Crohn's Support Network - CSN IS RELAUNCHING WITHIN THE NEXT COUPLE OF WEEKS!

In Autumn of 2009 I created an online support network for people, along with their loved ones, living with Crohn's Disease or another Inflammatory Bowel Disease (IBD). The reception was astonishing and we grew fast to have thousands of members from around the world!

You can join at the new address www.CrohnsSupportNetwork.com (CSN). JOINING IS FREE (we gratefully accept donations) BUT WE HAVE BEEN CLOSED TO NEW MEMBERS FOR SOME TIME! I AM EXTREMELY PLEASED TO ANNOUNCE WITHIN THE NEXT TWO WEEKS A REVAMPED CSN WILL BE OPEN TO THE MASSES!!

-Jason

An Update, a Return, and a THANK YOU!

Hello Everyone ...
I wanted to first off say thanks everyone for all the continued support, and kindness. It is the amazing people in my life (my amazing father, and incredible friends) who have helped keep me positive, and helped keep (if not increased) my strong drive to fight. Since discovering I was in end stage liver failure in Oct 2010 it has been a tough road of up's and down's. While being worked up for a liver transplant some issues, and eventually (finally) some diagnosis's happened. It became harder and harder to breathe so the doctors thought it was a syndrome related to liver failure. After many (MANY) procedures and tests it was discovered the nerve to my diaphragm (phrenic nerve ) was no longer working - causing a paralyzed diaphragm. This causes pressure on my lungs, and de-oxygenation from the diaphragm not allowing them to inflate properly. I am on constant oxygen along with an external breathing device called an AVAP machine. It pushes pressure into my body forcing the diaphragm up. It has a very delicate sensor in it that changes pressure depending on my breathing. I only have to use  it when I feel tight, short of breath, and always when I sleep. After three sleep studies the results were disturbing - showing I stopped breathing around 130 times a night. I was also never entering REM sleep causing extreme fatigue.
As time went by I started developing other symptoms of what seemed like nerve issues. I was getting contortions along with muscle seizures. They tested my leg which showed a great deal of abnormalities; an MRI was then performed of my lower back. The MRI showed an incredibly rare condition called Epidural Lipamatosis (EL). EL consists of many hard tissue masses which form in the body - but extremely rarely in the spinal region. The masses are crushing the nerves in my back from the T-12 (mid back) all the way down to my Thecal Sac (right where the spine ends at the hip/pelvis). Due to the nerve damage it became incredibly difficult to walk, since the nerves in my spine which control my legs can not support weight anymore w/o buckling. I have been wheel chair bound a few months now. I also moved into a wheel chair accessible apartment; something that has made my life much easier as I have access to everything.
The kicker unfortunately is due to the paralyzed diaphragm surgery of any kind is out of the picture. The transplant team and other doctors agree that being int-abated during surgery would lead to very bad results. I would either never have the tube removed, and in the case of the transplant not survive the surgery. I have been taking care of myself, and the doctors follow me closely via blood work and frequent check ups.
The time will eventually come where a serious decision will have to be made ...but I am nowhere near there right now. As I mentioned earlier I have good days and bad days ...but truly the good outweigh the bad. I refuse to roll over and give up which has helped keep me here. I am just grateful that through all of this I have some truly great experiences, and I know it could always be worse.
I have returned slowly to The Crohn's Disease Support Network (CDSN) on a regular basis...something I am very excited about. I also have been offered some truly amazing opportunities (more to come on this at a later date...once everything is cemented). It has been fantastic to return to CDSN! The warm responses have been incredibly humbling, sweet, kind, thoughtful and so much more!
I hope this message finds everyone well! I have rambled on for so long you probably all need a nap after reading this haha. Thank you again from every fiber in my body!
Always - Jason

What A Long Strange Trip It's Been ...

