Monday, December 19, 2011

The "Book"

This Blog is a "Warehouse" storing my thoughts and experiences related to "Living with AIP".
Then to turn my Blog into a Book...
As time has passed it has become really really hard for me to try to pull it all together. I feel eroded from the inside out.
Editing, formatting, publishing and marketing a book feels like mission impossible with my current challenges.
My nervous system has taken a serious hit from the chemical corrosive nature that chronically Active AIP creates. After trying to "push through it" and battling with denial... I am learning to accept that I have to respect my limitations... and try hard to make the most of better days.
Finishing a well written book that will help others navigate their rare disease is still my personal goal!!

But...
I think that I need to consider re-investing in regular Blog Writing...
Because it is therapeutic... it is good for me.

My family is as supportive as they know how to be... but writing allows me to vent and sort my thoughts in a calmer... more organized... less dramatic... more healthy fashion.

Since October 2011 I have reached out to "Modern Medicine" in hopes that they could help me feel better... I had done that multiple times in my life with statistically poor help to stress benefit. This time has turned out to be little different... now what???

More Spirituality...
More Alternative and Cognitive/Mental Therapies...
Daily Vegetable & Fruit Juicing???

I am tired out...
but soldiering on in hopes of learning to dance well with my health situation.

December 2011 Letters to Doctors

December 1, 2011
Dear Doctor__ (a letter faxed to my primary Care Physician),
First, I want to wish you and your family a very happy holiday season. You have been a kind, invested partner in my health journey and I sincerely appreciate you. Next, I want to update you on my ongoing health situation. Since our last meeting I have continued to struggle with daily health issues that inhibit my quality of life. I remain unable to work. I now regularly see you for Primary Care (Q6ms), a Hematologist (Q2wks), a Hepatologist (Q6ms), a Psychiatrist (Qmo), a Therapist (Qwk), an AIP Specialist (PRN) and an Endocrinologist (Q12ms). I also participate in monthly SKYPE meetings as part of my participation in the Mount Sinai NYC long-term AIP clinical study. I follow a gluten free, dairy free diet (reality= 90% of time). I avoid all AIP triggers within my control. Regarding current treatment… I take Gabapentin 300mg TID, Phenergan as needed for nausea and agitation, Estrace QWK, Naproxen PRN for moderate pain, and weekly Dextrose infusions the day after Estrace . The Hematologist is collaborating with Mount Sinai regarding AIP Tx Mgmt… we are considering regular Hematin infusions… but are trying the dextrose first because I seem to have good improvement with dextrose alone (1 to 2 days of lasting benefit vs little to no lasting benefit with Hematin). Prior to Thanksgiving I went to the hospital’s Out-Pt Special Procedures Unit to have an Ultrasound Guided PICC Insertion… they tried 3 times and failed x3. I have been getting peripheral lines each Dextrose infusion at present. I have follow-up visit with the Hematologist tomorrow (12/02/2011), hopefully we will make a new plan.
The daily neuropathy pain in my limbs has significantly decreased with the Gabapentin. My mental health and cognitive ability continue to be a challenge. The Psychiatrist has been following me with monthly visits and I now see a good Therapist weekly.
With a “heavy heart” I went to the Social Security Office 11/23/2011 and officially applied for long-term disability.
I am scheduled to see you again in January 2012, but wanted to connect with you to let you know where I am in my health journey now.

Best Wishes & Blessings~
T. Suzanne Jaynes-Emmert

December 2, 2011
Dear Dr. ___ (a letter read to my hematologist at office visit),
At times I have trouble finding my words when I need them. I have found that writing out my thoughts ahead of time can greatly improve the clarity of my communication. Please hear me when I say how much I admire your knowledge and medical insight. We have partnered in my health journey for almost 4 years now. I respect you and trust you to take care of me when I am at my worst. Due to the neuro-corrosive nature of my illness, my intellectual stability has become unreliable. It is becoming increasingly difficult to manage my disease as independently as I use to. I am at a point where I must rely on others to help navigate my path. The problem is that I have a growing lack of confidence due to multiple human errors and a lack of both investment and thorough follow-through. My anxiety and stress level have been negatively impacted by seeking medical help. I feel scared and lack the confidence that I want to have in the medical profession.
Examples:
-Medical Providers that I am entrusting my emotions, my physical body and my very life to, cause me great anxiety due to a significant knowledge deficit related to the Pathophysiology of Acute Intermittent Porphyria… the Porphyria Drug Database is rarely referred to without my prompting and I fear receiving a drug that will cause me further problems… improper porphyrin tests are ordered and incorrectly gathered during acute attacks… I am not sure if staff are aware of the risk of respiratory paralysis when inpt… I don’t feel heard when I say something makes me feel worse after trying a new treatment… I try to provide educational resources (CEUs, brochures, scholarly articles) but I feel my attempts to educate fall on deaf ears.
-Plan of Tx discussed at office visit differs from executed medical follow up
-Ultrasound Guided PICC Placement Failed x3 with significant pain
-Lack of Care Planning Re: PICC teaching/management after 4 nurse:pt discussions
-Direct untruth Re: Home Health Referral to Amedysis… not done

Positive Action Steps I have taken:
-Set up Case Management through my Health Insurance
-Started a medical coordination log
-Engaged a new Therapist with AIP experience (2 patients)
-Monthly Psychiatry Appointments
-Write out organized thoughts for medical appointments prior to visit

Idea for Treatment Plan:
-Discuss potential impact of 1 or 2 dextrose infusions qwk… Diabetes risks… other risks… monitoring protocol… what if Diabetes develops? How to manage both???
-Arrange Port placement in January (consider liver biopsy?)
-Request a 23hour obv to receive Hematin and dextrose after placement… to follow up anxiety and stress of procedure
-Continue weekly dextrose the day after Estrace at infusion center with a peripheral line until port in place
-After port… if advisable 2x/wk dextrose… infusion center or HOME infusions???
-After port… Hematin as advised

-??Wt management thoughts to minimize diabetic risk? Current GF/Dairy-free, ~1800 cal/day, exercise QD as tol, conscious of glycemic index, thyroid goitergens and liver: protein relationship

Thank You for valuing my situation and allowing me to be heard.
I look forward to your thoughts and comments.

Respectfully~
T. Suzanne Jaynes-Emmert
AIP Patient

December 2, 2011
Dear Dr. ___ (letter read to Psychiatrist at office visit),
At times I have trouble finding my words when I need them. I have found that writing out my thoughts ahead of time can greatly improve the clarity of my communication. Please hear me when I say how much I admire your knowledge and medical insight. I am glad that we have partnered in my health journey and I appreciate your investment in assisting me in the management of a rare medical situation.
The gabapentin continues to help ease my everyday neuro-sensory hypersensitivities and peripheral neuropathies.
I have found the Klonopin is something I cannot take… my Porphyria is too sensitive to small triggers at present and I would feel more comfortable with a PRN antianxiety medication that is safe for AIP people.
I have been using the Phenergan for nausea, agitation and restlessness. How do you feel about a low dose pill form that I could use more regularly for the agitation and restlessness?
Action Steps I have taken:
-Set up Case Management through Health Insurance
-Started a medical coordination log
-Applied for Social Security Disability
-Engaged a new Therapist with AIP experience x2
-Write out organized thoughts for medical appointments prior to visit

Tentative Treatment Plan:
-Change PRN Anxiety Med to AIP Safe
-?Phenergan pills for agitation/restlessness
-Therapist Qwk

Thank You for valuing my situation and allowing me to be heard.
I look forward to your thoughts and comments.

Respectfully~
T. Suzanne Jaynes-Emmert
AIP Patient

Sunday, October 9, 2011

Blog to Book

Wow, I can't believe that it has been a month since my last entry!So very many things have happened since then. Life is relentless that way... nothing stops the forward motion of it all.
Sometimes life's perseverance feels like a blessing...
and others can seem insensitive and progressively cool.
My life is always a "mixed bag" of both... vacillating as my journey unfolds.

The one year caption of, "My Life with Acute Intermittent Porphyria" has come and gone now. My next writing phase will be identifying a blog to book program and converting this past year's blog entries into a book format. After doing a little research it looks as though I have quite a lot of work ahead of me, editing, front book work, back book work, publishing and distributing the finished project. The thought of all the foreign tasks seems unmanageable...
Thankfully, I am empowered by a strong internal motivation to turn the challenges of my experiences into a "helping hand" extending a sense of understanding and validation to others who struggle with AIP worldwide.

Saturday, September 10, 2011

Life's Path...



The "Spirit of Me" feels lighter today...
I feel uplifted, positive and yes, even hopeful.
I think I had started loosing hope for a "better tomorrow" a while back... each day things just seemed to feel less than par... or worse.
I really struggle when I find myself in those ruts.

There are several aspects to contend with in deciphering why I vacillate in this cyclic, manic-depressive type fashion...

My twin eight year olds' behavioral-mental wellness challenges that beat on my stress nerve like a iron mallet to a piece of glass... sharp edges and complex shatter patterns... the whole package ignites my Porphyria on a daily basis like gas on a flame. My goal is to identify and secure solid therapeutic treatment to help them heal.
When I am "sick", my mind, body and soul become depleted... and my family initially goes on "auto-pilot". If my "illness" persists long, the whole family dynamic turns chaotic and dysfunctional. My husband becomes overburdened, resentful and angry. My children become needy, craving attention, acting out to get it, matters become even worse.
I feel physically miserable, emotionally drained and mentally "not myself". Guilt strikes me... I feel guilty sometimes about the fallout that these AIP periods cause. Sometimes I get angry... angry because it is not MY fault! I don't want to be like this! If the STRESS level weren't consistently SO unbelievably high in my home life... AIP might be much less active.
Usually after a spell of feeling just awful, and unable to do much more than rest in the bed... I will wake up one day feeling like a normal person... no stifling pains, no nausea... I have emotional calm, mental clarity. I want to take on the world... make up for all the time I "lost".

