rare dominant (sorry, long post)

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rare dominant (sorry, long post)
« on: December 01, 2016, 06:26:21 PM »
I have two randparents from Sicily. My dad had Thalassemia Beta Trait, I and all of my siblings have it (4 total), my three children have it. This does not fit the typical pattern, so I have been wondering if we might have the rare dominant form.

I have some questions and am extremely appreciative for any guidance offered!

How to get genetic testing? Would testing one of us be sufficient to diagnose, and if so, which generation? My two grandchildren?

I have high blood sugar, but not diabetic levels, which is actually easy to control by avoiding sugar and carbs. What is the relationship between high blood sugar and Thalassemia? Beta-something?

I especially want to help one grandchild (10) who is very overweight, and I would love to be able to help her normalize her weight as it seems to be affecting her ankle bones. She also has diagnosed brain issues, anxiety, panic attacks, tantrums, even bedwetting, as she just can't wake up once she falls asleep.

My mother, who did not have it, used to say her kids all had tired Italian blood, as she was used to seeing more active children than we were. My children also exhibit fatigue, they are all in their 30s, and cannot wait for the weekend to sleep in, even though they are all sports active. Not me, all I can do anymore is walk.

I have so many issues from head to toes. Over the years (63) I have had what I feel are too many medical conditions. Foot pain (diagnosed plantar fasc.) Shin splints if I walk without warming up with a long period of slow walking (had this in chilhood and teen years, not age nor weight related.) Childhood inability to keep up with other kids in running and sports, I was miserable in hockey and lacrosse, ok in gymnastics. Joint pain and muscle pain especially if I overdo exercise. Very painful cellulitis on upper thighs and hips. Kidney stones. GERD. Several occasional extreme gastric pain episodes with visits to the ER, unexplained by gall bladder problems, there were no stones.  Unexplained abdominal pains. Cardiac vasospasm (recorded during an angiogram.) Angina. Overdoing physical activity causes typical heart symptoms, headache, angina, left arm pain, etc, but no enzymes. Minor heart arrhythmia. Cerebral vasospasm. Dizzyness. Sleep apnea with CPAP treatment. Fibromyalgia and myofascial hardening. Early stage osteoporosis. New last year is mild asthma. New this year is high blood pressure, which began after a cardiac episode in August, and since then exercise intolerance, (cannot push through it, get faint, nausia, headache.) And the aforementioned high blood sugar. Very short stature, the only person in my elementary school grades and in high school who was shorter has dwarfism. My kids are shorter than predicted too, well below the 10% line on the growth chart. Fortunately, they are all intelligent, and responsible adults. So, is it possible some of these are Thalassemia-related?

I went to a local "blood dr" last year wanting to finally nail down the cause of my fatigue, as the CPAP helps, but didn't cure it, who basically read me a textbook description. I already know I have Thal B from testing done during my first pregnancy, and fetal compensators. This doctor ignored my question about the dominant pattern I wonder about in my family, on both expensive visits. He said I have enough iron, no treatment needed, Thal B is symptomless.

Again, thank you for any help with this. It will help 9 people in my immediate family, and possibly cousins too.

Re: rare dominant (sorry, long post)
« Reply #1 on: December 03, 2016, 04:47:46 PM »
no reply, moderator please delete, thx

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Offline Andy Battaglia

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Re: rare dominant (sorry, long post)
« Reply #2 on: December 03, 2016, 06:21:50 PM »
You do realize that this is a volunteer effort mostly run by one person who also administers several different thalassemia related groups on Facebook, and all for no charge. I also average about a 60 hour work week at my business, so I can't always get to the posts immediately.

If you do want help, before I can answer much, I need to know about your history in regards to iron. Have you ever taken iron supplements for more than one month at a time.

I can answer the DNA question. It really wouldn't matter which thal minor in the family was tested. From your description of symptoms, I think a DNA analysis is necessary to see exactly what is carried, as there are a host of health issues involved, and knowing what you are dealing with from the thalassemia standpoint could be a big help in determining whether or not some of these issues are related to thal or something else.
Andy

All we are saying is give thals a chance.

