The Doctors & the Patient.

25 04 2010

Once there was a woman with secondary Addison’s disease. She took her replacement medicine (cortisol, thyrax, growth hormone, DHEAs) as she should and everything was reasonably under control.

However, gradually she began to feel weak. She often disliked food, sometimes to such an extent that she had an urge to vomit. After a month or so, she began to feel dizzy, especially when standing up. Then her heart suddenly banged very quickly against her chest. It felt frightening.

Her work wasn’t going well in this period. Feeling weak and not being able to sleep, she could not concentrate and often felt like she could just cry or layĀ  down on the floor, because she was so extremely, extremely tired.
But it was a busy and important period. She should write a research proposal, finish a paper, and present a poster at a congress. Furthermore her paper was chosen for a press conference, that was recorded live.

Nobody took her serious, no one thought that she might be ill. She did recognize some symptoms that could be manifestations of an Addison crisis (weakness, dislike of food). However there was no reason for a crisis: she took all her medicine and had no flu or fever, thus there was no need to raise the cortisol dose.

Before she went on vacation she just wanted to check with her endocrinologist, but the endo was on vacation too and according to the policy of the hospital she had to be referred by her GP. Her GP also had vacation, so she went to the locum. He reserved his lunch time for her, which was really nice.

Because this doctor wasn’t familiar with the disease, he looked it up in a book and decided to determine whether she had orthostatic hypotension (drop in pressure and dizziness when standing up), because this is typical for an Addison crisis (she learned then). She had to lay down and stand up several times. He failed to demonstrate orthostatic hypotension (but she felt it was taking him too long to measure the blood pressure after standing up; she didn’t say anything though).

Then this GP phoned the attending endocrinologist at the hospital. This was one of the endocrinologists who had been in charge of her during the years. Usually she got a new doctor each year.
From the conversation she could infer that this endocrinologist didn’t think there was really much wrong. Afterwards the GP urged her to take her medicine (as if she didn’t take enough) and to take more if necessary. He gave her a form for blood tests, but urged her only to do the tests when it was REALLY REALLY REALLY necessary…..

She felt bad, like a show-off. She excused herself, she was sorry to have bothered him. She never did the blood test, but took some extra cortisol though.

She went on holidays (why would she stay home?), but from there it only went worse. She couldn’t sleep at all. She had gradually lost up to 8-10 kilos in 2-3 months time (about 15% of her body weight). She was dizzy and nauseous and regularly had migraine .

Back home she went to her own GP. He didn’t examine her, but sighed: “This diseases is too complicated. I think you better see your endocrinologist….”

So she phoned her (own) endocrinologist and she was invited to come after the regular consultations. The doctor did a physical exam and noticed orthostatic hypotension. Blood tests were done, which revealed a low blood sodium (125), not as dramatically low as during her first crisis (106), but still abnormal (<140). The next days the sodium dropped further and the creatinine got outside the normal range, so the endo proposed to hospitalize her, saying: “this is not an emergency, but i don’t trust it, because this is not like you. It is not like you at all. I don’t want to wait for the weekend.”

Her endocrinologist had to convince the internist to make a bed available for admission.

During the intake, the two internists behaved very skeptical. Even though the lab results pointed at a too low cortisol level (later confirmed by the lab). One of them kept repeating that a cup of salty soup would pick her up (lack of salt mainly plays a role in patients with primary Addison’s disease, who also lack aldosterone). He would even say that after she recovered by her cortisol infusion. The recovery was very quick. The other internists said that it was a miraculous metamorphosis.

Well that female patient? That was me, six years ago.

And all doctors were males except the doctor who finally hospitalized me.

Yes of all doctors I had seen as an Addison patient over the years, she was the only woman. Many of the male practitioners were excellent doctors, but none of them would have hospitalized me because his intuition told him “something was not o.k.” Maybe I’m wrong and it is just by chance that this happened to be a female doctor. But somehow I feel that, on average (!) female doctors listen a little bit more to their heart, and male doctors a little bit more to their brains.
It is only intuition, but then I’m a woman. šŸ˜‰
My doctor was not interested in a career as the other doctors, she didn’t like the competition, she didn’t like the hierarchy and she didn’t care for a publication in top journals. Her patients came first.
She has now moved to another (non-academic) hospital and I have a new doctor again. A man, indeed.

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This post is submitted to the upcoming Grand Rounds at Chronic Babe, with the theme Babes:
Your post might be about what it’s like to have women patients. Or to be a woman patient, or a woman who’s a health care provider. It could be about trends in health care for women, or how awesome women are, or how annoying they are. It could be about a particular patient who was very ladylike, or someone who totally wasn’t. It’s your choice. Whatever you send, just please make sure it’s related to the Babes in some way, or else I won’t be including you. This is Grand Rounds, ChronicBabe-style, so it’s Ladies Choice!