I did the 26 blood tests and now I have been to my Primary Doctor. All tests back so far area within normal parameters. My liver enzymes are slightly elevated. For the first time in many years all of my vitamin levels, especially Vitamin "A" have stayed within normal limits without high doses of supplements! My vitamin levels normalized after I went gluten and bovine dairy free. My theory is that I have a sensitivity to gluten and a known sensitivity to the whey in bovine dairy... each time I consume these products I develop significant indigestion, gas, bloating, headaches and joint pain with swelling within 24 hours. Anything that causes these types of reactions is obviously irritating one's digestive system... with irritation comes swelling and decreased performance ability. I believe that my nutrients were not being absorbed properly due to on-going irritation... even with significant amounts of supplements in addition to wholesome foods.
An interesting tid-bit I was told long ago is that "when someone has frequent urinary tract infections Vitamin "A" stores can be depleted"???? Also, "people who have both a Vitamin "A" deficiency and an iron deficiency (anemia) can be difficult to heal the anemia issues"?????
I will keep to my new way of eating... because it makes me feel better.

My doctor was her pleasant self as always. I wasn't feeling my best that day... but I was ok. She spent about 40 minutes with me, evaluating my situation. My next visit is scheduled in 3.5 months... short-term disability renewed... long-term disability denied! Denied! Denied? My doctor knows that STRESS is an enormous AIP trigger. She also knows that my son's untreated psychological/behavioral condition is my primary STRESSOR. With proper treatment his behaviors will become regulated and my STRESS will be significantly lessened. Lower stress levels create the possibility that I may return to some form of nursing work.

I was angry with her initially... I have tried and tried... I haven't found anyone who can help him... what if I never can! Ahhh a mental switch flipped for me! I can, I will, and I MUST find the right intervention to help my son! There is NO other option. Finding him a solution to his challenges provides the only solution to a large part of my challenges (in theory anyhow).

I went to the hospital today. Externally I looked like I was dressed for a yoga class. They said, "no metal at all on your clothing for an MRI". Internally I was a little nervous. I have never had an MRI before. What if the IV contrast made me sick? I didn't know what to expect at all. I was digging up harbored anger and resentment about always being alone at every new or "scary" point in my health management. I shook it off... it wasn't positive or helpful. The woman who did my MRI was very kind and the procedure wasn't bad at all.

After the MRI I remembered to ask for a "disk" copy. The Liver Specialist had requested I bring him one. Then I went to see who was sitting at my old desk...
Yeah two of my favorite coworkers! We had a good visit and I went down to our main department area. My boss was in a meeting so I scheduled an appointment with her for this coming Tuesday. Then I spoke with our Pediatric Social Worker. She asked and I summarized my son's situation. She gave me the name of a local specialist that might be able to help! Then I went in to talk to the lady that I was hoping to "job-share" with come April 2012. She has a small office to herself and reviews patient charts filing appeals for denials, and submitting clinical information for insurance company review. She had devastating personal news... her husband was quite unwell. She would like to start job-sharing as soon as possible. Hmm I thought I wonder if I am ready to try? I wonder if my doctor thinks I'd be ready for this type of work? I wonder if my old boss would let me give it a try? So many, "I wonders"... so much happened in just two hours time yesterday morning. I meet with my boss on Tuesday, till then I will pray for guidance and call the suggested doctor for my son first thing Monday morning.

As I readied for bed last night I reflected on the day. Isn't it ironic that you can wake up on one path in life... then go to bed that very same day with a whole new direction available and ready for exploration... life is exciting, scary at times... but NEVER hopeless.

Friday, September 2, 2011

Bits and Pieces of Me Lately

As noted, I have really been struggling lately.
I am schedualed to see my Primary Doctor next Wednesday.
I have my first MRI, with IV contrast, next Friday.
I follow it all up with an evaluation by my Hepatologist.

I am currently receiving "Short-Term Disability" due to a "Medical Leave of Absence" from my half-time job as a Nurse Care Coordinator at the local Emergency Department. I love the nursing profession and am proud to be called a nurse... but I don't know if I will ever have my disease "regulated" enough to be productive as a reliable "employee" again. That makes me so sad.

My joy is that I still have the ability to write, to share, to validate and to help other "patients" like myself via my written story.



Just the other day my daughter dropped in for an unexpected visit.

It was wonderful to see her.
Even though she lives minutes away... she is 20 years old and keeps a very busy life. As we sat and talked on the living room sofa, I confessed that I had been really struggling with AIP and with life. I apologized to her. Many times when we see each other I bring up AIP.
"I don't want, nor have I ever wanted AIP to be our strongest common ground".
My problem is... when I am with "you" (my daughter) "you" really "understand" what the "storm" feels like inside my body... you understand without trying to find words or make analogies.
I have been feeling like an alien on a planet where no one understands me...
I feel confused...emotional... secluded... in pain and medically without hope.
With you I feel validated, understood, believed in.

I am truly grateful that my daughter has not experienced AIP issues to the degree that I have and do... yet she is the only one who can, "see beneath my skin".

For such a young woman she showed great kindness, warmth and depth of character in comforting my spirit that day.

She does worry me regarding her own AIP disease management... she turns 21 soon and is planning to "go out"...
Alcohol has been a significant trigger for her in the past.
She is also considering re-trying hormone contraception, which has also been a significant trigger for her in the past.
She has a clear understanding of AIP, so I know that as an adult she will make her own choices... as we all do.



Today I reached out for the "strength" that connecting with a sibling can give.

My brother and I have known each other all of his life and most of mine, 39 years now. In many ways we are much a like. We are fueled by determination, perseverance, internal motivation and ethical responsibility; sharing common perceptions about our upbringing.
In other ways we compliment each other... I am deeply empathetic, I thrive on sharing the depth of my soul with a small handful of close family members... I wear my heart and the truth on my sleeve for all to know who I am, and what I am "about".

I have always known my brother to be an "excellent poker player" in the game of "real life"... rarely does he show the world how he truly feels or what his plans are... "his thoughts are his own". Many, many times I wish I were more like him... because openness brings pain and insult when the world is not as kind or caring as one might hope.
Together, I guess we as siblings are a "balanced set".

Life has inspired change in both our lives in the past year...
My sons were diagnosed with some significant behavioral issues.
My brother sadly divorced early in the year after a long term marriage.
Our beloved grandmother died in early Autumn. (Probable AIP)
Our father died in early Winter, after a long dance with many health issues throughout his life. (Diagnosed with AIP days prior to death.)
My brother happily remarried on New Years Day 2011.
My AIP remained chronic, vacillating in severity throughout the year.

During this past year, under significant circumstances, I have felt my brother begin to share more of himself with me. Although I know he is not "magically super-human" to me, my brother has a very strong spirit! I do love that about him... like grandma, dad, grandpa Wood, and mom (who thankfully is still here on earth).

Just like "borrowing a cup of sugar from a good neighbor", I need to "borrow 3 cups of extra strong spirit" to get me through an "empty spot in the cupboard of my life".

My text to him today read:
Thank you for trying to return my "I miss you" text with two phone calls... that I unfortunately missed.
Days of late are extremely trying...
My son's behavioral/ psychological situation will NOT be denied and commands constant attention/ investment.
My mind... my body and my spirit are drained.
I search daily for the "silver lining" or the "light at the end of the tunnel".
Nothing leads me to the comfort of answers, interventions or change.
My Porphyria has been constantly "prodded" by the situation, until it now "ROARS like a Lion".
Days are hard and I feel "jailed" by life.
Any insight that you might have would be appreciated... in lieu of your physical presence... that always provides a surge of strength to a sister's heart.
With Much Love/ Your Sister

26 Tests for the Hepatologist (Liver Doctor)

I had an order for blood work to be drawn, one week prior to my upcoming abdominal MRI, so today is exactly one week prior.
"Fastng" began at 8pm last night.
I dropped the children off at school around 8:50am this morning and drove directly to the lab to have my labs done as ordered.
I signed in at 9:18am and one hour later I had my 26 tests (13 tubes) drawn from my right arm. (Over $3K without insurance... $15. copay with insurance... thank God for my husband and his job!)
I have been having a lot of "Porphyria" activity this week... but after having an EXTREMELY stressful morning with my son and fasting for 14 hours, when I asked the lab technician if I could use the restroom, my urine looked like "dark ice tea" and smelled of "old dirty socks".
"Here we go again!" I thought as I rushed to the closest "Starbucks" where I ordered not one, but TWO breakfast sandwiches and a "Tall Skinny Hot Chai". I ate every bit of it all as I drove home...let the dog out...then back in... and now find myself in bed typing my thoughts and day away until I can calm my body enough to rest a bit.

Wednesday, August 31, 2011

Information and Support That Help Me with My Health & AIP

- Blog Browsing: looking at other people's web logs to see what they have to share about their situations... gastroparesis, peripheral neuropathy, dealing with chronic disease in general.
Just choose a web browser and type in "____ blog".

- Reading Published Memoirs: examining how others, "tell their story" in memoir format... gaining insight regarding how various chronic health situations change people's lives.
Just go to the local public library and type in "memoir" as the key look-up word.

- The Magazine "Life Extension" provides very in-depth information about the latest integrated medical research findings around the world... they do "push" their vitamin/other supplements.

