New Book about Thalassemia (Cooley's Anemia)

I am still waiting for my copy.  I hope to receive it soon.  I am glad that it is easy to read, I think that this was the intention of the author - to write a book that everyone can read and learn from. 

Sharmin

Some highlights of chapter 1, Introduction

Chapter 1

Introduction

The author explained the hemoglobin molecule is made of beta and alpha globin together with heme. The two (Beta and Alpha) globins acts as two fingers and heme as thumb to hold the oxygen just like a pitcher holds the ball in a baseball game or a bowler in cricket game. The hemoglobin molecule catch and hold oxygen from lungs and pitches the oxygen to tissues in the body just like the pitcher or bowler does in their respective games. The difference is that each hemoglobin molecule pitches the oxygen with precise accuracy billions of times in a day for months. The amino acid that creates the alpha and beta globins are formed in chromosomes 11 of our DNA or our genetic makeup which lies in the marrow of bones. In thalassemia, due to absence of healthy beta gene (one finger is missing to hold the oxygen) resulting in the inability of hemoglobin to hold the oxygen properly. The normal production of alpha globin tangles the hemoglobin molecule as the balancing beta globin is absent to balance the oxygen resulting in the destruction of hemoglobin molecule in early stage. We know the story what happens next……increased hemolysis, expansion of bones due to extreme activity of bone marrow etc etc etc.

Red blood cells, like all other cells, are made in the bone marrow cavities inside our bones. The earliest red blood cells somehow know that their main job is to make large amounts of hemoglobin. The scientist don’t know how they know, but they know. The earliest red blood cells made in our bone marrow have nuclei that carry our chromosomes and DNA. After dividing a few times, these nucleated red blood cells in the bone marrow, brusting with hemoglobin, become smaller, lose their nuclei, and leave the marrow to enter our circulating blood.

Starting chapter 2
 

Thank you so much Canadian family for the information, very simple and informative

manal

Yes it seems quite simple and easy to understand,i wonder if i can get my hands on it  i'd love to read it.

Zaini.

Chapter 2 Love and Loss

Very depressing, I warned my wife not to read it, yet she did and could not sleep well. The chapter is about thalassemia patients and their families in 1970's and 80's and their hardship......

No other comments on chapter 2.....

Yes Canadian family, it is very clear from the tiltle.. its better to think positive

manal

Canadian Family,

Thank you for sharing.  Chapter 2 is one that I personally feel afraid of reading as well.  It is sad to know that parents who were in our shoes in earlier times had to go through such heartbreak.  I hope that within the next decade things will change drastically enough - that they will no longer have to suffer at all.

Sharmin

That reality about thalassemia was what drew me to help in the first place. The thal reality that Lisa grew up with was dismal and colored her own beliefs about her life. In her mind, thals were very lucky to make it to 30 years old. I had no idea back then that there were already thals in their 40's. Perhaps if some of these people had been more open to sharing and helping, I may have been able to do something for Lisa. I do know that what I have learned over the years could have made a difference to her health, but it's too late for that but I can help others and do my best to do so.

I want to say that the old reality is no more and that reading about it can give you different perspectives, and I hope one it does give everyone, is the perspective on how much thal treatment has advanced in such a short time and not only does that mean thals can expect to live far longer, but also that we can all expect more improvements and better lives for thals. I know it's sad to read about how it was but don't let it affect how you approach each day. Please remember that Dr Bank has lived through much himself and I am sure he has lost many friends to thalassemia and there is no way he could write a book about thal without remembering those who are gone and providing the perspective on how much different things are today. From one who has lost many dear friends to thal, I will say that it is so sad and at times it is hard to go on and stay involved, especially when I realize there are people I know who will die because they refuse to work hard enough to stay alive. I still don't know what to do when people won't chelate enough and probably never will know what to do, but I have learned to help them in any way I can, even if just to help them find some acceptance of their decisions and some peace of mind. Meanwhile, I hope you all realize what a difference you make to your children and how much your constant intervention does for them. There are people here whose kids would no longer be with us if the parents had not stayed on top of situations and acted when necessary. Take some strength in what you have shown already and know you will always make a big difference in the lives of your kids.

