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.

Too Much, and Not Enough

Too much pain. Too much nausea. Too much time spent in the clinic trying to pinpoint the problem. Too much gazing up at the ceiling of a hospital room.

Not enough food. Not enough fluids. Not enough relief. Not enough time for a reprieve before the next treatment begins.

Jordan spent the whole of Monday afternoon in the ER checking out chest pain. He spent Tuesday, from about 11 AM to 5 PM, in the clinic, looking for some help with pain management and nausea. Back again on Wednesday, he sought help for both again, with nausea and vomiting at an intolerable intensity. from 10:30 AM to 5 PM, they worked with him, sending him for an abdominal X-ray, administering morphine, enema, kytril and saline solution with potassium. Finally, he rushed home to pick up a few things, then proceeded on for admission to the hospital, where he seems to be improving.

Why all the discomfort of this week? The verdict (although it's felt more like a sentence), is that he was given "too many" Neupogen injections to stimulate white blood cell production, which led to excessive pain. Then, he was given too much morphine to control the pain, which led to a cessation of peristalsis, and/or iileus bowel obstruction; hence the nonstop nausea and emesis (barfing). Just for you, a couple of definitions follow.

Peristalsis: The wavelike movement of intestinal muscles that propels food along the digestive tract.

Ileus: Obstruction of the intestine due to it being paralyzed. The paralysis does not need to be complete to cause ileus, but the intestine must be so inactive that it prohibits the passage of food and leads to blockage of the intestine. Ileus commonly follows some types of surgery. It can result also from certain drugs, injuries, and illnesses. On listening to the abdomen with a stethoscope, no bowel sounds are heard (because the bowel is inactive). Also called paralytic ileus.

For his pain control, it's Fentanyl to the rescue. This is an opioid analgesic with a potency approximately eighty times that of morphine. Fenntanyl transdermal patches work by releasing fentanyl into body fats, which then slowly release the drug into the blood stream over 72 hours. Oh, and fewer Neupogen injections! The doctor said we'll cut back from nine to six next time.

For the ileus bowel obstruction, no more mor"fiend"! A diet limited only to clear fluids seems to be helping. Over the last few hours, he's been able to keep down some 7-Up and ginger ale.

All his counts are on the way down (HGB from 9.0 to 7.2 since yesterday), so he'll have 2 units of packed red blood cells today, and then, if all goes well, he'll be released later this afternoon. With the WBC's dropping from a high of 2.1 yesterday, down to 1.6 this morning, we hesitate to celebrate. After the first treatment back in Jan., the rising WBC had us on cloud nine when it reached 2.2, and the next morning when it dropped to .6, we were in despair, with good reason.. It meant the rhabdo was on the move again, in a big way.

What You Can Do:
Please continue praying for Jordan to be strong and well. And will you send him a birthday greeting? He turns 26 on Sunday, the 16th. Thanks a trillion.

The Trend is... There Is No Trend

From one day to the next, we don't know what to expect. Jordan has a few good days in a row, and then suddenly, he's back in the throes of nausea and pain. Last Thursday's 12-hour nosebleed and transfusions were followed by more nosebleeding late Friday evening, which precipitated a night spent in the hospital with yet another transfusion of platelets. Saturday and Sunday went well though, lulling us into believing all would be well until treatment time came around on the 18th, just after Jordan's birthday on the 16th, and ST. Patty's Day on the 17th. Good, thought we, all we'll have to think about for the next few days is planning a great birthday bash for our green-eyed ladie.

But no such luck. Jordan's second bout with bone pain was at hand. Wrist, shoulder, spine, and sternum, to be exact. One of the drugs in his chemo cocktail (Doxorubicin) carries the risk of cardiotoxicity. Oxycodone and Hydrocodone provided welcome relief when the discomfort began on Sunday, but when he began to experience increasing chest pain and shortness of breath on Monday, they were no longer effective. Our friend, Jane, usually a model driver, sped him to the ER on the recommendation of the medical oncology team. After an EKG, cardio panel, X-ray, CT scan, and maybe a few other things, it was determined that the pain was likely due to bone pain caused by the Neupogen injections, and not a cardiac event after all. Six milligrams of morphine sent the pain packing, and another four mg a little later, slammed the door in its face!

