Now that our family is back from a wonderful and diverting beach vacation, we will share the liver biopsy news that we received while at the beach. It isn't a perfectly clear picture--because apparently pediatric livers are not as easily staged as adult livers--but we've learned more detail about the current state of Thomas's liver damage.
In the journalistic style of most important news first, Thomas's liver is in "at least Stages 2-3 Fibrosis" (out of 4 stages).
How Was this Diagnosed?
The medical team has always suspected Thomas sustained liver damage, but it wasn't much talked about because so many other medical issues were in crisis mode. The liver needs time to heal, so it remained a background issue.
Thomas's Liver Function Tests have remained elevated since December 2020 when he experienced acute coagulopathy (requiring 93 blood transfusions over one month) and his hepatic duct occluded. Liver Function Tests (LFTs) comprise about a half dozen tests, such as ALT and AST, and which all paint a picture of liver health. We want a liver that has very good blood flow, that can excrete bile appropriately, and that cleans toxins from the blood efficiently.
Thomas's LFTs reached levels 1,000 times higher than normal range during his most sick time during the hospital. Having liver enzyme levels that high is a massive "hit" to the liver. Since then, the levels have come way down but are always above normal range. If he takes any hepato-toxic drugs (which many are), his levels go up. If he gets a common virus, his levels go up.
After 17 months of watching this, I persisted in asking if this is Thomas's new baseline and asking why. When one reads layperson's articles online about liver health, one reads about the amazing regeneration properties of this organ. For example, I've read that if a person suffers acute acetaminophen poisoning and 50% of the liver dies completely, the the remainder of the liver will regrow in as quick as 30 days. Wow! Seems like the liver can recover from anything!
Sadly, the liver cannot recover from scar tissue, otherwise known as fibrosis or cirrhosis. Scar tissue cannot become healthy tissue.
In the last couple of weeks, Thomas underwent:
- liver blood labs
- a complete abdominal ultrasound
- manual examination
- a FibroScan
- a liver needle biopsy
We are very pleased with Thomas's new hepatologist (liver doctor). She is board certified in Pediatrics and Gastroenterology, and she founded the GI department of Levine Children's Hospital. She has then specialized in hepatology for the last decade or so, managing the liver transplant team. Plus her bedside manner is incredibly warm and maternal.
On manual examination, Thomas's liver is perceptibly "hard" (doctor's word). The hepatologist taught me how to palpate Thomas's liver and even I can feel it.
She then did a Fibroscan test on Thomas, which sends ultrasound waves through his liver to test resistance. She hoped for a score of <7 and said that a score of 14+ represents fully developed cirrhosis. Thomas scored 17, which earned him a fast ticket to a liver biopsy.
The liver biopsy done just one week ago produced a very detailed report that I won't share here. Suffice to say, there are numerous fascinating cellular changes observed that go into declaring a stage of fibrosis. Thomas is diagnosed with Stage 2-3 fibrosis, with possible areas of Stage 4 where he was not biopsied (as suggested by cellular changes seen). His particular liver scarring is mottled throughout, while a liver biopsy removes a core from only one particular spot, making perfect diagnosis unattainable.
With info from blood labs, manual exam, Fibroscan, and biopsy, now the doctors watch symptoms. Thomas does not show symptoms of the liver failing which occur at Stage 4 (jaundice, spidery veins on abdomen, red palms, etc.).
Prognosis
The hope and prayer is that Thomas's fibrosis is not progressive. Most situations of liver fibrosis are due to progressive diseases, so both the doctors and we parents are flying a little bit blind knowing that Thomas's fibrosis was due to an acute injury (all of December 2020). The only way we know to find out if the fibrosis is progressive is to watch and wait. Thomas will continue to have liver labs done every 2-3 months, see the hepatologist twice yearly, and have another liver biopsy next year.
