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Medication and Treatment II
This the second of our Medication Q & A pages with Dr Levy - please view the first one here.
We're really grateful that the expert based at Massachusettes 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.
These answers relate to medication and treatment of Superficial Siderosis and its associated symptoms. If you're looking for something specific, please use your internet browser's page search facility (usually in the top right hand corner of your screen) to search particular keywords or alternatively, browse down the page to learn more about how Superficial Siderosis can affect everyday life and what can be done to help. If you'd like to broaden your search, you may find what you're looking for on another part of the Silent Bleed site - please use the search box in the header above.
Question:
I have been advised that Deferiprone or Ferriprox for the treatment of early stage Superficial Siderosis is only available in USA. Are you aware of there any proposed Clinical Trials in Australia, in particular Melbourne ? or a Specialist we can contact regarding these medications?
Answer:
There are no trials for superficial siderosis using Ferriprox anywhere in the world at the moment. However, we are planning another trial in the near future which will likely include sites in the US and UK.
Question:
A family member who suffered a major spinal cord injury decades ago has had two MRIs that show SS and has experienced several years of hearing loss and cerebellar ataxia. This family member also has a history of DVT and is on OAT. In your webinar, you suggest that all SS patients should be initiated on Ferriprox, since doing nothing means inevitable progression. As far as I know, my family member's physician and neurologist have not discussed initiation of Ferriprox chelation. How difficult and expensive is it to access Ferriprox? My family member is insured through a state insurance exchange. What can we do to try and access the drug? And is the DVT/OAT a contraindication?
Answer:
The drug is very expensive in the US but there are sources online that are cheaper and there is a considerably cheaper generic version of deferiprone called Kelfer that is made in India. For my patients whose insurance companies do not cover Ferriprox, I recommend trying to obtain the drug online.
Question:
Are there any more radical treatments being considered such as flushing a iron removing fluid around the brain?
Answer:
Radical treatments have not been considered seriously. Several patients have suggested it but regulatory authorities would never approve something without extensive testing in animal models. And that would take lots of time and money.
Question:
Are there any problems taking antihistamine meds for hayfever (eg Loratadine or Chlorphenamine) with Ferriprox?
Answer:
There are no problems taking anti-histamines with Ferriprox.
Question:
CT scan or MRI?
My 81 year old mother was diagnosed with superficial siderosis with deposits on her brain stem, cerebellum and spinal cord. She is had a series of operations over the years for cervical spine stenosis, including rods and a cage put in to stabilize her. We always thought her symptoms were because of arthritis, but it seems as if her balance issues, pain, neuropathy, and her deafness can be from this.
In addition to this problem my mother also has antiphospholipid syndrome (autoimmune disorder that makes blood clots) and has been on warfarin for over 20 years. She has been falling alot, and her doctors have always told her to go to the emergency room if she hits her head because of the coumadin. Everytime this happens...we go...and she has a ct scan that has never seen the siderosis. It is my understanding that MRI will pick it up - NOT CT scan.
Considering that she has a history of siderosis plus on a blood thinner, if she falls and hits her head, should she be getting a ct scan or MRI in the emergency room? Will a fall impact this condition in any way?
Answer:
A CT scan is better for visualizing fresh blood from a trauma. And it’s super fast. So if your mom falls and bleeds in her head somewhere, a CT scan is what you want to get.
A CT scan won’t visualize the superficial siderosis, which is old blood products deposited on the surface of the brain. Only an MRI can see that. Superficial siderosis evolves slowly and can be followed yearly or every other year. No need to get an MRI with falls.
Question:
Can a person who is diagnosed Superficial Siderosis and also has Marfan's Syndrome take Ferriprox safely?
Answer:
As far as I know, yes. I have had two other Superficial Siderosis patients with Marfan’s Syndrome.
Question:
Can patients who have cochlear implants go on Ferriprox as MRI's are no longer possible.? Do you need the regular monitoring by MRI to measure progress. Is there any other way to measure progress?
Answer:
There is no other way to monitor progress if you can’t get MRIs. Clinically, the expectation is that you will worsen even while you chelate because the iron is still there (albeit reduced in amount hopefully) and is still toxic. So if you can’t get MRIs, the only way to know if it worked is to take it until the iron is all (or mostly all) gone and you clinically stabilize or improve. Alternatively, you can have the magnet of implant removed (short procedure) and then replaced (another short procedure) after the MRI.
Question:
Cerebral Spinal superficial siderosis. Hi, my husband and I have been trying to find help for three years. We have been to countless doctors including the Mayo Clinic in Minnesota, looking for help. He had surgery in December and his most recent MRI shows the surgery was not a success. Looking for any amount of help we can find.
Answer:
Dr. Kumar at the Mayo Clinic in Rochester, MN, and I are the only two neurologists who see patients with superficial siderosis in the US. Dr. Kumar’s expertise is in the surgical approach to finding and stopping bleeds. I can’t imagine a more experienced team. But I also have neurosurgery colleagues at Johns Hopkins and UCLA who I’ve worked with before. My area of expertise is in the non-surgical management of superficial siderosis although I do refer patients to surgery when they really need it.
