Thalassemia Patients and Friends
Discussion Forums => Thalassemia Major => Topic started by: Christine Mary on November 07, 2006, 04:12:22 PM
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hi everyone.
i thought you'd like an update on Lauryn. Her hgb dropped from a 9.9 to a 9 in 3 weeks. I am a little depressed knowing the day will come shortly that she will have to tx. the md says her body is starting to recognize the "failing hgb"levels. her body is working hard right now. :'(
the dr. spoke of the port again, and i frankly told him i would only resort to that in a "life or death" situation if need be. he understood my point, and hopefully we will never have to discuss it again. other than her hgb dropping, she weighs 12.9 lbs!( she was born at 7.5) lol. she is also a little over 22 inches long. she def. is a big girl.:) her heart and spleen are perfect. her nails and tongue are pink and her eyes are as white as cotton. considering ALL factors, she is doing great!!! :cloud9 he wants to see us in 2 1/2 weeks, and im not sure if we'll need to tx her then. he says he wants to tx her when she reaches the 7ish range. hopefully that will be some time from now. :pray maybe after the holidays?? :dunno
is there a possiblilty she can maintian a certain # for a prolonged period of time? Like,can she remain a 9 for 3 months??? That would be miraculous!!! wouldnt it? :love
i know its natural to have ups and downs,but if anyone can lift my spirits up,please do. :sigh i know if anyone can it would be you guys. hearing about the 19 yr old who just passed,made me feel horrible.
im hurting so much for my baby. :'(
Love to all,
Christine
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Hi Christine,
It is good to hear that Lauryn is doing good and from your posting I see that see that she did not need the tx yesterday .... that's real good. So, don't feel down .....
I do have a word of advice though :) Since Lauryn's hemoglobin is at 9 now, I would suggest that you monitor her closely so that you can pick up the first signs of anemia quickly and early - listlessness, lack of interest in feeding, paleness of lips, etc. I am sure that she will not show these signs in weeks to come and will be able to maintain her hemoglobin in the high 8's (given that she dropped just 0.9 in her hemoglobin count in 3 weeks)
You had indicated in your post that Lauryn's doctor has indicated that they will wait till her hemoglobin falls to the 7 range before they transfuse. Our daughter's doctor (at the Children's Hospital, Boston) had a different take on this - he indicated that for infants he tries to keep the pre-transfusion hemoglobin level in the high 8's. Different doctors have different opinions on this so don't be alarmed :)
I would appreciate comments from the other members of the forum on this as I am not an expert on this .. and do not have any experience except for what I hear about my daughter from her doctors.
Take care .. hugs to Lauryn
Bostonian
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Bostonian,
I think what we are seeing is a change in thinking regarding Hb levels in the very young. Previously, thals have been pretty much lumped together regardless of age in terms of what Hb level to maintain. The newer approach takes into account that children, and especially infants are developing at such a fast rate that low Hb levels can have a significant negative impact on development, and thusly, higher Hb levels are now being recommended so that as close to normal physical development can take place. Whereas an adult may be able to get by at an Hb of 7, an infant will not develop properly with an Hb that low.
Christine, I would seriously consider a visit to the Oakland Children's Hospital for advice. The first years of your child's life are so important in terms of physical development, especially the bones and organs and everything must be done to ensure that it proceeds. A comprehensive care program early on will make sure that Lauryn's development is at its optimum. I agree with Bostonian that you should closely monitor Lauryn for any changes in health or behavior and report these to your doctor.
I don't know if the Hb drop can be accurately predicted but so far it has been fairly normal, which would require transfusion to begin at 5-6 months if it continues at this rate.
