Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x1, nausea
 GU- altered voiding, neuro-bladder holding urine
 Muscular- ok
 Immune- ok
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- clear thinking, calm
 Sensory- no problems
 Pain/Comfort- legs and heels dull ache, nausea
 Sleep/Rest- fair night sleep (took Ativan) asleep at 12midnight & up @ 6:30am, teach at community college 8am to 2:30pm, active evening
 Self-Propelled Disease Management:
-maintain appropriate carbohydrate intake
-medication as prescribed
-stress management

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x1, nausea
 GU- altered voiding, neuro-bladder holding urine
 Muscular- ok
 Immune- ok
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- sad & tired due to family-turbulence/late night, clear thinking, calm

 Sensory- no problems
 Pain/Comfort- legs and heels dull ache, nausea
 Sleep/Rest- poor night sleep (took Ativan & Naproxen),ACUTE family-turbulence with 19 year old daughter interaction until after midnight. Up at 6:20am for 8hour work day. Extremely tired afternoon.
 Self-Propelled Disease Management:
-maintain appropriate carbohydrate intake
-medication as prescribed
-seeking effective problem-solving strategies to assist with family-turbulence.

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x1, no problems, ate pizza for supper
 GU- altered voiding, neuro-bladder holding urine
 Muscular- ok
 Immune- ok
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- clear thinking, calm, good multi-tasking
 Sensory- no problems
 Pain/Comfort- legs and heels dull ache
 Sleep/Rest- good night sleep (took Ativan & Naproxen,busy day at work, busy evening w/family
 Self-Propelled Disease Management:
-maintain appropriate carbohydrate intake

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- Nausea most of day, BM x1, Staying Gluten, Soy and Dairy Free (& caffeine free)
 GU- altered voiding, neuro-bladder holding urine
 Muscular- ok
 Immune- mild seasonal allergies
 Cardiac- heart palpitations & racing in night...clears with bearing down
 Respiratory- no problems
 Psychiatric- irritable, delayed word recall, delayed complex thinking
 Sensory- eye pain, headache, more with light
 Pain/Comfort- moderate pain between shoulders, intermittent right lower leg pain
 Sleep/Rest- poor night sleep, to bed @ 10p...up at 3am..void...heart issues...awake till 5:30am...up at 6:30am...to work for 8.5hrs at hospital..Very tired all day.
 Self-Propelled Disease Management:
Family member's thoughtlessness sabotaging good health, addressed family re:issue.
Good Carbohydrate Loading to calm Porphyria
Rest all evening
To bed for the night at 8:30pm with Ativan and Naproxen

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- Staying Gluten, Soy and Dairy Free
 GU- no problems
 Muscular- ok
 Immune- ok
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- no problems
 Sensory- OK
 Pain/Comfort- moderate pain between shoulders
 Sleep/Rest- good night sleep, busy day Church, shopping, domestic duties, no rest... tolerated well
 Self-Propelled Disease Management:
Prepared Course Outline for, "Providing Care for Seniors: Meeting the Challenges of Aging". (I will be presenting this new 3 day program for my local community college in November.)
Noting how vital it is to establish resources for support, treatment and education when facing chronic situations.

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x2 (no problems), staying Gluten, Soy and Dairy Free
 GU- no problems
 Muscular- weak, mild activity intolerance(continued improvement)
 Immune- seasonal allergies/ "hay fever" (continued improvement)
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- no problems
 Sensory- OK
 Pain/Comfort- moderate pain between shoulders
 Sleep/Rest- good night sleep, busy day cleaning no rest
 Self-Propelled Disease Management:
feeling good "back on the Wagon" ...100% Gluten, Soy and Diary Free for 3days
cleaned my personal home office today, re-organized area, purged