- "Advance Magazine for Speech-Language Pathologists & Audiologists", vol.18, NO.44, November 3,2008 had an excellent article (pg 7,8,9 & 34) "The Narrative Self Restoring Patient Identity in Neurogenic Disorders" written by Jason Mosheim (www.advanceweb.com/speech).
For me the article validated how I feel alienated about interacting with other people at times. Sometimes communicating is extremely difficult during an active AIP phase. This article discusses this issue in relation to several neurogenic disorders and offers ideas on how to improve the situation.
This article references a book that I have not yet read, but plan to. The book is called, "Neurogenic Communication Disorders: Life Stories and the Narrative Self" written by Shadden, B., Hagstrom, F., Koski, P. (2008)
For more information contact: Barbara Shadden, PhD, bshadde@uark.edu

- The Book, "PORPHYRIA THE UNKNOWN DISEASE" written by Diana Deats-O'Reilly
This book provides a good basic overview about what Porphyria is. It also presents several patient case studies to demonstrate what it is like to experience active episodes of Porphyria. I found this book to be an excellent springboard for researching AIP prior to my official DNA diagnosis.

- The Book, "Porphyria A Lyon's Share of Trouble" written by: Desiree Lyon Howe
Ms. Howe's book gives a personal account of her life with AIP. She is inspirational in demonstrating determination to assist others with Porphyria. She is one of the initial founders of the American Porphyria Association (APF). The APF is a significant resource for information, assistance and support for those with Porphyria.

- "UNSTUCK Your Guide to the Seven-Stage Journey Out of Depression" by: James S. Gordon, MD
This book gives great natural interventions to help combat the symptoms of depression that all people with AIP are prone to.

- "Healing the Planet ONE PATIENT AT A TIME... A Primer in Environmental Medicine" by: Jozef J. Krop, MD, FAAEM
This is one of my favorite books. I learned so much about the human: environmental interaction and how one impacts the other. So very many helpful insights...I think I will sit and read it again!

- "The NEW GLUCOSE Revolution Low GI Gluten-Free Eating Made Easy... The Essential Guide to the Glycemic Index and Gluten-Free Living" by: Dr.Jennie Brand-Miller, Kate Marsh and Philippa Sandall
Long term hypovitaminosis (low blood levels of essential vitamins) was one outcome of my gastrointestinal vitamin absorption dysfunction. After reading this book I became better at identifying long-acting carbohydrate choices that are gluten-free. After trying a gluten-free/ bovine-dairy-free diet for several months my vitamin levels are now within normal limits (without supplements) among other resolved issues. This book is packed with excellent health information!

- "Notes on Nursing What It Is, And What It Is Not... Florence Nightengale with an introduction by Ramona Salotti"
This book represents the foundation of my beliefs in nursing as a career/ calling.

- "Animal, Vegetable, Miracle... A Year of Food Life", by: Barbara Kingsolver with Steven L.Hopp and Camille Kingsolver
A New York Times Bestseller! A city family goes rural and learns to live off the land (literally) and appreciate a "simpler" way of life.

- "Health Promotion Strategies through the Life Span", by: Ruth Beckmann Murray and Judith Proctor Zentner.
The title says it all. A great resource book for positive health maintenance strategies.

- "Integrative Health Promotion... Conceptual Bases For Nursing Practice", by: Susan Kun Leddy
The title says it all. A great resource book for positive health maintenance strategies.

- University of Iowa College of Nursing Online Human Genetics and Genomics Courses.

- The Genetics Education Program for Nurses (GEPN) at Cincinnati Children's Hospital Medical Center... Web-Based Genetics Institute... and other online programs.

- Genetic Nursing Credentialing Commission (GNCC): web search/ information

- Certified Nurse Life Care Planner Certification Board: web search/ research

- Epigenetics web search/ research... how environmental influences effect gene expression

- Porphyria Nurse Care Coordination... web search/ research

- Some Informational Web Sites:
ehsc.science.oregonstate.edu
nursece.com
geneticalliance.org
cdc.gov
doesitruninthefamily.org
http://www.healthyhomestraining.org

- Yoga Nursing

- Digital Health Records/ Health Information Systems: web search/ research

- NeuroSensory Dysfunction/Impairment... also known as Sensory Processing Disorder: web search/ research

- The "Yacker Tracker Deluxe" sound monitor related to noise level control

- The Surround Air XJ-3800 Intelli-Pro Air Purifier: seven filters one machine (a HEPA filter, an activated carbon filter, an ionizer, a photocatalytic (TiO2) filter, a germicidal UV lamp, a washable pre-filter, and an electrostatic dust collector)

- 5 Stage Reverse Osmosis Water Filter System With Storage Tank

- Many books and publications by Dr. Andrew Weil

- "Environmental Medicine" web search/ research

- "Environmental Health Nursing" web search/ research

- "American Holistic Nurses' Association Guide to Common Chronic Conditions: Self-Care Options to Complement Your Doctor's Advice" by: Carolyn Chambers Clark

- "The Omnivore's Dilemma: A Natural History of Four Meals" by: Michael Pollan
An excellent book about the food we eat...its quality and content, where it comes from, how government regulates food and marketing nuances. I used the audio book format over a very long drive.

- "In Defense of Food: An Eater's Manifesto" by: Michael Pollan
This book seemed to be a continuation of his first book, but still good information.

- "The Maker's Diet" by Jordan Rubin
The author of this book tells his story of terrible illness only to become well by following dietary/ lifestyle guidelines outlined in the Bible.

- Relaxation and Meditation... web search/ research

- Rare Disease Advocacy Research Education... Rare Disease Network

- "The Comfort of Home... A Complete Guide for Caregivers" by: Maria M. Meyer with Paula Derr, RN

- The Chronic Fatigue Immune Deficiency Syndrome Association of America

- Stanford School of Medicine "Chronic Disease Self Management Program"
(http://patienteducation.stanford.edu/programs/cdsmp.html)
This is a wonderful program offered via many "Area Agencies on Aging" throughout the United States. I also believe that there may be online options available.

- Many "Scholarly Research-based Articles" about Porphyria retrieved via "EBSCOhost" academic search.

- Anatomy and Physiology Text Books

- Books about The Liver

- Books about Genetic Disorders

- The "Guided Care Nursing Program" @ (http://www.guidedcare.org/nurse.asp)

- The Farm Sanctuary @ (http://www.farmsanctuary.org)
A "Field Trip" to the New York sanctuary was an unbelievable "eye opener" for me! I was so overcome with the reality of our "real world" that I had to leave the building weeping, during the initial presentation...I wept without control at the animal cruelty and at my own personal naivety related to my unquestioned belief in: an invested government "focused on" the best interest of the people it serves. Soon after this experience I listened to Michael Pollan's book "The Omnivore's Dilemma: A Natural History of Four Meals" which served to reinforce my Farm Sanctuary "Awakening".

- Multiple Chemical Sensitivity- web search/ research

- Herbert Bonkovsky, MD @ (http://www.porphyriafoundation.com/about-the-apf/scientific-advisory-board/dr-herbert-bonkovsky)

- Karl E. Anderson, M.D., F.A.P.C. @ (http://www.porphyriafoundation.com/about-the-apf/scientific-advisory-board/dr-karl-anderson)

- The American Porphyria Foundation (APF) @ (http://www.porphyriafoundation.com/about-the-apf)

- The European Porphyria Network @ (http://www.porphyria-europe.org/)

- The British Porphyria Association (BPA) @ (http://www.porphyria.org.uk)
There is a list of Porphyria Associations all over the world, printable from this website

- Books and Articles on, "Half Acre Hobby Farming" & "Sustainable Living"

- Information from varied media sources on keeping "Back Yard Laying Hens" and most recently "Nigerian Dwarf Dairy Goats"

- The Christian Bible
I began reading my Bible last year about the same time I started this Blog/Book. My intent is to read the Bible like a storybook, cover to cover. At present I remain about one-third of the way through. For such a widely referenced, well-known book, I am sad to admit it is my first time reading it cover to cover.

He Laughed

Having been on a "Medical Leave of Absence" from work for several weeks now, my husband asks..."what will you do if you aren't ever able to return to work"? I responded, "I will apply for permanent Social Security Disability and Medicare Supplement to help with our finances". As for life in general... "on my 'good' days I will finish my book, edit it and work on publishing it... then I will put together my next book on 'International Adoption & Trying to Heal RAD'... that is of course in addition to all of my domestic wife and motherly responsibilities". He responded with laughter... I was hurt and confused. "How should I interpret your laughter" I asked. "Are you mocking me... telling me you think of my aspiration as a joke... or maybe that you believe I lack the talent for the job".

His rationale for laughter:
"I believe that you have the capacity to write, and write well. I have known many people who aspire to write a book... I have known no one who has completed the job and become famous". "How are you different"?

How am I different?
I have no aspiration to "become famous" or make tons of money. My motivation is a drive to share information about what it is like to be a Registered Nurse who lives with a rare genetically inherited disease, Acute Intermittent Porphyria (AIP). My audience is most likely limited to people who have the disease, people who have loved ones with the disease and medical personnel who want more information about AIP. The obvious lack of support shown by my spouse this day served to "fuel my fire" even more to "make this book happen".

As my pre-set time frame of one year (9/17/2010 - 9/17/2011) is quickly ticking to an end, I struggle to include all of the elements that I want and need to share. I am hoping that during my "editing phase" (post completion date) I can identify the pieces I have neglected and modify the final product to include a comprehensive presentation of real-life information.

It has been even more difficult to write lately, as my disease has been very active and I struggle to, "think straight". Writing in small segments, when my environment is well controlled is ideal yet rare to come by.

