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HBP Fiasco #3 + The Resolution: March 2019 – June 2020

March 2019

Three months-on with an anti-hypertensive medication that I was sort-of tolerating (atenolol) and with a new one (valsartan), I was hopeful in succeeding with my 11-month journey to control my high blood pressure (HBP) and then Wham! I was struggling to maintain consciousness.

Suddenly, the 10-15 mile hikes and 25 mile bike rides on my calendar were non-starters: my systolic blood pressure (BP), the upper number, dropped 75 points in 25 minutes and the lower number dropped by half. When that happens, one’s heart rate is supposed to pop-up to compensate, to keep you alive, but my primary medication, the atenolol, does its job by suppressing heart rate, so my heart rate merrily dropped further.

At one point, Bill said he had been 10 seconds away from calling 911, which I could feel by the tension and moisture change in his hands, but what would they do? My heart rate wasn’t responding to the adrenaline my body was kicking out in the crisis, so our Epipens in the ready would have done no good either. Fortunately, like the day before, after about 90 minutes of high alert for both of us, the same crisis passed. I could bear to open my eyes, then a bit later, to sit up. Frequent monitoring again showed wide BP swings throughout this second sick-day.

I’d decreased the newest medication, the valsartan, the evening after the first episode with little effect, so next up would be decreasing the first medication, the one that was destroying the “keep her alive’’ heart rate response. All we could do was hope that I would get improvement overnight, that it wouldn’t take days for the dose adjustments to extract us from this drama. Had I been descending a mountain on my bicycle when those sudden drops in blood pressure occurred, I could have had a fatal crash.

We didn’t know why I was experiencing these big BP drops but it seemed pointless to seek professional help: doctors’ frustrations with managing my uncontrolled BP at scheduled appointments were vented on me in the form of negative comments, which hadn’t yet had a positive therapeutic effect. And like before, our feeble interpretations of the unexplainable events would be dispatched with distain. It took another week of feeling weak and tired after 3 days of severe low BP events to feel stable again.

Going Dairy-Free
These terrifying BP drops in March of 2019 had occurred mid-morning on days #7, #8, and #9 after withdrawing dairy from my diet; my BP dropped precipitously for several hours, then returned to my typically too-high levels.

Ten days prior, I had concluded that I might have a diary allergy and that, just maybe, there was a link between having a dairy protein allergy and hypertension. I decided to go dairy-free, even though there is no association between the two in either main stream or alternative medical literature. It was plausible that a dairy protein allergy could create inflammatory damage to the inner lining of my blood vessels and some consider hypertension to be a disease of inflammation so, in my mind, it was worth a try. Being desperate to find a solution to my HBP issues, I was willing to try almost anything. It could be a double win if I was indeed allergic to dairy proteins to stop consuming them.

The dairy allergy hypothesis soon looked true based on improvements in my cognition, rashes, and gut. Blood tests a month later when at home confirmed that I was allergic to both milk proteins, whey and casein. I already knew from elimination diet testing on my own years prior that I was lactose intolerant.

Abruptly withdrawing dairy from my diet was associated with transient, catastrophic drops in my BP but didn’t trigger the long term BP improvement I craved. There was no way to know if withdrawing dairy had anything to do with the BP crises or not.

Interestingly, 13 months later, again when on 2 different medications, I had a similar BP crash. It only occurred on 1 day instead of 3, and my BP didn’t drop as low. It too was totally out-of-the-blue. Both experiences renewed my commitment to take charge of my medications, to do what I needed to do to keep them from killing me, directly or indirectly.

About a month after the 3 day episode of abrupt BP drops in March of 2019, I took myself off of the atenolol because its heart-rate lowering effect was devastating to my athletic performance. A month later, I discontinued the valsartan while overseas because it was affecting my muscles, resulting in dangerous stumbling on steep trails. I took my chances on only protecting myself from a stroke by using acupuncturist prescribed herbs through the summer, though they never improved my BP.

In the fall, I took another “medication holiday,” like I had done the previous fall, by delaying the latest antihypertensive (doxazosin) from my internist so as not to risk a show-stopping, low BP event while in the Grand Canyon. There we do the epic Rim-2-Rim-2-Rim hike of 46 miles and 11,000’ of elevation gain in 2 days and then repeat it again about a week later. The isolated interior of the Canyon would be a terrible place to suddenly have a repeat of these severe, unpredictable, hypotensive events.

I invited a Traditional Chinese Medicine (TCM) practitioner to continue treating my hypertension from the perspective of his discipline in weekly appointments while we were in the Palm Springs area for 4 months over the winter of 2019/2020. We had begun this journey together early in 2019 with 9 acupuncture treatments in weekly appointments and while traveling over the summer and fall of 2019, I sought the services from 4 different acupuncturists, including 1 in Italy.

