Mega Steroids Day Four plus IVIG

It's nine o'clock and Jenna has been at it since we got here at three this afternoon.  She is doing round four of Solumedrol and just started her first round of IVIG.  I talked to Dr. Guthery yesterday about her not being able to have a BM without an enema ever since she had the barium.  He told me then that the IVIG had been approved by our insurance, so she was going to start it soon.  I wasn't sure if he had ordered it for today, but it turns out that he did.  She had to do the Solumedrol first and then IVIG after that.  She was able to tolerate speeding up the steroid, so that took an hour off of that infusion time.  The IVIG is supposed to run over three hours, but there is still plenty left in the bag and we are at the three hour mark.  This will be a long night.

Dr. Guthery ordered an abdominal X-ray to check stuff out and he said that she looked pretty good, as far as the amount of stool in her colon went.  He recommended taking a higher dose of miralax yesterday to help her move her bowels, and we talked more about that today.  He told me to have her take one square of ex-lax twice a day, so she can add that to her growing list if meds.  At least it comes in chocolate.  We also talked about the MRI and I am supposed to call tomorrow to schedule it for next week.  I will hopefully be able to schedule it for Thursday or Friday when we are down here for the next round of steroids.  Jenna told him about the rumblings low in her belly and he thinks it is probably just air moving through.  She was also concerned at the frequency of her hiccups, but he didn't have any thoughts on that.

Jenna has been asleep for abut an hour and a half now.  She gets so exhausted by the end of the day that she asks for us to have family prayer so that she can go to bed.  She was on the beginner swim team last year and I have been concerned about signing her up for it again.  She complains of muscle pain and weakness often and she gets fatigued easily.  Dr. Guthery said that it might actually be good for her.  I will need to discuss it with the coach and make sure that he understands.

In ther non-hospital related news, today was the kids' field day at school and tomorrow will be the last day.  Hallelujah.  Paige is working at the pool as a lifeguard and it opens on Saturday.  David and I have been working a lot out in the yard.  It was neglected for a long time before we moved in.  It is coming together nicely!

Mega Steroids Day Three

Zofran is a miracle drug.  I requested an order for some today, given her history of nausea over the last two infusions.  It can cause constipation with chronic use, but the doctor agreed that she could have one dose to get her through today.  So far, so good, and it may have even helped with the metallic taste in her mouth because she said it isn't nearly as bad as it has been.

She put her room service menu to good use again and had more popcorn, beef jerky, mozzerella sticks, and a root beer float. She is excited to not have to come back again until Thursday.  The veins in her hands need a break.

Today we are thankful for good nurses.  All of them that we have had over the last three days have been fabulous.

Mega Steroids Day Two

Today was kind of rough.  Jenna started the steroids yesterday and had the second round today.  We went to the Rapid Treatment Unit, or RTU, at Primary's to get it done, since it is done by IV instead of oral.  Methylprednisolone, or Solumedrol, is a mean drug.  The run time for it is one to two hours, but we went over two hours to see how it tolerated it.  It turns out that she doesn't tolerate it well.  It gives her a horrible taste in her mouth and makes her feel nauseated.  About three-fourths through the first dose yesterday, she puked and felt so much better.  Her favorite thing, or really the only good thing, about the hospital is that they give her a room service menu and she can order at any time.  Denver Children's used to give us a $6 cafeteria coupon for an all day infusion.  Room service is way better for her, although the cafeteria in Denver is about a thousand time better than the main cafeteria at Primary's.  Any-who, she ordered a bunch of food that she didn't feel like eating until after she puked.  She magically got her appetite and down a whole bowl of popcorn.

She got her food ordered first thing today and went to town on it while we waited for the IV team to come and start the IV.  She prefers them because they have a cool lidocaine shot that isn't really a shot, more like a puff, and it numbs the site pretty well without having to poke her.  It took them two tries to get a good one started.  It didn't take long for the bad taste/nausea to settle in.  Unfortunately, she wasn't able to puke and make herself feel better.  She was miserable all the way home.  I made her take her miralax before bed and that was the winning ticket to puking.  Hopefully now she will be able to sleep. 

