Pure Autonomic Failure/PAF 1: Self-Care Summary

Warrior, Jean Cacicedo, 2024. Paper, printed, stitched. 15″x15″x1/2″

DAILY MANAGEMENT (HABITS)

  • Rise slowly: pause sitting on the bed edge before standing; pause again before walking
  • Sodium: 6–10 g daily helps retain fluid and support blood pressure (follow your doctor’s target, cardiovascular disease is affected by salt)
  • Medications: Use a pill organizer vs taking meds from bottles so that you never lose track of what you’re taking. See medications below
  • Fluids: 2–2.5 liters of water daily; drinking 500 mL quickly before standing can raise BP acutely; many people take electrolytes with this first water of the day
  • Compression: waist-high compression stockings or abdominal binder are best, thigh-high next best, knee high better than nothing — put on before getting up if possible
  • Meals: small, frequent meals; limit simple carbohydrates to reduce post-meal BP drops
  • Heat: avoid hot showers, baths, and prolonged outdoor heat; use cool water; plan outdoor activity for cooler times of day
  • Sleep position: elevate head of bed 10–20 degrees to reduce overnight BP fluctuation, particularly supine hypertension. Note that elevation is for entire torso vs. just an extra pillow (see photo in complete patient guide (3).
  • Quick fixes: if you feel faint, cross your legs, tense your leg muscles, or squat — these temporarily raise BP
  • BP monitoring: Upper arm, validated device. Measure after 5 minutes supine, after 1-2 minutes sitting, 3 minutes after standing. Log everything (time, position, symptoms. Patterns are of utmost importance
  • Exercise: Regular exercise is important in the overall management of PAF. Exercise in the presence of PAF is a real challenge. Do what you are able. Anything is better than nothing. Water-walking in diaphragm to waist deep water is a great way to extend how long you are able to exert yourself
  • Safety: When feeling unsteady or likely to feel unsteady, use a walker or rollator. Make a personal commitment to sit down, even on a sidewalk when feeling faint. Don’t worry about appearances.

Note: PAF management requires balancing low standing BP against high lying-down BP. Medication changes should always involve your physician. See medication and blood pressure monitoring section in separate post.

🚨  RED FLAGS — Seek Emergency Care Immediately

●     Loss of consciousness or fainting with injury

●     Sudden severe headache (may signal hypertensive crisis)

●     Chest pain or pressure

●     Confusion, slurred speech, facial drooping, or one-sided weakness

●     Fall with possible head injury or fracture

●     Inability to urinate for more than 8 hours

For the ER: PAF patients may present with very low blood pressure on standing and very high blood pressure when supine. Both are features of the disease, not separate emergencies. Tilt the patient slowly. Avoid aggressive IV fluid boluses without monitoring. Contact the patient’s neurologist or autonomic specialist if possible. We made a laminated PAF and medication card to carry and show ER staff if necessary

This guide is for general educational purposes only and is not a substitute for professional medical advice. Always consult your physician regarding your specific condition, medications, or treatment. Medical information changes frequently; verify drug names, dosages, and current guidance independently. The authors disclaim liability for any injury or damage arising from use of this information.

Pure Autonomic Failure/PAF 2: Definitions, medications, BP measurement

Crazy Heart, Jean Cacicedo, 2024. Paper, printed, stitched, 15″x15″x1/2″

What Is Pure Autonomic Failure?

The autonomic nervous system (ANS) runs the body’s automatic functions — the ones you never have to think about: blood pressure, heart rate, breathing regulation, digestion, bladder control, sweating, and the adjustment of blood flow when you move from lying down to standing. In a healthy person, this system operates continuously in the background, making hundreds of fine adjustments every hour.

In Pure Autonomic Failure (PAF), this system breaks down. The nerve cells responsible for autonomic signaling degenerate, and the body loses the ability to regulate these functions automatically. PAF is caused by the abnormal accumulation of a protein called alpha-synuclein in autonomic nerve cells — the same protein implicated in Parkinson’s disease and a related condition called Multiple System Atrophy (MSA). Unlike those diseases, PAF affects the autonomic nervous system almost exclusively, sparing the parts of the brain that control movement and cognition.

PAF is rare — precise prevalence is unknown, but it is significantly less common than Parkinson’s disease. It typically appears in middle to older age and affects men and women roughly equally. The cause is not fully understood, and there is currently no treatment that slows or reverses the underlying degeneration. Management focuses entirely on controlling symptoms.

PAF and MSA: an important distinction

Patients and families are often told, or read online, that PAF can “convert” to MSA — a more serious and rapidly progressive disease. While this does occur in a subset of patients, current research suggests it is around 30%, less common than once believed. PAF tends to follow a slow course over many years. If you develop new neurological symptoms — changes in movement, balance, speech, or cognition — report them to your physician. These may warrant re-evaluation of your diagnosis.

What PAF affects

  • Blood pressure regulation: the most prominent problem; blood pressure falls when standing (orthostatic hypotension) and often rises dangerously when lying down (supine hypertension)
  • Heart rate: the normal increase in heart rate that compensates for a BP drop is absent or blunted in PAF
  • Temperature regulation: sweating is reduced or absent (anhidrosis), impairing the body’s ability to cool itself; heat intolerance can be severe
  • Bladder and bowel: urinary urgency, incomplete emptying, constipation, and less commonly fecal incontinence
  • Sexual function: erectile dysfunction in men; reduced sensation in women
  • Pupillary response: slower adjustment to changes in light

Prognosis

PAF is a chronic, slowly progressive condition. Most patients live with it for many years and, with good symptom management, maintain meaningful quality of life. The disease does not directly cause death, but its complications — falls, injuries from fainting, cardiovascular stress from BP swings — require active management. A small percentage of PAF patients do eventually develop features of MSA or Parkinson’s disease; regular follow-up with a neurologist or autonomic specialist is important.

