2 year old and 9.8 hb

Hello!  My son just had his 2 year appointment with a new to us pediatrician, we recently relocated to another city.  The new pediatrician had my son do a finger prick to check hemoglobin and lead levels, and his hb came out at 9.8 twice.  The  person doing the finger prick said they would want to do a vein draw to check for iron deficiency.  I explained I have beta thal minor and my son gets a supplement with iron, so I was doubtful it was iron deficiency and more likely Thal.  My son is not a huge fan of meat, but does eat some, so at his 18 month appointment I had asked my original pediatrician about his iron level, and he checked his eyelids and said his coloring looked good, development was good, and said if I was worried about it to give him an iron kids multivitamin, which I have been 4-5 times a week.  I wanted to wait to test my 2 year old until he could sit better for a blood draw and understand what is going on.  My husband does not have thal, or any other known blood disorders.  My original pediatrician didn't do any finger pricks with either of my boys, and based things on the physical exam.   Would 9.8hb be pretty common for a 2 year old with beta thalassemia minor?  My son is developing appropriately, and has no other concerns.  Should I just do the vein draw?  He screamed the entire appointment and I had to hold him down for the exam, shots , and finger pricks:(. Any other parents with potential beta thal minor kids?  When did you test?

Thanks for any words of wisdom,
Crissy

I don't think the test is necessary. He's already taking iron. His Hb is near the low end of the range for thal minor, so he may also be a bit iron deficient. Has he been taking iron for long? What type of iron is he taking? I really think all it will take is making sure the iron deficiency is corrected, if it is present. If the Hb does not improve after 2-3 months, he probably isn't deficient. If the Hb improves, the iron should be continued until the Hb stops rising.

Hi Andy, he takes Poly-Vi-Sol, a liquid multivitamin, which has 10mg of iron (ferrous sulfate) in it.  He's been taking that for the past 6 months.  I was giving that to him 4-5 times a week, but could bump it up to 7 days per week and see if there's any improvement.  Would there be any concern over giving him iron if it is thal minor alone? 


Thanks for your help!
Crissy

It's a fairly low dose of iron, so it shouldn't be a problem. Has it affected his Hb at all since he started taking it? I think daily would be a good choice right now. Is anything known about his thal gene and if it's beta zero?

I do not have any background on my families thal gene, other than I was tested when I was 11 for Von Willibrands and thal after 6 years of being told I was iron deficient.  The doctor said I had beta thal minor, but not Von Willibrands.  My thal side of family is Sicilian in background.  Is there any testing I could request on myself that could help for gene information for him?  I have always had symptoms like fainting, fatigue and a poor immune system, as have my mother and grandmother.  My Hb is typically 11.  I was told my grandmother had Thal intermedia. 
This was the first hemoglobin check he has had.  My previous pediatrician did not do blood tests unless he had a concern with the physical exam.  I started giving him iron after his 18 month visit because I was worried he didn't eat very much meat, and my original pediatrician said it would be fine to give him. 

Thanks Andy!
Crissy

Crissy,

A DNA analysis would tell you the beta thalassemia mutation that you both have. His would be the same as yours. Diagnosing Von Willebrand's is difficult. Thal minor can be diagnosed in most cases with a complete blood count and a hemoglobin electrophoresis test. Do you know if these tests were done and that's why your diagnosis was changed to thal minor?

Andy,
I'll see if I can get a copy of my medical records from my hometown.  I know I was tested there first and failed a blood clotting test. I was then referred to a specialist in a larger city and they were the ones that diagnosed thal minor and ruled out Von Willibrands.  I believe there was electrophoresis, CBC, and platlet count testing, I'm not sure what else was done.  My paternal grandmother was diagnosed with Von Willibrands around that time, and her doctor had said anyone else who got frequent bloody noises and bruised easily in the family should be tested, so that's why my parents took me in for further testing, which I'm glad they did since I was not iron deficient and the iron supplements made me sick!  Hopefully my records will have the  DNA analysis, otherwise, I will request testing so I can get more info for my son.  I sure appreciate your advice.  I'll l you know what I find out!

Thanks,
Crissy

An update, I haven't had any luck with DNA analysis for my thal gene to see if we are beta thal zero.  I have asked my primary and I mentioned it to the pediatrician, but I think it is not something that is commonly done around here.  My son had a vein draw 3 weeks  ago.  I got a verbal report of Hemoglobin 9.8 and Ferritin 33.  She said his iron binding capacity was normal, but she wanted to consult a pediatric hematologist.  We just heard back that the hematologist agrees with the thal diagnosis, and only recommends iron if he is a poor eater, which he is.  Is there anything else I can give him supplement wise to help with his hemoglobin?  He's been sick frequently the last few months; pneumonia, bronchitis, stomach bug,  lots of fevers, and he has been saying he is tired. He just turned 3.  I have consistently been giving him Poly-Vi-Sol with iron.  Thanks for any suggestions you have.  I truly appreciate this group:).

