New Doctors, More Testing, and Surgery

Back in April, I finally got an appointment with the neurologist here in my town after waiting over a year for my new patient appointment. Although I had a neurologist up in Denver in the interim, he was simply just an okay doctor in my opinion – while he supported my theory of a POTS diagnosis (which I ultimately received last summer), he kept referring everything to my cardiologist and didn’t really seem that interested in doing his own investigation of my symptoms. He was also an hour’s drive away when there was little to no traffic, so I was obviously excited to finally have a specialist here in town. Immediately after meeting with the new doctor, I could see why there was such a long wait to get into the practice – she was fabulous!

After reviewing my case in detail, she was horrified that no one had done an MRI of my head as of yet – Thank you! That’s what I’ve been saying – especially considering the number of head injuries I’ve had back when I was cheerleading. She also ordered another Electroencephalogram (EEG) since evidently my first EEG was only reviewed by the technician who performed it and was never signed off by my neurologist, which was a lovely finding I might add. Thankfully, my EEG came back as being normal again, confirming that I was NOT having temporal lobe or partial seizures as both neurologists had suspected based on my primary neurological symptoms of migraines, cognitive impairment, visual aura, and olfactory hallucinations. Basically, the negative EEG results confirmed that I was suffering from Status Migrainous or intractable chronic migraines as originally suspected. Although I had started taking gabapentin after my first appointment with the neurologist, which seemed to provide a little bit of relief of my symptoms, the doctor decided to also start Botox Injections since the migraines were confirmed to hopefully get them under control.

I had my first injections back on May 4th and so far they have really helped. Although my migraines have not completely disappeared as of yet, they have been less intense and a lot less frequent than they were prior to the injections. However, they do say it can take a few rounds before they will know if it will work effectively, so I’m hopeful that they will only continue to improve after my next appointment with neurology in August. Nevertheless, I was much more anxious about what the MRI would [or would not] show than the results of the EEG.

To my surprise, the MRI showed that my brain was fine. There were no brain lesions, tumors, growths, signs of stroke, etc.,  which was really good news. The rest of my head, though, not so much…

MRI of the Head/Brain W/O Contrast

FINDINGS:

The brain parenchyma is unremarkable. The diffusion weighted images demonstrate no evidence of recent infarct.No evidence of hydrocephalus. The pituitary gland, pineal gland, and corpus callosum regions are normal in appearance. There is no intracranial hemorrhage. No extra-axial fluid collections are present. The orbits, cavernous, and para cavernous regions are unremarkable.

The paranasal sinuses demonstrate complete opacification of the right
maxillary antrum, with maxillary sinus wall thickening, suggesting chronic sinusitis. There is a rim of T1 shortening and associated susceptibility within the mucosal thickening, which could represent inspissated mucus or possibly allergic fungal disease. There is no associated expansion or erosive change identified to suggest mucocele. There is a retention cyst within the right sphenoid and likely along the roof of the left sphenoid. Mild mucosal thickening within the left maxillary antrum.

The calvarium, skull base, and craniocervical junction are preserved. Normal vascular flow-voids are identified.

Impression: 

  1. Unremarkable MR appearance of the brain without contrast.
  2. Paranasal sinus disease as described, including complete opacification of the right maxillary antrum, with suggestion of chronic maxillary sinusitis. Ring of T1 shortening and susceptibility within the right maxillary antrum could represent inspissated mucus or possibly allergic fungal disease. 

So what does all this mean? According to the neurologist, it means that I needed a quick referral to an ENT for evaluation. Great – another new doctor.


At the same time all this was going on, I also had two other MRIs  (aside from the one on my brain)  to evaluate the acute pain I suddenly was having on my right side that further radiated down into my pelvic region. Although the first MRI was supposed to be on both my abdomen & pelvis, it only was approved as an abdominal MRI. Not surprisingly, since it didn’t show anything other than the vascular compressions seen on my earlier CT scans, they ended up having to order another MRI of my Pelvis a few weeks later. Thankfully, given the amount of time that had passed between tests, the concern about appendicitis had pretty much been ruled out.

MRI of the Pelvis W/ and W/O Contrast

Findings:

Pelvis Mesentery: Small quantity of free fluid within the posterior dependent pelvis, primarily within the rectouterine pouch of Douglas with trace fluid also adjacent to the lower aspect of the uterine fundus.

GI:  Small tubular structure originating from the base of the cecum, just below the terminal ileum, with location and appearance most consistent with the appendix, normal in appearance and size (for instance image 41 series 6). No abnormal
surrounding T2 signal and no abnormal contrast enhancement about the terminal ileum or appendix region. Normal appearance of the terminal ileum (for instance image 37 through 39 series 6).

Uterus: Uterus is normal in size and appearance, with normal anteflexed uterine
fundus. Cervix is normal in appearance by MRI evaluation.

Adnexa: There is a 1.8 cm peripherally enhancing cyst in the right ovary, with mild heterogeneous internal T2 appearance, with normal internal T1-weighted isoechoic appearance. Several small follicles in bilateral adnexal regions.

Impression:

  1. Small 1.8 cm peripherally enhancing cyst in the right ovary. Small quantity of free intrapelvic free fluid. Right ovarian cyst may represent a previously ruptured right follicular cyst. Per current radiology criteria no further specific follow-up for this right ovarian cyst is required.
  2. No MRI findings to indicate appendiceal pathology, including no abnormal edema or enhancement surrounding the cecum or appendix.

