Ask the Doc: Q & A with Edward Kasarskis, MD, PhD

Edward Kasarskis, M.D., Ph.D. is Director of the multidisciplinary ALS Center at the University of Kentucky Neuroscience Center in Lexington, Kentucky, professor in the Department of Neurology at the University of Kentucky, and Chief of Neurology at the VA Medical Center in Lexington KY. 

	Dr. Kasarskis

Organ Donation and ALS

Q: I just saw an episode of Grey’s Anatomy that featured a younger person with ALS who used an advance directive to discontinue his life support so he could donate organs. It disturbs me that the show featured an ALS patient, since I understand that the majority of if not all state donation systems refuse to accept organs from people with ALS, apparently because the cause of ALS is not completely understood. What should people know?

A: Yours is an insightful and complicated question.  The answer will not be brief.  Unfortunately, we don’t know the exact cause of sporadic ALS and this uncertainty breeds caution around the issue of tissue and organ donation.  Although there is an extremely small probability that donated organs or tissue could cause any problem whatsoever in the recipient, there is still some level of lingering concern that the disease might be transmissible from one human to another in rare cases.  Research scientists have tried to transmit ALS from human tissues into recipient research animals and have not been able to do so. 

In my opinion, the background concern comes from the group of so-called "prion diseases." Think back to the Mad Cow Disease epidemic in England and all the concerns surrounding this.  Prion diseases are a family of rare progressive neurodegenerative disorders that affect both animals and humans.  Considered to be “transmissible spongiform encephalopathies,” they are notable for their long incubation periods, neuronal loss, and the lack of an inflammatory response to the disease.  It’s believed prions are abnormal transmissible protein agents that cause the normal brain proteins to fold in an abnormal way, which leads to brain damage and usually results in progressive dementia and not ALS. The diseases usually progress rapidly and are uniformly fatal.  The best-known example in humans is Creutzfeldt-Jacob Disease (CJD).

How does all this relate to organ donation?  The organ donation laws vary state by state.  It appears that the intent of these statues is to maximize the number of organs for donation while keeping the recipients safe from a transmissible disease.  So if the cause of an illness (such as ALS) that resulted in the donor's death is not known, some states might not accept a tissue or organ donation from such a person.  The unstated concern is that a progressive disease in someone that looks like typical ALS might be a transmissible disease.  Theoretically I understand that might happen, but it is a very, very rare occurrence.  

That said, it’s important to emphasis that we do not believe ALS is transmissible.  Families ask me that question all the time, and I tell them that if ALS were infectious, I would have caught it a long time ago!  Lou Gehrig was worried about this very issue and actually wrote a letter from the Mayo Clinic reassuring his wife that she could not catch ALS from him.

The overwhelming likelihood is that it would be very safe to receive organs and tissues from an ALS patient, and if your state allows it, such a donation would be a reasonable thing to do.

Know that in addition to donating for transplantation, the need for tissue for research is great. There are many academic medical centers and research organizations that collect tissue donations from patients, and the VA also has a tissue bank. There is also the opportunity to donate one’s body to medical schools for teaching purposes. These resources are needed for a wide range of important studies. Gifts for these purposes make a substantial contribution to furthering medical knowledge about ALS and other diseases. 

If you’re interested in being an organ donor or making a contribution to science, don’t assume you can’t.  Raise the question and find out what the rules are in your state. Work with the donor staff at your hospital or health center to learn more.

If a person with ALS who depends on a respirator to breathe decides to withdraw respiratory support – as in your example from Grey’s Anatomy -- know that the individual can be kept comfortable, be weaned from the ventilator and can pass away peacefully.  The prospect of organ and tissue donation can be brought up before that decision, or afterwards, and the person who is next of kin would be in the position to put that possibility forward.  

These are very complicated issues that cut across law, medicine, religious beliefs, and ethical concerns.  Every hospital and hospice organization has individuals or teams to help with discussions about the ethical questions.  

In short, a wide range of organ and tissue donation options are available to people with ALS, depending on their state and on whether they are also interested in making a donation to education and research.

If you would like to submit questions for a future Q & A, please send your questions to theexchange@alsa-national.org. Please understand that we won’t be able to address all questions and we won’t be able to respond to individuals personally.