Dr. Bohnsack came in to see her today.  He told us that the MIBG from yesterday was kind of inconclusive.  I asked him to clarify what that meant because that is the scan that makes neuroblastoma light up.  If I actually understood what he said, he said that it looked hot around her adrenal gland, but that the radiologist was unsure if it was accurate because her stomach is distended and pushing things over to the right.  The way the iodine goes through the blood vessels, it may just be a collection of iodine in that area.  I think.  So they are trying to decide if the next best thing is to do a CT or an MRI to figure it out.  He has a call into the oncology department to see what they want to do.  He is also going to be going on vacation next week, so hopefully we will at least get the next step figured out before he leaves. 

25.2 kg was her weight today.  That's 55.44 pounds. 

The Jenna Chronicles

One of the things that I regret the most is that I didn't do more journaling when Jenna was going through all of her treatment for neuroblastoma and opsoclonus-myoclonus syndrome (OMS).  I am pretty good at remembering the big details, but it would be nice to be able to go back and read the little details.

Jenna is eleven now and having more medical issues.  She has been complaining of GI trouble off and on for years.  We have taken her to see different doctors over the years, but none really ever helped her or really spent much time trying to figure out what the issue was.  In February of this year, her belly was quite distended and painful, so I took her to see our family doctor and was referred to a pediatric GI.  She saw him and he scheduled an EGD and a colonoscopy for a week and a half later.  (P.S. We don't see this guy anymore.)  Her pain and distention got worse before that date.  After vomiting ten times in a day, and realizing that she had not had a bowel movement in a week, we ended up at the emergency room at Ogden Regional.  They took an abdominal x-ray and the size of her colon was shocking!  No wonder she felt so painful!  They sent us down to Primary Children's in Salt Lake.  Over the course of the next few days, she had two surgical disimpactions done and cleaned her out.

Her new GI doctor, Dr. Guthery, put her on a miralax regimen to keep her bowels regular and hopefully shrink her colon.  He also tested her for paraneoplastic antibodies and voltage gated calcium channel (VGCC) antibodies, both of which were positive.  This is where her situation gets interesting, and also confounding.  Paraneoplastic means related to cancer.  We know that she has had cancer, but what we don't know is if she has had these antibodies since then.  No one is routinely tested for those.  The VGCC antibodies are most often associated with Lambert-Eaton Syndrome, but she doesn't seem like a classic case of that.  It is often associated with small cell lung cancer.  What we also don't know is where in the disease process these antibodies show up.  Do they show up before the tumor can be detected?

Jenna had good days and bad days over the next two months.  Dr. Guthery ordered an upper GI with a small bowel follow-through on April 29th to see if her small bowel also had motility issues.  That required that she drink barium contrast and it plugged her up and made her hurt.  On May 1st, she still hadn't passed the barium and was in so much pain that she was admitted back to PCMC.  They gave her some IV hydration and toradol for pain.  Because she already has gut motility issues, she is not able to have any narcotics for pain or zofran for nausea because they cause constipation.  The toradol worked great and she slept for the first time in days.

During that admission, we had a parade of doctors seeing her.  Dr. Guthery came in and said that the x-ray that she had there showed lots of barium that she still hadn't passed.  He was thinking that her condition might be autoimmune, but there is no particular syndrome or disease that he could point to. He was referring us to the oncology department to explore the possibility of a neuroblastoma recurrence or other caner, and to a rheumatologist to explore the possibility of it being autoimmune.

Dr. Bohnsack is her new rheumatologist.  We talked a lot about treatment options and what would be the best way to start.  He suggested pulse steroid treatments to suppress her immune system.  This means that she will do three days of high-dose IV steroids and then four days off, repeated for a few weeks.  The idea is that if she has a positive response, meaning that her gut motility improves, it will indicate that it is in fact an autoimmune problem.  She is set to start those tomorrow.