Although it states in the report that no further up was needed for the ovarian cyst according to radiological criteria, my gastroenterologist disagreed and, therefore, referred me to my gynecologist for further evaluation.


At first, my gynecologists wasn’t extremely concerned about the findings of the report. However, there was some concern was that the cyst did have a heterogeneous appearance, although this could mean anything from being benign growth all the way to cancer. He sent me for a follow-up transvaginal ultrasound, which he said was the preferred method for evaluating ovarian cysts, just to be safe.

US Pelvic Transvaginal

Findings: 

The myometrium is heterogeneous but no distinct mass. The endometrial stripe is within a normal range for age. There is a small amount of free fluid. Both ovaries are well-visualized. In the right ovary, there is a partially collapsed but otherwise simple appearing cyst in the right ovary, which on my repeat measurement is 17 x 9 x 15 mm in size. The larger area measured by the technologist is not well-defined and likely includes normal variant parenchyma. Otherwise, there are simple follicles

Both ovaries are well-visualized. In the right ovary, there is a partially collapsed but otherwise simple appearing cyst in the right ovary, which on my repeat measurement is 17 x 9 x 15 mm in size. The larger area measured by the technologist is not well-defined and likely includes normal variant parenchyma. Otherwise, there are simple follicles under 1 cm in size in both ovaries.

Impression:

  1.  17-mm partially collapsed but otherwise benign-appearing right ovarian follicle.

The good news was that the cyst itself was benign. The bad news, however, was that the cyst had virtually stayed the same size during the month and a half between testing, despite being collapsed in both scans. Basically, the cyst seemed to be refilling itself over and over with fluid (likely blood), thus explaining the free fluid throughout my pelvis that should not have been there.

The doctor gave me a shot of Depo-Provera, which contains the hormone progestin, in hopes of getting rid of the cyst since it likely wasn’t going to go away naturally on its own. If the shot didn’t help to get rid of the cyst in 6 weeks, the doctor informed me that it would have to be surgically removed. Great

Lucky for me, however, the Depo did its job and the cyst was virtually gone during the next transvaginal ultrasound.


Just as one surgery was crossed off my list, another one was added. After I met with my new ENT about the results of my head MRI, he ordered a CT of the head to confirm the extent of the damage marked in my first scan. As expected, it only accentuated the fact that I would need surgical intervention on my sinus cavity.

CT Sinus Complete

Findings:

The sinuses are normally developed bilaterally.

The frontal sinuses are normally aerated. Minimal thickening cannot be excluded in the left ethmoid sinus. Right ethmoid sinus is normally aerated.

There is marked diffuse opacification of the right maxillary sinus, consistent with marked chronic inflammatory disease, and associated thickening of the inferior and lateral maxillary sinus bony wall. Small areas of probable calcification representing debris are noted within the chronic inflammatory tissue.

Minimal thickening is noted involving the left maxillary sinus.

The right ostiorneata complex is obstructed. The left ostiomeatal complex is patent.

Mild thickening is noted in the right component of the septated sphenoid sinus .

There is very minimal undulation of the nasal septum, but no significant deviation.

I just had endoscopic sinus surgery this past Friday, which including a septoplasty, turbinate reduction, antrostomy, and sinusplasty. So far, everything has been going good, except that the recovery has been much harder than I had originally expected. I’ll go over all the fun details, with pictures, hopefully in my next update.

Although all of this may seem like a lot, I still haven’t gotten to the best part – A new vascular surgeon and substantially larger surgery here in the near future – but that is definitely a story I’ll have to share another day.

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I swear I knew it all along…

After my venogram in December and the fiasco at my follow-up appointment to discuss surgery, I had pretty much given up hope that A) we had finalized a diagnosis and B) that I would ever get better. Unfortunately, my next round of follow-ups with the specialists weren’t much better.

By the time my follow-up with gastroenterology had rolled around at the end of January, I knew there really wasn’t much left to do as every GI test had been run up to this point and I was expecting to be let go after a full year of testing with really no results. As I’m sitting there in her office, explaining how once again I won’t be having surgery as expected, she asks me why they offered me the one surgery in the first place if they knew it wouldn’t help. I honestly have no idea what to tell her – Why don’t you ask them?  – I don’t dare say that out loud, though.I look over to see my husband is once again fidgeting in his chair, I can tell he’s getting angsty. However, I wasn’t anticipating what happened next.

In almost slow motion, I watched him mouth the words …because nobody takes her pain f***ing seriously….one syllable at a time. He didn’t just say that out loud, did he? The doctor immediately asks him to leave. Okay, so I guess he really did say that out loud after all. I can tell my doctor was not amused as my husband  gets up from his chair and storms out, and I just sit there, on the exam table, in shock. Now, what? I wait for her to say something but I’m having a hard time holding back the tears in embarrassment and frustration. Really, though, my husband was right – no one does take my pain seriously and I’m becoming more and more tired each day. I really just want to go home at this point.

But somehow, the torture isn’t over for today. She says she wants me to have an anorectal manometry done to rule out pelvic floor dyssynergia for constipation that I have on and off. This is a joke, right? Nope, guess not. She thinks it’s in my head…. that maybe somehow I forgot how to have a bowel movement… I think she is far off with diagnosis. She thinks I should do it anyway. I agree… knowing I”ll prove her wrong… but finally able to leave. I’m not sure how this could get any worse, really… but somehow it always does.


WHAT IS ANORECTAL MANOMETRY?

