Childhood Bone Marrow failure/MDS

[Cupcakegirl]

My 9 year old daughter has a low white blood count. 2.2 as of 2 weeks ago. She had a bone marrow biopsy and the pathologist said he is not going to rule out MDS. The hematologist is not treating my daughter for anything...we just get blood work every month. My daughter is so tired she cant go to school.

We have been dealing with this since Feburary last year. She has fevers ranging from 99.1 to 102.3 and doctors where we are are not doing anything. They said they might repeat the bone marrow biopsy in January.

My daughter has a identical twin sister and her twin does not have any of these issues of symptoms and her blood work is fine. I am so confused as to what to do. Our house was tented for termites and that is when all of this started.

Should I be pushing for the doctors to do anything and is there anything I could be doing to bring her WBC up? I just bought a juicer because the doctors dont know what is wrong with my daughter.

Any help or advice would be appreciated! Blessings to you.

[triumphe64]

Where are you in California?

[Cupcakegirl]

Southern California. I have contacted City of hope but we have not heard from them yet.

[Neil Cuadra]

Cupcakegirl,

I'm very surprised that the doctor hasn't given you more information by now. It might be a good idea to start making a list of questions for the next appointment with your daughter's hematologist.

There are many possible causes of fevers or a low white blood count. The information you've posted isn't enough to conclude that it's a bone marrow problem, or that it isn't.

Here are some questions that might help you learn more about what's going on:

What causes of your daughter's symptoms does the hematologist consider to be possibilities?

What sign is there that it could be MDS? What about aplastic anemia, which for children is more common (actually, I should say "less rare") than MDS?

What other tests have been performed and what tests besides another bone marrow biopsy should be considered?

Is her fatigue due to a low red blood cell count (anemia) or is her red blood cell (RBC) count normal?

You can find other possible questions here.

Let's hope your daughter doesn't have a bone marrow failure disease, but instead something more easily treated.

I suggest that you get the free information packet from the Aplastic Anemia and MDS International Foundation (AA&MDSIF) and ask them to include information about both aplastic anemia and MDS, unless the doctor has already ruled out aplastic anemia.

[Cupcakegirl]

Hi Neil,
Thank you for your reply. My daughter had a bone marrow biopsy in October 2012.
The doctors she has seen (rheumatologist, neurologist, oncologist, hematologist, gastroenterologist) have ruled out everything under the sun it seems like. She does not have Anemia at all. She has a positive ANA so they thought she had Lupus. But they have done so many tests to rule that out.
They have ruled out Epstein Barr virus. They have ruled out the bacteria.
And allergies. The only thing I have not asked them to test for is chemicals. But I dont know who to take her for that.

Her bone marrow biopsy has concluded that she has a Hypocellular bone marrow with decreased trilineage hematopoiseis. I dont know what that means. It says dyspoiesis in all three cell lines. The pathologist notes say there are adequate numbers of myeloid percursors present and the possibility of this representing the early stages of marrow failure syndrome cannot be excluded.

I dont know how to read the rest of the report. Her hematologist is so crowded that I waited 2 hours and didnt get to see the doctor to ask questions last time we were there because she had 90 patients and was the only doctor on call. The specialist was super flustered. (as was I because she wouldnt give me the time of day other than tell me to reschedule and take my co payment) They dont know what is wrong with my daughter and she keeps getting fevers, cant make it through the day at school, and looks pale. They tell me to get her blood work repeated every month and when we go to the doctor it is just the same thing, come back in a month and we will see if she is better or not.

All we know is that she has low WBC 2.2 and that her lymphocites 1137 are also low Neutrophils 774 are low and her monocytes 167 are low. This has been up and down since February 2012.

I have contacted City of Hope with (no reply yet) because no one can tell me why...they say she has had a virus for almost a year, (which she has not even been sick but once) and that it will go away, but she keeps getting tired, keeps getting these phantom headaches, fevers, body aches etc. She has had a MRI of her brain for the headaches with no conclusion besides a cholesterol granuloma which they said is normal and every single blood test known to man. She was passing out giving blood because they were doing it so often.

thanks again Neil for your reply.