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Invisible Chronic Illness: Addison’s Disease

17 08 2009

This week the Grand Round will be hosted by Invisible Illness Week, a blog dedicated to the National InvisibleĀ  Ilness Week, which runs September 14 -20, 2009. The purpose:

National Invisible Chronic Illness Awareness WeekĀ  (..) is a worldwide effort to bring together people who live with invisible chronic illness and those who love them. Organizations are encouraged to educate the general public, churches, healthcare professionals and government officials about the impact of living with a chronic illness that is not visually apparent.

The theme of the Grand Round is, not very surprisingly: Invisible chronic Illness.

I won’t write about this professionally -being a librarian-, but I will speak from my own experience.

As many of you know, I’ve the chronic illness Addison’s Disease. Not that I feel ill. It doesn’t affect me, really… Not anymore.. I think.

But many people with Addison’s disease suffer silently from this disease. And like many other diseases this disease is seldomly understood by partners, colleagues, friends ….. and doctors.

Before I explain more about Addison’s disease, first let me say that almost every disease is “invisible” to others. People can never fully understand what an illness means to someone suffering from it.

Ball-and-stick model of the cortisol (hydrocor...

Cortisol, Image via Wikipedia

Patients with Addison’s disease make no or too small amounts of cortisol, a hormone made by the adrenal cortex. Cortisol has a bad reputation as the stress hormone among many people. It doesn’t deserve this reputation as this hormone is vital to life. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior (Wikipedia)

Too much of this hormone causes Cushing’s disease, too little causes Addison’s disease. If you want to know what Cushing does to your body and mind, then please read the letter of Kate when she was first diagnosed with Cushing’s, at Robin’s “Survive the Journey”.

Here, I will confine myself to Addison’s disease. It is a very good example of an invisible yet serious disease.

There are 3 forms of Addison: primary (defect in the adrenal cortex itself, often also leading to a defect in aldosteron production), secondary Addison (by a defect in the hypophysis or hypothalamus) and iatrogenic Addison (caused by overtreatment with corticosteroids)

Here some reasons why the illness, although “invisible”, can have great impact on your live.

1. Diagnosis.

well-ville.com/images/adrenalQA2.jpg

Diagnosis is often a challenge, especially in patients with primary Addison, most of whom look healthy because of their pigmented skin. Nowadays, the main cause of primary Addison’s disease is immune destruction of the adrenal cortex. This has often a slow onset and in 50% of the patients the diagnosis takes more than 2, sometimes even more than 10 years [1]. 38% of the patients even experience vague complaints, that can later be attributed to Addison, during 11->30 years before diagnosis [1].

Before the diagnosis is made, people with Addison’s Disease often feel extremely tired and miserable. Even when the disease fully manifests itself the symptoms are largely vague and aspecific. The most common symptoms are fatigue, dizziness, muscle weakness, weight loss, difficulty in standing up, vomiting, anxiety, diarrhea, headache, sweating, changes in mood and personality, and joint and muscle pains. Often the symptoms aren’t taken seriously (enough) or the illness is mistaken for anorexia or depression.

My secondary Addison was the consequence of 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 life. I felt awful, weak, (well I lost >3 liters of blood to start with), couldn’t give breast milk (no prolactin), and I disgusted food so much, you can’t imagine. I couldn’t get anything down my throat, only the look of it made me vomit. And I felt so bad not being able to care for the baby, but I just couldn’t. I couldn’t even stand for more then a few minutes, couldn’t walk.Ā  And then there was unstoppable diarrhea, dizzyness, and speaking with double tongue. And practically no one took it seriously, not the gynaecologists, not the nurses, not the paediatricians, nor my friends or family.

But this was only one week. How would it have been if it durated 5 or 10 years?

2. Grieve and adaptation.

Once the disease is diagnosed you have to learn to live with a body that has let you down (grieve) and you have to learn to become confident again (adapt). You also have to find a new balance. I’ve lost a few hormones overnight (ACTH, cortisol, thyroid hormone, growth hormone, prolactin, gonadotrope hormones) and believe me, it took me a few years to feel reasonable normal again. It is quite surprising how badly I was informed. Very little information about the risk of an Addisonian crises, the dosing of cortisol under various conditions.
It was also confronting how little people wanted to know about the disease or what I had been through. Visitors after the birth wanted me to be euphoric and didn’t want me to go into any detail of what had happened. They cut me short by saying: “But you have a lovely baby”. Somebody cried that she didn’t want to hear it. So I stopped trying to speak about it.

I took no sick leave, immediately went back to work. My boss – a nephrologist, never asked after my health, not once.

As I said it took a few years before my “come-back”. I didn’t feel myself. It was as if I couldn’t think, as if my head was filled with cottonwool. Afterwards I think the main reason for improval was the reduction of the cortisol from 30 mg to 12.5 per day and the use of DHEAs plus that I regained confidence in myself.

