Help need guidance

[Jx3]

At the age 60 yrs old, my father was diagnosed with AA in Early Dec 2011 and has received the ATG Treatment. As expected he has had to have several transfusions of blood and platelets with the longest time period lasting about a week in between transfusions. As we all know the doctors have told us to not really worry about the blood levels and the platelet levels but to keep an eye on his WBC.

About a month ago he went to the specialist at Vanderbilt for a monthly checkup. The doctor’s told him that he needed to have a higher level of WBC b/c they have been staying around .8-1.0, or he would have to have a Bone Marrow Transplant. Dad is scheduled to go next week to have another bone marrow aspiration completed to find out for sure on his levels. With in the past three weeks he has had a steady increase from 1.0 to 1.3 to 1.7 (last week).

Today when he went in to have his blood work checked. His WBC has fallen back to 1.3. When I called to check on him I could tell in his voice that he is devastated as expected and packing his hospital bag for another round of transfusion. It seems like my mother and sister are just hanging on by a thread before the have complete melt down and we can not get all of our questions answered. We also have read in all the forums that changes may take longer than 3 months but the doctors are pushing to get him on the transplant list.

I guess what I am getting at is what is the probability that this lower level is just a bump in the road or is the likelihood that his body did not react to the ATG and that a bone marrow transplant is in the future?

[Greg H]

Hey Jx3!

I'm an MDS patient, not an AA patient, and I had Campath instead of ATG, but I can tell you for sure that it is not a good idea to get all worked up about one blood test.

WBC levels bounce up and down a good bit from week to week, as the body responds to little infections that we don't even notice.

I have my CBC every other week. Here's the track of my WBC counts since November:
3.2
2.7
2.3
2.3
2.2
3.2
2.8
2.9
2.9
2.8
3.7
My counts are better than your Dad's, of course, but the point is: look at the variation -- up and down, up and down.

You and your Dad should not fret and wait to see what the next CBC says, and the one after that, and the one after that, before you start packing your bags.

Take care!

Greg

[Marlene]

When it come to the WBC, you need to look at the ANC (absolute neutrophil count) also known as absolute granulocyte count. The labs have a WBC differential which breaks down the make-up of the total WBC. They do percentages as well as an actual number. With the percentages, you want to see the neutrophil % to be higher than the lymph %. You also want to track the ANC # along with the WBC. The ANC is needed to fight infections.

Like Greg has stated, counts will fluctuate all the time. Even though we want to see a steady climb, most find that one day they're up and the next they dropped back down. So what you need to do is follow the trend of the count. As long they trend upwards overtime, that's good.

The way I look at it is if he got to 1.7 once, he'll get there again. What is his ANC count?

[Jx3]

Our local hospital lab does not show the results of the ANC.

I appreciate your answers. I think that is alot of our problem with our nerves and emotions is that we have alot of questions that are not getting answered, leaving alot for us to worry and wonder.

[Neil Cuadra]

Originally Posted by Jx3

Our local hospital lab does not show the results of the ANC.

If you have your father's blood count lab report then you can compute your ANC from his white blood cell count and other numbers in the report, as long as the blood tests they did include the "differential" test that Marlene refered to. That means that they counted the various types of white cells, not just the total white cells. They tell you how many neutrophils, how many lymphocytes, etc., or tell you the percentages of each of these types of cells.

You can use our ANC Calculator to help you. Or, if you don't mind sharing your father's lab results, post them here and we'll help you figure out the ANC. Look for the WBC and any lines that say polymorphonucleocytes, neutrophils, eosinophils, basophils, lymphocytes, or monocytes. Sometimes they use abbreviations like POLY, PMN, NEUT, SEGS, BANDS, EOS, BASO, LYMPH, or MONO instead of the long names.

[Hopeful]

I think that you need to look at all the blood levels, not just the WBC. An increasing WBC is sometimes the first sign of a response to ATG. You should look at his platelets and HGB as well because if they are rising (or the need for transfusions is decreasing), that is another sign of response.

Your father is very early in his recovery, as he only had ATG in December. I would be very skeptical if his doctor was pushing a BMT, especially since he is older! It sounds like his specialist is not following standard protocol. Do you know how many other AA patients he has seen/treated?

Is your father still taking cyclosporine?

I would seek out another AA specialist now for a second opinion.

[Jx3]

I was able to figure up his ANC and it is not good. Which is a very disheartening result. It appears that he is in the severe level of ANC.

With that in mind can the weekly blood work show a completely different result than the Bone Marrow Aspiration show?

