Changing care (for Addison patients)

19 10 2008

This post is inspired by the theme for this weeks Grand Rounds at PalliMed, a Hospice and Palliative Medicine Blog: “Changing Goals of Care”. According to Christian Sinclair, M.D. of Pallimed:

It can be changing the goals in any direction, not just the curative towards palliative route, although I expect that is a common touchstone for many in the medical field.

‘Goals of Care’ is a subject that is outside of my area of professional expertise, being a medical biologist and an information specialist.

But as a consumer and patient I can easily see how I would like health care to change.

  • affordable healthcare for everyone who needs it
  • More personal and personalized care
  • And -indeed- more attention for palliative healthcare (my mother in law has a bearable life, since low doses morphine were prescribed)

But those issues can be better addressed by persons in the field. I just simply want to restrict to “changing care in a very specific area, adrenal diseases, simply because I’m a hands-on expert, having secondary Addison’s Disease (Sheehan’s syndrome)”.

Main conclusions:
Healthcarefivers look (and act) beyond your specialty! Try to be a good generalist as well. Please adapt protocols if it suits the patients. Take the patient seriously.

Diagnosis
Primary Addison (damage or destruction of the adrenal cortex) as well as secondary Addison (absent pituitary signal(s)) often have a slow onset and are difficult to diagnose.
In theory this may be different for Sheehan’s Syndrome. According to Google Knol:

Sheehan’s syndrome (…) is a condition in which the pituitary gland is injured as result of heavy blood loss during complicated childbirth. This heavy loss of blood deprives the pituitary gland of oxygen and other nutrients and leads to necrosis (death) of pituitary tissue and therefore pituitary failure (hypopituitarism). Failure to produce breast milk after delivery (due to lack of the pituitary hormone prolactin) may be a presenting sign of Sheehan’s syndrome. Fortunately, Sheehan’s syndrome is now rare cause of pituitary failure, particularly in developed countries as a result of improved obstetric care.

Looking back I’m stunned that Sheehan was not directly diagnozed by the gynecologists themselves.
And perhaps even more surprised why it happened to me in the first place, being hospitalized in Europe, and having a previous cesarean. (For good reason it is: “Once a cesarean, always a cesarean” According to present protocols I had many negative predictors for success (no prior vaginal birth, short stature, age >40, induction of labor, gestational age almost 43 weeks, failed second stage), but worst of all they didn’t take me serious when I said I didn’t feel well and got a sudden neck pain. When standing up I fainted. So I have every reason to believe all this could have been prevented).

I lost more than 3 litres of blood (and had puerperal fever as well), developing all signs of Sheehan (and Addison crisis) in the days that followed: breast milk “disappearing”, loss of appetite, severe muscle pain, fatigue, headache, lethargy, extreme nausea, diarrhea & vomiting and finally speaking with double tongue, feeling like I fell when lying down, sensitive to cold etc. But nurses pressed to try to give breastmilk (till bleeding), reprimanded me in presence of other patients (you have to break the circle, please do your best (!) and eat something; you have to take care of your child, come on!) and a psychiatrist was being ordered. Finally (after 10 days), when I plead them to check whether I was not dehydrated, they did some tests and found out my blood Natrium was dangerously low (106; normal 140), and could apparently not be corrected by giving saline transfusion. I “missed’ this part, but when I woke up the internist told me proudly he found out I had Sheehan (practically no cortisol or any other hormones under regulation of the anterior hypophysis). Normal natrium levels were achieved after giving cortisol-replacement.

I’m by no means an exception. Addison’s disease is often missed or diagnosed late. That early diagnosis can be a challenge is frequently addressed in the medical literature and many poignant examples can be read on patient forums. In fact I know very few prompt and swift diagnoses.

For instance (from the Newsletter of “The Canadian Addison Society”, issue 27, 2002

After being admitted and discharged what seemed to feel like every weekend, I was finally admitted for bronchitis that affected my asthma. I went on Prednisone* to treat the infection. I felt much better to my surprise. After being “cured” of bronchitis, back in the hospital I went. The pain was unbearable; doctors were questioning if I was anorexic, I saw a psychiatrist who put me on Paxil because I “appeared” to be depressed. Demerol became my new best friend and was the only thing that put me at ease.
My mother continued to stay by my side the entire time. Whether it be stroking my hand, brushing my hair, or encouraging me to walk just a few steps a day. This felt like a marathon to me; in reality it was only a few steps.
After every “possible” test was completed my internist had suggested performing one more test. The results had come back positive! Addison’s Disease….**

(*Prednisone is a glucocorticosteroid that can replace cortisol; this patient also had pigmented handpalms, specific for primary Addison.)

well-ville.com/images/adrenalQA2.jpg

The same is true for other adrenal diseases. Cushing’s Disease (excess of cortisol) is often mistaken for (manic) depression. See for instance wrongdiagnosis.com or here (Dutch).