I gave acupuncture and TCM a good go with a total of 35 treatments over 13 months from 5 different people, supplemented by Chinese herbs from 2 practitioners, but none of it cracked the code on my HBP. I performed prescribed acupressure and did some yoga-like poses to improve my flagging Yin energy. The acupuncture may have lowered my BP a tad, it did make my BP a bit more stable, and it definitely helped with the medication side-effects but after those 13 months, it became clear that it wasn’t the solution I needed. I would however recommend others try acupuncture for their hypertension because it is an effective treatment for some people.

A Year Later
In February of 2020, I was panicking about having a stroke and on the recommendation of a physician friend who had retired from Loma Linda Medical Center in southern California, I saw a nephrologist there. I had an appointment with a nephrologist at home on the books for April, but I was too scared to wait. Loma Linda accepted self-referrals and it was a little more than an hour drive from our trailer park in Palm Springs, so it was worth a try.

This doctor (a vegan) was convinced that my higher protein, high fat, keto diet was to blame for my HBP and that if only I too were a vegan, that my BP would drop to 110/80 in 10 days. To the amazement of people who knew how committed I was to my 6 year stint on the keto diet, I accepted his challenge and took 2 weeks to transition my diet from eating a pound of meat a day to none and spent 2 weeks as an ovo-vegan (I still ate 2 eggs a day for the protein). Not surprisingly, there was no change in my HBP. I resumed my keto diet and he added a second antihypertensive to my regime (lisinopril), the same drug that I initially took almost 2 years ago.

By May of 2020, a couple of months into the COVID-19 pandemic when 85,000 Americans had died, including 1 friend, I was totally discouraged. I had stopped documenting my ever-hopeful journey to find a fix for my hypertension for over a year. I had given TCM a good try. It was the same old story: failure on more anti-hypertensives and more doctors expressing their anger/frustration at me with bullying and vivid images of my life being changed forever by a stroke.

In my “leave no stone unturned” approach, I’d also tried hypnosis, guided visualization, and breathing technics. There had been treatment strategies from 4 naturopaths and 5 physicians. I’d used 7 different antihypertensives and some of them twice. I taken the drugs solo and in combination, two and three at a time. Each drug had its trademark mix of side effects, including headaches, dizziness, abdominal cramping, loose stools, malaise, depression, brain fog, fatigue, and sapped athletic ability. Side effects would hit like clockwork. The headaches were dismissed or attributed to my HBP even though that had never been my experience. My first internist labeled me as having psychogenic reactions that were systematically sabotaging my medications and making my BP go up on the drugs. I kept thinking “If I am so awesomely powerful, why wouldn’t I employ those talents to make my BP go down??”

The Answer
Finally, on June 16, 2020, I had a precise diagnosis, I had the answer to why I had HBP despite my high level of fitness and lack of usual risk factors: I had Liddle Syndrome and its hallmark is hypertension. Very few patients with hypertension ever know the source of theirs, but I was one of the lucky ones. I now knew. And better yet, there was a cheap pill to treat it.

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I bought these tulips to celebrate my BP drop after the first week on the amiloride.

We had had a preliminary celebration a month earlier when Bill recalled the Portland nephrologist mentioning the name of this condition, among others, when we were eating lunch on the trail. We actually had internet service at our lunch perch in the Colorado National Monument and since I needed to extend our stop to stretch out a painful buttock muscle, I suggested that he read to us about the condition.

We’d been stunned when 4 days after I took the new drug, when I first checked my BP, that it had dropped into the acceptable range of 135/90. It was astonishingly fast, especially given that I’d never responded to an antihypertensive. We were excited and intrigued by my response to amiloride, a response which is characteristic of Liddle Syndrome.

I wasn’t textbook at all for Liddle Syndrome but Bill, and later my nephrologist, made the diagnosis on the basis of 2 important findings: my HBP didn’t respond to the 7 other antihypertensives I’d taken and that it did respond to amiloride, an old, largely ineffective diuretic. Those observations were definitive in the eyes of my nephrologist.

It was a genetic mutation that was raising my BP. I either inherited it from my mom (who stroked from her poorly controlled HBP) or I have a de novo mutation. Liddle Syndrome is very, very rare and I am a variant of even it, especially since my HBP didn’t present in childhood.

The mutated gene in Liddle Syndrome causes too many sodium reabsorption channels to be present in the kidney, resulting in too much sodium being reabsorbed from the urine, causing excess sodium to be retained by the body, leading to hypertension. The amiloride works by inactivating the excess sodium channels in the kidney that accumulate in Liddle Syndrome. These critical sodium channels occur in tissues throughout the body and we are curious to see what other positive changes in my health that I might notice in the coming year from taking amiloride.