(Retrieved from the Motility Society Website)

Anorectal manometry is a test performed to evaluate patients with constipation or fecal incontinence. This test measures the pressures of the anal sphincter muscles, the sensation in the rectum, and the neural reflexes that are needed for normal bowel movements.

PREPARATION

Give yourself one or two Fleet® enemas 2 hours prior to your study. You can purchase the Fleet enema from a pharmacy or supermarket. You should not eat anything during the two hours prior to the procedure. If you are diabetic, this may involve adjusting your diabetic medications. You may take regular medications with small sips of water at least 2 hours prior to the study.

THE PROCEDURE

The test takes approximately 30 minutes. You will be asked to change into a hospital gown. A technician or nurse will explain the procedure to you, take a brief health history, and answer any questions you may have. The patient then lies on his or her left side. A small, flexible tube, about the size of a thermometer, with a balloon at the end is inserted into the rectum. The catheter is connected to a machine that measures the pressure. During the test, the small balloon attached to the catheter may be inflated in the rectum to assess the normal reflex pathways. The nurse or technician may also ask the person to squeeze, relax, and push at various times. The anal sphincter muscle pressures are measured during each of these maneuvers. To squeeze, the patient tightens the sphincter muscles as if trying to prevent anything from coming out. To push or bear down, the patient strains down as if trying to have a bowel movement. Two other tests may be done: first, an anal sphincter electromyography (EMG), a test to evaluate the nerve supply to the anal muscle; second, measurement of the time it takes to expel a balloon from the rectum. After the examination, you may drive yourself home and go about your normal activities.

Anal Sphincter EMG

Anal sphincter electromyography (EMG) is recorded with a small plug electrode placed in the anal canal. The patient then is asked to relax, squeeze and push at different times. The anal sphincter muscle electrical activity is recorded and displayed on a computer screen. Anal sphincter EMG confirms the proper muscle contractions during squeezing and muscle relaxation during pushing. In people who paradoxically contract the sphincter and pelvic floor muscles, the tracing of electrical activity increases, instead of decreasing, during bearing down to simulate a bowel movement (defecation). Normal anal EMG activity with low anal squeeze pressures on manometry may indicate a torn sphincter muscle that could be repaired.

Balloon Expulsion Test

For this procedure, a small balloon is inserted into the rectum and then inflated with water. The patient goes to the bathroom and tries to defecate (expel) the small balloon from the rectum. The amount of time it takes to expel the balloon is recorded. Prolonged balloon expulsion suggests a dysfunction in the anorectum area. What can be learned from anorectal manometry? The anal and

WHAT CAN BE LEARNED FROM ANORECTAL MONOMETRY?

The anal and rectal area contains specialized muscles that are helpful to regulate proper passage of bowel movements. Normally, when stool enters the rectum, the anal sphincter muscle tightens to prevent passage of stool at an inconvenient time. If this muscle is weak or does not contract in a timely way, incontinence (leakage of stool) may occur. Normally, when a person pushes or bears down to have a bowel movement, the anal sphincter muscles relax. This will cause the pressures to decrease allowing evacuation of stool. If the sphincter muscles tighten when pushing, this could contribute to constipation. Anal manometry measures how strong the sphincter muscles are and whether they relax as they should during passing a stool. It provides helpful information to the doctor in treating patients with fecal incontinence or severe constipation. There are many causes of fecal incontinence. Weak anal sphincter muscles or poor sensation in the rectum can contribute to fecal incontinence. If these abnormalities are present, they can be treated. Biofeedback techniques using anal manometry and special exercises of the pelvic floor muscles can strengthen the muscles and improve sensation. This can help treat fecal incontinence. There are many causes of constipation. Some involve sluggish movement through the whole colon, whereas others involve the anal sphincter muscles. In some patients with constipation, the anal sphincter muscles do not relax appropriately when bearing down or pushing to have a bowel movement. This abnormal muscle function may cause a functional type of obstruction. Muscles that do not relax with bearing down can be retrained with biofeedback techniques using anal manometry.

Risks of Anorectal Manometry

Anorectal manometry is a safe, low risk procedure and is unlikely to cause any pain. Complications are rare: it is possible that a perforation (tearing) or bleeding of the rectum could occur. Equipment failure is a remote possibility. If you are allergic to latex, you should inform the nurse/technician before the test so that a latex free balloon can be used.


Let’s just say I was less than thrilled to have to do this test. Just the idea of having to take two fleet enemas and administer them on my own was enough to get me to freak out. Not to mention, since the test was over an hour and a half drives away, they told me to do it the night before. I was still petrified that I would have some embarrassing accident for sure. Not only did the cleansing make me super sick and dizzy, passing out every few minutes, but also had a lot of pain and bleeding afterward as well. It was almost as bad as the colonoscopy prep, only luckily I didn’t have to drink anything. Up until five minutes before leaving, I was still passing a good amount of blood. Nevertheless, I went to my scheduled appointment anyway.

Luckily, my nurse was very nice and calmed me down as I was very tense going into this test. Still, I was in a lot of pain and worried I’d embarrass myself so I was quite reserved. Along with using a balloon they also did the sponge test to see if I could “pass it”. Let me tell you, it felt like I was trying to pass sandpaper. It was horrible and I was in tears. The nurse tells me everything was within normal limits but a doctor would double-check my results to be sure. I figured my test would be normal anyways and I was just happy I was able to go home to recover.

