The last 24 hours were pretty scary.
“Fuck.” That’s what I woke up thinking this morning. With my little man, Drex, right next to me waiting to pick up where we left off on the latest update of Super Mario Run the night before. I’m like “Not now dude. Watch some FGTeev and I’m going back to sleep for a bit.” He’s like “cool.” Then asks “How’s Lilla?”
Well, that’s a loaded question. But he understands, so I answer with a somewhat confident, somewhat scared “She’s okay. Right now, she’s okay.” He gives me a hug, then presses play on his video.
When Lilla was 3 months old, she was diagnosed with RSV. For those that aren’t aware, here’s the writeup on WebMD:
Respiratory syncytial virus (RSV) is a common, and very contagious, virus that infects the respiratory tract of most children before their second birthday.
For most babies and young children, the infection causes nothing more than a cold. But for a small percentage, infection with RSV can lead to serious problems such as bronchiolitis, which is inflammation of the small airways of the lungs, or pneumonia, which can become life-threatening.
Babies with more serious cases may need to go to a hospital, where their treatment may include oxygen, IV fluids and medications to open the airways.
Lilla was one of those babies with more serious cases. That’s when the breathing treatments started. She’s 7 now. Well, she would say “I’m basically 8 already.” I love this girl. So much energy, so many hopes and dreams and goals to accomplish. Wants to be a Ninja Warrior. Wants a motorcycle for her sixteenth birthday. Is planning, and preparing to be on America’s Got Talent in a couple years. And boy does she get mad if anyone gestures, hints at or makes reference to something a girl can’t or shouldn’t do. Especially when compared to boys.
On any day, if Lilla has a chance to play, she goes all out. Because she might not get that chance tomorrow, or for the next couple weeks. It’s her body that often holds her back, and because of that, she’s filled with a lot of rage. The multiple high ankle sprains, twice-broken foot and broken arm are definitely partial to blame. Her lungs, by far the biggest culprit.
Until a couple months ago, no matter how nasty and croupy the cough, how long it persisted, how frequent the breathing treatments, how recent the last round of steroids, it always came back to asthma. And the same treatment plan put right back into effect: nebulizer every four hours (often doubled), multiple antibiotics, back-to-back steroids and whatever else her doctors want to throw into the mix. Every Fall, every Winter, every year. Always right at the start of school. Same questions, same explanations, same comments.
Asthma is a pretty common word. Most people have somewhat of an understanding of what asthma is. There’s tons of information, specialists and support groups. But as Lilla’s diagnosis, it didn’t always make sense. Margaret, my wife, always questioned it. We’ve never heard Lilla wheezing due to activity. She doesn’t have a single allergy. The breathing treatments never really seem to help. And the condition only ever seems to get bad when Lilla is getting sick. But we took the diagnosis as it was, because there was nothing else. All other theories seemed to lead to dead ends and more medical bills. Then things changed.
Lilla was on her second week of a croup sounding cough. She just seen a new pulmonologist days earlier, where he changed her inhaler and added an acid reflux medicine. Margaret gave the new meds a few days, but when nothing changed, decided to page the on-call doctor. When the doctor heard her coughing over the phone, he said “That doesn’t sound like an asthma cough. That sounds like something else. Can you take her to your Asthma doc in the morning and run the breathing test? If her lungs are operating at full capacity, then it’s not asthma.”
We did exactly that. The next morning we’re at the asthma doc. Lilla performs the breathing test, and her lungs are operating at full capacity. She is immediately scheduled for a bronchoscopy. They put a camera through her trachea and into both sets of bronchial tubes. The outcome: we have a new diagnosis. Lilla has Tracheobronchomalacia (TBM). It’s actually a pretty simple explanation. Her trachea and bronchial tubes are misshaped. It’s severe in her right lung, moderate in her trachea and left lung.
