About Caden and His Family

Caden's Current Condition

As of July 16, 2005

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. All of the items listed below are related to this primary diagnosis. Caden will recovery from most of the complications listed below during his childhood and will lead a fairly normal life.

Heart

Caden still has an Atrial Septal Defect (ASD) and a bicuspid aortic valve that was not repaired during his surgery at six days old. These defects were present when Caden was born, but the surgeons had to focus on the most life threatening heart problems at that time.

Future heart treatment - The cardiologists are monitoring and will determine when to correct them.

Swallowing

This is Caden's most serious problem today. Caden has a condition called Achalasia that basically means that the upper sphincter of the esophagus does not function properly. Caden still does not swallow anything. Caden can not even swallow his own secretions or saliva. This lack of swallowing causes Caden to get suctioned as much as several times per hour to help keep his saliva and secretions from getting into his lungs. Getting fluid in the lungs (aspiration) can cause pneumonia or even drowning in extreme cases.

Feeding - Caden gets his food through a feeding tube (G-tube) that is surgically inserted into his stomach. The food is a special order formula.

Future swallowing treatment - The swallowing problem can be treated with botox injections into the muscle tissue around the upper sphincter of the esophagus. This will relax the muscles and allow food, saliva, medicines and other things to travel into the esophagus and then into the stomach. Caden is scheduled for the botox treatment on August 3, 2005.

Reflux

Caden's reflux was recently corrected with a Nissen Fundleplication. The reflux was a severe case of projectile nasal reflux. Caden has not experienced reflux since the procedure was performed on June 6, 2005.

Complications of Reflux - Caden still has retching spells. Retching is the act of refluxing without anything coming up. Caden turns red in the face, experiences difficulty breathing, and experiences significant discomfort. This retching is helped best by attaching a syringe to Caden's G-tube and allowing his stomach to vent out. 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.

Immune System

This is one of the most complex issues related to Caden's health. Caden has been diagnosed with a mild to moderate immune deficiency. This basically means that he is more vulnerable to viruses than the average person. Caden recently received his first round of vaccinations. Caden will have blood test performed regularly to monitor his immune system.

Immune System Treatment - There is 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 h4 enough to hold its own.

Calcium Deficiency

Caden was diagnosed with a calcium deficiency 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.

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.

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Current Challenges for Caden's Family

As of July 16, 2005

Nursing : Staffing

The problem is that there are no nurses. The nursing agencies require that a minimum of a LPN care for Caden. That is their requirement, not ours. It is a liability thing with them since Caden requires suctioning and the nurse has to deliver medication and food via his G-tube.

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.

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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 echocardiogram 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 condition. Our strength comes from God. I am currently 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. You can read more about my progress on my personal blog The Land of Ozz.

This page was last updated on July, 16 2005.

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