Help me regarding treatment plan for AA patient

[dhruba_bd]

Dear all,

I am Dhruba from Dhak, Bangladesh. My wife(Sraboni Samadder, Age 23 Year) has been diagonised Aplastic Anemia. On 30th July, 2010 I first did her CBC test and found WBC=1.82K, Haemoglobin=4.82g & Platelet=38K. That time she was supervision under Prof. HAM Nazmul Hossain, Head, Medicine Dept. Dhaka Medical College. He suggested to transfused 2 bag of compack cell and transfer her to Prof. Dr. Monzur Morshed, Head, Clinical Haematology, Bangabondhu Shekh Mujib Medical University. He checked the CBC and Bone Marrow examination report and told me that my wife is suffering for Severe Aplastic Anemia. Also suggested to do Bone Marrow Transplantation ASAP. After another 3 bag blood transfusion her WBC reaches to 4.00K, Haemoglobin=12.0g & Platelet=80K. As their is no facility to do BMT we went to Cristian Medical College(www.cmch-vellore.edu) for further treatment under supervison of Prof. Dr. Alok Srivastava, Head, Clinical Haematology. Here triphal biosphy, Bone Marrow examination were again performed and result was same(Aplastic Anemia).
Dr. suggested to take ciclosphorine 300 mg daily(150mg+0+150mg). She started to take ciclosphrine from 24th Sep. Her blood count on 04th November was WBC=2.93K,Haemoglobin=5.9g and Platelet=54K. My wife feel very tired so I transfued 1 bag blood again. My wife has only 1 sister and yet we dont do HLA typing. Dr. told that ciclosphorine minimum trial period is 3 month, our next appointment with doctor on 31st December.

Now please suggest me what to do.
1. Try ciclosphorine for at least 3 months?
2. Do HLA match with her sister and do BMT as soom as possible?

if wait for 31st december and minwhile Haemglobin decreases under 6.0 gm then transfused whole blood cell or only RBC?

Please answer my questions, I am in a very critical position. Your little bit help can save my wife life.

Thanks,
Dhruba
Cell: +8801717251152

[Lisa V]

Dhruba, in the US the standard is: if the patient is young (less than 40 yrs.) and has an HLA matched sibling, then transplant is usually recommended. If there is no match, or if the patient is older, then IST (ATG + cyclosporine) is usually the first line of treatment. In order to make a decision, you will need to first find out if your wife's sister is a suitable match. If not, then IST is the next step. If that doesn't work, then it's time to search for an unrelated donor.

Is ATG treatment available in Bangladesh? The rate of success of cyclosporine alone is not nearly as good as it is in combination with ATG. I would be concerned about that.

[dhruba_bd]

Originally Posted by Lisa V

Dhruba, in the US the standard is: if the patient is young (less than 40 yrs.) and has an HLA matched sibling, then transplant is usually recommended. If there is no match, or if the patient is older, then IST (ATG + cyclosporine) is usually the first line of treatment. In order to make a decision, you will need to first find out if your wife's sister is a suitable match. If not, then IST is the next step. If that doesn't work, then it's time to search for an unrelated donor.

Is ATG treatment available in Bangladesh? The rate of success of cyclosporine alone is not nearly as good as it is in combination with ATG. I would be concerned about that.

Dear Lisa,

Thank you very much for your reply. ATG treatment is not available in Bangladesh as there is no specialized hospital to support Haematology patient. Her doctor told us to try cyclosporin at least 3 months. But now she need blood transfusion. As far I know that blood transfusion can make BMT more complex. Now I want to know what will more feasible for me.

1. Wait 3 months and continue cyclosporine?
2. To take decision for BMT?

Thanks,
Dhruba
+8801717251152

[Lisa V]

Dhruba, the first thing you need to do is have your wife's sister tested.

BMTs don't happen quickly, it will take time to do the testing and make all of the other necessary arrangements. Since she has already been taking cyclo since September, 3 months is not that far off. Even if her sister is a match, I doubt they could arrange to do the BMT before then, and if she shows improvement by then, you can always cancel it. You are not committed to go through with any procedure if her circumstances change. The doctors should tell you that.

Yes, the more transfusions she has, the more risk of developing antibodies that can complicate a BMT. That is not a reason to avoid transfusions, however. To not get needed blood or platelets would be much riskier for her health in general. It is unavoidable, but there are many people here who have had quite a few transfusions and still had successful BMTs.

