New to SAA

[Sarah Roman]

Hi Its good to be able to talk,

Please help I have been diagnosed with SAA and really not sure where to begin, I am English but live in Spain and my consultant is Spanish so its quite difficult my husband is Spanish so of course that helps but really seeking some help and advice on this illness not even sure wheter to call it an illness. Not sure on how servere it is but I have been prescribed Cicloprosporine 200g a day, only been taking it for 3 days and wondering when it might take effect and what to look iut for, I have read allsorts of stroys and really need to know if thus is life threatening and help to deal with this diagnoses. I am sporty and full of energy my job is activities co-ordinator swimming,walking,keep fit and laughing yoga! Now I have no energy and moments feel quite lifeless, shortness of breath and heart palpitations and feeling scared. First time on the forum I hope I have not gone on to much but really grateful for any help. Many thanks

[DanL]

Sarah,

a diagnosis of SAA can be a little frightening. it can lower your normal blood counts of white blood cells, red blood cells, and platelets. These deficiencies may cause you to become more prone to getting ill, to bruise or bleed more from small bumps or cuts, and may cause shortness of breath due to lower red blood cell counts.

There are a lot of treatment options available for SAA, many of them starting with immune suppression, by taking drugs such as cyclosporine, prednisone, or some other drug. Many patients are eventually treated with anti-thymocyte globulin, (ATG), and then have another immune suppressive drug for maintenance. If none of these reach a solution, a bone marrow transplant may be offered, if you have a suitable match.

SAA, like most bone marrow failure diseases is complex and highly variable based on the patient. It is normally good to seek out second opinions and it is crucial to find a doctor or team of doctors that you trust and are very comfortable with. You should be able to talk to the doctor about any of your questions or concerns and get responses that are thorough and help you understand what your disease is, and what to expect from treatment. Take your time and learn a little bit at a time -- too much information, especially from the internet -- can be overwhelming, and even worse, outdated and incorrect. There have been a lot of advancements that aren't fully published, and many of the statistics that you will see will paint a picture that you will find to be unpleasant. Since each patient is different, it is really hard to glean valid information for your case, so please keep this in mind when you review articles on the internet. Most of this information lag is due to the nature of statistics. It is hard to guess what impact the advances in diagnosis and treatment has done when you try to do a 10 year study on outcomes. In my case, MDS is not generally diagnosed in people under the age of 40. In fact the average age of diagnosis in the US was 71 when I was diagnosed. The other issue was that there are generally only about 15 de novo mds cases diagnosed in people over the age of 18 and under 40. The only people in that age range that get the disease are people who were born with trisomy 8, or people who have had prior cancers treated by chemotherapy.

That is my cautionary tale about information on the internet - keep all data in check. The treatment options are generally pretty current, but don't tell you much about your individual case.

The key is to be strong, keep active, believe in yourself and the people around you, and stay positive. Get good doctors, stay with your treatments.

Good luck in your journey.

Dan

[Hopeful]

Hi Sarah,

SAA is a very serious, life-threatening disease. It seems to strike the young, and your story of being very physically active one moment to being diagnosed with this illness is not atypical.

It is very important to be diagnosed and treated by a doctor that has expertise in this disease, as it is rare.

SAA is a diagnosis of exclusion meaning that your doctor should have run you through a bunch of other tests, including a bone marrow biopsy, to rule out a number of other conditions that can mimic the symptoms of this disease.

The gold standard of treatment for someone with SAA, without a matched sibling donor, is ATG plus Cyclosporine. Cyclosporine alone is not a typical treatment except in the elderly or in third-world countries where ATG is not available. It can work, but the success rate is not as high as when it is combined with ATG. It will take months before you will know whether it is working.

What are your blood counts (HGB, ANC, platelets) and do you have a copy of your BMB report? What is your age? Do you have the option of going to England to see a specialist, as there are a number of people on this forum that are being treated at Kings.

If this is truly SAA, time is of the essence!