After years of non-recognized Cushing one of my fellow patients was treated by many specialists. One expert (being an orthopedic, I believe) totally missed the Cushing, because she fixated on other causes of the severe osteoporosis and didn’t notice the patient’s bruises, mania, belly fat, striae to name just a few other symptoms, typical for Cushing. Missing her diagnosis means she is mostly in a wheel chair now, and not able to do the things she liked to do (for those interested and able to read Dutch she has written a book about it: “Aftakelen and Ophijsen”)

Action (in case of a crisis)
With hormone replacement therapy, most Addison patients disease are able to lead normal lives. However extreme stress can precipitate an Addison crises, which is a medical emergency. Patients therefore often wear alert bracelets or necklaces, so that emergency personnel can identify them as having adrenal insufficiency and provide stress doses of steroids in the event of trauma, surgery, or hospitalization.

Luckily I don’t seem very vulnerable to crises (still producing aldosterone), but the one time I had something like it (presumably due wrong capsules, thus more insidious), family physicians reacted inadequatly. One gave me a lab form emphasizing twice that lab tests should ONLY be done when I was really, really ill. Very stupid, because determining Natrium costs nothing compared to hospitalization, and my pride prevented me taking the test, afraid that I made a fool of myself. My own physician said a few weeks later that I should consult a endocrinologist, because he found Addison “much too difficult”. I thought that wasn’t bad, but my endocrinologist didn’t agree, because “he would have been too late in case of a real emergency”. (I had a Na of 123, but was hospitalized, because my endo (a wonderful female doctor) found I behaved differently and wasn’t ok – I also lost >18 pounds in 2 months)

But there are far more upsetting stories of other Addison crises. Even in this era there are unnecessary deaths due to  inadequate intervention. What is also worrying is that paramedics often miss the alert bracelets. A Dutch paramedic wrote on the bulletin board of our patient’s association, that paramedics don’t even look at it, because they aren’t allowed to do anything going beyond first aid and stabilization. However, if my husband may give me an intramuscular injection of corticosteroids, why can’t a paramedic? It is the most essential emergency measure that can and should be taken. He advised that we would bundle our forces with other patient groups to change the protocols of the ambulance personnel. Paramedics won’t do anything when they are not legally entitled to.

I also hear from many Addison patients that it takes ages before there is adequate action. Apparantly routine tests have to be performed first. A nurse even told me that glucose is tested first, because it is such an easy and fast test. O.k. an addison crisis is often accompanied by low blood glucose. So what? Get those corticosteroids in!!! Intravenous injection is often difficult, because of the low blood pressure. It often takes too long and often fails, at least that is what I hear from other patients.

Iatrogenic Cushing and Addison

Apart from natural causes, Cushing and Addison’s disease can have a iatrogenic cause (unintended harmful effects by a physician’s activity, manner, or therapy). It is well known that longlasting treatment and/or high doses of corticosteroids can give Cushing-like symptoms as well as Addison-crises in case of sudden withdrawal (because of feedback mechanisms the body can’t make cortisol any longer).
Laurens Mijnders has developed long lasting Addison’s Disease because of his asthma treatment. His letter in Contrastma, a paper of a Dutch Asthma Foundation (Astma fonds) evoked many responses of patients who had used high doses corticosteroids (up to 50 mg/day Prednison per day). The reactions showed that doctors had given little or no information about adverse effects of corticosteroids and had never warned against a possible Addison crisis (see here).
An endocrinologist revealed at a meeting that they still regularly see Addison crises in patients who received high-dose steroids for their asthma, rheuma, dermatologic or other inflammatory condition
Of course some of these diseases can only be controlled by corticosteroids, but the treating physician should try to sail safely between Scylla and Charybdis, and prepare the patient for any (anticipated) danger.

Wasn’t it: “Primum non nocere” (Latin for “First, do no harm”)?!

Thus physicians, look beyond the border of your specialty and always take patients seriously, please?

Addison's disease info (nvacp)
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12 responses

22 10 2008
Robin

Excellent post! I saw this by way of Dr. Shock’s blog. I am a Cushing’s Disease survivor. Many of us who have surgery also deal with adrenal insufficiency. And I was misdiagnosed (or undiagnosed, really) for well over 20 years with Cushing’s. You make some great points. Thank you.