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Offline Dori

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Re: rare dominant (sorry, long post)
« Reply #3 on: December 05, 2016, 08:26:01 PM »
Yes, what about your iron levels? What is your nor,al hemoglobine?

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Offline Andy Battaglia

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Re: rare dominant (sorry, long post)
« Reply #4 on: December 08, 2016, 04:58:13 PM »
Sorry if I couldn't provide instant gratification. It's a shame anyone feels they are automatically entitled to that. We really have an impatient world.
Andy

All we are saying is give thals a chance.

Re: rare dominant (sorry, long post)
« Reply #5 on: October 29, 2017, 01:11:55 AM »
Hi Andy,

Sorry I didn't respond to your posts. I never checked back. I didn't mean to appear impatient with you at all, and was dismayed to read your comments today. Oh dear. I was just uncomfortable with my very long list of problems.

So to answer your question about iron, I was on iron supplements several times in my 20s. The doctor who first told me about my thalassemia prescribed it.

In 2015 I asked for a referred to a hematologist and here are the results from the blood screening he highlighted or underlined, there are two different reports, so there may be duplication.


Hematology CBC and Differential
RBC H 6.27
Hgb  12.4
MCV L 63.5

Flowsheet print request
WBC 7.4
RBC  6.45
Hgb  12.5
Hct.  40.5
MCV L 62.7
MCH L 19.4
MCHC L 30.9
RDW H 14.7
Platelet 261
(more things here, not highlighted)
Hemogloblin F 1.4
Hemogloblin S Not applicable
Hemoglobin A2 H 4.7
Sodium 140
Potassium 4.2
Chloride 103
CO2 24
AGAP 13
Glucose H101
BUN 12
Creatinine 0.7
BUN/CRE Ratio 17
Calcium  9.5
Albumin 4.4
Total Protein 6.9
Globulin 2.5
Alk Phos 97
A/G Ratio 1.8
Bili total 0.4
AST 18
ALT 25
GFR *90
Iron 81
Iron Sat 28
TIBC 286
Ferritin 156.5
Comment:
"Elevated hemoglobin A2 suggests beta thalassemia trait. This is associated with microcytosis and absent or minimal anemia, but without clinical problems."

He said my elevated blood glucose is not related to thalassemia trait, nor are any of my other health problems.

Three months ago I was reading about Intermittant Fasting, and decided to try it. I have lost nearly 15 pounds. I keep a food diary in Cronometer, follow Varaday's Every Other Day diet (averaging 1000 a day), low carbs, ketosis, fasting 16 to 20 hours between dinner and breakfast, limiting carbs plus protein to a range of 60 to a maximum 100 grams per day (this keeps my fasting blood sugar down), no grains, no evening eating and using atkins/banting food lists. Yep, 9 things, 10 if I add walking.

During the fasting periods my blood sugar does some wild swings, so once again I was looking for reasons, thought perhaps the thalassemia does have something to do with my high fasting blood glucose and found my own post from last year.

I sincerely apologize for irritating you, and for another stupidly long post. Answer it or not, or delete the whole damn mess.

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Offline Andy Battaglia

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Re: rare dominant (sorry, long post)
« Reply #6 on: October 31, 2017, 08:37:30 PM »
Your iron numbers are within range now, so iron would not be needed.
If the doctor is monitoring your glucose level, make sure that the HbA1C test is not used, as it gives incorrect readings in thal minors and has no usefulness.
I would suggest taking a magnesium supplement before bed at night. It calms the body and makes it easier to sleep. I use it for restless legs and it's effects are almost immediate. It can also help if you have heart palpitations, as magnesium and calcium control the heartbeat. Testing will not tell you if you're deficient, as 99% of the magnesium in your body is not in the blood.
B12 may also be of help. Not only is it needed for energy and blood production, it was discovered a few years ago that B12 is essential for bone formation. Sublingual tablets work best.
Has your vitamin D been checked? My asthma has improved greatly since raising my D level above 40.
Andy

All we are saying is give thals a chance.

Re: rare dominant (sorry, long post)
« Reply #7 on: November 01, 2017, 06:29:29 AM »
Magnesium, B12, and Vitamin D, will do! Thank you for the advice.

 

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