Day +87

Day +87 post bone marrow transplant 

Today Henley had a CT scan on her lungs. Although we don’t have the official report yet, her pulmonary doctor saw the same “cloudy” spots in her right lung that she had two months ago. We aren’t overly concerned though because her lungs sound really good. So we will go forward and just continue to keep an eye on that. 

Tomorrow morning bright and early Henley will have her bone marrow biopsy. She’s scheduled as the first case. 

Please pray that all goes well. No complications and that we have excellent doctors and that Jess doesn’t have to exhaust herself arguing to take Henley back for sedation like she has in the past.

Hospital Stay Begins for BMT

I posted this to Facebook on Tuesday. 

Thank you for all the prayers. Henley is doing ok - blood cultures have been repeated and will continue to repeat every 24 hours. 

~~~LONG POST~~~

Here's a little background as to what happened yesterday. First, she slept for a solid 6 hours during the night and had to be woke up for feeding. Very unusual for her. Second, she had blood that had backed up into both lines. This isn't ideal, but a call to the on-call doctor resulted in them telling me to re-flush both lines. I checked in with them again a couple hours later because her behavior continued to be unlike her. She was foggy acting, only nursed for 5 mins and hadn't smiled in an hour and half. The on-call told me it was probably because she was just tired and had a really long week - being anemic again didn't make sense because she had a red cell transfusion on Friday. 

My intuition told me it was more than that. I went with the on-call's thoughts for about 30 mins and then she started to feel warm to me. Took her temp and it was 100.1 - this technically doesn't meet "fever protocol" but we immediately decided to take her to the hospital and call the on call dr yet again to notify them. 

Once in the "ED" (emergency department) - they did labs and decided based on her behavior they would do blood cultures too. Those culture results take time to come back - usually 24 hours + They did IV fluids and she officially reached "fever protocol" and so they they did IV antibiotics. She seemed to pep up a little bit after fluids, nursed and we were sent home. The next couple hours she acted fairly normal. We were told we could give her tylenol and she fell asleep. I grabbed a much needed shower and went to bed too (I have been fighting a terrible cough and am still worn out from last week).

About 10 minutes after I climbed into bed Kyle's phone rang and it was the hospital telling us that Henley's blood culture was growing a bacteria and she needed to be brought to the hospital and admitted directly to the 4th floor ASAP. 

It was really upsetting, but we quickly grabbed what I needed and I took her in. 

Since getting her last night, additional cultures have confirmed there is a blood infection. Her team is optimistic that we can get this under control and continue on the current timeline of transplant - BUT there are not guarantees and things will be evaluated on a daily basis. She's continuing to get IV meds - a different kind than was given in ED. Her lab work doesn't look great. She's still very anemic and close to needing another transfusion of red cells and her platelets hit new all time lows everytime they are checked. I feel like it won't be long before she starts getting platelets transfused daily. 

Here's a few more bits of info/educational stuff based on dr conversations we have had -- 

A blood infection has really been our greatest fear for months. Neutrophils are responsible for attacking bacteria and she doesn't have many of those and the ones she has are known not to functional normally in SDS patients. 

Red blood cell transfusions should last awhile - sometimes up to 120 days... in the past Henley's red cell transfusions have gotten us by for awhile.

Platelets can sometimes need to be transfused multiple times a day. They are very short lived in the blood stream and it appears Henley's body is making very very few of these right now. Her doctors also suspect the ones she is making don't function normally. 

On a spiritual note when we got to the hospital room last night Henley started waving - smiling and then clapping her hands. She was looking at "someone" that I couldn't see. At one point she was laying on her back in the crib holding her unicorn and just smiling and waving like crazy at the ceiling. I managed to grab a couple pictures and prayed it was angels that were responding quickly to all the prayers being sent up. 

Thank you for being our prayer warriors. Thank you for helping us through this difficult journey. We have been told we are here, inpatient for the "duration" - likely til June.

I’ve had lots of requests for our mailing address and from some people asking if we have a Go Fund Me page. We do have a youcaring page 

https://www.youcaring.com/jessandkylejohnson-1150906

that was set up by my cousin for us. Kyle and I have struggled to express how others can support us. We know we need help- but it’s hard to figure out exactly what to say sometimes. We are especially grateful for prayers, because we need strength, patience and continued faith right now more than anything.

29 days til transplant

Day -29.     29 days til transplant 

We’ve had a crazy week of driving and it’s only Wednesday. Here’s a short update. 

Henley’s been struggling with her nutrition and weight gain for about 3 weeks. We saw her GI in Sioux Falls today and tweeked a few things, hoping we see some immediate improvement. 

Tomorrow will be a rest day. I’m fried and hope to nap when Henley naps. 

Friday we see an orthopedic doctor to review Henley’s skeletal abnormalities. This is a specialty that we haven’t yet seen- but her skeletal survey at diagnosis did note problems with her spine, hips and ribs. We just don’t know much about them. 

Weekend will be filled with more rest before starting work up Monday. 

Monday Henley will have surgery to place a central line and do another bone marrow biopsy. 

I can’t remember all the other appts that follow through the rest of the week without looking at the schedule. Taking things day by day. 💚

T-Shirts and Milestones

