Pituitary Patient Resource Guide Fifth Edition

PUBLISHED BY THE PITUITARY NETWORK ASSOCIATION

© COPYRIGHT PNA 2013, ALL RIGHTS RESERVED

Library of Congress Catalog Card Number: 2013942368

ISBN: 978-0-9895570-0-9

PITUITARY NETWORK ASSOCIATION

P.O. BOX 1958, THOUSAND OAKS, CA 91358

PHONE: (805) 499 9973 FAX: (805) 480-0633

WWW.PITUITARY.ORG

TAX ID: 33-0530465

The fifth edition of the Pituitary Patient Resource Guide was compiled and published in Thousand Oaks, California by the Pituitary Network Association (PNA). Executive Editor: Lewis S. Blevins, Jr. MD, PNA Chairman: Robert Knutzen, MBA

All editorial and graphic contributions to this publication are used by permission from the authors.

All rights reserved. Written permission must be secured from the publisher to reproduce any part of the book in any form for sale or commercial distribution.

IMPORTANT NOTICE

This publication is intended to function as a patient/physician peer guide, not a medical journal, and should in no way be considered an endorsement of any particular organization, physician, treatment method, or medication. The information contained within this book represents the contribution and opinions of various individuals, and while the information has been reviewed by the PNA editorial committee for appropriate content, neither the PNA, its members and advisors, nor the editors and publishers and their staffs are responsible for the accuracy of any of the information contained herein.

As always, you must be responsible for your own life, heath, and medical care. We urge you to investigate, ask questions, and make your own informed decisions.

In addition, neither the PNA scientific advisors nor the medical members of the PNA have been responsible for the compilation, placement or wording of the information contained within the guide.

Contents

SECTION ONE: INTRODUCTION

Introduction

By Lewis Blevins, M.D.

Chairman’s Foreword – Welcome to the Future

By Robert Knutzen, M.B.A., Chairman, PNA

Foreword

By Sharmyn McGraw, Pituitary Patient

The Powerful Patient: How to Rise Above Adversity

By Robert Knutzen, MBA and Annie Hamilton

How Patients Can Help Prevent Misdiagnosis

By Tammy Mazzella, PNA

Board of Directors

Scientific Advisory Board

PNA Mission Statement

SECTION TWO: SYMPTOMS AND SIGNS OF PITUITARY DISEASE

Chapter One: Types of Pituitary Disorders and their Manifestations

Introduction to Pituitary Disorders: Pituitary Tumors and Hypopituitarism

Daniel F. Kelly, M.D., Garni Barkhoudarian, M.D., and Pejman Cohan, M.D.

Acromegaly – GH-Secreting Adenoma

Adult Growth Hormone Deficiency

Antidiuretic Hormone Deficiency Causing Diabetes Insipidus

Craniopharyngioma

Cushing’s Syndrome and Cushing’s Disease – ACTH Secreting Adenoma

Deficiency of ACTH and Cortisol

Deficiency of LH and FSH (Hypogonadotropic Hypogonadism)

Endocrine-Inactive (i.e., Non-Functional) Adenoma

Hypopituitarism

Prolactinoma

Rathke’s Cleft Cyst

Thyroid Hormone Deficiency (Hypothyroidism)

TSH (Thyroid Stimulating Hormone) Secreting Tumor

The Prevalence of Pituitary Adenomas: A Systematic Review

By Shereen Ezzat, M.D., Sylvia L. Asa, M.D., Ph.D. William T. Couldwell, M.D., Charles E. Barr, M.D., M.P.H., William E. Dodge, M.S., M.B.A., Mary Lee Vance, M.D. , and Ian E. McCutcheon, M.D.

Can Pituitary Tumors Be Inherited?

By Robert F. Gagel, M.D.

Chapter Two: The Diagnosis of Pituitary Diseases

Acromegaly

By Shereen Ezzat, M.D., F.R.C.P.(C). F.A.C.P.

Prolactinoma

By Michael O. Thorner, M.B., D.Sc., Edited by Shereen Ezzat, M.D.

Cushing’s Syndrome/Disease

By Shirley McCartney, Ph.D., Christine Yedinak, D.N.P., and Maria Fleseriu, M.D., F.A.C.E.

Cushing’s Syndrome: An Update

By Laurence Katznelson. M.D.

Gonadotroph And Other Clinically Nonfunctioning Pituitary Adenomas

By Peter J. Snyder, M.D.

Multiple Endocrine Neoplasia Type 1 (MEN1)

By Stephen J. Marx, M.D.

Autoimmune Pituitary Disease or Lymphocytic Hypophysitis

By Patricia Crock, M.D., M.B.B.S., F.R.A.C.P.

