Question for Andy...

Just reading thru the post...My 3yr old got MRI and T2* done in Children's Boston when she was 2.5 yrs old for baseline heart and liver iron. Since she is too young, she had to be given anesthesia.

Bostonian_04 i hope your little daughter is well.  It is good to have T2* done at a young age for a child especially for a parents peace of heart and mind and efficiency in addressing any issues.
Hoping she would not need anesthetic in future.

Emby 

I want to follow this up with information acquired at the NYC conference. I posed this question to Dr John Wood, who is very cooperative at every conference and he told me that the goal now is to do the first scan one year after transfusions begin. This will usually mean the child has to be sedated, but the benefit of discovering any early iron loading issues far outweighs any concerns over the child's reaction to having to be scanned. This is just one area where there is a constant evolution in the thinking about how to approach various issues in thals and it's great to hear the doctors talk about the progress being made in understanding how to best manage thalassemia treatment. I know everyone is anxiously awaiting a cure, but we do have to deal with what is and having these improvements makes a great difference in the management of thalassemia, which leads to better quality of life for thals.

Bostonian_04 i hope your little daughter is well.  It is good to have T2* done at a young age for a child especially for a parents peace of heart and mind and efficiency in addressing any issues.
Hoping she would not need anesthetic in future.

Emby 

Hi Emby,

Thanks, my daughter is doing well ..... she is scheduled for her annual CMR for T2* and liver iron (R2*) this December. Since she is a toddler, she would have to be anesthetized for the procedure this year as well. I guess this will be the case for the next few years as well till the time she grows up enough to lie still during the procedure ....

Bostonian

Hiya Andy,

Yes the final decision is with the patient.....but then when i am making clear my decision why does my boys Doc look at me as if I'm from another planet !! 

Hope you are keeping well Andy.  I know from some of your posts that you have been not too well......you are a diamond Andy (or are tanzanites worth more!?!) may God have Mercy on you and keep your light shining the brightest!

I need to ask something about our boys.  They have both complained about this to their Doc.  I would like to know your opinian.  This morning my eldest got up with a slightly uncomfortable feeling in his chest. He went off to college as usual  leaving us in anticipation of how went his first day of exams.   He text us to say it went well except that he had an excruciating back pain.  He was relieved to have made it through exam time.  The feeling of tightness in his chest travels down to the lower back, causing it to pain and making it difficult to breath. 
Their ECG has always come back normal and their cardiologist has said that this was nothing to worry about.  Could this be just muscle spasms? what do you think?  It always starts that way with discomfort in the chest receding to lower back with difficulty in breathing and pain that could last for anything upto 5 minutes to half an hour. 
Would love to know what your opinian is on this.

Emby         

Emby,

Because of the history of low hemoglobin levels, I would suggest asking at the thal center about investigating the possibility that extramedullary hematopoesis has developed in the boys. What happens is that red blood cells grow outside the bone marrow and develop into masses that can press against the organs or the spine, and this can become painful. The best way to prevent this is by not allowing the Hb to drop low. Hydroxyurea is also sometimes used to shrink the masses. Ultrasound can be used to determine if these masses exist in the boys.

And, I am doing much better. My shoulder and back finally healed and I re-did my work space at work so that I am no longer stretching to reach the keyboard on my laptop.

Thank you Andy for a quick response,

Regarding extramedullary hematopoesis, would this not have shown up on the MRI scan they recently had? Also their DEXA scan came back as normal, would the masses on the bone not cause weakness to it?  This pain thing happens to them only occasionally, if they had this would they be in pain most of the time?.......you know thal doesn't rule their lives, they have too much stuff to be getting on with but me.....I obcess about things briefly, deal with issues then let it go to the back of my head!!

Really happy to hear you are keeping well.

Emby   

It won't be revealed by MRI and the DEXA scan would scan only the boe. These masses grow outside the bone and tend to cause discomfort more in times when the HB is low and they expand. The symptoms fit but it is not the only possibility, but I would request an ultrasound by a technician who understands what to look for.

Reading up on it, hydroxyurea is an option for treating it but radiation is a better form of treatment other than surgery which may remove the masses for sometime but may grow back again.....God Willing, I'm hoping it is not this......will ask for this to be investigated.
What do you think are the other possibilities?......the Doc thinks i worry too much (ask many questions) but there is a difference between worrying and keeping informed.....still..the Doc feels i worry too much..
   

Radiation is usually reserved for severe cases where spinal pressure is painful enough to require action. The hydroxyurea can have the added benefit of boosting the Hb level and has been found to be very safe in the doses used with thalassemics. One other possibility is simply the pain associated with low Hb, as the bone marrow begins to expand to try to produce RBC's. This expansion is felt in the bones and can be quite painful. There are very good reasons to keep the HB above 9-9.5 all the time.

Thanks Andy,

.......... ...thats me at the moment though not quite flat on my face yet at 2am...then it will be

Who put together these smileys...some are hilarious...that great big syringe one...haha

The smiley's were all added by Danielle, our co-administrator. We don't see much of her on the site these days but she still takes care of the technical stuff. One good note was she recently told me on Facebook that her ferritin is really low now that she's been taking Exjade regularly. Her T2* also came out good.

Danielle has matched human moods and emotions perfectly with the smileys....i have read some of her posts..she has such a strong personality.  She has been through so much in her life but carries herself with incredible strength...there are some very insperational people on this site.

Yes, Danielle has had to deal with many serious health issues that have nothing to do with thalassemia, in addition to osteoporosis, which is thal related. She has also been busy with school as she furthers her career, so she doesn't have a lot of free time.
Facebook becomes a good way to keep up on a lot of people who don't visit here so often.