Newly Diagnosed 8 yr old son. ANC and platelets all over the place.

[curlygirl]

Hello,

On 26 April, I ended up taking my 8 yr old son to the hospital after he, his older brother (10) and my husband had a horible upper resporatory virus. Both boys looked like they were punched in both eyes for a month with the periorbital circles around their eyes. Eventually my older son got better and my younger one didn't. He was also very tired and very pale. At the hospital his hemoglobin was 2.7, platelets 7, and WBC 2.2. He did test positive for a rhinovirus/entervirus, and on the HHV-6 PCR. His ANC was around 650. He had a bone marrow biopsy in the hospital the showed 10% cellularity but was encouraging because it showe only 33% lymphcytes in the marrow (it was 75% in the blood) and percursors of recovery.

It has been 5 weeks and he has been all over the place. During his week in the hospital his ANC would jump daily from 630, to 450, to 775, to 835, to 520. Two weeks ago his ANC stayed in the 200-350 range. He did have a weird jump that the doctors though was a mistake in the machine but now I'm thinking not:

13 May: ANC 350
15 May: ANC 630 (4 pm)
16 May: ANC 200 (9 am)

He was recently improving and then bottomed out again yesterday. Here are his recent counts for the last week and a half:

21 May: ANC 520
24 May: ANC 720
28 May: ANC 780 - had made 37K platelets over the weekend for a total of 70k (33k was left on 24 May from last transfusion - first time making platelets)
31 May: ANC 390 (6k platelets)
4 June: ANC 168

Has anyone ever seen numbers like these before? I was wondering if anyone else has. Our doctors say that they haven't. They decided to do another biospy yesterday but I'm not sure what that will tell them, as it's a pinpoint in time and we know he's not doing well.

He is also now developing petechie a lot this week around his face, ears, and neck for the first time in the 6 weeks this has been going on. It's spreading today but my doctors aren't concerned - I'm not sure how long to let it go. He had 32k platelets left from the last transfusion yesterday, but had another biopsy yesterday, and our next appt isn't until Fri.

My older son has been tested to see if his bone marrow matches my younger son's for a transplant, but it doesn't.

Thanks!

[curlygirl]

P.S. I apologize for my typos (I was typing fast). Also, I'm happy to meet you although I'm sorry that we're all here.

[Neil Cuadra]

curlygirl,

ANC is computed as the product of WBC and the percentages of certain types of white cells as found in a white cell "differential" test. When the white count is stable this produces a measure of susceptibility to infection. If your younger son is still battling an infection it could be making his white count highly variable, producing the unstable ANC result. If the infection has already cleared up then I don't have a theory about the unstable ANC.

The doctors have their work cut out of them, keeping his counts up and finding an appropriate treatment plan. What diagnosis have they given you?

If you're trying to keep your son away from sources of infection, following his lab results, working with the doctors, and asking them your questions at each appointment, then you are doing just what you should be doing. I hope they soon figure out what's best to do for your son.

[curlygirl]

Thank you, Neil! My doctors have decided that it is Aplatic Anemia and have recommended Cyclosporine and ATG. My son also tested slightly positive on a PNH test. The doctors were ready to start but then my son's numbers jumped into the 700s, so we put a hold on it thinking it may increase (as opposed to decrease). It was so sad when we went back in and found out he went from 780 into the 300s in three short days. My son was so excited that everyone else was excited (even the doctors) that he was getting better. Except for the first night in the hospital it was the only time he started to cry. He did seem under the weather again in the days prior to his ANC collapse and had the dark circles under his eyes again.

We did go to Hopkins where we got my older son tested to see if he was a match for a BMT (our hospital does not perform BMTs). When he wasn't, Hopkins talked about their High-dose Cyclophosphamide treatment. After looking into it my husband and I decided that we'd rather stick with the Cyclosporine/ATG treatment. Also, my son really likes his doctors and nurses currently.

Thank you for the response! It is great to have input and it makes me calmer to have people to trade ideas with. Please take care.

[Neil Cuadra]

curlygirl,

ATG treatment for aplastic anemia is the tried and true method, routinely successful and usually well tolerated. There are no guarantees with this disease but youngsters are definitely in the most successful treatment group.

You might want to read Evan MacNeil's profile. Evan was 9 when he started having symptoms and he and his mom were gracious enough to share their stories with us. You can exchange messages with his mom if you want to (see the links at the bottom of the page).

