How Did We Get Here? June's Story, Part III (By Whitney)

Part III: August 2010- June, 14 months.

I went to get June from her crib one morning. I picked her up and smelled bad breath. Uh-oh...we know what that led to last time! But that must have been just a fluke, that wouldn't happen again.

June was fine all day and spent a few hours with close family friends while Tony and I attended a wedding reception that evening. We picked her up and on the drive home, we could hear her breathing. She sounded a little stuffy, but did not worry too much about it.

The next morning, June had a full-blown cold. Heavy, labored breathing, congestion, and mild fever. A temperature of 101-102 did not mean a lot to us because we had already been in the 104s! She was very clingy and seemed weak, but I thought, "well of course she is weak, she is sick." I called the nurse to ask what we could give June for a cold, and she gave us the typical instructions: humidifier, tylenol, liquids, etc. She asked me to describe June's behavior. She paused, then said, "It is okay for a baby to have a cold. It is okay for a baby to have a mild fever...but she shouldn't be weak." Meanwhile, I'm whispering to Tony..."I don't know, do you think she is weak??" I had mentioned that we were waiting for the test results on Addison's, but remember-I had not read about Addison's at this point, so I had no idea what it was. Based on what had happened in February, the nurse said she would feel better if we took June to the ER at Children's Hospital. I hung up the phone and sighed in disbelief. I couldn't believe we were taking June to the ER for a cold!

When we got to the ER all I could think was, "Oh geez, they think I am that parent! Here we are with a child with a cold...wasting their time...they think I am overreacting..." I admit, I felt a little embarrassed. With the help of the tylenol, June's fever had even come down to 99! Grrreeeatt...even the nurses think I am being ridiculous!

Pre-cortisol

When our ER doctor came to see us, I know he was thinking the same thing I was! He was oh-so-casual and hooked June up to an IV to hydrate her and said we would soon be taking her home. He left (probably rolling his eyes and wondering why we were taking up space in the ER). When he came back to check on June, I said, "I don't know if this has anything to do with it, but she is being tested for Addison's." Well, well, well...did HIS attitude change! He paused and said, "I'll be right back."

The doctor came back in, sat on the bed, and was much friendlier with us. He said he had spoken with Endocrinology (we were due to see them the following week) and that he would be admitting June.

We got the diagnosis the following morning. The endocrinologist overseeing our case was wonderful and really stressed the importance of understanding what all of this meant. She said that most likely, the February episode had been an Addisonian Crisis, and she was amazed that June had bounced back from it so well. She was also amazed at how well June had been ever since. Her cortisol level was below 1! Her ACTH was a whopping 3,006. And we would later find out that her aldosterone level was undetectable.

Post-cortisol

Just a month earlier, I had felt so silly asking our pediatrician about June's skin tone. In the hospital, our doctor looked me in the eye and said, "You asking about her skin saved her life." That statement took my breath away, and only at that moment did I finally understand what this all meant. I also tearfully thanked June's pediatrician for taking me seriously. He could have easily blown me off and said, "It's the middle of summer, you just got back from Phoenix, let's check her this winter." Everyone in the hospital told us how lucky we were, and now knowing more about this disease, I would have to say I agree.

A petition to support the initiative to get Solu-cortef available through EMS.

http://www.change.org/petitions/adrenal-insufficiency-united-create-protocols-for-adrenal-insufficiency#

Also check out Adrenal Insufficiency United : http://adrenalinsufficiencyunited.org

SICK DAYS

Everyone gets sick. When kids start school or daycare it seems as if they spend the first few months continuously ill as their immune systems adjust to the new environment. For most parents, they cope with the runny noses, fevers and upset stomachs with extra rest and fluids. For parents of kids with Addison's or adrenal insufficiency, we walk the tight rope between illness and crisis. Even a small illness can be a crisis or can turn into one quickly. Here's some things I learned in a recent bout with Stomach flu in our house.

The National Endocrine and Metabolic Diseases Information Services list the symptoms of adrenal insufficiency as chronic fatigue, muscle weakness, loss of appetite, and weight loss. You could also see nausea, vomiting, diarrhea, low blood pressure, depression, irritability, salt cravings, hypoglycemia, headache, and sweating. According to the National Institutes of Health symptoms of stomach flu include nausea, vomiting, diarrhea, low blood pressure, hypoglycemia,weight loss, fever and chills. They are too similar to tell apart.  If adrenal insufficiency isn't treated, it can turn into an adrenal crisis- sudden pain in the abdomen or back, severe vomiting and diarrhea, dehydration, low blood pressure and loss of consciousness. So how do you tell them apart? You can't and that is why it is always important to stress dose at the first signs of illness. Adrenal insufficiency often progresses slowly and the change in syptoms are barely noticeable. By the time you notice symptoms, it's time to stress dose.

