Bracy Family Support

A couple of weeks ago, my 3-year-old niece, Felicity was admitted to Golisano Children's Hospital in Syracuse to treat a case of pneumonia. Her lungs cleared up but her oxygen levels remained low and her lung functions continued to decline. Further diagnosis and a bronchoscopy revealed that she has bronchiolitis obliterans or 'popcorn lung' and her lungs are in very bad shape. She is still being evaluated but will likely need a lung transplant and extensive care. This has been incredibly hard on my brother (Adam) and sister-in-law (Amanda) as they have watched her health deteriorate so quickly and without any kind of warning. 

Felicity is the sweetest little girl. She is always smiling and loves to play with her little sister Gwen and her aunts and uncles. It breaks my heart that she is going through this life-altering event but she's strong and I know she will overcome it.

In the meantime, I am starting this GoFundMe to try to provide support to the family so they don't have to worry about some of the associated expenses on top of everything else that's going on. We are very grateful for any contribution you are able to make and would also appreciate all of the thoughts and prayers you can send their way.

**Update**

Thanks to everyone for the incredible support so far! Adam and Amanda are very grateful for everyone's generosity and that they can focus on getting the best care possible for Felicity. They would also like everyone to know that any funds that they recieve that aren't used for basic necessities while traveling and staying with Felicity will be sent as a donation to the chILD Foundation (Children's Interstitial Lung Disease) to support research into these rare lung diseases and develop effective treatments. 

As for Felicity's current condition, they are trying to get Felicity moved to a hospital that is better equipped to handle her situation. To confirm her condition it's sounding like they will have to go a step further than the bronchoscopy and perform a lung biopsy.

**Update from Adam**

PRAISE THE LORD AND THE SMALL ARMY PRAYING FOR FELICITY! We have just been told that transport to Pittsburgh has been approved. They will be arriving today, time TBD but we have been advised to pack and prepare to go. It has been and overwhelming 24 hours but our prayers have been answered. She will be receiving some of the best care in the country. We will continue updates if her condition changes.


**Update**

Felicity arrived safely at UPMC Children's Hospital in Pittsburgh Yesterday evening. She is such a strong little girl - according to the team in the helicopter she did great :) Adam and Amanda are settling in there as we await the next course of action. Thank you all so much for your continued support.


**Update**

Please pray for Felicity. They hooked her up to ECMO last night and she was doing well till this morning. Her oxygen saturation started to fall again and they found some fluid around her heart (not enough to be dangerous at the moment). They're currently repositioning the cannula that enters her heart to see if that resolves the issue and prevents any more fluid from collecting. She's keeping everyone on their toes and has multiple teams of doctors working together.

**Update 4/28**

Yesterday they swapped Felicity's ECMO circuit proactively to avoid clotting issues. She also had a high resolution CT scan and we are waiting for the teams to review it.

Felicity had a stable night and they are adjusting some of her meds to keep it that way. She still has some air around her lungs that they are continuing to monitor. They want to perform another bronchoscopy but are holding off for now. Her plasmapheresis is continuing and she is getting pediasure through her ND tube.

Today they removed the neuro muscular disruptor with the hope that she will move more lung secretions on her own, help reabsorb the air around her lungs, and move slightly to keep her muscles functioning. She is still heavily sedated.

This evening her eyes will move occasionally as well as her hands and feet. She has some incredible will power. We were already saying prayers with her, reading, singing, and playing movies and TV shows for her but we are going to double down so she knows she isn't alone. It is wonderful to know she is still there and still fighting. We continue to pray for her recovery so she can get back to being a fun loving little angel.


**Update 4/30 **


We will be meeting with Felicity's intensivist, pulmonologist, and hopefully the immunologist in the next couple days to discuss Felicity's status, course of care, and any continued diagnostic measures. Immunology is confident she does not have an autoimmune disease but the lung inflammation means there is at least an autoimmune component. It is difficult to say if the inflammation is still active or if it has passed. X-ray imaging lags behind actual progress so the most reliable information we will have is the ventilator trend. The less pressure it takes to provide a certain volume the more compliant and healthy the lungs are. Establishing a good data trend will take some time.