[Neil Cuadra]

Sarah,

I'm sorry to hear that you've been diagnosed with severe aplastic anemia, and that a language issue makes it hard to communicate with the doctor.

Hopeful is correct that you'll have to take this diagnosis seriously. SAA (severe aplastic anemia) is more serious than MAA (moderate aplastic anemia) but not as dire as VSAA (very severe aplastic anemia). I don't know if the diagnosis cutoffs are the same in Spain as in the U.S. but it's still an indication of how serious the problem is. The "severe" in SAA usually indicates that two or three of your blood lines (red cells, white cells, or platelets) are noticeably low. Your fatigue and shortness of breath match the symptoms of an abnormally low red blood cell count.

Reading stories like those you'll find in these forums can show how SAA affects patients, but you'll want to start with authoritative information. The Aplastic Anemia & MDS International Foundation is the best source of information I know. Their AA page covers the basics, and you can get more details by having them send you their educational materials (click on Aplastic Anemia in the "Bone marrow failure disease" box).

While reading about AA, I suggest that you write down questions to have your husband ask the doctor for you. One of them should be whether you are a candidate for antithymocyte globulin (ATG), as Hopeful mentions.

Good luck getting the information you need.

[triumphe64]

Originally Posted by Neil Cuadra

Sarah,

I'm sorry to hear that you've been diagnosed with severe aplastic anemia, and that a language issue makes it hard to communicate with the doctor.

Hopeful is correct that you'll have to take this diagnosis seriously. SAA (severe aplastic anemia) is more serious than MAA (moderate aplastic anemia) but not as dire as VSAA (very severe aplastic anemia). I don't know if the diagnosis cutoffs are the same in Spain as in the U.S. but it's still an indication of how serious the problem is. The "severe" in SAA usually indicates that two or three of your blood lines (red cells, white cells, or platelets) are noticeably low. Your fatigue and shortness of breath match the symptoms of an abnormally low red blood cell count.

Reading stories like those you'll find in these forums can show how SAA affects patients, but you'll want to start with authoritative information. The Aplastic Anemia & MDS International Foundation is the best source of information I know. Their AA page covers the basics, and you can get more details by having them send you their educational materials (click on Aplastic Anemia in the "Bone marrow failure disease" box).

While reading about AA, I suggest that you write down questions to have your husband ask the doctor for you. One of them should be whether you are a candidate for antithymocyte globulin (ATG), as Hopeful mentions.

Good luck getting the information you need.

Some of the AAMDS materials are in Spanish.

[Sarah Roman]

Hi, many thanks for your replys!

I have my note from the hospital maybe you can help.
My RBC is 9, WBC 1970 platelets 24 000 and VSG 7 which I dont understand ???
Be so grateful for some help. I have to go back to the hospital 22nd July and from there I will see what to do as the ciclosporine/sandimmune might start to take some effect. I have moments where I feel fine but moments where I feel tired,weak,sad,and very lonely. I have been taking the tablets for 4 days now I have lots of bruises. How long does the treatment take to get into my system. Also people around me dont seem to realise this is serious as the spanish are very blasay when it comes to illnesses. Also my fingers tremble and my lips,heart palpitations is this all correct??
So grateful much love Sarah x

[triumphe64]

"my fingers tremble"

This is common. Cyclosporine depletes magnesium which can cause this.
Also the dose may need to be adjusted. Ask your doctor.

[Sarah Roman]

Thanks for that I go back in 3 wks should I wait? Do you have any other side effects?

[Hopeful]

Hi Sarah,

Are you on Sandimmune or modified cyclosporine?

What are your absolute neutrophil count and hemoglobin levels?

Did you have a bone marrow biopsy done?

While on cyclosporine, the doctor should be checking your cyclosporine trough levels, at least initially, to get the right dosage while avoiding toxicity. I wouldn't wait 3 weeks to check this. They should also be monitoring your kidney and liver functionality.