There is a $1400 genetic test for Liddle Syndrome that I won’t bother with: interesting but of no utility since I have a treatment. I’d pay $300 for the genetic test out of curiosity, but that’s it. The nephrologist didn’t mention it. She did however add a bit more good news: Liddle Syndrome does not contribute to chronic kidney disease—the chemistry behind this source of hypertension is different than the usual disease.

I had 2 low lab numbers that were consistent with Liddle Syndrome but more importantly in making the diagnosis, as my Portland nephrologist said, my body is “exquisitely sensitive” to the treatment. The smallest pill is 5 mg, which is what my doctor prescribed, but I’d only been taking half that for 5 weeks. I had had so many bad experiences on HBP medications that I never started with the full dose. Fortunately, my nephrologist was satisfied with my reduced BP and felt no need to push it lower by taking more amiloride. And indeed, she was right again, it is a “lousy” diuretic, I had no clue that I was on a diuretic. None. Phew!
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Cutting, crushing, & dividing my amiloride tablets into “pixie dust”.

Five weeks after starting the drug and getting my official diagnosis, I cut my dose in half and my BP didn’t change, it stayed at 135/90. A week later, I cut it in half again, down to 0.6mg/day, and my BP held. We had no explanation as to why my “exquisitely sensitive” body managed to correct its genetic error with so little medication. It was like it only needed a whiff to change course. We quietly wondered if I really had Liddle Syndrome or if something even weirder was amiss.

I had headaches, dizziness, GI upset, and cognitive dysfunction side effects from the amiloride, which was difficult, but at least my BP was down and I was no longer a stroke risk. As hoped, cutting my dose in half, and then half again, diminished my side effects, though didn’t eliminate them. I did need to continue taking Imodium with my medication in the morning to quiet the substantial abdominal cramping I had for several hours every day and again at bedtime to be able to sleep through most of the cramping.

The nephrologist said that it could take 3, possibly 6 months, for my body to adjust to the many changes that the drug triggers, though that was not a promise. We crossed our fingers and hoped that I would again feel like the person I used to be.

On the heels of my triumph came fear, fear of abruptly being hospitalized with COVID-19 or something else and hearing a doctor in the background say “Double her dose!” I feared that if I wasn’t able to advocate for myself from a hospital bed that my HBP would be treated with the standard antihypertensives, which don’t improve my HBP and make me ill. The standard response, like from my first internist, is to double the dose, then double it again, where upon I would get sicker and sicker.

It was fruitful panic: in about 24 hours I’d selected an attractive medical alert bracelet on which I could have my medication and dose engraved. I’m not a jewelry person, but I was delighted with the thought of having this particular piece: it would protect my health in an emergency and be a pleasing reminder of my victory after 2 miserable years of humiliation and struggle.
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A visible victory symbol for me & medication guidance to others should I be hospitalized.

I would like to believe that with sharing my difficult journey in bringing my hypertension under control with an acceptable level of side-effects, I have emboldened others to keep pressing for a better treatment plan for themselves. Bullying of hypertensive patients is a known phenomena, one that I believe arises because of the limited number of strategies that physicians have available to them and because, by some estimates, about half of the patients cannot tolerate their prescribed medications. Regrettably, a culture of blaming the patient has arisen. If you have an unsupportive care provider, do what you can to find someone who will be an advocate for you instead of being a demoralizing, erosive influence.

My Liddle Syndrome is quite rare, an unlikely outcome from my journey, but it must be under diagnosed given how hard it was for me to obtain this clarity. My mutation is labeled a ‘variant,’ in part because I didn’t have the classic presentation of HBP in childhood and because my lab values for aldosterone and renin weren’t as low as expected for the Syndrome. But in further reading about this mutation, it is clear that many with the condition are variants. In one family of 5, each member’s condition presented differently. If your hypertension does not respond to the usual treatments, consider asking your prescriber for the diuretic amiloride. My HBP decisively responded in days to this old drug. He or she can evaluate if there would be any harm in giving this long shot treatment a try, a treatment that can be diagnostic.

Out-of-Pocket Costs of My 2 Year Treatment Journey: $21,500
Direct Costs: $6300
..seen by 5 MD’s: 2 internists, 2 nephrologists, 1 cardiologist
..consulted with 4 naturopaths
..2 treatment sessions with a hypnotist
..35 acupuncture appts with 5 different practitioners + Chinese herbs on prescription medications
..2 devices

Indirect Expenses (triggered by medication side-effects): $15,200
..4 electric bikes; a pair in 2018 and a more suitable pair in 2019
..shipping our custom touring bikes from Italy to home in 2018