6 year old son newly dignosed... Questions

[Bolinger]

Im new to the forum so Im sure that some of my questions have already been answered. My wife and I feel that we need another form of support other than the doctors. Why not try from someone who is living and dealing with the same things we are. I will try to keep this as short as possible. Mid February my son who is six years old began vomiting and having diarrhea. It was over a two week period and seemed to only happen at night on Fridays and Saturdays. Then throughout the week he was fine. Then again on the following Friday and Saturday, same thing. When he got sick again on we decided that it wasn't a bug and he needed to be seen. That night during his bath, we noticed what looked like hives around his waist band. We gave him benadryl for the hives and took him to the doctor first thing the next day. Because of the hives, his pediatrician decided to do blood work. She called a couple of hours later and said that we needed to pack a bag and get him to ------- Children's hospital which is about an hour away. Looking back we remember a lot more bruises than normal. Then we just thought is was bruises that every six year old boy gets... We were admitted due to his platelets being at 14. We stayed about 3 days, received a transfusion, and were discharged with no diagnosis. A week or two later we were back in the ER and admitted to the HEMOC floor again where we stayed about a week. The Dr was very puzzled every time they came in the room. After several days they did a biopsy and aspirate. The Dr said that he was 99% sure it was aplastic anemia. But again, he was puzzled and said that our son was an "enigma", he didn't fit the typical AA signs. This is what has my mind racing now, but I will get to that in a minute. They discharged us and told us that they wanted to monitor him and continue supportive care. Easter weekend came and we went to Texas to see family. We set an appointment at another Children's Hospital for a second opinion since we were close by anyway. On the way home, we received a call from our Dr in Arkansas saying that they received paperwork from our second opinion and wanted to start ATG the next day. Up until they received the paperwork on the 2nd opinion, the plan had been to monitor him for a couple more weeks and see if his counts would change. We were admitted the next morning and over the next few days he received the ATG's. Since discharge, he has been on multiple antibiotics, prednisone for 2 weeks, cyclosporin, and a few others. Its been 4 weeks since treatment and we are receiving platelets every 7-9 days. His levels will go down in single digits about that time. The Red Blood Cells drop in the 7's after 10-14 days. WBC about 1.5, Neutrophil 3, ANC about 40 and sometimes lower.

Now I get to my questions????? With the Dr saying that he was an "enigma" and didnt really fit the normal AA profile, could there be something we are missing? Is there anything else this could be? Could there be toxins in his blood causing the problem? Would they have tested for toxins like in a toxicology report? Could it have been a medication he previously took? We moved here September last year and live in a newer rental home. Could it be something in the house?

I know typically it takes 3-6 months to see any kind of results after ATG, but should be see some kind of increase yet? So far everything has decreased since treatment.

Sorry for the long post but I wanted to give everyone the complete rundown and hopefully get some insight, inspiration, motivation, something. Thanks

[sstewart09]

I'm not familiar with ATG treatment, but I wanted to recommend an excellent support group for your family. "Band of Mothers in the Fight Against Aplastic Anemia" is a Facebook group geared towards pediatric AA patients.

[NLJabbari]

Hello Bolinger,

From the description you've mentioned re: your son's presentation of symptoms, that sounds pretty typical of an AA patient to me. The rash on the waist (Petichae) due to low platelets, bruises, etc... The only thing you mentioned that is somewhat different would be your son vomit/Diarrhea episodes. I've often heard of allergy-like symptoms, coughing, tiredness, post diagnosis. I tend to think that his body was trying to fight some type of virus, but due to the low WBC/ANC was having a difficult time and hence it kept reoccurring. On the other hand, could this virus have been what triggered AA? That is the question that most doctors can't answer. The majority of AA patients are diagnosed as "Idiopathic" AA. Perhaps that's what your son's doctor was referring to as "enigma"...???

Was your son on any particular meds in the months prior to diagnosis? Any antibiotics, or allergy meds, etc.?

My son was 10 y-o when he was diagnosed. Prior to dx'es he was as healthy as could be. Very athletic and full of energy. About the only thing he struggled with were seasonal allergies. His Pedi. doc recommended OTC quick soluble Allegra and one nighty dose of Benadryl at bedtime as his allergies seemed to be a bit more than the usual. In hindsight, this particular year, he struggled the most with his allergies, followed by a nagging little cough, then came bruises that at the time I attributed to (Karate Lessons and Baseball) finally the Petichae and fever that prompted me to take him in! Unbeknownst to me at the time; this might've been a clue to something wrong going on in his immune system.