Where to get help

  • Dysautonomia International: dysautonomiainternational.org — patient advocacy, physician directory, research updates
  • Facebook: there is a PAF group with a collective wealth of information. This group also serves as a portal to biweekly Zoom meetings with Dr. Goldstein usually present.
  • Autonomic specialty centers: academic medical centers with dedicated autonomic programs (Stanford, Mayo Clinic, Vanderbilt, NYU, and others) offer the most experienced evaluation and management
  • Your care team: a neurologist with autonomic expertise should lead your care; your primary care physician plays a key role in day-to-day management and coordination
  • See references for additional resources

Medications commonly used in PAF

Medications for PAF almost always require adjustment, or “titrating to effect,” when starting a new one. The central issue is what effect a medication has on BP and how you feel through the day. PAF management requires balancing low standing BP against high lying-down BP. Medication changes should always involve your physician.

Medication Purpose / Key notes
Droxidopa (Northera) Raises standing BP; first-line for OH in PAF
Midodrine Vasopressor; waking hours only — do not take within 4 hrs of lying down
Fludrocortisone Helps body retain sodium and fluid; raises blood volume
Pyridostigmine Mild BP support with less effect on supine hypertension
Melatonin / clonidine Sometimes used at night to reduce supine hypertension — discuss with physician

BP monitoring at home

  • BP cuff: upper arm, validated device
  • Positions: measure after 5 min supine, after 1–2 min sitting, immediately on standing, and again at 3 min standing
  • Log everything: time of day, position, symptoms, meals, activity — patterns are what matter
  • Pre/post meal: check BP before and 30–45 min after eating to identify postprandial drops

This guide is for general educational purposes only and is not a substitute for professional medical advice. Always consult your physician regarding your specific condition, medications, or treatment. Medical information changes frequently; verify drug names, dosages, and current guidance independently. The authors disclaim liability for any injury or damage arising from use of this information.

Pure Autonomic Failure/PAF 3: A Complete Patient Guide to Management

Dysautonomia Offering. Jean Cacicedo, 2024. Paper, cloth, stitched. 15″x15″x1/2″

PAF is rare, and many clinicians — including in emergency settings — have limited experience with it. This guide is designed to help you understand each aspect of your condition, monitor it at home, and take informed action. Bring it to appointments. The section on each problem includes what to tell clinicians who are unfamiliar with PAF.

1. Orthostatic Hypotension (OH)

Orthostatic hypotension — a drop in blood pressure upon standing — is the cardinal sign of PAF. It is present in virtually every patient and is usually the most disabling problem.

The problem

When you stand, gravity pulls blood into the legs and abdomen. A healthy autonomic system instantly tightens blood vessels and slightly raises heart rate to maintain pressure to the brain. In PAF, this reflex is absent or severely impaired. Blood pressure falls, sometimes dramatically, within seconds to minutes of standing. In some cases there is “impaired Valsalva maneuver,” i.e., a rapid and profound drop in BP after straining at stool or having a bowel movement.

What it feels like

  • Lightheadedness, dizziness, or a sensation of “going gray” when standing
  • Generalized weakness or heaviness in the legs
  • Feeling vaguely unwell, foggy, or drained — especially after meals or in heat
  • Blurred or tunneled vision
  • Neck or shoulder ache (the “coat hanger” pattern) from poor perfusion to neck and shoulder muscles
  • Symptoms that improve promptly when lying down

What can go wrong

  • Fainting (syncope) and falls — with risk of injury
  • Reduced blood flow to the brain over time, with potential for cognitive effects
  • Supine hypertension: the same regulatory failure that causes low standing BP often causes dangerously high BP when lying down — treatment, including self-care, must balance both
  • Labile BP: wide swings between high and low stress blood vessels and organs
  • Post-meal (postprandial) hypotension: BP drops further after eating as blood shifts to the digestive tract
  • Exercise intolerance: it is not just the heart and brain that are affected by OH. Muscles also need blood to function optimally.

Testing

Medical:

  • Orthostatic vitals: BP and heart rate measured in three positions
    • Protocol A (autonomic lab standard): 5 min supine → 1 min standing → 3 min standing
    • Protocol B (some clinical settings): supine → seated 1–2 min → standing 1 min → standing 3 min
    • A drop of ≥20 mmHg systolic or ≥10 mmHg diastolic confirms OH
    • In PAF, heart rate does NOT rise significantly when BP drops — this distinguishes PAF from most other causes of OH and is an important clinical clue
  • Tilt-table test: clinic-based; controlled reproduction of positional BP changes
  • 24-hour ambulatory BP monitor: captures full day/night BP profile including supine hypertension

Home monitoring:

  • Upper-arm validated BP cuff (not wrist)
  • Measure after 5 min supine; after 1–2 min sitting; immediately on standing; at 3 min standing
  • Keep a log: time, position, symptoms, meals, recent activity
  • Morning pre-rising readings often capture the highest supine BP

Bring your log to appointments. Clinicians unfamiliar with PAF may not order orthostatic vitals routinely. A home log over days or weeks gives a far richer picture than a single office reading.

What you can do

Daily habits:

  • Rise slowly: sit on the bed edge before standing; pause again before walking
  • Avoid constipation and straining to have a bowel movement; allow for recovery after bowel movement
  • Sodium: 6–10 g daily (your physician may set a different target)
  • Fluids: 2–2.5 liters daily; 500 mL drunk rapidly before standing or arising from bed gives an acute BP boost
  • Compression: waist-high stockings or abdominal binder — on before getting up
  • Meals: small and frequent; limit simple carbohydrates
  • Heat: avoid hot showers, baths, outdoor heat — all worsen OH
  • Bed elevation: raise head of bed 10–20 degrees to reduce overnight fluid loss and moderate supine hypertension. Note that elevation is for entire torso vs. just an extra pillow

    One means of elevating torso: 4×4 supporting piece of 3/4″ plywood at an angle. There are also mechanical head-elevators available from mattress retailers

  • Physical countermeasures: leg crossing, tensing leg muscles, or squatting can temporarily raise BP when symptoms start
  • When feeling unsteady or likely to feel unsteady, use a walker or rollator. Make a personal commitment to immediately sit down, even on a sidewalk, when feeling faint. Don’t worry about appearances.

Medications:

  • Droxidopa (Northera): norepinephrine precursor; raises standing BP; first-line for PAF
  • Midodrine: vasopressor; waking hours only — do not take within 4 hours of lying down
  • Fludrocortisone: promotes sodium and fluid retention, raising blood volume
  • Pyridostigmine: modest BP support with relatively less effect on supine hypertension

Note: medication changes should always involve your physician. The OH–supine hypertension balance is delicate.

