Bethany and Heart Surgery

This a post to show that Bethany could have had her heart surgery before she deteriorated more and it became critical and a not so idea situation. These posts will show that Kate stated in Bethany’s Caring Bridge that she was steroid free and she stayed that way for 4 months. The end of Oct 2011 she began to get more ill again and was on steroids again and ultimately had to be put on stronger medicines also. She would go another year trying to wean her again before they were able to do her surgery.

The reasons behind not doing her surgery the end of 2011 was Joshua being to ill for her to be in Portland for 2 weeks. Mind you when she did finally get her surgery she only spent 5 days inpatient. I am sorry but with 4 adults in the home someone could have been by Bethany’s side when she needed proper medical care.

There were occasions when Bethany was very ill and Joshua needed to be away at the hospital and it was never an issue. Joshua went to the hospital and got the care he needed. If anyone wants proof of that I will find the post that overlap and post them.

*The rest of this post will be really long as I am going to post postings from CB and will keep the entire posts in tact.*

  • Written Jun 13, 2011 9:33pm

Bethany saw her rheumatologist today. She has been pretty grumpy due to joint pain since her steroids were weaned down. Dr. K seemed disappointed that she has not responded to treatment as had hoped she would & said he felt it was time to try injecting cortisone into Bethany’s joints to try & knock the arthritis down that way. So, on Wednesday morning, Bethany will be sedated & will have injections in her ankles, knees, wrists, fingers & possibly her elbows & shoulders (it will depend on how things look to the doctor when he has her sedated & completely relaxed).

She’s seeing her cardiologist tomorrow. I don’t know if he’ll want to schedule a heart cath yet or not, but I’m hoping he says it can wait for another 6 months. I’m really in no rush to go the heart cath + surgery route with Bethany. We know it’s in her future, but hopefully it won’t be any time soon.

  • Written Jun 15, 2011 9:36am

The cardiologist appointment didn’t go as I had hoped. Bethany needs open-heart surgery to re-do the repair that was done in Ukraine, but her arthritis needs to be under better control first because her being on steroids puts her at risk for a lot of complications, primarily problems with wound healing. Her rheumalologist will try to get the JRA chilled out so the cardiologist can do surgery in a couple months.

  • Written Jun 18, 2011 12:47am

We’re home after our week up in Portland. To summarize what we learned with regards to Bethany, the rheumatologist injected cortisone into her joints & we’ve taken her off Prednisolone completely in order to get her into good enough condition to undergo open-heart surgery before the end of the year so the cardiologist can repair her leaking mitral valve & fix the holes in her heart (one in the atrial patch that was placed in Ukraine & the other in her ventricle). He will also try to lower her pulmonary hypertension through some procedure, but I didn’t fully grasp what that was & I figured it wasn’t something I needed to pursue understanding of until the time got closer to her actually having surgery.

Bethany is almost 30 pounds and still 34 inches tall. Her rheumatologist said she won’t grow as long as she’s on steroids, so I’m hopeful that once the cortisone & Prednisolone are out of her system, she will be able to finally begin adding some height to her frame.

The decision to take her completely off steroids was one that Dr. K & I discussed in depth. Ultimately, the truth is that juvenile rheumatoid arthritis causes pain every day in a child’s life & there is really no way to get rid of it completely. As such, as heartless as it may sound, Bethany will have to learn to live with a certain degree of pain. It is our hope that she will be able to live without daily steroids. It is our further hope that being off steroids will not result in her arthritis flaring up & that whatever pain she has will be tolerable for her. Only time will tell whether or not this goal is possible to achieve, but it is what I’m praying for. It’s just not healthy for a person to remain on steroids forever… it will wreck their body & cause lots of problems. I want what’s best for Bethany, which would be to not be on steroids, so it’s something we’re going to try & see how she does with it. We will continue giving her chemo once each week, along with her other medications.

