Linda Lenz
Lenz Associates, LLC
S77 W16199 Mariner Ct.
Muskego, WI 53150

Ehlers-Danlos National Foundation

Development of a Life-Saving Medical Presentation - Medical


Concept, research, creative direction, script and package copywriting.  The Ehlers-Danlos presentation was distributed to 7,000 physicians world-wide and has been credited with saving at least four lives as a result of the treatment information.


Ehlers-Danlos Syndrome EDS – Emergency Physicians Reference CD
Here is a condensed list of life-saving surgical and post-operative suggestions for patients with Ehlers-Danlos Syndrome – Vascular, Type IV.  Although considered rare, clinical diagnosis of EDS Vascular is often difficult.  In a trauma situation do not assume that your EDS patient has been typed correctly.  EDS Vascular is a life-threatening connective tissue disorder that affects all tissue, arteries and internal organs making them extremely fragile.
Roughly one-half of all cases of Vascular EDS are new mutations with no family history.  The half are familial, inherited from an affected parent.  Vascular EDS is autosominal dominant.  Continue through the CD or booklet after this list, for more detailed information.

  1. CT scans or MRIs – immediately
  2. No arteiographics, enemas, or endoscopies
  3. Non-invasive techniques only – no stress/tension on skin, organs or vessels – extreme care during physical exam or passing nasogastic tubes
  4. Anesthesiologist please note: when intubating – fragile mucus membranes throughout – a lower peak volume pressure may be necessary
  5. Vascular surgeon’s assistance anticipated in every surgery – meticulous, gentle handling of internal organs and vessels
  6. Plastic surgeon’s presence may be necessary
  7. Aneurysm – a small soft-tipped catheter with micro coil (memory) has been successful in some cases
  8. Abdominal aneurysm – Double woven velour/Teflon grafts
  9. Colonic rupture – consider permanent colostomy/ileostomy to reduce the risk of recurrent perforation
  10. Padded clamps with red rubber catheter covers (Fogarty Hydrogrips)
  11. Use Lange’s lines for incisions – whenever possible (Teflon sutures)
  12. Incision pressure – use 1/3 to ½ less pressure, with meticulous, gentle dissections – avoid tension/stress on suture lines
  13. Ligation of vessels – use surgical hemoclips and umbilical tapes where anastomosis is required, buttressed sutures by Teflon or felt pledgets
  14. If necessary, the sacrifice of a non-essential organ or limb to save a life must be considered

Condensed Emergency Post Operative Suggestions
Monitor for:

  1. peritonitis, pneumoperitoneum, and/or other infections
  2. ruptures, cysts and abscesses
  3. wound dehiscence, ileus, gastrointestinal bleeding
  4. arteriovenous and/or intestinal fistula
  5. aneurysms, embolus, hematoma
  6. liver: bleeding, changes in pressure and/or function

Use or be aware of the following:

    • wound packs and abdominal binders (reduce risk of incisional hernia)
    • IV placement: may be problematic due to fragile veins (If necessary, permanent access port catheter has been used)
    • Less IV pressure: slower rate when administering fluids
    • Immediate evaluation – of any change in vitals or additional complaints
    • The most non-invasive post-operative care available is recommended
    • Be vigilant – as status can change abruptly with this patient