Addison’s Disease

By Eileen K. Corrigan, N.I.H., Retired

Pituitary Cancer

By Hasnaian M. Khandwala, M.D., Ian E. McCutcheon, M.D., F.R.C.S. and Keith E. Friend, M.D.

Chapter Three: Mental and Physical Complications of Pituitary Disease

Heart Problems in Acromegaly

By Philippe Chanson, M.D.

Pituitary Disease: Association With Cognitive Dysfunction

By Wesley P. Fairfield, M.D.

Headaches and Pituitary Tumors

By Peter N. Riskind, M.D., Ph.D.

The Oral Cavity and Pituitary Tumors

By Eric C. Sung, D.D.S.

Keep an Eye on Yourself

By Nicholas Volpe, M.D.

Surgical Correction of the Acromegalic Face

By J. Timothy Katzen, M.D., Mohammed S. Kabil, and Hrayr K. Shahinian, M.D.

Human Sexuality

By Robert Knutzen, M.B.A.

Hormones and Sexuality

By Sandy Hotchkiss, M.S.W., BCD

The Mental Health Side of Pituitary and other Neuroendocrine Disorders

Linda M. Rio, MA, Marriage and Family Therapist

Sick of Being Sick and Tired: When Depression is Not Depression

By Michael A. Weitzner, M.D.

The Stress Response and Its Implications for Pituitary Tumors

By George Chrousos, M.D. and Robert Bock

Emotional Disorder Medications

Chapter Four: Common Issues for Women

The Menstrual Cycle – Getting the Vital Respect it Deserves

A Compilation of Abstracts Presented

Psychosocial Aspects of Pituitary Disease in Women

By Donna E. Stewart, M.D., F.R.C.P.C.

Pituitary Disease During Pregnancy

By Martin N. Montoro, M.D. and Jorge H. Mestman, M.D.

Chapter Five: Common Issues for Children

Panhypopituitarism in Childhood

By Mitchell E. Geffner, M.D.

Pediatric Pituitary Tumors

By Cheryl A. Muszynski, M.D.

Chapter Six: Common Issues for Men

Androgen Deficiency and Hypogonadism

Chapter Seven: Testing

Progress in Pathology: What We Have Learned About The Causes of Pituitary Tumors

By Sylvia L. Asa, M.D., Ph.D.

Laboratory Assessment of Pituitary Gland Function

By Jon M. Nakamoto, M.D., Ph.D. and Delbert A. Fisher, M.D.

SECTION THREE: TREATMENT OF PITUITARY TUMORS

Chapter One: Medical Options

Indications for Treatment of Microprolactinomas: An Update

By Anne Klibanski, M.D.

Chapter Two: Surgical Options

Management of Pituitary Adenomas

By Edward R. Laws Jr., M.D., F.A.C.S. and John A. Jane Jr., M.D.

Surgical Management of Pituitary Tumors

By Sandeep Kunwar, M.D. and Charles B. Wilson, M.D., D.Sc., M.S.H.A.

Pituitary Surgery: The Fully Endoscopic Supraorbital Approach

By Hrayr K. Shahinian, M.D. and Yoon Ra, M.S.

Skull Base Approaches for Pituitary Tumors – and Intraoperative MRI to Confirm the Amount of Pituitary Tumor Removal

By Robert J. Bohinski, M.D., Ph.D., Michael J. Link, M.D. and John M. Tew Jr., M.D.

Endonasal Endoscopic Pituitary Surgery: Evolution of the Classic Transsphenoidal Approach

By Daniel F. Kelly, M.D., Garni Barkhoudarian, M.D., Chester F. Griffiths, M.D., F.A.C.S., and Kian Karimi, M.D.

Transsphenoidal Resection of Pituitary Adenomas in an Intraoperative MRI Unit

By Claudia H. Martin, M.D., Richard Schwartz, M.D., Ferenc Jolesz, M.D., and Peter Mcl. Black, M.D.

Surgical Management of Clinically Nonfunctioning Pituitary Adenomas

By Joshua Rosenow, M.D., and William T. Couldwell, M.D., Ph.D.

Chapter Three: Radiotherapy Options

Gamma Knife Radiosurgery for Skull Base and Pituitary Tumors

By Ajay Niranjan, MD, MBA, L. Dade Lunsford, M.D., and John C. Flickinger, M.D.

Neuroendocrine Complication of Radiation Therapy for Non-Pituitary Tumors

By Anne Klibanski, M.D. and Howard H. Pai, M.D., FRCPC

Chapter Four: Hormone Replacement Therapy

Pituitary Hormone Replacement in Women

By Jennifer Larsen, M.D.

