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Serious care of SCLS

knackll Message
19 Oct 2012, 01:27 PM

Hello, I just wanted to chime in as it helps me to deal with this very horrible disease my wife tries to exist with. We are currently dealing with our 10th hospital ICU visit in 1 calender year for an episode/attack of SCLS. This usually evolves a minimum stay in the ICU of roughly 10 days not counting step down. Yesterday, she was scheduled to enter the ICU as an non-emergency inpatient visit to receive IVIG. While waiting for it to come to the floor, she had another SCLS episode/attack. She was in her 4th week from the last ICU visit of SCLS at which time she received 2gr/kg. 2 weeks ago after leaving the hospital, she met all at once, with her new team of doctors, which included an immunologist, pulmonologist, & neurologist to devise a plan to prevent these episodes from happening or at least reduce the effects of it. I'm very glad that we have metamorphosis from a 1 man team to several doctors who have really taken an interest in helping us treat this disease. The plan that was devised was a inpatient ICU visit to receive 2gm/kg over a 48hour period. This would be just for a trial period, not to be a permanent procedure. The doctors are just concerned and want to be proactive just in case. The reason for the IVIG to be administered as a walk-in admission was that she had an anaphylactic attack as an outpatient receiving IVIG at the same hospital a couple months prior. There were some procedures that were not followed that I believe led up to that situation, such as not been pretreated with anything prior to the IVIG. Anyways , the plan this time was to admit her for 2 days to receive the IVIG and release her. Well, that didn't quite work out as while waiting the several hours for the IVIG (another story for later) she went into an episode which caused her to crash as she always does. I guess one good thing, at least she was in the right place to have the attack. After changing the course of action from a simple non-emergency visit to a full blown SCLS episodic attack visit the IVIG miraculously appeared from pharmacy. Obviously, she was headed for an episode/attack regardless of the timing of the IVIG being administered yesterday. What seems to bother me, is that I struggle to find other people diagnosed with SCLS who seem to have this disease as bad as she does. Maybe they just don't report it I'm not sure. She is attacked every month with a vengeance. It attacks her heart even though she has been given a permanent pacemaker (bp dropped 50/25 as always), Her lungs (plural effusion happens), Bladder (loses sensation to urinate), bowels, kidneys, & possibly pancreas just to name a few. Her body completely non-profuses. Something we started to notice is the warning signs have started to change. In the past, the common symptoms were swelling/redness/nausea/bp drops. All this starts the week prior to the actual physical episode that hospitalizes her. Now, the signs have diminished or have disappeared and she only experiences nausea and bp fluctuations. Whats even more disturbing is when we arrived to the ICU yesterday, her stats were perfect. They couldn't of been any better. However, within a hour and half she crashed. Very little warning signs. Just when I thought I was becoming an expert in her disease, the pattern of attack has changed. All the results remain the same for the most part, but the oncoming symptoms have changed. I'm starting to wonder if the prior IVIG treatments have something to do with that. Is there anyone else aware of someone having the same recurring month to month attacks the require ICU visits every time? What also has become kind of sad, maybe not, is that we have come to know quite a few of the doctors and nurses. I guess as a positive way to look at it is we are giving them an education to the disease that very little is known about. We have had several of the same physicians & nurses on our case from prior stays have somewhat of an understanding of what it is and how to treat her. That's not to say that once in a while we come across a new cowboy who wants to change the world. We just can not break this vicious cycle. The doctors are making a valiant effort, but just can't seem to get a head of this. There was talk last night of possibly administering IVIG every 2 weeks in an effort to ward off any pending episodes or attacks. I asked about increasing the amount dosed and was told that the quantity is not as much of importance as is the frequency. I'm not an expert on IVIG so I can't comment to that, but certainly am not opposed to the idea. At this point we are struggling to cope with this every month occurrence as it just will not stop. Also, on a side note, I know it has been requested to send some blood samples and I am still working on trying to orchestrate that, however the hospital seems to be somewhat reluctant. I'm not giving up on trying to have that accomplished. Anyways, thanks for reading. knackll
aporzeca Message
20 Oct 2012, 03:00 AM

Dear Knackll: I’m so terribly sorry to read this update on your awful ordeal! The only good thing is that the doctors and nurses are getting to know your wife and her case, are giving her the recommended dose of IVIG, and are starting to pull together to try to help her in an effective manner. However, it sounds like they could use some help. Let me repeat 2 of my earlier suggestions: 1) Have your hospital send to Dr. Druey at NIH refrigerated blood from the day of your wife’s admission. (They usually keep any blood drawn for a week.) Her blood needs to be studied with the powerful tools available at NIH to determine what (if anything) is wrong — or especially wrong — with her. For instructions on how to ship the blood, which must be chilled but not frozen, have the hospital contact Dr. Druey and/or his team, see “Disorder Details” in this site, and scroll towards the bottom for contact information. And do this every time she lands in the hospital. Insist on it. 2) Have her doctors make arrangements to have your wife seen at NIH (in Bethesda, MD, just outside Washington DC) as soon as possible. For this purpose, they must contact Dr. Druey and send him all of her medical records along with a referral letter. Dr. Druey will be able to confirm her SCLS diagnosis, or else he will have her examined by his colleagues until the right diagnosis is obtained.
Josephite Message
7 Nov 2012, 04:13 AM

Knackl, I'm so sorry to hear of your constant episodes and trips to the ICU. My husband's oncoming signs also constantly change, but even at it's worst, my husband's episodes were every 2-3 months (not monthly as you experience). I sincerely hope that a treatment regimen that helps is discovered. I am curious why they give your wife IVIg as an ICU "walk-in". My husband gets his monthly IVIg in a Day Medicine unit at the hospital, which allows him to make scheduled appointments. I`m not suggesting doing the same would reduce your wife`s episodes. But, it would be easier on you guys to go in for an appointment rather than walk in and wait until they can fit you in. I know how exhausting it is when the condition is out of control. I am sending you all my best thoughts, and hope you both get a deserved break soon.