Day 5, Cushing’s Awareness Challenge

In Day 9 on April 9, 2015, I wrote about how we got the Cushing’s colors of blue and yellow.  This post is going to be about the first Cushing’s ribbons.

I was on vacation  in September, 2001 when SuziQ called me to let me know that we had had our first Cushie casualty (that we knew about).

On the message boards, Lorrie wrote: Our dear friend, Janice died this past Tuesday, September 4, 2001. I received an IM from her best friend Janine, tonight. Janine had been reading the boards, as Janice had told her about this site, and she came upon my name and decided to IM me. I am grateful that she did. She said that she knew that Janice would want all of us to know that she didn’t just stop posting.

For all of the newcomers to the board that did not know Janice, she was a very caring individual. She always had something positive to say. Janice was 36 years old, was married and had no children. She had a miscarriage in December and began to have symptoms of Cushing’s during that pregnancy. After the pregnancy, she continued to have symptoms. When discussing this with her doctor, she was told that her symptoms were just related to her D&C. She did not buy this and continued until she received the accurate diagnosis of Cushing’s Syndrome (adrenal) in March of 2001. Tragically, Janice’s tumor was cancerous, a very rare form of Cushing’s.

Janice then had her tumor and adrenal gland removed by open adrenalectomy, a few months ago. She then began chemotherapy. She was very brave through this even though she experienced severe side effects, including weakness and dizziness. She continued to post on this board at times and even though she was going through so much, she continued with a positive attitude. She even gave me a referral to a doctor a few weeks ago. She was my inspiration. Whenever I thought I had it bad, I thought of what she was dealing with, and I gained more perspective.

Janice was having difficulty with low potassium levels and difficulty breathing. She was admitted to the hospital, a CT scan was done and showed tumor metastasis to the lungs. She then was begun on a more aggressive regimen of chemo. She was discharged and apparently seemed to be doing well.

The potassium then began to drop again, she spiked a temp and she was again admitted to the hospital. She improved and was set to be discharged and then she threw a blood clot into her lungs. She was required to be put on a ventilator. She apparently was at high risk for a heart attack. Her husband did not want her to suffer anymore and did not want her to suffer the pain of a heart attack and so chose for the doctors to discontinue the ventilator on Tuesday. She died shortly thereafter.

Janice was our friend. She was a Cushie sister. I will always remember her. Janine asked me to let her know when we get the Cushing’s ribbons made as she and the rest of Janice’s family would like to wear them in her memory. She said that Janice would want to do anything she could to make others more aware of Cushing’s.

The image at the top of the page shows the first blue and yellow ribbon which were worn at Janice’s funeral.  When we had our “official ribbons” made, we sent several to Janice’s family.

Janice was the first of us to die but there have been more, way too many more, over the years.  I’ll write a bit more about that on Day 21.

Day 4, Cushing’s Awareness Challenge

This is one of the suggestions from the Cushing’s Awareness Challenge post:

“Give yourself, your condition, or your health focus a mascot. Is it a real person? Fictional? Mythical being? Describe them. Bonus points if you provide a visual!”

 

Our “Official mascot” is the zebra.

Our mascot

Our mascot

In med school, student doctors are told “When you hear hoofbeats, think horses, not zebras“.

According to Wikipedia: “Zebra is a medical slang term for a surprising diagnosis. Although rare diseases are, in general, surprising when they are encountered, other diseases can be surprising in a particular person and time, and so “zebra” is the broader concept.

The term derives from the aphorism ‘When you hear hoofbeats behind you, don’t expect to see a zebra’, which was coined in a slightly modified form in the late 1940s by Dr. Theodore Woodward, a former professor at the University of Maryland School of Medicine in Baltimore.  Since horses are the most commonly encountered hoofed animal and zebras are very rare, logically you could confidently guess that the animal making the hoofbeats is probably a horse.

zebra-mug

A zebra cup my DH bought me 🙂

By 1960, the aphorism was widely known in medical circles.”

