Kwiecinski Syndrome: Help for Karen (Nesoff) Kwiecinski

"Our girl is sick...". All I can think of is Aurora telling Rosie that in Terms of Endearment. Not because I think death is coming, but because of the word "our". Our girl. That's how I think of Karen. She's everyone's girl. I thought I knew a lot of people, but I bet when it comes down to it, she blows me out of the water. She's just that kind of person who is outgoing and fun, and people just want to be around her. I know because I've known her for over thirty-five years. People just gravitate to her and always have. Most of the boys I liked in elementary school liked her. In my mind it was that she had bigger boobs, but really it was more. It's who she is as a person. We don't need to see or speak to each other for years but the second we talk again, it's like no time has past. And she's always the same, genuine person who I've seen do so much for others.

When I think back, I have so many memories with her. We practically lived at each other's homes as kids. We fought like sisters. We tried make-up together at the mall. She looked better and her sister's friend told me I looked like a clown. Something extremely traumatic happened to me when I was nine. The first place I ran to was her house. Her parents were away but she was ready to just let me stay indefinitely. We would steal her mom's eyeliner so I could draw freckles on me like Karen had. She was over for dinner on a Saturday night when my parents ritualistically ordered Chinese from (the now defunct) Hong Hing. I'll never forget her calling her parents excitedly to tell them how she had her first spare rib. She loved it! Bob and Sandy- not so much. Pig wasn't on the menu at the Nesoff house. Oops.

We went on our separate paths over time only to be pulled back together for this event or that. Two reunions, deaths, etc. But we were always the kind of friends who could catch up for hours. While planning our 20th reunion, somehow it ended up being just Karen and I, instead of a "committee", for most of it. It was fine. One night, she called me at midnight or so. My husband was sleeping. My phone was about to die. So I'm laying in our third bedroom, which I basically made into a closet, under my vanity table, tethered to my plugged in cell phone. I guess my talking woke up B, he came in to what looked very suspect- his wife on the phone, in the dark, under a table, at one o'clock in the morning. He asked who I was talking to. I said- "Nesoff!". Yeah, for the first time ever, he checked my phone the next day to see if I was telling the truth. (I was!)

Karen & my mom at my Sweet 16.

When my mom passed away in 2009, she just showed up. A lot of people showed up for the memorial service and funeral. But she really showed up. She dealt with all the food for the shiva and just hung around as much as she could. I'll never forget that. Then I saw her at the funeral of another mom of a classmate a few years later. You can just always count on Karen to be there for anyone who seems to be in need. When I think of her, I just think of someone who is always willing. Not that many people are that willing. When I found out what was going on with her, the first thing I thought is that there HAS to be a ton of people who feel the same way I do. That feel like they would want to help her in any way they can and return some of the numerous favors she's given over the years.

Months ago I begged her to let me set up a fund for her. I was on my way to a mammogram and I was relentless with texts. It took her three or four months for her to agree. She just isn't accustomed to accepting or asking for help. Finally, at her breaking point, she relented and here I am.

She wrote her story for me- It's partially in a timeline/journal form and part like just telling me her story. I'm leaving it mostly as is, and it gives more of an in-depth explanation of what has gone on over the past year and where she is now. Reading it really just brought tears to my eyes. No one should have to go through something so horrific. Well, there are some people....but I won't go there.

So let's help our girl....

If you don't want to read the gory details and just want to donate: http://www.gofundme.com/helpkarenk


Karen's Story:

It began on Monday, November 17^th, 2014:

I was moving some things around in my house. All of a sudden I felt a pain in my upper back. I just assumed that I pulled a muscle, so I put a heating pad on it and rested on the couch. As I slept that night, the pain rapidly spread throughout my entire upper back. The following day (Tuesday), the pain continued to progressively spread. I’m not one to run to the doctor, but I knew something wasn’t right. So I figured if the pain continued I would go the following day.

Wednesday morning I woke up and the pain had spread through my entire body. I called the doctor and was able to get in to see him that day. He had no idea what the problem was, and could only assume that I had acquired some sort of virus. He gave me an antibiotic and some anti-inflammatory pills and told me to follow up with him in about a week.