3. Comorbidity

With cortisol I lost some other hormones which are also essential. Patients with primary Addison often miss aldosteron as well, which makes them more liable for an Addisonian crisis. Primary Addisonians may also have other immune diseases, like autoimmune thyroid disease, gonadal failure, type 1 diabetes and vitiligo.

4. Addisonian crisis

An addisonian crisis is an emergency situation, with possible fatal outcome, associated mainly with an acute deficiency of the glucocorticoid cortisol. This occurs in (extremely) stressful situations. Some Addisonpatients are more prone to it than others. You can -and should – take precautions, like wearing alert bracelets or necklaces, so that emergency personnel can identify adrenal insufficiency and provide stress doses of steroids in the event of trauma, surgery, or hospitalization.

Some Addisonians fear these crises so much that they dear not walk or run alone. Many Addison patients don’t go to a country far away, some don’t even pass the border (and you know the Netherlands aren’t that big).

5. Addison’s disease can be treated but not cured.

Addison patients are treated with corticosteroids like hydrocortisone and are substituted with other hormones that they may lack. Without treatment, the disease is lethal, with treatment the disease is not cured. I do feel all right now, but many of my fellow patients don’t. I think that the following excerpt from a Seminar of Wiebke Arlt and Bruno Allolio about adrenal insufficiency [2] makes this very clear.

Despite adequate glucocorticoid and mineralocorticoid replacement, health-related quality of life is greatly impaired in patients with primary and secondary adrenal insufficiency. Predominant complaints are fatigue, lack of energy, depression, and anxiety. In addition, affected women frequently complain about impaired libido. In a survey of 91 individuals, 50% of patients with primary adrenal insufficiency considered themselves unfit to work and 30% needed household help. In another survey of 88 individuals the number of patients who received disablility pensions was two to three times higher than in the general population. The adverse effect of chronic adrenal insufficiency on health-related quality of life is comparable to that of congestive heart failure. However, fine-tuning of glucocorticoid replacement leaves only a narrow margin for improvement, and changes in timing or dose do not result in improved wellbeing.

References

  1. Zelissen PM. Addison patients in the Netherlands: medical report of the survey. The Hague: Dutch Addison Society, 1994.
  2. Wiebke Arlt, Bruno Allolio. Adrenal Insufficiency, Lancet 2003; 361: 1881ā€“93 , full text on http://www.addisonssupport.com/Documentation/adrenal-insufficiency-2003.pdf

Earlier posts on the subject:

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Election Day at Grand Rounds 5.7

4 11 2008

Grand Round is now up at Nurseratchedsplace of Mother Jones, RN.

This Round, called Election Day at Grand Rounds doesn’t only give a great wrap up of this week’s medical blogposts, but also reviews the health history of former presidents. According To Mother Jones:

“The President of the United States has a few things in common with people like Joe the Plumber. From time to time, everyone gets sick and needs to see a doctor. I remember when George H.W. Bush vomited all over a Japanese Prime Minister during a state dinner. And we all remember the attempt on Ronald Regan’s life. Whatever happens, the president’s physician stands ready to care for their famous patient and members of the First Family.225px-john_f_kennedy_white_house_color_photo_portrait

My blogpost on the importance of early intervention in Addisonian crises is introduced by the story of President J.F. Kennedy who

“started taking steroids to treat colitis. This caused many long-term side effects that haunted him for the rest of his life. Kennedy suffered from Addison disease as well as chronic back pain possibly related to weakened bones caused by steroid use.”

The latter would also have fit with a previous post on Addison’s Disease showing that part of the Addison Diseases are iatrogenic, caused by treatment with high doses of corticosteroids.

For the complete review of all this week’s medical blogs Ć nd of presidential diseases please visit Election Day at Grand Rounds. Great Job, Mother Jones!

Who missed the previous Grand Round, well it passed by with the speed of light. Hurry up. You might catch up if you take the emergiblog speedway here.

Next Week Grand Round will be up at Musings of a Distractible Mind. Here you can find details how to submit your post.







The importance of early intervention in Addisonian crises

27 10 2008

In a previous post entitled ā€œchanging care for addison patientsā€œ (see here), I mentioned that Addisonā€™s disease is often misdiagnosed and Addison crises not adequately dealth with.

ā€œIā€™m by no means an exception. Addisonā€™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.ā€

ā€œā€¦But there are far more upsetting stories of other Addison crises. Even in this era there are unnecessary deaths due to inadequate intervention.ā€

While preparing this post I came across a recent paper in ā€œHet Nederlands Tijdschrift voor Geneeskundeā€ (something like the Dutch Lancet) with a relevant clinical lesson on this very subject. It is entitled:

ā€œAddisonian crisis in patients with known adrenal insufficiency: the importance of early interventionā€, written by Mulder of the group of Professor Hermus from the Universitair Medisch Centrum St Radboud, Nijmegen.

The paper decribes 3 fatal cases of Addisonian crisis in patients with adrenal insufficiency, which formed the basis for the development of a regional protocol to prevent any further unnecessary death from Addisonian crisis (see PubMed abstract here).

