Our Worst Nightmares Don’t End When We Open Our Eyes

The ten weeks since our daughter, Stephanie, was diagnosed with primary sclerosing cholangitis (PSC) have been a blur of hospitals, tests, and surgical procedures. A steady stream of GI specialists, radiologists and surgeons have poked, prodded and asked the same questions over and over. But despite their collective wisdom, they seemed to have no idea why Stephanie appeared to have skipped over the beginning stages of this normally slowly progressing liver disease and landed somewhere closer to the end.

Well, that’s not exactly true . . . they had one idea.

In most PSC cases, there was an initial flare-up, usually blocked bile ducts, that gave rise to the diagnosis. After the patient was treated they usually went off to wrap their mind around this dreadful illness. They were told that it would rear its ugly head from time to time, slowly damaging their liver which would shorten their life considerably unless they received a liver transplant down the road. If there was any good news it was that they had time some time. On average people stricken with this disease had eight to ten years before the circumstances became dire.

I’m sure we got that same speech from a well-intended GI doc, or maybe from several. And I’m sure there were days early on when I believed what they said was true. Of course, we had time. So, in the beginning I went to bed each night expecting the next day would be better. But that didn’t happen for us.

I’ll never understand it. Steph barely had any symptoms when she was first diagnosed at the end of January, and I think I even convinced myself that maybe she would be on the longer end of the average. I remember thinking, Why not? She was younger than most people diagnosed with PSC and other than this awful disease, she had always been the healthiest person in our family.

Last week I was organizing a file folder full of information I had been collecting about PSC and came across some notes I made one night when we were in the hospital – probably one of those sleepless nights when my head understood more than my heart was ready to feel. I had researched the best diets to fight liver disease and the detail in my notes startled me. My usually sharp mind was a bit foggy as I had completely forgotten that I had done all this research and even started a Pinterest board of recipes that could help us combat our new enemy. Like I said, it’s been a blur.

Those notes were dated February 6 . . . I was obviously still an optimist then.

Not that I didn’t still wake up every day hopeful that our fortunes would take a positive turn, but sometime in the last few months I had lost that naïveté that accompanied pure optimism. You know, the thing that allowed you to believe with all your heart that there was no possible outcome except a good one. I’m not sure its disappearance was related to any single event; but I had it back then and now I don’t.

Maybe it happened with the first biopsy. During one of several procedures to implant biliary drains to relieve her blockages, this one on the left side of her liver, they encountered a mass. They took a tissue sample through a technique called biliary brushing. It’s just what it sounds like, they used a tiny brush to scrape some tissue off the mass and had it analyzed. The pathology report was negative for malignancy. But Stephanie’s doctor downplayed it, which looking back on it seemed odd.

To me, “not malignant” could be nothing but great news . . . but then, I was still new to all of this.

A few days later, we were referred to a hepatologist at Vanderbilt and our new doctor’s number one concern was the suspicious mass that we were trying so hard to believe was a non-issue. We had come to understand that the “not malignant” finding only meant that the specific tissues collected were not cancer, but it didn’t mean that there wasn’t cancer present. He used the term “very concerning” about six times in a five-minute conversation to describe what he saw in her scans and by the time we left his office we had scheduled a second biopsy.

In subsequent conversations, her new doctor explained that one complication associated with PSC was bile duct cancer – cholangiocarcinoma –  and it drastically changed the course of the disease. Bile duct cancer is very rare and typically aggressive, and it could explain why her progression seemed to be so accelerated. Since no treatment could begin without this diagnosis, it was critical that they answered the cancer question first. Her blocked bile ducts and all the normal PSC type concerns were on the back burner until the bigger issue was clarified.

The second biopsy was performed on February 23. In addition to using the brushing technique for a second time, they also collected a larger sample through a fine needle aspiration which seemed to give them confidence that a diagnosis could be made. But it didn’t; the biopsy was inconclusive. The pathology report said something like “while the tissue appears suspicious, malignancy cannot be confirmed.”

Not the “yes” answer they expected . . . but also, not the “no” answer I prayed for.

