When is it appropriate to wait, and when should we take action?

My sister, Angie, was admitted to the Hospital on Feb 17th, as she was confused, had a fever, behaving strangely, very aggravated and then finally was unable to control her bladder. They did an MRI and diagnosed her with ADEM within 24 hrs. The DR admits that he has never treated ADEM (we are in a tiny hospital in San Antonio) and we (the family) are worried that he is not moving fast enough. He started her on the steroid treatment on the 18th and she showed slight improvement, so he waited 5 days and did a second MRI. It showed that the lesions had worsened. Then he started her on IVIG on 3/10. She has finished it and we see no change in Angie. Now he plans to wait until Thursday 3/20 for another MRI. WE are climbing the walls! Angie cannot speak, only paddles her feet and fingers, she is alert only partially, seems to understand us some of the time. I am afraid that the longer this DR drags his feet, the harder it will be for Angie to recover from the damage. Any thoughts? Am I just impatient?

We have tried to put our DR in touch with Dr Wingerchuck at Mayo in Arizona as ADEM is his specialty, but he has been on vacation. UGGGHH.

Has he mentioned plasmapheresis? That is another common treatment when the steroids have little effect. I hope you get answers soon- I would be climbing the walls as well. When my son was sick, our local hospital thought he had meningitis and was just waiting for him to get better. Despite his condition worsening daily, they kept telling us to be patient. I am an RN and my husband is an ER doc. Finally, my husband basically demanded a CT scan and when that was obviously abnormal he was transferred by helicopter to our children’s hospital where he was properly diagnosed and treated. Thank God he has made a complete recovery but I don’t know what would have happened if we hadn’t acted. Also, had my husband not been a doctor, we would probably have just continued to wait and see… He may have died or had a much less successful recovery. It sounds like your sister’s doctor is doing the right things, one of the hardest things with this disease is the wait and see times and the very gradual nature of the recovery. There must be someone else you can get a second opinion from? Thursday seems like a long time to wait with no improvements.

DO NOT WAIT!!! Get another opinion today. Then get a third. I was not correctly diagnosed for 5 months which has lead to permanent neurological damage. She should have another MRI to see if the inflammation has stopped otherwise the damage is continuing. That having been said, it will take time for the myelin to regenerate and that will be when the recovery is evident. It takes up to two years for you to know what the final level of disability will be.

I am in Austin & suggest you try contacting UT Southwestern in Dallas. I believe they have a neuro unit that understands the myelitis diseases. Call them asap & see if you can have Angie transferred to their care. If not, check with - what's the name of the Army hospital in San Antonio? Brooke Med Ctr, I think. They have a good reputation & they're right there if you can get in. But I'm not sure they have experience with ADEM, either. Call the hospitals & if you need help navigating the departments, there's usually a patient advocate that can help you. Push, push, push for her! Good luck to you.

Hi,

I agree with other comment,s time is important. I was first put in induced coma, then on steroids, then IVIG which didn't work for me, then they tried plasma pheresis, which worked. This took about 8 month's back in 2011, I have recovered a lot, but because of the scaring from the lesions on the spinal cored and the back of the brain, I have been left with some disabilities. It was a tough journey, but I'am thankful to be alive. I do not believe you are being impatient, my wife had to get in there and fight for me. I suggest go for the plasma pheresis.

Good luck and I hope your sister Angie get's the best treatment and makes a full recovery.

By the way it is not good me suggesting my Neurologist, because I live in Australia

Cheers Rob

Two years? Can the recovery period actually extend that long? That helps me to not give up hope. Thanks. --Paul

SweetKnits said:

DO NOT WAIT!!! Get another opinion today. Then get a third. I was not correctly diagnosed for 5 months which has lead to permanent neurological damage. She should have another MRI to see if the inflammation has stopped otherwise the damage is continuing. That having been said, it will take time for the myelin to regenerate and that will be when the recovery is evident. It takes up to two years for you to know what the final level of disability will be.

Cedarmama:

We actually mentioned it to him 2 weeks ago, and he said that "the literature" says that one is supposed to administer this treatment OR the Steroids, which she got first. This is the first indication that he is not the right man for the job.