Americans With Disabilities Act: AIP

Proposal for "Reasonable Accommodations" at Work

Friday September 24, 2010

Dear Human Resource Representative,

I have been a job-share employee in the Patient Care Management Department, for approximately 6 months now. Emergency Department Patient Care Coordination (Wound Ctr & Out-Pt Recovery Unit coverage as well) is an excellent fit for me. I am a dedicated employee, I love my job, and I feel I do my job well. I have a strong work ethic and hope to have a long career here.
I have Acute Intermittent Porphyria (AIP), a rare genetic metabolic disease affecting my heme synthesis process and causing neuro-toxic episodes. I try very hard to manage my disease well and minimize the potential of acute exacerbation. I do not want to draw attention to myself by asking for “special considerations”, but the reality of the situation is that Porphyria is inborn, incurable and does affect my life.
In a proactive attempt to maximize my career potential with the hospital, I would like to discuss the possibility of outlining some reasonable accommodations that won’t put an undue hardship on the hospital.

1. Modified Leave Package

- I am concerned that with the hospital’s standard unplanned leave/ occurrence policy that I will be reprimanded if my disease activity causes more than 3 occurrences in one year.
- I do my best to come to work as scheduled, but there are times when it is unsafe to do so.
- Can we please discuss possible modifications?

2. Rest area at lunch

– Some days with Porphyria, I am not feeling well, but it is mild and I am certainly able to work. These days mornings are manageable but afternoons can be challenging.
- I have found that if I lay down mid day, in a quiet, darkened place with minimal sensory stimuli, the rest gives me enough energy to get through the afternoon much better.
- Can we please discuss a possible arrangement?

3. Flexible Scheduling

- I am a salaried employee scheduled to work from approximately 8a to 4:30p, due to the needs of the job most days (85%) I do not leave until after 5 or 5:30p. Is it possible to track extra hours worked, so that when my disease occasionally flares I have some “banked” time???

4. Work space environment

- I have light sensitivity related to neuro-sensory sensitivity. I have been told that if I bring in a lamp, it can be inspected for safety and if approved I can use it in my work space in lieu of the overhead florescent lighting.
- Having active AIP causes Multiple Chemical Sensitivities that when exposed to can trigger acute neurological attacks, this includes cleaning chemicals. My work space is already set up ideally. I keep the sliding windows closed when cleaning chemicals are being used in my area.

5. Eat while working in personal workspace

- Carbohydrate intake/maintenance is an essential part of my disease management. I currently pack my food and eat at regular intervals throughout the day, while working in my work space (never in front of patients or during interpersonal interactions.)

Thank you in advance for your professional consideration and confidential discretion. I look forward to collaborating with you to identify reasonable accommodations that don’t put an undue hardship on the hospital.

Respectfully~
T. Suzanne Jaynes RN, BSN, ED-CC
Patient Care Management Services

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x1 (no problems)
 GU- no problems
 Muscular- weak, "heavy feeling limbs", activity intolerance(improved compared to yesterday)
 Immune- seasonal allergies/ "hay fever" (improved compared to yesterday)
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- mild cognitive "fog", mild word recall delays (improved compared to yesterday)
 Sensory- chronic light sensitivity to eyes, sensitivity to noise and slight odors
 Pain/Comfort- R lower leg mild ache (less than yesterday)
 Sleep/Rest- good night sleep, good morning, moderately tired from 2pm on
 Self-Propelled Disease Management:
took Ativan and Naproxen at bedtime last night with good results
written accommodations proposal submitted to my supervisor in a proactive attempt to protect job status

Narrative Note

Today was much better than the picture yesterday's anxiety had painted.
My internal milieu was calm and peaceful.
I had a good night's rest and it felt good to be engaged in life...making a difference.
I do reap great personal reward as a Nurse, coordinating care for people in there time of need.

My morning went by without a problem, then around 2pm "the wind left my sails".
I became so heavy and tired I could hardly hold on. Every task became a major undertaking and I couldn't wait to get home and lay down.

It is now 7:22pm and I am ready to go to bed for the night.

Tomorrow is another work day!!!

