we're really grateful that the expert based at Massachusetts General has been working with us since 2015, and we've built up quite an archive over the past few years. With a little sifting you should find the answer you're looking for. However, we know we haven't covered everything, so if there's something else you need to know, please contact us - we'd be happy to forward your question. It's part of what we do.
Question:
As well as Superficial Siderosis my son also has retinitis pigmentosa. With the advice of a nutritionist we are looking at his diet. A number of the things she is recommending have a significant iron content, eg blue/green algae/spirulina/dark greens etc. Provided my son follows your advice about avoiding iron/zinc/vitamin c rich foods 2 hours before and after taking the Ferriprox is it ok for him to eat these? The nutritionist said that these were ok for haemochromatosis. Thank you.
Answer:
The only concern with a high iron diet is that iron the gut can bind Ferriprox in the gut. The complex of the two is not absorbed so you will lose both. SS is not an iron overload disease as the total body iron is normal. It’s an iron distribution disease: the iron is distributed in the brain where it's not supposed to be.
Question:
Baclofen pump and SS
I have Superficial Siderosis and investigations found a disc causing bleed it was removed and bleed stopped. I was left paralysed T8/9 after surgery and have painful spasms . Baclofen pump is being offered but I am concerned if this will cause another bleed as it goes into spine. Any advice would be gratefully accepted as no one I've seen so far know much about SS and a baclofen pump
Answer:
Baclofen pumps are good when you respond well to oral baclofen pills, but you need so many pills that it makes you sleepy. Baclofen pumps allow you to achieve the
same level of anti-spasticity control but with lower levels of medications.
Before installing a pump, make sure you maximize medical management including combination of medications like muscle relaxants (baclofen, tizanidine, cyclobenzaprine)
and anti-seizure drugs (gabapentin, carbamazepine).
Question:
Blood brain barrier question
https://www.nature.com/articles/s41467-019-12426-9
There are current studies being done on using ultrasound waves to get meds past the BBB to treat other diseases such as ALS, MS, etc. Is this new technology a future possibility
for SS sufferers? Could Ferriprox possibly ever be injected straight into the brain, to more effectively target the bad iron in the brain as opposed to the good iron in the blood and body?
Answer:
Ferriprox (deferiprone) has no trouble getting into the brain. That’s why we use it for superficial siderosis. Within 7 minutes of absorption in the gut, deferiprone is detectable in the spinal fluid.
So this technology will not help in this regard.
However, there are other iron chelators that cannot get into the brain. We don’t currently use them for superficial siderosis for that reason. But if there were a way to get them in – perhaps with devices
like these – it might be useful.
Question:
Can you explain the relationship between iron and ferritin? My iron levels are staying between 10 and 30 and my ferritin is 15. Is that good?
Answer:
Ferritin is a protein that binds to and carries iron around the body. A ferritin level of 15 is fairly low and indicates that your circulating iron reserves are low. Maybe due to Ferriprox, if you're taking Ferriprox. That would be expected as Ferriprox depletes total body iron stores as well as depleting the iron salts from the surface of the brain and spinal cord. We follow ferritin levels to make sure that over time it continues to decline indicating effective chelation.
Question:
Chance for operation. This has me a bit worried. I have a neurosurgeon who is willing to do the operation to help with all the iron but I am slightly worried about what he says. With the amount of iron I have, he doesn't believe it's a bleed, he reckons it has to be multiple bleeds or the wire in my neck is possibly starting to corrode (for lack of a better word). I fractured the hinge bone between C1 and C2. He believes they might not have used or had titanium 26 years ago when they put the wire in and might have used steel. He has told me that even if the operation is a complete success, there is a chance I will come out worse than I went in. From the MRI he showed me it looks like the bleed or source is from my spine as the iron seems to be like a perfect 3-4 mm border around my cerebellum area (I think that's what it is called ). Are there any tests I can get without opening my neck? I mentioned does he think getting a spinal tap do any good to see if there is any blood there but he said what good would that do? (I have to admit I couldn't answer besides saying well I would know now if there was blood there) I could do with some words of wisdom. Thank you.
Answer:
It’s very likely that your C1/C2 dural defect is the location of the bleed. You don’t need another procedure to confirm it, but if you really want it you can do a CT myelogram. The CT myelogram also includes a lumbar puncture as part of the test so you can get both in one test. The lumbar puncture will tell you 1. If you’re bleeding and 2. How much you’re bleeding.
