Jasper Against Batten

We are back in the Netherlands for well over 4 months now and the dust has settled. Pretty much all the reconstruction that we envisioned has been completed, our car has Dutch license plates, and the last boxes from the relocation left our house many weeks ago.

We even had our first US visitors over. Jeroen’s best friend Cole was here with his twin sister and mom to check out our new environment.

Jeroen is doing very well in school (taking into consideration that he was in a US Montessori school for nearly 4 years). An awesome report card is probably the best evidence of this. He only needs to do a bit of extra work with regards to the spelling from the previous grade to get up to par with the rest of the group. He is also starting to understand and appreciate his new sport, soccer, better every week.

We are also reaping the full benefits of our relocation now re: Jasper.  First of all, we get a few hours per day of professional support at home (which really makes a very significant difference). The two of us no longer have to do everything ourselves.  Furthermore, we got some appliances to make things a bit easier.  We got a very nice bed that’s adjustable in height (can be tilted and is totally safe so Jasper can’t hurt himself or fall out of bed). We also got a stair lift and another special hoist, to get Jasper in and out of bath without sacrificing our backs, is on its way.

Jasper has been attending his school (day care center) ‘De Ster’ now for a while. After a rough start (he needed some time to get comfortable with the new place), he seems to like it a lot. He also gets his weekly therapy there to keep him in good shape as long as possible.   Since we tweaked his meds (after consultation with his new hospital, the AMC), he’s suffering much less from muscle cramps. As a result of this, Jasper is a happy camper most of the time.

The epilepsy is also well under control; with a seizure about every three weeks we’re still pretty close to our long term average. This is mainly thanks to our hospital, the AMC, where they went out of their way to import Jasper’s primary seizure medication Depakote from the US. This particular drug is unfortunately not available here in the Netherlands and it really means the world to Jasper in terms of seizure control.

It’s almost Christmas and despite my previous remarks about being settled in by now, this is a special time of the year and we have special memories attached to it. It is one year since we were staying in NYC for almost a month when Jasper got his gene therapy surgery at Cornell University Hospital.  Back then, we were walking through Central Park on an almost daily basis, but today we’re walking through Woerden (a small town in the very center of the Netherlands). So there have been a lot of changes over the last 12 months…..

With JAB, we have not been proactive in the fundraising due to the relocation. However, we still get donations from time to time.  Additionally, we had a very nice fundraiser back in September that was organized by Bcause. This organization put together an awesome rally with old sports cars and and made JAB the charity of the day. This generated almost 10k USD, hence a big ‘thank you’ for Bcause! Next to that, we have Jolanda who collected a few hundred euros during the final live performance of the band Nederbob (which already did a performance for Jasper a while ago); and the Protestant Church of Linschoten supported us again with a similar amount.

As for the research; at this point in time, we are working solely with the UNC, where we are funding a follow up study to an earlier one with gene therapy. We recently received some preliminary results that look (again) very promising. Next year on March 13,^th the International Congress on Neuronal Ceroid Lipofuscinoses (Batten Disease) will take place in London. Hopefully, our UNC researcher will be able to present his results there to the Batten community.  We are already contemplating which study will have to be designed (and funded) beyond that in order to make it compelling enough for the FDA and a PI (Primary Investigator) to approve and move forward with a clinical trial.

For everyone who follows us: we wish you a happy holiday season and a happy New Year!

Here is a quick update from the Netherlands. We’re back in the Netherlands for a bit over a month now and things are moving along. The trip over here with Jasper went well but in the days after the trip, he clearly suffered from the sudden change of environment / time zone which was evidenced (among other things) by a couple of seizures. However, after a rough first week, he started to do better and right now, everything is pretty stable.

After the first week, he also started at his new medical daycare center; De Ster. His first day there was not so good but after that, things have been much better! Jasper likes his new environment a lot and is doing very well. Just like the LightHouse in Chicago, he gets a lot of care and attention. One nice thing here is that he has more similar aged peers in his group.

We took Jasper to the AMC (Academic Medical Center in Amsterdam) to check in there as they have become our new primary hospital since leaving Children’s Memorial Hospital and the RIC in Chicago. Everything went well and other than adding another medicine against the muscle tightness (Baclofen), nothing changed.