Cedarmama said:

Has he mentioned plasmapheresis? That is another common treatment when the steroids have little effect. I hope you get answers soon- I would be climbing the walls as well. When my son was sick, our local hospital thought he had meningitis and was just waiting for him to get better. Despite his condition worsening daily, they kept telling us to be patient. I am an RN and my husband is an ER doc. Finally, my husband basically demanded a CT scan and when that was obviously abnormal he was transferred by helicopter to our children's hospital where he was properly diagnosed and treated. Thank God he has made a complete recovery but I don't know what would have happened if we hadn't acted. Also, had my husband not been a doctor, we would probably have just continued to wait and see... He may have died or had a much less successful recovery. It sounds like your sister's doctor is doing the right things, one of the hardest things with this disease is the wait and see times and the very gradual nature of the recovery. There must be someone else you can get a second opinion from? Thursday seems like a long time to wait with no improvements.

Thank you Connie. On it.

ConnieM said:

I am in Austin & suggest you try contacting UT Southwestern in Dallas. I believe they have a neuro unit that understands the myelitis diseases. Call them asap & see if you can have Angie transferred to their care. If not, check with - what's the name of the Army hospital in San Antonio? Brooke Med Ctr, I think. They have a good reputation & they're right there if you can get in. But I'm not sure they have experience with ADEM, either. Call the hospitals & if you need help navigating the departments, there's usually a patient advocate that can help you. Push, push, push for her! Good luck to you.

I agree with others about aggressively seeking another opinion. Has your doc been consulting any specialists? Sometimes a literature search is not enough.

With regards to his statement of plasmapheresis OR steroids, I believe that they are not normally administered at the same time as the plasmapheresis will remove the steroid from the blood, negating the effects. My son had his steroids for a course first, followed by 8 plasmapheresis treatments in 10 days. Not at the same time… But definitely both treatments. He then had ivig. Improvements started a few days after the ivig was complete. He was on a high oral dose of prednisone through the entire treatment period which was tapered after a few months. If your sister is not improving, I would think ANY possible treatment should be considered. Good luck to you and your family-?

Thank you so much. This situation is so very hard. When we press the DR, he just resists, and its not a great feeling. I am not sure how to go about getting a second opinion for her as she is trapped in this bed, but I am going to continue calling around. I have asked our DR to consult with a specific DR from Mayo, and our DR did attempt to call him (and I called Mayo to leave a message with that DR as well) but no answer as of yet.

I would also like to add that with a very cursory internet literature search, steroid treatment AND plasmapheresis treatments are both recommended by many sources. It is very difficult when confronted with a physician with whom you disagree. You need to keep in mind that they usually are trying to provide the best care they know how. Their disagreement with you is not normally a result of not caring. That said, being anadvocate for your sister is very important as well if you don’t think she’s receiving every treatment possible.

Our thoughts exactly. I do not think our DR is unkind or uncaring, just inexperienced, and after calling around, I am finding that it is difficult to get a consult or second opinion without moving her.

I am in Canada and don’t really get how your health care system works… Does your insurance provider pay for transfers for second opinions or would you have to pay? With such a rare condition, is there provision for getting best,most specialized care? In our situation, we live in a mid- size city with a proper hospital, pediatrics dept etc but when they realized that his condition was beyond their expertise, the standard of care was transfer to a facility that has the best, most specialized care… For him, a children’s hospital with a neurology department. I imagine it would be the same for patients in the US? Perhaps even more so for you with the legal system supporting you? No one wants to be sued…

Hi Sweetknits -- you said you waited 5 months to be diagnosed. I've already been through ADEM with my son. But I know a lady who had a viral illness, followed by terrible head pain which doesn't go away. She's had 3 MRI's. She now is noticing cognitive deficits. I'm wondering what you experienced during those 5 months. Is it possible to see an MRI and not diagnose it? My son's case was severe and so they diagnosed him right away. just wondering why it took 5 months and what your symptoms were.

