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Maybe call your regular doc and see if you can get a sterile tube to collect fluid in, and see if your doc is able to just order the beta transferrin test with the associated blood draw. It seems like a pretty basic lab, I only went to ENT because I have very complicated headaches and I was looking for more opinions and they happen to be familiar with csf leaks. There are also ways to try to trigger more fluid called valslva maneuvers, usually involving straining or bending forward, but I would only do with a doctor helping.
Typed a reply last night but it never showed up. Weird.
There is a condition fairly recently named called Mast Cell Activation Syndrome or MCAS that you should look in to, and it commonly coexist with POTS and EDS. It is basically when mast cells that the immune system stores pre-made chemicals in get triggered to dump their contents (degranulate) inappropriately. The process acts quite differentry from traditional allergic reactions, and most of the time triggers won’t show up on traditional allergy testing. Stomach pain is quite a common symptom, uv sensitivity less common but I’ve heard more than a few patients mention it. There are a myriad of different skin reactions I’ve seen described. Other common reactions are flushing, hives, swelling, blood pressure goes high or low or fluctuates between, headaches, and quite a few suffer anaphylaxis with throat closing/swelling or loss of blood pressure and need epi pens for this emergency situation. There is no clear explanation of how MCAS gets kicked off, some like me have had symptoms since childhood but got worse suddenly at some point, others it’s out of the blue and suspected to be from insect venom, viruses, powerful antibiotics, etc. Common triggers are alcohol, insect venom, nsaids, opiates, antibiotics, fragrances/smells, foods, various medications or the fillers or colors in them, stress, weather, and the list goes on.
Treatment is usually antihistamines to block the cascade of histamine released from the mast cells, you will see references on this site to zantac+zyrtec being a good starting point to try. There are also mast cell stabilizers that help keep the mast cells from degranulating.
Since you are going to a dermo, you could start with asking for testing for mastocytosis, which is a related condition but consists of the body over producing mast cells. It is more rare than MCAS, but must be ruled out. Most dermo’s should be at least a little familiar with mastocytosis since the disease often includes skin involvement. First test is generally a blood test for Tryptase, a high level would point to possible mastocytosis. You could ask the dermo to biopsy any spots that regularly itch or swell up and ask for them to be specifically stained to look for mast cells.
It’s tough to be concise with this info, but there are now many resources out there. Tmsforacure.org is the US Mastocytosis Society site, mastocytosis.ca is the Canadian site. Feel free to ask me any questions, I’m a moderator on one of the Facebook groups.
Cheers,
LeslieI want to bump this thread and add my thoughts rather than start a new post, hoping Dr. Diana will weigh in. I am from the MCAS world and have been taking high dose hydroxyzine and 4x daily cromolyn for a few years mainly for helping chronic headaches and facial numbness. My other triggers are pretty well defined (medications and foods) and managed just by avoiding them. At first the hydroxyzine was a miracle and the headaches and numbness were gone, but the symptoms slowly returned. I added the cromolyn a year? or so later, and again it helped at first, but then I had the same issue after a few months that Dr. Diana describes in the videos: I absolutely hit the wall and I thought I was going to drop dead (not exaggerating). My functional doc ran new adrenal saliva tests and I was critically low, so started daily hydrocortisone which helped the energy but I immediately gained 25 lbs and the headaches are as bad as ever (suspect a csf leak, still looking). After seeing the latest videos, I suspect that the MCAS meds had blocked too much acetylcholine which whacked my HPA axis causing the cortisol issue. The hydrocortisone is treating the symptom, but not the source, and I’d rather not have to stay on it. I also have autoimmune hypothyroid and have had exercise induced headaches for at least 20 years, so my issues could be very long-standing. I likely have EDS or chronic hypermobile, but not yet diagnosed.
My question is this: How do we find the balance between histamine blocking meds needed for MCAS which happen to block acetylcholine, and the necessary amount of acetylcholine for the rest of our systems to continue to function? Will increasing acetylcholine reduce the need for histamine blockers?
Also, is there any specific treatment for enlarged internal jugular vein that starts this whole cascade? What imaging should I request to look for this?
An ENT can evaluate if the drainage is CSF fluid by running a beta transferrin test on collected fluid along with a blood draw at the same time.
