Monday, January 21, 2013

Annie's Adrenal Crisis

"Those who don't know history are destined to repeat it." Edmund Burke

In light of those words of wisdom, let me tell you Annie's story again. My thought is that if you or someone you know ever shows symptoms like Annie had, you will run to the doctor--preferably a diligent endocrinologist--and avoid the tragedy that happened to us.

Six years ago today, on January 21, 2007, my three and a half year old daughter, Annie, suffered an adrenal crisis that resulted in a severe, hypoxic brain injury. We put her to bed with a little cold, and the next morning we found her blue, eyes staring straight ahead, teeth clenched, and arms pulled up to her chest. She was in an on-going seizure after her blood pressure dropped and her blood sugar had dropped, causing her to go into cardiovascular collapse.

In the year prior, beginning in January 2006, she had had multiple episodes of dehydration, stomach pain, nausea and vomiting. She had been hospitalized for three days, we had taken her into the ER three times, and to the doctor many times in between. No one diagnosed her with Addison's disease, or adrenal insufficiency. No one referred us to an endocrinologist.

She took three hour naps. She was thin. She craved salty Goldfish, pepperoni, and pizza. She also began to be fearful of little things like walking down steps alone--things she had previously been doing fine by herself. And her skin got progressively darker until she had a tan that prompted a friend to start calling her "Malibu Barbie."

Then one night in January I put her to bed with a cold--and she almost died as she slept.

Addison's Disease is a disease of the adrenal glands, and results in adrenal insufficiency, or AI. AI can also be caused by secondary problems such as tumors of the pituitary gland, or adrenal glands, or injury. The adrenal glands produce cortisol, which is necessary for your body to fight illness and stress. If your adrenal glands don't work, you are at risk of an adrenal crisis--a life-threatening event.

The treatment for Addison's, or adrenal insufficiency is simple: hydrocortisone tablets, usually taken three times a day, to mimic the way our adrenal glands supply our bodies with cortisol. During times of illness or stress, the dose is increased double or triple to mimic what the body does naturally to fight off infection. In times of crisis, an injection of liquid cortisol (Solu-Cortef) is given.

Annie was saved from dying that morning, but not until after she had already sustained a catastrophic brain injury. She lived for four years without ever regaining the ability to walk independently, say more than 5 words, or eat. And her brain function went from precocious 3 1/2 year old to infant--overnight. Annie passed away on March 25, 2011 of complications of the flu. We grieve the loss of that blond-haired, pink-bowed sassy little girl, but are continually comforted that we will see her again in heaven soon.

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What does this all mean for you?

If you or someone you know has weakness, or gastrointestinal symptoms such as nausea, vomiting, weight loss, dehydration, and has unexplained darkening of skin color--think adrenal insufficiency. Adrenal insufficiency can develop at any age--young children, teens, adults--boys, girls, men or women. Get to a doctor--an endocrinologist--and insist on a simple cortisol test. It could save your life!

If you are a parent of a child diagnosed with adrenal insufficiency:

1. Be diligent in times of stress or illness. Trust your gut--if your child looks or acts the least bit sick, assume they need a stress dose of hydrocortisone.

2. Train family members, caregivers, teachers, and school nurses on stress-dosing and giving the emergency injection. Train them on recognizing the subtle signs that your child needs more hydrocortisone. Train them on what an adrenal crisis looks like for your child, and what may cause it.

3. Learn as I did that calling 911 is not a substitute for giving the emergency injection in a crisis. Driving to the ER is not a substitute for giving the injection. Every second counts. Get over your fear and queasiness and give the injection first and then go get help.

4. Join our Facebook group Parental Support for Adrenal Insufficient Children or see our Facebook page "Cupcakes for Annie"  and get support from other parents.

5. Finally--Read, read, read about AI. Learn about the subtleties of your child's brand of AI. You will become the expert on your kid. Be a bulldog advocate for them. Don't be pushed around by anyone who minimizes or brushes off what you know to be true.

Remember to learn from my history--Annie's history--and recognize the signs of adrenal insufficiency. If her story saves one life, it helps to redeem our loss.

Jean



8 comments:

Anonymous said...

