Mom dx with AA possible MDS

[Lamilu]

Hi Everyone,

Thank you in advance for any information.

My 68 yr old Mother is in the process of being dx with AA. She has had 2 bone marrow biopsies now. The last one Feb.7. After a heck of a time I finally got a hold of her hematologist here in Edmonton at the University of Alberta (Dr.Larett). She told me that they think Mom has AA and are waiting to get final results on whether it is MDS or not. Which she said would take 2-3 weeks. My mom has been very week and tired. Her bloodwork had fluctuated. Platelets being the worst. She has had 1 hemo transfusion and 4 platelets. when i talked to he dr. on Wednesday they told me that if there were beds available Mom would be starting treatment (ATG, Cyclo and Steroids). She said maybe Tuesday Mom might be admitted. I'm not really familiar with what is normal for counts and what is not but here are numbers for this morning. Hemo at 91, Platelets were at 23 after having a transfusion last saturday and are now going down again at 19. She will probably need another Plate trans on Monday because it keeps going down to 9. Her neutrophils are at 0.4 which is what is the most concerning for me now. I left a message for the Dr. to tell her I think Mom should be admitted before she gets sicker. but I haven't heard anything back. What should I do? Is this super serious for someone of her age? Or should we calm down and just let things take their course? Any advice would be MUCH appreciated. Oh ya, Mom also has problems with her heart. Thanks!!!!

[Birgitta-A]

Hi Lamilu,
You know since I have MDS I don't know much about AA but here you will get the normal counts. Different labs can have a little different reference values but they are always written on the lablists:
http://www.lymphomation.org/CBC-blood-counts.htm

As you have understood hemoglobin, white blood cells (specially the neutrophils) and platelets are the most important counts.

A hemoglobin of 91 (or 9.1) is quite good - many patients manage well with that but your mother has heart problems that make her feel the low hemoglobin.

When her neutrophils are 0.4 she can easily get infections - if they have done the examinations like BMB she should at once have Neupogen or a similar drug for her white blood cells.

It is a problem with platelet transfusions because they never last long - hopefully the platelets will increase when she receives steroids.
Kind regards
Birgitta-A
70 yo, dx MDS Interm-1 May 2006, transfusion dependent, Desferal and Exjade for iron overload, Neupogen 2 injections/week for low white blood cells after neutropenic fever Sept 2007.

[Lamilu]

Thanks so much for your reply. Any information helps. I have been on the net researching every bit of info I can find. Never hearing of AA or MDS before, I have had my work cut out for me. I was wondering, when I looked up my Moms doctor, she a specialist in MDS. Does that mean she is a specialist in AA as well or are they two totally different diseases? Also my Mom was switched from one Dr. to this one and I am wondering if there is more too it. When I spoke with Mom Dr. yesterday she said my Mom would be fine even if her counts went to nothing, as long as she doesn't get a fever not to worry. I had read about the shots you can get or even just being put onto antibiotics for preventative measures. I don't understand why her Dr. isn't doing this if it is that serious. I don't want my Mom to get left in the dust because there are no beds available at the hospitals here in Edmonton. Should I then push for my Mom to get these shots to help with her neutrophils? My Mom also has what is called Afib. Her heart beats are irregular and at times she has had to get "shocked" into beating properly again. She is worried because her heart is going haywire. Is this from not getting enough oxygen? One more thing. Is being dx with MDS mean a worse outcome than AA? Sorry for all the questions but I'm very worried and I know that talking to people who are in the same situation is the best way to get answers. Thank you!



Daughter to 68yr Mom in the process of being Dx with AA or possible MDS.

[Lamilu]

I wanted to clarify. the doctor said Mom would be fine if her neutrophils went to nothing. Not all of her counts. Just the neutrophils.

[Birgitta-A]

Hi Lamilu,
As far as I understand MDS is a more severe diagnose than AA. Patients with AA can be cured with drugs and only few patients with the type of MDS that is on the border to AA perhaps can be cured with drugs.

Then I have understood from other members that neutropenia in AA patients isn't as dangerous as neutropenia in MDS patients. I still think 0.4 is very low but your mother doesn't need antibiotics now.

AA and MDS are both bone marrow diseases and your mother's doctor should know much about both diseases if she is a specialist in MDS.

In any case you could look at these advices for patients with neutropenia. It is old but all the neutropenia sites look the same to me. I live like I have neutropenia since I had neutropenic fever Sept 2007 and have not had any infections (knock on wood).
http://www.neutropenia.ca/about/living.html

Heart fibrillation is quite common - your mother must have medication (like digitalis) for that symptom. Be sure that she is getting the best possible care for her heart.
Kind regards
Birgitta-A

[Lamilu]

Thank you Bergitta for taking the time to respond. I feel a bit better now. When my Mom gets her bloodwork done tomorrow she is going to ask about that shot that will help with her Neutrophils. Thanks again!!!!

