new member questions

[kellym]

hello, I'm new to this site, I have a 5 year old daughter only just diagnosed with aa in dec 2010, the doctor took a blood sample from my eldest child to see if she would be a match for bone marrow but it turns out she is not, so they are talking about atg treatment, I've looked all over the internet and seem to be going in circles.
my question is is atg a cure ?
does it include a long hospital stay?
I'm not seeing the doctor again until next week where I'm sure they will explain more
sorry if these questions have been asked elsewhere
thanks

[triumphe64]

This site can also be of great help.

http://www.aamds.org/aplastic/

[Ryan Jay]

Hi there,

I guess I can answer some of your questions.

The hospital stay for ATG can range from a week to two weeks or more depending on whether you have any side effects. I was in on a Monday and out the next Sunday. Not too sick, it just konked me out for the week. I felt WAY worse the next few weeks and then I came out of it.

As far as a cure...what is a cure? This is something that doctors talk about a lot. If you define cure as her counts going back to a normal/near normal range and her being able to live a normal life...then yes, ATG can be very effective.

No matter how successful your treatment, this is a disease that you will need to keep an eye on for the rest of your life. For me, it has become part of the routine of life. Wake up, go grocery shopping, take the trash out, go to the gym, check my cyclosporine trough at the hemotologist's office, walk the dog.

I would suggest you keep in contact with other patients though. This can be a very lonely world at times and there are some things that only other AA patients can help you with.

Ryan

[Lisa V]

The standard answer to the first question is that ATG is not a cure, only a BMT is a cure. That is pretty subjective, though. What they mean is that with ATG there's always the possibility of a relapse. That is true, but some people do not relapse, so for them, it might as well be a "cure". We've also heard from people who have relapsed after a transplant, so to my mind the distinction is not nearly as clear as the standard answer would suggest.

The bottom line is that ATG is a much less complicated procedure than a BMT, and the hospital stay and recovery time should be much shorter. Different medical providers seem to differ on how long they like to keep you in. My husband had no serum sickness issues, and he was in and out in less than a week the first time. On the other hand, I've heard of patients in other areas being held for several weeks just for observation, whether or not they had any adverse effects. I'd ask your doctor what the policy is at your hospital, Kelly.

Meanwhile, you might find it helpful to do a forum search for "ATG". You will find several descriptions of what the process entails and what to expect. Some people tolerate it better than others.

[kellym]

thankyou for the replys, it's great to know that people are managing this condition, I'm so glad to have found this site as it all seems very frightening at the moment

[Ryan Jay]

We've all been there. For me, the WORST was the first week after the diagnosis. I was in some dark places at times.

Once things switched from, "Oh my God, I have this horrible illness" over to fight mode, things got much better.

My advice to you is to go to the AAMDS website and download some of the webinars from Dr. Neil Young, Dr. Ron Paquette...and Dr. Margolis. They are very informative.

This can be beaten!

Ryan

[Neil Cuadra]

All patient families should get the free information packet (http://www.aamds.org/aplastic/information_packet/) from the AA&MDSIF.

[kellym]

thankyou for the info, we have to go for more tests next week and hopefully I will know where we go from there
we have had nearly 6wks of testing and it's the waiting and not knowing what's happening that's hard, from what I've read I will have to get used to that!
it's good to know other people are doing well

[Lisa V]

Yes, the waiting and the second-guessing is hard on everyone, especially at first. After a while it gets a little easier, especially when the counts start to creep up instead of down, but the idea that this is something you can be done with quickly and move on and forget about is something you'll probably have to let go of. This is your new reality, for better or for worse. That's not necessarily all a bad thing, even though it probably feels like the bottom has dropped out of your world. You can use that to connect with others who are going through the same thing, and to educate yourself and become an active participant in your daughter's treatment. Those are the things that helped me to cope during the early days, and I still value the sense of community I've found on forums like this one.

[kellym]

I am slowly get used to it, it amazing the things you get used to over time, my daughter is coping very well if you didn't know you'd think nothing was wrong with her, apart from pesky cough that won't go away!