By Bengt-Ake Bengtsson, M.D., Ph.D, updated by Dr. Peter Sonksen

Androgen Deficiency in Women With Hypopituitarism

By Karen K. Miller, M.D.

SECTION FOUR: LONG-TERM CARE

Pituitary Tumors – Long Term Outcomes and Expectations

By Edward R. Laws, M.D., F.A.C.S., and John A. Jane Jr., M.D.

By Brooke Swearingen, M.D.

SECTION FIVE: THE PATIENTS’ EXPERIENCE WITH PITUITARY DISORDERS

Patient Story

By Sharmyn McGraw

Patient Story

By Esperanza Ramirez

SECTION SIX: PATIENT RESOURCES

Chapter One: Physician and Hospital Listings

Physicians

United States

International

Hospitals/Medical Centers

United States

International

Pharmaceutical Companies/Corporate Listings

Chapter Two: Additional Help and Resources

A Layman’s Quick Checklist For The Acromegalic’s Symptoms, Tests, Remedies

Learn More About Clinical Trials

Glossary

SECTION ONE

INTRODUCTION

Introduction

By Lewis S. Blevins, Jr., M.D.

I am delighted to have worked with so many fine individuals to prepare this fifth edition of the Pituitary Patient Resource Guide. This work came to fruition as a result of the passion and drive exhibited by the staff and volunteers affiliated with the Pituitary Network Association; many of them are pituitary patients that have benefited greatly from the organization and its publications. I greatly appreciate the support of contributing authors, many of whom revised their chapters on short notice, and especially those who’ve contributed new works for this particular edition. It is our hope that this guide will serve as a resource for those with newly diagnosed and established disorders of the pituitary. May it also serve to educate not only patients, but their families, healthcare professionals, Allied health professionals, and friends of those affected by disorders of the master gland. Knowledge is the greatest defense against any illness. Educate yourselves...... and share what you have learned!

Lewis S. Blevins Jr., M.D.

San Francisco, California

July 2013

Chairman’s Foreword

By Robert Knutzen, PNA Chairman

WELCOME TO THE FUTURE (An overview and introduction)

It’s a strange way to introduce a book but strangely, in this case it fits the situation perfectly! In working with Tammy, Jackie and Fran in our offices under the guidance and Editorship of Dr. Lewis Blevins, I am again reminded of how far and wide we will travel to find the best Present and Future paths to follow in order to bring you, your family and current physicians as up-to-date as we can on modern Pituitary Medicine.

Very recently I wrote a foreword to another book and I pointed out that the latest and best information is sometimes too big a leap for both Patients and Physicians to immediately grasp. Wasn’t what I heard and learned 5-10-20 years ago about Pituitary Medicine truthful and good enough? Yes, it is/was, at the time you were told. However, the advances in all forms of Pituitary Medicine (and all other fields of Medicine) is moving so swiftly as to leave a lot of previous beliefs and knowledge in the dust, no longer of value to you and your physicians/healthcare providers. Some of it, in fact, may be injurious to your well-being.

Hence, welcome to the future, if only for a few months! Being as-good-as, is not good enough. Being ahead and striving to be better-than, is what we aim for. As a matter of fact, I am convinced that the PNA saves many, many lives every year, because we aim to bring the future of medicine to you. Unfortunately, most of us/you, with Pituitary Disease/tumors were attacked by a sometimes relentless foe which requires time, effort (and Money) to combat. BUT, with this new book you should win a lot faster and easier than before. Initial symptoms may be severe but the diagnosis, treatments and recovery should be a lot easier, less debilitating, less painful and, hopefully, a lot less costly and hopefully only have to be done once!

In future years we sincerely hope that we will be in the vanguard of bringing you better tidings, better results and a speedy return to normalcy in your personal and professional life.

On behalf of all of us at the Pituitary Network Association, we deeply and sincerely thank our present Pilots for introducing us to the future and a better life. As was said a few years ago by our now older Generation; KEEP THE FAITH, BABY!

Foreword

By Sharmyn McGraw, Pituitary Patient

I started my journey to help others affected by pituitary tumors and/or hormonal disorders in April 2000. I never thought in a million years I’d still be beating the same dead horse, and although we have moved a golf ball about one inch closer to the sun, we still have only touched the tip of the iceberg with understanding the relationship between the pituitary gland’s function and our quality of life.

