The Basics

Caden Gabriel is the son of Hank and Sherry Osborne.  Caden was born on November 2, 2004 (Election Day). Our family lives in Goose Creek, SC which is just outside of Charleston.

Caden has endured numerous complications associated with a 22q11.2 chromosome deletion that was detected before his birth.  This disorder is also called DiGeorge Syndrome.

Caden has one older brother Riley and one younger brother Josiah (JJ).

Name Meanings and Popularity

Caden is an American name defined as “fighter” according to BabyNames.com. The name Caden is growing in popularity and is nearing the top 100 most popular names given to boys in the United States according to the Social Security Online’s Popular Baby Names page.

Gabriel is defined as “God is my Strength” according to Baby Center. Gabriel has been hanging around number 30 on the top 100 most popular names according to the Social Security Online data.

This child has lived up to his name!

As you read Caden’s Page you will clearly see that Caden is a fighter with the God as his strength.

Caden’s Current Condition

As of January 2009

DiGeorge Syndrome or VCFS

The primary diagnosis for Caden is DiGeorge Syndrome or Velo-Cardio-Facial Syndrome (VCFS). These names are used to describe a micro deletion of a small band on the 22nd chromosome. Caden’s diagnosis is also referred to as a 22q11.2 micro deletion. All of the items listed below are related to this primary diagnosis. Caden will recover from the complications listed below during his childhood and will lead a fairly normal life. We learned of the 22q11.2 diagnosis just a few days before Caden’s birth. We learned about halfway through the pregnancy that Caden would be born with multiple heart defects and likely some other medical complications.

Caden was seen by Dr. Elaine Zackai and Donna mcDonald-McGinn at the 22q and You Center at the Children’s Hospital of Philadelphia (CHOP) in May 2008.

Heart Defects and Surgeries

Caden was born with five separate heart defects in addition to having a right-sided aorta. The defects were an interrupted aortic arch type B, VSD, ASD, enlarged PDA, and bicuspid aortic valve.  While the right-sided aorta is not considered a defect it did cause the interrupted aortic arch repair to be somewhat of an exploratory surgery surgery.  The right-sided aorta appears in about 5% of the general population.

On September 22, 2008 Caden had a Ross Procedure to repair a bicuspid aortic valve.  The surgeon also removed a pacemaker that had been in place since June 2006. Now that Caden has a foreign tissue valve on his pulmonary artery, he will be followed every six months to make sure the valve is sustaining the flow required by his body. Most heart patients who receive a donor valve at Caden’s age have it replaced with a larger valve before they reach their teen years.

On June 15, 2006 Caden had the pacemaker implanted. This was primarily due to “heart blocks” detected after a heart catheter that was done in March 2006. Several halter monitors were done that continued to show heart blocks. The pacing device was recommended even though Caden has recovered from every heart block on his own with the exception of the first one he had during the heart cath procedure back in March. That event required chest compressions for about a half minute until they could get the medication in him to stimulate/speed up his heart signals. Read below for what led up to Caden getting the heart cath and read HERE for the details of the heart surgery to put the pacemaker in and other thing that was done (see swallowing section). The 6 week pace maker checkup showed that it was not longer working because it didn’t fire consistently when they tried to raise his heart rate with the pacemaker. The Holter monitor they put on him that day came back as having irrethemias. There were plans made for a pace maker repair but it got canceled because of illness discovered at the pre-op appt. It was discovered at the pre-op, when they interrogated the pace maker, that the it hadn’t needed to fire. They then turned the pace maker off and the surgery was rescheduled for Nov. 06. We decided against having the surgery and cancelled the date.

On Feb. 14th 2006 Caden went in for a routine cardiology checkup visit. Caden had a scheduled sedated echo cardiogram to look at his aortic valve that was repaired on 11-8-04. To our surprise, and the cardiologists, they diagnosed Caden with a Supra Valvar Aortic Stenosis. The simple definition for that diagnosis is a narrowing of the aorta just above the aortic valve. This narrowing is not in the same area where Caden’s Interrupted Aortic Arch was repaired shortly after birth. Based on this new information, the doctor’s plan was for Caden to go in for open-heart surgery by the end of March 2006 to repair the newly diagnosed condition. Regular updates were made on Caden’s condition on the main page.