We are still expecting a bronchoscopy later today or tomorrow. Today is her last day of plasmapheresis. She has been meeting her nutrition goal as of yesterday. We will continue to update her condition as information is available to us. Thank you again for your prayers and support.


**Update 5/2**

I apologize for not posting an update yesterday. I will try to include as many details as possible. We spoke with immunology and rheumatology yesterday and pulmonology today. Rheumatology and immunology both agree that there is nothing affecting the entire body and that the issue is relegated to her lung tissue as there is an absence of inflammatory markers in the blood. Pulmonolgy thinks she could still be a fit for cryptogenic organizing pneumonia but can't prove it without a biopsy. The air leak in her chest has resolved and the intensivist and radiology think her x-ray this morning showed some marginal improvement of the lung tissue from the previous morning. Right now they want to see how she does over the weekend. If there is no clear improvement by Monday they will revisit the biopsy. Below is a summary of her condition and treatment.

ECMO:
She remains on ECMO and will be until she regains significant pulmonary function. Both Felicity and the ECMO circuit are heparinized to mitigate clotting risks. She has been on a blood pressure medication to lower her pressures which can spike when her heart starts fighting the ECMO. The surgical team is discussing switching from veinous-arterial to veinous-veinous ECMO since it is less sensitive to positioning, would not have issues fighting with her very healthy heart, and would carry less risk of clots in bad places.

Ventilation:
The ventilator is running at lower pressures and conservative oxygen saturation to keep her lungs functioning as best they can. High oxygen and high pressure have a negative effect on lung healing. NG tube is still in place for relief of stomach pressure. The intensivist has discussed a tracheostomy which is more comfortable for long term intubation. It may happen this coming week depending on a possible biopsy. She is still sedated but they have removed the neuromuscular blocker so that she will be able to move on her own. The long term goal is to get her comfortable and awake so that she can move independently and work with the occupational therapist and physical therapist to make sure her conditioning does not diminish. Her tidal volumes will be one of the key indicators of improvement or worsening of her lungs.

EEG:
For the duration of her time on ECMO her brainwaves will be monitored as a means of detecting possible clots in the brain.

Nutrition:
Felicity is up top maintenance calories and is tolerating the feeding through the ND tube very well. Nothing but pediasure for now. They will be discussing vitamin supplements with the nutritionist to make sure that she is well balanced and had no deficiencies.

Plasmapheresis:
Felicity finished her five day course of plasmapheresis on Tuesday. No significant improvement resulted from it.

Corticosteroids:
Treatment continues. They have done one round of pulse steroids since we have arrived and may do another next week.

Azythromyacin:
An antibiotic that has anti-inflammatory properties and is frequently administered for inflammatory lung diseases.

Surfactant:
Normally produced in healthy lungs, they do not believe Felicity is deficient but are administering it in the hopes it will recruit deflated alveoli and increase her lung capacity. It is a liquid administered through her breathing tube and allowed to sit for an extended time for best results. She received one treatment yesterday and another today.

IVIG:
Essentially the hope is this will bind to receptors involved in the inflammatory process and prevent inflammation.She started receiving this yesterday.

Metformin:
Normally used for type II diabetes, there is data to suggest this alters the energy usage in the macrophages in the lungs and subsequently the associated signaling involved in the healing process resulting in less scar tissue forming. She started receiving this yesterday as well.

Imaging:
Besides the ventilator, her morning chest x-rays will be important to ascertaining her clinical condition and will continue. Her high resolution CT scan was impressively thorough but did not result in any eureka moments.

Genetic Testing:
Testing has been sent out to see if she is predisposed to any illness.

Overall:
Right now the plan is to stack the odds in her favor and see if she begins to recover on her own. No progress will most likely lead to a biopsy and those results will inform further steps.

