Sep 08 2009

The Road Not Taken (and why I wish it had stayed that way)

Two roads diverged in a wood, and I —

I took the one less traveled by,

And that has made all the difference

– Robert Frost

Ahhh, the allure of independence, free will, and personal freedom. The free spirit in all of us knows that the road less traveled is the one to take. It’s where all the surprises and excitement exist. It’s where spontaneity is born. It’s where all discoveries occur. It’s the path that seduces our “Inner Lewis & Clark” to push ’round the next bend in the river. But if you’re a patient with a rare disease navigating a health system, the road less traveled can be a downright lonely and inhospitable place.

Let me explain.

crossroadsI just had a visit with the transplant team at MCV on August 25th and it looks like I’m at a crossroads in the progression of my liver disease. Here’s a quick review of the facts. I’m on the transplant list, but with a relatively low MELD score. As I’ve mentioned before, the MELD score quantifies just how sick a liver patient is, and consequently establishes the priority in which available livers will be provided for transplant recipients. Complicating matters is the fact that while the MELD score effectively measures severity of illness in the vast majority of liver patients, it is a notoriously unreliable measure for patients with my specific condition, Primary Sclerosing Cholangitis (PSC).  So, although I’m on the transplant list, I’m so far down the list that it’s unrealistic to expect a match from a deceased donor any time soon.  While I wait for a liver to become available, I face a risk of cholangiocarcinoma (bile duct cancer), a known risk for patients with PSC and very-nearly a terminal diagnosis (NOTE: There is still a great deal of controversy related to liver transplants in patients known to have cholangiocarcinoma. While cholangiocarcinoma can’t be considered a purely terminal diagnosis, the outcomes are very grim. The most recent issue of the medical journal Transplantation sums up the issue nicely in the abstract found here). So, my crossroad has two choices: (1) wait, and hope that I don’t get cancer, or (2) try to move the timetable up dramatically by identifying a living donor.

In the meantime, I’ve been seeking the advice and guidance of the transplant team at MCV, and I’ve been getting two widely divergent sets of opinions from them about the course of action I should take. Here’s a quick, highly oversimplified summary of their views and of mine:

  • The Hepatologist: Believes I should wait patiently. He is not a fan of living donor transplants because he believes the complication rate for the recipient is substantially higher than in a deceased donor approach. He also feels that the risks associated with the surgery actually outweigh the risk of getting bile duct cancer, given my current MELD score.
  • The Transplant Surgeon: Believes I should move ahead aggressively with a living donor option. Prefers the living donor approach over the deceased donor option. Says that while living donor complication rates used to be higher than with a deceased donor, the gap has nearly closed, and in the specific case of his patients, the complication rates are the same. Is very concerned about the risk of cholangiocarcinoma.
  • Me: I believe I am a better-than-average risk for the surgery. I’m otherwise healthy and have a high level of understanding about what is required post-transplant in order to stay healthy. I further believe that I am a worse-than-average cancer risk. I have nothing to quantify this assumption, nor do the physicians have any hard evidence to counter my assumption. Admittedly, I’m placing significant weight on the risk of cancer as a result of spending the last 22 months of my mom’s life watching her die from cholangiocarcinoma, so my motives may be more emotionally driven than quantitatively driven. Bottom line, I’d like to be transplanted now and a living donor could make that possible.

Although the hepatologist and the surgeon don’t see eye-to-eye on my case, I remain 100% convinced that they are each spending an extraordinary amount of time studying my specific situation, and that they are each providing me with the best advice based on their individual medical judgment. The fact that they disagree has nothing to do with being right or wrong, and has everything in the world to do with making decisions based on incomplete data. I’m unusually fortunate that my team of providers not only tolerates my routine input, challenges and opinions, they have actually encouraged it. They have expressed appreciation that I am so willing to be vigilant about my condition, to study the latest data, and to be actively engaged in my care. To be candid, not all health care providers appreciate that level of involvement. I’m thankful that mine do.

So, although we don’t all agree, not surprisingly we’ve all settled on a compromise. I’m now on 90-day cycles for visits to MCV. When I’m there, I’ll go through the usual battery of blood work and physical examination, but I will also meet with the surgeon and/or the hepatologist. They have agreed to look beyond the MELD score and have indicated that they will be asking for more of my input related to symptoms I’m experiencing. Catherine has pointed out that I have a tendency to gloss over how poorly I’m feeling, and that now isn’t the time to be stoic. I’m a very lousy patient in that regard, since I find few things as unpleasant as talking about how sick I am. I thought I could hold off that conversation for another 40 years or so once I was institutionalized with my fellow octogenarians somewhere. No such luck. So, every 90 days we’ll go through the same debate again: transplant now or transplant later.

