By Lisa J. Markland
Originally posted on FB in the Addison’s Disease Support Group. To respect privacy some names have been changed to initials.
As the ADSG’s founder, along with the group’s admins, I’d like to let you all know of the very sad passing of several of our members. I also want to take this opportunity, as I’ve promised to each of the families who have lost loved ones recently who were group members (listed below), to enhance awareness of how preventable most if not all of these deaths were. We all need to be super-vigilant that our doctors are doing their jobs and that we stay on top of things like our dosing, stress dosing, labs, emergency kits and medical alert bracelets. These things together can almost always save lives affected by AI. This will be a very long post because I’m going to add in a repost of what I wrote when we lost 6-year-old M. back on Christmas eve/Chanukah 2016. It’s geared for parents but really is so pertinent to ALL of us– every one of us diagnosed with AI of any kind (I am not editing the old post, so please bear with some of the information, written around the start of 2017). The message never gets old and can’t be restated enough, as long as we’re still hearing of deaths from AI. Please do your part to help yourself and others by spreading the word and doing what’s needed and ensuring your doctor has ALL the information he/she needs to help you properly. If it’s not correct, don’t agree. Be as educated as possible, even more than your doctor. Our group also has amazing resources in our files tab, so please be sure to read through it all. This is your life and it’s too important.
In just the last few months, and in addition to the many lives we’ve known have been lost from our group from AI-related death or suicide since I started this group in early 2012, the following members are being honored with this post and your proactive efforts to help us raise awareness and prevent additional loss of life:
K. and her mom, L.: K. was being helped by one of our amazing members who’s also on our ADSG Advisory Board (AAB)… and was hopefully going to join the group this past month when she unfortunately passed away from her under-treated AI. I was working to get her a free medical alert and other resources. She wasn’t even being treated by an endocrinologist and wasn’t on Florinef, but had been diagnosed for several years with primary Addison’s.
She was still fighting for her emergency injectable kit and more. Shortly after her death, her mom L. died as well, likely from the stress of losing her daughter. L. had been diagnosed with primary Addison’s as well, and likely died from not properly being able to stress dose while grieving the recent loss of her daughter.
On a very similar note, it was just this week I learned of the loss in January of another member, C.T., who had joined the ADSG after losing her dear husband last year, J.T., to Addison’s. Despite years and years of ER visits and more, he was not properly diagnosed or treated and the hospitals really dropped the ball, as did his doctors. C. died of a heart attack, likely a second case of death from a ‘broken heart’ in not that many months’ time that we learned about. C. was here in the group for her husband J. and had posted when he passed, looking for support. I had been digging deeply into his medical records for her, explaining what I could about what they showed to help her family understand better what happened to J.. They left behind a very amazing 21-year-old son, C. who I spoke with by phone today. He is determined to prevent deaths from AI and will hopefully soon be in our group to help others.
We also briefly had a member over the last month named A. who was in the group with his husband, C. (they both had left the ADSG for another group). A. died from what we understand was complications of AIDS, which he was open about. Per a long conversation with A., he was not clearly AI-diagnosed but was in our group to learn more about his high steroid doses and related symptoms.
We also know that one of our own past admins, J., had recently gone into adrenal crisis and was found unresponsive, ending up in ICU and is still fighting back in rehab, but hanging in strong and hopefully will make a full recovery. We’ve seen too many times when someone goes into adrenal crisis and is not treated emergently with stress doses and/or the Solu-Cortef emergency injection, that by the time they get to an ER or A&E they may have lapsed into seizures, coma… and when death does not occur, they often end up with permanent brain injury and disability. We need to also prevent this, which has affected several kids/teenagers and adults in our group as well. We wish J. ongoing luck and send our wishes for a full recovery. We are always here for you!
We send our collective love, support and heartfelt condolences to each of the families who have lost loved ones and hope we really can make something positive come from each loss of life we learn about in the group. We will continue to be here for each of these families in any way we can and wish them all the very best. Following is the post I mentioned from last December. It’s still very relevant and I hope you’ll take the time to read through it entirely, as it may save your life or that of another.