Important note: maintaining optimal physical condition is helpful in maintaining maximum physical and mental well-being. This is true for anyone, and especially for people with PAF. A significant challenge in regular exercise is that decreased blood pressure means decreased blood perfusion into muscle tissue, i.e., the muscle machine of the body is running on less fuel. Water walking (walking in midriff/diaphragm or waist deep water) is a great way to exercise. I can walk for 45 minutes nonstop in water, but only for 10–15 minutes out of the water before falling BP exhausts me. Why does water walking work? There is just enough pressure from the water to prevent pooling of blood.

2. Supine Hypertension

Supine hypertension — high blood pressure when lying flat — occurs in the majority of PAF patients and is the flip side of orthostatic hypotension. The same failure to regulate vascular tone that causes BP to fall when standing allows it to rise unchecked when the body is horizontal.

The problem

When lying flat, blood no longer has to fight gravity to reach the brain. Without autonomic regulation to keep vessels appropriately relaxed, pressure builds. BP while supine can reach 160–200+ mmHg systolic in PAF patients — even patients whose standing BP is dangerously low.

What it feels like

  • Often no obvious symptoms — supine hypertension can be silent
  • Headache, especially at the back of the head, when lying down
  • Nausea or a sense of pressure or fullness
  • Nocturia (waking to urinate frequently) — the kidneys excrete excess fluid in response to high overnight BP
  • Worsened morning OH: overnight pressure natriuresis (fluid loss through urine) reduces blood volume, making the standing BP drop worse the next morning

What can go wrong

  • End-organ damage over time: heart, kidneys, and blood vessels are stressed by chronically elevated overnight BP
  • Hypertensive crisis: very high BP (typically >180/120) can cause stroke, heart attack, or acute kidney injury
  • The treatment paradox: medications that raise standing BP can worsen supine hypertension; this is the central management challenge in PAF

Testing

  • 24-hour ambulatory BP monitoring is the most informative test — captures the full night/day profile
  • Home BP check before bed and immediately upon waking (before rising) reveals the overnight pattern
  • Keep a log alongside OH readings — the relationship between morning supine BP, overnight nocturia, and morning OH severity is diagnostically useful

What you can do

Daily habits:

  • Elevate the head of the bed 10–20 degrees — reduces venous return to the heart and moderates supine BP
  • Avoid lying flat during the day; use a recliner or wedge pillow for rest
  • Time medications carefully: midodrine and fludrocortisone should not be taken close to bedtime
  • Limit fluid intake in the evening

Medications:

  • Short-acting antihypertensives at bedtime are sometimes used (e.g., losartan, melatonin in higher doses, nitroglycerin patch) — but only under physician supervision; these can cause dangerous morning OH if they act too long
  • The goal is to reduce nighttime BP without depleting blood volume or causing excessive morning hypotension

3. Temperature Dysregulation and Anhidrosis

PAF impairs the body’s ability to regulate temperature. The primary mechanism is anhidrosis — reduced or absent sweating. Sweating is the body’s main cooling system, and without it, body temperature can rise rapidly in warm conditions.

What it feels like

  • Little or no sweating even during exercise or in heat
  • Feeling overheated or flushed in warm environments
  • Heat intolerance: fatigue, weakness, and worsened OH symptoms in warm weather
  • In some patients, excessive or abnormal sweating in isolated areas (compensatory hyperhidrosis)

What can go wrong

  • Heat exhaustion or heat stroke — both are genuine medical emergencies for PAF patients
  • Heat dramatically worsens orthostatic hypotension — hot environments are doubly dangerous
  • Exercise in heat is high-risk; even a warm shower can trigger a significant BP drop

Testing

  • Quantitative Sudomotor Axon Reflex Test (QSART): a specialist test measuring sweat gland function at multiple sites — available at autonomic centers
  • Thermoregulatory sweat test (TST): maps the pattern of anhidrosis across the body
  • Home observation: note sweating (or its absence) during exercise, warm environments, or mild exertion

What you can do

  • Avoid heat: hot showers, saunas, outdoor exertion in warm weather, heated car interiors
  • Cool proactively: cool water, cooling vests, air conditioning, damp towels on the neck and wrists
  • Exercise with caution: water-based exercise (swimming, water aerobics) is often better tolerated because the water assists cooling and exerts pressure on limbs
  • Time outdoor activity: early morning or evening when temperatures are lower
  • Alert others: people around you should know you cannot cool yourself normally and recognize signs of overheating
  • Emergency: if body temperature rises above 104°F / 40°C, this is a medical emergency — call 911

4. Bladder and Bowel Dysfunction

The autonomic nervous system controls bladder and bowel function. In PAF, both can be significantly affected, though severity varies widely among patients.

Bladder — what it feels like

  • Urgency: sudden, intense need to urinate with little warning
  • Frequency: urinating more often than normal, including at night (nocturia)
  • Incomplete emptying: the bladder does not fully empty, increasing infection risk
  • Urinary retention (less common): inability to urinate, requiring catheterization
  • Urinary incontinence in some patients

Bowel — what it feels like

  • Constipation is the most common bowel symptom in PAF — slowed gut motility
  • Bloating, early satiety, and nausea (gastroparesis — delayed stomach emptying)
  • Less commonly: fecal urgency or incontinence

What can go wrong

  • Urinary tract infections from incomplete bladder emptying — these can be serious and recurrent
  • Severe constipation leading to fecal impaction
  • Straining to have a bowel movement can cause syncope (passing out)
  • Gastroparesis can complicate nutrition and medication absorption
  • Nocturia from both supine hypertension (pressure natriuresis) and bladder dysfunction disrupts sleep significantly

Testing

  • Post-void residual (PVR) ultrasound: measures how much urine remains after voiding — simple, non-invasive
  • Urodynamic testing: specialist evaluation of bladder muscle and nerve function
  • Gastric emptying study: nuclear medicine test for gastroparesis
  • Home: keep a bladder diary (timing, volumes, urgency episodes)

What you can do

Bladder:

  • Take your time urinating, let it all out
  • Timed voiding: urinate on a schedule (e.g., every 2–3 hours) rather than waiting for urgency
  • Pelvic floor physical therapy like Kegels can improve urgency and incomplete emptying
  • Medications: anticholinergics (oxybutynin, tolterodine) reduce urgency; discuss with your physician as these can worsen cognition in older adults
  • If retention is significant: intermittent self-catheterization may be necessary
  • Stay well hydrated — counterintuitively, restricting fluids can worsen bladder irritability

Bowel:

  • High-fiber diet and adequate hydration are the first interventions for constipation
  • Do not stand up immediately after a bowel movement, to avoid a sudden BP drop
  • Regular scheduled toilet time (e.g., after breakfast) uses the body’s natural reflexes
  • Osmotic laxatives (polyethylene glycol / Miralax) are well tolerated for chronic constipation
  • For gastroparesis: smaller, more frequent meals; avoid high-fat foods; your physician may consider prokinetic medications

When to Seek Emergency Care

🚨 Call 911 or go to the ER immediately for:

  • Loss of consciousness or fainting with injury
  • Sudden severe headache — may indicate hypertensive crisis from supine hypertension
  • Chest pain or pressure
  • Signs of stroke: facial drooping, arm weakness, slurred speech, sudden confusion
  • Fall with possible head injury or fracture
  • Inability to urinate for more than 8 hours
  • Body temperature above 104°F / 40°C

For Emergency Clinicians: PAF patients present with very low blood pressure when upright and very high blood pressure when lying flat. Both are features of the disease. Tilt position changes slowly. Avoid aggressive IV fluid boluses without monitoring BP in multiple positions. Hypotension that responds to lying flat and recurs on standing is likely orthostatic, not hypovolemic. Consult the patient’s neurologist or an autonomic specialist when possible.

Resources and References

Researched and drafted by Charles Kemp and Jean Cacicedo with the assistance of Claude (Anthropic AI). This guide is for general educational purposes only and is not a substitute for professional medical advice. Always consult your physician regarding your specific condition, medications, or treatment. Medical information changes frequently; verify drug names, dosages, and current guidance independently. The authors disclaim liability for any injury or damage arising from use of this information.

Pure Autonomic Failure/PAF 4: Notes on the Lived Experience

Holding My Heart. Jean Cacicedo. Wool, dyed, stitched. 2026, 34”x38″

“It’s hard. The main thing,” Jean says, “is unpredictability.”

“The main thing is unpredictability — if and when you’re going to feel bad. Obviously after every meal, but there are other times as well. The lethargy. The physicality of it — kind of numb throughout the body. Exhausted. Balance issues when walking — I feel like I’m walking drunk, that’s why I have to sit a lot. I feel like I’m on display. Disconnected. Don’t feel right. Don’t feel sick. Not getting blood to your body — it feels like the walls closing in. It happens a lot. Feels like you’re going to faint even after getting ready for bed — but not every night.”

That description — precise, physical, disorienting — is what PAF actually feels like from the inside. No test captures it. No chart note records it.

The complexities of daily management (medications, fluid, sodium, activity, etc.) coupled with whatever other health problems one has can be overwhelming. For example, the effects of PAF on blood pressure and heart rate have, I believe, led to persistent atrial fibrillation (AF) and congestive heart failure (CHF).

Technical medical discussion paragraph: AF and CHF coupled with the exercise intolerance inherent in PAF create challenges in daily life and the work to improve physical conditioning. When CHF is paired with PAF there is a therapeutic conflict between the high sodium demands of PAF and the low sodium demands of CHF. There is also therapeutic conflict between the high fluid volumes needed in PAF and the lower fluid volumes needed in CHF. If this seems technical and complex, it is. The neurologist and cardiologist seem to find it so as well. In our “siloed” health care system the neurologist wants the cardiologist to deal with heart issues and the cardiologist wants the neurologist to deal with autonomic issues. However, these are inextricably bound together in the understanding pathophysiology of PAF. Teaching is minimal. The bottom line: I figure it all out.

The heat intolerance of PAF means that one literally cannot function in a hot climate or on a hot day in a cool climate.

Severe dry eyes require constant management.

The chronic constipation of PAF is complicated by occasional episodes of sudden, severe diarrhea.

When flying with PAF, respiratory distress may be experienced while the plane is ascending and descending. Thus flying is a fearful event. I just purchased an oxygen concentrator to use while flying and have exercises that may help. We will see.

As with other chronic illnesses, PAF affects the entire family; it is truly a family illness.

Invisible and unexplained

PAF is classified by the NIH as a rare or orphan disease. That rarity has consequences that go beyond statistics. Most physicians have little familiarity with it — and some seem to reason that if they haven’t heard of it, it probably isn’t serious. Emergency personnel are largely unaware. Some know something about the more common dysautonomia POTS (postural orthostatic tachycardia syndrome), but PAF is less common and less recognized. Friends and family don’t understand what’s wrong, and explaining it soon runs into blank looks.

The institutional consequences can be surreal. My HMO refuses to include PAF in my chart’s problem list, on the grounds that PAF does not appear in the ICD-10, the World Health Organization’s International Classification of Diseases. No code, no diagnosis. Never mind that the condition exists and requires active management. The problem list is precisely what emergency personnel read first when a patient comes in unable to speak for themselves.

I live with moral injury related to the injustice of medical dependence on a system and individual doctors that will not give me the diagnosis that I live with and the collaborative health care I need.

The work of it

Managing a complex, dynamic chronic illness is genuinely demanding. Symptoms shift hour to hour, day to day, week to week. Staying ahead of them requires attention, discipline, and knowledge that most patients have to acquire largely on their own.

I’ve been fortunate to see an out-of-network autonomic specialist once a year. The Facebook PAF group provides information and community, and coordinates a biweekly Zoom support meeting — often with a prominent autonomic specialist in attendance. These are the resources that actually help. My HMO provides neither education nor support for this condition.

The social calculus

PAF quietly reshapes ordinary life. The postprandial BP drop means that walking to the car after lunch out is an effort requiring calculation. Sitting around the table after dinner becomes difficult. A museum is best taken in small increments, with a rollator — a wheeled walker — as an essential tool. Socializing requires planning that healthy people never think about.