Question:
Could this improve the efficacy of Ferriprox? https://neurosciencenews.com/nanoparticle-bbb-17515/...
Answer:
The technology in this article is describing a lipid nanoparticle that can transport its contents to the brain. This might be useful for getting a new or better iron chelator in to the brain. Ferriprox is itself a lipid particle so it has no trouble getting into the brain. Within seven minutes of ingestion, Ferriprox is already in the brain. The difficulty lies in prying the iron molecules off ferritin proteins so they could be chelated by the Ferriprox.
Question:
Currently my husband's GP does not seem to think that he needs any specialist care at all! His hearing is deteriorating quite rapidly. Is there any value in him seeing an auditory consultant on this matter rather than just attending the hearing aid clinic?
Answer:
Your husband may be a candidate for a cochlear implant to improve his hearing. Not all patients are good candidates so it's important to get an expert opinion.
Question:
Diagnosed with SS with one strange symptom.
14 years ago I suffered with a suspected csf leak. Remained in the supine position for nearly a year to alleviate the headache and vomiting. No leak was found but
the symptoms at the time were classic of a csf leak with low pressure headaches. IV caffeine finally got me standing. Since then I have suffered with migraines. Recently the migraines and thunderclap type headaches and vomiting increased and that is when an MRI confirmed SS. Subtle but obvious I was told. No typical symptoms have started but what has occurred continuously for 6 months is some wet substance coming out of just my right ear, but only when I sleep on the right. It happens every night without fail.
 Doctor so far cant explain it and Im yet to do a CT scan with dye. My question is have you heard of this strange symptom and could it be linked to SS?
Answer:
It does not seem likely to me that your CSF leak 14 years ago was into your ear because you would have to have leaked a lot of fluid out of your ear at the time enough to cause sagging of the
brain, etc. Nearly all CSF leaks are in the spinal column somewhere.
Question:
Dr. is there any reason to find where the blood is leaking into my brain. when we were there, you didn't mention any kind of search for it. Maybe in my case, it didn't seem of concern. Of course, I may have missed the whole conversation.
Answer:
In some situations, it may be worth finding the bleed. Those include situations like bleeding tumors or spinal dural tears that can be corrected. It may be better to try to chelate first and then pursue the bleeding source later. Each case is unique in this regard.
Question:
Ferriprox for 70-year-old. Thirty-five years ago my husband was diagnosed with bilateral hearing loss. We were told that this was a result of radiation he received after removal of a benign brain tumour at age 11. About 10 years after the hearing loss diagnosis he began to have slurred speech. This was followed about 5-10 years later with movement and balance problems and be began to walk with a wide gait. Over the years he lost much of his sense of smell and taste. The latest problem in the past year has been memory loss which seems to be getting worse. He was finally diagnosed with SS a year ago. Would Ferriprox typically be recommended for a 70-year old with these symptoms? Is there a doctor in the Philadelphia area who has expertise with SS?
Answer:
Ferriprox takes a long time to remove iron from the brain. Depending on the amount at onset, it often takes many years (5-10 years) for most patients to see a benefit. Monthly blood tests, fatigue and the cost of Ferriprox take a toll over the years. In the meantime, the progression often continues but hopefully slows a little. We are working on making this process go faster, but in the meantime, this is all we have. So it’s a major investment and you’re right to take this into consideration when you’re making your decision.
Question:
For SS related nerve pain, I usually try one or more of the following: amitriptyline, Lyrica, gabapentin, Topamax, carbamezapine, Cymbalta. I use a trial-and-error approach as each individual has a slightly different response. I'm asking a question here that may have been answered by Dr. Levy already. But hoping for input from anyone that can help. ---- was diagnosed with SS over a year ago. He is in a Rehab Facility in NH but they are not aware or know anything about SS. He is in a wheelchair and has a feeding tube. One of his major complaints from the beginning is his left hand always feels cold. I have tried socks, mittens, heating pads an wrapping his hand in blankets. But nothing helps. His hand feels fine to the touch but its on the inside, well that is how he describes it. Lately he complains that it hurts feeling like pins and needles are sticking him. We ask his doctor about the gabapentin but he was afraid the side effects would be worse. Is this a common problem with anyone else? If so what suggestions or ideas do you have that could possibly help?