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Christine,
Hang in there, girl. We are all hoping for the best for Lauryn. I am glad to hear that she has good color and appears to be doing well at least for now. I agree with Andy that a trip to Oakland would be extremely beneficial - for you and for her. You could get some answers to your questions, and Lauryn would be seen by specialists who deal with thalassemia everyday. Call today! I was able to talk to Dr. Viichinsky (international specialist) on the phone when I called from my hospital bed years ago. I was totally frustrated about not getting any help after years of trying and just called. What a shock to be able to talk to a thal doctor. That was several years ago, but you could call his excellent nurse, Dru Foote, and talk to her. They are really nice. You could even e-mail Dru at: dfoote@mail.cho.org
and ask her questions, sometimes it takes awhile for an answer, or just call Children's Hospital at Oakland and make an appointment with the thalasemia team.They know the latest thinking and trends in treatment. I have been to hematologists, medical schools, the Mayo Clinic, etc., but they just didn't see enough thal people to have many valid answers. That's what is good about Oakland - they see many thal people.
Another thing I did was just e-mail other thal specialist doctors at the thal centers to get their opinion on things. It's hard for them to give answers when they haven't seen the patient, but sometimes they have insights. I got replies from them, or from their nurses.
Good luck, Christine. If travel money is an issue, maybe the thal organizations could help pay for a trip. :bighug Jean
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Hi Christine.
look at it this way 19 good years is better than nothing,
none of us knows how long we will live,yes you are entilltled to feel sad but remember you are doing the very best for your daughter and you need all your strength and a clear mind to get on.beside ,I believe there will be a cure in few years time and Lauryn stand a good chance of getting that benefit.
So chin up girl :bighug and get ready for your next adventure;
Love kathy
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Dear Christine
What about the DNA test, you said you will take a sample when you do the CBC. Were you able to do so??
Anyway, cheer up and thanks to God that till now , we are in no need for transfusion.
Dear Andy
You always keep saying that children Hb has to be always in the range of 8 or 9 so that normal growth won't be affeced . So what about my son and how can he keep growing with this HB (lowest 6.1 and highest 7). Keep in mind that when i first discovered, he was 6.4 and no one knows how long he was living with this level. I am checking his growth in terms of weight and hight and he is within the normal range. My question is: Can there be anything that is not developing well and i do not recognize. He is active and smart. What else should i look for??
Thanks
Manal
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Christine,
Transfusion normally occur when the Hemoglobin is between 8 and 9, its the standard in Canada. A child will develop normally if this level is maintained, however no matter what; the transfusion will not be delayed farther than five weeks. In our case, my daugther is maintaining a hemoglobin of 10.9 to 11.3 (average) even after five weeks. We have asked to delay the transfusion by six weeks but it can't be done as they have to follow the protocol.
Lauryn's level is 9 right now and my guess is in 2 and half weeks if it drops further (I hope not) than she may need a transfusion, but normally doctors give a child full chance to see if the Bone marrow may work a little bit (they can go upto 7 to check that). So keep your hopes up and pray.
Regards
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Hi Manal,
I know that some of us (which could have strong will powers) can manage to continue even with extremely low HB (like me :biggrin) but it is not really wise to do that for small children especially when their rate of growth is higher compared to adults. Keeping them in low HB might not effect their behaviour but it could effect other things that are invisible to us such as retarded growth, organ development and bone marrow over work which can deform fragile bones.
Maybe it was keeping me in low HB that has enabled me to unlock the secret of fountain of youth :winky
We don't want that to happen to any of us do we?
Take care, Peace!
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Hi Christine!
Don't worry too much. We all are with you. The Laryun looks great in the new Display Picture.
Cheer up and hopefully her HB will mantain at 9 :thumbsup
Take care, Peace!
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Hi Manal,
The most important thing is that your son has his growth and development closely monitored. Intermedias do make some useful blood and the Hb does usually stabilize at a level of 6-8, so they are not in the same danger as majors concerning dropping Hb. Hb of 6-8 is just one more step for majors as their Hb drops to the 3-4 range, so it is necessary to start transfusion and soon as it is evident that the Hb drop isn't going to level off. With intermedias, transfusions are not recommended until there is obvious evidence that the low Hb is causing developmental problems.
If you can show this to your doctor he may be able to tell you if this is a test they can do there. This article says this is a definitive test for determining when intermedias need transfusion.
From http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3426965&dopt=Abstract
A simple index for initiating transfusion treatment in thalassaemia intermedia.
