Thalassemia Patients and Friends

Discussion Forums => Thalassemia Minor => Topic started by: TKDgirl7 on January 26, 2019, 10:20:41 PM

Title: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on January 26, 2019, 10:20:41 PM
Hi,
I was looking for advice for improving my son's hemoglobin and iron.  I feel like I am failing because he is such a picky eater.  We tried shakes, we tried multiple introductions of foods, he gags and cries or flat out refuses to eat and he has slowly lost weight since the start of the school year.  We also do Spatone iron 3 times per week and smarty pants multi vitamins. I had him back to the doctor last week to have his blood checked as he is always cold and falls asleep frequently.  Here are the labs I got back if anyone has any suggestions I would appreciate it.  I still have to connect with the doctor on Monday to talk through the results. 

Differential 5 part Manual
Neutrophils Manual  57%
Lymphocytes            32%
Reactive Lymphs      4.0%
Monocytes Manuel    6%
Myelocytes Manual   1%
Neutrophil Absolute   4.02k/ul
Lymphocytes Absolute  2.26k/ul
Reactive Lymph            .3 k/ul
Monocytes Absolute      .42k/ul
Myelocytes Absolute      .07k/ul
Anisocytosis                  Slight
Poikilcytosis                  None Seen
Polychromasia              0-1/HPF
Microcytes                    Slight
Macrocytes                   Slight
Elliptocytes                   Slight
Schistocytes                  Slight
Target Cells                   Slight
Stomatocytes               Slight
Platelet Estimate           Normal
Hypochromatic Cells      Normal

CBC with PLT
white blood cell count   7.06k/ul
red blood cell count      5.14m/ul
hemoglobin                  9.7g/dl
hematocrit                    31.6%
MCV                            61.5fl
MCH                            18.9pg
MCHC                          30.7 g/dl
RDW                            32.5fl
Platelet count                390k/ul
MPV                             11.4fl
Absolute NRBC's            0k/ul
nRBC                            0

TIBC                           315ug/dl
Ferritin                        41.2ng/ml
Iron                            30ug/dl


Thanks for any support you can provide!
Crissy
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on January 29, 2019, 06:02:00 PM
An update, our pediatrician is referring us to a pediatric hematologist.  Since there are none in our area we are being referred out.  Hopefully we can figure out what’s Thalassemia and if there’s anything else going on. 
Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on February 16, 2019, 08:57:13 PM
I don't know if iron is much of a problem for him. His numbers are in normal ranges. Discuss this with the hematologist.

Smarty Pants vitamins are excellent quality, but he may need more folate (so glad to see folate on the label, rather than folic acid). You may be able to find a liquid folate that is easy to take. Something like this with bot folate and B-12 may be helpful. https://www.amazon.com/Designs-Health-Liquid-Natural-Servings/dp/B0041RBW1E   I would suggest a drop 2 times daily. I would not suggest it after dinner, as the B-12 may keep him up.

I hear so much from parents about kids being very picky eaters. Keep experimenting with nutritious foods and see what he will eat. Also, talk to him and ask him which foods he likes. Will he eat yogurt? It may help with his digestion and make eating a more positive experience for him.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on February 18, 2019, 12:44:20 PM
Thanks Andy, I will look into a liquid folate.  We love Smarty Pants vitamins, when I did 23 and me testing I found out I have a MTHFR mutation so I scoured all over to find a prenatal with folate for my third baby.
I’m hoping the hematologist can help or refer for the food issues, they seem to be sensory related.  He is cold and tired and pale everyday.  He goes to preschool 3 days a week and that really wipes him out.  He usually falls asleep for 2-3 hours after even though he gets a nap there too.  I was curious if you knew if it was typical for a child to have lower hemoglobin than the parent with Thal?  I am usually in the 10.8-11.5 range, but the  three tests my son has had have shown him  at 9.7-9.8.  Our appointment is this Friday, I’m hoping it will go well!

Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on February 19, 2019, 04:03:10 AM
Oh also, Andy, would you have any good resources on beta thalassemia zero mutation?  I’ve been googling to try to find Hb ranges or more information about moderate anemia in Thal minor, but I’m not getting anywhere.  Thanks!  I appreciate you sharing your wealth of knowledge! 
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on February 22, 2019, 08:51:38 PM
An update, we had the appointment today.  The doctor was great.  We are officially testing my son for thalassemia, it’s always been suspected/assumed but not confirmed.  She’s also doing genome testing and a test for immune level.  She confirmed that it would be expected he and I would have anemia symptoms.  She also said she would write a letter for his school so he can have modifications to physical education and so they can understand his anemia may limit some things.  It was such a relief to talk to someone who acknowledged there are symptoms with Thal minor.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on March 01, 2019, 04:19:31 PM
One more update, the tests are all back.  It is confirmed that he has the beta Thal zero mutation.  His ferritin also dropped to 19 so we will be starting a 3 month treatment of iron.  We have also been referred for genetic counseling which should be very insightful.  My son will now have annual check-ups with the pediatric hematologist.  I’m very glad we made the trip to see a specialist.  Also, I got the liquid folate in the mail yesterday so we’ll be starting that too.  Hopefully between the iron and the folate my son will be feeling a little more energetic soon.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on March 03, 2019, 05:41:30 PM
It is quite common for the symptoms of parent and child thal minors to not be the same. This is usually the result of other genetic modifiers and can affect the total hemoglobin level.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on March 03, 2019, 05:51:13 PM
It's great to have the proper diagnosis.

I do not know of any good source about thal minor or beta zero thal minor. There just has not been enough study of thal minors. It would be nice to match reported symptoms with the genotype of thal minors. My experience tells me it would show that beta zero minors do have more symptoms that beta+ minors.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on March 04, 2019, 08:08:33 PM
This is good to know. I am sad he may always be in the 9 range, as it does impact his energy level.  I worry how taxing school is going to be for him.  His brother is very athletic, I think that will be tough too.  It is good to have a diagnosis at least we can explain things to him.  I remember being so frustrated as a child getting sick all the time, and not being able to keep up with my sister, but I didn’t get a diagnosis until I was older, and the I was told it should affect me in any way.  I am glad there is more information on the various mutations now.  I would love to see research on the mutation types and symptoms experienced too.
It is quite common for the symptoms of parent and child thal minors to not be the same. This is usually the result of other genetic modifiers and can affect the total hemoglobin level.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on June 25, 2019, 04:31:13 AM
A three month later question...my son has not responded to the NovaFerrum prescribed for his iron deficiency.  His ferritin is now 18.7 after taking the supplement every morning.  His hb has not changed either.  He is still a very a very picky eater, but since he’s been home me for the summer I’m able to maintain his weight with frequent snacks, just not the kind that will boost his iron..  I had him on Spatone iron water before, but was told it was not high enough in iron to get him back where he needs to be.  I had a difficult time absorbing iron during my pregnancies is this a Thal thing or something else going on?  I’m worried he’ll need infusions and he’s terrified of needles, my husband and three hospital staff had to hold him for the last blood draw:(. Any suggestions to help him out? 
Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on June 27, 2019, 10:12:03 PM
Was he on the Spatone long enough to see if it was raising his ferritin? Even though the ferritin is at the low end of normal, it's not terrible and the other iron numbers are in range.
As far as snacks with iron, will he eat dried fruit, like raisins or apricots?

I do think being a beta zero thal minor is more of the source of his low Hb and low energy than iron is. If he will take a sublingual B-12 tablet, it may help his energy some. They are small, taste sweet and melt under the tongue and are very easy to take. I take them myself several days a week when I have more physical work to do.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on June 29, 2019, 03:55:38 PM
Hi Andy,
He was was on the Spatone for around two years (1 packet 3 times a week), during that time he had a couple of blood checks, but was in the 30-40ish range for his ferritin when he was checked.  Around December, I requested testing because he became noticeably more pale and even more fatigued than usual, along with weight loss.  That test showed his ferritin was still in normal limits, but by the time we met with his Hematologist in February it had dropped to 19, which was when he was prescribed the NovaFerrum. So now he's still has symptoms and hasn't improved his Ferritin even with the 3 month prescribed treatment.
The hematologist called me back and is recommending he do one iron infusion to see how he responds, she was concerned about giving him iron because of his thal, so he will only do one and see if his energy improves.  She is also recommending he see a Gastroenterologist to see if there might be some bleeding that is causing the iron deficiency. 
I'll definitely try some dried fruit, thank you!  He's also been getting super liquid folate each day from your recommendation earlier.  Hopefully with the summer off to relax we can focus on his nutrition and more rest he will regain some energy:). 
Here are his most recent labs:
Component                         Your Value             Standard Range
Ferritin                                18.4ng/ml            22-274ng/ml
Reticulocyte Count %            1.9%                   0.5-2.0%
Reticulocyte Count Absolute   0.096%               0.026-0.095 M/uL
Immature Retic Fraction        13.2%                 2.3-13.4%
Reticulocyte Hemoglobin       22.3pg                 24.1-35.8 pg
White Blood Cell Coount        5.41 K/uL            5.5-15.50 K/uL
Red Blood Cell Count            5.09 M/uL            4.00-5.20 M/uL
Hemoglobin                         10.0 g/dL            11.5-15.5 g/dL
Hematocrit                           32.3%                35.0-47.0%
MCV                                    63.5 fL                77-95 fL
MCH                                   19.6 pg                25-35 g/dL
MCHC                                 31.0 g/dL             32-36 g/dL
RDW                                  33.8 fL                 37-50 fL
Platelet Count                     312 K/uL              150-440 K/uL
MPV                                   10.7 fL                 8.0-13.0 fL
nRBC                                  0.0                      0.0-0.2 #/100
Absolute NRBC's                   0.00 K/uL            0.00-0.01 K/uL
Neutrophils Relative Percent    30.0%               32.0-52.0%
Lymphocytes Relative Percent  57.9%               40.0-60.0%
Monocytes Relative Percent      9.8%                1.0-11.0%
Eosinophils Relative Percent     1.3%                0.0-6.0%
Basophils Relative Percent        0.6%                0.0-2.0%
Immature Grans Relative Percent .40%             0.00-0.42%
Neutrophils Absolute                   1.63 K/UL      1.76-8.06 K/UL
Lymphocytes Absolute               3.13 K/UL        2.20-9.30 K/UL
Monocytes Absolute                  0.53 K/UL        .05-1.70 K/UL
Eosinophils Absolute                 .07 K/uL          0.00-0.93 K/uL
Basophils Absolute                    0.03 K/uL        0.00-.30 K/uL
Immature Grans Absolute          0.02 K/UL       0.00-0.03 K/UL

