Her Story
A little bit of background for those of you who do not know Julie's history. Julie sustained a Suprasellar Germinoma brain tumor at the age of 14, underwent surgery to resect 90% of it, followed by chemotherapy and radiation treatments. She remained in remission for 20 years, right up until her death at the age of 34 on May 16th, 2018. As a result of the location of the tumor and the subsequent damage to the pituitary, Julie lived with panhypopituitarism. Therefore, she took several medications to replace the hormones once controlled by the master gland. So.. what happened? The coroner's report in essence said that her heart stopped. No trauma, no heart attack. Dr. Mary Sutton, Julie's Neuro-oncologist of 20 years at Children's, explained that Julie's death was most certainly caused by adrenal insufficiency -and then crisis. After the autopsy, Dr. Sutton was proved correct. Julie's cortisol level dropped so low that she fainted, then went into a coma as her vital organs eventually shut down.
Here is what we have learned subsequent to Julie's death, about Adrenal Insufficiency (AI). And these are in no particular order.
1. Death by adrenal crisis is highly preventable.
2. People who live with Julie's complicated medical history require clear and specific guidelines for managing their circumstances, with regular educational follow-up. This goes for their caregivers as well. As an aside, but related to this topic of regular educational follow-up, I would like to mention another important component to Julie's circumstances. Brain tumor survivors can have a wide variety of late sequelae, including cognitive damage which includes short term memory loss, and other neural complications. However, Dr. Sutton and Julie's entire family would all tell you that she was very high functioning as a brain tumor survivor. She drove her own car, she traveled on her own, she was able to maintain employment. All-in-all, Julie was able to function as a normal 34 year old woman.
3. Adrenal Insufficiency can be difficult to identify and distinguish, because each one of the classic symptoms of this condition are easily explainable by other, more common circumstances. For example -and I will specifically talk here about Julie's symptoms over the last 18 months of her life.
a. Weight loss. Julie steadily lost weight over the course of 18 months, for a total of 125 pounds before her death. It was gradual and it was consistent. She was not trying, she did not put herself on a diet, she did nothing out of the ordinary other than walk more with her new job. Therefore, her father and I assumed, as did Julie that the weight loss was the result of her job as a delivery person for a sandwich shop. This was a new job and required more walking than she had ever done in her prior positions.
b. Chronic fatigue. Julie complained to us more and more about how tired she felt every day. We chalked it up to her job, getting in and out of the car, working in the heat, just being worn out from the physical exertion. We also chalked it up to her hypothalamic somnolence which is another classic late effect of a brain tumor survivor. Julie required an enormous amount of sleep.
c. Muscle weakness. Julie consistently felt very weak. Again, we thought it was the result of her physical activity on her job. We also thought it was the result of no longer working with her personal trainer, because the only trainer she was willing to work with moved away right around the time Julie started her position with the sandwich shop.
d. Loss of appetite. Julie was eating less- as we look back now. I was not around Julie 24/7 because I worked full-time, she was working and we did have different schedules. But I did spend a lot of time with her and I did notice that she was eating less. Honestly, we thought it was again from the exertion of her job, the heat of the summer and the fact that Julie was notorious for snacking all day long and then acting like she hadn't snacked all day long- (so that I wouldn't get aggravated with her. :)
e. G.I. symptoms. -vomiting, diarrhea or just a general feeling of "being sick to your stomach". Julie frequently had G.I. issues- since she was a little girl. We attributed this to her biology: her father and she seemed to have the same sensitive stomach. And obviously, when Julie was very sick (with more than one day of illness), we had her in her family doctor's office right away because of fear of infection and/or dehydration.
At this point I'd like to segue into a couple things about Julie and illness: From the onset of Julie's brain tumor d/x and resection, we were all schooled on "sick day rules" and stress-dosing of hydrocortisone. We were told consistently by both Dr. Sutton and Dr. Cohen that Julie had to be her own adrenal gland and therefore, triple her hydrocortisone as soon as she was feeling ill. This could be from fever, sore throat, G.I. illness, etc. We knew this and we were all vigilant about this, especially Julie herself.
But there are four critical pieces of information that none of us knew:
First, stress-dosing of hydrocortisone should also include times of overheating, as well as times of emotional stress.
Secondly, with G.I. symptoms, particularly more than a day of vomiting and diarrhea, it is unlikely that oral hydrocortisone is sufficiently absorbed into the bloodstream.
Third, we had no idea that there was such a thing as an injectable form of hydrocortisone. The more I read, the more I am certain that we should have had a prescription for this on hand, particularly in light of Julie's regular bouts with G.I. illness. The most common injectable product being used currently by AI patients is Solu-Cortef. Julie and I would have made certain that she had a prescription always filled and on hand for this injectable hydrocortisone. We would have used it whenever she had these sorts of symptoms. We just did not know it existed.
And finally, we had no earthly idea that our daughter could die from such a thing as low cortisol. We just did not have the education or the knowledge. If someone had said to us at some point along the way: "Your daughter could potentially die if she doesn't have enough cortisol in her bloodstream", I believe our daughter would be alive today. Please take a look at the picture below. This was taken at a friend's wedding just four days before Julie's death. Does this look to you like someone who is dying? We still cannot believe it. Our suspicion is that Julie's baseline, everyday level of cortisol was far too low to sustain her size, activity, her unique physiology, her lifestyle. None of us knew this. If we had suspected anything we would have requested that blood work be done frequently and consistently. I have also come to understand now, after talking with Dr. Patel that measuring blood levels of cortisol is complex, as the body absorbs cortisol at different rates throughout the day, and as needed according to stress. Additionally, he has explained that patients such as Julie are especially complex, as the levels of other hormones, such as ACTH and TSH also impact absorption and need for cortisol.
I have learned recently from my readings that there is current research in place on the design of a pump similar to that worn by diabetic patients- which will be able to both measure continual cortisol changes within the body as well as administer the needed dosage automatically. To my knowledge, this device has not yet been approved by the FDA in the U.S. It has been researched and approved in the UK and is being used by AI patients in the UK with tremendous success.
In sum, Dan and I strongly believe that we must come together as a community of families and medical practitioners in order to: (a) implement significant changes to the education of AI patients, their caregivers, physicians and all ancillary medical personnel and (b) support research into more effective treatment protocols for AI.
With your generous donations to the Julie Theresa Jones Fund, we are certain that lives will be saved from Adrenal Crisis, and families will be spared the unbearable pain and heartache of losing a loved one from such a preventable death. This is truly what Julie have wanted. Here is the link to her donation site:
https://foundation.uc.edu/donate/JulieTJones
God bless each of you.
