May 2002 Updates
5/28/02 Update

We got released from the hospital today.  Ben's white blood counts were low, so we have to be careful and watch for fever and infection (see counts here).

We received Dr. Jaffe's pathology report today, it is attached here.

I am concerned that a new CNS lesions is showing up on the MRI.  Dr. Manwearing was not sure if it was a lesion and said we need to wait and see.  This lesion would be in the cerebellum, where all the others are in the cerebrum.

Ben tested positive for Rota Virus - he seems to be feeling better the last couple of days.  Diarrhea is much better.

We had to take Marie to the ER today.  She has been sick with rota virus for about 8 days.  She was dehydrated and refused to drink.  We will see how she does tomorrow.

5/26/02 Update

Ben's scalp lesions are getting more numerous, redder, larger and the older ones are showing yellow raised centers.  I will add some pictures later.  What does this mean?  We do not know - maybe they flair up with the chemo, maybe the disease is progressing.

Ben had some blood in his stools and we were all worried, but today he seems to be doing better.  The tests were negative yesterday.  Probably an aggravation from the diarrhea. (See Dr. Notes for 5/25)

His blood counts are a little lower for WBC ANC & Hemoglobin, but not low enough to be concerned. (See Dr. Notes for 5/25).  He is handling the Methotrexate chemotherapy well, just gives him diaper rash.

The new hospital is nice.  It just opened this week.  Still not like home, the rooms are no bigger, but it is much more comfortable.  We should be home Monday night.

5/24/02 Update

Just a few quick updates - Ben was really having a rough time the last couple of nights, not getting any sleep.  We are not sure what it was from, it could be so many things - antibiotics, chemotherapy, rota virus....  We took him into clinic today, and they decided to hydrate him because he has lost a couple of pounds over the last two weeks.  They also gave him some anti-nausa med. and some morphine.  We were going to go into the hospital tomorrow for his Methotrexate and Vinblastine, but they decided to keep him tonight and start.  We will be in the hospital until Monday or Tuesday.

Ben was 7 months old yesterday.

Marie is starting to do better, we have to be very careful what we feed her, she is still having stomach pains and cramps.  Carol went to urgent care yesterday and got some IV fluids, she was dehydrated from being sick.  She is starting to feel better.

5/22/02 Update

...and finally some good news....
We went for Ben's fourth MRI today.  Dr. Manwearing called us after he reviewed the results.  He said the main lesion has not grown and we can delay the next MRI until six weeks from today!  We do not know (cannot know) what to attribute the stop in growth to, but we are happy the lesion is not larger.  We were concerned that we would be going into brain surgery soon, but it looks as if that is safely put off.  We needed some good news!!!  Note: The lesion did not shrink, and we need to examine the other lesions for growth.

...and some not-so-good news...
One of Ben's scalp lesions is becoming raised and a little yellow (see picture below).  All the others have been flat.  What does this mean?  We do not know.  He is also getting a couple more red spots or lesions above his eyes & on his forehead.

   Poem by Cathy Kush

5/21/02 Update

Dr. Jaffe has reported back and concurs with Dr. Dehner, Ben has JXG.  This is the news we expected, but were not hoping for.  I have added to the Dr. Notes.

It has been a rough several days, Marie has been very sick for two days, and Carolyn was sick before that.  Today, Carol, Carolyn's mother started getting very sick as well.  We think it may be the Rota virus - we were put in a hospital room with a little girl that had it (we left as soon as we learned she had a virus), they moved her out of the room, but we may still have contracted it from the room.  You do not start showing symptoms for several days, and you are contagious for several days after you are done feeling sick.  Vigorous hand washing is supposed to prevent transfer.

Marie has not been able to hold anything down.  She is dehydrated - we went to the doctor today and they gave us some nausea medicine so we could get some fluids in her.  We have Ben quarantined with Carolyn to the master bedroom.  We are praying he does not get sick.

Ben's eyelid looks back to normal.  All his medications are keeping Carolyn & him very busy - Ben has bad diarrhea from the antibiotics, he throws up from the Prednisone and he is not eating well.  Carolyn spends a couple of hours or more giving him his morning medication, and then it starts all over again for lunch and then for dinner.  Ben will only take the Prednisone when he is in the bathtub, so he is getting three baths a day.  He has been going through a ton of outfits from the diarrhea and from throwing up the medications.

Tomorrow we go for Ben's fourth MRI of his head.  We are meeting with Dr. Manwearing, the neurosurgeon, directly after.  I should point out, that we are not judging the effect of the chemotherapy - we hold that judgment until the six week point.  That initial response is very important, so the six week milestone is key.  Tomorrow we will be judging whether Ben is in danger or not (for hydrocephalus or thalamic invasion).

