A.A. 4 yrs. and counting not much.

[julia69]

My son has A.A. it's been four years and we just keep getting in a constant rutt.We've tried A.T.G. twice horse and rabbit and we have done daclizaumb at N.I.H. Nothing get's us past 30,000 plts. everthing stay's the same.Our doctor now say's he expects to see an improvement during puberty , he's 10! What do you think?Sounds like DOC . IS BUYYING HIMSELF A COUPLE YEARS OF KEEPING US QUIET!
Any thought's?
Julia

[Ruth Cuadra]

Hi, Julia.

There actually is some basis for your doctor thinking that puberty might cause a change in your son's aplastic anemia: male hormones (androgens) are sometimes given to stimulate the bone marrow to become more active.

Is your son currently on any immunosuppressants? If so, has your doctor suggested trying to taper the dosage to try to get a little more response (that is, more production) from your son's bone marrow?

If your son's counts do not allow him to live comfortably, I would not allow the possibility that changes will occur during puberty to silence you. One of our members, Wendy Beltrami, has a son who is now about 15 and has been dealing with AA for 8 years or so. Although he has had ups and downs, it sounds as though he has been able to live a mostly normal teenage life. I hope she'll see this thread and be able to offer you more advice.

Hope this helps.
Ruth Cuadra

[Wendy Beltrami]

Hi again Julia,
I responded to the other thread but I would like to say that Grant's onset of puberty has been very slow and late compared to most of his peers and especially his brother. It is happening, but slowly. It did not improve his AA, however we are trying male androgen therapy now (Danazol) hoping for some improvement.

Grant was diagnosed 12/4/98 at the age of 7 and since that time, he has had a fairly normal life. Lots of blood draws, and no contact sports but he has just developed other interests.

I would be REALLY REALLY happy with 30K platelets. We would kill for that right now. It has only been this past year since he has been transfusion dependent that it is starting to cramp his style a bit.

Grant's platelets were around 30 and we started getting antsy and wanted to try something new. MMF was what we chose (mycophenolate) and it was disasterous and dropped his red cells drastically and did nothing for platelets. It was the beginning of a serious decline and he has been transfusion dependent ever since!

Wendy

[julia69]

Originally Posted by Ruth Cuadra

Hi, Julia.

There actually is some basis for your doctor thinking that puberty might cause a change in your son's aplastic anemia: male hormones (androgens) are sometimes given to stimulate the bone marrow to become more active.

Is your son currently on any immunosuppressants? If so, has your doctor suggested trying to taper the dosage to try to get a little more response (that is, more production) from your son's bone marrow?

If your son's counts do not allow him to live comfortably, I would not allow the possibility that changes will occur during puberty to silence you. One of our members, Wendy Beltrami, has a son who is now about 15 and has been dealing with AA for 8 years or so. Although he has had ups and downs, it sounds as though he has been able to live a mostly normal teenage life. I hope she'll see this thread and be able to offer you more advice.

Hope this helps.
Ruth Cuadra

Thanks for the posting,Gabriel is taking about 400 pills a month.His kidneys are showing signs of distress from the gengraf.They are not going to lessen the dose cause he is so stable at 28,000 plts.My concern is these counts have never changed in 4 years unless he goes severe which has been twice and it's almost like his body knows no different.I contacted the Aplastic Anemia foundation about the puberty thing and they say it's very rare.

[julia69]

Originally Posted by Wendy Beltrami

Hi again Julia,
I responded to the other thread but I would like to say that Grant's onset of puberty has been very slow and late compared to most of his peers and especially his brother. It is happening, but slowly. It did not improve his AA, however we are trying male androgen therapy now (Danazol) hoping for some improvement.

Grant was diagnosed 12/4/98 at the age of 7 and since that time, he has had a fairly normal life. Lots of blood draws, and no contact sports but he has just developed other interests.

I would be REALLY REALLY happy with 30K platelets. We would kill for that right now. It has only been this past year since he has been transfusion dependent that it is starting to cramp his style a bit.

Grant's platelets were around 30 and we started getting antsy and wanted to try something new. MMF was what we chose (mycophenolate) and it was disasterous and dropped his red cells drastically and did nothing for platelets. It was the beginning of a serious decline and he has been transfusion dependent ever since!

Wendy

I know I should be happy with high 20's low thirtys,but I can't be all the time.That would be throwing in the towel.I am frustrated with the lack of cures or treatments.I am frustrated with the system.I can't be thirlled that my son drags his body out of school cause he's exhausted,that the house is covered with bloody tissues,that he has no want to eat cause his mouth taste like blood and cyclosporine.That he has blinding headaches from low hmg. and throws up blood.I can't be thrilled that he pick's up every germ cause his immune system is flawed and he misses school or his count's drop even more .
I am thrilled I have my son but I will not except that this is the best medical science can do.
Thank you for the posting and best of health for everyone battling marrow failure.
Julia

[Marlene]

Hi Julia,

I know it's very difficult to watch the ones you love struggle with this disease. I'm surprised your son has that much bleeding with that platelet count. So a couple of things come to mind.

First, the next time he gets blood drawn, make sure they run test to check his clotting time. I know it sounds bazzaar but there are clotting factors which play an important role in blood clotting....it takes more than platelets. Even when my husbands platelets were at 8K, his clotting times were normal. So get them to run that test along with a chem panel to check his liver enzymes. The liver plays a key role in clotting factors. Vitamin K is also important for clotting. When John had bleeding, I would make up some gelatin for him to drink. I would get the plain old Knox gelatin powder and mix it up orange juice per the direction and have him drink it. It's soothing to the tummy and has clotting factors to help with bleeding.