We need to keep moving forward; we need to keep pushing for more public awareness and for better education for our medical communities, we need to stand up for a disease that is so grossly misunderstood but affects so many lives. Making a difference, caring about people who are very sick, constantly trying to educate doctors and running a non-profit is challenging, to say the least, but the dedication and hard work the PNA has done and continues to do doesn’t just make a difference, it actually saves lives. There truly has not been one other organization that has made the powerful and positive impact that the team has made at the PNA.

Unfortunately, because of the lack of public awareness and education, someone suffering with an endocrine disorder can take years to get a correct diagnosis. And, in far too many cases, he or she never gets a diagnosis at all. But for those of us lucky enough to have found a correct diagnosis and the experts to help us; I am sure we all agree: so much more needs to be done.

I dedicated myself to helping others and I could not have done it without the help and support of the PNA. I often have people contact me and say, Sharmyn I want to do what you do. I want to help patients, so if you ever need me to talk to someone that’s sick or just had surgery, I will be happy to talk with them. I can tell them my story. And over the past decade I have heard this same thing many times. People want to help and they think that others need to hear their story. And they do – Amen to that! But other patients don’t necessarily need to hear your story – you’re singing to the choir. You need to tell your story to the public, to your community, and there’s no time in history that has made it any easier than today with the power of Social Media. If you want to help, then do it.

There shouldn’t be one person who’s had a pituitary tumor who doesn’t have the link to the PNA posted somewhere on their social media page. This patient resource guide is the only one of its kind. It’s written by the cream-of-the-crop pituitary experts and it will help save lives. Send everyone you know the link to get a copy of this guide book as well as information about pituitary tumors and/or hormonal disorders. Because if you want better education about pituitary disorders, if you believe patients deserve better healthcare, more pituitary experts, then just do it.

Having an unexpected illness, or watching someone close to you suffer, is devastating. The overwhelming frustration I think we have all felt and wrenching anguish often spurs ordinary people to make extraordinary changes. So do it!

I know from my own personal experience, (having suffered with Cushing’s disease for a very long time before getting a correct diagnosis) that education about pituitary disorders has been extremely empowering. I’ve been facilitating a pituitary patient support group under the direction of Dr. Daniel Kelly at the Brain Tumor Center in Santa Monica for over a decade and it continues to grow stronger each year. Dr. Kelly and our team are dedicated to making a difference in patient’s lives, other pituitary centers of excellence across the country mentioned in this guide book are dedicated to improve patients’ lives, and the PNA is dedicated to making a difference for patients, their families and their medical team – Are you?

The material in the Resource Guide is invaluable. It’s written by some of the top pituitary medical experts in the world. In this current age of the internet, we have so much information at our fingertips. But over and over I meet people who are horribly misinformed. That is why I value the work the PNA has done to get current and accurate medical information to patients and medical professionals.

With the right tools and the willingness to try, I believe ordinary people can make an extraordinary difference for others as well as themselves. The Resource Guide is an excellent tool that will benefit anyone one who reads it. Will you be part of the solution and join our efforts?

Thank you and many blessings,

Sharmyn McGraw

www.hormones411.org

THE POWERFUL PATIENT

How to Rise Above Adversity

By Robert Knutzen, MBA and Annie Hamilton

Pituitary Network Association

Many newly diagnosed patients find themselves feeling as though they’ve been thrown into the deep end of an ocean of information, treatments and symptoms with no purpose or end. We’re here to put answers in place, some order if you will, as there is nothing more important to the patient – both novice or seasoned – than a fresh and willing attitude toward your own wellness and health.

It’s also helpful to your physicians, surgeons, families and friends for you to maintain an attitude of openness as it helps them treat and understand you with greater efficiency. Obviously this makes life easier for everyone.

There are three distinctive but equally important aspects to the hormonally challenged patient. Dealing with the medical community is an interesting while often daunting task although the presence of a tumor does take on overriding importance to physicians. Often times, quality of life can be compromised not by the removal of the tumor itself but by the inadequacy of the follow up of your physicians understanding of the need to regularly monitor your hormone replacement. Medications in proper doses, perhaps altered at regular intervals should be offered at either annually, bi-annually or quarterly appointments, depending on your illness and severity.

If your physician is not offering this, it’s probably time to seek out a new medical provider. Your medications probably need adjusted if you’re lethargic, having unusual mood swings, out of control (or frequent) headaches, seizures, irregular menses/periods, depression that isn’t lifting, you’re unable to eat or cannot stop eating, or feel like taking your life. Please don’t be embarrassed – your life is worth getting help, isn’t it? Pituitary disorders are etched in hormones and this stuff is completely biochemical and if things aren’t working then naturally you’re going to feel strange if the train is temporarily derailed. You’d be surprised how wonderful it feels once your hormones are once again balanced. Give yourself a chance to view life through the lenses of a patient that’s been treated for years, successfully and doing well. It can be very uplifting. You are worth it and we’re with you every step of the way.

