Prince George’s woman works to raise awareness about rare disease

Posted on September 26, 2013 by MaryO

Lanham resident to speak at Patient Education Day event about Cushing’s disease

By Sophie Petit Staff Writer
stacy

Greg Dohler/The Gazette

Lanham resident Stacey L. Hardy, a survivor of Cushing’s disease, will speak about her experience with the pituitary disorder at an upcoming event at Johns Hopkins Hospital.

Stacy L. Hardy of Lanham described herself as athletic, which is why she became concerned when in 1996 she mysteriously gained 240 pounds that took five doctors 14 years to determine she had a potentially fatal disease.

Now Hardy said she wants to raise awareness among others who may unknowingly have Cushing’s disease, but are unaware of the symptoms and treatment.

It wasn’t until 2010 that Hardy, now 43, was diagnosed with the disease, a rare disorder that causes the body to release too much cortisol, the body’s stress or “fight or flight” hormone, said Gary Wand, a pituitary gland specialist at The Johns Hopkins Hospital in Baltimore.

Excess cortisol causes weight gain, especially in the stomach, extreme fatigue, muscle aches, anxiety and depression, he said.

“I didn’t even know what Cushing’s was. I was ready to just live with [the symptoms],” Hardy said, adding that by the time she was diagnosed she felt so tired she could barely move.

At 5 feet, 4 inches tall, Hardy said she reached 365 pounds during her struggle with the disease.

“We knew something for a while wasn’t right, but I never thought it would be something like that,” said Hardy’s daughter, Paij Hardy, 21, a student at Baltimore City Community College.

Just three out of every one million people are diagnosed with Cushing’s each year, said Wand, who estimates he sees 30 patients per year worldwide.

In 2011, Hardy underwent 16 hours of surgery at Sinai Hospital in Baltimore to remove four tumors from her pituitary gland, located at the base of the brain that controls the release of cortisol.

Today, she is 100 pounds lighter, with the weight still rapidly coming off, and said she is determined to serve as a lifelong support and education source for her fellow “cushies” — others with Cushing’s disease.

Hardy will speak Saturday at the Johns Hopkins Pituitary Gland Center’s fifth annual Patient Education Day, an event to raise awareness about the disease, Wand said.

Since the pituitary gland is the size of a kidney bean, Hardy underwent several brain scans before doctors, who previously suggested she might have leukemia or needed to diet and exercise more, could tell there were tumors on her gland, she said.

Hardy’s experience with delayed diagnosis and misdiagnosis is not unique, Wand said.

Cushing’s is a “subtle” disease, which is difficult to diagnose, and not everyone exhibits the same symptoms, he said.

If left untreated for more than a decade, the disease is fatal, but removing the pituitary gland tumors has proved extremely successful, Wand said.

“I’m evidence that there’s help out there,” Hardy said. “I can move. I can almost run. I can bend over and pick up a box. Oh my goodness, I can dance.”

From http://www.gazette.net/article/20130926/NEWS/130929354/1077/prince-george-x2019-s-woman-works-to-raise-awareness-about-rare&template=gazette

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Mifepristone in children with refractory Cushing’s disease

Posted on September 14, 2013 by MaryO

Introduction

This study is being done to examine the effects of a medication called mifepristone in children with Cushing’s disease. This medication has been approved by the Food and Drug Administration (FDA) for use in adult patients with Cushing’s syndrome. It is not FDA approved for use in children.

The study will investigate how children’s bodies absorb and process mifepristone, how it works in children and what effect it has on the use of sugar in the body, on the child’s weight and on growth hormone. An important part of the study is to determine the proper dosing and to evaluate the side effects of mifepristone in children.

Children 6 to 17 years old will be enrolled in the study if they have had surgery for Cushing’s disease and currently have elevated cortisol levels.

To get started, please click here.