Second, I would post on www.aplasticcentral.com also. There are quite a few parents with children who have AA and many people with good solid experience with cyclo. John never took cyclo but from what I've read, it's a balancing act and blood levels need to be checked all the time. It may be helpful to read some of the past post there. It may give you what you need to push this issue with your doctors.

Finally, how low is Gabriel's HGB? I assume if he get really bad headaches he's still transfusion dependent for red cells? If not, the headaches can be from the cyclo.

Take care,
Marlene

[julia69]

Wow I never heard of clotting time,and liver enzimes.I know they are concerned about his kidneys.The headaches are horrible.While he is in distress the blood just pours out of his nose.It's pretty scarey.I will go to that web site you mentioned.What is your husbands partial remission numbers?
Thanks so much best wishes,
Julia and Gabriel

[Marlene]

Johh did High Dose Cytoxan at Johns Hopkins in April of 2002. So we are into his fifth year of recovery and it's been a long one. He was transfusion dependent for 20 months on platelets, and 22 months for red cells.

His counts are still low but we are seeing a very slow upward trend. There were times his count would not budge for 6 -8 months, then make a small improvement and hold there again. So John has not been one of those where once the counts start climbing, they do so fairly quickly.

As of February 16, 2007: WBC - 2.7, ANC - 1.8, HGB - 10.8, Platelets - 43.

These are an all time high for him. His platelets finally started to move last September. Til then, they hung out around 18- 20K.

We would love to see these get closer to normal range. But I am truly amazed at how well he does with low counts. He's only caught the flu one time in the past five years. And with counts lower than these, he broke his wrist in 2004, ruptured his quad tendon, and had surgery for it in Oct 2006. I could not believe how quickly he recovered.

Our approach is to try to do as much as possible to make sure we get proper nutrition, avoid as many of the bad things as practical and to ensure we have a partnership with our doc. There is nothing at this time can medicine can do for John that will help his bone marrow. So we're pretty much on our own and get supportive care when needed. I forgot...there is one thing we are doing. John is on Exjade for his iron overload. Too much iron can effect your BM so we are anticipating that as his Ferritin levels come down, we will see improvements.

I think your instincts on the cyclo should be explored. I saw you posted on the other site. You may want to start a new thread there about Gabriel and see what kind of response you get. Be sure to post his treatments, dates, latest counts, and cyclo levels and how much he takes a day. 400 pills sound like a lot.

Hand in there.....Marlene

[Wendy Beltrami]

Julia,
obviously, your son's platelet levels present themselves in a very different manner than Grant's do. It was insensitive of me to say that I would be happy with 30K platelets. After 8 years I think Grant's body has just adjusted to these lower levels. He doesn't have bloody noses until he hits around 4K and even then, he often does not have them if he is religious about using his saline nasal spray. We recently got a 3 pack at Costco. I highly recommend it. The telling evidence for Grant is big dark blood blisters in his mouth and of course lots of bruising when his platelets are really low.

Certainly if my son was having the day to day issues that your son is having I would not be happy either. There is nothing, I repeat NOTHING worse than seeing your child's quality of life suffer with this disgusting disease.

Grant has been on Danazol for about 5 weeks with nothing much to show for it but MAYBE an increased retic count. One time is not a trend, so I will hold my breath until the next draw. His absolute Retic count went from 26 to 48 on this last count.

Starting zenapax is the next thing we are trying and that is in the works now.
A 30 min infusion every 2 weeks. 4-6 treatments.

We'll keep you posted.

Wendy/mom to Grant

[julia69]

No worries, If I was a little more detailed about his condition ,you would have known.As we all know counts can't always tell the hole story.Are you being treated at N.I.H. FOR THE MALE HORMONES and the possible zampac?
THANKS,
Julia

[julia69]

Originally Posted by Marlene

Johh did High Dose Cytoxan at Johns Hopkins in April of 2002. So we are into his fifth year of recovery and it's been a long one. He was transfusion dependent for 20 months on platelets, and 22 months for red cells.

His counts are still low but we are seeing a very slow upward trend. There were times his count would not budge for 6 -8 months, then make a small improvement and hold there again. So John has not been one of those where once the counts start climbing, they do so fairly quickly.

As of February 16, 2007: WBC - 2.7, ANC - 1.8, HGB - 10.8, Platelets - 43.

These are an all time high for him. His platelets finally started to move last September. Til then, they hung out around 18- 20K.

We would love to see these get closer to normal range. But I am truly amazed at how well he does with low counts. He's only caught the flu one time in the past five years. And with counts lower than these, he broke his wrist in 2004, ruptured his quad tendon, and had surgery for it in Oct 2006. I could not believe how quickly he recovered.

Our approach is to try to do as much as possible to make sure we get proper nutrition, avoid as many of the bad things as practical and to ensure we have a partnership with our doc. There is nothing at this time can medicine can do for John that will help his bone marrow. So we're pretty much on our own and get supportive care when needed. I forgot...there is one thing we are doing. John is on Exjade for his iron overload. Too much iron can effect your BM so we are anticipating that as his Ferritin levels come down, we will see improvements.

I think your instincts on the cyclo should be explored. I saw you posted on the other site. You may want to start a new thread there about Gabriel and see what kind of response you get. Be sure to post his treatments, dates, latest counts, and cyclo levels and how much he takes a day. 400 pills sound like a lot.

Hand in there.....Marlene

His cyclo. level was 250 ,The doc liked it there.His crit. level was high so he's drinking more water, let's hope.Gabe takes many pills since diag.
Thank's for all your help.
Best Health for all,
Julia and Gabe