For the next few days I basically just laid in bed crying. None of the meds that the doctor prescribed for me were helping yet, and the pain was getting worse and not missing an inch of my body. My husband had asked me several times if I wanted to go to the ER, but I just didn’t know what to do. So finally on Friday afternoon I called him at work and told it was time I went. He took me to St. Joe’s Hospital in Wayne because it was close. We spent several hours in the emergency room and the only determination was that my vitals were way off. My blood pressure was very low and my resting heart rate was very high. They gave me dilaudid through IV for the pain, and strongly recommended that they admit me in order to determine why my vitals were so off. Well, after one night of unbearable pain in the hospital, the attending physician had no answers for us and told us that there was no reason for me to stay any longer since they couldn’t do anything for me.

I visited my doctor again the following two Mondays in a row, actually having my parents drive me because I was just in that much pain. So after this third week my doctor told me that it’s time for me to go to a specialist because this was going on too long and was getting beyond his expertise (being that he’s a general practitioner). I got a few recommendations for rheumatologists, and most had a 4 month waiting period. I was able to contact one practice that let me explain my situation, and within an hour the assistant called me back telling me that one of the doctors heard my story and would be able to see me that same afternoon, which was a miracle.

December 10th, 2014:

I got to the appointment and he found that my resting heart rate was 122. He was extremely concerned so he did an EKG and some other tests, and told me he may want me to go to the ER for a cat scan. I was getting scared. I got to the ER at Valley Hospital again. They brought me in for the cat scan and by the time I came through the end of the tube, the rheumatologist was standing at the foot of the machine. I mean, this guy just met me that day and dropped everything to help save my life.

The results of the cat scan showed that I had pericarditis, which means that I had fluid around my heart. All of the doctors were still leaning towards the idea that this started from a virus. However, there was still no way to find out how I got the virus. I wasn’t contagious, but it seemed to just be attacking my immune system.

After many tests and 4 days in the hospital, I was free to go. All of the cardiac tests showed that my heart was in perfect shape, so it was an enigma as to why I had fluid around my heart. They were able to reduce the fluid around my heart to a normal amount before they released me.

January, 2015:

I went home for 2 weeks and still I was feeling horrible, from head to toe. I had seen the rheumatologist several times by now, and still they couldn’t figure out what was wrong with me. They had contacted other doctors and were collaborating with each other, but even together they couldn’t figure it out.

My rheumatologist had done some deep research and had finally found an interventional cardiologist. I had no idea what that meant, actually I’m still not sure I fully understand. But I was like their dream patient…my case was an enigma to all of them. Still nobody could figure out what was wrong with me.

I met with a cardiologist at this point. My doctors had recommended that I see a cardiologist because of the pericarditis. He told me that my heart was probably the strongest organ in my body, but once he saw what was happening with my hands, he personally contacted my rheumatologist, who now wanted to see me the next day.

So I went to see him the following day and he put me back in the hospital. This time I visited him on a Wednesday and he scheduled me to come in on Saturday morning through the ER. And yet another co-pay.

January 17^th, 2015:

I spent a couple of hours in the morning there in the ER and was moved to the ICU for an IV treatment called Iloprost. It was supposed to help stop the progression of whatever this was. The average duration of this particular treatment was normally 3-10 days. I was hoping for the 3 of course. So I spent several days with an unbelievable amount of doctors visiting me, a toilet in my bedroom like a jail cell and a nurse with an office in my room, for 10 days. I didn’t even care about lack of privacy at this point, I was just in so much pain.

During that time I was consistently receiving the IV Iloprost treatment, about 1000 mg of IV prednisone per day, anti-nausea meds (Zofran) through IV as well as Dilaudid through the 6 minute push button for pain. At least it helped me sleep.

Probably one of the most significant and horrifying changes happened while I was in the hospital during this 10 day visit…

The only way to describe it is it was like a nightmare. I laid in a hospital bed, with doctors and nurses surrounding me constantly, and literally watched my fingers turn black in front of my eyes. I was actually watching the necrosis creep around my fingers by the minute, and there was nothing I could do to stop it. Or the doctors. They continued all of my IV treatments and upped dosages to do everything in their power to stop the progress. But nothing was working. I cant explain the feeling of watching your fingers die in front of you and having no control over it whatsoever. Horrifying doesn’t even begin to explain it.

One of the nurses actually told me that the doctors were so baffled with my case that they were referring to it as “The Kwiecinski Syndrome”, because there was no name for it. They’ve just never seen this happen before, and especially so quickly. I always wanted to be famous, but not this way.

