March-2000

 Dear Family and Friends,

I apologize for taking so long to write another update on Shauna. Blame it on technology - computer technology, that is. My Compaq PC decided not to wake up one morning a few weeks ago. Some computer savvy people I know say that I strangled it to death! I guess I should have left a few more sectors free. Long story short, it was in the shop, and as my e-mail address book was there with it, I couldn't easily update everyone at once. So now it's back with a little more memory and a little more hard drive - hoping to make it last a few more years.
As to Shauna, she had her bone marrow biopsy and aspirate done on February 24. She was doing so well I was feeling assured that the test would come back negative for disease. We were crushed when the doctor called on Friday to say that there was still disease in the marrow, less than 10%. The next week was really hard on us all. It was our belief based on conversations with her doctors, that if the marrow was not clear after two rounds of chemo, the chances were high that it was not going to get clear. And now she's been through three courses of chemo, and still disease.
We were feeling a bit in limbo from that point on. Would we go ahead with the protocol and hope that course four would do the trick? Would we start palliative (quality of life, life prolonging) treatment?

What was next?

We scheduled a meeting with her doctors to correspond with admission for a fourth course. Unfortunately, Shauna's platelets were too low for chemo to continue. So the meeting was rescheduled. Platelets were still too low but we decided to go ahead with discussion anyway. Then doctor rescheduled. More time in limbo.
To help me maintain sanity, I spent a lot of time on the Internet searching for cases similar to Shauna's. I found a neuroblastoma listserv that has been very helpful. I posted an introduction and received several responses. Most all of them said that Shauna is much too early in the game to give up on getting her marrow clear. Many had up to 9 courses before the marrow was free of disease. I started to feel renewed hope. I learned about other methods of treatment and other chemo drugs. I learned about the various trial studies happening around the country. I learned to be strong enough to face her doctors with more questions and ask for more options.
The meeting finally took place last Thursday. The doctors recommend another course of chemo, possibly using one of the drugs she's already had. Unfortunately, it's the one that kept her in the hospital for almost a  month. If after three weeks, her marrow is clear, we go forward with stem cell harvesting and bone marrow transplant. If no clear, we talk again. The doctors said that we could possibly go ahead with transplant without a totally clean marrow. The stem cells could be 'purged', a process intended to remove residual cancer cells. But this process damages the stem cells and lengthens the transplant recover period. The doctors are concerned that Shauna's lung condition would cause her to have complications.

So they presented us with something to consider. There is a study being done at Texas Children's Cancer Center in Houston. It involves a vaccine developed from the child's own cancer cells. The intent of the vaccine is to administer it to children in remission such that their own immune  systems can learn to fight the neuroblastoma and prevent relapse.  To date, the vaccine has been used to help relapsed patients get back into remission and has been fairly successful. With Shauna, we would use it either to fight off remission (if we can clear her marrow and continue with transplant) or as a treatment if we can't clear the marrow. There would be no guarantees.
We've decided to get the vaccine created for Shauna as there is no cost to  us and the procedure is minor. It would then be there if we needed it. It takes 40 days to create the vaccine. Meanwhile, Shauna will have a fourth course of chemo and we will again check her marrow. So, Shauna goes inpatient on Wednesday. Please keep her in your prayers.

Take Care,
Kathy Palmer
Nevada City, CA
 

June 2000

Hi to family and friends:

I feel like I can breathe again. Last week was a good week for us all. It started with Shauna's admission on Monday (6/19). She needed conditioning (i.e., roto-rootering) prior to surgery. Wes and I both stayed in the hospital room expecting to be running her to the toilet and changing bed linens all night. But, without going into details, the drug worked without meeting our expectations and we all got a good night's sleep. The surgery to remove her primary tumor was very successful. The tumor had shrunk to the size of a walnut and was encapsulated, meaning round, smooth, and without tendrils. No organs other than the left adrenal gland were involved. The gland had to come out as well. It was all done laproscopically and she has three small scars on her left side.

She spent one night in ICU and three more in the Oncology wing. The tumor was biopsied and sent off to study its characteristics. By Thursday, we got the news that her DNA index was 1.45, or that the cancer cells have 1.45 times the DNA of normal ones, which is supposed to be linked to a good chemo-sensitivity. We still await news on additional factors that will tell us whether Shauna's prognosis is better than we had thought to this point.