SweetKnits said:

DO NOT WAIT!!! Get another opinion today. Then get a third. I was not correctly diagnosed for 5 months which has lead to permanent neurological damage. She should have another MRI to see if the inflammation has stopped otherwise the damage is continuing. That having been said, it will take time for the myelin to regenerate and that will be when the recovery is evident. It takes up to two years for you to know what the final level of disability will be.

My initial symptoms were onset of what I took to be the flu on Thurs Jan 15, 2009. I woke up with aches in my shoulders and back with feeling of being feverish. I went to work anyway but after an hour I called the doctor and went in for a prescription of Tamiflu. The doctor looked me over, took my temp and agreed. I went to get the prescription filled at the pharmacy down the hall. I turned it in and sat down. Three hours later I woke up, feeling even worse. I waited until there was no one in line then went to get my drug. While standing at the counter, I felt faint, told the clerk I think I'm going to pass out. Next thing I remember I was in the ER getting a CAT scan, a heart work up and a whole bunch more tests. Nothing. The diagnosis the next day was no flu on the culture but rest as clearly I had something. Sunday, my adult children and all the grandkids came over to make tamales. I though I had overdone it and caused my carpal tunnel problems to flare up because my right arm felt like it was on fire. I took NSAIDS and ignored it. Then, one week after the fainting incident I woke up with the right side of my body numb to the touch and cold. Back to the doctor, to my Family practitioner, who said, "I think you have MS." Off for the first MRI. It is always bad when they call you to come in for a second MRI at 6:30 the next morning and worse when the neurologist wants to see you at 5 PM on a Friday. Bottom line: I was told MS would be the good news, that it appeared I had a grade two or three astrocytoma in the medulla oblongata that was completely inoperable. A lumbar puncture that evening was followed with a horrible weekend.

The following Friday, with my right side still numb and icy cold to the touch, I was given some pretty grim prognosis. Then the neurologist went on vacation. By the following Monday the pain started. The skin on the left side of my head felt like it was being blow torched constantly. I couldn't brush my hair, wash my face or put my headset on at work. I kept calling for help and finally went to the ER where they put me on a palliative dose of steroids and sent home. The steroids helped quiet the pain a bit, but then the cognitive problems started. I was still trying to work and not succeeding very well. I was taking a large amount of steroids, lots of drugs for pain, meds to stay awake, and others to quell the paresthesia. I had 4 more MRIs over the next couple of months , all coming back with the same diagnosis, major astrocytoma 5 cm long encompassing half of the width in the medulla oblongata above the transverse process. No radiation or surgery possible due to location and chemo not advised. Lovely.

Meanwhile my husband of 35 years could not cope. I took a demotion and cut in pay and moved in with a good friend since I could not care for my own home any longer. She laughingly tells people that I moved in 6 years ago and was supposed to be dead by the end of summer. 5 months after the first symptoms - the feeling of coming down with the flu and passing out in the line at the pharmacy - I saw a new neurologist. I chose him at random because I liked his face and he had stated his interest in unusual situations. He looked at my first two MRIs and said, "You do not have a brain tumor. Yes you have a lesion in the medulla oblongata but you also have 12 other inflamed areas in your brain although the remainder are just pinpoints in comparison. You have Acute Disseminated Encephalomyelitis." High dose steroids and a six week course of doxycycline for its immuno-suppresent effect brought the progression to a halt.

But I still had serious problems with cognition and lots and lots of pain from the demyelinated areas. It wasn't until I was diagnosed with a second auto-immune disease which we now believe was the catalyst for my ADEM that the symptoms were finally conquered, nearly 3 years after they started. Once my undiagnosed Celiacs disease was uncovered and I went on a rigidly gluten free diet, I began recover quickly. I still have nerve damage. I still have pain and I have good days and bad. I will always wonder what if I had been diagnosed in a timely manner ? What if I had insisted on the second opinion when i first asked for it less than two weeks in? What if...