Daughter of Sharon (68)

[Birgitta-A]

Hi Lamilu,
We hope that your mother's counts have improved!
Kind regards
Birgitta-A

[Lisa V]

Lamilu, the fact that your mother has had 2 BMBs and they are talking about ATG makes me think that her marrow is probably hypoplastic (empty). Most MDS patients have hyPERplastic marrow (too full), and those patients don't generally respond to ATG. There is a much smaller category of hyPOplastic MDS patients, which is very similar to AA and often difficult to distinguish from it. The main difference seems to involve having some sort of chromosomal mutation, so that is probably what they are waiting to find out. Cytogenetics testing takes a bit longer than just looking at the cellularity.

The good news is that hypo MDS patients tend to have a better response rate to ATG, so if this is the case, it may be successful regardless of whether she is technically classed as AA or MDS. You do want to get an accurate diagnosis before rushing into anything, but it may not necessarily change the course of treatment or the prognosis. My husband's diagnosis was changed from SAA to hypo MDS after his first course of ATG, and it didn't affect his response to the second round. If anything, it helped explain why he relapsed after the cyclosporine was tapered off, so now we know not to attempt another taper.

Currently there doesn't seem to be any corresponding regimen for the hyperplastic forms of MDS (MDS is not really one disease but a group of related syndromes) that has the same sort of success rate as ATG does for AA. There are medications that can help with some of them, but as far as I know none of them is likely to bring about full remission. Somebody may correct me if I'm wrong about this.

I wouldn't say that your mother will necessarily be "fine" if her ANC drops to zero, but if she observes certain basic precautions she can get through it. I would advise wearing a surgical mask, limiting her exposure to people, particularly if they are sick, eating only foods that can be peeled, scrubbed or cooked, etc. Even if they were to start the ATG right away, her counts are likely to drop before they come up. Many patients go through a period of neutropenia following ATG, and since her white count is already pretty low, you should expect that. Also ATG tends to "eat up" platelets, so she may require even more frequent transfusions for a while than she's getting now. Eventually, though, it should start to turn around.

Best of luck,

[Marlene]

To echo Lisa's comment....John's ANC was zero for 3 months until his white cells kicked in again. He was on quite a few drug for infections but as soon as his anc reached .350, they pulled the drugs as long as there was no fever. So our doc at Hopkins feels you have the ability to fight infections with an ANC of .350. It took a long time for the ANC to get up to 1.0 without neupogen. I think it was about 1.5 years after treatment.

This of course is for AA and MDS. I feel the cells seem to work better in those with AA since they are "normal" cells.

[Lamilu]

Thank you for the information. My Mom actually was admitted and started ATG last night. They gave me a copy of her blood work as well as their findings. The blood work I kind of understand now but the comments they have about her dx I'm confused. I'm not really sure what means what. They started her ATG yesterday at about 3:30pm. At about 7pm she began to get hives. They stopped the ATG and gave her Benadryl. Then started it back up at a slower rate. By 1am they stopped again because she had more hives, chills and a fever. At this point that is all I know. When I called, the nurse said they had to wait for the doctor to come in and check her out before they could decide what to do next. So now I'm wondering, if this doesn't work then what next? What else is there to try that and older person could handle? Or will they possibly just keep going with the ATG and my Mom will just have to get through it? My worst fear is Sepsis as I have read that it is a possibility. Also, I did read in her chart "The peripheral blood smear shows subtle features of hyposplensim" which from what I just read is Sepsis. right? My Mom is really scared. So am I. It does help so much to have somewhere to come to ask questions or just to vent. thank you.


Daughter to Sharon 68 AA

[Lisa V]

Lamilu, chills and fever are absolutely normal for the first infusion of ATG. It is not an indicator that anything is going wrong, it's just a serum reaction which usually passes after a few hours. I don't know of anyone who has NOT had this reaction, but sometimes an inexperienced medical team can mistake it for symptoms of infection and administer antibiotics (this happened to us). Hopefully they will not do that with your mother unless they have some concrete evidence something else is going on. Normally they just give Tylenol and wait it out.

Hives indicates an allergic reaction, so slowing down the rate and increasing the Benadryl are also standard procedure. Whether or not she will be able to continue will depend on how severe her reactions to the ATG become. This is something for the doctors to determine, but she may have to tolerate a certain amount of discomfort for a while.

Hyposplenism is not sepsis, it is reduced spleen function. I don't know a lot about the spleen, but I found this quote from Williams Hematology:

"The spleen culls aged and abnormal cells from the blood; removes intraerythrocytic inclusions through a process called pitting; sequesters approximately one third of the normal intravascular platelet pool; removes bacteria, foreign particles, and tumor cells from the blood; and by virtue of the T and B lymphocytes and macrophages in the white pulp plays a role in immune surveillance and antibody formation. Exaggeration or impairment of some or all of these splenic functions results in hypersplenism or hyposplenism, respectively."