Answer:
Localized sensory issues could be due to SS but can also be due to other problems such as degenerative disc disease. The treatments are different so it's worth getting it checked out. Those medications you listed are good for SS related nerve pain. Physical therapy, epidural injections and/or surgery are options for disc disease too. Gabapentin is among the safest of all of these and I don't hesitate to use it.
Question:
Funding for Ferriprox. I have SS. I have been operated on by Mayo at Rochester and they were able to stop the bleed. Dohmen said that if I take Ferriprox it could reduce the iron deposits in my cerebellum which could lessen some of my symptoms, but I am finding difficulty in paying for the drug. Any suggestions will be appreciated.
Answer:
Dohmen should have access to the Co-Pay Assistance programs. They should be able to refer you to one. If you are ineligible or don’t have access to one, a lot of people order the Ferriprox online from outside of the US.
Question:
Has anybody had symptoms of SS reverse?
Answer:
Rarely but yes. In order for symptoms of siderosis to reverse, the iron has to disappear and the damaged tissue has to heal. In my patients who have been on Ferriprox a long time (> 3 years), sometimes they see some improvements. Most commonly I've seen improvement in sense of smell – probably because olfactory (smell) neurons can regenerate whereas most other nerves in the brain cannot.
Question:
Hi there I'm messaging from sheffield UK on behalf of my dad. He's 73 and been diagnosed with SS around 2000. He's in the advance stages with total hearing loss, nystagmus, loss of bladder and bowel control and in a wheelchair as he can no longer walk. I've heard about Ferriprox and wondered if it can be used in the advanced stages of SS. I've never heard his consultant mention this drug.
Answer:
Ferriprox is approved in the UK and should be accessible. There is no other iron chelator that gets in the brain so it's the only option for treatment at this time. No telling if your dad could see the benefit of Ferriprox since it will take several years to remove the iron.
Question:
How many cases of full recovery are known?
Answer:
Only a select few who have been on Ferriprox a long time (>4 years).
Question:
I am a mother of 7 children. I have had five of them from birth on and I started having migraine headaches while I was pregnant with my 3 year old Son.
For weeks I would have them everyday and they were the kind that shut me totally down and I had to rest them off. I starting using a tincture of wood betony and only 4 drops of it and it helps my migraines go away and not get so bad. Then when I was pregnant with our 2 year old, I had a weird episode. One night my husband found me laying on our living room floor, apparently my oldest daughter couldn’t wake me up and when my husband came home I was checked out and I only vaguely remember laying down and my daughter
giving me a pillow to lay on but nothing else. The doctors told my husband that I was dehydrated and needed fluids, but I dont remember anything of that week other than what I was told and only a few tiny details’ my husband said I was extremely confused.
 I was a CNA for nearly twelve years and saw a lot of confusion in people and I cant understand what happened to me because I felt totally disconnected and like I was in a fog. Later when I talked with my OB he said he had no idea what caused it. Also that same year during one of my migraine headaches, I tried to call my mother in law to ask her to please come help me watch my kids so I could take a rest because of my migraine- my speech came out totally jumbled. That also- no one could explain to me. They even
sent me home with a monitor of some kind but it showed nothing. Then in 2020, when my youngest was born I had speech problems when the OB was trying to speak with me, in the beginning stages of childbirth and another migraine headache. He would ask me a question and the answer I had was on my mind but I couldnt get the words to come out. So he had me get a CT scan and then later referred me to a neurologist- who then had me get 2 MRIs ( because he didnt start with the one that has contrast) and thats when they
saw superficial siderosis around the thalamus of my brain. So, they said that at some point in time I had a bleed there. Would that have been from my unexplainable episode? Or from a car accident I had in 2010? Or from before that? It doesn’t change anything, but I just wonder about that episode. Its a mystery to me and I felt like I was in a thick fog for weeks after and thats not normal. What do you think happened to me to explain it? Dr. Levy, if I didn’t have any further bleeding 2 years ago when they did an MRA, and they said I didn’t need a Neurosurgeon- then do I need to worry about further bleeding? My grandmother died of a hemorrhage inside of her brain, I don’t want that to happen to me. My family relies on me and I want to be here for them but how am I supposed to know if its bleeding again when I went so long without knowing there had been a bleed to begin with? I have headaches most of the time but what does a brain bleed feel like? Any incite you have would be wonderful. Thank you for your time, and for sharing your knowledge on this rare disease.
