Summarized by PBSN Vice President Frank Walker
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PRUNE BELLY SYNDROME NETWORK 5th ANNUAL MEDICAL CONVENTION BOSTON, MASSACHUSETTS July 27-31, 2005
Hello friends- For anyone who is curious how this year's 5th annual PBSN convention went in Boston, here’s the long detailed version……..
Wednesday, 7/27:
Everyone checked in & we had an informal “Meet & Greet” get-together at dinnertime. Caught up with many old friends & met a few new ones. Four different countries were represented this year……….a family from Mexico with a PBS child, a PBS father from Ireland, & a PBS adult male & his girlfriend from Quebec, Canada & the rest from all over the US.
The themes of this convention were “PBSN Goes To Beantown” & “You are not the only one.” Signs stating both were all over the main floor of the hotel & shirts were given out stating both.
My little “PBS Brother” Daniel is getting big & is as adorable as ever. Been pretty healthy lately, thank God. His parents & his sisters are as wonderful & supportive as ever.
The family from Mexico have a 10 year old boy named Max who has PBS. Max is wheelchair bound & has no use of his legs & has no kidneys. Although I was told he could speak Spanish he only made sounds (no words) anytime I saw him. From what I was told he was given no outlet or access to treatment for his PBS all this time, which is why he is like he is today. I actually thought he was much younger than 10, to be honest! The family speaks very little & broken English. Luckily another couple who was there were bilingual & spent the weekend translating all that went on for them. They apparently received overwhelming amounts of info all week long & now know how & where to treat him. Max was always smiling, blowing kisses & hugging everyone……..beautiful little boy. Lynn’s husband Paul joined us on Friday & upon seeing the kind of wheelchair Max was using & how uncomfortable it was, Paul went back home to New Hampshire (90 minute drive ONE way) to bring back a wheelchair they had used for Daniel at one point that they didn’t need anymore & was much more suitable for Max. They gave his family the chair on Saturday & they were beside themselves with joy & gratitude.
Also met Jacob of Illinois, a 4 year old adorable little boy with curly short blond hair. He remembered me from last year & asked if I brought my guitar. Told him yes & he tells me he wants to play someday too!!!! :) I bought him a beginner guitar book later in the week & wrote in it, “To the world’s next rock star!” LOL! He became my buddy for most of the week & I spoke with his mom about his condition. He constantly gets painful urinary tract infections, has to self-cathaderize daily & has very little appetite. His doctors can’t understand why the UTI’s keep happening so often & aside from giving him medication, basically told his mom that he will “grow out of them”, but what does he do in the meantime? Meanwhile he’s another happy, playful little boy despite all this & anytime we went somewhere that weekend, he asked to sit with me on the bus (where he’d sometimes curl up sitting next to me & fall asleep, LOL!) & walk with me throughout the trips. I would try my best to encourage him to eat & drink “a little bit extra to get big & strong” & sometimes it actually worked! Coming home from the final trip Saturday night, he asked me if we were going home now. I told him no, just going back to the hotel but were all going home the next day. He then said “OK because I don’t wanna leave you!” And both of us well up with tears in a huge long bear-hug……….killed me!!! I’ve heard from his mom since then that they had to rush him to the hospital as they got home on Sunday with 103 fever & another painful UTI, which they have given him CIPRO for. They will very shortly insert a feeding tube into his stomach area to ensure he is fed properly, given his low appetite.
Caleb of Washington DC is almost 4 now. Back in NY 2 years ago he was dealing with PBS, kidney dialysis, & had no use of his legs. Today he walks with help of a walker, & received a donated kidney from his mother, earlier this year, but now also deals with Cerebral Palsy.
Two female PBS patients joined us this time, one adult & one 13 year old. Very interesting to hear things from their perspectives, given that 95% of PBS patients are male.
Met several other families & talked with them throughout the weekend about their own personal PBS stories, all different yet stemming from the same basics & all very gut-wrenching, some being told there was no hope early on, etc.