[Sarah Roman]

Hi hopeful,

Thanks for your reply! Well sorry to say I have not asked consultant to many questions as the language barrier. Well my bloods are 9 Hemaglobin 1970 white and 24 000 platelets also vsg 7 not sure what that means? Thats all the info I have on my bloods. Yes I did have a biopsy and last thurs is when I recieved the news. I am on 1/2 presidone and 200 mg sandimmune also another for my blood to stop the bruising and a stomach protecter. Do these levels relate to anything you have ever had? I am so keen to have as much info as I can so I can learn and move forward. I have actually called the consultant and I will be seeing him this Friday, 2 weeks earlier as I am not feeling that good and also I agree with you I feel to start it should be more frequent. I still keep thinking this is in my head maybe its denial/ shock also I keep thinking if I take a paracetamol like You would for a headache it will all go away! Sorry if I twitter on but its so good to talk in my own language and express myself a bit !

Big hugs and thanks for help!!!

[PaulS]

Hi Sarah - sorry you have to go through this - it must be especially scary and difficult having to understand complicated medical information presented in a foreign language. I don't have experience with SAA but I do have plenty of experience with prednisone. How much are you taking? Your post mentioned 1/2 but I don't understand what that means - 1/2 what? Prednisone can cause all sorts of ups and down - high doses (50mg per day)made it difficult for me to sleep, made me jittery and agitated - short tempered - anxious - and ravenously hungry -also reduced muscle and made me diabetic. I'm down to 15mg/2.5 mg every other day and the effects are far less pronounced.

My guess, from some internet research, is the VSG is the "velocidad de sedimentation globular" - which is the same thing as the erythrocyte sedimentation rate (ESR) or Sed Rate - which roughly speaking, measures inflammation in the body - which may be associated with a variety of conditions including autoimmune - here is a link describing the test - it sounds like your results are not abnormal - but please don't rely on me - ask your doctor for clarification - I am not a doctor and don't speak all that much Spanish - http://www.healthline.com/health/esr#NormalResults6

Hope that helps a bit - best wishes,
Paul

[Sally C]

Hi Sarah,
I did a search on the definition of the CBC test. Here is the link. I hope this helps and I wish you well!!
http://www.webmd.com/a-to-z-guides/c...lood-count-cbc

Best wishes,
Sally

[Sarah Roman]

Thanks Paul and Sally thats a help I am now taking 15mg every day so thats not so bad is it? Thanks for the websites I am going to take a look, I am back at hospital Friday so just preparing a list of questions any thing you think I should ask as this is so new my daughter speaks Spanish and google translate will help me to figure out my answers. When I recieved my results last wk the consultant said in my case he thinks that ciclosporine will be enough????? To soon for him to deciede this No?
Thanks again for responding xx

[Hopeful]

Hi Sarah,

I hope I am not too late in getting back to you!

I would ask your doctor how many SAA patients he has successfully treated with immune suppressive therapy. You need to know this, as his treatment is not conventional. It will be an uncomfortable question to ask, but you must do it. If he has only treated a handful, it is critical that you find another doctor. Cyclosporine alone is not the treatment of choice for SAA.

Get a copy of your bone marrow biopsy results and all of your lab work. What other tests were done to rule out other diseases? Did they do a differential to see your Absolute Neutrophil Count? What is your bone marrow cellularity? Do they think your low blood counts are the results of a virus/infection, chemical exposure, or drug? Are your counts starting to rise?

SandImmune is an older drug that is less prescribed these days. The newer modified form of cyclosporine (like Gengraf or Neoral) has better absorption in the body. The dosing of modified cyclosporine and SandImmune is very different. So, if you have the option, you should switch to the more conventional modified form of the drug. Just be sure that they don't give you the same dosing!

Prednisone is also a warning flag to me that your doctor may not be an expert in this disease (or that he is uncertain of the diagnosis). The SAA experts will say that prednisone has no place in the treatment of SAA.

Do you have other options for a second opinion there?

Be brave! This is a serious disease and if your doctor's aren't treating it seriously, you need to go somewhere else.

