DISEASE EQUALITY- DIABETICS AND ADRENAL PATIENTS BOTH DESERVE ACCESS TO INFUSION PUMPS

DISCLAIMER- This post should not be used to diagnose, treat or manage any condition. Management of adrenal disease is serious and requires medication that is managed by a reputable endocrinologist. There is no cure for adrenal disease and this post is not suggesting this treatment is a cure, only a management tool.

Adrenal Insufficiency and Diabetes are both difficult diseases to manage and this Foundation sympathizes with T1D and T2D patients. Both are serious, endocrine disorders and we are in no way downplaying the battle of diabetic patients, we are simply using it as a comparative tool to explain adrenal disease, as most people are unaware of it.

Adrenal insufficiency is a disease where the adrenal glands fail to produce the proper amounts of steroid hormones. There are many different forms of adrenal disease, but the treatment for all forms is the same- steroids for cortisol replacement.

Type 1 Diabetes is the disease where the pancreas fails to produce the correct amount of insulin, thus rendering someone insulin dependent.

Both of these diseases are endocrine disorders.

Both of these diseases require life-long replacement therapy.

The adrenal insufficient person is dependent on cortisol.

The diabetic is dependent on insulin.

Both of these diseases are life threatening.

Both of these diseases require daily monitoring.

Almost every cell in the body has cortisol receptors, making it a crucial hormone. This hormone impacts multiple functions of the body. Without adequate levels of cortisol, the body will go into an adrenal crisis which will result in organ failure and eventually death.

Cortisol impacts blood sugar levels, metabolism, stress response, inflammation levels, aids in the immune system, affects the metabolic processes such as the salt and water balances within the body and it also greatly impacts the circadian rhythm.

Unlike diabetic patients, adrenal disease sufferers have no meter to check their cortisol levels. They must be constantly vigilant of their own personal signs and symptoms of low cortisol. and require an emergency injection if their levels drop too low.

The standard treatment for all adrenal disease patients is daily cortisol replacement medication- steroids.  Medications such as prednisone, dexamethasone or hydrocortisone are prescribed to replace the deficits of steroid hormone in the body. Steroids have a myriad of side effects ranging from weight gain to emotional disturbances. Long term steroid use has been linked to damage of the bones, eyesight and even muscle tissue.  Yet, this is the only treatment for adrenal disease.

In a normal person, during situations of emotional or physical stress their body releases more cortisol. The excitement from a happy event, the sadness from a death of a loved one or the strain from exercising are examples of things that would cause the body to release more cortisol.

In an adrenal insufficient person, this does not happen. They have to artificially manage their cortisol with pills. Their personal cortisol needs may differ from day to day. No two days are the same and it is a struggle to regulate proper cortisol levels.

The most commonly prescribed steroid for adrenal insufficiency is hydrocortisone (HC). This is the bio-identical steroid medication. This medication has a blood serum half life of 90 minutes and must be taken multiple times a day.

Most adrenal patients struggle with quality of life due to this mismanagement. Oral HC must be processed through the stomach and the liver before reaching the blood stream.  This causes a constant rise and fall of cortisol levels, which results in subpar function.

Below is what the natural circadian cortisol rhythm is supposed to look like-

naturalCircadian

This is the circadian rhythm of an adrenal patient on HC.

UnnaturalCircadian

(This image was created using Clearly Alive‘s Steroid Dose Plotter)

Notice how it is a constant up and down motion? This causes fatigue, headaches, irritability, blood sugar issues and lowers quality of life.

The quality of life is vastly poor due to this lack of balance. Oral HC cannot do what natural cortisol can.

But there is solution to help create a more natural circadian rhythm!

The use of solu-cortef (inject-able version of cortisol when mixed with saline) used in an insulin pump programmed to disperse cortisol according to the natural circadian rhythm by programming rates of delivery into the pump.

This therapy bypasses the gastric passage and is able to deliver cortisol in a more natural way. With a pump, an adrenal insufficient patient can receive a constant supply of cortisol and will not suffer the ups and downs with oral HC replacement. Side effects due to mal-absorption are decreased and patients have been reported to have improved sleep, weight management and experience an overall improvement in their energy levels and sense of well-being.

This therapy can literally give adrenal patients their lives back, and yet so many are unable to receive this treatment.

Professor Hindmarsh, is a pioneer for this life changing treatment. He has done much international research and has proven that every single patient he has placed on this therapy found improvement. Yes, 100% success rate! That is unheard of in the rare disease community.  Granted, this is a small group of patients who’ve been exposed to the pump so as awareness grows that stat may fluctuate.