You mention that your son was excited when everyone else was excited. That brings to mind something to keep in mind: that kids take their cues from the adults around them and especially from their family members. If you are optimistic and confident he's likely to be the same and if you let yourself get discouraged he's bound to sense it. Nobody can feel positive all of the time, especially when facing medical unknowns, but the more you can keep your own spirits up the more everyone else will feel that "team spirit". And it really helps!

[curlygirl]

Neil, thank you! David had the first initial symptoms - the bruises on his shins that when I asked how he got them he didn't remember, and even last week the petechia in the crease of his neck (he got it from looking down while playing Wii in the Drs. office) It's uncanny! Until these last few weeks I didn't even know that bone marrow could fail. We are going to start ATG on the 24th. I do have one more question. At our hospital they see this about once a year. They would do it in a PICU with a ton of other sick kids in there - they don't have private PICU rooms. Does anyone feel this would be ok? I'm not a fan of the idea. My son likes his Doctors, to the point where he does like to go see them, but I feel like I should transfer to a Hospital that would let him do this in a private room to stay away from germs?

[ssdavi71416]

Curleygirl

There are several facebook groups with AA patients and moms. The one that comes to mind is caregiver angels.

Most of us had no idea prior to having this disease that the bone marrow can fail. It is a complete shock. ATG treatment can have severe allergic side effects and can need ICU treatment if a reaction occures. I was fine during my treatment, but they did need to slow it down at one point due to chills and such. My Hematologist refered me to a teaching hospital (Emory)and suggested that I have the treatment at Emory...Mainly because of the inexperience of the staff at his hospital. When at Emory they waited until the second shift came on because the second shift nurse had performed many ATG treatments. I believe you and your son would be much more comfortable and avoid exposure to viruses and bacteria in a private room. I wish you well. Thank you for sharing your story and I hope and pray that your sons recovery is speedy!

[ssdavi71416]

The link below is from Neal Young at the NIH. It addresses many questions. The Title is "How I treat acquired aplastic anemia" by

Phillip Scheinberg and Neal S. Young

http://bloodjournal.hematologylibrar...1185.full.html

[KMac]

Hi Curlygirl,

Regarding your question on whether ANC & Platelets bounce around for anyone else with aplastic anemia, my ANC bounces around even more than your son's. Some examples below:

- My ANC was 200 at diagnosis.

- Went down to 0 during ATG.

- Went up to 650 within about 2 weeks post ATG.

- Fell back down to about 100 a week or so after that.

- Started responding shortly thereafter to regular neupogen shots.

- Went as high as 8000+ with neupogen.

- Fell back down to 240 after stopping neupogen.

- Rose to 2800 without neupogen, due to fighting a cold.

- Now, 15 months post-ATG, it bounces around between 500 and 1700. I never know what it is going to be when I go to clinic for my CBC.

My platelets bounce around too, but not as much as the ANC (between about 110 and 150 recently). My Hgb is more stable.

But (knock on wood) I feel pretty good and don't get sick. This is a strange disease. My most recent bone marrow biopsy showed a good number of white blood cells in the marrow, so my doctor thinks it's a mobilization issue, i.e. as my body fights to recover, the marrow sporadically distributes neutrophils, and hopefully as time goes on this will become more stable. That is his optimistic hypothesis. But on the other hand, he's sometime concerned I may still have an active autoimmune process destroying cells, so we consider another round of ATG if my counts were to fall more. I am hoping his optimistic hypothesis is correct!

I think my doctor is excellent. But with aplastic anemia, when counts bounce around, the reason is often a mystery to even the best doctors.

But despite these uncertainties, the ATG/cyclosporine treatment has given me my life back. I work, ski, climb mountains - I am so grateful for it.

I hope for the best for your son as he goes into ATG treatment.

Best Wishes,

[curlygirl]

Thank you, Scott and Kevin! It actually makes me optimistic that my son's bone marrow has spurts of activity; as if it may respond well to the ATG because it is waiting for something to help spur it along. We live closer to CHOP than Emory so I am thinking of moving there for treatment, although my husband would like to stay where we are. Also, our doctors do correspond with Dr. Neil Young at NIH. He offered to enroll my son in a study that includes a platelet growth medicine in addition to the ATG & Cyclosporine but I think we'll see how his first round of ATG goes and may do that if he needs a second. I'm glad you're both doing well and have high hopes for my son, too! Thank you for taking the time to answer my questions!