It might help you to read the transplant section of this forum, just to get a better idea what is involved.

All the best,

[Hopeful]

Hi Dhruba,

Cyclosporine alone has been used successfully to treat SAA. The response rate is lower than if used in combination with ATG.
http://www.ncbi.nlm.nih.gov/pubmed/16270758

As Lisa pointed out ATG or BMT are typically the first line of defense for SAA. However, if ATG is not available, cyclosporine is an alternative.

While waiting for the response, can you start the HLA testing on her sibling? This process takes time so it would be beneficial to start it now.

Also, you may want to have her EPO levels measured to see if she is a candidate for EPO shots as an alternative to RBC transfusions.

Also, do you know her absolute reticulocyte count? A high reticulocyte count at diagnosis is a good predictor of response to IST.

[dhruba_bd]

Originally Posted by Lisa V

Dhruba, the first thing you need to do is have your wife's sister tested.

BMTs don't happen quickly, it will take time to do the testing and make all of the other necessary arrangements. Since she has already been taking cyclo since September, 3 months is not that far off. Even if her sister is a match, I doubt they could arrange to do the BMT before then, and if she shows improvement by then, you can always cancel it. You are not committed to go through with any procedure if her circumstances change. The doctors should tell you that.

Yes, the more transfusions she has, the more risk of developing antibodies that can complicate a BMT. That is not a reason to avoid transfusions, however. To not get needed blood or platelets would be much riskier for her health in general. It is unavoidable, but there are many people here who have had quite a few transfusions and still had successful BMTs.

It might help you to read the transplant section of this forum, just to get a better idea what is involved.

All the best,

Dear Lisa,

Thank you again for your informative reply. Actually In Bangladesh, I can do HLA A, HLA B and HLA C typing. My Bangladeshi doctor told me that to select my wife sister as a donor need to do some other HLA typing. So I cant do the HLA matching now. My next appointment fixed on 31st December in India. I am now worried that am I getting too late?
I transfued 1 bag compack cell but still my wife feel very much weak. Will I transfuse more? If transfuse then transfuse only RBC or whole bag?

[Hopeful]

In the US, it is my understanding that they do not transfuse whole blood for AA. They only transfuse RBCs, platelets, and on rare occasions, WBCs.

Also, irradiated RBCs are preferred, as your wife is a candidate for a BMT.

[dhruba_bd]

Originally Posted by Hopeful

In the US, it is my understanding that they do not transfuse whole blood for AA. They only transfuse RBCs, platelets, and on rare occasions, WBCs.

Also, irradiated RBCs are preferred, as your wife is a candidate for a BMT.

Thank you very much for your reply.

[dhruba_bd]

Originally Posted by Hopeful

In the US, it is my understanding that they do not transfuse whole blood for AA. They only transfuse RBCs, platelets, and on rare occasions, WBCs.

Also, irradiated RBCs are preferred, as your wife is a candidate for a BMT.

Thank you very much for your reply. Yesterday I did the blood count and found WBC=2600,HB=5.8 and Platelet=40K. So, today she take 1 bag RBC. As I already told you that my next appointment on 31st December.

Should I wait for that day?
At which point I need to transfuse Platelet?

Thanks
Dhruba
Dhaka,Bangladesh
Cell:+8801717251152

[Greg H]

Originally Posted by dhruba_bd

At which point I need to transfuse Platelet?

Dhruba,

My platelets are currently at 30K (I'm in hospital at the US National Institutes of Health), and I have been told by my doctor's that they will transfuse platelets if the count gets down to 10K. Below that, things get dangerous. I think some folks may use 20K as the marker, but here it is 10K.

Good luck! Your wife is lucky to have you looking after her.

Greg

[Hopeful]

Hi Dhruba,

I would usually get 2 units of RBCs when my HGB was in the low 7's. A HGB in the 5's is pretty low. I'm not sure if your units are the same as ours, but does she get enough boost from just one bag of RBC's?

As Greg pointed out, a platelet count of 40k is pretty good in the AA world. I would transfuse when platelets were below 10k.