I have suffered a myriad of chronic skin issues for decades. I’ve been treated for everything from acne to eczema to folliculitis with very little imorovement. Fast forward a bunch of years to after I started suddenly reacting to an odd assortment of foods and eventually found out about mcas, and I was finally able to tie alcohol, then nsaids and aspirin, and anti seizure meds (zonisamide) to my skin outbreaks. The key was that I had a huge skin reaction to mobic, and then I started having reactions within a few hours of having g alcohol, so I was able to identify the specific triggers. My spots are more like folliculitis, but on the mcas groups we have had numerous discussions of the weird skin presentations. Note that mcas folks can be sensitive to any med including antihistamines, usually from fillers and colors but also possible tO be sensitive to the actual antihistmine. In my opinion, it’s more likely you have some other chronic triggers happening and the zyrtec didn’t really have any part in it. For me, elimination of the triggers was the only relief, I was taking huge doses of antihistamines and using topical steroids with no affect.
Oh, and when I was trying to test whether the fluid draining from my nose was CSF, I went to an ENT here in Denver that specializes in the repair of sinus CSF leaks. You might want to check locally for an ENT with some CSF leak experience, mine was much more aware of all the symptoms. He couldn’t do anything because my imaging didn’t show the leak yet, but is there waiting if we ever turn anything up, at least.
Leslie
So true migracie! I was bedridden for much of the day for almost 2 years, just couldn’t make it through the day without lying down all afternoon. This was a result of the constant pain, but also crushing fatigue. I was being actively treated by my neurologist, trying pretty much every migraine treatment out there with very little effect and quite a few medication reactions. So glad you are getting some impact from Zyrtec/Zantac. I was able to try some different antihistamines with a cooperative doctor that didn’t see any harm, and I found that hydroxyzine is my best med. I take 50 mg 4x day, which would completely sedate most people (they use 100 mg pre surgery as a sedative). When it started taking away some of the numbness and tingling, I simply dosed based on when symptoms returned. Sadly the symptoms have exceeded the ability of the hydroxyzine to control them, but it still helps me be somewhat functional. I also took cyproheptadine (Periactin) daily for awhile, which helped a little, but could be contributing to my high prolactin levels so I’ve stopped for now. Cromolyn sodium was a huge help, also. Feel free to contact me for additional help with mast cell stuff. We also have some very active Facebook groups with many knowledgable members.
Leslie
Thanks for the reply Dr. D. I have, in fact, read that high ICP goes along with leaks. Sometimes the high pressure is though to be contributory to the leak, others it is thought to result after the leak as a compensatory reaction to the fluid leaking and the pressure going low. The thing that really makes me wonder is that my son has the (what we think of as) classic progression of large head until age 2 and then sudden drop off of his growth. His failure to grow was so severe he was eventually treated with growth hormone, was not producing any of his own (pituitary anyone?). We were fortunate to find a doctor to treat his growth at all, and we recently consulted with a premier pediatric endocrinologist who was surprised that an MRI was never done. Since he has normal growth hormone now for his age, the MRI is not indicated now. He has no headaches, but is irritable and has severe difficulty concentrating. Are there other symptoms we could look for that might validate that he really should have an MRI?
@susan_sk it is really good to hear that description of the headache from Dr. Schievink, mine started with progressively worsening numbness then went to getting a pressure related headache by late each afternoon. The really frustrating thing is that I did mention the postural component when the pattern first started, but was dismissed as “everyone feels better lying down”. Then the pattern went chronic and I was in so much pain all the time that there was no longer a postural component. It wasn’t until mast cell meds that I got a little control of the symptoms and noticed that I was worsening from being upright. Since I previously had more classic migraine with aura, all my symptoms were immediately diagnosed as just a different type of migraine. Sitting on the couch seems to be the worst, something about slouching and tipping the head forward.
So nice that Dr. D has the academic chops to take this issue on with her medical background and her willingness to be the guinea pig. For my part, I’m willing to share all of my remaining brain power and the knowledge I’ve gained about mast cell disorders. I’m also willing to share my medical records and imaging, and would volunteer for any research purposes. So if you need any of my records or need to examine me, Dr. D., I’m in!
Leslie
Did you get the myelogram done? Get any results? I’m currently getting scheduled for one. Suspect CSF leak, all low pressure symptoms, nothing on MRI, lumbar puncture results normal. Just curious about your experience.