Friday, August 26, 2011

Wine with Dinner

I had some delicious wine with dinner tonight. It is my husband's birthday. My AIP very rarely can deal with any alcohol at all anymore. It really does seem to be both an age, and a life-time of accumulated exposures to triggers situation. In my late teens, twenties and early through mid thirties I was able to tolerate ocassional alcohol consumption the same as any other regular person... not so today! One glass and it's on! My switch is flipped... I cannot urinate... I cannot sleep (it is 1:20am)... I am irritable and restless... "It is what it is"... I made a poor choice joining my husband in having that glass of wine with dinner! Hopefully things will slowly calm and pass...I will be extremely "kind" to my AIP tomorrow...carbohydrates, lots of rest, and if possible, a low stimuli envronment.

This week has been SEVERELY stressful and my AIP has been down right evil to me. My husband (a teacher) returned to work last Monday and I have been home alone doing my best to take care of our twin 8yr old boys with significant behavioral issues (more about that in my next book). Life goes on in spite of AIP... and I truly prefer a life with my family, than a life alone without them. I continue to do my best to make it work.

Saturday, August 20, 2011

Scary Places Without Support

As a nurse and a caregiver I have experienced the effect of chronic disease consuming an individual's personality and social conversation... I do not wish to be that type of person. I want people to enjoy my presence not be irritated by it. I choose to stay in "controlled environments" whenever I can without saying a word. We recently rented a Lake House for 2 weeks on either side of my daughter's wedding. I spent most of my time inside the Lake House with a cool drink and a big comfortable chair while most everyone else sped around on water crafts, jumped off the dock and played in the water. My husband has brought up the point several times since that "he didn't think I had a good time"... "You hardly left the house". Oh, on the contrary my dear... I loved every minute of quiet, peaceful conversation, beautiful views of both nature and of my family having fun!Outside the humidity, temperature, biting insects, commotion and noise was out of control! Inside my environment (and new found Magnum Ice Cream Bars) was perfect for me!

I try not to verbalize my chronic state of AIP related aches, pains and psychological challenges on a regular basis... it sucks the life out of my companions and it negatively effects me. That said... sometimes... many times I feel so alone in the world... other people who don't have AIP don't understand the nuances of the illness. I have family members with AIP but one is young and only has limited insight into the type of support I seek. I want to talk about how it has effected my life and see if it has done the same to theirs... it sounds so very selfish... but it is not from that part of my heart... I just don't want to feel alone with AIP... it scares me... sometimes I feel like it takes me "down a road" that I may not be able to find my way back to normal... that scares me deeply.
But my siblings with the disease act as if it doesn't exist. I truly am glad that their AIP has remained latent enough to ignore.

The on-line support group I belonged to just closed. The leader's wife had AIP and died last year... he needed to move on to other things.

The "Virtual Group with Video Chat" that New York Mount Sinai is setting up is still in the works.

Some days I feel like an alien... no one around like me.

I was given the name and contact information of a woman with AIP who lives in my state... maybe I will call her...

New Medications

So a bit earlier I discussed how I agreed to begin a regime involving daily medication... I was definitely a must do situation!
The first night I took the Neurontin and the Xanax ER I was able to sleep ALL NIGHT!
It was the best night sleep that I had in years! No lie!
For the first week the Neurontin was at bed only then starting week 2 I was to increase the dose to every 12 hours (2x per day). It has been several weeks now, and believe it or not I actually feel very positive about taking my new medications. The effects last about 8 or 9 hours, so there is a little gap of increased issues, but I feel at least 50% better than before medication.

The medications seem to provide a "buffer" between my neurosensory system and the world (both stress and environmental triggers). As the weeks go on, insomnia, pain and emotional/psychological instability still escalate in parallel to the stress of my home life.

My home life can really "blow you over"... I deal with it as best I can... as anyone would. What are my choices??? Live as a hermit, a recluse, in the mountain forests of my childhood... rubbish... some days I can hardly walk to the bathroom! Besides... could you imagine the guilt and enormous sense of loneliness???
Forget it! My husband, my children, my friends, associates and community members are stuck with my presence... although I do consider the benefits of solitude, peace and serenity that eloping with AIP from human interaction... "Utopian Seclusion in Nature"...

Even "Good" stress gets to me... oh how I struggled to appear "pulled together" and "June Cleaver-ish" for my daughter's recent wedding day! My younger daughter has AIP as well and found her bridesmaid role a challenge in the midst of managing her eruption of ever-irritating AIP symptoms.

One day I forgot whether or not I had taken my medication... I was afraid to take it and overdose... so I didn't take it (I now have a pill box). By 1pm I was absolutely certain that I had not taken the morning medication! My nerves were jumpy at any little thing... my legs (especially the right shin) were radiating with pain... my emotions were up...down... all over! It was a terrible day! I took my night time medications at 6pm and could not go to sleep until about 3am!! Awful!!! The next day remained a challenge but with each day things have settled a bit more.

My very best days "health/ wellness-wise" are the days I stay at home in an environmentally controlled atmosphere (children outside in pool or playing...until school starts in a few weeks!). Stress is low, the temperature is comfortable, no scents, low sensory stimulation, and daily afternoon naps! I have even been able to return to stretching with the yoga ball and walking for 30 minutes on my elliptical trainer a couple times a week.

I find the more tired out I get through the day the less my legs work and the less I am able to understand what people say or do. I appear drunken when I walk and my brain just stops working... I can't even write... until I take a nap (usually about 2hours, 2pm to 4pm). When I wake up I feel much better and am able to continue with my day until about 10pm, when I go to bed for the night (up again at 9am).

I have a follow-up visit with my Primary Doctor in a few weeks... we will see what she thinks...

A Long-Time Friend

I saw a long-time friend who has had a difficult life the other day...
As we "caught-up" in the post office parking lot... he shared his current situational status with me...
I need to remember the advice I gave him that day, and apply it to my own life EVERY DAY!!
"I don't believe it is what you do for a living that makes the biggest difference in life... it's how you choose to live your life that really matters!"

What do people see when they think of me?
Even with my custome health struggles, am I a "model citizen"... do I conduct myself in a positive exemplary way? Do I complain too much? Am I kind, thoughtful and morally just????

If Not A Nurse...Then What???

As I left my doctor's visit it went through my brain...
What if I am unable to continue as a Nurse???
What if I need to consider permanent disability?
I am definitely unstable, unreliable and my illness is easily triggered!
I have spent my entire life setting nursing career goals!
I spent some time mourning the potential loss of continuing an active nursing career.
I spent more time crying and trying to figure out where I go from her?
Who am I?
What makes me...well what makes me...me??
Granted my emotional and psychiatric states were pretty unstable for a while... but these thoughts stayed on my mind even after the new medicine "kicked in".
Staying home on Social Security Disability, in my mind was equal to my career dying.
Being a nurse allowed me to focus on the needs of others...which took the focus off me.
Being a nurse allowed me to feel like I make a difference in the world.
How will I make a difference in the world now?? Struggling some days, just to make it through the day???
Then I remember... "God is in my heart" even when I am absolutely miserable!! My Life Has A Purpose... and it is NOT always about me. I am learning to "Let Go"... I am learning to "Let Life Just Happen"... I am learning how to prioritize "life's really important objectives"... I am learning to apply my nursing experience, knowledge, skills and abilities toward other life activities (writing) and aspects (family)... I am learning that I can still make a difference and direction will come when I stop fighting against the current... accepting and loving myself as I am... where I am.

A Visit to my Primary Doctor

My life has been very different since my most recent "Acute" Porphyria episode began... my body's response to in-hospital treatment did not follow the expected "bell curve" and the way in which I interface with the world is now significantly changed. Just a short time ago I was dreaming up ideas of furthering my nursing career. I considered a Doctoral bridge program that when completed would allow me to practice as a Psychiatric Nurse Practitioner and Behavioral Health Clinical Specialist. I thought I would be able to help people better manage their chronic health situations... providing them the support, expert guidance and professional collaboration that I wish I had available to me right now.

Today my reality is quite different than it was the day I wrote about becoming that person... my AIP has contributed to an internal state of constant "dis" "ease"... This past 12 months of writing in a "nut shell"... when my AIP is quiet... I want to do it all! I enjoy living life passionately in forward motion... going...doing...seeing...smelling...touching...experiencing it all! I feel as if I have been let out of a cage... I crave "real" living... being the "real" me!
Then I do too much or some life event triggers another round of increased illness.

It is extremely difficult to ride this cyclical, whirling dervish, called, "life with AIP" and not want to give up hope. Depression, anxiety and suicide statistics are higher for us with AIP... I have no idea what the biochemical contribution is... but heck just read and listen wouldn't anyone have issue with this un-chosen lifestyle???

I went to see my Primary Doctor a few weeks ago because I continued to have a lot of neurosensory, cognitive/psychiatric/emotional, insomnia, and pain. We talked about my medical situation as she evaluated me for over an hour! One thing she said to me... well it was more of a question... "how is your spiritual life??". How is my spiritual life... hmmm... actually my spiritual life is the only part of me that feels Well! Without the belief that God is with me in my heart... I would have succumbed... succumbed to it all, a very long time ago. I used to feel as if I were being punished... and that is why I would be made to feel so miserable... that feeling is not a useful one and only serves to make matters worse... instead I now try to tell myself that God has a purpose for my "life's journey" and it is not always about me! I wish I never came to know the awful nuances, the pain, the disruption and the hopeless loneliness of societal ignorance related to active Acute Intermittent Porphyria (AIP)... but I have become intimately aware of all that is AIP... and I am forever grateful that my "Spiritual Life" is healthy!

My Primary Care Physician (PCP) is like none other I have ever known... each visit is at minimum one hour... evaluating and collaborating... investing in trying to help my mind, my body and my soul. The time has come for me to make some big changes in accepting and taking care of my mind, body and soul. She assisted in setting up a Medical Leave of Absence from my job. I agreed to begin taking medication on a regular and consistent basis. We agreed that these steps are necessary at this point in time. I began taking Neurontin 300mg 2x daily, and Xanax XR 1mg 2x daily. Taking daily medication is a big step for me... but without some help I felt as if I were headed back to the hospital... either 2 South for Hematin... or 3 South for psychiatric stabilization.