[dhruba_bd]

Originally Posted by Hopeful

Hi Dhruba,

I would usually get 2 units of RBCs when my HGB was in the low 7's. A HGB in the 5's is pretty low. I'm not sure if your units are the same as ours, but does she get enough boost from just one bag of RBC's?

As Greg pointed out, a platelet count of 40k is pretty good in the AA world. I would transfuse when platelets were below 10k.

Her look the below WBC count and Neutrophil count and give your kind feedback.

On 04.11.2010----------WBC=2930 & Neutrophil=28.3%
On 20.11.2010----------WBC=2600 & Neutrophil=31.2%
On 30.11.2010----------WBC=2500 & Neutrophil=28.0%

From the above count what you think,
1. her condition is getting worse day by day?
2. Can we say that Cyclosphorine does not work?
3. She need to do BMT/ATG as soon as possible?
4. Is she is a severe phase of Aplastic Anemia?

Someone told me that I am getting late. Please help me, I am very much worried.

[Hopeful]

Hi,

I am not a doctor, but to me her WBC looks stable. How are her platelets doing now? This is typically (but not always) where you would see the improvement first. Patience is key. If you see her platelets going up, that is a good sign that the Cyclosporine is working.

The severity of her AA can only be assessed by her bone marrow cellularity, reticulocyte count, platelet count, and absolute neutrophil count (ANC). In either case, it would not effect the treatment at this time, since you said that ATG is not available.

I would not be overly anxious to jump to a BMT if there isn't a facility with lots of experience in doing this. It is a very risky procedure. Sometimes ATG is even used as a pretreatment regimen for a BMT.

Waiting is the hardest part in all of this! Your wife is young and that works in favor for a positive response to IST. I wish you both well!

[dhruba_bd]

Thnak you very much for your kind reply.

Please look below for her Platelet count and Neutrophil count and give your kind feedback.

On 04.11.2010----------ANC= 829.19 and Platelet=54000
On 20.11.2010----------ANC= 811.20 and Platelet=40000
On 30.11.2010----------ANC= 700.00 and Platelet=40000

From the above count what you think,
1. her condition is getting worse day by day?
2. Can we say that Cyclosphorine does not work?
3. She need to do BMT/ATG as soon as possible?
4. Is she is a severe phase of Aplastic Anemia?

Someone told me that I am getting late. Please help me, I am very much worried.

[Hopeful]

Originally Posted by dhruba_bd

From the above count what you think,
1. her condition is getting worse day by day?

I can't speculate on this.

Originally Posted by dhruba_bd

2. Can we say that Cyclosphorine does not work?

I think it is too early to tell one way or the other. *Early* response to ATG with cyclosporine happens within the first 3 months, but it can and frequently does take longer. Cyclosporine alone is not as powerful as ATG. So, it seems likely that it may take longer in some cases.

Originally Posted by dhruba_bd

3. She need to do BMT/ATG as soon as possible?

If she has a perfect sibling match, BMT is the first choice for treatment.
Otherwise ATG is the preferred treatment. Is this an option for you?
If ATG is unavailable, cyclopsorine seems like the next best thing. It is a treatment, and if her disease is immune mediated, should gradually slow the disease's progression.

Originally Posted by dhruba_bd

4. Is she is a severe phase of Aplastic Anemia?

The criteria for severe aplastic anemia are two of the three in a hypocellular marrow:
* Absolute neutrophil count less than 500
* Platelets less than 20k
* Absolute reticulocyte count less than 60,000 /uL

Don't get caught up in the staging. Since she is transfusion dependent, her disease is serious.

Also don't get caught up in weekly blood count changes. Look at the trends month to month.

Hopefully, her doctor will provide some reassurance at the next appointment. It would be good to understand why he thinks the diseases is immune mediated and may respond to IST.

[dhruba_bd]

Dear all,

I have a good news. My wife has an identical HLA with her elder sister. Plz chcek (http://www.facebook.com/photo.php?fb...67869816581260) for the reports. And BMT date is fixed on March as there is no date available before that. Doctor stop Cyclosphorine.

Now please check the follow CBC trend and give me advise.