This immediately confirmed two things we’ve struggled with her entire life:
- The length of her coughs and illnesses: when Lilla gets a common cold, it sometimes takes multiple weeks to recover. When Lilla’s body starts to produce mucus, it can get stuck in various airways. It can block the airways, causing her to go into an asthma-like attack. The mucus can build up and get infected, leading to worse conditions and longer recoveries.
- Her croup cough: it’s not a croup at all. Due to the shape of her trachea, like an instrument, she plays a different tune than we do. Only the trained ear can tell the difference. And since her body is consistently trying to move out the mucus, her cough often lingers well after she’s over the sickness. The cough can get us some annoyed looks while at the grocery store. F em!
So what now. We know what it is, but there’s little information, specialists or support groups. We found one support group on Facebook. We found one specialist in Boston (we’re in Detroit). For over 7 years, we had a day-to-day plan, a sick plan, and an emergency plan for Lilla. Now we’re starting at ground zero.
The treatment plan changed. We still use the nebulizer, but a different type of medicine (Atrovent instead of Xoponex — well, both at times). And we have added The Vest, the same type worn by cystic fibrosis patients. She wears it 20 mins in the morning and 20 mins at night. When sick, we increase the nebulizer and vest to four times/day.
Last weekend Lilla caught Influenza (Flu). Her body started to produce mucus. The treatments and vest are used to loosen the mucus and keep it flowing, so she can eventually cough it up. The cough and fever got so bad on Wednesday that Lilla was hospitalized — her heart rate elevated and oxygen levels dropped, but everything stabilized and she was released a few hours later. We learned new information: on top of TBM and Influenza, Lilla also has pneumonia. Wednesday through Friday night were filled with late-night/early-morning coughing fits.
Last night, I woke up at 3:30am to Margaret yelling at me. “Iain…I need you, right now. Go sit with Lilla.” I walk into Lilla’s room. She’s sitting up on her bed, breathing treatment mask on, coughing uncontrollably and looking very scared. She has to focus on trying to breathe, so she doesn’t say much. I sit next to her and pull her tight. I hold her hand. She squeezes hard. She whispers “I’m scared Dadda. I think I’m going to die.” It was probably the most real thing I’ve ever heard in my life. Margaret was organizing to go to the ER. She called the UMHS on-call doctor, woke up our eldest daughter Genevieve to let her know we were going, packed a bag and started the car. Drex woke up to all the commotion and came in the room crying. He laid on me while I held Lilla. I never felt so unqualified in my life. I grabbed Lilla by both hands, looked her in the eyes, told her she wasn’t going to die and just said “breathe with me.” She really tried to, but kept coughing. Once Margaret was ready, we got her in the car and to the nearest hospital. Lilla stabilized and was back home by 6am.
Now, Margaret and I sit here tonight. Knowing we both should be sleeping. But we can’t stop thinking about the last 24 hours and the next 24 to come. If she can get through the pneumonia, she will return to her normal.
As far as long-term. There’s no cure for TBM, but her airways will grow as she does. So the hope is that they self-correct to rid most of the issues. And then she’ll be performing at America’s Got Talent, showing off her instrument, in a couple years.
The frustrating part, and I have to imagine this goes for any rare or complex condition, is having to teach and prove to every person and doctor that you know what you’re talking about. That you’ve done your research. That you’ve tried EVERYTHING. Just listen. At both hospitals this week, doctors immediately tried to diagnose Lilla as something much more common, that you are trying to tell them it’s not. But that’s just how it is. With pretty much everything it seems.
UPDATE: Lilla has been accepted to Boston Children’s Hospital, where they specialize in TBM, for extensive evaluations and testing. She’ll be going December 6–8 (the 8th is her 8th birthday). Based on their findings, she may be recommended for surgery the following week (14th). Surgery can lead to an in-patient recovery up to 10 days (which could mean Christmas in Boston as well). Also, they are very concerned about Lilla showing up sick to Boston — which means they couldn’t perform what they need to. So they don’t want her attending school from now until then. This is all a lot to handle and figure out, especially with our other children who will be home, so wish us luck.