Sbyrakis S, Karagiorga-Lagana M, Voskaki I, Efthimiou H, Karaklis A.
School of Health Sciences, Division of Medicine, University of Crete, Iraklion, Greece.
In order to develop an objective test for discriminating between patients with thalassaemia intermedia requiring blood transfusion, and those not likely to require transfusion the medullary width (MW) in the midpoint of the second left metacarpal and the bone mass were measured in 34 normal children and in 37 patients. In patients, blood haemoglobin (Hb) and serum ferritin concentration were measured and cephalofacial deformities (CFD) were scored. The mean values of MW were 0.37 +/- 0.06 and 0.44 +/- 0.1 cm (P less than 0.01) and the bone mass 0.091 +/- 0.012 and 0.078 +/- 0.02 (P less than 0.005) in normal children and patients, respectively. In 13 of these patients who had MW more than 2 SD above the mean of the controls, i.e. more than 0.5 cm, regular blood transfusions were instituted. Measurements of MW 12 and 1 month before and 12 months after the initiation of transfusions showed an increase from 0.50 to 0.60 and a decrease to 0.49, respectively. Bone mass measured at the same times decreased from 0.083 to 0.045 and increased to 0.071, respectively. These changes were visible radiologically. It is concluded, therefore, that the measurement of MW seems to be an objective, simple test for discriminating between patients requiring or not blood transfusions, and that bone deformities will be reversible if transfusions are instituted using as criterion the MW (greater than 0.5 cm) regardless of age or haemoglobin concentration. This test may help clinicians to decide about the optimal time for institution of regular transfusions in patients with thalassaemia intermedia.
PMID: 3426965 [PubMed - indexed for MEDLINE]
I would also like to ask Jean what her experience growing up was. Was your development normal as a child? Do you feel transfusion would have helped you as you were growing or was it only as an adult when the problems became severe enough to warrant transfusion?
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Many thanks to you Sajid and Andy.
Andy, i will discuss this test with the doctor in the next visit and let you know.
thanks
manal
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Hi Christine,
thank you for inviting me to join this forum. I was busy last time and had not time to write. But now here I am.
Lauryn is in aperfect state. A decreasement of Hb from 9,9 to 9 is not so much in three weeks for a thal major, but it shows that production of adult Hb increas and the fetal Hb decrease. That is normal. The oppinion when it will be the right time to transfuse first time change from Doc to Doc. Some says the Hb should not underline 8 g/dl other means that only if a person can't manitein by herself an Hb of 7 g/dl you will be shure that it is a thal major and not intermedia. It is difficult to decide which oppinion will be better because both aspects can be right.
No one of use can predict when Lauryn will have her first transfusion. But annyway she is under control and more. She is growing up in a family who loves her so much. I think that is what she will need for all her life. It dosent matter if she is Thal or not. You can't take away the thal, but you can give her all your love. I know what i am speaking from. She will be greatfull for your support it dosn't matter how thinks will go.
Best wishes
Gabriella
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Hi all,
Since I didn't know any of my hemoglobin levels until I was in my 20's, (when I found out I had thal intermedia), I can't give much advice on levels in children. I know I was sick a lot as a child, and I would have simple illnesses longer and more intensely than others in my family. But when I felt well, I had lots of energy. I think I subconsciously had this feeling that I'd better do everything I could while I could, until I was down again. I am sitill like this today!
My spleen was enlarged, but they didn't correlate that with thal at the time. I also had pains in my legs, that my pediatrician said were growing pains. Otherwise, I developed fairly normally - went through all developmental milestones at the normal times. I am 5'3".
It wasn't really until my early twenties that I started getting more and more sick with infections and very tired. My spleen was getting bigger and bigger. I seemed to be really sensitive to smells, chemicals, foods, etc. I was hospitalized for 10 days because I couldn't keep food down. Finally I found out what I had, and the doctor said my body was used to a low hemoglobin, and that I was just fine with a hemoglobin in t he 7 to 8 range. Then over the next years I had many problems with pain, infections, etc., and I finally, iin my 40's had my spleen and gall bladder out. The large spleen was making me really sick.
