Ambition is a wonderful thing. When I started this blog, I had it all mapped out in my mind and since that time, life has gotten in the way. Soooo, here's a new post!

During the time since N.'s initial, life-saving surgery, there has been a long row to hoe. As I mentioned previously, she has done well in recent years but getting there was never a walk through the tulips by any stretch of the imagination.

The first time I met N. was well before all of the medical problems. She was 14 at the time I was first introduced to her family and she was healthy, energetic and vivacious. Into all the things she wanted to do at the time. Horseback riding, roller-skating (sorry if I just aged you, honey!), etc. As they say, she, "had a lot of juice!" I was a frequent guest in her family's home for a few months and then was away for a couple of years.

It was during that time that she endured the worst of the medical procedures, the accompanying pain and risk to her life. After being away for a while, I went back to visit her family and renew the friendship I had previously had. During that time and in spite of the medical issues, N. had grown to 18 years old, had pretty much overcome most of her most pressing health issues, was working at a medical services company and doing quite well.

After a while, we started dating. It was immediately obvious that N. wasn't able to move around with any real endurance any more. She mentioned that after the surgery and when when she was more or less recovered, she would find herself exhausted just walking from the couch to the bathroom and back. (As a side note, at the onset of the illness, such a lack of energy was also a problem.) By the time we started dating, she had made progress but getting from the car to the front door of a grocery store was a major drain on her energy. It made it difficult for her to move around much but she kept working on it. Over time, she has been able to come to a nearly normal activity level, however, it's very easy for her to overdo it and find herself exhausted way too soon!

We were married three days after her nineteenth birthday. We had gone to her doctor at the time to discuss the issues of childbearing for N. and the doctors were adamant: No kids allowed. The strain on the liver from the Budd-Chiari had already taken its toll on her liver and to impose the biological task of bearing a child would have been too much. It wasn't a pretty picture that he painted for us.

About three months into our marriage, N. got sick again. She wasn't feeling well at all. It should be noted that during the time of our marriage, for some reason, her periods were very unusual. I remember that she would have a period sometimes for an overly long period of time with excessively heavy flow. Or she would have a couple within short order of each other. One would end and another begin about two weeks later. One time, there was an extra two weeks between two periods which caused us no small amount of panic given that we weren't supposed to let her get pregnant! After a few of these disorderly periods, she started to have abdominal pain again and went to the doctor.

The diagnosis was that she had developed what they called, "chocolate cysts," or, "blood cysts," on her ovaries. She was right back into surgery again to have them removed. One of her ovaries was completely removed and they left a part of the other in order to preserve her ability to keep producing her female hormones. This was supposed to be a safeguard against cervical cancer, etc.

She recovered from this surgery over approximately six weeks but her healing process was somewhat extended. At the time, N. was still being treated with coumadin in order to keep her blood from clotting up in her body, to try to circumvent stroke, etc. As a result, of course, healing from her surgery was somewhat extended as her incision took an inordinately long time to heal and required extensive care to keep it orderly and eliminate the dangers of it.

She went back to work, things resumed, more or less normally! Stairs were always an issue as were hills. Walking uphill wore her completely out, even a gentle slope. Elevators and escalators were her friends! She didn't have any major issues with her health for about a year and then, the previous year's chocolate cyst problem recurred with her remaining part of an ovary complete with the strange menstrual cycles.

Once again, surgery was required to remove the remaining part of an ovary. Again, it took about six weeks for her to heal completely and with the same care requirements and concerns. She healed up well, however, and resumed her life. Pregnancy, of course, was no longer an issue and we proceeded to stop worrying about her getting pregnant. I'll be the first to admit that it was nice to not have to use condoms any more! (N.'s doctors had never wanted her to go on birth control pills because they were concerned about their potential effect on her liver. At the time, we were considering the possibilities of engaging the services of a surrogate mother so we didn't want me to get a vasectomy either.) Of course, N. will be the second to admit it was nice to get rid of the condoms!