Hi everyone it's been a while since I've posted an update of what 's been going on. I hope everyone is doing well, and life is treating them right. In my last blog posted I mentioned an MRI of my lumbar indicated a rare condition called Epidural Lipomatosis. This is when masses form around the spine compressing it. In my case it is severely compressed from my T-12 down to my Thecal Sac (which is nearly completely compressed). The nerves are damaged to the point that the compression has effected the part of my spine that controls my leg muscles; my legs have become severely weakened and can no longer support weight. I have been wheelchair bound now for about 3-3.5 weeks. It was an incredibly fast change, but I am adjusting. It has actually made life easier because before the wheelchair I was using a walker, and basically dragging the lower half of my body. This was putting an immense amount of pressure on my back, and left foot - causing excruciating pain. The downside to all this is I need to now look for wheelchair accessible living. The condo I live in now I am restricted to the main floor - which only has one half bathroom. All the bedrooms and full baths are upstairs - while the washing/drying is downstairs. My dad and I are currently looking into places to live. On top of all that it just isn't safe where I am living now, plus my loved ones are concerned about my safety. My doctor also insisted on a nurse who comes to the house and monitors my health situations, since I have so many serious medical issues. I have also started physical therapy and occupational therapy so my muscles do not atrophy. It has been tough but the way I look at it is if I get better that would be amazing...if not then I adjust and life keeps moving forward; either way life must keep moving forward.

As far as the severe rigidity I was experiencing an Endocrinologist discovered my calcium levels were critically low, and my vitamin D was undetectable from a blood draw. Getting infusions and taking mega doses of supplements has incredibly helped. I hope to soon be on maintenance doses which I will probably be on forever. It is wonderful though to not feel like I am turning to stone. The muscle contortions and seizures though have continued. The doctors feels it is related to the nerve damage I have in my back, as most of the contortions are mostly lower body.

I know quite a few of you have been reaching out to people and asking how I am. I am incredibly touched and grateful - but please respect these people as they have a lot going on themselves. If anyone ever has any questions you can also always email me at CrohnsDiseaseSN@gmail.com It would greatly be appreciated if you take these methods instead of reaching out to others.

I can't think of anything else going on - but it definitely has been a long strange trip .... and my life has taken many changes in a short amount of time. I don't handle change well - so it has been challenging at times, but the support and outreach from many has absolutely made it a lot easier; I can't thank you all enough. I will update more as I reintegrate back into the normalcy of life, but it is going to be a slow process. I really am so grateful and wish everyone nothing but the best. More to come soon .... - J

The view from my hammock .... rough eh? :-P

How Can One Person Have So Many Medical Issues?

Well I have to say I'm very frustrated, a bit depressed, and honestly getting angry. As I broke down in my last blog of all the issues going on - yet another has popped up. I had am MRI of my lumbar (lower back) because of the findings the EMG indicated of my leg. The MRI definitively indicated a very rare disease called Epidural Lipomatosis (EL). EL is actually so rare there is hardly any info to be found on it via the internet. What is happening is a 1/4 of my spine (section T12 Vertebrae to Thecal Sac ) are masses of tumor like fatty tissue compressing the nerves and narrowing the Thecal Sac.  This is causing painful back pain, leg pain, and unfortunately weakness. The main treatment for this is spinal surgery - something I can't have due to my paralyzed diaphragm and liver failure. I see the neuro muscular doctor again on Tuesday May 21st, and then a spinal surgeon/specialist upon referral.

I truly am having a problem wrapping my head around all this. I just do not understand how ONE person can have so much fraking wrong with them; it seems like a big cosmic joke. My father and I are currently looking into an adjustable bed, a chair that rises up so I can get out of it, and home nursing care. I am also facing a motorized wheel chair, as the nerves and narrowing worsens from the masses. Eventually my legs will be so weak I won't be able to use them.

I know I've said it numerous times but thank you so much to everyone for the supportive, sweet, caring comments. The outpouring from people has truly been moving - and thoughtful. I will continue to make updates as I can. I say goodbye for now ... wishing everyone the best. -J

I Think The Right Word is RIDICULOUS!

