Ehlers Danlos

Ehlers-Danlos Toolkit for School Nurses

 

Disclaimer: This toolkit is intended to provide resources for school nursing practice as it relates

to the care of students with Ehlers-Danlos in Oregon Schools. This is not medical nor nurse

practice advice and is not prescriptive as it relates to individual students or individual nursing

practice. These resources do not override individual assessment, and clinical judgment. Nurses

are responsible for their own assessments and practice.

 

Disease Description Pertaining to School 

Ehlers-Danlos Syndrome (EDS) is a rare hereditary condition involving the connective tissues of

the body (Malfait et al., 2017). It is estimated that 1 in 5000 individuals worldwide are affected

by EDS, but a new study conducted in Wales suggests a higher prevalence (Demmler et al.,

2019). This condition can create joint hypermobility, skin elasticity, and tissue fragility. There

are currently 13 subtypes of EDS, with each subtype having a unique impact on the body

systems (Malfait et al., 2017). Individuals with heritable connective tissue disorders, like EDS,

have reported increased fatigue, pain, disability and decreased general health when compared

to individuals without. It is important to assess the student’s EDS type and individual impact

when identifying accommodations (Warnink-Kavelaars et al., 2021). These symptoms can

greatly impact a student’s ability to access their education and can impact educational gains. It

is crucial to assess each student with EDS individually as health needs and accommodations will

vary.

 

For a quick summary of the The 2017 international classification of the Ehlers-Danlos

syndromes, please click on the link below. It is important to note that not all criteria are needed

for a diagnosis. For more information on diagnostic criteria, please review the The 2017

international classification of Ehlers-Danlos Syndromes at the link found in the basic disease

information section.

 

Summary of 13 EDS diagnostic criteria

 

Ehlers Danlos have a variety of subtypes that impact various systems in the body, most notably

vasculature. Depending on systems impacted, supportive information may be found in the

following toolkits:

Cardiac

Seizures

Bleeding Disorders

 

Basic Disease Information

For a complete review of the 13 EDS types, please review the following:

Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L.,

Bowen, J. M., Brady, A. F., Burrows, N. P., Castori, M., Cohen, H., Colombi, M., Demirdas,

S., De Backer, J., De Paepe, A., Fournel-Gigleux, S., Frank, M., Ghali, N., … Tinkle, B.

(2017). The 2017 international classification of the ehlers-danlos syndromes. American

Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 8–26.

https://doi.org/10.1002/ajmg.c.31552

 

Legal Practice Standards

At the forefront of nurse case management for school nurses, is the understanding of scope of

practice as it relates to case management and delegation of nursing tasks in a community

setting:

● Oregon State Board of Nursing Oregon Nurse Practice Act

● Oregon Revised Statute ORS 339.866 Self-administration of Medication by Students

● Oregon Administrative Rule OAR 581-021-0037 Administration of Medication

 

 

Evidence Based Practice

Evidence Based Practice Grid

 

Public Access Resources

Note: the following are national or regional resources that serve as a reference, they may or

may not be consistent with Oregon law and do not supersede Oregon Nurse Practice.

 

School toolkit for JHS and EDS

A free resource for staff to help support pupils with joint hypermobility syndrome (JHS) or the Ehlers-Danlos syndromes (EDS)

EDS School Toolkit

 

Genetic and Rare Diseases Information Center

GARD is a public health program of the National Institutes of Health (NIH), National Center for

Advancing Translational Sciences (NCATS) that provides health information about rare diseases for the public.

Ehlers-Danlos Syndromes

 

Go See the Nurse: https://rarediseases.info.nih.gov/diseases/6322/ehlers-danlos-syndromes

 

Ehlers-Danlos Syndrome (EDS) – Guide For The School Nurse

 

Professional Development

 

Ehlers-Danlos Society

EDS ECHO is a series of educational programs for healthcare professionals across all disciplines

who want to improve their knowledge and ability to care for people with Ehlers-Danlos

Syndrome (EDS).

Ehlers-Danlos: Project Echo

StatPearls

Ehlers-Danlos Continuing Education

Ehlers-Danlos Society

Information for Healthcare Professionals

Chronic Pain Partners

Ehlers Danlos Syndrome Nursing Education Program

National Institutes of Health

● Miklovic T, Sieg VC. Ehlers-Danlos Syndrome. [Updated 2022 Jun 9]. In: StatPearls

[Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from:

https://www.ncbi.nlm.nih.gov/books/NBK549814/

Osmosis

Ehlers-Danlos syndrome (Year of the Zebra) (7:57(=)

 

Templates

 

The following templates are modifiable for use by school nurses as it relates to their practice

and should be used consistently with Oregon State Board of Nursing, Delegation Laws and local

school policy.

 

Individualized Health Plans

An individualized health plan is the totality of the plan that includes all applicable procedures

for students with chronic disease. Because these are intended to be individualized, the attached

is an example of a compiled IHP:

Ehlers-Danlos IHP Template

Ehlers-Danlos Teacher Notification Template

 

Authorizations

Authorization for Specialized Care Template

 

Prescriber Order Templates

Prescriber Order Template

 

Medication Administration and Self-Medication Agreement

Medication Authorization

 

504

Ehlers-Danlos SAMPLE ACCOMODATIONS WITH RATIONALE

 

Author: Kristiina Gangner

Peer Reviewer: Jan Olson

Editorial Reviewer: Ann Occhi

 

References

Demmler, J. C., Atkinson, M. D., Reinhold, E. J., Choy, E., Lyons, R. A., & Brophy, S. T.

(2019). Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum

disorder in Wales, UK: A National Electronic Cohort Study and case–control comparison.

BMJ Open, 9(11). https://doi.org/10.1136/bmjopen-2019-031365

 

Feldman, E. C., Hivick, D. P., Slepian, P. M., Tran, S. T., Chopra, P., & Greenley, R. N.

(2020). Pain Symptomatology and management in pediatric Ehlers–danlos syndrome: A

Review. Children, 7(9), 146. https://doi.org/10.3390/children7090146

 

Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L.,

Bowen, J. M., Brady, A. F., Burrows, N. P., Castori, M., Cohen, H., Colombi, M., Demirdas,

S., De Backer, J., De Paepe, A., Fournel-Gigleux, S., Frank, M., Ghali, N., … Tinkle, B. (2017).

The 2017 international classification of the ehlers-danlos syndromes. American Journal of

Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 8–26.

https://doi.org/10.1002/ajmg.c.31552

 

Miklovic T, Sieg VC. Ehlers Danlos Syndrome. [Updated 2021 Jul 10]. In: StatPearls

[Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from:

https://www.ncbi.nlm.nih.gov/books/NBK549814/

The Ehlers Danlos Society. (2020, May 28). The types of EDS. The Ehlers Danlos Society.

Retrieved from https://www.ehlers-danlos.com/eds-types/#mEDS

 

Warnink-Kavelaars, J., de Koning, L., Rombaut, L., Alsem, M., Menke, L., Oosterlaan, J.,

Buizer, A., & Engelbert, R. (2021). Heritable connective tissue disorders in childhood:

Increased fatigue, pain, disability and decreased general health. Genes, 12(6), 831.

https://doi.org/10.3390/genes12060831