It started out as a stomach ache in my house. My son, who would eat anything in sight- turned down another piece of pizza. The mommy alert was heightened. By evening, he was vomiting.  Since I have 4 kids, we institute an immediate quarantine. It was a miserable night and following day. By the second day, I was ready for a break and it hit the second kid- my adrenal insufficient kiddo. As luck would have it- if you could call it that- we had a pre existing appointment at the pediatrician's office. I took her in and she was nice enough to vomit in the doctor's office in between passing the football with her brother down the hall of the clinic. They looked her over- probably a virus-keep stress dosing her and call if it gets worse. I triple dosed her, she didn't vomit and she was obviously feeling better by the end of the day. The next day we went back to normal dose and her sister was hit with the puke bug. I got distracted. I was sleep deprived. My littlest one was dehydrated and I was busy trying to keep her fluids down- I probably missed the signs that my adrenal insufficient(AI) kiddo wasn't 100%.Looking back, I should have continued the stress dosing, especially since the bug was still ravaging our house.

We had a day "off", no vomiting and then the AI child wakes up with a tummy ache. She didn't eat breakfast. I gave her the morning hydrocortisone and fludrocortisone- she immediately threw them up. Here we go. I spent the next 2 hours trying to get her to keep her meds down. No luck. I had leftover anti nausea meds from when she was in chemotherapy and I gave those a try. She threw that up. I tried her meds again. Threw up again. I tried the antinausea again. It stayed down longer, but still came up. She would take a plain sip of water- it would come up. We officially missed her first dose. It was lunch time. On a normal day- missing a dose of hydrocortisone is serious. I usually notice the emotional symptoms first- she gets weepy and way too emotional over small things. That is my cue that something is up. On a sick day- missing a dose can be life threatening. I page her endocrinologist who immediately tells me to give her the shot. I look at the needle and the 100mg of Hydrocortisone and wonder if it's really necessary. I look at my child, getting increasingly lethargic and vomiting bile and realize it is.

I plunged the yellow stopper, releasing the powder into the solution. It was cloudy and I swirled the container until it was mixed and clear. I attached the needle to the syringe. I peeled back the little yellow circle from the top of the vial and wipe the vial top down with an alcohol wipe. I plunge the syringe in the vial and draw back mostly air.  I remove the syringe, plunge the air out and try again. It helps to pull the needle down a bit in the vial- you get less air that way. Her doctor and I decided to give her half of the shot. The shot is 2 mL, so I just measured out 1 mL. This was my first mistake. In the future, I will give the whole shot. When my daughter was in chemo, they gave her massive doses of steroids and we suffered from the roid rage fall out-I've been preconditioned to dread that. This situation was different. If I am in the situation again where she needs the shot- I will give her the whole shot. Her body needed the boost and she probably would have recovered quicker if I had given her the whole thing. Lesson learned. By the time we got to the ER, she was pretty lethargic- despite 50 mg of Hydrocortisone. They gave her 30 mg more there. She never did have a fever. Fever is a symptom of infection and that is why stress dose is required then.

Giving the actual shot wasn't as bad as I feared. I pinched a big chunk of her thigh because I didn't want to jab her in the bone. I have been reassured that the needle will not hit the bone- and after giving the shot, I will concede that. It does make a slight popping sound when it pierces the skin- which really creeped me out. I think the best way is to be quick- jab quickly, plunge, remove quickly. I can only say this, now that I have done it. I dreaded the shot. It scared the bejeezus out of me. My reccomendation is to practice. This is tricky since you cannot practice on a person. If you have an extra supply of syringes- fill one with water and jab a pear, apple or potato with it. It helps to have a dry run. If you don't have extra syringes- you can buy insulin syringes at a drug store or walmart to practice with. It's not the exact same because insulin syringes are much smaller and designed to go subcutaneously(under the skin) whereas it is important for the Hydrocortisone to go directly into the muscle- hence the big scary needle and the big thigh muscle. The important thing is if you are worried- to practice, it can set your mind at ease. It also helps to go over it with a neighbor or friend who is close by. That's what I did- so when it comes down to an emergency not only do you have support, but you have back up. The other thing you can do is practice plunging the hydrocortisone act-o-vial when the vial expires. Be sure to check the expiration date when you check your emergency kit.

