Grandson 8 years Hemoglobin Bart Thalassemia

My grandson had two hospitalizations one at 5 years old and the other last week.  He had severe abdominal pain, vomiting, and exhausation.  We were told that they could not be sure that these episodes were related to the Thalassemia.  We're frustated because we want him to receive proper medical care and we just do not know who to contact for answers.  We live Portland, Oregon area.

Any suggestions??

Do you have anymore information about his thalassemia? Hemoglobin Barts refers to alpha thalassemia. Does he have to take blood transfusions? Was his hemoglobin level checked during these incidents?

He has not received any treatment for his condition, and he has never received a blood transfusion.  We were told last week that his blood test results were consistent with someone with Hemogolbin Barts, and that when he is older he will probably need a blood transfusion.  His blood was tested during the first event, but HB was not mentioned.  We just thought that it could be related this time, because his symptons were similar to the first event, and the physcians could not explain why he was in so much pain.  They performed a Catscan, x-rays, blood tests, urinalysis...and they thought it was his appendix.  The also, said his potassium was a low, so he was given medication that he had to drink to increase his potassium levels.  They also, kept him on a bland diet, because everytime he would eat he would vomit and the pain would return for about one hour.  He received morphine to control the pain.  After four days his pain stopped and he could eat without pain.  We left the hospital with no specific diagnosis. The only information we have received is a letter from the State when he was born, because they test all newborns for thalassemia.

I would suggest a proper diagnosis be made. There are varying degrees of alpha thalassemia and this needs to be determined. There are four alpha globin genes and one, two or three can be deleted in alpha thal. In 3 gene deletion alpha thal, hemolytic crises can occur as a result to exposure to certain foods, primarily fava beans, and also to certain chemicals, such as naphthalene.
A list of things to avoid can be seen at
http://www.thalassemiapatientsandfriends.com/index.php?topic=3410.msg34830#msg34830

In a hemolytic crisis, the red blood cells break down quickly, which can be reflected in a swollen spleen and gallstones. Both can cause severe abdominal pain. At this point, this is pretty much a guess because I have little to go by, so I would suggest testing to find out exactly what type of alpha thal is involved. To find a thal familiar doctor in your area, please contact Eileen Scott at CAF. She should be able to guide you. If this is 3 gene deleted alpha thal, I would recommend that the child eventually be seen at Oakland Children's hospital, probably the most experienced center on earth when it comes to alpha thal. But first it must be determined if his problems are being caused by alpha thal so do contact Eileen and get started.


Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999

Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org

Andy,

Thank you so much for the information you provided, it will allow us to move in a postive direction to enure my grandson receives the medical care he needs.  We will contact Eileen, so that we can find a physcian in our area with knowledge of alpha thal.  Thank you for the "things to avoid list" too!  I am so thankful I found this website!!!   

Thank you!!!

Cheryl