[Marlon + Ness]

Hi Hunter,

Good to see other users of RCH4 on here. I think we are in a very lucky position being able to have this drug. I know when my wife first activated things were very stressful for me, as I had seen my wife’s two sisters and mother die from this horrible disease and so I thought I was going to have the same thing happen to my wife. Her decline was very rapid to start with in the first months and I remember the absolute panic. We were lucky to be offered RCH4 and started taking a couple of months after activation and have been using for 6 years now. Her progression then stopped and we almost take it for granted now that there is no progression and life is somewhere near ‘normal’ and so my stress levels are very good. Don’t think I’d be quite so relaxed if things took a turn for the worse again.

[Marlon + Ness]

Hi Amanda,

My wife (Vanessa) has been on a trial drug called RCH4. She has been on it for over 5 years now. Her ALSFR-S score has frozen at 45 after stabilizing when she started taking RCH4. The drug is struggling to get financial backing to get to FDA trials which is a shame but has recently been recognized by the FDA and given Orphan Drug designation. Hopefully, things move forward soon. My wife has SOD1 Familial and is the only remaining triplet after her sisters and mother passed very quickly after getting the disease. Here is a link to how well she is still walking. https://youtu.be/goobRa-3b7c

 

[Marlon + Ness]

 

Results from RCH4 can’t be denied. View the chart from their website http://www.rch4.com

[Marlon + Ness]

Results from RCH4 can’t be denied. This chart is from their website http://www.rch4.com

https://i.ibb.co/mJnqqLf/Ness-Als-Netpiccture.png

and below is my wife’s ALSFR’S chart which is amazing considering the terrible fate of the rest of her family.

https://i.ibb.co/4WJTjh4/Ness-June21.png

[Marlon + Ness]

So the suppliers of RCH4 must be supplying someone data on this drug, as I believe they have just been granted Orphan Drug status which is great news. I would say that if the FDA could scrutineer the drug and believe it has some benefits others might start to take it seriously now and get things moving.

My wife is up to nearly 5.5 years now on RCH4 and is still holding in at an ALSFR-S score of 45 which is just amazing.

[Marlon + Ness]

A Happy New year to everyone on ALS News Today. I’m very happy to have my wife around for another year. It has been 5 years since my wife’s first symptoms of this awful disease. We thought the worst once we realized what was going on because her two sisters and mother all died within 18 months of onset of MND. Lucky for us we were accepted as recipients of RCH4 (a trial drug) which we then received at no cost to us. It has proved to be a godsend because since starting the drug, her progress has halted.

Shortly after onset, we were having to move her around in a wheelchair for any distance. Here is a video of her walking today which I would say is pretty good for 5 years on.

https://www.youtube.com/watch?v=goobRa-3b7c&feature=youtu.be

Here below is a copy of her ALSFRS-S chart which is produced by the RCH4 suppliers each month. It also shows the disease timelines of her triplet sisters and mother and how short they were 🙁

https://i.ibb.co/BgCXZ6R/Dec2020.png

 

 

 

[Marlon + Ness]

Just wanted to update on my wife who has MND and has been taking RCH4 for 4.5 years now, which has been going really well for her. For those who don’t know my wifes story, she is the only remaining triplet sister who is still alive. She lost her two sisters and her mother, all to this awful disease. They all died withing 18 months of first symptoms! We searched for any kind of treatment that may have a chance of working, as mainstream was offering nothing, and still offers nothing! We dicounted loads of things we could prove didn’t work and costs loads of money. We then came across RCH4 on their website and seemed to offer something slightly different that we though was worth a chance. We applied in Jan 2016 and started receiving in Feb 2016 at absolutely no cost! The rest is history and my wife is still with me. Her ALSFR-S score has frozen at 43 as can be seen on the chart below. If you have any questions on RCH4, let me know and if I can help answer, I will.

Marlon

[img]https://i.ibb.co/QpCdp0P/Nessy-chart-Aug-2020.png[/img]

[Marlon + Ness]

ALS victims represent 0.3% of the population. Remarkably, 28% of PALS have a history of going to the gym or similar strenuous activity more than once per week. This is pointed out in the RCH4 website, also confirmed on Utube.