When my follow-up with GI came around in March, I was far from prepared when the doctor told me that my manometry was positive and that I did, in fact, have pelvic floor dyssynergia. Excuse me, what? She’s sending me biofeedback therapy twice a week. Great, I think, just what I have time for. I honestly don’t know how this is possible based on my symptoms, but I schedule therapy anyway.

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After weeks of going to biofeedback therapy twice a week, my symptoms were anything but better. In fact, the extra added stress of trying to make it to therapy twice a week, on top of everything else, likely made everything worse. It was actual torture and I was starting to feel like this was punishment for my husband disrupting the appointment. The therapist also focused more on relaxing my vaginal muscles more than relaxing anything else, which I found to be useless in all honesty. How was this teaching me how not to be constipated, again? They kept asking me if I was eating – of course I’m not eating, that’s what causes the pain, but nobody listens to me. The honest truth, each and everyone one of my muscle in every area of my body  hurts. They are tense because I am always in intense pain every single day. I’m getting angry. I ask for a copy of my test results because I honestly don’t understand how I have this condition in the first place and all the results in biofeedback therapy are coming back as normal.

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When the report finally comes in, my test was borderline at very best. I knew it! I likely didn’t have completely “normal” results because I was in pain while “pushing” and now I’m really angry that I have wasted my time.

Within a few hours of reading the test results, I get a message from the radiologist that I had sent my GI scans to a few weeks back to review for me. I  had asked him to look at them for me since every doctor kept denying that I had superior mesenteric artery syndrome when the reports kept saying I had it, or the doctor would say I have the right angle for the condition but because I didn’t have dilation in my other GI scans, I couldn’t have it. Sure enough, the radiologist had confirmed not only the angle and the aortomesenteric distance, but the dilation of my duodenum was missed in three separate scans.

According to Karrer et al. (2015), the “CT criteria for the diagnosis of superior mesenteric artery syndrome include an aortomesenteric angle of less than 22 degrees and an aortomesenteric distance of less than 8-10 mm” (para. 1). My aortomesenteric angle was 17 degrees and my aortomesenteric angle was 4mm.

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When I went to my next biofeedback appointment, I share the diagnostic news with my therapist. She asks me if biofeedback has helped at all, which I of course reply that it hasn’t. She feels like the diagnosis is incorrect as well and decides to dismiss me from therapy. I have yet to follow-up with GI doctor but I have an appointment scheduled with her in a couple of weeks. Not sure how this is going to go, but I think this will be the last time that I see her – officially. Things were missed in my testing that was super important and  I could have been treated over a year ago when the testing first mentioned the findings of all these conditions. Again, if only these doctors would have listened to me or checked the scans themselves. My husband was right, despite his behavior that day. On top of it all, I was diagnosed with a condition that I feel was more of a punishment than something I believe the doctor would honestly never think that I have since I do not have 99% of the symptoms of pelvic floor dyssynergia.Needless to say, it’s going to be interesting to an interesting follow-up. Maybe I’ll bring my husband with me one more time…

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References:

Karrer et al. (2015). Superior Mesenteric Artery Syndrome Workup. Retrieved from http://emedicine.medscape.com/article/932220-workup

Motility Society (2005). Patient Information on Anorectal Monometry. Retrieved from http://www.motilitysociety.org/patient/pdf/Anorectal%20Manometry%20Patient%20Information%208%205%202005.pdf

 

Viper Venom Blood Testing

I’ve had a few people ask me about this test today after I posted about it in my list of blood tests that I’ve had in the last year, so I figured I’d share some more information about it.

For starters,  the test is officially known as the Dilute Russell’s Viper Venom Time (DRVVT). While it’s not a rare test per say, it’s not generally  ordered as a part of routine blood work . Typically, a doctor will order this if they are looking for specific markers relating to certain blood clotting disorders, such as those in Antiphospholipid Syndrome (APS). Still, when I called for my test results the nurse who read my result had never even heard of this test and had no idea what the result meant – other than it was flagged as being abnormal (more about that later). Nevertheless, the test itself is really interesting (and slightly confusing as well).

Although my test was done in the laboratory, this video will give you a good demonstration of how and why viper venom is used to test the coagulation times of human blood.

*Trigger Warning*

If you have a phobia of snakes – DO NOT watch the following video

Pretty amazing, huh?

Also kind of scary to think about what happens to the body when an individual is bitten by such a venomous snake. Luckily, though, that wasn’t the case here.

About the Test:

According to the Practical Haemostasis Website, the “Russell’s viper venom [RVV] isolated from the snake Daboia russelii [orrussellii – contains a potent activator of factor X which in the presence of phospholipid [PL], prothrombin and calcium ions clots fibrinogen to fibrin. In individuals with a lupus anticoagulant [LA] the antibody binds to the phospholipid inhibiting the action of the RVV and prolonging the clotting time.
As the RVV directly activates factor X, the test is unaffected by deficiencies of factors XII, XI, IX or VIII. The DRVVT is frequently combined with a platelet neutralisation procedure to demonstrate the phospholipid specificity of the antibody” (para. 1).

One thing to note here is that the Lupus anticoagulant does NOT test for lupus  – although the test may be positive for some individuals if they have both Lupus AND APS. It’s merely an immunoglobulin that is associated with an increased clotting time.