Oh, but that sneaky nemesis, nausea, slipped through the keyhole to continue the assault. Jordan and Brianna have had very few chances to go out and have a little fun together for the last three months. He's had his eye on a Vietnamese restaurant located just a short walk from the hospital, so they decided to linger for awhile, in order to enjoy an early dinner there, and wait for Dan to finish up at work and bring them home. They ordered two dishes to share, a pork and vegetable stir fry with rice, and a wonton soup, both of which met with their approval, and reminded them of their carefree days in China. Unfortunately, what made the meal most memorable wasn't the food or the ambience, it was Jordan suddenly grabbing the most accessible container, which turned out to be Brianna's bowl of soup, and throwing up in it. Over twenty-four hours later now, the nausea continues unabated. The bone pain also returned, more morphine was administered today, potassium too, and at this point, no one feels like venturing a guess as to what the night, not to mention the next few days, may hold.

Happily, he has successfully avoided all mouth sores this time. The bone pain, while more than annoying, has been less debilitating than it was after the first treatment, when it was nothing short of excruciating, and had us all running scareder than scared. We're hoping and praying for an uninterrupted string of good days, because the next treatment is just around the corner, sure to present its own raft of challenges to overcome.

Transfusions

It started with a trickel, just a slow, steady tricle of blood from his nose, at about nine o'clock yesterday morning. With platelets in full nosedive mode, a nosebleed is nothing to . uh, sneeze at. Since they were down to 13k on Wednesday, after three hours of drip-drip-dripping, we decided to call the clinic for guidance, even though Jordan had an appointment already scheduled for two pm. They said to come on down, so we called Hank, our chauffeur for the day, and made haste for the clinic downtown. Labs showed the platelets had fallen to 6k, the lowest to date! Hemoglobin was at 9.0 (low, although it has been lower), so they decided to fix him up with a couple of units each of platelets and packed red blood cells. Jordan and Brianna were comfortably ensconced in a private room, compliments of St. Luke's Regional Medical Center, until nearly 11 pm, when the transfusion was finished and Dan transportted them home. That pesky proboscisbleed didn't stop until about nine pm! Hmmm, even though they made it home before the midnight hour, the transfusions appear to have had a transformative effect upon Jordan.

Before

After

March 6:

WBC 0.2

RBC 3.05

HGB 9.0

PLT 6.0

Neuts 0.00

Count Them One by One

It's definitely good times when Jordan's home, buuuut, when he's home, it's back and forth every single day, rain or shine, feeling bad or feeling fine, to check those temperamental blood counts. You never know who to put your money on, the WBC's, hemoglobin, the platelets, or what. It's a real horserace. One count pulls ahead, almost reaching an acceptable level, then suddenly, it loses its place and falls to the back of the pack. Another count slowly starts to make its move, steadily gaining ground, and then, oh! down he goes. Now come on, you reds, whites, neuts and platelets, you're getting Neupogen injections, plenty of transfusions, and chemo to knock out your opponents, so let's get going.

Our Hall of Thanks

Jordan is home! Everytime he's released from the hospital and he and Brianna walk through the door, it feels like a holiday. He tolerated this cycle of VCD better than the first cycle while inpatient. At that time, violent vomiting caused small hemorrhages of his eyes, and he reacted poorly to several antinausea meds (antiemetics). No Compozine, Zofran, or Aloxi for him, ever. The latter decreased his heart rate from a norm of about 70 beats per minute for an adult male, to the mid 30's for a couple of days, and the former produced a strange sensation that he was unable to communicate until it subsided. Calling it discomfort would be an understatement; and calling it pain would be inaccurate. Dr. Z. explained it as an allergic reaction that causes the synapses to fire randomly, sending mixed signals throughout the body. Fortunately, he responds well to Ativan and Marinol, which he takes as needed, and 30 minutes or so before meals. Presently, fatigue is his main side effect, but we are on the lookout for those dreaded mouth sores, which proved so distressing from about 10 days after the first round of chemo and persisted for 2 interminable weeks. He is diligent about salt and baking soda mouth rinses, in hopes of staving them off completely this time. He began the first of 7 Neupogen injections yesterday to stimulate white blood cel production, and this should safeguard him from them as well. So first in the hall of thanks....is Jordan.