[squirrellypoo]

I am slowly get used to it, it amazing the things you get used to over time, my daughter is coping very well if you didn't know you'd think nothing was wrong with her, apart from pesky cough that won't go away!Honestly, as someone who had AA and ATG as a 5 year old, let me tell you, it was way worse on my parents than it was on me! I didn't really understand what was going on, but things were new and I kept getting presents and lots of yummy applesauce (my mom figured out she could crush my pills into it and I wouldn't care!). Frankly, my only last effects from it was a superpower for needles!

I see you're in the UK - it might be an idea to see if your haematologist can send her BMB results down to Prof Marsh at Kings College Hospital in London for a second set of eyes on it. She's the UK's leading specialist and researcher in AA and she is WONDERFUL. She's also very interested in childhood AA and the longevity of ATG remission in patients, too.

[kellym]

she is enjoying all the presents and fuss too! ill look up that professor, that hospital is not far from me.
We saw our consultant yesterday and they are planning atg treatment in about four weeks, so im pleased something is getting started at last.

[Stacerph]

Kelly--

I'm sorry to hear about Ella's diagnosis. You didn't mention whether she has severe, moderate, or mild aplastic anemia; the difference is determined by counts at diagnosis. As someone mentioned, ATG can bring remission, sometimes for a long time. It isn't considered a cure.

The best ATG regimen according to Dr. Margolis at this time is the high dose 4 day treatment, rather than a lower dose 10-day regimen. Dr. Margolis at Children's Hospital of WI is VERY accessible, and he will talk with you, even if you are not his patient. I would recommend giving him a call. He is really amazing, and he will answer your questions.

Good luck. Feel free to email me if you would like to talk about ATG or BMT for AA.

Staci

[kellym]

hello, thanks for the reply, my doctor hasnt said if she has severe or moderate, shes only just been diagnosed and they have done another bone marrow aspirate this week to see if she has worsened.
Im guessing she has severe, she has platelets now 3/4 times every nine days and two blood tranfusions since dec 19.
In heself she is well and has lots of energy and is not tired, maybe as she is young its helping her cope better, i can hear her running about upstairs as i type lol
is the doctor you mentioned in the uk?

[kellym]

i forgot to add that her bone marrow cellularity is 20% is this severe?

[Hopeful]

Hi Kelly,

I would question your doctor as to why she wants to wait 4 weeks. There is a lot of evidence out there that prompt treatment (<21 or 27? days from diagnosis) leads to a more favorable prognosis. Perhaps this is just another reason to expediate the consult with another doctor!

Time is of the essence when dealing with a disease like aplastic anemia! I had about 2.5 months between diagnosis and treatment and things went downhill pretty fast towards the end of the wait.

Here is the criteria for severe aplastic anemia:

Any 2 of these in a hypocellular marrow (hers is):
* Absolute neutrophil count less than 500
* Platelets less than 20k
* Absolute reticulocyte count less than 60,000 /uL

I wish you and Ella the best! Continue to be strong and advocate for your daughter!

[Jen B]

Hi Kelly,
My son was 3-1/2 when he was diagnosed in December of 2008. His big brother wasn't a match for him either. We went through horse ATG and he had a partial response - enough to keep him out of the hospital for long chunks of time. Some patients go for a second round of rabbit ATG if the horse doesn't help, but that is being discussed whether or not the second round or straight to transplant is a better option.

This disease requires so much waiting and patience - of which I have very little. Hope for remission with the ATG, but put her name on the list to look for a match in case transplant is the way you have to go. You don't want to have them start looking when you are in need at that moment.

Feel free to e-mail me if you want to chat.

[kellym]

hello, thankyou for the messages, you are all very kind. We are still waiting to find out when our treatment is going to start, im seeing the consultant next week and he will tell us, i just want to start getting on with it now, i think he has left us waiting as he wanted to see if she had a virus and if her counts would improve by themselves before starting treatment, they have stayed the same though neither any worse or better.
Is it usual for neutrophils to go up and down? as hers is now at 0.4 where a few weeks ago it was 0.1
i wish you all good health!