Why? Because those of us who DO have a rare disorder know from personal experience what it feels like to be dismissed by a doctor or in many cases, multiple doctors. Many physicians have completely lost the ability to even imagine that zebras may exist!  Cushing’s is too rare – you couldn’t possible have that.  Well… rare means some people get it.  Why couldn’t it be me?

Although one of my signature images has a zebra, many have rainbows or butterflies in them so I guess that I consider those my own personal mascots.

I posted this in 2010 in 40 Days of Thankfulness: Days Twenty-Two through Thirty

I have a special affinity for rainbows. To me, a rainbow is a sign that things are going to be ok.

Years ago, our little family was in Florida. I felt guilty about going because my dad was terminally ill with his second bout of colon cancer. I was worried about him and said a little prayer for him.

I was lying on the beach while DH and our son were in the ocean and I looked up and saw a rainbow. It was a perfectly clear, sunny afternoon. I even called the people out of the water, in case it was something I wanted to see that didn’t really exist. They saw it, too.

Where in the world did that rainbow come from, if it wasn’t a sign that everything would be ok?

Butterflies are something else again.  I like them because I would like to think that my life has evolved like a butterfly’s, from something ugly and unattractive to something a big easier on the eye.

My Cushie self was the caterpillar, post-op is more butterfly-ish, if not in looks, in good deeds.

From July, 2008

For as long as I can remember, I’ve loved butterflies for their beauty and what they stood for. I’ve always wanted to shed my cocoon and become someone else, someone beautiful, graceful.

One of my first memories as a kid was knocking on the back door of my house and when my mom answered, I’d pretend to somehow be an orphan, looking for some kind person to take me in. And I would try to be that different child, with new habits, in the hopes that my parents would somehow think better of me, love me more as this poor homeless kid than they did as their own.

The butterfly was trying to emerge but it never got too far. Somehow, I would slip into my original self and be a bother to my parents.

Hope springs eternal, though!

Day 3: Cushing’s Awareness Challenge

me-tired

Sleep.  Naps.  Fatigue, Exhaustion.  I still have them all.  I wrote on my bio in 1987 after my pituitary surgery “I am still and always tired and need a nap most days. I do not, however, still need to take whole days off just to sleep.

That seems to be changing back, at least on the weekends.  A recent weekend, both days, I took 7-hour naps each day and I still woke up tired. That’s awfully close to taking a whole day off to sleep again.

In 2006, I flew to Chicago, IL for a Cushing’s weekend in Rockford.  Someone else drove us to Lake Geneva, Wisconsin for the day.  Too much travel, too Cushie, whatever, I was too tired to stay awake.  I actually had put my head down on the dining room table and fallen asleep but our hostess suggested the sofa instead.  Amazing that I traveled that whole distance – and missed the main event 😦

This sleeping thing really impacts my life.  Between piano lessons, I take a nap.  I sleep as late as possible in the mornings and afternoons are pretty much taken up by naps.  I nod off at night during TV. One time I came home between church services and missed the third service because I fell asleep.

I only TiVo old tv shows that I can watch and fall asleep to since I already know the ending.

Since  mid-February, I have been doing physical therapy twice a week for 2 hours at a time for a knee injury (read more about that in Bees Knees).  I come home from that exhausted – and in more pain than I went.  I know it’s working and my knee is getting better, but it’s such a time and energy sapper.  Neither of which I can really spare.

Maybe now that I’m nearly 10  years out from my kidney cancer (May 9, 2006) I could theoretically go back on Growth Hormone again.  My surgeon says he “thinks” it’s ok.  I’m sort of afraid to ask my endo about it, though.  I want to feel better and get the benefits of the GH again but I don’t want any type of cancer again and I certainly can’t afford to lose another kidney.

I’ll probably just muddle through without it.  I always laugh when I see that commercial online for something called Serovital.  I saw it in Costco the other day and it mentions pituitary right on the package.  I wish I could take the people buying this, sit them down and tell them not to mess with their pituitary glands.  But I won’t.  I’ll take a nap instead because I’m feeling so old and weary today, and yesterday.