[evansmom]

Hi there,

This kind of sounds like a slow smouldering aplastic anemia to me. If your daughters bone marrow biopsy only showed a trilineage decrease in cell precursors, and no abnormal cells, chromosomes, etc., I can't see at this point how it could be MDS. The only other thing is that it is abnormal to have a positive ANA, and Lupus is difficult to diagnose and often goes undiagnosed or improperly diagnosed for years. Of all the specialists your daughter has seen, it appears that you haven't gone the Immunologist route.
Perhaps this would be worth pushing for given the positive ANA.

If this is indeed a slowly developing aplastic anemia, and your daughter's hemoglobin and platelets are at an acceptable level and, most importantly at this point, her neutrophil (ANC, granulocytes) count STAYS ABOVE 500 CONSISTENTLY, then routine blood work, a BMB in the not too distant future and the 'wait and watch' approach is appropriate.

If the doctors truly believe this is a virus that will eventually go away, then surely they have done complete viral panels that would have revealed elevated copies of something. What was elevated??

The only other thing I can think of at the top of my head that you could discuss with the hematologist is a medication trial of acyclovir (to address this virual theory) or a trial of Lupus treatment. I'm not sure these approaches would be wise for fear of dropping the other counts, but given this has been going on for 11 months, it's worthy of discussing, even if it is unconventional.

Best of luck and keep your daughter away from damp, dark potentially moldy places, non-domestic animals, birds and any place that would harbour bird droppings such as window air conditioning units in tall buildings where you'd likely see pigeons, etc. perched. Review on a reputable web site the risks for fungal infections in a neutropenic patient. As far as I can see, this is where your daughter's biggest risks lie right now.

Take care and keep us posted.

[Cupcakegirl]

Hi Nicole,
Thank you for your reply. I am going to write all of this down and ask the specialist at our next appointment with them.

Hanna has had Viral Panels, and nothing came up when they did those, except they said she did at one point have the Epstein Barr virus, (which everyone gets at some point in their lives) I feel like they are just telling me it it a virus because they simply do not know why she is feeling this way and why her blood counts are low.

As for Lupus, They have done every single lupus test, even testing that they do only for people with lupus, and all of it has come up negative.

Someone said she could have Lyme disease, because that is almost impossible to diagnose.

I really wish I had some kind of answers for her. I just feel like if it IS a virus, why hasn't it gone away after almost a year? Seems odd to me.

Her Hematologist said her bone marrow is only working at 10%.
I dont know anything about Anemia, but her RBC has never been low except one time. and it is always on the low end of normal.

Thank you again for your advice and reply.

[Sally C]

Hi Cathleen,
Now that you mention Lyme Disease that was the first thing I though of after reading your post. I have had it twice - my doctor said it was a reactive test so it may or may not show the disease.
I had the body aches, headache, very fatigued - all the symptoms except for the characteristic bulls eye.
He decided both times to treat me with 2 weeks of Amoxicillin. He said I would feel better after 3 days of medication and he was exactly right. Maybe it wouldn't hurt for her to go through a cycle of Amoxicillin just to see.
It can be a very debilitating disease.
Best of luck and God Bless,
Sally

[Cupcakegirl]

HI Sally,
Did you ever get your blood counts when you had the Lyme symptoms? Were your white counts low like this? Sometimes Hanna has months where the wbc counts are higher than others. Thanks for your input.

[Sally C]

Hey again Cathleen,
I did not have any blood work done when I had it. Actually my husband is the one who has MDS.
But it is my understanding that counts can be affected when one has an infection.
Good luck - I'm sure this is quite upsetting.
Take care,
Sally

[julieluvsmarco]

Hi I was just browsing and found ur thread.my daughter Marie had aplastic anemia and the way she started off showing symptoms is very similar to what u are experiencing.we were seen in so many children hospitals till we were fully diagnosed at Miller childrens hospital in long beach the hematologist there are just amazing they are very helpful and are very informed with children blood disorders ..