The cases

Patient A was a 47 year old male with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Since this leads to deficient glucocorticoid and mineralocorticoid hormone production, replacement therapy consisted of daily replacement with glucocorticoid (hydrocortisone, HC) and mineralocorticoid (fludrocortisone).
A got a sudden gastroenteritis (acute abdominal pain, watery diarrhea, no fever), for which he doubled his HC dose. The next day he became weak and dizzy. The consulted physician didnā€™t deem parenteral cortisol (proposed by the patientā€™s partner) necessary, but prescribed loperamide instead. Indeed the diarrhea improved, but the condition of the patient worsened overnight, his temperature dropped to 34,4 C, he was confused and finally became comatose. Upon arrival at ED the hypotensive patient developed ventricular fibrillation. The neurological sequelae after CPR were so severe that active medical treatment was withheld, after which the patient died.

jmr_photo/2738016554/

The other two patients had panhypopituitarism and adrenal insufficiency secondary to their ACTH deficiency. With respect to replacement of adrenal hormones, these patients only require replacement of (ACTH driven production of) glucocorticoids, not mineralocorticoids. (On the other hand, they need extra replacement of other hypophysis-(regulated) hormones, like levothyroxine, gonadotropins and growth hormone).

Patient B, a 28 year old male got a sore throat and fever (41 C), for which he didnā€™t increase his HC-dose. His mother called a physician in vain: patient B didn’t respond and was found dead two hours later. Obduction showed tonsillitis, bronchopneumonia and an enlarged spleen, indicative of sepsis. This all took place in one and a half day.

Patient C was vomiting and had fever during a couple of days. Soon after her doctor visited her, she suffered a cardiac arrest and died. Her family physician was not familiar with her medical history nor with the prescribed medication. In retrospect, patient C had poor treatment compliance (never came to a consult and didnā€™t take replacement medication, including HC, for a year).

Conclusions

Even patients known to have adrenal insufficiency can develop a life-threatening Addison crisis in case of inadequate adjustment of the glucocorticoid dosage during intercurrent illness. Treatment consists of a high parenteral dose glucocorticoids, preferentially HC (because this also has a mineralocorticoid action).

The chance of hypovolemic shock accompanying a crisis is greater in patients with primary Addison, lacking mineralocorticoids (case A).

Preventive measures

These casualties led to a new protocol. According to the authors:

“Patients with known adrenal insufficiency, as well as their relatives and general practitioners, should repeatedly receive verbal and written instructions on how to deal with physical and severe psychic stress. We teach the patients and their relatives how to use an emergency injection of hydrocortisone, and the patients can consult the on-call endocrinologist by telephone 24 hours a day.”

I. Points to be adressed in the yearly instruction of patients with primary or secondary adrenal insufficiency, preferably in presence of his/her partner or close relative:

  • explain importance of glucocorticoid use.
  • describe the symptoms of an Addisonian crisis
  • give instruction on increasing glucocorticoid dose in case of illness or severe stress
  • stress the importance of an alert bracelet
  • verify whether the patient has an emergency ampule with hydrocortisone (i.e. Solucortef) at home
  • give instruction on the use of an emergency intramuscular injection (standardly given by a nurse)
  • inquire about traveling abroad, provide letter with advice in case of (written in English) if required*
  • provide written information, including telephone number of on-call endocrinologist (24 hours a day service)!!
  • In addition the family physician receives a yearly letter with a standard treatment advice in case of an imminent Addisonian crisis. He is advised to inform his colleagues at the Central GP post.

II. Advice to patients with primary or secondary adrenal insufficiency for dosage of cortisone in case of stress. Normal Dose is 15 to 30 mg HC daily (or equivalent dose of other glucocorticoid)

  • outpatient or dental interventions (i.e. local anesthesia): double HC dose before intervention
  • fever (>38 C), severe psychological stress** (difficult exam, death family member): at least triple HC-dose, i.e. 60 mg in the morning and 30 mg in the evening, taper till normal dose after symptoms are relieved. Contact doctor if there is no improvement.
  • vomiting or diarrhea, unconsciousness: parenteral administration of 100 mg hydrocortison by patient or partner (im) or physician (im, iv); direct consult of on-call endocrinologist, always check afterwards at ED
  • surgery or hospitalization: the treating physician should contact the patientā€™s endocrinologist for advice on dose adjustments.

What is special about this protocol is the 24h endocrinologist on call service, the earlier (and consistent) referral to endocrinologists and ED, in case of possible emergency, and the structural approach: all patients with adrenal insufficiency, including their relatives and physicians, are well-informed about the preventive measures that should be taken (including HC emergency ampule and alert bracelet).

That is a great improvement! Hopefully other regions and countries will follow this example.