And tomorrow…

Longer-Acting Growth Hormones Promising for Adult GH Deficiency

Two investigational long-acting growth-hormone (GH) replacement products hold potential for less frequent dosing and improved adherence among adult patients with proven growth-hormone deficiency.

Adult growth-hormone deficiency is a rare disorder characterized by the inadequate secretion of the growth hormone from the pituitary gland. It can be hereditary; can be acquired as a result of trauma, infection, radiation therapy, or brain tumor growth; and can even emerge without a diagnosable cause. Currently, it is treated with once-daily injections of subcutaneous growth hormone.

The new results, from a 26-week phase 3 trial of Novo Nordisk’s once-weekly growth-hormone derivative somapacitan and a dose-finding phase 2 safety study of Versartis’s long-acting recombinant growth hormone somavaratan, both in adult patients with growth-hormone deficiency, were presented here at ENDO 2017: The Endocrine Society Annual Meeting.

“Compliance is often a problem with daily growth-hormone injections in children and even with adults,” session moderator Luma Ghalib, MD, assistant professor in the division of endocrinology, diabetes, and metabolism at Ohio State University Wexner Medical Center, Columbus, told Medscape Medical News.

“Patients will often stop taking the daily medications, sometimes because of the cost but also because the daily injections are cumbersome. So the two longer-acting agents that have been studied will be an amazing breakthrough if they get [US FDA]-approved.”

But, she cautioned, longer-term data are needed. “In the long term, we worry about the metabolic effects. We know growth hormone can increase insulin resistance and diabetes, so we have to keep an eye on the peaks.”

And, she added, there could be a small risk for regrowth of the pituitary adenoma that caused the growth-hormone deficiency. “The risk will probably be slim because we haven’t seen regrowth with the daily dosing, but it hasn’t been studied.”

Once-Weekly Somapacitan Found Safe, Well-Tolerated

Gudmundur Johannsson, MD, PhD, professor and chief physician at the University of Gothenburg, Sweden, reported findings from the 26-week multicenter, multinational, randomized open-label parallel-group trial of somapacitan, a reversible albumin-binding human GH derivative intended for once-weekly subcutaneous administration.

A total of 92 adults (aged 18-79 years) who had been previously treated with once-daily growth-hormone replacement for at least 6 months were randomized 2:1 (after a 1-day washout) to either once-weekly somapacitan or once-daily somatropin (Norditropin, Novo Nordisk). Doses of both were titrated for the first 8 weeks to achieve normal insulinlike growth factor (IGF)-1 levels (target 0–2 standard deviation scores) and remained fixed for the subsequent 18 weeks.

Patients were around 50 years of age, 45% female, with body mass index 28 kg/m2. After remaining stable in both arms following titration, mean serum IGF-1 standard-deviation scores at week 25 were 0.22 for somapacitan and 0.35 for somatropin.

The primary outcome, incidence of adverse events including injection-site reactions, was similar between the two groups. Total adverse events occurred in 53 of 61 (86.9%) with somapacitan vs 21 of 31 (67.5%) with somatropin and included nasopharyngitis, headache, fatigue, dizziness, and arthralgia. Serious adverse events occurred in four (6.6%) with somapacitan and two (6.5%) with somatropin.

Of more than 1500 somapacitan injections given, there were two mild, transient, injection-site reactions (hematoma and bruising). No antibodies to somapacitan or GH were detected.

At week 26, patients’ scores on the Treatment Satisfaction Questionnaire for Medication-9 (TSQM-9) for convenience, effectiveness, and satisfaction all favored somapacitan over somatropin.

Additional phase 3 trials in adults with growth-hormone deficiency are ongoing, as well as a phase 2 trial in children, a Novo Nordisk spokesperson told Medscape Medical News.