And then there is the gap between appearance and reality. People assume that if you look fine, you are fine. You may look great. You may not feel great. That gap — between the face you present and the body you’re managing — is one of the quiet costs of living with PAF.

Finally

Having PAF is hard, stressful. I’ve experienced almost everything we’ve written about in this guide, except for phenoconversion and some of the testing. I am grateful to my autonomic specialist consultant, to friends and family who have stayed with me, to doctors who have tried to understand and help, and to the PAF Facebook group.

We, Charles and Jean, are grateful for one another.

This guide is for general educational purposes only and is not a substitute for professional medical advice. Always consult your physician regarding your specific condition, medications, or treatment. Medical information changes frequently; verify drug names, dosages, and current guidance independently. The authors disclaim liability for any injury or damage arising from use of this information.

Chronic pain

I’ve Looked at Pain from Both Sides Now…

Before implementing any part of these pain management strategies you must first consult with a healthcare professional.

The purpose of this post is to give people practical tools for managing chronic pain. 

I’ve looked at pain from both sides now, from other people’s pain in hospice care and primary care to my own chronic pain experiences. Here I want to discuss how to manage chronic pain and provide background on why pain is so problematic for so many people. Discussion includes effective medical management, random tips, terms and definitions, difficulties (real and imagined) in pain management, and my background for writing. Topics are ordered from my sense of which are likely most relevant to people in my position – a pain patient. While some information and principles here apply to end-of-life care, this is written for adults with chronic pain not related to the end of life.

Reading this requires effort. Pain is a complex problem encompassing all realms of being – physical, psychological, social, environmental, and even spiritual. I tried, but often failed to keep it uncomplicated.

Remember, for chronic pain there is seldom a single answer or “magic bullet.” Pain is complex, the answers are complex, and and complete relief is very difficult to achieve.

Managing chronic pain (pain that lasts more than three months) Overall references:

  1. https://www.cdc.gov/opioids/healthcare-professionals/prescribing/guideline/recommendations-principles.html#follow-up,
  2. https://www.cdc.gov/mmwr/volumes/71/rr/rr7103a1.htm

The CDC notes that, “chronic pain can lead to impaired physical functioning, poor mental health, reduced quality of life, and contributes to substantial disability and death each year” (Reference 1 above). In the following discussion, I will cite some risks in taking pain medications. These should be understood in relation to the preceding risks of pain. In other words, there are significant risks in pain per se and in the effective treatment of pain. Some institutions and individuals have misinterpreted earlier CDC guidelines, in particular the 2016 Opioid Prescribing Guidelines so that medications are under prescribed and human suffering is increased. The 2022 guidelines seek to clarify and correct “misapplications” of the 2016 guidelines (1, 2).

In all cases, an accurate diagnosis of the cause(s) of pain is essential. Even when the cause of pain has been diagnosed, rapid worsening or onset of pain should always be evaluated by a competent medical professional.

Medications for pain

If pain can be managed with acetaminophen (Tylenol) or NSAIDS (non-steroidal anti-inflammatory drugs such as ibuprofen or naproxen), great, you’re fortunate and there is no need to read further. NSAIDS are generally more effective than acetaminophen but must be taken with food as they are irritating to the GI system. No problem – just have a few bites of non-spicy food before taking an NSAID. Note that NSAIDS should not be taken with “blood thinners.”

For moderate to severe acute or chronic pain not controlled with NSAIDS or acetaminophen, opioid medications such as oxycodone, hydrocodone, or morphine are commonly used either in immediate release (IR) formulations such as Percocet or Norco; or extended release (ER) such as OxyContin or MS Contin. Regardless of the opioid formulation, IR or ER, other medications such as acetaminophen or NSAIDS are often taken along with the opioid.

Common side effects (SEs) of opioids include respiratory depression sometimes leading to death, tolerance, dependence, nausea and vomiting, constipation, sleepiness, dizziness, depression, decreased testosterone, itching. Respiratory depression is the most significant SE and is increased in “opioid-naïve” patients with high dose opioids or concomitant use of opioids + alcohol, tranquilizers, or gabapentin. Note that even regular users may experience serious side effects from (especially) high dose opioids or mixing medications as described above. Some SEs decrease with time or adjustment of dose. Nausea is a very common early side effect and should be treated with meds such as ondansetron – usually for about 3 days. Constipation is inevitable and is treated as described below under random tips. Also see below for discussion of tolerance and dependence. Tolerance is basically inevitable. Knowledgeable clinicians will address tolerance with increased dosing or closer intervals of administration – up to a point. That point is usually if dependence develops, and even then, opioid therapy may be warranted.

(Many people are under the impression they are allergic to codeine or other opioids because they were nauseated when they first took it. Nausea is a common side effect, seldom an allergic reaction.)

In the case of neuropathic (nerve) pain, the first drugs of choice are non-opioid medications such as anti-seizure meds (e.g., gabapentin, carbamazepine) or certain antidepressants (e.g., duloxetine, Elavil). These may be combined with opioid medications and/or NSAIDS such as ibuprofen or naproxen. Combining gabapentin with opioids increases the risk of overdose, but concomitant opioid therapy may be indicated. Concomitant NSAIDS or acetaminophen are often helpful. Success in reducing pain with (for example) gabapentin alone in doses of 1200-3600 mg/24 hours ranges from 30-40%, with >50% of patients experiencing adverse effects. Common side effects include somnolence, dizziness and difficulty walking. References:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452908/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6464253/,

https://www.nytimes.com/2019/05/20/well/live/millions-take-gabapentin-for-pain-but-theres-scant-evidence-it-works.html

How to manage pain

Take enough of the right medicine at dosing intervals according to the medication(s) duration of action or effect. For example, oxycodone in immediate release form such as Percocet has an effective duration of action of at best 4-6 hours, while oxycodone in the sustained release form such as Oxycontin has an effective duration of action of around 8-12 hours.

When pain is an ongoing problem the ideal situation is to take an effective dose at intervals that maintain a relatively stable state of pain relief without significant impairment. It is generally better to take medications on a schedule to prevent pain rather than waiting until the pain increases. If you spend enough time with your pain, you may discover it’s worse in the morning or late afternoon, so dosing may need to be adjusted accordingly. One person may get by with just a morning dose, while another may take an opioid only in the afternoon and evening.