[Sally C]

Hi Curlygirl,
You mentioned Dr. Neal Young at NIH and what I assume is Promacta (Eltrombopag) for platelets.
If you are interested please read my posts under Clinical Trials. This drug and NIH performed close to a miracle with my husband Don (he has MDS). They had such success with their AA patients that they decided to try it on MDS patients. Don was their first MDS guinea pig and he has been transfusion independent for almost 2 years after having over 125 platelet/RBC transfusions.
NIH is a wonderful, healing place on the cutting edge. And Dr. Young is considered the #1 hematologist in the world. They have a beautiful facility for children and their families to stay.
If you have any questions about NIH should you decide to go that route, please feel free to contact me at shcalvert3@aol.com.
I wish your David the best!
God Bless,
Sally

[curlygirl]

Sally,

Thank you for your reply! I just read your thread about your husband and the NIH clinical trial. I am very excited for him and for you that it worked! Thank you for sharing!

[Sally C]

Dear Curlygirl,
Thank you for your kind words. I wish you and your son the best.
God Bless,
Sally

[KMac]

Hi CurlyGirl,

I concur with Sally, that it sounds like a wonderful opportunity to try the trial with ATG/Cyclosporine/Eltrombopag at NIH. That eltrombopag seems to have success helping all 3 lines of blood in some AA patients:

Here is a great article on that with before and after pictures of bone marrow:

https://ash.confex.com/ash/2011/webp...aper36762.html

...I was hoping to get an appointment with Dr, Young at NIH myself, in particular concerning my ANC, but he reviewed my records and basically decided I was doing too well to see him right now. I guess that is a reason I can be happy with! All the same, it would be nice to have the opportunity to be seen by him. But I understand he and NIH are very busy and have to prioritize who they can see when.

That brings up a point about AA treatment though that NLJabbari and I have discussed on this forum. It seems once you have a response to treatment, even a partial response, a patient can kind of get stuck in a weird "holding pattern" where they are doing too well for further treatment, but still not doing as quite as well as we'd like.

I bring this up because if your son had a less than desirable response to ATG/cyclosporine, I am not positive NIH would provide an opportunity to try eltrombopag after that. They might, perhaps it is something you could ask them.

I understand it is a huge decision, but I think personally if I had the opportunity to try eltrombopag right off the bat along with my other treatment, there's a good chance I'd take it. But of course every drug has a risk, so it is not an easy choice.

[KMac]

P.S. Sally that is such encouraging news about your husband's response to eltrombopag!

[Sally C]

Hi KMac,
You are correct about Don's response to Promacta (Eltrombopag). It is nothing short of amazing. And as you said with the AA patients, it also raised his red cells so he became transfusion independent for blood transfusions as well.
We are very blessed as the whites and reds are now normal - platelets last check about 123,000. There was a time that Don was on our Oncologist's "death list". Sometimes it's just a matter of finding the right drug. It's easy to deal with being on no MDS meds with those counts!
Thank you for your kind words. I wish you the best.
God Bless,
Sally

[curlygirl]

Thank you, Kevin! I talked to our doctor and she is reinvestigating the private/isolation room option. If the private room is not an option I am working on talking my husband into transfering into the NIH trial. Take care!

[curlygirl]

Hello! My son completed his ATG over the weekend. He had an allergic reaction to it despite pre-meding with benadryl and prednisilone. He broke out in poison ivy-like hives all over his body during treatment, so they had to slow the infusions down from 6 hrs to 12 hrs and give him intravenous hydrocortisone. He also developed some fevers. Then on the night the fever broke his temperature went down to 35 degrees celcius and he had a respiration rate of 7 breaths per minute so that shook them up. That said the room was freezing at night. He has a habit of kicking his covers up and I would wake up and cover him up again, but at one point I had to fall asleep so that was when his temperature dropped.

After the first treatment his WBC number dipped to 0.8 with 26% lymphocytes, then during the 2nd treatment the WBC went to 1.3 but 87% lympocytes. I forget the 3rd day's number (analogous to the 2nd), but on the last day he had WBC of 0.6 with 56% lymphocytes. He required 3 platelet transfusions during the treatments. The bizarre thing was that his hemoglobin jumped up and down during the treatment. He started with HGB in the high 9s from a transfusion, went up to 10, went down to 8.6, jumped up to 9.7, and then fell the day after the treatment ended to 7.8 (with WBC back up to 2.8, 75% lymphocytes). I would think that the 9.7 was a mistake except that the reason I asked for his lab work was that this was the day after the fever and temperature drop, and his temp was back to normal, and I kept thinking that his skin looked so pink, as if he still had the fever. So I aksed for the labs thinking "I swear he looks like he made blood" and he did, but I can't figure out how, because his reticulyte count was still 0.010. It stresses me out of course because his HGB shouldn't jump around all over the place, and it's doing it like it did when he was first admitted to the hospital with the virus before he started to get better and then didn't. I hate this disease because there's no normal so you can't even tell if his recent plummet is normal. :-(