How It’s Used:

From The American Association for Clinical Chemistry

Lupus anticoagulant testing is a series of tests used to detect lupus anticoagulant (LA) in the blood. LA is an autoantibodyassociated with excess blood clot formation. LA testing may be used to help determine the cause of:

  • An unexplained blood clot (thrombosis) in a vein or artery
  • Recurrent miscarriages
  • An unexplained prolonged PTT test (PTT measures the time it takes in seconds for a person’s blood sample to clot in a test tube after reagents are added.) LA testing helps determine whether a prolonged PTT is due to a specific inhibitor, such as an antibody against a specific coagulation factor, or to a nonspecific inhibitor like the lupus anticoagulant.

LA testing may also be used:

  • Along with tests for cardiolipin antibody and anti-beta2-glycoprotein I to diagnose antiphospholipid syndrome (APS)
  • Along with tests such as factor V Leiden or proteins C and S to help diagnose an excessive clotting disorder (thrombophilia)
  • To determine whether the lupus anticoagulant is temporary (transient) or persistent

LA cannot be measured directly and there is no single test or standardized procedure to detect the presence of LA in the blood. A series of tests is used to confirm or rule out the autoantibody:

  • It is recommended that two tests be used to detect lupus anticoagulant. The most sensitive tests are dilute Russell viper venom test (DRVVT) and a LA-sensitive PTT (PTT-LA), one that uses low levels of phospholipid reagents. Follow-up testing is performed to confirm or exclude the presence of lupus anticoagulant. These may include:

  • Mixing study: an equal volume of patient plasma is mixed with “normal” pooled plasma and a PTT or DRVVT is performed on this mixture.

  • Correction/neutralization: an excess of phospholipids is added to the patient sample and a PTT-LA or DRVVT is performed. (When PTT-LA is measured, the assay is called a hexagonal phase phospholipid neutralization assay).

The Laboratory Method:

From Practical Haemostasis

Pooled normal plasma is mixed with diluted PL at 37°C. Diluted RVV and then calcium chloride are added and the clotting time is measured. The test is then repeated using patient plasma and the ratio of test:normal plasma is calculated.

Reagent Explanation
Platelet poor Plasma A source of coagulation factors, particularly thrombin and fibrinogen.
Dilute Russell’s Viper venom Typically this is diluted to give a clotting time in normal plasma of between 30-35s as this optimises sensitivity to antiphospholipid antibodies.
Phospholipid To provide a surface for thrombin generation. This should be diluted to a sufficient degree that it becomes the rate limiting step and any inhibition by a LA prolongs coagulation.
Calcium To initiate coagulation

My Test Results:

DVRTT

Although my test results were out of the normal limits, it is not considered a positive test because my result would need to be higher than normal range (slower clotting time) and mine was below the average (faster than the normal clotting time).  Make sense? No idea if a low score has any clinical significance – haven’t found anything that even mentions it.

Below are some explanations about the meaning of the results and how to determine a positive test.


Result Interpretation:

From The American Association for Clinical Chemistry

The results of the series of LA tests either lead toward or away from the likelihood of having LA. The laboratory report may be somewhat complicated, but it usually provides an interpretation of the results and states whether LA is present or absent. LA testing results, like those of other tests for clotting disorders, are difficult to interpret and are best evaluated by physicians with experience with excessive clotting disorders.

Although the initial tests performed for LA may vary, they usually begin with a PTT that is prolonged. A PTT that is normal (not prolonged) may mean that there is no LA present. However, the test may not be sensitive enough to detect LA and the LA-sensitive PTT (PTT-LA) may need to be done.

Additional details on LA testing results

Lupus anticoagulant testing is often done in conjunction with tests for cardiolipin antibody and anti-beta2-glycoprotein I antibodies to help diagnose antiphospholipid syndrome. The results are interpreted together, along with clinical criteria, in order to make a diagnosis.

Results of other tests that may be performed to help rule out other causes of a prolonged PTT include:

  • If a thrombin time test is normal, then heparin contamination is excluded as a cause of prolonged PTT.
  • If a fibrinogen test is normal, then it is likely that there is sufficient fibrinogen for clot formation.

Other tests that may be done to help confirm the diagnosis of a lupus anticoagulant include:

  • Coagulation factor assays – these may be ordered to rule out factor deficiencies that may cause a prolonged PTT and bleeding episodes; a panel of factor assays may also help in detecting lupus anticoagulant.
  • Complete blood count (CBC) – the CBC test includes a platelet count; mild to moderate thrombocytopenia (low platelet count) is often seen along with the lupus anticoagulant; moderate to severe thrombocytopenia may develop in patients receiving anticoagulant (heparin) therapy for lupus anticoagulant-associated thrombosis.
  • Tissue thromboplastin inhibition test (rarely performed nowadays)

Clinical Explanation:

From Practical Haemostasis

If the DRVVT is not prolonged then the correction test (sometimes referred to as the neutralisation test) is not indicated.

DRVVT Interpretation
DRVVT
Ratio [Test DRVVT/control DRVVT]
Reference Range: 29-42s
Reference Ratio: 0.9-1.05
Ratio [Test DRVVT/control DRVVT] >1.05 Possible LA
Exclude deficiencies of factors II, V, X, fibrinogen or another non-LA inhibitor
Prolonged DRVVT which corrects with normal plasma Clotting factor deficiency
[A weak LA can sometimes be masked in a 1:1 mix with normal plasma and some labs recommend a 1:4 mix [Normal plasma:Test plasma] to try and minimise this]
Prolonged DRVVT which corrects with PL Lupus anticoagulant

Calculating the percentage correction:

1. The percentage correction is calculated using the formula below where ‘+ PL’ is the DRVVT with additional phospholipid i.e. the neutralisation step.