By now you’ve probably heard conflicting stories about the ‘rarity’ of pituitary disorders and aren’t sure what to believe. Since our inception, we’ve set out to distinguish fact from fiction and, in fact, commissioned a study with many of the world’s leading endocrinologists, neurosurgeons, pathologists and medical mathematicians/statisticians to arrive at a reasonable, recognized, starting place. The Prevalence of Pituitary Adenomas, a Systematic Review, by Shereen Ezzat MD, at the University of Toronto; Sylvia Asa MD, PhD. At the University of Toronto; William Couldwell, MD. At the University of Utah; Charles E. Barr, MD, at MBA at Roche Laboratories, Inc; William E. Dodge, M.S., MBA; Mary Lee Vance MD at the University of Virginia; and Ian McCutcheon at the University of Texas does exactly that. We have the answers and no evidence exists to the contrary.

The study was supported by the Pituitary Network Association and published by the American Cancer Society in June 2004.

In reviewing dozens of cases over the past 80 years on the prevalence of Pituitary tumors alone (not counting Addison’s disease, Sheehan’s syndrome, inflammations, enlargements, etc) our experts reached a consensus; at least 16.7 of the populace develops or harbors pituitary tumors. Many estimate it’s much closer to 30%. The clinical significance of such tumors only reflect what individual physicians find significant. (which does not mean it’s necessarily accurate) if your own doctor doesn’t believe your symptoms (headaches, weight gain, personality changes, rage, apathy, weight loss, weakness, depression, lactation or inability to lactate, hot flashes, headaches, skin problems, mouth sores, allergies, sexual dysfunction, etc) are clinically significant and discount it then you could continue to suffer as a result of inadequate treatment.

To us, as patients with twenty years of interacting with hundreds of thousands of other patients – that is hardly an acceptable conclusion. All pituitary tumors and conditions, in the absence of clear and proof of medical cause of the patient’s discomfort, must be viewed as significant.

We share some patients’ information with various world experts in medicine, and collect and read the experts’ opinion and findings from around the world. Yes, headaches, joint pain, mental/emotional disorders, sexual dysfunction and eating disorders are very often related to pituitary tumors and hormonal dysfunction. The overwhelming numbers of pituitary patients are not hypochondriacs nor are they ‘crazy’. Sick? Yes. In need of expert care? Absolutely. Can they improve their quality of life? You bet! Will their outcomes improve? Certainly.

DIAGNOSIS

Getting a correct diagnosis is critical and too often the most difficult part. Pituitary medicine is the miracle branch of hormonal health care. It’s hard to believe but these small, clinically insignificant by the uniformed often turn into Cushings disease, Acromegaly or something equally sinister, often causing irreversible damage simply because someone didn’t take the time to be curious enough.

As a rule, always at a minimum, get a second opinion. Without exception the opinions must be from experts. In other words, a general practitioner probably is not a pituitary expert. They may wear a lab coat and have an M.D. behind their name but unless they specialize in the field of pituitary medicine you must not deviate from this rule. It will always come back to haunt you later on.

For patients and their families and friends, pituitary disease is much like watching a slow-moving train wreck – one that your average physician seems ill equipped or too untrained to prevent, understand or appropriately treat correctly. The burden of finding the right specialists for your situation will rest ultimately with you and you alone and you must take responsibility for this seriously. We’ve taken some of the guesswork out of it for you with our website at www.pituitary.org (you can also track your symptoms, read about various disorders and look up blood tests to ask for so you’re better informed)

Don’t try to be your own doctor or diagnose yourself online, this is a dangerous game to play. Take the time to find specialists and listen to what they have to say. Take a loved one or friend with you who can take notes while you listen to them and offer moral support. Don’t get discouraged, never give up. The average time between onset of illness and diagnosis is extraordinarily long. With your help, we will create sufficient awareness in the medical community and the public to reduce this time from an average of seven years to one or two.

Practitioners normally use the term benign to differentiate non-cancerous from cancerous tumors. We of course disagree with this distinction as noncancerous does not mean harmless or unimportant and it’s often not the tumor that kills you, it’s one of the hundred of related disorders from cancers to stroke that follows the initial tumor that happens because of the tumor.

Most patients seek help for various ailments from many providers from family doctors, gynecologists, internists, neurologists, chiropractors, neurosurgeons, dentists, eye doctors, skull base surgeons and mental health professionals etc., before a curious M.D. has a eureka moment. In far too many cases, medicine is prescribed, treatment is recommended or the patient is simply told, there is an incidental finding of a microadenoma, but not to worry, it’s too small to be of concern! that is when you know you’re in the office of someone who has no idea of what he’s talking about.

