I talk a lot on this blog about the diseases we treat for Von. Yet, I don’t really tell you about the nitty gritty of how this effects Von. It’s not something I really enjoy thinking about let alone talk about. There are things that I find more enjoyable, and I can name a few: cleaning out the cat box, walking the dog in negative temperatures, running a marathon after being awake for 2 days and with no food, and finally watching the Green Bay win any football game. I guess you can see this is really hard for me, but it’s something that is always on my mind and a reality that no matter what I do I cannot escape. There are mornings I wake up, and I look around and I think today is going to be a better day. I say my prayers, and I kiss the morning sun. Yet, I know that soon I will have to wake up and help my 3 year old battle another day. This is done with sheer determination and I know that we can get through it, but it doesn’t mean it’s not without some heartache.
Everyday we wake up to the reality that left alone without any medications, Von’s body would quickly shut down within days. He would go in to respiratory failure, cardiac failure and his blood sugar would plummet to zero. It’s a scary reality when you know that you can’t miss a single dose of medication or your child could end up in crisis. I can tell you a few things I know about Adrenal Insufficiency. I know a lot of you still don’t really understand it, and I get it how many of us really don’t remember anything being discussed about the pituitary gland in Human Biology. Actually, how many of us actually took the class Human Biology. Most pre-med students take it in college, but average non-genius folk like me didn’t and when we got diagnosed I took a crash course in the endocrine system.
The first thing I learned is there is a tiny gland the size of a pea in the your brain, and it’s located right behind your eyes near the optic nerves. A stalk connects the gland to the Hypothalamus which is the part of the brain that tells the hormones to all release. Did you get that? The pituitary gland talks to the hypothalamus through a stalk and the Hypothalamus talks to the body and tells the other glands to release their hormones. This tiny little pea shaped gland controls your bodies sex hormones, growth hormone, your thyroid, your kidneys ability to absorb salt and water, your adrenal glands, and it releases the love hormone oxytocin. Hormones and hormonal balance goes a lot beyond what you and I learned in sex ed. There are way more hormones than estrogen or testosterone making our bodies function. Now knowing all the amazing things this gland does, imagine you just didn’t have one! No that’s not possible, right? WRONG! There is a chance you could be born without one, the one you have stops working, or a tumor envelopes your brain and crushes your pituitary gland. All of these would render you with Panhypopituitarism. This means your pituitary gland does not work. So in order for hormones to be released we have to replace them all with synthetic medication, hope it works, and know that it’s not a perfect science and at any point the drugs could not work properly which could put you in a position where you are on the brink of life or death.
So back to Von, sorry for the long tangent. Von’s is absent. Completely absent. We found this out on January 16, 2013. The MRI confirmed an empty sella (the sac that protects the pituitary gland). That was when we got the diagnosis and when doctors said “Your son has a life threatening disease.” We go to many doctors to treat all the issues that have come up since that diagnosis. His endocrine team manages this disease. This means we need to get a lot of lab draws, daily medications, injections daily, and daily monitoring of all his symptoms. Daily checks of body temp, heart rate, lung function, and watching him carefully to ensure he’s not pale or lethargic.
Adrenal crisis is really scary. I can’t even say that it’s really scary. It’s more like you feel like everything around you has stopped and you can’t believe that this is happening. When Von has a crisis it is difficult to pinpoint why they happen. However, we know good and bad stress can not be good for Von. If he’s overly stressed by an event that is suppose to be happy, he can actually start to crash. It happened at Christmas. He became quiet, pale and tired. He was whiny and was yawning. We knew we had a short window to give him extra medicine before it became critical. Christmas is suppose to be happy, but even happy stress overwhelms him and he needs that cortisol to surge his body. Unfortunately he makes no cortisol, so he needs more medicine to manage it. Then we have to HOPE that his body digests and absorbs the medicine. If his body doesn’t absorb it, it’s bad news.
Last month Von’s body wasn’t working right. He got a virus and doctors believe he wasn’t absorbing his medicine. His heart started beating irregularly, his beats per minute were low, and his blood sugar plummeted to 44. We had to give him an injection and rush to the hospital. A simple virus landed us in the hospital for 2 days. I felt like a failure, and was reminded how fragile he is, and I got the reminder from his doctors that this is just what life is going to be like. “Be prepared to spend time in the hospital,” they told me. It’s hard to have to rely on the hospital for these situations because it means we can’t do a lot of things and can’t go places that are far from pediatric hospitals. It makes travel difficult, and because there is no standard of care in ER’s across the country it makes travel scary. This past few months 3 patients on social media support boards dedicated to Adrenal Insufficiency have died due to protocol issues in the hospitals. Doctors that refused to give life saving medications, or stopping care sooner than the patient could handle. The sheer discrepancies from state to state and doctor to doctor puts patients with the disease at risk daily of one bad experience at the ER ending up in death.
So what does all of this mean for us? It means our life is a lot more structured than the average family. It means when we hear your child coughing we might leave the room or the party. We don’t go out as a family frequently from November – April. Von has nurses 12 hours a day 6 days a week that monitor his multitude of diseases and manage his medications. Von has 4 therapists, 9 doctors and 5 nurses that care for him and help him develop. It means we live on the edge a lot. Sleep is hard to come by, and over indulging in ice cream or wine can happen without even thinking about it. Stress is always high, and tempers can flare in an instant. Every day I pray to God. I pray for two things. I pray for strength for Todd and me to get through another day of managing the diseases, and I pray for Von to continue to develop and beat the disease. Somedays I pray that a cure will come. Even though I know this disease is incurable and it will eventually take his life, I will never not pray for a cure. It’s the only thing that would make this all go away. Every day I ask thousands of people that follow us for prayers, and we need them. Trust me we need them. I will keep on fighting, but this is what it means to have a life threatening illness for us.