The first two years in my Mom's words...
Neurological sydromes are on the rise (or maybe just more documented) and understanding them is so hard. I will try to explain things simply - so much of what I have read on-line is confusing and hard to follow. These first two years were the fastest and longest two years of our lives. Looking back, I don't even know if it was all real - because parts of it certainly don't feel like it. Those early stages of how will we ever get through this, the Meghan who fought every new thing we tried, and all the traveling we did! I think I cried every night the whole first year. And from Meghan's point of view - I think everything was suspended in time.
Since the making of Meghan's awareness video September 2014, we have met twelve families with someone touched by POTS. New research indicates that as many as 1 in 100 teens suffer from some form of dysautonomia (a blanket tern for a neurological dysfunction of things that your nervous system controls automatically such as heart rate, blood pressure, digestion, sleeping etc). For some reason, 80% of those diagnosed are female - and most take several years to find answers. POTS (along with other neurological syndromes) is often referred to as an invisible illness and I think a lot of people don't believe that the person is really sick or suffering from real symptoms. My best advice - don't tell someone who suffers from one of many invisible illnesses that they don't look sick. Or that they look like they are feeling better. They will remember those words always.
I have been trying to document things - there is so much and I want to keep things manageable to read. So this very first (long) entry of the blog is sort of a summary of things - if you can call it that. Future entries will be from me and some from Meghan as she is ready.
Everyone's experiences are so unique. That's partly why it is called a syndrome - a collection of symptoms - some different, some the same from person to person. A lot of what I am writing is not believed by every doctor or every researcher. It's just my interpretation of what has happened to us. Patients share a very similar underlying theme of frustrations in dealing with doctors along the way. Make sure to take everything you read or things you hear from doctors with a grain of salt. Be smart but also trust your instincts.
A brief description of POTS and neurological conditions is also probably necessary. I can explain it to someone and then explain it again a month later and I know they still aren't sure. It really wasn't until I did the awareness video with Meghan that things came together for me - and this was almost a year and a half into the process. Neurological disorders are those that affect the nervous system - such a complicated system in our body - and still so much is unknown. One part of your nervous system controls automatic functions such as heart rate, blood pressure, digestion, bladder function, sweating, body temperature, sleep cycles, tear & saliva production etc. So for example - when you lie down, sit up, stand up or exercise, your body is constantly and automatically making adjustments to your heart rate and blood pressure. When you stand up your blood pressure has to increase to pump blood from your feet to your head - which is harder for your body to do then when you are just laying down. Unless you are doing heavy exercise, you probably don't even notice, and certainly don't even think about it. This does not happen correctly when someone has POTS. Blood pressure does not increase when you stand up or when you exercise - this causes a racing heart, dizziness, light headedness, and release of adrenaline to compensate. Even with all of this going on, the POTS patient looks fine on the outside - no one can see these changes. Meghan's worst symptoms included an almost constant feeling of nausea, sporadic light headedness or dizziness, stomachaches every day, extreme hot and cold spells, anxiety caused from the racing heart and adrenaline release, fatigue and joint pain. She would often crawl up stairs or go up stairs without bending her knees because her joints hurt so badly. But the blood pressure and heart rate issues are the defining symptoms of POTS. The long list of other symptoms range from patient to patient.
Starting with a timeline of events: Right before Memorial Day in 2013, Meghan found a deer tick on her arm. You almost couldn't see it - like a tiny scab the size of a pin head that she said wouldn't come off. I knew right away what it was and tried to remain calm. She took precautionary antibiotics for 3 days and numerous people told me to relax. Three weeks later the dizziness started - and sporadically she wouldn't be able to finish swim practice (she swam 5 days a week). It frustrated all of us because swimmers are crazy people who practice on Christmas Eve and New Year's Eve and have rare breaks during the year - so when you start missing practices they don't handle it well. :)
We may never know for sure if the tick bite started this whole process but it very well could have. This is definitely an unknown and some doctors think yes and some think no. She currently does not have Lyme's disease and most likely never will. But the bite could have triggered her body to begin producing antibodies that subsequently turned bad. People that develop POTS, have the perfect underlying conditions - a few examples in her case: naturally low blood pressure, very low heart rate, low blood volume, a family history of auto-immune type diseases, hyper-sensitive nerves and an over achieving or driven personality with LOTS of energy. That is a very simplified example of some of the things Meghan already had (and it can be different for each individual) but these are often common things that other POTS patients share. Then some sort of triggering event occurs. It could be an illness that is taxing to your body like mono or other virus. It could be a concussion. It could be another auto-immune disease such as Sjogren's that the person develops. And now they are finding a link between tick bites, lyme's disease (which is also a neurological condition), and POTS. So basically, a neurological interruption (trigger) to your nervous system occurs, when conditions are right, that throws everything out of whack. There are some patients who never know what triggered their illness - but often times there is a defining event.