Saturday, July 16, 2011

Online Porphyria Support Group... Today's Post

Thank You Lynda & Eileen for sharing your Porphyria experiences!
Your validation has come just when I needed it the most! I have been struggling with my chronic AIP issues for some time now... amen to the fact that people and stress escalate the neurosensory overload! I am at a very difficult point at present...my nursing career has been a huge part of my life... like sand through my fingers I am losing my career... beginning the disability process... it is extremely hard for many reasons... I try with every bit of energy that I have, but I just can't do it anymore... my AIP is triggered easier and easier these days... smells, lighting, motion, the volume, pitch and speed of other people's talking... my body feels as if it is made of lead... I have more and more trouble focusing... remembering details... making connections... the pain in my legs and the random "bee-like stings" is awful (as you know)... I am getting more and more anxious when I am planning to leave home... not because I have a phobia... I have always been social and fun-loving... but because I cannot control the environment and my symptoms will probably worsen no matter how careful I am. It is like living in a bad dream. I try every day to keep a positive mind-set... to look for the little things that make life special... some days are a terrific challenge. I have a husband and twin 8 year old sons... all with ADHD... I am extremely invested in eating right to optimize my health (Gluten Free, Soy Free, Bovine Dairy Free, Organic, Non-Goitergenic, AIP-friendly)... I sauna...I yoga... I use my elliptical exerciser daily when able... I read and research to optimize my wellness... I joined the AIP Clinical Study... I try to gently educate my health care providers when needed... I have had dextrose IV treatments... I have had Panhematin treatments... they usually help with the acute issues but have no lasting effect. To the world outside of my body I look about 20 pounds overweight, tired with dark under eye circles... but otherwise normal... normal... the same as everyone else... they don't understand... they can be condescending and both intentionally and unintentionally hurtful... I appreciate that I am "Preaching to the Choir"... It is so very comforting to know that there are other people who understand what I am talking about... other people who can relate... other people like me. Thank You so very much for being there, for sharing your experiences... and for listening.
Best Wishes~
Tracie

Wednesday, July 6, 2011

I Pray That God is With Me



Neuropathies (central, autonomic and peripheral): headache, bilateral leg pain (bones/joints), heels like pins

GI: burning nausea

GU: dysuria

Muscular: weak, heavy, poor endurance, fatigued

Psychiatric: cognitive slowing, confusion, memory lapses, restless, irritable, emotionally labile,

Sensory: neuro-sensory hypersensitivity (sound, light, motion, touch), ringing in ears

Pain/Comfort: leg bones radiate with pain, headache through eyes to back of skull, insomnia, restless/ cannot find a comfortable position

Sleep/Rest: see Pain/Comfort

Self-Propelled Disease Management:
Ugh I am so incredibly frustrated! For the life of me I cannot figure out where the "shut off" valve is to my Porphyria... Since my recent hospitalization, complete with IV Dextrose & Four Panhematin Treatments... I came home and over the following week seemed to gradually return to my normal baseline self... I went back to work last Thursday June 30th and my family went out of town for the holiday weekend... the house was calm and quiet...work was fine... it was a bit of a challenge to make it through all 8 hours of the work day with low endurance... but it was ok...then Friday came and went without too much issue (still a lot of low endurance/ fatigue)... I was off work and home Saturday and Sunday... I knew I should take advantage of the quiet time and rest... but when I looked around the house... well it hadn't had a good cleaning in weeks due to my health... I felt disgusted by the scene and began scrubbing, cleaning, laundering...etc. when I finished everything it was Sunday afternoon! The house looked great but it came with a price... I started feeling a "little cloudy" mentally... I forgot a teapot on the stove and burned out all of the water... I went outside and left the kitchen sink running... when I came back in there was water everywhere!!! I went to bed early Sunday night, but couldn't fall asleep until after midnight with Ativan, Naproxen and Magnesium! Monday July 4th I was late to work...still not feeling "myself" and worked till 2pm... I came home and stayed in for the night... Tuesday I worked 8am to 4:30pm, came home, crawled to the couch and put my feet up... 45 minutes later my family returned home from their trip. It was good to see them, the energy they brought through the front door was bittersweet... I truly had missed them all... but as the night progressed so did the energy, volume and negativity. I could feel my nervous system struggling, firing off like a fireworks display... I gently reminded them that these behavior choices are unhealthy for everyone... they agreed for the "millionth" time and I went to bed at 10pm. I struggled at work today, my co worker's perfume put me over the edge and I finally left for home at about 10:30am... A quiet, dark, cool place to rest works best to counteract my hypersensitive nervous system... a warm, weighted heating pad works best for my leg/ nausea issues. I try so hard to keep a positive outlook... I search for the "meaning"... I try to discover what I can do to change my situation... but today I feel powerless. I pray that God is with me.

Saturday, June 25, 2011

About My 5 Day Hospital Stay

This is my fourth day home from the hospital and I am still weak, "heavy" feeling and have little endurance. I am not complaining... my pain and discomfort level is at about a level 2 out of 10 which is a huge improvement!
My struggle now is re-creating a sense of stability... this episode was one of the biggest I have had. My "mental self-talk" is insecure about my abilities... and about future planning... the perpetual "rug" is apt to be pulled from beneath me again at any time... leaving me dysfunctional wherever I am... work, home, driving, traveling... it is scary.
I was told, "I waited too long before seeking medical attention"... my reply is... how do I know when to go??? Some times simple rest and carb-loading reverse the escalating symptoms of AIP... other times things cross a point and spin wildly out of all control! How do you apply logic to all of this? Not to mention...that "Resting" on too many work days will put one "Out of Work" permanently.
I was told, "It is our medical opinion that you should manage your stress more effectively to avoid AIP triggering"... I agree. How? I am an uber nutrition "Freak", I exercise daily, Sauna regularly, Vent issues, Proactively spear-head problems as they arise... seek professional and personal support for my children's on-going behavioral challenges... talk to a marriage counselor... and Pray A Lot!!!
Wrestling with a sense of hopelessness can lead to depression for me. I am trying to focus my energy on the positive attributes in life... donning negativity filters... avoiding anything and anybody that doesn't see "the glass half full".
Until my energy is re-established I find, for me, it is best to stay close to home... it requires a fair share of "negativity filtering" right here... and that is all the energy I can muster until tomorrow... then maybe I will venture out...

A 5 Day Hospital Stay

Thursday, June 16, 2011

AIP Triggered By... The "Energizer Bunny" on "Crack Cocaine"

Part 1

It is not quite one week into "Summer Vacation" for my family...

-Twin 8 year old adoptive sons, with Reactive Attachment Disorder(RAD), Anxiety & Attention Deficit Hyper-Activity Disorder (ADHD)...

-Husband (School Teacher, also off for the summer), with ADHD...

The "Raw Energy" that exudes from these three individuals could power a large city!

The energy is delivered in a variety of formats...
-fun-loving playfulness
-outdoor enthusiasm
-sports participation
-multiple partially-completed home renovation projects
-behavioral rages & fits
-impulsivity
-stealing
-lying
-fabricating untruthful stories
-undermining
-manipulation
-deciet
-trickery
-blatent disrespect
-unkind remarks
-thoughtlessness
-embarrassment
-aggression
-violence
-constant LOUD voices
-self-focused, lack of empathy for others
-irrational thought processes
-multiple, simutaneous, stimulating, sensory imputs (TVs, Radios, Computers, Video Games, Conversation, etc.)
-never ending supply of "High Gear" energy in motion (good & bad)


Part 2

Pair this trio with a wife/mother ...a Thyroid that runs low... and an extra large genetic helping of Acute Intermittent Porphyria...
Not a pretty sight! Believe me...
I am dog tired most of the time and now that everyone is home for the summer I get very little rest time.
During the school year I work part-time....this allows me several days to rest while everyone is in school... I still have to manage the home...doctors appointments and food shopping... but I have a balance that I am able to manage most of the time.

Today I feel overwhelmed and hopeless... Porphyria has been "on my heels" for a few days now... I am anxious about my escalating symptoms... I had to work today... my cognative recall is slipping even with basic concepts....eye /light pain...headaches...leg pain...nausea... emotional instability... restlessness... irritability...

I can usually "calm the beast" by removing sensory stimulants...resting... increased carbohydrate intake... and more rest... BUT... did you read Part 1???

Part 3

I don't know how to best cope... I am run down, tired out, and over stressed... I try to see the light at the end of the tunnel... but today I cannot.
Others I reach out to are unfamiliar with the dynamics that complete our home...
They politely listen as I vent... this helps some.

I pray...I eat right... I exercise daily (when I am well enough)... I pray some more... I try to live a lifestyle of healthy choices...of love... of kindness and respect... I have sought professional intervention for my family... counselors... psychologists... medication... physicians... specialists... religion/faith/spirituality... I try very very hard to be positive in effectively managing my Health Situation but today...
today I am struggling...
today I am discouraged...
today I feel hopeless...
today my body hurts, I am physically, emotionally and cognitively uncomfortable
today I can't think straight or recall common words or processes
today I wish I were stronger in every way
today I am angry with my family for "triggering my Porphyria" with their issues
today I am angry with myself for being weak
Today I wish Porphyria would go away!
Today I really HATE Porphyria... I am tired of this same old cycle!!!

Thursday, June 9, 2011

DNP

I have almost completed the Johns Hopkins, "Guided Care Nursing Program" (GCN)...
What a fabulous program!
I have enjoyed every minute of course work!
I am currently working with one of the Emergency Department Physicians where I am employed to create a Care Planning Program for patients with chronic health issues that cause recurrent hospitalizations...very exciting!