Anyway, I didn't start on regular transfusions until I was 52! I wish I could have started on them earlier because my hemoglobin was going in the 6 range by then, and it was difficult for me to stay healthy. If I could have started in my 20's, that would have been great. I started having a lot of pain in my left cheekbone and my hips, shoulders, legs, etc. My bone marrow was working extremely hard. They think I also have fibromyalgia, so I don't know how much that affects everything.
I hope this helps - sorry to repeat my story for the 700th time for those who have heard it. I think a person can usually do fairly well with intermedia until he or she gets older. This is just my story though, others may have different experiences. All in all, I have done fairly well. Jean
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Hi,
My son was first diagnosed at 11 months with HB of 5.3. It has taken a bit of toll on his development. He was able to lift his head up muchat that time, because it was so big compare to his body. I feel that it is not advisable to let the HB get too low before transfusion. His upper jaw juts out so much he now has a speach problem. We are now trying to fix his pronunciation problem.
I have also refused a port. It was difficult to watch sometimes when he has to restrained by 5 adults, and they can't get the IV in until the 7th try, and that he would has screamed for 1 and a half hours. However, it'll pass. Since he was 2 years and 3 months, he sticks his own arm out and tells the doctor when he wants to be poked.
Best wishes to you.
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Hi Jia,
That's a brave son you have!
May God Bless him!
Take care, Peace!
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Hi Manal;
I hope you are feeling better I'm thinking of you,
Regards Kathy :smile2
:flowers :flowers :flowers :flowers :flowers :flowers :flowers :flowers :flowers
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Hi all,
Since I didn't know any of my hemoglobin levels until I was in my 20's, (when I found out I had thal intermedia), I can't give much advice on levels in children. I know I was sick a lot as a child, and I would have simple illnesses longer and more intensely than others in my family. But when I felt well, I had lots of energy. I think I subconsciously had this feeling that I'd better do everything I could while I could, until I was down again. I am sitill like this today!
My spleen was enlarged, but they didn't correlate that with thal at the time. I also had pains in my legs, that my pediatrician said were growing pains. Otherwise, I developed fairly normally - went through all developmental milestones at the normal times. I am 5'3".
It wasn't really until my early twenties that I started getting more and more sick with infections and very tired. My spleen was getting bigger and bigger. I seemed to be really sensitive to smells, chemicals, foods, etc. I was hospitalized for 10 days because I couldn't keep food down. Finally I found out what I had, and the doctor said my body was used to a low hemoglobin, and that I was just fine with a hemoglobin in t he 7 to 8 range. Then over the next years I had many problems with pain, infections, etc., and I finally, iin my 40's had my spleen and gall bladder out. The large spleen was making me really sick.
Anyway, I didn't start on regular transfusions until I was 52! I wish I could have started on them earlier because my hemoglobin was going in the 6 range by then, and it was difficult for me to stay healthy. If I could have started in my 20's, that would have been great. I started having a lot of pain in my left cheekbone and my hips, shoulders, legs, etc. My bone marrow was working extremely hard. They think I also have fibromyalgia, so I don't know how much that affects everything.
I hope this helps - sorry to repeat my story for the 700th time for those who have heard it. I think a person can usually do fairly well with intermedia until he or she gets older. This is just my story though, others may have different experiences. All in all, I have done fairly well. Jean
I'm quoting this post both for my own reference and to remind myself to write a more thorough and coherent response when I wake up in the morning!
With that said, I'm absolutely amazed at how similar my own story sounds compared to yours!
I have a form of alpha thal called Hemoglobin H Disease and my Hg count is consistently at a high 7 and I too have 'grown used to' my hemoglobin.
My illnesses have always been rather frequent and took longer to deal with, but other than that and some of the 'random' (hey, -I- thought they were random!) symptoms you described (including the horrible leg pains that I thought only I ever had growing up!), I was 'fine' until more recently when I hit adulthood and suddenly, I didn't feel so 'fine' anymore. Either that or my coping issues suddenly became more apparent as adult concerns and responsibilities and activities started coming into play.