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NIH: An Open-Label Study of The Safety, Pharmacokinetics and Pharmacodynamics of Mifepristone in Children With Refractory Cushing’s Disease

Posted on September 13, 2013 by MaryO

This study is currently recruiting participants.

Summary

Number 13-CH-0170
Sponsoring Institute National Institute of Child Health and Human Development (NICHD)
Recruitment Detail Type: Participants currently recruited/enrolled
Gender: Male & Female
Min Age: 6
Max Age: 17
Referral Letter Required No
Population Exclusion(s) None
Special Instructions Currently Not Provided
Keywords Child;
Cushing Syndrome;
Metabolism;
Mifepristone;
Pharmacokinetic-Pharmacodynamic
Recruitment Keyword(s) None
Condition(s) Cushing’s Syndrome;
Cushing Syndrome
Investigational Drug(s) Mifepristone
Investigational Device(s) None
Intervention(s) Drug: mifepristone
Supporting Site National Institute of Child Health and Human Development

Background:

– There are currently no approved therapies for children with Cushing’s disease who are not cured by surgery alone. A drug called mifepristone has been approved to treat adults with Cushing’s syndrome and elevated blood glucose caused by Cushing’s. The drug is marketed under the name Korlym(Registered Trademark). The study drug may have a different effect on a child’s body than an adult’s, so researchers want to know how much of the drug to give children and what effect it will have. They want to learn if mifepristone improves Cushing’s disease in children as it does in adults. They also want to know about the drug’s side effects in children.

Objectives:

– To study the effect of a medication called mifepristone in children with Cushing’s disease that has not been helped by pituitary surgery.

Eligibility:

– Children ages 6 to 17 with active Cushing’s disease following pituitary surgery and who have a body weight higher than expected for their height and age.

Design:

– Participants will be screened for up to 8 weeks with a physical exam, medical history, and medical tests including blood tests and X-rays.

– Participants will take tablets of the study drug each day for 12 weeks.

– Participants will stay at the clinic for 4 nights at the beginning of the study. They will have three 1-day visits during the study. They will stay at the clinic the last 3 days of the study.

– At these visits, participants will be given several tests. In one test, a small wire is inserted under the skin of the belly and a small monitor is attached taped to the belly. In another, the participant drinks a liquid and blood samples are taken.

– Follow-up visits will occur 4 weeks and 12 weeks after the study ends.

–Back to Top–

Eligibility

INCLUSION CRITERIAPatients who are eligible for enrollment must meet the following eligibility criteria:

– Males and females 6-17 years at informed consent

– Active Cushing’s disease as demonstrated by the following:

–24 hour Urinary Free Cortisol greater than the upper limit of normal for age on two urine collections during screening and

— midnight serum cortisol > 4.4 mcg/dL (mean of two determinations on a single day at 2330 and 2400 during screening)

– Previous trans-sphenoidal surgery (TSS) for ACTH secreting pituitary tumor at least 3 months prior to screening

– Increased body weight defined by BMI Z-score of 1.5 or above

– Able to provide consent/assent

– Able to swallow study drug tablets (not crushed or split)

– Willing to use non-hormonal method of contraception in patients of reproductive potential

– Primary health care provider in home location

EXCLUSION CRITERIA:

– Hypercortisolism not due to Cushing’s disease.

– Type 1 diabetes mellitus

– HbA1c geater than or equal to 9.5% at Screening

– Body weight < 25 kg

– Use of certain medications that are CYP3A substrates with narrow therapeutic ranges, such as simvastatin, lovastatin, cyclosporine, dihydroergotamine, ergotamine, fentanyl, pimozide, quinidine, sirolimus, and tacrolimus during the 4 weeks prior to starting study drug. Use of these medications is also prohibited until 2 weeks after end of dosing.

– Use of certain medications that are strong CYP3A inhibitors such as itraconazole, nefazodone, ritonavir, nelfinavir, indinavir, atazanavir, amprenavir, fosamprenavir, boceprevir, clarithromycin, conivaptan, lopinavir, mibefradil, posaconazole, saquinavir, telaprevir, telithromycin, and voriconazole during the 2 weeks prior to starting study drug.