Since Shauna's marrow situation (about 5% disease after 4 rounds of chemo) hadn't been evaluated since receiving a fifth round of chemo, Wes and I pushed for a bone marrow biopsy and aspirate, which was done on Thursday. On Friday we received the best news of the week - under the microscope (can detect 1 cancer cell in 100), Shauna's marrow is clear! In Dr. Yim's own words, "I'm really surprised - her marrow is clear". We were later laughing and hugging nurses in the hallway when the doctor overheard and said, "Don't open the champagne just yet". I say, celebrate the small victories and open that bottle of champagne. If, 5 years from now she's still with us and NED (no evidence of disease), we'll open a case! We are waiting on the pathology results from a lab in LA to determine if there is any minimal residual disease in the marrow (this test can detect 1 cancer cell in 100,000 cells).

So, here's the new treatment plan (changes every month, doesn't it?) This Friday, Shauna has a full body PET scan. This is primarily to check the primary tumor site and the metastasis to the right sinus. The doctors also suspect a metastasis in the lower left lung. Areas of active cancer cells should show up on the scan. With the results of the scan, tumor biopsy, and bone marrow aspirate, we will sit down with Dr. Yim (hopefully next Wednesday). Shauna will also be seen by a Radiation Oncologist to determine if she is a candidate for radiation. So, radiation may or may not fit into the picture. If there is no minimal residual disease, she will have a round of chemo followed by harvesting of her stem cells in preparation for a bone marrow transplant. If there is disease, she'll probably still get more chemo followed by another biopsy. The transplant would take place at Oakland Children's Hospital. It would involve the highest dose of chemo yet and her recovery period would be the longest. I'm terrified for her but at the same time, I suddenly feel there may be hope of winning this monstrous battle. For now, the vaccine at Texas Children's will be placed on the back burner (not such a bad thing because the vaccine trial is still on hold by the FDA).

I'm sitting here at the keyboard, eyes closed, trying to capture in words how I'm feeling about all this now. The stress is still there, yes, definitely still stressed, but somehow it feels different. Maybe this will describe it - Wes and I are (I'm embarrassed to say) sometimes relaxed about making Shauna sit in her car seat. How can we make her endure a discomfort that we can relieve with all that she endures and we have no control over. On Friday, as we were driving home from the hospital, Wes said we should make her stay in her car seat, because perhaps now her chances of dying in a car accident were greater than her dying of cancer.

Please continue to keep Shauna in your thoughts and prayers - it appears to be working!

Love to all,

Kathy

October-2000

 Dear Family and Friends:

I must have sat down to post an update on Shauna a dozen times over this past month. But I wanted to give the task quality time and there just didn't ever seem to be any. I my last post, I announced that Shauna was classified as NED (no evidence of disease) and we were headed down the path for a Peripheral Stem Cell Transplant (similar to a Bone Marrow Transplant). In late July / early August, we took Shauna to Children's Hospital of Oakland for the process of collecting her stem cells for the transplant. It took 5 days to collect enough cells for the process, partly because her system wasn't ready to shed the cells at the start of the process and partly because her central line was of a small diameter and the pheresis machine had to be run slowly.

Next came the waiting period to hear if the collected product was free of Neuroblastoma cells. Results: some of the daily collections were and some weren't. But one day's collection was negative for NB and large enough for the transplant. So we proceeded with the pre-admission staging tests (bone scan, chest x-ray, eye exam, hearing exam, bone marrow biopsy and aspirate, kidney function, dental exam). It was during this battery of tests that NB was found in the aspirate and back in her right sinus. The transplant was cancelled.

Well, by the very next day, Shauna was admitted to Sutter Memorial Hospital in Sacramento for chemo. She was placed on the Topotecan/Cytox trial. She had 5 days of chemo and was re-staged on September 14th. Results: no change in disease, classified "stable". The doctor said that Topotecan is a slow acting drug and expects to see better results after the second round (finished this past Friday). Shauna's doctor said he'd like to do 4 rounds of these drugs, every 21 days. If after any round she's NED, off she goes to Children's Hospital of Oakland for the transplant. If her disease remains stable, we discuss another course of treatment. If her disease progresses, she goes back to high dose chemo. So, we're once again considering an anti-Id study or a gene therapy vaccine. We'd prefer the autologous gene therapy vaccine, but the cells collected from Shauna's marrow back in April might not survive long enough to create the vaccine. They may have been too damaged by previous chemo.