Answer:
Headaches can occur from brain bleeds, but they can also be due to a number of other causes. If you have migraines and the headaches are controlled with migraine meds, its probably migraines. If you want to know if the headaches are due to brain bleed, the best thing to do is get a lumbar puncture when you have a really bad headache. If there is no blood in the spinal fluid, its probably not a brain bleed.
If you’re worried about aneurysms, you should talk to aneurysm expert (not me). But, it sounds like you may had an aneurysm rupture perhaps that deposited the blood
on the thalamus? Its an odd place to have blood from classical superficial siderosis sources which is usually in the spinal column.
The event that found you on the floor, perhaps you had a seizure? Did you discover that you bit your tongue at the time or lost urinary control when you got up? Or perhaps you took an extra medication by accident for the migraine. I’m not sure!
Question:
I am aware that the trials for Ferriprox are ongoing. What do you hope that taking it may achieve? Or maybe the results will be infinitely variable like the disease.
Answer:
The goal of the Ferriprox trial is to see if removing the iron from the surface of the brain results in clinical benefit. With about half of subjects completed, my hunch is that two years is too early to tell. Most patients only begin to see reductions in brain iron by MRI at two years. It could take several more until most of the iron is gone. Only then would I expect healing to begin.
Question:
I am having major back surgery in a week. It's to repair damage of 2 broken vertebrae (T12-L1). Should I discontinue Ferriprox until discharge from the hospital, in case I possible infection? In an earlier answer you mentioned that people with SS take longer to recover from surgery and anaesthesia (Q10). Why is that and about how much longer?
Answer:
It's always a good idea to stop the Ferriprox whenever you're at risk for infections. To answer your other question, SS patients have reduced "neurological reserve." Neurological reserve is your capacity to resist neurological stress. Kids, older folks, and anyone with neurological disease have less neurological reserve. Depending on your reserve, you may take more or less time to bounce back from anesthesia.
Question:
I caught a cold - actually more sinus than anything else - typical sinus symptoms: sore throat, yellowish green mucous, burning eyes, slight cough. Should I stop the Ferriprox? Should I get an antibiotic as soon as possible ? This is new to me. Haven't had a cold in over 3 years.
Answer:
It's always safest to stop Ferriprox with an infection, just to be sure. Then you can quickly restart it when you're feeling better.
Question:
I have been on Ferriprox 500 mg per day I'm now trying to increase it, so one day per week I now take 1000 mg, since then I awake with numbness in my arms right down to the tips of my fingers, in which i then have no feeling in at all. It wears off after 20 mins or so. What do you think is the cause of this, what can I do about it?? Been on Ferriprox a year and a half.
Answer:
That sounds like either positional nerve compression during sleep or perhaps degenerative disc disease. It could be due to SS but I would check the other two possibilities first.
Question:
I have been taking Ferriprox for three and half weeks now, taking one tablet twice a day (as it stands at the moment) but they are looking to increase it to a maximum of three tablets in a 24 hour peirod. My last blood count was 5.5 and last couple days I have developed a needle type pain in the left side of my tummy (right at the side) I know it is a side affect but it is a needle type pain and driving me crazy. I think it's a side affect of the drug, should i be worried?
Answer:
The best way to know if this is a side effect of the drug is to reduce the dose or stop it altogether for a period of time. If the needle feeling goes away, it's a side effect. If the side effect persists, you should find the highest dose that you can tolerate even if it's only one or two tablets a day.
Question:
I have found that the use of cannabis helps me with my coordination the following day. Takingj ust one joint per day in the evening helps me greatly with my balance, headaches and leaves me feeling more awake during the day. Is there any evidence or trials to back this and would you recommend it? If I could get a regular supply of canibis, it would replace 6 tablets a day which cater for the symptoms above.
Answer:
I think medical marijuana is probably good for many neurological conditions. I suspect it's good for some of the symptoms in SS too. I don't know any suppliers. Medical marijuana is legal in my state but there are no dispensaries yet so I don't have any experience using it in my patients.
