Recording, Resources + Notes from Orthostatic Hypotension in PD, MSA, and LBD Webinar

Brain Support Network and Stanford University co-hosted a webinar last Monday, September 18th about orthostatic hypotension (OH) in Parkinson’s Disease (PD), Multiple System Atrophy (MSA), and Lewy Body Dementia (LBD).

———————–

RECORDING

We’ve posted the webinar recording here —

https://youtu.be/-FzsgUfQ_xI

———————–

SURVEY

If you listen to the webinar recording, please take LESS THAN FIVE MINUTES to answer six questions on our survey.  See:

https://www.surveymonkey.com/r/QGHVV85

———————–

RESOURCES

For additional information on the topics addressed during the webinar, see:

Orthostatic hypotension –
parkinsons.stanford.edu/orthostatic_hypotension.html

Parkinson’s – parkinsons.stanford.edu

Make an appointment with Dr. Santini at the Stanford Movement Disorders Center –  650-723-6469

Multiple System Atrophy www.brainsupportnetwork.org/msa

Lewy Body Dementia www.brainsupportnetwork.org/lbd

———————–

NOTES

Our terrific volunteer, Denise Dagan, took notes from the webinar.

Webinar
Orthostatic Hypotension (OH) in Parkinson’s, Multiple System Atrophy, and Lewy Body Dementia

Speaker:  Veronica Santini, MD, movement disorders specialist, Stanford University
Host: Candy Welch, former MSA caregiver, Brain Support Network
September 18, 2017

 

SANTINI’S PRESENTATION

Topics for this webinar are:
* Describe symptoms associated with orthostatic hypotension (OH) in
– Parkinson’s Disease (PD)
– Multiple System Atrophy (MSA)
– Lewy Body Dementia (LBD)
* List the conservative and medication interventions used for treatment

Normal Blood Pressure Response to Gravitational Change
Gravitational Change = changing from lying or sitting to standing, even climbing stairs.  Gravity pulls blood into the legs and belly (up to 1 liter, or more).  That means less blood goes to the heart, resulting in up to 20% less blood leaving the heart and consequent blood pressure decrease.  Normally sensors in the neck see less blood pressure and sends signals to close blood vessels, increasing blood pressure.  Important organs get nutrients and oxygen.

In OH the sensors are not working properly (baroreceptor reflex is dysfunctional), so blood vessels don’t close.  They stay open and blood pressure drops, causing symptoms.

Common symptoms include:  lightheadedness, dizziness, almost passing out, weakness, fatigue, visual blurring, headaches.

Less common are:  buckling legs, walking difficulties, confusion, slowed thinking, shortness of breath, imbalance, jerking movements, neck pain/“coat hanger headache”, chest pain

Rare symptoms include:  stroke-like symptoms, weakness or numbness, abnormal cramping/dystonia.

Evaluation of OH includes:
– History of autonomic symptoms
– “Orthostatic” blood pressure (BP) = measure BP in both laying and standing postures.  OH is defined as a drop of the systolic >20 or diastolic >10
– Neurological examination
– Autonomic testing can be helpful in distinguishing PD/DLB from MSA

Approach to Treatment of OH:
Conservative therapy first, then adding Medications and, if necessary, Combination therapies (both conservative and medications, even a combination of medications)

Goals of Treatment:
1. Prevent loss of consciousness (this leads to falls and potential injury)
2. Prevent close calls (almost losing consciousness and)
3. Identify and prevent symptoms of OH (leg weakness, falls, somnolence, confusion)
4. Improve fatigue, exercise tollerance and cognition

Actions to Avoid:
– Standing motionless
– Standing too quickly
– Working with arms above shoulders
– Hot environments (anything that leads to sweating)
– High altitude
– Hot baths
– Fever
– Dehydration !!!
– Vigorous exercise
– Fast or heavy breathing
– Large meals
– Alcohol
– Straining with urination or defecation
– Coughing spells