The pump is not a cure for adrenal disease, but it is an option and a ray of hope for those who are bedridden with no quality of life.

If there is another option for adrenal disease, why is the pump therapy only available to diabetics?

Are adrenal patients not worthy of a better life?

Adrenal patients should have the same rights as diabetic patients. All diseases should have the opportunity to receive the best treatment and care possible.

PumpAware

Adrenal Crisis: Another eye opener into why we need awareness

Personal Experience of an Adrenal Crisis.

July 5th- I was having a lot of weakness in my legs and I felt like I had run 10 miles. I couldn’t catch my breath when I was talking. I started to feel really different, something wasn’t right. Ended up having to use my wheelchair because my legs couldn’t support me. I got really dizzy as my husband was pushing me to the couch, and then I couldn’t breath. I was having a very hard time getting air. 911 was called and they took me to the ER.

The Dr. refused to give me hydrocortisone until he talked to my endocrinologist.

He ordered a chest X-ray and CT scan, bloodwork and urine sample. All those came back great (They were testing for heart attack symptoms), which we told them several times, I was in an adrenal crisis.

Still no hydrocortisone.

They gave me meds to slow my breathing. Once it hit the IV my whole body tensed up, I was shaking, I couldn’t move and I couldn’t breath. I started to fade, the nurses voice and my husbands were in the distance. I was scared because everything was black and they were next to me but sounded like they were so far away.

Then I stopped breathing.

The nurse was yelling at me to breathe and was pounding on my chest with her fingers, she kept telling me this and pounding on me, again I slipped into the black and her voice was far away. She slipped some oxygen on me, finally, and I was brought back. After that everything leveled out.
Since I wasn’t having a “normal” adrenal crisis, they sent me home.

I rested all day but I still couldn’t use my legs, not even enough to use to get myself in the wheelchair. It was really scary. For all of us.

July 6th-I was just exhausted (but that’s normal). I was able to eat without having that horrific pain. (July 3rd and 4th I did Ex-lax and water to see if that would clean up stomach up enough where I could eat without the pain, nausea, and severe bloating).

This was a reminder of the severity of my disease and that I need to really take it easy, not push myself, and let go of the things (and people) that cause a lot of stress in my life.

I’m so proud of the boys for stepping up the way they did. My middle son took my youngest to the bedroom and kept him calm and distracted while the EMTs were here. My oldest held it together, tried to keep me breathing, was telling the EMTs over and over that I needed my shot, what disease I had and that they needed to give it to me. He answered all the questions, because I couldn’t talk, he even corrected my husband about my age.

My husband handled it okay. He was scared and broke down a bit, but my oldest did good at calming him as well. I’m thankful they did amazing at advocating for me. I’m so proud of them. I just hope that doesn’t happen again, because I was terrified.

I can’t tell you how often this happens, people denied hydrocortisone because the doctors don’t know anything about Addison’s disease.

Instead of listening to the patient, they deny them the one thing that will save their life. I get that there are procedures, but most people (like me) are told “no.”

I know of four people in groups I follow on social media (in the year I’ve been apart of it) who have died after going home from the ER because the doctors refused to give them hydrocortisone. They were told they were fine or over reacting and that nothing was wrong with them.

This really angers me that there is no understanding of this disease and doctors don’t take it seriously because they don’t know or don’t understand it.

I want to make my voice heard and I am doing what I can to bring awareness.

-Chronic Mom, Adrenal Alternatives Foundation Contributor

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Recently passed California bill harmful to pediatric adrenal disease sufferers

 

The SCR- 110 Sex Characteristics Bill is a bill California presented that was amended in the Senate on May 9 2018.

The context of the bill is coming from the wake of the gender queer and transgender issue. This bill prevents parents from making a decision on whether a child should be deemed male or female In those born with ambiguous genitalia.

Where this is concerning is there are genetic deformities and congenital diseases that are negatively impacted by this bill.

In these specific cases, this bill negatively impacts children born with diseases such as congenital adrenal hyperplasia. CAH is a life threatening disease which can cause children to be born with ambiguous genitalia, hormonal issues and requires life long steroid cortisol replacement medications.

Most pediatric endocrinologists agree that when CAH causes genetic abnormalities or genital mutation, these cases require medical intervention.

The danger of this bill is that these children will not receive the care that they need because of laws passed by politicians NOT educated informed physicians regarding medical care.

The concerning part is when decisions of parents and physicians are taken away by legislators who have no education regarding the dangers of improper medical treatment with diseases such as CAH.

It is understood, the concept of not wanting to cause someone to have gender dysphoria or to be forced to choose a gender; but in this case these specific medical issues need to be addressed.