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x1 (no problems)
 GU- no problems
 Muscular- weak, "heavy feeling limbs", activity intolerance
 Immune- seasonal allergies/ "hay fever" (sinus congestion, sneezing, itchy eyes)
 Cardiac- occasional, intermittent heart racing
 Respiratory- no problems
 Psychiatric- mild cognitive "fog", mild word recall delays (simple tasks take longer, complex tasks slow to accomplish)
 Sensory- chronic light sensitivity to eyes, sensitivity to noise and slight odors
 Pain/Comfort- R lower leg mild ache
 Sleep/Rest- good night sleep, good morning, EXTREMELY tired from 2pm on
 Self-Propelled Disease Management: stay home extra rest yesterday, spoke with job supervisor today, proactive attempt to protect job status... requesting "reasonable accommodations" for "chronic disabling condition" (will outline in writing to submit)

Narrative Note

Well it was a week ago this morning that I was discharged from my local hospital, after 20 hours of IV Dextrose.
I felt absolutely terrific the morning I left the hospital. I came home and took it easy. As the day went on my nervous system became increasingly more irritable. I wasn't scheduled to work again for 7 days so I decided to manage by resting at home and maintaining carbohydrates orally. It has been a week of moderate turbulent nervous system activity coupled with the discomforts of chronic flaring thyroiditis.
Today I find my mind catastrophizing... I am anxious and intolerant of my family members... I am scheduled to work tomorrow... what if I am still having "neuro-turbulence" ? What if I can't manage it at work... if I miss too much work I could lose my job. I am also scheduled to teach a 5 day Certified Medicine Aide class for my local community college. That means that between the two jobs, I am working full weeks, for the next 3 weeks. Then I am scheduled to go on vacation with my mother, back to my home town. Oh how my heart has been set on that get-away with mom... I feel overwhelmed, over-booked, and anxious about how things are going to turn out in the midst of uncontrollable "neuro-turbulence" and the lurking potential of AIP escalation.
I tell myself to stop. Calm Down! It is hard to do, especially when I never know when AIP will "turn-up the volume" and "sucker-punch" me when I need to be at my best. Ativan helps in these situations, but I am a, "pill-phobic" person by nature (I absolutely hate taking medication). This morning, I took the Ativan and I am beginning to calm down.
I need to remember that when I am at peace with my disease I feel better. It seems that when I have an extended period of relative, "neuro-calm" (like this summer) I like to forget that my disease is part of who I am and how I need to manage my life. When considering life: I need to take a less intense perspective. I need to look at my date book and avoid long stretches of back-to-back obligations. I need to schedule "Rest Days" to give my nervous system a break. If the day comes and I am feeling great, it becomes a happy gift. My family and I can take a spontaneous adventure, or I can do a project that I have wanted to do. If the, "Rest Day" comes, and I have been struggling to get through each day prior, it feels as though I have finally crossed the finish line! Yeh Me!!
I rest, I eat carbs, I try to keep sensory stimuli to a minimum, I even take medications to help sometimes. Then after a few days I usually feel well enough to go through another cycle. This plan works well for me when I stick to it and when my family supports it. When I veer from the plan it becomes a gamble... a roulette of sorts...occasionally it turns out alright, but the majority of the time over the last several years... it has turned out very badly.
Fortunately I had a fairly "neuro-calm" summer. I was faithful to "the plan". Then as the end of summer approached... I veered from the plan...I took a gamble... I over booked and under-rested myself. I gambled and I lost. The added stress of being frustrated at my disease is a vicious cycle... It is really my own fault for having magical thinking... these situations don't just disappear.
As a nurse I have seen it with many many diseases... Diabetics, Bipolar patients, and the list goes on. When you feel better you think...I don't need this "treatment plan" anymore... and boom there we all are with a BIG reminder that, "Oh Yes we do"!!