My two granddaughters, Olivia and Kinsley, also have Addison's Disease. Olivia had a crisis after suffering from undiagnosed Addison' s at about 15 months old. Ironically, it was the same day as Annie's. She continued to suffer from low blood sugar and seizure episode s until finally diagnosed with Addison's at 3 1/2. Little sister, Kinsley, was diagnosed st 7 months old after being rushed to the ER having a seizure. In the ER she was having a crisis and stopped breathing. Thank God, the doctor was in the room and bagged her for the twenty minutes it took to stabilize her.
Both girls are on daily doses of hydrocortizone. They continue to be monitored by an endocrinologist. Annie's story is very dear to our family. She is forever in our thoughts and prayers. Annie is a guardian angel to all children
susuffering from AI issues
Thank you for sharing her story and inspiring others to spread the message of awareness.

Jean said...

Thank you so much for writing! When I first read about Olivia on Dusty's Addison's Support blog, I just wanted to call Amy up and pick her brain. We have since connected over the Facebook "Parents of Adrenal Insufficient Children" page--thank God for that support system!

Then when I heard that little Kinsley had AI too, I just was awed that Amy manages TWO little ones at the same time. It is a huge challenge. And you know from their brushes with death how incredibly important it is to stay on top of everything.

God bless you, Amy, Olivia & Kinsley with good health and peace in any crisis as you walk this AI road together.

Terri Bebingh said...

I stumbled across your blog posting because I am searching for parents detailed events of what AI looks like and how a crisis was handled. My 19 month old daughter was diagnosed c-php so she suffers AI. I am planning a training course for our parents and caregivers on how to use the cortef and asses a crisis properly. I plan on sharing your story as this gets down to the nitty gritty and fearful aspects of this condition. Thank you for sharing your story. Sending blessings to you and your angle. Im so sorry for your loss.

Jean said...

Thanks, Terri--and thank you for sharing Annie's story with your parents and caregivers. I am very interested in helping however I can.

If I could add anything, it's to urge caregivers to err on the side of caution when assessing children. They can drop like rocks, going from fine to life-threatening in a matter of minutes.

Also, don't forget to encourage your parents to check out the FB page Parents of Adrenal Insufficient Children for lots of support.

Martin Norrman said...

Hi
My name is Martin and I started the Swedish Addison association. I´ve a question and wonder how I can mail you?

/Martin

Jean said...

Hi Martin--

Under "More Information" on the side bar there is a link to contact me.

Congratulations on launching the Swedish Addison Association--looking forward to hearing from you!

Jean

Patricia Barber said...

This story grieves me as I also suffer from Addison's. I am in my fifties, and so I was able to do my own research and basically diagnosed myself. Children don't have this opportunity. I am currently just out of the hospital in New York, as I was visiting my son's family. I started vomiting profusely and after two hours, my husband administered the solu-cortef shot. I had been carrying this shot in my purse for years. The ambulance came immediately after I received the shot. In the ambulance, I was not aware of much, but I do remember the EMT saying he could not find a pulse or blood pressure. However, slowly the steroid shot kicked in and I was at least conscious, but still had very low blood pressure, I ended up having mesenteric panniculitis of the small intestines. It meant I had inflammation and a severe infection there as well. The Endocrinologist at the hospital said I was their new "poster child" because if I had not received my shot I would be on a ventilator in ICU or in heaven. My greatest concern is the lack of knowledge among the medical staff. This is a serious disease that is often fatal, and there should be much more training out there for the medical community!! Ambulance personnel should carry solu-cortef shots and know when to give them. My youngest son will get married in November and I will get to be there because my husband knew the signs of an adrenal crisis. How many medical personnel missed the signs in Annie? I pray her death will not be in vain, but will spur a movement to educate all about this terrible disease.

Jean said...

Thank you for kind words, and for sharing your story, Patricia. I am so glad your husband had the presence of mind to give you the Solu-Cortef injection. He did save your life!

And yes, it's hard for people with adrenal insufficiency to understand when you call 911, you might be met with EMS professionals who are unfamiliar with this rare disease. That's why we started Adrenal Insufficiency United, and why we do Cupcakes for Annie (https://www.facebook.com/CupcakesforAnnie) each year. Our awareness and training has saved lives, as attested by both EMS and ED staff. Awareness saves lives.

Annie's death is not in vain. We do miss her terribly, but we are grateful to the people who help us share adrenal insufficiency awareness. We want to do our part to prevent others to suffer what happened to her.

And beyond that, we know that Annie is with Jesus. That is our ultimate comfort.

Thanks again, Patricia for writing--and keep that hubby close by!