Thank you for reading. I also have two questions:

1) Since our stay was extended due to the fevers and allergic reaction, the PICU nurses forgot to give my son Prednisone by mouth for a day and a half after his ATG ended. The doctor restarted it afterward. That shouldn't hurt anything, should it? As far as I can tell the prednisone is for preventing serum sickness and not trying to cure the AA? Will it hurt him that he was accidently taken off of it cold turkey and then started up again?

2) My son's urine has become rust colored. I'm not sure if this is hemolysis from his PNH clone, as his bilirubin jumped to 1.7 during treatment but is trending back downward (1.2 at discharge), or if it could just be a side effect of the cyclosporine. I am just worried about him rushing through his red blood cells before his appointment on Fri. Our hemotologist didn't seem worried about it but it seems odd to me?

Also, his blood pressure has risen from the cyclosporine. They've put him on 3 magnesium tabs a day as his magnesium was falling hoping that would help. Hopefully it will.

Thank you very much for reading! Take care and good luck to everyone!

[Hopeful]

Originally Posted by curlygirl

After the first treatment his WBC number dipped to 0.8 with 26% lymphocytes, then during the 2nd treatment the WBC went to 1.3 but 87% lympocytes. I forget the 3rd day's number (analogous to the 2nd), but on the last day he had WBC of 0.6 with 56% lymphocytes. He required 3 platelet transfusions during the treatments. The bizarre thing was that his hemoglobin jumped up and down during the treatment. He started with HGB in the high 9s from a transfusion, went up to 10, went down to 8.6, jumped up to 9.7, and then fell the day after the treatment ended to 7.8 (with WBC back up to 2.8, 75% lymphocytes). I would think that the 9.7 was a mistake except that the reason I asked for his lab work was that this was the day after the fever and temperature drop, and his temp was back to normal, and I kept thinking that his skin looked so pink, as if he still had the fever. So I aksed for the labs thinking "I swear he looks like he made blood" and he did, but I can't figure out how, because his reticulyte count was still 0.010. It stresses me out of course because his HGB shouldn't jump around all over the place, and it's doing it like it did when he was first admitted to the hospital with the virus before he started to get better and then didn't. I hate this disease because there's no normal so you can't even tell if his recent plummet is normal. :-(

I'm sorry that you both had such a harrowing ATG experience. While I was getting ATG, my counts were jumping all over the place. The doctors only looked at them to decide whether it was time for another transfusion. After the ATG was over, I felt great as my HGB rose to a new high before slowly dropping again. So don't be surprised if that happens as well. Also, be aware that Prednisone will cause a false increase in WBC. I can't remember if ANC is falsely affected as well.

Originally Posted by curlygirl

1) Since our stay was extended due to the fevers and allergic reaction, the PICU nurses forgot to give my son Prednisone by mouth for a day and a half after his ATG ended. The doctor restarted it afterward. That shouldn't hurt anything, should it? As far as I can tell the prednisone is for preventing serum sickness and not trying to cure the AA? Will it hurt him that he was accidently taken off of it cold turkey and then started up again?

It shouldn't matter at all.

Originally Posted by curlygirl

2) My son's urine has become rust colored. I'm not sure if this is hemolysis from his PNH clone, as his bilirubin jumped to 1.7 during treatment but is trending back downward (1.2 at discharge), or if it could just be a side effect of the cyclosporine. I am just worried about him rushing through his red blood cells before his appointment on Fri. Our hemotologist didn't seem worried about it but it seems odd to me?

This could also be a sign of the strain of all the drugs on his kidneys. It is important that your son drinks a lot of liquids while on cyclosporine. I am not sure how you do this with an 8 year old! I think I would give him any sugary drink that he wanted, as long as he kept drinking! Check his urine in the mornings to look for signs of PNH.

Originally Posted by curlygirl

Also, his blood pressure has risen from the cyclosporine. They've put him on 3 magnesium tabs a day as his magnesium was falling hoping that would help. Hopefully it will.

High blood pressure is one of the side effects of cyclosporine, as is low Mg. It's good that they are starting the Mg early!