So basically, since the spleen appears to play an important role in filtering out impurities from the blood, a loss of function could put one at an increased risk for infections. That does not mean that sepsis is the inevitable result, it just means that her immune system is compromised, which you already know. In fact, if it's only the T and B lymphocyte function that is being affected, that may be kind of a non-issue at this point because those are what the ATG is intended to knock out anyway (in order to short-circuit the autoimmune attack that is thought to be causing the AA in the first place). What is more important at this point is that she has enough working neutrophils to take up the slack, but as Marlene has stated, it is still possible to go quite a while without them, so long as she is being monitored and managed for any flare-up of infection.

Just judging by the quote above, it doesn't sound as if her spleen function would have any impact on neutrophil production. It may be affecting her platelet production, but I'm not sure exactly how. Just to put things into perspective, though-- in the old days before ATG and BMTs, the first line of tratment for AA was removal of the spleen. I don't think it was terribly effective in a lot of cases, but they certainly wouldn't have done that if you couldn't exist without a spleen. At any rate, we are fortunate to have better options these days.

Hope this helps some,

[Lamilu]

Ok then, I will try not to worry so much. I have passed this info onto my Mom as she is pretty scared.

Thank you!

[Lamilu]

My Mom is home from ATG treatment now. She had many side effects including, rigors, fever, low oxygen, severe headaches, large hives covering her whole body and was quite depressed. She was sent home yesterday afternoon. Last night she still had a few hives but nothing that was to bothersome.

One thing that I wanted to ask anyone who has had ATG before. Mom ended up getting fluid on her lungs that has made it hard for her to breathe. They said it was because of all the fluids going into her IV. She was sent home with diuretic pills and told she would be fine. Has anyone experienced this before? I never came across this symptom. I'm also quite worried about it. She is breathing better but it just seems weird they would send her homelike that. Her counts are also down. Her Hemoglobin was at 88 yesterday morning. Her WBC 1. not sure of the others as her blood work just says "pending". Hopefully they will get a Hemo transfusion into her shortly as she was going to the clinic for more blood work today.

Thanks!!

[Marlene]

John had pleural effeusions (sp) also. The diuretic should help but they need to monitor her potassium very closely as well as magnesium and calcium. Diuretics deplete potassium. So make sure they check this at her appointment tomorrow.

They gave John IV lasix which worked better than the oral pills. I assume they did a chest xray to assess the volume of fluid around her lungs. They should give her IV diuretics with her transfusions and run them very slowly.

They do resolve as you heal. If she's too uncomfortable or if breathing becomes worse, be sure to tell them. John's were not resolving and he had further complications with fluid in pericardium sac. They finally had to drain the fluids.

[shirah hadasha]

Dear Lamilu,

This is my first posting. I have no advice but I can tell you a bit of my story. Let me start out by saying that I am going to be 68 in two weeks. I was diagnosed with AA about 12 years ago. My doctors tried ATG and that did not work for me (extract of horse was attempted twice, with unpleasant side effects both times) though it has for others. Injections of epogin and neupogin didn't make me fee too good either, but I stayed with the treatment and after some months it started to work.

I guess what I am trying to do is encourage you to keep doing what you're doing: to persevere, to ask questions of your doctors and to use this forum to stay in touch with your compatriots.

We will never meet, but I personally wish you all the best.

Shirah

[Gloria J]

Dear Lamilu,

ATG is not an easy treatment, and to have other medical issues going on, I'm sure, only complicates matters. The rigors, fever, hives - have they evaluated her for serum sickness because of the ATG?

Your Mom will probably still get transfusions fairly frequently (every two-three weeks) until the ATG kicks in. In my experience, it wasn't until almost six months post-ATG, that I could go a month in between them.

All of the different meds she is taking are to help ameliorate the side effects of the Cyclosporine, and they will decrease as she eventually tapers off the Cyclosporine. Make sure they monitor the level of Cyclo in her blood (blood is drawn just before your next dose) so she is not taking more than she needs.

The next few weeks will not be easy, but encourage your Mom to hang in, she will feel better. One last thought - I never had Neupogen shots for the WBC. My counts tended to bounce up and down, so I have always been very careful in public, and avoided situations where I might encounter risks of infection.

Best wishes to your Mom, and let us know how she progresses.