Notes and Sources:

Sources: Mulder AH, Nauta S, Pieters GF, Hermus AR. Addisonian crisis in patients with known adrenal insufficiency: the importance of early intervention. Ned Tijdschr Geneeskd. 2008 Jul 5;152(27):1497-500. [Article in Dutch] (see PubMed abstract here).
* The Dutch Addison and Cushing Society NVACP since long has a small booklet “SOS stressboekje”, which is specially designed to inform physicians abroad when on vacation. Short guidelines for dosages of (hydro)cortisone in stress and medical information for physicians is translated in 6 languages.
** Advices based on what is usually advised in the literature. There is little evidence for a particular dose in case of physical or psychological stress.
Photo’s acknowledgments.
Burning and burned matches derive from Flickr, respectively from
http://www.flickr.com/photos/bholak/309005330/
and http://www.flickr.com/photos/jmr_photo/2738016554/

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De Nederlandstalige samenvatting van het artikel:
Addison-crisis bij patiƫnten bekend wegens bijnierschorsinsufficiƫntie: het belang van vroegtijdig ingrijpen
A.H.Mulder, S.Nauta, G.F.Pieters en A.R.M.M.Hermus in het Ned Tijdschr Geneeskd. 2008 5 juli;152(27)

Dames en Heren,
PatiĆ«nten met een bijnierschorsinsufficiĆ«ntie kunnen over het algemeen goed functioneren indien zij worden behandeld met glucocorticoĆÆden en – in geval van een primaire bijnierschorsinsufficiĆ«ntie – mineralocorticoĆÆden. Tijdens ziekte, koorts en ernstige psychische stress is de natuurlijke
behoefte aan cortisol verhoogd. PatiĆ«nten met een bijnierschorsinsufficiĆ«ntie moeten in deze gevallen dan ook de substitutiedosering glucocorticoĆÆden verhogen. Alhoewel zij tijdens de poliklinische controles hierover uitleg ontvangen blijken de instructies niet altijd adequaat te worden opgevolgd. De ernst van de situatie wordt soms door de patiĆ«nt zelf, en soms door de geraadpleegde huisarts of specialist, onvoldoende onderkend.
Met de beschrijving van de volgende drie ziektegeschiedenissen willen wij onder de aandacht brengen dat een addison-crisis bij patiĆ«nten met een bekend hypocortisolisme levensbedreigend is, en dat vroegtijdig adequaat ingrijpen noodzakelijk is. Tevens beschrijven wij de maatregelen die wij namen om patiĆ«nten nog beter te informeren over glucocorticoĆÆdgebruik bij lichamelijke en psychische stress en om de bewustwording bij medebehandelaren te verhogen.





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.

ā€˜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 toĀ  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)




Blog Spam and Spam Blogs (1)

11 09 2008

Flickr.com cursedthing (CC)

We all get our spam once in a while. Most of the time spamfilters block them. Askismet works well at this blog. Often you recognize spam by the hyperlinks or the words, i.e. “viagra”.

But sometimes spam is not so obvious. In 2 separate post I would like to give some examples of less obvious blog spam, spamblogs and something in between.

Acccording to wikipedia:

Blog spam is done by automatically posting random comments or promoting commercial services to blogs. Any web application that accepts and displays hyperlinks submitted by visitors may be a target.

Conversely, spam blogs are usely fake weblogs where content is often either inauthentic text or merely stolen (scraped) from other websites.

All spam artificially increases the site’s search engine ranking, which often results in the spammer’s commercial site being listed ahead of other sites for certain searches, increasing the number of potential visitors and paying customers.

Blogs & Spam: “Spam” by request?

David Rothman describes at his blog how he is often mailed by people asking him to post about their site, which often is “just a lousy site solely meant for pharma marketing”. He refuses if the site isn’t really useful, but apparently many of his fellow health bloggers aren’t that fussy, since those particular sites often manage to get mentioned on other health blogs anyway. David hopes that the blog-reader will read through this, but is that really the case? The blogger may be considered an expert in the field (that’s why he receives an email) and people may be inclined to take his word for granted. Striktly taken this may not be spam, but it sure works the same way.

Spam Blog (1). “Spam” hidden behind “Breaking Health News”

About a week ago, I had a look at WordPress.com and saw an interesting featured post with the (WordPress) tag “Health”.
At WordPress “Featured Posts” are at the top of a tag list -in this case “Health”-, which increases traffic to such posts). The subject captured my attention, because it was about Addison’s disease (which I have). I read it.

Somebody with primary Addison (Primary Adrenal failure, which leads to inability to make the hormones cortisol, aldosterone and dehydroepiandrosterone (DHEA)) asked whether the menstrual irregularity she developed a year ago could be caused by the replacement therapy with Hydrocortisone and Fludrocortisone and if this could lower her fertility.

The answer (see here) was rather lengthy, it discussed the causes of menstrual irregularity, primary Addison’s disease, replacement therapy, that (the often not replaced) DHEA might improve general well-being, and finally comes to possible explanations:

  • changes in menstrual cycle could be related to too much or too little of the replacement hormones
  • recurrence of menstrual cycles was reported in one patient treated with DHEA (also considered as a supplement, by the way).
  • advice: consultation of an endocrinologist.