So far, she seems to be tolerating the change all right, but that could be because she got cortisone injected into her joints & they’re not hurting as badly as usual. Again, time will reveal more & her treatment plan will evolve as the need arises. Hopefully, though, she’ll be stable enough within 2-3 months to schedule her heart surgery & we can then get that over with. It would be great to get Bethany to a place where she’s healthy (or as healthy as she can be) so that we could maybe see more of her personality when it’s not clouded by symptoms of her heart condition or arthritis.

  • Written Sep 11, 2011 5:41pm

Bethany’s rheumatologist called to chat. I was supposed to take her & Joshua to Portland for appointments, but with Joshua being terminally ill & unable to travel anymore, I had to cancel the doctor visits. Dr. K wanted to know how Bethany was doing off steroids, though, so he called me.

I told him that Bethany was doing really well, actually. She’s lost 5 pounds (that was steroid weight), and her hair is growing & looking so much less brittle and ‘straw-like’ than before. Her moods have improved & she is happier than she was when on the steroids. She still has many challenges ~ not least of which is her autism ~ but her body is obviously happier when it’s not being given Prednisolone. She’s had times when we thought she might be beginning to flare, but then things have settled down each time (prayer works). Dr. K was very happy to hear such a positive report.

He told me that he does NOT think Bethany has a second autoimmune disease. He believes that the test showing her genetic marker for a spondyloarthropathy does not mean anything important. As such, we’re back to her having “just” S-JRA. That’s fine with me since that one can cause enough trouble all by itself. I’m relieved that Bethie doesn’t have more going on; that’s a blessing.

With regards to Bethany’s cardiac situation, we are seeing more blueness in her hands & feet & she tires out more quickly than she used to. I agreed to schedule her surgery once things with Joshua are resolved, so we have that on the ‘things to do’ list.

From a medical point of view, other than her heart, Bethany is doing all right. Her arthritis appears to be in remission right now & the only medication she receives is a weekly shot of Methotrexate. She & Hannah will be getting their AFOs on Thursday and it’s my hope that Bethany spends more time upright once she has a solid base of support. She has such low tone in her trunk, hips & knees that getting her to a point where she can learn to walk will take a lot of work. I am hopeful, though, that we can achieve that goal sooner than the physical therapist & orthotist anticipate it occurring. Other milestones we are working toward include teaching Bethany to eat solid food & overcoming her extreme aversion to using her hands. I want to be able to send her to preschool next fall (I am not going to attempt to homeschool either her or Hannah) & it would be great if she could get something out of it.

When I schedule her heart surgery, I’ll update the blog again, but I don’t anticipate having much to say here until then.

  • Written Oct 4, 2011 10:31pm

Bethany has a kidney infection. Poor baby girl had to get labs drawn & that took 3 different needle pokes to accomplish. Each poke drew blood, but her veins would blow before the phlebotomist was able to get the full volume needed for the tests that the pediatrician ordered. It was so sad! She also got cathed for a urinanalysis and, once we knew that she had at least a UTI, the nurse gave her a shot of Rocephin.

The labs showed that Bethie’s illness does not appear to be related to her arthritis, which is wonderful. We really need to keep her off of steroids so that she won’t have any complications with her heart surgery & I didn’t want to see her ‘set back’ by months if she had to start steroids again and then wean off of them, so her not needing them is a huge relief.

We’ll find out tomorrow (I think) what bacteria the lab grew out with the urine culture. If it’s a drug-resistant e-coli like she’s had twice before, it’ll require IV antibiotics, but if that happens, I would push for her to get a PICC line so I could give her meds at home. I would not want to leave Bethany in the hospital without me (even though she’d have her big sister with her, so she wouldn’t be alone), but I couldn’t leave Joshua (http://www.caringbridge.org/visit/theyoungestof7) for 2 weeks, either. Adding in the fact that we’re moving in 9 days and, well… the timing of a hospitalization right now would be tough, so I’m praying that it doesn’t happen. I’m praying that whatever bug has invaded Bethany’s kidney will be treatable with oral antibiotics and that it stays in her kidney & doesn’t get into her blood.