5/18/02 Update

Ben did well at his clinic visit on Friday.  The whole thing only took a couple of hours.  Ben was given his IV push of Vinblastine through his porta-catheter.  He was also given some antibiotics because they were not 100% sure that his swollen eyelid and slight temperature was not infection.  His eye is a little less red today.

Ben is smiling at his Grandma Stokman below.

5/16/02 Update

Ben's eyelid has stayed about the same - the swelling has not increased markedly.  He still has a slight fever and we are giving him Tylenol for it.  The doctors have conferred and decided to wait and see how it progresses without antibiotics.  They do not believe this is an infection - it is some kind of swelling caused by either the lesion in his eye acting up or perhaps by the chemo doing its job.  We do not know which.  Hopefully the swelling does not increase.  Going into the eye would be a good opportunity to get a biopsy sample that might conclusively diagnose this disease, but we are currently not willing to take the risks just to get a definitive diagnosis - it would not change our plan of action anyway.  If the neurosurgeon has to remove Ben's largest brain lesion (to prevent thalamic invasion or to relieve hydrocephalus), then the eye doctor will go from the inside to remove the orbit lesion at the same time.  This will actually be safer and easier.  All the details of the discussion are captured in the Dr. Notes.

Ben has had a little trouble with the chemotherapy - he has been throwing up the Prednisone a few minutes after he takes it and he has been getting better at spitting it out.  He starts to cry when he sees daddy coming with the bib and the droppers full of medicine.  His little cry is heartbreaking.  We have been trying many variants - with flavoring, with food, and crushed pills vs. liquid.  Tomorrow we go to clinic to get his Vinblastine.  It will be our first visit to the new Phoenix Children's Hospital.

Dr. Jaffe has finally received the samples from Ben's biopsies (scalp and brain) after a 13 day mail delay!  It will be extremely interesting to see what results he returns.

Dr. Jubran has given me permission to post her wonderful presentation on LCH.  This is the same presentation she gave us at the LA regional meeting for the Histiocytosis Association on April 27th.

Below is a picture of when we brought Ben home from the hospital just after he was born.  We told Marie that Ben was her birthday present (the next day was her 2nd birthday).  As you can see, she was very happy and proud.

5/15/02 Update

Ben's eyelid started swelling again - last night we started seeing a noticeable change.  Today is is about halfway shut and Ben has a slight fever and is fussy.  His eyelid is red, pink and purple.  It is also swelling out.  This is almost a repeat of what got us to the hospital the first time, though it seems to be happening at a slightly slower pace.  We went to see the eye surgeon first thing this morning.  We are currently trading surgery with antibiotics.  It does not appear to be an emergency condition at this point.  I have updated the Dr. Notes with our discussions and options (see the section dated 5/15/02 - about 1 1/2 pages).  Below you can see the change in Ben's left eye:

5/14/02 Update

We just returned from the hospital after three days.  Ben had his first dose of chemotherapy.  We are giving him three chemos, Prednisone (3 times daily by mouth), Vinblastine (Every Friday by IV at the clinic), and Methotrexate (every other week by IV, three days in the hospital starting on Fridays).  He seems to be handling it well.  His immune system will not take a hit for a couple of days yet.  He was a little nauseous, but they gave him a few medications and it seemed to take care of that problem.   It is a little difficult to give him the medication orally, he gets better at spitting it out each time!  In the pictures below we are celebrating mother's day in the hospital.

5/10/02 Update

The doctor's notes have been updated per our discussions with three doctors today (see section dated 5/10/02).  The notes give some of the concerns we traded.  With Dr. Manwearing's help, we have decided to start chemotherapy tomorrow.  He believes we have a small window before we would need to do brain surgery to alleviate the hydrocephalus.  We will monitor Ben closely for any symptoms and hope that the chemo will reduce the size of his lesions or halt growth all together.  We will hopefully be admitted to the hospital at 11:00 AM for the first round.  Wish us luck.

Check out the new frequently asked questions page.

5/9/02 Update

We went for the MRI today.  Disappointing results, Ben's tumors have grown.  Click here for a comparison. Click here for an animation.
Tomorrow we see the neurosurgeon.  The decision we are going to be evaluating tomorrow is if we should start with brain surgery on the largest (yellow) tumor, or if we have enough time to try some chemo.

I have added a link to Dr. Grois's article about CNS involvement with LCH - 'Central Nervous System Disease in LCH'.  This seems to be the most comprehensive article on brain involvement for LCH.  For JXG, there is not much knowledge about CNS involvement.  The bad news - cases with central nervous system involvement are typically the JXG cases with a poor outcome.  JXG lesions do not seem to subside with chemotherapy.  There aren't any medications that are clearly effective in causing the tumors/lesions to decrease in size.