Then the search for answers. Each doctor we saw had an explanation that made sense and we would leave and think everything was going to be okay. She had a virus. She was dehydrated. She was having optical migraines. She had vertigo. She was faking. She was anorexic. She wasn't eating properly. I had that poor girl eating so much healthy weird stuff and smoothies with dark cherries and ginger. :)
She struggled but swam the rest of that first summer (2013) and even qualified for a meet she desperately wanted to go to in Kansas in the beginning of August. By September, she couldn't make it through any of swim practice. It was devastating. She cried every night and begged me to take her to the pool. She said if I wouldn't take her she would get someone to pick her up. At an appointment for a regular physical with my own doctor in October that year, we talked about Meghan and all of her different doctor visits with no results. He vaguely remembered something they had talked about in medical school that involved a "switch" that sometimes gets turned off in your body for your everyday functions but couldn't think of any other details. I went home and started googling (one of my favorite things). I came up with a general medical website that talked briefly about dysautonomia - a vague description and some similar symptoms. This seemed like a possibility of what was going on but I needed to find a neurologist who would see us and listen (there are not a lot of pediatric neurologists in Wisconsin) - and I had already exhausted my efforts and patience with Children's Hospital. I even had at one point questions at Children's Hospital insuating that maybe Meghan was not being properly cared for at home. It was a scary time. We had lots of talks that summer about whether she thought she had anorexia or if she was pretending or if something WAS really wrong. She would look at me and I could see in her eyes that she was not pretending and she was afraid we might never figure out what was happening. Then in August a pediatric cardiologist that was leaving Children's that summer because of his own frustrations gave me the name of a doctor in a neighboring community who was a pediatric neurologist - I decided to try her.
In November (after more weeks of waiting) we finally got an appointment. The doctor thought I was crazy. Well, actually she thought Meghan was. She said no, none of the things that I told her about Meghan were really happening. It was too rare and some kids act out for different reasons. She said Meghan might benefit from seeing a therapist and gave me some names. Meghan started crying and the doctor said I had to leave the room to talk in private because it was too upsetting for the child! She said Meghan had a lot of anxiety and maybe we should take things more slowly. I refused to let it go and told her that there had to be more she could do. I was desperate! She agreed to refer Meghan to Froedert for a tilt table test (used for diagnosing neurological type disorders). And with A LOT of luck, this was done right before Thanksgiving. After finally seeing the results almost 2 weeks later in December, the neurologist confirmed that yes, Meghan did have dysautonomia - which as I said before is a blanket term for someone experiencing certain dysfunctions of their nervous system. I asked her if it was all in Meghan's head and she QUIETLY said no. I thanked her and we never went back. Now realize - the part where we had lucked out getting an impossible appointment for the tilt table (me speaking loudly to a scheduler, not yelling of course, followed by an extremely rare cancellation) and the part where we had a doctor speak rudely and condescendingly to us at Froedtert and the part were I yelled 'I knew it' when the neurologist walked momentarily out of the room, and the weeks we had to wait in between each step - were all left out of my story. Meghan doesn't like those parts. I am trying to leave out the crazy or rude things that doctors have told us along the way - it's very hard and there are many stories! I think people don't believe the stories I tell about how some doctors have treated us.