I've heard similar stories from other parents.

I hope your little boy will do fine and that he has a good response to this ATG treatment. While everyone is different, the numbers you've posted below seem about average for this period of treatment that your son is in. In my experience, a response happens gradually and the cell-lines respond at different rates. Seems like his WBC is showing a response, yet Neutrophils are low. Is he getting any Growth simulators like Neupogen?

[Bolinger]

Thanks for the reply. The hives were actually pink in color and raised where you could feel them. They ranged in size from dime to half dollar and all different shapes. Kind of like an allergic reaction to med would be. Problem was at that time he wasnt on meds. As far as the "enigma", we at one point had about 9 doctors circled around his bed just stairing at him. Just makes me as a parent wonder and think we are missing something. As far as the other questions I had, it makes me look back over the years or months leading up to it and think about chemicals I have used in the yard, household cleaners, interior paint, deck stain, etc.... the list could go on and on. I guess I am just reaching. Today was a hard day. Just like many others on here Im sure, we went in this morning hoping to see something positive to only to end up needing red blood cells when he just had them 2 fridays ago. Platelets were at 39 so he will be getting platelets wednesday at our clinic visit. By then they will be below 10, a week following the last transfusion.

As far as meds leading up to all this, I cant think of anything. He had a couple cold last year but nothing major.

They havent given any type of growth stimulators. I ask about them prior to treatment but was told that they werent going to give them.

[ssdavi71416]

With AA the treatment usually doesn't include growth factors. The Drs want the marrow to do it job on its own. Growth factors might slow the production of marrow. The thing to remember is that AA is probably a group of related occurances as opposed to a specific illness. Platelets only last 2-3 days from my experience. It is difficult to determine what the cause might be and I would not worry about it to much. Hopefully everything will begin to work soon. Have the Doctors discussed bone marrow transplant at this point? They probably will wait at least 6 months for the ATG/ cyclosporine to work, but it is worth talking about BMT. I wish you all the best

[Bolinger]

They have talked about a BMT and would have probably done it right away if we had another kid and they would have been a match. I know what you mean as far as what might have caused it, but as a parent, I guess in the back of my mind I keep thinking or hoping, that maybe its not AA. Theres a small part of me that keeps wondering if it could be something else. I just dont want to miss anything.

[Hopeful]

Hi Bolinger,

Be sure to get a copy of your son's lab and BMB results every time he gets one so that you can spot the trends and subtle changes. Also, don't be afraid to ask the doctor what specifically makes your son "an enigma".

Did the doctors run your son through a battery of tests to rule out everything else (arsenic, parovirus, heavy metals, etc.)? AA is a diagnosis of exclusion.

[Marlene]

Also tests to rule out any genetic anemias like Fanconi and Diamond-Blackfan anemia. Even though my husband was diagnosed at 51 yrs, they still ran the tests.

Get all of the test results. Make sure they have checked nutritional levels on B12, folate, b6, copper, iron and zinc. B12 should be at least at 500. Anything below that should be investigated. Any highs or lows in B12 and folate indicate a possible conversion problem.

Look at potential dietary problems with wheat, gluten, additives, etc.

[NLJabbari]

Hi again Bolinger,

I agree with most of the comments posted here as far as all the testing that is required and the fact that you should keep copies of all tests/labs and results.

From the information you've shared, I assumed your child had been diagnosed with either Severe or Very Severe Aplastic Anemia (SAA or vSAA). It should have been classified as either an Identified Source e.g. Hepatitis, Idiopathic (Unknown Source) or Genetic Form e.g. Fanconi etc. To the best of my knowledge these tests for classifications were done prior to treatment with ATG.

Regarding the use of Growth Stimulator(s), more specifically WBC stimulator GCSF such as Neupogen. Back in 2004 when my son was diagnosed; it was part of post ATG Protocol. At least most of the people (parents) I spoke to back then and a bit after had mentioned a short course of Neupogen as part of their child's post ATG protocol. I fully understand that might not be the case any longer as there was controversy as to whether or not it was beneficial.

With AA, you really have to be patient and take it one day at a time. Do the best you can for yourself and your child on a daily basis. Slowly, but surely changes will come about. Keep a journal and jot down your thoughts, daily occurrences, questions for the doctors or even questions for us on marrow forums.