Conservative Management:
– Water ingestion (60oz/day!)
– Salt tabs, dietary salt (chips, pretzels, nuts, deli meats, soups, tomato juice)
– Head of bed elevation 10-20 degrees/4” or 10cm (reduces postural change extremes, and urination)
– Physical maneuvers that raise orthostatic blood pressure (standing calf exertion, raise one leg on a step, knee bends, single knee kneel)
– Cooling vests, leg sleeves, binders around the abdomen after eating to prevent blood rushing to gut for digestion

Medications:
Fludrocortisone (Florinef)
Mineralocorticoid, a-1 agonist = woirks by expanding blood vessel volumes
Dose 0.1-0.5mg/daily
Should be used carefully due to rise of volume overload, electrolyte abnormalities
Additional side effects: headache, swelling, weight gain, high blood pressure lying flat.

Midrodrine
Peripheral z1 agonist = Works by squeezing blood vessels
Dose 5-10mg 3x daily
Common side effects: pupil dilation, goose flesh, tingling, itching
Can also cause high blood pressures when lying flat.

Droxidopa (Northera) (Newest FDA-approved Rx)
Norepinephrine (NE) pro-drug but the exact mechanism of action is unknown.
Studies have shown low standing NE
Dose 100-600mg 3x daily
Common side effects: headaches, dizziness, nausea, blurry vision, high blood pressure
Can also cause high blood pressures when lying flat.

Doctors advise against lying down when using all of these so you don’t raise blood pressure too high. Never take them before bedtime so blood pressure doesn’t go to high while sleeping.

Non FDA-approved Pharmacology:
Pyridostigmine (Mestinon)
Improves standing BP in patients w/OH
Does not increase BP when lying down
Effective alone or w/Midrodrine
Side effects: diarrhea, salivation, nausea, vomiting, muscle cramps, twitching\

Yohimbine
a-2 adrenorectptor antagonist = increases norepinephrine and BP
Side effects: confusion, increase in heart rate, headache, or tremor
Medication interactions
Regulation of supplements

———————–

QUESTIONS AND ANSWERS  (all answers are by Dr. Santini, unless indicated)

Q:  What can caregivers do to help?

A:  Be the squeaky wheel by reminding your family member to keep hydrated, eat salty foods (even it that means the two of you eat different meals), help them check blood pressure throughout the day.  Also, give your doctor a symptom report so he/she has a full picture of challenges at home.  Doctors can’t fix what they don’t know about.  Sometimes patients get used to having low BP, so they don’t report changes to their doctor.  Caregivers can be more objective in how things used to be before BP issues arose, like seeing increased falls, more sleepiness, etc.  Caregivers need the right amount of support, as well.  Sometimes, the doctor can arrange for a nursing assistant to come into the home to do BP checks, or provide other services.  Just let your neurologist know if you are feeling the least bit overwhelmed.

Q:  How do you keep someone safe with OH without confining them to a wheelchair?

A:  Doctors should make sure the patient’s BP is good enough to have a full and active life.  It is a step-wise process, so be patient, but patients and their families or caregivers should be persistent.  Make sure all aspects of the patient’s health influencing BP is investigated, the big picture is formed and all therapies possible are attempted.

Candy:  We had a tilting wheelchair for my husband, who had MSA, so when he was feeling faint they could tilt the chair back making it easier for the body to maintain blood pressure, and preventing him from feeling awful or passing out.

Dr. Santini:  Neurologists are often able to write a letter to your insurance company recommending such a chair so that it is covered by insurance.  They are very expensive, but insurance did pay for Candy’s husband’s tilting wheelchair.

Q:  How does blood pressure affect brain function?

A:  There are several philosophies, but it is thought the blood carrying oxygen and nutrients doesn’t get to certain parts of the brain when BP is low.  The most upper parts of the brain affect both thinking and leg function.  Lack of oxygen and nutrients to these parts of the brain can cause all the symptoms mentioned; visual blurring, headaches, neck pain, dizziness, etc.

