September 29, 2007 - Terry - Hastings, East Sussex (England)
Relationship to NBIA individual: mother of Paul

Paul has continued to take 5000mg B₅ daily and things are changing. We went to London in July and Paul's DBS stimulation was changed very slightly to help his mouth. This it appears to have done. Paul is now able to walk better than he has for over three years. Paul still freezes and falls but he can now walk uphill again. This improvement has been  gradual so I feel it may be due to the B₅ rather than the DBS.
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July 13, 2007 - Terry - Hastings, East Sussex (England)
Relationship to NBIA individual: mother of Paul

Paul started on B₅ after the conference on May 10th. He started on 1000mg a day ie one 500mg tablet in the morning and one in the evening. Paul doubled his dose weekly and since the 7th of June he has been taking 5000mg per day. 5 tablets in the morning and 5 in the evening. We have not really noticed any difference but Paul will continue to take it for a while. Paul's only problem since the conference has been a deterioration in his mouth, his swallowing is not so good and he is dribbling a bit. We have seen his doctor in London and he tried changing his DBS settings but this has not helped. We are returning to London on 18th July so we will see what happens then.
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January 11, 2006 - Joan - Las Vegas, NV (USA)
Relationship to NBIA individual: mother

My son was diagnosed in June of 2005. He had few physical symptoms but we noticed a few ticks and changes in personality. I started him on 500 mg of B₅ and gradually I have increased it to his current dosage of 10,000 mg per day. He takes 2500 mg 4Xs a day. He has not complained of any stomach aches or anything. I really don't know if it has helped or slowed down the progress but I will continue giving it to him. The doctor has assured me it is probably going out in his urine and down the toilet. There once was an "unidentified substance" in one of his urine tests so we took  him off B₅ for a week and the next sample was fine.
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January 1, 2003 - JianFeng Xu - Guangzhou, GuangDong (China)
Relationship to HSS individual: father

My son is 4 years old and is diagnosed with HSS. I tried to give B₅ 750mg/day for 1 mouth, but there was no response.

December 1, 2002 - Anabela - Niskayuna, NY (USA)
Relationship to HSS individual: mother

I have two daughters Sabrina who is 8 and Alyssa who is 6. They both have HSS. We have tried B₅ for months now and with no results. This is the second time that we have started with B₅ then stopped. We went up to 2500mg per day and they both complained of stomach pains. Sabrina was always in constant pains that would not go away. She was always uncomfortable then during her G-Tube operation doctors found an abnormality in her stomach lining and decided to do a biopsy and discovered that she had H-Pylori (a stomach bacteria that can cause ulcers). Has anyone had any of the same conditions? I would like to hear from you. Also, has anyone experienced a lot of cold sores in their mouth? Especially on the tongue? Sabrina gets them so often that I wonder if it is a reaction to the medication or not? My email is fbela65@hotmail.com.
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July 9, 2002 - Martin Family

Hello, We inform you that Lydia is taking, from September 01 to April 02, acide pentothenate (1000 or 1500 mg), without any result and we stopped to start with Vitamin B₅ (in powder) with dosage of 5000mg a day, (from April till now) without change of state. We would like to know first, what you think about the one or the other vitamin and those dosage that we are giving now and secondary, if you advice other medicament. With our best wishes. Family MARTINS

Reply from Dr. Susan Hayflick regarding this post:
It sounds like the Martins have tried two different forms of pantothenic acid. The pantothenate they tried first was most likely a pill form. Then it sounds like they stopped and started a larger dose of a powder form. B₅ and pantothenic acid are the same thing. It shouldn't matter if Lydia takes it as a pill or a powder. The body still breaks it down and uses it as the same chemical.

There may not be any change in condition even with high doses of pantothenic acid. This supplement is probably only effective if there is a small amount of enzyme working in the body. Some patients with PKAN have no enzyme, and the supplements won't help.

It is best to start with a small dosage and gradually build up to a level (not exceeding 5000 mg/day) that can be tolerated. If experiencing diarrhea or stomach discomfort, the dosage should be gradually reduced to a level which is better tolerated. Information about the dosage best for you or your loved one should be discussed with a physician.

It can be useful for the group to hear personal accounts, as we have heard that B5 helps some patients and not others. There have been no formal studies to determine if B₅ really works, and for whom it works.
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June 26, 2002 - Cuxhaven, Niedersachsen (Germany)
Relationship to HSS individual: I have HSS

I just wanted to let you know that my blue/green marks on my legs went away since I got Grape Extract. The German Grape Extract didn’t work anyway, so we bought the American at Vitamin World, and my marks went away. Our non-profit organization is working from now on, so let me know, if you got more information. Greetings, Saskia
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May 27, 2002 - Thomas - Hagen (Germany)
Relationship to HSS individual: Dear Saskia...

Original submission in German: Hallo Saskia! Ich habe den Bericht über Dich und Deine Krankheit bei Akte 02 gesehen und bin von Dir und Deinem Kampf gegen diese heimtückische Krankheit sehr beeindruckt! Mach weiter so!! Alles, alles Gute. Thomas Sorry, but my English is not so good. I wish Saskia (and all others) hope and good Luck.

