Seattle Bound 10/25

Evelyn continues to run 102 fevers despite ibuprofen & Tylenol. Her pain has spiked, especially after the aspiration this morning. Preliminary results from the aspiration show a possible infection, so the fluid was sent for culture. It is still very difficult to say what is going on in her leg. The all-around conviction is that this central line needs pulled. The doctors feel that our best bet for treating this infection with its complexity and variables, along with the fact that she will need a new line placed some time down the road, means we should probably be in Seattle. They have Pediatric Infectious Disease, Pediatric Orthopedics, Etc; a very diverse and specialized unit that can hopefully get a plan in place for recovery. There is talk about possible cleaning of the joints to remove the fluid if it is infected, but they are leaving that up to the Seattle team. Life Flight is here now to take her & Kevin by airplane. She is comfortable with strong pain meds & her fever has decreased for the time being. Hoping for a smooth ride over this Evergreen state! Remember us.

Night 1

Evelyn had an okay night last night, although her fevers have returned and she had a “toxic” reaction after her antibiotics were given last night. We are so glad we brought her in when we did! The hospitalists and Infectious Disease doctors strongly feel after having 5 days of very strong antibiotics that the chances of clearing Ev’s line of infection is slim. They are willing to try adding on a second antibiotic as well as “locking” her line with the strongest one (they leave the antibiotic sitting in her line for at least 4 hours, so this will be tricky with her TPN needing to run over 22 hrs in a day). They do not like to wait too long while they are actively sick, since this means we are only losing ground, so if this does not show significant improvement in a reasonable amount of time, they will need to remove the line. The bug that is growing is called coagulase-negative staph, a relatively reasonable bug to treat through in a line infection, but after finding that the bug she is growing is the same bug she was infected with 3 weeks after the onset of her foot pain back in May, this makes us wonder if it is a bone infection, and in that case the two sites of colonies (line and bone) will likely feed each other. The line will most likely have to go. She also has the worry of her fresh vascular stent (from January) being a potential area of growth, since a new sheath of vascular cells is created over top of it that heals in the months after it is placed, thus a perfect breeding ground for bacteria as well. This is less likely, since scans showed it was fine during May’s infection. They performed an Echo on her last night to check this out; they hope to have results sometime this morning. She will also have an aspiration of her knee and ankle sometime today to culture the fluid and test for white blood cells and other markers that would indicate infection.

She was made much more comfortable after receiving IV Tylenol & Toradol (ibuprofen) last night.

Thank you all for your encouraging words, thoughts and prayers. We can surely feel them! Will update as we can.

Inpatient

Ev is being directly admitted to the hospital. Her MRI yesterday showed fluid buildup that the radiologist feels we should definitely aspirate and culture. Her headache has worsened today, she is very dizzy and her blood counts from yesterday have significantly decreased since Friday. Her blood cultures from yesterday have already come back positive today. Please remember us that God will give strength, as He always has in the past.

More Good News…

Here we are again sending another post out, but it’s worth it, we promise! Doctors decided they will send her home instead of admitting her since she’s had no fevers today and seems to be improving. She will go home on two antibiotics, both of which will only be once daily, and tomorrow they should be able to determine which medicine the bacteria is susceptible to, so we may be able to reduce down to one again. We have never been able to leave this early on with a bacterial line infection, but there’s always a first for everything! Thankfully the bug doesn’t seem as vigorous as others she’s experienced. Thank you for the prayers!

Ev to the ER…x 2

(10/19/17) We’ve had a busy week with Evee, as she had a severe bout of pink-eye (that doesn’t seem too contagious…yet) that has cleared up significantly after starting antibiotic drops with steroids in them. Then, Tuesday night/Wednesday morning (4am) she starting running a fever so we brought her to the ER. It got up to 104.7 in the ER but came down with IV Tylenol to around 100. She was sent home after blood cultures were done & a dose of antibiotics was given, since her labs looked “okay.” At this point I felt things could go either way, viral or bacterial, so we kept up with Tylenol and as the day wore on yesterday she began to perk up. Her last dose of Tylenol was at 8 pm last night and she woke up without a fever this morning, so I really started to believe maybe it was the bug the little girls had last week. So, I was much surprised when the ER called around 10:30 this morning to say she has a line infection! So we headed back in today to get treated and hopefully this will be a quick stay since she is already doing so much better!

We do have a bit of good news though; we’ve finally received insurance approval to have yet another MRI done, since the rheumatologist in Seattle was not satisfied with the quality of the other images. Her bone scan of her leg & foot does certainly show something is going on, but doesn’t really tell the nature of what it is. So, Monday the 23rd she is scheduled to be under sedation & hopefully these images will be more successful. I will try to keep everyone updated as I can! Thanks for continuing to remember us in prayer.