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM x2 (no problems)
 GU- no problems
 Muscular- weak, "heavy feeling limbs", activity intolerance
 Immune- seasonal allergies/ "hay fever" (sinus congestion, sneezing, itchy eyes)
 Cardiac- occasional, intermittent heart racing
 Respiratory- no problems
 Psychiatric- mild anxiety, feeling "unsettled" within
 Sensory- chronic light sensitivity to eyes, headache
 Pain/Comfort- achy body
 Sleep/Rest- mildly tired/sleepy, broken night sleep
 Self-Propelled Disease Management: stay home extra rest, health related research/ reading

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels
 GI- BM (no problems)
 GU- no problems
 Muscular- weak, "heavy feeling limbs", activity intolerance
 Immune- seasonal allergies/ "hay fever" (sinus congestion, sneezing, itchy eyes)
 Cardiac- heart racing/palpitations during night (wake out of sleep)
 Respiratory- no problems
 Psychiatric- calm, no problems
 Sensory- chronic light sensitivity to eyes
 Pain/Comfort- Intermittent shooting R lower leg 6/10
 Sleep/Rest- mildly tired/sleepy, broken night sleep
 Self-Propelled Disease Management: stay home extra rest

Narrative Note

This past week has been turbulent and consistently inconsistent related to my Porphyria management. I remain in forward motion trying to get beyond this state of "increased activity" but seem to be having some trouble.
These "colicky" episodes are not unfamiliar to me... sometimes a "spell" can linger for weeks or months before I feel consistently well again.

What happens when these periods come...
I have some energy in the morning so I do normal life/family things...then I crash a few hours later...feeling like I haven't slept in a month...my limbs feel like they weigh 500 pounds each...I drop everything I pick up and if I don't eat A LOT of carbs I get VERY irritable, intolerant of everything... then more nausea, pain, etc. ... I try very hard to eat the carbs.
The carbs do help calm my porphyria, but they don't take it away.

I have been trying to keep a gluten-free/dairy-free diet because I feel SO much better when I do (a lot less GI issues and a lot less joint pain/headaches). The problem with keeping this sort of diet is planning ahead and stocking the pantry with "Good Carbs". When I haven't been disciplined enough to plan ahead the "Good Carb" pantry is bare and "Bad Carbs" are readily available. I find myself eating processed foods, pastas, breads, icecream...anything to calm the internal roar of my "Porphyria Beast"... the result is initially positive with a Big "backfire".
So today I am trying to get back on track from yesterday, when I ate enough "bad carbs" and dairy to support a small village for three days!
Let's not even talk about my once (now doubled) size 6 figure...I do miss that, and am forever trying to get back into those jeans I keep in the back of my closet.

My husband believes that exercise cures all, and as a nurse I know that consistent physical exercise is key to a healthy lifestyle. During my "good stretches" I try very hard to exercise on a regular basis, but when the volume is turned up on my porphyria I struggle to exercise. There have been times that I try to "push through it...be tough"... I get on my elliptical trainer and either my legs and feet go completely numb or I can't expand my lungs to breath, or both...VERY scary! Not the same as being unfit... because on any other non-porphyria day I can do an hour at hard levels without issue.

The Key Elements of My Porphyria Disease Management are:
1. Educating myself about my Porphyria and understanding how my body works
2. Adjusting my lifestyle to optimize well being
3. Making Peace with, "The Cards I Have Been Dealt"
4. Asking God to "hold my hand" mentally when the "ride" gets scary

I have had an extended "good" phase that lasted almost all summer, for that I am so grateful. Now that Porphyria's volume has turned up again... I am writing again...using writing as a coping tool with the goal of helping others... I am going to church again...sadly I seem to get "too busy" during my "good phases" to do the things that mean the most to me... what irony!