Nothing really wrong with this. However a more plausible explanation wasn’t mentioned, i.e. that the reduced cycling might be due to the disease itself. Nowadays the main cause for primary Addison is auto-immunity, and auto-immunity often doesn’t come alone. Gonadal failure can occur in approximately 5% of the woman with auto-immune Addison’s (Williams Textbook of Endocrinology, E-medicine).
For instance in 100 Dutch patients the distibution was as follows

… In 47% of the patients with autoimmune Addison’s disease at least one other autoimmune disorder was present. Primary hypothyroidism had the highest prevalence (20.5%), followed by vitiligo (9.6%), non-toxic goiter (8.4%), premature menopause (7.3% of the women) (….).
From: P.M.J. Zelissen et al, J Autoimmun. 1995 Feb;8(1):121-30.

I tried to place a comment. However, comments were closed (at the date of posting). Odd. I must say that I already found it weird for a patient to start with I actually have an interesting question.” No one says that, but rather:

Help, I’ve Addison and my menses become irregular, I want to have children, so I’m afraid that I’m becoming less fertile. Can this have anything to do with the corticosteroids I take?”

An even closer look points out that:

  • both the Q & the A are written by the same person.
  • The automatically generated “Possibly Related Posts” only link to posts at the same blog
  • as do all “so called comments” (so a kind of self-ping).
  • There is no info whatsoever about who is behind this site.
  • The tab “About” is really the tab Pharmacy Store, where a bunch of “high quality medications” are offered.
  • If I click on fosamax (which a lot of ex-Cushing (panhypopituitary) Addisonpatients need), I ‘m linked to a really (recognizable) commercial site: see here

Is this so bad? Well at least as bad as a lot of commercial-pills-selling-sites that don’t look like commercial-pills-selling-sites. It is quite misleading to use a blog on “breaking Health news” as a cover-up for real intentions: selling. Readers cannot respond, only trackback. Furthermore, in this particular case, the information was not really adequate for patients either (although “partially prepared” by pharmD candidates). One may also wonder why such a post becomes the featured Health blog at WordPress. Well, it will have suited them (and their tag “health” is well-thought-out).

But there are better (or really worse) examples of real spam blogs. Two examples will be given in the next post (see here).

Flickr.com cursedthing

———————-

We hebben allemaal wel eens last van spam. Meestal wordt spam wel door spamfilters geblokkeerd. Askismet houdt in ieder geval het nodige tegen op dit blog (700 spam). Vaak herken je spam wel aan de (vele) hyperlinks of termen als “Viagra”.

Soms is echter niet zo duidelijk dat het om spam gaat. In tenminste 2 berichten wil ik voorbeelden geven van minder evidente blogspam, spamblogs en wat daar tussenin zit. Het zijn dingen waar ik toevallig tegenaan gelopen ben.

Eerst wat definities. Volgens Wikipedia :

Blog spam is done by automatically posting random comments or promoting commercial services to blogs. Any web application that accepts and displays hyperlinks submitted by visitors may be a target.

Conversely, spam blogs are usely fake weblogs where content is often either inauthentic text or merely stolen (scraped) from other websites.

All spam artificially increases the site’s search engine ranking, which often results in the spammer’s commercial site being listed ahead of other sites for certain searches, increasing the number of potential visitors and paying customers.

Blogs & Spam: “Spam” op verzoek?

David Rothman vertelt op zijn blog dat hij vaak een verzoek per mail krijgt om een post te plaatsen over een bepaalde site, terwijl het gewoon om een belabberde farmaceutisch e-commerce site gaat. David weigert dit als de site slecht is/zijn lezers niets biedt, maar kennelijk zijn z’n collega bloggers niet zo kieskeurig: vaak worden dergelijke sites binnen no time wel op andere gezondheidsblogs besproken. David hoopt dat de lezers van dergelijke blogs hier doorheen kijken, maar ik vraag me af of dat werkelijk zo is. Degene die erover schrijft op zijn blog wordt al gauw als expert gezien (daarom kreeg hij ook dat verzoek) en lezers zullen al gauw geneigd zijn wat hij bespreekt voor waar aan te nemen. Strikt genomen is dit wellicht geen spam, maar het resultaat is hetzelfde.

Spam Blog (1). “Spam” verborgen achter “Breaking Health News”

Ruim een week geleden zag ik een interessante post bij de “featured posts on Health” bij WordPress.com.
Bij WordPress komen “Featured Posts” bovenaan de posts met een bepaalde tag, in dit geval “Health” te staan. Ze worden daarmee extra in het zonnetje gezet en krijgen extra veel bezoek. Maar in dit geval trok ook het onderwerp mijn aandacht, omdat ik het zelf heb: de ziekte van Addison.