We are determined to make sure Felicity know she is loved and cared for during this scary experience and are taking steps to decorate her room, set up a DVD player and streaming, read books, say prayers, and generally interact with her in a very positive way. We thank God every day for the support and prayers flooding in on every side. Thank you everyone.


**Update 5/4**

I am sorry to report today that Felicity had another air leak around her heart yesterday. It improved overnight but there is more air today. They have reduced the ventilator settings and her attending has told us that he wants to do another chest CT on Monday. Following that, most likely they will proceed with a biopsy. He also thinks it is time to begin workup for a lung transplant.

Felicity's gut is also not cooperating (side effect of sedation and pain killers). Last night when suctioning air from her stomach a good deal of pedia-sure came up as well. She will be started on IV nutrition this evening through her central line for the time being. As soon as they can get her intestines moving again they will try to resume the ND tube feeding.

They have also stopped the Metformin due to increasing lactate levels in her blood. Those levels are back to normal now. 
 

**Update 5/5**

Felicity's condition at the moment is unchanged. She is on IV nutrients but they also gave her something to help her gut. They started her back on a very slow feed through the ND tube and are continuing to monitor her digestive progress. We were just informed by her intensivist that pulmonary does not believe a repeat CT is necessary or would give them any additional information. Surgery is currently discussing the best way to obtain a biopsy and the procedure could be scheduled for tomorrow. They are also discussing switching her from VA ECMO to VV ECMO while she is in the operating room. A tracheostomy was on the table but they are waiting to hear from transplant pulmonology to see if a tracheostomy would hinder possible future transplant efforts.

On a non-clinical note, we do have some good news. Today Felicity received confirmation and first holy communion, as well as anointing of the sick for the 5th time. If you feel inclined to offer some prayers for her, to celebrate receiving these sacraments, they would be most appreciated. We are thrilled that she was able to receive these two sacraments of initiation prior to what promises to be a difficult week. Thank you again everyone for your support and prayers.


**Update 5/7**

The team has decided to go ahead with the biopsy. She is scheduled for Thursday afternoon. They will be taking two tissue samples from separate lobes so results can be corroborated. At that time the surgery team will also swap her ECMO circuit and attempt to switch from VA ECMO to VV ECMO. 

The past couple days she has been bleeding from one of the central lines in her thigh and had multiple bandage changes. Once she is on the VV ECMO it should hopefully stop since they can reduce the heparin in the ECMO circuit.

Another small success last night was her first stool since she has been intubated. She in now being given what is essentially Narcan through her feeding tube several times a day. This is supposed to minimize the sedation effects on her gut. Her feeding rate has been increased again though she is still on IV nutrients for now. She has OT and PT sessions each day to help prevent muscular and neurological breakdown.

Depending on what is found during the biopsy, we may not receive any information until this coming Monday or Tuesday. Thank you again for your prayers everyone.


**Update**

Some updates as of this morning. Last night Felicity had an issue with her heart beat skipping which is all I understand at this point but will be speaking with her doctor soon to get more information. She is stable now and resting. Her meds were adjusted this morning as well. There was a meeting this morning with apparently 40 medical professionals concerning her case and the plan over the next few days. They have decided not to switch from VA to VV ECMO when she goes down for the biopsy due to the cardiac event last night. The biopsy will also be first thing Thursday morning instead of the afternoon. Right now she is scheduled for 7:30 am.

They will be starting a glycerin suppository to help her stool and have taken her off the EEG for now so she can have a break. She is also getting her mattress swapped out tomorrow for an alternating pressure mattress. This has a pneumatic pump with 4 outputs controlling various zones of the mattress. The adjustable pressures will help her be comfortable and also stimulate her skin to prevent breakdown. Regarding the air that has been in her chest; this mornings x-rays showed it to have resolved almost completely.