Use of these medications is also prohibited until 2 weeks after end of dosing. Grapefruit and grapefruit juice are prohibited during this time frame.

– Use of certain medications that are strong inducers on CYP3A such as rifampin, rifabutin, rifapentin, phenobarbital, phenytoin, carbamazepine, St. John’s wort during the 2 weeks prior to starting study drug. Use of these medications is also prohibited until 2 weeks after end of dosing.

– Use of medications used to treat hypercortisolism from the duration indicated below prior to Day 1. Use of the medications is also prohibited until after the end of study 4 week follow up visit.

–steroidogenesis inhibitors such as ketoconazole, metyrapone: 4 weeks

–cabergoline, bromocriptine, somatostatin analogs such as octreotide, lanreotide, pasireotide long acting formulations: 8 weeks (immediate release formulations: 2 weeks)

–mitotane: 8 weeks

– Use of systemic glucocorticoid medications beginning 1 month prior to screening or anticipated use of these medications except for the treatment of adrenal insufficiency. Use of glucocorticoid medications is prohibited during the study until after the end of study 4 week study visit.

– Inflammatory, rheumatological, proliferative or other disorder(s) that would be anticipated to worsen with glucocorticoid blockade (e.g. inflammatory bowel disease, rheumatoid arthritis, psoriasis, etc.).

– Uncontrolled hypo- or hyperthyroidism.

– Uncorrected hypokalemia (< 3.5 mEq/L). The screening period may be used to correct hypokalemia prior to starting study drug. Use of potassium and/or mineralocorticoid antagonists is permitted during the study.

– QTc geater than or equal to 450 msec on Screening electrocardiogram

– Unexplained vaginal bleeding in females and/or any history of endometrial pathology.

– Positive pregnancy test in females.

From http://clinicalstudies.info.nih.gov/cgi/detail.cgi?A_2013-CH-0170.html

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Hormonal disorder may make weight loss more challenging

Posted on September 11, 2013 by MaryO

(HealthDay News) — Losing weight is never a piece of cake, but there are some medical conditions that may make it even more difficult.

The American Academy of Family Physicians says these conditions may make weight loss more of a challenge:

  • Hormonal disorders, such as diabetes, hypothyroidism, Cushing’s disease and polycystic ovary syndrome.
  • Cardiovascular problems, including heart-valve disorders and congestive heart failure.
  • Disorders affecting sleep, including obstructive sleep apnea and upper airway resistance syndrome.
  • Eating disorders, such as bulimia and carbohydrate craving syndrome.

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One Of A Kind: Despite use of only one lung, right guard Dillon Reagan helps solidify Jacks’ offensive line

Posted on August 28, 2013 by MaryO

The Big Uglies. The Big Nasties. The Hogs.

All terms used to describe a football team’s offensive line.

More often than not, it takes a special breed of player to be willing to do battle in the trenches.

Humboldt State right guard Dillon Reagan can without a doubt be bestowed with the title of special.

From overcoming Cushing’s Syndrome — a rare disease which left him with the use of only one lung — to the depression that was associated with the disorder, the Issaquah, Wash. native has definitely taken the road less traveled.

”The hardest part,” Reagan began, “was the first four months. (Doctors) didn’t know what was necessarily wrong with me. I was misdiagnosed as bipolar.”

Reagan displayed the initial symptoms of Cushing’s in 2009 after earning second-team All-State honors as a freshman at College of the Redwoods. A disorder caused by a tumor on one of the endocrine glands, Cushing’s causes a massive secretion of hormones which can affect behavior and physical appearance.

It did that and more to Reagan.

”I had full-ride scholarships taken away,” he said.

A full-body scan revealed a softball-sized tumor wrapped around his heart and left lung. Open heart surgery remedied the situation but his left lung useless.

