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    Ehlers-Danlos Syndromes (EDS) : Fiona Li Pharm D Candidate University of Saint Joseph School of Pharmacy

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    Diogo Capella
    Based on the document, the patient has Ehlers-Danlos syndrome (EDS), specifically vascular type. She presented with severe right lower quadrant pain and vomiting. Her treatment focuses on pain management with opioids and NSAIDs, as well as monitoring for complications specific to vascular EDS such as bleeding disorders.

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    Ehlers-Danlos Syndromes (EDS) : Fiona Li Pharm D Candidate University of Saint Joseph School of Pharmacy

    Uploaded by

    Diogo Capella
    52 views22 pages
    Based on the document, the patient has Ehlers-Danlos syndrome (EDS), specifically vascular type. She presented with severe right lower quadrant pain and vomiting. Her treatment focuses on pain management with opioids and NSAIDs, as well as monitoring for complications specific to vascular EDS such as bleeding disorders.

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    danlos sindrome

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    Based on the document, the patient has Ehlers-Danlos syndrome (EDS), specifically vascular type. She presented with severe right lower quadrant pain and vomiting. Her treatment focuses on pain management with opioids and NSAIDs, as well as monitoring for complications specific to vascular EDS such as bleeding disorders.

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    52 views22 pages

    Ehlers-Danlos Syndromes (EDS) : Fiona Li Pharm D Candidate University of Saint Joseph School of Pharmacy

    Uploaded by

    Diogo Capella
    Based on the document, the patient has Ehlers-Danlos syndrome (EDS), specifically vascular type. She presented with severe right lower quadrant pain and vomiting. Her treatment focuses on pain management with opioids and NSAIDs, as well as monitoring for complications specific to vascular EDS such as bleeding disorders.

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    of 22

    Ehlers-Danlos Syndromes(EDS)

    Fiona Li
    Pharm D Candidate
    University of Saint Joseph
    School of Pharmacy
    Objectives

    Describe common manifestations and


    pathophysiology of Ehlers-Danlos Syndrome

    Discuss current treatment & management


    options
    Patient Background

     22 year old female

     Chief Complaint: Severe right lower quadrant


    pain, with vomiting

     Past Medical History:


    Ehlers-Danlos Syndrome
    Recurrent urinary tract infections
    Patient Background(Cont.)

    Social History: Marijuana use, smoking

    Allergy: No known allergies

    No remarkable lab results


    Patient Medication
    Home medication Hospital medication
    Pantoprazole 40mg PO qDay Pantoprazole 40mg IV push BID
    Hydromorphone 2mg q4hr prn Hydromorphone 1-2mg IV once
    Medroxyprogesterone q90days Senna 8.6mg po BID
    Diazepam 5mg QID, prn Lorazepam 1mg IV push q4h
    Docusate 100mg po BID
    Ketorolac 30mg IV once
    Ceftriaxone 1gm IV q24h for 4 days
    Levofloxacin 750mg po q24hr for 8d
    Ondansetron 4mg IV once
    Promethazine 25mg IV once prn
    Tamsulosin 0.4mg po qday
    Acetaminophen 650mg po q4h prn
    Continuous infusions NSS 1000ml
    Ehlers-Danlos Syndrome(EDS)
    Hereditary connective tissue disorder (autosomal
    dominant or recessive traits)
    Prevalence: 1/10000 to 1/25000
    20,000-50,000 EDS patients in the US
    Signs/Symptoms
    Joint hypermobility
    Skin hyperextensibility
    Tissue fragility
    Pain
    Aly Abayazeed, Vascular type Ehlers-Danlos syndrome with fatal spontaneous rupture of a right common iliac artery
    dissection: case report and review of literature. J Radiol Case Rep.2014 Feb;8(2):63-69.
    Signs & Symptoms
    vEDS

    https://www.google.com/search?q=ehlers&biw=801&bih=811&source=lnms&tbm=isch&sa=X&ei=bls1VfrzD8alNoXwgKgP&ved=0CAgQ_AUoAw#tbm=isch&q=e
    Classification Of EDS
    Six subtypes of EDS
    Classical
    Vascular
    Hypermobility
    Kyphoscoliotic
    Arthrochalasia
    Dermatosparaxis

    Aly Abayazeed, Vascular type Ehlers-Danlos syndrome with fatal spontaneous rupture of a right common iliac artery dissection: case
    report and review of literature. J Radiol Case Rep.2014 Feb;8(2):63-69.
    Jakob Burcharth,Jacob Rosenberg, Gastrointestinal surgery and related complications
    in patients with Ehlers-Danlos syndrome:A systematic review, Dig Surg 2012;29:349-357.
    Vascular Type Of EDS