The 4 board members (President Kurt Walker, Secretary Lynn Brokus, Treasurer Chrissy Mason & myself) met for a while that evening to go over several aspects of the PBSN as an organization & among other things, discussed how to get more involvement from our community.
We also changed the Board-position terms from 2 years to 4 to better be able to carry out all plans made. So the next election will be in 2008.
Thursday, 7/28:
Ed Lowe’s Long Island Press article came out that morning & we pulled it off the Internet to include it in our agenda for the week. Upon my initial reading of it I was overwhelmed with its contents (still am) & was very grateful for this kind of exposure. However there was one minor misprint within it, which said that all fundraising that I’ve been involved with raised $1600 over the last 3 years when it is actually $16,000. Aside from that, it is perfect & I wrote a humbled thank you note to Ed for his time & generosity.
http://www.longislandpress.com/?cp=154&show=article&a_id=5061
After breakfast (complimentary & made to order courtesy of the hotel every morning, WOO HOO!), the morning was spent as a Vendor Fair. Several medical companies set up display tables showcasing various products they make that pertain to most PBS patients’ daily medical needs. One table was set up by a grandmother (I think her name was Joanne) of a PBS baby, which stood out for me. She wasn’t a company, just someone who wants to help. (Her grandson was also expected not to survive birth & his family was advised to abort….he’s 2 now…..go figure…..) All “PBSers” at some point wear an elastic girdle-like binder around the waist to tighten the stomach area & keeps everything protected. The ones I wore as a kid were a basic rectangular shaped simple fabric with Velcro ends to hold it together & were usually quite irritating & uncomfortable. Modern ones are still the same basic fabric but are more contoured along the sides to fit a body’s shape. But still they are not very comfortable. Joanne machine-sewed a binder for her grandson made up of soft foamy fabric, similar to what you’d see in a Sealy-Postropedic (spelling?) Bed commercial, & again contoured for a better shape. Once the surrounding shape was created, she would machine sew lines all across the binder to give it extra-risen padding throughout it, making it much more comfortable & still serving its purpose. I tried one on & it was amazing….I WISH I had this when I needed it! Getting something like this through normal insurance channels would cost at LEAST $80 minimum per binder. At her table, she had all the fabric & a tape measure & offered to measure each person (adult or child) who needed it & only charged $40 for the cost of the materials….all because she only wanted to help. She got a lot of business that day & again, brought me to tears.
After lunch, Kurt Walker spoke to the room. He & his wife Jen organized this year’s event & he works as an IT person for Boston Children’s Hospital (BCH), where he used to visit on several occasions as a PBS patient. Most of the speakers were from this hospital. He told us that the bulk of the cost of this convention was paid for by the past year’s benefits we held (which included giving 11 families $500 each toward their hotel room bills) & he made a point of acknowledging me & everyone at home for our efforts. Very uncomfortable moment because everyone stood up & applauded. He then spoke of his own letter writing campaign which raised almost $5000 earlier this year & asked for more input from our community as a whole from here on in. Our new annual goal is $25,000 to allow us to provide us the best access to help for conventions & other things throughout the year while still having some money left in the bank for other unexpected things that may come up, promotion-wise, etc.
Each speaker delivered Powerpoint presentations on each topic & our hope is to display each of them on the PBSN website ASAP, as well as ones from previous conventions.
The first speakers for the convention were a PBS mother named Mary & her 14 year old son Danny, who has PBS, from Wisconsin. Scoliosis & Kyphosis are major “side-effects” to PBS & Danny had a severe case of both, hunched back & all. Doing the bulk of the work himself, he created his own Powerpoint presentation pertaining to surgery he had a few months ago where his spine was severed & RE-attached with rods to the point where he is now in a permanent upright position, can no longer slouch, & actually gained 4 inches in height immediately after the surgery! He’s a soft spoken & shy kid, but he gave an amazing presentation & answered everyone’s questions…..His mom & we all were very proud of him!