I took my daughter into the ER and she was admitted for 2 days. I don't know if I could have avoided the hospital admission altogether- but if I had given her the shot earlier, it may not have gotten as bad as it did. The body needs the burst of hydrocortisone when it's sick- this is never the time to float a dose. I hadn't realized in how short a period of time she could go from bad to worse. In the morning she was just sick- by lunchtime we were diving into crisis. My doctor gave my the guideline- the oral hydrocortisone has to stay down for an hour to really "count". I was just shooting for 20 minutes. In the future- if there are multiple vomiting episodes in one hour , I will give her the shot. If she has diarrhea more than 2-3 times in an hour- she probably is not absorbing as much hydrocortisone as she needs and I will give the shot. It is important to give the shot before going to the ER or clinic- if you think they might need the shot- they need the shot. I will definitely call her endocrinologist if I am concerned and worry less about "bothering" them. That is what they are there for- had I called earlier- they might have been able to get her into the clinic for fluids and extra hydrocortisone and we might have avoided a full blown crisis and a 2 day respite in a hospital room. There are no little stomach bugs with a child who has Adrenal Insufficiency.

Annie Sullivan's Memorial

Please take a moment to read about a little friend, Annie Sullivan. She and her family battled Addison's for years and her mom Jean keeps a blog of their trials. On Friday March 25, Annie became an angel and all of our hearts became a little heavier. Please keep her family in your thoughts and prayers as they remember their very special little girl.


http://annaleesullivan.blogspot.com/2011/03/annies-memorial-service.html

Medical Identification Jewelry

At our first endocrinology appointment following diagnosis, we were handed a pamphlet to order a medical alert bracelet and a temporary bracelet. It was a plasticky thing that opened with little scraps of paper inside to write information on. I wasn't sure who came up with that as a good idea because my 4 year old proceeded to dismantle it and toss the papers to the ground. I'm pretty sure I tossed it in the trash as we exited the hospital. When I got home, I began my research on bracelets that might be able to keep up with a kid.

Adrenal Insufficiency requires immediate attention in the case of an emergency. On multiple sites, it is listed as the first reason a medical identification tag is needed. Yes this is because it starts with "a", but the fact is- it's listed. Many parents reason that their child will never be unattended and therefore jewelry is not necessary. Kids wander off, accidents happen- it is necessary. When minutes make the difference between life and death, a medical alert bracelet can make sure your child gets the medicines they need. I know far too many parents who spent months and years trying to find out what was wrong with their child, only to finally be told it was adrenal insufficiency. Why would we expect emergency medical professionals to be able to diagnose it in a few minutes?

If you do a web search for medical ID bracelets, you will find dozens of retailers. You can get ready made, custom made, bangly, sparkly, sporty and pricey bracelets. They are gold, silver, titanium and swarovsky crystal. You can get them at Walmart, Costco, Amazon and other retailers- even ebay. You can also find necklaces, pendants, watches, keychains, shoelace charms and USB flash drives. You have sites that tell you to put every piece of identifiable information possible in a 2 inch spot and others suggest less. So what's a parent to do?

WHAT DO I GET?

Plan for the worst case scenario- your child is found unconscious and no one knows what is wrong or their history. It is most useful for them to have identification on their person. What is the first thing an emergency responder does when they check a victim? They take their pulse. This is why bracelets are so popular and useful- the wrist is used to get a pulse. The neck can also be used and is the second site generally looked at to make sure there is an open airway. A down side to necklaces is that they can be overlooked if they are long or fall down into the clothing and sometimes  they get in the way of  every day activities. There are watches, shoelace tags and keychains- but emergency personnel are not to trained to look for those- they are trained to look for a bracelet or necklace upon initial exam. These identifiers are useful reminders though for caregivers or less emergent cases.

I have heard that many people don't like to wear medical alert jewelry because it is an announcement of a disability as well as a painful reminder of their condition. I can appreciate that, but if they do not get timely diagnosis and aid in an emergency situation, the only reminder of their condition will be a tombstone. It may be harsh, but it is the reality.  Many of the bracelets have gotten more stylish and have a variety of wristband options- anything from sportsbands to leather to silver to gold to crystal beads. I used to worry that EMS might not recognize a pretty bracelet as much as a standard issue- but if it comes down to something or nothing- something (pretty) is always better.

