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.hematologylibrary.org/content/120/6/1185.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/webprogram/Paper36762.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]

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.



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.


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.



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]

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/bsb/client/CL_DEFAULT.asp?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.

[curlygirl]

Hopeful, the doctors checked his Creatinine level yesterday and it's getting lower than it was on the ATG. It's down to 0.33. Also his urine is looking better. His bilirubin went up to 1.7 during the ATG and is trending downward; yesterday it was down to 0.8. So it looks like there was some hemolysis going on in the liver but it is resolving. So things are getting better. Thank you for helping me know what to ask at the appointment!

[Hopeful]

Hi curlygirl,

Glad things are getting better!

For my benefit...did I do the cyclosporine calculations incorrectly or is your son on a high dosage as part of his protocol?

Also, are his doctors checking his cyclosporine trough levels to avoid toxicity?

[curlygirl]

Hi curlygirl,

Glad things are getting better!

For my benefit...did I do the cyclosporine calculations incorrectly or is your son on a high dosage as part of his protocol?

Also, are his doctors checking his cyclosporine trough levels to avoid toxicity?

Hopeful,

You calculated it correctly! They are using the dosage outlined by Dr. Neil Young in the article ssdavi71416 (Scott) linked to "How I treat acquired aplastic anemia" - "We initiate CsA on day 1 to a target trough level between 200 and 400 ng/mL, starting at a dose of 10 mg/kg per day (in children, 15 mg/kg per day).79" So our doctors started at that level and are then going to check and taper it each visit. We go back again tomorrow.

What I find fascinating about all this is that I notice that my son gets hemolytic anemia when his ANC drops below 200. That's only happened twice: once in May and again on ATG. Usually he is around 400. That actually sounds hopeful because secondary autoimmune hemolytic anemia tends to occur in lymphoproliferative diseases. That means his hemolytic anemia is probably secondary to another autoimmune disease, in this case the aplastic anemia. So it gives me hope that he will respond well to the immuno-suppressants.

Thank you for checking up on us! I will let you know what our cyclosporine end dose ends up being and if there are any effects on my son's kidneys.

[Hopeful]

It sounds like your son is in good hands :) It's interesting that pediatric patients start at a higher cyclosporine dosage than adults!

[curlygirl]

Hopeful (et al.),

I wanted to follow up on the Cyclosporine treatment, since you were helping me with it. My son stayed in the 260ish level for the last two weeks. Yesterday his level shot up above 400 so they are lowering the dose to 1 ml two times a day.

I really wanted to thank you for warning me about the WBC count and the prednisone. He weaned off the prednisone a few days ago and his WBC counts dropped from 6000-7000 back down to 2800. Oddly his ANC went UP with the WBC drop, because the 6000-7000 was 90-95% lymphocytes, which was higher than he had even going into ATG. So I think it was the prednisone bumping up the count. I think they were true white blood cells but not the type we wanted :-p So thank you for warning me. I didn't feel at all upset when I saw the 2.8 number because you had warned me to expect it.

His reticulocyte percent is above 1% though, which I'm taking as a good sign! It was sitting at 0.4-0.6% prior to ATG.

Thanks and Take Care!

[Sarah H]

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.

Their comfort with their medical staff is extremely important. My daughter was diagnosed with SAA March this year and had BMT April as her brother my 3 year old son was great match.
I have asked doctors and nurses not to mention ideas of getting better or going home or similar around 8 year old as they take everything on and get sad when it doesn't happen. They feel personally responsible for getting sick again or not getting better. They feel like it's their fault everyone is sad and stressed and if only they could do it right, they can get better and it will all go away. Which it won't of course.
We say 'when' or 'we hope' or 'let's just do what we need to do and just think about that'.
Lots of things about treatment are different in Australia, like there is only 1 hospital to get treatment in the whole of north east Australia! but taking care of a child is the same everywhere. They have to be helped to feel it's not their fault and no one is put out by them or they can't wish for things to change. They just need to focus on drinking their water, taking their meds, eating without being too fussy, telling the truth about how they feel, resting when they're tired and keeping busy when they feel well...those are things they can control.

[curlygirl]

Hello, everyone! I haven't posted on my own thread for a while but wanted to when things started looking up! My son hasn't needed a platelet transfusion since 29 August. They've been above 30 for three weeks. For the last two weeks his reticulocyte % was in the 3s and today was almost 5%. It's been two weeks since his last blood transfusion and his hemoglobin is still above 8. He had a little metamyelocyte in his white blood cells, too. He also had nucleated red blood cells in his blood back in mid-August. So there appears to be a good left shift in his cells (http://www.pathologystudent.com/?p=318). His RDW, which sat at in the 12s for most of his illness, shot up to 17 last week and 19 this week, which, thanks to Kevin (KMac), I also read can be a sign of improvement. So things are on the up and up!