So now what. Meghan has dysautonomia. What does that mean and what do we do? We still hadn't heard the words "POTS" at all yet! No one in Wisconsin seemed to know anything about it - at least not among the doctors we had seen so far. And we had literally seen 15 different doctors from all different specialties. But we knew her body wasn't controlling her blood pressure and her heart rate correctly. I was disappointed (that's putting it mildly) in doctors in Wisconsin so I thought maybe the Mayo clinic could help us. Yes, that's it! Nope. I was wrong. Initially, I thought it was great...we went there in January of 2014. We spent 4 days of doing tests (mostly repetitive tests because they only use their own results - which is wasteful and expensive). We talked to several nurses and assistants who seemed to understand and sympathize. They said she was deconditioned - which typically happens when someone is bedridden for months or even years on end and then tries to get up and do stuff. Unfortunately, I was too stupid at the time to doubt anything they were saying. She was a competitive swimmer and a child who rarely sat down - of course she wasn't deconditioned.
We again went home thinking all would be fine. They told us she had a mild case of something called POTS but not to worry because she would grow out of it - and exercise, lots of water and salt were all she needed. Ummm. Not so much. That is actually old thinking and true for a miniscule amount of people with POTS. Most people with POTS do NOT outgrow it. And although exercise definitely helps - you still have to understand what is happening to your body and why and the limitations. The next few months nearly killed all of us - because we thought consistent exercise (30 minutes everyday) and water and salt were the answers. Also, they said a therapist would help with Meghan's growing anxiety. Okay. So we add that, too. Another mistake. Because her heart rate changes sporadically through-out the day (imagine how you feel when your heart suddenly starts racing - you feel nervous), Meghan's body was tricking her into feeling nervous and anxious. POTS tricks your body into feeling anxiety; but anxiety does not actually cause POTS. This is very important to understand. Very. So if you go to a therapist who doesn't understand the underlying health condition, they get frustrated when they can't help you with your anxiety because they think it is all in your head. Unfortunately it is not in her head at all! After we stopped going to see the therapist, a weight was actually lifted. She had convinced me briefly that Meghan was acting out on purpose and could somehow control it. It wasn't until we did the video that I completely understood how that all worked. I can tell when Meghan is hiding extreme dizziness or anxiousness from me. Her personality changes and she often acts out. Or if her blood sugar level is low. I have become much better at handling this or knowing what she needs to help get it under control. Medication can also help control some of the symptoms. That was actually something Mayo discouraged us from trying with Meghan - when actually a few medications that she currently takes have greatly improved her quality of life. More on that later.
Back to the Mayo Clinic almost killing us - Meghan and I religiously started exercising 30 minutes a day and drinking salty things like broth and eating pickles (well she did that part) or salt water or anything to get her salt intake up into the 7000mg a day range (they recommend the average adult to get 2300mg or less). She would gag on the broth and even gag on pickles near the end. She would cry and struggle to do basic daily activities let alone 30 minutes of exercise. After 6 weeks of suffering (they told us to be patient because it would take time), I called our nurse at Mayo and said it wasn't working and that it seemed to be getting worse. She basically said we hadn't done it long enough to see improvement. Click. That was the end of that nonsense. What next?
I felt defeated. Discouraged. Alone. If Mayo couldn't help, who possibly could? Fortunately, my discouraged spells are always short lived and I started scheming new things in my head. I am so skeptical of people and things and answers. I don't take no for an answer - especially if I don't think it's the right one. Back to google I went. I literally had a huge stack of things I googled, printed, highlighted, organized by subject that I would take from appointment to appointment (doctors often disliked me from the start and would sigh out loud when they saw my papers). Eventually, I come upon a website called POTSgrrl. It is a blog about a woman from Long Island who developed POTS in her 30's (she had the right underlying conditions all along just hadn't had a trigger yet). She suffered a concussion while snowboarding that triggered her POTS and found she had Sjogren's syndrome as an underlying cause. She became bedridden for several years and started this incredible movement in research in neurological illnesses. It's mind boggling to me the lack of funding and research that has been done for neurological illness. If you google Lauren Stiles you will come up with pictures of her and article after article of all the things she has done to help those with dysautonomia. She is a lawyer and a marine biologist and kids about wanting to become a doctor. She is an over-achiever type who wouldn't accept the ridiculous answers many doctors gave her (like depression because she hadn't had any babies yet, etc). Her blog was the first time I read about a treatment called IVIG. More googling. IVIG (in a nut shell) is just the antibodies taken from the blood of literally thousands of people and mixed together into this amazing concoction. It is given through an IV and it looks like a liter of saline if you were to see it. But I kept hearing about how hard it was to obtain and how doctors didn't really know why or how it worked yet. Oh. And it's really expensive.
