situation changes for me...

Micky,

Your son's spleen is enlarged which is a little worrying and you realized why it was enlarged. It is all because the spleen was working hard for quiet some time to destroy low quality RBC produced by your son's bone marrow, the bone marrow was working hard to produce enough RBC to cope with the hb requirement of the body but due to low hb the body requirement was never satisfied, the bone marrow worked hard and keep producing low quality RBC, the spleen keeps destroying it. Can you think how much stress your son's body was taking. Do you realize since spleen worked so hard and since it is enlarged and so its capacity to destroy the RBCs in future. The over working of bone marrow can lead to deformaties in a child and osteoprosis.

Alomost everybody on the forum tried to give you advice to avoid Mr. Mathur but remained on guard with you providing support and advice. You wrote that Mr. Mathur is annoyed. Can you ask Mr. Mathur what plan he has for the enlarged spleen (a major source of destroying the RBC). Obvoiusly, he does not know enough and not realize the implication of his own treatment.

I am so upset right now, your son is dear to us all please take care of him.

Micky,

You are doing the right thing at this time so please be strong. It won't be as bad as you think. Kids get poked with needles all through childhood. Keep your child occupied during the transfusion. Read to your son, share some special time with him. When I visited the thal center in Dubai and saw the young kids getting transfused, there was no sadness. There was just a bunch of kids playing games, watching tv, and having fun while they transfused. Your approach can make a big difference. Go in with the attitude that you will be spending some quality time with your son and try to make it a fun experience for him. The context that you create can make a big difference in how he reacts to the situation. Don't ever show fear as your son will see it. Be strong and think about the friends you have here who want to see the best for you and your child.

I also want to repeat that a one time transfusion will not make your son transfusion dependent. I am absolutely certain your son is an intermedia and not a single person in this group can tell you yet if your son will become transfusion dependent. Only time will tell. Hb level is not the sole criteria for determining when and if an intermedia should transfuse. Your son needs blood now because of recent illness. This is common among intermedias, but it does not mean that this will be his routine. In addition to Hb level, the whole clinical picture must be observed. Bone development is a key. If your child does not have proper bone development and is showing abnormalities, regular transfusions will be required. If bone development is normal, even if slow, and the rest of his clinical picture is satisfactory, regular transfusions should not begin at this time. Only a competent doctor who understands thalassemia and specifically thal intermedia, can help you make this decision. Thal intermedia is a tricky condition and it takes a trained doctor to advise you correctly. I hope you will have your son throughly examined and that his Hb is tested regularly. As the parent, you have the responsibility of being objective and honest about his health. When you can do this, you will know when your son needs help. If his health is suffering, you must get him transfused. Please talk to a medical doctor who understands thal intermedia. Many intermedias can live without transfusions but at times, a transfusion may be necessary. The fact that your son has maintained an Hb of over 7 when not sick does give hope that he may fall into the category of intermedias who do not transfuse regularly. I would say that at best, Dr Mathur's treatment has resulted in some small Hb increase, but I feel you might get the same effect from much more inexpensive methods like wheatgrass and folic acid.

Do not rush into regular transfusions until you are certain your son cannot maintain an Hb of at least 7 as regular transfusion will make him transfusion dependent. If his Hb has dropped back down below 7 after a few weeks you may have no choice but no one knows if this will happen. The months ahead will be very important in determining your son's future. It is essential that you find a doctor who understands the differences between major and intermedia and which criteria must be used to make this determination about regular transfusions.

None of us have seen your son. We don't know how he looks or what his activity level is like. In this group we have heard how Manal's son is a bundle of energy in spite of low Hb. Hb is one factor but not the only one. It is very important that you are objective when assessing your son's health. If he can keep up with the other kids, and seems normal, then you may be able to avoid regular transfusions. If he is regularly sick and lacks energy, you may have no choice. Please step back and try to make an objective assessment of your son's health, as this will aid the doctor in determining whether regular transfusions should begin. We can give you information and give you examples for comparison but we can't make this decision. Please pursue proper care from this point on. There are many good medical doctors in India. Always seek competent care for your son.

Transfusion from parent to child is possible if there is a match. However, there is one very important process that must be done to make sure the blood is safe. Some years ago, a child in Australia got the hiv virus from a transfusion in what doctors called "a one in a million" chance. Odds mean nothing when it is your child. In response to parents who wanted to provide their own blood to their child, the hospital established the following criteria. Please take note of this one crucial guideline:

Directed blood donation from first-degree relatives is associated with an increased risk of graft-versus-host disease.  To prevent this, all directed donations must be irradiated prior to transfusion.

http://www.rch.org.au/bloodtrans/circumstances.cfm?doc_id=9184

Directed Blood Donation at RCH

    *
      Safety Issues
    *
      Blood Group Compatibility
    *
      Eligibility for referral
    *
      Steps for directed blood donation
    *
      What does a directed donation blood pack look like?

