Organ Donations from Individuals with Rare Diseases
Organ donation is often the final treatment option for patients who cannot be otherwise treated with pharmacological agents or sustained with machines, usually because the patient’s own organ is beyond treatable and cannot carry out its function.1 While much research has gone into using animal or synthetic organs, human-to-human organ donation still represents the most viable option, especially in cases where time is limited. The process of human organ donation involves many individuals, procedures, rules, and tests. It starts off with an individual making the important decision to be an organ donor and ends with organ transplantation in the recipient.2 While organ donation saves lives, the decision to be a donor and other personal factors have resulted in an organ shortage in Canada. In 2018, over 4300 patients were waiting for an organ donation, and 223 people died waiting for a transplant.1 As such, many campaigns have been launched to encourage people to register to be a donor, both while they are living and following death. However, one group of potential organ donors that is often excluded from such campaigns are those with rare diseases, out of fear that any organs they could donate can carry their rare disease, which would be passed on to the recipient.
Recent studies suggest that organ donations from donors with rare diseases are safe and provide the recipients with new hope that would otherwise not exist. Many European countries have responded in recent years by loosening organ donor requirements to include people with rare diseases, as long as their disease does not currently, and is not known to, affect the organ they wish to donate.3 In Canada, however, the rules surrounding organ donation are much more stringent. For example, a person with a rare disease may be able to put their name on an organ donor registry, but should they be matched with a recipient, the additional screening will eventually rule them out. The purpose of this article is to provide evidence to support the viability of organs donated by individuals with rare diseases and to bring about re-evaluation and reform of current policies in Canada. Not only could this strategy help mitigate the aforementioned organ shortage in Canada, but it will also remind individuals with rare diseases that they too can give the gift of life.
With an underrepresentation of rare disease organ donors, it only adds to the already existing problem of frequent organ shortages around the country. This problem then further extends to many individuals. First, patients in need of an organ donation are forced to wait even longer when organ donations are low. This wait can be especially elongated depending on the severity of their condition. In Canada, a “first-in, first-out” policy is not used when it comes to organ donation.4 Someone in urgent need of an organ donation may spend less time on the waiting list than someone else who has been waiting longer with a less severe condition. Second, people with rare diseases who wish to be organ donors are barred from having the opportunity to do so, even when their rare disease does not affect an organ they wish to donate. There is often fear that because all organs carry the same genome, there is the potential for transferring the rare disease to the recipient, which can result in financial and legal consequences for the health care system.4,5 Lastly, the health care system is placed under greater strain as waiting lists become longer and patients in need of a transplant must occupy hospital beds and other resources even longer.
In Canada, the complex process of organ donor recruitment to organ donation matching is overseen by an appointed organization in each province that works in conjunction with Canadian Blood Services (CBS).4 While CBS works separately from the federal government, their funding and regulations are dependent on Health Canada. To be an organ donor, registration occurs on a province-specific registry website, such as beadonor.ca in Ontario, and according to these same websites, people with rare and severe diseases can register. Patients in need of an organ transplant, on the other hand, are placed on the National Organ Waitlist, which is operated by CBS. Once a registered donor has been matched with a patient in need of an organ, follow-up screening takes place to determine the risk of using a given donor’s organs. It is most often in this step where many registered donors who have a rare disease are turned away out of fear that their organs may inflict harm on the recipient. Before organ harvesting and transplantation, tests and medical histories are obtained about the donor. This information is sent to public health units who will ultimately decide whether to accept the organs or not. In most cases, knowing that the donor has a rare disease will result in the denial of the organs. The recipient may also be the one who makes the decision of whether to accept organs from someone who has a rare disease. In some cases, these patients will accept this risk because they know they will die without the organs. The key point here is that the recipient or their family must be aware that an organ is coming from someone who had a rare disease to prevent any legal issues at a future point, should the recipient contract the disease. Doctors may also recommend to the patient whether they should accept the organs or wait longer for another donor to come along. Currently, the most common use of organs from people with rare diseases in Canada is for research purposes or tissues transplantation, but not direct organ transplantation.1
Canada uses an opt-in policy for organ donation while other countries, especially those in Europe, use an opt-out system.6 An opt-in system requires the general population to choose to be an organ donor, whereas an opt-out system assumes all members of the public are donors unless they choose not to be a donor.6 Interestingly, in most countries where opt-out systems are used, organs from donors with rare diseases are more frequently transplanted. Both systems give the final say to the individual to choose whether they want to donate their organs, but opt-out systems generally result in higher donation rates as opt-in systems require the individual to take the first step.6 Furthermore, for donors with rare diseases, Canada has a major problem where the decision to accept their organs often falls into the hands of medical specialists and not the recipient. While this policy was put in place to ensure that medical professionals can use their knowledge to assess the risk that transplanting an organ may pose to a recipient, it denies the recipient the right to make that choice for themselves. Most European countries let the recipient or their family make the choice to accept a donor from someone with a rare disease. One Italian study even showed that of all 16 recipients who received organs from a donor with a rare disease, either all lived to follow-up (81.25%) or died (18.75%) due to causes unrelated to the rare disease.7 The only conditions that would absolutely prevent someone from being a donor in these countries are certain very transmissible diseases (ie. Ebola virus) or active cancer, but individuals with most other diseases (even genetic ones) are not restricted from registering to be a donor.
