Please Advise me!!

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Offline vampirebaby

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Please Advise me!!
« on: November 20, 2011, 03:21:19 PM »
Hello All,

Hope you're all doing well.

My daughter Eva, now 9 months, has never been transfused yet. Her Hb has been around 7.2-7.5 this month. The doctors want two consecutive Hbs of 7 before they decide to transfuse her. Can someone please tell me if it's really worth waiting? Is 7.2 low enough to cause facial/cranial bone deformities?? When I run my hand over her head, I can feel this protruded area near her soft spot (anterior fontanel) which could be just an area of normal skull fusion but it can also be an effect of thal. I should also mention that Eva has started to look very pale but at the same time is very active and feeding fine and not cranky at all. She's her usual self. Please give me your opinion on the subject, I'm really worried and would greatly appreciate any help.

Kindest Regards,
Sameet

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Offline Andy Battaglia

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Re: Please Advise me!!
« Reply #1 on: November 20, 2011, 08:52:25 PM »
Sameet,

This is a tough situation. Yes, the standard guidelines do say don't transfuse until there are two successive Hb readings below 7. I believe that at this point, the long time standard of judging by failure to thrive must come into play. Is the child growing at a normal rate? Is there a marked difference in her appearance and activity levels? If the answer is yes, then transfusions should begin. If there is not much change in these factors, then the advice will be to continue to delay transfusions until either the Hb drops below 7 or there are changes on her growth and activity level. Have the doctor examine her forehead and make sure the bone growth is normal.

I almost hate to reveal this, but if you feel that transfusions should begin, making sure Eva is very well hydrated before her Hb may make the level look slightly lower, as the added water volume does dilute the Hb a bit. If you are certain, this may be a strategy to get her started on transfusions.
Andy

All we are saying is give thals a chance.

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Offline vampirebaby

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Re: Please Advise me!!
« Reply #2 on: November 26, 2011, 06:08:37 PM »
Hi Andy,

Thanks alot for your response.

We collectively decided to have Eva transfused, mainly because her growth had slowed down. She's only 7Kg so they transfused 100mL only. Do you think that's an appropriate amount according to her weight? Also, I'm currently in Pakistan and the doctors here are a bit laid back. They didn't ask for a post-transfusion blood test to be done. Is a post-transfusion blood test necessary??

Aslo, we haven't been advised when to go back to have her Hb retested? Is 3 weeks a reasonable interval?? Do ALL thal majors require blood 3 weekly?? or could it be longer/shorter??

I'd really appreciate your help.

Thanks & Regards
Sameet


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Offline Andy Battaglia

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Re: Please Advise me!!
« Reply #3 on: November 26, 2011, 06:49:59 PM »
Hi Sameet,

Yes, the low volume of blood at this weight is appropriate. As Eva gets older this volume will increase. 3 weeks is an average for thal majors, but individuals will vary, so do get tested every 3 weeks, but especially at a young age you can expect some variation as to how often blood is needed. It may be that sometimes after 3 weeks, you are told to come back one week later for transfusion. This is based solely on the Hb level at the 3 week point. Post transfusion Hb testing is not required, as this can be estimated fairly well based on pre-transfusion Hb and the volume of blood transfused. testing would only be required more frequently if the Hb is too low after 3 weeks. The advice today is to maintain the Hb above 9.
Andy

All we are saying is give thals a chance.

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Offline vampirebaby

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Re: Please Advise me!!
« Reply #4 on: November 26, 2011, 07:01:09 PM »
Thankyou so much Andy! I hope you know how great you are!

Can you please also tell me what this genetic terminology means? IVS 1-1 (G>T) and IVS 1-5 (G>T)

Thanks again :)

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Offline Andy Battaglia

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Re: Please Advise me!!
« Reply #5 on: November 26, 2011, 07:23:50 PM »
IVS 1-1 (G>T) and IVS 1-5 (G>T) are the two beta thalassemia mutations that are present. They are 2 of the 6 most common thal mutations in the Indian sub-continent. IVS 1-5 (G>T) is a severe mutation often found in thal majors. IVS 1-1 (G>T) is a less severe mutation and is often found in thal intermedia. The two combined are likely to result in thal major, but would also account for why she has not needed transfusions earlier.
Andy

All we are saying is give thals a chance.

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Offline vampirebaby

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Re: Please Advise me!!
« Reply #6 on: November 27, 2011, 05:13:36 AM »
Thanks Andy :) That makes sense now!

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Offline vampirebaby

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Re: Please Advise me!!
« Reply #7 on: December 13, 2011, 07:56:12 AM »
Hi Andy,

Hope you're well. I need some help from you again if that's okay.

Eva had her 1st ever transfusion about 3 weeks ago. We're gonna have her Hb tested again this week. What should her pre-transfusion Hb be? If it's sitting at 9-10, would she still require a transfusion?

I understand that the Hb needs to be maintained above 9-10 but what if it's around 7-8 before her next transfusion? Does that mean she requires a transfusion sooner than 3 weekly?? Is it harmful to drop to 7ish briefly before a Tx??

Thanks alot in advance,

Regards
Sameet

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Offline Andy Battaglia

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Re: Please Advise me!!
« Reply #8 on: December 14, 2011, 01:33:12 AM »
Sameet,

Hb should not be allowed to drop much below 9, as it will have to be made up at some time with more frequent transfusions. Generally, you get around one point of Hb increase per unit of blood, so transfusing at 9 means less blood is needed. If you let it drop to 7-8, the child will not be as healthy and will need more blood during transfusions. Transfusing blood stresses the heart, so it's best to establish a routine that does not require more blood at one transfusion. Sometimes the blood is affected by antibody reactions and does not last as long. During those times, transfusions become more frequent. Outside of that and during illness, the Hb should not drop too fast. 3-4 weeks is pretty standard for that age, and a pattern will be established by how long she can stay above 9. If the test is above 9.5 transfusion may be put off for a week.
Andy

All we are saying is give thals a chance.

 

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