I started getting sick all the time all year round once I hit adulthood, which was 6 years ago. They were so frequent and bad that my doctor thought I had an auto-immune disorder and for the last 4 years, I've been dealing with severe chronic depression.
I tried a trial run on transfusion therapy and it bumped my Hg levels to 9.6, but I really didn't notice much, if any difference, and it discouraged me from considering transfusion therapy.
But the more I've thought about it, the more I wonder if maybe I should seriously consider it as an option. ???
Thanks so much for posting your experience and thanks to Andy for linking me here!
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Dear Christine,
I wish you could talk to my mother; she found out that I had Hemoglobin H Disease when I was not even 2 years old and I'm sure she went through a lot of the same worries and fears that you're having right now and would be able to at least share some thoughts with you.
:hug
But since my mother can't be here right now, I'll share my memories of growing up as a child with an intermediate form of alpha thalassemia. After reading Jean's story and comparing it to my own memories, I realize that the symptoms of the intermediate forms of both types of thalassemia are quite similar.
My Hg count has always been somewhere in the 7 range (I will make an important note and say that my Hg levels, while low, are persistent and constant at this level and for the most part, doesn't fluctuate much unless provoked by illness or something else) and I never did transfusion therapy or was recommended to do transfusion therapy. This was back in the early 80s, so there wasn't too much research done on thalassemia yet, especially in my neck of the woods, I was told, but my mother was told at the time that my own body would adjust to my lower Hg levels.
And it's true. My body did learn to adjust to my lower Hg levels as far as I'm concerned.
As for my symptoms or evidence of my anemia, my most noticeable symptoms came in the forms of fatigue, leg pains/growing pains in my legs from %$#@ for several years, enlarged spleen, higher iron counts, and a definite penchant for catching colds.
I caught colds easier than most kids and took longer to get rid of them. Despite having caught pneumonia and bronchitis on multiple occasions, I never had a transfusion even though my Hg count did drop. I was hospitalized on several occasions because of the severity of the colds, but more often than not, my own mother who is a former nurse, took care of me at home and was very very mindful of getting me well as soon as possible.
My mother told me that the doctor advised her to:
- Watch out for fevers - NEVER let a fever get too high and go unchecked
- Don't let an illness prolong itself - Especially if there is fever involved, don't let an illness go unchecked
- Monitor the spleen size - Though that's more of a doctor's job ;)
- Monitor growth - Again, more of a doctor's thing
- Watch for iron absorption in the stools if there's iron supplements being given (iron deficiency -can- happen with thalassemic patients!) - Stools that are dark indicate that iron is being absorbed
- Make sure I have a healthy diet that is lower in iron and rich in antioxidants
- Make sure I don't overwork myself/push myself too hard in physical activities
- Rest when I need to
I know for HgH Disease patients, the symptoms don't necessarily show themselves until later and that the Hg levels sort of plateau out and settle into a number range and stay there. In my case, my Hg levels settled on the 7 range and once it settled, it seemed that my own body resettled itself out according to the Hg levels.
I'm not sure if Lauryn's thalassemia condition does or will do the same, but if it is the sort to 'settle' out at a number, that's the better time (so I have been told by my former pediatrician) to measure how 'anemic' she is because then you have a 'base' number to compare with.
As far as I know, I was at an 8ish when I was first diagnosed and I've been in the high 7s since, and now at 24, I'm a grown adult - 5'4", healthy looking with no noticeable bone issues except in my pinky finger on my left hand.
But I have an intermediate form of alpha thalassemia and not beta thalassemia, so I don't know if the symptoms for someone with beta thal in my Hg range are different or the same/similar as mine.
I've lived with my 7 Hg all my life and only did a trial transfusion run 2 years ago and during my last checkup, my hematologist was very pleased that I had turned out the way I did and really, I have to give all the credit to my mother.
She was the one who loved me and nursed me in sickness and in health for all my childhood life and during my more trying years in my adult life.