TRIP TO BOSTON CHILDREN’S HOSPITAL
Day 2 Update (12/6): Lilla had her first appointments today; mostly discussions and a couple breathing tests. Dr. Jennings, the primary specialist for TBM, is out with the flu. We met with the pulmonary and GI teams who will be conducting the bronchoscopy and endoscopy on Friday. Basically, just filled them in on Lilla’s past.
Other than that, our hotel was not ideal (to put it nicely), so we moved out of it today, and into an Airbnb a few blocks from Fenway park. It’s walking distance from the hospital.
Tomorrow we have a pre-operative appointment to prepare for her scopes on Friday (her 8th birthday).
Day 3 Update (12/7): We had a morning pre-op appointment today. A lot of discussion around Lilla and family history, and met with anesthesia to discuss tomorrow’s plan. Pretty straight-forward. However, we were surprised when we noticed the two doctors we met yesterday were not scheduled for her scopes, and it was two docs we hadn’t heard of. We’re not concerned, but we hate surprises, and would have felt much better if they explained this to us.
It lasted about two hours, and then we went adventuring. Hit a pretty awesome pizza place (Penguin Pizza). Then Margaret and Lilla’s first train ride. Then ice skating downtown. Then a walk on the harbor. Then back to the apartment. We’re beat.
Tomorrow’s endoscopy (upper GI) and bronchoscopy are scheduled for 2:30pm. Lilla can’t eat after midnight, and we have no idea what she’s going to be like after, so she wanted birthday dessert tonight (specifically creme brulee). So we got that from Yard House.
Lilla’s in good spirits. A bit scared. And still sad it’s happening on her birthday, with a chance she has to stay the night. Once the scope is done, we should have pretty clear direction going into next week’s surgery. There’s still a (small) chance surgery will get cancelled if they don’t feel it’s necessary.
Tomorrow is a big day for our little (soon to be 8 year old) football playin’, dirt bike ridin’, emoji pants wearin’, sweet lovin’ girl.
Day 5 Update (12/9): Yesterday, Lilla had her bronchoscopy and endoscopy (upper GI). She had a rough night coughing, we had to double up her breathing treatments, but that was normal with her prior scope. Pending any biopsies, her GI checked out fine. No changes yet, but this may rule out any form of acid reflux (currently they have her on Zantac). Her bronchoscopy basically validated our findings from Univ. of Michigan. See attached photos for a better understanding.
The good news, scary news, and main reason we went to Boston — is that the surgeon confirmed that she’s most likely a candidate for the Posterior Tracheopexy that only Boston Children’s Hospital specializes in. The surgery is scheduled for this Thursday (12/14). Dr. Jennings (the TBM master) was out sick this week, otherwise he would have conducted the scope, so we have to wait for him to review the photos and provide final recommendation. Lilla will also have a CT Scan to ensure this surgery is the correct option (i.e. if they find that a blood vessel is putting pressure on the trachea, it becomes a different surgical option). Margaret and Lilla will meet with Dr. Jennings after the CT Scan on Wednesday to discuss the final plan for Thursday’s surgery. I’m on my way back to Detroit right now, to see Genevieve and Drexel, and will be back in Boston Wednesday night.
For those that aren’t aware, Drex (our 6 yr old son) also has TBM. However, he doesn’t experience the breathing attacks like Lilla does. He has an appointment with the U of M pulmonologist on Wednesday that I will be taking him to — to discuss his treatment plan. There’s a good chance we can be going through this same process with Drexel in 2018.
Lilla was not aware of the next surgery. We have been taking it day-by-day. She thought yesterday was it. So Margaret and I explained everything to her last night. She cried. She’s afraid. But she understands. She even said “this is a once in a lifetime opportunity”. Boston has had amazing success with this surgery. It’s life changing for most people. Hopefully, that will be the story for her.