Somavaratan Dose-Finding Study Yields 2-Week Response

Kevin CJ Yuen, MD, MBChB, medical director of the Swedish Pituitary Center, Swedish Neuroscience Institute, Seattle, Washington, presented findings from an open-label, multicenter phase 2 study of somavaratan, a novel long-acting form of recombinant human growth hormone. The study aimed to evaluate starting dose, dose titration plan, and safety and to determine the IGF-1 response with 30-day dosing.

Patients were allocated into three starting dose cohorts: 0.6 mg/kg/month for those aged 35 and older, 0.8 mg/kg/month for those younger than 35, and 1.0 mg/kg/month for women on oral estrogen, regardless of age. All received five monthly subcutaneous doses of somavaratan with a target IGF-1 standard deviation score of 0–1.5. In all, 32 of 49 patients completed the study.

The most common adverse events were injection-site reactions (19.4%) and headache (11.1%), mostly mild or moderate. No severe adverse events were deemed related to somavaratan.

Mean IGF-I SDS increased from -1.32 at baseline to +2.31 at 7 days after the first dose, with subjects within each cohort who received higher doses tending to have higher IGF-1 responses. Following the last study dose, IGF-1 standard-deviation scores returned to baseline by day 22.

Thus, Dr Yuen said, twice-monthly administration will be studied going forward. Starting somavaratan dose and administration frequency are being investigated further in the extension study and then will be carried forward in a new phase 3 study.

Speaking about both products, Dr Ghalib told Medscape Medical News: “We are waiting. Less frequent dosing will make our lives and definitely the patients’ lives a lot easier.”

Dr Johannsson is a consultant and/or speaker for Viropharma, Shire, AstraZeneca, Novartis, Otsuka, Novo Nordisk, Merck, Serono, Pfizer, and Ipsen. Dr Yuen is an investigator and/or medical advisory board member for Pfizer, Opko, Novo Nordisk, Versartis, and Sandoz. Dr Ghalib has no relevant financial relationships.  

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ENDO 2017. April 1, 2017; Orlando, Florida. Abstract OR22-1, Abstract OR22-2

From http://www.medscape.com/viewarticle/878088

Day 2, Cushing’s Awareness Challenge

The Seven Dwarves of Cushing's

So, these are only seven of the many, many symptoms of Cushing’s.  I had those above – and I often felt like I looked like one of those little bearded dwarves.

Cushing’s affects every part of the body.  It’s not like when I had kidney cancer and only the kidney was affected.

Here are some of the many areas affected.

  • Progressive obesity and skin changes
  • Weight gain and fatty tissue deposits, particularly around the midsection and upper back, in the face (moon face) and between the shoulders (buffalo hump). Some symptoms such as sudden weight gain, are caused by excess cortisol. The excess cortisol in the body does not increase protein and carbohydrate metabolism. It slows or nearly disables metabolism function, which can cause weight gain (fat accumulation) in the buttocks, abdomen, cheeks, neck, or upper back.
  • Loss of muscle mass. Some areas of the body, such as the arms and legs, will remain thin.
  • Pink or purple stretch marks (striae) on the skin of the abdomen, thighs, breasts and arms
  • Thinning, fragile skin that bruises easily
  • Slow healing of cuts, insect bites and infections
  • Acne

Women with Cushing’s syndrome may experience:

  • Thicker or more visible body and facial hair (hirsutism)
  • Irregular or absent menstrual periods

Men with Cushing’s syndrome may experience:

  • Decreased libido
  • Decreased fertility
  • Erectile dysfunction

Other signs and symptoms include:

  • Fatigue
  • Muscle weakness
  • Depression, anxiety and irritability
  • Loss of emotional control
  • Cognitive difficulties
  • New or worsened high blood pressure
  • Glucose intolerance that may lead to diabetes
  • Headache
  • Bone loss, leading to fractures over time
  • Hyperlipidemia (elevated lipids – cholesterol – in the blood stream)
  • Recurrent opportunistic or bacterial infections
Think you have Cushing’s?  Get to a doctor and don’t give up!

MaryO
         MaryO