His ANC continues to swing wildly. It's gone from 400 to 800 to 1300 to 650 to 900, all over the last three weeks. His neutrophils are definitely trying to make a comeback, though. Twice they've hit above 40%, which is higher than when he was diagnosed.

I'll make sure to post whether things go up or down. I of course hope they continue to go up, up, up! Thanks for everything everyone's taught me on the board and for the hope you've given to me and my family!

[Chirley]

All sounds very positive. I love reading about metamyelocytes and left shifts etc.

Did you think that you would even know those terms let alone what they mean, 5 years ago?

It's all a learning experience.

[curlygirl]

Chirley,

I wouldn't have even thought 6 months ago that Aplastic Anemia even existed! I'm a research at heart and by profession, as I told my boss at work, this would all be a really interesting academic exercise if it wasn't real life. I'd rather not be reading it in real life but it's better to be as informed as possible. Thank you for responding!

[curlygirl]

Hello, all! As a short update, my son hasn't needed a platelet transfusion since late Aug and a blood transfusion since early Sept. His white blood cells haven't really gone up, and his hemoglobin was slowly declining, but now has been hanging out at 7.6 for the last 6 weeks. Wish it was more (and going up!) since you don't feel good when your hemoglobin is 7.6 but it's better than being below transfusion level! While I was reading about the anniversary of JFK's assassination, I happened to read about his "blood disorder", which now has a name (Addison's disease), so I was telling my son how one of our great Presidents had an autoimmune blood disorder as a child that made him very sick, but he grew up and became President of the US. That made him pretty happy, thinking that he could be President one day :-). Happy Thanksgiving!

[Heather8773]

Awe I love that story you told your son!
#heartwarming
God bless!

[curlygirl]

Hello! I realize I post all over the place but I last posted on my beginning thread in Nov 2013. It's been 17 months since ATG and this week my son's numbers all hit low normal for the first time. WBCs have been normal for a while but it took longer for blood and platelets to catch up:

WBC = 5.9
Hemoglobin = 13.1
Platelets = 152

His platelets still swing wildly, up to 40k by appointment. And he still gets marrow growth "spurts". He'd have a jump in numbers every 3 to 4 months, then stay stable for a while or start to trend slightly down, then jump up higher. We could tell last week that his hemoglobin was probably higher because he had more energy consistently than he had in a long time.

He's still on 50 mg cyclosporine each morning and evening.

We went to get our Christmas tree last weekend and my husband took us to Home Depot and I asked why we were getting it there and not our normal place. He replied it was cheaper and that we got it there last year and I just didn't come (as in he was annoyed that I didn't come.) So I looked at the kids and actually said I couldn't remember why my younger son and I didn't go get the tree last year. Then a few minutes later I remembered that it was because my son had been in the hospital at the beginning of last December with his temporary relapse (in conjunction with a respiratory infection). So while I still usually have anxiety it was the first time that we both actually forgot about it. :o

[Hopeful]

Hi curlygirl,

I am happy to read that your son is doing so well! Have a wonderful Christmas :)

[KMac]

Hi Curlygirl,

So glad to hear about your son's improvement! His counts really look great! My recovery has been slow and sporadic, but good, so much like you describe with your son.

Some of the nurses at my hospital have anecdotally observed that patients who get better slowly, actually seem to get better more thoroughly, and stay better. Not a scientific study on that, just an observation on their part, that I sure hope is correct and significant.

Merry Xmas!

[curlygirl]

Hello, everyone! Thanks for following me so far! My son is starting to relapse. We dropped his dose of cyclosporine to 25 mg morning and evening and very quickly his platelets fell from 150k to 44k. They are now in the 30s. His Hemoglobin has fallen in the last 6 weeks from 12.7 to 11.0, and I'm sure is lower: he went back to school and has the light purple bruises across his legs. We've raised the dose to 150 mg of cyclosporine day and night (at this level his trough concentration is between 200-300) but the bruises are still there. Hoping for the best as we removed his port last November. His doctors and we were again disappointed - we were at the visit to actually take him off of cyclosporine completely - he had a tan from swimming all summer and looked fantastic. People still tell us how great he looks. I'm ready to go to BMT but his doctors and my husband want to try a 2nd dose of horse ATG and never go below 50 mg of cyclosporine day and night for the rest of his life. My son really wanted to go off the cyclosporine. HEveryone please think good thoughts that the cyclosporine will work - mainly because he had such a bad reaction to the ATG last time (hives, fevers, and then hypothermia) that I'd rather him not go through it again.
Thank you!

[Fana]

Hi curlygirl
I am so sorry to hear about your son's relapse.i can't imagine the pain you must be going throug.my son is one year post BMT this September he is also tapering his tacolimus so for so good.hope you also can make a good decision regarding his treatment.praying for your son.