In the mean time we also moved into our new place. The first month, we rented a place with some furniture.  Last week the container with all of our belongings arrived so we could finally start to settle in at our new house. With almost 360 boxes and other packages, it is a lot of work to unpack and find a spot for everything, but we’re making good progress. It’s also very helpful to have Jasper’s wheelchair and stander back after a month!

Nothing groundbreaking in the world of research but the UNC group, has already started their study that we’re (co-)funding and everything is moving along.

As for the fundraising: in July Jolanda raised over 1700 euro by participating in a Dutch walking event (Vierdaagse van Nijmege) and sponsoring JAB. This month (September 15)Bcause.nl (a Dutch organization) is putting together a business tour program and a part of the revenue generated is donated to JAB. We’re very excited about this!

On a more personal note; we’ve mixed emotions about being back in the Netherlands. One the one hand we’re happy to see all our family and friends and we love our new house. But on the other hand, we very much miss Chicago and all the people we’ve left behind there…..torn between two worlds…..

It has been a bit over two months since our last update. The relocation to the Netherlands is getting near. Only two more months in Chicago before we move back to the Netherlands. In the mean time we have made good progress on our relocation checklist:

First of all, we bought a nice and spacious house in the Netherlands (Woerden).  This will enable us to accommodate Jasper and all his ‘special needs’ tools (stander, wheel chair, swing) on the ground level of our (new) house.

Furthermore we found a really nice school (medical day care center ‘De Ster’ in Vleuten) for Jasper. This will be a natural follow up from the current Chicago Lighthouse school that he currently attends.  The same school will also provide daily support outside the regular school hours. This is something we look forward to as the care of Jasper is not very easy anymore.

We also found a great new school for Jeroen that should be a pretty good fit after almost 4 years of US (Montessori) elementary school.

Next to that we have had to research the availability of medicines in the Netherlands and find suitable alternatives for the ones that are not available there.

Last but not least; we also decided on our future hospital; the AMC (Amsterdam Medical Center) has a special division for metabolic diseases which includes also Lysosomal Storage diseases that are related to the central nervous system. We expect that this will be a perfect fit for Jasper.

So the most important components are in place, but next to the above, there are of course numerous bigger and smaller things that need to be addressed to ensure that our relocation will be a smooth one. We did the same thing almost 4 years ago so we are rather ‘experienced’ on this topic. However, the ‘Jasper component’ adds a lot of complexities to this process.

While Jeroen is very aware of the upcoming change (and, just like ourselves, has mixed emotions about it), Jasper doesn’t realize any of it. He lives in his own little world of school (Lighthouse), therapies / exercise and listening (he can no longer see) to his favorite movies.

In our previous journal post, we mentioned that Jasper bounced back remarkably after a very rough stretch following the gene therapy at Cornell in New York City last December. Unfortunately, the positive progression didn’t continue. Now, we have periods in which Jasper is pretty happy and comfortable. But we also have periods in which Jasper is totally unhappy and uncomfortable. We feel that the progression of the disease is slowly continuing despite the therapy that he underwent. In the last 2 months, Jasper has lost all his eyesight, his seizure activity has increased, he’s totally tube fed now and his hands are more and more clawing (which, together with the blindness makes it basically impossible to grab his toys). Jasper‘s sleep rhythm is also out of sync with ours; he’s usually awake at 5 am and often much earlier (3 or 4 am). This deprives us also from a lot of sleep, which doesn’t make things easier. There are still positive things though; when he’s in a cheerful mood he can still smile and chuckle about certain scenes in his favorite movies or about us making funny noises.  These moments still can make our day!

In the fundraising arena, we have our loyal supporter, Jolanda who will again participate in the ‘Vierdaagse van Nijmegen’ (a 4 days walking event in the Netherlands). We continue to receive (sometimes pretty significant) donations but haven’t been very active ourselves as we’re too tied up with the upcoming relocation.  The care for Jasper also demands more and more time and energy. Besides, we decided to take on a slower pace anyway in January (after a brutal 20 months following the diagnosis).  We also want to make sure that we spend enough time with Jasper! Nevertheless we expect to beef up things a bit more once we’re settled in in the Netherlands and some things are already in the works.