2. The percentage correction of the ratio is calculated using the formula below where again ‘+ PL’ is the DRVVT with additional phospholipid. The final result is multiplied by 100 to convert it into a percentage:

This latter calculation is that currently recommended by the British Committee for Standardisation in Haematology [BCSH.]

In the following example:

Sample dRVVT Clotting Time [s] Ratios
Patient Plasma 69.2 s [Patient Plasma]/[Reference Plasma] = 1.82
Reference Control [Normal] Plasma 37.9 s
Patient Plasma + Phospholipid 39.5 s [Patient Plasma + Phospholipid]/Reference Control [Normal] Plasma +  Phospholipid] = 1.21
Reference Control [Normal] Plasma +  Phospholipid 32.5 s

From the data above: [1.82 – 1.21/1.82] x 100 = 33.5% correction. So in a patient in whom the dRVVT is prolonged as in this case [69.2s] the 33.5% correction is consistent with the presence of a lupus anticoagulant.

3. What constitutes a positive correction ratio? Most laboratories regard a correction of >10% with PL as being a positive test.

In individuals in whom a LA is identified, the test should be repeated in 12 weeks. It should also be remembered that not all tests including the DRVVT will identify all LAs and therefore, if the index of suspicion that a specific patient has a LA then other tests should be undertaken e.g. Silica Clotting Time [SCT]. Finally – the causes of a LA should be screened for e.g. ANA, drugs, viruses etc.


References: 

American Association for Clinical Chemistry (2014). Lupus Anticoagulant Testing. Retrieved from https://labtestsonline.org/understanding/analytes/lupus-anticoagulant/tab/test/

Practical Haemostasis  (2016). Dilute Russell’s Viper Venom Time [DRVVT]. Retrieved on June 6, 2016, from http://www.practical-haemostasis.com/Thrombophilia%20Tests/APS/drvvt.html

Sports One Media (2012, July 12). Viper Venonom Turns Blood Into Jelly [Video File]. Retrieved from https://www.youtube.com/watch?v=PwT8vDzjCSQ&list=PLXGuqM-FQFlipPDC8hcLvG7CQ2F8divMo&oref=https%3A%2F%2Fwww.youtube.com%2Fwatch%3Fv%3DPwT8vDzjCSQ%26list%3DPLXGuqM-FQFlipPDC8hcLvG7CQ2F8divMo&has_verified=1

A Review of my Medical Journey From the Last Year

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Since restarting my search for a diagnosis in February of last year when I started at the research hospital, I’ve been through a lot of doctors and a lot of medical testing.

Well, it got me thinking –  Just how many tests have I done?

 Being as OCD as I am, I decided to look through my list of notes and test results so that I could write it all down (in list form of course). Eventually, I’m hoping to upload some of my test results – along with an UPDATED diagnoses list – for comparison.


Doctors I’ve Visited

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Primary Care Doctors:

  1. PCP-A
    • Scarred and butchered my scalp biopsy (with no anesthetic, by the way), forgot to order tests, couldn’t fill out paperwork, the office staff was horrible, the list goes on and on…
  2. PCP-B
    • Told me she’d review my record and call me with a plan- never called after multiple attempts over 2 months. I went as far as to file a complaint with the office manager, who promised me at least a call, which never happened.
  3. PCP-C
    • Told me I was simply dehydrated and depressed, despite having initial diagnoses at the time.
  4. PCP-D
    • FINALLY – a good PCP and my current doctor.

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Medical Specialties:

  1. Vascular Surgeon #1
  2. Vascular Surgeon #2
  3. Vascular Surgeon #3
  4. Neurology #1
  5. Neurology #2
  6. Dermatology #1
  7. Dermatology #2
  8. Cardiologist
  9. Electro-cardiologist
  10. Immunologist
  11. Rheumatologist
  12. ENT/Allergy
  13. Gastroenterologist
  14. GI surgeon
  15. Urologist
  16. Gynecologist #1
  17. OGynecologists #2
  18. Physical Therapist
  19. Optometrist
  20. Psychologist
  21. Neuropsychologist
  22. Chiropractor
  23. LCSW
  24. Nutritionist

Procedures (Under Sedation)

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  • April 2015 – Esophageal Dilation and Internal Biopsies
  • May 2015 – Endometrial Ablation
  • December 2015 – Venography

Medical Testing and Imaging

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2015:

March

  • CT Abdomen & Pelvis
  • Pulmonary Function Tests – Pre & Post
  • Spirometry
  • Allergy Test – Skin Scraping

April 

  • Abdominal X-Ray
  • Tailored Barium Swallow
  • Colonoscopy & Endoscopy
  • X-Rays of both Ankles

May

  • Abdominal Ultrasound RUQ
  • Gastric Emptying Study

June

  • CT Angiogram Abdomen & Pelvis
  • Electroencephalogram (EEG)
  • Spirometry
  • Fecal Cultures

July

  • Electrocardiogram (ECG)
  • Echocardiogram with Agitated Saline

August

  • Skin Biopsy
  • Cardiac MRI
  • Exercise Stress Test
  • Holter Monitor
  • Spirometry

September

  • X-Ray of Spine

October

  • Sitz Marker Study
  • Upper GI Series
  • Small Bowel Follow Through
  • Hydrogen-Methane Breath Test
  • Spirometry
  • Abdominal X-Ray Supine

November

  • Hepatobiliary (HIDA) Scan
  • Electrocardiogram (ECG)
  • Holter Monitor
  • Echocardiogram