So they continued to try everything possible to help me. What an amazing crew these guys were. And they only met each other because of my case. But I felt I had the best team because they really cared. They were doing everything in their power and knowledge to heal me.

While I was in the ICU, I was taken for all sorts of tests, including a couple of MRI’s. I needed to have a bilateral hand and arm MRI but because of the contrast, they couldn’t do both sides at once.

The day after I went for my first MRI, I had my first major surgery a Thoracic Sympathectomy. Essentially what they did was laproscopically went in near both of my armpits, 2 holes on each side. Once they were in they collapsed my lungs so they were able to cut some nerves in order to open some blood vessels. The only side effect would be the change of where my sweat glands were. Since the surgery, I no longer sweat on my head, but mostly on my stomach and back. This was one of the side effects that my husband and I laughed at because I always sweated anyway, what was one or two more places?

So finally after 10 days in the ICU, I was set free (finally on January 26^th). But only for a short time. My fingers were still getting necrotic and the doctors had outpatient treatments for me to go to.

In addition to all of the treatments these doctors were trying, they had given me an unbelievable amount of medications to take at home. The cocktail of meds they put me on was because they just didn’t know how to stop the progression, and wanted to try everything possible to open my blood vessels. I was taking pills 4 times a day, including 17 pills in the morning alone. I mean I was taking everything from cholesterol medication (even though I don’t have high cholesterol) to Viagra (and I don’t have Erectile Dysfunction)! I laughed at all of the years I had received and deleted email offers for Viagra. All of these medications have secondary purposes, and my doctors wanted to try anything to stop the necrosis from progressing even further. Now insurance wont cover the costs of Viagra for a female. It is only to be prescribed for a male for Erectile Dysfunction. They don’t care about it being prescribed to try to open blood vessels. So there went another hundred bucks per month. Luckily, I was allowed to stop taking them after only a couple of months. In addition to the oral medications, I had to get a shot (blood thinner) 1-2 times every day, and I’ve never been a big fan of needles to begin with. I have been working closely with a pain management group in order to relieve the pain, but also be able to function.

February 6^th, 2015:

The rheumatologist set me up to go to Weil Cornell/New York Presbyterian on a Friday night. He had spoken with a colleague of his at the hospital and I was to go in through the ER and they would admit me through there. He thought these doctors may have known more about my condition than my doctors could figure out, so anything was worth a shot at this point. Unfortunately, the hospital was so over capacity that there were beds in the ER hallways, one of which is what they gave me. There was not one bed available in the hospital for them to admit me. After several hours I noticed one open room and being that I’m not shy, I asked if I could get into that room instead of laying in the middle of the hallway. Finally, after several more hours, a small team of doctors came in to see me. They did a Doppler check (a small machine that is able to detect a heartbeat on hard to hear areas) on my hands/fingers and feet/toes. Although they weren’t able to hear the heartbeat clearly on a few of my fingers, they simply had nowhere to put me in the hospital, so they sent me home early Saturday morning with no more knowledge or relief than I went there with.

February 10th, 2015:

I went back in to Valley Hospital. The doctors wanted to try one more round of Iloprost to try to stop the progression, since nothing seemed to working. By now, most of my fingertips had become black and some were as hard as a rock.

In addition to the Iloprost and 1000 mg of Prednisone through IV each day, there was another treatment that they wanted to try to help stop the progression…Chemotherapy. But there were two choices. The first could put me in early menopause, and we weren’t ready to have them make that decision for us. So I chose the second chemo, which was called Rituxan. They figured they’d do the first round while I was in the hospital in case any allergic reaction occurred. They started that Wednesday night (February 11^th) and the rest of the treatments were to be done at an infusion center.

I went for 4 weeks, once a week, for treatment. Basically I sat in a chair for 4 hours every Thursday from 9-1 and received, through IV, a round of Prednisone (steroid), Zofran (to stop any nausea) and Benadryl (in case of any allergic reaction), and then the chemo. Luckily the Benadryl would knock me out so I’d sleep through most of the treatment. Unfortunately, the next few days were always hell.

I felt extremely wiped out the entire day of treatment. Then every Friday, Saturday and Sunday I felt nauseous and completely exhausted, typical chemo side effects (or so I had read). I couldn’t do much but lay on my couch and sleep to feel better.

Finally the chemo ended and it seemed to have stopped the progression of the necrosis. What had already become necrotic was awful and apparently couldn’t be reversed, but at least it was no longer continuing to progress.