[Heather8773]

Cupcakegirl

Has she had a flow cytometry test? If not I think it's worth looking into

[Cupcakegirl]

Hi Heather,
No she has not had a Cytometry test yet.

We just took her to City of Hope and they agree with her other hematologist it could be a virus still (that has lasted for one year) or now they are saying she could have the symptoms of developing aplastic anemia. They are doing the watchful waiting. Because her red blood cells and her platelets are stable most of the time they dont know what she has. Her white blood count is always super low. They dont know why. One doctor suggested because they think my daughter could be depressed. That comment made me super mad. She is a very happy child and never complains about anything. (she is an identical twin) and her sister would tell me if she was upset or sad.

My daughter just tells me that her body is tired all the time. And she has headaches all the time. The doctors are telling me a low white count would not make her this tired. I don't know what to believe anymore. It has been a year of no answers and no help. We are told to take her to get blood work when she spikes a fever. She has thrown up blood a few weeks ago and they acted like it was no big deal.

I was told to not worry about my daughter and that this will probably go away or get worse. And they wont treat my daughter with anything till they know what is causing the low counts.

I think more and more about going to a holistic doctor because I dont know how to get my daughter playing with her friends again and running with her twin sister. She naps after school and is so pale like a ghost.

Thanks for all of your input.

[evansmom]

I am reminded of a young man in my neighbourhood who, for a number of years, had fevers that came and went in cycles. He'd be well for three weeks, then bedridden with fevers, fatigue and headache for one week. This would go on and on. He also could not get a diagnosis. He travelled with his fiance to Mexico for vacation (in my opinion, no one should travel outside their country when they suffer a mysterious undiagnosed illness) and sure enough, he became very ill and was hospitalized down there.
To make a long story a bit shorter, at the time he became acutely ill, his body was riddled with an etremely high number of copies of the EBV which then led to an immune dysregulation disease called hemophagocytic lymphohistiocytosis (HLH).

There are other things to consider as well: Cyclic Neutropenia, or perhaps low IgG, IgA and /or IgM levels.

[Hopeful]

Hi Cupcakegirl,

Just a random thought to add to the mix...

Since your daughter is only 9, do you remember her getting EBV (mono)? I remember getting it in high school and being extremely fatigued for months and months. Can they tell if she is recovering from a recent infection of EBV by the panels?

[Heather8773]

Cathleen

I really hope you get a dx soon. Maybe wait on holistic until you have a dx.


Your her mama and you sound like an amazing one it takes a lot of strength to keep fighting when you keep being told things "will either get better or worse"
I had some really good advise that keeps poping in my head for you
Your her mom and you know what's normal for her and what's not. If you have that little voice/gut feeling or things just are not adding up. Listen to it.
I'm am sure it's incredibly hard to make the choice to get more painful test for your baby girl when you DRs are hopeful it will disapear.
Have you contacted a specialist in bone marrow disorders in your area?
You are in my prayers.

[Cupcakegirl]

We have moved out of our townhouse and to house a different city because well I have 5 kids and we needed a larger space.

Hanna's Blood counts have been increasing towards the normal ranges since we have moved. I believe it had something to do with the mold and the tenting we had done in our townhouse. I think the air there is toxic.

Hanna is still experiencing Hip and bone pain. and Many headaches. So I am not going to write all this off yet. Even though on paper she looks like she is getting better, and she is feeling ok for school, She is having major hip pain. Since she is an identical twin, I dont think this is due to growing pains, because usually they have the same "growing issues" or pains.

I am thankful for the bone marrow biopsy getting put on the shelf for now again, because the last one was horrible.

Thank you all for your kind words, and encouragement. Life is a journey...isnt it?

[Cupcakegirl]

My daughter is still having extreme hip pain even when she is swimming or running. So they did another EBV pannel on her and they are positive and they both say High.

The EBV VCA IGG is 3.83 HIGH
and the EBNA IGG is 5.00 HIGH.

I dont understand why they would do this if they think it is Aplastic Anemia, or if they think it could be a leukemia. Her ANA is positive and her pattern is speckled at 1:80. I dont like it that she is still having leg and bone / hip pain.