You want the identification to have a caduceus(serpent and staff) and cross(some have 4 arms like a t or an x and some have 6 like an *) symbol on it- that is universally recognized as the medical alert logo. It can be on a big charm or a little charm- but you want that charm to be visible and readable. You want it to be activity appropriate. If your child is not very active- a light beaded model might work. My daughter snapped off 2 stainless steel models and a sportsband model in a 6 month period. I generally go for the buy one get one free sales when I can. If nothing else, I have a spare. I did notice on the more delicate model we had, it had a raised medical symbol- that sheered right off- I would recommend getting one with the engraved medical symbol. I also noticed the sportsband got grubby and dirty and started to smell after awhile- definitely something to think about if you have a kiddo who is already sensitive to germs. I also recommend getting one right away- it took a little while for my daughter to get used to her bracelet, but now she doesn't notice it anymore.


WHAT DO I PUT ON A MEDICAL ID BRACELET?

Put yourself in the emergency situation and in the role of the emergency medical professional. What piece of information does that person need in the first minutes of an emergency to treat your child as quickly as possible? Adrenal Insufficiency. I put it first, so it will be the first thing they see, it is a rare condition, they may or may not immediately know what treatment or medication is needed. Hydrocortisone(or whatever medication your medical professional recommends for your child during an emergency.) NAME. For my child, I want them to know her name so they can address her or if she is not with me- I also want them to be able identify her and be able to contact someone who can help inform them on her condition, whether it be a neighbor or school official or coach. Phone Number. I put my cell number since I always carry it with me. Some people put a doctor's number- it should be a number to someone who knows about your child's condition and will answer!  If you are uncertain what to put, your physician can be really helpful in this area. Both the Medic Alert and American Medical ID sites have recommendations. Some tags can be engraved on front and back, some only on the back- this can be a consideration if your child has mulitple conditions. This is not a time to engrave lactose intolerance or color blindness, we are looking at acute and life threatening conditions, like adrenal insufficiency.

WHERE DO I GET MEDICAL ID BRACELET?

This is the tough one. The answer depends entirely on what you want. One of the most well known retailers is Medicalert.com  They offer jewelry as well as a medical database service with your child's history in it. Emergency personnel can call the 24 hour service and be alerted to the child's meds, history and needs.  There is a service charge and a monthly fee for this that runs $20-$30 a month. There is a good selection of ID tags, with rounded edges.  Although I prefer their jewelry, you cannot buy it without joining their service and I don't need it, so I go to americanmedical-id.com . The prices are reasonable, they too offer an optional database service and a fair selection but there are some drawbacks. One of the drawbacks is that the bracelets do not come in half sizes- this is fairly problematic with kids. Too tight- they are miserable- too loose and it is gone. I solved this problem by enlisting a few links of chain and some help from a jeweler neighbor.   The edges are only more rounded in the more expensive models, which is disappointing. My daughter is very active and I worried about her scratching or gouging herself, but it hasn't been a problem. Another complaint is that the engraving is difficult to see. The manufacturer carries a warranty on this but also emphasizes that the engraving is legible when looked at directly and not casually or from a distance as to protect the person's information. I think that is a valid point.  There is merit to having your information out there when necessary and transmitting it with neon lights.

If you are in the market for kid specific, sporty, prettier, custom, or unique looking jewelry- friends have reccommended two places. Lauren's Hope @ Laurenshope.com and Petite Baubles Boutique @ petitebaublesboutique.com Many jewelers carry medic alert bracelets in their stores- I have seen them at Walmart even. Some can do engraving on site and others have to mail it out. If you find a piece you really like and that works- you can always have it professionally engraved and many engravers can incorporate the medical alert symbol.  The most important thing is that you find something your child will wear and that they wear it! It could save their life!

Medicalert.com
americanmedical-id.com   
Laurenshope.com
petitebaublesboutique.com

Adrenal Insufficiency from a Child's Perspective

Thousands of teens struggle each year to come up with good essays for college entrance exams. There are questions about accomplishments and overcoming obstacles. Many soon-to-be adults struggle to define or appreciate any life threatening or altering experiences they have had because they simply have not had them. Kids with adrenal insufficiency face barriers that normal kids do not. As parents we often wonder if they grasp the seriousness of their situation while at the same time wanting to encourage them to reach for their dreams and do the things they love. When 10 year old Landon was asked to write an essay about "breaking barriers", he captured the heart of what it is like to live with a disease and persevere. Sometimes you can't go through, you can't go around, you can't go under- sometimes you just have to break through and Landon is doing just that!  If this doesn't win the contest, I don't know what will!