She was 24lb, 11oz today ~ that’s a loss from a few months ago, but since she’s been throwing up, no one was surprised ~ and she’s 35 inches tall. Our ped’s nurse calls her a peanut, which is an apt name for Bethie considering she will be 4 years old next month.

  • Written Oct 25, 2011 10:15pm

Bethany is having a major arthritis flare. Her inflammation markers are WAY up & she is miserable, with fever, shaking, swollen hands, fingers, wrists, ankles, feet & knees. She cries if we move her at all & she throws up some of her feeds. Poor baby girl is hurting terribly and I feel so sad for her.

She’s back on Prednisolone to control her flare & Zantac to protect her gut while she’s taking the steroids. It will be awhile before she is off of them completely, which means we won’t be able to do her heart surgery soon after her brother passes away, as planned (Bethie has to be off steroids for at least 2 months before surgery can be done due to the risk of serious complications). I’m frustrated by this development because it’s not in Bethany’s best interest to be postponing the surgery & the only reason we have thus far is that I can’t be 250 miles away from Joshua for 2 weeks. She was satting at 92% today, whereas she typically has been in the 95-97% range, so that makes me a little nervous, and her hands & feet remain cold despite having a fever of 102+, so that makes me hope her pulmonary hypertension isn’t worsening.

I feel sad that Bethany has to be back on steroids. She has become SO much happier since getting off of them & losing all of the steroid weight she’d gained (she now weighs a healthy-for-her-35-inch-height 24lb,11oz). I had to give her a “pulse” of steroids tonight ~ that’s one big dose of steroids to kind of shock her system & hopefully begin knocking down her immune system so it will chill out again. Tomorrow, she’ll start getting steroids twice a day. I’ll take her in for more labs in a few days to determine if we’re getting this flare under control or not. Hopefully we’ll see her ferritin, CRP & sed rate lowering. If not, she’ll have to be put on a higher dose of steroids until her immune system begins behaving again.

This bites. 

JUNE 18, 2011 to OCTOBER 25, 2011 BETHANY WAS STEROID FREE AND COULD NOT HAVE HER SURGERY BECAUSE JOSHUA’S ILLNESS TOOK CENTER STAGE. PAY NO MIND TO THE FACT THAT BETHANY WAS GETTING MORE BLUE AND TIRED. SHE COULD HAVE JUST AS WELL PASSED AWAY DUE TO HEART FAILURE WHILE WAITING. SHE WAS ONLY REQUIRED TO BE STEROID FREE FOR 2 MONTHS SHE DOUBLED THAT AND STILL NO SURGERY. 

By the beginning of 2012 she was so bad off she was having another medicine added because the steroids weren’t controlling her arthritis.

  • Written Jan 31, 2012 6:31pm

Bethany’s arthritis is flaring even on steroids. Her rheumatologist seemed disappointed. She was rashy, swollen joints, painful & had a 101 fever when he saw her today. Our next step is to have Dr. K petition our insurance company to cover Actemra, the only medication approved by the FDA for treatment of children with systemic JIA. He said they’ll cover it; he doesn’t foresee there being any hassle about it since she’s been on steroids & methotrexate for a year without gaining remission, so she obviously needs something different. Once we have approval, Bethany will come back to Portland to get a PICC line & have her first infusion & then we’ll do infusions every 2 weeks at our local hospital with our pediatrician overseeing things (in consult with the rheumatologist). If the Actemra helps her, then the rheumatologist said he would want Bethany to have a port placed for permanent central line access. I’m to ask the cardiologist tomorrow if it will be okay to do the PICC & will Bethany be okay to have a port placed down the road, as well.

Meanwhile, cardiology called again & said that the cardiologist won’t be available tomorrow, but because we traveled 250 miles for the appointment, they are putting Bethany in with a different doctor, who can do her echo. I have no idea if this guy she’ll be seeing is any good & I have no idea if he’ll be able to answer my questions regarding being able to do surgery even with Bethany on steroids (rheumatologist said we are “nowhere near” being able to get Bethie off steroids, so cardiologist needs to figure out a Plan B if we’re going to get surgery done any time in the foreseeable future). I’m feeling a bit like tomorrow will be a waste of time since we don’t get to see “our” cardiologist, but who knows… maybe this other guy will be okay & will provide answers. What I think will happen is that he’ll say that I need to talk to our regular cardiologist since he (our regular guy) knows Bethany better & knows what’s going on in conjunction with rheumatology. Still… maybe I’ll be pleasantly surprised & he’ll be a great guy who wants to be really helpful. I can dream, right?