This bill takes autonomy away from parents and physicians to make that choice.

Children, especially females born with CAH typically have ambiguous genitalia that needs to be corrected. These children can also suffer from hormonal issues such as polycystic ovarian syndrome.

This bill will inhibit the proper care of these children.

The treatment of CAH has nothing to do with gender identification.

Bills like this take the power of medical decisions out of parent’s and medical professional’s hands.

Below is a brief excerpt of the bill so you can fully understand the context.

This bill was amended by Senator Wiener, and is as follows-

This measure among other things called upon stakeholders in the health professions to protect children born with variations of sex characteristics.

Whereas between 1% and 2% of individuals born with variations of sex characteristics; which may include differences in genital anatomy from a hormonal variations or internal reproductive structure: Whereas intersex refers to the rare variety of physical indicators that indicate the differences which occur about the same frequency as do green eyes.

Whereas the majority of babies born with these variations are healthy and do not require medical or other interventions related to sex characteristics until puberty.

Beginning in the 1950’s physicians in the United States began performing irreversible surgery on intersex infants without medical justification and attempt to surgically or hormonally force them to conform to what the  physician perceived as a typical male and female body.

Source- https://leginfo.legislature.ca.gov/faces/billTextClient.xhtml?bill_id=201720180SCR110

So what can we do about it?

The Judiciary Committee phone number is (916) 319-2334.

It is imperative that we have representation from patients, family members and medical professionals to stop this bill.

This is not an attack on the gender-queer or transgender population, this is a protection for children born with a serious, life threatening, incurable disease.

 

This bill states- “California should serve as a model of competent and ethical medical care and has a compelling interest in protecting the physical and psychological well-being of minors.”

 

If that be the case, I urge Senator Wiener, Senator Glazer and Assembly Member Limón to reconsider this bill and amend it to protect children with CAH.

Please call and request an amendment to the SCR-110 Sex Characteristics Bill in favor of children born with CAH.

Do You Think You Can Cure Me?

It’s happened to all of us. Even the non-chronically ill. Someone has suggested a sketchy miracle cure. Maybe they told you eating ‘healthy’ or going organic would have prevented or fixed your condition and will save you from taking your medications?

Why is this so frustrating? Their heart is in the right place is it not? What’s the problem?

When you tell me eating vegetables or a certain trendy food would prevent my condition, it insinuates that my choices have caused my condition and all of the resulting health problems. That if only I had an organic farm and bought a juicer I’d be cancer and ‘pharma’ free.

My cancer is genetic. I could not prevent it, and all of the organic vegetables in the world will not cure it. That’s not really how cancer works. You can’t just throw ‘healthy stuff’ at cancer and make it go away. Individual cancers behave differently and the mechanisms of their growth and death need to be understood on the cellular level for best treatment. No amount of kelp will do that.

Of course, a healthy lifestyle is a benefit to anyone with or without a medical condition, but should not be used in place of treatment. Many of the miracle stories you read about conveniently leave out the part where their “natural miracle cure” also involved some surgery, chemo, and/or radiation. That doesn’t get as many facebook likes now does it? These stories are dangerous misinformation. Please stop sharing them.

Next, can we please stop judging people who have to take medications? This implies I’ve failed some how because my life depends on medication. I would so love to toss all of my pills into the trash. That would be a dream, but guess what? I’d die. I’d suffer greatly, and die shortly thereafter. I cannot trade my medications for coconut oil or anything else on a grocery store shelf (or farm.) Maybe I am a slave to ‘big pharma.’ I like living though. I’ve got a family, so I’ll keep taking my meds and keep living as long as I can. For me, that means meds forever, and I’m okay with that.

I value and protect my health. I do appreciate your concern for me. Cancer isn’t fun, and you want to help. I want there to be a miracle cure too, but rather than cling to false hope, I will keep using treatments that are proven effective. I’d rather not hear any more cures. Instead let’s talk about enjoying life, savoring the moment. That’s the real way you “beat cancer.” One breath, one day at a time.

10 Things I Wish My Endocrinologist Would Have Told Me….

I was diagnosed with Adrenal Insufficiency on March 23, 2016. When I met with my first Endocrinologist, we’ll refer to her as Dr. W, I knew nothing about Adrenal Insufficiency so I took a notebook with me, prepared to take lots of notes. In a nutshell, this is what Dr. W told me…”Take Hydrocortisone twice a day, I’ll see you every 6 months for labs, and you’re going to be able to live a normal life again!” The “2016 me” was beyond ecstatic, after suffering with symptoms for over 8 years. The “2018 me” rolls my eyes and wonders how this doctor ever made it through medical school, let alone becoming a “specialist” in medical care.