My original plan had been to complete the GCN certification program then to sit for the ANCC National Certification in Nursing Case Management...
I still intend to complete these 2011 goals.

My thought is that gaining formal education and credentials to couple with my personal experiences in Chronic Disease Management will really hone my expertise in the field.
Ultimately my life goal is to have a significant helpful impact on supporting individuals with chronic health issues.

In thinking about life... and life goals I did a bit of web searching...
Are there professionals that specialize in "Counseling" Individuals on how to best manage their chronic health issues???
Not just medically managing the disease, but actually supporting the person living life with a chronic health issue or issues... helping him/her navigate a life impacted by chronic issues...

There are some Professional Chronic Disease Self Management Support Resources (PCDSMR)out there...but the only PCDSMR a person could independently choose, call up, make an appointment, go to see, and potentially have insurance cover the fee for service was a Health Psychologist.

At first I thought... I want to be a "Health Psychologist"!
Then I began to think... what about Nursing???
In 2015 all Advanced Practice Nurses will be required to have a Doctorate Degree to enter the practice...
Besides, there are no specialty programs for Chronic Disease Management...
But...
Supporting Chronic Disease Self Management is definitely a behavioral/counseling intervention...

What about the
Doctor of Nursing Practice (DNP) in Psychiatric Mental Health Nursing at
The University of Tennessee Health Science Center (UTHSC) College of Nursing???

Just a thought...
It is a three year hybrid program... online classes, local clinical arrangement, and some on-site sessions in Tennessee...

I would love to tackle this program gaining the credentials necessary to provide PCDSMR to individuals who need help... and the ability to bill insurances for services rendered!

It might be magical thinking...I tend to forget the limitations that my AIP puts on me... but maybe???

I am always looking ahead...identifying options and opportunities to grow within my specific life situation... I stay flexible and open... but ceasing to grow would be synonymous with death in my dictionary.

Medications That Have Helped with My AIP

Occasionally/ As Needed
Ativan- PO Q6h/ PRN- Neuro-Sensory Calming
Naproxen- PO Q12h PRN - Pain & Discomfort
Glucose Tabs- PRN to Avoid AIP Crisis
Cranberry Capsules- PRN As Directed- To avoid UTI during episodes of neurogenic bladder dysfunction r/t AIP

Magnesium- PRN As Directed- Neuro-Sensory Calming
Phenergan- PRN Q6h- Neuro-Sensory Calming and Nausea Control

During AIP Crisis
IV Dextrose
IV Morphine
IV Phenergan Alternating with IV Zofran
IV Ativan
IV Hematin

Chronic Disease Self Management Support

-Guided Care Nurses (GCN) at Primary Care Practices
-Case Managers (CM) provided by Medical Insurance Providers
-Care Coordinators (CC) while in the hospital
-Social Workers (LCSW/ MSW) located within facilities and within community-based settings
-Counselor/ Therapist- Health Psychologist, LCSW, APRN-CNS, APRN-CRNP, other...
-Stanford University's Chronic Disease Self Management Program (web site)
-Resource Books
-Disease Specific & General Category Organizations- APF, NORD,
-Disease Specific & General Category Support Groups- online groups, Facebook, etc.

Items That Help My AIP

Meltzers Ginger Mints- to calm nausea
Farinfared Sauna- sweat out toxins, giving the liver a break

Glucose Tabs... watch out for metabolic spikes...only use if necessary (to avoid a crisis)...try to keep steady long-acting carbohydrate state in lieu of fast acting carbohydrate spikes

Reference Books
Instant Oatmeal- great for quick carbohydrates... I prefer cinnamon and apple

Air Purifier- controls your environment, and reduces additional toxins that your body has to metabolize

Organic Food- reduces toxins from pesticides, hormones, antibiotics, etc.
Water Filtration- reduces contaminates? toxins

AIP Dx and printed APF material in car, in purse, at work, at home- keep printed information with you should you need it (it has helped me several times).

Steam Mop (no chemicals)
APF (or National Porphyria Organization in Country of Origin- Reference and support
Chronic Disease Management-Personal Support
Heel Pillow to raise heels off the bed- decreases peripheral neuropathy discomfort
Pulse Ox- to test O2
Incentive Spirometer- to test lung function, esp r/t AIP crisis
Neuro Test Kit- reflexes, hot/cold, sharp/dull
Ear Plugs- sensory calming
Eye Pillow- sensory calming
Weighted Blanket- sensory calming
Weighted Heating Pad- sensory calming
Under Eye Concealer- Cosmetic Dark Circle Cover Up

Medical Alert Bracelet or Necklace with Disease Name on front and key chain flash drive encryption information on the back

Medical Alert Bracelet or Necklace with Medication Name (if appropriate...for me Neurontin) on front and key chain flash drive encryption information on the back

Encrypted Digital Medical Record on flash drive attached to key chain or in bracelet fashion...many doctors are vaguely familiar with AIP and therefor having your comprehensive medical history at their digital fingertips helps direct your prompt treatment.

People "Like" Me

Living with a rare chronic disease is a daily dance...till death we do part.
Sometimes I am at peace with Porphyria and "we" get along well...
I know what Porphyria "likes" and "dislikes"...
I try to maintain "peace" in our relationship by doing the things it likes and avoiding the things it doesn't.
Sometimes I get frustrated because no matter what I do Porphyria will flare-up without reason... that's just the way it is.
Navigating the nuances of Porphyria coupled with Autoimmune Thyroid Dysfunction and a Gluten Sensitivity can be absolutely crazy...
I have come to find that I always do best when I use my experiences as a knowledge base for something bigger than me...
Meaning...
if the challenges in my life can provide me with knowledge and insight that might help other people... well, then I cope better... I keep a more positive perspective on life.

I started this Blog on that very premise.
I hoped to pour my thoughts, research, personal happenings and feelings into one vessel (this Blog) then convert it to a book to be distributed to anyone who might find it helpful.

Honestly I am vulnerable... I get discouraged and even depressed at times...
I ask myself..."is this stuff really helpful for others?"...

Today I noticed the Blog Stats for the first time...
It was emotionally moving to realize that ~2,000 people visit this Blog from all over the world!!!
What a gift of encouragement!

Regenerated with encouragement, I will continue to do my best to complete the Blog to Book plan!!!
The Blog component should be finished September 2011.
I have reviewed a few Blog to Book programs and a few more self publishing options...

Email (nostalgicnursing@gmail.com)... insights welcome!
Best Wishes~

Monday, April 18, 2011

Image Insights

Physical Body Maintenance and Personal Presentation have always been important to me.
I have always felt the need to "do the best I can with the cards that I have been dealt".
Not from a place of narcissism...or conceit... or even social comparison...
For me...I have been taught since birth to take care of things...avoid being wasteful...make things last!
I strive to apply this concept to my health and appearance.
I encourage family members, friends and patients to do the same.
I believe that "walking the talk" sends a strong message of living by example.
If I were to guide someone to improve well being by investing in themselves yet I appeared to only half-heartedly care for myself (ie. yellowed teeth and ill fitting clothes)...what message am I sending???
A message that I don't truly value myself or the words I speak...because I don't "practice what I preach".
I have found that without a doubt people judge how well you will be able to assist them by how well you take care of yourself.

My routine involves:
Exercising Regularly (elliptical machine and resistance bands)
Stretching Regularly to maintain flexibility (gentle yoga poses)
Eating Mindfully according to my individual metabolic chemistry
Maintaining Stylish Healthy Hair
Participating in Therapy to work through life challenges (as needed)
Keeping my nails (fingers and toes) well groomed and looking nice
Following Up with my Health Care Providers as ordered to maximize optimal wellness
Drink Plenty of quality non-chlorinated/ non-fluoridated, natural water
Observing Current Fashion Cues...dressing modestly...(enhancing to positive aspects of my body type)
Cleansing, exfoliating, hydrating and moisturizing my skin (and using sunscreen)
Brushing and Flossing my Teeth
Whitening my teeth to remove stains
Seeking meaningful human interaction daily!
Avoiding negativity whenever possible
Continuing Education
Taking Saunas to detoxify my body naturally
Spending time every day with my 14 pound Silky Terrier

Even with all of that "front loading" to maximize wellness...I struggle with health and self-esteem challenges.
With AIP, Early Menopause (due to hysterectomy), Autoimmune Thyroid Disease my metabolic efficiency has been severely impacted. I have gained 40lbs in 4 years and gone from a 6/8 to a 14/16. It is very hard for me to accept the new me.
My face is round, my eyes have become deep set with dark under eye circles.
My skin tone and elasticity has changed...giving way to fine lines and wrinkles.

In my best attempt to manage I have learned that:

Re: Clothes
High waisted, full A-line dresses that have 3/4 sleeves, a V-neck and fall just below the knee are the most flattering for my new body type (large pear).
Pointed toe shoes and accent jewelry complete my best look.
I love the "BORN" shoe line for a modern fashionable look with excellent comfort and durability.
Re: Hair
Shoulder-length or longer full hairstyles with layered, "piecie" bangs are best for my head/ face shape (large and round)
Re: Make-up
A good under eye concealer is a must! Moisturizing, hydrating lotion or cream, organic mascara, eyeliner, eye lash curler, light rouge, matte loose finishing powder and tinted lip balm finish my best look.

Seeking Updated Professional Advice I have scheduled a few hours at a local day spa requesting the following services:
-A one hour make-up lesson to discuss my personal concerns and learn new techniques
-A hair lesson with a Master Stylist to discuss my concerns and learn new techniques
-An eyebrow wax and shape

Look Your Best = Feel Your Best = Act Your Best = Impact the Well being of Others the Most!!!