[kellym]

We now know it's severe aa after last weeks tests and are going to be admitted to hospital in two weeks to begin ATg treatment, so fingers crossed it works, I'm am worried about the serum sickness I've been reading about though, but pleased treatment will start at last, it's been a long two months!

[Hopeful]

That is good news that they are moving forward with the treatment.

Try not to get too worked up about the serum sickness. Trust me, it will be harder for you than for her! Just remember that for every horror story that you read, there is another person that will have breezed through it.

Try to focus your nervous energy through the hospitalization on things that you have more control over. For example, ensure that all nurses/doctors/visitors are keeping things as sterile as possible. Her neutrophils will go way down before they go back up and the risk of infection is serious. Be vigilant that proper protocol is being followed for sterilization of her PICC line before things are injected into it. Also double check all drugs administered and their dosage.

I wish you both the best!

[kellym]

hello hopefull, thankyou for the advice i will make sure everyone is keeping things sterile, she is being treated in a cancer hospital and it is the cleanest hospital ive seen here (im in the uk) and has amazing childrens wards which im pleased about as our local hospital where we have transfusions is not the nicest place and i wouldnt want to spend a lot of time there!

ill try not to worry about the sickness i have read some horror stories though, but you cant help but read them, i find it helps to know all i can about this illness...still cant quite belive its happened.

all the best :)

[Jen B]

Ethan got through horse ATG pretty well, just a few headaches and after the fact, some transient rashes. Everyone is different and she may have a breeze of it! Keep positive...

[Lisa V]

Just about everyone I've heard from has had some initial flu-like symptoms (chills, fever, aches) during the first few hours of infusion, but that is not serum sickness, so don't be alarmed by that. It will pass quickly, and with any luck it should be smooth sailing after that.

[kellym]

Thankyou for the replys, we only have a few more days until we go in, I was feeling really positive, but now feel terrified by it all, I just pray ellas not going to too unwell with it all we have managed to keep her well the last few months. Hope you are all keeping well

[Ryan Jay]

Try not to bite off more than you can chew. Focus on day to day successes and not, "Oh my God, what if?" stuff.

Every day I would write about something that went well in a log book.. That seemed to work well.

You are going to be in really good hands through this process. ATG is usually administered by the cream of the crop.

Anything that you need help with, you can always come here and ask.

Ryan

[kellym]

That's good advice, my mum said that too, I tend to worry about things that havnt even happened!
I've been keeping a diary the last few months it helps me to remember everything and keep notes of her counts and how she feels.
It really helps to talk to people that are going through the same thong and have the same worries as others don't really understand, I've learnt a lot from this site.

[Neil Cuadra]

That's good advice, my mum said that too, I tend to worry about things that havnt even happened!
I've been keeping a diary the last few months it helps me to remember everything and keep notes of her counts and how she feels.
It really helps to talk to people that are going through the same thong and have the same worries as others don't really understand, I've learnt a lot from this site.
We were told that it helps to put a calendar on the bulletin board (or the refrigerator) and note which days are better than the previous day and which are worse than the previous day. You can draw up and down arrows, draw :) and :( faces, or use stickers. It can help everyone remember that there are good and bad days but more and more happy faces as you make progress over time.

[Karenish]

I have just found this site having been diagnosed with SAA in the first week of 2011! great start to the new year! not!! I am nearly a young 51 year old female and of course hit the internet with gusto and scared myself nearly to death! But reading all of Ryans input has made me feel human, yes I went on a huge emotional roller coaster, how can this happen to me, I have never knowingly hurt anyone...etc etc. and as a health care professional (occupational therapist) thought I was immune due to all the injections we have to have.
I am naturally a very positive person but having now lost control over my health I could only see the "dark" side. But Ryan you have at least put a more positive bend on the whole thing. So ATG is not a cure....but it can give me a relatively normal life back, with a strong emphasis on infection control and hand hygiene, something I receive annual training on. I wont be able to eat shell fish anymore, but heck thats a small price to pay and all the other things!
So really just wanted to say a big thank you to Ryan, wish I had found him sooner lol xxx Karen:)

[Gloria J]

Hi Kelly,

It seems about the time your Ella would be starting ATG. I'm hoping it is going well with no complications. As treatments go, it can be one of the easiest. There are always meds they can give you to counteract the bad stuff.

