Issue #12September 2011
In This Issue
It's in the Bag(s)!
School Days... School Days
Remembering Kevin
Show Us Your Logo!
Be a "Know it All"
DBAF Journal Club

It's in the Bag(s)!

Lambs in FL

Tige, Vickie, Griffin & Amelia Lamb

"When it comes to fundraising, it's a no brainer for us.  We can't change the fact that our daughter has DBA, but we CAN do everything in our power to raise research money for finding better treatments and ultimately a cure!  Her quality of life depends on it, how can we not do something?  The DBA family may be a small group, but we're mighty, and together we can accomplish a lot!" ~ Vickie Lamb 


Vickie and Tige Lamb, from Lenexa, Kansas, have organized and participated in a half-marathon, century bike race, cyclo-cross bike races, mini golf tournaments, bake sales, "dress down" days, and more to help raise funds for the DBA Foundation, in honor of their daughter, Amelia.  


Vickie is now spearheading an online fundraiser that all our families and friends can participate in -The Mixed Bag Designs Online Fundraiser!  
What is Mixed Bag Designs you ask? Well, think eco-friendly, fun, fashionable and the ability to help a worthwhile cause all rolled into one! 

These re-usable bags are made
from woven polypropylene - one the most popular plastics for making food containers like yogurt cups. 10-40% of the polypropylene used in the bags is recycled from post consumer use. Most of the bags hold up to 50 lbs, because of the plastic fibers woven into the bags. There are lots of great patterns and various sizes. Check it out for yourself!

Simply go to
Mixed Bag Designs, enter your order, pay for it and it will be shipped directly to you. At checkout, type 69293 in the "School/Fundraiser ID#" field.   THIS IS VERY IMPORTANT SO WE RECEIVE THE CREDIT!

Thank you for your support!  40% of all orders placed will be donated to the DBA Foundation.  Please pass this along to friends, family, neighbors, or anyone that might be interested
. This fundraiser will run through the end of this year, however, do not delay in placing your order today. Order a few for yourself, check them out, and then order some more later in the year... they make great gifts!   


The Diamond Blackfan Anemia Foundation sincerely thanks Vickie and Tige for all their hard work and continued efforts. It is because of the commitment of our families and friends that we are able to fulfill our mission for patients... for families... for research.  




Upcoming Events



DBA Fishing Tournament 

September 17, 2011 (rain date)  

Four Oaks, NC  

Tracy Adams


Friends of DBAF Golf 

Outing & Silent Auction 

September 17, 2011  

Cherokee Hills Golf Club
Valley City, Ohio

Jim and Carol Mancuso

Kevin J. Gately Foundation
Golf Outing 
October 17, 2011  
Black Swan Country Club
Georgetown, MA


Jack's Fight for a Cure
DBA Dinner & Dance Gala
to benefit DBAC 
November 11, 2011  
Orangeville Agricultural Center
Orangeville, Ontario

Janet Pereira

Ongoing Fundraisers 


Mixed Bags Designs

Vickie Lamb
Use Code: 69293  

To order online visit:




Wristbands Available

Twila Edwards 

Tribute Cards Available

(2 styles)

In honor of...

In memory of...


Dawn Baumgardner


donation donation

DBA Cookbooks Available


Betty Lightner


To download your order form:

cookbook cover 

Good Search/Good Shop

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

Just download the GoodSearch - Diamond Blackfan Anemia Foundation - DBAF toolbar at


Search the Web now Money-saving coupons


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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 Diamond Blackfan Anemia Foundation is YOUR Foundation!  We encourage you to share your ideas, photos, and stories for our website and upcoming newsletters. Contact us at

School Days... School Days   


It's back to school time for our students bringing new pencils, new opportunities, and new fears to many! Below is a guidance sheet provided by the National Institutes of Health outlining positive actions and a checklist for schools and families to use to facilitate a coordinated approach in meeting the needs of students with chronic illnesses. This is only a guideline and may help in achieving the best plan for your child.


Chronic illnesses affect at least 10 to 15 percent of American children. Responding to the needs of students with chronic conditions in the school setting requires a comprehensive, coordinated, and systematic approach. Students with chronic health conditions can function to their maximum potential if their needs are met. The benefits to students can include better attendance, improved alertness and physical stamina, fewer symptoms, fewer restrictions on participation in physical activities and special activities, such as field trips, and fewer medical emergencies. Schools can work together with parents, students, health care providers, and the community to provide a safe and supportive educational environment for students with chronic illnesses and to ensure that students with chronic illnesses have the same educational opportunities as do other students.

Family's Responsibilities

  • Notify the school of the student's health management needs and diagnosis when appropriate. Notify schools as early as possible and whenever the student's health needs change.  
  • Provide a written description of the student's health needs at school, including authorizations for medication administration and emergency treatment, signed by the student's healthcare provider.   
  • Participate in the development of a school plan to implement the student's health needs:

Meet with the school team to develop a plan to accommodate the student's needs in all school settings.  

Authorize appropriate exchange of information between school health program staff and the student's personal healthcare providers.  