If you’re anything like me, I love knowledge! As soon as I was diagnosed, I started spending hours upon hours researching my new diagnosis. I found support groups on Facebook, with people who were members that each had their own Endocrinologists and ranged from being newly diagnosed to having Adrenal Insufficiency for decades. I quickly realized how much Dr. W did not tell me, and after having a couple more appointments with her, I realized that I knew more about my illness than she did. As you can probably guess, I fired Dr. W and found Dr. C, who is very knowledgeable and has an appreciation of the fact that I probably know just as much about Adrenal Insufficiency as she does.

Over the past couple of years, there have been many newly diagnosed people who have joined the support groups on Facebook and have posted questions, desperate for answers, just like I had been at one point. Over time, I have become very frustrated because of the overwhelming number of newly diagnosed people who’s doctors were basically telling them the same thing Dr. W told me, and nothing more. So, I figured I would compile the Top 10 things I wish my doctor would have told me when I was newly diagnosed. I’m not a medical professional, and before you change anything in the care of your Adrenal Insufficiency, you should consult with your doctor.

1.“Taking Hydrocortisone 2 times per day will not be enough!”Hydrocortisone stays in a body anywhere from 4-6 hours at a time. When I took my Hydrocortisone twice a day, I would feel great for about 2 hours each time I took them, but felt horrible the rest of the day. Little did I know, after the Hydrocortisone metabolized out of my body, I had no cortisol in my system, which is why I felt so horrible and continued getting sick. A normal body creates cortisol 24 hours a day, so it would make sense to take the medication that is supposed to replace the lost cortisol, as closely as possible to how a normal body would create it. In order for that to happen, a person needs to take Hydrocortisone between 4 to 6 times per day, depending on how quickly their body metabolizes the Hydrocortisone.

2. “How much and when to take Hydrocortisone matters!”In order to mimic a normal body’s output of cortisol, you have to take Hydrocortisone in a way that will be as close to how the body produces cortisol as possible. How do you do that? You have to follow the Circadian Rhythm dosing schedule. For me, since I metabolize Hydrocortisone every 4 hours, that means I need to take it 6 times a day in order to have 24 hour coverage. Is it a pain in the butt? It sure is….I have to set alarms and even wake up in the middle of the night to take my meds. Is it worth it? It absolutely is! Before I started following the Circadian Rhythm dosing schedule, I just guessed at how much I needed to take every time. I knew that normal bodies had higher amounts of cortisol in the morning and very little at bedtime, so I just took the highest amount when I woke up and tapered down during the day, and took my last dose at bedtime. This schedule not only had me feeling awful when I first woke up and having highs and lows throughout the day, but I was also over replacing my cortisol at times during the day and I had no cortisol in my body at night and early in the morning, when a normal body would have cortisol.

3. “You will have to stress dose!”Do what? Dr. W never told me that there would be times that I would need to take extra Hydrocortisone. In a normal body, anytime the body needs extra cortisol, the adrenal glands produce more instantly. This is not the case for people with Adrenal Insufficiency. At times when we need more, our bodies metabolize the Hydrocortisone faster and are left searching for more. How much extra to take and when to take it really depends on what is currently going on inside of your body. If you are having additional emotional stress, even if it’s good stress, like attending a wedding, you’ll have to take a small amount extra. If you have a stomach bug or an infection, you’ll have to double the amount that you take during the day. And if you’re puking or having surgery or get into an accident, you’ll need 100mg of Solucortef, which is the emergency Hydrocortisone. It’s very important to know what your symptoms of low coritisol are and to listen to your body.

4. “You will need to carry an Emergency Injection with you at all times!”Not only did Dr. W not tell me about Emergency Injections, when I asked her for a prescription for one, she told me I didn’t need it. Make no mistake, not having an Emergency Injection on you at all times can be deadly! I didn’t realize just how important having one at all times was until I had an Adrenal Crisis, wasn’t breathing, and ended up “dying” three times in one night. Where was my Emergency Injection? Sitting at the pharmacy because I hadn’t gotten around to picking it up. I have had a few times where I’ve gone into an Adrenal Crisis and wasn’t breathing, and after my husband injected me with the Emergency Injection, I started breathing within a minute or two. Dr. C makes sure that I can have several on hand at a time, with plenty of refills. She also advised me to inject as soon as I cannot keep my oral meds down if I am puking.

