Today marks almost two months since my last blog and it is hard to believe just how fast time is passing by…
 
On May 19th, we celebrated ten months with our miracle boy. It has been an incredibly long journey, but thankfully Caleb has overcome every obstacle and bump along the way. Caleb is a very happy boy and he can light up a room with his infectious smile. His resilience is amazing and we are reminded daily of just how far he has come. 
 
For the most part, since our transition to Mt. Washington Pediatric Hospital on February 11th, things have gone smoothly. However, it would not be Caleb without his share of complications. Caleb has continued to heal and is getting bigger and stronger each and every day. Caleb weighed 3 pounds 2.09 ounces at birth and is now weighing in at 18 pounds 13 ounces.  Caleb continues to receive physical therapy, occupational therapy, and speech therapy almost daily and he especially enjoys his play time with child life.  Caleb is usually content hanging out in his crib watching his mobile and playing with his rattles and rings that hang from his activity gym.  He also enjoys his custom rocker, playing on his mat, bath time, visitors and of course snuggle time.  On April 30th at 285 days old, we were finally able to take Caleb for a leisurely stroller ride outside.  Since he had only gone outside for transports back and forth to Johns Hopkins for appointments, this was an exciting time for us, but he was a bit unsure of the sunshine.  We walked around Mt. Washington, put his feet in the grass for a few mintues, and captured this experience and his reaction on camera.  We are looking forward to many more of these firsts for him.
 
Caleb has had additional follow-up appointments with cardiology and neurosurgery.  The echocardiogram showed that his heart is functioning normally and that the pulmonary hypertension that he once had was fully resolved.  This is great news but since this is something that can change momentarily, he will be monitored closely and he will continue to take a medication to help relax and widen the blood vessels in his lungs to decrease the pulmonary blood pressure to his heart and improves its function.  His head ultrasounds have also remained stable and they have made a few adjustments to the setting on his shunt.  He is at the highest setting meaning less fluid is being drained.  Caleb will have repeat head ultrasounds every 1-2 months to ensure that the shunt is working properly as these often need to be replaced within six years.  
 
Before leaving Johns Hopkins, we had a family meeting with Caleb's general pediatric surgeon and he had stressed the importance of letting Caleb heal.  Knowing the difficulties of what another surgery would entail, he had made the decision to send Caleb to Mt. Washington with a nasogastric tube (NG tube), which is a special tube that carries food and medicine to the stomach through the nose.  However, as Caleb began to get better and better each day and there was talk about sending Caleb home, there was a huge concern that the NG tube was not ideal for an at home setting.  One of the most common complications of enterrally fed patients through an NG tube is aspiration, which can ultimately lead to pneumonia.  The NG tube can become malpositioned during routine use and since Caleb will be on the go when he comes home, this was not something the doctors were willing to risk.  

So, after several conversations between Caleb's doctor at Mt. Washington and his surgeon at Johns Hopkins, Caleb was set to undergo his 6th surgery to have a gastrostomy-jejunostomy tube (G-J tube) placed.  A gastrostomy (stoma) is a surgical opening made through the skin of the abdomen (belly) and into the stomach and the G-J tube is a “tube within a tube” placed through this opening.  One tube ends in the stomach and the other extends into the jejunum (the small intestine).  This allows food and medicine to be given directly into the stomach or jejunum instead of through the mouth and can safely be done at home.  Hopefuly, the G-J tube will be temporary and will allow Caleb to get enough nutrition to grow, develop, play, learn and recover at times of illness.  Caleb's original surgery date was postponed due to him contracting a norovirus infection, which was characterized by a fever and the sudden onset of severe vomiting and diarrhea.  This is highly contagious and occurs most frequently in closed environments such as hospitals.  Caleb's symptoms lasted 4-5 days and the virus seemed to make it's way around Mt. Washington infecting staff and several other babies.  After what felt like an eternity, Caleb's surgery took place on May 5th. The surgery to place a G-J tube is typically pretty quick and simple, but Caleb's lasted about four hours and was much more complicated than expected...of course!  Due to Caleb's congenital diaphragmatic hernia (CDH) and the displacement of his abdominal organs, his stomach was not easily accessible.  Although the end result was the placement of Caleb's G-J tube, it entailed making an additional incision which was utilized to move his colon and kidneys, correct the malrotation and twisting of his intestines, and a 2nd CDH repair.  While performing the surgery, Caleb's bowel was damaged and three centimeters had to be removed.  Caleb's recovery was longer than anticipated, but thankfully his respiratory status remained stable.  In order to administer his nutrition and fluids while his abdominal area healed, a central line was placed and caused his next setback a few days later.  Caleb had suddenly spiked a fever of 104°, which remained on and off for days.  It was determined that Caleb had a central line blood infection and he received a seven day course of antibiotics while at Johns Hopkins.  In the past, Caleb had developed severe sepsis from a central line infection and as a result, he coded and needed chest compressions.  So, as you can imagine, this was an extremely scary time, but once again, he bounced back and made a full recovery.  When Caleb arrived at Johns Hopkins for his surgery, he was completely wean off of his pain and sedation medication (clonidine, valium, and methadone) for the first time in his life.  The weaning process had been long and difficult at both Johns Hopkins and Mt. Washington so it was difficult knowing that another surgery meant more drugs to control his pain.  We are three weeks out of surgery and Caleb is still receiving oxycodone, but the hope is that after another slow wean, he will get his last dose by the end of the week.  