Directed blood donation of red cells, from parents to their child may be available in certain circumstances. The following information is provided as a guide to determine which families may be eligible for referral to the haematologist for further discussion, blood group testing and referral to Australian Red Cross Blood Service (ARCBS).
Safety Issues

Parents may request directed blood donation in the belief that this is a ‘safer’ form of transfusion. Several large studies have shown that the prevalence rate of infectious disease markers is not necessarily lower in directed donors compared with volunteer community donors.

Directed blood donation from first-degree relatives is associated with an increased risk of graft-versus-host disease.  To prevent this, all directed donations must be irradiated prior to transfusion.
Blood group compatibility

Being the ‘same’ blood group as the child does not necessarily mean that a parent’s blood is compatible. The haematologist will take into account:

    *
      the child's blood group
    *
      the potential donor’s blood group
    *
      if the child is a neonate - the maternal blood group, including presence or absence of atypical antibodies and ABO antibodies
    *
      Kell compatibility - it is the practice of the RCH Blood Bank to issue Kell compatible blood to female transfusion recipients

Eligibility for referral

The following criteria must apply before families are referred to the haematologist for further discussion:

    * There must be a reasonable likelihood that the child will require at least one blood transfusion.  In most cases this will be elective surgery with a greater than 10% likelihood of requiring transfusion.
    * The transfusion episode should be expected to occur within a single episode of care and with the expectation that the likely transfusion volume can be met by a directed donor.
    * The alternatives to non-directed (homologous) blood including autologous pre-donation, blood salvage and other techniques are either not appropriate or not available.
    * There must be adequate time for collection. ARCBS requires a minimum of five (5) working days between blood collection and likely date of transfusion.  More time will be required when more than one unit of blood is requested.
    * The parent(s)/guardian must be eligible to donate blood and fulfil all ARCBS donor eligibility criteria.

Referral is not appropriate in the following circumstances:

    *
      Mothers who have had premature rupture of the membranes (PROM) or chorioamnionitis, who are febrile or taking antibiotics are not eligible to donate blood because of the risk of bacterial infection.
    *
      Patients who have an anticipated need for Fresh Frozen Plasma (FFP) and/or platelets are unsuitable for directed donation.
    *
      Directed Donation for children with malignancy who may require future bone marrow transplantation is not recommended.

Steps for directed blood donation
1. Does the clinical situation fit with the guidelines above?

If yes, a referral may be made:

    *
      for outpatients refer to the Transfusion Clinic - held weekly on Monday afternoons.  In general, referral should be made four to six weeks prior to elective surgery. 
    *
      for inpatients referral can be made directly to the Haematology Registrar.  Please note the minimum time required.

The referral must provide the following information:

    *
      date of the planned transfusion
    *
      procedure being performed
    *
      anticipated likelihood of transfusion
    *
      number of units required (total anticipated blood requirement)

2. The Transfusion Clinic

    *
      The haematologist will meet with the parents to discuss the issues and arrange for parental blood group testing where appropriate.
    *
      If parental blood group testing is compatible with the child, the haematologist will refer the parent to ARCBS for blood collection.

3. Provision of directed blood donations

    *
      ARCBS will collect blood from the eligible parent.  Once infectious disease screening is complete (and negative), the directed unit will be forwarded to RCH Blood Bank.
    *
      A crossmatch request with recipient blood sample must be received by the hospital blood bank not more than 72 hours before transfusion.  The request form must indicate that Directed Units are available in addition to the usual information.
    *
      The RCH Blood Bank will issue the directed unit(s) to the child if required.
    *
      In the event that a patient requires more blood than the directed donor has provided, the directed units should be used first.
    *
      Any unused directed units will be destroyed on expiry. Directed units will not be re-issued for transfusion to any patient other than the intended recipient.

4. Administration of directed blood donations

Blood product administration, investigation of transfusion reactions and all other procedures relating to the transfusion of directed donations must proceed as per established hospital guidelines for the transfusion of non-directed blood.

I,ve also heard that a close relative should not be the donor,specially if you wanna go for BMT in future,plz check this out with ur doctor.

Micky,

I know he would cry,ofcourse it's his first time,but plz rest be assured that it's for his safety and health,be strong your self ,try to divert him with new toys or anything he likes,wish you good luck.We are all here for you.

ZAINI.

my thoughts are that he should be on a hypertransfusion regimine until his spleen and liver return to their normal size. 
What are your thoughts andy?

I don't think hypertransfusion will be applied unless it is established that excessive hemolysis is taking place. At this point, it is important to take one transfusion and see what happens to the Hb after that and how quickly it drops. The spleen is commonly enlarged in intermedias and is one of the reasons that splenectomy has traditionally been part of the treatment for intermedia.