Thank you for your responses!

[Greg H]

Jx3,

The pathologist will do a differential (the breakdown of bloods cells by type) on the marrow aspirate, but what you're really looking for in the BMB results is the answer to why your Dad's counts are low.

So, the first question will be whether the marrow has the expected number of cells in it or whether it has more than expected (hypercellular) or fewer than expected (hypo cellular). Most MDS folks are hypercellular; aplastic anemia is hypocellular; but some folks with MDS are hypocellular, too.

Next stop is blasts. Blasts are immature white cells. In normal marrow they make up 1-5% of the cells. More than that, and you have excess blasts, which is not a good sign. More than 20 or 30% blasts, and it starts to look like leukemia.

Next stop is dysplasia: a fancy word for messed up cells. The pathologist will look at all three blood lines (lineages): reds, whites, and platelets. Cells go through a multistage process in the marrow, from the original stem cells, through a variety of "progenitor" cell types, and then onto the final product, namely, the blood cells that circulate in the body. The pathologist will be looking for messed up cells anywhere in this process. Your Dad might have dysplasia in the white cell line only, or in whites and platelets, or in reds only, or in all three.

If he has dysplasia, then he'll mostly likely be diagnosed with MDS. How many lines are messed up has some significance for treatment decisions, so its something that you and your doctor will pay attention to.

Next stop is cytogenetics. This has two parts: karotype and FISH.

Karotype involves actually growing out a few bone marrow cells until they are ready to divide, and then killing them in order to spread out all the chromosomes and look at them. This is where you find out if your Dad has any chromosomal abnormalities. Whether he has them, and which ones, can affect both prognosis and the recommended course of treatment.

FISH is a fancy way to for specific chromosomal abnormalities that are known to be common in bone marrow failure diseases. Not all BMBs involve FISH.

So that's what you can expect in terms of data.

Give a shout if that raises any other questions.

Take care!

Greg

[Jx3]

This pass week dad had his follow up BMA and the results of it shows that he has not improved at all since the AGT treatment. So it looks like we will be starting the process of finding him a donor. Right now we are waiting to see if his brother is match or not, then on to the international donor list.

[Neil Cuadra]

Jx3,

I'm sorry that your dad's condition hasn't improved yet. But he has multiple paths to success at this point and still has a number of factors working in his favor.

First of all, if his status is unchanging, even with low blood counts, then he's better off than if his counts were dropping. Second, it's been only a few months since December and ATG can take longer to be effective. ATG often makes things worse before making them better, and it's hard to be patient month after month, but your dad could start to see benefits soon. Third, he's eligible (not too old) for a transplant. Fourth, he has a brother as a potential sibling match. Fifth, a search of the bone marrow registry may identify other potential donors. And last but not least, you are looking out for him. It's hard to overstate the importance of the support you are giving your dad.

Looking for a donor is a reasonable course of action and it doesn't mean that you are giving up on ATG, just that you're preparing a backup plan.

I'm sure it's very disappointing that your dad hasn't shown improvement already, and it's still a serious disease with risks that can't be ignored, but as your patience is tested I hope that you and your family can remain optimistic, see the positive possibilities, and keep your chins up. Good luck.

[Karenish]

JX3 - please don't think the ATG has failed yet. I had mine in Feb last year...we waited and waited...nothing, then all of a sudden 10 months later the counts started to climb. I am no way out of the woods yet, but hb is about 8.5 plates 30 whites nearly 3 and neuts nearly 2 Keeping well and last transfusion Dec 22nd 2012..... Hold on in there, Your doc may want to do another bone marrow aspiration after about 6 months to see if there is any change, mine did and said he could see a very slight improvement which is why we waited for a while...looks like it paid off.....I am well aware that my recovery can now take years. who knows how long my marrow was taking a hammering from the rogue T cells so those wee blood making stem cells have to re boot.
xxxxxx

[Faithx4]

Hello-
I am Jx3's sister. I have been a frequent visitor of this site since Dec, but this is my first time to post. I appreciate each one of you that took the time to respond to my brother's post! The information and/or feedback gained from this forum is more valuable to me than any medical article published.
Our family is strong in our faith and trust that God is in control. However-as we begin this next phase we are struggling with the risk's associated with the road ahead and the unknown. The doctor told my parents that time is of the essence--his new sense or urgency is concerning. The weekly transfusions don't appear to be giving dad the "boost" they once provided.
I would love to hear from others who have successfully conqured BMT--especially those around the same age as my dad. Also, dad's only sibling is 68 years old--if he is a match will age be a factor in the success of the transplant? What happens if my uncle isn't a match and a match is not identified on the donor registry? I am scared....I worry that dad's age, my uncle's age and "time" is not on our side.