Caleb has since been transported back to Mt. Washington and is back to himself again.  His therapies have been restarted and they are attempting to bottle and spoon feed again.  For kids with CDH, feeding by mouth is a struggle and it could take months to even years before Caleb is receiving his full daily nutrition this way.  In the meantime, he will be on continuous feeds through the jejunum port of his G-J tube and medication will be administered through the stomach port of his G-J tube.  No matter how long this takes and how frustrating it may be at times, we will continue to live by our motto from the beginning of this journey...slow and steady wins the race!

The talk of discharge has surfaced and it could be right around the corner as long as the little guy can stay healthy!  Caleb has been approved for nursing, but typically only night hours are given so we will need to fight for day nursing shifts since we must continue to work.  I wish, as his parents, we could take over his care at home but unfortunately this is not an option.  Finding a reliable nursing agency is hard, but it is even more difficult to locate qualified nurses in our area that can meet Caleb's needs and who have experience operating a vent and working with a child with a tracheostomy and G-J tube.  He will obviously remain Mt. Washington until around-the-clock nursing can be arranged and we can fully trust those individuals with Caleb.  This is the most exhausting and stressful aspect of him coming home as we have heard many horror stories of families in the same position.  We are continuing to take advantage of the many training opportunities at Mt. Washington to make certain we are capable of acting in an emergency situation and performing some of the necessities such as administering medication and feeds through his G-J tube, changing his tracheostomy strings (holds the trach in place) twice a day, and inserting a new tracheostomy once a week.  In an attempt to make things easier for when he comes home, Caleb's doctors are trying to make a few weans on his vent, but only time will tell how he will progress and handle the changes.

More to come soon...

Keep fighting, Caleb and stay healthy!!!

I honestly don't even know where to begin. The last two weeks have been filled with good days, bad days and everything in between. The last I wrote, Caleb was recovering from eye surgery and his 2nd CDH repair.

Thankfully, Caleb's follow up appointments with the eye doctor showed that his eyes responded perfectly to the surgery and he should no longer be in danger of further retina eye disease. The goal of the surgery for retinopathy of prematurity is to stop the progression of the disease and it appears it has at the moment; however, a degree of Caleb's peripheral vision may be lost. He will have follow up exams every few weeks while at the hospital and then he will need regular, yearly eye exams for most of his life. This was such a relief and some much needed good news...

When Caleb came back from his CDH repair surgery, the pediatric surgeons expressed their concern over whether or not the two additional patches would hold since there was not much viable tissue in the surrounding areas. Our biggest fear came true just thirteen days later when we learned that Caleb had reherniated again. Although this is typical after an initial CDH repair, it usually does not occur until months or even years later. The panic set in and my mind started racing. The next few days were a blur as we tried to process everything. We met with the surgeon and he addressed what felt like a million of our questions. Unlike the last recurrence, they do not believe that emergency surgery is required since only a small portion of his stomach is up in his chest and it should not be causing Caleb any distress. In addition, they do not believe he has fully recovered from the last repair and they want to make sure he is strong enough to withstand another surgery, if and when that decision is made. We discussed the latissimus dorsi muscle flap repair, which is another approach besides the patch, but Caleb is just not big enough yet. After discussing our options it was determined that the plan of action would be to monitor the reherniation closely and most importantly get him bigger and stronger. His lungs are very immature and there is a lot of scar tissue so his respiratory function will be their primary focus.

This week, the NICU doctors will be consulting with the pulmonary team, who deals with diseases involving the respiratory tract and manages patients who have needed prolonged mechanical ventilation. Although Caleb went several weeks with no mechanical ventilation, he still needed the help of continuous positive airway pressure (CPAP) and the nasal cannula. They had been unable to wean this support and his tachypnea (rapid breathing) was still present and at its worst. As a result, they have mentioned the possibility of Caleb needing a tracheostomy, which is an opening into the windpipe (trachea) that is held open by a tracheostomy tube and helps you breathe when the usual route for breathing is impaired. After the initial shock, we have started to prepare ourselves for this procedure and focus on some of the benefits. Caleb will be able to breathe easier as it offers a more stable and secure airway, he will not require as much pain medication and sedation, and he can begin developing at a normal pace. As parents, we will be able to see his adorable face (entirely), enjoy his coo’s as only a baby can do, see him smile, and we will be able to hold and interact with him again. If this is determined to be the direction in which the doctor’s proceed, the hope is that tracheostomy would be temporary and can be removed once Caleb’s lungs grow and develop over time.

