We are at our limit. Please, anything will help.

[rtoth256]

This year has been an utter nightmare for my wife and I, and is starting to tear us apart.

My wife was recently (as of September/October) diagnosed with POTS.

Nothing has helped. I am at my limit. I need help. No help is coming, so I am here.

A medical background:

• 26yo Female.
• Type 1 Diabetic (see timeline below: no islet cell antibodies, just GAD65 antibodies).
• Has prior diagnosis of Neurofibromatosis Type 1.
• Has hypothyroidism (unsure whether this is Hashimoto's, thyroid antibodies are being checked).
• Full spinal fusion in 2010 due to severe scoliosis.
• Has Tarlov Cyst in lower spine, present since roughly 2015.
• History of vasovagal syncope (multiple incidents from 2014 to March 2022, attributed to dehydration).

Current symptoms:

• Heart rate spike when standing. Highest we've seen is 140.
• Light-headedness when standing or sitting.
• Blood pooling in legs after periods of standing or sitting.
• Constant 4/10 headache that does not vary in intensity with any treatment (NSAIDs, rest, hydration, steroids, etc.)
  • She wakes up with it, she goes to sleep with it.
  • This is, apparently, the hardest symptom to understand. She is never without pain.

A timeline:

• Mid-May, 2022: Wife had bouts of urinary urgency. Thought it was a UTI, used OTC medications to clear it up.
  • She's doesn't usually have UTIs, so this was odd. No painful urination, just urgency.
• May 31st, 2022: Urinary urgency wouldn't go away with OTC medication, went to clinic for antibiotics.
  • Urine test revealed glucose present in her urine. Blood test revealed moderate hyperglycemia (230mg/dl).
  • Diagnosed as a pre-diabetic, type 2. At 120lbs and 5'4", this seemed incredibly unlikely.
• June, 2022: Management of blood sugar through diet, exercise and routine seems to have little effect.
  • Blood glucose levels are sporadic, carbohydrate management seems to work only half the time.
• July 9th, 2022: Woke up with a steadily progressing headache, capping out at 4/10 on the pain scale.
  • Tried excessive hydration, no relief.
  • Blood sugar was normal, not hypoglycemic or hyperglycemic.
  • Advil, Tylenol, NSAIDs, no relief.
  • Resting/sleeping, no relief.
  • Her headache wouldn't go away. Constant, unending, she wakes up with it and falls asleep with it.
• July 13th, 2022: The day her world ended. In addition to her constant, unending headache, she started to develop severe bouts of light-headedness.
  • Light-headedness only when standing and sitting, not lying down. Essentially renders her bedridden.
  • Clinic visit same-day revealed elevated white cell counts indicating some kind of infection.
  • Physician prescribed antibiotics and said she just had a "sinus headache" despite no sinus symptoms.
• July 20th, 2022: Clinic visit with her primary doctor as a follow-up.
  • Injection of high-dose toridol to attempt to break her headache. Does not work.
• July 22nd, 2022: Her symptoms get worse. Several days on antibiotics leads to an allergic reaction.
  • Hives, non-painful and non-itching cover her entire body. Only noticed when we lifted up her blanket when she woke up.
  • Went to emergency clinic, essentially told to start antihistamines and stop taking antibiotics.
  • Now listed as an "allergy". No prior medication allergies found, and she's been on this particular antibiotic prior.
• July 29th, 2022: Her symptoms exceed what is tolerable and we take her to the ER.
  • Incredible light-headedness when standing.
  • What appears to be blood pooling in her feet/legs when standing or sitting.
  • ER tries to treat her constant headache with migraine cocktails to no avail.
  • CT w/angiography "unremarkable", MRI shows slight sign of vasculitis.
    • MRI technician and physicians were unaware of NF1 diagnosis: slight enhancement of corpus callosum deemed "acceptable" but still noted.
  • Given IV fluids, discharged with no relief in headache or postural symptoms.
• August 1st - 7th, 2022: Her neurologist sees the word "vasculitis", wonders why she's been discharged and sends her to another ER to be admitted.
  • Leading to one of the worst weeks of her life, she's admitted to the ER.
  • Spinal tap, blood sample collection, MRIs.
  • Admitted to hospital's neurology wing.