Next to speak were 4 young “Child Life Specialists” from BCH, 3 female & one male, all in their 20’s. While all the adult talks were going on, they spent time with the kids in a separate room. They planned events for the little ones & the teens, & even separated them into PBS patients & siblings of…… They brought anatomically correct dolls with them to help them deal with their own medical needs on a “Sesame Street” level. They would each get an opportunity to “play doctor” & treat the dolls medically as they would get treated by their own doctors, to get a better understanding as to why certain things are necessary & hopefully find a fun way to deal with things, even painful ones. They were also given projects to do, including filling a box with cutouts from magazines. The cutouts were either pictures & words that they could relate to emotionally (relating to dealing with their own PBS or their siblings’) & then they would discuss what each thing meant. The conversations were staggering, according to the 4 specialists. “These kids know more about PBS than you THINK they do, so PLEASE don’t be afraid to talk to them about this. Don’t dress it down or blow it off to spare their feelings.” One main discussion with the kids was “What does PBS mean to you?” & the answers were amazing on so many levels, ranging from things like “PBS means my mom & dad taking my brother to the doctor every other day”, “PBS means doctors telling my mom that I should’ve been aborted”, “PBS means getting picked on at school a lot”. But when siblings were asked if they’d “rather not have PBS in the family, would their lives be better?”, EVERY one said no “because our brother/sister wouldn’t be here.”
One other doctor spoke that day, pertaining to Bladder Function in PBS. Kidney function is a major side effect to PBS (was for me).
After dinner, we made time for fun. Kurt & Jen Walker put together a Powerpoint Slideshow of all 4 previous conventions (I’ve only been involved in the last 3 including this year), put to music lasting about 20 minutes, finishing up with “Not The Only One.” Very emotional……..
I played a few songs live including “Only One” which is hard to do on any given normal day! Forget about trying to play it TO the people I wrote it about! But got through it best I could & we all had a good cry over it. Lots of CD’s were sold throughout the week & hopefully it will be passed on & promoted by the community. A few people did some Karaoke too & fun was had by all!
Friday, 7/29:
After another complimentary breakfast (YAY, LOL!), it was a jam-packed day…… 3 speakers while another psychosocial workshop was in progress for the kids by a different person.
The topics touched upon on this day were (please forgive my memory on some of these,........getting old & forgetful, ya know! LOL!):
Prenatal Diagnosis & Intervention- Given by a doctor who was very generous, helpful, & entertaining. There is surgery available while still carrying a PBS child, but only in EXTREME emergencies usually dealing with lung/breathing issues. Anything else can wait til after birth, no matter what level of severity. While there are still no definitive causes known for PBS, there are some recent & well thought-out educated theories being discussed…..none of which are genetic, oddly enough. I was always led to believe that PBS also caused kidney & urinary-related side-issues, but it may actually be the other way around! When ureters are blocked for many of several reasons, it is possible that THAT is causing the IN-ability of formation of abdominal muscles. Of course with funding for research into causes & treatments, better progress could potentially come forward. But I digress……….
Frontiers in Kidney Development & REGENERATION- The doctor who spoke on this topic is hoping to do away with the need for Kidney transplants in favor of rejuvenating EXISTING kidney tissue through research, but admitted this is a long way away (probably not in our lifetime). Mostly theoretical but good to know for down the road.
The Present & Future of Organ Transplantation- Presentation given by the boss of the previous doctor who hopes he will be put of business eventually, but in the meantime here is what is happening currently & for the immediate future.
The remainder of the afternoon was devoted to taking a “Duck Tour” of Boston. We rode around in duck shaped buses/boats (yes, both in one!), some of which were authentic ones used in WWII I believe, which drove us through the streets of Boston pointing out historic sites & then converted to a boat to ride us through the river & see the town from that perspective….very fun time! Afterward we all had free time to walk around the city for about 2 hours, then meet up again to ride back to the hotel. Once back at the hotel, we all just unwound….some of us hanging at the bar talking & watching TV.
Saturday, 7/30:
2 things planned for the morning, then off to Kurt & Jen Walker’s house about 45 minutes away for a BBQ.