[Hopeful]

It is always wise to start a donor search early. However, it would be unusual to proceed to a BMT so quickly after ATG - especially in an older patient. Do your father's doctors suspect that his AA was caused by environmental factors or secondary to another illness/treatment? Are you seeing someone who is an expert in AA? Has the doctor discussed the possibility of using growth factors to try and raise his WBCs while waiting for the marrow to heal?

Just as a side note...I think it took a year after ATG for my BMB to show signs of recovery. My blood counts started to improve before it showed up in my marrow.

[Faithx4]

Hello Hopeful and thanks for responding.

The past 7 months have been a whirlwind on one hand, yet on the other it feels like time is standing still and someone has their finger on the rewind button.
Dad was fine one day and in critical condition the next. In September dad had labs drawn for a routine physical . That same day the doctor called to inform him that he needed to be admitted to the hospital immediately due to critically low levels. Looking back he was experiencing some of the warning signs--bruising, tired and shortness of breathe. Dad has been an outdoorsman all his life. We blamed the above symptoms on the hot summer days and his passion for working in his garden after a full days work.
Below is a copy of some of the journal entries on dad's CaringBridge site. I share this so you have a better understanding of how quickly, in comparion to others, this whirlwind has been.

--------On September 9th 2011, Bob had blood drawn for a routine yearly physical. We were quickly notified by our family physician that his blood levels were low, particularly his platelet count, hemoglobin and white blood cells. The original concern was a reaction to a prescribed medication, but other medical conditions were also mentioned.

Throughout the next weeks his levels continued low and even worsened despite treatment. His initial bone marrow test did not reveal anything or provide a diagnosis. We were referred to Nashville to a specialist who wanted a 2nd bone marrow test. It also did not reveal anything. We were told to be patient and wait to see if an underlying disease process was emerging. He was told that he might have aplastic anemia or MDS ( pre-leukemia). He has received several platelet and blood transfusions while he "waited."

His blood levels continued to drop. His overall condition seem to deteriorate over the weeks. On Dec. 8th, he had his 3rd bone marrow test and was told it would be a week before he would hear from thee results. Once again the waiting continued.

His health progressively worsened. Over the last 2 weeks he had a transfusion reaction to platelets, experienced confusion and short term memory loss and they feared theat he might of had a stroke. The need for supportive transfusions treatment increased in frequency. Even after receiving blood and platelets he developed an uncontrollable nose bleed,which is a potentially severe complication of low blood platelets. We notified the Drs in Nashville and by the Grace of God all of the right people answered the phone and were attentive of his need for urgent care. Within the hour, he was notified by the Drs in Nashviille that his bone marrow showed aplastic anema, and for him to come to Nashville that night. The Drs were immediately aggressive to start his care. Praise God!

Today is Saturday the 10th, Bob has received 2 units of blood and will get a PIC line for better IV access. He will start his Chemo on Monday,12th.

To us he has always been special. But now we know that he is truly one in a million. Aplastic anemia is a rare blood disease that affects 1-3 out of one million people .

Jump a couple months to February 2012
Journal Title:Crunch Time

Bob went to see Dr last Thursday (2/9). He said that he wanted Bob to have a bone marrow biopsy in 4 weeks. He needs to have an increase in cellularity of the bone marrow and an increase his lab values. If not, the Dr said that he will have to pursue a bone marrow transplant. That scared us. Bob is showing a little improvement in white blood cell levels, but he needs a lot more. He has been getting platelets once a week and PRBCs about every 10-12 days. He does not receive white blood cells in the transfusions, his body has to make them. An increase on his blood tests would indicate that he is making blood cells and that his bone marrow is functioning.
So needless to say it is crunch time! We ask for your prayers. We know that God is in control and he is the Great Physician. We know that all who read this will sincerely pray for Bob. Please pray every day for the next 4 weeks.. We have Faith that God will answer prayers!
March 2012

[BJournal Title: ]The Road Ahead[/b]

Bob has to have a bone marrow transplant. His Dr called today and said that there is no improvement in his bone marrow and that he wants to get started as soon as possible. His brother will be tested to see if he is a possible donor. If not then they will go to the national donor registry. If his only brother is not a match, it may take 3-4 months to find a match. The important thing at this point is for Bob to stay well. We have accepted that this is the Lord's will and have Faith that he will be with us throughout. A transplant is the only cure for Aplastic Anemia. Although it will be a very long road, It is God's will that Bob will be cured, and we will give God all the praise and Glory. Please continue to pray for Bob and all of us as we embark on this journey.