Keep us posted on Ella, and stay strong for her, and you! It's a lot to deal with, but you can always find someone here who has probably had the same experience. I know that helped me greatly, just to read that I wasn't the only one going through it.

Best wishes,

Gloria

[kellym]

Hello, thanks again for the lovely messages
Ella started her ATg this morning, she had a test go for a hour and didn't get any reactions, so they started her on her full bag of it this afternoon, they have given her all sorts of meds before hand and I cant recall them all, the ones I can are puriton and antibiotics and some fungal meds. She seemed to be doing ok until a few hours ago she started to get a tempreture, the highest it's gone is 91 they've given her paracetomol and some pethidine but it doesn't seem to be working so they are going to try another med soon, aside from the temp she's been ok blood pressure fine and even has been up walking around which suprised the nurses!
I'm glad the ATg has started but I think it's going to be a long night as I'm worried about her temp.

[ljensen3]

Hello Kelly,
I hope all is going well with Ella. I do have a question for you, was Ella ever diagnosed with MAA? My 7 yo son was just diagnosed MAA and I am not sure what the progression from MAA to SAA is. Any information that you could share with me would be greatly appreciated. I will keep Ella in my thoughts :)
I can be PM'd or my email is on here also.

Thank You,

[Neil Cuadra]

Leanne,

Aplastic anemia is classified by blood counts and bone marrow cellularity. The distinction between MAA and SAA is based on cutoff values, not symptoms, so they aren't distinct diseases, just measurements along a scale from less serious to more serious.

They call it severe aplastic anemia when you have bone marrow cellularity of less than 25% and at least two of the following three count ranges:
absolute neutrophil count < 500/µL
absolute reticulocyte count < 20,000/µL
platelet count <20,000/µL

Do you know the numbers for your son?

[ljensen3]

Hi Neil,
Thank you for your quick response. At his last BMB in Sept. 2010 his cellularity was 40-50%. I'm sorry but I am new to this and I am not quite sure how to do the conversions but his lab report from 4/13/11 said his absolute neutrophil count was 3.18. His absolute retic count was 16.5 and his platelet count was 234. All labs seem to have their own reference ranges and he has been going to 2 different ones, one where we live and one at the specialist at Devos. Do you know how much the WBC and RBC count? And if the MCV has anything to do with it? His MCV has been high since 10/08, when this was first discovered it was at 90.4 and the last time it was between 94.9 at the BMB and 97 5/2/11.

[Neil Cuadra]

Leanne,

You can check the units on the lab report, but I think that they are reporting thousands of cells per microliter (K/μL) rather than in cells per microliter (/μL) as I used. If so, then your son's numbers are
absolute neutrophil count = 3,180/μL
absolute reticulocyte count = 16,500/μL
platelet count = 234,000/µL

Not the SAA range.

WBC and RBC and platelets (cells in circulating blood) are important in determining if he needs transfusions or what side effects of low counts he's at risk for. The ANC indicates the level of neutropenia (risk of infection). 3,180 is in the normal range (no neutropenia). The ARC is a measure of immature (baby) red cells and provides clues to the production (or lack of production) in the bone marrow.

MCV is mean cell volume, meaning a measure of red blood cell size. Those MCV numbers don't seem high to me. Are they really higher than the reference ranges shown on the lab reports? If he does have elevated MCV it can be a sign of folate deficiency or vitamin B12 deficiency.

