The new "something" is Porphyria

After nine months of job hunting, Steve has finally landed himself a regional sales management position in a start-up urology company. I must say a big thank you to all of you who have kept him in your prayers and passed on resumes for him. It certainly is nice to be on a payroll again. The best news of all is that he doesn't need to travel much. After 18 months in his last position of traveling continuously, it's nice not to have to find rides for the kids and myself.

My Kindle has just been updated with a free application which has increased the font size, and now I am able to read it again and not just listen. Yeah! It's also been a nice bonus that the kids are back in school. Tyler is a sophomore and Alyssa is in 2nd grade and they are both doing well.

Medically, since my last post, I've had two root canals, two crowns, and still require more dental treatment but can't afford it at the present time. My dentist told me that the reason I'm having such bad decay is because of the brain stem radiation back in October 2006. I was never warned of this being a side effect.

After returning home from the hospital with the kidney/blood infection, I received antibiotics through August 9 through a pic line. About a week after the pic line was removed, I started feeling as if I was getting another infection. I had low-grade temperatures and felt flu-like and very achy. I had blood work done. My white count was elevated but they could not find a source for the infection so they decided to just watch it. This continued and yet the achiness has increased in intensity, I'm very fatigued, and I sleep 10-12 hours per night. Since coming home I've lost another 10 lbs and have not been able to gain it back. The doctors were still perplexed as the blood work had not pointed to a source of infection. So I went to see an infectious disease doctor in mid-September, and he seemed to feel that I had Lupus or a condition called Porphyria. My symptoms at this point were frequent and painful blisters on my hands and feet, and my skin discoloration had worsened on my arm.

Later that day I saw a dermatologist who saw the blisters. Dr. Brieva did two skin biopsies to determine what type of condition it could be. He felt that my skin discoloration and thinness was not just related to steroid use in the past and that there was some other factor. About 10 days later, the dermatologist called with the skin biopsy results and said I have Porphyria and ordered lab work and urine tests to determine which of the eight types I have. As I have no history that I know of in the family, he feels it probably started as a result of long-term antibiotic use over this past 10 months.

Porphyria is a rare disease of the blood that causes fatigue, weight loss, low-grade temperature, neuropathy (nerve pain), blistering, skin pigmentation change, nausea, etc. -- everything I have. My pain seems to have settled in my upper back and upper arm. I'm still waiting for results from the Mayo Clinic lab as to which type of Porphyria I have.

Other than all this nonsense, I truly enjoyed the summer and it was nice to finally have a summer this year (last year was cold, crappy, and I felt horrible). Hard to believe I've aged so much but I even had my 30th high school reunion on September 18th.

I recently have seen the eye doctor, Dr. Cohen. Again, he said not much changed but I still see crappy. We'll be following up with infectious disease and whoever follows Porphyria. I never had a chance to start chemo treatments for my eye or kidney with all that's happened in the last year. The good news is my internist said he phoned in a strong narcotic for me. Looking forward to hopefully finding some better relief today for my back. The pain is draining my body of energy.

It's almost full circle now. My brother passed away October 22nd of 2009 and I remember blogging from the lobby of Northwestern Memorial Hospital. With everything happening this past year, I realized I never fully grieved. He comes to mind often and I really miss him.

On a brighter note, Alyssa is also a year older, turning eight this Saturday, and we will be hosting a spa party with 20 of her closest friends. She's so excited. It will be quite fun. Ooh la la!

Seems like it's always something...

From Suzanne: It's been a while since we've posted anything. That is a good thing actually. Kim had been feeling really good and getting out to enjoy the summer. Last week, however, that all changed... I just spoke to Kim on the phone and wanted to update you on the latest. I will do my best to summarize the events of the past week.

At the beginning of last week, Kim started feeling ill and had a fever around 103-degrees. She was achy and decided that it must be the flu. As the week went on, her fever continued and she began feeling disoriented. Finally, on Thursday, a friend convinced her that she needed to see a doctor a.s.a.p. She went to Condell Hospital in Libertyville and was admitted. Apparently, she had a bladder infection that became a kidney infection and ultimately spread to her blood, i.e. she had sepsis. She was also very dehydrated, so they started administering fluids and antibiotics.

She was transferred to Northwestern hospital on Sunday, where she continued treatment. She was in ICU at first. Her heart rate was in the low 20's and they wanted to keep a close eye on her as sepsis is extremely dangerous. They did an ultrasound of her kidneys to see if the infection had caused any further damage & it seems like they are stable. She will have an MRI in the coming weeks to be sure.