On March 23, we were back at Vanderbilt for a third biopsy. We had spent four weeks in limbo since our last visit; this diagnosis stood squarely between us and our path forward. I wish we could have used those four weeks for some adventure that brought enjoyment to Stephanie. But life these days was far more restricted; her weakened condition and the nagging glitches with the drains have drastically hindered her mobility. She’s lost more than twenty pounds and much of her stamina along with it. Her emotions are fragile, and she lived in fear of the next medical procedure. Maybe it all seemed like a blur because ours had become a life I’d rather forget.

Gone were all my lofty aspirations for this year. Now my joy came from waking up at home rather than in a hospital room or knowing my daughter made it through the night without needing oxycodone. I dreamt of things like Steph being able to shower or having a day without bile bags to be emptied or soggy bandages to be changed. I prayed she would once again move freely,  to sing and dance and not  be worried about triggering pain or dislodging a drain.

Instead, our life stood still . . . waiting for the diagnosis that would tell us if we’d ever feel whole again.

Certain words were guaranteed to devastate, no context could soften their blow. As soon as I answered the phone and caught the hesitation in his voice, I knew I was about to hear one of those words. And then finally, he said it. “… it’s cancer…” I don’t remember the words before or the words after. I don’t think it even registered that tears were streaming down my face until I tasted salt on my lips. As I tuned in and out, words like rare and aggressive were followed by talk of oncologists and no cure and limited treatment options. He said he’d give me a little time, that we’d talk later. He had obviously done this before.

Seven hours passed. Darkness had replaced light, in the sky and in my heart. I climbed in bed beside Stephanie and watched her sleep while it all sank in. After weeks of repressing my worst fears, I allowed all the feelings of pain and loss to run free. My agony wasn’t just for me; in those moments I was suffering everyone’s pain – incredible family and friends that had been so loving and loyal to her over the years. I don’t think for a minute this is only hard for us, it’s tragic for everyone in her life. And how could I not worry for the younger ones, cousins and nephews, with such tender hearts that were soon to lose a precious slice of innocence. But mostly I was sad for Stephanie; it terrified me to think of the pain and anguish this new enemy would inflict.

Sleep was not my priority, I knew from experience I had a short window to grieve and then brace myself for the journey ahead. I thought back to the hours and days after Stephanie was born when I struggled to understand why this had happened to me, why my daughter had been born with Down syndrome. I questioned if I could handle having a child with disabilities; if I could deal with the long, hard road ahead. Those first few nights were filled with the same darkness and sorrow that occupied me now. Eventually I came to understand that what my baby needed was simple. She just needed me to be her mom –  to love her and care for her and advocate for her. I couldn’t think too far ahead, I had to take each day as it came.

And so now, I knew what I had to do … I just needed to keep being her mom.  A good mother is the glue. If she’s strong, everyone is strong. If she’s calm, everyone is calm. But that doesn’t come easy when your heart is aching, when your soul is crushed. As first light peeked over the horizon, I knew I wasn’t there yet. I was still too empty to be a good mother. It was too soon to talk about what’s to come. I wasn’t ready to hear that the only options were choosing between bad and worse. I wasn’t ready to make decisions no parent should have to make.

But I would get there . . . what choice did I have?

In these last few months, I think maybe I’ve found a little insight into that very first question I had when Steph was born. The “why me” question. I could see now that Stephanie wasn’t like most kids who just needed a mom to give her a good start in life… she needed a mom that could go the distance. I think it was always intended for me to take her all the way through this life.

I intuitvely knew my heartache wouldn’t stop me from ultimately fulfilling the promise I made to her more than thirty years ago – somehow, I would summon the strength to be the mother she needed. Now more than ever I had to help her find a little happiness in each day and protect her from as much of the pain as I could. Of course, in the wee hours of the morning after receiving such devastating news, it all seemed impossible. How I could bring her cheer when my heart was shattered, or even give her a smile when I couldn’t stop crying?

Four days have passed since that night. I wish I could say I’m ready to take this on. I’m not. But my spirit is resilient, and my faith is still strong. Stephanie and I are blessed with amazing support from family and friends, so I know in the days ahead we will find a new rhythm to our life; one that guides us down the path no mother ever wants to take her daughter.

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