Leslie
Hi, I can comment a bit about the mast cell side of things. Flushing tends to result from high levels of prostaglandins, which are released by mast cells. In mast cell activation syndrome (MCAS), mast cells tend to be badly behaved and will release their chemicals to improper triggers. Heat is a common trigger for many. Daily histamine blocking medications can help (like Zyrtec or Allegra here in the US), as can mast cell stabilizers (cromolyn sodium or ketotifen). There are also h2 histamine blockers more commonly known for acid blocking like Zantac or Pepcid that should be tried along with the other histamine blockers. Worth noting that ketotifen is terribly hard to obtain in the US because of its FDA status, it’s legal but not manufactured here. It can be gotten from overseas or compounded, but most doctors here aren’t familiar with it or the process. If you can get it OTC in Australia, I would recommend trying it. Prostaglandins can be tested via a 24 hour urine test for prostaglandin f2a, which I believe is a metabolite. High prostaglandins are commonly treated with aspirin, but be very cautious since those with MCAS are often triggered by NSAIDs (aspirin, ibuprofen, naproxen), and only do with doctor supervision. I’ve seen in our MCAS groups that mangosteen supplements are supposed to help regulate prostaglandins, but I haven’t tried myself. Incidentally, I have high prostaglandins but no flushing. It is also worth becoming familiar with common mast cell triggers and determining if any make your episodes worse so you could avoid the triggers. Reactions to triggers may be subtle; I was having low level reactions for years to ibuprofen showing up as skin eruptions around my mouth, but no doctor could ever figure it out. It wasn’t until ibuprofen also started making me itchy that I figured it out, avoiding has greatly reduced my skin reaction. Another common trigger you should be aware of is alcohol. I finally figured out I was having similar low level chronic reactions to just small amounts of alcohol, beer and wine being worst. I stopped having any about 18 months ago, and when I think it’ll be ok “just this once”, I now pay massively with a quite spectacular reaction. All of the little triggers that you get exposed to regularly can add up to a very mysterious reaction like flushing. Daily meds can help, especially to help you determine if you are looking in the right direction, but it usually comes down to identifying your own personal triggers and avoiding them.
Leslie
I totally relate about the not knowing when to get glasses with the changes in vision. A couple years ago I got a pair when things were bad, then had to go back and tell them I couldn’t see through the glasses a few weeks later. All my testing including eye pressure was different just a few weeks apart. I try to get examined when things are pretty good now, so I can use the glasses more of the time. Hope you are getting some progress with your other problems.
Leslie
I also get small amounts of clear nose discharge when leaning forward or exercising. It is not enough to properly test for CSF fluid, though. I attempted to collect enough over a week, and results were negative.
Leslie
So I have a very similar problem. I have symptoms of low CSF pressure/volume that have been chronic for at least 5 years and are getting much worse recently. I have constant numbness and tingling in my forehead, worsens to pain and stiffness in my neck, pain at the back of my head, tingling and pain in the top of my head, and numbness and tingling in my hands, legs, and feet as soon as I stand up in the morning. The symptoms increase over the day, and I can notice some relief of symptoms by lying down. Exercise makes things much much worse, and will generally trigger a “worst headache ever” migraine within a few hours. Also triggered by weather, altitude, and hormones. As the symptoms worsen, I get a distinct feeling of suction inside my head basically behind my sinuses. When it is really bad, I can sometimes feel the “bubbles” when I lie down and some of the areas equalize in pressure. It is just like when you clear your ears for altitude, but behind the sinuses.
At first I was unsure if my symptoms were more indicative of low or high CSF pressure. I have distinct worsening of symptoms with bending over and with any Valsalva maneuvers, which is supposed to be more for high pressure. I got my PA at my neurologist to let me test Diamox, and I got distinctly worse feelings of suction, so I’m fairly sure I have low CSF pressure, possibly with a leak. I have quite a few instances of trauma and car accidents over the years starting with passing out on a driveway chin first at age 13, and headaches first started in my teens, so there is the possibility of a trauma induced CSF leak. I have also had tinnitus that worsens when the headaches get bad as long as I can remember.