Kim had a pic line inserted and will be coming home today. She will continue with IV antibiotics for 13 more days. Her heart rate is now in the 50's, which is low but acceptable. Not surprisingly, she has very little energy. She has also gained 24 lbs. in water weight since entering the hospital, so that is uncomfortable as well. It'll probably be 4-6 weeks until she's feeling back to normal. They want her to do physical therapy to strengthen the muscles in her neck. That should help with some of her pain.

Regarding the eye situation, that seems to be stable for now, although Kim says she sees "like crap." LOL She also recently had Part 1 of a root canal done & goes back for Part 2 next Monday. Poor girl never gets a break!

Comfortably numb... (actually un-comfortably numb)

I saw the eye doctor again on June 7th. Dr. Cohen was pleased that the fluid was decreasing and the bubble seems to be shifting out of my central range of vision. It seems like my vision is getting a little better now, so I am glad I did not get the steroid injection. His big concern still is the optic nerve lesion because it is large and leaking. He's afraid if I do PDT again that it would really compromise my vision. Therefore, he wants me to hold off until there is some further change in the size or amount of leakage from the optic tumor. I follow up with him on July 2nd.

I've been very preoccupied this summer with my Aunt Winnie, the travel agent who I went to Florida with. Unfortunately, she has been suffering from medical issues herself. She required surgery three weeks ago for a pinched nerve & after being in rehab for a week, had to undergo another surgery due to complications and signs of infection in her lumbar incision. It turns out that she has MRSA, a really serious type of staph infection which is very resistant to treatment. She's very frightened now, as she has to be on antibiotics the rest of her life. So, please send prayers her direction too.

Over the past couple of weeks, I have noticed tingling and numbness in different parts of my body - e.g. my arm & fingers have been tingly and my pinky is completely numb. The sensation in my right arm is really off. It feels like it's asleep and I keep shaking it to wake it up. Then I noticed that it started progressing into my right foot, causing numbness in several toes, my heel and my shin is tingly also. After the symptoms worsened, I contacted my neurosurgeon, Dr. Bendok, who suggested I go right to the emergency room. He was concerned that I had a stroke or a recollection of fluid in my spine. Unfortunately, Steve had the stomach flu so my friend, Darlene, drove me down to Northwestern. They kept me there all night and I finally had an MRI of the brain and cervical spine done at 5:00 a.m. this morning. Things looked good and there was no sign of a stroke. However, they are still really concerned with the numbness and its cause. They gave me the option to stay and do another MRI of my thoracic spine. They think that maybe there are tumors there that could be the culprit. I chose to come home & am scheduled to go back on Friday for MRI's of the brain, cervical and thoracic spine (3 hours!). Then I see Dr. Bendok on the 28th. Another possible reason for the numbness could be hyperstimulation of the nerve root. Your nerve endings regenerate about six months after surgery, so it could just be that I'm having a hypersensitive reaction. So, they put me on Lyrica, which will help calm down the nerve endings if this is indeed the problem. I'm very anxious because my symptoms increase daily. I am also nervous because once there is nerve damage, you sometimes never get that feeling back.

A BIG thank you to Darlene for taking me to the hospital and staying with me all night. It was like a sleepover. We talked and laughed all night. Unfortunately, the bed we were sharing broke in the middle of the night!

Hopefully, this will all be resolved soon and I can get on with enjoying my summer. Hope everybody else is doing well...

No steroid shot for now...

I returned home from Philly on Saturday, after spending a few days with my sister, Robin, who lives in the Poconos. On Monday, I went to see Dr. Cohen (my eye dr. here) in order to get the steroid shot in my eye. However, on Sunday, I felt that my vision was improving a little and less foggy. I told the nurse this & requested that they perform another test prior to doing the injection. When he compared the new test with the one from the week before, he thought the fluid levels had gone down slightly. Therefore, we decided to wait on the injection. I will go back to see him next Tuesday. If things stay the same or get better, I will not need the injection & he will just keep watching the eye closely.

On Monday, I also (finally) spoke with my kidney doctor. He felt that there were only slight changes and not to worry about the small growth in my left liver. I will follow up with him in the next month to discuss a plan (possibly chemo or surgery to address my kidney cancer).

Then I talked to my infectious disease doctor from Northwestern. Although my bloodwork came back okay last Friday, he wants to repeat it and see me in his office since I have felt achy and fatigued for the past few weeks. I will see him next Monday.

Whew! That's all the medical news for today. Enjoy the last few days of freedom before the kids get out of school : )

Update from Philly...