A large number of both Prolactinomas and Cushing (ACTH) causing tumors, for instance, are too small to even be seen on an MRI. (30 % or more don’t show up at all) An MRI is not the ideal way to screen for a pituitary tumor. Your physician should give you a physical, take a detailed medical history with complete current condition and use blood tests (sometimes urine also) or sputum/spit to aid in diagnosis. An MRI (with contrasting agent) is ideally used to determine the size and direction of growth of the tumor; a CAT scan just doesn’t have the same value or accuracy.

Yes, you can and should protect yourself. In addition, medical science is making rapid strides toward helping us. Even good information travels slowly in the medical community, so your family doctor may not know about these advances for years after their discovery. It’s up to you to stay informed which is precisely why we were formed in 2002.

TREATMENT

Diagnosis, treatment and maintenance usually depends upon the team approach, an extremely serious modus operandi on your part, and the demand that no corners are cut on the part of either party. What this means is complete communication at all times. You must insist upon professional treatment by your endocrinologist, surgeon, radiologist and other professionals so you can get on the road to wellness. It also means when recommendations are made you must follow them in order to not cut corners with your own care. What this means, of course, is that with careful treatment and open communication you can usually return to the state of hormonal balance that you enjoyed prior to the onset of your tumor or disorder. Sometimes this entails surgery, radiation, pharmaceuticals or a combination of the above. That said, experts usually treat and control your disorders but often you’re a patient for the duration of your lifetime. To get your blood/hormone readings into a normal range requires a highly specific skill set, one altogether overlooked by the mainstream medical folks and putting your house in order is another issue altogether.

We firmly believe that the appropriate treatment regiment must include the nurturing of the mind. It’s been understood throughout history from Hippocrates to Dr. Harvey Cushing that in the treatment of the body for various illnesses one must not overlook the importance of caring for the mind. In this text, pituitary/hormonal disorders are likened to a slow-moving train-wreck and the damage can be every bit as dramatic and catastrophic. If you were to look carefully behind the door of most pituitary/hormonal diagnosis, you might find patients with shaky marriages, unstable friendships and family relationships, career lows, unfinished dreams, a collage of physical and psychosocial complaints, eating disorders, chemical dependencies, depression, feelings of worthlessness and apathy. It’s important to understand that beyond diagnosis and treatment, patients must be responsible for the repair and maintenance for these issues. It can be managed successfully with therapy, pharmaceuticals, proper nutrition, journaling, exercise, and open communication.)

Reaching out to others in similar circumstances is helpful and recommended, as long as the environment remains positive and constructive. Letting go of lingering anger is an important part of moving life forward as harboring negative feelings only breeds anxiety and anger. Such feelings can lead to further illness, poisoning others around you. This will mean different things to different people; for some it may mean forgiving your doctor(s) for not diagnosing you as early as you should have been treated, for others, it’s creating an understanding within your family that allows you to get well on your own time while keeping your relationships intact. These are personal and individual areas of concern that should never be dismissed.

Treatment is far reaching and may touch on areas in both body and soul. First and foremost, without accurate diagnosis, your treatment is likely not worth much. Your endocrinologist should be advising you on medications and treatments on a regular basis. It’s imperative to see an endocrinologist who is an expert, following any surgical event, for hormonal adjustments and who will follow you for rest of life care. This person must be the health gatekeeper of your body, the one who should understand everything that is going on from stem to stern. Never be embarrassed in their presence. If you are – it’s time to find a new gatekeeper.

This person will advise you of where you need to go for adjustments as needed (surgeries, therapies, mental help, bone, oncology, etc) At all times, keep in mind that only the best trained in the field is good enough for someone with your particular area of symptoms – most physicians simply don’t see enough pituitary patients (or they see them but cannot properly identify their disorders so on to the next physician they go!) to qualify as a true expert and as such, we must see the subspecialty in pituitary medicine in order to not fall through the cracks further. (do not let your family, friends or co-workers convince you that the ‘best doctor in town’ is the person you ‘simply must see’ – trust us. The person you must see is the expert trained pituitary specialist they’ve probably not heard of who may be hard to get an appointment with but is worth it once you’ve made it to their office because you’re likely diagnosed correctly the first time) Note: bedside manner is less important than expertise and skill. You do not need a specialist who holds your hand and agrees with your opinions, you’re there for their skill set and healing, not to make friends.