Your story is very moving for me, because I have had an experience that is in many ways similar. Almost exactly a year ago (I turn 67 in a few days), I came down with a severe gastrointestinal disturbance that lasted several days. Over the next few weeks, I gradually developed neurological symptoms -- difficulty with balance and walking, paresthesia on my right side, headache, and double vision in my right eye. I had seen my PCP a couple of times during this period and he eventually set up an appointment with a neurologist. I woke up one Monday morning and when I brushed my teeth I couldn't control the facial muscles around my mouth. Even though my appointment with the neurologist was the next day, the mouth thing scared me and I went immediately to the ER.

In the ER they gave me an MRI. Shortly after I got out of the tube, a doctor came up to me and said, "You have multiple abnormalities on your brain that look exactly like the lymphoma that killed my mother." No kidding, that's exactly what he said and how he said it. I'm still pissed off when I think about that, so unprofessional.

I was admitted to the neurology ward and over a couple of days received several tests, the purpose of which was to decide if I in fact had cancer or something else -- late-onset MS, ADEM, a couple of other things. Finally, I had a brain biopsy -- they put you under, drill a small hole in your skull, push the needle an inch or so into your brain, and grab a small amount of tissue.

Shortly after the biopsy I was visited by one of the neurologists -- I had seen several in the hospital -- who said I had brain cancer and would be seeing a neurosurgeon shortly. I saw the neurosurgeon, who said I would also be seeing a neuro-oncologist. Major bummer.

Then something really weird happened. About 20 minutes after my little tete-a-tete with the neurosurgeon, another neurologist came into my room and told me, "You don't have brain cancer, you have ADEM; the pathologist misread the slide from the biopsy." At that point, I was like, "Okay, whatever."

They kept me in the hospital, giving me three days of IV methylprednisolone. After that I was discharged with a three-week course of oral prednisone to take at home. That's all the meds I've had and it's been a year.

I have recovered quite a bit -- I'm back at work -- but I still have some residual symptoms -- fatique, but that's gotten a lot better; minor headache, kind of like I've had a little too much to drink; minor numbness, which for some reason seems to move around a little; balance and walking much improved, but I still have a little of a "drunken sailor" at times. Some days my residual symptoms are worse than others; I have good days and bad days, which can be worrisome. I try to stay optimistic, telling myself that this is all part of the healing process, and in absolute terms I really have improved a significant amount over the past year. Based on the admittedly limited reading I have done, I have come to this conclusion: on an individual basis it is impossible to predict how complete one's recovery will be and how long it will take. We read about "averages," but those are virtually meaningless to the individual patient, in my view. Nobody really knows. I've got my fingers crossed about remylination and continued healing.

Anyway, take care and have hope. You may still be gradually improving. I'd appreciate any comments or questions. This support group and message board has been very helpful.

Wow, Rich! Sounds all too familiar. I am 6 years post ADEM. I have been stable since the discovery of the Celiac disease and the elimination of gluten from my life in all forms - food, lotion, makeup, shampoo, detergent, treats for my dogs, etc. BUT, one small contamination incident, (such as the day my son was turning hot dogs on the grill at a family reunion. He turned mine in its new foil pan with the same tongs he was using on the remainder resting directly on the public grill), brings on rapid return of the ataxia, pain, cognitive disfunction, narcolepsy, and visual changes. I spend a week or more in bed and wind up back on steroids again. Sadly, while the small intestine problems from gluten are reversible, those of us whose bodies create antibodies to assault the gluten that wind up attacking our central nervous system are not so lucky. Each episode causes a bit more damage, a bit more fatigue, a bit more pain, which does not go away. My extreme response to contact with gluten is why my neurologist is fairly certain the undiagnosed Celiacs was the catalyst for my particular bout with ADEM. This is one of the reasons I am training a gluten detection dog, to help prevent accidental contamination and the return of all those awful problems.

Deborah

Rich said:

Your story is very moving for me, because I have had an experience that is in many ways similar. Almost exactly a year ago (I turn 67 in a few days), I came down with a severe gastrointestinal disturbance that lasted several days. Over the next few weeks, I gradually developed neurological symptoms -- difficulty with balance and walking, paresthesia on my right side, headache, and double vision in my right eye. I had seen my PCP a couple of times during this period and he eventually set up an appointment with a neurologist. I woke up one Monday morning and when I brushed my teeth I couldn't control the facial muscles around my mouth. Even though my appointment with the neurologist was the next day, the mouth thing scared me and I went immediately to the ER.