Communicate significant changes in the student's needs or health status promptly to appropriate school staff.

  • Provide an adequate supply of student's medication, in pharmacy-labeled containers, and other supplies to the designated school staff, and replace medications and supplies as needed. This supply should remain at school.
  • Provide the school a means of contacting you or another responsible person at all times in case of an emergency or medical problem.
  • Educate the student to develop age-appropriate self-care skills.
  • Promote good general health, personal care, nutrition, and physical activity.

School District's Responsibilities

  • Develop and implement districtwide guidelines and protocols applicable to chronic illnesses.
  • Guidelines should include safe, coordinated practices (as age and skill level appropriate) that enable the student to successfully manage his or her health in the classroom and at all school related activities.
  • Protocols should be consistent with established standards of care for students with chronic illnesses and Federal laws that provide protection to students with disabilities, including ensuring confidentiality of student health care information and appropriate information sharing.
  • Protocols should address education of all members of the school environment about chronic illnesses, including a component addressing the promotion of acceptance and the elimination of stigma surrounding chronic illnesses.  Develop, coordinate, and implement necessary training programs for staff that will be responsible for chronic illness care tasks at school and school related activities.
  • Monitor schools for compliance with chronic illness care protocols.
  • Meet with parents, school personnel, and healthcare providers to address issues of concern about the provision of care to students with chronic illnesses by school district staff.


School's Responsibilities

  • Identify students with chronic conditions, and review their health records as submitted by families and healthcare providers.
  • Arrange a meeting to discuss health accommodations and educational aids and services that the student may need and to develop a 504 Plan, Individualized Education Program (IEP), or other school plan, as appropriate. The participants should include the family, student (if appropriate), school health staff, 504/IEP coordinator (as applicable), individuals trained to assist the student, and the teacher who has primary responsibility for the student. Health care provider input may be provided in person or in writing.
  • Provide nondiscriminatory opportunities to students with disabilities. Be knowledgeable about and ensure compliance with applicable Federal laws, including Americans With Disabilities Act (ADA), Individuals With Disabilities Education Act (IDEA), Section 504, and Family Educational Rights and Privacy Act of 1974 (FERPA). Be knowledgeable about any State or local laws or district policies that affect the implementation of students' rights under Federal law.
  • Clarify the roles and obligations of specific school staff, and provide education and communication systems necessary to ensure that students' health and educational needs are met in a safe and coordinated manner.
  • Implement strategies that reduce disruption in the student's school activities, including physical education, recess, offsite events, extracurricular activities, and field trips.
    Communicate with families regularly and as authorized with the student's health care providers.
  • Ensure that the student receives prescribed medications in a safe, reliable, and effective manner and has access to needed medication at all times during the school day and at school related activities.
  • Be prepared to handle health needs and emergencies and to ensure that there is a staff member available who is properly trained to administer medications or other immediate care during the school day and at all school-related activities, regardless of time or location.
  • Ensure that all staff who interact with the student on a regular basis receive appropriate guidance and training on routine needs, precautions, and emergency actions.
  • Provide appropriate health education to students and staff.
  • Provide a safe and healthy school environment.
  • Ensure that case management is provided as needed.
  • Ensure proper record keeping, including appropriate measures to both protect confidentiality and to share information.
  • Promote a supportive learning environment that views students with chronic illnesses the same as other students except to respond to health needs.
  • Promote good general health, personal care, nutrition, and physical activity.


Student's Responsibilities

  • Notify an adult about concerns and needs in managing his or her symptoms or the school environment.
  • Participate in the care and management of his or her health as appropriate to his or her developmental level.

 Wishing everyone a safe and productive year!



Remembering Kevin 

Kevin J. Gately

1981 - 2010 


Kevin Gately's short life and tragic death has touched the hearts and lives of many DBA patients and families. (To read Kevin's Legacy, written by his mother, Sally Thompson Gately, download our Spring 2011 Newsletter.)  


 Kevin's amazing family continues to touch our DBA community. Motivated by their love and desire to memorialize Kevin's life, the Kevin J. Gately Foundation was established to support Kevin's charitable interests. The DBAF is honored to be chosen as the recipient of their hard work. We are extremely grateful to the KJG Foundation for their generous support allowing us to support DBA patients, families, and research.

On October 17, 2011, the KJG Foundation will be hosting their 1st Annual Golf Tournament at Black Swan Country Club in Georgetown, MA. All are welcome! Please visit for information on the tournament and sponsorship opportunities.

Take the Challenge ~ Show Us Your Logo 

Bethany tatoo
 T-shirts, hats, coffee mugs, face paintings,  tattoos, bags,  pumpkins ... our logo is showing up everywhere! We are thrilled that our beautiful logo is proudly being worn and displayed by patients, families, and friends.

 When it comes to their feelings about their son, Lane, Tina and Anthony Bethany of Florence, MS, wear their hearts on their sleeves.  Well... almost! They wear their dream of a cure on their arms...a dream we all share! 