Just curious...what is your son's dosage of cyclosporine and what is his weight?

[Neil Cuadra]

Originally Posted by Hopeful

Also, be aware that Prednisone will cause a false increase in WBC. I can't remember if ANC is falsely affected as well.

A false WBC reading will produce a false ANC.

[curlygirl]

Hopeful,
Thank you for responding! My son weighs around 60 lbs and is on 2ml of cyclosporine twice a day. I certainly hope the ATG/cyclosporine works. It would be so sad to have my son go through this and have it not work. The day after his ATG ended and we were still in the hospital he asked what would happen if this didn't work, and I said "We'll just do this again," and he gave me a look that I associate with people at work when they're thinking "what are you thinking, you moron?" but don't say it out loud. It was actually pretty funny but of course sad. Thank you for your responses, too. He is drinking more now that he's feeling better so hopefully that will help the urine situation. They'll check his creatine at his appointment on Fri. He's very pale so I know he needs blood. I'm hoping he will be ok until his appt. I'm not good at distinguishing what his skin looks like when his hemoglobin is in the 5's vs. the 6's, but I can tell he's below 7. He seems to have energy, though.

Thank you for responding to my questions and making me feel better!

[Hopeful]

Hi curlygirl,

I'm glad I could help. I think that it is a good sign if your son is energetic. I know for myself that I felt better after the ATG, even though it took a while for it to show in my counts.

Some follow-up questions for you...
Is your son on Sandimmune or Gengraf/Neoral?

Is he taking a total of 4 ml of cyclosporine a day?

Did the hospital do a skin test before administering the ATG? (It is water-under-the-bridge now, but I am curious.)

Also, did you ever check out the webcasts/webinars on PNH and AA on the AA&MDSIF website? They are very good.
https://live.blueskybroadcast.com/bs...?Client=680927

[curlygirl]

Hopeful,

He is on Neoral. Yes, it is 4ml a day total.

They did not do a skin test. I asked about one but they said they didn't need to do it because they'd continue the ATG either way (if he was allergic or not). There are three doctors in the pediatric hematology office in my hospital and the brand new one of course has the weekend shift.The other two have done this before but I don't think he has, because the other two know what I mean when I ask a question and this doctor just blinks at me when I ask him a question like he doesn't understand what I'm talking about. Even my father noticed that. Honestly at this point through all my reading I know more than he does. My husband doesn't like him either but he's the one that gets the worst shifts at the hospital for being the youngest member. At one point he was given our case for regular clinic visits and we asked to be switched to the other doctor.

I haven't watched the webinars but should, thank you!

[Hopeful]

Hi curlygirl,

It sounds like that hospital is a little inexperienced in the administration of ATG. They need to do a skin test to determine whether a patient is severely allergic, as some patients can go into anaphylaxis shock! Depending on the degree of reaction to the skin test, they would know up front whether to slow down the infusion rate or not do it at all. It sounds like you will have to be on your toes when working with this doctor/hospital. You may also want to look for a second opinion in parallel at a different facility.

I am not a doctor, but from my calculations, your son is on a very high dose of cyclosporine right now. Some of the newer research says that patients should stay not exceed 5 mg/kg/day but some of the older ATG protocols would start patients out at a much higher dosage (like 12mg/kg/day).

I think your son is on the equivalent of 15 mg/kg/day. Here is how I got that number:

60 lbs = 27kg
There is 100 mg/ml in the liquid form of Neoral
4 ml = 400 mg equivalent
400 mg divided by 27kg = 14.8 mg/kg/day (your son's current dosage)

You definitely, want to discuss this with your son's doctor soon. It could be that they are using a different protocol and have plans to lower the dosage. It could be that I am not doing the liquid form conversion right. Or it could be, like happened in my case, that they are using an older protocol and forgot to lower the dosage. The sooner you can correct this, the quicker your son's kidneys will thank you.

[curlygirl]

Thank you, Hopeful, I will ask tomorrow!

Really our doctors are good. We did get a second opinion at a research center farther from home. My son really likes his doctors and nurses. While getting his ATG he even said to me, "I miss the clinic." Then another night he was worried that once he got better he wouldn't get to see his doctors and nurses anymore. I reassured him that he would be going back a lot for a while. I realize that this isn't necessarily the most important thing but they are very good with him and he was overwhelmed by the size of the waiting room and number of people waiting in it at the research center and didn't want to go back.

I'm sure the doctors are doing a good job, I just get more worried each time he gets pale and needs blood.