maintaining hb levels

hello again...

please andy i was wondering if you could help.  my older son has now been receiving less blood than before for almost three months now.   his hb was 9.4 may, 8.3 on 5th june, and 25th june  
7.8.  he is due for a blood transfusion saturday 4 july.  i know his hb is low and i spoke to his consultant about this.  she seems to want to continue with her formula for the amount of blood my son has (desired hb 13 - actual hb x body weight kilograms x 3).  she told me it could be a viral thing (he has not been sick) and something about heamocrit (sorry about spelling).  can you explain to me something about this and how can he maintain his hb at 9.5.  his hb was dropping even with formula of desired hb 14 being used:

oct 2007 hb 9.7
nov   07  hb 7.8
dec   07       8.8
dec   07       9.5
jan    08       9.9
feb   08        10.2
march 08      9.6
april  08        8.6
may  08        8.3
june  08       8.4
july   08       9.2
july   08       8.6
august  08   not recorded!
sept   08      9.6
oct   08       8.8
nov   08       8.4
dec   08       8.7
jan    09       10.3
feb   09        8.4
march  09     8.9
april     09     9.8

what would you say i need to look at.  also if he has regular blood transfusion of 4 or even 5 units of blood every month (about 1000mls - 1250mls) would this be not good?  as i have mentioned before his ferritin is 257 at present and he is on desferal and ferriprox because of alot of iron around heart (from t2 scan).  he had an oppointment with endicronologist (spelt wrong again!?!)
and his vit d level was significantly low so he is taking calcium sandoz 5ml twice daily and ergocalciferol solution 3.3mls once daily for 28 days then once weekly till next appointment, 3 months time.
he also had dexa scan and awaiting result.
he is not feeling  particularly tired with low hb and continues with physical activities (loves football !).

look forward to hearing from you

emby  
 
    
 

oops....should be ''maintaining'' hb levels....sorry !  

Hi Emby,

Perhaps your consultant is unaware of the updated guidelines for treatment of thalassemia in the UK that were released in 2008. If not, please show this to her. If she continues to insist on maintaining a lower Hb level, please contact Emma Prescott at Whittington hospital. I will send you her email address in a PM. She should be able to help you.
Dr. Farrah Shah, Specialist (Thalassemia Physician)
Emma Prescott, Specialist (Thalassemia Nurse)

The Thalassemia Clinic, Mercers Ward
Whittington Hospital NHS Trust
Highgate Hill, London, N19 5NF

Phone Number
02072723070


http://www.ukts.org/pdfs/awareness/ukts-standards-2008.pdf

6.2 Standard
• Haemoglobin levels should be maintained
above 9.5-10g/dl.

I agree completely about maintaining a higher Hb level because it has been proven that the closer to normal Hb that can be maintained, the fewer overall problems patients will have.

thank you andy.....

Hi Emby
Are your boys taking vit E? It really made a difference in my son`s pre tx hb. For more than a year his pre tx hb was hovering between 8 - 9 gm/dl. Its five months since I started him on 100IU vit E( natural mixed tocopherols) and the last four months his hb has been over 9.6 ( twice over 10) with the same volume of blood ie about 15ml/kg.

maha

hi maha,

i am not just yet....thank you for that...i probs start giving him that.  my younger sons hb was maintaining well before the tx change.  i want his doc to change it back.  i will be talking to her after blood test this week.       

hi,

i have just read something about vit e...am worried if taking it would affect sirum ferritin levels and also not found something that say it could increase hb level...please may i have more info on vit e and thalasseamia....what else could be taken to increase hb...thanks.

Hi Emby,

I doubt anything else can raise hb levels than blood itself. Why don't you try shorter transfusion intervals for one or two times and see what happens?

Lena.

Hi Emby,

Vitamin E would have no effect on serum ferritin levels. It was first established in the late 1970's that thalassemics were typically deficient in vitamin E. Supplementation has long been recommended. There is a belief that vitamin E can extend the lives of red blood cells by its antioxidant effect.

http://emedicine.medscape.com/article/958850-treatment

Vitamin E deficiency: Vitamin E deficiency has been reported in patients with severe thalassemia. Some of the hemolysis in this population was attributed to peroxidation of the RBC membrane lipids by an iron-mediated free radical effect. As an antioxidant, vitamin E is expected to decrease cell toxicity.

I believe it is one of the most important supplements because of this antioxidant effect, which helps protect the organs from iron damage. Antioxidants in general are highly recommended for everyone but especially thals because they do need to try and protect their cells from iron oxidation which damages and destroys cells. Diets high in antioxidants and antioxidant supplements can play key roles in optimizing the health in thalassemia. As far as safety, doses up to 1000 IU natural vitamin E daily have shown no ill effects and many thals take that daily dosage. For teens, I would suggest 200-400 IU daily of natural vitamin E. Do not use what is known as dl-tocopherol. It is a synthetic form of E with very little value and can be harmful in high doses. The label should say d-tocopherol. Mixed tocopherols are the best, as they combine the various forms of vitamin E as found in nature. Look for mixed complex vitamin E. I have taken 400 IU daily for 35 years. I am absolutely convinced about its value.

thank you lena and andy,

they are having blood tests today...am hoping, inshaallah, hb not dropped further...i need to convince my sons to take it because they won't take anything unless its prescribed by their doc.

keep well

Good luck for the tests Emby,everything will be alright Inshallah .

Zaini.

thanks zaini

hi,

got hb results.....older one 6.8..gotta have 1300mls tx.  little one hb 8...having 1000mls tx using old formula.
asked about vit e but was told that it is not normal practice to give to thals over here  but i asked doc to please look into this which doc agreed to.

its not fair on the boys, when their old formula was already working for them...perhaps needed a bit of tweaking, to have inadequate tx then to have a whole lot (sigh!)

God willing, inshaallah,  all will go well tomorrow.
hope you all well.
 

 

hiya,

just want to ask if it is safe if my boys are exposed to swine flu?.....spreading like mad here !!

Hi Emby
Is that formula actually used by your doc? Even when the desired hb is 14 as per the formula my son should recieve 180ml which is exactly 12ml/kg. If the desired hb is 13 then he should have 135ml which is far too less than what he is actually recieving ie 225ml as per the 15ml/kg rule. Your boys problem is just that they are getting less blood than required. My paediatrician also feels 12ml/kg  every THREE WEEKS works the best, but we were more comfortable with 15ml/kg every four weeks. I feel you should talk to your doc about it.

take care
maha