    Vascular type EDS, 5% of all EDS cases


    Deficiency of collagen type III
    Decreased/absent collagen synthesis or
    abnormalities in collagen secretion
    Poor prognosis
    Most severe subtype
    Life threatening vascular ruptures
    Aneurysm formation
    Aly Abayazeed, Vascular type Ehlers-Danlos syndrome with fatal spontaneous rupture of a right common iliac artery dissection: case report
    and review of literature. J Radiol Case Rep.2014 Feb;8(2):63-69.
    EDS Diagnosis
    Classification and diagnosis of EDS is difficult
    Diagnosis
    Clinical symptoms: Beighton Score,
    Brighton/Villefranche criteria
    Laboratory studies
    Qualitative and quantitative testing of
    collagen subtypes
    Genetic mutation analysis
    Positive family history
    Jakob Burcharth,Jacob Rosenberg, Gastrointestinal surgery and related complications in patients with Ehlers-Danlos syndrome:A systematic
    review, Dig Surg 2012;29:349-357.
    https://www.google.com/search?q=ehlers&biw=801&bih=811&source=lnms&tbm=isch&sa=X&ei=bls1VfrzD8alNoXwgKgP&ved=0CAgQ_AUoAw
    https://www.google.com/search?q=brighton+criteria&biw=1600&bih=837&tbm=isch&imgil=uA4I-rvyVPNe9M%253A%253BGnc3YhGvGc4kdM%253Bhttp%25253A%25252F%25252Fjaoa.org%25252Farticle.aspx%25253Farticleid%2525253D2093276&source=iu&pf=m&fir=uA4I -
    http://www.bing.com/images/search?q=willefranche+criteria+&view=detailv2&id=A4E24D324E13213A98B2A02A7199B93CDE73D8E9&ccid=O6tQzHTF&simid=608020249839668791&thid=JN.JPPNn7U8onSyZtPnYskdhw&first=1&selectedindex=3
    EDS Management
    Patient education
    Prevention and early recognition of
    injuries/complications
    Monitoring & Interventions
    Particular manifestations
    Complications with each forms of EDS
    Medical alert device
    No medical treatments

    Susan P Pauker, Joan Stoler. Clinical manifestations and diagnosis of Ehlers-Danlos syndromes. Uptodate, Mar 2015.
    EDS Management-Joint Protection
    Principle of management:
    Optimize function, relieve symptoms, and prevent
    injury
    Joint protection and function
    Preserve and protect joint function
    Prevent recurrent joint dislocations, chronic joint
    pain and early onset of osteoarthritis
    Swimming, walking, and taichi
    Vitamin C 500 mg daily
    Avoid carrying items weighing> 5 lbs
    Susan P Pauker, Joan Stoler. Clinical manifestations and diagnosis of Ehlers-Danlos syndromes. Uptodate, Mar 2015.
    EDS Management-Pain Management
    Chronic musculoskeletal pain, resembles
    fibromyalgia
    Associated with joint damage, with neuropathic
    features
    Acetaminophen
    NSAIDs (avoid in patient with easy bruising)
    Opioid medications (controversial)
    Ice on the joints
    Meditation/relaxation techniques
    Claudia Celletti,Marco Castori. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility
    Syndrome/Ehlers-Danlos Syndrome Hypermobility type. Biomed Res Int. 2013; Jul 14
    Ballantyne JC, MaoJ. Opioid therapy for chronic pain. N Engl J Med. 2003 Nov 13;349(20:1943-53)
    EDS Management-Skin Fragility/Bruising

    Protective bandages/pads over exposed


    areas
    Avoid heavy exercise, contact sports
    Avoid aspirin, NSAIDs, anticoagulants
    Recommend Vitamin C, 1-4 g daily in adults,
    it shows decrease bruising and improve
    wound healing

    Susan P Pauker, Joan Stoler. Clinical manifestations and diagnosis of Ehlers-Danlos


    syndromes. Uptodate, Mar 2015.
    EDS Management-Bleeding Disorders
    Vasopressin analogue, desmopressin (DDAVP)
    Tranexamic acid
    Recombinant FVIIa
    Avoid drugs that interfere with hemostatic
    process(ASA, NSAIDs, anticoagulants)
    Avoid invasive vascular procedures whenever
    possible
    Ultrasound guidance if it is unavoidable to
    central venous catheterization
    Thomas wilesmann, Marco Castori. Recommendations for anesthesia and perioperative management in patients
    with Ehlers-Danlos syndrome. Orphanet J Rare Dis. 2014;9:109
    Monitoring
    Non-invasive monitoring
    Cardiovascular disease
    Ophthalmology
    Spine disease
    Individualized depending on the
    severity of disease and specific
    manifestations , tailored to the type
    of EDS
    http://www.bing.com/images/search?q=Ehlers-
    Danlos%20spinal&qs=n&form=QBIR&pq=ehlers-
    danlos%20spinal&sc=8-20&sp=-1&sk=

    Thomas wilesmann, Marco Castori. Recommendations for anesthesia and perioperative management in patients
    with Ehlers-Danlos syndrome. Orphanet J Rare Dis. 2014;9:109
    Patient Case
    Pain management
    Hydromorphone 1-2 mg IV q4hr
    Ketorolac 30 mg IV once
    Acetaminophen 650 mg po q4hr prn
    Lorazepam 1 mg IV push q4hr
    Ice on joints

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