[Sarah Roman]

Hi hopeful thanks for getting back!

Well I went yesterday to the hospital I find it so hard to talk to them as its not my native tongue. My HBC is now 8.7 before 9 my white has improved alot its now 4000(950) before 1970 but dont kniw what the number in brackets mean?? Can you help? My olatelets is now 22000 before 24000 so I was a little dissapointed please may I ask do these numbers look like a case of severe I think not?? I asked about hypocellur and she said that I didnt need to worry I am feeling a little lost? Please hopeful I am really struggling to come to terms with this as my brain is great its just physical, I feel like I can everything just get worn out. Do have lots of expericance with this disease ? Is it really this complicated and life threatning?? Please help??

[Hopeful]

Sarah,

SAA is a rare, very serious, life-threatening disease. Before the 1970's (when ATG was invented) people with SAA would die from the disease within 6-9 months. That is how serious the disease is.

Because the disease is so rare, there many good doctors who know very little on how to diagnose and treat the disease. That is why it is so important to see someone who specializes in AA and hematological diseases. They will be excited to see you because the disease is their passion and the patients are few. Usually these doctors can be found at universities or teaching hospitals.

Many other conditions can cause pancytopenia so the doctor should be running a ton of tests on you to rule out other conditions (B12, folate, iron, copper, HIV, parovirous, lupus, PNH, hepatitis, heavy metals, inherited conditions, etc.). It is only when you are negative for everything else that they will consider it AA. Did they do this? Recent infections/viruses, drugs, and chemical exposures can also cause low blood counts, so they should have asked you about your history.

To be considered SAA, you would meet 2 of the 3 criteria:

ANC (Absolute Neutrophil Count) < 500
Platelets < 20k
ARC (Absolute Reticulocyte Count) < 20,000

I don't know your specifics but perhaps they are treating this as Moderate AA??

Here is an excellent reference on how AA is treated by 2 experts in the field:
http://www.bloodjournal.org/content/...o-checked=true

Also, there is a ton of information at the AA&MDSIF website, starting with the very basics:
http://www.aamds.org/diseases/aplastic-anemia

I don't know when your previous blood test was, but it doesn't look like much has changed since that one. Small fluctuations in HGB, Platelets, and WBC are considered normal. Those are small fluctuations. Your WBC is falsely elevated because of the prednisone. So don't get too excited about that! Perhaps the 950 is your ANC??? That is the number of importance here, as it determines how susceptible you are to infections.

I am not a doctor or in the medical field. I am a patient with AA/MDS overlap for 8 years (!) that has been successfully treated with immune suppressive therapy I was also very active at diagnosis and didn't believe my doctor at first when he told me that I had to take this disease seriously! I continue to live an active lifestyle. So don't think that this is the beginning of the end!

Good luck and find that second opinion! You could try calling the US based AA&MDSIF to get their assistance with this:
(800) 747-2820, option 1

[Sarah Roman]

Hi Hopeful!
Many thanks for your response you are being extremly helpful. I have been tested for most things And the doctor diagnosed AA he is the top hematoligist at Almeria hospital so I have to have respect and faith in him I have decieded to wait till I go back on the 21st of June to see my results before I get a 2nd opinion. I cant believe that thus disease is so life threatning its a real struggle to think that this is the reality. I have good days and bad today I have energy and because of this I think I Oh I am fine now! but I know its not, I am nit working at the minute the doctor said I should just be resting and taking it easy its really hard when your brain is saying the opposite. I am glad that you are well I hope my outcome will be good to.
Many Thanks to you Sarah x

[Sarah Roman]

Sorry Hopeful yes I had a biopsy and he said that my marrow bone was working less then 50% and then they put me on my medication presidone 15mg and cyclosporine 200mg and then said to go back 21stJune and take it from there another biopsy and medication. Hoping it will all be on the up. So the numbers I gave you dont come across as Severe just to out me at ease a little.
Thanks again xx