Caden still has a diagnosis of a bicuspid aortic valve which was not repaired during his surgery at six days old. This defect was present when Caden was born, but the surgeons had to focus on the most life threatening heart problems at that time.

The cardiologists had planned open heart surgery to repair the Stenosis. Read HERE for the details of the heart catheter mentioned above that was performed in March 2006.

Swallowing – Tube Feeding

Caden’s swallowing functionality is one of our main concerns. We are working with several speech and occupational therapists as well as talking to doctors at MUSC and other clinics around the country who specialize in treating swallowing issues in young children. It is a general consensus between all parties involved right now that Caden’s problems seem to still be medical. Caden does not have an aversions to having things in his mouth, but he does still struggle to get the least little bit of food down.

Caden is 100% tube fed through a g-tube. He even gets his hydration through the tube. Caden has been given very small amounts of food by mouth during feeding therapy only. Nearly all of that has come back out his nose or had to be suctioned out using the portable suction machine we carry everywhere we go.

This has been Caden’s most persistent problem since birth next to the heart issues. And first let me say that the doctors, nurses, and therapists have only found one article in medical journals on this problem. Even that article was sketchy at best. Caden has been diagnosed with a condition that is often called Achalasia which basically means that the upper sphincter of the esophagus does not function properly. His ENT doctor says that there is a “discordination” with his swallowing. Meaning that the upper sphincter doesn’t open and allow things to go down the same time that he swallows. Caden is usually able to clear most of his own secretions and saliva… that is all we’ve seen these days. This lack of swallowing functionality does occasionally cause Caden to get suctioned using a portable suction machine to keep him from gagging on and getting fluid into his lungs. Getting fluid in the lungs (aspiration) can cause pneumonia or even drowning in extreme cases. Caden has always protected his airway amazingly well and he has become better able to handle his secretions as time goes on. These days Caden can blow most of his secretions out his nose.

Feeding – Because of the above mentioned swallowing issues, Caden gets all his food and water through a feeding tube (G-tube) which is surgically inserted into his stomach. His formula has been switched to Nutra Renal, a highly concentrated and special order formula. This switch was made so we could go from 4 to 3 feedings a day without having to increase the volume of milk he receives per feeding. He also receives bolis of water 4 times a day. Caden’s endocrinologist directs us on the formula to use because of a diagnosis of Hypocalcemia.

Past swallowing treatments – Caden has had his esophagus scoped completely and has had 3 botox injections into the muscle tissue around the upper sphincter of the esophagus. Theoretically, this relaxes the muscles to allow food, saliva, medicines and other things to travel into the esophagus and then into the stomach. He got the first botox treatment in August 2005, then again in September 2005, and in November 2005. Caden has been able to handle his secretions for the most part since these botox injections. He has also had his Left Subclavian Artery surgically clamped off because it was discovered to be protruding into his esophagus and possibly preventing swallowing ability. This was done by the heart surgeon during the pace maker surgery back in June 06. We still have not seen the manifestation of swallowing from this procedure; however, there hasn’t been any side effects of clamping off the artery either.

We have tried using Neurontin (a neurological medication used to treat Epilepsy) for GI and to see if there is any improvement in swallowing since that “discordination” is possibly neurological. no improvement was seen for Caden. In the future, the ENT may want to do a “Z Plasty” on the palate to correct the diagnosed Sub mucus Cleft Palate. This surgery is done to help with the formation of certain speech sounds that require pressure (IE. D, B, P, and G sound). There has been NO research saying that the Z Plasty is done to correct swallowing.

Caden has had a sphincter plasty that had no affect on swallowing and little affect on speech development.

Future swallowing treatment – We are hoping and praying for a miracle in the area of swallowing. It seems that we have tried most all of the viable options with no success. It is time to give this issue to God.

The only medical option on the table is a myotomy of the upper esophageal sphincter (UES). This is an irreversible surgery that involves cutting the muscle tissue around the upper opening of the esophagus. There is very little research showing success with this procedure in children. It is more commonly used on elderly patients who have trouble swallowing. Our ENT has been reluctant to recommend this procedure for Caden.

Sub mucus Cleft Palate

Caden has also been diagnosed with a Submucus Cleft Palate. This is a high soft palate. It is very common with 22q11.2.