Any info that I could gather to ask the doctors about this would be great. I appreciate everything everyone posts.

[Cupcakegirl]

Hanna is going in tomorrow for another bone marrow biopsy, her blood counts dropped again and some of the other tests were wacky. She is scheduled for a MRI. She is scared. I am scared to do this again, last time it was not pleasant.
Wish us luck.

[Neil Cuadra]

Cathleen,

I wish they could give you definitive answers and stop the hip pain, but for now I just hope you and Hanna can get through this appointment.

If the previous bone marrow biopsy was painful, make sure the staff knows it and they use enough painkillers this time. An MRI can be disorienting and very noisy. They have medicine that can reduce anxiety if you want to ask about it.

Hanna will probably take her cues from you, so if you're brave and confident about getting through the tests, she may be too. But I guess you've been through plenty of medical exams and tests already, so you probably already know the best ways to help your daughter. Good luck.

[Heather8773]

Cathleen
Thank you for updating us. I really hope they have the answers this time... I think the worst is waiting for the unknown.
Neil had some great points. Hopefully they can give her something for pain this time!

[Cupcakegirl]

Thanks for your tips Neil & Heather, I asked for something stronger for her and They were gracious to give her something for the pain that has been helping. Thank you again for your suggestions.

We should find out Friday the results of the biopsy. Her clinic is so busy but I like the fact that her Doctor apologized to her yesterday for not knowing what is going on with her...he promised to get to the bottom of it with another doctor...he was telling her that she is a little mystery.

I am having trouble getting the MRI scheduled, My insurance is an HMO and I am blessed to have that, but it is a all day process to get anything done right.

Both Hanna and I are exhausted, She has been sleeping much of this day already, I think sleep is the best, poor girl.
(I have 4 other children to run to school, etc so I am thankful she is resting)

I will post again sometime when we find out what the results were.
Thanks for "reading"

[Cupcakegirl]

Biopsy was not conclusive. AGAIN.
Her Doctor Personally called me this evening said her biopsy was NOT normal, but her cellularity numbers are slightly better from last time.... so that part is encouraging. What isn't encouraging is that Hanna has to have another bone marrow biopsy (#3) in a few months and continue to see the specialist if her blood counts stay stupid like this. We still have to schedule the MRI. Waiting on the insurance for that. I need to make an appointment with her Pediatrician to find a different rheumatologist. ....why cant this just be over with? UGH. I have been crying since talking with the doctor about this. I just want my baby well. This is not fair to her!

After talking with my husband, I think we are done with the Biopsies on our sweet girl. We are not letting them do anything further unless there is a severe change in her blood work from now. I am going to talk to her pediatrician and request all the rest of her blood work that I don't have and the MRI and biopsy report when we are done with that this next week. The last year for Hanna and our family has been Hell with all the doctors visits and I just want her to not have to worry. Since the bone pain is not explained other than they know "something is wrong" but they don't now "What" .....I feel lost about how to help her. Her condition is labeled "chronic neutropenia" for now.

Today has been so super emotional....I am exhausted. Thankful for Easter Break..... I just wanted to hear she was all better and that we can stop this madness.

[Sally C]

Dear Cathleen,
I am so sorry for what your Hanna is going through. It was very difficult to get a firm diagnosis for my husband's MDS. We finally went to The National Insititutes of Health in Bethesda, Md. After 6 months of trying to get a diagnosis, The NIH diagnosed right away. They have a wonderful children's building for families to stay in and are cutting edge in blood disorders. All medical tests/treatments are free to the patient. I understand MD Anderson in Houston is very good also.
I wish your Hanna well.
God Bless,
Sally

[Cupcakegirl]

Hanna's Monocytes are High, Her WBC is low at 2.2. Sometimes it fluctuates. I feel like, do I do anything with this or leave it alone and let her live a normal life? They tell me they dont know what is wrong....City of hope said that some people just live with low counts.... Thank you so much for your help and your suggestions. I may contact that hospital.

Blessings to you all and your families....
Cathleen