The morning events were:
PBSN Open Forum: General discussions among the community covering several topics, including plans & ideas for the PBSN with help from its members. This was originally slated for after the medical presentation, but the flight that my doctors were taking was delayed, so we initiated the discussions while waiting for them to arrive (& my pacing back & forth in worry that they would get there too late in time to make any real impact, LOL!) & continued it once they concluded. The doctors stayed for & even took part in the 2nd1/2 of the general discussions after their presentation…& stayed to have lunch with us to discuss things privately with members.
Management of the Abdominal Wall Defect in PBS by Muscle Transplantation: My surgeon Dr. Ralph Ger is now retired & in his 80’s, but is renowned for developing & performing breakthough surgical methods, mostly dealing with muscles & unclosable wounds.
Since his retirement, much of the work he used to do is now being done by Dr. Elliot DuBoys of Woodbury, although HIS current main line of work is general plastic surgery.
Both doctors spoke on behalf on this procedure & as it turns out, Dr. DuBoys was a resident in the hospital when he assisted Dr. Ger during MY surgery back on August 18, 1982, which I was unaware of. Dr. Ger’s article on my surgery can be found here: http://www.prunebelly.org/DrGer.html
Dr. DuBoys conducted most of the presentation himself, although Dr. Ger DID speak at length as well.
Dr. DuBoys started by discussing general reasons for transplanting muscle from one part of the body to the other, usually dealing with victims of accidents or ulcers.
Once the discussion turned to PBS, I was the guinea pig up on the screen! Most of the pictures used in their presentation can be seen in the article, but quite a few were ones I never saw before OF ME! They also showed a “cough-graph”, similar to a lie detector test graph, of me pre-surgery. When muscles are intact & you cough, the graph remains still because everything is in place & still. Without the muscles, the graph is LITERALLY off the page because nothing is being protected & everything shifts during the cough. That FLOORED me, to know that was me causing that to happen, & typical of me, I was in tears through most of their presentation from being reminded of my “previous life”.
The surgery basically stems from building up my quadricep/thigh muscles, from which they took one of the 4 in each leg & moved them up into the abdominal area. The 3 remaining muscles in each leg then expand to make up the difference & there is NO loss of leg usage at ALL! At the conclusion it was a 95% correction of the abdominal area in my case & sadly, I am the ONLY PBS person to have it done.
Others who have considered it in the past have been advised against it for various “risk-factors”, which these doctors say are false! The problem is that within the medical community there are several political & ego issues to deal with, yet the CATCH 22 is that only after at least a half dozen of these surgeries are done, will it only THEN be recognized & considered worth looking into.
My doctors’ purpose for coming to us is to try to persuade individuals to do this to raise our numbers & chances for normalcy. Practically anyone can have it done, after figuring out individual insurance issues.
Several questions were raised & asked during their presentation: -The mother who donated a kidney to her son Caleb, asked if she could also donate muscle. The answer is no, only an identical twin can because they know that the muscle will grow properly in size & speed.
-How early an age can this procedure be done? They cannot get any definite answers from pediatric colleagues & their best educated guess currently is around 12 or 13 years of age, because the muscle growth won’t have far to go.
-What can be done UNTIL the age of 12 or 13? There are several COSMETIC procedures that can be done that are similar to “tummy-tucks”. The excess skin can be folded over like a binder to tighten the area & give you extra protection. The only downfall to that is that most doctors lead you to think that’s as much as they can do, but it DOESN’T fix the problem of lack of muscle! The question then becomes if someone does the cosmetic surgery at an early age, can they still have THIS done at pre-puberty age? The answer, which we weren’t expecting, is YES, ABSOLUTELY!!!! They can go back & re-open the cosmetic procedure AND transplant the muscles. LITERALLY at that moment, I & the entire room gasped!!!! NEW HOPE!!!!!!
-An adult PBS male from Canada looked into having similar surgery done, but taking muscle from the shoulder rather than the leg per his doctors & he asked for Dr. Ger & DuBoys’ opinions………..”DON’T YOU DARE do that! You will lose the use of your shoulder! You always use your legs everyday & they build on their own. We’re very surprised someone would offer that & we STRONGLY are opposed to it”.
