Working through it 

Reiss has had her moments of anxiety and fear today, but has also showed a glimpse of a turning point in the right direction. 

She fought us when we asked that she move to a chair to help her lungs and muscles strengthen.  However, once she was there, she was fine and watched tv and munched on a banana.


Reiss had three lines taken out, her chest tube shortened and a bulb added, and is only on 1/2 liter of oxygen. Additionally, she started to eat more this evening and drink more also. 

We tried to help her stand up for a couple seconds between the chair and moving her back to the bed,but she was scared and in pain. It was short-lived, but still was a small step. Walking was not in the cards for us today. Hopefully tomorrow we can get her up and moving. 

A tough day

Reiss is having a tough day today. She is hurting, which is making her sad, anxious, mopey, and inpatient. 

Two of her lines have already been removed, which is good. We are going to be off of the oxygen today, as well as up and walking again. 

 The Classic Repair

I thought I would share with you a schematic of what Reiss’ Glenn heart looked like versus the classic repair she had on Wednesday, May 25. The drawing below was completed by Dr Mckenzie. 

While the apex of most hearts points to the left side of the body, Reiss’ apex points to the right (dextrocardia). Additionally, Reiss’ heart sits in the center of her chest, as opposed to the left side in normal patients. 

  • SVC – For the Glenn procedure Reiss had at 10 months, the superior vena cava (SVC) was rerouted from its connection at the right atrium and placed directly on the right pulmonary artery. This was the only way Reiss’ heart supplied blood to her lungs. For the Classic repair, this was reversed, and the SVC was put back on the right atrium. 
  • VSDs – the picture on the left shows two ventricular septal defects (VSDs), or holes in the septum between the two ventricles. During her procedure last week, these two holes were patched. 
  • Pulmonary Conduit – Reiss’ pulmonary artery was completely closed at birth (pulmonary atresia). The Glenn bypass option, as mentioned above, is what helped carry blood from the upper portion of her body into the lungs, bypassing the heart. In the Classic repair, her native pulmonary artery was ligated and a pulmonary conduit with valve was added, connecting her right sided, left ventricle to the pulmonary arteries. 
  • Pulmonary Branch – during her repair last week, the surgeons noticed that the pulmonary branch arteries were narrow. They widened them with a balloon catheter to help increase the blood flow through those vessels. Later in the week, they noticed a portion of the left pulmonary artery (LPA) was narrowed even further. Before placing her pacemaker today, they also widened this LPA. 

The Classic repair will allow more blood flow to the lungs, hopefully decreasing the likelihood of collaterals and hemoptysis. We have already noticed a difference. With pulse ox readings in the mid-90’s, Reiss no longer has a bluish tint to her lips and fingernails. 

UPDATE | Breathing tube out

They finally took the breathing tube out in the last 30 minutes. Reiss was ready for it. She instantly wanted water and food, and was mad when I couldn’t give anything to her. 

Reiss was talking instantly, asking the doctor why she couldn’t have any water and cracking jokes. When she figured out she wasn’t going to get any liquids, she opted for TV and rest time. 

So, for now, she rests. We are praying for a non-eventful day and night. 

UPDATE | Back in CVICU

Reiss is back in her room now. She is still intubated and sedated, and will continue to be like that for a couple of hours as she wakes up from the sedation. 

She is on oxygen and has a bandage wrapped around her abdomen around her pacemaker site to reduce the opportunity for fluid buildup. 

UPDATE | Finishing up

They have widened the pulmonary artery and taken Reiss off of the bypass machine. The pacemaker has been placed and the team is making sure all the parameters are set properly. They should be closing her up now, then the surgeon will be come to see us in about 20 minutes. 

UPDATE | In the OR

They took Reiss back to the OR after giving her sedation medicine for the separation from Roy and me. She went back to sleep easily. 

Emotions are high at this point – more than for her surgery a week ago. First, the fact that her heart stopped twice yesterday has us freaked out. Second, the emergency that delayed us included the need for CPR. We walked by that room as we left Reiss with the anesthesiologists and our minds turned to the worst in regards to Reiss. 

Please pray that God guides the hands of the doctors and nurses as they reopen Reiss’ sternum for this surgery. 

Pray Reiss stays RKT -Reiss Kaderly Tough. 

When a heart stops

Reiss gave us a scare over night. Around 11:45p, she passed out. They checked everything, then decided to push fluids, calcium, and potassium, as well as add oxygen.  

The doctors blamed it on a lack of fluid intake – knowing that Reiss had not been able to keep any solids or liquids down yesterday and released most of the bowels in her system. 

All went back to normal until 1a. It happened again.  This time, she puked afterwards. 

The team reviewed everything and decided both fainting spells were because of what is called Syncope. Her heart had stopped.

Her heart had stopped? But that doesn’t make sense. We have had multiple conversations with multiple specialists who told us she has a beautiful escape rhythm. Even if the leads were failing further, she should continue at her native rate.  

Turns out, that didn’t happen. Her heart stopped. Twice

We are all watching her for the next two hours with 2-3 back-up plans if an emergency situation arises.   7:15a – we get a permanent pacemaker to resolve this. 

3rd Open Heart Surgery

We talked to Dr. McKenzie’s nurse practitioner this evening about the plan for Reiss’ third open heart surgery in 2.5 years.

After further review of the CT scan, echo, and Reiss’ stats, the team decided a pacemaker is necessary.  As mentioned in our post yesterday, Reiss is already at a high risk for needing a pacemaker in general because of her specific type of heart defect.  Instead of trying to wait a couple days longer to see if her heart would conduct properly, the decision was made to go ahead with the surgery.

