I think we in the Hermansky-Pudlak Syndrome community understand the gift of life better than most. We wait with anxiety and anticipation with our friends in need of new lungs. We pray that some family out there somewhere will have the heart and the strength to make a gift of their loved ones lungs when they are in such pain and grief themselves.
As HPSers, we can give as well as receive. We have it in our power to give the gift of life and hope to our HPS friends for years to come. For us, the gift might not be as straight forward, but it is as precious all the same. We can give the gift of tissue for research.
We can do this in lots of ways. If you have a planned surgery or biopsy, contact the HPS Network and let us know. This way, we can consult with the researchers to determine whether what you’re having removed or sampled could have some value to our research. For example, last year an HPSer having an overy removed let us know. We didn’t think it would be of interest to researchers, but in fact, they wanted it. Another HPSer, after years and years of fighting bowel disease, had an ostomy. He donated his removed colon to HPS research. The Network contracted with NDRI to have it tissue banked, and now it could very likely be more valuable than gold to HPS bowel research.
Over the years, as HPSers have either received lung transplants, or sadly passed away from the pulmonary fibrosis of HPS, they have donated their old lungs to research. While research might not have been able to save their native lungs, they gave us a precious gift. These samples can be so precious to research, and with a rare disease like HPS, they are made even more precious because they are so hard to obtain.
What we’ve learned over the years, however, is that making this gift isn’t just a matter of telling your doctor and signing a consent form. Even when HPS patients have been eager to donate their tissue, the system creates a lot of obstacles. It’s necessary to not just have a signed form, but an action plan that can take some time and effort to put together.
It shouldn’t be so hard, but it is.
Here is an example of why.
Several years ago an HPS patient made our job easy. They came to us eager to donate their lungs for research. We all hoped this person would get a lung transplant and live a long life with new lungs. But, that wasn’t to be. The patient signed consent forms with the NIH and with her hospital. They made their wishes very clear to their doctors and their family. Even as they were critically ill, they were asking loved ones to be sure their lungs went to research. They saw it as a gift for the future of their HPS friends – a final legacy to help us find a treatment.
We thought everything was ready to go to make their final wish come true. Yet even with all this advanced planning, there was trouble. When the patient passed away, it wasn’t during normal business hours. The pathologist would not come in to prepare the tissue samples on off hours. By the time he got to it, too much time had passed and what we really needed was no longer possible.
We learned a lot from that case. We learned that doing all the things you are supposed to do isn’t always enough. Now, one of our advocacy priorities is to try to improve this system. Perhaps we need to help our HPSers speak directly to the head of pathology to plead that they understand how rare and precious these gifts are and to help them understand what a vital role they play in our gift of life. Perhaps we need to find a way to allow NDRI (our tissue procurement company) to deal directly with the transplant surgeon as well as pathology. Honestly, we aren’t sure what the plan will look like. It will also have to be different depending on the patient, the nature of the tissue donation, and where the patient is located.
The part I struggle with the most is having this discussion with HPSers who are sick or who are hoping for new lungs. I feel like I’m being some kind of vulture after body parts. I know it has to be done. I know many HPSers actually find meaning in being able to give this gift to all of us. Still, it’s so hard.
I will never forget how easy my friend Elsie made this process. Elsie loved her HPS family so much. This was very important to her. She made it easy for me to talk to her about the subject because she brought it up. She flat out told me not to feel funny about it because it was something she wanted to do. She gave me permission to have the conversation, and that alone was a huge gift. She took away that feeling of guilt I sometimes have when I have to bring up this subject.
So, in closing, please help us by being willing to make these gifts of life. Call the HPS Network office and let us know it is something you want to do so we can help you do it. If you ask us, it makes the process so much easier.