Daily Post

 Neuropathies (central, autonomic and peripheral)- chronic heels (can't stand to let them touch the bed at night, hard to walk on sometimes...irritable)
 GI- BM (no problems)
 GU- no problems
 Muscular- weak, "heavy feeling limbs", activity intolerance
 Immune- seasonal allergies/ "hay fever" (sinus congestion, sneezing, itchy eyes)
 Cardiac- orthostatic hypotension (unable to stand during church/mass d/t extreme light-headedness)
 Respiratory- no problems
 Psychiatric- calm, no problems
 Sensory- chronic light sensitivity to eyes
 Pain/Comfort- Intermittent shooting R lower leg 6/10
 Sleep/Rest- Very tired/sleepy
 Self-Propelled Disease Management: see today's narrative note

Daily AIP Blog

 Neuropathies (central, autonomic and peripheral)- both heels
 GI- chronic mild nausea, increased gas (after eating wheat/gluten), no BM
 GU- dark urine (mild tea color)
 Muscular- exercise intolerant, weak, tires easily, hard to lift
 Immune- seasonal allergies ("hay-fever")
 Cardiac- no problems
 Respiratory- no problems
 Psychiatric- no problems, calm, tired
 Sensory- eyes sensitive to light (mild)
 Pain/Comfort- intermittent shooting pain with dull ache background (R lower leg & between shoulders/down spine)
 Sleep/Rest- little rest (busy day), overnight sleep was good
 Self-Propelled Disease Management: carbohydrate maintenance, self awareness (blog expression)

First 24 hours

 Neuropathies (central, autonomic and peripheral)- Heart, Heels, R Lower Leg
 GI- Chronic Mild Nausea
 GU- Dark Urine
 Muscular- Weak
 Immune- Seasonal Allergies
 Cardiac- Occassional Palpitations/ Racing
 Respiratory- No Problems
 Psychiatric- Vulnerable, Slight Fuzzy Thinking, Pretty Good
 Sensory- Eye Pain R/T light
 Pain/Comfort- Intermittent Right Lower Leg (shooting/aching 10 out of 10)
 Sleep/Rest- Poor, Insomnia (trouble falling/staying asleep)
 Self-Propelled Disease Management: New Rx: Shaded Progressive Lense Eye Glasses, Ativan QHS to assist with sleep

Real-Time Life w/AIP Posting Template

 Neuropathies (central, autonomic and peripheral)
 GI
 GU
 Muscular
 Immune
 Cardiac
 Respiratory
 Psychiatric
 Sensory
 Pain/Comfort
 Sleep/Rest
 Self-Propelled Disease Management: (free text)

Real-Time Life w/AIP

As initially proposed, "the blog plan" is to go "real" starting tomorrow... September 17, 2010, the 2 year anniversary of my official AIP diagnosis. As life would have it (and as the nature of the disease process goes) I found myself hospitalized the night before last due to an acute attack.
As previously shared, I spend my every day life as a Nurse Care Manager and Health Educator. I manage my own disease and direct my educational goals with my, "professional" eye.
However...
As I lay awake to the high pitched squeeeeak of my IV infusion pump my thoughts slowly and gratefully cleared.
I began to evaluate the situation from the patient perspective...

- How scary it is that my health care providers know so little about my disease process...
- How my heart would race then flip/flop like a fish out of water...
- How terrified I felt earlier that day when I could not understand ordinary words, how to use my cell phone to find a number, or remember how a commode flushes...
- How I went without food/ disease essential carb-loading from 10am til 8pm... (because dietary staff do not understand the connection between AIP and carbohydrates)
- My Health care providers were very kind, and provided excellent care within their current knowledge base...
- I have no desire to offend or alienate my caregivers in my time of need... their collaboration is vital to my recovery!

Now the day after discharge I am at home, the house is quiet and I am reflecting on my recent experiences. I try to learn from every life experience. How can I improve things for the next time? My next time or any one's next time...
My intervention was to outline and post "AIP Nursing/ Healthcare Provider Essentials". I will offer a print copy to each of my doctors and to the nurses on the unit I stay on in the hospital. To improve my future treatment even further, I will carry a printed copy with me in my shoulder bag and keep a copy in my automobile with the rest of my "Emergency AIP Information".