Use combinations of medications such as oxycodone and ibuprofen. Oxycodone and hydrocodone are often given in pills such as Percocet or Norco containing the opioid and low-dose acetaminophen (Tylenol). Norco 10/325, for example contains 10 mg of hydrocodone and 325 mg of acetaminophen, which is equal to one tablet of regular strength acetaminophen, so this acetaminophen component is clinically negligible for pain relief. To get any pain relief from the acetaminophen component It is thus necessary to take extra acetaminophen with the total acetaminophen dose not exceeding 1000 mg 4 x day. Most first time users will definitely feel the opioid effects of hydrocodone 10 mg. In fact, the CDC recommends sometimes starting with a low dose of 2.5 mg, i.e., half a 5 mg tablet.

Note that opioids and NSAIDS act in different ways on pain. Opioids act on the central nervous system, while NSAIDS inhibit an enzyme that plays a part in pain and inflammation. The daily limit for ibuprofen is 1200 mg. Acetaminophen blocks pain receptors and the daily limit is 4000 mg. https://www.ncbi.nlm.nih.gov/books/NBK547742/#:~:text=The%20main%20mechanism%20of%20action,the%20lack%20of%20these%20eicosanoids.

Opioid dosing should be based on severity of pain, patient conditions, patient responses to treatment, and CDC or other reputable source guidelines. Dosing should be understood in terms of morphine milligram equivalents (MME). The link below discusses means of conversion. Examples of the MME of common medications/dosing:

Hydrocodone 5 mg 4 times day/24 hours MME = 20 mg morphine/24 hours by mouth or about 7 mg morphine intravenously (IV) or intramuscularly (IM)/24 hours.

Oxycodone 5 mg 4 times day/24 hours MME = 30 mg morphine/24 hours by mouth or about 10 mg morphine IV or IM/24 hours. https://www.cdc.gov/drugoverdose/training/dosing/accessible/index.html

Note that dosing of morphine or other opioids taken by mouth is different than when taken IV or IM, e.g., 10 mg morphine IV is about the same as 30 mg morphine or 20 mg oxycodone by mouth (except IV has a much quicker onset of action). https://www.capc.org/documents/download/324/

The CDC notes that there are increased risks of opioid-related harms in doses of any opioid greater than 50 MME/24 hours, especially in opioid-naïve patients and/or with concomitant use of tranquilizers or alcohol. The CDC states that clinicians should avoid “increasing dosage to more than 90 MME/day or carefully justify a decision to titrate dosage to more than 90 MME/day.” Note that when pain is severe, this caution does not preclude taking more than 90 mg MME; rather it requires “carefully justifying a decision” to go past 90 MME. https://www.cdc.gov/drugoverdose/training/dosing/accessible/index.html

In summary, for moderate to severe chronic pain not managed by non-opioid measures take enough opioid medicine at frequent enough intervals to keep the pain at bay. Combinations of opioid and NSAIDS are usually required. Other meds may also be required for neuropathic pain. In most cases, alternative methods should also (concurrently) be employed.

Random tips

The risk of falling is increased with opioid medications.

Mixing opioids with alcohol, tranquilizers, or less commonly, gabapentin increases the risks of impairment and dangerous side effects such as falling or respiratory depression.

Understand what addiction is and is not. See terms and definitions below.

The ideal is pain-free and alert – which is very difficult to achieve if you’re living a relatively normal life. But you should not be suffering and the pain should at a minimum be bearable.

Numeric pain ratings are subjective (“On a scale of 1-10 with 10 being the worst, how would you rate your pain.”), but they are what we have.

Qualify your rating, e.g., “Most of the time when I’m walking, working, or just living my life, it’s a 7. If I’m completely immobile it’s a 5.” Don’t say your pain rating is an 11 – unless you’ve been burned, have advanced cancer, are trying to pass a large kidney stone, and so on. Don’t exaggerate. Tell providers you’re not going to embellish or exaggerate anything.

Constipation is basically inevitable with opioid use. With frequent opioid use staying well-hydrated and fiber intake helps, but a stool softener and stimulant laxative are usually also needed.

Alternative methods include the below. These are not substitutes for effective medications, rather each one can be part of a comprehensive pain management regime. “All these things help some people some.” (https://www.nature.com/immersive/d41586-023-00869-6/index.html)

  • Meditation – Jon Kabat-Zinn is one well-regarded source of information on pain meditation.
  • Cognitive behavioral therapy (CBT) – a psychological, goal-directed approach in which patients learn how to modify physical, behavioral, and emotional triggers of pain and stress, i.e., strategies for how to deal with it.
  • Certain medications for depression or for seizures, some of which can also treat pain. Note that pain often leads to depression and anxiety, hence treating these problems may help independently of the pain relieving qualities of, for example, duloxetine.
  • Pain patches such as Tiger Balm.
  • Interventional therapies, like steroid injection or nerve blocks.
  • Exercise and weight loss. Water walking is a good option as body weight is less of a factor. PT is sometimes used.
  • Hot or cold water bottle.
  • Other therapies such as acupuncture and massage. Chiropractic interventions are helpful for some, but others report increased pain or injury from “adjustments.”
  • Distraction or being engaged with things other than the pain.
  • Maintaining social connections.
  • Lifestyle adaptation responses such as simplifying meal prep, decreasing activity at times when the pain is usually worse, limiting activities that may worsen the pain, planning distracting activities around times when the pain is worse, giving medications time to work (plan inactive time between dosing and going forward with the day), doing activities likely to increase the pain at times when pain tends to be less.
  • Accepting the presence of pain and concomitant life changes in a positive manner.

Personally I utilize more than half of the above measures, while trying to not have the pain or pain relief measures dominate my life.

What about marijuana, including CBD and THC? Some people find these helpful in reducing pain per se or in changing the subject from the pain to being stoned. I am skeptical of some of the claims of the more messianic advocates.

Terms and definitions (https://www.cdc.gov/opioids/basics/terms.html)

Chronic pain: Pain that lasts more than 3 months.

