Josh

Josh suffered from Vascular Ehlers Danlos Syndrome, the most severe form of EDS. The connective tissue in the arteries (including the thoracic and abdominal aorta), intestines, uterus, and other hollow organs may be unusually weak, leading to organ or blood vessel rupture. Such ruptures are most likely between ages 20 and 40, although they can occur any time, and may be life-threatening.

Here is Josh's story, as told by his wife Deb.

On the morning of January 30th, 1998, I found myself suddenly widowed at the age of 29 and the single parent of a beautiful seven week old son.  Around 11:45 that morning surgeons were struggling to explain how their efforts had failed to save the life of my 27 year old husband, Joshua Doss.  He had just died in surgery from a ruptured abdominal aortic aneurysm. 

He had recently been diagnosed with a genetic connective tissue disorder called Vascular Ehlers-Danlos Syndrome (vEDS) which causes the walls of arteries to be significantly weaker; leaving him at extremely high risk for arterial dissection, aneurysm, or rupture.   Our newborn son was just seven weeks old when Josh died.  Two weeks after his death I received confirmation from our geneticist that my son, Karsten, had the same life-threatening disease as his father. 

The summer before he died, in the eleventh week of my pregnancy, Josh survived a splenic artery dissection that nearly took his life and left him hospitalized for ten weeks.  Through the diligence and commitment of our local internist, we were sent to a geneticist at Emory University in Atlanta who diagnosed Josh with the vascular type of Ehlers-Danlos Syndrome.  Because of his disease, Josh’s body didn’t make enough Type III collagen which is like the glue that holds the cells together. We didn’t realize it at the time, but his body, his vascular system, was literally coming unglued.   

In December of 1997 Josh was admitted to the ICU at Emory after doctors discovered bilateral iliac aneurysms which were causing excruciating abdominal pain. That same night while nurses were settling him in his room, my water broke.  It was five weeks too soon. 

I was taken to a nearby hospital where friends and family surrounded me; Josh and I would not be together for the birth of our son.  The night our son was born, Josh’s mother stayed on the phone with him throughout the delivery, keeping him up to date on how I and the baby were doing. 

Five days after he was born, I took our son to meet his daddy for the first time.  Still in I.C.U., still covered with I.V.’s and monitors, Josh held his son in his arms for the first time and together we cried tears of joy and gratitude.  The fact that Josh was alive to see Karsten at all was a gift; we were both keenly aware of how serious things were. 

We had six short, precious weeks together as a family.  We weren’t always under the same roof because Josh was in and out of the hospital many times. But whenever possible I was there with him, baby stroller and all. 

We were staying at his parents’ house on the night before Josh died.  Around 10:15 I found him crumpled on the floor, crying out in pain from an aneurysm we never knew he had.  His abdominal aorta had ruptured and within 14 hours he slowly, painfully bled to death.  Surgeons tried to repair the artery, but his disease had left his tissues so weak, so fragile, that they literally fell apart when touched by the surgeons’ hands.  After completing the autopsy, doctors learned that Josh had been living with multiple aneurysms, fistulas, and dissections for years. 

Receiving my son’s diagnosis two weeks later was the single darkest day of my life and if I’m honest I must say that I still have not completely come to terms with it. Having that knowledge, though, regardless of how painful it may be, is exactly what allows my son to have a much better chance at surviving than his father ever did. 

Because of the lessons learned during his Josh’s illness, I am able to fight for our son’s life with my eyes wide open, fully aware of what risks are involved, and do all I can to try to ensure that things will turn out differently for him.  In the fight against vascular complications, knowledge really is power; and education and preparation are the two most important weapons I have. 

Groups such as the TAD Coalition and its member organizations are working hard to get accurate, life-saving information into the hands of patients and physicians on aortic complications and the warning signs that can’t be ignored.  It’s because of resources like these and the people who work so tirelessly to create them that I have real hope that my son’s life will turn out differently.