4 11 2008
Dutch Grand Round: Grote Visite | Dr Shock MD PhD

[…] MedLibLog has two important posts about Addison’s Disease. The first is about the trouble diagnosing Addison’s disease, the second post is about the importance of early intervention in Addisonian Crisis. Her own […]

4 11 2008
Election Day at Grand Rounds 5.7 « Laika’s MedLibLog

[…] The latter would also have fit with a previous post on Addison’s Disease showing that part of the Addison Diseases are iatrogenic,… […]

17 08 2009
Invisible Chronic Illness: Addison’s Disease « Laika’s MedLibLog

[…] an injury to the pituitary gland as result of heavy blood loss during complicated childbirth (see previous post). The week between the cause and the diagnosis of the disease, was the most terrible week of my […]

8 03 2010
Janet Gale

agree totally with your comments. Have just been diagnosed sheehans after pph in 1987. 23 years of hell. Feel as if ive been tortured. Symptoms have been horrendous. Drs thought it psychological despite classic history and symptoms.

24 01 2010
AMY LAUREN METTER

MY 11 AUTOIMMUNE DISEASES AND THE LACK OF COHESIVE CARE THAT I HAD TO CHANNEL MY INNER ERIN BROKOVITCH IN ORDER TO SAVE MY LIFE!
WORST OF ALL OFFENDERS WAS MY GP WHO AFTER GETTING MY BLOOD WORK
(HIGHLY SENSITIVE CRP WAS 8.1 LOW FREE T’3, PREMATURE OVARIAN FAILURE AND HORMONAL IMBALANCE, POSITIVE ANA AND HIGH SED RATE)
TOLD ME I WAS DOING MUCH BETTER AND SHOULD BE FEELING VERY WELL!!!!
I SPENT THE ENTIRE SUMMER IN BED FEELING AS IF I WOULD DIE IF I DID TO MUCH!!! I ALSO SPENT THE WHOLE SUMMER ANGRY AT MYSELF FOR BEING SUCH A WIMP!!!! IN OCTOBER MY AM CORTISOL LEVEL WAS 3.893!

17 04 2010
Irreversible Effects of Previous Cortisol Excess on Cognitive Functions in Cushing’s Disease « Laika’s MedLibLog

[…] of ex-Cushing patients with longlasting if not irreversible health) problems in my previous post here. [added 2010-04-17) To realize how this affects daily life, I recommend to read the photo-blog 365 […]

21 04 2010
Grace RN NJ

At the tender age of 58 I have finally been diagnosed with Sheehan’s Syndrome, despite being symptomatic for the past 29 years. I was diagnosed with severe adrenal insufficiency July 2009 at a local university-based hospital in the US, then poorly medicated. Endocrinologist #3 recognized that I have had untreated hypothyroidism and started Synthyoid-2 weeks ago! Despite being a nurse and providing a full patient history-including amniotic fluid embolus and DIC in 1980 requiring 14 or so units of blood and blood products, no healthcare provider even would consider a diagnosis of Sheehan’s, saying it would have been picked up soon after birth. I’m here to tell you it can take 30 years to get diagnosed!!

So despite being unable to lactate and profound, debilitating exhaustion that progressed to frequent near-syncopal episodes (passing out) no one even would test me for adrenal or thyroid disease. When one is this tired, pursing doctor after doctor just uses up too much energy.

In the US, may I highly recommend the Massachuttess General Hospital Neuroendocrine Study Center in Boston. I was seen there a week ago and diagnosed quickly with additional lab work showing a DHEA deficiency and low free T4. I am to return in a month for a growth hormone stimulation test, the only true way to diagnosis adult onset growth hormone deficiency which is common in Sheehan’s Syndrome.

23 06 2012
carrie

“pursuing doctors after doctor just uses up to much energy”, amen this is so true. I felt so sick I could barely move and then I would push myself only to get there and be treated like I was a hypochondriac… It became the saddest part of my life… and I still am sick. I find that every time I get a new doctor they seem to want to challenge the diagnosis all over again,., Its a tough road to trave..

23 06 2012
carrie

“pursuing doctor after doctor just uses up to much energy”, amen, this is so true. I felt so sick I could barely move and then I would push myself only to get there and be treated like I was a hypochondriac… It became the saddest part of my life… and I’m still sick. I find that every time I get a new doctor they seem to want to challenge the diagnosis all over again,., Its a tough road to travel..

Repl

5 07 2012
diane

Is it possible to have cushing’s one point in your life and addison’s at another point?

24 04 2014
Irreversible Effects of Previous Cortisol Excess on Cognitive Functions in Cushing’s Disease | Laika’s MedLibLog | CushieBlog

[…] of ex-Cushing patients with longlasting if not irreversible health) problems in my previous post here. [added […]

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