Preparedness is Key

Last night, I had to sit back and wait, helpless, while a fellow AI warrior slipped into adrenal crisis.

You see, I help moderate an online support group for people with adrenal insufficiency. Someone reached out to us for help, but we didn’t know where they were, how to reach them. Their online profile was locked down tightly, for security, and there was no way to know if this person survived the night.

**As I’m writing this, a friend of this person reached out to let me know that this person lived through the night… And I’ve burst into tears!**

On another group, a member frantically asked what they needed to do because they were afraid a crisis was imminent. They didn’t have a doctor to reach out to late at night, and didn’t know if their fear was warranted or not. They are new to this diagnosis.

Today, another person reached out for help. This person didn’t know how to utilize their emergency injection. The solu-cortef act-o-vial was cumbersome and confusing. Another moderator was able to reach them by phone, and talk them through it while they called for an ambulance.

Another person lost their life last week due to an adrenal crisis in their sleep.

Far too many doctors see a patient with adrenal insufficiency and assume that taking a little steroid pill twice a day will allow someone to live a regular and full life. That is simply not the case. The human body produces cortisol on demand, and the needs shift based on activity, stress, injury, and illness. These are regular daily variables that cannot be planned for. Anticipating a stressful situation isn’t something healthy people even consider.

For an AI patient, though, we MUST anticipate every potential scenario.

It’s flu season, so we carry extra hand sanitizer, wear masks, and get our flu shots.

Walking around the block on a hot day, we must bring water with electrolytes as well as a cell phone and our emergency injection.

Grocery shopping can often cause enough stress that a stress dose is necessary.

These variables are not considered by most doctors. They don’t understand how individual the cortisol response is.

The best way to educate medical professionals about the variability of adrenal insufficiency is to raise awareness.

Speak to your providers about your experiences, and don’t be afraid to advocate for yourself. If your medication regimen isn’t working, speak up.

Remember, EVERY adrenal insufficiency patient MUST have an emergency injection on hand – and a back up!

Take the time to educate yourself about the risks. Whether you are a patient, friend, or caregiver, if you are around adrenal insufficiency, it may fall on you one day to save a life!

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Genetics?

I want to scream

I want to cry out at God, the Goddess, the Creator

I want to ask a million different questions, but mostly I want to know WHY

Last summer we suspected our youngest daughter may have adrenal insufficiency, too. She was cranky, weak, tired, dark circles under her eyes… Everything I remember feeling for many years prior to my own diagnosis.

We had her cortisol tested. It was low, but not scary low. Low enough for follow up though.

We saw a pediatric endocrinologist, who reluctantly agreed to perform an ACTH stim test, which our daughter passed, though not with flying colors.

The consensus was to wait and watch.

For the past few weeks, she’s been exhibiting symptoms again, most dominantly the extreme fatigue and pain of waking in the morning.

It was time to check her cortisol again.

With a reference range of 5-25, hers came back as 2.2

2.2

According to Cleveland Clinic, any AM cortisol below 3 requires immediate diagnosis and treatment, no further testing required.

We are going to pursue more testing anyway. We need a solid diagnosis, unquestionable by other doctors down the road. But my precious baby is joining the ranks of the AI community, and I wish like hell she wasn’t!

I am angry

Scared

Tired

I don’t know what else to do. I cannot protect her from this. If I had a miracle, I’d give it to her.

Her older sister is heartbroken. She has a more clear understanding of how dangerous this disease can be.

Our youngest had a million questions tonight when we talked with her about why we need to see more doctors. She’s not scared, just curious, and I’m grateful for that. Her biggest concern is whether her medicine will taste bad. But I think she’s excited about the prospect of knowing why she feels so sick all the time.

I am, too, but I wish this wasn’t the case. I’m grateful it isn’t a terminal diagnosis, but it’s still life threatening. Now we get to navigate childhood with that fine balance between protecting her and stifling her. I want to sign her up for a hundred activities so she never misses out on an experience, but I also want to put her in bubble wrap so she can never be hurt.

It’s going to be a challenge, of course. And I find myself grieving all over again.