  • Several days of attempted treatment and scans with no relief.
    • Suspected CSF leak leads to CT myelography. Tarlov cyst found, but no leaks.
    • Several MRIs of her head, neck and spine, all show normal physiology.
    • Placed on several drugs to possibly treat her headache, no relief.
    • Placed on 500mg of IV prednisone trial for 3 days, no relief.
      • I'm starting to think (or hope) that this was just a placebo, but cannot confirm.
  • Discharged. No relief. No diagnosis. Nothing.
• August 25st: Met with an Endocrinologist.
  • Discussed existing diabetes diagnosis, antibody tests ordered.
  • Discussed possible pheochromocytoma and pancreatic/renal involvement.
    • Ruled this out with blood serum tests, but haven't completed 24hr urine test yet.
  • Positive GAD65 antibody test, but no islet cell antibodies.
  • Confirmed as a Type 1 Diabetic, qualifying for insulin and other medications and devices.
• August 31st: Met with Cardiologist.
  • Fitted with Holter monitor prior to referral. Data upon review shows no arrhythmia. Does show tachycardia.
  • Placed on a trial of Midodrine to see if it helps with her symptoms.
  • Also placed on a trial of Metoprolol.
• September 22nd: Autonomic testing (tilt table).
  • Pursued referral to the only autonomic specialist in the state.
  • Tilt table test findings are as follows:
    • Severe blood pressure drop when standing for medium to long periods, ending with a BP of 80/90.
    • Blood pools in her feet and legs, most noticeable at the 5 minute mark.
    • Heart rate skyrockets into the 120s and beyond, with a gradual ramp-up as blood starts pooling.
    • Venous return is poor. 5-10 seconds on capillary refill.
    • Sweat test was compromised by a faulty sensor.
  • Confirmed diagnosis of POTS.
  • Blood work for a number of antibodies pending.
• October 2022: Numerous medications tried with little to no effect.
  • Bisoprolol, tapered to max dose: No effect.
  • Metoprolol: Little to no observed effect.
  • Propranolol: No effect, slight chest tightness.
  • Pyridostigmine: No effect, diarrhea/fatigue.
  • Midodrine: No effect, chills/hot and cold flashes, fatigue.
• November 13th: Frustrated with literal months without any relief, had her take the following cocktail:
  • 400mg of Metoprolol Succinate, spread across two doses (200mg earlier in the day, 200mg at the time of taking the cocktail).
  • 15mg of Midodrine.
  • 180mg of Pyridostigmine.
  • Took a downward turn with side-effects, but resting heart rate lowered to 55-60 BPM when supine.
  • Slept through the night.
• November 14th: A ray of hope. She woke up feeling the best she's ever felt.
  • Heart rate more normal than I've seen in several months.
  • Venous return looked incredible. Little to no blood pooling.
  • Light-headedness not entirely gone, but significantly better. Could tolerate hours of standing and sitting.
  • We both cried in joy and shared a sunset together.
• November 15th: Blood work. She needed to be fasting and off all medications for the day, and had around half a pint of blood taken.
  • Obviously weak and fatigued, rested most of the day, but started again on 400mg of Metoprolol.
  • The blood draw essentially knocked her out for the day.
• November 16th: A critical mistake almost cost her her life.
  • A perfect storm sent her into syncope.
    • 500mg of Metoprolol Succinate, qualifying as an overdose.
    • 360mg of Pyridostigmine.
    • A hot shower.
  • Had to do chest compressions as I couldn't get a pulse.
  • ER visit and glucagon got her back in "normal" ranges.
  • Physicians Desk Reference lists 400mg as the max dose for Metoprolol Succinate.
    • We knew this beforehand but wanted to test the waters. Never again. Always adhere to PDR's max dosing requirements.
  • This erased all of the hope we had on Monday. Back to symptoms.
• November 24th, 2022: Loss of hope, the future.
  • Completely burnt out.
  • Our marriage is strained, as this turned from "we'll get you better" to "our lives, especially hers, have been stolen from us".
  • Trying to reproduce what happened on November 14th by re-trialing the medications individually and in combination.
  • Contact with doctors is sparse due to under-staffing.
  • We are on our own.