Dr. Fair is the oncologist fellow that we talked to.  He said that given the type and stage of neuroblastoma that she had initially, the odds of having a relapse are very slim.  He did think, however, that given her other issues it was reasonable to do some scans that will rule it out.  Most neuroblastomas will light up on a MIBG scan.  She is having one of those tomorrow.  We went to PCMC today and she had the contrast injected.  She will also be having a neck/chest/abdomen/pelvis CT scan to rule out any other types of cancer, hopefully done in the next couple of weeks.  She was supposed to have that one done last week, but she still hadn't passed the barium.  Daily enemas over three days got it out enough that she can have her scans this week and start the steroids.

That puts us mostly up to date.  Jenna is in good spirits for the most part.  She is used to hospitals and doctors, so that is helpful.  She has missed a lot of school.  We are thankful that we are at the end of the school year.  Please say a prayer for us that the MIBG will come back negative for neuroblastoma and that the steroids will work.

Rain in Due Season

I was just looking out the window this morning and noticed that it was raining. It got me thinking. We had a super dry winter last year, followed by a super dry summer. Elder Russell M. Nelson came to our Stake Conference on Auguest 21st and blessed the valley with rain. I hardly think it is coincidental that it has rained almost every single day since. He also pointed out a scripture. Leviticus 26:2-4 says:

2. Ye shall keep my Sabbaths, and reverence my sanctuary: I am the Lord.

3. If ye walk in my statutes, and keep my commandments, and do them;

4. Then I will give you rain in due season, and the land shall yield her increase, and the trees of the field shall yield their fruit.

My Little Fish

Paige, Jenna and Jack have been at John and Marsha’s for the last two weeks. They had a full session of swim lessons, along with swimming at Karen’s and Emma’s just about every day. This is the result.

Jenna is a very cautious person.  It took her forever to feel comfortable putting her face in the water.  I am so proud of her!

Jack took off right where he left off last year.  He is such a big boy!

French Silk Pie

Every week I make a pie, I learn something new.  The last time I baked the shell empty, which is actually called “blind baking”, it puffed up and shrunk.  I was told that to keep that from happening, I should put dry beans in a pie tin and then place it inside the pie and bake it that way.

I tried that, but I couldn’t get the pie tin to nest right inside the shell without mashing the fluted crust, so I just dumped the beans on top of the raw crust. 

That didn’t work, either.  The beans kept the bottom of the shell from baking at the same pace as the top.  They also made little bean-shaped rivets and it still puffed up.  I had to pick them out and put it back into the oven.  I think this made the shell too hard.  You can see in the pictures that the crust was very flaky, but it was just over-cooked.

The filling was made using this recipe.  I wanted to use cocoa instead of baking squares, just to cut down on cost.  I don’t know if there is a difference in taste or not.  I guess I’d have to make it with the squares and compare the two. 

I also used Egg Beaters, instead of regular eggs, because they are pasteurized.  The first time I had French Silk pie was when I was pregnant with London.  I was seriously in heaven eating it until I learned that it had raw eggs and then I cried because I couldn’t finish the piece I was eating.  (I didn’t even eat cookie dough when I was pregnant.)  So not having to worry about getting salmonella from the eggs was a great thing. 

Also, you’ll notice that the edge of the crust is all jacked up.  That came from me putting the crust down on a rack by the back door so that it would cool faster and Paige plowing through it on her way in through the door.  Luckily, all was not lost.

Overall, this pie was delicious, with the exception of the hard crust.  I knew that the crust would be the toughest part of this, so I’m okay just doing it again next week.  I ordered some ceramic pie weights and a silicone pie crust shield from amazon, so I am expecting to have better luck with the blind baking.

I am actually going to make two pies this week because we have a chuckwagon dinner/potluck at the church on Friday.  I’m planning on a lattice crust apple pie and a lemon meringue pie.  Stay tuned.