Iemand met primaire Addison (uitval van de bijnieren waarbij de oorzaak in de bijnieren zelf ligt, niet in de aansturing. Hierdoor worden de hormonen cortisol, aldosteron en dehydroepiandrosterone (DHEA) niet meer gemaakt) stelde een vraag over haar sinds een jaar vaak uitblijvende menstruatie. Ze wilde weten of dit iets te maken kon hebben met de substitutietherapie met Hydrocortison and Fludrocortison.

Het antwoord (zie hier) was nogal weinig to the point. Het volgende werd breeduit besproken: de oorzaken van onregelmatige menstruatie i.h.a., primaire Addison, substitutietherapie, dat het vaak niet gesubstitueerde DHEA (eigenlijk ook vaak gebruikt als voedingssupplement) de kwaliteit van leven kan verbeteren, om tot slot met enkele mogelijke verklaringen te komen:

  • veranderingen in de menstruatiecyclus kunnen samenhangen met te weinig of te veel vervangende hormonen (maar niet door fysiologische doses, hetgeen het streven is bij vervanging).
  • Ć©Ć©n patient kreeg weer een normale cyclus na gebruik van DHEA (overigens werden ook de andere hormonen beter ingesteld)
  • tot slot een algemeen advies; ga naar je endocrinoloog.

Hier is niet echt wat mis mee (vooral met het laatste advies). Zij het dat een voor de hand liggende verklaring niet genoemd wordt, namelijk dat een onregelmatige cyclus en verlaagde vruchtbaarheid ook kunnen samenhangen met de ziekte zelf. Tegenwoordig is de belangrijkste oorzaak voor primaire Addison autoimmuniteit (afweerreactie tegen eigen weefsels/organen) en autoimmuniteit komt vaak niet alleen. Uitval van de geslachtsorganen kan in zo’n 5% van de patienten met primaire Addison voorkomen (Williams Textbook of Endocrinology, E-medicine).
Bij 100 Nederlandse patienten was de verdeling bijvoorbeeld als volgt:

… In 47% of the patients with autoimmune Addison’s disease at least one other autoimmune disorder was present. Primary hypothyroidism had the highest prevalence (20.5%), followed by vitiligo (9.6%), non-toxic goiter (8.4%), premature menopause (7.3% of the women) (….).
From: P.M.J. Zelissen et al, J Autoimmun. 1995 Feb;8(1):121-30.

Ik probeerde een reactie te plaatsen op de blogpost, maar dat was niet meer mogelijk. Nou ja niet meer: het was de dag dat het bericht geplaatst was. Raar. Ik moet zeggen dat ik al mijn wenkbrauwen fronsde bij het zien van de aanhef I actually have an interesting question.” geen patient begint zo, maar zegt eerder:

Help, Ik heb Addison. Mijn cycli worden onregelmatig en ik wil nog graag kinderen hebben, dus ik ben bang dat ik minder vruchtbaar wordt. Kan dit komen door de corticosteroiden die ik ter vervanging inneem?”

Geintrigeerd ging ik verder op zoek.

  • De Q & de A bleken door dezelfde persoon geschreven.
  • De automatisch gegenereerde “Possibly Related Posts” linken alleen naar berichten op het blog zelf.
  • Dat geldt ook voor alle commentaren (een soort zelf-ping).
  • Er is nergens info over wie er achter de site zit.
  • De tab “About”/”Over” is eigenlijk de link naar de “Pharmacy Store“, waar een reeks “high quality medications” wordt aangeboden.
  • Als je bijvoorbeeld op fosamax (vaak gebruikt door ex-Cushing Addisonpatienten) klikt kom je op een duidelijk herkenbare commerciele site terecht: zie hier

Is dit zo erg? Nou dit blog is net zo erg als die pillen-verkopende websites die er niet uitzien als pillenverkopende websites. Het is nogal misleidend om je blog te presenteren als een blog over “breaking Health news” om je werkelijke bedoelingen te verbloemen: pillenverkoop. Lezers kunnen niet reageren, alleen trackbacken. Verder was de informatie ook voor patienten niet helemaal volledig. Je kunt je ook afvragen hoe zo’n blog nou een featured Health blog bij WordPress wordt. Nou, het was wel lekker meegenomen (en ze kennen niet voor niets de tag “Health” toe).

Maar er zijn betere (or eigenlijk slechtere) voorbeelden van echte spam blogs. In de volgende post (zie hier) zal ik er twee bespreken.





Visualize your blog (words) with Wordle

3 08 2008

I just discovered Wordle through Thomas of “Biomedicine on Display” (blog of Medical Museion, University of Copenhagen), who wrote several posts on this new web toy. Thomas created a cloud of his own blog links, and of the World top blogs on Health and Medicine. I’m on these clouds, but couldn’t find my name. Can you? (it is weighted according to importance šŸ˜‰ ).

Wordle is a free online Java ap created by Jonathan Feinberg, which generates word clouds from any given URL, RSS-feed, del.icio.us user names, or text you provide. The nice thing is you can tweak your clouds with different fonts, layouts (horizontal, vertical, random etc) and color schemes.