Q:  Are there any new blood pressure (BP) treatments?

A:  Yes, the newest is Northera.  Anecdotal evidence shows it to be quite effective.  But, the old ones are tried and true and new ones can be significantly more expensive.

Q: How do BP medications interact with Parkinson’s medications?

A:   There are several issues here.  Parkinson’s disease and atypical parkinsonian syndromes, like Lewy Body Dementia and Multiple System Atrophy cause problems with orthostatic hypotension.  So, the disease itself causes OH problems.  Almost every medicine doctors have to treat parkinsonian syndromes also drop blood pressure, unfortunately.  Patients should understand they need not suffer.  Let your physician boost your BP with some meds, then get your PD symptoms under control with other meds.  It is more meds, but if it improves your quality of life because you can move better and you can think and not be dizzy, etc. it’s probably worth it.  I frequently see patients who are not taking enough carbidopa-levodopa because it lowers BP.  I boost the BP, then add enough carbidopa-levodopa to improve mobility.  It’s a trade-off, but I feel quality of life is the most important thing while the patient is well enough in other ways to be active without feeling dizzy.

Q:   Can beta blockers help?

A:   With beta blockers you have to be careful. Beta blockers are often used for tremor control. We use those that don’t affect BP too much. They can be helpful for people who have very elevated heart rates.  Usually, the best treatment is to use the BP boosting agents. Oftentimes, in the absence of Northera, which can sometimes cause an increased heart rate, if you treat BP, heart rate can come down.

Q: What foods and supplements are best for OH?  Anything to be avoided?

A: It’s more how often you’re eating and how much you’re eating.  The bigger the meal, the more your BP can drop afterward.  If you are susceptible to BP drops after meals, an abdominal binder can be helpful.  Put it on about 10 minutes before a meal and keep it on for an hour afterward.  I recommend several small meals throughout the day, rather than three big meals. As far as what meals are best, we know some people have more difficulty with digestion of gluten or lactose.  Try going gluten free first for a couple weeks to see if it makes a difference for you.  If not, return the gluten and try going lactose free for a couple weeks.  It’s a good test to see if you are one of those with these digestive issues.

Q: What do you do if you have both OH and hypertension?

A: This is by far the most challenging of the group.  You have to decide on goals of care. Most commonly, people have hypertension, or high blood pressure, when they are lying flat. In that case you should avoid that flat position during the day.  At night we sometimes give a short-acting high blood pressure medicine, something like captopril, clonodine, etc.  It is more challenging when people have wide swings in BP.  It is extremely common in MSA and advanced PD.  Even standing or sitting people will have very high blood pressures, with systolic in the 180s of 190s.  Others will have extremely low blood pressures standing, with systolic in the 70s of 80s and they are passing out.  One thing doctors will do is ask patients to take their BP before they take the BP boosting medicine.  Then, the doctor will advise against taking the BP boosting meds when BP is already high, but to take it later in the day.  Sometimes, a person will need to avoid everything causing high BP.  Sometimes not treating high BP is the best option, even though that would normally not be recommended.  You have to treat which is causing the most symptoms and affecting quality of life.

Q: Can salt tablets help?

A: Yes, if you don’t like eating salt.  Talk with your doctor.  Taking a 1 gram tablet of salt in a tablet works better for some people than having salty meals.

Q: Can OH cause shortness of breath?

A: Yes!  It’s a common symptom because the upper lungs aren’t seeing as much blood as they usually do when BP is normal.  Gravity is pulling the blood down and those upper lung fields feel like they’re not breathing so people feel short of breath.

Q: Why does BP drop with exercise?