In her mind, thals were very lucky to make it to 30 years old

This thinking is still present,my own aunt who lost her daughter to thal back in 80s,still had the same perspective about thal,when i told her about new treatments and technology and how it had helped thal's life span and over all health,she was like "yeah may be" .

   I hope you all realize what a difference you make to your children and how much your constant intervention does for them

Credit goes to this forum Andy,it has helped us and encourage us in learning ways in which we can help our kids,it amazes me when i tell people about this forum and they don't join it.

Zaini.

Chapter 3, 4, 5 and 6 tells the stories of families in early 60s to 80s.....

Part 2

Dr. Arthur Bank describes how he came with his conclusions and different mutations of beta thalassemia major (he talked about his experiments) in 60s, 70s and 80s. He talked about the management of thalassemia in modren days. Sadly, he admitted that eventually this condition despite all medical advances would kill its victim. He still thinks the lifespan of 30s to 40s at this time.
I found Dr Arthur Bank is inconclusive re. the cure of thalassemia at this time. He seems of the view that there is a lot to be done...you climb one mountain and see another mountain waiting for you. He talked about different treatment options (we all know, fetal hemoglobin etc.).

Still reading.

Canadian Family thank you so much for sharing what you have read with us.  I am still waiting to receive my copy - but it has been so nice to read your comments. 

I have much respect for Dr. Bank - after knowing him for 10 years and learning what a kind and caring person he is.  I do hope that his prognosis about the life span of thalassemia patients proves to be wrong however.  Dr. Vichinsky's opinion on this is that thalassemia patients, with full compliance, can live a full life span.  Perhaps, here is where the problem lies - many thals are not compliant and as soon as things go wrong they completely lose control of the disease.  If thals keep their iron levels down to begin with - if problems (such as antibody issues) occur then the problem will not spiral out of control.  In this way, Dr. Bank's prediction is correct.  We realize this when we try to get certain families to take proper precautions, or even join this site.  Some people don't take the time, that is so unfortunate. 

My hope is that with proper transfusion/chelation and supplements thals should be able to live a full life.  Dr. Vichinsky mentioned that he has 50 year old patients, who did not enjoy the medical advancements that we do these days - therefore our children have much more hope of living into their fifties and beyond.  What has and will continue to separate the survivors from those that unfortunately succumb to this disease is compliance.  Recently, a thal major became a grandfather - to me this is encouraging.  I have thal major friends who are in their 40s and they have children - and they show no signs of slowing down.  It has been so hard reading this 'expiry date' for years, I hope that it does not have to apply anymore. 

Our goal is a group is to increase the life span of every thal patient that comes to us for help - and my goal as a mother is to dedicate my life to preventing this disease from claiming my son as a victim.  My prayers are that thalassemia will not beat us - and that we are able to get rid of thalassemia. 

If genetix pharmaceuticals has stumbling blocks I hope that they are able to defeat them - and those that they cannot defeat - perhaps Errant Gene or some other group using stem cells can overcome these mountains.

Thanks again Canadian Family god bless all of our children 

Sharmin

I also am unable to understand Dr Bank's point of view,i also think that with proper transfusions and compliance with chelation our kids will have a very fair chance to live a normal life span.

Zaini.

I never understand why people say that about thal life spans, especially when as Sharmin said, there are patients alive today in their 50's (I know several) and they did not have the advantages that are here today when they were growing up. I truly believe as Dr Vichinsky does, that patients who are serious about compliance with all aspects of treatment will live long lives.

Science always comes up with facts and figures to put in their books. I think what Dr Banks is forgetting while discussing the disease is the human being who is going through the disease and the family who is watching their beloved suffering.

Dr Arthur Bank is basically a scientist and I found in the book he does not want to talk about anything else other than what he knows today or making a claim. He did mentioned his recent talk with one of the higher official of the CAF where he said"I did not want to raise the false hope'. For example, later he did remotely mentioned about the thalassemia patient who became grandfather but he stopped making a statement about the full life span of thalassemia.

I think the statement of him re. 30s or 40s is worldwide and is based on today’s figure (he did admit the huge difference of medical care between developed and under developed countries). He mentioned about the very good quality of life for thalassemia these days. Well that is his personality.

Tariq,

I fully agree with your post.