May 2012                                                                                              Volume 20
In This Issue
Camp Sunshine
Children with Chronic Illness
Show Us Your Logo!
DBA Fact #9
Journal Club
National Family Meeting at
camp logo


The Diamond Blackfan Anemia Foundation, Inc. (DBAF) is proud to announce our eighth national family retreat. This retreat will take place during the week of  July 8-13, 2012 at Camp Sunshine, which is located at Lake Sebago, Maine. Air carriers fly into Portland, Maine and ground transportation is provided by Camp Sunshine to the campsite.


Final selection of families chosen to attend is determined by Camp Sunshine.  Completed applications and physical examination forms should be returned to Camp Sunshine as soon as possible, due to the limited availability of family accommodations.  Travel expenses are the families' responsibility.  


If you have any questions regarding Camp, please contact Dawn Baumgardner at 716.674.2818 or 


Additional information and applications are available at

Upcoming Events


DBA Family Meeting 
July 8 - 13, 2012 
Camp Sunshine
Casco, ME 
Dawn Baumgardner 

Friends of DBAF Golf Outing
& Silent Auction  
September 15, 2012 
Cherokee Hills Golf Club
Valley City, OH
Jim & Carol Mancuso
Ongoing Fundraisers

Family Letter Writing Campaign                           
Pre-printed letters and envelopes have been created for you to send to your contacts! Call or email for more information.
Dawn Baumgardner

Wristbands Available 
Twila Edwards
Tribute Cards Available
(2 Styles)
In honor of...
In memory of...
Dawn Baumgardner 
 donation donation

5" x 5" Decals Available
Dawn Baumgardner
window sticker 

Cookbooks Available 

Betty Lightner
To order online, visit:
Cookbook order form
cookbook cover



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Raise money for DBAF 
just by searching the web and shopping online! 

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:: 716-674-2818
The Diamond Blackfan Anemia Foundation(DBAF) is committed to keeping you updated and connected to the entire DBA community. The DBA Foundation is YOUR Foundation!  We encourage you to share your ideas, photos, and stories for our website and upcoming newsletters.  Contact

Children with Chronic Illness: Dealing with Emotional Problems and Depression

(Source: from the American Academy of Pediatrics)    

Healthy kids logo

Feelings of sadness, depression, or being overwhelmed may come and go for both you and your child. This is normal and healthy. As tumultuous as these times can be, most youngsters and their families emerge with few if any long-term behavioral problems or lasting psychological scars. In fact, the majority of children really do manage their situation well, despite riding an emotional roller coaster on occasion. Researchers believe that the likelihood of emo­tional and behavioral problems associated with chronic illness has been de­creasing recently because parents, school staffs, and health-care providers are learning more effective ways to help children and their parents meet their psy­chological needs.


Nevertheless, children who have a chronic illness or condition often feel "different," socially isolated, and restricted in their activities. They may have school problems and feel overprotected. They may experience recurrent fear and pain. When these emotional difficulties are not dealt with, they can lead to anxiety, sadness, withdrawal, rebelliousness or a decreased interest in school.


School-age children rarely state that they are sad or depressed. Instead, they may withdraw from friends and family or exhibit rebellious or angry be­havior. They may do poorly in school. They may interfere with their medical treatments, perhaps by refusing to take medication as scheduled. They might experiment with alcohol, drugs, or early sexual activity. Or they may run away from home or contemplate suicide.


Make an ongoing effort to discuss with your child what he is experiencing. Do you think he is displaying signs of despair and hopelessness related to his illness and future? Encourage him to talk about these feelings with you or with another trusted adult. Because your child may not even be aware of his feel­ings, try beginning these conversations with statements like "If I were you, I think I would be feeling..." or "I have read that many kids with this condition feel lonely and sad. How do you feel?"


Some parents are hesitant to discuss feelings about the disease with their child, in an effort to protect the youngster from emotional hurt. Most experts, however, disagree with that point of view. Children can usually adjust much better to an unpleasant truth than to the perception that their parents are up­set and hiding something from them. If parents and children do not talk openly, the opportunities for misinterpretation are high. A youngster's imagi­nation can run wild, and fears may emerge or be exaggerated.


Thus, it is best to make a commitment to be as communicative as possible. Remind your child that he is not going through this alone and that you will re­main a constant source of love and support. Many studies show that the key to a child's resilience is a relationship with a caring, loving, accessible adult-someone the child can count on and trust.

If you are concerned about your child's coping with these stresses, talk to your physician. If your youngster is exhibiting destructive or unusual behav­ior, if he refuses to take his medication, if he is severely withdrawn or if his schoolwork has deteriorated, your doctor might recommend some counseling for the child or the entire family.

Show Us Your Logo!    


Lola with stickers

We are making it easy to Show Us Your Logo and to help spread the word about Diamond Blackfan Anemia! 


Four year old, Lola Willford, was excited to receive her beautiful DBAF stickers. One immediately was stuck to the front window of the Willford's West Jordan, Utah home.  Lola said, "I want the window to look pretty, mom!!"  Thanks Lola and family for telling the world you want a cure for DBA.

To get yours, make a minimum donation of $5 for each decal to the DBA Foundation by check (via mail) or credit card/PayPal. Visit our website's Fundraising Activities Page for more information.  Many thanks to Michelle Holdren for making these stickers available! 


Here's the challenge: We would like to see how many places we can show off our logo! Snap a picture sporting our logo and send us your story. Draw it, print it out, wear it, wave it, tattoo it, carve it, stick it... be creative!  Take us to school, on vacation, to the hospital, on a plane, to the game, in your home... anywhere!  Show us your logo!  Send your photos and stories to  
DBA Fact #9

Ellen Muir
Our Facebook Page posts DBA facts written by DBA nurse, Ellen Muir, RN, MSN, CPON. We are pleased to share these facts with our patients and families. Thanks, Ellen!