Last week our Twinkle Little Star turned 9 months old! In energy and spirit she continues to amaze us. I wish I could say the same about her bone marrow, but all three cell lines continue to drop off. I wonder if we will be able to make it to transplant without blood transfusions. She hasn't needed a transfusion of platelets yet, but that could be coming if those numbers continue to dwindle. On Friday she was at 74 and the normal range for platelets is 150-450. What's going up, is her weight. Slow and steady seems to be our normal right now. Not getting earth shattering gains, but we're gaining. We may need to increase her GCSF dosing (this is the shot I give her, so her marrow makes neutrophils), but this isn't unexpected due to the fact she is likely outgrowing her current dose. 

So to celebrate her 9 month birthday, Henley decided to give us a real run for our money. Last week she had an allergic reaction to dairy, which resulted in a 40 mile ambulance ride to the closet hospital. I've now added an Epi pen to our bag of goodies that must go everywhere. THEN the following day she was playing next to a wooden busy box toy, doing her usual bouncing dance an hit her head. It caused a deep enough cut next to her brow that we needed to head back to town and have that clued shut. She is really keeping us on our toes. 

The last thing I want to mention in this post is that we have started to sell some T-shirts as a fundraiser for bone marrow transplant. I'll post the link below if you are interested in buying one. There is also a great conversation starter shirt that has been really popular -- STRAIGHT OUTTA NEUTROPHILS :) A play off the movie STRAIGHT OUTTA COMPTON. I'd love to come up with another design that has a more western, cowboy spin on things to use as a future fundraiser for the Shwachman-Diamond Syndrome Foundation (by the way, I've been recruited as an Advisor to the Board and am helping them amp up their social media outreach!). The foundation does so much good for our small SDS community and we want to give back to them when we can. 

https://logomagicinc.com/healinghenley/shop/home

He said YES!

http://www.shwachman-diamond.org/rare-disease-day/

#curesds #bethematch #imgettingmarrowed #godisgood 

28,842 people have been reached by our post on Facebook about Henley's upcoming transplant! What a great opportunity to share the importance of donating your bone marrow and about research for Shwachman-Diamond Syndrome. 

Recovering From RSV

The twins are playing perfectly with our new in home helper and Henley is taking a nap. I started cleaning the entry way and mudroom and instead decided to take a rest, drink a cup of coffee and engage in some therapeutic writing instead. It might sound silly, but all these hours behind the wheel leave me lots of time to think, but not much time to unload those thoughts. So a lot of the time I just talk to God and ask for guidance and clarity, healing and rest! But sometimes I get 5 minutes to sit down...

Henley and I came home Saturday night, after being gone for a week again. She is not 100% but the worst of the RSV seems to be behind us. In addition to that her tube site FINALLY looks really good and that gives me more comfort being back home as well.

I came across this really need video that explains the function of neutrophils:

https://www.youtube.com/watch?v=FZxf1QDcEO0

We continue to boost Henley's neutrophils to an acceptable range with the GCSF shot she gets every other day. Then we do blood work twice a week to ensure the shot is working like it should. Without the shot she has ZERO.

Henley did have a shot a little over a month ago called Synagis. We credit another SDS family for sharing their story about fighting RSV for making us aware of this shot. Although Henley did still test positive for RSV, we believe that it probably lessened the symptoms. Being in Fargo and so close to the hospital (literally less than 5 minutes) made us so much more comfortable. She was also evaluated everyday from the day she first showed symptoms. There's no doubt that all the prayers you sent up for her helped keep her out of the hospital and gave her little body strength to fight through this awful illness. Now I'm just praying that her healing continues. She's still coughing and has a runny nose, but she's doing so much better.

We have learned so much about how his awful flu and RSV season is affecting so many families. I'm begging you, if you are sick, if you are coughing, if you are sneezing, if you have a tickle in your throat - please take measures to protect others around you. Use antibacterial gel, wash wash wash your hands. Wipe down surfaces. I think the flu and RSV are so strong this season that this is example of when sharing germs or thinking germs help build a healthy immune system is NOT the case. Although Henley did not end up being in the hospital inpatient - her and I did have to spend an entire week away from home again. I'm so happy to be home again with the twins and Kyle.

Two States in One Day

January 18

It was a busy day and a challenge to get out of the house on time. First stop was the new Sanford Hospital in Fargo to see a pediatric surgeon who looked at the gtube site. They agreed that the small boil that had formed above her tube likely needed to be drained. So some numbing cream and a scalpel later we had all sort of stuff coming out, including what appeared to be a couple stitches that had not dissolved as planned. They did collect some of the drainage to culture, so hoping to hear back on that soon.

From there I ran through (well actually I DROVE through) a drive through and hit the interstate to make it to Sioux Falls, SD in time for an afternoon appointment with Henley's GI dr. As much as an inconvenience as it is to travel that far, we really love Dr. Doubledee. We made some changes to her nutritional plan and hope that over the next 6 weeks they pay off. We'll stay in touch with him via email until then. He goes to a remote clinic in Aberdeen once a month, so we will save some miles and see him there the first week of March.

The most irritating part of my day was dealing with Sanford (specifically a nurse manager named Kristi) and trying to arrange for Henley to receive a shot she needs TOMORROW in Fargo instead of Dickinson. It took about 3 hours, multiple phone calls, then working with our insurance and our pediatrician before she finally agreed to do the shot. It was completely ridiculous. Henley is blessed to have an pediatrician that has made himself available to her whenever needed because he recognizes the complicated life we have signed up for. She's also blessed to have an advocate assigned to her at Blue Cross Blue Shield. Both hit the phone hard advocating to get this shot done. What's incredibly sad is that not every child is that fortunate and for truly NO LEGITIMATE reason Kristi was being difficult. So disappointing.