Question:
I have recently been experiencing twitches. First in the back of my shoulder blade, lasted 12 hours, then in my eyelid, about 3 hours. Both have gone away, but were very annoying. Is this from SS? Is there anything I can take/do if they come back?
Answer:
Probably from the SS, yes. If it persists for weeks, you can get it Botox’ed.
Question:
I have restless legs at night and twitch and jerk, can't stay still. Ropinirole 1mg ineffective. Any suggestions?
Answer:
Trial and error of medications like Lyrica, gabapentin, Cymbalta, amitriptyline and Topamax are worth a shot.
Question:
I just got Diagnosed with Superficial Siderosis. I also have Cervical Stenosis disease of the spine. I had my first Neck Fusion last month - C3, C4. I am going next week to see my Hematology doctor in Las Vegas and I need another Spinal cord surgery - C5, C6, C7 are all damaged The cords are flat and the pain is bad. I am also Bipolar 2 and Borderline Personality Disorder. My depression is really bad. I take an anti-seizure med for Mood Disorder and Lamical and Clonazepam for Anxiety Panic Attacks. My neck is in so much pain the Perzact is Interning with my Bipolar med which cause a life threatening rash. Should I get another spine? My lower lumbar back really is in pain.
Answer:
The decision to undergo surgery can be difficult especially since SS patients take longer to recover from both the surgery and the anesthesia. If you're unsure about advice given to you, I would recommend a second opinion.
Question:
I just had my second MRI yesterday after about a year on Ferriprox. My neurologist is thinking that he might want to take me off the drug if he sees no improvement. He is worried that my ferritin levels are too low...in the 20's. I know it can take longer than a year to see results. I really don't want him to take away my only hope. What is your advice?
Answer:
To help your neurologist understand what to expect, we generally start to see improvements on MRI after 2 years of Ferriprox, sometimes 3 years if the siderosis is heavy to begin with. We also expect ferritin levels to decline—that’s a sign the Ferriprox is working. While you’re on Ferriprox, it’s not a useful marker of available iron because available iron stores are being actively chelated. A better marker of iron deficiency is blood counts as the bone marrow is them most sensitive organ to iron deficiency.
Question:
I would like to know if there is any medication I can take for pain for my back? My Doctor says my SS is extensive.
Answer:
For SS related nerve pain, I usually try one or more of the following: amitriptyline, Lyrica, gabapentin, Topamax, carbamezapine, Cymbalta. I use a trial-and-error approach as each individual has a slightly different response.
Question:
I'm a 36 year old from Sydney, Australia and last year was diagnosed with SS possibly resulting from a rapid CSF leak at C4/C5 level (they're not 100% sure if this is the cause of SS). Symptoms have been persisting for years now. My neurologist is keen for me to have the leak repaired as he believes its causing an unsourced bleed. The iron build up is around the base of the cerebellum. Problem is the hole in the dura causing the CSF leak is anterior and the best option for repair is posterior entry according to the neurosurgeon. This is risky as they'll have to bypass some essential nerve roots and spinal cord to seal the leak. Not many neurosurgeons have done a lot of these procedures in cervical region nor have much experience with SS. Is there any way to know if SS is progressive and am wondering if there's a better option than surgery? I'd be grateful if any of you have any input as questioning if I'm choosing the best option.
Answer:
SS is progressive and stopping the bleed is probably a good way to stop the extent of brain involvement. Surgery is risky, yes, but because it’s at C4/5, an anterior approach is probably the best way to fix this. If it were higher, it would be too risky; lower would be inaccessible. You're kind of lucky it’s there, I guess. Glue injections and blood patches are other ways to seal the dura but they are transient and depend on inflammation to trigger healing. Surgery is more definitive and probably the best option in your case. After surgery, you’ll still need to chelate the iron off your brain and spinal cord. The iron that's still there will continue to be toxic until it's gone.
Question:
I'm having more surgery on June 2. My orthopedist is replacing the worn out liner in the acetabular component in my left hip. They'll probably want to give me heparin shots. Will this be okay, and for how long? Should I have my surgeon contact you? Every time I have surgery?