I have no idea what to do anymore. Someone, please help. I'll give you any data you want. It is impossible to continue like this.

[Looking_for_light]

 

So sorry and saddened to hear this. I'm in a state of despair due to being a carer for a relative with suspected dysautonomia and suffering from other serious conditions. 

Wonder what experienced people here think? 

Did they tell her what subtype it is? Possibly sounds more like neuropathic, perhaps with hypovolemia? 

Have renin and aldosterone been tested? 

Compression garments up to the waist for the blood pooling?

More salt or salt tablets? 

Eating extra fluid - e.g soups? 

Possibly enquire about trying another option - Fludrocortisone (Florinef)? 

It will be very frustrating but many things need to be tried for a while to see improvement. 

Really hope things improve soon. Take care. 

 

 

 

[rtoth256]

We don't have a confirmed subtype yet.

Renin and aldosterone haven't been tested. We've tried compression garments, but they don't seem to have a noticeable effect.

We've elevated the sodium in her diet but still no effect, though we haven't tried the capsule form of sodium supplements, just the tablets.

We've requested Fludrocortisone, but her cardiologist "doesn't usually prescribe that", so we're being left in the wind.

We live in the PNW, so I've always thought Mayo was out of reach.

Thank you for all the well wishes.

[rtoth256]

She had a CT of her abdomen to screen for pancreatic cancer, confirmed no tumors on her adrenals.

Quest didn't draw labs for standing catecholamines, said "they don't do that test". Trying to find a lab that will draw them.

She is being treated for hypothyroidism and has been since she was young, using synthetic thyroid hormone.

We've checked all of her hormones multiple times. She's had a glucose tolerance test; her pancreas is just not producing enough insulin to keep up, but there's no evidence of damage or islet antibodies.

Nothing happened prior to all of this occurring. It happened suddenly, out of the blue. The only immunization she had this year was a COVID booster (Moderna) in January, with double Pfizer shots last year.

Her headache doesn't qualify as a migraine because it never goes away. Ever. That's the hardest part for any clinician to understand: they just class it under migraines. It never goes away.

I'll try to get in touch with Dr. Wright, but I suspect we'll be on a long, long waiting list. The autonomic clinic at UW has a waiting list of 300+ people.

[Looking_for_light]

You're right that would be classed as

new persistent daily headache

rather than migraine.

Worth looking that term up to look for treatment options. 

Have you considered mast-cell activation as a possible factor? Worth looking to see if it resonates with the overall picture. It causes headaches too. 

So nice to feel that can help people. 

Issue over here is sadly hardly any knowledge, care or help, hence our desperation to seek guidance from patients. 

Very best of luck to you both

 

[Looking_for_light]

Not come across evidence on specific diets for persistent daily headaches.

A healthy, anti-inflammatory, Mediterranean-style diet with lots of fiber would keep bowels and sugar stable. 

[MikeO]

33 minutes ago, rtoth256 said:

The autonomic clinic at UW has a waiting list of 300+ people.

is this in wisconsin or oregon?

[rtoth256]

She's having her tryptase levels checked via the bloodwork we submitted, that appears to be a test for MCAS.. so maybe? I've looked at MCAS but there's no "allergic reaction" type symptoms that she has apart from that one reaction, so I moved on to other causes.

[rtoth256]

Just now, MikeO said:

is this in wisconsin or oregon?

Washington state.

[rtoth256]

Adding to this, ANA and ANCA were normal, C3 and C4 were normal.

