New to AA

[Donna]

Hi everyone,
I am Donna, mother of A.Jae. She is 16 and we just found out that she has Aplastic Anemia. She is in the moderate stage. We have tested siblings to see if they are a match. Won't know until next week. The doctor wants to monitor her on a monthly basis. Is there anyone else that is going down this same road as we are. I have not found anyone yet with a female, at her age. I wonder how long can she live like this, in the moderate stage. What does life have to offer her down the road? She is a very special child. She is my hero! Never complains. Except when she does not feel like riding her horses, but really wants too. She pushes herself to ride. My heart hurts for her. I know I must remain positive. I would love to hear from someone who could advise, or could tell me what they would do differently.

God is good:
Donna, mother of A.Jae

[Marlene]

Hi Donna,

Welcome and sorry that you need to be here. As you already know, they don't usually treat moderate AA. There are only a few options out there for SAA. Bone Marrow Transplant and immunosuppressant therapies. I would recommend you research all of them before making any decisions. Any path you choose, be sure to work with a doc/health care team familar with AA. I would recommend you speak with at least, Dr. Neil Young at NIH regarding ATG therapy and then also, Dr. Brodsky regarding High Dose Cytoxan. These are your options if you don't have a matched donor sibling for a BMT. There's a couple of other docs that parents like really like. Ones in wisconsin and the other at the Cleveland Clinic. Can't remember their names though. I'm sure other will know.

AA strikes children as young as two month old to older adults. There's another site you can post to find other mothers/fathers whos children have AA. Its www.aplasticcentral.com Just click on the forum at the top to get to it.

Again, now is the time to research all your options and varous hospital so that you can make an informed decision if needed.

If at any point A.jae needs blood products, be sure they are irradiated, leukocyte reduced/filtered.

You can live a very long time with moderate counts so that's good. You'll be amazed at what the body can handle. So what are her counts now and are they stable?

Marlene

[Andrea Pecor]

Hi Donna,

A.Jae sounds like an amazing person - I'm so sorry she has been diagnosed with aplastic anemia. I am also a horse lover though I haven't ridden for years. I was diagnosed with severe aplastic anemia when I was 24 years old - that was 27 years ago. Moderate AA can be a chronic condition for years, and A.Jae could lead a completely normal life as long as her counts are somewhere near the normal range. It is wise, though, to monitor her counts regularly so you can be on top of it if something changes.

I agree completely with Marlene that now is the time to do your homework - it will serve you well in the future if things change with A.Jae. Finding out if any of her siblings are a match for a potential bone marrow transplant is important, and could prove to be her "ace in the hole" if one of them is a match. I also agree with Marlene about being evaluated by Dr. Young at NIH. That institution is remarkable and Dr. Young's team and the work they do there is world class. If you decide to have A.Jae evaluated there, the AA&MDSIF will reimburse up to $500 of associated travel costs. (Let me know if you would like details about this) On the other hand, I would recommend you scrutinize the results of Dr. Brodsky's research very carefully before considering treatment with high dose cytoxan.

You'll find this site very helpful and supportive - just let us know how we can help.

best,
Andrea

[skoopman]

Hi Donna,

The two other dr's that Marlene was referring to are Dr. David Margolis at the Children's Hospital of Wisconsin in Milwaukee (that's who my daughter was treated by) and Dr. Macejewski at Cleveland Clinic (he treats adults but consults on pediatric cases, I know a mom who uses him for her daughter). The girl who sees Dr. Macejewski was diagnosed as MAA but she did have ATG treatment. Not many people do it, and I know she did a lot of research to figure out if they should treat her or wait for it to get worse.

I also agree with Marlene in that you need to find a dr who has experience with AA, preferrably in kids. Research all your options and figure out what is best for you.

What state do you live in and where is she being treated?

Suzanne

[Sherri]

Hi Donna-

I am the other Mom Suzanne is speaking of. My daughter was much younger than yours when she was diagnosed. She was 5, but is now 7. You will get thru these initial feelings- it isn't easy- but you and your daughter will become so much stronger as a result.
My first question is - what are your daughter's counts and has she had a Bone Marrow Biopsy? What determined the diagnosis of MAA? When Annalyse was first diagnosed she had low normal RBC numbers (they just hadn't had a chance to drop yet) an ANC of 600 and a Platelet count of 31. (31000). She was borderline SAA but her marrow cellularity was 80%- so they didn't even diag Aplastic Anemia at first. Then her marrow went to 40% at her 2nd BMB 2 months later. That led to the MAA diag . However- she also had PNH , which will show a higher % of cellularity due to the PNH cells. So she was probably SAA or very close.
My dgtr sees Dr. Maciejewski at the Cleveland Clinic. Prior to ATG we discussed doing Dacluzimab (Xenapax) but decided on ATG. Dr. M told me last week that they did the Xenapax on a small MAA child recently - normal counts within a couple months. This is one thing for you to look at as far as treatment. This may be comforting- I remember Dr. M. saying that with MAA you don't "get on the mortality boat". You live with MAA- it's not life-threateni ng. You will find you are soon back to "normal" - it's just a different normal.

[jancye]

Hi Donna

Its early days for A.Jae.... Read everything you can as knowledge is power. Colleen was diagnosed with AA at 18 and has just turned 22 and is doing really well after ATG. No meds for the last 18 months and her counts are back to normal. Her HB is 135, Platlets 200 with White count sitting on 3.9. It was very scary there for a while but we got through.