Careful treatment/examination could/should include a bone scan, dermatological evaluation, colonoscopy, and most definitely an opthalmological exam thorough being the operative word. Of course, each patient and case is unique and different. There are no quick fixes or cookie cutter approaches yet available for pituitary treatment.

MANAGING THE INSURANCE MESS

It’s logical that most insurance carriers struggle against paying claims for two reasons; both of which are critical to understand in order to receive treatment as well as for practical reasons, such as getting your doctors paid. Obviously with the passing of Obamacare, the structure of insurance contracts will likely change somewhat and it’s an area we’ll be watching very closely.

Insurance carriers earn money based on reserves required to pay claims on behalf of their policyholders. When the claims paid are less than the funds pooling in, that yields a profit. When claims paid are greater than funds pooling in there will be a shortfall, which presents business issues for the insurance carrier, their financial holdings and stockholders. It’s commonplace for carriers to ‘hang on to claims’ for as long as possible as the longer the funds are allowed to sit in reserves the more money will be earned on the funds that are pooled. Many states have enacted legislative measures to ensure that claims are paid in a timely fashion (Medicare claims must be paid within a few weeks) or the insurance company will be subject to fines and penalties. The rules differ from state to state but it’s not unusual for doctors to reject insurance contracts unless they obtain favorable financial terms in writing, up front. What it means to you, the pituitary patient is that you must fight to have your claims paid.

Note: ask your insurance carrier what their rate of pay is for physicians is and if they refuse to tell you, your physician likely is more than happy to spill the beans. Tip: letter writing campaigns often go a long way, especially if you get the press involved.

Few physicians understand pituitary/hormonal disorders and the same is true of insurance carriers, unfortunately. They must understand fully what it is they are paying for and it is your job to inform them completely so they can help you. Much of the headache can be eradicated by taking a few steps: 1) Have a copy of your insurance policy sent to you and read through the entire document, fine print and all. Knowledge is power, especially where insurance rights are concerned. 2) Obtain pre-authorization, in writing, whenever necessary, from your carrier (usually not necessary with a PPO) as well as from a third party administrator in charge of claims processing. 3) Ask your physician (s) for a list of codes he or she will be using and submit them to your carrier with an explanation as to what each means. 4) Keep a list of state and federal authorities who can jump in and help when you need them (PNA members can easily access such lists through our website at www.pituitary.org)

Don’t forget to copy HR directors when you write appeal letters. Their job is to handle insurance issues for their companies and group insurance policies review on an annual basis. For policies of companies that are problematic, many will terminate contracts for more favorable arrangements for employees. (the squeaky wheel, etc – especially if the individual is a valued employee – so be polite but firm in your protests!) the same goes for companies that are known to underpay physicians. If your doctor lets you know they’ve dropped their contract with your insurer due to shoddy contract conditions, LET YOUR EMPLOYER KNOW, get your pen writing! Reimbursement and regulatory problems have plagued physicians for many years now and it helps for the patient community to get involved. This is an area where I’m louder than ever! Too many people act like doctors should give away free care (most give away more free care than any of us would be comfortable knowing) yet it’s evil for them to earn a living? Spare me!

You will find often that your insurance policy doesn’t allow you to be treated by experts and ignorance of these very issues can stand in the way of treatment without persistence. It’s up to you to copy pages from this resource guide and help your insurance carrier to understand what you’re dealing so that they’re able to help you. One way around this sticky situation is to make sure you have a PPO insurance policy which allows you to seek treatment wherever you wish rather than only within the confining guidelines of an HMO. One is of course more expensive but the freedom is worth it.

In each state in the US and we hope in most provinces and countries there is an insurance commissioner or an appeals board that you can turn to for help. You may need an attorney to help you –not to file a lawsuit – but to negotiate on your behalf with the powers that be in order to ensure that you receive the best possible and most cost effective treatment. Keep in mind that it is far less expensive to treat you correctly once, than haphazardly and incompletely treat you multiple times, often with dreadful results. Thousands of patients have been misdiagnosed (or under-diagnosed) and mistreated (or under-treated) multiple times, with financial, physical, and emotional costs that are overwhelming to patients, to their families and friends, to employers and to society in general.

OUR ADVICE: FIGHT FOR YOURSELF. YOU MUST BE YOUR OWN ADVOCATE.

REST OF LIFE CARE

With appropriate treatment and therapies, there is no reason why you should not live a long and happy life. In order to achieve this very commendable, yet achievable goal it is imperative that you form a rest of life team of experts to keep an eye on you after the acute phase of treatment is over. The thought leader should be your endocrinologist. (You may only need to see him twice per year or Annually, again, depending on your illness and the severity) your family physician should be kept in the loop regarding your treatments and he or she should coordinate notes and records with your endocrinologist as well as the other specialists who care for the others who care for the rest of your body (eyes, sexual health, emotional health, heart/lungs, skin, teeth, virtually everything) check for cancer regularly (breast, vaginal, prostate in particular) leave nothing to chance.