I have done extensive research on CSF leaks and low pressure symptoms, and it is interesting that the longer the symptoms have been chronic the less likely it is to be associated with positional worsening, meaning that the distinct worsening to very severe symptoms when standing is lost. I was able to reduce the daily symptoms by strictly limiting exertion for a few months, but they returned full force the first day I added any exercise back in, and have remained back at the severe daily level since, which to me also indicates a possible leak. I had a MRI with contrast done again in December 2014 with nothing notable for CSF pressure low or high, but according to my research there are a significant percentage of patients that show nothing on MRI especially when the leak is chronic for extended times. There is a study out there that actually shows that the imaging is distinctly different depending on the time since the leak began, and shows how easy it is to miss the “window” when you might see something on imaging. I also had a lumbar puncture to test pressure in January 2015 with no notable results. This is also known to be possible to be in normal range but still have a leak or low pressure/volume.
I have been diagnosed with MCAS, mostly evidenced by a sensitivity to many medications like opiates and NSAIDs as well as insect venom. I started daily antihistamine treatment summer of 2013, winding up on a particularly high dose of hydroxyzine daily and noticing an improvement in the daily facial numbness and headaches. I started taking the mast cell stabilizer cromolyn sodium August 2014, and had an additional improvement in numbness and headache control. I have no real idea of how these medications are impacting my low CSF symptoms other than possibly lessening the nerve reactions?
I recently found out that EDS likely runs in my family. I probably had enough signs as a kid to be diagnosed, but due to limited activity over the past number of years I’ve become quite stiff. My mom and daughter have pretty classic signs of EDS, though. This is important because connective tissue disorders are implicated in increasing possibility of CSF leaks through tears in a weakened dura. Also important because “brain sagging” is more likely from the reduced pressure/volume with EDS, putting more pressure on cranial nerves.
I also now have several endocrine issues, which tracks with CSF pressure issues causing pituitary dysfunction. Of course, when I consult with “real” endocrinologists I get laughed out of the office because my test results just don’t indicate things like full adrenal failure. I am fortunate to have an integrative doc who is willing to treat my hypothyroid with compounded t4 (I react to synthroid, armor, and tyrosint). He also diagnosed my critically low cortisol via saliva tests and I’ve at least improved the deathly fatigue with cortisone replacement treatment. I have had a high result on prolactin, which I’ve seen mentioned in research on CSF leaks/low CSF pressure. I also had a very low in normal range ADH (anti-diuretic hormone) which could be increasing the problems by keeping me more dehydrated because I immediately pee out everything I drink. I even have times where my skin dents from just momentary pressure. I’ve been diagnosed with Hashimoto’s hypothyroid for 15 yrs.
I am getting my neurologist to order a full spine and cervical myelogram, which is supposed to be the best chance of seeing a leak on imaging. If nothing is visible from that, I plan to have my imaging records sent to Dr. Linda Gray-Leithe at Duke who is a neuroradiologist that specializes in detecting CSF leaks. Apparently she is able to see things on imaging that are missed by most everyone else. She performs mainly epidural blood patches for repairs I think. Another option is Dr. Schievink at Cedars Sinai, who is also very experienced with leaks and does surgical repairs.
I am basically driving this bus myself to try to get a definite diagnosis and possibly attempt a repair. It is exhausting and frustrating, and really difficult to constantly go up against disbelieving doctors to get what is necessary. I am putting this info up here hoping that my research and experience might help others.
Leslie
I had a horrible skin reaction (mast cell related for sure) to zonisamide in December. The worst part is that it seemed to be helping my constant head pressure/tingling/numbness that regularly leads to migraines. I’ve seen some comments referring to odd seizure reactions for mast cell triggers on the Facebook groups, so it could be your personal mast cell reaction type. I get skin reactions mostly around my mouth to medications and alcohol, and all the head symptoms chronically. I’ve also seen mention from some experienced with MTHRFR issues that EDS symptoms can improve with treatment/supplementation for the methylation issues, so I don’t think it’s much of a stretch to suggest that a possible mast cell trigger med like zonisamide could trigger a worsening of EDS symptoms. I get similar reactions from nsaids and from alcohol, worst other than zonisamide was mobic, the really strong nsaid. Thought it worth mentioning for those not as familiar with mast cell issues, since the reaction signs can be subtle and quite delayed. It took me many years to figure out some of these triggers, and then only because the reactions started happening soon enough after the trigger to relate them.