But I still wasn’t getting better. My fingers were still necrotic. It was the scariest thing of my life. The necrosis had made those parts of my fingers black and they felt completely dead. I just had no feeling in the tips whatsoever.

During this time, I had met with a hand surgeon at Mount Sinai. They did x-rays and it appeared that the necrosis had not spread to the bone. However, he did tell me that in his professional opinion, I would ultimately need to have some fingertips amputated. The dreaded “A” word. He couldn’t figure out either why my fingers had become necrotic so quickly, turning from perfectly normal to completely necrotic in the matter of 2 weeks.

 

Additionally, he found that although my radial arteries were perfect, my ulner arteries (on my wrists) had stopped before reaching my hands. This was the case on both hands, which lead us to the possibility that I may have been born with this. The other possibility was that whatever possible ‘virus’ they thought I may have had in November/December had caused the effects of my Raynaud’s to accelerate and have a spasm that affected my ulner arteries. We just found it bizarre that both hands were symmetrical.

So I met with another hand surgeon at NYU Langone, who was referred to me by the doctor at Mount Sinai. Although this doctor couldn’t figure out either what may have caused my situation, he agreed that surgery was imminent and needed to be done as soon as possible. I wanted to have both hands done at once, but he explained to me that I would have no use of my hands whatsoever. That honestly never even crossed my mind. I decided to do my left hand first, because it was in worse shape than my right.

We discussed the ultimate reality that at least two of my fingertips would need to be amputated, but we decided to go with an auto-amputation. This means that we would let the fingers heal as much as possible on their own before removing any of them. The benefit of this method is that it allows the finger to heal more on its own, and I would have the chance of losing less of a portion of my fingers. Otherwise, if we were to amputate sooner, a larger portion of each finger would need to be removed.

March 18^th, 2015:

We went to NYU for my next surgery.

The surgery that was to be performed was a digital sympathectomy, along with a vein graft. Essentially what he did was cut across the palm of my hand and cut the nerves in order to open blood vessels. Additionally, he needed to take a piece of my vein from another spot on my arm and replace the missing ulner artery. While they were performing the surgery, they found that there was a blood clot in my hand. Again, the cause was unknown, and could have been from a possible spasm. Still nothing was for sure except that the clot could have become worse if they hadn’t gone in when they did. A surgery that we expected to last about 3-4 hours ended up taking about 7 hours, but it was deemed successful.

I stayed at the hospital for 3 more days after the surgery and was finally released (for a surgery that was scheduled as outpatient). They kept me because the situation and surgery were more severe than they had anticipated once they went in. my case is obviously not a traditional or routine one.

After surgery, I was unable to do pretty much everything. I couldn’t drive on my own (my parents, sister, and sister-in-law all took turns taking me for oxygen therapy…that will be explained in a moment). I couldn’t even shower alone. It was humiliating for me to have to ask my husband to shower me. He wanted to do whatever he could to help, it didn’t bother him but it bothered me that, even though it was temporary, I felt like I had become an invalid. I’d stand there with surgical gloves on (because it hurt so much just for the water to touch my fingers), crying in pain and humiliation the entire time. Even before the surgery, showering was extremely difficult because my fingers were in such unbearable pain. I couldn’t even touch a soft towel without it feeling like it was comprised of needles. My fingers were just that sensitive and painful.

Just before I had gone in to NYU, I received a letter from NJ State Disability, stating that I would be covered through March 18^th. Once my doctor contacted them and explained that I was having major surgery on the 18^th, they were nice enough to extend it to March 21^st, the day I was released from the hospital. With everything else going on with my health, they apparently haven’t realized that they are prolonging my recovery by holding payments. I’m physically unable to work, and my doctors had filled out every requirement and then some, just to confirm this information to disability. So after a few more weeks I was told that they would extend it to May 16^th. Again, my doctor sent letters to them explaining the severity of my situation, but no response. As a matter of fact, as of now (the end of August), I still haven’t received a dime from them since May 16^th. What bothers me is that people can fake injuries and receive long-term disability, but then someone like myself, who truly needs it, must wait several months with no word. I was told that I just needed to continue to wait because that is the process.

So back to treatment…

March 23^rd, 2015:

My rheumatologist and I had discussed the possibility of adding a hyperbaric oxygen chamber as my next treatment. So I had met with the wound care center at Hackensack Hospital prior to my surgery and we decided that my best bet was to start just after the surgery.