December

  • Venography

2016:

January

  • Renal Ultrasound

February

  • Anorectal Manometry
  • MRI Abdomen

March

  • Tilt-Table Test
  • Electrocardiogram (ECG)

April

  • MRI Pelvis
  • MRI Head/Brain

May 

  • Transvaginal Ultrasound
  • Renal Ultrasound
  • Electroencephalogram (EEG)

June

  • Transvaginal Ultrasound
  • CT Maxillofacial

Blood and Urine Screening

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  • Urinalysis and Urine Cultures
  • Routine Blood Screens:
    • Comprehensive Metabolic Panel (CMP)w/ and w/o eGFFR
    • Complete Blood Count (CBC) w/ and w/o Differentials
    • Electrolyte Panel
    • Liver Panel
    • Kidney Function Tests
    • Celiac Disease Panel
    • Thyroid Screens
      • TSH
      • TSH 3rd generation
      • T4
    • Electrolyte Panel
    • Lipid Profile
    • Cholesterol Levels
    • Serum Vitamin Assays
      • Vitamin D
        • Total
        • D3
        • D 25-Hydroxy
      • Vitamin B12
      • Folate/Folic Acid
      • Calcium
    • C-Reactive Protein
    • Antinuclear Antibody Panel (ANA)
    • Serum Iron Testing
      • Iron and Iron Binding Capacity
    • Rheumatoid Factor
    • Creatinine with eGFR
    • Erythrocyte Sedimentation Rate (ESR)
    • Amylase
    • L-Creatinine
  • More Specialized Blood Screening:
    • Antibody Screening
      • IGA
      • IGE
      • IGM
      • IGG
      • CK
      • CS
      • Tissue Transglutam AB IGA
      • IGE-RAB, Chronic Uticaria
      • IGE Receptor Ab
      • DNA AB (DS)
      • anti-RO/SSA, and Anti-LaSSB
      • CCP Antibodies
      • Beta-2 glycoprotein IgGG and IgM
      • Cardiolipin
    • Sjorgen’s SSA
    • B. burgdorferi Screen Rflx
      •  B. burgdorferi Ab, IgG,IgM
      • B. burgdorferi Ab, IgG (WB)
      • B. burgdorferi Ab, IGM (WB)
    • Growth Hormone Somatotropin Test
    • Cortisol (AM)
    • Blood Clotting Tests
      • Prothrombin Time (PT)
      • International Normalised Ratio (INR)
      • Dilute Russell’s Viper Venom Time (dRVVT)
    • L-Protein Electrophoresis
    • Nutritional Panel w/ Prealbumin
    • Blood Typing

Life After Venography

Recovery from the venogram was much worse than I expected. Originally, they told me I’d be in pain for the next 48 hours and then I’d be fine, but put me on restrictions for 7 days. I guess I didn’t realize how much you actually use your neck, as I could barely move at all for the entire week following. All I could do was sleep. The day after the procedure, I started coughing up blood, as well as blowing it out my nose, and my pelvis hurt more than it had even before the procedure. I also couldn’t see clearly either. It was as if my normal vision (even with glasses) suddenly changed overnight. Worried, I called the hospital, but the on call doctor thought my symptoms were unrelated to the venogram. Just weird they started the morning AFTER the venogram. Not long after I get off the phone with him, the hospital calls back letting me know that I have an appointment for a follow-up in two days (which I didn’t make – I was told to call to schedule). At that point, it was now the weekend, so I called bright and early that Monday morning to reschedule, since I was already scheduled with cardiology (at a different hospital) at the same time.

When I go to the follow-up a week later, the receptionist checking me in tells me that they’ll be calling me shortly for my ultrasound. Confused, I questioned her about it and she said, “Oh, never mind. It is an appointment, but there is no doctor written on the schedule…how odd!Um, okay… that doesn’t sound good, but she doesn’t act concerned so we (my husband and me) take a seat in the waiting room and actually get called back to the exam room rather quickly – I think it was in less than 5 minutes from sitting down. After the nurse does her thing, she says it’ll be just a few minutes until the doctor comes in. After an hour goes by of us waiting in the exam room, the nurse finally comes back in to check up on us, saying that she just realized that it had been just over an hour since we checked in and that the doctor was scheduled for surgery at the time of the appointment… but he should be almost done. About 10 to 15 minutes later, a doctor that looks familiar, but is not MY doctor, walks into the room.

He introduces himself and says he will be helping Dr. J with my surgery and that he assisted with my venogram. Okay, so that’s why he looked familiar. He proceeds to go over the findings of the test by drawing me a picture and telling me, in the most basic way possible, that I have Nutcracker Syndrome. Um, yes, I know. It’s already been confirmed multiple times by multiple doctors. I don’t think he knew anything about me, really. This became more apparent when he then tells me to they want to do surgery to stop my hematuria – only I don’t have hematuria as a symptom and I haven’t since I was in my teens and early 20’s. He seems confused by this and asks me what, exactly, are my symptoms.