Monitoring Transfusional Iron Overload

As part of a large hematology team at Cohen Children's Medical Center of New York, we see hematology as well as oncology patients, as many centers do.  Several clinicians (doctors, nurses, nurse practitioners and physician assistants) see and make treatment decisions for a variety of patients with very different diagnoses.  For this reason we found it important to develop a consistent plan of care and treatment for those who are at risk for transfusional iron overload (see attached document).  Please feel free to print and share with your treating clinicians.  Use it to formulate questions and have a discussion with your clinician to develop a plan that meets your needs.


Methods of determining degree of iron overload: 

Serum ferritin Ferritin is a protein found inside cells that stores iron so your body can use it to make red blood cells.  Since DBA individuals do not make their own red blood cells, ferritin is not used and builds up with each transfusion.  A ferritin test indirectly measures the amount of iron in your blood and can be used as a 'marker'.  Ferritin is not always accurate as it is affected by factors other than iron loading such inflammation.  Ferritin is useful over time to monitor a trend, up or down, to see if chelation is helping to reduce iron burden.  This may be misleading as it does not reveal how much iron is stored in the organs and tissue.  Chelators will pull the iron from the tissue into circulation and the ferritin level will go up. 


FerriScan/ T2*/ R2 are all magnetic resonance imaging (MRI) based technologies for the non-invasive measurement of iron concentration. The difference between them is the method used to calculate the amount of iron stored.  FerriScan is just the brand name for R2 (like Xerox is for photocopy machines).  Not all MRI machines are equipped with the software to do this type of testing.  I would advise you to call your radiology department directly and ask to speak to the radiologist to see if they can do a T2* (* = star) for liver and heart iron quantification.  Disadvantages include possible increased reading if there is any metal in the field of study, such as metal clips if your spleen was removed.  Most 'hardware' is now MRI compatible, which means it won't be attracted to the magnet.  You may feel a warm sensation if you have a port or a joint replacement.

Liver biopsy is an invasive procedure, requiring anesthesia and a biopsy needle to obtain small pieces of liver.  Disadvantages include risk of bleeding, risk of infection and the possibility of obtaining a piece of liver that does not have as much iron as another area of the liver.  There is no way of knowing which area of the liver to take a sample, as it does not store iron evenly, so 3 or 4 different areas are usually biopsied. This procedure is usually done by a gastroenterologist or interventional radiologist and requires you to lie still for 4- 6 hours after the test, without eating, just in case there is bleeding and you need to go to the operating room to stop the bleed. Advantages over other methods of testing include the ability to actually see the condition of the liver and look for cirrhosis and fibrosis.

Gene testing for hereditary hemochromatosis provides information on likelihood but not level of iron loading.  The presence of this gene will cause transfused individuals to load iron even more quickly than those not affected by the gene.  If the test is positive for even one copy of the gene, DBA patients may need to come off transfusions and try steroids or have a stem cell transplant as maintaining iron balance will be a very difficult battle.  Disadvantages are that there are a number of hemochromatosis genes, but only one, HFE, can be easily identified.


Ellen Muir, RN, MSN, CNS                                                           877-DBA-NURSe (322-6877)                                         

DBAF's Monthly Journal Club

Steve Ellis
Steven R. Ellis, PhD
DBAF Research Director

 This month's Journal Club is a long awaited paper. It is a collaborative effort between the folks at the DBA Registry (DBAR) in New York and the National Cancer Institute (NCI) in Rockville, Maryland1.  The two groups got together and carefully analyzed the incidence of various forms of cancer in patients within the DBAR.  This study represents one of the most comprehensive analyses of cancer incidence in DBA patients. (abstract available).


Of 608 patients included in the DBAR, there were 15 cases with solid tumors, 2 with leukemia, and 4 with myelodysplastic syndrome.  Of the 608 DBA cases in the DBAR, 589 had no cancer or MDS.  The numbers don't exactly add up as 2 of the reported cases with MDS also had either a solid tumor or leukemia.


What do these numbers mean?  The cancer numbers seem low, at least numerically.  But the important consideration is: What would the numbers be if you looked at 608 people in the general population? The manuscript reports that the risk of all cancers in DBA was increased approximately 5.4 times that of the general population.  While this number is significant, it was substantially below that reported for Fanconi anemia and Dyskeratosis congenita, two other inherited bone marrow failures syndromes. 


Like the general population, the risk of cancer increases with age with a median age at presentation of 41 years in DBA patients.  This is compared with a median age of presentation of 66 years according to the SEER database at the NCI (  The authors state that this new information on the incidence of cancer strongly suggests that patients with DBA should undergo counseling and surveillance for cancer at an age earlier than that of the general population.     


One thing missing from the current analysis is whether individuals with mutations in certain DBA genes have an increased risk of cancer relative to others.  The reason for this is that gene discovery still lags behind the clinical information available for DBA patients, and the fact that some patients included in these analyses are no longer available for current genetic analysis.  As we close in on identifying all affected genes in DBA patients, these relationships, if they exist, should become more apparent. 


Since I am neither a physician nor a genetic counselor, my role with this Journal Club is to bring this paper to your attention so you can discuss it and its implications with your health care provider.  I would very much encourage you to do so as there is considerably more information within this manuscript than outlined above which will factor counseling and surveillance strategies. 


1.          Vlachos, A., Rosenberg, P.S., Atsidaftos, E., Alter, B.P., and Lipton, J.M. (2012) Incidence of neoplasm in Diamond Blackfan anemia: areport from the Diamond Blackfan Anemia Registry. Blood 119:3815-3819