Q&A with Dr. Jeanne Boudreaux

November 9, 2014 – CAF periodically receives questions from patients on a wide range of topics related to thalassemia and its treatment. We have shared a few of these questions with Jeanne Boudreaux, MD, of Children’s Healthcare of Atlanta. We thank Dr. Boudreaux for sharing her expertise with us and with the thalassemia community.

If a patient’s hemoglobin is high (they are getting transfused every two weeks), why would the patient still feel fatigued or look pale?

Dr. Jeanne Boudreaux

How pale someone looks is a function of many factors. The level of anemia is the one we address with transfusion. Short intervals between transfusions can decrease the swings from low (pre-transfusion) to high (post transfusion). However, there are many other factors including baseline skin color and tone, sun exposure, degree of underlying jaundice, degree of iron overloading (bronzing), external temperatures (cold causes blood to flow away from the skin, giving the appearance of pallor), etc. In addition, low blood pressure and blood sugar can cause acute pallor.

With fatigue, again the hemoglobin is a big factor, but there are other factors to consider. Importantly, thalassemia patients are at risk for hypothyroidism and should be screened regularly. Blood sugar abnormalities, both high and low, can contribute. Poor heart function from chronic iron overload should also be considered. And then there are the rebound effects of caffeine, too little regular exercise, too little sleep, depression, side effects from some medications, etc.

The bottom line is that if the anemia has been optimally dealt with, then these other causes should be considered by you and your health care provider.

Is washed blood safer than blood that is not washed?

WashedUnitsThere are advantages and disadvantages to washed units. Generally, washed units are used in patients who have IgA deficiency or who have developed transfusion reactions that don’t respond to more conservative measures alone (leukocyte depletion, antihistamines, steroids). During the processing of a unit of PRBC’s (packed red blood cells), about 70 % of the plasma is removed that contains the proteins that cause those reactions. For most patients, that is sufficient. Washing the units requires an extra step, causes some loss of RBC’s (red blood cells), adds time and cost to the processing and requires the blood to be transfused within a few hours or the unit is rendered unusable.

Does washed blood eliminate the possibility of contracting Babesia and Chagas disease through blood transfusion?

No, the Babesia parasite lives both inside the red blood cell and in the surrounding plasma. Washing attempts to get rid of all of the plasma but does nothing to any RBC’s with the parasite inside. Irradiation and leukocyte depletion is also ineffective. Prevention of transmission through blood transfusion is through donor screening for a history of the infection; this is currently used here in the U.S. For Chagas disease, most U.S. blood suppliers test every donor once to see if they have evidence of previous infection. It is questionable whether irradiation, leukocyte depletion or washing helps to prevent transmission.

What are the advantages/disadvantages of using a port in pediatric patients? Are there age recommendations for its use? Do repeated transfusions (not using a port) have consequences for veins?

PortInfectionPorts can be used in any patient where IV access has become an issue. Often with young children, use of EMLA (a cream which causes the skin to feel numb) or similar cream, combined with distraction techniques with child life specialists and with IV access teams using devices that help locate good vessels is all that is needed. But we are not into torture and if getting IV access creates repeated episodes of extreme anxiety, then a port should be considered. The disadvantage is that ports can get infected, so if a patient with a port has a fever, they should seek medical care immediately to rule out a line infection. Ports require anesthesia to put in and take out and can increase the risk for thrombosis (blood clots). Peripheral IV access can cause some scarring of veins. Rotating sites helps decrease this issue.

What are the factors that determine how much blood a person gets when transfused – hemoglobin level? Height? Weight? Age? Can you get too much blood during a transfusion?

How much blood and how often to transfuse is a complex issue which a patient and provider must work through. It is based on age, weight, your hemoglobin abnormality (some mutations are more severe than others), etc. If your spleen has been removed, how much you need may change. In addition, if you have developed any alloantibodies, your transfused units may not last as long. Washing can cause some loss of RBC’s. Also there are periods of increased metabolic needs, e.g. pregnancy, puberty, heart dysfunction, etc. The degree of iron overload might also need to be factored in. Other issues to consider are lifestyle and distance from the transfusion center. Bottom line is that you and your provider must work together to determine your individual goals and modify as needed.

Yes, you can get “too much” blood. Over-transfusing can cause fluid overload and excess heart strain and increase iron overload without additional benefit.

Are there any ways to increase hemoglobin in a thal intermedia patient with autoimmune hemolytic anemia (AIHA)?

There are medications that can help treat AIHA, e.g. steroids, rituximab. Splenectomy has also been used. The risk and benefits of the latter must be weighed when considering splenectomy in a patient with thalassemia intermedia.

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