I should point out that PSC patients get accustomed to this level of ambiguity very quickly, but it’s no less frustrating just because it’s typical. Most PSC patients spend months, even years undiagnosed or misdiagnosed. After diagnosis, the course of action doesn’t get much clearer because there’s so little known about the disease, its cause, and treatments. Let me offer a quick synopsis of a common conversation someone newly diagnosed with PSC might have with their physician. All of the following is based on actual experiences…

  • PSC Patient: Wow, so I’ve got this disease. Any idea what caused it?
  • Doc: We have a few theories. It might be an autoimmune disease, but we really don’t know.
  • PSC Patient: I did some reading and see that a transplant may be required. Am I going to need a transplant?
  • Doc: You might, but we really don’t know. The disease manifests itself very differently in different people.
  • PSC Patient: So, what’s my prognosis?
  • Doc: I don’t know.
  • PSC Patient: Is there any chance my kids will get this? Should they be tested?
  • Doc: No, no. PSC is not known to be hereditary.
  • PSC Patient: Wait a second. My mom had PSC. Doesn’t that seem strange if it’s not hereditary?
  • Doc: I didn’t say it wasn’t hereditary. I said it wasn’t known to be hereditary. Big difference.
  • PSC Patient: Wow, do you use a scalpel to split hairs that thin?
  • Doc: What…huh?
  • PSC Patient: Sorry, that was supposed to be a joke. I understand there’s a risk of getting cancer. I guess we should monitor that pretty closely, huh?
  • Doc: I wish we could. Unfortunately there’s no real reliable test for cholangiocarcinoma.
  • PSC Patient: Okay, well what do we do now? Is there any sort of treatment?
  • Doc: Well, “treatment” may be too strong a word, but we do have some pills that you can take (Ursodiol). There’s no clear evidence that they work. In fact, we’re pretty sure they have no impact whatsoever on the course of the disease, but at least they are outrageously expensive.
  • PSC Patient: I don’t have any symptoms now. When can I expect that to change?
  • Doc: Sometime between tomorrow and 30-40 years from now. Did I mention that the disease progresses at very different rates in different people?
  • PSC Patient: Yes, you mentioned that. Is there anything more that I should know?
  • Doc: I don’t think so.

Maybe the little one-act play above portrays an unreasonably cynical point of view, and there is definitely a small-but-growing body of knowledge that is being slowly accumulated as more research is conducted, but in general, this is what life is like when you have a rare disease. I’ve met with numerous physicians who have dedicated their careers to studying liver diseases, and a small handful of physicians whose field of study has been focused exclusively on PSC. In both instances, the body of research and quantitative data that they can rely upon is pitifully small. Worse yet, in many cases the new research absolutely contradicts the earlier research (e.g., Ursodiol. Remember, that’s the incredibly expensive drug I have been taking in megadoses diligently since September 2000. I had been cautioned that it might not actually affect the underlying disease and that it could possibly just make my lab scores look better. Well, the most current research indicates that high doses of Ursodiol actually INCREASE the rate of bile duct cancer vs. patients who take nothing at all).  So my exchanges with physicians gradually become less about data and research and more about hunches and intuition.

Okay, a quick reality check. It would be fair to accuse me of exaggerating in my description above to make a point, but I think most PSC patients who read this will recognize the frustrations associated with the ambiguities of our disease. Before I am accused of spreading misinformation, though, here are some hard facts. There is no known cause for PSC. There is no known prevention for PSC. There is no known treatment for PSC with the exception of a transplant. The few treatment options that exist are focused almost solely on managing symptoms. What works for one PSC patient may have no effect on another.

So, this is the road less traveled, albeit probably not the one that Robert Frost had in mind. The path is being carved daily and there are as many dead ends as there are discoveries. In the words of my six-year-old daughter quoting Rabbit in Winnie the Pooh’s Heffalump Movie, “This expedition is fraught with danger.” I couldn’t have said it better myself.


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  1. Pete Weisberg

    Unbelievable. And I thought constipation was bad. Bill, I really have a strong feeling though this is going to work out. Go with your gut ..on second thought, maybe your heart.

  2. Stacey

    Unfortunately for us both, my liver is unusable. I would be willing to donate, and I do mean that with every fiber of my being. I have been a donor since 16, and a nurse, as you know. My kid-16- is a donor. I guess it’s just something I have always beleived in. However, my autoimmune disease rules me out. I checked. And no, the beer and trips to Harry’s bar didn’t do it! All I can do is get the word out, and that I shall continue to do. I will keep reading…..

  3. Alison

    You’re on my mind every day. Hang in there.

  4. Nannette

    Your story hit home. My husband is not on a transplant list YET but I have a feeling he soon will be. It’s a very difficult journey. I will continue reading your story and will think of you often.

  5. markchatterley


    I’ve just been diagnosed with PSC and have stumbled across your blog. Thank you for taking the time to write all this down. Some of it has scared me, but to be honest, I would rather be prepared.

    I have also started a blog which I hope will help people like yours has helped me.

    Thank you. I look forward to your future posts.


  6. Randal


    Catherine sent me to your blog. Your use of humor, faith, honesty and even a good clinical verbatim are refreshing. I think about you a lot and am pulling for a transplant…soon.

  7. TLBarwick

    do you ever get a burning pain on your side (upper abdomen)? I spoke with my liver doctor about, she doesn’t know why I have it, but ordered an MRI. I am holding for my G.I. doctor to call me back in the meantime.

  1. A Bright New Year Begins with a Perfect Match « Bill Varner's Transplant Journal

    […] Next Curt and Alison traveled to Richmond to begin the testing for compatibility. This testing is not insignificant. It includes interviews with psychiatrists and a social worker. It also includes more traditional clinical exams like blood typing, tissue antigen typing, a chest x-ray, an electrocardiogram, a pulmonary function test, a cardiac stress test, an MRI, and a hepatic angiogram. The hepatic angiogram is saved for the very last test because it is an invasive procedure and there’s no reason to put a prospective donor through that test until the’ve passed the preceding tests with flying colors. Alison’s hepatic angiogram was put on hold until the transplant team could agree on whether or not the timing was right for me to move ahead with surgery, a process that added at least three months to the journey and only recently concluded with the green light (for more details on this, see my earlier posting). […]

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