Tulips and Rembrandts: Home and Holding Steady

To begin with, Elizabeth has remained “stable” since leaving the hospital. Her ascites has remained the same and her diarrhea continues unabated. As soon as we push in 5 ounces of formula into her G-tube, approximately the same amount comes out the other end! We are very thankful for the absorption capacity of modern disposable diapers and in-home laundry machines. Because of her frequent diapers, she’s also developed a whopper of a diaper rash- the kind that brings her to cry and say “awe-done” every time we have to change her. She is still very weak, preferring to sit on the couch or in our arms rather than crawl around the floor with her toys. And she frequently asks to be put to bed by sweetly pleading “nigh-nigh.” Though we’re pleased that she’s sleeping from 7:30pm to 9:30am, we’re anxious to have our energetic Elizabeth back.

Tomorrow, my dear mother is heading back to California. We are naturally anxious about whether we’ll be able to manage on our own. This week brings many doctors appointments as we try to put the pieces together and chart a course for Elizabeth’s recovery…which brings me to the new revelations of the last several weeks.

After a couple of weeks of unabashed emotionality about Elizabeth’s hospitalizations, (thanks to my admittedly unabashedly emotional wife J) I thought we’d start the week off with as more medically-oriented update. (be warned...it's long!)

First, we learned that Elizabeth's heart is having new problems. Specifically, over the last 6 months, the left side of her heart has stretched much larger than it should be. For the cardiologists among you, she has a dilated left ventricle, but still with normal wall thickness and ejection fraction. The cause for this new finding is uncertain. It could simply be the result of her new anemia and worsening fluid retention. If so, there’s a chance it could reverse if we manage to reverse these other two problems. The more dire possibility is that her heart muscle has a genetic abnormality in its structure. We'd discard this explanation except that these genetic defects also cause generalized muscle weakness, something Elizabeth has had since birth. The doctors are starting to wonder if there might be a connection. At this point, there is no treatment needed for the heart problem, just close observation and monitoring.

Second, Elizabeth’s portal vein thrombosis is NOT improving like we’d thought. Based on recent imaging studies, it looked like Elizabeth's body had begun forming for venous shortcuts to get blood around the blocked area. Everyone was hopeful that these “collaterals” were becoming big enough and numerous enough to keep her abdominal blood pressure low. Recent events and tests overwhelmingly show that these collaterals are inadequate for the job.

When the blood pressure in the abdomen gets too high, several things happen. The blood tries to find a new way back to the heart by creating varices (dilated veins) in the esophagus, stomach and spleen. To get an estimate for how high her abdominal blood pressure is, the doctors put a camera through her mouth, down her esophagus and into her stomach (an endoscopy) to look at the varices. They found that her varices had worsened since her endoscopy one year ago, increasing from a Grade 3 to Grade 4 (5 is the worst). They also found evidence that they are under considerable pressure, with several showing “cherry red spots.” When these spots are seen, there is a high risk of rupturing in the next 6 months. Without meaning to be dramatic, these varices are ticking time-bombs. If one were to rupture, Elizabeth would begin vomiting blood. Without immediate, highly specialized medical care, she could potentially die from such a hemorrhage.

Another effect of high abdominal blood pressures is the accumulation of ascites (fluid inside the abdominal cavity surrounding the intestines). Elizabeth’s last week in the hospital occurred because she developed a large amount of this fluid, further indicating how high her abdominal blood pressure is.

A last important effect of high abdominal blood pressure is a secondary build-up of pressure in the abdominal lymphatic system. The lymphatic system is responsible for taking nutrients absorbed from the intestine back to the blood stream. If it is under pressure, the nutrients can’t be absorbed and are left in the intestine where they can contribute to diarrhea. Elizabeth’s persistent diarrhea suggested that this might be occurring. In confirmation, dilated lymphatic vessels were seen during her endoscopy. A biopsy was taken which will likely confirm this problem.

Needless to say, we are very concerned. The doctors are as well and are urging us to pursue “an aggressive conservative” approach. A “conservative conservative” approach would involve starting a blood pressure lowering medication in the hopes that it would lower her abdominal blood pressure enough to decrease her risk of variceal rupture. An “aggressive aggressive” approach would involve taking her the liver surgeon and having shunt procedure or liver transplant performed despite the risks incurred by her young age and small size. The “aggressive conservative” approach involves two interventions. First, blood pressure lowering medications would be started to try and lower her abdominal blood pressures. Second, endoscopy would be performed every 3-4 months to monitor the varices. If a varix appeared ready to rupture, the gastroenterologist would inject it with alcohol to make it scar down instead. This type of a procedure sounds risky, and it is. Because Elizabeth’s esophagus is just slightly bigger than the camera tube they use, there’s a chance her esophagus could be ruptured. Additionally, with each alcohol injection, there’s a decent chance of causing the varix to rupture instead of scar down. And lastly, closing off the collateral flow that occurs through these varices can have unintended consequences. Primarily, it risks making her ascites worse or causing an “encephalopathy.” An “encephalopathy” is a condition in which the function of the brain is impaired. In this case, toxins the liver usually removes would build-up in the blood stream and begin affecting the brain’s function. Because Elizabeth’s brain is still developing, the damage could be permanent. If scarring down the varices in a sequential fashion increased her ascites or caused encephalopathy, we’d quickly be forced to try the shunting or transplant option.

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Whew! That’s a lot of information to process. If you stuck with us through the whole thing, you deserve an honorary doctorate…at least in Elizabethology. If you skipped down to the double-spaced new paragraph hoping the details had ended, that’s OK too. Here’s the wrap-up:

The events of this last month have reminded us how tenuous Elizabeth’s health is. We’ll be in touch after our round of doctors appointments this week. We really appreciate your prayers. It’s very clear to us that without the constant prayer covering she’s been getting, she may not have made it this far.

Goodnight,
Matt (and the girls)

Tea Parties with Grandma are an essential part of our daily routine now and will be very much missed when she leaves tomorrow! Though we have a little in-house debate about whether the tea pouring out sounds like: "shhhh" or "glub glub glub"...So far Grandma's voting for "glub glub" (in solidarity with her beloved son) and Elizabeth is taking my side and saying "shhh". Time will tell...