nwbsaw

Has any one found any thing that will stop me from dropping my blood pressure and passing out? I have tried theophylline, brethine, steriods and many other things. I am now starting on thalomid. How are you coping with this disease?

aporzeca

You seem to have the even more rare, chronic version of this (for all practical purposes, incurable) disease. One of the other members of this Community has it also.

Have you/your doctors consulted with Dr. Philip R. Greipp of the Mayo Clinic, who is this country’s leading authority on SCLS?

Also, have you/your doctors considered using long-term, monthly IVIg treatment, which has been tried with partial success in Europe? Have them check out the just-published articles posted under Disorder Resources, “High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome” and discuss it with Dr. Greipp. It’s risky, expensive and experimental, though.

It would be good to know more details about what exactly you’ve tried (dosage, frequency, etc.) in such a short time.

judithdavis3

I frequently have attacks every 5th day for the past 2 to 3 years. I keep records and rate them on a scale of 1-10 depending on the severity. An "8’ is when I can’t sit up because I am too light headed and dizzy and nauseous and hypotensive. I go to the ER when I am that sick. I stopped having bad attacks June 2006 when my doctor stopped my theophylline and terbutaline and I started taking singulair. I have been hospitalized for fluid in my lungs during recruitment phase and dehydration from stomach virus with a leak since 6/2006. I hydrate at home when I leak-not a problem usually because I am so thirsty. It would be a problem to hydrate if I was nauseous and couldn’t drink. I was drinking 6 bottles of water a day and was told this could drop my sodium levels so I try to drink some gator ade with the water. I also avoid caffeine at all costs.

nwbsaw

Thanks for your help. My Dr. has consulted Dr. Greipp. I am to sick to try to go see him. I have been in the ICU in my community hospital in 6 times since the first of June. Usually I require 10 to 15 liters of fluid and dopamine or levophed to keep my blood pressure up. I was transported to a hospital in Salt Lake City 200 miles from my home. I was there for more than a week while I recovered from a pericardial window placed for a cronic pericardial effusion. I have a port-a-cath that was placed in May and my wife who is a nurse will give me fluids at home with milder attacks.
I just started thalidimide and we have discussed IVIg treatments but don’t have the funding or medical back up. yet to do that. I guess that will be our next step if this doesn’t work.
I have been on a high dose theophylline (1800 mg a day) for 6 months. We decided to stop it when I went into arteral flutter. I have had blood clots in my lungs twice. Once in August last year and two weeks ago. I am on comidin for the rest of my life. I was also on singular for nine monthes. I am also on predinsone.

judithdavis3

Have you seen the IGIV article on pub med? You would be a perfect candidate for this therapy. The French swear by it for SCLS. I was unable to get insurance auth and since have been doing fairly well so have not persued it but if I start getting sick again I will use that article to get insurance auth. I go to a community hospital also (but am a Hopkins nurse for 30 years-pediatric infusion). I have been out to Mayo twice and am followed by Dr Greipp. I try to stay away from the hospital and away from IV fluids. The hospital and IV fluids seem to mess me up more than without. The literature says NOTHING will bring the BP up-only the leak stopping will bring the BP up. I got rhabdomyolysis from the 20 liters of fluid they gave me before I was diagnosed and had kidney failure as a result and permanent nerve damage in both legs from the fluid over load. The French specialist Dr Amoura says 2-3 liters only should be given to prevent fluid over load in recruitment. I would agree to more fluids if I was getting shocky. How did you find the SCLS rare share? I am glad you did-there are so few of us with SCLS.

nwbsaw

I was trying to find some one that had SCLS so that we could talk and share. I was even trying to maybe set up my own web site and was asking my son who is a computer engineer to help me. He found this site for me. We live in a small community and it has been a challenge to find the help we need. I seem like no one in the west has even heard of the disease let alone knowing or wanting to know what to do for it. We are blessed to have a family practice Dr. who is a personal friend. With out him we would be in sad shape. He is will to go to bat for us with our insurance. We just have to exhaust all other treatments. We will have to find someone that is willing to do the treatments. Thanks for talking to us. (By the way my husband, Nolan is the one with SCLS and I am Wendy his wife. He is just recovering from another episode.)

judithdavis3

Wendy It is very good to have connected with you and Nolan. I learn so much by speaking to others with SCLS. What I just realized yesterday is that all 3 of us on here with SCLS had clotting of some kind. I had 4 strokes in 2004-my initial hospitalization eventually resulting in dx. SCLS supposedly does not cause coagibility issues but I am now suspicious that it might even if indirectly. There are so few of us left alive that every bit of info gathered is significant. Dr Greipp is supposed to offer my email address as a contact to all SCLS patients. Please feel free to email me any time-judithdavis3@hotmail.com. No one on the east coast has ever heard of SCLS as well and no one seems totally interested. It falls under hematology but they all have bigger fish to fry than SCLS. Please email me so we can exchange info. I would love to talk to you in greater detail.

jisenhour

Well the GW university hospital in DC is aware of it now. I’ve been in and out of the ICU for the last three years but I have to agree that from my perspective there is not a lot of interest in long term care of this syndrome.

aporzeca

Dear jisenhour: I have developed a good relationship with a top Internal Medicine MD and a top Critical Care/Pulmonary medicine MD at Georgetown University Hospital, and they ARE interested in SCLS, so if you’d like to switch from GWH to GUH, let me know and I’ll give you a referral.

jisenhour

I’d love a referral. If you need my contact information just let me know. My email address is in my profile I believe.
Thanks again for this forum.

aporzeca

No, jisenhour, email addresses are not disclosed, so write to me at aporzeca@american.edu and I’ll tell you who to contact at Georgetown University Hospital.