DiMattia here.

Hi my name is David DiMattia I am new to this site, I'm 31 Italian & Korean  born and raised in America.

 I have hemoglobin e beta o thalassemia

@ age 4 I had my 4.5 lb spleen removed, I have had Many Many Many blood transfusions mostly as a child,  in early 2000 I had radiation treatment on my back because the bone marrow was growing outside of my spice and compressing nerves in my cord causing extreme pain along with paralyzing me from the middle of my torso down.  The radiation worked I was able to walk again and for the most part the major pain went away.

Medications
Exjade
Penicillin
Folic acid
Neuroton for pain
Felden for joints
Enalapril for shortness of breath/heart

Some of My symptoms
PAIN
Pain in back
Pain in legs
Pain in feet
Pain in Joints
Pain in neck due to supporting my big ass head all day
Ear pain and loss of hearing in right ear due to marrow growth (radiation pending)
Shortness of breath
Fatigue / Weakness

ME & My HEALTHY 11 y.o. Son Ocean

Di Mattia,

Welcome to the board!  It is great to have you here, it sounds like you are a real trouper and fighter - as you have overcome so much.  It is nice to see you and your healty son in the picture:) 

We have so much to learn from you and hopefully we can help you here as well    Please keep us posted as you go through the radiation for your ear. 

Best,

Sharmin
 

Hi David,

How low is your hemoglobin level allowed to drop before you get transfused? I am concerned about the extramedullary hematopoiesis for which you received radiation treatments. These usually only happen so severely when the HB is kept low.

How low is your hemoglobin level allowed to drop before you get transfused?

I was actually wondering the same thing.  If your hg is being allowed to drop very low perhaps an adjustment needs to be made in order to prevent further complications relating to extramedullary hematopoiesis.

Sharmin

Thank you guys.

Andy my hemoglobin is normally around the 7's if i get into the 5's or lower i receive transfusions.

David,

The masses that were irradiated, the extramedullary hematopoiesis, are a direct result of the low Hb level. When your bone marrow can't make enough hemoglobin to adequately serve your body's needs, attempts will be made to grow red blood cells outside the marrow, usually within the body cavity. This can result in pressure on the spine and organs and can cause serious permanent damage if left to grow. This can even occur at the cellular level where it looks very much like lymphoma in scans. It is not and any thal diagnosed with lymphoma needs to be aware that it may not actually be what it appeared to be in a scan. The drug Hydroxyurea is often used to help shrink these masses. Hydroxyurea is also a hemoglobin inducing drug, and should be considered as a possible therapy in thal intermedia. Have you ever tried it? Raising the Hb level is a key component of the treatment for extramedullary hematopoiesis, so I am upset to see that your Hb has been kept so low. This is an outdated style of treatment for intermedia and is now rejected by most experts, because iron chelation drugs are available to counter the iron load, and patients do much better when the Hb is kept above 9 at all times.

Where are you being treated? Are you open to the idea of being seen once per year at a Thal Center of Excellence in the US? I think this is the key to living a fairly normal life as a thal, especially as you age. A good treatment program can keep patients healthy by doing an annual assessment and fine tuning the treatment program. I would also suggest that you register with CAF. They are very good about finding the best centers in an area and also providing information about the various Centers of Excellence.

Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999

Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org

I am a great believer in the comprehensive approach to care that is found at this type of center around the world. I do not believe you are getting the comprehensive care that you need, especially being that you have the responsibility of a family.

I wanted to ask something about what you were told about the odds of having thal children. There is absolutely no difference in the odds from one pregnancy to the next. It is purely chance. What I do want to know is your wife a carrier? If not, there is no chance of you having an intermedia thal. Minor or non-carrier only if your wife is not also a carrier.

Andy

I have never even heard of Hydroxyurea but you can bet your sweet bottom that I'm going to ask my dr about it next visit.

Yeah from my teens till now my treatments have for the most part been very little other than the radiation as long as my hb was around 7 my doc felt I was doing fine, he did want me to get on iron chelation around 2000 when he was putting me on radiation but at that time I did NOT want the Desferal thing put in my chest so I refused for about a year or two then I said okay I'll do it and just think of it as another piercing.
But then my doc said let's hold off they are working on this oral chelation pill and he told me that for the next 5-6 years till exjade was approved by the FDA.

I'm being treated by Dr. Troy Guthrie @ 21st century oncology but kind of feel my Dr  has been a little lazy or un educated in my illness.  I tell him I'm having pain and the meds he is giving me is not working and he does nothing.  All of my visits are less than 10 mins face to face time I never really leave my dr feeling satisfied.

I'm will register w/ CAF & yeah I'm down for the once a year check up thing.

Thanks for all your help so far.

So my doc said NO to the Hydroxyurea said its used for sickle cell & not FDA approved for thal.

Hi Dimattia and welcome to the site

Actually I think that your doctor is not an expert in thal because hydroxurea is being used for thal intermedia. Not only this but some thal major patients use it in order to increase the gap between transfusions.

It would be great if you follow andy's advice and go to a comprehensive thal center in order to be in good hands
Feel free to discuss anything you want
Manal

So my Doc did some reasearch and yes he has put me on Hydroxyurea 500mg a day for one month then he is going to check my blood work and if all is good go up to two pills aday for a month check blood work if all is good up to 3 pillls a day and he said no more than 4 pills a day if all goes well for me.

He also put me on Mag Oxide 400mg pill 3 times a day I dont know exactly what it does but he said it works with the Hydroxyurea.

Best of luck and keep updating
Manal

Welcome DiMattia.

1 of 3 targets for radiation the other 2 are a little lower and on each hip bone area on my sides
Radiation starts Monday

Hi DiMatta,

I usually do not assume things, but it looks like your doctor does not understand how to manage the thalassemia. Also, I think he might be experimenting on you. One day he said NO to Hydroxyurea and next day he is ready to give it to you and said, lets see what happens, he is not sure himself.

Please contact CAF mentioned above.

4 weeks of radiation done on my lower back and pelvic area.

started radiation on my mid and upper back

today my lower back started hurting to the point i could not walk, what the hell i thought this radiation was supposed to stop the pain not make it worse.