EMOTIONAL WELL BEING

One aspect of pituitary/hormonal disease is that often overlooked, even by those with the very best of intentions, is mental/emotional health. It is a subject about which a few doctors know a lot, but most know very little. It is an issue of immense importance to you, your family, friends and society in general. It does not do anyone any good to cure you (chemically/hormonally) if you don’t also get well enough to function normally and rationally for the rest of your life! You are entitled to enjoy your life, job and family and function as you did before. There is no shame in taking properly prescribed medicine which permits you to be calm, relaxed, sharp and functioning at normal speed. Paying attention to the well-being of your family relationships, career and general social interaction is extremely important and must be a part of your recovery plan. Bridges which you may have thought were forever burned behind you can be rebuilt and relationships restored.

Seek the help you need from experts in the fields indicated above and know that a pituitary/hormonal tumor/disease is not the end of the highway, merely a fork in the road. I like to keep in mind Olympic gold medalist Scott Hamilton’s words, "Everything that I’ve ever been able to accomplish in skating and in life has come out of adversity and perseverance"

How Patients Can Help Prevent Misdiagnosis

By Tammy Mazzella, PNA

Recently an article caught my eye and it reminded me about how important the right diagnosis really is. The article was about Misdiagnosis and how it is much more common than you think. Those of us who have a pituitary or hormonal disorder know that it is very common. We know that it sometimes takes years to get a proper diagnosis. But the interesting part of the story was about how the problems start with communication. Because pituitary and hormonal disorders are still considered rare in most cases the patient has not heard of these disorders and in too many cases neither has their doctor. Here at the PNA we know that pituitary and hormonal disorders are not rare, just rarely diagnosed properly. One in five people have some form of pituitary or hormonal disorder, that does not sound rare to me.

So how can we help ourselves get better care from our doctors? Well if you are reading this you have already made a good start. Purchasing the Pituitary Patient Resource Guide will give you the resources necessary to get the best care available. Joining the PNA is another great way to keep up with new breakthroughs in pituitary medicine. It is an excellent way to find out about the medical experts available that have experience with pituitary medicine. PNA membership also gives you access to a large community of people who understand what you are going through and can help guide you through your journey to better health. If you are not a member yet, become a member, work with us to help all pituitary patients get the attention of the medical profession and the health care industry. Help us spread awareness about the symptoms and help other patients get an early diagnosis.

There is a page on our website that I refer to quite often. The page starts with a list of symptoms:

Headaches, particularly with a sudden onset and in the center of the head

Sexual difficulties (painful intercourse, low libido, erectile dysfunction)

Depression and/or changes in mood (anger, depression, anxiety), bipolar diagnosis

Recent and or sudden onset of family, friend relationship difficulties in conjunction with physical symptoms on this list including loss of relationships resulting in isolation, loneliness

Infertility

Growth abnormalities/changes especially of the hands (large fingers), feet, head, jaw and separation of teeth

Obesity (especially in the central area of the body), especially weight gain over a short time, rounding or moon face, a large bump/hump on the back on the neck/back. Note: if you suddenly are unable to manage your weight with proper diet and increased activity/exercise, this is a hormonal red flag.

Eating disorders: excessive weight loss, gain, bulimia. (loss of interest in food or uncontrollable urges to eat)

Vision changes including loss of peripheral vision, blurring etc. especially with sudden onset

Skin thinning, stretch marks, bruising easily, acne and cuts or abrasions/pimples that don’t seem to heal

Carpel tunnel syndrome

Menstrual difficulties (irregular periods, discontinued, painful, no ovulation, anything not usual for your cycle), early menopause

Fatigue, weakness in the limbs, general muscle weakness

Hypertension

Arthritis, aching joints, osteoporosis

Loss of sleep, changes in sleep cycle

Memory impairments, poor concentration

Unusual hair growth (on chest, face etc.) or hair loss (falling out in clumps)

Skin and hair that becomes extremely dry, scaly and discolored.

Lactation not associated with pregnancy (breast leakage...even for men)

History of emotional/physical trauma and/or chronic stress

Depersonalization i.e., feeling detached from one’s own body/mind

This is a huge list of symptoms and one or more of these symptoms may not mean you have a pituitary tumor, but they should prompt further investigation. So often these symptoms are treated as individual problems and not looked at as a whole picture. Sometimes you need to step back