1. Laika’s URL – no english stopwords
2. Laika’s URL – no dutch stopwords

Above are two word-clouds of my blog, created by entering the URL of my blog and removing common words. However, since my blog is bilingual, removing English stop words leaves Dutch stop words and vice versa. Thus the two versions shown above most prominently show the stopwords of the other language, but that gives a funny effect. Wordle only shows the words of recent posts, those posts which show up when you type in the URL.
The variation is endless. Even when you refresh, you get a different picture.

Pictures can be saved online in the “the Gallery”.

3. Laika’s old category cloud – not weighted

Next I created a category cloud from my (old) categories (I need to update them). First I didn’t weight the words, entering each word once (3). But in the next series I gave the categories weight by repeating them as many times as they were used in my blog (4,5). I just show fig. 4, because ‘Social Networking’ was completely out of the cloud, which is -I think- a contradictio in terminis. Really amusing.

5. Category Cloud - weighted

Cat-cloud - weighted

4. Cat-cloud - weighted

Addison's disease info (nvacp)

6. Addison's disease info (NVACP)

Finally I copied the text from a Dutch Patient website (the NVACP) about Addison’s disease (which I have). This illustrate one of the applications of World: you can immediately grasp what the text is about. It can be used to visualize your presentation or even as an educational tool. (e.g. in this post Wordle is used to prepare a school lesson on the environment). But you can use Wordl for many purposes, i.e. make postcards, write a poem etcetera (see this post for some ideas).

Well anyway, playing with Wordle is great fun and a bit addicting.

Want to create your own: click here

——-

Een heel leuk nieuw web-speeltje: Wordle.
Toevallig leerde ik het kennen via Biomedicine on Display” (blog van het Medical Museion, Universiteit van Copenhagen), waar Thomas enkele berichten over deze applicatie plaatste.

Thomas maakte een woordwolk van zijn blogroll, en van de “Top Health and Medicine Blogs”. Ik sta ergens op deze wolkjes, maar heb ze nog niet kunnen localiseren. Nu ja wat wil je met meer dan 800 blogs, naar belang gerangschikt šŸ˜‰

Wordle is een gratis Java applicatie, ontwikkeld door Jonathan Feinberg.
World genereert woordwolken vanuit een
URL, RSS-feed, del.icio.us gebruikersnaam of aangeleverde tekst. Het mooie eraan is dat je de wolken op verschillende manieren kunt opleuken. Je kunt heel veel verschillende lettertypen, lay-outs (verticaal horizontaal, kris-kras) en kleurenschema’s toepassen. Plaatjes kun je bewaren in “the Gallery”.

Ik heb eerst woordenwolken van mijn blog gemaakt door de URL in te voeren en de opdracht te geven stopwoorden te verwijderen. Maar mijn blog is twee-talig. Verwijder ik de nederlandse stopwoorden, dan komen juist de engelse stopwoorden prominent in beeld, en vice versa. Maar ach, dat is eigenlijk wel grappig. Wordle toont trouwens alleen een wolk van de laatste berichten, d.w.z. dĆ­e berichten die je ziet als je de url intypt. Bij mij gingen de laatste berichten juist over Twitter en PubMed.
Je kunt eindeloos varieren. En zelfs als je ‘refresht’ wordt het plaatje weer helemaal anders qua kleur-compositie.

Vervolgens maakte ik een wolk van mijn (oude) categoriĆ«n (althans het is het plan ze drastisch te vernieuwen, er komen nog teveel woorden van de Spoetnikcursus in voor die ik niet meer zal gebruiken). Eerst voerde ik de termen in enkelvoud in (3). Maar daarna gaf ik er gewicht aan door ze zo vaak te kopiĆ«ren als ze op mijn blog voorkomen (4,5). Fig 4 laat ik zien omdat ‘Social Networking’ er helemaal buitenhangt, eigenlijk een contradictio in terminis, maar wel amusant.

Addison's disease info (nvacp)

6. Addison's disease info (NVACP)

Tenslotte kopieerde ik tekst over de ziekte van Addison van de Nederlandse Vereniging van Addison en Cushingpatienten (NVACP). Dit illusteert meteen een van de mogelijkheden van Wordle: je ziet in een oogopslag waar de tekst over gaat. Daarom kun je met deze tool gebruiken je presentatie visualiseren, of het als een leermiddel inzetten (zie bijvoorbeeld dit bericht, waar Wordle is gebruikt voor een les over het milieu).

Maar je kunt Wordl op veel manieren toepassen, je kunt er bijvoorbeeld ansichtkaarten mee maken of een gedicht. (zie dit bericht voor enkele ideeen).

Nou, hoe dan ook, een beetje spelen met Wordle is erg leuk ĆØn verslavend.

Wil je zelf aan de slag, klik dan hier!

Binnenkort meer…