Scoliosis

Caden has been diagnosed with scoliosis. The most recent measurement of the curve was 53 degrees.  The curve was first detected in early 2006.  There was a fairly significant progression of the curve during a follow up visit during July 2006 when we learned that we needed an MRI of the spine.  The MRI could not be performed at that time because Caden had a pacemaker implanted in June 2006.  The pacemaker was removed in September 2008 clearing the way for the MRI.  We have seen two orthopedic surgeons at MUSC and one at the Children’s Hospital of Philadelphia (CHOP).

Reflux

Caden had a severe case of projectile nasal reflux. This caused “failure-to-thrive,” lots of suctioning, and contributed to developmental delays (because mobility was limited). Caden’s past reflux problems were corrected with a Nissen Fundleplication. He has not experienced reflux since the procedure was performed on June 6, 2005. This procedure made it possible for him to come off of the Previcid, Zantac, and Reglan medicines.

Complications of Reflux – Caden still has what’s called “retching spells”. Retching is the act of refluxing without anything coming up. Caden turns red in the face, difficulty breathing (briefly), significant discomfort, sweating/clamyness, and at times a zoning out where he just tries to go to sleep. We have helped the retching by attaching a syringe (without the plunger) to Caden’s G-tube and allowing his stomach to vent out. This also helps to relieve any air/gas in his system. It normally takes just a few minutes for the retching to end and for Caden to go back to what ever he was enjoying before it began. We almost always have to suction him when he retches because of the massive amount of secretions it produces (we still don’t understand the connection!) The retching has gotten much better over time. Now he only occasionally retches, usually right after his feedings, and they are not as severe as they used to be.

Immune System

Caden was diagnosed with a mild to moderate immune deficiency at birth. This basically meant that he was more vulnerable to viruses than the average person. Caden has received all his vaccinations to date. We took quite a while before getting the live ones which are MMR and Varicella (Chicken Pox). Caden had blood tests performed to monitor his immune system and be sure it was strong enough to withstand the vaccines.

Immune System Treatment – There’s no treatment needed, but rather precautions. Caden should not be exposed to anyone who has recently received a live vaccine or who has a viral infection. Other than that, his immune system is believed to be strong enough to hold its own and his blood cell count is rising.

Calcium Deficiency

Caden was diagnosed with a calcium deficiency called “Hypocalcemia” at birth through a blood test. Caden was prescribed a calcium supplement that is given via his G-tube three times per day. Low Calcium can cause seizures if not monitored and treated properly. Praise God, Caden has never had a seizure! We are continuing to believe and claim that Caden will come off this calcium supplement though the endocrinologist has not been so encouraging.  As of the Fall of 2008 we are down to 2.5 ml of calcium once per day.  We started at 6 ml three times per day at birth.

Small Finger on Left Hand

Caden’s smallest finger on his left hand has a knuckle that will not straighten. It is frozen at a 90 degree angle. This will likely require surgery in the future to correct but not until Caden’s hand has matured more.

Left Ear

Caden has an area of his left ear that is folded over and fused. This will be corrected once the ear has grown more if it has not already be corrected by the healing graces of God. We have seen a noticeable difference in the appearance of Caden’s ear in recent months. Praise God!

Previous Challenges for Caden

Nursing : Staffing

The problem was that there were no nurses when needed. Now Caden gets along fine without 24/7 supervision. He sleeps in his own bed and does not require suctioning for the majority of the night. That was not always the case. The first 6 months at home where horrible.

Nursing : Medicaid

Caden has Medicaid through TEFRA Medicaid. This program is also called the Katie Beckett Medicaid program. This form of Medicaid pays for doctor visit co-pays, hospital deductibles, and prescription co-pays where Caden’s private insurance does not pay. What this Medicaid program does not pay for is private nursing care. Coverage for private duty nursing care in the home can be paid for by Medicaid, but it is a separate application process known as a Medicaid Waiver in South Carolina. The application process is very confusing and not everyone involved in the application process even knows all of the steps required to get the application processed. Caden’s application has been processing for a few months now and we still do not know if or when he will be approved. Even if Caden was approved for the Medicaid waiver today we would not be able to utilize the funding due to staffing shortages mentioned above.

Alternative Solutions for Nursing – The first solution is that we accept help from anyone we know who is (a) willing to help and (b) can handle it. Some people find it difficult to care for Caden. Sometime Caden fights pretty hard when getting suctioned, but it has to be done. He is getting stronger and harder to restrain.