More google searches keep bringing me back to a site called Dysautonomia International. Lots of good information but how could I know if it's a good source? It was international after all!. At that time they were hoping to do a study for people with POTS and were looking for donations to fund the research. For some reason, I couldn't let it go - although I didn't have any idea where the money was really going and if the site was legitimate. I literally stared at it for about a week - finally my husband said to do whatever I needed. So I donated. Within a few hours, I got an email from someone thanking me. It turns out to be Lauren Stiles - the woman I had read so much about and the author of the potsgrrl blog. She is probably the most inspiring person I can think of ever. Ever, ever. You have no idea. Really. We ended up talking on the phone and she introduced me to a whole new way of thinking, doctors, and studies. It was the best thing I did through all of this. Really.
She gave me names of doctors all over the country and opened my world up into research and knowledge that no doctor we had encountered could even begin to understand. Meghan and I got on a plane in June of 2014 to see a pediatric rheumatologist in Boston. One of the few direct flights left out of Milwaukee! We left early one day, saw the doctor, and Meghan was back to school mid-morning the next. We wanted to rule out some common underlying auto-immune diseases that often accompany POTS and to just talk to someone who knew things! Lauren also told us about new studies that had made some exciting discoveries linking POTS to a specific bad antibody that your body produces making it an auto-immune type disorder (announced February 2014) - which could be the answer as to why IVIG works for people with POTS. (Remember IVIG is a mixture of thousands of people's good, healthy, fighting antibodies). By now we are over a year into the journey - and we still don't have a doctor in Wisconsin for Meghan.
The next doctor that we decide to see we met by chance through a friend's husband, who is a doctor, who knows someone who has POTS. She is in her 30's and saw someone in a town an hour north of us who was looking to start a dysautonomia clinic through our healthcare network. I decided it would be nice to have a cardiologist closer to home for regular stuff. There are no "just POTS" doctors. Typically you have a neurologist and a cardiologist that work together - here it was no different. We meet the doctor and instantly I like him - he's a great listener and got Meghan on fludrocortisone, propranolol, and midodrine - common POTS medications if you actually go to a doctor who knows what's going on - and gave me some hope. I tell him all about our travels, Dysautonomia International, Lauren, and the new thinking on POTS being an auto-immune type syndrome. And then I tell him I want Meghan to try IVIG. I was afraid to ask him because most doctors have not reacted positively when I even bring it up. So, I just blurt it out. He is interested in all of the newest findings and says we should meet his neurologist who would determine the need for IVIG - I am thrilled! But...it turns out to be another disappointment, dead end, and another rude, condescending doctor who talked down to us. She asks me where are the studies supporting such things. She says she isn't familiar with POTS being auto-immune. She said I was wasting her time - and her other patient's time! I stare at the floor and I do not speak the rest of the appointment. I cried all the way home.
But again - this does not stop me. Meghan is slowly progessing to a worse state. She has nerve damage in her feet called neuropathy - it starts out as tingling and pain sensations and becomes an area where nerves are no longer feeling sensation (later this starts in her hands). She has increasingly worse hot and cold spells - she uses cold packs from the freezer that wrap around her neck to try to cool down. She goes to bed with an ice cream pail (just in case) each night with terrible nausea wishing she would throw up so she could feel better. Joint pain causes her to struggle with stairs - her Dad carries her to bed or she crawls up the stairs. Dizziness, spinning rooms, lightedness occur through-out the day. We decide to have her stop doing gym at school - gym days were hard for her to recover from. The school easily gets it set up so that in the fall when school starts again, I can pick her up during lunch and study hall to go the Y each day to do exercises that suit her needs. I document her exercise and submit it quarterly.
During the summer of 2014, we fly to Washington D.C. for a 3 day conference (click here to find out about watching informative videos from the conference) that Dysautonomia International holds each year to listen to top neurologists and other specialists talk about new findings and different things patients find helpful to make it through the day. We meet Lauren in person. I tell her I want to have Meghan try IVIG but we can't find anyone who will prescribe it - and that I want to get her into a study. Meghan spends a lot of time laying in bed at the hotel the conference was at because of dizziness and nausea. But we do find a study that Meghan can take part in.