Question:
Do you know of a neurologist in Britain that specializes in SS?
Answer:
I don’t know any that have a particular interest in SS.
NOTE FROM THE SILENT BLEED TEAM: We know that Prof Werring at UCL's Queens Square centre has a special interest in the condtion and now quite a few patients in his care. That said, there are also now other doctors in the UK, based around the country. Please contact us for more information.
Question:
Do you still see a case for use of Ferriprox/deferiprone if: 1. A Siderosis bleed has been sealed surgically; and 2. The patient does not have ongoing ferritin in their spinal
fluid (if that was confirmed by lumber puncture)? In the above 1and 2 scenario do you believe that Ferriprox/deferiprone would still be a viable treatment to attempt to chelate the historical existing deposits on the brain and spine or, based on research,
do you see the value in its use only for patients who have ongoing bleeds as evidenced post surgery by ferritin in spinal fluid? Thanks
Answer:
Ferriprox (deferiprone) can do two things: 1. Remove acute blood products. It does this easily. For those with ongoing bleeding, this medication can help to prevent additional iron deposits from accumulating.
2. Remove old blood products. This is harder to do once the iron is bound to the surface of the brain. But eventually, after several years, deferiprone will start to remove those old iron blood products.
Question:
Doctor search. Hello, my mother has recently been diagnosed with superficial siderosis. We are trying to find a doctor that has knowledge of this condition. Do you have any recommendations in the Midwest?
Answer:
Dr. Raj Kumar at the Mayo Clinic in Rochester, MN, is a world expert in superficial siderosis.
Question:
Dr Levy There is a post on Facebook referencing a product by the name Ironology. Ferriprox and some other supplements for SS are brought into the thread and there seems to be substantial confusion on what works and how. Dr. Levy, I am completely ignorant to what you can/will comment on, but was wondering if you could offer some clarification? Thank you!
Answer:
Ferriprox belongs in a class of medications called iron chelators. Iron chelators are simple molecules that bind iron. When you take an iron chelator, it binds iron in your body and then the kidney excretes the product into the urine. Ferriprox is unique among FDA approved iron chelators in that it is the only one that can get into the brain to bind iron there. Ironology looks like a company that sells both iron supplements and a pill that blocks iron uptake in the gut. The ingredients report curcumin, which is found in the spice turmeric, which is a weak iron chelator. The idea is that the curcumin taken with your meal will prevent the iron from being absorbed into the body. This might be useful if you have too much iron in your body, for example. In superficial siderosis, though, the total iron level is normal. Reducing the body’s iron stores does not “draw” the excess iron out of the brain, unfortunately. So this approach is not helpful. There are some reports that curcumin can get into the brain so perhaps this supplement can be helpful in removing iron from the brain directly. Similarly, there is another vitamin called IP-6 (inositol hexaphosphate) which can also weakly chelate iron and may be able to get into the brain. To be more certain if these are helpful, we need conduct appropriate trials. In the meantime, however, there is probably no harm in using them.
Question:
How is Feriprox dosage decided, is it on weight?
Answer:
Our first trial based the dose on weight – 30 mg/kg/day divided into the two daily doses. But since the drug only comes in 500 mg tablet pills, we standardized the dose to 1000 mg twice daily which is approximately 30 mg/kg/day for a 70 kg person.
Question:
I have been on Ferriprox for almost a year now, without any problems on my weekly labs. Is there a time when I can cut down on the frequency of my labs being drawn? Thank you.
Answer:
Based on our experience with lab monitoring, we feel that as long as your blood counts are stable, you should be able to monitor labs monthly, not weekly any more.
Question:
I have been taking Ferriprox for twenty months and my last MRI showed no change. Would an intermittent bleed need to be found? Is it likely the bleed could become a bigger bleed?
Answer:
Iron from active bleeding is much easier for Ferriprox to chelate than iron bound to hemosiderin already stuck to your brain. If you are seeing no progress by MRI, my only suggestion is to keep trying it. I’ve seen only a rare few cases that did not improve by MRI eventually (4 years or more in some cases).
Your bleed hasn’t changed in 15-40 years. It’s unlikely to change now unless you do something traumatic or surgical.
Question:
I have been taking Ferriprox over 4 yrs now. I had some balance improvement by the end of the first year. In the past 6 months I have been experiencing vertigo/dizziness, pain in my lower back & the back of my head, and sharp pains in my feet, legs, and eyes. Along with all of this, I still have continued loud ear ringing and extreme fatigue. Can all of this be related to Superficial Siderosis? Thanks for all you do! God bless you!
Answer:
Vertigo/dizziness, pain and loud ringing in your ears (tinnitus) are almost certainly due to ongoing issues related to superficial siderosis. The extreme fatigue is probably due to the Ferriprox. To address the fatigue, you can try to reduce the morning dose.