Answer:
heparin is really important following procedures like these and it's always OK to use heparin when you need to. Any short term bleeding from heparin is minor to the SS bleeding that has occurred over decades. On balance, the answer to any question about using heparin and other blood thinners is temporarily is almost always “YES.”
Question:
I've been having a problem with recurring urinary tract infections. Is this a result of SS? I've been trying to drink at least 8 glasses of water a day. I've got problems with dry eyes, brittle nails, and the bottom of my feet feel like leather, very dry. Are all these because of SS and what should I do about the UTI's? I have to wear Poise pads because of leakage, and that seems like when the UTI's started. Could that be a contributor, too?
Answer:
SS can cause bladder problems and that predisposes to infections. In addition, UTIs can exacerbate the leaking. Drinking a lot of water is good. You can try things like cranberry juice to acidify the urine – sometimes that helps. In more severe cases, we often use preventive daily antibiotics.
Question:
I've been having terrible uncontrollable loose stools I am unaware when it happens, it's been going on for about two years, but now it's worse than ever. I've been put on 8 Imodium per day which isn't working, have you any ideas as to what I can do? I was diagnosed 24 years ago.
Answer:
I can suggest three options: 1. Bulking agents to make the stool thicker and less runny: Metamucil for example + bananas. 2. Bowel training program to empty once daily. Difficult diet to maintain though. 3. Tincture of opium to slow the gut. Very effective but hard to find.
Question:
I've been taking Ferriprox for 2 years now, and my iron levels on the brain have almost been depleted. My symptoms are still uncomfortably strong, no hearing, some improvement in my balance, and very loud tinnitus. I've been taking OTC pill called Lipo-Flavonoid for about 5 months which is supposed to help with the tinnitus. It hasn't helped much with that but it has a lot of vitamin C. Should I give that up, as it is probably counteracting the Ferriprox. My next MRI should be in a couple of weeks, early June.
Answer:
The only trouble with vitamin C is the same as iron (answer above). It binds Ferriprox in the gut and you’ll lose both. If your MRI is improving, hopefully the healing will begin and you will regain some neurological function.
Question:
Ibuprofen
I read a great deal of the Q/A, and ask if ibuprofen is ok for headaches. My husband is newly diagnosed, has extensive SS in the cerebellum and is being told not to take ibuprofen. Other prescribed meds are not working for him. Codeine just knocks him out and he does not like that. He says the ibuprofen is the only thing that helps.
Answer:
Ibuprofen is probably fine. Ibuprofen makes platelets a little less sticky and therefore the implication is that it would make any internal bleeding from SS worse. Surgeons ask you to avoid ibuprofen before a surgery to avoid bleeding complications. But no one has tested ibuprofen in SS, I’m not sure it’s a fair extrapolation. Plus, every medication decision is a balance between benefits and side effects – so if ibuprofen is the only thing that helps, it might be the best option.
Question:
In one week I took two of these injections called (iFilgrastim (Zarzio) injections) because my neutrophil level went as low as 1.0, after these injections the levels went from 1.0 to 7.6 (10*9/L) it had never gone that high since monitoring started back in 2012 and the low levels were the reason why I could not be prescribed Ferriprox. Are you aware of these injections?
Answer:
Filgrastim is a hormone that stimulates the bone marrow to make and release neutrophils. This should only be used sparingly and not as way to repeatedly keep counts up while on Ferriprox. The better approach would be to modify the dose and schedule of the Ferriprox.
Question:
Iron infusions
I was recently diagnosed with SS after an MRI ordered because of vertigo. I have a history of iron deficiency anemia and have been working with a hematologist and getting intravenous iron infusions. I read that SS patience have normal iron levels, so I am wondering if the infusions were a bad idea and made the situation worse. Also, would I be a candidate to take ferriprox because my iron levels are already so low?
Answer:
Iron infusions into the blood stream are not generally harmful. When you bleed into the brain, most of the iron that deposits comes from blood cells. If you have transient higher levels of iron in the blood from the infusions at the time of a bleed, the contribution is probably minimal compared to the amount of iron in red blood cells.
Iron deficiency anemia can make chelation challenging because chelation therapy is not directed to the brain. Chelators remove iron from the whole body. When I treat my iron-deficient patients with chelation therapy, I often will use chelation for 5 days per week and iron supplements (rapidly absorbed iron supplements, not the slow release form) for the other 2 days per week.