A: Sometimes it will raise, sometimes it will drop.  You may notice basketball players wearing sleeves on their ankles and legs.  Those are compression sleeves to help adjust BP.  When we exercise, the blood vessels open up so all the blood flow can get to those muscles that are working so hard.  The problem is that in OH we don’t have those extra reflexes to boost the BP back up.  Sometimes vigorous exercise can drop BP in people who have OH.  Those leg and arm sleeves can be very helpful in that case.

Q: Can OH lead to sudden death?

A:   It is a more rare circumstance.  It can certainly lead to heard dysfunction, and that could lead to sudden death.  We know that in autonomic dysfunction people can also have arrhythmias, and that can lead to sudden death.  If not exactly OH, sometimes it’s the autonomic failure that involves the heart that can lead to sudden death.

Q: Is OH more severe in MSA than in PD or LBD?  Is treatment of OH different with these three diseases?

A: Treatment tends to be similar but you have to be ready as the patient, caregiver, and healthcare provider to accept more OH in MSA. OH is typically more severe in MSA than in PD or LBD.  Sometimes very advanced PD or LBD (10+ years) may have severe BP swings, but MSA is more severe because OH occurs early in the disease course.  BP swings/OH is one of the most prominent symptoms people have in the entire MSA disease course.  Treatment goals in MSA may be different from PD and LBD as more accepting of BP swings.

These questions were sent in during the webinar:

Q: Someone has MSA w/OH but also supine hypotension (low blood pressure lying down).

A: This is easiest to treat because you just need to boost BP in all positions (lying down, sitting, and standing).  I would be concerned something else is going on and would recommend autonomic testing to determine that.

Q: Someone has primary autonomic failure (PAF) with possible MSA.  Does OH occur in PAF?

A: Oh, yes!  This whole category of PAF is a difficult one.  There is a current study looking at the natural history of primary autonomic failure.  Based on that research they are finding some of these patients eventually meet qualifications for an PD or MSA diagnosis.  Some people just have PAF, but not significant PD symptoms or progressive parkinsonism.  The main symptoms these patients have is OH and they really suffer from that.

Candy: This is how my husband was diagnosed with MSA.  First, doctors diagnosed PAF.

Q: Northera doesn’t help.  Should I stop and restart it?

A: No.  Sounds like you need to adjust the dosage.  Tell your doctor.  Sometimes, you just need to call or email, rather than make an appointment to see the doctor, for a medication adjustment.  Sometimes, they may ask you to come in in order to understand the problem.  If I had a patient report this to me and the patient was at the max dosage of 600mg/daily, I would cover all the bases with the patient.  I would reassess everything, confirming that the patient is drinking enough water, eating enough salt, wearing compression stockings. Does this patient tolerate Florinef and, if so, can we retry it?   Are you on an effective dose of Florinef or Midodrine, would adding pyridostigmine help the situation? When things get really tough, I sometimes temporarily reduces the anti-Parkinson’s medications (carbidopa-levodopa or dopamine agonist).  Sometimes reducing the PD meds isn’t what’s necessary, but increasing carbidopa can reduce side effects of levodopa, sometimes.  It’s worth looking at.

Q: Can coconut oil help OH?

A: Harmful? probably not, unless you have high cholesterol.  Ask your doctor if you should or should not be eating coconut oil, based on your health numbers.  There is no evidence that it helps.  It’s just the new magic for everything.

Q: Questioner feels faint while having a bowel movement. What can be done?

A: Either urinating or defecating activates the opposite side of the autonomic nervous system, lowering blood pressure.  People have passed out on the toilet.  Bathrooms are dangerous with hard surfaces to hit your head on when passing out.  The answer is to treat the constipation so there is no straining.  Don’t treat to the point of diarrhea because there can be straining with that, as well. Any of these may help:  Miralax, Senna, Cholase, or any stool softener. Another solution may be to put your feet onto a stool so knees are raised while on the toilet (Squatty Potty) can help defecation without straining.  Massage the lower belly while trying to poop can help move your bowel.  Best to poop after a hot meal and around the same time every day.