So, for the time being, we're back in limbo, taking one day at a time. Shauna is doing well. She is tolerating the two chemo drugs well but still needing transfusions between courses of chemo (keep donating those blood products!!!). I met a man at the local Big-O Tires shop who said he donates platelets as often as they allow. I wanted to hug him!

That's it for now. If you'd like pictures of the apheresis machine, Shauna and Kayla at the circus, or Shauna at Chevy's, please email me privately. Take Care everyone.

Kathy

February-2001

Shauna has been cleared for take off!!! The doctors at Children's Hospital Oakland say we can start planning to return home - not just as far as Sacramento - but HOME !!! Okay, so we just got settled in at the Berkeley house, now we switch gears and figure out how/when to move East. But not too soon for us. Although it's mostly been a positive experience, here in the Bay Area, as the odds would have it, our car was broken into last Saturday night - right in front of the house! Admittedly, I kept too much stuff in it and it was a tempting target. The neighbors tell us they keep nothing more than their child car seat in their car. Still, the perpetrators got away with a lot of booty and did damage to the car in the process. If you've ever been the target of a robbery, then you know the feeling of violation and vulnerability that follows.

Moving on, it's day +97 or +61 (post transplant) depending on which stem cell infusion you go by. Shauna's needing platelets about once a week and packed red cells about once every two weeks. She's still on TPN (intravenous nutrition) but is keeping down 3/4 pre-transplant volume of food (by G-tube) so I expect the TPN will be stopped soon. Her energy and spirits are up. We are hopeful.

Take Care everyone,

Kathy

April 24, 2001

I'm sorry that it has taken me so long to provide an update on Shauna. It is infinitely more difficult to report bad news than good.

It is with great sadness that I tell you that Shauna is loosing her battle against cancer. It was discovered in mid March that she had relapsed, just four months out of transplant - the cancer was again in her marrow and sinuses. We tried another type of chemo on a Phase II Trial, but within three weeks, the disease had progressed. The doctors say this is major progression. For many reasons, we have turned to home care and Hospice. Enough is enough! She has fought a brave battle, but her little body can't take any more poison, and her head can't cope with any more procedures.

Unfortunately we were unable to take her Make-a-Wish trip to Disneyland this past weekend. The pain medication has her sleeping most of the time and she is easily agitated. I am hoping that Make-a-Wish can bring a little bit of Disney to her instead.

Well, my little Tinker Bell is sleeping sweetly now and it's a good time to get a few things done. Thank you all for your continued support through your thoughts, prayers, and e-mails.

Take Care,

Kathy

May 9, 2001

 Shauna Marie Palmer

February 13, 1996 – May 6, 2001

Our little princess, Shauna Marie Palmer, passed away peacefully at home on Sunday evening, May 6^th. Just as she came from my womb, she took her last breath while lying on my chest with Wes nearby. She bravely fought an 18-month battle with cancer but in the end, her battle was lost. In our hearts, she will forever be five years old.

Although brief, Shauna's life was full. She enjoyed many vacations including a trip to Disney World in Florida. Shauna found much joy in her Disney videos of which she had committed each to memory and knew every song. She especially enjoyed the Disney princess characters and insisted on only wearing their character nightgowns. Shauna loved being in the water, be it in a tub, pool, lake, or at the water park. When she wasn't in the water, she was pouring 'tea' from one of her many tea sets. She will be remembered for her bravery, perseverance, impish smile, and ability to inspire. Throughout her lifelong medical ordeal, she was known to always remark, "Don't worry. I'm fine."

Shauna’s memory will be served at a Celebration of Life gathering at her Nana’s house on May 21, 2001. I know that you will all be there in heart if not in body. If you would like to e-mail me a precious moment, memory of Shauna, or send your well wishes, I will print them out to be stored in her Memory Box.

Thank you all for your generous support during these last few trying months, her lifelong struggle with her syndrome complications, and her fight against cancer. You helped me to remain strong, stable, and sane.

Memorial donations in Shauna’s name appreciated to The Noonan Syndrome Support Group, Inc., P.O. Box 145, Upperco, MD 21155, an organization offering information and support to those whose lives are touched by Noonan syndrome.