Please keep Felicity in your prayers as the biopsy approaches. There is significant bleeding risk due to the heparin, and significant risk of a persistent air leak due to the compromised lung tissue. We are hopeful that the biopsy will provide new information that will direct her course of care. The pulmonologists have informed us that about 10% of pediatric lung biopsies are inconclusive so there is a chance we may have no further information.

Thank you everyone for continuing to follow Felicity and pray or her.


**Update**

Thank you everyone for your prayers. We are relieved to report that the procedure was completed with zero complications. Felicity is back in her room resting. They will be monitoring her closely the rest of the day to make sure there is no bleeding as the slowly increase her heparin back to full dose. Results from the biopsy should be available early next week. 

The entire procedure was accomplished with only minor incisions for tools and the camera. She does have a second, smaller diameter, chest tube which they placed through one of the minor incisions they made for the procedure. The Vas Cath in her right thigh had been bleeding for several days due to the heparin and was removed in the operating room since it hasn't been used and won't be necessary as she is on ECMO. They are switching out her ventilator for a system which sends an air current past her endotracheal tube so she can go on complete lung rest for the next seven days.

Besides the procedure going smoothly we also have a couple positive notes to share from this morning. The surgeon and intensivist both said that visually, her lungs look better than they were expecting. The intensivist in particular was pleased to see this. We don't want to speculate at this point so we will be waiting to see what the biopsy can tell the team. Finally, Felicity has stooled. It was "legit," in the words of our nurse. We are extremely happy that her bowels are functioning and she should be able to resume nutrition through her feeding tube in the near future.


**Update**

Felicity is heading back to the OR this afternoon due to hemothorax around her right lung (blood in the chest). The plan to wash out her chest and possibly replace her chest tubes or place another chest tube. She is back on heavy sedation and a neuro-muscular disruptor.

Please pray for her this afternoon, that all goes well during the surgery and that she makes a swift recovery.

**update**

She’s on her way back to her room. They did end up placing two new, larger chest tubes and removed quite a lot of blood. Praying she gets some quiet rest tonight.


**Update 5/13**

Felicity's pupils are different sizes and they are concerned a clot may have reached her brain. They are preparing for a CT scan of her brain. Please pray!


**Update 5/14**
Thank you all SO much for your continued support and prayers. I’m sorry for the silence since yesterday morning. This has been the longest, hardest week since this all started. Felicity’s CT scan yesterday came back without any signs of a blood clot. They aren’t entirely sure why her pupils were different sizes or why her EEG scan looked different. But in any case, she’s stable right now. They are constantly adjusting her medications to keep her safe and comfortable. We should have the results from the biopsy soon, maybe by tomorrow afternoon. There’s a chance that they won’t be able to determine what caused this, even with the lung tissue samples. We’re hopeful though that they found something meaningful. And hopeful she’ll recover with no future problems!


**Update 5/15**

Unfortunately there are no conclusive answers at this time. We met with the head of pulmonology who told us the biopsy results were inconclusive but did give them some new information. No disease has been identified but they are fairly certain she does not have an interstitial lung disease. What he observed in the samples was some fibrosis (scarring) but it is not severe. He is having her steroids increased to help prevent further scarring. He also observed that the alveoli and bronchioles were clogged with something. That something has yet to be identified. He performed another bronchoscopy and a partial lavage of all five lung lobes to see what they can get back. He did mention that her airways as far down as the bronchoscope could go looked healthy.

The pulmonologist is ordering a new medicine that is typically used for malaria but was mentioned as a contributing recovery factor in another pulmonary case. When we asked him if it is possible for her to recover from her current condition he said it is possible but he couldn't give us a probability. One outcome seems just as likely as another at this point.

Genetic testing was sent out a while back and should be coming back soon. It may or may not give them an answer but it seems to be the last diagnostic attempt on the table.

Since Felicity has not had any air leaks she is slowly being brought off of lung rest settings on the ventilator. They will be monitoring her x-rays to see how much of the lungs they can recruit. 