My Barrier
By Landon Adzima

...
I bet you don’t know what Addison’s Disease is. John F. Kennedy had it and so do I. It is a rare, life threatening disease.



When you have Addison’s disease, your body won’t produce Cortisol (Adrenaline). Cortisol helps your body respond to extreme stress such as; trauma, injuries, broken bones, sickness and intense physical activity. As a result, I have to take medicine three times every day to replace the Cortisol my body doesn’t produce. In addition, I have to carry a crisis kit that includes a shot of Hydrocortisone (not the cream for itching), pills of Hydrocortisone, an Epipen, and an inhaler. I also wear a Medic Alert Bracelet at all times that has my medical issues engraved on it.



One big problem I have with this disease is fatigue. If I have a lot of physical activity, I don’t eat often or don’t get enough sleep I become very fatigued. I have been hospitalized because of my disease. When I get really sick my body can’t take it very well. I have to get my medicine through an IV. It can be scary.



After telling you about my disease, you would never think I would be a wrestler. Well I am a wrestler! The one major thing that is hard about wrestling is the constant pounds I take to the mat and the complex body bending moves. I also get fatigued so easily during and after matches. For example, one time I had a rough first match and got so fatigued that I was not able to recover physically in between matches. I ended up losing my next match and was wiped out for the rest of the weekend. In order to prevent this I have to take double or triple doses of my medicines. This is called Stress Dosing.



There is no sport to compare to wrestling. It is the roughest, most strenuous sport. My teammates are phenomenal! When I am wrestling the whole team is cheering for me. When another teammate is wrestling the whole team is cheering for him. My coach says there is no sport where the team is so close and teamwork is so important. The team is not a team it is a family.



Although wrestling is a tough sport, and even tougher for me because of my disease, I still have persistence and determination. I am always determined to try my best and have fun. I also work my hardest every single practice and tournament. I am persistent to keep wrestling no matter how I feel physically. In wrestling you win some matches and you lose some matches. When I do lose, it breaks my heart. My coach and teammates always tell me to keep my head up no matter what.



Most of the people that know about my disease and the conditions I have to overcome are amazed that I am a wrestler. Thanks to my pastor, my family, my team and many other people, I have been able to overcome this tough and exhausting barrier and I am able to do what I love-Wrestle!

That bridge fell down... (By Whitney)

Remember the bridge that June and I were going to tip toe over? The one to Mother's Morning Out? Well, it collapsed.

On Monday, I went to meet with the director of the program and one of the teachers to go over specifics of adrenal insufficiency such as what to look for, and how and when to give an injection. There were about 10 other children there that day, and they were just sitting down to lunch when June and I showed up. I brought a snack for June and sat her down at the table with the kids. As I made my way out of the room, she got up from the table to follow me and then a teacher led her back to the table. No whining, no tears (from either of us)-phew! After my meeting, I poked my head into the classroom to see how June was doing. She was fine, and I asked if I could sit in the back and watch her play for a little. It took about 5 minutes for June to even notice I was there! And that felt great! When she did see me, she ran over to me, smiled, and ran back to where the kids were playing. Then she started to laugh and spin in the middle of the carpet! I was soooo happy!!! June was so happy and just doing her thing. Big sigh...we were ready! I signed June up for Friday and the following Monday-just for a couple of hours.

Tuesday morning, June woke up with a low grade fever. It slowly crept up to the flashing 101 mark; she was weepy, weak, and just not herself. We TD that night and gave her tylenol, then checked her throughout the night. On Wednesday, we visited the pediatrician and he diagnosed her with an ear infection.

That wasn't what I was upset about. No Mother's Morning Out, he said.

It took me nearly two months to make the decision to start MMO and I was FINALLY happy with the decision to start June this month.

You know, here is the endo subtly telling me to toughen up and treat her like any other child (with extra precaution of course), and then when I try...the pediatrician is saying that she is not ready for that kind of germ/virus exposure. So then WHAT exactly?!?

(Virtual scream)

Here is my problem-both doctors are right, and then my own opinions are in the mix somewhere, too. How do I keep everything straightened out!? And here is another problem-there is no right answer...and I like answers!

So long for now Mr. Bridge. As we got closer to you, you were looking pretty good. We're going to walk away now, but we will revisit in a few months. Hang tight!