I tell him the list (ULQ abdominal pain that can also radiate to the right or pelvic region on both sides, constant nausea, dysphagia, pain after eating, alternating diarrhea/constipation, rectal bleeding with or without mucus or undigested food in stool, dizziness, syncope/pre-syncope, occipital neuralgia, skin rashes, burning sensation under skin, insomnia, sleep apnea, chest pain, asthma/allergies/anaphylaxis, olfactory hallucinations, hair loss, incoordination/balance issues, severe fatigue, heart palpitations, arthralgia and myalgia, muscle and eye twitching, livedo reticularis, nevus anemicus, random swelling of my lymph nodes, and abnormal menstrual cycle before endometrial ablation). I also tell him my current diagnoses aside from the NCS (SMAS, MTS, IBS, Hypoxemia, POTS, Pericardial Effusion, Ventricular and Sinus Tachycardia, Cardiomyopathy, Alopecia, SIBO, and some underlying autoimmune/infiltrative disease that is tricky to diagnose, but the doctors are fighting about it being lupus or not). Maybe it was the number of symptoms/syndromes I named off, I don’t know, but he just kind of brushed it all off since none of it fit with the Nutcracker diagnosis.

Instead, he moved on with the conversation by showing me where all the collaterals are forming (where the body develops new blood circulation pathways to flow through since my renal vein is essentially blocked) and we watch some video from the venogram.

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Photo retrieved from Dr. Scholbach’s Website, 2015

Basically, a lot of my blood is flowing into my lumbar plexus and pelvis, causing the pelvic congestion syndrome.

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Photo retrieved from RIA Endovascular Website, 2015.

The blood that is making it through the renal vein is then going up into the hemiazygous vein.

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The rest of the blood, however, is flowing retrograde toward the IVC, and the doctor says that it’s not likely May-Thurner Syndrome but the retrograde flow instead (although, again, Dr. J said I absolutely had MTS based off my CT Angiography, which we watched together during my consult as well).

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Retrieved from the University of the Cumberlands website, 2015

This doctor then tells me that I have two options to fix the Nutcracker Syndrome; a stent (which I interrupt him and tell him that’s not an option) or the LRV transposition surgery, which they believe, due to my age, would be the best option. I ask him about the SMA Syndrome, which Dr. J said I had based on the CTA as well, but this doctor tells me there’s no way I have that. I tell him that GI surgeon confirmed the angle for SMAS and wrote it down as a diagnosis in my chart, but said he wouldn’t treat it without other proof.

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Retrieved from Dr. Sathaye’s Blog, 2015

Basically, this doctor says that since I have no confirmed blockage they won’t treat it and that they can only treat what they have confirmed – the Nutcracker Syndrome and the Pelvic Congestion Syndrome (they plan to “tie off the vein” where it goes into the pelvis). Great…

We go over the surgery, recovery, etc.  My husband asks him if surgery will treat any of my primary symptoms. He basically stutters, saying there’s no way to know if it’ll help at all, but it may decrease any flank pain, hematuria (which, again, I don’t have) or pelvic pain caused by the PVCS, and that it has to be done. “So none of her primary symptoms?” – “Not likely”. I ask him if this could be causing any of the problems with my heart or the POTS. “Not likely influencing any of that either. I don’t suspect that this surgery will help those problems, but you never know.” We basically end the conversation there.

As the nurse is getting us checked out she says, “For someone with as many symptoms as you, you look really good.”  Excuse me?

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After 20 years of pain and no diagnosis, you learn to hide it pretty well…

 “We have another patient with all the same things as you and she doesn’t look well at all….She is horribly skinny and sick.

Wow.

I didn’t know how to respond to that, so I just said,

“Well I keep my weight up by drinking 1 or 2

high-calorie Starbuck’s drinks a day.

Starbucks?

She doesn’t understand how that’s even possible.

“It’s 500 to 600 calories a drink. Keeps my weight up

and is the reason why I don’t ‘look sick’.”

She’s in shock.

You mean, you don’t eat food?

No… I CAN’T eat food.”

She’s speechless for a moment and then tells me that the doctor’s scheduler will call me to set up a surgery date, before walking us out. Before we left, though, she was nice enough to print out my venogram results so I didn’t have to wait for medical records to send them out by mail.

So, after all of this, I’m at a loss about what I should do.

Primarily, the biggest concern is having a major surgery without fixing my primary complaints first– you know, the ones that have taken away my ability to work and live a normal day-to-day life.

My husband doesn’t want me to have it done because he knows I can’t handle any additional pain on top of what I already have going on (that surgery won’t fix) and he doesn’t think it would be worth it. He also doesn’t trust the hospital, as I do after this whole affair. When the hospital called to schedule a few days later, I told them I would have to call back after I’ve gotten my affairs in order, talked with my other doctors and moved some appointments around, and talked with my school about taking at least one semester off. I also plan to call around to some other doctors out of state to see if they can review my tests, as well as my other doctors, although I obviously didn’t tell them that.

When I called my cardiologist to see if he had any objections or concerns I should be aware of about surgery, he recommended that I wait until my cardiology testing is done because he can’t guarantee I’d be okay with such an intensive surgery or recovery. Still, I need some opinions or input as to how to move forward once I am cleared for surgery. I don’t want to be noncompliant, but this is an invasive surgery and I want to make sure that I don’t do anything to make myself worse or choose the wrong thing. So far, this is one of the biggest medical decisions I’ve had to make.

Nonetheless, I’m losing hope that I’ll ever feel better as it seems to only be getting worse over time, and honestly don’t know what to do about anything anymore. I was so ill last week that I lost 8 pounds in three days from not being able to consume coffee (or food, obviously), but I am running out of tests that can prove the SMA Syndrome in a way that they would accept in order to treat it.

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So what do you think?

Should I move forward with surgery at this location after how they’ve handled everything else?

Is it even worth having the LRV Transposition if it won’t likely help any of my primary symptoms?