As a brief note, sexual activity has been a source of frustration for N. We suspect that as a result of her illness, her body didn't get completely into the cycle of puberty that most women have and as a result, she doesn't lubricate as normal women do. It took us a while to find a lubricant that was suitable for her to use (Eros) and didn't just add to her discomfort. She also lacks the elasticity that most women have and the commencement of sex has ever been painful for her for the most part. For a while, doctors prescribed various hormonal prescriptions but they generally ended up making her ill and they soon ceased to be an option. In spite of her frustrations in this regard, she is still able to engage in sex without too much difficulty as long as we take the time to do it right, as the song goes!

Coumadin was a normally recurring theme for N. Doctors were ever concerned about making sure that she didn't have any propensity to clot off and cause other problems such as strokes, blocked circulation in her legs, etc. Over time, however, Mother Nature has adjusted her blood chemistry so that her need for coumadin has been eliminated.

Speaking of Mother Nature, she has done an amazing job of taking care of N. Here are some examples along with some other effects of the disease:

- Blood flow. Since the amesio-atrial shut has been clotted off, there is no way for her body to move blood directly from the liver to the hear as it should do. Nature, however, has taken it upon itself to reroute her circulation so that her blood actually moves backward through her veins to accommodate this need. As a matter of fact, her veins actually flow in both directions simultaneously, especially near the liver. More than once over the years, ultrasound techs have come running out of an examination room in order to locate his buddy techs so they can come and check it out! When the new color Doppler ultrasound was first invented, N. was invited to go in so they could test it out on her! As far as we know, her body is unique in this regard. In order to make the blood flow as it does, Nature had to greatly increase the size of her veins to accommodate this requirement and has also increased the number of veins in her body, especially in her torso. Some of them are visible at the surface so you can imagine what it must look like inside. More on this later.

- Blood composition. As mentioned above, her body no longer requires a thinning agent like coumadin. Also, just last year, she went in for her usual blood tests and was told that her red blood cells are larger than they are supposed to be. I assume that they carry oxygen better but I don't really know what it means to have enlarged red blood cells. The doctor didn't seem to think it was a problem, however, so we are leaping to the conclusion that it's a good thing! Pro-times are pretty much normal and over the years, blood test show that her liver is actually functioning just fine.

- Liver. Blood tests show it's functioning fine. The liver is the only organ in the body which is capable of repairing itself. Hers seems to be pretty much repaired now with a slight cirrhosis or inflammation still present. Once in a while, she will experience a couple of days of pain and then it will dissipate and she'll be fine again. These occurrences have become rather rare over the last couple of years and she's able to recover much more quickly. We've speculated that these episodes are related to stress but we don't know for sure. It could be that once in a while, Nature has to take a few moments to cleanse or purge or whatever.

- Gall bladder. We're not sure if this is a result of the Budd-Chiari syndrome or not, but her Gall-bladder has a large "soft" stone in it. As long as N. manages her diet, it doesn't cause her any grief, but occasionally, it will pain her. The first few times, it put her in the E.R. just for some heavy-duty pain drugs. Lately, it hasn't been that severe, but it is something she has to pay attention to. Mostly, if she avoids too many sequential days of overly greasy foods, she's okay but I believe that's pretty much true for most people anyway! Doctors fear that they cannot do any surgery because the network of Nature's collateral veins in the area make it highly risky. All they don't need is to be working on her and inadvertently cut through a significant "new" vein and she'll bleed to death on the table. Less risky to leave the gall bladder alone in her case.

- Collateral circulation, though a good thing for N., has it's problems too. The most significant has been several episodes of bleeding from her esophagus. She has veins visibly protruding at the surface of her body but by the same token, they protrude in her esophagus as well. The problem there, however, is that they are subject to the normal chemical bath that happens with everyone but with her, that chemistry can cause the vein to start to leak a bit. The blood then goes to her stomach and causes nausea and dizziness and everything else associated with blood loss. Fortunately, the nausea is an early warning and we can get her to the E.R. right away.