Colleen does not have a sibling match but we live as a family day by day and for now she is really well.

Know that people are thinking of you and your family.

Jancye (Australia)

[Donna]

Hi everyone,

Thank you all for the encouraging words. A.Jae's siblings were tested and they were not a match. We live in the state of Mississippi and are being treated at the University of Mississippi Medical Center. Dr. Bigalow is her doctor. I ask Dr. B if she has treated anyone with this disease before and she said that she had. Her patient was 19 years old. I am still a skeptic about treatments. Her hemat. score was a 29 and her white cell count was a actually up. I am writing down all of the counts at every visit. I do not have them with me right now. A.Jae has had a BMB. I will find out what the density is. The Doc told me when she informed us that she had MAA. I was shocked by the news and can't remember any of the numbers. Can someone give me a list of what I need to keep up with on our monthly visits? I would so much appreciate it. A.Jae started school this week and has been experiencing shortness of breath it seem more frequently. Thanks again for the kind words and the information. I would be interested in speaking with the doctors that you mentioned. Sincerely Donna, mother of A.Jae

[Lisa V]

Donna, writing down her counts is good, but they should be able to give you a print-out if you ask for it. Much easier, and you may find it helpful to keep a chart or spreadsheet to track A. Jae's progress. That will give you a feel for how quickly things are changing, if they are.

For AA, the counts we usually look at are Hgb (hemoglobin), Plt (platelets), WBC (white blood cells/count) and ANC (absolute neutrophil count). Hgb is about the only one you can really "feel", since it measures the oxygen carrying capacity of the blood. Normal range for Hgb is 12.5 - 18.0, but many AAers are able to adapt to lower levels and function okay with 9 or 10. It's an individual thing, but they usually will transfuse if it gets down to 8 or lower.

Normal platelets are 130 -440(k), but if you have at least 50k you are considered pretty "safe" from most bleeding issues (except in the case of severe trauma, which is not safe for anyone anyway). The usual tranfusion target is 10k, but some people find that they can let it go even lower without problems. Others have easy bruising and petichiae (fine red spots) at higher levels, so those are things you have to be on the lookout for.

Normal WBC is 4.8 - 10.8, but again, it is possible to adapt to much lower levels, as long as she's not having problems with infection and illness. The most important component of the WBC for AAers is the neutrophils, since they are the prime infection-fighters. Lymphocytes fight infection too, but they are also thought to be the culprits in AA, having launched an autoimmune attack. The usual immunosuppressant treatments for AA (ATG, cyclosporine) are designed to knock out certain of the T-lymphocytes and keep them from destroying the marrow's ability to manufacture stem cells. They don't target the neutrophils, so as long as there are sufficient numbers of them, the body should be able to handle most things. Different labs record ANC differently, and you may have to add the segmented neutrophils and the bands to arrive at the number. The normal range is 1.8 - 7.5, but again, many people can get by with less, you just have to monitor the incidence of infections. You don't want the ANC to get below 0.5. That is known as severe neutropenia, and may necessitate wearing a mask, avoiding crowds, eating only foods that can be cooked or peeled, etc.

When you say A. Jae's white counts was up, do you mean up from when they first measured it, or higher than normal? If it's the second, it doesn't sound like AA. Too many white cells are more associated with some forms of MDS and leukemia.

Hope this helps. One thing we've all learned is that while "normal" is the goal, "not normal" doesn't have to mean you can't have a normal life. You just have to monitor at what level problems start to occur. That's why most doctors don't recommend any treatment for MAA, since quality of life is not usually significantly affected at that level. I'm assuming A. Jae is not having to be transfused at this level. The main goal of ATG and other treatments is to become transfusion-independant, so it is usually not recommended until it becomes SAA or vSAA. I know it's hard to sit around and do nothing and wait, but there is a lot of that in AA, so it's best to come to terms with that.

[theresasc]

We have a 17 year old daughter. She was diagnosed February 8, 2006 with MAA. She received 4 blood transfusions over the next 45 days. During that period of time we researched supplements and started her on one particular supplement. Her blood level went from 7.8 to 9.0 over the next three months. We switched to another supplement that was more specific in nature and her blood level went from 9.0 to 10.9 in less than 3 months. All other blood counts increased with the hemoglobin. As of last week, her hemoglobin was 11.5. Last school year, she played Volleyball, did Cheerleading, and other normal activities. Besides the February to April 06, she has lived a normal lifestyle. She did not change her diet. The final solution was a liquid vitamin and flaxseed oil to deliver the one supplement that my research showed would effectively help correct the issue.

Yes God is Good!
Theresa

[Donna]

Hey everyone,

A.Jae is doing o.k. She still gets very tired and shortness of breathe. I read what Theresa wrote and was wondering, if your daughter was MAA, why did they give her transfusions. What kind of vitamin does she take and why the flaxseed oil? A.Jae has trouble sleeping at night time. Her body will not let her rest. Her doctor does not want to put her on any medication which is fine by me. She takes a Centrum vitamin and a calcium pill everyday. We do not go back to the doctor until Nov. I would like to know more about the vitamins.

Truly concerned,
Donna, mother of A.Jae

[theresasc]

Diamond V Mills-----Epi Cor

[theresasc]

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