Well, that's a very good question.
Scott has a genetic disease /disorder called Ehlers Danlos Vascular type (VEDS) previously refered to as type IV EDS.
Here are a couple good links with lots of super medical mumbo jumbo
http://www.edstoday.org/info/types/vascular.htm#Links"
http://ghr.nlm.nih.gov/condition=ehlersdanlossyndrome
In a nutshell. Scott's DNA, specifically COL3A1 is mutated (Scott's is a G when it's supposed to be an A). His body doesn't produce the correct amount of collegen. Collegen is what holds your body together. Most EDS patients have problems with their joints, and thin skin and then there are VEDS patients like Scott.
Here's some good information on COL3A1 http://ghr.nlm.nih.gov/gene=col3a1
Scott's problems are in his vascular system. The lack of collogen makes Scott's arteries weak. This weakness causes aneurysms, and AVM's (Arterio-Venous Malformations). His arteries are very soft, we've heard doctors refer to stitching Scott up is like trying to stitch Jello. Take a moment and picture taking a needle and thread and pulling in through Jello without causing it to split. So not easy. On one of Scott's recent surgeries Dr. Jacobs described his femerol artery as piece of wet tissue paper.
VEDS is the only fatal type of EDS. There is no known cure and the only thing you can do is manage the "fallout" from an event. Which is why the best vascular surgeon at RUSH (in my opinion - and lets face it my opinion is the only one that counts) is on my speed dial.
At any given moment Scott has numerous aneurysms in his body. Most of the time we don't know they are there until they rear their ugly little heads in the form of a rupture or bleed.
We currently know of several aneurysms that Scott has existing in different parts of his body, the most serious of these is in the hepatic artery. This is the only blood supply for the liver. Should this friendly little guy decide to cause trouble it would mean serious business and in all reality probably death.
So if you see Scott please be sure to not look in the general direction of the hepatic artery and for God's sake if you do make sure you talk slow and gently to it.
Please do not provoke the hepatic artery. Thank you for your consideration.
History -
Scott has had 6 aneurysms rupture making life very complicated.
What we now know was the first aneurysm was in Scott's right leg in Feb 2000. The doctors incorrectly diagnosed the rupture as compartment syndrome and performed a faciotomy.
Number 2 - The second aneurysm was in his left leg. This was when he was first clinically diagnosed with VEDS December 24, 2002, a short three months after our first date (and yes it was Christmas eve - my hubby has always been the best gift giver). Scott had his first angiogram the day after Christmas 2002 and his genetic study was completed with a VEDS diagnosis in March of 2003. The treatment of this as well as the diagnosis was done at Advocated Luthern General.
Number 3 - Fast forward to 2004, Scott had an aneurysm in his ulnar artery in his left arm. The vascular surgeons at Luthern General were terrified by Scott and kicked us to the curb telling us to go seek help at RUSH.
Best thing that could have ever happened. We found our miracle worker; Dr. Chad Jacobs.
No intervention was necessary with that aneurysm it resolved itself and occluded(closed off) the ulnar artery.
Number four - (bored yet?) Number four was a biggie. Scott's splenic artery ruptured. This was the 90day stay at RUSH from May 13 - Aug 13 2008. I blogged about stuff at that time. If you've got several hours you can read it here www.livejournal.com user name katesdaddy.
This stay was a huge wake up call. Scott spent much of this time very close to death. He was intubated with a ventilator breathing for him for long periods of time. He had days where he would have procedure after procedure. We chased and chased, fix one problem, develop another. Dr. Jacobs was nothing short of phenomenal. But in the end it was Scott's body that did the real work. Dr. Jacobs just gave him the time he needed to recover.
Numbers 5 and 6 - during the '08 stay at RUSH Scott developed radial aneuyrsms in both arms. Dr. Jacobs performed surgery on both and tied off the radial artery in both of Scott's arms. If you're keeping track in Scott's left arm he has lost 2 of the 3 sources of blood supply.
And then lucky number 7 - that would be where this blog started.
Angiography has played a huge role in keeping Scott alive. Scott most certainly wouldn't be here with out it. But, from an emotional stand point angiograms are the hardest things for Scott and I to handle. Which leads me to ...
Family History -
We have trouble dealing with angiograms because Scott's mother (Paula) passed away from complications from an angio. Her VEDS was undiagnosed at the time, the doctors fixed what appeared to be a typical bleed resultant of a fall she took. Upon completion of the angio Paula became seriously ill and died on the operating room table while the doctors tried to fix her bleeding.
While I was never blessed enough to meet her I can't help but think of her death everytime Scott has an angio procedure. Even though Scott has had more of them than I can count, every one of them gives both Scott and I serious pause. They will never be easy, but having a fantastic team of IR's (interventional radiologists) that all know Scott inside (literally) and out makes us feel a little more at ease. Those guys truly know how fragile Scott is.
Scott's sister Tamara also had VEDS. Her's was diagnosed after years of bowel problems. She fought the disease her entire life. She passed at the age of 29 having spent the last 53 weeks of her life in the hospital.
Looking back now, the doctors did so many things incorrectly in treating her. If they knew then what we know now... She still would have passed but she may have been here a little longer and she would have had a better quality of life.
Part of what we love about Scott being a patient at RUSH is all the medical advancements that they make every day (hello Bio-Glue). So many doctors, nurses, techs etc. are able to learn from Scott. Maybe they can learn enough to cure this disease.
Knowing that VEDS is genetic we always get the question what about Kate? Kate is fine, perfectly normal. We achieved pregnancy by using every medical advancement in fertility options available,
Preimplantation testing, also called preimplantation genetic diagnosis (PGD), is a specialized technique that can reduce the risk of having a child with a particular genetic or chromosomal disorder. It is used to detect genetic changes in embryos that were created using assisted reproductive techniques such as in-vitro fertilization. In-vitro fertilization involves removing egg cells from a woman’s ovaries and fertilizing them with sperm cells outside the body. To perform preimplantation testing, a small number of cells are taken from these embryos and tested for certain genetic changes. Only embryos without these changes are implanted in the uterus to initiate a pregnancy.).
Kate may have been created in a petrie dish but she's perfect. Absolutely, completely perfect.
We know that Scott will leave us too soon. I know I will have to explain to Kate that her daddy is in heaven. I know I will have to find a way to move forward with my life without Scott in it.
When we first got Scott's diagnosis Scott told me I could leave, said he wouldn't blame me, wouldn't hate me, after all we'd only been together 3 months and we already knew he had MS (multiple sclerosis) (when it rains it pours around here). The answer I gave him then is the same as it is today. It doesn't matter if we're given 5 minutes, 5 days, or 50 years; my life would never be complete without having him in it.
We live in every moment, soaking it all up and feeling grateful. Facing each day with as much grace, love and fun as we can. We choose to not see everyday as the last day but to embrace each day as the gift that it truly is.
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