Here's the challenge:  we'd like to see how   many places we caDBAF  Logon 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... 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 

Be a "Know it All"

The DBA Foundation strives to keep you updated. Some information is best sent to a street mailing address. Please take a moment to ensure we have your correct information. Please submit a current registration form. 


If you have any questions, contact Dawn at  


 Journal Club 
Steve Ellis
Steven R. Ellis, PhD
Research Direct

Who was Otto Warburg, and what does he have to do with DBA? Well, Otto Warburg was a great German biochemist who won the Nobel Prize in 1931 for his studies on cellular respiration. Warburg's work laid the foundation for oxidative metabolism, the process by which cells derive energy from nutrients via the consumption of oxygen. For example, the complete oxidation of glucose to carbon dioxide and water, which requires oxygen and is referred to as respiration, produces over 30 molecules of ATP, the energy currency of a cell. In contrast, anaerobic glycolysis, a process that occurs in the absence of oxygen and converts glucose to lactic acid  produces only 2 molecules of ATP. One could therefore assume from these observations that respiration is a much more efficient means obtaining energy from glucose than is glycolysis. Interestingly, Warburg found that many tumor cells obtained most of their energy from glycolysis rather than respiration, even though oxygen was available. This property of tumor cells was ultimately named after its discoverer and is referred to as the Warburg effect 1.  One rationale for this property of tumor cells is that by preserving the carbons of glucose as lactic acid rather than converting them to carbon dioxide, these carbons can be used as the building blocks for cellular components needed for tumor growth.  


So, what, if anything, does Otto Warburg have to do with DBA? Well, let's just refer to this as my "six degrees of separation" Journal Club. And DBA, of course, is all about red blood cells; whose job is to transport oxygen, not consume it. So how then do red blood cells get their energy? Well, red cells get their energy by glycolysis so as to not consume the precious cargo that they will ultimately deliver to respiring cells of the body. In contrast to Warburg's tumor cells, which presumably could carry out respiration but choose not to, red cells are incapable of respiration because they have lost their mitochondria, organelles in which respiration occurs. Thus, we have this tenuous link between red cells and many tumor cells, in that they both rely on glycolysis as a primary source of energy.


However, our six degrees of separation between Otto Warburg and DBA wouldn't be complete without bringing in the p53 protein. The p53 protein is a major tumor suppressor protein and many tumors have lost p53 function. Why? Because p53 is often referred to as the guardian of the genome, and when cells begin to go genetically haywire as cancer cells often do, p53 gets activated and this either causes cells to quit dividing or commit suicide. Many tumor cells however, inactivate p53 to escape this fate. Interestingly, p53 also is thought to regulate cellular metabolism by favoring respiratory growth 2. As such, loss of p53 function could contribute to the changeover of metabolism during tumorigenesis away from respiration to aerobic glycolysis, thereby favoring the Warburg effect.


The p53 protein should be familiar to those of you that regularly follow DBA research because defects in ribosome assembly are thought to be another means of activating p53 and possibly explain the enhanced cell death observed in red cell progenitors in the marrow of DBA patients.3 But one of the problems with this theory as an explanation for the clinical features of DBA is why red cell progenitors would be so sensitive to p53 activation. Why doesn't p53 activation and cell death occur to all cells in a patient with DBA? If one thinks about the role of p53 as a potential regulator of cellular metabolism, this selective effect on red cell progenitors might be explained. For if p53 is activated in red cell progenitors promoting respiration at the same time red cell progenitors are activating differentiation programs that will ultimately lead to mitochondrial loss and glycolytic growth, it would seem that these progenitors would be getting mixed signals which may in turn sensitize cells to cell death programs. The figure below visualizes the six degrees of separation (in blue) between Otto Warburg and DBA.

 Warburg effect diagram

Some have argued against this far-fetched perspective on DBA because loss of respiratory function occurs relatively late in the red cell developmental program, while the progenitors lost in DBA patients are much earlier in the pathway. I, on the other hand, think that the signals that drive cellular differentiation may occur in progenitors long before the outcomes of these signals are evident in the observable behavior of more differentiated cell types. As such, mixed signals could conceivably influence decisions made by progenitors even though respiratory function is not fully lost until the reticulocyte stage of red cell development. 


I raise these dubious connections more as food for thought than a definitive last word on the red cell specificity of DBA. The manuscript that resurrected these thoughts and provoked this journal club edition has nothing to do with either Otto Warburg or DBA but instead provides an additional link between cellular metabolism and cell fate decisions 4.             

1. Warburg O. On respiratory impairment in cancer cells. Science. 1956;124:269-270.

2. Matoba S, Kang JG, Patino WD, et al. p53 regulates mitochondrial respiration. Science. 2006;312:1650-1653.

3. Dutt S, Narla A, Lin K, et al. Haploinsufficiency for ribosomal protein genes causes selective activation of p53 in human erythroid progenitor cells.  

Blood 2011;117:2567-2576.

4. Yi CH, Pan H, Seebacher J, et al. Metabolic Regulation of Protein N-Alpha-Acetylation by Bcl-xL Promotes Cell Survival. Cell 2011;146:607-620.