On the research front, we’ve made some progress. First, we finally got the results of a drug repurposing study that we started long time ago (and that took much more time than we envisioned).  From the 2000 drugs we tried, there was actually one compound that showed a small but significant change in the production of the missing enzyme. We have shared our results with a researcher who’s currently in the process of doing a similar study. So they can take our results as a point of departure. Hopefully something good comes out if this if a combination can be found that would further enhance the missing enzyme production to a level that it might have some therapeutic benefit for the Batten patients.

Furthermore we are going to do the follow up study with the UNC/ Samulski lab that we mentioned earlier. We did a gene therapy study with them last year and the results looked very promising. We therefore tried to do a follow up study with them through a fund matching program. We were rejected the first time but tweaked the proposal and resubmitted it. This time we had more luck and got approved. This follow up study will be used to validate the results from the first study (that was done with a rather small sample). This could potentially be a great and non-invasive therapy so we think it is worth pursuing.

Also some bad news from the research world; at the beginning of April, the US company StemCells Inc announced that they would discontinue their Batten Disease program. They did the first clinical trial with stem cells on 6 Batten patients a couple of years ago and were in the process of recruiting patients for a second clinical trial. Unfortunately, they had very strict eligibility criteria this time. This resulted in insufficient available candidates to participate in this study (in particular since they also had to ‘compete’ against the ongoing gene therapy study at Cornell in New York City). A very unfortunate event!

To end on another negative note; last time we ended our journal with the announcement that Tom, a Dutch boy, passed away. In the mean time, patients of this horrible disease continue to pass away; as recent as last week we got an email from a Spanish family that we met last year during a Batten disease family event in Chicago; their daughter Arene passed away…..Will the hurting ever stop?

It’s going well with Jasper. We have had a few rough months behind us after the surgery back in December. Our once ‘always so happy’ boy had some difficult days especially in January. He was in pain and we could only guess what was wrong with him. Slowly but surely, our brave Jasper is winning back terrain on this disease and it’s progression. Now we have our happy boy back-for most of the time anyway. He is happy and “talks” up a storm. Last Saturday, he gave me the best gift ever. Out of the blue, he all of a sudden said “Pip”. That one short word I hadn’t heard in a year and a half and craved so much. It took me two seconds to realize what he had said but Bou heard it too. He only said it one time but it made my day/week/ month. Oh, those small joys.

Jasper finally received his stander last month. It’s a huge thing that helps him to stand on his own. It will help him to get in a different position which is good for his little body, his bowl movements and social interaction with us. He does have to get used to it though and only uses it a few minutes a day. But we should be able to use it for longer periods at a time. Jasper is able to work with both his hands again. He is practicing letting go of an item he is able to pick up himself now. He does need help finding the item because his eyes definitely are worse. We suspect he is almost blind. Although I saw him squinting his eyes when the sun was right in them the other day. So apparently there is light going into his eyes. He is also practicing sitting more independently. Working with Nicole, our super occupational therapist, makes him happy again. He smiles when he hears her coming in the house. We started with a new physical therapist recently and are very happy with her. The funny thing is that she is Dutch but lives here in Chicago. She speaks Dutch with Jasper and he thinks that is really funny.

Sleeping is going well off and on…and changes day to day. He often wakes up because of his legs kicking and moving involuntary. He gets totally fed up with them but that too seems better to cope with for him.

Jasper still loves to attend the Chicago Lighthouse school and is less sad there too compared to two months ago. The school keeps adjusting to Jasper’s ever changing needs.

I do have some news to share with you. We have decided to move back to the Netherlands. It was a hard decision but while Jasper still can enjoy his family (grandparents, aunts and uncles) and they can still enjoy him, it’s probably the best thing to do. It is hard to leave the people here in Chicago we grew to love and who always supported us, behind. It’s hard because we have a good relationship with our trusted doctors and therapists and we have to find them all again in the Netherlands. It is good to go back and be with our parents, family and friends again. It’s good also to meet or meet again with people who helped and supported us in the Netherlands while we were here. But we are not saying goodbye yet. We will do that the end of July. We are now looking for a new home, schools and organizing help for Jasper.