Oh ~ I forgot to mention, Bethany is 29.1 pounds (13.2 kg) & STILL 35 inches tall. The rheumatologist said that kids with systemic JIA are stunted in their growth & that Bethany really won’t grow as long as she’s on steroids. Add that to Bethie having Down syndrome (where the average adult female height is 4’9″) AND having been profoundly neglected & malnourished in a Ukrainian orphanage for 3 years (thus affecting her height, too) and we don’t know if she’ll get more than 1-2 inches over 4 feet tall. I guess that since no one ever expects her to be able to walk, at least she won’t be super-big, so that’ll make it easier to move her from place to place.

  • Written Feb 1, 2012 9:18pm

Today’s appointment with the cardiologist left me feeling truly dejected. He was a nice guy ~ infinitely more personable than our usual cardiologist ~ and he impressed me at the beginning of the appointment by sharing that he had called the rheumatologist prior to our arrival to discuss the unique situation that we have in Bethany. That was a point in his favor, for sure, & I appreciated that he already had an understanding of what was going on with my daughter before he ever met her.

He had me bring Bethany into the echo room & lay her on the table. Bethany never fights any kind of medical procedure, so she lay still & quiet for the duration of the echo, which was about 45 minutes long. During the exam, the cardiologist would quietly speak to the tech, asking her to do different things & focus on different areas. They spoke low enough that I could not really understand anything being said, but then, after repeatedly requesting one view that the tech was struggling to get, the cardio raised his voice enough that I clearly heard, “You have to hold the angle! Everything is leaking in there!” Then he told her to let him do it, himself, and they switched places.

Let me tell you, “Everything is leaking in here,” is nothing a parent wants to hear with regards to their child’s heart. I bit my tongue to not say anything for the remainder of the test.

After the echo was complete, the doctor told me to get Bethany dressed & then he’d come in to discuss the results with me. Five minutes later, we were sitting opposite a table & he was laying out the details: moderate leakage of the mitral valve, mild-to-moderate leakage of the tricuspid valve, mild leakage of the aortic valve, moderate pulmonary hypertension (increased since her last echo), an ASD (hole between the 2 atrium of the heart) and a VSD (hole between the 2 ventricles of the heart). The ASD has gotten a little bigger since her last echo. The VSD is not large. Oh, and “her lungs aren’t perfect,” which was explained to me as being the result of her pulmonary hypertension causing her lungs to look “fuzzy” during the echo.

Surgery is needed, but with Bethany being steroid-dependent & having her arthritis out-of-control, there is absolutely no way the cardiothoracic surgeon will touch her yet. At this point, her heart defects are not life-threatening. If they were, I was assured the surgeon would do the necessary repairs regardless of Bethany’s inability to heal properly. For now, we can wait & try to get her arthritis under control by using the Actemra infusions & (hopefully) subsequently wean her down on steroids. The cardiologist said they’d be willing to do the surgery if Bethany can be brought down to a low dose of steroids (though they’d anticipate problems with healing and/or slow healing, so she might wind up staying in the hospital for longer), but when I asked what dose that would be, the cardiologist admitted he didn’t know yet since they don’t have a set protocol for this. He only said it would have to be substantially lower than what she’s currently at. Thankfully, her heart CAN be fixed (with the exception of the tricuspid valve; the cardiologist told me there’s not much they can do about hers). Prayerfully, we’ll be able to get her heart repaired sooner than later.

