Thursday, September 16, 2010

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
 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
 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
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

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