If-if-if his fibrosis is not progressive, then the hepatologist feels "encouraged" that his liver "should last his entire lifetime." If his fibrosis is somehow progressive and he reaches Stage 4, the only known treatment then is a liver transplant (which can be done from a living donor).
It is still a punch in the gut to be told that we can hope Thomas's liver will last his lifetime because it never occurred to us to consider otherwise. Even though this situation has existed for nearly a year and a half, we parents did not really know it.
How Much More, Oh Lord?
Thomas remains with a mostly blissfully innocent attitude because, despite how much educating we do about his own condition, at six years old he cannot possibly comprehend the implications. So, we parents carry the emotional weight of knowing about:
- his total gastrectomy which makes it very challenging it is for him to eat food, causes malabsorption of nutrition, and puts him at high risk of kidney stones
- his reactive hypoglycemia due to the gastrectomy (causes blood sugar fluctuations that are wildly high and low if untreated)
- his pancreatic exocrine insufficiency because without a stomach to "send messages" to the pancreas, the pancreas does not know to release enough digestive enzymes to the intestines
- his splenectomy, making him vulnerable to certain infectious diseases
- his adrenalectomy (losing one gland) which makes him vulnerable during stress to lack of steroids
- his cholecystectomy, making it difficult to digest fat and then leads to malabsorption of fat-soluble vitamins
- his history of cancer and our having to watch for late effects from chemotherapy (such as some measurable hearing loss he already has, plus numerous things that could reveal themselves later)
- his history of so many GI surgeries, which has resulted in a major amount of scar tissue in his peritoneal cavity, which will mean a lifetime risk of abdominal adhesions, which can quickly lead to intestinal obstruction, a life-threatening emergency
- his acute kidney injury that means doctors anticipate a lifetime of hypertension
- and now his acute liver injury that has resulted in serious fibrosis.
Practical Considerations
Thomas needs to avoid anything hepato-toxic as much as possible. Unfortunately, many medicines damage the liver. I think of the lifetime of aches and pains human beings experience and how the rest of us pop an ibuprofen or acetaminophen . . . he is supposed to avoid taking those. We can use prudential judgement, but mostly Thomas is going to have to grow up learning to tolerate and accept pain and fevers in a way that modern humans with access to a drug store no longer have to do. For example, already if Thomas is having growing pains typical of little kiddos, he wakes up several times overnight and I sit next to his bed rubbing his legs with Biofreeze (topical menthol) and soothing him while he cries himself to sleep. Also, he already would have needed to greatly limit alcohol intake as an adult due to his total gastrectomy, but now with liver involvement, this kid is going to have to grow up to be a teetotaler.
I know people are helpful to want to offer herbs or alternative treatments that are "liver-cleansing," but those would not be appropriate for Thomas. None of them are supported by double-blind, placebo-controlled studies and many products on the unregulated supplements market are outright dangerous.
If I let my mind wander to whether Thomas ever had a relapse of cancer or any particularly serious disease or injury requiring really serious medications, that sets me off having an anxiety attack to think about the effect on his liver. So, I need to focus on the worries of today and none tomorrow.
Help on the Horizon: St. Erasmus and Boston Children's
We have borrowed a first class relic of St. Erasmus (aka St. Elmo), the patron saint of liver disease. Apparently he was disemboweled and this relic is a bit of his intestines! Now we have a new patron saint on our team of St. Benedict (kidneys) and St. Jude (impossible causes) and both St. Thomases (the Apostle and Aquinas). I also pray to St. Becket (Weinert) and St. Vincent (Baran), two little boys who died during Thomas's time of medical crisis.
We are headed to Boston Children's Hospital in mid-May and, with this new diagnosis, were pleased to discover that Boston has one of the top worldwide teams treating pediatric fibrosis. Apparently they are the best hospital in the United States to treat children with liver damage caused by TPN (which Thomas did have to receive for nearly six months). There are MDs and PhDs there whose research focus is how to regenerate pediatric livers from fibrosis. Believe me, we'll be begging to get their involvement!
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