This news, coming so soon on the heels of the rheumatology appointment yesterday, hit hard. I want to cry ~~ Bethany has needed heart surgery for almost a year & we can’t do it because we can’t get her arthritis into remission & her off of steroids long enough to make surgery possible. Meanwhile, her heart slowly gets worse & that is negatively affecting her lungs. Her joints are swollen ~ she won’t use her hands at all because her fingers are so puffy & painful. Her right ankle is trying to “freeze” into an abnormal position that keeps her toes pointed downward, so I have to gently force her foot into a 90 degree angle & then use her AFO to keep her foot locked in proper position. My maneuvers cause her pain, but I have no choice but to do it.

Sometimes I am so utterly overwhelmed by how much can go wrong inside one little person’s body. First Joshua & now Bethany…. it’s just so sad. If I force myself to focus on the positive, all I can come up with is that at least there is one treatment option for Bethany. That’s better than we have for Joshua. For Bethie, it’s all riding on the Actemra infusions, which need to be approved by insurance in order to start. We need this medication to be approved & we need it to work. It’s the only medication approved for children with systemic JIA. What we really, really need is for the Actemra to make it possible to wean Bethany off steroids in a fairly-rapid manner without lots of adverse side effects so that we can safely get her heart surgery done.

I feel horrible for her. This is worse in some ways than what Joshua is going through because at least we can control Joshua’s pain. And on the flip side, at least Bethany isn’t terminally-ill. The sweet dolly, though… she got hit with so much in her little life & it just doesn’t make sense to me.

Once again, I am holding on to the things I know to be true ~ God is in control, He has a plan for Bethany’s life that is being worked out day by day & He is good all the time, even in situations that look anything but good to me. Those truths have gotten me through the hard times with Joshua & I believe they’ll get me through the hard times now with Bethie. I just wish it weren’t this way for my little girl…. after living through hell for almost 3 years in a Ukrainian orphanage, I wanted so much to give her a happier life. It’s disappointing that I can’t.

BETHANY WAS OFF STEROIDS FOR 4 MONTHS JUST A FEW MONTHS BEFORE THIS.

Starting the weaning process again.

  • Written Mar 8, 2012 3:21pm

We’re back in Portland. Yesterday, Bethany had her PICC dressing changed & labs drawn, then we headed over to see her rheumatologist. He was pleased with how Bethie is looking & I was pleased because he outlined a plan to get her off steroids. We are going to wean her very slowly since Bethany’s little system has shown us repeatedly that weaning is tough for her. I’ll reduce her steroid dose by 2mg every week, so if everything goes as planned, she will be done with Prednisolone in 8 weeks. That means that if all goes well, we could be scheduling Bethany’s heart surgery in another 4 months or so.

I asked Dr. K what happens if Bethany doesn’t tolerate being weaned from the steroids. He said, “Then we would have a problem.” We’re hopeful, however, that Bethie will do just fine & that in another 2 months, things will be looking much better for her with regards to having her arthritis controlled & being able to look at getting her heart taken care of.

Her lab results yesterday were as follows:
WBC 11,400 (down from 37,700 last week)
RBC 4.48 (down from 4.78 last week)
CRP <1.0 (about the same as last week’s 0.61)
sed rate 6 (up from 2 last week)
ferritin 786 (up from 712 last week)
hemoglobin 11.8 (down from 12.7 last week)
hematocrit 37.8 (down from 40.8 last week)
platelets 299,000 (down from 330,000 last week)
AST (SGOT) 34 (same as last week’s 34)
ALT (SGPT) 27 (up from 26 last week)

Tomorrow, Bethany will have an MRI of her spine to evaluate for an occult spinal dysraphism. Her appointment with the neurosurgeon isn’t until July 3rd (yep, 4 months from now), but I am assuming Dr. W will look at the MRI long before 4 months from now. I’ll get a copy of the MRI before leaving tomorrow & if I see anything obviously amiss, I plan to walk upstairs to Dr. W’s office & ask her directly to look at the scan. It wouldn’t matter if the MRI showed something that would require surgery ~ until Bethany is off steroids, she can’t have any kind of operation & her heart would take priority over her spine ~ but I’d like to know whether or not neurosurgery is also in Bethie’s future.