On 04.11.2010----------HB=5.9,WBC=2930, ANC= 829.19 and Platelet=54000
On 20.11.2010----------HB=5.8,WBC=2600, ANC= 811.20 and Platelet=40000
On 30.11.2010----------HB=7.2,WBC=2500, ANC= 700.00 and Platelet=40000
On 14.12.2010----------HB=5.8,WBC=2700, ANC= 540.00 and Platelet=41000
On 31.12.2010----------HB=7.5,WBC=2390, ANC= 499.51 and Platelet=41000

Please note that she took total 3 bags RBC during this period on 11.11.2010, 21.11.2010 and 15.12.2010.

Now if I want to do BMT on CMC, India(http://www.cmch-vellore.edu/PatHaema...0/Default.aspx) then no date is available before March, so what should I do? Also note that it is the best institute for BMT in india and my wife is an OPD patient(patient ID:782971D) of this hospital under supervision of Dr. Alok Srivastava, MD,FRACP, FRCPA, FRCP.

1. Wait till March?
2. Do BMT in other hospital?

[Neil Cuadra]

Dhruba,

It is wonderful news that Sraboni's sister is a match. It is worrisome that the transplant cannot be arranged at Christian Medical College before March because her ANC has been dropping. Transfusions are keeping her hemoglobin up but her neutropenia (lack of white cell neutrophils) is now on the borderline between "moderate" and "severe", which means she is in increased danger from infections. You should do what you can to keep her away from sources of infection (such as other people who are sick) and I think you should ask her doctor what to do about the neutropenia if you have to wait months for the transplant. Perhaps her doctor will have advice about reducing the chance of infection or will want to treat her for the neutropenia.

Christian Medical College does about 80 allogenic transplants a year. If you know of another treatment center that also does transplants it would be worth finding out if they have similar experience and can arrange a transplant sooner than March. Waiting that long increases her chance of getting an infection and also increases the number of transfusions she'll need.

Did her doctor explain why they stopped cyclosporine?

If Sraboni had been at Christian Medical College originally they probably would have used immunosuppressant therapy with ATG or ALG. (You found that there is no ATG treatment in Bangladesh but there is at CMC. I can tell from their research reports.) But with a transplant coming up I doubt they will recommend ATG now.

Are you having to travel back and forth between Dhaka and Vellore or are you staying in India now?

[dhruba_bd]

Dear Neil,

Thank you for your reply. I requested many times but CMC couldn't fixed a date for BMT before March as all the slot already been booked before March. We will go Vellore on February for pre-transplantation work. CMC is the best hospital in India for BMT and also the cheapest. Actually the total treatment cost is far beyond my capacity. Many people help me to raise fund for my wife's treatment. You can visit (http://www.facebook.com/home.php#!/p...6581260?v=info) for details. Till I am lack of money to finished the treatment. So I have no other option than wait. Other hospital of India charges more(at least double) than CMC.

Doctor stopped Cyclosphorine and suggested to take Danazol capsule IP 200 mg(1+1+1) a day.

CMC suggested BMT as 1st option not ATG.

Now I am in Dhaka and start for Vellore on 7/8th February,2011.

Again thank you very much for your reply. Please pray for her early recovery.


Thanks
Dhruba
+8801717251152
+8801912101407

[Lisa V]

Dhruba, I am glad to hear that your wife's sister is a match, but sorry you have to wait until March for the transplant. As Neil says, the main concern now is her ANC and the increasing risk of infection. Does your doctor in Dhaka have access to G-CSF drugs like Neupogen or Neulasta? These are growth factor injections that can stimulate white cell production. Unfortunately they are very expensive, and it sounds as though you are already going to be hit very hard financially.

The alternative is to take as many precautions as possible to keep her from being exposed to sources of illness or infection. She should avoid any unnecessary contact with other people and wear a surgical mask if possible. Eat only foods that can be peeled or cooked to eliminate bacteria on the surface. This description of the "neutropenic diet" may be written with US patients in mind, but it may help give you an idea the kinds of things to be avoided: http://www.rd411.com/index.php?optio...ets&Itemid=383

I hope all goes well!

[Greg H]

Hi All!

Would it make sense for Dhruba to obtain some ciprofloxacin to ward off infection, assuming he can find it affordably?

The protocol I am on prescribes 500 mg 2x/day as long as ANC is under 500.

Greg

[dhruba_bd]

Dear all,

Today I got the CBC result and found that

Total Leucocyte count=2250 and Absolute Neutrophil count =261.

As you already knew that her BMT date is fixed on March. So, what will I do now? Please suggest me.


Dhruba
+8801717251152