Felicity's sedation has been much more stable for the last 48 hours so today they temporarily discontinued the neuro-muscular inhibitor to make sure that she retains motor control function. She did just fine but there was a significant amount of twitching as her muscles "woke-up" which was difficult to watch. She is back on the inhibitor now. 

For those praying for Felicity we would ask that you pray for her lungs to heal and heal quickly. The longer her lungs stagnate, and the longer she is on ECMO and sedation, the greater the risk of something going wrong. Thank you everyone.


**Update 5/22**

Felicity will be heading back to the OR tomorrow morning due to another hemothorax. They’ll be doing the same chest washout they did last time, and hopefully the bleeding will resolve. She has been relatively stable this past week. Her chest X-rays are looking more like they did pre-biopsy, which is an improvement. She’s still sedated and muscle relaxed a little bit, and she can answer some questions with a nod. She’s still on ECMO, the ventilator, has chest tubes, arterial IV’s, etc etc. Her favorite thing right now is music therapy provided by the hospital (awesome!) and lullabies we sing her. We are still patiently hoping & praying for this sweet girl every single day.


**Update 5/24**
Thank you for your continued support everyone! Felicity’s chest washout yesterday went well. They were able to remove about 70% of the clotted blood and change the tubing on her ECMO circuit. She was off of blood thinners for about 20 hours (which is unconventional for ECMO patients because of the risk of blood clots developing in the circuit & causing major issues like stroke) to see if that would help stop the bleeding in her chest. So far her chest tubes are not putting out as much as they were previously, and her chest tube dressings are clean. Thank goodness! They will be doing a full broncoscopy lavage of each of her lung lobes later this morning. It’ll be much more extensive than previous broncoscopies. They can’t get to the smallest airways with a broncoscopy, but the goal is to clear out her larger airways as best they can. Then over the next few days they’ll continue trying to recruit her lungs with the ventilator. She’s been very comfortable on her current sedation & pain medications, which is nice to see. Keep this cutie pie in your prayers, please!


**Update 5/27**
Just a quick update for everyone...our sweet pea has had a rough 24 hours. They’ve been adjusting her sedation medications and also trying to recruit her lungs. She’s no longer on the paralytic so she’s breathing fast with her belly again and she’s tremulous. They’re trying their best to keep her comfortable but responsive enough to answer questions to let us know how she’s feeling. As far as lung recruitment goes, the X-ray shows a bit more air in her lungs, which is good. However, there was also a new pneumomediastinum (air around her heart) on her X-ray this morning. After her persistent problems with air leak in the past they are keeping a close eye on it. They’re going to hold off on further recruitment maneuvers for now and try to retain the progress she’s made so far.

**Update 5/28**
Over the last several days the doctors have been performing recruitment maneuvers to try and increase Felicity's tidal volumes. Her lung x-rays have been showing more air in her lungs though the change is not dramatic. As Felicity's tidal volumes increase the treatments such as surfactant, mucomyst, and albuterol will be more likely to reach the alveoli.

Early this morning Felicity's x-ray showed a pneumomediastinum, almost certainly due to efforts to recruit her lungs. This appeared smaller than most previous incidents but her ventilator settings were slightly decreased in response. Follow up imaging showed that the condition was not worsening and in the next day or so the air should reabsorb.

Her sedation continues to be a struggle to pin down, primarily because of her length of stay and concern over developed tolerance to various medications.

**Update 5/28**

The pneumomediastinum has decreased slightly but is still there. Recruitment maneuvers have been postponed until it has fully resolved. 

Her inflammatory markers have been trending up over the past week and there will be an official consult today with infectious disease and general surgery. It may just be a general response to everything that is going on but of course they are concerned about infection.

Behavioral health is now monitoring her due to her length of stay and sedation problems. They will be watching for behavioral changes and indicators of ICU psychosis.

We are exceedingly grateful for the prayers that continue to flood in for Felicity and our family, and are remembering all of our patrons and benefactors in our prayers.