5. “You will gain weight!”Say it isn’t so! I have always been a very thin person. I could eat whatever I wanted and never had a weight problem. At my second appointment with Dr. W, I was horrified when I had gained 15 pounds. Dr. W told me not to worry about it because it was just the Hydrocortisone getting my body metabolized, and that I shouldn’t gain anymore. Now, I look back at those 15 pounds and how horrified I was, and only wish that I could go back to that weight. I kept gaining weight and had no idea why. I rarely eat sugary foods and I hadn’t changed my eating habits to support the weight gain. I saw many posts on the support groups of people who had gained weight, so I thought it was just something that happened and couldn’t be controlled. Finally, one of the Admins of one of the support groups on Facebook wrote a post about why people gain weight on steroids. Basically, she said that carbs instantly turn into insulin, so our bodies have a huge rush of insulin every time we eat carbs, and that turns into fat. Seriously? I love carbs! Give me bread and pasta and I’m a happy girl. Well, now I’m a chubby, happy girl. After doing some research, I found that following the Ketogenic way of eating was one of the only options for losing weight while on steroids. When I follow that way of eating, I do lose weight, but when I give into my carb cravings, I start gaining again. So, carbs and I had to break up.

6.“You’ll need to take a Calcium supplement and other supplements!”I have a good friend of mine that is a doctor. After I was diagnosed, he asked me how much Calcium and Vitamin D my doctor had me on. “None” was not the answer he expected. He told me the importance of taking Calcium and Vitamin D every day, because steroids rob your bones of Calcium and it can lead to Osteoporosis at a very young age. Also, because the Adrenal Glands control so many of your hormones, it’s important to have your other hormones tested to see if you need to be on any other supplements. For me, I have to take a DHEA supplement every day. I didn’t even know what DHEA was until recently. I was feeling sluggish and had to take naps during the day. Dr. C tested my DHEA levels and they were low. Now, I take a DHEA supplement every day and my energy level has improved tremendously.

7. “The days of going to the hospital are not over!”Not only are the days of going to the hospital not over, now that you have been diagnosed with Adrenal Insufficiency, you are going to have to be very knowledgeable and become a strong advocate for yourself for when you do have to go to the hospital. I thought my hospital days were over…I was wrong. I also thought that since I finally had a diagnosis, doctors would treat me with the correct treatment plan and would finally stop treating me like my symptoms were in my head…again, I was wrong. Unfortunately, I’ve been to the hospital many times since being diagnosed. I have found that there are two types of doctors. The doctors who took an 8 hour class in med school that think they know “everything” about Adrenal Insufficiency. And the doctors who admit that they don’t know as much as I do about it and ask me how to appropriately treat me. You will quickly figure out which kind of doctor you’re dealing with, and you need to be prepared to deal with the first type. I have “fired” doctors in the hospital because they refused to listen to me and have actually caused me to go into an Adrenal Crisis, in the hospital, under their care.

8. “Be prepared for an Adrenal Crisis!”Some people with Adrenal Insufficiency can go years without having an Adrenal Crisis. Other people have several a year. Knowing the symptoms of low cortisol can be detrimental to keeping you out of an Adrenal Crisis. Knowing the signs of an Adrenal Crisis will save your life! Always make sure you have your Hydrocortisone and your Emergency Injection with you at all times. Last summer, I was camping and decided to go out on our boat without any meds. I started having low cortisol symptoms, so my husband drove the boat to shore so that I could walk back to our campsite and take my Hydrocortisone. However, I got lost while looking for the campsite. By the time I finally found it, I was already in an Adrenal Crisis, and ended up spending the next two weeks in the hospital because of the severity of the Crisis. Also, making sure you always wear a Medical Alert ID bracelet is essential and making sure your loved ones know what to do when you do go into a Crisis is crucial.

9.“You are not alone!”When I first got diagnosed with Adrenal Insufficiency, I felt very scared and alone. I had no idea that there were thousands of people, from all over the world, just like me, who were living with this disease. Once I found the online support groups on Facebook, I felt a sense of peace. Suddenly, I could find someone to talk to or ask for advice 24 hours a day. Some of my best friends now are people I’ve met online in the support groups. Although I love my friends who do not have Adrenal Insufficiency, they don’t completely understand it, so it’s nice to be able to talk to people who are living with it every day like I am.

10. “This is your new normal!”Based on what Dr. W told me, I expected my life to go back to the way I felt prior to diagnosis and prior to having symptoms. I’m a little hard headed, so I waited over a year before I finally came to the realization that this life was my new normal. I will always have Adrenal Insufficiency, I will always have to take replacement steroids to live, I will always have to be prepared for good days and bad days. The sooner you can accept this, the happier you’ll be. Just remember, you’re stronger than you think you are! You are an Adrenal Insufficiency Warrior!