Trying to be patient...

Isabel did not move out of the ICU this week. It was so hard to be at the brink of a major step forward and for it not to happen...again. Although she has been doing so well, her potassium levels were low (mainly due to the diuretics that she is on to help her heart) and she required a higher supplement. Her belly was bloated, gassy, and distended earlier this week too, which can happen as a result of the electrolyte imbalance. She has had a gassy, distended belly many times before, but it has to be taken more seriously now that she has had abdominal surgery and a bowel perforation. So to be extra cautious, the docs stopped her feeds and took daily x-rays waiting for her bowels to move all of the gas through. I am happy to report that, as of today, her belly and x-ray looks great. They are restarting her feeds at 1/4 of her total volume and will increase them every 6 hours until she is back up to her full volume. At the same time, they will be working on getting her oral potassium supplements to a stable and adequate dose. This did push us a little off track of our goal and we are now looking at going  home at the end of January. Sincerely hoping that will be the case.


The Stable Vent Unit, that she will be hopefully be in by the first of the year, is a step-down unit, but they are much more strict about taking patients. It's essentially where ventilator dependent patients, who are otherwise ready to go home, make a stop. It is SVU team that makes sure that we, the parents, are ready to take on all of her care, that we have all of the supplies and equipment we need for home, and that we have our home nursing care arranged. They will also get to know Isabel before discharge because they will be the ones following us once we are home. They will manage her ventilator care and the weaning process for when she is ready to come off of the vent. So, they essentially want her "perfect," without the need for anymore changes to care or medication.

Tomorrow

Isabel has been doing extremely well. So well, in fact, that she is supposed to move out of the ICU and to the Stable Vent Unit tomorrow! It makes me so nervous to even write this. So hoping the transition goes well. Right now, our goal is to be home mid-January! Stay tuned for more of those details. Until then, here's a little story...

The other night when I was holding her, she kept turning her head to look at the TV. All that was on was Pandora Internet Radio, but she kept staring. So I sat her up facing the TV, with her back against me, and I put on the Disney movie Cars. She loved it! She just sat there with me for over an hour! Every now and then she would lean her head back and look up at me. It was so great! Me any my girl just hangin' and watching a movie.

Thankful

Isabel had a great weekend. Lots of snuggle time. Mom and Dad finally got some rest too.

Pediatric Surgery is very happy with how she has healed and recovered from her perforated bowel, and what we were waiting for happened...she pooped! Things are moving and working again, so they have started to feed her and switch IV medications back to oral (through feeding tube). They are increasing her feeds very slowly so not to overwhelm or stress her belly. She should be back to her full volume of milk by Wednesday.

November 16

From Dan:

Hi everybody.  Saturday was an extremely hard day for the Bumford family. I would have liked to share this story then but it all came at us at high speeds with very little time to think let alone write.

Amy and I got a phone call from Izzy's nurse around 10:30 in the morning. Her nurse said that they were worried that Izzy's bowel had a puncture from her recently placed feeding tube. They sent her down for a test to see if their worries were correct. Before they could conduct the test, Izzy's blood pressure dropped like a rock (a normal symptom for somebody with sepsis or infection). They rushed her back to her room and worked frantically to get her blood pressure stabilized. With a lot of meds they were able to do so. They then spent some time trying to figure out what to do next. They completed the test to see if her bowel was punctured and it was. The contents of her bowels had leaked out into her abdominal cavity.

Shortly after everything was stabilized they had Izzy set to head down for emergency repair of her bowel. When she went down we weren't sure that we'd ever see her again. It was a long 4 hours of waiting. Thankfully our parents and friends were able to be there with us. The repair went very well and Izzy has been stable ever since. The docs have been able to take her off of all blood pressure meds (woo hoo!) but have had to increase her pain and sedation meds while she recovers from another painful surgery.

We are so proud of the strength and fight that our sweet girl continues to show us. Despite all of our struggles we continue to praise God for the miraculous things that he is doing in and through Izzy. And we continue to thank Him for surrounding us with great friends and family that continue to hold us up through these trying times.

Getting caught up

Since my last Facebook post (see below), Isabel has had a few ups and downs, but as of today she's doing extremely well.

(Facebook 10/24/13: Isabel is having a truly fantastic week. All of her meds, except one straggler, are now given orally through her feeding tube. She was put on the small home ventilator on Monday for what we thought would be a 2 hour trial, and she has been on it ever since! She is awake, happy, and according to one the case workers, "doing so well"! Lots of smiling doctors talking about Little Miss Izzy Bumford. Depending how things go in the next couple of days, we may even be out of the ICU as early as next week. Just have to wait and see. The next stop is the Stable Vent Unit and then home. Lots to think about, but we're feeling very encouraged.)

We're still in the PTCU (Pediatric Cardio Thoracic ICU). Just a couple of days after that post, Isabel was having a hard time breathing and started to spike fevers. She came down with a minor case of tracheitis and pneumonia and had to go back on the conventional ventilator for about a week. It was a really hard week because she required more support medically, as well. She is still having fevers off and on and the docs cannot seem to pinpoint a cause. It's extremely frustrating! They have ruled out infection numerous times, have tried to find a correlation to a medication, and haven't found anything. During all of this they have stopped and started slowly weaning her pain and sedation medications, methadone and Valium, and are beginning to think that minor withdrawal is the cause for the fevers. Unfortunately, that is very hard to prove, and they need to rule out any other possible causes first. These fevers are especially difficult because they have been preventing her from making progress in other areas, in particular, having a procedure to get an abdominal feeding tube. We'll need to be in the PCTU until after she has this done, and doctors/surgeons will not perform any procedures on a patient who is has a fever or has had one in the last 48 hours.