In the ER they gave me an MRI. Shortly after I got out of the tube, a doctor came up to me and said, "You have multiple abnormalities on your brain that look exactly like the lymphoma that killed my mother." No kidding, that's exactly what he said and how he said it. I'm still pissed off when I think about that, so unprofessional.

I was admitted to the neurology ward and over a couple of days received several tests, the purpose of which was to decide if I in fact had cancer or something else -- late-onset MS, ADEM, a couple of other things. Finally, I had a brain biopsy -- they put you under, drill a small hole in your skull, push the needle an inch or so into your brain, and grab a small amount of tissue.

Shortly after the biopsy I was visited by one of the neurologists -- I had seen several in the hospital -- who said I had brain cancer and would be seeing a neurosurgeon shortly. I saw the neurosurgeon, who said I would also be seeing a neuro-oncologist. Major bummer.

Then something really weird happened. About 20 minutes after my little tete-a-tete with the neurosurgeon, another neurologist came into my room and told me, "You don't have brain cancer, you have ADEM; the pathologist misread the slide from the biopsy." At that point, I was like, "Okay, whatever."

They kept me in the hospital, giving me three days of IV methylprednisolone. After that I was discharged with a three-week course of oral prednisone to take at home. That's all the meds I've had and it's been a year.

I have recovered quite a bit -- I'm back at work -- but I still have some residual symptoms -- fatique, but that's gotten a lot better; minor headache, kind of like I've had a little too much to drink; minor numbness, which for some reason seems to move around a little; balance and walking much improved, but I still have a little of a "drunken sailor" at times. Some days my residual symptoms are worse than others; I have good days and bad days, which can be worrisome. I try to stay optimistic, telling myself that this is all part of the healing process, and in absolute terms I really have improved a significant amount over the past year. Based on the admittedly limited reading I have done, I have come to this conclusion: on an individual basis it is impossible to predict how complete one's recovery will be and how long it will take. We read about "averages," but those are virtually meaningless to the individual patient, in my view. Nobody really knows. I've got my fingers crossed about remylination and continued healing.

Anyway, take care and have hope. You may still be gradually improving. I'd appreciate any comments or questions. This support group and message board has been very helpful.

Update:

Angie was discharged from the Hospital on 3/21 and sent to a Neuro Rehab center in Austin. This Neuro finally got in touch with Dr Wingerchuck at Mayo, and he is insisting on getting her started on the IPEX--we are trying to find an Neuro that will administer on outpatient status so that she does not have to leave the center. There is very little change in her, still not speaking, can barely move her extremities, but she is really trying! Hoping that this will be the magic wand!

I do not see anyone on here who can speak to such a complete loss of faculties in either themselves or a loved one, or am I missing someone?

I wonder how much pain she is in, how much she understands and what she needs or wants--especially when it comes to visitors. Normally, she is a very proud person that would probably not want any pity, or to be seen so compromised, but it just seems like it would be great for her to see some friends. Too soon? We just cannot decide, as it seems unfair to speak for her. Thoughts?

Thanks for the support! It has really helped!

I am so glad you have found help! I don't get on this forum often but was pleased to see your update. Have you established a way for Angie to respond to your questions so she can say Yes or No to a visit from friends? She's prob pretty darn bored being inside so much, maybe a visit would be a nice break. Also, it would be good for her friends to get a better understanding of what she's going through, if you can prepare them so they don't react in a way that would upset her. Although my son was the one with ADEM, I also have a daughter-in-law who had a stroke at age 28 & it has left her with multiple permanent disabilities. Only two of her dearest friends visited the hospital & I think they are the ones best able to see how far she's come. They do their best to see her the way she always was, treat her just like one of the girls still. They talk to her about celebrity gossip, boys, etc. Or talk about stuff they used to do in college. Just good times they've had & will have in the future. Something to look forward to, propel her forward. Even if it's not the same, it can still be a good time. I am wishing you both all the best.