 

[Marlon + Ness]

Dagmar,

What my wife have noticed over the last 4 years is that if overexcertion in any kind of exercise is done, she will then find that she suffers cramps that night and can notice a degrade in strength in that area over the following few weeks. We have had this happen several times. We have now learnt to take life as easy as possible and it really helps. Ness still gets around and so keeps active but we just avoid over excertion. My wife is not the best eater and so my be linked to her body just not getting the instant energy required when aready depleted muscles need the extra energy to compenate. One thing that seems very common is that most people who activate with ALS are sporty people who train. The charity who supplies us with RCH4 has always said to us is that you rarely find a large person come down with ALS which seems very true (my wife was a runner)

ANyway, I just wanted to update on Nessy’s progression. We are up to 4.5 years on RCH4 and the progression is still halted which is great news for us. We are convinced that the drug is doing a great job for us, especially when compaired to the rest of her family who all passed away with 18 months of on-set. I’ll try and upload her ALSFR-S chart below.

[img]https://i.ibb.co/G3mVCq2/Ness062020.jpg[/img]

[Marlon + Ness]

Hi all,

It’s been a while since I reported in on this forum but I’m happy to report my wife’s progression is still halted and we have just gone past 4 years on RCH4 which is awesome when my wife’s two triplet sisters and mother all died within 18 months of onset of this disease. Ness is leading a reasonably normal life but has lost some strength and stamina in her legs, but this has not got any worse in the last couple of years. I see a few comments on exercise on here and I just wanted to add our experience. We have found that if you exert too much, it seems that remaining muscles get over exerted and actually progresses your disease further in those muscle groups. We went on a holiday to Sydney a couple of years ago and Ness ended up doing loads of walking. We found that at night she really suffered from cramps and her legs weakened over the next couple of weeks and she never got that strength back. We now manage any exercise very carefully and have had no more progression (obviously this is also helped by RCH4 as well). Since doing this Ness has not had any cramps or twitches. Diet is also very important and we have found as advised by the charity that sends us the RCH4, that the high carbohydrate foods are good for you. Dieting and eating salads does not go good with ALS – you must battle loosing weight. Initially we were asked if Ness is vegetarian as well, because allegedly this also does not sit well with ALS and surviving as well. Now this is just advice from our RCH4 supplier, but we have found by following the advice, my wife is still on the planet so I’m very thankful

[img]https://i.ibb.co/LRkY63X/Jan-2020.png[/img]

[Marlon + Ness]

Jean-Piere,

You are correct, my memory let me done. I just looked up the actual gene test and it is  p.lle114Thr. I’ll try and put the gene test on here, but have not worked out how to attach pictures yet

Marlon

[Marlon + Ness]

Here is Nessy’s ALFFR-S chart for the last 3 years with her sisters and mothers timelines on as well, which shows a good comparison between her and her sisters.

[img]https://i.ibb.co/8MvGgZM/Nessy-June-19.png[/img]

[Marlon + Ness]

Hi there,

My wife has been on RCH4 since Feb 2016 and we believe that it has halted her progression. I see there is a bit of interest on the forum here so if you have any questions let me know. My wife’s story is here if you want to read about it https://rch4als.com/our-stories/f/vanessa%E2%80%99s-story

The reason we think RCH4 is having an affect is because my wife is one of three medically identical triplets. All diagnosed with familiar SOD1 thr114lr varient. Her two sisters have already died and went within 18 months of first symptoms. Their geneticist proclaimed that Vanessa will follow a similar timeline. Vanessa (Ness) is still walking around and functioning nearly normal (just a bit slower and tires easily) nearly 3.5 years later. If anyone wants to talk to us, send a message or ask away about our experience.

Marlon

[Marlon + Ness]

Jean Pierre, RCH4 does indeed have Orphan Drug Designation (ODD) status. That is a different thing to Marketing Authorisation (MA).
ODD is a major step in getting marketing authorisation.

Go to https://www.accessdata.fda.gov/scripts/opdlisting/oopd/
Put in RCH4 and you will be seen that it is certainly designated.

[Marlon + Ness]

https://www.accessdata.fda.gov/scripts/opdlisting/oopd/detailedIndex.cfm?cfgridkey=795520