Evee’s Foot Pain

We would like to add a little update since last Thursday to let everyone know how things are going with Evelyn’s foot/knee.  We have allowed her to “baby” it for the past week and the swelling has gone down significantly; however, the pain is still excruciating if she uses it at all and if it gets bumped. After hearing from the Pain Doctors that they are concerned it is not the Pain Syndrome (a.k.a. CRPS) we felt it would be best to expedite the process of getting her evaluated sooner rather than later, especially since it seemed the pain had worsened in the last several weeks.  So, at the beginning of the week, Kevin brought her into the ER and they did an MRI without contrast, with a plan to use contrast at the end. Unfortunately, Ev was unable to finish the MRI to get the contrast study done. From what they did gather, Evelyn’s MRI showed a “change” compared to the MRI done in July, but other than that it was not very comprehensive. There was obvious effusion, or fluid buildup (hence the outward appearance of swelling), but no obvious tumor, infection, or injury. The rheumatologist from Seattle said on Monday that they would still like something with contrast so as to rule out infection or malignancy that may possibly be festering.

On Thursday (10/5), she had a bone scan that showed her knee and her foot bones were definitely inflamed. We finally received report today (Saturday 10/7). The radiologist does not think that the bone scan is consistent with Complex Regional Pain Syndrome, and wonders if Evelyn could be suffering from an autoimmune adolescent disease called Chronic Recurrent Multifocal Osteomyelitis (CRMO), which is in easy terms inflammation of multiple bones. Often, this disease is known to plague kids with long-term GI inflammation (go figure!) or skin psoriasis and can take years to diagnose. Evelyn’s pediatrician is passing this information on to the rheumatologist in Seattle again (they also have a radiologist who will be reading it on their end as well) and hopefully we can get a plan going forward. Usually, this inflammatory disease is a diagnosis of exclusion, meaning they must rule out infection of some sort before giving her a formal diagnosis, so we will wait until Monday to see if the rheumatologist would like to aspirate the bone or swelled tissue first or start some steroid injections as a trial run to see how she does. Thankfully, Evelyn has had IV ibuprofen (Toradol) going for the last several days and her inflammation has improved significantly. Unfortunately, it is not a long-term solution, so hopefully we can get a plan set in place quickly and bring her relief soon!

Seattle Trip

So! An update finally on the status of Evelyn’s surgery in Omaha, but it may not be what we all were expecting.

As many may know, back in May, Evelyn’s right ankle began to give her grief, to a point where we went to numerous doctor appointments, ER visits, x-rays and even an MRI to pinpoint the reason for her pain. Nothing was at all definitive and we were left thinking that she may have a pain syndrome that is causing her nervous system to not work right. Since then, her ankle has had times that are better, but just recently it has flared up again and the pain and swelling is now in her knee as well.

We took a trip today (9/28/17) to Seattle Children’s to visit with the Pain Specialist about Evee’s pain to learn techniques to help her cope. However, the Specialist who saw her was somewhat concerned because not all of the symptoms Ev is presenting with necessarily go with the pain syndrome they were thinking. They quickly added on labs and an X-ray of her foot and knee, as well as a visit with a rheumatologist. The rheumatologist was ever gracious and stayed after hours to see her! Between the labs and the rheumatologist’s exam they cannot be sure that it isn’t an infection, but the impression we were left with is it most likely is not an arthritis nor the pain syndrome as they had first thought.

So, for now, until we rule out infection, we will most likely need to wait on her intestinal surgery. Omaha is working with insurance still to figure out paperwork before we can schedule; now we can see very clearly why God has delayed her surgery! For now, we must be patient and hope and pray that God will reveal what is next for little Ev, in His time.

Transplant Evaluation

On August 14th we flew to Omaha for an Evaluation for an Intestinal Transplant. After 4 days of testing and meeting the transplant team, the Intestinal Rehabilitation Specialist/Transplant Surgeon, Dr Mercer, told us that he thinks Evee is a long ways from transplant, since her liver is still quite healthy (this starts to break down with long term use of IV nutrients).  His first questions for us were whether or not she had time in her life where her intestines worked at all, and after hearing that she has had times, although they certainly weren’t perfect, even when they lasted a week, he felt that there is still hope for finding out what makes Evelyn’s guts tick. He would like to work with the intestines she has before going the transplant route. His plan is to repair a dilated section of her small intestine and he is hopeful, since she still has a large amount of her own small bowel still and there is a non-dilated section to work with if all else fails. They said to expect any number of hours for the surgery itself. Her stay will be 2 weeks in the hospital and two weeks staying close by; after this we would plan to see him routinely as needed.  So a month in Omaha on our own schedule is much better than the 6 months we expected for transplant with only 6 hours notice.  The risk of the surgery is much less than transplant and Dr Mercer is optimistic it will help her. If you would like to meet Dr Mercer, here is a video from their website; it is about a girl who had a surgery done by Dr Mercer as well. This is called the STEP procedure, which is Plan B if he finds it a better option for Evee, once in surgery. The surgery he is planning to do does not need to necessarily lengthen her bowel, as the STEP procedure does, but simply make its diameter smaller by cropping the intestine down its length, making all the intestine the same width. If much of her bowel is lost he will utilize the STEP method.

So for now transplant isn’t in the near future, thanks be to God.  We hope to schedule surgery for early September, but are still working on getting insurance approval.