The organization JAB will continue to exist as we remain committed to finding a cure for this terrible disease. The center of gravity will probably shift to the Netherlands but of course we will keep in touch with the researchers and other families in the US. We’re still active and last month we got a couple of nice donations and a Dutch band Nederbob did a free concert at a place in Woerden (the Netherlands) called ‘het Bierhuys’. A warm thank you for the folks from Nederbob and het Bierhuys for putting this together!!

Not many updates from the research corner. Last month was the annual Lysosomal Storage Disease conference in Las Vegas but nothing new and groundbreaking came out of that conference as far as Batten Disease is concerned. Within the Batten Family Coalition we’re again looking at a couple of studies. But nothing concrete yet. In earlier updates we mentioned that we put in a proposal at the University of North Carolina for a grant matching scheme but our proposal did not make it to the finalists. We’ll tweak it a bit and try it again in April. Hopefully we have more success this time as we’re eager to move this forward and have our preliminary results validated!

We want to end this journal entry with the news that on March 19 Tom, a Dutch boy with LINCL, passed away. He would have celebrated his 8th birthday the next day. We wish his parents, family and friends all the strength they need to face the coming time.

Jasper is still not back to his ‘normal’ pre-treatment level, but he’s still significantly better than in the first few weeks after the surgery. Maybe we’ll see some further improvements in the next couple of weeks, but it’s hard to have real high expectations on this topic.

Currently, we are having issues with the fact that Jaspers ‘ muscles are really tight and that causes a lot of discomfort (muscle cramps) for him. Of course we started to use muscle relaxers and pain killers but the results are not so great.

Anyway, last Wednesday, we celebrated Jaspers 6^th birthday. Jasper got a bulk load of postcards (and electronic cards) in honor of his birthday and he actually had a really good day.  the Sunday before, we had his birthday party / fundraiser at the LakeShore Athletic club in Chicago. Despite the fact that the Chicago Bears had to play a really important game, we still had a good turnout. A warm thank you to the (new) management of the LakeShore Athletic club, Sarah Ihmels, the volunteers from Starbucks, Bob Love and Sidney green of the Chicago Bulls and all other vendors  that contributed to this event!

Furthermore; yesterday another fundraiser  in the Netherlands. Jolanda van den Berg uit Woerden (Netherlands) and 20 of her colleagues from 4 DMS (a Direct Marketing Center in IJsselstein, Netherlands) spent a great part of their Saturday to contact about 1000 households and asked them for a one time donation. They did a great job and a 100 people actually made a pledge!

Also, on February 18th, students at a private school, Pingrey, in New Jersey will be having a special Bake Sale/Pizza Sale to raise money and attention for us. Thank you to Natalie Lifson and her friends for that!!

As for the research; in our previous update we already mentioned that we’re in discussion with the University of North Carolina (Samulski lab). They have a matching grant program for which we have applied. In March, we’ll know if we’re accepted in this program and get our study funded against 50% of the costs! We keep our fingers crossed.

Again, we have to admit that it has been awhile since we updated our journal. Sometimes we’re so busy with the ‘regular’ care of Jasper and all of the ‘normal’ things in life that we just don’t get to it. And sometimes there is just not much to say because things have been slow. Since we’re dealing with a progressive and degenerative disease we’re happy with a status quo at this point in time!

     In November, we had the LI NCL research conference that the BFC initiated and funded. The BFC is the Batten Family Coalition; a group of US family foundations that, in conjunction with the BSDRA, tries to find good science and move things forward.

The conference was a big success; all the ‘big names’ from the Batten research field were present and they all presented their latest research. There was also ample room for discussion so the scientists and the families had sufficient possibilities to challenge each other and to share views.

The preliminary results from a study with gene therapy that we (JAB) initiated and funded at the University of North Carolina were also presented. They look very promising so we certainly are going to follow up on that with additional funding!

Furthermore, it is of course, always a pleasure to check in with the other families and with some of the researchers.

After that, the month December came and that month was certainly not slow……

Around Thanksgiving we got a phone call from Cornell University. They asked for an update on Jasper’s condition.  A few days later we got an invitation to come to NYC for a re-evaluation (the first evaluation took place last spring).