Thanks Andy!
Crissy




Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on July 06, 2019, 06:10:49 PM
You can try the iron infusion and see if it helps the symptoms. I don't think the ferritin measurement has much relevance in light of the other iron numbers being in normal range. If there is a significant increase (at least .5) in Hb after the infusion, you can assume the iron helps. If the Hb doesn't really move, iron isn't helping.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on August 17, 2019, 03:35:13 AM
Hi Andy,
We did end up doing the infusion and have seen a marked improvement in his energy and mood.  His ferritin is now a 56 and his hemoglobin moved up to 10.5.  The results will be reviewed by the hematologist and then we are going to see gastroenterology to see if there’s anything going on there. 
We also had a CBC done for our 8 year old son; he’s never had a blood draw, his first pediatrician said they weren’t necessary.  His hemoglobin came out at 10.9, I was shocked, he has never shown any signs of anemia.  We are going to check ferritin and then do testing for Thalassemia.  I’m just so surprised one son is so much more impacted than the other. 
Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on August 17, 2019, 07:08:57 PM
There can be other genetic factors that moderate the effects of thalassemia, so one child may have a factor that helps, while another does not. One of the most common modifiers is carrying both alpha and beta thalassemia. One of our moderators has a beta zero major son. Both parents carry thalassemia. The mother is a typical beta minor with low Hb. The father also carries alpha minor, and his Hb is above 14 and he runs marathons. Both parents carry beta zero, but in the father, the presence of the alpha thal means that the two types of globin are produced in similar amounts, whereas, in beta zero alone, there is a large deficit of beta globin, creating an imbalance with the alpha globin and it is this imbalance that is responsible for many of the problems of thal minors.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on August 20, 2019, 03:46:24 AM
That’s really interesting!  I’m not aware of any alpha thalassemia in our family, but I don’t think anyone has been tested either.  My guess is something from my husbands side must have benefited my older son.  Both the boys will go to the hematologist for follow up in a couple of weeks.  My daughter had her blood checked and was in normal range for a one year old, so I’m optimistic she does not have Thal.  We still have to figure out the iron for my middle son, I was reviewing my numbers and I run on the low end for ferritin as well, so I’m wondering if we just don’t absorb it well? 
Title: Re: 4 Year Old Beta Thal zero advice
Post by: Andy Battaglia on August 21, 2019, 11:34:17 PM
I have theorized that the reason thal minors often have low serum ferritin is because iron is kept in use by the constant overproduction of red blood cells. Since iron is recycled and not eliminated by the human body, the amount of iron present would still be the same, but it would not be concentrated in ferritin, but rather it will be found in the constant use and recycling. Doctors get alarmed about low ferritin, but the iron bound by ferritin is what your body is currently not using. I don't think in the context of thal minor that ferritin is a very useful measurement.
Title: Re: 4 Year Old Beta Thal zero advice
Post by: TKDgirl7 on August 24, 2019, 01:33:40 AM
That’s an interesting theory. I know I really struggled with my iron during my pregnancies, but otherwise I think I do okay even though I’m on the lower side of normal.  I’ll be curious how my oldest’s numbers look, he eats meat unlike my younger son.  His platelets were slightly higher than normal too, which my younger son’s are not.  I will be relieved once all of this is sorted out.