Question:
I have been taking supplements in a smoothie with non-heme iron 4 hours before Feriprox to be sure that they do not coincide and have much more energy, is it ok to continue like this?
Answer:
The best way to know is to check monthly ferritin levels. If the Ferriprox is chelating your total body iron, ferritin levels will decline over time.
Question:
I have mild superficial siderosis and have been on Ferriprox for three years. I have just received my latest scan results and it shows no change. I do a lot of exercise, mainly free weights and low impact cardio and I walk my dog every day. Could the frequent change in blood pressure close an intermittent bleed? Could my workouts increase a bleed and could the Ferriprox be just working on new deposits?
Answer:
To answer your first question, it depends on the source of the bleeding. Exercise and exertion may cause re-bleeding if there is shearing pressure on the bleeding site, but I don't think it's due to changes in blood pressure. In answer to the second, Ferriprox is very good at chelating fresh blood. I think there is plenty of Ferriprox to chelate both the fresh blood and the continue to work on old iron deposits.
Question:
I recently went for a follow up MRI for arachnoiditis post subarachnoid haemorrhage/intraventricular haemorrhage . My new diagnosis is siderosis of the lumbosacral spine. Have you ever seen arachnoiditis and siderosis occur together in the spine? Are the physical pain symptoms the same?
Answer:
Yes, superficial siderosis and arachnoiditis co-occur. When bleeding occurs in the spinal column, the fresh blood triggers an inflammatory reaction in the lining around the spinal cord. That process is known as arachnoiditis. Symptoms of arachnoiditis all come from dysfunction of nerves trying to pass through the inflamed lining to the lower abdomen, lower back, pelvis and legs. It’s also very painful. Arachnoiditis is treated using anti-inflammatory medications.
In contrast, superficial siderosis is caused by the long term consequences of chronic or intermittent bleeding over a long time. In response to bleeding, the brain and spinal cord produce a protein called ferritin to chelate and neutralize the free iron from the blood breakdown products. This product of ferritin and iron is called hemosiderin and it is insoluble. It is unable to be filtered back to the bloodstream so it collects in the spinal fluid. By gravity, it collects around the spinal cord when you’re awake, in the back of the brain when you sleep on your back and in the front of the brain when you sleep on your tummy. For the most part, hemosiderin is effective at suppressing the damage caused by free iron but over years and decades, the iron can escape from time to time and cause damage to the brain/spine tissue underneath. The areas most vulnerable to iron damage are the nerves going to the ears causing hearing loss, the nerves in the balance center of the brain (cerebellum) and the nerves in the spinal cord causing weakness, numbness and bowel/bladder/sexual dysfunction. We treat superficial siderosis by stopping the bleeding and chelating the iron out of the brain with iron chelating medications.
Deferiprone or Ferriprox
I have been advised that Deferiprone or Ferriprox for the treatment of early stage Superficial Siderosis is only available in USA. Are you aware of there any proposed Clinical Trials in Australia, in particular Melbourne ? or a Specialist we can contact regarding these medications?
I don’t know of any clinical trials of deferiprone in Australia but the medication is generally available worldwide including in Hong Kong and other places near you. It’s also available to purchase online. There are also generic versions called deferiprone and Kelfer that are much cheaper.
How to access Ferriprox
Dr. Levy, a family member who suffered a major spinal cord injury decades ago has had two MRIs that show SS and has experienced several years of hearing loss and cerebellar ataxia. This family member also has a history of DVT and is on OAT. In your webinar, you suggest that all SS patients should be initiated on Ferriprox, since doing nothing means inevitable progression. As far as I know, my family member's physician and neurologist have not discussed initiation of Ferriprox chelation. How difficult and expensive is it to access Ferriprox? My family member is insured through a state insurance exchange. What can we do to try and access the drug? And is the DVT/OAT a contraindication? Thanks!
Ferriprox is expensive in the US, but it is fairly easy to access online (in the US) and available in many countries. There is also generic versions (deferiprone) and in India it’s called Kelfer that are less expensive. It’s always worth requesting insurance coverage for these medications before paying for them out of pocket.
Using blood thinners can increase the bleeding risk in the brain and spinal cord in superficial siderosis. However, blood thinners are generally life saving for people with blood clots and other disorders. In most cases, even if they worsen the siderosis, I continue to use them in patients who need them. You can use chelating agents while using blood thinners at the same time.
Question:
I try to eat a healthy diet but I worry that I'm eating too much iron. My ferritin is 10 and iron is 17, could the Ferriprox be just working on the iron in my blood and not getting to the iron in my brain?
Answer:
The Ferriprox is certainly chelating iron in your entire body, including freshly ingested iron, as well as iron in your brain and other organs. There is no way to target to Ferriprox to any particular iron store – it gets everywhere. The only thing to avoid in this context are iron pills which will bind the Ferriprox in the gut and you will excrete both pills.