Neuropathic pain: “Nerve pain” or pain from insult to the nervous system. Neuropathic pain is burning, tingling, stabbing, or shock-like. Pain classified as neuropathic includes evoked pain, which is pain from stimuli that ordinarily would not cause pain, such as touching or brushing against skin. Diabetic neuropathy, post-herpatic neuralgia, alcoholism, and HIV are some common causes of neuropathic pain. The other major class of pain is somatic pain or pain from the body other than nerves (see following).

Somatic pain is “characterized as well localized, intermittent, or constant and described as aching, gnawing, throbbing, or cramping” and arises from the skin or musculoskeletal system. (https://www.ncbi.nlm.nih.gov/books/NBK12991/)

Visceral pain is “poorly defined and diffuse and commonly described as deep, gnawing, twisting, aching, colicky, or dull.1 It is usually associated with autonomic features (e.g., sweating, nausea and vomiting) and highly emotional (e.g., anxious, feeling of impending doom)” (From the American Academy of Physical Medicine and Rehabilitation: https://now.aapmr.org/differential-diagnosis-and-treatment-of-visceral-pain-in-the-pelvis-and-abdomen/

Opioid: Substances that “interact with opioid receptors on nerve cells in the body and brain, and reduce the intensity of pain signals and feelings of pain” (CDC, 2). Examples include morphine, Dilaudid, oxycodone, hydrocodone, codeine, and of course, the mother of all opioids, opium. Fentanyl is a synthetic opioid and Tramadol is similar to opioids.

Tolerance: Decreased therapeutic response to opioids. Tolerance is an expected phenomenon that builds over time. To counteract tolerance opioid dosing is increased most commonly in amount, but also in frequency of dosing.

Dependence: when the body adjusts its normal functioning around regular opioid use. Unpleasant physical symptoms occur when medication is stopped suddenly.

Addiction: Addiction occurs when attempts to cut down or control use are unsuccessful or when use results in social problems and a failure to fulfill obligations at work, school, and home. Opioid addiction often comes after the person has developed opioid tolerance and dependence, making it physically challenging to stop opioid use and increasing the risk of withdrawal.

Nausea and vomiting: As noted earlier, nausea and vomiting from opioid use is common and occurs most often in the early days of therapy and is readily treated with anti-nausea drugs, especially ondansetron (Rx only).

Constipation: “Chronic constipation is infrequent bowel movements (fewer than 3/week) or difficult passage of stools that persists for several weeks or longer.” https://www.mayoclinic.org/diseases-conditions/constipation/symptoms-causes/syc-20354253. Treatment should be focused on prevention and includes a high fiber diet and plenty of water. Fiber and water are usually not enough and a combination of stool softener and stimulant laxative may be needed.

Why is it so difficult to treat pain effectively?

Chronic pain is a complex physical problem that is incompletely understood and the patient experience is subjective, i.e., there are no labs or images that necessarily confirm that pain is present or absent. Also, the mainstay of effective treatment for moderate to severe pain is opioid medications and opioids have several drawbacks, including significant side effects, cultural influences, and legal issues. Moreover most physicians are not well-trained in pain management and the aforementioned CDC 2016 guidelines have been widely misinterpreted (see CDC 2022 guidelines for further discussion of this).

A personal note: I was talking last year with a physician who has been involved in almost 200 medically assisted deaths. He told me that none of those patients were in hospice care and seeking release because of pain. In other words, hospice is able to manage pain effectively – and so can you, at least tolerably.

Background related to writing this

I was the founding Director of the VNA Home Hospice, the first hospice to provide care in Texas. I have studied pain and have written a book (published by Lippincott) on end-of-life care and have written related articles and chapters in journals and texts. I have cared for a number of patients with pain. I have had chronic pain for several years.

End Notes

Existential philosophers and therapists emphasize the importance of confronting the basic conditions of existence, which include suffering. By deeply reflecting on pain, one can grapple with profound existential questions and perhaps arrive at a deeper understanding of life’s meaning.

Consistent communication with healthcare providers is vital when adapting one’s life to manage chronic pain. Some, but not all can provide guidance tailored to individual needs and conditions. It’s also important to remember that what works for one person might not work for another; finding the right combination of treatments and adaptations often requires time and experimentation.

Aging and the end of life

Van nap time in a church parking lot in Berkeley Hills; SF Bay/Oakland in far distance; simple pleasures

Recently, a friend of a friend decided to voluntarily stop eating and drinking (VSED). It was not a matter of advanced disease, but rather a long life, ever-increasing disabilities, and ever-decreasing capabilities. Overall, it was an apparently positive experience except for a brief period at the very end, during which his wife reported that he seemed agitated (but not necessarily distressed – she was distressed, though). A detailed and positive account of a death from VSED is found at ~19:00 in the below YouTube video from Ashby Village.

Ashby Village – Charting your end-of-life journey (includes a comprehensive update on current medical, legal, and other issues)

Concurrently Jean and I have been working on issues related to aging and enjoying life, including staying in our home as long as possible and what to do when things go wrong, as they inevitably will. AARP and other sources have resources/ideas for structuring such planning. The exploration and documentation of issues and resources is a lengthy and detailed process!

Those we leave behind will appreciate the documented work done. Excerpts from what we’re working on:

“The money conversation”

Point Reyes. Taken from van; nap time; living the good life

Access to information
General – log-ins phone, computer, business, household, etc.
Money – bank, savings, investments account #s
Health – Advance Directives, Medical Power of Atty, health provider log-ins, medications taking with dosing and frequencies, other
Legal – wills, medical

Emergency Basics
Essential contacts
Important contacts
Alarm, keypads, extra keys
Medications, other important items location

Near Bodega Bay; about to take a nap with soft wind blowing over the mighty Pacific

Team
Who will do what such as open mail, pay bills

Local resources
Medical, caregiving providers
Funeral, related
Community groups

Things I need to do

“Shrink and disappear, little wretch!”

Third and last radiation treatment…

“Shrink and disappear, little wretch!!!” Message to the tumor from my niece, Mary.

The way it went for me: Jean drove us in the van to Dublin where the Kaiser radiation oncology unit is. The first time in Dublin we got there early, had a snack, hung out in the van, and went to the cancer center. The second time we took a sandwich and went to a park that Jean found via google and had a picnic and a nap with a cool breeze playing over us. The third time was like the second. I love it so much that Jean likes picnics and naps together like this.