In no particular order, other recipients of our sincerest gratitude include the following:

For posting blog comments:
Julie, Amy, the Chipmans, Dave & Kathy, Lucinda, Barb, Jamie, Amy & Matthew, and Rebekah

For knitting the beautiful afghan:
Cindy, Cross's mom

For the cheery cards and posters:
Jana and the Primary children, Lucinda, and Sister Wheeler & Sister Tupa

For chauffeuring us to labs and appointments:
Karen, Lorrie, Jolene, Cindy, Erin, Verna, Gladys, Diane, Kirt, Joyce, andprobably many more to come

For prayers:
So very many faithful friends and family members

For flowers and crafts:
Don & Janet, Joan, Jean

For food:
Karen, Cindy, Kirt, Lorrie, Jana, Lois, and Joan

For big surprises:
Nauvoo's Christmas delivery, a huge stocking from an unknown Santa, for Greg and Neil's wild, wintry ride from Denver to Boise, the Lims' gift, and an unnamed gift from the Victory View ward

Family visits from far away, visits from friends close at hand, all the phone calls, e-mails, posts from CLLC Friends and Nauvoodles, mean so very much to us. Many thanks to the doctors, nurses, social workers, technicians and to the charity of St. Luke's, for a multiplicity of highly-specialized, invaluable contributions

To our Father in heaven, we are most deeply indebted.. And we will never, ever forget.

Catching Up Is Hard to Do

From Bonnie on 12/13/07

We so hoped for a conclusive diagnosis today, but it still isn't in. According to the doc, a sample of bone marrow has now been sent on to the premier bone marrow pathologist in the nation, Kathryn Foucar at the University of New Mexico. Unfortunately, he still suspects Jordan has either ALL or AML, not the B-12 autoimmune deficiency. Jordan was discharged from the hospital on Monday, but will return if the diagnosis of either leukemia is confirmed. Until it is, I guess he will be treated with antibiotics and blood transfusions. He had his first transfusion last Friday, and will likely have another this Friday. Counts improved for about 3 days, and are heading down again. Platelets went from 38k on Monday, to 28k today. HGB went from a low of 6.6 last week pre-transfusion, to 10.something on Monday. But now they're at 8.8.

From Bonnie on 12/17/07

I hoped the doctor was making a mistake in suspecting leukemia, and he apparently was. But that didn't turn out to be good news. The nationally known pathologist thinks this may be a metastatic rhabdomyosarcoma (RMS). Jordan will have a CT/PET scan Wednesday to investigate further, and another appt with Dr. Z. We are just now reading about RMS. It occurs mostly in children younger than 10 years of age, and has a 70% cure rate if caught early. The outcome is poor if it has become metastatic, as this appears to have done.

Because Wednesday's PET scan was negative, and rhabdomyosarcoma usually presents with a primary tumor site, Jordan's medical data was sent on to Stanford University for evaluation by a second pathologist. A very slow pathologist, it turns out.. Appointment after appointment, we heard, "Sorry, still waiting on Stanforrd." From Dec. 17th to Jan. 10th, we waited for that report. As a small diversion, the possibility of neuroblastoma was raised, sending us on another fruitless hunt. John, from the CLLC Friends site even sent us an article he found on rhabdomyosarcoma mimicking acute leukemia, which we passed along to the oncologist way back on Dec. 17th, but I can't say if it ever saw the light of day.

At long last, Stanford's report arrived, with an article referenced in the footnotes which led Dr. Z to confirm the diagnosis of rhabdomyosarcoma. Ironically, the article in question, Hematologic masquerade of rhabdomyosarcoma, is very similar to the one we had offered 3 weeks earlier.

The first two rounds of chemotherapy, while uncomfortable and difficult, have brought about marked improvement in the bone marrow's production of red and white blood cells and platelets. Transfusions are becoming necessary less frequently, and our hopes rise as the blood counts creep upward. It's true that Jordan has spent more time than he'd like in the hospital, but on two occasions, he was given a four-hour pass for a change of scenery. He requested a drive in the mountains, and Dan made it happen. I tucked a borrowed sled into the car, just in case Brianna could be coaxed into giving sledding a try. For a girl who grew up in southern China, this would be monumental. It took only a little arm-twisting, and a lot of courage, and she was off! Better yet,, Jordan followed suit! And now, friends, we're all caught up. :)