My Goal:
By providing direct information in an easy to follow format health care professionals are empowered to gain comprehensive insight into understanding AIP assessment and treatment creating optimal patient outcomes.

AIP Nursing/ Healthcare Provider Essentials

Potential for Altered Functioning due to
HMB- Synthase(Porphobilinogen- deaminase) Deficiency

 Blood
 Risk for Hypercholesterolemia
 Risk for Adult onset IDDM
 Risk for Menstrual Cycle Luteal Phase Acute Porphyria Attack
 Risk for Primary Hepatocellular Carcinoma
 Liver
 Kidney function R/T AIP (porphyrin/precursor accumulation/excretion)


Potential for Altered Functioning due to
PBG & ALA accumulation and related Neuro-Toxic effects
(Axonal Myelin Sheath Changes- Polyaxonal Motor Neuropathy)

 Sensory (vision, hearing, touch, taste & smell) perception
 Neuropathies (central, autonomic and peripheral)
 Cognitive Ability (Confusion, Poor Word Recall, etc.)
 Psychiatric Stability (Hallucination, Anxiety, Depression, Emotionally Unstable, etc.)
 Paralysis
 Neuro-Cardio ( Irregular/Ineffective Heart Rhythms)
 Symptomatic Cardiovascular Autonomic Dysfunction/ Neuropathy
 HTN
 Respiratory Insufficiency/Paralysis
 Endocrine
 GI Insufficiency
 Vitamin and mineral nutrient metabolism/ storage
 Bowel (constipation/ diarrhea)
 Nausea and Vomiting
 Urinary System (altered voiding abilities, frequent UTI, etc)
 Exercise Intolerance
 Neuromuscular Ability
 Neuro-Immune
 Pain
 Insomnia
 Restlessness
 Generalized Irritability
 Nonspecific Sense of Discomfort
 Increased Sense of “Overwhelming”
 Oxidation and Oxidative Stress creating an Increased Risk of Disease

Assessments & Interventions
(Evaluate/Address Essential Life Functions FIRST followed by all others)