The second solution is for us to mitigate the need for nursing. The need for nursing stems from Caden’s lack of ability to swallow because he could aspirate on any fluids that gather in his throat. We have scheduled the (botox) treatment for August 3, 2005. We hope to mitigate the need for in-home nursing care with this treatment.

Summary of Caden’s Past

This web site was started in November 2004 to keep family and friends updated on Caden’s condition after his birth. We also use Caden’s Page to post pictures of Caden, his brother Riley, other family, and friends. Sherry and I knew as far back as July 2004 that our new baby boy Caden was going to be born with a fairly serious heart defect. He was diagnosed during the pregnancy with and Interrupted Aortic Arch (IAA) type B with a ventricular septal defect (VSD). The OB wanted Sherry to get an amniocenteses to check for Downs and other possible chromosome abnormalities linked to these heart defects but she did not want to. Later in the pregnancy (34 weeks) she had got so big (51 cm) across the belly that they suggested an amnio reduction to give her comfort. The condition was called polyhydramnios. She agreed to the amniotic fluid reduction and we ask that some fluid be sent off for chromosome testing at that time. The results came back positive for a 22q11.2 micro deletion. We got the results from the test approximately five days before Caden was born.

The micro deletion on the 22nd chromosome is most commonly referred to as DiGeorge or Velo-Cardio-Facial Syndrome (VCFS). This all means that the 11.2 band of the Q (long) arm of the 22nd chromosome is missing. The visible symptoms of this chromosome abnormality vary greatly. There is a long list (over 180 items on VCFSEF.ORG) of possible complications associated with 22q11.2 deletions. Most people are believed to only show signs of just a handful of these symptoms. Caden is displaying close to a dozen of the symptoms associated with DiGeorge and VCFS already with five of them being related to his heart alone.

Once Caden was born on Election Day 2004 he went directly to the ICU. He was put on prostaglandin to keep his PDA open until surgery. The PDA is a natural bypass that all newborns have to allow for blood from mom to be distributed to all parts of the baby’s body before birth. It normally closes automatically within a few days after birth. The PDA closing on Caden would have been fatal. The doctors did a fetal echo cardiogram and found that Caden not only had an IAA TYPE B with VSD, but he also had an Atrial Septal Defect (ASD). That was not all. Caden had a right-sided Aortic Arch. This only occurs in about 5% of the general population. To our knowledge, this particular medical university had never seen a right-sided IAA TYPE B. That said cardiologists and surgeon did not paint a very rosy picture for us. They said that to some degree that they would have to wait and see what Caden’s anatomy was really like once they got in there for the surgery. All went well through the surgery at six days old for Caden. Later the evening after surgery Caden had fluid start to build up in the chest cavity. The surgeons had to open Caden’s chest back up and leave it open for a few days to relieve the pressure. They also had to add other drain tubes to help fluid drain from around Caden’s heart and lungs.

Caden spent exactly one month in the PCICU at MUSC after his surgery. He then went to his own room on the cardiac ward of the Children’s hospital at MUSC where he spent another four weeks. There he had difficulties swallowing and experienced severe nasal reflux. He was diagnosed with conditions called dysphasia and GERD. He failed two barium swallow tests while in the hospital. Caden could not even swallow his own secretions. The answer to this problem was to give Caden a G-tube. This is a tube that is surgically inserted into the belly to allow him to be fed directly into his stomach with a pump. We were told that Caden would remain on this pump until he was able to swallow which could take anywhere from months to a couple of years. Caden’s reflux has become one of the most troubling problems at home. He could not ever be left unattended due to the risk of aspiration or drowning on his reflux. The reflux was completely unpredictable other than it got worse with congestion.

Caden receives home therapy and nursing care when it is available and when funds are available to support the care.

Caden was initially discharged from the hospital on New Years Day 2005. He has been hospitalized a few other times due illnesses and surgeries.

We consider this web site to be a ministry tool. My wife Sherry and I share our thoughts, feelings, and lessons learned though our experiences with Caden’s many medical conditions. Our strength comes from God. I have been working on a book that I plan to share with other parents who have children born premature, with life threatening illnesses, or become sick and require special care. My intention is to let people know that they are not alone if they are going through, have gone through, or know someone going through a similar situation with someone they love.

This page was last updated on January 2009.

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