Initial Visit w/ A Hepatologist



Outside of visiting a Porphyria Specialist, for formal diagnosis, and my new AIP Clinical Study participation... I have been at a disadvantage in securing a long-term medical provider to manage my health needs. The providers that I have approached know little about AIP and do not seem to have the time to invest in learning (even with printed material distributed at office visits). This has been a particularly difficult dynamic for me...knowing more about AIP than the professionals I put my life in the hands of...well...it provokes fear, insecurity, and anxiety. I continue to politely offer educational information...I keep a current Personal Health Record (Formal AIP diagnosis letter...copies of all recent lab/ imaging results...relevant medical history documentation and professional AIP educational literature).
This particular new patient visit was different!
Dr. M knew quite a bit about AIP!
Dr. M educated me on the nuances of the disease and offered long-term medical collaboration.

Dr. M's AIP Liver Surveillance Plan entailed:

1. MD office evaluation Q6months
2. Blood work Q6months
3. Liver Imaging Studies Q6months (Ultra Sound Q12ms/ alternating with MRI Q12ms)
4. Eventual Liver Biopsy to determine cell status/ level of disease impact on liver
5. Scope to assess Gluten Intolerance and Poor Vitamin Metabolism/ Absorption
6. Long-term collaboration and disease management support
7. If ever necessary... due to AIP related cirrhosis or primary liver cancer... a liver transplant
8. The option to receive the monthly Panhematin treatments (recommended by Mount Sinai)at his facility, under his medical guidance.

At the close of my appointment I became moved by the new found sense of medical security Dr. M had provided me.
I am so very grateful to be blessed with Dr. M as a knowledgeable new member of my health care team!!!

Sunday, April 10, 2011

2011 Porphyria Awareness Week

Written for the April 2011 Emergency Medicine News Letter
@ my Regional Medical Center

April 16- 23, 2011 is Porphyria Awareness Week

As a member of the American Porphyria Association (www.porphyriafoundation.com ), and as an individual with Acute Intermittent Porphyria (AIP), I have been asked to raise awareness of this rare disease. AIP is a genetically inherited disease that alters specific enzymes of the heme synthesis pathway. In times of crisis the accumulation of neurotoxic byproducts cause a cascade of potentially life threatening events including, but not limited to: pain, nausea and paralysis. AIP also has a significant relationship with the liver. AIP is treated with carbohydrate loading, IV Dextrose, IV Morphine (for pain) and most importantly IV PANHEMATIN (http://www.aiporphyria.com ).

It has been a long journey to diagnosis and treatment, confirmed by genetic testing at Mount Sinai Hospital in NYC. To raise awareness at our medical center I will be distributing AIP information (including a free online CME opportunity) outside of the upcoming ED Staff Meeting 4/14/2011.

Thank you for your time and professional consideration.

Best Wishes~

Johns Hopkins Scholarship to Reach a Goal

The Guided Care Nurse

In Guided Care, a specially educated registered nurse plays a critical and central role in ensuring that patients receive high-quality and coordinated care. Guided Care Nurses reconnect people to the center of care, are empowered to take control of their patients’ care, provide personal support to each patient individually, and offer support to family caregivers.

To become recognized professionally as a Guided Care Nurse, a candidate with a nursing degree and a current license must complete an accredited online Guided Care Nursing course. Upon completion of the course, the nurse is eligible to take an online examination leading to the American Nurses Credentialing Center's new Certificate in Guided Care Nursing.


(http://www.guidedcare.org/nurse.asp)

Email to my Supervisor

I know that the hospital and our department have financial restraints related to approving continuing education opportunities.
I have invested a lot of myself in trying to participate in the JHH “Guided Care Nursing: program.
I have submitted two separate employee request packets over the past 6 months for this program and have not heard back.
I am SOOO Happy to find out that there is now a 100% scholarship available through JHH!!!!
I have completed and submitted all but one requirement….
They require a letter of support from my Supervisor.
I have written a proposed letter…it is attached… if you are ok with it can you Please print it on facility letterhead and sign it???
I need to get it in ASAP to be considered for the next class.
Thank You So Much… I really Appreciate Your Help!!
I truly believe that this information can help improve ED Pt Care Coordination!!!

The Letter

Monday March 28, 2011
Dear Sir/ Madam,

I am aware that our Emergency Department Patient Care Coordinator (ED-CC) has applied for your “Guided Care Nursing” course scholarship. She submitted the essay copied below. The facility and I are indeed congruent with our ED-CC's goal to improve Emergency Department Care Coordination by integrating the principles of “Guided Care Nursing”.

I have been actively searching for a method to finance the Guided Care Nursing Course since I discovered it about six months ago. The day I wandered into the general information web page, describing exactly what a “Guided Care Nurse” does I knew it was the “Cinderella’s Slipper” that I had been seeking. I have been a Registered Nurse working in Case Management settings for seventeen years. “Guided Care Nursing” is a concept that I have always believed in and informally practiced as often as possible.
Currently I am employed by a large medical center, as a Patient Care Coordinator in the Emergency Department. I assist patients, medical staff and others with planning appropriate care, placement, resource intervention, and long-term medical management. We have a large population of patients that are “repeat customers”. Many of these patients have multiple chronic conditions with little to no follow-up support system. They use the Emergency Department as their sole primary resource.
A scholarship would provide the means for me to complete the “Guided Care Nursing Course” and broaden my skill base. I would be better equipped to assist these “repeat customers” with innovative chronic health management strategies. This would not only impact optimal patient wellness, but create improved financial responsibility.
Currently my hospital is fiscally burdened with uninsured and minimally insured “repeat customers” who use the Emergency Department as their sole primary resource in managing multiple chronic issues. Implementing “Guided Care Nursing” Strategies as part of Emergency Department Patient Care Coordination would seriously benefit the patients we serve and the financial impact absorbed by the hospital.

Respectfully,


Email Notification of Award

Dear Participant
Congratulations! You have been awarded the Online Guided Care Nursing Course Scholarship to attend the session dated May 2, 2011 – June 10, 2011. We look forward to your participation in this continuing education program. Course logistics for the May 2nd session will be sent to you in the near future. If you have any questions or require additional information, please feel free to contact our office at 443-287-4745 or via email at ijhn@son.jhmi.edu.


Follow Up

I am excited to gain insight and intervention strategies to improve our ER patients quality of care/ quality of life.
My thought is that the Care Coordination/ Case Management Skills I gain, will also add to my knowledge base as a community resource in Chronic Disease Health Care Management. As life slowly unfolds and my career path becomes illuminated... I feel drawn to assist people who are faced with the challenge of navigating life with chronic health issues.

After completing this program (& Guided Care Nursing Certification Exam) I hope to begin preparing for the ANCC National Case Management Certification Exam for RNs.
( http://www.nursecredentialing.org/NurseSpecialties/CaseManagement.aspx )

Continuing Education Interests:

Medicare Guidelines
Environmental Health
Genetics/ Genomics
Geriatrics
Autoimmune Disorders
Acute Porphyria
**

Friday, April 8, 2011

Participating In A Clinical Study


This day last week I went to New York City for the first time in my life.
The anxiety of anticipation and expectation was by far, greater than any anxiety related to the actual experience... although subway navigation during rush hour on a Friday morning when computers are down... well, that was a bit "nerve racking"... especially with twin 7 year old boys prone to impulsive behaviors and high energy levels!

After a 2 hour navigational adventure, I arrived at Mount Sinai ready to participate in a Clinical Study designed to examine the long-term effects of Acute Intermittent Porphyria. I respect research and value evidence-based decision making. I am excited to be a part of the study.
The initial process was pretty standard, involving paperwork...consent review and signature... then a thorough question and answer session. Looking back, my only regret was that I should have eaten better and had more time to transition... I felt more "scatter-brained" than I would prefer but I gave them access to this blog and copies of my medical record... they have a good amount of solid information to start with.
About my "colicky-cranky" AIP...
I explained that significant stress is an unavoidable component of my daily existence. After failed in-vitro attempts we were overjoyed to build our family via international adoption. Unfortunately our ,now 7 year old, twin sons have significant behavioral demands linked to early life challenges. I try to be very careful in managing my lifestyle choices to avoid triggering my AIP... and yet it remains a constant "background music" almost every day of my life. Stress and illness seem to be the triggers I primarily cope with now that I am post-menopausal (2nd to a total hysterectomy in 2008).
The suggested treatment plan is to receive monthly Panhematin infusions as crisis prevention. I agreed.
I haven't started yet...but I fear the risks and the unknown.
Even though my quality of life has been significantly altered...I am familiar with my AIP... I am used to it.
It would be so awesome to regain some vitality... but is it worth the risk of getting a clot and becoming paralysed or getting phlebitis... or, or, or...
Sometimes it is just NOT helpful to be a nurse... my mind races with the "what ifs"...
I will try the infusions... because physically I feel like my body is decomposing daily... more and more toward a place that I really don't want to be.
I will see a Liver Specialist next week (to establish long-term monitoring) and I will set up an appointment with a Thyroid Specialist to deal with my Thyroid issues.
I am so very fatigued both mentally and physically by constant health issues that make me feel terrible... BUT I will continue to try my best to be positive, to be hopeful and to seek solutions.

Life Is Hard


Living the way I feel I should, involves tremendous amounts of selfless giving.
Tonight I feel like I gave my all today and I have nothing left.
I started the day at 6am fasting for some routine blood work and a liver ultra sound... by 8:30am I had completed those tasks and was on-duty at work... drinking instant oatmeal as I juggled multiple issues.
As the day unfolded... human need exploded all over the Emergency Department.
I worked diligently to meet the biopsychosocial needs of my patients and was fairly successful by days end.
I like making a positive difference... it is the gas that keeps my engine running.
There was a time,not so long ago when I was out of work for about a year...
My health presented similar challenges then as it does today... only without the reward of making a positive difference in the lives of others on a daily basis... I became unsure why I existed...without purpose...
Physical endurance and pain continue to challenge me in my desire to maintain a half-time professional career... but I am determined to push through as long as I can.
As a nurse I have the opportunity to see human situations that give me perspective on life...
I am truly blessed in so many ways... so very many people have much, much worse conditions... I remind myself to be grateful when self pity or other unhelpful feelings consume my thoughts.