Translation: I saw the report on you and your illness in document 02 and am impressed by you and your battle against this very insidious illness! Thomas
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May 14, 2002 - Saskia - Cuxhaven (Germany)
Relationship to HSS individual: I have HSS

Dear Patti, I got Grape Extract, and my condition seems to stand, from the time, when I wrote you first time! I am very happy about it, maybe you can tell others from my experiences with the grape extract! thank you, I hope to meet you soon, bye. greetings from Germany!
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May 7, 2002 - Carol - Blackstock, On (Canada)
Relationship to HSS individual: mother

My son started taking 500 mg. daily of B₅. We increased about every two weeks and he is now taking 3000mg. daily. So far we have not noticed any improvement. His condition seems to have worsened in the last several months.
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April 5, 2002 - Anna - Sugar Land, TX (USA)
Relationship to HSS individual: cousin

I wrote Dr. Hayflick some time ago about my 8 year old cousin in Italy, Luca, who was diagnosed around age 5. Since then, we have sent to his mother supplies of the B₅. Prior to taking the B₅, he was not able to move any parts of his body, was always very sad, and was at the point where he could not swallow. In December, he started taking 500 mg per day and after a month, went up to 1000 mg per day. He is now able to swallow and seems to be more alert and happy. However, he is still not able to move about and uses only two fingers to grab. We are also debating whether we should try increasing the dosage.
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March 18, 2002 - Saskia - Cuxhaven, Germany
Relationship to HSS individual: I have HSS

I’m taking every day 10 times 500 mg of Vitamin B₅, we started with 500 mg, then we gave me more until I got 10 times 500 mg a day! I was not able to walk, not able to stay standing, and not able to sit, now I can walk almost ordinary, and I can stand a longer time then before, now I would just wish that I can someday sit again! I would be happy to get an E-mail from you! saskia983@compuserve.de 

• After reading Saskia’s experience (above), Patricia V. Wood, President of HSSA, emailed the following questions to Saskia:
  "Thank you for sending in your B₅ experiences. That is very positive that you had such good results. At what dose of B₅ did you first start noticing a difference? How long have you had HSS and at what age did it start? Has your speech been affected by the disease and did the B₅ help in that area? Have you had any side effects from that large dosage? Many of the families have complained of stomach problems with much lower doses - has this been a problem for you at all?”
    
• Saskia responded to Patricia's email with the following:
  “Dear Patricia Wood! To your question: Yes, my speech changed when I was 14, but nobody cares, cause they never thought that I could ever have a disease like that! I have HSS since May last year, it started in May, when I was not able to walk like anybody else! At about 4000 mg a day it started getting better, but every time we gave me a larger dose, it seems to get better, but two or three days later, I am doing bad as before, there are good and bad days. I am waiting that there will be only good days for me! Yes, I had stomach problems, too! I have a question to you: Can I take more than 5000 mg a day? I thank you, for writing back, Saskia.”
    
• Patricia then asked Dr. Hayflick (researcher who discovered the gene) about the level of B₅ and if it could be increased to more than 5000 mg of B₅ per day?
  Dr. Hayflick’s response to Patricia’s question:
  "There really is no medical experience with the dosing of B₅ in PKAN, so we are in uncharted waters. It is important to be prudent about use of any medication and be certain that decisions are made with a physician.
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January 5, 2002 - Hilde - Aartselaar, Belgium
Relationship to HSS individual: mother

Since last October we have been giving Stijn our youngest son of 8 years 500mg vitB₅ and this twice a day. Since December we noticed that his speech improves and that his motor system is more controlled. We would really like to thank you we hope to hear very soon about other positive results. best regards, Hilde.
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January 5, 2002 - Sabrina - Marion Center, PA (USA)
Relationship to HSS individual: Private duty nurse, friend

I am happy to report my fiend is doing lots better. Started her back on 250mg with g-tube feedings. And her brother is still refusing to take meds as they are crushed and he spits it out so we will try the liquid in a drink for him. My e-mail is now potts@yourinter.net
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January 3, 2002 - Sabrina - Marion Center, PA (USA)
Relationship to HSS individual: Private duty nurse, friend

Hello from Sabrina my second time to write. This is a blessing for me as I am a nurse and friend of 2 special children with this disease.  E. is 17yrs and D. is 7yrs. Could someone let me know where to get liquid B₅ as little D. refuses a lot of the time to take his meds any way we hide them. My e-mail is dsplpn@yourinter.net. Any advice is appreciated as well as any support I can offer others. I will keep you updated on E. and D. The severe abdominal pain has past and we will start E. back on her B₅ at 250mg and go from there.
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December 30, 2001 - Norma - Milwaukie, OR (USA)
Relationship to HSS individual: mother
 