I’m on the tray

At the cancer center I check in, wait a short while (meanwhile, Jean was running errands, walking), and they take me back, through the corridors, through the massively heavy 4” thick door into the room. I lie down on the plastic mold of my shoulders/torso (made on the previous planning visit) on the plastic tray and grasp the handles over my head. The radiation therapists move me in small increments until several bony landmarks of my body are aligned with the machine that will beam the radiation into my body, into the tumor. They leave the room and it’s just the machine and me. The plastic tray and I slide into the machine and I’m lying still, breathing however it is I’m breathing (it seems a little fast to me) and there are beams of light (red and blue? I’m not sure) and some things like appendages – to my left a flat thing, a blank plastic rectangle about 24” x 14” and to my right, a round thing, maybe 22” across with a ~8” glass square in the center like a window and there’s a third thing like a rectangular box and those three things slowly, silently rotate around me, sometimes it seems on different axes. So I lie there, very still and after about 15 minutes the machine powers down and the plastic tray and mold and I slide out. The RT is there. “Do you need help sitting up?” “Yes, thank you.” Experientially, it seemed like nothing happened except a little discomfort. Actually a lot happened. Beams of seriously high energy that breaks the DNA of the cancer cells and thus their ability to replicate/grow/spread.

The first time I was irradiated one of the radiation therapists and I talked for awhile. She told me that school for radiation techs takes about two years. School for radiation therapists is an additional 1.5 years, so 3.5 years to be there, getting it all right every time. I used to teach that part of being a patient was literally giving your body to another person/people and that trust was essential. True. The therapist was helpful to me – moving me a little farther along in the process of integrating these new realities, becoming little more connected. I felt like she was a truth-teller. Can’t do any better than that.

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For about 6 or 8 months I took Phana for weekly chemotherapy infusions and related at Presbyterian and MD Anderson. At Presbyterian when someone finished a course of chemo they rang a bell and everyone clapped. “They’ll never ring that bell for me,” she said.

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Phana and me, 30 years before she passed. Sigh.

Even caught early, with good odds of surviving it’s still realistically pretty heavy shit to have lung cancer. I feel almost dramatic when I say that, but you know, it is pretty heavy.

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Route 66

Many years ago I had a patient who had triplegia as a result of a radiation injury. He walked into the hospital and came home paralyzed in three of his four limbs. He lived with a prostitute and some other people in a shotgun house on the frontage road of a freeway. (A shotgun house is a cheaply built house, narrow, one room after another so that a shotgun fired through the front door would clear the whole house.). There was a bedpan and they put him on it and because the people in the house didn’t know anything and he couldn’t feel anything they left him on it for a couple of days. I got there and we got him off, but the blood supply to the tissue at his sacrum had been cut off for several days and over the next week or so, he developed a large, deep decubitus ulcer at his sacrum. I kept it as clean as I could with some help from the woman. She was a heavy drinker (they all were) and that and the nature of her work meant that she was gone or indisposed a lot. She was a nice person and I dug her, but she wasn’t dependable.

Several times when I was there the man was watching a TV program about sewing called Sew What’s New, which seemed odd since the program was hosted by a man all tricked out in pastels and lace and the live TV audience was all older white women and the patient was from streets, maybe pimping the woman, who brought her tricks through his room and on into her room. The ulcer/infection went into his bone and he became septic and died.

Way up into the alpine… rock, snow, ice, air.

A year or so later I saw the woman on the street. We talked for awhile. She still seemed nice and she still had that unpleasantly sweet alcoholic smell.

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My friend Joyce had 10 radiation treatments a year or so ago. Plus some other stuff. She’s doing fine. No evidence of recurrence. I know I’m getting off lightly, so far.

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David and me backpacking in Grand Canyon

When I was in my early 60s I began to look around, thinking about what I would do with the rest of my life. One answer was already clear: I would continue backpacking as long as I was able. The more time I could spend in the wilderness, the better. Wyoming’s Wind River Mountains became my place. I was in the Titcomb Basin four times, along the lakes, to the foot of the glaciers, in the alpine. The alpine, higher than any tree can grow, like magic to me. The Cirque of the Towers, the Highline Trail, Jean Lakes, Knapsack Col, Twin Glacier, snow, ice, rock, milky glacier run-off water, air, tiny alpine flowers, on and on. Also Big Bend, Sangre de Cristo Mountains, New Mexico, and more.

Another answer was to keep on traveling as long as we could. A typical trip was tickets from Dallas to San Francisco to see David to Hong Kong to Hanoi and 6 weeks later return from Bangkok to HK, SF, and home to Dallas. During that 6 weeks we’d ride buses, trains, and planes to Sapa, Hue, Saigon, Phnom Penh, Luang Prabang, Chiang Mai, Bangkok, etc. After Leslie passed away, Jean and I traveled to NYC, Barcelona, Granada, all over Wyoming, Colorado, and of course, California.

The Atrium Obscurum crew, meeting before the gathering

Sonic Bloom in Colorado

I also reconnected to my hippie roots through connecting to psychedelic medicine and to the psytrance scene. Talk about blessings! Try taking this in: I’m sitting with Jeff, my best friend since the war in Vietnam – we’re by a dirt dance floor somewhere deep in a forest in East Texas. We’re rolling very, very strong, I’m sitting next to a man I’ve loved since 1966, the music is loud, people dancing, dancing, I’m dancing, the stars above, pounding music through the night, into the day, and into the next night, and along the way I took the music in and was experientially connected to the psychedelic trance scene. The connection deepened over time and I joined Atrium Obscurum, a crew that was putting on forest gatherings in Texas. I went to gatherings in Texas, Oklahoma, Colorado, New Mexico, and Arkansas. Sometimes I taught classes on the end of life, PTSD, and psychedelic medicine. It was an amazing time in the reality of the global underground.

https://ckjournal.com/psytrance-trance-trance-culture

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When I finished my course of radiation, the nurse asked if I wanted to ring the bell. I said, no thanks – in tribute to Phana and because (so far) I’m getting off very lightly.

From our deck – Mt. Tam in the distance