There have been at least five episodes of this problem over the years and she has been well-served by the doctors. They have done two things to solve this problem. The most immediate is that they do a blood transfusion. Next, they do a chemical cauterization of the points of bleeding in her esophagus. I don't recall the term for it. Sclerotherapy or something like that. Lately, they have "banded" the veins in her esophagus to prevent them from bleeding in the first place and that has seemed to be a "permanent" solution. The banding closes off the veins which are protruding from the inside of her esophagus, eliminating the circulation therein and the veins are reabsorbed by the body. Of course, if they aren't there any more, they can't just start bleeding again whenever they feel like it!

There is more to tell, of course. More later!

--Wag--

A killer disease

I'm not sure why I'm putting this blog up. I'm sure I'll know by the time I'm done. I think there is room for hope for people and my wife, N., is a prime example of that hope.

When she was 16 years old, she was smitten by a killer disease called Budd-Chiari Syndrome. To this day, nearly 20 years later, I'm not 100% sure of the details of it, what causes it or even what it actually is. I do know it nearly killed my dear wife before we even knew we were going to date each other, much less get married a few years later.

She endured more than one major medical procedure including a massively invasive surgery wherein the doctors inserted a mylar mesio-atrial shunt (forgive my spelling if necessary) which saved her life. At the time, the doctors told her if it clotted off, she'd die within a year. It clotted off right away. That was, as I mentioned above, nearly 20 years ago.

There are better ways to solve this problem now. Ways to give a survivor much better odds of survival and a "normal" life. To their credit, they did a wonderful job of it 20 years ago. Did they get lucky? Probably. Did they have skill? Most assuredly. They deserve a great deal of credit.

I should mention their names: Dr. Ronald Busuttil of the UCLA Medical Center and Dr. Telfer Reynolds of the USC Medical Center. They saved her life. In addition, I should mention another doctor who has done amazing things with her over the last 13 years. Dr. Kenneth Horwitz. Dr. Horwitz has been willing and able and above-the-call-of-duty anxious to do everything in his power to improve her quality of life as much as possible.

These three men have been miracle workers in every sense of the term.

And yet, even they have expressed a certain degree of amazement at the steady health and progress of N. through the years. For example, she called Dr. Reynolds a few years ago, mostly just to touch base. The first words out of his mouth were, "You're still alive?" He was amazed. "I want to see you!" So, we went and saw him. I went just to meet him and during our meeting, he said, "I have a class who I'd like you to meet." Next thing you know, we're in a classroom full of med students and N. is on an exam table being poked and prodded by the students.

A curious observation worth mentioning which was evident not only in this setting but also throughout the time I've been with N. is that women doctors are brutal. When the men doctors treat her, they are gentle and considerate and compassionate. The women doctors, on the other hand, poke too hard, prod too hard, cause additional pain and have very poor bedside manners. Generally speaking, of course. There are exceptions to the rule, no doubt (although we haven't encountered any yet!)

N. and I have been married nearly 17 years. They've been wonderful and miserable. Frightening and lovely. The roller-coaster ride has been a thrill and a terror. If I have the impetus to finish this blog the way I'd like it to be, you'll see much of that through my eyes. But here's the most important thing I wish to present through all of this: Quality of life is what you make of it. It's been a lesson learned over 17 years by the example of my wife to me. It cannot be overstated.

Through these years, N. has had a positive mental attitude that puts most athletes and high-acheivers to shame. She loves life. She wishes for it to be what it is. She doesn't complain about her illness (though she seeks help without hesitation when she needs it). She knows more about her condition than most doctors. (I've seen her make references to anatomical terms and had E.R. doctors have to go look them up!)

I believe her doctors have performed amazing things with her life and given her the opportunity for fulfilment. But I also believe she's done as much to cure herself and prolong her life as anyone else who has been a part of it.

This blog, hopefully, will give that story. If you were to look at N. today, you probably would not think there was anything wrong with her. Talk to her, relate to her, do things with her, she'd seem as normal as anyone else. But the more you were to take time to be with her and befriend her, the more you'd know that she has a solidity of character rarely matched by anyone other than those of extreme accomplishment. Other people who have had major medical problems can relate somewhat, assuming they have also survived againts the odds. N. is an example of the kind of person who has had a greater hand in the quality of her own life.

--Wag--