Additionally, they found some narrowing of the left pulmonary artery (LPA), just distal of the conduit/pulmonary branch connection.  The narrowing, according to the NP, is pretty significant.  If Reiss didn’t already need a pacemaker, this could be ballooned through a cath procedure.  However, Dr. McKenzie will already have her sternum 0pen for the pacemaker procedure, so they will address the issue then.  Most likely, they will augment the artery with a patch material to widen the vessel instead of trying to balloon it again.IMG_6147

While our pulse ox saturations have been better than usual with readings in the low 90’s, they have room for improvement.  By adjusting the LPA, we hope to bump that up to 100.

Side note – I know you were wondering if Reiss finally had a BM. She did!  We are glad that happened before they put more sedation and other meds through her system, which could all add to the issue.

Tomorrow

Reiss will be NPO (no food/drink) at midnight today in preparation for a 7:15am start – the first case in the morning.  She will need to be on the heart/lung machine again for a small amount of time while they repair the pulmonary artery, but at least the surgery will be shorter than her previous one on Wednesday, which lasted 11 hours.  The NP projected the surgery to be complete by noon.

FullSizeRender (4)Tomorrow will be different than Wednesday.  Reiss will not be as carefree and just jump on the anesthetist’s back to go to the OR.  Thankfully, she already has IV’s and other lines still in and working. They will give her a medicine to make her loopy and easier to separate from her family.

Reiss will be groggy, recovering from the surgery for the rest of the day Thursday.  Friday, however, will start a new physical therapy regimen – including getting up and walking. Walking!  I’m so excited to get her up on her little feet.  I believe wholeheartedly that once she gets up and walking, her recovery will progress significantly.

The Pacemaker

SFullSizeRender (2)ome pacemakers are attached to the heart through a vein, and nestled up underneath the patient’s collarbone.  However, in a child the size of Reiss and with her defects, they chose an Epicardial option.  The leads are sewn into the external surface of the heart and the pacemaker is placed in the upper abdomen under the rib cage.

The pacemaker is pretty small (pictured, left).  The dimensions are about 2″ x 2 ” x 0.5″.  The picture only shows one lead with two branches, but Reiss will have two – one will have both branches sewn into the right atrium, while the other will reside in the right ventricle.

The pictures below show a diagram and description of the epicardial option and how big the pacemaker is in reference to Reiss’ hand.

FullSizeRender (3)IMG_6164

What to watch for at home

Here are some things we will need to watch for that will alert us to pacemaker, leads, or battery failure while at home.

  • Twitching in her stomach or chest muscles
  • Hiccups that won’t stop
  • Vomiting
  • Trouble breathing
  • Dizziness
  • Constant Fatigue
  • Chest Pain
  • Extra skipped heartbeats
  • Call the doctor if she falls or is hit on the pacemaker site

Luckily, we will go home with a bedside monitor.  This monitor will download all of the pacemaker’s daily information and send it directly to Texas Children’s Hospital without us having to do a thing – other than sync the two daily.  The receiver is mobile and can go where we go if we want it to.  This will help put our minds at ease if we feel like her pacemaker may not be functioning properly.

Also, we will go home with a pacemaker identification card for when we travel.  For instance, we would have to present this to airport security if we chose to fly.

Reiss’ Fixed Heart

Fixed heart.  This sounds foreign to me, and, technically, is a complete misnomer.  Reiss’ heart may be mended, made to function to increase quality of life and function, but it is most definitely not fixed.

Congenital heart defects (CHD) cannot be fixed.

It is a lifelong battle.  For some, it means transplants, multiple cath procedures, or open heart surgeries.  It is lifelong maintenance, pacemakers, testing, and follow-up appointments.  It includes machines at home, tubes, syringes, medicines, and becoming OCD with antibacterial solutions.  Some children live into adulthood, while many children lose the fight too early.

There is not a cure for CHD.

While we will want Reiss to experience life to the fullest, there will always be something she is not allowed to do – contact sports, MRIs, or anything that could compromise the safety of Reiss and/or her pacemaker.

Reiss’ mended heart will allow her to be around family more often, play more, do more, learn more about life, as well as greatly extend her life in comparison to what the Fontan promised.  We are ready to get the pacemaker placed, start her physical therapy regimen, and begin to make large steps in the recovery process.IMG_6167.JPG

Additional procedure and other issues

We have only briefly talked to the nurse practitioner about a supposed plan for Reiss this week.  Here is a quick summary of what we know(ish).

  1. The CT Scan showed some narrowing at the left pulmonary artery where it connects with the conduit from the surgeon placed in her heart last week.  We are not sure what the exact issue is, or how they may fix it.  The surgeon may just wait until he is physically looking at it to make a decision.
  2. Her heart continues to not conduct.  So, they are 99% sure she will need a pacemaker.
  3. The pacemaker placement and a fix, whatever that may be, for the narrowing of the pulmonary artery can happen at the same time.
  4. We are looking at possibly tomorrow, but do not have a firm date/time.

Reiss continues to have issues with headaches and an upset stomach.  So far today, she has only been able to keep down a small amount of food after puking a couple times.  We are getting a little more aggressive with making sure she has a BM. Hopefully that is the source of her upset stomach and discomfort.  They have started her back on an IV fluid to help reduce the probability of dehydration.

On a good note, she has been on room air for over 24 hours – and her pulse ox has stayed in the mid-90’s!  We are ecstatic about this!  Previously, with her pulse ox in the low 80’s/high 70’s, her body was creating extra vessels (called collaterals).  These collaterals were bursting, causing her to cough up blood (hemoptysis).  With our pulse ox in the mid-90’s now, and hopefully that increases after we correct the pulmonary narrowing issue and heal, the risk for hemoptysis should decrease as time goes on.

We will let everyone know when we have a firm date/time on the next procedure.