At his age, it's not easy to give a lot of supplements, so a good multivitamin with minerals would be a good addition. When he is a bit older, you might want to try some individual supplements, like folate and vitamin E. A liquid vitamin D supplement might be easy to give and is advised, as it helps with tiredness and improves immune system health. Sometimes kids will go for smoothies and you can get a lot of healthy fruits and veggies into a child with smoothies. It's often a challenge to get thal minor kids to eat well, so any creativity that may get him to eat is recommended.

I wouldn't stay with the iron too long if his Hb doesn't rise. Long term iron supplements can eventually cause health issues and even damage to organs in thal minors, as iron will build in the body. It's not a good ongoing practice for thal minors.

Thanks for the supplement suggestions Andy!  I just got my son's lab numbers, does this look pretty typical for a beta thal minor toddler (3 year old)?

Component                  Your Value           Standard Range
White Blood Cell Count   5.88 K/uL         5.50 - 15.50 K/uL
Red Blood Cell Count           4.82 M/uL        4.00 - 5.20 M/uL
Hemoglobin                   9.5 g/dL        11.5 - 15.5 g/dL
Hematocrit                           29.7 %       35.0 - 47.0 %
MCV                                   61.6 fL        77.0 - 95.0 fL
MCH                                   19.7 pg        25.0 - 35.0 pg
MCHC                           32.0 g/dL       32.0 - 36.0 g/dL
RDW                                   33.4 fL       37.0 - 50.0 fL
Platelet Count                   326 K/µL       150 - 440 K/µL
MPV                                   10.9 fL       8.0 - 13.0 fL
Absolute NRBCs                   0.00 K/uL       0.00 - 0.01 K/uL
nRBC                            0.0 #/100        0.0 - 0.2 #/100

TIBC                                    321 ug/dL      260 - 445 ug/dL

Component                  Your Value                     Standard Range
Iron                                   34 ug/dL                      60 - 160 ug/dL

Component                   Your Value                    Standard Range
Ferritin                           36.3 ng/mL                 22.0 - 274.0 ng/mL

Component                          Your Value                 Standard Range
Neutrophils Relative Percent   39.0 %                    32.0 - 52.0 %
Lymphocytes Relative Percent    56.0 %                    40.0 - 60.0 %
Monocytes Relative Percent            5.0 %                    1.0 - 11.0 %
Neutrophils Absolute                    2.29 K/UL                    1.76 - 8.06 K/UL
Lymphocytes Absolute   3.29            K/UL   2.20            - 9.30 K/UL
Monocytes Absolute                     0.29 K/UL               0.10 - 1.24 K/UL
Total Count                              100     
RBC Comments                          Microcytic/ Hypochromic     Normocytic/ Normochromic, Red cell morphology consistent with age
Platelet Verification                 Platelet count adequate     
Polychromasia                            0-1/HPF /HPF                 0-1/HPF /HPF
Elliptocytes                                    Rare                       None Seen
Schistocytes                                    Rare                       None Seen
Target Cells                                    Rare                       None Seen

Thanks,
Crissy

It does look normal for beta minor, although the Hb is a bit lower than typical. This could be related to diet, and at that age, it can be very difficult to get a child to eat enough nutritious foods. I always suggest that you find some smoothie recipes that can include fruit and raw veggies. Most kids will drink a good tasting smoothie. I would also suggest a B-12 supplement, as it may help raise the Hb a bit. At his age, a liquid supplement would be best.

Thanks Andy, that's a relief.  I was worried he might be iron deficient even with the supplementation we've been doing. I will try some smoothies, he likes Naked Juice so at least that's a start.  On a positive note, he's now eating a little meat and managed to gain a pound.  I really appreciate your advice, and I am so thankful there is a place for me to connect with other thals and be able to ask questions:)

Crissy

Hi Andy,
I found out my son and I are beta-zero by doing 23 and me testing and loading the raw data into Promethease.  I tried looking up beta zero, but beta zero sickle cell kept coming up in my searches.  I'm mainly just wondering if there are any good articles I can read on this, and if there are any implications health wise for me and my son? 

Thanks,
Crissy

Beta zero means that globin gene produces nothing, so the one good gene is left to try and produce enough beta globin to make sufficient hemoglobin, which it cannot. In thal minors, it most often means a lower Hb and more need to address the nutritional aspect of thal.