Me

My husband

Our child

And no idea why the three of us have this rare disease…

And a prayer that our oldest never shows signs of having it, too…

For now, I wait impatiently for more answers, clear directions where to go next for her. I’m building my arsenal for her school, to raise awareness and provide protocols to keep her safe. I have a different focus in my awareness attempts, now with even more urgency.

I’ll probably never get an answer why, so instead I will try to focus on HOW.

How can I make the biggest impact for my daughter’s future?

And how do I ensure that she lives her best life?

Mostly, though, how do I manage my fear… It is loud.

Surviving a Crisis

Death.

The concept is so vague, when you think about it.

But I realized last week that the bell will toll for me much sooner than I’d like.

I went in for a routine surgery. Placement of the port would allow for easier access to my veins both for administration of IV fluids and steroids, and for regular blood draws. Those with chronic illnesses are often blessed with junk for veins as well, and that is certainly true for me.

I could feel myself groggily waking from the anesthesia, and as the nurse stood by me, the radiology technician came back in for another x-ray. They thought maybe they’d punctured my lung.

After a second x-ray, and despite my complaints of lung pain, they sent me home, believing that my lung was fine. Within a few hours, the pain was unbearable, and it was hard to breathe.

Another x-ray at the ER confirmed: pneumothorax. Fairly quickly, the surgeon arrived to place a chest tube. I recall them administering stress dose steroids, and anesthesia, and then I don’t remember much else.

My husband told me later that while they were placing the chest tube, I had an adrenal crisis, which caused them to need to give even more steroids to stabilize me. And then I was admitted to the hospital, chest tube in place, and on oxygen and high doses of hydrocortisone.

For the next few days, my blood pressure stayed frighteningly low at 90/60.

One of the things I’ve learned is that when my cortisol is low, I am not always clear about what’s going on around me. I was quite comfortable to just sleep on the hard bed, and barely moved. By day 4, I realized that my blood pressure had been low, as had my blood sugars, both signs of extremely low cortisol. I was still unstable and floating on the edge of crisis.

The problem was that my body clears hydrocortisone much more quickly than it should. No idea why, it just happens. The typical Addison’s patient would be given IV solu-cortef (hydrocortisone) every 6-8 hours. They were giving it to me every 4 hours, which is how often I take my solu-medrol (methylprednisolone), which is supposed to last 8-10 hours.

By the 4th hour, my cortisol was far too low, and I was slipping toward crisis again.

During a conversation with the Dr, I crashed again, with no warning. It was then that we decided to try a constant IV drip of solu-cortef, meaning that even though I wouldn’t have the peaks of cortisol like typical circadian rhythm patterns, I would also never have the lows.

Overnight, my blood pressure stabilized, and we reduced the overall amount of steroids by half, maintaining my stability.

The next doctor on duty panicked. And I learned quickly that small town doctors are never going to know what to do with me. Based on calls to two endocrinologists, this doctor was going to cut off my continuous steroids and only give me solu-cortef every 6-8 hours, managing symptoms only by confirmed lab work and blood pressure, not my own physical symptoms.

If left to the care of this doctor, or transferred to another hospital with these outdated endocrinologists, my life was going to be at risk very quickly.

Thanks to the actions of the first doctor stabilizing me, I was able to go home at the end of day 6, and am now managing with solu-medrol every 4 hours – and no later. If I’m even a few minutes late, I feel it. I’m still on high doses of steroids and will be for a few weeks as I wean down to normal again, but normal is subjective.

My quality of life suffers greatly. I’m still trying to get hooked up with a cortisol pump, because I know that it will give me a stability and freedom that I haven’t known in many years.

I’m so tired of fighting for my life, and fighting for proper care, and as I sit here swollen beyond belief, I realize that this is exactly what my life will be.

My agenda includes to push for better testing and treatment for myself and others, a solid treatment plan in place for my next crisis event, and writing legislation to allow paramedics and EMS to use patient provided medications in a crisis, in order to save lives.

I’m in pain tonight, still scared, and absolutely terrified of death. I’m not ready to go yet. I have more to do.

And the absolute kicker?

The port doesn’t even work.