[Lamilu]

Mom is feeling a bit better now. She is to go three times a week for blood work. Her counts were down today so she is going for a platelet transfusion tonight. She has sores in her mouth that she got Akabutus mouth wash for. She is weak and has tingly lips. She said it feels like pins in her lips. Otherwise doing good so far. I have to thank you all for your replies. I know without a doubt that not having the information that you gave us would have made things worse for her (and me). Every time I was not sure about something, or my Mom wanted to know, I came on here and asked and you took the time to reply. Please know it has REALLY helped! My mom is not too computer savvy but I"m desperately trying to get her to come on here and talk. I hope she will. I know it would make a difference for her. One thing I notice is that people don't take this disease seriously. If you didn't know my Mom was sick, you maybe wouldn't notice anything different besides her being a bit slow. Have any of you dealt this feeling before? I think people expect to see something visual when someone is REALLY sick but I know that is not always the case. I knew before as my 5 year old has severe arthritis. We have a fairly large family and my sister and I are the only ones that are REALLY scared for my Mom. Unless my brothers just don't want to face it. I suppose everyone deals with things in a different way. Anyways, thank you again. Hopefully my mom and I will be able to help others. I know I will be back again asking more questions of you in the future.


WBC 1.8
Hemoglobin 94
Platelets 7
Neutrophils 1.2

[Neil Cuadra]

Originally Posted by Lamilu

One thing I notice is that people don't take this disease seriously. If you didn't know my Mom was sick, you maybe wouldn't notice anything different besides her being a bit slow. Have any of you dealt this feeling before? I think people expect to see something visual when someone is REALLY sick but I know that is not always the case.

Lamilu,

How people handle a serious illness in the family depends so much on individual personalities and roles in the family. Some people step forward, some retreat, some want all the facts, and some don't want to hear any bad news. When a parent is ill, care often falls to the oldest child, the relative who lives the closest, or the most nurturing member of the family. The caregiving work is rarely shared equally and it can put a strain on everyone.

For those who don't want to face the severity of AA and MDS, closing your eyes to the problem is a lot easier when it's a disease you never heard of, when people avoid calling it cancer, and when you can attribute the outward signs like fatigue to "getting old". When you say aplastic anemia people may think of run-of-the-mill anemia from low iron and think it's not a big deal. The flipside is that patients probably don't want to look sick, even if helps convince people that they need support.

If you want family members to understand more about your mom's condition, my suggestion is to give them information about it that they can absorb at their own pace. You could email them status reports, or give them a copy of the literature from AAMAC. I doubt it'll sit there unread. Still, remember that having them worry won't accomplish much. If you can use help from family members, I suggest asking them for something specific: to visit Mom, to do an errand, to be available to listen when you want to talk, or whatever you and your sister need in order to help your mom.

[Lamilu]

Thank you for giving me some suggestions. I will email the PDF I found at the AA and MDS site. It is very informative. I have talked to my brothers about what AA is and what it could mean in the future, but I don't think they understand. And of course my Mom downplays everything because she doesn't want anyone to worry over her. Maybe if they read some of the information themselves they will understand it better. I do need to be more specific with them in saying exactly what is needed. I am the youngest of 5, but I am the closest with my Mom. I am the "caregiver" type of personality. I suppose it comes more natural to me. Thanks Neil for your reply. It helps to hear someone else's view on things.

[Marlene]

The term "aplastic anemia" really doesn't reflect the seriousness of the disease. It sounds very benign. Like Neil said, most think it's easily corrected with iron supplements or by eating red meat. We finally just started to tell people that John had catastrophic bone marrow failure to drive the point home when needed. We then told them the medical label for what was going on in his bone marrow was called Severe Aplastic Anemia. I'm not one for drama but I found this was a good short cut to getting the point across about the severity of SAA.

[Lamilu]

I agree with you. They need to change the name or something so people don't confuse it with regular anemia. It would help.

Thanks!

[Lisa V]

I'm with Marlene. After a while we just started telling people he has a bone marrow failure disease. That pretty well gets the point accross, and avoids the confusion with iron deficiency anemia or the issue of what to call it since they found his chromosome mutation. AA or MDS, it's still bone marrow failure, and even if people don't understand exactly what that entails, it sounds serious.

We've also dealt with the issue of his not looking sick. After his first ATG people took it seriously for a while because he did look and act differently. Most of that was just the drug side effects though: puffy face and short temper from the prednisone; hand tremors, tiredness and loss of voice from the initially high dose of cyclosporine. Once we tinkered with his meds, he looked and felt much more normal, and everyone assumed he was "all better". I even had one person tell me "Gee, I heard he was at death's door, but then I saw him driving around!" Like he was faking it, or we were all being drama queens or something? I don't know. I just have to calmly explain that no, he's not all better, but he's undergone treatment, is currently stable on medication, and that we have a normal life, for the time being, anyway. If they want to know more, I'll tell them more, but these days it's not so much of an issue anymore because his counts are greatly improved and he really does feel fine. Hopefully he can continue like this for a good long time! Life is good again, but I don't forget what it was like for a while, and relapse is never far from the back of my mind.