She never let my illnesses, though frequent, go unchecked for long and was always at my bedside when I had bad fevers. She gave me very nutritious meals from babyhood to adulthood and encouraged me to listen to my body and to its needs. She also treated me like I was any other normal kid otherwise and I was, as long as I didn't have to run to keep up or stay up late at nights to write papers.
Christine, I know you're worried, but cheer up! It's scary to have to deal with something so unpredictable as thalassemia, but there are others here who have been through it and there are other parents who have raised children with thalassemia.
I've been through a bit of a ride myself in terms of my thalassemia, but my mother's love and care pulled through! Mother's intuition frequently knows best (in my mom's case at least) so if you follow your heart and listen to what it says, I think you'll be able to make the right decisions to give your daughter the healthiest outlook possible. :)
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And Christine?
I live in northern California and I have been to CHO (Children's Hospital Oakland) before. :)
I'll be more than happy to help you get more information on CHO and if you happen to come this way, I'd certainly be glad to meet you if it's something you'd like to do.
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Zadkhi,
I'm glad you mentioned CHO because I was going to suggest you visit there. I am curious as to what they recommended in terms of transfusing. Also, has any doctor ever suggested trying hydroxyurea?
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Hi, Zadkhi,
Too bad I couldn't have met you the times when I went to Children's Hospital in Oakland! We do have similar stories. Before I went on regular transfusions, I tried a trial every other month for 6 months. That didn't make much difference in my health. My average monthly hemoglobin was not being raised enough (my opinion). So, I didn't start transfusions for awhile longer. Then I started doing worse, and I started monthly transfusions, with the goal of keeping my monthly hemoglobin level at a mean of 12. It's been 3 and a half years, and I am so much healthier. I don' think doing a short trial will really tell you that much (my opinion), when your body has gone for years at a low hemoglobin. It takes awhile for your body to adjust to a healthier level of health (if that makes sense).
Good luck, Zadkhi, and nice to meet you. If I go back to Oakland, I will get in touch with you. My brother lives in Pleansanton. :hithere Jean
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OH MY GOODNESS!
I LIVE IN PLEASANTON!!!
:rotfl
Ohhh my gosh, why couldn't I have met you before?! This is just completely amazing! :lol
My biggest Eeeeep! factor in going on regular transfusion therapy has been both the worry about iron chelation and overload and money-wise as transfusions are very costly and I currently don't have medical insurance because I'm stuck between almost graduating from university and finding a job. :huh
I'm definitely thinking very seriously about it though and I fully intend to discuss it with my hematologist the next time I see him. Your story has given me more reason to really consider that avenue of treatment. :)
And definitely let me know if you come by the area!
Who would have thought that I'd meet someone who could theoretically be so close by?!
:yahoo
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And to Andy -
Honestly, it's been several years - over 2 - since I've visited CHO. Where I DID get counseling from them in regards to my thalassemia, I didn't do treatment under them because I was under the care of Dr. Lewis from Alta Bates at the time and had landed myself at CHO because of a mild thalassemic emergency.
Dr. Lewis was very encouraging in wanting me to look into CHO though, despite it being a Children's Hospital and all, and I do believe I'll be following his advice. :)
On a related note, the Northern California Comprehensive Thalassemia Center @ CHO recently sponsored a walkathon for thalassemia in Berkeley! I was in attendance and it was awesome to see thalassemia activism at work!
My initial impression of CHO and of the doctors that have worked with CHO or are working with CHO is that they're very caring and dedicated people and have quite possibly one of the strongest comprehensive thalassemia centers in the US.
I'd love to be able to work with them to help promote thalassemia awareness and if possible, be a patient under them as well.
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I CAN'T BELIEVE YOU LIVE IN PLEASANTON! That is just so amazing. I will definitely contact you if I go out there. I haven't been there in a few years. CHO is a really great thal center. I want to go back there.
Transfusions are really expensive, and so is chelation. One would have to be very wealthy or have insurance to start treatment.
What are you majoring in at the university? Take care, Jean