In mid-September, we travel almost 900 miles to meet a doctor with a limited IVIG study and a pediatric cardiologist he consults with on several patients. I can't believe how much they know. I am a wreck the whole trip. Meghan has to qualify and be approved for the study. We leave there knowing her blood pressure and heart rate changes meet the requirements but are not certain of the remaining criteria. We have to wait almost 3 weeks more - some of the new testing procedures failed on initial blood tests. I begin to doubt whether or not this will really happen. Medical research is full of set backs and retesting and tweaking of procedures. I try to be patient.
Currently IVIG is not a drug listed or approved for POTS - there are actually no approved drugs for POTS at this time! You have to find a doctor to prescribe it "off-label" and why I had such a hard time getting it for Meghan to try. Most insurance companies do not cover off label treatments (or at least really expensive ones)...so the hope is that when there is documentation of the results it will lead to IVIG becoming a prescribed treatment for POTS and lead to less problems with insurance coverage.
In November of 2014, Meghan and I stop exercising at the Y. She has far too many dizzy spells, terrible stomachaches, diarrhea (remember your nervous system controls digestion), lots of anxiety, random heart pains, and joint pains. She hardly eats anything all day because of the nausea she constantly feels or the stomachaches the food causes. I pick her up from school during exercise time to just give her some rest to finish the school day. But on the outside - she continues to fool people. No one really knows what is going on inside her. She smiles and acts like everything is normal. She would cry when she got home from school and lay on the couch hoping to feel better. POTS can be a progressive disorder and we were definitely seeing a faster progression then we had previously. But thankfully we also get confirmation that Meghan does meet the requirements of the study!
I talk on the phone with his consulting doctor who is swamped by patients because he can't turn anyone away. So many are suffering. He will be the prescribing doctor of the IVIG and the first dose will be done at their hospital in another state, November 30th to December 1st, 2014. We will have it done through the night since it can take up to 12 hours the first time and Meghan can sleep through a lot of it. We don't know how she will react so they do it in a hospital setting at a very slow rate and she is giving a high dose of Benadryl and a steroid to help avoid any allergic reactions. She is also given pain medications for migraines which is a common side effect of the IVIG. They also give her a liter of saline before the IVIG and a liter of saline after the IVIG. So she is pretty water logged - maybe even a little puffy around the eyes when we leave. But it goes fairly smooth - some throwing up during the beginning of the infusion, most likely from the high dose of Benadryl. We fly home that night after several hours of observation.
The next few days are much harder than expected - she develops meningitis type symptoms that can occur after IVIG or other types of drugs (cancer treatments are another example). She wakes me the next night at 3am with a horrible migraine, feverish, neck pain, vomiting, and a panic that won't subside. I debate whether or not we should go to the emergency room. We make it until 6am and I am able to email with the consulting doctor who answers me immediately. He helps us through it and prescribes a strong pain killer and nausea medication. The first treatment of IVIG is typically the hardest and each subsequent dose is easier - you also learn what types of medications the patient will need to keep side effects under control - everyone reacts a little differently. After about 15 hours of the delayed reaction and horrible pain, she feels better - almost like a switch turned off. It took about 4 days before she felt completely "normal."
I haven't talked much about insurance. Our insurance has been great about covering all the doctor visits - just a few bumps here and there. I went ahead with the IVIG without knowing what they would say or do. Frankly, I did not care. We were doing it. It took a bit, but the letter did come denying the first two treatments. The 3rd treatment was already scheduled and soon the infusion center called and said they could not keep the appointment until insurance agreed to pay or we paid up front. Both the researcher and his consulting doctor write letters to our insurance company and we appeal the denial. More waiting but eventually they agree to one year...without the fight I was expecting.
We decide the treatments will be monthly - usually during the first week of each month. Subsequent doses will be done at the hospital about an hour from our home - the researcher's consulting doctor is still the prescribing doctor but it is overseen by her new cardiologist here. This is a relief - I don't know if we could physically and mentally fly 900 miles each month with no direct flights. So yay! We can do it in Wisconsin. Meghan is terrified to do the next dose because of the meningitis type symptoms of the first dose. We talk about how if we stop, we will go back to the way it was before and the hopelessness we felt. She agrees we need to keep going. It goes smoothly and migraines seem to be her biggest side effect. Typically lasting 2 days. We experiment with some different medications and by the 4th dose have gotten it down to a small headache here or there while taking migraine medication. She actually had none after the 5th dose.