After her appointment tomorrow, we’ll return home, begin weaning B from steroids, do weekly labs & bi-monthly Actemra infusions & see how things go. I’m glad we finally have a plan of action & am praying things go the way we’re hoping they will.

  • Written Mar 12, 2012 8:16pm

We’ve weaned Bethany down just 2mg on her Prednisolone & now, 5 days later, she is increasingly irritable & beginning to vomit again, both of which are early signs that a flare is right around the corner. She gets her 3rd Actemra infusion on Wednesday. I’m interested in seeing what her labs look like. We’ll wean her down another 2mg on Wednesday, too, so I guess we’ll get a really good idea of how well the Actemra is working when we see whether or not it prevents her from flaring.

Here’s hoping it’s doing what it’s meant to. I am really not up for dealing with another flare so soon after the last one. I’m fairly certain Bethany isn’t, either.

I haven’t heard any results from the MRI she had on Friday. I’ll share results when I know anything.

  • Written Apr 12, 2012 12:30am

Today was an insanely-long day. Joshua had an MRI at 6:15am in Portland. Afterward, we drove home, where I dropped Joshua off & picked up Bethany in a 5-minute pit stop, then headed to the hospital 40 miles away for her Actemra infusion.

Since Bethany has no PICC (we’re going to try to use peripheral IVs for her infusions until we can get her a central line ~ a port ~ during her open-heart surgery), phlebotomy was called over to draw her labs (she’s a very hard stick). Then we waited for the results to come back. When they did, and everything was deemed by the pediatrician to look good, pharmacy was called to mix the Actemra & then the infusion was given. We were at the hospital for “just” 5.5 hours tonight. Still, it was a loooong 5.5 hours since I had to hold/wrestle with Bethany for most of it. She wanted to pull out her IV & I had to keep her from doing so. To say she was unhappy would be putting it mildly, but she survived it & was fine by the time we got home.

She is still grumpier than usual & she’s grinding her teeth all the time, but her labs look good, so they tell me she’s fine. Since I don’t know what Bethany’s default personality is (she’s been flaring or on high-dose steroids ever since we brought her from from Ukraine), I don’t know if she’s just naturally a grouchy child. It could be the autism affecting things, as well. Who knows?

She’s 3 weeks away from being weaned off steroids, which is great news. Once she’s off them, I’ll contact her cardiologist to ask how soon we can schedule her surgery.

Oh, speaking of surgery ~ Bethany will be having her adenoids removed & tubes placed in her ears on April 23rd. The ENT won’t need to use stitches (she cauterizes the vessels after removing the adenoids), so she doesn’t believe Bethany being on steroids will cause a problem. We’re hoping that removing her adenoids will open her airway enough to help reduce her sleep apnea since there’s no way Bethany would keep a CPAP on her face at night (the ENT said she wouldn’t even bother trying). We’re also hoping that the ear tubes will help with the mild hearing loss Bethany has. She doesn’t have fluid behind her eardrums, but she does have severe retraction of one eardrum.

  • Written Apr 21, 2012 10:25pm

I hate when I get behind in updating here because by the time I do get around to writing, there’s so much that has happened that I don’t know what I should say! I’ll just go with the abbreviated version of things that happened this week. 🙂

Bethany was supposed to have surgery to remove her adenoids & place tubes in her ears on Monday. That’s been cancelled. I took her to her pre-op appointment at the hospital only to find they had not scheduled either her pre-op or the surgery because her ENT had decided she wanted written consents saying Bethany could have surgery from her rheumatologist & cardiologist. Then anesthesiology decided they didn’t want to risk putting Bethany under because of her heart defect. I guess it didn’t matter to them that the cardiologist said it would be fine. What does he know, anyway, right? (yes, I’m being facetious) So… because it turned into such a hassle, we’re waiting until after she’s had her heart surgery, spinal cord surgery & has had a port placed. THEN we’ll deal with the ENT issues.

