Discussion Forums > Thalassemia Major

New to Thal Major - lots of questions

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germ79:
Hello to all!  My wife and I just discovered this wonderful forum and have some questions for you all.  My wife posted this on another forum as well, so I thought I'd just ask it here too.  This seems like it is one of the more active Thal forums. 

Thank you in advance for your time!

Jeremy

My husband and I are considering the adoption of a little girl from SE Asia who is Beta Thal Major. A year ago we adopted a little boy whose birthmother was Thal Trait but our son did not inherit any form of thal. For some reason God keeps putting these children in our hearts! We would adopt this little girl without question if we had no other children but we have 2 young boys and worry about how the needs of this child will affect our ability to meet the needs of our other children. I hope no one is offended by this - we know that all children come with risks and our other children are not "perfect" either (just "perfect" for us!) but we want to make sure that we are capable of giving her what she deserves.

I want to know what the day-to-day looks like for a person with Thal Major. Other than transfusions and chleation treatments, how much medical care is needed? Are there frequent hospitalizations? How long does it take to complete a transfusion? I'm sorry if these are ignorant questions - we are really at the beginning stages of research and I'm just trying to gather a clearer picture of what our lives would look like. I welcome any input or information anyone can give me.


Also...

More questions as we are still considering the adoption of this Thal Major little girl. I know there are a lot of options when it comes to chelation so there isn't one easy answer, but many of the resources I find state that it is "painful." I think one of the hardest parts of this decision for my husband is knowing that we will have to watch our child experiencing pain. Can you tell me what exactly is painful? Is it the port or the site or it is it the actual process? Sorry if this is dumb but we have never seen anyone with a pump or catheter or seen the process occur. Also, can you tell me about the transfusion process - I understand that it takes several hours depending on the amount being transfused but are you able to move during that time? Do they sedate babies? This little one would be about 1.5 yrs when we receive her. Also, (we will have her file reviewed by a physician so I'll ask the same questions then) but should we be concerned that she is 6 months old now and already receiving transfusions? Most families I read about have children who don't receive transfusions until they are over a year. Is this an indication of the severity of her condition?


Thank you for all the support and listening to all our questions.

Jeremy and Diana

Andy Battaglia:
Hi Jeremy and Diana,

I'll leave some of these questions for the parents in the group to answer but I can answer a couple.

Chelation has changed. While many patients are still using desferal, which requires a needle to administer over 10-12 hours, many patients have now switched to the oral chelator, Exjade. In the US, Exjade is approved for ages 2 and up. It is a once a day oral medication that is mixed with water or juice and drank. It has made life much easier for thalassemia patients and has made it much easier to have a normal life.

Early transfusion is becoming the norm with patients, as it has been found best to not delay so that development goes at a normal pace right from infancy. In the past, transfusions were often delayed until the hemoglobin dropped below 7-8, but now it is believed that it is to the child's advantage to start earlier and maintain the Hb above 10.

There is regular monitoring that needs to be done for all patients but for the most part, this consists of checkups, blood tests and the occasional scan to check for heart function and iron loading. Treatment has advanced greatly over the past 30 years, so please do not be frightened by anything you read from outdated sources. The prognosis for patients born today is far better than it was before the age of chelation, and the earlier treatment begins, the better the ultimate outcome will be. Today, there are patients who are in their 50's and they did not have the treatment that is available today while they were young, and in fact, chelation didn't even exist when they were young. Patients today have a bright outlook and future developments in treatment and even a cure, will only continue to improve life for thalassemics. In the US, there are even comprehensive care centers for thalassemia, located around the country, where an annual visit can provide both the total monitoring needed, and also create a treatment program based on each child's specific needs.

 It does require more dedication than raising a non-thal child, but it shouldn't take anything away from your other children. In many ways, it is a positive for the other children as long as they are taught to embrace and not resent the new child, but this is true in all adoptions.

I'd like to hear from some of the other parents and what their view is of raising both thal and non-thal children.

germ79:
Andy,

Thanks SO much for your reply.  It was wonderful to hear your positive outlook on raising a thal child.  Like you said, I would love to hear from other families too. 

We understand that this is going to be different than raising a non-thal child, but we are feeling very positive about it.  From all we've read, it really seems that it's all about what you make of it. 

Thanks again and I look forward to hearing some more responses!

Jeremy (and Diana)

PS.  We are still wondering why some articles describe the chelation process (using desferal) as painful.  Is it actually painful having the stuff injected or is it just uncomfortable being hooked up to the machine, or just a plain 'ol inconvenience? 

Andy Battaglia:
Desferal is painful in that it requires a needle to be inserted under the skin, usually in the abdomen or thigh and left for long periods. The desferal itself, can also cause irritation at the injection site and a lump after long term use. Imagine sticking a needle under your skin 5 nights a week and you will have some idea why it is considered painful. It has been a necessary evil for patients and a big reason many patients have not fully complied with treatment, especially as they get older and more involved socially. Most kids don't want to have to be pumping desferal when they're out with their friends. Oral chelation provides the potential for much higher chelation compliance and the resulting benefits in terms of good health.

germ79:
Thank you for the clarification, Andy.  I didn't know if the pain was from the obvious repeated needle pokes or something more. 

A couple more questions for you then (if you don't mind)! 

1.  I've read that most people chelate for 8-10 hours per night.  What if you don't sleep that long as you get older?  You just start before bed?  Chelate less?

2.  I've also read about a portacath.  Is that commonly used?  It SEEMS that it would be a good alternative to getting poked every night, but then again, you'd have to have the portacath inserted and in all the time.  Do you know anything about them?  Is that a good idea?  For kids too?

J

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