[NLJabbari]

I almost forgot to mention....regarding the hives on your son's waistline. Could he possibly have developed allergies to elastic or latex?
Interesting, as a young child, my son was sensitive to the elastic and metal buttons on his pants. I recall having to put a bandaid on the inner clasp of his jeans etc...I had totally forgotten this since he no longer displays any such allergic reaction.

Just thought I'd share this

[Bolinger]

They did rule out all of the genetic anemia's such as Fanconis. After testing, it was ruled as idiopathic. Not sure what other test were run, I remember the parvo virus, HEP, HIV all came back negative. There might have been a few more that I am missing. As far as testing for the thing that "Hopeful" mentioned such as arsenic and heavy metals, I don't remember anything ever being mentioned about that. That's what I was referring to as the "toxicology report". I probably have the name really messed up though. I don't recall them ever testing for anything like that. We talked about mine and my wife's professions, me being active duty military and possibly bringing back something from my 2009 deployment to Iraq or the fact that my wife is a mortician and possibly contracting something while embalming? Being in Iraq I was exposed to who knows what...They said that this was very unlikely and of no concern. But as far as I know, there were no test for potential toxins.

We have an appointment tomorrow morning so I will definitely have a lot of questions for them. They also have never mentioned anything about nutrition levels or dietary problems as Marlene mentioned so I will ask. Another thing, is it normal to have a PICC Line or does anyone have a port? We have had the PICC Line since April 2nd but are wondering if the port would be better? With the PICC he is unable to bath himself because of getting it wet and we keep having issues with it clotting and having to make trips to the ER and hour away.

Thanks again for all of the information. Sure beats Google!

[NLJabbari]

That's a good question. I would also ask the community on FaceBook's "Band of Mothers in the Fight Against Aplastic Anemia" Some of the members might have good advice as it applies to Pediatric Patients.

My son had a Double-Lumen picc line and had no problems with it besides the inconvenience in the shower.

https://www.facebook.com/BandOfMothe...Anemia?fref=ts

[Sally C]

Hi Bolinger,
My husband has had a picc line and now has a port. I think anyone who has had both would go for the port hands down. It's just under the skin above the nipple. There is so much less risk for infection. Also the only maintenance he has to do for his port - now that he is transfusion independent - is have it flushed with saline and heparine every 6 weeks. If he was still getting transfusions that wouldn't even be an issue.
Otherwise he doesn't even know it's there. No restrictions with showers. swimming or anything else for that matter as it is completely under the skin.
I'm so sorry for what your son is going through.
I wish you well.
God Bless,
Sally

[Marlene]

Since he's had digestive issues, ask to have them check for the h.pylori bacteria. It's been linked to blood disorders in some.

Don't let them dismiss the nutritional aspect of his health. Even if he's not deficient in any areas, low normals at his age would cause me to wonder why. It may not be the reason for the SAA, but you need adequate levels of B12, folate,etc to heal the marrow and restore counts. B12 serum is not a conclusive indicator of B12 status. There's been so much new data around B12 and folate that many doctors are not aware of and may dismiss it. So just know that if it's below 500, there are more test they can run.

[Hopeful]

Hi Bolinger,

I had a 24 hour urine test to check for heavy metals and arsenic. You would remember if this test was done I would recommend doing it if you are unsure about current environmental exposures, as this is something that you can control. You can drive yourself crazy trying to figure out the cause.

[NLJabbari]

Bolinger,

Do you know whether your son is CMV pos. or neg.? CMV (Cytomegalovirus) is a virus that many are carriers of. It is also a virus that can cause problems in transplant patients.

I tend to think ahead and try to prevent whatever I can and that is within my ability. My son is CMV- and therefore Only received CMV- blood products. One of his doctors didn't think it was important as her argument was that nowadays most blood products are Irradiated. Needless to say, I fired her! I spoke to the director of our local blood bank and she advised me that even "Irradiated" blood products have a small chance of being CMV+ The Unit bag should display an additional sticker stating that the Unit has been specifically tested for and is CMV-

Also, when you get a chance you might want to check-out some of the Webinars in the AAMDS website. There is one specific to blood compatibility and some updated information as it applies to patients with blood disorders that are in need of transfusions. One interesting fact that I learned in this Webinar is that patients who present with a PNH clone whatever size, should ONLY receive "Type Specific" RBC's.

My son's new doctor who is also a Professor of Hematology at Stanford University agreed with me and has attached a Perm. message to his files that the transfusion dept. would see should he need a transfusion. Luckily, he has not needed one in a few months.

Just another bit of info. I thought I'd share with you.