[rtoth256]

No immediate symptoms from the COVID shots, I will definitely put her on a trial of H1/H2 blockers. Her C-peptide levels were low, and she has routine thyroid panels every year to see if she needs to adjust her dose (she's been on 75mcg of Levothyroxine for 12+ years now).

She has a full spinal fusion, so manipulation is impossible (she cannot move her spine).

B12 and Vitamin D were low, so she's taking supplements for both.

[rtoth256]

Something to add...

• Her Diastolic BP is high, sometimes in the low 80s, sometimes in the 100s.
  • 80/90 was seen during her tilt table tests, recent test on 10mg of Midodrine (no Metoprolol) showed standing BP of 115/105.
• Her last cholesterol test showed she has 259 mg/dL of Cholesterol and 170 mg/dL of LDL Cholesterol.
• We're re-trialing Midodrine with more controls and measurement. Midodrine and Metoprolol seem to be a common-ish combination... hoping they yield results.

Does anybody have any literature on the interaction between alpha agonists and beta blockers? I'd like to know if there's a compensatory beta stimulation effect when someone's put on an alpha agonist.

[Pistol]

@rtoth256 I have HPOTS with high BP, especially the diastolic. My BP is currently controlled but it took many years to find the right meds. Before seeing my excellent autonomic specialist I was seen by various cardiologists that did not know anything about POTS. Since Midodrine is a first attempt meds for POTS I was put on it, but of course it did not help. Once I got in to see the specialist he took me off it right away and said that Midodrine should not be used in people with high BP - like in HPOTS. It is usually used for LOW BP. 

The subtype is AVSOLUTELY important because different types will respond to different meds. Even a physician with little knowledge of dysautonomia should know that!

[Looking_for_light]

@PistolIs high dBP especially characteristic of H P? If so, do you know the mechanism of that? 

If so, my relative's sadly textbook in that regard too! Devastating symptoms are a living nightmare. 

Do both BP measures have to go up by a certain number on Tilt to confirm H P? 

Understand its mechanisms a lot more after you described vasoconstriction - thanks! 

Your CCB can also be used for SVT. Do you have that too? Is it (or other arrythmias) common in H P? 

Any specialist (rare as hen's teeth here!) would agree with us - subtype is vital! Highly concerning hearing that a top cardio here thinks it isn't, but doc isn't a specialist, but a cardio with an interest in the subject - different! 

Have you heard any members with H P mention any helpful treatment with any uk docs?  

Dire situation.   

Thanks! 

 

On 11/26/2022 at 9:45 AM, Pistol said:

@rtoth256 I have HPOTS with high BP, especially the diastolic. My BP is currently controlled but it took many years to find the right meds. Before seeing my excellent autonomic specialist I was seen by various cardiologists that did not know anything about POTS. Since Midodrine is a first attempt meds for POTS I was put on it, but of course it did not help. Once I got in to see the specialist he took me off it right away and said that Midodrine should not be used in people with high BP - like in HPOTS. It is usually used for LOW BP. 

The subtype is AVSOLUTELY important because different types will respond to different meds. Even a physician with little knowledge of dysautonomia should know that!

Thanks! 

[Looking_for_light]

@Pistol As well as feeling horrendous for sufferers, does the increased circulation of stress hormones in H P and lots of adrenaline dumps etc do permanent damage the body (with no treatment)?! 

Thanks! 

 

[Guest KiminOrlando]

Was just coming here to discuss a Mast Cell disorder. Evidently it is a spectrum. Tryptase can be negative and you can still have mast cell issues to a degree. May also want to consider making an appointment with Vanderbilt Cardiology to see if they will refer you to their Autonomic Clinic and maybe their Mast Cell specialist. 

My Mast Cell doctor has me taking 2 OTC Xyzal, Quercetin, and 2 Pepcid AC per day. Also was told that meds can trigger 'leaky' Mast Cells. BETA blockers were one of those meds. 

This case is very complex. In my opinion you need to be at Mayo Rochester, Stanford, Vandy, Cleveland Clinic- some place like that. 