The hyperbaric chamber is known for healing wounds, and we were hoping it would help the ulcerations (necrosis) on my fingertips heal. We breathe in only 21% oxygen when we breathe on a normal basis. While in the hyperbaric chamber, we breathe in 100% oxygen.

They lock me in what looks like a clear MRI tube, and seal the door. It’s basically a simulation of what Navy divers use to decompress. For the first 15 minutes, they bring you down the equivalent of about 2 lengths below the sea level (for me I went 33 feet down). Your ears pop for the first 15 minutes during the ‘dive’. For the next 40 minutes you lay there at that depth. Then you get an air break and you put on a mask that helps you breathe in regular air for 10 minutes. The next 40 minutes are the same as the previous set of 40 minutes, where you remain at the same depth. Then for the last 15 minutes, they bring you ‘back up’. I had gone in for a total of 2 hours per day, 5 days a week, for almost 5 months.

During this time I was still visiting with my doctors and trying to get surgery scheduled for my right hand. I wanted to get it over with so I could get back to normal as soon as possible.

April 2^nd, 2015:

I wasn’t feeling right and took my temperature at night, and again the following morning. I was in an incredible amount of pain again and now had a temperature of 102.6. My entire body felt achy. All of my muscles were extremely fatigued. Once again, I looked like a 90 year old woman just walking down the few stairs in my house (split level so about 7 steps per floor). So I called my rheumatologist and he sent me to Valley Hospital yet again. More IV drugs, another 3 days in the hospital, and a diagnosis of pericarditis (fluid around my heart) again, along with Pleural Effusion (fluid around my lungs). I was in so much pain it was just unbearable. We believe the reason this happened was because I was weaned off the Prednisone too quickly in order to have my surgery in March. Luckily we were able to get it under control and cleared up and I was feeling better in a few days.

Because of this, I was told that I needed to go back on the high dose of Prednisone, and because of the Prednisone the surgery for my right hand was delayed. It was originally supposed to be 3 weeks after the surgery on my left hand and I finally ended up being operated on 3 months later instead. I needed to be slowly tapered off the steroid this time to avoid any further complications.

June 17^th, 2015:

Finally the day had come to operate on my right hand. I had also come to the most difficult decision of my life…that it was time to amputate the index finger on my left hand (the surgeon had told me it would be removed when I was ready, and I felt that the auto amputation did as much as it was going to do on the index finger at this point). The top 1/3 of my finger was basically hanging on by a thread. I cant even begin to describe the amount of pain I was in. The surgeon was happy with my decision once he saw the condition that the index finger was in. And apparently I made the right decision because an infection had developed in the bone on my index finger, which would have been worse had we waited longer to amputate it.

It took another 6+ hours of surgery for the digital sympathectomy on my right hand. Wouldn’t you know it? They found a blood clot in the palm of my right hand that was even worse than the one they fixed on my left hand. It probably grew because of the length of time that we had to wait to do this surgery. Regardless, I was lucky that they were able to get rid of it before it got rid of me.

 

Today:

 

So as of today, I have some good days and some bad. There are some days I don’t even want to get out of bed, and other days I just push myself to do anything to give myself a purpose. Being unable to do many physical things that I’ve always been able to do. It’s a horrible feeling being told by doctors that I’m not allowed to work, that they will reevaluate me in October. I’m trying to feel useful but I’m limited to what I can do.

As of now, I have one more surgery scheduled on September 16^th, for the amputation of the top portion of my left pinky. The rest should ultimately be OK, although it will take a long time until they do heal.  After healing from my next surgery, I will be sent for occupational therapy to restore my motor skills in the amputated fingers. Even today I have difficulty bending my index finger.

My bills are continuing to come in stacks. It makes me sick every time I open the mailbox.  We have insurance, but there is still a co-pay for every visit to every doctor and hospital. I never imagined how they could add up. The chamber, for example, sends one bill for a co-pay for every single session. I went almost 100 times. And that’s just one doctor. But disability is still taking their time. I even sent them pictures of my fingers, but they still haven’t remitted anything to me in over 3 ½ months. I just don’t get it.

All of my doctors have recommended that I move to a warmer climate because the cold weather is not good for me. We’ll see how this winter goes and if its anything like this past winter was, we’ll start considering the options. But this is my home so I'm ready to go there just yet.

With one of her friends. Still smiling...