Iron and the lungs

There is reason to believe that iron can settle into the lungs and interfere with lung function.  Time to time I have heard of thals experiencing breathing issues - sometimes when the hemoglobin is low and that may be related to lower O2 and lower oxygen carrying capacity of red blood cells when the patient is due for transfusion.  I do think that high iron content in the lungs can cause low lung function.

Little A had some issues with breathlessness last fall - and breathing tests showed that he had low normal results.  Now that his iron levels have come down the levels have all increased to well within the normal range.  Another test will be repeated in 6 months (at the same time as his next SQUID test) to see the relation between iron levels and lung function.

The solution to this - as well as any other iron related issues in the body is - chelation and antioxidants.  Chelation to remove the iron from the body and antioxidants to bind free iron so that it cannot damage the lungs. 

Sharmin

The effect of iron on the lungs has been the subject of several small studies, with somewhat conflicting results.

http://radiology.rsnajnls.org/cgi/content/full/229/2/507

Although iron deposits have been found in the macrophages in alveoli and in the perivascular tissue of patients with ß-thalassemia major at postmortem examination (30,31), the relationship between iron deposition in the lungs and pulmonary dysfunction is unclear (31). The proposed mechanisms of airway obstruction in ß-thalassemia major include oxidative damage as a result of free iron deposition within the airway epithelium (10,11). This theory is supported by the identification of iron-laden macrophages and lymphocyte alveolitis within the bronchial mucosa and in bronchoalveolar lavage fluid, suggesting that free iron deposition is a toxic mechanism.

Other suggested mechanisms are bronchial hyperactivity (32) and chronic immunologic factors (6) related to blood transfusion and disproportionate and/or excessive alveolar growth relative to airway growth caused by hypoxemia or hypoxia, a chronic abnormality in patients with ß-thalassemia major (33). It is known that serum ferritin levels do not necessarily reflect total body iron stores because they vary during the process of chelation and because ferritin is an acute-phase protein as well as a product of hepatocellular damage (34). It is therefore not surprising that reports of correlation of serum ferritin levels with pulmonary function have been contradictory; some researchers have shown a positive correlation (3,6,7), while others have not (4,5,9,10).

Chronic low oxygen levels also play a part in lung function in thals and this is another reason why keeping the hemoglobin levels up is important.

It's really good to see these positive reports on little A's health and it's a testament to the value of compliance with the various areas of treatment. The comprehensive approach to thalassemia treatment goes beyond the transfusion-chelation routine to include all aspects of physical and psychological needs of the patient. This program of treatment can prove very costly, so many aspects will not be followed in other treatment centers and in fact, not all are followed even in the comprehensive centers themselves. And this is actually an area where we can serve an important function in the international thalassemia community by promoting the concepts and methods that are included in the comprehensive approach to thal care. There are many things that patients and parents can do for themselves to improve health once they have the proper knowledge, and we regularly see patients doing better with health issues when they follow the advice they receive here. We are seeing how important supplements are for thals and the drastic improvement in health that is seen when deficiencies are corrected. We see how much better patients do with chelation when they learn the proper methods and mental approach. There are many things we do that help to spread the concepts of comprehensive care and this is a low cost approach that has major benefits. We use this group to educate patients, parents and even doctors to help further the understanding of the many issues involved with thal and the many facets of treatment. Our promotion of the comprehensive approach to thalassemia treatment is already seeing results and the various posts we read here show that patients are becoming more informed and are effecting changes in their local treatment centers and even furthering the understanding of thal care among medical professionals. This is only possible because the members have been willing to learn and to share what they learn.

Thanks for the info Sharmin and Andy,

Chelation is a necessary part of thal's life,but its really sad to see that sometimes people do not understand that,i met today two youngsters who think just ferriprox is enough for them,although they have high ferritin levels,above 3000,and i don't think ferriprox alone can lower it.
I really feel sad for them,they have a life,they can have a chance at life and they are deliberately loosing it.
One of the two youngsters's mom was there,and she said i can not force,he is old and won't listen to me,she was sad,i wish i could have done something for her.

Zaini.

