Ah yes, the Levine hypothesis. I wanted my son to have an echo done 18 months ago, because a cardiologist had suspected the reason for his POTS symptoms was because he "might have Ehlers-Danlos Syndrome". This was based on the association of orthostatic tachycardia with hypermobility, or Joint Hypermobility Syndrome. My son is very hyperflexible, and has a few of the other conditions that have been associated with hypermobile EDS, (such as soft skin, acid reflux, anxiety, numbness and tingling).
However, I wasn't convinced he had EDS. I felt there could be other explanations for his severe orthostatic hypotension and weakness. He was due for another MRI, with general anesthesia, but when I took him in for a heart exam first, he only got an EKG and orthostatic vitals. Although the pediatric cardio said he had autonomic dysfunction, I needed to get a geneticist to "confirm Ehlers-Danlos" before they would do the echo.
Two months later, the geneticist's report said my son "met the criteria for EDS Hypermobility Type", and ordered the echo. At this same time, I ran across Dr. Levine's article and read it with interest. Although I knew my son was deconditioned from having POTS (or at least orthostatic hypotension) for two years, and that deconditioning was NOT the cause of his illness, the possibility of him having a smaller-than-normal heart size, or decreased left ventricular mass, was intriguing, due to the fact that his body had been deprived of almost all growth hormone for at least five years at that point (because of a pituitary tumor).
I printed out the abstract of Dr. Levine's article, along with another article (unrelated to Dr. Levine's) entitled "The Severity of Growth Hormone Deficiency Correlates with the Severity of Cardiac Impairment in 100 Patients with Hypopituitarism". This other article stated that impaired left ventricular function and reduced cardiac size was a common finding in patients with childhood-onset growth hormone deficiency. (It even said that withdrawal of growth hormone replacement therapy was followed by decreased cardiac size and performance).
I showed both articles to my son's endocrinologist just one month before he started growth hormone therapy, and told him I wanted the echo done asap, to document whether or not his heart was abnormally small before growth hormone. I thought this could be the answer to his extreme orthostatic weakness and tachycardia! I thought if anyone was going to have POTS due to a small heart, it would be my son.
To make a long story short(er), my son got an echo, but only minor pulmonary regurgitation was noted; no mention of any impairment of function, or smaller than usual size. There was also no aortic dilatation seen, or any other cardiac problem associated with Ehlers-Danlos (thank goodness!)
Perhaps if he were to get some dynamic testing, something abnormal might be found; a stress test or other functional testing. Nothing unusual is ever found when he is quietly lying still!
Now we are still having the problem of doctors believing that all my son needs to do is exercise and drink lots of water, eat salt etc. They are still focusing on the deconditioning because it's the only thing they see. Even after three years. Finally six weeks ago, his new neurologist put him on Midodrine, but so far it's not helping.
I don't think Dr. Levine believes deconditioning is the ONLY cause of POTS. He specifically excluded people from his study who had autonomic dysfunction that would *not* respond to exercise training. I can understand people getting upset with a generalization of their condition associating it with a negative character such as the Grinch. This IMO was a poor example for Dr. Levine to choose, mostly because not all, (in fact, very few) POTS patients have an undersized heart. Some don't have hypovolemia either, or only mild cardiac hypofunction.
I was pleased to read the article by Dr. Fogoros, which I found just two days ago. It needed to be written, to give some balance to Dr. Levine's study, a better perspective of the complexity of POTS, and a voice to the need for all dysautonomia patients to be treated with care and respect for the suffering they live with daily. I don't believe the small subset of POTs patients in the study represents the majority with this diagnosis, and it was wrong for Dr. Levine to imply this, to support his hypothesis.
To Dr. Levine,
I am an 18 year old female with POTS and I am appalled at the mere idea of renaming this condition to Grinch Syndrome. It is absolutly disgusting to even think about. I understand that the research is sound and the findings are secure, but I believe adding a Dr Seuss refernece to the name of a condition that is this severe completely undermines the extremes if the disease. The symptoms associated with POTS are not something out of a light hearted childrens book. POTS is a life altering condition that steals youth from the young and makes each day of struggling with symptoms miserable. It is only with a vast amount of medication, an altered lifestyle, and the acceptance that I cannot function anywhere near the normal standard set by my peers that, I can make it through the day. Those three things make miserable days livable. I believe that renaming the syndrome is rude and childish. I struggle to get out of bed some mornings, so knowing I do that and realizing my disease has the word Grinch in it would be a mockery. I am cannot think of anything more disgusting. With all do respect until you've had this happen to you you have no idea what it's like to live with this. So labeling POTS with the name Grinch Syndrome is infuriating. I understand there are much more radical and severe prognosises one can recieve, POTS isn't the end of the world, but it is a war every second of the day to get a broken body to function properly. I hope I do not come across as a complaining child but the idea to rename POTS is rediculous. If anything add some sort of cardiac acronym to the name, but for the dignity of the individuals who have it, and the diseases name itself, do not rename POTS.
I wonder if the point of the renaming was to try to humiliate all with P.O.T.S. in a generalized, broad-brush approach that those who have it have it as a consequence of laziness?
Even as a very active child, I would get short of breath with certain activity for some reason.
I was diagnosed with P.O.T.S. back in 2004 after first being diagnosed with I.S.T.. Years later, I had an echocardiogram which states my left ventricle (which Levine says is reduced in size in his study in P.O.T.S. patients) is normal in size. My own testing in the past with urine revealed a sodium content a different article seemed to indicate would be consistent with hypovolemia.
I admit in recent years, I have become more sedentary. But would a sedentary person's normal-sized heart have its rate jump to 160 even though the head of my bed was elevated, as Dr. Levine mentions regarding the beds in their study?
Would a sedentary life-style cause my heart rate to be over 100 at some point all hours but one (that one likely while sleeping) in a 24 hour period in my most recent holtor monitoring? Would sedentary living cause some blood pressures this year to have pulse pressures so low as to one of those days cause repeated sinking feelings in my chest?
Would just deconditoning cause excessive thirst and excessive urination? Would it make one's vision start to leave from raising one's arm/s above one's head?
Dr. Levine admits they did numerous things other than just exercise, such as raising the head of the bed and increasing daily intake of sodium and water. They also conducted their autonomic function tests after careful regulation of intake of sodium, potassium, calcium, etc. prior to testing, likely not a simulation of real life conditions for these individuals.
This therefore, I believe, is a muddled study, and Dr. Levine's wanting to rename the condition to a fictitional character's name, a character held in contempt at that, is far beneath the dignity of the medical profession. It is disrepectful and degrading to all those who suffer from this life-altering condition called P.O.T.S..
I saw dr levine i had prayed for the day. My dr was a god send and he came highly recommended. I felt so beat up by this illness, betrayed by my body. He was going to be my great shining hope. Cant even begain to describe what my husband and i enduted prior to traveling to see him. I was desperate for his expertise. Lets just say i left crying (not in a good way) was told by someone very close to him, most leave that way. But i still have great drs in my corner that are a blessing and finally i feel more like myself enjoying life!!! Living so make sure you see a caring dr because god knows its what you need