 Confirm Medical Diagnosis AIP
 Discontinue all AIP triggers (including unsafe medications)
 Start IV Glucose
 Provide IV Panhematin as directed
 Provide safe pain control
 Initial Diagnostic Tests, if patient has not been officially diagnosed with AIP (24hr urine for ALA & PBG, Blood, Stool, Genetic Confirmation)
 AIP Rapid Test
 CBC
 Nervous System Assessment/Testing
 Sensory Function Testing (vision, hearing, taste, touch, smell)
 Blood Work to determine vitamin and mineral levels
 Assess Cognitive Changes
 Liver Function Testing/ Imaging
 Evaluation: Cholesterol Level
 Peripheral Pain/ Tingling/ Numbness Evaluation
 Evaluation: Cognitive Changes/ Confusion
 Evaluation: Paralysis
 Assess for signs and symptoms of Cardiovascular Autonomic Dysfunction/ Neuropathy * Provide further Cardiac Intervention as appropriate
 24 hour Halter Monitor/ Cardiac Telemetry
 Evaluation: Rapid Heart Beat/ Tachycardia
 Evaluation: Irregular Heart Rhythms/ Palpitations
 Evaluation: High Blood Pressure
 Assess Orthostatic Hypotension
 Cardiac Evaluation/ Functioning
 Evaluation: “Heavy Chest Breathing”
 Evaluation: Difficulty Breathing/ Shallow Breathing
 Assess Respiratory Autonomic Dysfunction via Direct Observation (i.e. changes in depth)/Auscultation and Consistent Assessment of Incentive Spirometer (sustained maximal inspiration, SMI) Ability *Provide Mechanical Ventilation when appropriate
 Assess Pulse Ox Reading
 Pain Evaluation
 Evaluation: Nausea/Vomiting
 Evaluation: Neuro-Psych/ Mental Health Changes
 Evaluation: Irregular Voiding Patterns/ Pain With Urination/ Low Back Pain
 Evaluation: Dark Discolored Urine
 Urine Culture & Sensitivity
 Urinary Analysis
 Kidney Function Testing
 Patient Education S/S UTI, UTI Prevention Strategies, UTI Treatment Strategies (No Sulfa Drugs)
 Urinalysis Dip Sticks for home use
 Neuro-Muscular Testing/ Evaluation
 Muscle Weakness/ heavy feeling
 Evaluation: Numbness/ Tingling/ Paralysis
 Recommend: Quality Sleep/ Rest
 Recommend; Quality organic foods high in anti-oxidants
 Recommend: Sweat- to expel toxins and reduce liver workload
 Recommend: Full Spectrum Lights In Winter
 Recommend: Shaded Eye Wear R/T Light Sensitivity
 Recommend: Quality Stress Management
 Recommend: Daily Non-impact Exercise as Tolerated
 Monitor Frequency and duration of bacterial, viral and other illnesses to determine immune deficiencies
 Assess Insomnia
 Assess Restlessness
 Assess Generalized Irritability
 Assess Nonspecific Sense of Discomfort
 Evaluation: Pain Scale
 Sleep Study
 Assess Increased Emotional Instability
 Assess Increased Sense of Overwhelming
 Provide Calm/Reassuring AIP Knowledgeable Professional Intervention
 Provide/ Recommend Low sensory stimuli environment
 Teach patient to keep copies of AIP diagnosis medical confirmation letter in their automobile and in their shoulder bag at all times. (for emergency situations)
 Teach patient to keep copies of basic medically approved/authored AIP disease and treatment information in their automobile and in their shoulder bag at all times. (for emergency situations)
 Teach patient to wear a medical alert bracelet at all times to help speed proper emergency treatment when necessary.
 Teach patient to track menstrual cycle, note luteal phase can trigger acute episodes.
 Develop a specific “Neuro-toxic signs and symptoms check list” prior to physically assessing patient, as pt responses may seem vague and disconnected to the inexperienced Porphyria Healthcare Provider (Know what to look for).


Non-Porphogenic Medications & Supplements as determined by individual situation
(*ALWAYS check APF Drug Database to ensure safety!)

 Maintain Appropriate Carbohydrate Intake
 Magnesium
 B-12
 Fish Oil
 Vitamin and Mineral Supplements as determined by noted deficiencies
 Ativan
 Phenergan (promethazine)
 Naproxen
 Neurontin (gabapentin)
 IV Glucose (non-corn-based is better for some)
 IV Panhematin via Central Line
 Beta Blockers
 Avoid Chemical Birth Control (Use Physical Barriers)
 Lupron injections to Suppress Hormone Cycle and reduce frequency of Luteal Phase AIP attacks (or permanent surgical alternatives)
 Maintain Adequate Hydration
 Pain Medication/ IV Morphine
 Pure Cranberry Supplements (discouraging bacteria growth during neurogenic bladder episodes)


Helpful Lifestyle Recommendations:

Written Disease Management Plan, Designed via Physician and Patient collaboration
Full Spectrum Lights
Farinfared Sauna to sweat out toxins
Elliptical Exercise Machine for non-impact exercise
Yoga
Weighted Sensory Blanket
Shaded Eyewear
Natural Heating/Cooling Pad
Scent-free organic products
Water Analysis (Identify water quality)
Water Filter/ Purification System (Reverse Osmosis)
Air Filter/ Purification System (UV/Carbon/Hepa- Triple Combination in one machine)
Home environment Soil testing (Identify quality/ toxins)
Consider an organic, gluten-free, bovine-dairy-free diet full of fresh whole foods (non-processed)

Necessary Education:

Acute Intermittent Porphyria (AIP)
AIP Triggers
Multiple Chemical Sensitivity- Lifestyle Choices/ Environmental Set Up
Natural/Nonchemical Sensory Dysfunction Treatments
Neurologic System Function/Dysfunction
Self Propelled Effective Disease Management