Friday, March 18, 2011

Enzyme Follow-Up



What about enzyme replacement to treat AIP?
"Porphozym by Zymenex"...

I sent an email to one of the top AIP Specialists in our country asking him this question.
He responded within 24 hours!!!
What a thoughtful person.

His reply basically stated that "Porphozym" was found to be ineffective in clinical trials and was therefore never offered as treatment to the general population.

IV Hematin remains the treatment of choice for AIP.

Colonoscopy


At 9am this morning I was wheeled via stretcher to the procedure room for a colonoscopy.
At 41 years young I had my first colonoscopy 3 years ago because my father struggled with Cancer that began in the colon.

I am glad I went 3 years ago... they found and removed an abnormal area.
I didn't need to return for 3 years!
Now...after today... I am cleared for 5 more years!!!
The Colonoscopy is a great Cancer prevention/ early detection tool that I promote Whole-Heartedly!!

But...
It is a little harder for people with AIP.
My first experience was awful! I was absolutely miserable and stayed unwell over a week!
But...
I didn't know that I had Acute Porphyria back then!

My AIP Colonoscopy Protocol:
1. Schedule the procedure first thing Friday morning (if possible)
2. Take Thursday and Friday off from work (and make sure the weekend is clear of obligations)
3. If I still had a menstrual cycle I would try to schedule the procedure the week or two after menstruation ends
4. I use a "Dulcolax" and "Mirilax" colon "cleanse" process outlined by my Gasteroenterologist
5. The day before the procedure I am only allowed "clear" liquids...so I drank GREAT quantities of blue "Gatorade" to keep my carbohydrates from dipping too much... this also helps in avoiding dehydration
6. I thoroughly went over my AIP diagnosis with the GI doctor and the Anesthesiologist... giving them both printed literature I ordered from "The American Porphyria Foundation"
7. I determined ahead of time which medications I would be receiving and made sure that they were AIP "SAFE"
8. The nurse started an IV infusion of dextrose as soon as I arrived
9. I packed a small meal for after the procedure
10. I went home and rested for the remainder of the day

Look Good = Feel Good


My Porphyria activity/ severity can be clearly gauged by how dark my under eye circles become.
I honestly look like Old "Uncle Fester" from the "Addams Family"!!!
If I am super sick...well it just doesn't matter how I look.
But...
If I can manage to remain actively engaged in life I do my very best to do so.
I try to invest in my appearance... I want to do the best with what I have... It keeps self esteem at a positive end of the spectrum and can lift spirits too!
So...
The dark circles are a real problem!
Hiding them with traditional makeup is impossible...I have tried them all,
I did some research and found a "medical make-up" called "Dermablend"...
I bought the "Quick-Fix Concealer" made for "severe skin flaws"...
It works very well and I don't see "Uncle Fester" when I look in the mirror anymore!!!

Filling In The Gaps


It has been over a month now since my last entry.
Life has been pretty challenging between then and now, but not all bad.
My Porphyria calmed a bit and we traveled to see friends and family...
Being with the ones you love is like food for the soul!

Then after returning home a respiratory virus took over our home and local community.
The curious connection I noted was that as I "cranked up" the juicer with fruits and vegetables I was able to "crank up" my immune system!
I never got the full-blown illness everyone else did, which was great!

However...

As my immune system was going "full tilt" my Auto-Immune Thyroiditis became a big swollen issue of dysfunction... super tired...super weak...severe headaches...no endurance...significant neck/ throat discomfort...then after about a week of this my Liver began to feel irritable and swollen...I began retaining fluid...especially in the abdominal area... Thankfully, I never developed any Porphyria issues this episode.

It appears that when my immune system is turned up...it protects me from illness, but attacks my Thyroid too.
Then my Thyroid in its dysfunctional state forms an "unhealthy relationship" with my already burdened Liver causing increased malfunction.
The "Up Side" to all of this is that as my immune system gears down after the threat of illness is removed... my Thyroid reduces in size and improves functioning.
As the Thyroid improves...so does its "relationship" with my liver...
then my Liver begins to return to baseline... the fluid stops accumulating... and I can re-engage in a semi-normal life...smile!
Some might ask...why didn't you go to the doctor???
I have... thus the complete Thyroid "testing / work-up"..several times.

Diagnosis:
Chronic Auto-Immune Thyroiditis with benign nodules...
intermittent episodes of Hypo-Thyroidism and even occasional Thyroid Storm if manipulated/ palpated (physically touched)

Treatment:
Tried Synthroid...did not tolerate... migraine headaches, increased "storming" (firing erratically... too high...then too low)and a whole-body sensation of being "squeezed"... these issues persisted for about three weeks after discontinuing the medication.

Final Medical Advice:
"Learn to cope with the episodes and monitor Thyroid/ nodules with regular testing"

So...I have been coping with these intermittent episodes for four years now.
My liver function has only been impacted during these "Thyroid Episodes" for about a year now.

My new proactive plan:
I found a Hepatologist and have an initial appointment next month.
I found a Thyroid Specialist and am in the process of setting up a new patient appointment.
I joined a Long Term Clinical Study: Acute Porphyria... my initial appointment is in about three weeks.

Wednesday, February 2, 2011

Heavy Metal Cells: Human Bean Bag



Today is my second day in bed, out of work and out of commission.
Two days ago my "Colicky- Cranky" Porphyria began to transition into something a little more...
Now my body feels as if every cell has become a heavy-metal-pellet and I am, in a sense, a giant 10 ton human bean-bag!
Wow what a picture huh!
My limbs feel so heavy to lift, my body feels so weak I drop everything and can only walk short distances without rest... showering leaves me feeling as if I ran a 10k...
I slept almost 24 hours straight and the nausea, eye, ear, limb and joint pain are beginning to subside... Gatorade and Quiet, Dark resting spaces help a lot! Phenergan, Naproxen and Ativan have also helped. I have found that chocolate, peanut butter, oatmeal no-bake cookies are great for calming AIP too!

I saw this flare-up coming... I have been juggling life at top speed...feeling OK...not taking rest periods...becoming increasingly tired...until I became so fatigued that I couldn't even think straight. The world around me sees me doing "it all" and pushes harder...expects more and more... they can't see what happens on the inside...and sometimes they don't understand when an Acute Phase of Porphyria strikes...
They can lack compassion and become frustrated with me...
I can become frustrated with my own body...not performing the way I want it too...
Again...another lesson in letting go of my drive to be in control.
I am NOT in control and I must accept that AIP and I will coexist for the rest of my lifetime...
I have to be kind to myself, listen to my body, nuture myself...stop the negative self talk and educate the world around me... forgiving the ignorant for their unkind harshness.

This is not a new challenge for me... just another learning cycle.

What about enzyme replacement?
Porphozym by Zymenex... it appears to be available in some areas of the world...
When I am feeling a bit better I will research it some more.

As a nurse, I feel that dextrose IVs are helpful and a fairly easy treatment intervention when oral carb-loading isn't working
but...
Heme Infusions via a central line can present a slew of added risks, if the attack is NOT at the life-threatening stage and if the healthcare team has Little to NO experience/knowledge about AIP!

I feel like there is no one to take care of me...and I get scared.
My AIP specialist is several states away and hasn't seen me since my diagnosing visit going on 3 years ago! He is more of a researcher than a direct care provider...he says any follow up questions I have can be handled by phone... I feel unimportant and lost medically...as I stated in a previous post, my hematologist of 4 years doesn't even know that AIP is a disease of the liver...he is too busy to read the educational literature I provide for him.

Thus my quest to create the role of Porphyria Disease Management Nurse...so that others like me might have someone to talk to...a resource...an educator... Diabetics (another type of metabolic disease) have this type of system with the DNE (Diabetic Nurse Educator).

The American Porphyria Association does a terrific job of providing printed educational materials and advocating for Porphyria Sufferers in general... but due to liabilities cannot veer too far into medical advisement...

Well... I am getting tired out and need to rest...more thoughts to come!

Saturday, January 29, 2011

Colicky-Cranky Porphyria



Although my official diagnosis is established as Acute Intermittent Porphyria, I propose that my condition is better known by me as...
"Chronic Colicky-Cranky Porphyria"
My "Acute" times are truly "Intermittent"...but long episodes of dis-ease, generalized ill-feeling, mental/emotional fragility, poorly defined but ever present pain, GI dysfunction, nausea, headaches....and such can last days...weeks...even months.

It is as if my internal environment just feels very "Colicky & Cranky".

*Quiet, low stimuli environments really help but in REAL Life with kids, careers and spouses this intervention is hard to arrange.
*Ativan, Naproxen, Phenergan and Ginger help a lot...but can cause significant drowsiness...caution is needed when health care career and child rearing responsibilities are to be considered.
*Prayer and Spirituality are comforting
*Calls to loved ones helps
*Writing/ Creative Expression helps vent some of the "yuckie feeling"
*Exercise as tolerated definitely helps...but too much and everything gets MUCH worse...balance custom to the situation is key.
*Strict adherence to my nutritional plan helps keep extra health stressors at bay so my body can focus on calming it's current challenges
*Extra Sleep!!! Is one of the best treatments I know.
*Being kind to myself...hot cups of tea...pretty flowers...warm fuzzy blankets...