Day 8 Update (12/12): Big day tomorrow. Decisions get made. At this moment, the Posterior Tracheopexy surgery has been rescheduled for Friday. Based on the information BCH currently has, Lilla is being considered for a thoracoscopic version of the surgery which requires different/robotic equipment. Tomorrow, we will have three appointments: Pre-Op, CT Scan, and final recommendation/discussion with the surgical team. At this point we know the posterior (back) wall of her trachea is collapsing the airway (100% during coughs), and we don’t suspect any GI (esophageal) work needs to be done. The CT Scan will determine if there’s any other contributing factors, like a blood vessel that is wrapped around the left bronchi. If the CT Scan is clean, Lilla will most likely proceed with the thoracoscopic surgery on Friday. If the CT is not clean, then it becomes a bigger deal than it already is, and the original version of the surgery (plus whatever else), will most likely be the case on Friday.
Since last week’s scopes, we’ve learned a lot more about the surgery. We knew it was major, but we didn’t realize how intense it is. We spoke to the team, and surgeon, multiple times yesterday to clarify any questions or miscommunication. We also learned that Dr. Jennings, the lead surgeon who was out with the flu last week, will not be returning this week either. To be completely honest…that was a lot to take in. We also connected with other parents, one who’s child is having the exact same surgery on Thursday. Lilla can’t wait to meet her, and already made her a present. As parents, we questioned a lot over the last couple days. We had multiple freak outs. And we made the decision that I needed to fly back to Boston a day early (tonight), to be with Margaret and Lilla for all appointments tomorrow. All three of us need to be 100% confident and 100% clear on our expectations (No Surprises!) going into Friday.
Here’s a few cute pics of Lilla exploring Boston over the last few days.
Day 9 Update (12/13): Nothing abnormal in today’s CT Scan. Whew! We met with the surgeon to discuss everything. No question went unanswered. Unless something changes once they get inside, Lilla will have the thoracoscopic Posterior Tracheopexy on Friday (she asked to see the robots, and her surgeon said to remind him). The surgery will consist of four small incisions on her side/back, and should last between 5–7 hours. However, if the surgeon doesn’t feel the robots are able to do exactly what he needs, then he may choose to revert back to the open surgery, which would consist of an approximately 6" incision. And a more intense recovery. Upon completion, she will have a lung drainage tube for a couple days (or longer if needed).
When it comes to recovery, he said if all goes as planned, and her body responds positively, she could potentially come home mid-to-late next week. We asked about resuming normal activities and physical activity (Lilla is planning to play basketball in January), he said to let her body do the talking. There will most likely be no restrictions coming from them. And again, provided everything goes as planned, they will most likely ween her off the breathing treatments.
Other then that, Lilla made a friend today. Over the last few days, Margaret has been talking with the mom of another child (close to Lilla’s age) who is having the same surgery tomorrow. Lilla made her a care package, and finally had a chance to give it to her. She was happy to have someone to talk to, that can relate to what she has gone, and is going through. As usual, Lilla shined her light on everyone she touched today.
Here’s some pics from the day.
Day 11 Update / Happy Surgery Day (12/15): We knew the biggest challenge with this surgery was keeping Lilla healthy enough to have it. We thought we made it. And then at about 5:30pm yesterday evening, Lilla threw up. No biggie. She said her belly was ouchie. Then again, and again, and again. No fever. No other symptoms. We looked at each other like “Oh sh*t…here we go.” Margaret paged the on-call doc, their response “hmmm…let me get back to you with a plan on this one.” They called back and said if it continues, take her to the ER for evaluation, if she sleeps, they will check her at pre-op. Well, right when we thought we were clear, around 11:30pm after about 2–3 hours of straight sleep, Lilla threw up again. We packed some things, got a Lyft and arrived at ER by midnight.
She only threw up one time at the ER. They gave her an IV and some anti-nausea meds, and she seemed okay. Her surgery was scheduled at 7:30am; check-in at 6am. So we just got an early start. By 6am, her surgeon stopped by the ER. They moved us to pre-op. The anesthesiologist cleared the surgery to proceed. And they took Lilla into the operating room at 7:40am. It should last 5–7 hours. Now we wait.