[Hopeful]

Hi curlygirl,

I am so sorry to read of your son's relapse! Hopefully things will turn around on the higher dose of cyclosporine. I will keep him in my thoughts and prayers.

[curlygirl]

Thank you, Fana & Hopeful!

[curlygirl]

Hello! It's been a few months since I updated and wanted to again.

1) My son got strep twice over New Year's. Really it was one time but Amoxicillin didn't cure it so we had to get a second dose of antibiotics of Augmentin. His numbers always take a big jump on Augmentin. The excitement was that WBC went from 3.5 (bad), to 10.1 (also bad, for different reasons), to 5.5 (good). Also, on Augmentin his platelets went to 119k - a new high after falling to 35k after trying to taper the cyclosporine. At the same time I feel that: 1) Between ear infections and strep his only big jumps have been after Augmentin, and 2) the entire purpose of my original post - where he had 8k platelets, then made 70k on his own, then fell to 6k, was when he was on Erythromycin after he started wheezing - makes me think he needs a long term antibiotic. Has anyone else's numbers improved on an antibiotic? His numbers stay the same on cyclosporine but don't "go" up. I'm starting to get thankful for when he gets an ear infection because I can expect the jump in his numbers. I wish someone would just try putting him on 3 weeks of an antibiotic just to see what would happen but I can't get his hematologist or pediatrician to try it. His hemoglobin got down to 9.4 but is now back up 10.1.

2) He has one heck of a wart on his foot from the long term cyclosporine (it came back immediately upon restarting full strength cyclosporine). It is huge. I tried the salylic acid for 6 months and have now moved on to Vicks VapoRub, which is helping. It starts to die but then he takes a bath and the wart regenerates amazingly huge with water. Really, it's like a gremlin, I wish I could post a picture. We have an appointment with a dermatologist on Thurs but most treatments are contraindicated on immunosuppresive drugs. Does anyone have any advice?

Thanks! I hope you're all doing well!

[Cheryl C]

While on antibiotics my WCC will rise but this will be followed by a drop once the antibiotics are finished. I think this is typical.

I'm so sorry your little boy is suffering infections. It's hard enough for an adult but must be very difficult for kids. My thoughts and prayers are with you.

[Hopeful]

Hello! It's been a few months since I updated and wanted to again.

1) My son got strep twice over New Year's. Really it was one time but Amoxicillin didn't cure it so we had to get a second dose of antibiotics of Augmentin. His numbers always take a big jump on Augmentin. The excitement was that WBC went from 3.5 (bad), to 10.1 (also bad, for different reasons), to 5.5 (good). Also, on Augmentin his platelets went to 119k - a new high after falling to 35k after trying to taper the cyclosporine. At the same time I feel that: 1) Between ear infections and strep his only big jumps have been after Augmentin, and 2) the entire purpose of my original post - where he had 8k platelets, then made 70k on his own, then fell to 6k, was when he was on Erythromycin after he started wheezing - makes me think he needs a long term antibiotic. Has anyone else's numbers improved on an antibiotic? His numbers stay the same on cyclosporine but don't "go" up. I'm starting to get thankful for when he gets an ear infection because I can expect the jump in his numbers. I wish someone would just try putting him on 3 weeks of an antibiotic just to see what would happen but I can't get his hematologist or pediatrician to try it. His hemoglobin got down to 9.4 but is now back up 10.1.

2) He has one heck of a wart on his foot from the long term cyclosporine (it came back immediately upon restarting full strength cyclosporine). It is huge. I tried the salylic acid for 6 months and have now moved on to Vicks VapoRub, which is helping. It starts to die but then he takes a bath and the wart regenerates amazingly huge with water. Really, it's like a gremlin, I wish I could post a picture. We have an appointment with a dermatologist on Thurs but most treatments are contraindicated on immunosuppresive drugs. Does anyone have any advice?

Thanks! I hope you're all doing well!

Hi curlygirl,

I would definitely get the growth on his foot checked out right away. It could be that his body is trying to fight an infection, but because of the cyclosporine, it is not able to ward it off. However, maybe the antibiotics are helping his immune system fight the foot infection, but they are not given for long enough??? Just thinking out loud here...

I know that when my daughter had a skin infection, the dermatologist prescribed a month (!) of antibiotics to get rid of it, and it did take that long! So perhaps that is all your son needs to help his foot heal and his counts to recover??? It would be worth consulting with a dermatologist.

As another data point, when I've been on antibiotics for an infection, I never saw a rise in my counts.

[curlygirl]

Thank you both very much! It was indeed a wart, just a very large one. We ended up trying to freeze it off. It doesn't look infected now but he does have a large blood blister where it was. I've been putting neosporin on it. He had another much smaller wart his finger and we froze that one off too. The finger isn't looking near as bad so at least we caught it early. It turns out that warts are a hazard of cyclosporine, who knew? I will keep a watch on it and take him back in if it looks infected or if it starts to hurt. Thanks, again!