Let me just start with thank you so much to everyone. The support I've received has been truly moving, and honestly a light during a kinda dark time. It all started with Crohn's Disease. As a lot of you know I've been dealing with Crohn's for 27yrs now. At some point during the last 4yrs I contracted Hepatitis B. - unknowing to myself and my GI at the time treating me. I had gone through most of the Crohn's meds at the time, and was put on Humira. A very important thing about Humira is - having Hepatitis B and being on Humira is BAD. The combo of the two caused my liver to fail and extensive Cirrhosis; a liver transplant was necessary. When you are a candidate for organ transplant they work you up from top to bottom. Any issues - even small ones - could become serious due to the surgery and medications...this was Oct 2010. For some reason I started developing breathing problems. It was incredibly difficult to take a breathe in. By May of 2011 I required Oxygen all the time. Initially it was assumed I had Hepatopulmonary Syndrome. - which is caused from the liver and cirrhosis. After countless tests and about eight months of basically wasted time, it turned out I DID NOT have Hepatopulmonary Syndrome! Fast forward to the last few months ... I had an EMG done of my diaphragm and found that the Phrenic Nerve which controls the diaphragm was fried ...dead ...fired its last brain message - it will never work again. Due to the diaphragm the prospect of a transplant went out the window. The reason transplant is not possible is they put a breathing tube in for surgery, and recovery. The doctors feel strongly that once the breathing tube is put in I would never come off of it...ever. Right now docs are monitoring my liver very closely, and crossing bridges when absolutely necessary.

I was referred to a neuro muscular specialist because the odds of the nerve issue being isolated to my phrenic nerve was very low. For some unknown reason I started getting extremely painful muscle contortions/seizures, double vision, and eventually rigidity. I literally felt as if I was turning to stone; any movement and wowzers did it hurt. The neuro muscular doctor ran an EMG of my leg, and took blood to check levels such as calcium, vitamin D, etc. The EMG findings fit best with Motor Neurone Disease or  Lumbosacral Radiculopathy.  I need an MRI of my spine...which is this Wednesday May 7th. The blood work was interesting - it showed my calcium was critically low, vitamin D was undetectable, severe anemia, and a specific hormone was extremely elevated (the normal range is 0-65 ...65 being high. Mine was 820 (the endocrinologist actually said she had never seen a number that high). The hormone that was elevated is called PTH. It happens to be created by 4 tiny glands inside the thyroid called Parathyroids. When calcium is out of wack the body they over produce the hormone PTH. Since my calcium was critically low I have what is called Primary Hyperparathyroidism. In 95% of cases surgery is done to remove the over active glands. BBBBBBBBBBBBBBBBBBBBBBBBBBBUT doctors are very hesitant to do ANY surgery on me because of the diaphragm. Along with the lovely low calcium I have osteoporosis along with renal-hypertension. Ridiculous isn't it?

To break it down:
1. Crohn's Disease
2. Liver Failure w/ Cirrhosis
3. Portal Hypertension
4. Hepatitis B
5 Primary Hyperparathyroidism
6. Renal Hypertension
7. Paralyzed Diaphragm
8. An undiagnosed neuro muscular disease (still running diagnostic testing)
9. Osteoporosis
10. Critical Vitamin Deficiencies
11. And a crack in the instep of my left foot

I am going to have to wrap this up for now - my hands are starting to cramp up. I wanted to touch base and let people know what is going on. and of course to thank the amazing support in my life! I would be completely lost and alone with out all the amazing loved ones. Regardless of what happens as well ...I won't stop fighting until I physically and mentally can't do it anymore ...and I don't see that happening anytime soon.

a VERY short update

Hi everyone - I saw the Neuro-Muscular specialist today; his name is Dr Robert W. Neel. I again got the luck of the draw - he is FANTASTIC. Not only was he incredibly thorough - but kind and compassionate. He genuinely wants to help, and figure out what is going on. He looked right in my eyes and said, "I am sorry this happened to you. I am going to do anything I can to help you, and work to improve your quality of life". I could tell he meant every single word.