Started at 250mg in early August, increased at two week intervals to 1 gm. At this time daughter thought she had better balance and talked a little more clearly. I have not noticed this so it was short lived. After seeing Dr Hayflick the dosage was increased gradually every two weeks and is now on 5gms a day which is the maximum recommended. I don't see any improvement. She has had no side effects from the medication.
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December 29, 2001 - Sabrina - Marion Center, PA (USA)
Relationship to HSS individual: Private Duty Nurse (friend)
 
The family started giving their 2 children ages 17 and 7, 250mg. Increased it to tid then 500mg increased over two months to tid within 4 months. Stopped it for the 17 year old girl due to severe abdominal discomfort last week. This also occurs with pms symptoms. The 7 year old has never talked coherently he did seem to have better balance the past few months although his chewing is becoming worse at times.
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December 11, 2001 - Stan - Calgary, AB (Canada)
Relationship to HSS individual: family friend
 
Vitamin B5 is widely accessible in health food stores (i.e. 'Healing Alternatives') here in Canada. A 100 capsule (500mg each) container costs approx. CAD16.00
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November 8, 2001 - Fr Jacek - Gdansk (Poland)
Relationship to HSS individual: nephew
 
Please give me more information about B₅ because this medicine (vitamin) is unavailable - what we know - in Poland. It  would be great to try this way to cure my nephew.
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September 28, 2001 - Patricia - El Cajon, CA (USA)
Relationship to HSS individual: mother
 
We started Kimbi on B₅ on July 23rd at 500 mg. After two weeks we increased the dosage to 1000 mg. Two weeks later we went up again to 1500 mg where she is currently. We do plan to increase the dosage to 2000 mg in the near future.

We noticed a difference very quickly. Kimbi was more alert, had less dystonia and was making sounds like she wanted to talk. She would randomly answer questions appropriately with uh uh for no and yeah for yes. She did this approximately 10 times. She was also trying to say mama again. She had previously not said any words for at least 8 years so this was truly amazing. She also moved her one hand better and had some control. We also worked with her to use her eyes to communicate and she was opening them wide for no and closing them once slowly for yes. She never had this type of control previously.

The B₅ did seem to cause some stomach discomfort at some times, like a stomach ache, though it was not severe. She has not seemed bothered by it the past few weeks so perhaps her body adjusted.

It does seem like she has slowly lost some of these gains and the B₅ is not as effective at present. But the gift of seeing the possibilities and knowing that her mind is working and understanding has made this a wonderful experience.
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July 29, 2001 Question:  Should those with HSS start taking Vitamin B5? I noticed something called pantetheine and also pantothenic acid when I was looking for it at the store. What is the difference and which is preferable? Dr. Susan Hayflick Answers :  Based on our understanding of the enzyme block in PKAN, vitamin B5 may be beneficial. The problem in PKAN is that B5 is not converted to phospho-B5. So, the obvious fix might seem to be taking phospho-B5. However, this compound almost certainly will not be taken up by the body to be used and it seems not to be commercially available.  In PKAN, the enzyme is either not present or it is present and not functioning.  In those patients in whom it is present but working at only 1% of its usual capacity, loading up on B5 might "push" enough vitamin through the pathway to result in healthier levels of phospho-B5.  If B5 helps, it may only help in slowing progression of the disease. With a disorder that can be very unpredictable in how it progresses, this can be hard to prove.  It is certainly possible that vitamin B5 will not be beneficial in PKAN, but I suspect it will take time and patience to know. Vitamin B5 is also called pantothenic acid or calcium pantothenate. Pantetheine is a different compound and one that I think might be harmful in PKAN. In healthy people, vitamin B5 is virtually without toxicity. Subjects taking 10-20 grams per day reported only diarrhea as a side effect. While it is possible that B5 may have toxic effects in patients with PKAN, it is a water-soluble vitamin that is unlikely to build up and therefore cause problems. At this time, there are no pharmaceutical drugs or other compounds that can logically be recommended based on our new understanding of this disease. Therefore, the alternatives at this time are to try B5 or wait until we can learn more about the biochemical changes in PKAN. We have worked very hard to identify the gene and we are now focusing a great deal of effort on understanding the chemical changes in this disease. This will bring new ideas about treatments.  My recommendation for trying pantothenic acid is to start with a dose of either 250mg or 500mg orally or through a G-tube once daily. Try to keep good records to detect any changes, either positive or negative.  If there are no concerning changes after one or two weeks, consider doubling the dose by giving a pill twice a day. If there are still no problems detected after a few weeks, consider further increasing the dose, as tolerated. [Top of Page]

July 28, 2001 Question: How soon can we realistically expect any new treatments based on this discovery? Would it be possible to reverse the brain damage that has already occurred or do you think it would only be possible to prevent future brain damage? I understand you may not a have a definite answer but any ideas would be appreciated.   Dr. Susan Hayflick Answers: The PKAN gene discovery gives us ideas for new treatments. However, determining if there is a benefit from vitamin B5 or other compounds will take months or years. Being an optimist, I would say that there is much reason for hope in the discovery of new treatments for PKAN. We don't understand the process of brain damage in PKAN. It may be possible to reverse some of the damage caused by this disease as well as prevent further damage.

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