I believe my headaches are Mast Cell related as well. They get better with Mast Cell drugs and don't really get better with some migraine drugs.

Keep advocating for her. She needs you. 

[rtoth256]

Unfortunately, we cannot travel safely. We're effectively limited to in-state until we get some measure of treatment that allows her to travel. If I could, I'd have her at Mayo in a second, d*** the cost. If we can be treated remotely, that'd be amazing. Having someone look all of this over and help in throwing darts at the wall would put our minds at ease.

The 10mg Midodrine trial yielded some interesting results:

• She was tachycardic in bed prior to administration. That's not in line with the POTS/OH phenotype (you'd expect it with postural changes, but supine HR rises are weird).
• When the prodrug finally fully metabolized and hit its full peak, she began to experience chills and fatigue, not a "normal" response. Her BP was 115/105. Elevated BP, but high diastolic kept me thinking "that's not supposed to happen".
• Heart rate took a mild dip but ultimately stayed well above 75 even when supine. Supine BP was also high, with elevated diastolic BP.
• The real weird thing: her blood glucose went wild. Sawtooth waves. Sharp increases, and then sharp decreases, repeatedly, until the dose was out of her system.
  • This is not a researched effect.

I'm starting to think she may be hypovolemic. Looking into the stages of hypovolemic shock, she seems to be on stage 2. I have a working theory that increased pancreatic and liver perfusion from the midodrine is leading to increased, sporadic insulin production, which if she's hypovolemic could explain it.

You typically don't give antihypotensive agents to hypovolemic patients, as increasing blood pressure without increasing blood volume causes blood to circulate erratically instead of forming a smooth gradient, resulting in a "slingshot" or "rocket" effect.

Walking back my steps to the 14th, we cooked steaks that weekend and she ate them over the course of two days. Her hemoglobin levels are slightly low, as is her sodium, and heavy salt + iron-rich meat from well seasoned steaks might have contributed to a gain in blood volume. That, combined with high-dose midodrine probably gave her a solid night's rest where her system was functioning normally (normal organ perfusion), and the metoprolol kept her from feeling the heart rate effects of her blood volume decreasing the next day into Tuesday.

That's my current working theory. If she is hypovolemic, we need to take steps to treat that. I'm going to contact her endocrinologist to check her renin and aldosterone levels. Fludrocortisone and an excessive amount of sodium supplements are probably our next step.

Thank you all for your kindness. It's hard to feel hopeful. ♥️

[Guest KiminOrlando]

Fludrocortisone was difficult for me to obtain initially as well. I am hypovolemic. When I got it and started salt pills, it changed my life in 24 hours. It tanked my potassium though.

[Guest KiminOrlando]

The blood sugar thing is wild though. 

[Looking_for_light]

Sending thoughts to you both. 

[Looking_for_light]

Be sure to start at the lowest possible Florinef dose, assessment of calcium levels and bones, and have her eat more healthy foods rich in calcium, boron and vitamin k, eg leafy greens, broccoli, cauliflower, chickpeas, tofu, tempeh, tahini... etc, as it sounds as if it can play a role in osteoporosis, unfortunately. 

[rtoth256]

Will do. I'm going to base her trial diet on whatever she's deficient in w.r.t the latest blood work. Whatever she can't get/tolerate from food, we can supplement via direct supplements until she can tolerate it.

[Looking_for_light]

Do you, or anyone here, know all the signs and symptoms of hypovolemia? 

Are not feeling hydrated no matter how much is drunk, and a frequent sensation of dehydration with an extremely dry, taut throat at any time of day indications? 

Thanks!

[Looking_for_light]

What's w.r.t? Sorry, always lots of abbreviations here so it's taking time to understand them all. You sound extremely knowledgeable on so many medical matters, wondering if you're in the profession. If not, then you could have been! 

[rtoth256]

Haha, I'm getting that a lot from some doctors. "w.r.t" is "with respect to". I'm actually a programmer, so I'm trying my best to keep up with the literature and apply what I know from systems thinking.