6 4
General dentist 6 0
Psychologist 5 5

Because many families must travel when their children receive care or surgery, teams were asked about support given in terms of arrangements for lodging. Eleven of the teams indicated that arrangements were available for families (i.e., hospital rooms, local hotels, hospital affiliated hotels or apartments, and Ronald McDonald Houses).

Support groups and parent networks can be very important sources of information and comfort to parents of children born with craniofacial malformations. Eleven of the 15 teams said they were aware of community support groups or parent networks in their area available to families; however, only three teams sponsored any support groups or parent networks.

Finally, the survey recipients were asked to comment on any issues raised by the survey, or any other thoughts they wanted to share regarding treatment of children in New York State with craniofacial anomalies. Six comments related to medical insurance, specifically low reimbursement for care or denial for important services such as orthodontics and psychosocial care, especially for children as they enter the teen years. Other issues important to the responders included lack of institutional support for the team, problems related to referrals, laws they viewed as barriers to genetic testing, and the need for more consolidation of care to larger centers with comprehensive capabilities for pediatric care.

Discussion

New York is a very diverse state with population sizes that vary drastically among its 62 counties. Ten of the 18 teams are located in New York City or Long Island, while the remaining eight are located in cities across the State (Buffalo, Rochester, Syracuse, Elmira, Binghamton, Schenectady, Albany and Valhalla). The "upstate" cities vary in size, which may account for the large range noted for numbers of patients seen by each team. While the regions containing small to larger cities have organized craniofacial care teams, there are many areas in New York that are quite rural and distant from the existing teams. However, geography and transportation were not considered high on the list of noted barriers to care.

What did seem to be of concern to most respondents were issues related to insurance coverage. It is likely that team staff spend large amounts of time trying to encourage insurance companies to cover expenses for services and care of children that the medical community deem vital. Insurance coverage for care and treatment of children into the teen years and continuing into adulthood is important to the overall well being of each individual born with a craniofacial anomaly, and may play a significant role in individual's future well-being.

Reimbursement for services may have an impact on medical professionals who choose specialties that are important for the formation and success of interdisciplinary teams. Not all of the New York State teams have staff that encompass all the disciples recommended by the ACPA. Two-thirds of the respondents who said they felt limited to the services they could provide sited staffing issues as a problem. The ACPA recognizes psychological services as an integral component in interdisciplinary team care ; however, six responding teams did not have a psychologist or clinical social worker on staff to monitor the development of the children or to support the families.

New York State has 18 organized care teams identified by CMR staff; however, there may be other organized teams that were not identified. Furthermore, there may be children with craniofacial anomalies who are followed by physicians and surgeons who are very dedicated to the well-being of their patients but who do not work as a member of a team. While it is recommended that affected children be enrolled in interdisciplinary team care settings, ultimately it is the quality of care they receive that is important. Measuring outcomes of treatment and services received by children is a difficult undertaking. CMR staff are collaborating on several projects with researchers in Iowa, Arkansas and New York City to study the access to medical care and outcomes experienced by children affected by selected craniofacial malformations. The collaborative efforts also include a survey of primary care physicians and pediatricians in New York, Iowa and Arkansas to obtain information about their experiences in caring for children with orofacial clefts. The survey of craniofacial and cleft care teams in New York described here, in addition to the information drawn from the other research projects mentioned might allow for a more complete picture of the care available to children and their families of New York who are affected by craniofacial anomalies.

References

  1. U.S.Department of Health and Human Services. Healthy People 2010. 2nd ed. Washington, DC: U.S. Government Printing Office; 2000 Nov.
  2. American Cleft Palate-Craniofacial Association. Parameters for evaluation and treatment of patients with cleft lip/palate or other craniofacial anomalies. Cleft Palate-Craniofac J. 1993;30 Suppl:S1-16.
  3. Pannbacker M, Lass NJ, Scheuerle JF, English PJ. Survey of services and practices of cleft palate-craniofacial teams. Cleft Palate Craniofac J. 1992 March;29(2):164-7.
  4. Strauss RP, ACPA Team Standards Committee. Cleft palate and craniofacial teams in the United States and Canada: a national survey of team organization and standards of care. . Cleft Palate-Craniofac J. 1998;35(6):473-80.
  5. New York State Department of Health Congenital Malformations Registry. New York State Department of Health Congenital Malformations Registry summary report - statistical summary of children born in 2002-2004 and diagnosed through 2006. 2007.