On Wednesday, Bethany developed a rash that looked very JIA-ish in nature. Her labs looked good, though (I was told), so no one took my concern about the rash seriously. I mentioned at the ped’s office that she was much more irritable & had begun some self-injurious behavior. It was blown off. “But she’s grinding her teeth almost nonstop & that is always a sign of pain for her.” “Kate, her labs look good, so don’t worry.” The only thing the ped’s nurse said the ped was going to do was call the rheumatologist to ask him if we need to do anything different this upcoming Wednesday (infusion day) since Bethany’s triglycerides had had a “slight increase” from 112 (normal) to 203 (high). I questioned how having any lab value almost double in 7 days could be considered a “slight increase” and was told only, “That’s what Dr. S said.” Truthfully, I drove home feeling frustrated. I felt that no one was looking at Bethany as a human being… they were focusing on her labs & ignoring how she looked, clinically. Megan (my oldest daughter) shared my frustration, saying, “Mom, she could probably die & the doctors would scratch their heads in confusion, saying, ‘But her labs looked great!'”

By Friday night, Bethany’s hands, feet & ankles were swollen. She had a low-grade fever, green snot was coming out of her nose & from around her g-tube, she was throwing up part of every feed, her rash was worse & she was so miserable she did not want to be touched. I emailed our pediatrician, asking if she was going to be on-call this weekend, explaining what was going on with B & I sent a picture for her to see.

Saturday morning (today), I received an email response from Dr. S telling me to take Bethany to the office to be seen & not to delay going. I took her in & she was seen by a nurse practitioner who, thank you God, LISTENED to what I was saying. It was obvious Bethany was sick, too. She looked horrible. The NP called the on-call doctor to come check Bethany out. The MD called the rheumatologist, who agreed with the NP & MD’s diagnosis of sinus infection as a side effect of Actemra that triggered a flare of her arthritis. The question was whether the infection had worked its way into her system since she had green goop oozing from around her g-tube, as well. The goop was cultured & sent to the lab to see what bug(s) grow out, then Bethany was cathed & urine was sent to the lab to be cultured, as well. We went across the street to the hospital so labs could be drawn & then we came back to the doctor’s office so Bethany could receive Rocephin (via injection) in her leg.

Her lab results showed multiple abnormalities, some of which could indicate a serious bacterial infection (or worse). Because of that, Bethany will return to the ped’s tomorrow for another set of labs & more Rocephin. If anything grows out of the cultures (urine, blood or green gunk) that isn’t covered by Rocephin and/or can’t be treated with oral antibiotics, we’ll have to admit Bethany to the hospital for treatment. Obviously, I am hoping to avoid that scenario.

The biggest disappointment to me in all of this is that the rheumatologist quadrupled the dose of steroids Bethany is on. We had her *almost* to the point of being weaned completely off Prednisolone & to have her need to go back up in dosage, even for a short time to control this arthritis flare, feels like a huge step backward. I can’t help but wonder how we are going to get her off the steroids AND keep her infection-free long enough to make it possible for her to have all of the surgeries that she needs. Right now, it doesn’t look very encouraging for my Bethiebean.

It took until August 30, 2012 to get Bethany well enough for surgery again. And then it was just because it was needed so badly.

  • Written Aug 28, 2012 10:59pm

I don’t want to sound melodramatic, but I am feeling concerned & would greatly appreciate prayer for not only Bethany, but also Joshua.

His oxygen saturation, which should be in the mid to high 90% range, is going down into the 70’s when he is upright & talking during the day & into the low 80’s when he is sleeping. I am positive this is contributing to his head pain, which is getting harder to control despite our giving meds every 2 hours. He asks me to gently rub the back of his neck because it hurts. The tubing that runs along the top right side of his head & down underneath the skin along his neck into his abdominal cavity to drain excess spinal fluid from the shunt in his head seems to be stuck in scar tissue, so any time the tubing gets stretched (like when he turns his head to the left) or if someone touches it when washing his hair or rubbing his neck, it causes him pain. We are giving him oxygen more often during the day to help his O2 levels get back to normal more quickly after each apnea episode.