In the midst of all this, Reagan also developed diabetes.

It would have been quite easy for the 6-foot-3 kid from Washington to call it quits. No one would have blamed him.

But an offensive lineman never quits.

”I never changed my approach,” Reagan said. “It’s a position of dominance and perseverance. Being an offensive lineman helped me get through all that. It helped me not feel sorry for myself.

”It was a long road back to full speed, but, your body reacts to how you push it.”

And push he did.

Reagan hit the weight room and transformed his body — which ballooned to 380 pounds due to Cushing’s — back to the muscular 300-pound frame he showcased his freshman year. He returned to Redwoods for his sophomore campaign and again displayed the skills that made him a Division I commodity.

”I ended up getting All-California,” Reagan said. “And with the use of only one lung.”

Looking to further his football career at the university level, there was really only one option.

”I wanted to go to a good program,” Reagan said. “That clear answer was Humboldt State.”

Reagan noted the close ties Redwoods and Humboldt shared as a deciding factor. His coach at CR was Duke Manyweather, a former HSU player himself. Reagan also sought the guidance of Humboldt State strength and conditioning coach Drew Petersen during his road to physical recovery.

Reagan asked to join Humboldt State as a non-scholarship athlete during the 2012 Spring semester and head coach Rob Smith and his coaching staff were more than happy to have him.

The following season, Reagan showed why.

As a junior, Reagan started 11 games for the Jacks providing a stabilizing force for a unit which paved the way for running backs to gain 2,152 total yards. He also earned second-team all conference honors.

It is amazing how high Reagan has risen after seeing how far he had fallen. An inspiration and uplifting athlete, it is easy to label him special.

Just don’t tell Reagan that.

”It takes me a little longer to warm up and get to game speed. But I don’t want to be treated differently than my teammates,” he said.

Entering his senior season, Reagan is being counted upon to be a stalwart right guard as he is only one of two returning starters (center and good friend David Kulp the other) from last season’s road graders.

Reagan is more than ready.

”As an offensive lineman, you show up every day, no matter what happens outside of practice, no matter what’s going on at home, no matter how beat up you are. You do it again and do it every day,” he said. “We set the tempo for the rest of the practice, rest of the game. If we don’t know up, it’s hard for everyone else to show up.”

Three positions are up for grabs on the Jacks’ front line. Reagan likes what he is seeing from the player stepping up to the plate.

”Start with tackle,” Reagan began, “(Jonathon) Rowe has made tremendous contributions at camp. He’s really growing up for his in a short time. Jonathan Bajet, he’s moving over from the defensive line, and he’s been really neck-and-neck for a starting guard position. David (Kulp), he’s a great guy to play next too, a great guy to have in your corner. It just works. We don’t have to say anything, we know what we’re doing. And (Jarrett) Adams has stepped up a bit. In the last few weeks he’s learned how to play right tackle.”

Humboldt is still knee-deep in competition in preparation for the Sept. 7 season-opening home contest against Simon Fraser. Reagan notes practicing daily against a defensive line which features returners Alex Markarian, Silas Sarvinski and Tommy Stuart, to name a few, helps both the O and D.

”They are adapting to our fast offense,” Reagan said. “You’re only as good as you practice. No one shows up game day and plays good. We challenge each other every day. It gets intense. But it’s all out of competition. Competition is a thing that drives a football team.”

If everything falls into place, all the Jacks’ hard work will result in one thing: Great Northwest Athletic Conference supremacy.

There’s simply no better lasting impression for Reagan.

”A GNAC Championship,” he said. “That puts you down in the books forever, that GNAC Championship.”

Title or not, it’s a pretty safe bet Reagan has already left a lasting impression.

 

Ray Aspuria can be reached evenings at 707-441-0527 or raspuria@times-standard.com. Follow him at twitter.com/AirUpsa707

 

From The Times-Standard

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