About that feeding tube...Isabel has been tube fed since birth. Being on a ventilator initially prevented her from being able to feed by mouth, and since she was on a ventilator for so long she never had the opportunity to try. As many of you know, feeding by mouth can be challenging even for "normal" babies at birth as they learn to latch on to breast or bottle. This is further complicated by the fact that children with CHARGE syndrome have cranial nerve defects, which can affect swallowing. Despite all of this working against her, she does have a decent sucking reflex and takes a pacifier pretty well, so we are encouraged, but it will be a while. She will have to have swallow studies done down the road to make sure that she can swallow and that it all goes down properly. Until then, she will be tube fed. In pictures, the yellow tube in her nose is her feeding tube. What she will hopefully be having done later this week is a procedure to get a feeding tube port in her abdomen. Like the tracheostomy, it should only be necessary for the short-term, and is not permanent. The tube can be taken out in the future with no long term issues. It sounds much scarier than it is, and will be better for everyone in the long run. She won't have any more tubes on her face, in her nose, and down her throat, and the abdominal feeding tube can be disconnected when she is not being fed! I'll share more about the procedure and her specific tube soon.

On a very positive note, she recovered from the pneumonia and is back in the LTV, or small home ventilator. She has been on that for over a week now, and they have even been weaning some of the settings. She's a rock star! She shouldn't ever have to be back on the conventional ventilator except for when she has her feeding tube procedure. Sincerely hoping that this is the case. She has been spending a lot of time in her bouncy seat, and swing, which she loves. She also LOVES her mobile! She gets fussy when it stops and smirks when we turn it on again. I love that girl so much.

More of the Story

What we have not yet shared publically about Isabel is the cause of all of her medical issues. We have been reluctant for a variety of reasons, but now feel that we are ready. Isabel has been diagnosed with CHARGE syndrome. CHARGE is an acronym for several conditions that individuals can have, and until recent years the combination of some, or all, of these conditions was the only way that the syndrome was diagnosed.

This is the acronym that was used to diagnose CHARGE:

C - Coloboma of the eye (malformation of the iris and/or retina)

H - Heart defect

A - Atresia of the choanae (blocked nasal passages)

R - Retardation of growth and/or development

G - Genital or urinary abnormalities

E - Ear abnormalities

In 2004, a single gene mutation was discovered as one cause of CHARGE. Only about half of individuals with a CHARGE diagnosis have a mutation in this gene. Isabel’s genetic testing revealed that she does have this mutation. Sure we were nervous when we received this information, but overall we were relieved. In the weeks leading up to the formal diagnosis, many doctors suspected that she had CHARGE, some were positive that she did, others were not sure.  They were not sure if the test would be positive and give us an answer, or negative and give us nothing, and they were not sure if there was some other underlying cause for her issues. So, we felt relieved. We had an answer and we could move forward.

What we know about Isabel is that she was born with a heart defect (Incomplete AVSD), blocked nasal passages, and malformations of both her eyes and ears. Her nasal passages and heart have been repaired, although she will most likely need a follow up heart surgery sometime in the future. We know that she can see and hear, but don’t know to what extent. She will be having full hearing and vision exams in the near future to determine if she has any impairments in either of those areas.

Having both blocked nasal passages and a heart defect that causes increased work for breathing were the main reasons for having to be on a ventilator. Her lungs work just fine! Despite her lungs working well, being on a vent can stress the airways to the lungs making them weak (Tracheobronchomalacia). This is why she is still on the ventilator now, and will be for a while in order to give her airways time to grow and strengthen. Having a tracheostomy allows her to be on the vent, without holding her back in other ways. We’ll just have to wait and see how long she needs it.

What we also know about Isabel is that she has gorgeous, wavy, auburn hair, a dimple on her left cheek, dimples on her elbows, long skinny feet, toes, and fingers, and a spirit that won’t quit. She has a very strong grip, long thick eyelashes, and loves her pacifier. She has her daddy’s brown eyes and her mama’s knobby chin. Her fingernails grow really fast. She rolls her eyes like a teenager, and scrunches up her face to let Mama to know she is “done” with taking pictures. She loves to listen to music and the sound of Daddy’s voice. She has captured our hearts and the hearts of many of her caregivers.

There are still a lot of things that we don’t know about her growth and development in the future, but then again, no one knows what their child will be like, or what they will need. We are scared and nervous because we do not know what our lives will be like, and we are so excited to learn more about our little girl. Her likes and dislikes, her personality, who she really is, and how many lives she will touch through the journey. We love her more than we can even begin to explain.

Thank you all so much for your constant encouragement, love, support, and prayers over the past few months. It has been a crazy roller coaster so far, and we have a feeling that the ride is only beginning...   

Welcome

Welcome to our blog. Here we plan to share the life and journey of our beautiful, little girl. She was born 5 weeks early on Wednesday, June 26, 2013 at 3:18 am. She weighed just 5 pounds, was 18.5 inches long, and had a full head of hair. She is a dream come true and a blessing from God. Here is the first picture we have of her. 

She was on a ventilator. She had IVs going into and out of her umbilical cord. She had an IV in her head, and all kinds of other leads and lines. It was simultaneously the most amazing and most terrifying thing we had ever seen. Today, she is still in the hospital, but doing extremely well, and we look forward to the day that we can take her home with us.