We literally had just a few days to sort things out at home, book tickets and arrange hotel accommodation. We knew that we had to camp out in NYC for a couple of weeks if we would meet the eligibility criteria so it took quite some effort to sort everything out on such a short notice.

We flew out with the three of us to NYC on November 30. Jeroen stayed with his friend Cole and joined us a few days later. He had to stay in Chicago to take his Taekwondo (orange) belt test in that particular week.

On Thursday November 30, Jasper was admitted to the hospital for a 24 hour EEG, a neurological examination,  bloodwork and an eye examine under sedation.  Things worked out well and on Friday night we were discharged from the hospital. Jasper had passed the tests so we could come back on Sunday night for the real deal. In the meantime, Jeroen had arrived and he looked forward to his visits to Central Park. Unfortunately, the weather wasn’t so great on Saturday so he had to practice his patience.

Sunday night;  Jasper got re-admitted to the hospital for the pre-surgery MRI that had to be carried out the next day. They stuck a number of green little things on his head that help the surgeon later on to determine where to put the burr holes for the catheters. It made him look like a little alien.

Tuesday December 7 was the big day! Jeroen gave Jasper a big kiss on Monday night but we were both there to guide Jasper to the operation room where the procedure started at 7.30 am.  In the operating room we met the surgeon.  We had no further questions. For almost 18 months we had  been waiting for this day so we knew almost exactly what he was going to do. We gave Jasper a big hug and a kiss and then the sedation kicked in.  It was an emotional moment and I had to cry. It’s all the buildup tension that is released;  the fact that Jasper lies there so vulnerable but also the waiting, hoping, waiting, hoping, postponements and more waiting and hoping until this very moment!

Someone guided us to a family waiting room. Then it was just a matter of waiting and hoping that everything worked out well. Since the procedure takes about 7 hours in total we went outside to meet with our friend Alana (who came over to bring Jeroen to NYC and to support us) and Jeroen and had breakfast somewhere. After that, we went back to wait more.

Finally someone came over to pick us up. Jasper was already on his way to the pediatric intensive care. He would stay there until he was really awake. He slept almost the entire night but a few times he woke up for a little bit. The next morning they had to perform a post-surgery MRI to see if everything went well. Jasper was still so far out that they could do the MRI without an additional sedation. 

Then the process of waking up and being cognizant started. We knew it would take some time. Kids with brain damage  and neurological complication usually need more time than average to recover from a procedure like this.

The first few days things were going well; he sucked his thumb again and all the vitals looked good. But on Friday, we saw a subtle twitch in his face. It was so subtle that we barely noticed it. However, on Saturday the twitch was already more manifest and we brought it to the attention of the neurologists that were on duty. They were not too concerned and since everything else was good, they let Jasper go. At first we were delighted and happy to be out of the hospital.. but over the course of the weekend our happiness disappeared and we grew increasingly concerned.

Jasper took quite a few steps backward and it is hard to deal with for us. We know we have to be patient. It was a big surgery and kids with Batten are even more vulnerable and fragile.

He became basically bedridden and tube fed. “Luckily” we had the g-tube and could start to work out how that would work out best for him.

December 16, we went back to the hospital for a new evaluation that was part of the protocol. And of course to discuss the situation. The stitches were also removed from his head and that looked all good.

After a break of a few days, we went back into the hospital for the last time on December 20th. Jasper got hooked up for another 24 hour EEG, bloodwork and neurological assessment. The twitch had become slightly less.

After being released on the 21^st we went to the airport where we waited for our flight back to Chicago. The weather in NYC was fine but in Chicago the weather conditions were less favorable so we have some delay.  Eventually, we get boarded and we depart from NYC. Jasper cried a lot but eventually he became so exhausted that he fell asleep and slept for the entire duration of the flight.

When we arrived in Chicago, it was bitterly cold compared to NYC but we couldn’t care less! Never ever were we so delighted to be back home after spending more than 3 weeks between hospital and hotel!