I hope you will ask the pediatric hematologist to explain your son's lab test results. Maybe the correct interpretation depends on your son's age so one formula doesn't fit everyone. Even though other forum members and I are laypersons, talking to us may help you learn some of the doctor lingo and decide on the best questions to ask at your son's next appointment.

[ljensen3]

Hi Neil,
Thank you for the help with the counts. That is a relief. I know my story isn't quite as severe as others on here, but it is still a scary thing for us. Do you happen to know how fast it can get to severe? Is there other things we should be keeping an eye out for between his labs? Do you know the counts are for SAA in children? We would like to stay on top of things as much as possible. We are just thankful that we found out before it got to SAA. I know that they have checked for other things like liver, spleen, thyroid, MONO. I'm not sure if there were others but, they were all fine.
According to the ref ranges (75-87) for the MCV for his age they said it was high. That is why they are having a hard time figuring things out, his B12 and folate were fine and while his iron was low they were still high, which I was told that they should be smaller than normal. We will be going to U of M in 2 weeks so I will be sure to ask more questions. If you have any other suggestions on what we should ask we would appreciate it. Sorry to for bombarding you with all these questions.

[Neil Cuadra]

Leanne,

Since MAA and SAA are classified by lab test results on a scale, theoretically MAA could become SAA between one lab test and the next. But Andrew may never progress at all. I haven't seen a separate formula for diagnosing pediatric SAA; a lot of AA patients are children. But the classification is less important than the treatment (if any) that's required, so asking for recommendations about what to do (watch and wait? treat?) is something to ask the doctor. If you watch the test results over time you'll want to observe any trends (a count that keeps rising or falling over time) because that's the sign that something is changing (better or worse). That's what the doctors watch for too.

Your son's cellularity is about twice the SAA range but lower than a typical youngster. I'd ask the doctors what that tells them.

The main evidence I think you should watch for is any new or changed symptoms your son experiences. It might be helpful to record anything you notice on a calendar so you can tell the doctor how long a given symptom has been apparent. If there are no symptoms to report, that's fine! But doctors would rather have specific facts than vague reports.

If you didn't already get Your Guide to Understanding Aplastic Anemia from the AA&MDSIF, you should definitely ask for it here (https://www.aamds.org/aplastic/disease_information/educational_material/).

[JodyW]

Neil,
My son was diagnosed with Very Severe AA and I only see others reference SAA. Is it that VSAA is so rare you dont see it, or is it just referred to as SAA. His ANC was .01 and cellularity 4%.

[Ryan Jay]

Hi Jody,

Well, let me chime in on this one...and offer my non-expert opinion. MAA, SAA, VSAA were created a while back to determine how long an AA patient could be expected to live. Our prognosis back 40 years ago was very grim.

I think doctors have pulled back from these definitions a bit recently, and they even gloss over them at patient conferences. It would seem that the neutrophil count is more important in determining how quickly doctors need to move on your case.

If you are in the moderate category, the doctors may put you in a holding pattern, whereas more severe manifestations of the disease either require ATG or a BMT.

My hemotologist even told me that I could have either SAA to MAA depending on how much you value platelets and red blood cells. My whites and ANCs hung in there at about .800, but my platelets and rbcs got hacked to pieces. So, how do you define that?

I guess my advice is for you not to get too wrapped up in definitions that may not be clinically significant.

The big deal is response to ATG. When you go to that conference in October, try to sit next to Dr. Scheinberg at lunch. He is really friendly and knows soooo much about this illness. You'll love his presentation too.

Hope this helps.

[Neil Cuadra]

Jody,

Ryan Jay is right: getting the right treatment is what matters.

Doctors group patients into diagnostic categories so they can analyze statistics over a large number of patients. They do, however, use these categories to study how an individual patient can benefit from what is known about research on groups of other patients, such as what approaches work best for patients with milder or more severe AA.

If you want the formal definitions of MAA, SAA, and VSAA, see our Types of Aplastic Anemia page.

[JodyW]

Thanks appreciate the reply and will take your approach to this vs the alternative.