That is dad's timeline with Aplast Anemia to date. He is being treated at Vanderbilt Medical Center in Nashville TN. Any feedback from other patients that received their BMT at Vanderbilt is welcomed. My family has been very pleased with the dad's medical team. They all appear to be very knowledgeable and have experience with Aplastic Anemia--but who am I to judge....7 months ago I had no idea this dreadful disease even exisited!

[Neil Cuadra]

Originally Posted by Faithx4

However-as we begin this next phase we are struggling with the risk's associated with the road ahead and the unknown. The doctor told my parents that time is of the essence--his new sense or urgency is concerning. The weekly transfusions don't appear to be giving dad the "boost" they once provided.

Hi Faithx4.

As patients and caregivers it's hard for us to second guess the advice of someone else's doctor. You might want to get your dad a second opinion from another doctor with aplastic anemia experience. That could give you a better understanding of your dad's condition and his doctor's advice, or possibility a differing medical opinion that would help focus your questions on the choices that make the most sense.

I would love to hear from others who have successfully conqured BMT--especially those around the same age as my dad. Also, dad's only sibling is 68 years old--if he is a match will age be a factor in the success of the transplant?

Doctors usually prefer (a) the best matched donor, to reduce the risk of rejection and (b) younger donors, whose marrow can best contribute to long-term survival (although this may not be as important for a 60-year-old patient as it would be for a 16-year-old patient). They also want to avoid any risk to the donor, and older donors may have their own health conditions. So yes, age is one of the factors, to be weighed against how good the match is. If it turns out that there's a choice among multiple donors then the doctors can rely on research and statistics to recommend which donor is best to use.

What happens if my uncle isn't a match and a match is not identified on the donor registry? I am scared....I worry that dad's age, my uncle's age and "time" is not on our side.

If there are absolutely no exactly matching donors, doctors may consider a partially matched donor and compare that to the other choices: continued "wait and watch" with supportive treatments (transfusisions and/or growth factors) or another round of ATG. If it's clear that he needs to move forward with a transplant now, then you and your brother should learn all you can about what that entails so you'll be best prepared to help him through it.

[Jx3]

On July 6 dad received his BMT. He is doing good and is showing signs that his body has accepted the transplant. Since his 21 day mark which was on July 27 his WBC is at .2. Granted that is a low number it is still some sign of hope. Just wanted to let the ones know who was gracious enough to give me advise when we needed it! Thanks again and please continue to pray that his progress continues and he can be considered cured of this disease!

[Neil Cuadra]

Originally Posted by Jx3

On July 6 dad received his BMT. He is doing good and is showing signs that his body has accepted the transplant. Since his 21 day mark which was on July 27 his WBC is at .2. Granted that is a low number it is still some sign of hope. Just wanted to let the ones know who was gracious enough to give me advise when we needed it! Thanks again and please continue to pray that his progress continues and he can be considered cured of this disease!

This is big news, Jx3. Thank you for letting us know.

I'm really glad your dad got through the preparations and first weeks of his transplant. What was the lowest his WBC reached? If it went down to 0.0 and back up to 0.2 that would be a very good sign, showing first that his old faulty immune system was gone and then that his new transplanted immune system is starting to work.

[Jx3]

His lowest WBC was .1 before the transplant. As of Sunday it was .6. Also showing signs of improvement is his absolute Neutrophils of 380. Doctors are shooting for 500 for 3 days straight to consider him in the engraftment stage.

[LoveRapheal1]

I thought an ATG treatment would be a more sufficient procedure at that age instead of a bone marrow. You should consult another specialist. Good luck with your grandfather.

Originally Posted by Hopeful

I think that you need to look at all the blood levels, not just the WBC. An increasing WBC is sometimes the first sign of a response to ATG. You should look at his platelets and HGB as well because if they are rising (or the need for transfusions is decreasing), that is another sign of response.

Your father is very early in his recovery, as he only had ATG in December. I would be very skeptical if his doctor was pushing a BMT, especially since he is older! It sounds like his specialist is not following standard protocol. Do you know how many other AA patients he has seen/treated?

Is your father still taking cyclosporine?

I would seek out another AA specialist now for a second opinion.