In the mean time we’re back home for well over 2 weeks. We have much more experience with the tube feeding schedule and mechanics and the meds have been tweaked a few times. In the last few days  Jasper is certainly better than a couple of weeks ago. He seems to be more cognizant and awake, smiles and laughs sometimes and seems to enjoy watching his favorite movies. However, it is still a far cry from his pre surgery baseline.  He still cannot eat by himself, is wheelchair/ stroller bound and the twitch comes and goes (although it has become of a milder nature). We can only hope that sufficient rest, proper feeding and being back in his old rhythm will slowly bring him back to his old base level in the course of 2011.

In the mean time  the correct piece of dna has been applied to the brain cells that have been affected by the disease. Hence the production of the missing enzyme will now start in his brain. Jasper is the second kid that got this treatment this time around. So there is little or no information available concerning the further progression of the disease.

Since just a few days ago, Jasper is starting to say a few words and his neck and trunk muscles seem to get stronger every day. He is now (1/8/11) able to sit on his knees with heavy assistance and with his head up. He can put his thumb in his mouth without help and suck it really well.  He also started to say “hello daar (there)” which is a combination of English and Dutch. It came out of nowhere and he has repeated it a lot. So positive things are definitely happening.

The show must go on, so we move forward with our JAB organization to continue to find a near-term treatment for Batten. In the Netherlands,  the organization of a wine tasting event is in progress.  Here in Chicago, we have a fundraising coming up in honor of Jasper’s 6^th birthday that will be supported by The Chicago Bulls Organization.  We also are looking at new research opportunities that we would like to fund to follow up on the promising results of our first study at the University of North Carolina. There is still a lot of work to do!

We want to thank everyone for your support in 2010!  It has meant so, so much! We hope that you keep on supporting our cause and we wish you all a healthy and happy 2011!

It has again been awhile since our last update. We have enjoyed the long Summer and we are still enjoying the beautiful indian Summer! We made a few small trips in Illinois and Wisconsin and we had a couple of visitors over from the Netherlands (Parents, sister and next month a friend and co-founder of our Dutch sister foundation). So we can’t complain about a lack of attention! 

Actually, not much has changed since our last update in August. Jasper is pretty stable over the last couple of months. There are certainly good days and bad days and the difference between these can be enormous, but the overall change is minimal. Given that we’re dealing with a progressive disorder, we’re happy with this stability. 

Epilepsy is also still well controlled; 1 or 2 episodes per month and they always last a minute or less. 

In the mean time, Jasper got his wheelchair. However, we’re mentally not ready to use it yet and Jasper still does pretty well in his special needs stroller. Hence, so far the wheelchair has barely been used. But of course,  it will be just a matter of time before this will change. 

We’re still pretty active in the research field. Next month will be the first (late) infantile NCL conference ever. This scientific conference has been organized by the BFC; Batten Family Coalition in conjunction with the NIH. JasperAgainstBatten is part of this group of US families. All brains from the field will be present to either do a presentation or to participate in the discussions. We hope something good will come out of this and we look forward to it!

This month we also made it into the Dutch national papers. The Volkskrant (a quality newspaper) published a well written article about Jasper and another LI NCL boy from the Netherlands combined with input from (Dutch) doctors and scientists. 

We’re also still active on the fundraising front.  On 10-10-10, three colleagues from ABN Clearing Chicago ran the Chicago Marathon with JAB as their charity. They raised more than $7,000. And last Friday, we had a wine tasting event at Binny’s in Chicago Lincoln Park with live music from Ralph Covert. Many thanks to Binny’s and Ralph for their support!! The event had a great turn out; over 150 people attended the event. It’s very uplifting for us to see that people around us continue to support us and this cause!!

It has been awhile since we wrote our last update as we try to enjoy the summer. Jasper is pretty stabile at the moment although he has lost some more vocabulary. He is no longer able to say Bou or papa. But sometimes, a certain word comes back for a little bit. We recently started another powerful antioxidant called NAC and believe it or not, Jasper started to make more sounds right from the start. He even sometimes repeats words that we say

 Our speech therapist that evaluates his ability to swallow on a regular basis, thinks that it is still no problem to feed Jasper by mouth. Of course we have to blend the food first. We also ordered a wheel chair a couple of weeks ago. This week we got the word that it is ready to be picked up…

In the mean time the Chicago summer started. Warm temperatures almost every day with thunder storms, every now and then. Jasper is doing fine (of course given the circumstances we’re in). He eats well and although we suspect that his eyesight has further deteriorated, he is still watching his TV heroes, Dora and Boots on a daily basis. Unfortunately we can no longer ask him about what he still can(not) see .