Question:
Is there anything SS patients should avoid? Any activities that should be avoided? Any foods/medications that should be avoided?
Answer:
Activities to avoid: anything that re-tears the lining around your spinal cord (for most, that is the source of bleeding).
Foods to avoid: anything that will stick to the Ferriprox in your gut: foods that contain high vitamin C, iron or zinc content 2 hours before or after taking the medicine. No pills containing iron, zinc or vitamin C at all.
Question:
My ferritin level is still about 30 after two years on Ferriprox: does this mean the drug is not chelating the iron?
Answer:
It depends. Ferritin fluctuates a lot (in response to infections for example) but over time, a declining ferritin suggests you're becoming iron deficient. Iron deficiency is a good sign the drug is working. But you could have enough total body iron stores that you're not iron deficient despite the Ferriprox (yet). It doesn't mean the Ferriprox is not working -- an MRI of the brain showing a reduction in hemosiderin is the best indicator that Ferriprox is working.
Question:
My husband had his dural defect repaired C3-C6 area 10/13 at the Mayo clinic. As I read earlier on one of the questions, it looks to me that he would need to be on Ferriprox to help remove the iron deposits that remain on the brain and spinal cord. Is there currently any trials open for this therapy? We have investigated this therapy prior to surgery and and found it to be very expensive to use. Any suggestions?
Answer:
We only conducted one trial where the drug is paid for. The current trial just observes patients who already have access to this expensive drug. In the U.S., insurance covers the cost approximately 75% of the time. The other 25% have acquired the medication from online pharmacies or from foreign countries.
Question:
My husband suffers from constant debilitating headaches. He has tried all sorts of drugs over the last 20 years including being drug free for a year but nothing helps. Any suggestions?
Answer:
headaches in SS are often due to intermittent bleeds into the CSF. In these situations, it can help to stay hydrated and maybe caffeine (think: Starbucks Venti). It may also help to pursue the source of the bleed and stop it. Medications like verapamil may help, but medication benefits and side effects should be discussed with your doctor.
Question:
My son takes 3 Ferriprox tablets a day. Please can you advise on the best way to take these throughout the day in order to maximise their effectiveness. Is it best to take 1 tablet at a time with 7 to 8 hour intervals or can you take 1tablet in the morning plus 2 tablets at night?
Answer:
There is no known optimal regimen. I would say it's best to take pills twice a day to chelate all day and to use a higher dose at night to minimize the side effect of fatigue.
Question:
My zinc levels are a little low, 9.7 this month. How low can they be before its a concern and any ideas for increasing zinc without increasing iron
Answer:
When my patient's zinc levels get a little low, I recommend taking a 1 week break from Ferriprox and taking over-the-counter zinc tablets. The zinc level will bump up quickly.
Question:
Next step - diagnosed last October.MRI shows multiple microbleeds with no known cause. Spinal tap and spinal CT came back with no answers except low opening pressure. I had a CT Myelogram last week that showed no dural tears but the neuroanesthesiologist had trouble finding spinal fluid and went in 3 different places before he could place the dye, he said this was consistent with a leak. Still having severe headaches, what would be the next step?
Answer:
Probably the next step would be a blood patch. A blood patch would have the effect of sealing the leak for at least 3 weeks (which will give you some relief) and hopefully lead to healing of the defect that caused the leak.
Question:
Prochlorperazine
My GP has prescribed Prochlorperazine tablets for my spinning head.
It has had no effect so far, in fact, I continue to get worse!
Can they be effective in controlling the Ataxia or can you suggest an alternative?
Answer:
We don’t have any treatments for ataxia. I wish we did. The only thing I found that helps is vestibular rehab – physical therapy that focuses on balance. If anyone in the Facebook community has suggestions for medications, supplements or techniques that would help, please do share.
Question:
Should I be getting another MRI. It's been a year since my last one. Do we need a yearly MRI?
Answer:
Whether or not you’re on Ferriprox, it’s helpful to follow the course of disease or improvement with yearly MRIs. It's best to get the scan done on the same MRI machine at the same resolution. The best MRI sequence to visualize iron is called Gradient Echo (GRE). Using GRE, I can check for subtle changes over time.
Question:
So would it be possible to inject a chelator under the scalp, could that be a quicker way of getting rid of the iron?