-Another adult PBS male, 31 years old & new to our community, asked whether he needed it done…..whether it was necessary.
Dr. DuBoys asked, “Are
you limited in your daily life, medically or physically?” “Then I don’t recommend you have it done…….if you had any major limitations, especially with breathing & other body functions, then I would suggest you have it done, BUT if you want to improve on what you can do now, then yes I would recommend it then as well.” (They spoke at length privately afterward & numbers were exchanged)
-A PBS mother asked about how conditions are after surgery…..does everything function better? Dr. Duboys didn’t have an answer so I chimed in, “I can tell you YES……..you feel a difference in that something is there that wasn’t there before but recovery & rehab was not bad or long & breathing & other functions became much easier & manageable!!!!”
This discussion ran at least an hour longer than planned, but no one was in a rush to end it until everyone was satisfied. The doctors were VERY generous with their time & information & I strongly feel it is THE pivotal moment in care for anyone with PBS. It is NOW a very possible & doable reality for everyone after 23 years since mine.
Lots of people came to me with optimistic questions afterwards & I even spoke with the adult female about if she can consider this for herself. She said that at the moment, she can’t…….I don’t remember the term she used, but she has a related condition where all her insides are shifted over due to the lack of muscle keeping them in their proper place. She has to correct that first before anything else can happen, plus she also suffers from severe asthma. She also just married & would like to have children, so there’s a lot for her to do.
After lunch, we all bussed over to Kurt & Jen Walker’s house, about 45 minutes away, for a relaxing low-key BBQ, complete with a balloon walk for the kids & lots of conversation between everyone.
At one point I was holding a little 2 year old boy named Joey. He was hitting my face & playing “got your nose”, etc. He GOT it alright……his little fingernails were quite sharp & he dug them into the right side of my nose & I had to quickly put him down & get into the house without causing too much ruckus & let anyone see the blood gushing out non stop! No damage once it calmed down, aside from the fact that I had to wear a huge band aid on the side of my face for the rest of the afternoon, LOL! When Joey saw me with it on, he got upset that he gave me a boo-boo & I talked him down. How can ya be upset at THAT point when he was MORE upset about it??? ;-)
When we said goodbye
the next day & he saw the band aid was gone, he said, “No more boo
boo?”
:) Jacob was worried too, but was also relieved the next day. Too much, these kids……
Back to the hotel & again, everyone just unwound & did their own thing. Some of us again hung at the bar & chatted some more.
Sunday, 7/31: Another complimentary breakfast (LOL!) then slowly packing up & saying goodbyes. Kurt & Jen did an amazing job in planning this year. Previous conventions had a lot of info thrown at you but very little time to get to know each other. This year was the perfect mix of the two.
This was definitely the most effective & emotional convention yet & next year’s is already being planned for California! No definite venue yet, but the woman planning it knows people who work for both Knots Berry Farm & Disneyland, so those are both possibilities.
Have to figure out how & if I can afford to go there financially. Plus I’m not a big traveller & luckily despite some news reports in NY AND Boston about bomb threats in both train stations I needed to be in, everything was uneventful to & from this time! I haven’t flown in 30 years, so that, plus financials, have to be very thought out for next year.
My fellow board members are amazing people who are completely dedicated to reaching all goals for the PBSN.
Since the convention, one woman wrote in to the Support Forum saying that while we were all in Boston, her grandson was born 7/27 with PBS & died on 7/29 due to the lungs not receiving any necessary fluid.
Another woman is carrying a PBS baby & is being given the most negative news from her doctors & is begging for our help.
Many tears, but also much inspiration from it.
THESE are the reasons that the PBSN exists…we need help, attention, research, hope. There’s so much more to do. The Support Forum has become a private page on the site for members only for security reasons, but membership registration is still free & we encourage everyone to join & become active with us.
Thanks for reading & caring. -Frank W.MORE ......
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