First up is blood work, and getting me some comfort from the muscle spasms/contortions, and breathing issues. Working against the clock though as what ever the hell is going on it is progressing - fast. I will update more as I know things. A GINORMOUS thank you to Kelly and Patrick for taking care of CDSN, while being hugely supportive. Finally, thanks to all of you for the continued support and kindness ... it means more then any of you will ever know.

Where is Jason?

Well as a lot of you have noticed I've been kind of MIA from the wonderful world wide web. I have barely tweeted, posted a facebook update, or been active on CDSN. A lot has been going on, and I just haven't felt very social. I went to my liver transplant appointment to find out about listing, and the doctor explained why they aren't listing me. That is all I heard over and over as the doctor talked and explained why I wasn't being put on the organ transplant list. Apparently they didn't feel comfortable at the time with the Hepatopulmonary Syndrome diagnosis. I truly thought the doctors were full of shit. For at least half a year now, doctors have been telling me over and over that I have hepatopulminary syndrome - and the transplant doctors kept saying you don't. They sent me back to the pulmonary doctor to have yet another procedure. If I DIDN'T have hepatopulminary syndrome, and another issue, when they put the ventilator (breathing tube) in for the surgery (every patient has one)...that I would probably never come off of it. I was completely shocked! The procedure I had done is called a Pulmonary Function test. This showed how well and strong my lungs and respiratory system is - if it was not working right then I would be in serious trouble.

To my complete shock the procedure showed that in fact there were significant Neuromuscular abnormalities which the test flagged... and I DON'T have Hepatopulmonary Syndrome. Apparently my lungs were fantastic, but my diaphragm was not. It showed I had no trouble breathing out, but I couldn't breathe in because my diaphragm was only functioning around 21%. They set up a test called an EMG (similar to an EEG, EKG/ECG) but they stick an acupuncture type needle/probe into an area and listen to how well the nerves fired causing the area to properly work. The doctor put the probe into my abdominal muscle and I heard my muscle working, and nerves firing; I had no idea they could hear nerves and muscles working. The abdominal muscles sounded totally normal - which sounded a lot like static. The doctor stuck the needle into the diaphragm...nothing. It was pretty much completely silent; like someone turned the volume down. He told me to take in a deep breathe and it blipped maybe twice. Back in the abdominal muscle ...static - back in the diaphragm nothing. This essentially confirmed what the pulmonary function test indicated, a Neuromuscular disease (which there are 48 different kinds and which now has to be diagnosed.

Fortunately Cincinnati has the Neuro Muscular Institute - with some of the best doctors in the country. As soon has the doctor who performed the EMG filed his report the Institute would see me. I am very grateful that one of the doctors assistants is keeping an eye out for the report, and will call to get me scheduled as soon as it comes in.

I really am not one to be "whoa is me - life is terrible - person", and I am still not...but a part of me really just wonders how one person can have so much medical ailments hit them? From Crohn's Disease, to liver cirrhosis and failure, hepatitis B, and now a neuro muscular disease? The kicker really is - none of these are connected ... as in one caused the other. Yes - my liver got even more damaged because of the medication Humira and the combination of Humira ... but the Crohn's didn't cause either other issues.

I've been getting really bad muscle contractions and muscle seizures which are extremely painful. They've been in my legs, hip/back, and my hands. The spasms in my hand have made typing difficult. Besides just wanting not to discuss all this bullshit going on, the pain gets really bad when I get a muscle seizure. Thankfully they don't last long (90-180 secs, but I am talking 10/10 on the pain scale - and I virtually never say a 10.

That is what has been going on, and why I have been MIA lately. I really gotta thank Kelly and Patrick for taking care of CDSN. They have taken care of not just the site but the facebook group, and twitter account. I am a lucky guy to have them as administrators and friends, and even luckier to have Kelly in my life as someone I love very much.

I hope this answers a lot of peoples questions to what has been going on. I am very thankful and humbled by all the people who have asked if I am okay; it is very touching and helps a lot with keeping my spirits up. I am going to say goodnight, and sit back and relax. It's been a long day, and I need the rest. I hope everyone is well and life is treating them right. -J