With both surgeries just a day apart, Caleb's already immature lungs took a big hit and he still hasn't recovered. Although they tried to extubate a few days after his CDH repair surgery, he proved that he wasn't ready. He remains intubated and has needed more respiratory support over the last week then he has in a long time. In the midst of trying to recover from the surgeries, he has developed two endotracheal tube (ET) infections and a blood infection, which means more setbacks and antibiotics.

On a better note, Caleb’s first Halloween was one to remember. He was the cutest bat, monster, pumpkin, pea pod, and skeleton and being dressed in the crazy outfits didn’t seem to bother him at all. A special thanks to the nurses and RTs who helped make it so memorable!!!

The goal is to keep him comfortable and get him healthy (no more infections) in the days to come and to improve his respiratory status. Hoping he continues to tolerate the steady increase back to full feeds, slow wean of his methadone, and most importantly that his reherniation remains stable and unchanged.

We are beyond grateful for the continued support of our family, friends, and even those we do not know. Your thoughts, prayers, messages, and texts are what get Caleb and us through this extremely difficult time.

Stay strong and keep fighting, Caleb! 

Shortly after finishing my last blog about the good days, the bad days started to resurface quickly.

Once Caleb was stable enough after birth, they began routine eye exams every two weeks to screen for a condition called Retinopathy of Prematurity (ROP). ROP is a disease that occurs in premature babies where abnormal blood vessels grow in the retina, which is the layer of nerve tissue in the eye that enables us to see.  This growth can cause the retina to detach from the back of the eye, leading to blindness.  About 1,100–1,500 infants annually develop ROP that is severe enough to require medical treatment and about 400–600 infants each year in the United States become legally blind from ROP.  Following his exam on Monday, October 13th, I received a message from his doctor at the Wilmer Eye Institute and my heart dropped, as I knew that it could only mean bad news.  ROP is classified in five stages, ranging from mild (stage I) to severe (stage V) and we learned that Caleb had developed stage II "plus disease" meaning that his blood vessels of the retina had become enlarged and twisted, indicating a worsening of the disease.  Laser surgery (also called photocoagulation) was performed on Tuesday to help destroy the peripheral areas of the retina in hopes that it would slow or reverse the abnormal growth of the blood vessels.  Since the surgery focuses treatment on the peripheral retina, these areas will be scarred and some amount of Caleb's peripheral vision may be lost.  However, the goal is to preserve the central retina to allow him to be able to perform vital functions like seeing straight ahead, distinguishing colors, reading, etc.  Although ROP surgery has a good success rate, not all babies respond to treatment.  Caleb will be followed closely to see if his eyes are responding to the laser treatment, but we will not know the results and whether or not the progression of the disease was stopped until around October 28th.  If the ROP continues to worsen, Caleb may need additional laser and/or other eye surgeries.  Of all things that could happen, the potential for blindness is one of the scariest to me as a parent.  The waiting period continues to be extremely difficult and I can only hope that Caleb is lucky enough to see the world and all of the things that we take for granted on a daily basis.

Caleb's next obstacle and fourth surgery came on Wednesday, October 15th at just eighty-eight days old.  The doctor's plan for the day was to just monitor Caleb's pain and keep him comfortable since eye surgeries are very tough on babies.  That afternoon, a random chest x-ray was ordered to check the placement of Caleb's feeding tube.  It was discovered then that he had re-herniated and a portion of his bowel was once again up in his chest. Although we knew this was a possibility, nobody expected it to happen so soon after his initial CDH repair.  Caleb underwent emergency surgery that night.  It is believed that this subsequent surgery to repair the recurrence of his CDH was needed due to the severity of Caleb's CDH, his age at repair (twenty-four days old/31 weeks gestational age), and the fact that a GORE-TEX® patch was used during the initial surgery.  Often times, the patch pulls away from the chest wall, as the child grows, causing a recurrence.  It turns out that his diaphragm needed to be re-patched in two places and unfortunately, the general pediatric surgeons are not confident that the patch will hold due to the difficulty they had in utilizing the available tissue to make the repair.  The condition of the patch will be closely monitored by doctors to ensure that it remains intact.    

Caleb is three months old today and has now undergone more surgeries than some people have in a lifetime.  After seeing him do so well the past few weeks, I feel as if we are starting over.  He was re-intubated for surgery and will remain on the conventional ventilator until his respiratory support can be weaned, his feeds have been stopped, and his pain is being controlled with both methadone and morphine.  I miss my very alert little boy terribly and am longing for the moment when I can snuggle with him again.  

Through all of this, Caleb's strength is admirable.  We will continue to focus on his amazing progress thus far and stay positive as he recovers from the events that took place this week.  I can only wish that things begin to get easier for him...

We ask for your thoughts and prayers as we await the results of Caleb's eye surgery.