Rusty had his November monthly follow-up visit with Dr. Macy on Wednesday; Dr. Macy had decided last month to do more extensive tests on his liver this visit, as the test results have continued to decline over the past several months. Rusty has also continued to lose weight, even with the increase in his meals, which was a concern to Dr. Macy. Rusty had to fast before we took him in for the first round of tests; after his blood was drawn we took Rusty home and fed him double rations to get the liver, stomach, etc. functioning. We took him back two hours after being fed for another set of blood tests.
During the past few weeks Rusty’s intake of water has increased dramatically, including having to go piddle during the night (like his Dad); we took a urine sample with us. When they ran the tests on the urine, Dr. Macy and his tech came running out to the waiting room and accused us of “spiking” the sample, as the results were off the charts. We obtained another urine sample, using their sterile container, when we took Rusty home. When we returned, they ran the tests again and the results were still off the chart.
All of the tests were being sent to an outside lab for analysis and they hoped to have the results today. We had an extensive conversation with Dr. Macy this evening, as the lab results identified two new issues that we now need to address.
Dr. Macy said that Rusty “flunked” his liver tests. Rusty has a “mild portosystemic liver shunt”, which are abnormal veins that enable blood from the intestines to bypass the liver. The effect is ammonia and other toxins are not metabolized or removed from the blood. The majority of portosystemic shunts are congenital. Rusty’s shunts are not serious enough to require surgery; we can offset the effects of the liver by feeding him small high protein meals several times a day. No large meals!
Rusty has also developed “Fanconi Syndrome”, which is an impairment in the function of the kidneys that causes certain compounds which should be absorbed in the bloodstream by the kidneys to be excreted in the urine instead. Fanconi’s syndrome is generally an inherited disease that affects the proximal renal tubule and causes abnormalities in sodium, glucose, calcium, phosphate and amino acid retention. The disease can also be mimicked by certain toxins (see liver above) and drugs (Chemo) that affect the proximal renal tubule and interrupt normal functioning. Rusty is losing glucose, amino acids, uric acid and phosphate into his urine. This explains the increased water consumption and frequent urination. It also explains his weight loss as he is dumping calories into his urine. Fanconi Syndrome can be treated using potassium citrate along with other dietary supplements.
Rusty has to go back in tomorrow for a specialized test to determine the dosage of potassium citrate that he will need. Dr. Macy is not overly concerned at this time as he thinks that he can control the liver and kidney issues with diet and medications. Rusty has had his share of challenges during his young life.
The good news from this visit is there were no signs of the cancer returning; it has now been 22 months since his diagnosis and amputation. Rusty has had the fewest number of episodes of vomiting and diarrhea the past few weeks since his battle with cancer began. He has been off all meds and supplements and his appetite was normal; he was also happy, full of energy and looking his best since the amputation. You would not suspect that something was amiss from looking at him. Unfortunately, the next day after his tests, he again had another siege of vomiting and diarrhea; his weight has dropped to 30 lbs and we are feeding him four + meals trying to stop any more loss.
Hey Rusty, I hope that your tummy is feeling better. I’m so sorry you’ve got so much to deal with, I wish I could make it all go away. Please feel better soon OK?