Question:
I was diagnosed with immune deficiency around the same time it was found I have SS. I have had immunoglobulin therapy for 1 year now. I feel like when I have an intermittent bleed now my recovery from that episode is a little faster than before the IG. It could be wishful thinking but do think there is any benefit of me taking this for my SS? Do you think there is any connection between people who get SS and being immunocompromised?
Answer:
I cannot think of a link between your immune disorder and superficial siderosis. I also cannot think of how IVIG might be useful in your recovery from an intermittent bleed except that maybe the infusion is keeping you well hydrated. The only way I can like an immune deficiency with superficial siderosis is perhaps through an antiphospholipid antibody. These are antibodies that can interfere with the blood clotting cascade. You can test for these antibodies, but not while you're using IVIG regularly because of contamination. For what it's worth, I have never seen a case of superficial siderosis associated with this immunological blood clotting issue.
Question:
I watched the NBC Dateline program on Sunday (6/7/15) which was about the use of cannabis oil. The show focused primarily on children with epilepsy and neurological conditions. It is believed cannibis has helped or could help if it were more readily available. A neurologist stated that it was her opinion that cannabis could help in people with MS. Since SS mimicks MS in some areas. What is your opinion about it's use in SS? How difficult is it to get for medical use? Is Johns Hopkins planning any research on the use of cannibis oill?
Answer:
Cannabis would be expected to help with pain (neuropathy) and spasticity. For those SS patients with neuropathy and spasticity, cannabis would be expected to help. Unfortunately, no, we have no plans for testing cannabis at Hopkins.
Question:
I would like to receive the results of the tests with the drug feriprox that are carried out with SS patients. Reason: I was recently diagnosed with this disease, and here in Brazil there is no knowledge for treatment with iron chelators, so I would like to use the results of these tests to ask the health plan for coverage of the drug.
Thank you very much.
Answer:
This is the latest study we completed with Feriprox describing the benefits we saw:
https://pubmed.ncbi.nlm.nih.gov/29285884/
This article is free of charge to download and share.
Ferriprox after bleed is sealed
Question:
I'm helping a loved on to find a neurologist who has experience with treating superficial siderosis near VA and NC line. Area from Roanoke VA to Greensboro NC line, any advice?
Answer:
Superficial siderosis is incredibly rare. The only two doctors I know of who routinely treat superficial siderosis are me and Dr. Kumar at the Mayo Clinic. However, any neurologist can learn about superficial siderosis and consult us for advice.
Question:
If I have an intermittent bleed is there anything I should not be doing? I work out doing weights, shadow boxing, low impact cardio, Zumba and yoga but have done since before I started Ferriprox and my MRIs have shown no deterioration.
Answer:
Your MRI will not show deterioration because the timescale is too short. You would have to compare an MRI 4-5 years from now to see a deterioration. And you would not necessarily be able to correlate a change in that MRI to a few things you are doing now. So it’s not useful to use MRI as a measure of whether something is harmful.
The best way to know if these activities are leading to bleeding is to do the activity vigorous a few times and then get a lumbar puncture. If you see fresh blood in your spinal fluid, you have your answer. If your spinal fluid is clear, you’re probably OK doing those activities.
Question:
If the bleed has stopped and I come off the Ferriprox would I get worse or is it possible that I would stay the same with no further degeneration?
Answer:
Generally, iron deposits will cause degeneration of the underlying tissue over time, even if there is no additional bleeding. Consider that new iron deposit lie over the old ones so the toxic iron is the oldest iron deposits. However, stopping the bleeding can limit the extent of iron deposits on new areas of brain tissue.
Question:
In your webinair you mentioned IP6 as a theoretical, though untested, treatment for chelating the iron from the brain. As a family we have had a lot of success using more natural/nutritional approaches (e.g. my son who is autistic uses inositol for depression/OCD symptoms etc.), so we feel very comfortable using such approaches. My son who has SS takes Ferriprox and obviously would not want to stop doing so. Would it be safe to take IP6 as well as the Ferriprox?
Answer:
I think it would be safe, yes. I see no harm in taking both IP6 and Ferriprox.
Question:
Inosine
Inosine is available OTC. What would you advise people like me who are tempted to order and try it out on their own?
Answer:
Inosine hexaphosphate (IP6) has two properties that are appealing for superficial siderosis patients. First, the inosine compound is a neuroprotective agent that was shown to be helpful in other neurodegenerative
conditions like Parkinsons disease. Second, the phosphates provide a limited measure of iron chelation; it’s considered a weak iron chelator. Otherwise, IP6 appears to be harmless at the recommended dose (see instructions on the bottle).
Question:
Is Alzheimer's a form of dementia created by SS? What causes dementia in SS patients?
Answer:
No. Alzheimer’s is a dementing illness caused by a very different mechanism.