A proposed modification to Canadian policies would be allowing the recipient to play a larger role in the decision to accept an organ from a donor with a rare disease. This may help address the organ shortage and ensure that recipients are fully aware of the risks associated with accepting the organ. Some patients may wish to accept the organs because even if they were to develop the rare disease, it would be better than their current condition. For example, they may currently be unable to walk or eat on their own which can be a large burden on their mental health and on caregivers. However, by accepting the organ, these issues are “exchanged” for a relatively tolerable rare disease. Given that emerging studies also show that donors with rare diseases can safely donate their organs without inflicting harm on the recipient, it may be time to update the protocols that dictate organ donation. Additionally, while most rare diseases are genetic, some diseases only affect specific organs and therefore should not prevent the donor from donating organs that are unaffected by the disease.5 While Canada follows policies that try to minimize risk to patients, sometimes it is necessary to take calculated risks. After all, without the organs, many patients die each year from waiting for a “healthy” donor.
Daniel D'Souza
Works Cited
- Canada PHAof. Government of Canada. Canada.ca. https://www.canada.ca/en/public-health/services/healthy-living/blood-organ-tissue-donation.html. Published April 28, 2020. Accessed March 15, 2021.
- BC Transplant. http://www.transplant.bc.ca/. Accessed March 15, 2021.
- NHS Choices. https://www.organdonation.nhs.uk/helping-you-to-decide/about-organ-donation/who-can-donate/. Accessed March 15, 2021.
- A Complete Guide on Organ Donation in Canada. Closing the Gap. https://www.closingthegap.ca/a-complete-guide-on-organ-donation-in-canada/. Published February 28, 2020. Accessed March 15, 2021.
- White SL, Rawlinson W, Boan P, et al. Infectious Disease Transmission in Solid Organ Transplantation: Donor Evaluation, Recipient Risk, and Outcomes of Transmission. Transplantation direct. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6324914/. Published December 20, 2018. Accessed March 15, 2021.
- Ajnesh Prasad Professor & Canada Research Chair, Karly Nygaard-Petersen Doctoral student. An opt-out organ donor system could address Canada's shortage of organs for transplant. The Conversation. https://theconversation.com/an-opt-out-organ-donor-system-could-address-canadas-shortage-of-organs-for-transplant-145088#:~:text=In%202018%2C%20there%20were%204%2C351,lists%20for%20an%20organ%20transplant.&text=Passed%20on%20April%202%2C%202019,the%20idea%20of%20presumed%20consent. Published September 13, 2020. Accessed March 15, 2021.
- Peritore D, Trapani S, Rocca VL, et al. Rare Disease Patients as Potential Organ Donors. Transplantation Proceedings. https://www.sciencedirect.com/science/article/abs/pii/S0041134520304255?via%3Dihub. Published April 14, 2020. Accessed March 15, 2021.
- BC Transplant. http://www.transplant.bc.ca/. Accessed March 15, 2021.
Cite This Article:
D'Souza D., Chau J., & Mughal R. Organ Donations from Individuals with Rare Diseases. Illustrated by Z. Hasan. Rare Disease Review. April 2022. DOI: 10.13140/RG.2.2.18602.49600