Zaini,

Chelation two days a week with desferal on top of the Ferriprox would be great. Try explaining to them that desferal is more efficient than Ferriprox at removing iron from the liver, so the combination provides more than Ferriprox alone can offer. Two days a week is not a major hassle and should be possible. Do your best to get them to understand how much they can do for themselves with a little more effort. Ferriprox alone would be fine if their ferritin was lower but they need to get to that point before they use just Ferriprox.

I love the thalpal spirit that takes the information gathered here and spreads it to others.

Andy,

One of them is our forum member,but he isn't posting much,i asked his mum why and she said he is very busy with the job and everything.
Other patient was a girl,studying i university,around 20 years of age,i told her that if you don't want to use needles,and just want to take tabs,you can atleast switch to Asunra,but she gave me impression that she couldn't care less,its sad.i'll try to talk to them again when i meet them.

Zaini.

Since we have gotten back from Oakland, in addition to our previous supplements - I am giving him L-carnitine and alpha lipoic acid - also I have him skip rope every day.  I think that this will be good for his heart, lungs and his bones.  He is actually liking it and has been working on various tricks that he can do now that he is getting better at it.  I think that it will also increase his balance and coordination for his other sports. 

Skipping rope is an excellent cardio exercise and it is used by boxers to improve endurance and agility. It will serve him well when playing hockey.

Andy,

You are so right in what you said about Ferriprox alone not being effective, as far as liver is concerned.
I have my personal experience to deposit here:

My ferritin has been around 100 for the past 4 years and on 2007 I decided to chelate only on Ferriprox.No desferal at all.After one year only on ferriprox I did an MRI, and it showed a light deterioration on Liver (from T2*37 to T2*33) which, of course, is o.k and normal  as this rate  is considered safe and with no iron at all. However only ferriprox could not maintain the standard of T2*37.
Seeing this I started  desferal again 4 days a week, together with ferriprox of course.

I think combination therapy is a must.

Lena.

Lena,

Its really amazing for me,also your iron level and your chelation regimen,why do you have to do so much chelation with ferritin which is below normal range? Is it ok,chelators can't be harmful with that low iron?

Zaini.

Zaini,

One would think that having so low ferritin enables you to lower your chelation scheme. This is partly right. I have concluded that with 90-100 vials of desferal per  month and 7 tablets of ferriprox per day, I have succeeded in keeping ferritin at around 100. I take only 5 vials of desferal each time, as when I take 6 or more my pulse is slightly up and it returns to normal, 2 hours after the desferal has ended.
This of course answers to your question,that chelators can be harmful once your body is free from iron. However, here we keep about the same chelation scheme even if we have a normal  sf.
Once heart and liver are clear, chelation proceeds to other organs.Of course, if the chelators become toxic you should change the scheme.

Lena.

Its really amazing for me,as i always thought that one can go off chelation once iron is that low  .

Zaini.

No Zaini,

the only case you go off chelation is when you are cured by gene therapy and even then you have to chelate for a while after gene therapy for your body to drive off all of the iron it has.

Lena.

Can i conclude from Zaini and Lena discussion that

1- L1/kelfer should not be used alone no matter how your ferritn is?? So why some patients used them alone like Ashish, Sahil,....?

2-

Once heart and liver are clear, chelation proceeds to other organs

Does this mean that the iron overload doesn't start with these organ but iron is loaded in all organs at the same time. So if i am free of iron in the liver and heart, this does not mean that the other organs are free??? How can we measure iron in other organs???

manal

Manal,

About your first question,as we have heard before desferal is good at removing iron from liver,and ferriprox at removing iron from heart,so if you take ferriprox alone,i think there will be a risk that iron will get deposited in your liver,there was a short period of time when we were busy with some domestic stuff and everything was a bit hectic,so Little Z was on ferriprox alone and her ferritin increased.And we have heard that people who kept their iron levels low and were chelating,stil found outl they had iron deposited in their organs,so its a tricky one  .

Zaini.

Hi
I would like to add another question here. Why does it take 30 odd years for the ferritin to go below 500? Since chelation is done according to body weight, why can`t well chelated children also reflect such figures. Sharmin son was around 900, but since he was chelating from such a young age, why wasn`t his sf below 500. There are a few members in this site who have low sf levels and they age between 30-40.

maha