EDS & pregnancy

21 y/o G1P0 at gest week 26 c/o constant and intense lower abdominal pain, a feeling "pulling" pressure bilaterally and fatigue. As we discussed what round ligament pains are and why she may feel this, hers reportedly are "more severe." It was later discovered on history review that she had Ehlers Danlos Syndrome (EDS).

One source defines EDS as "an inherited genetic disorder that affects the body's connective tissues and prevents the body from functioning in the way that it should. Symptoms of EDS include:joints that move beyond the normal range of motion, which can lead to dislocations and chronic pain, extremely delicate skin that is susceptible to injury, overly stretchy skin, which makes the skin vulnerable to damage and exposes a person's internal organs to harm. EDS abnormalities such as these are caused by faulty collagen, which is the substance that gives strength and elasticity to connective tissues in the skin, joints and blood vessel walls."*There are 6 types or grades of EDS, one of the worst being Vascular EDS for obvious reasons.

With this and such patients in pregnancy, there is an increased risk for premature delivery, excessive bleeding (during or post c-section and vaginal delivery w/ tearing), miscarriage and other maternal complications. In utero, the vascular EDS may lead to placental rupture placing the fetus in demise. Excessive skin stretching (doughy), malleable bone changes and others can place the patient/client at risk for falls which can lead to a "bleeding events" (connective tissue disorder, as well).

Genetic counseling with a geneticist / expert genetic counselor (like Dr. Seibert......) during preconception, pregnancy and in the PP period is vital as there is no cure for this. The risk versus benefit of pregnancy and the impact on both mom and baby (medical/surgical complications, risk of genetic transmission to baby, lifestyle adjustments etc) should be discussed. Management of symptoms includes pain medications, warm compresses and physical therapy, referral for interdisciplinary/ancillary services (SW, MH, OT, Heme).

I found this very interesting. A "live" one!

* How stuff works. com