Many thanks to all of you who participated in our PCD Tissue Bank Survey.   64 individuals responded and the results are available at PCD Tissue Bank Survey. 
 

To protect patient privacy, our survey provider shields identifying information so that all we at the PCD Foundation see is the responses-not who sent them.  For this reason, I can't respond to the individual comments or questions that were sent, but will try to address most them for the group as a whole. 

 1.) Request for More Information About the Program, Annual Updates, etc.

The PCD Tissue Bank Program is a new collaborative initiative by the PCD Foundation (PCDF) and the University of North Carolina, Chapel Hill (UNC).  The reason you have not heard about this program before or that your surgeon/physcian did not mentioned it is that it didn't exist. In fact, we are still setting up program parameters, donation procedures, data access procedures, etc., so we still have a long way to go.  There are no other programs like this for PCD tissue, but we are modeling our program after successful tissue collection initiatives by other disease groups.  When complete, will make detailed program information and updates available on the PCDF and UNC websites and in hard copy format. 

 2. Role of Cloning Lungs for Lung Transplant as it Relates to the PCD Tissue Bank Program.

There is tremendous interest in the area of cloning (essentially growing new organs from cells retrieved by the person needing transplant) for transplant organ procurement.  This is not an immediate focus of the PCD Tissue Bank Program because we still have a tremendous amount of work to do in other, immediately critical areas and cloning science is really just in its infancy.  Also, the samples collected for the tissue bank will be coming from affected individuals.  Organs cloned from these cells would most likely have the same ciliary defects as the tissue that was removed because the defect in is the DNA, so the practical use of these tissues for cloning to provide transplantable organs is not clear right now.  However, tissues collected for the program will be available to any researcher presenting a valid, legitimate research proposal, so there is nothing to preclude the use of these cells or tissues in cloning studies.

 3. Survival Outlook for Lung Transplant Recipients

In 2006, The Journal of Heat Lung Transplant reported (using data from the United Network for Organ Sharing or UNOS) that the median post-lung transplant survival rate is 5.6 to 8.5 years.  These data are aggregate numbers, meaning they incorporate statistics from transplant for all underlying causes and from recipients of all ages.  I was not able to find updated statistics, but have contacted UNOS to if they have current data. From a historical perspective, median survival rates for lung transplant surgery have steadily risen and this trend is expected to continue.  
 

4. Situs Considerations in Tissue Collection

The terminology in the area of situs (positioning) is so confusing we are looking at a descriptive field for information about where the sample came from, rather than simple check boxes.  This is important because the term situs inversus is frequently applied to organ arrangements in PCD that are not true situs inversus, but actually are situs ambiguus (heterotaxy).  The medical terminology for heterotaxy syndromes is even less standardized than the terminology in PCD.  To be sure we get accurate information, we want specimen preparers to describe the actual situs arrangement they find, rather than using terms that can be confusing or erroneous.  As the survey data shows, there are not many PCD patients currently in line for procedures, so asking for a description on a few samples should not unduly burden our tissue collection partners. 

5. Advanced Directives for Post-Mortem Tissue Collection

According to Scott Randell, PhD, lung transplant coordinator at UNC, timing is the biggest factor in whether or not post-mortem tissues can be accepted for the tissue bank.  The key is whether or not an anatomical pathologist is available to look at the lungs and collect the tissue within six hours of death.  For patients at large or university hospitals this may not be a problem, but in the smaller more rural areas it could be tricky.  Dr. Randell is working with the PCD Tissue Bank group to provide written guidelines for Advanced Directive donation.
 
We are very excited about this program and believe it will advance efforts in PCD research nationally and internationally.  Thank you very much for helping us.
 

Sincerely,

Michele Manion