Question:
Is it Zinc, Iron and Vitamin C alone we should avoid in the two hours before or after taking Ferriprox?
Answer:
Yes. Those are the three things that can bind to Ferriprox.
Question:
Is sugar making my neuropathy-worse?
Answer:
maybe. If you are diabetic or borderline diabetic, sugar can cause neuropathy which will make your SS-neuropathy feel worse.
Question:
Is there anything else to take besides Ferriprox to get rid if the iron as I can't afford Ferriprox?
Answer:
Ferriprox is cheaper online at places like Canadadrugs.com and the generic is also available in India (marketed as Kelfer) but I can’t verify those sources as reliable. There are no other FDA approved iron chelators that can penetrate into the brain.
There is a vitamin compound called inositol hexaphosphate-6 (IP6) that has been proposed as well as green tea extract, but neither has been adequately tested.
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 temporarily is almost always “YES.”
Question:
Medication after a stroke. I was diagnosed with SS in 2006, started on Ferriprox in 2010 and I'm currently still on it. I was doing really well and my SS seemed stable. Then in 2013 I had a small stroke because of my SS and it was decided not to give me the usual medication after a stroke. Neurologically I deteriorated and I have now just had another stroke which has affected my right arm and leg. in your opinion should I be given the usual medication post-stroke which includes aspirin? Thank you and regards.
Answer:
Yes, you should take aspirin to reduce your risk of stroke. In the short and long term, strokes are much more likely to increase your level of disability compared to superficial siderosis. You will bleed a little more with the aspirin, but if you’re on Ferriprox, the additional bleeding will not add to the iron burden you already have as the Ferriprox is much better at binding fresh iron.
Question:
My husband's neurologist has told us that that there is a difference regarding the brain and meningeal siderosis, not systemic. He was not sure Ferriprox would be effective or even work in the brain which is not truly an iron overload. I didn't realize there was a difference. Is there nothing we can do for meningeal siderosis?
Answer:
Meningeal siderosis and superficial siderosis are the same thing. They refer to iron overload confined to the central nervous system. There are other iron overload conditions that lead to iron overload in the entire body, called systemic iron overload disorders. Ferriprox along with the other 4-5 iron chelators are all good for systemic iron overload disorders, but Ferriprox is the only one that can get in to the brain so it’s the only one we’ve tried for superficial siderosis.
Question:
My husband's neurologist has told us that that there is a difference regarding the brain and meningeal siderosis, not systemic. He was not sure Ferriprox would be effective or even work in the brain which is not truly an iron overload. I didn't realize there was a difference. Is there nothing we can do for meningeal siderosis?
Answer:
A Meningeal siderosis and superficial siderosis are the same thing. They refer to iron overload confined to the central nervous system. There are other iron overload conditions that lead to iron overload in the entire body, called systemic iron overload disorders. Ferriprox along with the other 4-5 iron chelators are all good for systemic iron overload disorders, but Ferriprox is the only one that can get in to the brain so it’s the only one we’ve tried for superficial siderosis.
Question:
My legs and hips are giving me fits every day. I'm on 10mg Baclofen 4 times a day. Does not seem to be helping. They tense up mainly in the thigh area and it gets really painful in my right hip where I have to take a very hot shower to hope it calms down. Exercise seems to make it worse. What do you suggest? It bothers my sleeping, too. I've been on Ropinirole for restless leg syndrome but that did not help either. I've been on Ferriprox for 3 years now. I've started losing a lot of hair and my fingernails are dry, splitting and peeling. Is this due to something the Ferriprox is doing? What should I do? Desperately needing advice.
Answer:
The spasms are probably due to hemosiderosis of the spine. That’s part of the Superficial Siderosis process. In addition to Baclofen, I often use anti-seizure medications like Gabapentin or Carbamazepine. You can ask your doctor for a trial (or me if I’m your doctor!). Your hair loss and brittle nails may be due to iron deficiency if you have no other explanation for them. One approach to long term treatment with Ferriprox is to try to use the Ferriprox every other month. In the off months, take iron pills to boost your body’s iron levels. It will presumably take longer to treat the siderosis but it may be more tolerable in the long run.
Question:
My mother suffers from SS she has been unable to take Ferriprox due to the cost. I am writing to see if the mediation has been approved for this disease so insurance will cover some of the cost. She really needs this medication to see if it can help slow this progress of this disease. She is almost completely deaf and it is very hard for her to keep her balance. This is causing her to lose faith and be very depressed I am really afraid for her and would appreciate any and all information that u could provide for me to try and help her. She is on Medicare and disability so there is really no money to buy it as I am disabled also.
Answer:
Ferriprox is not approved by the FDA for superficial siderosis. To get FDA approval would require an expensive trial. However, insurance companies will sometimes cover the cost of a drug that proves useful, even if the data does not meet FDA standards for approval. Our trial of Ferriprox in superficial siderosis may be one of those studies that insurance companies use. The data from the trial is currently being peer-reviewed and will hopefully be published in the next couple of months. Then it takes a few months for insurance companies to incorporate the findings and decide on coverage.