5/8/02 Update

No status updates, just additions to the web page:

I have added some animation of the tumors in Ben's Brain that really illustrate where they are and how big they are.  These are the 4/10 images.  The GIF files may take some time to load.  For each series; the left image is the raw MRI scan, the right image is an artificial highlight of each tumor in a different color.
For a back to front view click here.  For a bottom to top view click here.

I have added the CT scans from Ben's eye here.

I have added a link to Dr. Jaffe's article on 'The Histiocytoses'.  Page 8 has JXG information.  Page 4 starts the LCH information.  Note:  Cannot reprint.

I have attached  an article by Freyer, Kennedy, Bostrom, Kohut & Dehner on JXG and the 36 known cases of it.  'Juvenile Xanthogranuloma: Forms of Systemic Disease and their Clinical Implications'.

I have added a link to Dr. Dehner's pathology report on Ben.  I apologize for the fax quality. Best viewed printed out.

5/7/02 Update

Very little new information is available.  We are currently waiting on two events - pathology analysis and report by Dr. Jaffe and an MRI we have scheduled for Thursday.

Pathology
The pathology is important because we need to know if Ben has LCH or JXG.  We have two of the top pathologists looking at Ben's biopsy samples -  Dr. Dehner (an expert on JXG) & Dr. Jaffe (an expert on LCH).  We are eager for the results of Dr. Jaffe's pathology report to see if Dr. Jaffe concurs with Dr. Dehner, or if he has any doubts.  If any doubts turn up, we may need to go back into Ben's eye and get both soft tissue and bone samples from his orbit.  We may also want to take a third (larger) scalp biopsy.  We would then analyze these by electron microscope for something called Birbeck granules (presence shows LCH).  This has not been done before. From our own research of the literature and review of Dr. Dehner's pathology report (see comparison table here), we believe that Ben has what is called 'Early' JXG.  The term 'early' is not well defined.  Symptomatically or clinically Ben looks like he has LCH, pathologically or microscopically he looks like he has JXG.

MRI & Brain Surgery
We are also eager for the MRI because we want to know the current growth rate of the largest tumor (remember is was 10% in 10 days).  We are still very concerned about the largest tumor growing until it blocks off the ventricles and causes hydrocephalus.  To see more about the impact to Ben's brain, click here.  The doctors are trying to trade between brain surgery on the largest tumor, chemotherapy or a combination of both.  We may want to start chemotherapy before the surgery and hope for a quick reaction to the chemo - if we do not get a size decrease in the tumor then we many need to do the surgery.  We want to avoid radiation because of all its negative impacts on development.

More on JXG
JXG, or Juvenile Xanthogranuloma, is very rare, and because of a lack of any in-depth understanding, it is usually treated like LCH.  Multi-system involvement is even more rare.  So we are probably dealing with an atypical presentation of a VERY VERY rare disease with multisystem involvement (including the brain) with difficult pathology on top of that.  In addition, Ben's tumors are deep in the brain, which is not typical for LCH or JXG.  It is not known whether the JXG tumors in the central nervous system (brain) even respond at all to chemotherapy.

Sydney
The parents of Sydney (read more about Sydeny here & here) have been helping us out tremendously.  Sydney was born within a few weeks of Ben and had multisystem JXG.  She was originally diagnosed with LCH.  The diagnoses was later corrected to JXG.

Dr. McClain & Texas Children's Hospital
Several people have asked us how the visit to Dr. McClain in Houston, Texas went.  We are very grateful for the time and energy Dr. McClain has put into our case.  He met with us for several hours on Wednesday the 1st of May.  He is a phenomenal doctor, and he answered a ton of our questions.  He highlighted the uncertainty in a case like Ben's and he has been conferring with our local doctor, Dr. Etzl, about treatment options.

5/04/02 Update

We still do not have an answer on a firm diagnosis for Ben!  Our 2nd opinion Pathologist has given a diagnosis of something other than Langerhans Cell Histiocytosis.  His report says that Ben has Juvenile Xanthogranuloma (JXG).  We feel this diagnosis is even worse than LCH.  This disease is similar to LCH but usually presents as only skin lesions and resolves itself.  The more serious form that Ben has is even less understood than LCH and it is less clear how to treat it.  It is quite aggressive and does not always respond to chemotherapy.  In addition, the brain lesions are not typical.  Both LCH and JXG are treated with some combination of chemotherapy and steroids.  Right now we must wait until next week when our doctors will discuss which treatment will be best, given the two different diagnoses and the locations of Ben's lesions (brain, lung, orbit & scalp).  We are getting another MRI sometime next week to look at the brain lesions.  Ben's biopsies are being sent to another top pathologist with much experience in these histiocytic type disorders.  Hopefully he will give us a FIRM diagnosis.

 

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