Fear of Unread Research

One of the things I’ve learned over the past few years is that there’s an awful lot of emotions involved in chronic illness. There’s the grief in losing your health. Anger at your body for betraying you. Sadness that your entire experience is changing and not always in a good way.

One of the constants that I’ve come to find is frustration. Not just that I’m too tired to clean the house, or that six injections a day are hard to keep up with.

No.

Frustration at medical “professionals” who hold our lives in their hands.

There is constantly updated evidence for what to look for with adrenal insufficiency, both in initial diagnosis and crisis prevention.

Doctors and EMTs, however, do not stay updated on current research. Treatment, diagnosis, and prevention are all being based on research from the 1970s. Research that is four decades old is being used to determine how to manage this disease, despite how inadequate it has been proven time and time again.

In the last month or two, we have seen at least two more deaths due to preventable adrenal crisis.

Outdated information is still driving choices made in the back of an ambulance, in the emergency room, and in the ICU. This has proven to be deadly on many occasions.

Too often, patients are denied life saving steroids because first responders fail to recognize the urgency.

The most significant oversight is related to one specific symptom: blood pressure.

Old studies show that adrenal crisis has many symptoms, one of which is low blood pressure. But through more research, and anecdotal information from real patients, new evidence shows that blood pressure often rises prior to the fall.

Failure to recognize this as only one of many symptoms is dangerous. If a patient has 3 or more symptoms from the accepted list, they are at risk of crisis, and in need of immediate intervention.

These symptoms include, but are not limited to:

  • Low sodium
  • High potassium
  • Intense fatigue
  • Mental deficits
  • Profound weakness
  • Elevated heart rate
  • Low OR high blood pressure
  • Vomiting
  • Diarrhea

Emergency medical personnel are so hung up on the blood pressure, their textbook told them that only low was present. They cannot recognize that the other symptoms are present as well, and the crisis needs treated.

What they fail to recognize is that for a person with adrenal insufficiency, an emergency dose of hydrocortisone, or other corticosteroid, will not cause any harm. However, failure to administer this dose can be fatal.

It’s caused many deaths already.

It’s time for more awareness, more understanding, more funding, more research, and doctors to be proactive in reading research that already exists.

That’s, perhaps, what angers me most. The research that already exists. It is too often ignored. Maybe doctors have too heavy a patient load. But maybe they’re ignorant or lazy, too.

I heard today that a doctor claimed Addison’s disease is mental and can only be treated by a mental health professional.

And this is why people with AI are afraid to go to the ER. We are afraid that we may never go home.

The view from the other side

When I was diagnosed with adrenal insufficiency just over a year ago, our world was turned inside out. Suddenly, we had an answer for why I had been so sick, but a whole slew of questions. What caused it? How would I feel better? WOULD I feel better? Why me?

It seemed like every corner we turned, there was another question. For every answer found, several more questions came. I struggled with oral medications, constantly suffering from low cortisol symptoms I couldn’t control, and increasing doses of steroids just to be able to function.

Eventually, I heard about how several people have begun to use a modified insulin pump as a method to administer solu-cortef. These near constant infusions of steroids allow patients who are otherwise unstable to have greater control over their steroid dosing and symptoms. Many even become able to maintain lower doses of steroids for daily use, which helps to lower the risk of side effects like weight gain and bone loss.

Now, as I’ve spent more than a year learning about this mountain of a rare disease I have to maneuver every day, I’m finding that the world of advocacy is often overwhelming.

With no understanding of why oral medications don’t work for me, I’m resigned to six injections a day of solu-medrol, or methylprednisolone, just to maintain stability. My endocrinologist has no experience using an infusion pump for cortisol, and struggles to get in touch with one who can guide him through the process, from prescription to maintenance.

My ever growing mountain of used insulin needles feels like it will bury me alive, though it’s the only thing that keeps me so…

Now that I have a laundry list of diagnoses, which keeps growing, I’m learning that it isn’t any easier on the other side of diagnosis. Yes, I’m diagnosed, treated, and alive, but it often feels like no matter what I do, it’s always an uphill battle in front of me.

For now, I focus on gathering resources, making contacts with similar experiences, and hoping that I can just continue to put one foot in front of the other, taking one day at a time, until I reach the peak and can just enjoy the view from the other side.