If your insurance company does not cover Ferriprox, you can find the medication at online pharmacies at a significant discount. You’ll have to check the laws in your state about ordering for online pharmacies.
The bad news is that the Medicare is only required to pay for FDA approved medications. The company has decided not to pursue a trial for an FDA label for business reasons. However, the patent protection on Ferriprox expires late this year and there are likely to be inexpensive generic options on the market.
Note from the Silent Bleed Team: Dr Levy's study has now been published, please contact us if you'd like further details.
Question:
My neurosurgeon has suggested a diffused blood patch, I am also considering Ferriprox. I am in a wheelchair and balance issues are bad, do you have any suggestions which direction I should go? I am 78 years of age.
Answer:
It depends. The course of Superficial Siderosis is generally very slow. You may not see much worsening over the next few years. Similarly, a blood patch and Ferriprox will provide a benefit in the long term and may take 3-5 years to work, depending on your degree of siderosis. There are risks to blood patches and Ferriprox that you should weigh against the benefits. I’m not trying to talk you out of it but I do think you should have all of the information before making your decision.
Question:
My partner is being seen here in London at St.Thomas/Guys and our IFR application for treatment with Deferiprone/Ferriprox has just been denied by the NHS. Their reasons were mainly that there is no substantial evidence that the drug might work for patients with SS. I would like to appeal this decision, however in order to do so I need assurance that there is some precedent for this - that there are in fact patients in the UK who have been allowed this drug on the NHS. Given that the brain trauma that caused his SS occurred 30 years ago at age 18 months (removal of benign brain tumor), we do really need the drug to be administered as soon as possible. Would it be possible to send out a plea to your members to let us know whether the NHS has financed others as well? This would give us some ground to stand on for our appeal. Anything would be a great help, even anonymous information.
Answer:
I think Facebook is a great way to connect to other SS patients in the UK!
Note from the Silent Bleed team: this is all too common a problem, sadly but it doesn't have to mean defeat. There are patients in the UK who are currently on Ferriprox, it having been funded by the NHS. Please click on the Facebook buttons on this to find our support page. You can also use our contact page and we'll do what we can to help you personally.
Question:
My son has been on Ferriprox for several years. From time to time his copper levels are low and his consultant prescribes copper supplement with the instruction to continue with Ferriprox whilst taking the 3 months' supply of copper. Reading your responses to other people's enquiries would I be right in thinking you would recommend coming off Ferriprox for a week whilst taking the supplements then returning to the Ferriprox regime without the copper supplement?
Answer:
Ferriprox does not bind copper. You can use copper supplements whenever you need to without stopping Ferriprox. Ferriprox binds iron and zinc (weakly).
Question:
My son, who is on Ferriprox, takes the following supplements for his retinitis pigmentosa: cod liver oil (with vitamins A and D), lutein, Tudca (tauroursodeoxycholic acid) and astaxanthin. Are these all ok to use with Ferriprox?
Answer:
I think so, yes. The only things I know of that you can’t take with Ferriprox are vitamin C, zinc and iron.
Question:
My wife was diagnosed with SS some 7 years ago. It is the result of a car accident at the age of 12 (she is now 57!). She has the classic symptoms of hearing loss, ataxia and bladder problems. Her ability to walk has deteriorated over the last 5 years and she has been in wheelchair for 18 months. We have found an excellent (private) neuro-physiotherapy clinic which has helped in preventing further deterioration, if not a slight improvement, in her mobility. She has been on Ferriprox for 12 months now after we highlighted Dr Levy's study to our consultant. He has been unable to get funding for the drug (Trust/GP/NHS England) so is prescribing this privately, although monitoring (blood tests, MRIs etc) under the NHS. He thinks that the chances of getting Ferriprox approved for SS will be improved if Dr Levy's trial results are positive. The web page on /react-text ClinicalTrials.Gov react-text: 242 state that the estimated completion date of the study is June 2016. Do you know if the results have been published yet? If not, can Dr Levy give an indication of when they will be?
Answer:
he trial has completed and we are in the process of analyzing the data. It is taking longer than expected because we are developing a method of quantifying the iron on the surface of the entire brain by MRI. This has never been done before. We want to make sure this quantification process is valid before publishing it. We hope to have the results completed by October 2016. Then the results have to be peer-reviewed before they are published. That could take a few months as the experts will have to assess our MRI iron quantification process. Realistically, we are planning to present the final data at the American Academy of Neurology conference in Boston in late April 2017 and publish the results around the same time.
Note from the Silent Bleed team: if you'd like more information about the published trial, please drop us a line.
