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

PART I: February 2010-June, 8 months old.

It all started as a cold.

One morning, as I was getting June out of her crib, I noticed she had bad breath. That was weird, because normally her breath didn't smell like anything! As the day progressed, I noticed she was having difficulty breathing and was only breathing out of her mouth. And because she was still on the bottle at the time, June could only take a few big sucks and then she would gasp for air. Her temperature had also risen steadily for 24 hours, up to 104 degrees. We were doing what the nurses were telling us to do to treat her cold and fever.

The following morning, June's fever had dropped slightly so I felt a bit of relief. I sat down with her in my lap to give her a bottle. Soon after, she vomited it all back up. I called the nurse again and she thought it would be a smart thing to take June to the ER to treat her for dehydration. June was getting very weak. I drove her to the ER, and at every stoplight turned around to shake the carseat to make sure she was okay. She was barely moving or making any noise and it terrified me.

June was hooked up to an IV, given a chest x-ray, and tested for a urinary tract infection. Everything looked clear, and after some fluids she was able to sit up again. After 4 hours, we were sent home.

Soon after we got home, June vomited again. And it did not stop. We were instructed to give her teaspoons of Pedialyte using a medicine dropper. She kept some down so I felt better, then after several hours, it all came back up again. Called the nurse back. June was growing weak again, and she didn't even have the strength to sit up when vomiting. We were told to go back to the ER. It was midnight, I didn't want to go back and put June through that again. It had been a stressful morning, a long day, she just needed to get some sleep. So I was thinking, "we have to drive back there, hook up the IV again, more tests...my poor baby just needs to sleep." I truly thought she could keep a little liquid down and sleep it off, I didn't want to bother her; she was just so worn out. So I said to Tony, "Ok, if she throws up this teaspoon, we'll go." June threw it up, and off we went.

Her glucose had dropped to 23 (which we did not understand at the time, but later learned that one can lose brain function with a glucose level below 40). She was lifeless. I was fortunate enough to know the chief of the emergency room at our Children's Hospital, and he happened to have the midnight shift. We got VIP care. She had several tests done (what tests, I couldn't tell you, I was in a state of shock as nurses were running back and forth from our room). I was well aware, however, of our doctor administering a spinal tap on June. I bent over and cried my eyes out at the mention of meningitis. June barely made a noise when that huge needle went into her tiny spine, and that only told us how weak she truly had become. She had wires attached everywhere, and the sound of that damn blood pressure machine beeping still haunts us to this day!

June was admitted and we stayed at Children's Hospital for 3 days. The team of doctors could not figure out why she had gotten so ill, and so they boiled it down to a nasty stomach virus. All tests had come back negative (phew!).

When we got home, June had acted like nothing ever happened! Mommy and Daddy, on the other hand, needed about a week's worth of rest! So...la-dee-da...life went on.

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

Part II: Spring/Summer of 2010

June was about 10 or 11 months old when, one morning while changing her diaper, I noticed that her tummy seemed darker. I didn't really think anything of it. My mom has a darker skin tone, so I thought she could be taking after her. (Tony and I are fair). Towards the end of April, her legs were starting to get dark. Pittsburgh in April is still pretty cool, so I knew this was not from the sun. Getting into May, her legs were getting darker, tummy still a little dark, and her arms were now getting dark. Everywhere we went, and I mean e.v.e.r.y.w.h.e.r.e I would hear either, "Wow, she is so tan!" or "I guess you were just at the beach!" I am not exaggerating when I tell you that I probably heard this at least three times a day. I was close to making a t-shirt stating, "She is NOT tan!" and save myself the explanation.

As we got into the month of June, June's legs were even darker. As she sat in my lap in her onesie, her legs against mine, I just stared in complete awe of her skin tone. People had asked me if we spent a lot of time in the sun, or if the sun was hitting her legs while in the stroller. I think what they really wanted to say was, "don't you put sunscreen on your baby?" (One person asked me if she was eating a lot of carrots!)


In July was June's check-up with her pediatrician. I always go in with a list of questions, no matter how silly they seem. My mom kept asking me whether or not I was going to mention her skin at the appointment. I mean, yes I was going to, but I felt kind of silly doing so. Afterall, it was July 1, and we had just gotten home from a week in Phoenix. This doctor is going to look at me like I have two heads!

During the Q&A session of the appointment, I sheepishly asked, "Ok, her skin tone. She is really dark. Could I be missing something, or is this just her skin tone?" June's pediatrician did not look at me like I had two heads. Instead, he scribbled something down, told me not to Google it, and sent us to a pediatric dermatologist.

HA! Of course I Googled it! He had scribbled, "Addison's Disease." I skimmed the first article that I found, read the symptoms, and then saw that it could be fatal. I closed the page immediately and walked away saying, "He was right." But I did read those symptoms: nausea, weakness, fatigue, weight loss....June didn't have any of those. So she didn't have Addison's.

The dermatologist agreed with our pediatrician. She said that her skin was "definitely not normal." In fact, 5 other dermatologists from around the office came in to see June, and one asked to take pictures! They ordered bloodwork, and also made an appointment for us to see a pediatric endocrinologist in a couple weeks.


After two weeks we still had not heard the results. I was going to call on a Friday afternoon but we got caught up in a playdate, and I thought, "if it was bad, we would have heard by now." So I decided to call the following Monday. Well, I didn't get to make that call, and in the next section you will know why. (Side note: the dermatologist never got June's bloodwork from the lab, which not only infuriated the doctor for such a mistake, but also Tony and myself)

Lesson learned-call your doctor if you are waiting for results!

Three days before diagnosis.

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