From Suzanne:

Today, Kim was seen at the Wills Eye Center in Philadelphia. She flew out there yesterday. By some bizarre coincidence, a friend was traveling to Philly on the SAME day and on the SAME flight as Kim, and therefore, became her chaperone for the journey there. When she arrived in Philly, her sister, Robin, picked her up at the airport, stayed with her at the hotel & escorted her to her appointments.

I just talked to Steve and got a brief summary of what the doctors from Wills told Kim. I apologize ahead of time if any of this is incomplete or downright wrong! I will ammend it after I talk to Kim, but wanted to give everyone an idea of what was happening.

The doctors found that fluid is leaking from her optic nerve tumor and is causing something like a blister on her retina. This is what is causing her vision to be foggy. They said that this has nothing to do with the PDT (photodynamic therapy) treatment she had done a few weeks ago. Apparently, that was done on a different tumor? and the procedure is so mild that they said it would not have caused this. They are going to try giving her a steroid shot in the eye that they hope will reduce some swelling and hopefully stop the leaking. They are not sure that it will work & there are some risks associated with doing it (e.g. infection, glaucoma, etc.), but don't know what else to do. They said she could do it there or come back home & do it. Because there is some discomfort associated with the procedure, she decided she will wait until she returns home to do it. If this does not work, they mentioned returning there for more PDT.

Kim is staying in Philly again tonight and then I believe she will go to Robin's and spend the day tomorrow. She will most likely return home on Thursday?

To see or not to see... that is the question

Since my last post, I've seen the eye doctor several times. The blood on the retina has been reabsorbed and the swelling went down. Unfortunately, my vision is still significantly worse than before the procedure. The reason for this is that I now have a new problem. I had a scan of my eye and it showed that there is fluid behind the retina, which is causing distorted vision. The leakage of fluid is coming from the optic nerve tumor, which has sprung a leak. Dr. Cohen, my local eye doctor, has no answers for me and recommeded me to be see at the Wills Eye Hospital in Philadephia. There I will be seeing the top doctors in the country to see if they have any ideas of how to help me. These are the doctors to whom he presented my case a couple of weeks ago. Due to the urgency, I will be flying out next Monday and have an appointment on Tuesday (a 6-8 hour evaluation). Hopefully they will have a treatment plan for me, which may include a radiation implant or systemic chemo. My sister lives in PA & will meet me there to keep me company and help me get around.

Now for some good news... Yesterday, I had a repeat MRI of my spine to see if the infection was improving or was gone. It appears to be gone! Hooray! And, there didn't appear to be any change in the size of the tumors in the spine or brainstem.

This Friday, I will see my internist for a physical, bloodwork & EKG as a prerequisite to being seen at the Wills Eye Clinic.

On a completely separate note... I received a surprise book-on-tape in a bag from a mystery person. It came at the perfect time because I was going crazy not being able to see anything! I finished it in three days and would highly recommend it - House Rules by Jodi Picoult. So, thank to my secret angel!

I also want to take the opportunity to thank Brian and Darlene Schick for generously providing me transportation to Philly on a United buddy pass. Thanks also to Suzanne and Ray Baumruk for putting me up in a hotel while I am there. It is greatly appreciated, as Steve has still not found a job after six months out of work. If you have any tips, he is still looking for a medical sales or management position.

To all of the mothers out there, I hope you have a wonderful Mother's Day!

Will let you know what happens in Philly...

I can('t) see clearly now...

Last Friday, I had the PDT procedure done by Dr. Cohen. I had a weekend planned to clean the basement and go see some movies because I couldn't be outside in the sunlight. Instead, I spent the weekend blind as a bat! My vision has gradually decreased over the past few days. On Monday, I went back to see Dr. Cohen. He said it appeared that I have a leakage of blood behind the eye that is blocking the light as well as the ability to see things (in addition to my already poor vision). He said that it should improve over the next week. However, I called him today to tell him that it is still getting worse. He was surprised to hear that my vision had worsened rather than improved. If things continue to worsen through Friday, he said he would do surgery to irrigate the blood out of the eye and laser the vessel closed. He would probably do this next Monday or Tuesday. He will be out of town for the next few days at some vision conferences and will be presenting my case at Wills Eye Center on the east coast. He expects there will be many oncologists there & wanted to present it so that hopefully someone may know of a systemic treatment they could do to stop the optic nerve tumor from leaking and growing.

On another note... after three years, I decided I should make a trip to the dentist. I was hoping that at least one part of my body was in good shape, by not so. I need two crowns, a root canal and gum surgery. Yippee! Lucky me...

Tomorrow, I go get my pick line out (Yippee for real!) and have an MRI of my pancreas and kidneys. I'll write more later this week once I discuss the findings with the doctor.