Question:
Nutritional yeast. Dr. Levy, I wanted to ask your opinion of nutritional yeast as a dietary supplement for SS patients. My father suffered from Guillain Barre Syndrome in the late 70s that put him in the hospital for 8 weeks. As a part of his recovery, my mother introduced him to nutritional yeast, and he accredits nutritional yeast for the totality and speed of his recovery. It is my understanding that nutritional yeast has a positive effect on the CNS. Are you familiar with nutritional yeast and what is your opinion? Thank you. I look forward to hearing your opinion.
Answer:
Honestly, I do not know if nutritional yeast has any benefits. My first thought is that we consume lots of yeasts from bread products but I am not an expert in nutrition and there may be other factors that I am not aware of. I can certainly say that it’s not harmful to consume.
Question:
Research into the use of deferiprone Dear Dr Levy, briefly, my wife is a SS sufferer resident in the U.K. During a recent visit to my wife's consultant we were told the individual funding request made for the use of deferiprone in treatment was likely to be refused on the grounds of a) cost and b) the lack of conclusive evidence that deferiprone is an effective drug in the fight against superficial siderosis. It is intended to appeal the decision but obviously (a) would be easier to overcome if there was stronger evidence for (b). The U.S. ClinicalTrials.gov website indicates you have undertaken an "Observational Study of deferiprone in the treatment of SS." The estimated study completion date is shown as June 2017 but I have been unable to find any further information as to results. It would be appreciated if you would state: 1. Has the study been completed? 2. If so when will detailed results be published? If results have already been published would you let me know where they can be found or, perhaps, provide a copy of any publication? 3. If there are no plans to publish in the near future can you at least give an overview of the results, either positive or negative, which may assist in dealing with the NHS in the U.K. I am sure sufferers in other parts of the world will be interested as well. 4. In your opinion is the sample size of participants in the study trial sufficient in your mind to draw any firm conclusions as to the effectiveness or impact of deferiprone in patients suffering from SS. If so what are these conclusions? Your help would be much appreciated.
Answer:
The study has been completed and we published the findings last month. I made the study available to the Silent Bleed and we are in the process of trying to figure out how to make it available publically. You can find the summary at this site: https://www.ncbi.nlm.nih.gov/pubmed/29285884.
This was not a blinded study with a control group. Rather, this was an observation of 38 patients with siderosis who used Ferriprox and we are reporting the findings. There is a big difference scientifically and we cannot make the claim that Ferriprox works for SS patients even though it appeared to be effective in this trial. However, because SS is so rare, the NHS may accept this study as sufficient. Another thing to consider is that later this year the patent protection on Ferriprox expires and less expensive generic drugs will be become available.
As far as my understanding goes, this is available to those with SS only due to copyright.I do not own the copyright to the paper; the journal does. So if it were up to me, I would make it available to all!
Note from the Silent Bleed team: please get in touch with us for access to the paper, many thanks.
Question:
SS and Blood Thinners
Hello, I am a long time user of Warfarin. 22 years. I have had 2 pulmonary embolisms, 1998 & 2008. I had 2 brain bleeds in 2017. I also have 2 mutations that make me 6 to 8 times more likely to have clots. My doctors want me to switch to Eliquis. I plan to switch in a few weeks. I just wanted to know what Dr. Levy thinks? I have been to Cooper, Jefferson and Penn. Not one neurologist from the three ever had a patient with SS. I have an appointment at Johns Hopkins March 29th. Hopefully I can some answers and treatment.
Answer:
I wrote to you separately in an email. But to give some general advice to the community, I do not think there is much difference in the safety of different blood thinners in SS. Warfarin vs Eloquis and the others are all probably the same. They all make it more likely to bleed. But just like all medications, the risks need to be weighed against the benefit. For some people, stopping blood thinners will lead to blood clots and death; therefore, even if it worsens the SS, you have to continue taking them.
Question:
SS diagnosis and any value to treatment with Deferoprone
Would you support treatment with Deferaprone?
I have bee trying to get a teleb-appt with you for several weeks. Sadly, I found out on Tuesday, October 12, the Mass Gen is no longer permitted to do them.
I have a march 2020 SS diagnosis. It began in 2013 about a week after my 7th major spinal surgery. My first bleed in 13 eas followed by hearing loss in 14, balance issues
in 15, memory problems, brain fog and neuropathy in both feet around the same time. Things have gotten much worse during the pandemic.
My recent tests say it is static. My question is this. Would you support treatment with Deferaprone?
Dr. Ku:ar at Mayo says I have the worst spine he has ever seen.
Thank you four help
Answer:
The decision to use deferiprone to treat superficial siderosis depends on many factors including accessibility/cost, side effect profile (mainly fatigue), other co-morbidities. It also depends on your
age because in many cases of heavy siderosis, it can takes many years for the deferiprone to remove the iron deposits. In my experience using this chelator, it does work to remove iron deposits from the surface of the brain, albeit slowly. However, sometimes
the damage that was done by the iron toxicity is not easily repaired. Often, I set an expectation that deferiprone will be used to slow progression rather than to improve neurological function.