Answer:
people have considered injecting the drug into the spinal fluid to get to the iron faster. It won't. The drug is lipid soluble which means that no matter where you put the drug – in the gut, under the skin, in a muscle, in a vein or in the spinal fluid, it will distribute throughout the body quickly within a few minutes.
Question:
This is a rare disease right, but what do I do if the doctors around my rural area and in the Cities here don’t seem to care or understand what I am dealing with?
I had a doctor that was supposed to follow up with me and he never did after testing was done and I still have not heard from him. That was 2 years ago. Then the next doctor I saw I gave him a list of my symptoms and they all seem to me to be related to SS because they are new symptoms to me since my diagnosis and range from brain fog and feeling somewhat disconnected, difficulty getting my thoughts out, being more forgetful, stuttering, scrambled words, unable to get my thoughts out correctly, numbness, pain in feet that feels like shocks, unsteady, headaches, tired all of the time- even after a full nights sleep, extra moody, I have a temper now and I didn’t use to, hyper sensitive to touch at times, while cooking I have to be completely zoned in so I don’t mess up and forget what I’ve put in. Things take me longer to accomplish, time gets away from me and I have lost 2 hours of time at once and didn’t realise it until I saw what time it was and it only felt like a few minutes had gone by, and I feel like my hearing is not as good, oh and my ability to play the piano like I use to is harder for me- My fingers knew where they were supposed to go and I could play it without looking but it is now harder to remember where my fingers are supposed to go, all of these things added together feel like the only explanation is SS but the doctors around me seem indifferent or don’t care. I was told theres no cure and then that my symptoms are not related. Oh and how often should MRI’s be done? Is it weird to have a level of ferritin at 20 with SS? My doctor gave me an iron supplement and it feels weird to be adding iron. This is all perplexing.
Answer:
Superficial siderosis can cause cognitive issues but its not super common. It depends on where your iron deposits are, how much you have and what caused the iron deposits. Generally, its a good idea to make sure you don’t have an overlapping condition like a seizure disorder thats treated differently. Some people with siderosis are older and therefore prone to Alzheimers disease or vascular dementia as well.
I recommend trying to narrow down 2-3 most disabling symptoms to present to your doctors. They might become overwhelmed with a list of 15 symptoms (and they may dismiss all of them!).
The ferritin question is important. You don’t have an iron overload problem. Your total body iron is normal. Superficial siderosis is an iron distribution problem- the iron is in the wrong place. It should be concentrated in your blood, bones, liver and bone marrow, not in your brain.
With superficial siderosis, you might become anemic and need iron supplements. If you take Ferriprox which removes total body iron, including brain iron, you might become anemic as well. Ferritin represents your total body iron so if its low, it may be that you are anemic and need to take iron. When you take Ferriprox, however, the ferritin level may be artificially low and inaccurate.
Question:
Use of Botox to release strong stiffness of shoulder and neck muscles
I had brain surgery, meningioma, in 2006. A fluid bag developed into the place of the benign tumour. Bleeding was discovered in 2009.
Since the operation muscles of the shoulder have been very tight, also the large neck muscle next to the scar. No stretching, exercise have helped, or only for a day or two. My therapists have been cautious to handle the shoulder and neck treatment due to the concern of affecting the fluid bag. The left shoulder pulls the body forward and to the left. The tightness of the neck area may be one reason for the bleeding. MRI does not show it, though. A physiatrist now recommends the use of Botox shots to release the stiffness. On the shoulders I feel ok, but are there any reasons, not to take it to the neck muscles?
My SS symptoms (started to deteriorate two years ago even though the SS has spread very widely in the brain) are partial hearing loss, ataxia and balance, double vision in the left eye (3 times of prism helps), bladder problems (here also Botox has been used, I have not yet decided, if I should try). I have used Ferriprox since beginning of June. I just turned 70 years.
Answer:
Botox can be helpful in relieving muscle tension. The bad part of botox is that it may make those same muscles weaker. In the bladder, botox helps prevent spasms and leaking but too much leads to urinary retention. The good thing about botox is that is always wears off. It could take a few weeks to months, but it’s never permanent. So I do encourage my patients to try botox when Botox experts suggest it could be helpful.
Question:
Would magnets worn on the head help shift the iron deposits?