Misdiagnosed?

The jury's still out on that question, but we do know that Jordan should not have been released from the hospital on Thursday, the 13th. He was absolutely miserable all night, with uncontrolled pain and nausea, and very little sleep for him or Brianna. By Friday morning, we called Doyle and Phil for a blessing, after which Doyle transported us to the ER. Blood-flecked vomit was the last straw, giving us the final push out the door. Once at the hospital, there were more blood draws, abdominal and chest x-rays, liters of saline, 8 milligrams of morphine, and then it was back up to 4th South for admission. Grrr, we're thinking, just try dislodging us while Jordan continues to suffer with the same symptoms.

No chance of that, instead, we are introduced to a new medical crisis, hypercalcemia. Hy per cal whatia? Hypercalcemia: An abnormally high concentration of blood calcium. In myeloma, and other malignancies, the breakdown of bone,which is rich in calcium, is the main cause of high blood and urine calcium. The high calcium can contribute to weakness, loss of appetite, nausea, confusion, lethargy, and other symptoms. Its normal range is between 8.7-10.4. Jordan's was at 14.3, a medical crisis. and what do we do in times of crisis? Pray. And then? Go online to access the collective wisdom of our support teams. Friday night, I sent out a plea for input from the Rhabdo=-Kids listserve.

An excerpt from the letter of a rhabdo-friend, James Atkinson of North Carolina:

Bonnie, They probably will be able to get the hypercalcemia under control fairly rapidly, which is great since nothing makes you feel sick and wrong faster than too much serum calcium. Almost all of the symptoms you mention on the blog page could be attributed to hypercalcemia: nausea, loss of peristalsis, chest pain...all of it. Calcium is vital to every neural system we have, so when it gets out of whack like that, the body just does not work right. Because of the many unpredictable things that hypercalcemia can do to you, hospitalization is necessary. I will go out on a limb and say that I think that the Neupogen issue is a red herring. It can cause bone pain, absolutely, but not the other constellation of symptoms that you describe. My money is on the calcium this time.

From several articles provided by Michelle, another rhabdo-friend, I ten to concur with James on attributing the past week's woes to hypercalcemia. Along with the pain and nausea, Jordan developed a 102,8 degree fever, and delirium. To our great relief, he began to improve with a 1 miligram per hour morphine drip (yes I know I said no more morphine, but that was then, this is now), with IV Ativan, IV hydration, and two broad spectrum antibiotics, Vancomycin and Cefatazadime. Due to his hallucinations, we now know what lies buried deep within Jordan's subconscious mind. Anyone got a guess? It makes perfect sense, knowing Jordan. Fortunately, he is now lucid, the ileus has cleared, he's been able to keep food and liquids down today, even walking about a little for exercise. For the record, Zometa is the medicine being used to treat the hypercalcemia.

Meanwhile, James has raised another question which must be answered once and for all. It will be most uncomfortable to rehash the rhabdomyosarcoma diagnosis with our doctor, a man to whom we are deeply indebted, but we feel compelled to do so for Jordan's sake. The pathologists gave a differential diagnosis of either rhabdo or an acute erythroid leukemia. Here is James's mind-spinning correspondence:

A differential diagnosis of ARMS or acute erythroid leukemia suggests to me not a lack of guts but rather (i) lack of definitive data and (ii) an unwillingness to get the additional data, perhaps because of stupid bottom-line economics. Treatment for the two diseases is markedly different. I am surprised that they had "guts enough" to proceed with one protocol over another based on what amounts to a guess. From a cost standpoint, nothing is more expensive than a botched diagnosis. The bean counters never believe that. Diagnosis of AEL (and myeloid dysplasias in general) is determined by specific standards --- percentages of certain kinds of cells in the sample, among a constellation of other things. To muddy the waters, it appears that right now there are older diagnostic standards (FAB, or"French-British-American") and newer diagnostic standards promulgated by theWorld Health Organization, and that there is some degree professional disagreement between the two schools. The same patient might end up with different diagnoses depending on which criteria the reviewing pathologistuses. If the pathologists at UNM and Stanford were using the FAB criteria, their report should include additional classification data: classification M6a orclassification M6b. These refer to specific presentations within the marrow sample they had in front of them. If they were using the WHO standards, they also should have included specific blast measurements/counts, etc., that they used in order to generate the differential. It's such an odd differential that they had to base it on something ... more than just "small round blue cells" under the microscope. The cytogenetics of ARMS vs. AEL are different, so, again, if you can get someone to run the appropriate genetic assay, you should come up with a definitive lean one direction or the other. There may be separate assays for ARMS and AEL. I will say this: as rare as ARMS is in adults, acute erythroid leukemiaprobably is only marginally less so, and especially in people who have not previously been exposed to alkylating chemotherapy or benzenes. If you'replaying the numbers, that is to say, a de novo case of AEL overall probably is less likely than a case of ARMS, which is saying quite a lot given the utter rarity of ARMS in adults.

And if that's not enough, here's one more little tangle to unravel from another rhabdo-friend, Chris:

Bonnie, just to add to your already complex situation, if your son had ifosomide it is possible that he may have fanconi syndrome, it also causes a lot of those symptoms. My daughter has acquired fanconi syndrome from ifos. It damages the kidney tubules and affects the way the body converts vit D,. this inturn disrupts all the calcium, alkaline phosphatase, potassium, phosphate, and uric acid and glucose. It's worth asking the question as it is easily managed and saves lots of heartache.

Chris mum to Sarah erms 21/2 years off treatment.