Q: What about SSRIs (antidepressants) for OH?

A: Yes, Prozac has been studied for use in OH. There is some research that it can help boost BP.

Q: Is Parkinson’s with OH more severe than PD without or a more rapidly progressing form of PD?

A:   Everybody who has PD has a different form of the disease. I have heard the strangest symptoms that a neurologist would consider ‘off medication’ symptoms, or those not normally attributed to PD, but happen to be attributed to that person with PD.  It’s very common for people with PD to have OH.  They are just a little unlucky because with OH you get a lot of symptoms.  Although you feel horrible and like you’re dying, sometimes, it doesn’t mean that your PD is more severe, just because you have those symptoms.  It means it’s something we need to treat and get your quality of life better.

Q: It seems OH research is focused on MSA. Do you feel that is true, and if so, why?

A: Yes, it is very true because patients w/MSA have OH symptoms early and severely in the disease course.  Researchers feel that if they can develop a treatment for OH in MSA, it will help those with PD.  I feel more studies should be done for OH in PD because improvements in OH improves cognition and physical activity for patients with PD.  Up to 30% of newly diagnosed PD patients have OH, so they would benefit from OH research.

Q: If I have severe OH, what kind of doctor should I see?

A: It depends on the specialty at different medical centers.  If you come to see a movement disorder specialist at Stanford, I have had specialized training in treating OH.  But, some movement disorder specialists prefer you see an autonomic specialist if you have OH. Other specialists who can treat OH include cardiologists or nephrologists.  You just have to find the specialist most comfortable in treating OH at the medical center where you are being treated.

Q: Is OH caused by a pathology in the brain?

A: People with MSA, LBD and PD have an abnormal buildup of the protein alpha-synuclein in certain brain cells.  These people can be affected by OH.  Other atypical parkinsonisms, like PSP, CBD, etc. that don’t have alpha-synuclein don’t have OH so we feel there is a connection between OH and alpha-synuclein.

Q: Does Stanford have an autonomic testing center?  Do you know where other autonomic testing centers are located in the US?  What is the benefit of having this testing?

A: Stanford has a very good autonomic testing center.  It is especially useful for people who have diabetes and PD, or in cases where symptoms seem more severe than what would be expected in PD so you would like to gather more information to determine if it is really MSA.  For these people, it may be a good idea to have autonomic testing. Stanford is probably best place for autonomic testing on the west coast.  Mayo Clinic in the midwest, and there are several places on the east coast are terrific, like Beth Israel.

Q: Some research shows that doctors see OH and automatically diagnose MSA.  What’s happening here?

A: I see people newly diagnosed with PD who have some OH and they have been misdiagnosed with MSA.  They actually have PD, but because PD medications lower BP, the medications can make their symptoms look more like MSA early in the course of their symptoms.  When there is a question as to whether someone has PD or MSA, autonomic testing should be done to differentiate between the two.  Seeing a movement disorder specialist rather than just a neurologist because they are specially trained to use set literature criteria that helps to differentiate between these conditions. The history of a person’s initial symptoms helps me figure out an accurate diagnosis.  Also, seeing how a person’s symptoms progress helps to determine an accurate diagnosis.

Q: What does autonomic testing look like?

A: The patient lays flat on a special bed.  There are several tests.  In one they infuse a medication that causes sweating to see how autonomic nervous system responds.  They may also have the patient do deep breathing to see if their heart rate and blood pressure responds correctly.  They also suddenly change the patient’s position from lying to standing (by tipping the table up quickly) to see how heart rate and blood pressure system responds. Depending on the body’s responses to all these different tests, they can determine if they are normal or abnormal.  If there are abnormal responses, it the problem coming from the brain or from the peripheral nervous system. That can be helpful in differentiating between disorders.

Q: What about Methotrexate?

A: That can be used if there is an immune component to the patient’s autonomic dysfunction.