IF YOU ARE A PARENT WITH A CHILD OR TEEN WITH AI, PRINT AND READ THIS (this also applies to ALL of us):
For all our parents in the group, and in light of the recent and tragic fight and then loss of little 6-year-old M., the son of member A.S. , a very dedicated father who joined us in November 2015 after his son was diagnosed, I want to try to address the “what” and “how” of parents handling AI with their children. I’ve been getting many questions about this the past two days by PM but know I can help more people by posting, so although this will be very long, I promise it to be informative and crucial for all our parents to know. For our adults, nearly everything herein will apply to you also, so please take the time to read on. (For those who don’t know me, I founded the ADSG but have not acted in an admin capacity since May 2015, although I continue to work behind the scenes daily.) I will not dance around words in this post, as I’d rather err on the side of saving lives than padding what I’m saying with fluff, so I apologize ahead of time if any of this may sound frightening. But we all need to know this and we need to save lives as a result. M. deserves that from us and I’ve promised his dad I would personally ensure his son’s life with AI made a difference.
First, I want to emphasize that nothing I’m going to say is in any way meant to be or sound like ‘blaming’ for any of you, so please only hear this information for what it is: Information, to try to educate our members further on these key issues. Even if something horrible happens to someone’s child (or loved one of any age, or even ourselves), it’s nobody’s fault; sometimes things are beyond our control. And even if we “could” have done something we didn’t know to do or didn’t do, we all do the best we can with what we have or know at the time. We have to accept that and be gentle with ourselves when the outcome isn’t what we hoped. When dealing with AI, it’s important we all know the basics and be our own best advocates, even if that means knowing more than our doctors and then becoming their guide in helping us or our loved ones. I’m going to try to cover this here as a primer to AI for those who have a child newly diagnosed as well as for those who may just not have come across this information before. I’m as always going to include too much rather than too little information, so please just digest this as you can and ask questions if you have them.
We’ve had several deaths just this past year or so in the group involving children or young teens (as well as adults) and I always get asked what could have been done differently or how this can be prevented. This is being written to educate and even save others, and to take one step toward M.’s life having made a difference beyond what he could have possibly imagined at his young age. What I’m addressing here is to assist those with children, teens or even for themselves or an adult love one, although this will be written and geared toward our parents of children with AI. While there are no definitive answers and sometimes a loss of a child is just not in our control, I’d like to offer the following as things that each of you should ensure is done for your child (or yourself), and if you can’t get your doctor or child’s school to accommodate what you know is needed, speak up: Go higher if needed, find a more specialized endocrinologist, but don’t back down. It truly can be a matter of life and death. AI is a clearly life-threatening but overall manageable and treatable disease.
First, know your child’s diagnosis. If they are primary AI (PAI) vs secondary AI (SAI) vs CAH, it matters. It makes a difference in which steroids they will need, it may impact lifestyle differently, and it changes the follow-up labs that are needed. If you don’t know the difference between these diagnoses, which meds are needed and why, or what follow-up labs your child MUST be having (as well as which ones are useless once diagnosed and why) you need to become educated. Understand the results and help your child to understand the results and what’s going on in their body. It will make the journey less scary for both of you and knowledge is power. We can help you with language and tools that will assist with this if you get stuck.
Along with that, you need to know how to calculate your child’s “ideal” daily dose, which will change as they grow and mature. (It can change even for adults over time based on body weight and activity, other diagnoses, lifestyle, etc.) Then know how to dose throughout the day to mimic a natural cortisol rhythm, which is what their body would be doing if it were working properly. We are trying to replicate natural cortisol production with synthetic steroids, and in the case of treating PAI, also to replace the missing aldosterone hormone, a mineral steroid, which regulates sodium, potassium and fluid balance. We have a document on each of these topics that explains them well and fully. Don’t let your doctor say that dosing twice daily with hydrocortisone is adequate; it’s not and can’t fully cover 24 hours of cortisol replacement needs.
You need to know when to stress dose and why and to help your child learn this and to “listen to their body,” which is the #1 golden rule of AI. It will come over time from experience– and with your help and guidance as a parent. Keeping a journal of symptoms and medication doses, and even a food log, will help you to understand better what they may be experiencing and at what times and to differentiate this from other ‘kid’ behavior. There are ways to adjust steroids for this in most cases, but always work with your child’s endocrinologist to make changes. It’s often a tricky line to walk for parents.
