Ehler Danlos syndrome, more commonly referred to as Ehlers Danlos syndrome, is a connective tissue disorder that varies in severity. In mild cases, joint dislocation is common but few other symptoms are presented. Severe cases of Ehler Danlos syndrome can be characterized with heart problems and death. During pregnancy, connective tissue is forced to hold additional weight from pregnancy weight gain and weight of the fetus. For patients with Ehler Danlos syndrome this can result in pain and increased reports of dislocated joints.
Types of Ehler Danlos Syndrome and Pregnancy
There are at least 15 different types of Ehler Danlos syndrome. Each type is characterized with a separate set of symptoms.
Types 1 and 2 are considered Classical Ehler Danlos syndrome. These types tend to affect collagen and can present with skin sagging with type 1 Ehler Danlos syndrome being the more severe of the two.
Type 3 Ehler Danlos syndrome is known as hypermobility. Hypermobility presents with increased flexibility and can result in dislocation of joints, specifically hip and knee joints.
Type 4 is a vascular condition affecting both blood vessels and organs. It is estimated that 1 in 100,000 people (at most) are affected by type 4 Ehler Danlos. Physical characteristics associated with type 4 include larger than normal eyes, ears with no lobes and thin facial features.
Type 5 is a rare form of Ehler Danlos syndrome characterized with increased bruising of the skin and stretchy skin. Several patients have been seen with type 5 Ehler Danlos syndrome with a “floppy” mitral valve. This condition caused death in one patient.
Type 6 is known as Kyphoscoliosis. This rare form of Ehler Danlos syndrome has only been reported in about 60 cases. Type 6 Ehler Danlos syndrome is a progressive disease that results in a curving of the spine and muscle weakness.
Type 7 Ehler Danlos syndrome is broken down into A, B and C. Types A and B affect collagen with symptoms including looser than normal joints and hip dislocations. Type C is a skin disease that results in fragile skin that sags extremely. All subtypes of type 7 Ehler Danlos syndrome are extremely rare.
The remaining types of Ehler Danlos include Type 8, Type 10, Beasley-Cohen, Progeroid, Tenascin-X deficiency and unspecified.
How Will My Ehler Danlos Syndrome Affect Pregnancy?
In most cases, patients with Ehler Danlos syndrome tolerate pregnancy well. Women often report increased swelling or edema, hip dislocations and pain in joints during pregnancy. One study completed by The Department of Obstetrics and Gynecology at the Westeinde Hospital in the Netherlands reported several conditions and complications associated with Ehler Danlos syndrome and pregnancy. These included an increase risk of pelvic instability, bowel rupture, pre-term delivery and perineal wounds. In some cases pregnant women gave birth to infants with Ehler Danlos syndrome causing floppy baby syndrome.
It is important for women with Ehler Danlos syndrome to make the condition known to the attending obstetrician from the beginning of the pregnancy. If the obstetrician is not familiar with the condition it may be necessary to seek alternative or complimentary care from a joint or Ehler Danlos specialist.
