Getting started with chelation

Hi! My niece has a severe case of spherocytosis.  While I realize that is quite different from thalassemia, I think a brief history will show why she has more in common with the "thals."

Maddie is 2 years old and has been receiving regular blood transfusions since she was in the womb. She received about 4
fetal blood transfusions before they decided to deliver her at 31 weeks gestation. Since her birth, she
has been receiving transfusions about every 4-5 weeks (7 weeks is the longest she has gone).  She is located just outside Detroit, Michigan, USA.

She wasn't diagnosed with spherocytosis until recently. Part of the problem with diagnosing her is that with the frequent (and pre-birth) transfusions, they never had any of "her" original blood to test.  The diagnosis is just a working one and may not even be correct.

Anyway, she has an upcoming MRI scheduled in order to see the iron levels in her heart and liver. Her iron count was 1350 five weeks ago and is 1410 now, so it looks like they will be chelating her soon.

Obviously, we want to start off on the right chelator, but I realize that we may need to do some trial and error here.  I have some experience with lead and mercury chelation in myself and my own daughter (who I recovered from autism), so I'm not new to chelation in general -- just to iron chelation.

I have four questions:

1) I'm intrigued by IP6 and think we should start her on that as a supplement.  Does anyone have an idea as to the dosing for a 2 year old (she weighs 25 lbs.)?

2)  Also, that Exjade is oral is attractive, but I'm concerned about it being more suitable for children over 6.  Does anyone have a younger child on it?

3)  Is anyone dosing Desferal every 8 hours?  If so, was that recommended by your doctor or did you request that?  It seems like every 8 hours (instead of every 12) may be better based on the half-life of the drug.

4)  Has anyone tried lactoferrin as a supplement?

Thank you very much for any help you can provide!
Dawn

Welome Dawn to the website

I did a little bit about Spherecytosis and seems a little more like Alpha Thal(Major) where the child receives the blood while in the womb. As far as I know there are no Alpha Thal(Major) members on this website, but regarding chelation, I think thals can relate with removing the excess Iron.

Andy has mentioned he is going to get back to you after his research but here is a thought on one of your question

2)  Also, that Exjade is oral is attractive, but I'm concerned about it being more suitable for children over 6.  Does anyone have a younger child on it?

There is a member whose child is on Exjade. Canadian_Family is the username. You might want to send him a personal message and might get a reply. I think the child is less than 6 years of age

Hi Dawn,

There is a complete article on hereditary spherocytosis (HS)  at http://www.emedicine.com/med/TOPIC2147.HTM

Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity of clinical features, ranging from an asymptomatic condition to fulminant hemolytic anemia. The morphologic hallmark of HS is the microspherocyte, which is caused by loss of membrane surface area, and an abnormal osmotic fragility in vitro. Investigation of HS has afforded important insights into the structure and function of cell membranes and into the role of the spleen in maintaining red blood cell (RBC) integrity. An intrinsic genetic defect causes defects in membrane proteins.

The major complications are aplastic or megaloblastic crisis, hemolytic crisis, cholecystitis and cholelithiasis, and severe neonatal hemolysis...Hemolysis in HS results from the interplay of an intact spleen and an intrinsic membrane protein defect that leads to abnormal RBC morphology.

HS erythrocytes are caused by membrane protein defects resulting in cytoskeleton instability. Spectrin deficiency leads to loss of erythrocyte surface area, which produces spherical RBCs. Spherocytic RBCs are culled rapidly from the circulation by the spleen. Patients with HS develop splenomegaly...Anemia or hyperbilirubinemia may be of such magnitude as to require exchange transfusion in the neonatal period. Anemia usually is mild to moderate; however, sometimes it is very severe and sometimes it is not present. Splenomegaly is the rule, and palpable spleens have been detected in more than 75% of affected subjects. Severe hemolytic anemia requires red cell transfusions...Generally, the treatment of HS involves presplenectomy care, splenectomy, and postsplenectomy complications. A trend toward partial splenectomies exists in the pediatric population, which appears to control hemolysis and, at the same time, preserve splenic function...After splenectomy, RBC survival improves dramatically, enabling most patients with HS to maintain a normal hemoglobin level.Ideally, splenectomy should not be performed until a child is older than 6 years because of the increased incidence of postsplenectomy infections with encapsulated organisms such as S pneumoniae and H influenzae in young children...    * Lifelong folic acid supplementation is recommended for these patients secondary to low levels of chronic hemolysis. This is especially true for those who have not undergone splenectomy.

1) IP6 is very safe and may be of some use as a chelation supplement. Up to 1000 mg daily would be safe starting dose (much higher doses have shown no side effects). The capsules can be opened and mixed with liquid. It is also most effective when taken along with inositol.
2) Since the time when Exjade was first approved in the US, it has been approved for children age 2 and older. Studies have not found any reason to believe it isn't safe. The only problem may be getting a young child to drink enough liquid to take all the medicine. This is not a big deal compared to using a pump and needle.
3) Most patients take desferal in 8-12 hour sessions but no more than once daily. This is a matter of convenience (or perhaps, inconvenience). Desferal has a very short half life and is not active for long after the pump stops. The most efficient method is 24 hours a day but that is seldom done, except in cases of severe iron overload.
4) I don't know of anyone who has used lactoferrin, but it is found in milk products and they are known to help inhibit dietary iron absorption.

While posting, I see Maddie's mom has also joined and would like to say welcome. I want to assure you that IP6 is very safe and members of this group are finding it is a good supplement to iron chelation. I feel that since it works at the cellular level in all cells, that its effects are not as easily measured but it is working where it is needed. We have heard some reports of lower ferritin with its use and I highly recommend its use. One good thing is that splenectomy cures this disorder and chelation will not be a lifelong treatment. Partial splenectomy in children is becoming more favored and should be investigated, as this preserves the infection fighting capability of the spleen.

Does someone know a good hospital in Detroit? Thank you in advance.

Hi Dore ,
i hope this link will help you to find best hospital in detroit .. its a list of America's best hospital with their rankings and speciality ... check this link :  http://www.mlive.com/business/detroit/index.ssf/2009/07/metro_detroit_hospitals_place.html


Best Regards
Umair

Hey Dawn,

I first was also diagnosed with Spherecytosis and was because of that NOT giving blood transfusions. It all changed when my hgb dropped to cirital point, my parents noticed, and brought me in. Two years later I was giving the right diagnosis.

I will send you a private message too.

Dori