Hb level drop in one month- Thal Intermedia

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Hb level drop in one month- Thal Intermedia
« on: June 09, 2012, 09:30:06 AM »
HB level of a two years old patient dropped in one month from 7.3 to 6.6, in this one moth she suffered from throat infection as well, and took an antibiotics course.
My question is that is this an alarming sign? the doctor has ruled out blood transfusion for the time being.
plus are there any visible symptoms to check if the HB is dropping further?
She has been on folic acid dose since 6 weeks and has shown considerable improvement in apatite and activity.

plus would like to hear about the experience of people with hydroxy-urea? because the doctor has not started it yet, but intends to do so in a month or so.

Looking for advice
 

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Offline Andy Battaglia

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Re: Hb level drop in one month- Thal Intermedia
« Reply #1 on: June 09, 2012, 06:25:46 PM »
Any illness will lower the Hb level. In most cases, this should not be the justification for transfusion. More important is the normal health and growth of the child. If growth is slow and the child is lethargic, bone development must be assessed. If there are signs of abnormalities developing, the child should be transfused.
Andy

All we are saying is give thals a chance.

Re: Hb level drop in one month- Thal Intermedia
« Reply #2 on: June 16, 2012, 01:27:53 PM »
First of all, ThankYou loads, Thanks a bunch...this forum is a blessing.
and you're doing a wonderful job!

She remains a bit irritated in some parts of the day
also her weight was 10.5 kg a month ago but yesterday she was 9.5 Kgs, i'd rather be brief, 2 questions:

1. We got information from the internet and a doctor that if intermedias are transfused then their bone marrow might shut down and start relying on the external source of blood, is this true?

2. Also we're all too confused on the use and effectiveness of hydorxy urea, because one doc telsl us that WBC count will have to be monitored with weekly test, the other says there can be diarrhea and nothing else, another opinion is that hydroxy is only to be monitored if severe anemia is cured (its also written on the flap of the medicine)

What do u suggest for intermedias?

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Offline Andy Battaglia

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Re: Hb level drop in one month- Thal Intermedia
« Reply #3 on: June 16, 2012, 05:59:54 PM »
To answer your first question, the purpose of chronic blood transfusion is to ensure a high enough oxygen level to allow for normal growth and development, and of course to maintain life, but also to shot down the bone marrow, so that the production of ineffective red blood cells slows considerably. If transfusions are only taken occasionally, it will not shut down the bone marrow.

This is the protocol for monitoring when used with sickle cell. The monitoring for thalassemia should be similar.
http://scinfo.org/care-paths-and-protocols-children-adolescents/hydroxyurea-protocol
Quote
Dosage
Hydroxyurea 15-20 mg/kg p.o. q.d.(supplied as Droxia [200, 300, and 400mg] and Hydrea [500mg] capsules). All size capsules must be available for accurate dosing. Liquid suspensions, 100mg/ml in flavored syrup, are stable for at least 1 month and can be prepared for younger children. The dose may be increased by approximately 5 mg/kg/day every 8-12 weeks to a maximum dose of 30 mg/kg/day or until there is evidence of toxicity (see below).
Consider folate supplementation, 0.4-1 mg p.o. q.d.
Monitoring
1. CBC, reticulocyte count: baseline, then every 2 weeks until maximum dose tolerated without toxicity for 8-12 weeks; then every 4 weeks.
2. History and physical examination: baseline, then every 4 weeks until maximum dose tolerated for 8-12 weeks, then every 8 weeks. Be alert to the possibility of recurrent or new splenomegaly and risk of splenic sequestration.
3. Fractionated bilirubin, ALT, and creatinine: baseline, then every 12-24 weeks.
4. Quantitation of hemoglobin F: baseline, every 3 months x 2, then every 6 months.

I suggest taking magnesium and L-carnitine along with hydroxyurea if available, as they both have been shown to improve results.

With all that said, I am concerned about the weight loss. If the weight does not come back quickly since the illness is over, chronic transfusions may have to be considered. The growth and bone development of the child is most important and this cannot occur unless the child's needs are met. Some children cannot thrive at all with an Hb around 7, so the child should be closely observed. If there are changes in the bone development, then transfusions would be recommended.
Andy

All we are saying is give thals a chance.

 

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