Get to know your child’s symptoms. Low cortisol symptoms usually come in clusters, so if there’s only one thing, such as a headache, it may ‘just’ be a headache; if that headache comes with nausea and a rapid heart rate or internal shakes, it may be telling you it’s AI. Review our complete list of low cortisol symptoms in our files, as these vary from time-to-time and person-to-person… so don’t let your doctor invalidate your child’s own experience. This can be a long journey even for an adult. This means knowing your child’s low cortisol symptoms as well as knowing the difference between low cortisol and actual adrenal crisis. Know what brings on symptoms in YOUR child and don’t let a doctor or others tell you there’s a textbook answer to anything, whether that’s dose-related or about his or her symptoms and experience. The key to successfully managing AI is to control symptoms with steroids and lifestyle changes while not over-supplementing (your child can feel just as badly on too much daily steroid replacement as on too little, although the latter is what becomes life-threatening). For hydrocortisone there is no lab that can calculate the needed dose; for fludrocortisone, your child’s symptoms, renin and CMP will determine what’s needed.
If you’re ever unsure, err on the side of safety and give your child a small stress dose, usually about 5 mg HC (10 mg for older kids), and assuming their symptoms are not appearing critical, such as confusion, anything involving breathing or dizziness etc.— wait about 30-40 minutes and see if it helps. You can repeat this if it doesn’t. If at that point the extra doses haven’t made a difference, it’s possible that the issue is not low cortisol but something else that may require medical attention. If your child is sick and has a fever or infection, most likely they will be instructed to double or even triple their daily doses until the issue passes. If you’re ever in doubt and oral stress doses aren’t helping and your child seems to be going downhill, inject your child with IM Solu-Cortef and THEN get to emergency care (this is covered in more detail below). Over time, you and your child will recognize and differentiate their low cortisol symptoms from other issues and you’ll both know when and how much to stress dose. It’s important to remember that both emotional and physical stressors tax our cortisol reserve the same— so if you child is experiencing a difficult emotional time, they may also experience low cortisol and need a stress dose. This also will become more clear with time and experience and knowing your child’s disease.
You must pay attention to even low grade fevers, as anything over 99.5F (37.5C) requires stress dosing, as our body temperatures look lower when taking steroids than they really are (and our white blood count and esinophils may be slightly elevated without having infection, making it harder to know when to treat something with antibiotics). Although children’s fevers tend to run higher than adults, it’s always going to be safer to stress dose than not if you’re unsure. Diarrhea and vomiting can quickly become VERY dangerous, so please pay extra attention if your child is sick. While a routine cold should be manageable without extra steroids, one with fever or anything involving infection means a “healthy” body would produce extra cortisol, so you’d need your child to have extra hydrocortisone on board.
If you find that he or she is stress dosing often for symptoms, this likely means their overall dose isn’t enough or that their timing of doses isn’t meeting their body’s needs. It can also mean that they are not absorbing or metabolizing their steroids properly and may need a different form, such as injections or a pump to deliver this (we also know that some generics such as Q. contain ingredients that for many with AI make the person sicker; we also have more on this in our files). The latter can be checked via an inpatient day curve, which is one of the only exceptions after diagnosis where checking cortisol matters. In all other cases, never let our doctor tell you that your child’s cortisol is okay based on a lab (or that it’s low); this is fully explained in a files document as well and also applies to testing ACTH after diagnosis.
Your child MUST, must, must have an emergency injectable kit on him or her at ALL times. This is often the one thing that will save their life. If your doctor doesn’t understand why this is needed or thinks you’ll reach emergency care in time, assure him/her that that’s often not the case and help them to learn so they can prescribe this for you. Do not take “no” for an answer regarding an emergency injectable kit. There is no replacement option for this in many cases and it is critical to you and your child managing their disease. If the person is not readily near a hospital (on a plane, train, in a car, on a walk, etc.), or just plain gets really sick, vomits repeatedly and can’t hold in oral stress doses, it’s crucial they receive the intramuscular Solu-Cortef injectable immediately (in the U.S. the Act-O-Vial is the easiest to use). This cannot wait until help arrives or until someone drives them to an ER or A&E. It’s the same basic steroid they are on daily, but will get in faster and in a larger dose and may save their life. It’s inexpensive and EVERYONE with AI needs AT LEAST two vials and four CORRECT IM syringes and needles with them at ALL times. Notice the big capital letters. The same goes for oral stress doses. If you don’t have it with you you can’t use it when needed and emergencies with unpredictable consequences happen to us with some frequency. We have information for your doctor if they need help understanding the need for this, but do not back down if your child does not have this both at school, at home, and somehow on his or her person if away at a friend’s house or while at after-school activities, etc. We can help you with your school plan if needed also, but this must be in place 24/7. It is ALWAYS safer to stress dose or even inject than to not if you’re unsure, and over time you and your child will recognize the signs of needing this better, but always err on the side of using it vs waiting too long if unsure, and know the differences I mentioned between low cortisol and crisis. An occasional extra dose won’t hurt the person with AI if you’ve guessed wrong, but not having enough cortisol replacement if their body crucially needs it when placed in serious “flight or fight mode” can lead to adrenal crisis and even coma or death.