Question:
Should I be on Ferriprox? I had brain surgery and have cavernoma and have a bleed on the brain. I' waiting to hear back from my neurologist.
Answer:
A one-time bleed from a cavernoma may not cause superficial siderosis. Bleeds in the brain from all causes are very common, but superficial siderosis is very rare. We posit that superficial siderosis arises from repeated bleeding over a long period of time.
Question:
Someone suggested trying Parkinson's medications for tremors. What are your thoughts about this?
Answer:
It’s possible that medications developed for other purposes like Parkinson’s disease may work to ease symptoms in superficial siderosis. Many times in neurology, we use medications for all kinds of off-label indications. For example, we use lots of seizure medications for nerve pain. That’s because some of the mechanisms of action are shared. The mechanism behind Parkinson’s medications is to increase the amount of dopamine available in the brain. While I don’t think this mechanism is involved in Parkinson’s tremors, it’s not harmful to try the medication and see it helps.
Tremor is not a typical symptom in superficial siderosis but if that’s part of your disease course, please let the rest of the group know what medications work for your tremor.
Question:
Steroid therapy
My question is based on my past experiences with what I now consider to be flare-ups of SS. I've sought medical attention for years for head aches, lack of balance, numbness in hands, feet, weird behaviour, etc. I always ask for Decadron, because, in past, it has always seemed to clear things up quickly. Some docs will prescribe, some won't, but it's the one thing I always ask for nonetheless. Which leads me to ask...what is the
role of inflammation in the SS process, and would steroid therapy be a way to help SS victims through their more difficult times?
Answer:
There is some inflammation after bleeds. Bleeding into the spinal fluid can cause an arachnoiditis which is painful (headache and back ache) and associated with fever and stiffness. Steroids can help for
that. However, we don’t know if steroids have a long lasting benefit to reduce the iron deposits and reduce superficial siderosis.
Question:
Superficial hemersiderosis
I've had the phyzer vaccine in January 2021 since then 3 weeks later I couldn't move my right arm it was frozen I could not move it at all ... since then I very had a lot of things happen I had MRI scan and SS was diagnosed. They think the vaccine caused an allergy for me.am I able to fly with SS.
Answer:
This is an unusual way to get a diagnosis of SS. I suspect it had nothing to do with the Pfizer shot or the frozen shoulder. But if it led to an MRI, at least now you know about the SS and can try to address it before it causes symptoms. There are different types of superficial siderosis – including both harmless and harmful types so it’s important get some expert advice in your case. Yes, it’s safe to fly with SS.
Question:
Vibrations. Hi My mum complains of a feeling of vibrations. She feels it quite often and feels like she can’t get away from it. Is this someone that is related to SS?A: It could be related to siderosis.
Answer:
A sensation of vibrations is a form a neuropathy which can be due to siderosis of the nerves entering the spinal cord. Sometimes patients benefit from gabapentin or carbamazepine.
Question:
Why have people been told different times to take Ferriprox? Who is right....we're all taking different times?
Answer:
The issue regarding times of the day to take Ferriprox is based on absorption. Ferriprox binds to iron, vitamin C and zinc. If you consume any of those with your Ferriprox, they will bind together in the gut and the drug will not be absorbed into the body. The manufacturer recommends waiting 2 hours before and after Ferriprox before consuming anything that contains Vitamin C, zinc or iron. If you want to know if the Ferriprox is being absorbed, you can check blood levels of ferritin which should decline slowly over the course of the year
Question:
Zinc levels are supposed to be between 60 and 130 according to the lab. My level is 61. Should I be concerned? None of my doctors who are supposedly watching my levels have commented on this. My ferritin levels are now 5. Need some guidance.
Answer:
There is no concern in a low-normal zinc level. It’s still way more than enough. To boost your levels, stop the Ferriprox for one week and use a zinc tablet 2-3 times a day for a week. Your levels will bump up quickly. It’s good for you to have a low ferritin. It means the Ferriprox is chelating your body’s iron.
Question:
if a stroke patient can be rehabilitated to use their arms and legs or other body parts damaged from a stroke, is there a possibility that we as SS patients can retrain our brains to regain balance and muscle function?
Answer:
Yes! Rehabilitation takes advantage of your brain’s ability to generate new circuits and learn new tricks. Whether your damage is from stroke, trauma or siderosis, this phenomenon holds true. I highly recommend physical therapy, occupational therapy, vestibular therapy or whatever other targeted therapy you need to improve your neurological function.
Question:
if a stroke patient can be rehabilitated to use their arms and legs or other body parts damaged from a stroke, is there a possibility that we as SS patients can retrain our brains to regain balance and muscle function?
Answer:
Yes! Rehabilitation takes advantage of your brain’s ability to generate new circuits and learn new tricks. Whether your damage is from stroke, trauma or siderosis, this phenomenon holds true. I highly recommend physical therapy, occupational therapy, vestibular therapy or whatever other targeted therapy you need to improve your neurological function.
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