We spoke with Lilla a lot about the surgery yesterday, and she claims she’s not scared; she’s excited about a better future. And this morning, she reiterated that. This girl continues to blow us away with her inner strength.
Other good news…we got word that Lilla’s friend’s surgery went well yesterday. She’s currently in recovery.
Update#1: Doing well. Started with the robot/thoracoscopic approach.
Update#2: Nothing new to report, just that everything is going okay.
Update#3: They are finishing up, much earlier than we expected. Should see the surgeon within the hour.
Update#4: Spoke with the surgeon, all went well and as planned. No surprises. He left us both very hopeful. Lilla is being transferred to ICU, we should be seeing her sometime in the next hour. She will not be awake, and may not be today. Whew!
Update#5: We are with Lilla, she’s awake and softly talking to us and the nurses in the ICU. All is good.
Day 12 Update (12/16): Written by Margaret. Last night was a bit of a rough one. But we had an AMAZING nurse who stayed on top of Lilla’s pain and was so attentive to her. She had a few spikes of unbearable discomfort but they passed quickly. She was nervous to get her chest tube removed but faced it with courage and spirit as she does with everything . Her oxygen levels fall a little without the mask, but she is working up to room air. It’s so inspiring to watch the determined look in her big bright eyes when using the breathing exerciser. She does extra rounds and won’t stop til she makes the ball go all the way up. They have removed her catheter so now all she is hooked up to is two IV’s. She ate some soup and a tater tot and now is resting peacefully with her Enya playing. We should be moving out of the ICU and on to the floor soon. She is so excited to be reunited with Kate. One step closer to home and back to my babies. I miss them so bad my heart literally aches. This has been an experience words cannot explain. Saying it was a rollercoaster is an understatement. But I am seeing little by little the light in my angel coming back. That sparkle in her eye is shining bright. Without even trying she is warming and stealing the hearts of all the hands that have touched her and all people she has met.
Thank you all so much for all your continued love and support. Seriously we cannot express how grateful we are and how very blessed we know we are!!
Day 13 Update (12/17): Written by Margaret. I can finally ALMOST exhale. Lilla is no longer connected to anything. No tubes, no wires, no monitors!! We got our room and Kate is our roommate! The girls have been playing Roblox and laughing and are so sweet with eachother. The view from our new room is beauteous! Lilla is breathing at 100% on room air this is a first for her entire life!!!
Tomorrow we have an Esophagram scheduled at 2pm and if all goes well, Lilla and I will be headed home Tuesday morning!! Right now, she is eating mashed potatoes and chicken soup! She is tired and still has a lot of healing to do, but THIS GIRL IS ON FIRE!!
If they say to drink 4oz she makes a point to drink 8. If they say to make 1 ball on the triflow move, she won’t stop until she moves all 3. Determined and ready. Today, we painted nails and made art in the family room. Iain is on his way home to surprise Drex and hopefully we will be doing the same Tuesday. For the first time in a long time I can almost exhale.
Day 14 Update (12/18): Written by Kristy (Kate’s Mom). Kate was the luckiest girl this past week, she got to meet a friend for life, Lilla, who is going through the same surgery and issues she is. From pre op to post op, these two have been through it all this past week. Roomies and roblox besties! Lilla, we are going to miss you so much when you leave tomorrow! Your sweet smile lights up a room and you make pain easier to deal with! Safe travels! See you, same place, same time in 2018!
Day 15 Update (12/19): Heading home.
Day 16 Update (12/20): We’d like to thank everyone for their love and support through our journey. As of right now, Lilla has no restrictions — she can do whatever her body lets her do. She’s also off her daily breathing and vest treatments (that’s crazy); and will only be continuing with her acid reflux medicine. The next time she gets sick, we’ll see how her body responds, discuss with the BCH team, and use the treatments and medication as needed.