I’m worried about Joshua. I’m nervous about how he will do being away from home for potentially 2 weeks. Stress makes things harder for him & being away from home will stress him. I desperately want to be able to keep him happy & relaxed as much as possible and I don’t want him to “tank” while we’re up there.

I’m apprehensive about this upcoming surgery for Bethany, which I was told today is being done not because it’s good timing, but because her heart is bad enough we can’t wait any longer to repair it. It’s actually not the ideal time because she’s only been off oral steroids for 18 days & she got steroids injected just 6 days ago.

I’m nervous about my appointment with Dr. T tomorrow since it’s going to be a tough conversation.

I’m nervous about the pre-op appointment with Dr. I tomorrow since he will be discussing all of the concerns the surgical team has with regards to the repairs they will be doing to Bethany’s heart. He will also be the final word on whether or not Dr. W can do Bethany’s spinal cord detethering surgery on September 10th (Dr. K said it was fine, but wanted to pass it by Dr. I, too).

I’m nervous about the pre-op lab draw because if the Actemra Bethany received 4 days ago (which technically should not have been given so close to surgery, but we couldn’t risk not giving it to her & having her flare) has made her neutropenic (white cell count too low) or if her platelets (cells that stop bleeding) are too low, they won’t be able to do surgery at all.

So… yeah… prayers and/or words of support, encouragement & comfort would be really appreciated. I’ve said before that it’s difficult having 2 chronically-ill, medically-fragile kids and at times like this, it’s REALLY difficult. Not just physically, but emotionally. Add in being away from my other kids & husband and… well… it’s just a good recipe for feeling overwhelmed & really, really sad.

Bethany finally got her much needed heart surgery August 30, 2012 

 

 

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3 thoughts on “Bethany and Heart Surgery

  1. There are SO MANY problematic, heartbreaking and traumatic things here that it seems daunting to consider all of them, but one seemingly throwaway line has stopped me in my tracks.

    “I guess that since no one ever expects her to be able to walk, at least she won’t be super-big, so that’ll make it easier to move her from place to place.”

    I follow a LOT of “Christian parents adopting special needs orphans from Eastern Europe” blogs, ranging from mostly-typical kids with minor medical issues to those who were severely neglected on top of having major disabilities, and NEVER has a single blogger said anything like this. The trend, in fact, is to have SO MUCH HOPE and faith that their children will reach beyond their potential, that they will achieve much greater gains than anyone believes, and they are blessed to have the opportunity to interact in any way with their child. None of these parents, even those with children who seem devastatingly trapped in damaged bodies, has ever spoken of their child in this way. It is always, “No one expected my child to _____ and look what they can do now!” or even “No one expects my child to ____ but that is OK because they are loved”. Never, ever is it “No one expects my child to ____ but this horrible thing will make that circumstance easier for ME.”

    I know KP tends toward the sarcastic, but to speak of the child you rescued from an orphanage, your child who has infinite worth in the eyes of God and the adoption community and those who care about folks with any range of issues including Down Syndrome — to speak of this child as if she were no more than a figurine (not even a sentient, animal-like being) that needs to be moved from place to place, that the EASE of such a transfer based on her LIMITED GROWTH BECAUSE OF SEVERE NEGLECT…………this is horrifying. There is no balance with anything else she might say here – any pity or sorry feelings for poor Bethie over what she’s had to go through in her short life. KP is only thinking of herself here, and she has placed herself squarely as the recipient of any abuse &neglect her child has experienced.

    I read the Caring Bridge for years and just recently found out about the arrest and court case. It’s haunting, fascinating, gruesome and horrific. I once commented to ask about Joshua’s unnamed “connective tissue disorder”, as I have Ehlers-Danlos and was curious about whether his diagnosis was similar. I received a haughty response along the lines of “The entire family, including ME, has a very rare connective tissue disorder.” Jeeez.

    • Anna – I asked her the same question because I have Ehlers Danlos as well and got a similar response. I found it odd that she wouldn’t name it because everything else she loves to write out each and every diagnosis, the rarer the better.

      • The conviently never named “chromosome abnormality” also doesn’t match the description of any one I know.

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