Because of the lack of consistent medical intervention in this case, I don't think we really know enough to speculate on what course treatment will now take. It is important for Micky to understand that regular medical treatment is necessary and only after talking with a competent doctor, will he be in a position to know what to expect. I realize this is a trying time for Micky and I don't want to alarm him with speculation. We haven't seen his child so we are not in any position to tell him how his child is doing compared to other non-transfusing intermedias. This is why it is essential that a doctor who understands the difference in the approaches to treatment for intermedia and major is involved in his treatment.

Micky, I hope you take notice of the post I made about blood donation from close relatives and that the blood must be irradiated before given to the child. The risk of host vs graft disease is very real and should not be taken. This cannot be overemphasized and lack of facilities to irradiate the blood is why our fellow members have been told not to donate blood for their own children. Also, if you have paid for further treatment from Dr Mathur, please use it as it may have some small positive effect on Hb levels for your son.

hi. transfusion is done.. very painfull at starting for 3 hrs. then my son forgot and started playing but cant forgett this day ...

please now advice what to do. at the time of bt my son hb was 5.3, and he was given 200ml + about 250ml  of blood,  doctor told me spleen and liver is enlarge but with regular blood transfuion it wil get normal, and i should take more of bt in 3 week time for this enlarge spleen and liver untiel they get normal.  but as what andy says i should rather check this hb level , how it goes down so then should i wait?

otherwise also at that time my son was very active he was eating and playing though he dosnt walk for long time but for 15-20 min he can walk and dance like normal kids at hb level of 6.

now for slpeen as doctor said its 4-5 cms  long  he touched the stomach and observe this and for lever also , now we plan to go and o the ultrasound of stomach to see the exact of how much is slpeen and liver was enlarged.

so please how much HB SHOULD I MAINTAIN BEFORE GOIN TO BT incase hb is not falling that fast, also we had done the test for  TYPE OF THALASSEMIA MUTATION for my kid to know the exact type,i m aware whatever the result comes out so i need your advice on that also.

is there anything to worry about for enlarged slpeen 4-5 cms and liver- 6 cms?? or with regular blood transufion i can relax on this?

regs
micky

Hi Micky
250ml???I am shocked. Micky the amount of blood transfused should be between 12-15ml/kg. Was he monitored for BP, pulse etc... throughout the transfusion.Was his post transfusion hb checked? Hassan weighs 11.6kg and he was given 170ml. When too much blood is transfused at one time it puts a lot of pressure on the heart.
take care
MAHA

at the time of bt his hb was 5.3, and weight was 9.2 kg.  he was not check for bp or other things at the time of blood tranfusion but various test was done before starting it. doctor said the spleen and liver is enlarge and would take 4-5 months to get back to normal

Hi Micky
I feel he should have been given a max of 150ml this time and probably followed by 150ml again after 3-4weeks instead of giving 250ml at one go. This is what our hema told us . Are you taking him to a thal center.
Take care
Maha

Hi Micky,

I think 250ml is excessive, my daughter is 16.5kg and she gets 236ml every four to five weeks. I agree with maha that 150ml should be repeated after 3-4 weeks instead of 250ml at once. There has to be a regular blood pressure and pulse check up every hour during the blood transfusion to monitor the heart activity.

Where did you take your son for transfusion, if it was regular hospital set up and not a thal specialist clinic than the doctors may not know enough about the thal blood transfusion regimen.

I don't think the enlarged spleen and liver will come back to normal size in 4-5 months, it takes years and in some cases you have to wait for your son to grow enough such that the size is okay for him. When my daughter was first diagnosed as thal major at the age of 6 months, her spleen was already enlarged 1cm, we were advised by the doctors then that we have to wait until she grows. However, if your doctors are telling you 4-5 months than stay tuned for that.

I think you are on right track, please continue to see specialised thalassemia doctor and keep us posted.

Thanks.

Micky,

Because your son's Hb was already so low, he may need another transfusion in three weeks. It might take a couple transfusions to see what Hb level he can maintain on his own. If it continues to drop between transfusions, he will most likely need to stay on regular transfusions. For now, monitor his behavior and observe if there is a significant change in his energy level, his color, and his overall health. If these all improve, you may decide that transfusion is the best for him. I would also suggest starting him on some form of wheatgrass, along with IP6. Both are safe to take at all times.

Of course, we are all here to help guide you through these times.

All the best Micky,
We hope that your son's health improves quickly.  We are all here for you.  Hopefully your son will improve after a few transfusions and will be able to maintain his hg on his own. We're all here to support you. 

Sharmin

Micky,
We are praying for your son.

hi every1,
doctor suggested for 200ml of blood , but as nurse wasnt paying much attention also we were also not that alreat cauz my son was cryin a lot so by time we stop bt  it was about 250ml given , we did called doctro for this and she said it ok and give more of liquids to my son.

also after bt  today and yesteardy my son stomach is out and little hard we called doctor she said it happen after bt for 2-3 days.

ip6 i want to start to maitain iron from starting only.  so how much dose should i give to my son? as its one blood trasfuion is done yet?

please suggest.