The first dose took around 12 hours. Subsequent doses have been closer to 10 hours - one a little longer when she briefly developed hives and we paused the infusion to make sure it wouldn't develop into something worse. The goal is to get it down to about 8 hours. It is hard on her body to have it all dumped in at once or over a short period of time. She needs to have time to process it a little at a time.
Some patients have immediate and very notable, dramatic results from even the first dose of IVIG. Meghan did not. I was scared that maybe this was the wrong thing for her - even after dose 3 - I was worried. After everything we had been through and the expensive treatment costs. Sometimes when you fight really hard for something you find out it wasn't really what you wanted after all.
After the first dose, her overall nausea was slightly better. And she started eating a little. After the 2nd dose, we noticed less stomachaches and a little less joint pain. After the third dose, a little more energy, definitely able to do stairs better, and very little nausea or stomachaches. After dose six, I start to see some glimpses of the old Meghan - the Meghan who was wanting to be a part of everything and not miss out on one single thing. She wants to do the school zoo trip, the dance, sleepovers, the school Great America trip and anything else she can think of. She was excited to go to Florida over spring break - in the past she had dreaded any kind of traveling even if it was to somewhere fun. She just wanted to stay home. Do nothing. So the excitement for our trip was a wonderful sign. But she can still overdo it. She still has dizzy spells. Still can't do excessive exercising. Still has to drink 6 bottles of propel a day with salt added to it. We found a great lemon salt on Amazon - she gets close to 4000 mg of sodium from her propel and lemon salt drinks each day that help her to increase her blood pressure). She feels the effects if she doesn't drink her salty water. But things are noticeably improving.
I have left out other long complicated things we experienced along the way. She also has a connective tissue disorder called Ehler's Danlos that often goes along with POTS. It can contribute to the joint pain - we saw a physical therapist for a few months last summer. She also has some symptoms for and is part of another study for PANS/PANDAS. This can be caused by a bacteria that gets into the brain, causes neurological issues, and is often somehow linked to all of this POTS stuff or other neuro syndromes. It's very lengthy and complicated and even controversial so I left most of that out. She had an abscessed tooth removed that could have been the source of the bacteria. We have had lots of rough times of behavior issues and acting out and manic episodes early on - before I knew how to handle them or what was causing them. It was very hard on our family. Probably more so than the actual POTS initially. But I wanted to note this happened as well. There have been new findings that ADHD is also neurological based and I can say that sometimes some of Meghan's outbursts seemed like ADHD. Especially when we did too much or tried to do all our normal traveling we do for swimming or family. There where so many times I wasn't sure what would happen to us. I think the POTS symptoms exacerbated these symptoms many times over. We just didn't know early on. I can't imagine going through this without the internet. I felt so alone so much of the time. Researching online helped take away some of the helplessness.
In defense of the Mayo clinic, I do have to note that it is an enormously huge place and there are different types of thinking on POTS and even so within Mayo. We happened to be in probably the wrong area. They have just announced that they are also starting a study on the auto-immune possibilities. So this is good news - just didn't help us along the way. I also know that the therapist we saw for 6 months or so meant well. And that she really cared about Meghan. Just a good example of how little is known about the brain and the nervous system and how some of the symptoms can overlap for many disorders and anxiety issues - and people can get placed into the wrong group. The therapist really has to be careful to understand what they are dealing with before coming to conclusions. Seeing a therapist to help someone deal with a chronic diseases is different than having someone treat you for anxiety that isn't really anxiety. I should also mention that there are POTS patients who don't have the auto-immune antibody. That some have another form or other cause. Everyone is so different. Finding a good doctor who can help you sort that out is so important.
I am so grateful for the support she has received at her school. Everyone has been great. So many people in the office, teachers, and counselors have gone out of their way to help in any way. Without it, this process would have been so much harder. People with POTS also often describe a brain fog that they feel and how it effects their ability to concentrate. And tricked feelings of anxiety make it hard for tests and speeches. But these things are improving and she is noticeably able to concentrate on her homework and finds tests easier. All good things. I think the school is a great place for spreading awareness because of the number of people that can be reached. Teachers and staff can maybe help identify someone struggling and get them pointed in the right direction quicker. This has already happened more than once!