Because we suspect that Jasper’s (in)ability to walk also has been caused by muscle tone, we are reviewing again the casting option that we brought up earlier. Jasper will receive casts around his feet and calves this week. After one week, they will redo it but stretch his feet slightly more. Eventually we hope that Jasper can easily stand on the floor with his feet flat instead of the toe-walking that he currently does.

Jasper has only three weeks of vacation this Summer. That’s a good thing since he certainly needs his regular portion of therapy that he gets at school! Jeroen, to the contrary has almost 3 months of Summer break. After that, he will move into the 2^nd grade on the Oscar Mayer school. Apart from enjoying the Chicago Summer, we’ll also try to have a little vacation somewhere in the Midwest. Next to  that, Jeroen and I will probably visit the Netherlands for a week or so in August. We won’t take Jasper along as we do not unnecessary want to trigger a premature loss of skills by some self induced ‘stressful event’.

Everything is still moving forward in the world of science. A gene therapy study with the Beverly Davidson lab that we fund is almost finished. The preliminary results look promising. We’re also still working on the drug repurposing study. We expect to have results at the end of this month.

Fundraising remains a bit slow but we are still doing nice things. We still have our action with the big Dutch travel organization and at the end of this month we are the theme in a large tennis tournament in the Netherlands.  Furthermore, Jeroen’s old school in the Netherlands organized a fundraiser. That went also very well! A big thank you for the St. Dominicus school!  Fundraising in the U.S. on the other hand looks like it will materialize again in the next few months.

We try to post an update on our website on a monthly basis but this time we clearly didn’t make it. There aren’t that many new things that are going on and we’re actually in a bit of a slow period.

Fortunately Jasper is pretty stable after some significant decline in the first couple of months of this year. He is also in a much better mood recently. I guess he sort of accepted all the new ‘restrictions’ that were imposed upon him by the disease.

Last week we visited a speech therapist who is specialized in chewing and swallowing to have Jasper evaluated. Swallowing has become a less natural thing for Jasper and this is something we monitor closely. If little pieces of food or fluid get in his lungs because of the swallowing issues, this can result in pneumonia. Obviously something we certainly want to avoid. Fortunately it seems that we’re good for now so there is no need yet to get Jasper a feeding tube. But we did put ourselves over another mental threshold; we ordered him a real wheelchair. It will probably take a couple of months before he is here but we ordered a ‘Zippie’ in candy blue.

Although we get confronted with this situation on a daily basis, it still remains almost surreal. About 1,5 years ago our life was more or less normal. Our reality now is that we have to deal with a compromised child. A child that should have already be in a ‘full time’  school program to learn how to read and to write. A child that should have friends to play soccer with. However, if our telephone rings it’s never for a play date for Jasper. And if our doorbell rings it’s never a boy from the neighborhood that wants to pick up Jasper to play in the park…Other kids his age progress rapidly developmentally but Jasper has to fight to maintain whatever he has left in terms of skill set…..surreal.

Next to this big pink elephant we have, of course, our organization JasperAgainstBatten. Everything moves much slower than we want but we nevertheless achieved a lot in the last 12 months. We already raised a significant amount of funds and we are already funding various studies / research in the US and (before long) the UK. I think we have reasons to be proud of what we have accomplished. Furthermore we are working more closely together with a couple of other family foundations in the US. Every 2 or 3 weeks we have a conference call with about 6 families. We discuss topics like science, research, funding, fundraising and publicity. Hopefully we can achieve even more in our fight against this demon if we can work together in some aspects.

With regards to the fundraising; it’s a bit of a slow period but we participated this month and last month in two major running events with JAB teams. First we participated in Chicago with the Shamrock Shuffle run. Next we participated in the Utrecht marathon. In total we got a bit shy of 10k USD in pledges. Furthermore we got a couple of nice donations from the financial world!

 A large Dutch travel agency has generously reached out to help us; they start this month with the distribution of JAB poem booklets to about 30,000 of their customers. We certainly hope that this will generate a good response!