Similarly, your child MUST be wearing a medical alert bracelet always. If they don’t have one for financial reasons, contact ME. I run a program for the ADSG where we can buy medical alerts for ALL of our members with true financial need. Don’t let money be what stops this from being on your child at all times (the same goes for any adult in our group). You can also contact any admin if you’d like— but they will just refer you back to me, and please do not wait. It can’t help you if it’s not on your child’s wrist. We want to ensure EVERY member here (or their child) has a good quality, properly engraved medical alert. Get in touch if you need this help from our Medical Alert Assistance Fund and please don’t be bashful or feel badly about reaching out. Put safety over pride if you can’t afford this. We want to keep everyone in our group (or their child) with AI safe and alive and we can buy this for you.
Nobody with AI should be going into actual adrenal crisis with any frequency, as it’s almost always preventable with proper dosing, stress dosing, follow-up labs and care and injecting if needed. If someone does inject, they must always get to emergency care afterward for follow-up fluids and steroids, as it will get in faster but will not last longer than the oral medication. If your child is possibly going into actual adrenal crisis, or you’re unsure, always inject BEFORE going to or calling for emergency help. This can also mean the difference between life and death, as we’ve seen many times in the group. The exceptions would be when the crisis stems from surgery, serious illness or severe injury, in which case large doses such as in our injectables are nearly almost always needed and should be given prophylactically to prevent crisis symptoms. We have guidelines for dental and surgical care and stress dosing also in our files.
Lastly, ensure your child has the correct follow-up labs and care. They should be seen initially every three months or sooner if symptomatic. Once they are stable, they can be seen about every six months, but it should not wait longer than that. If they are diagnosed SAI they should have a CMP (complete metabolic panel) and thyroid labs done and if PAI, also thyroid antibodies if not previously checked. Also if PAI, they need to have renin checked along with their CMP and thyroid levels (TSH, T3, T4). Sex hormones, both androgens and estrogens, in both boys and girls, may also shed some light on changes you may see. Many of the normal changes in sex hormones in AI disease are able to be treated with supplements, but never add or change anything without checking with your child’s endocrinologist, who hopefully is specialized or at least able to say, “I don’t know” and find out for you.
This is all VERY important. I can’t stress that enough. Empowering your child to be in control of their disease and its treatment as their age and maturity allows will go a long way over time also. And being empowered yourself as a parent will ensure you have all the tools possible to help your child live a normal life and avoid common AI pitfalls. Early on you’ll be learning together, so keep communication open and let them know that it does get easier over time. Many of us, myself included, took a good year to get things figured out and many of us, myself included, have had various diagnoses since childhood, so just know and reassure your child that they are not alone. We also have a parent/child resources section in our files, which may help both of you. If you need further help understanding anything I’ve mentioned here, read through our files documents or tag an admin if you can’t find answers, but please look there first. We’ve spent years writing and assembling this for you and pretty much every answer is there for your reference (our files are currently undergoing some revisions but you can still find everything I’ve mentioned). We will do our best to always ensure you have accurate and correct information, but remember there are only a few admins (who also have very complex medical lives as do I) to almost 5,500 members and growing [7,000 as of this repost], so the more you can find on your own in our files or through posting, the more available their time and mine is to the group overall. I will also link you to an awesome video shared with us by member Jennifer Knapp, executive director of Adrenal Insufficiency United, that you would likely benefit from watching: https://youtu.be/uFw53YQIMQc.
As the group continues to support A. and his family [and all these families and individuals], let’s all do what we can to help make something good come from this for M. and all the other kids with AI who struggle each day. Become as educated as you can and help your child’s endocrinologist to best help you. And know that our group is there for you all in any way we can be to help with information and support.