Why we are here? Why is Tristan having surgery?

Many of you know Tristan’s story, but we thought it best to put it in writing so everyone knows the complete history.

It was February 2008 when we found out Becky was pregnant. It brought us much joy as we had been trying for our second child for some time. Along with the excitement, also came the worry of congenital defects. I (Adam) only have one kidney and with Becky having Congenital Contractual Arachnodactyly (CCA), we knew there was a 50% chance of our baby having either or both issues. At the sixth week of gestation, we started having ultrasounds and hoped to rule out any concerns.

It was during one of the many tests and ultrasounds that the doctors were able to begin to diagnose Tristan’s medical conditions. First, the doctor noticed that he had only one (solitaire) kidney, which also explained his single umbilical artery. Second, there were contractures of his knees and elbows, which confirmed that he had CCA. Third, an extra rib and hemi-vertebrae were found, which was something very unexpected. Although, finding all of these things in utero made us anxious, it also made it possible for us to specifically pray for the guidance in how to tend to Tristan’s long-term care.

In the months prior to his birth, we continued to see many medical doctors and planned for the possibilities of having a child with several medical conditions. When Tristan was born on October 7, 2008, he did spend 26 hours in the NICU and had several tests, but surprisingly although small was doing very well. The cardiologist saw him within his first few weeks and happily announced that she could clear him 100% for the minor heart issues found during his time in the NICU. The Ophthalmologist also cleared him after two visits before age one. After a few visits and ultrasounds with the Nephrologist (kidney doctor), the solitaire kidney proved healthy and to-date, he has been cleared until age four for follow-up.

The Physical Medicine/Rehabilitation doctor, who we saw prior to his birth, continues to monitor Tristan for his CCA. Since about one month of age, Tristan has been going to PT/OT once a week. Part of this connective tissue disorder leaves Tristan without the ability to straighten his knees and elbows, but with nearly two years of PT, we have seen significant improvement in Tristan’s ability to straighten his legs. We are now starting to discuss a more in-depth plan for getting his elbows to extend as well. At this time, continued therapy to help curve the affects of the CCA is our plan. CCA can have spontaneous improvement and we pray that he will be able to have full motion of his joints in the near future.

Tristan sees an Orthopedic surgeon for his most threatening condition, a hemi-vertebra (and extra rib.) By definition, the hemi vertebra is “a vertebra that is incompletely developed on one side”. Therefore, it creates a “wedge” in the spine that forces scoliosis. Tristan was born with a 30-degree curvature in the spine, because of this condition. In less than two years, the main curvature has worsened to 81 degrees, while adding two other smaller curvatures to compensate and to keep his head upright. Because of the rapid increased degree in curvature, surgery has become a necessity. Without the surgery to stop the curvature, we run the risk of the spinal column being pinched, which would leave Tristan paralyzed.



3D Composite of Tristan's Spine taken 9/7/2010



The first initial thought was to remove the hemi vertebrae and fuse the surrounding vertebra. This option would have shortened Tristan’s trunk and could have caused organ displacement as he grew. After Tristan’s Orthopedic surgeon reviewed the case more closely, it was determined that the fusion would not create enough stability. So after consulting with many other surgeons across the country, a decision was made to install a Vertical Expandable Prosthetic Titanium Rib (VEPTR).

There are only a handful of surgeons in the U.S. that do the VEPTR surgery, so in June of this year we began praying that God would lead us to the right surgeon. Although there is a surgeon at the UofM Childrens Hospital, which would be geographically convenient, our prayers and research led us to see Dr. Flynn at the Children’s Hospital of Philadelphia (CHOP). Dr. Flynn is the partner of the doctor who invented this procedure, which increased our confidence and if that wasn’t enough we also posed the questions to Tristan’s Orthopedic Surgeon here in Michigan, “If this was your child, and money and distance were not a question, who would you have do the surgery?” His answer was go out of state…see Dr. Flynn. Although everyone’s circumstances are different when faced with a decision of this magnitude, we are comfortable that the Lord has led us to the correct place.

We met Dr. Flynn in early July and we felt God’s peace immediately and knew we had met the doctor that would help our son. Since then we have been going to doctor’s to complete all of the pre-op care (including a complete pulmonary workup.) We have been making plans for the surgery, travel, and all of the post-op care. We leave for Philadelphia on Monday October 18 and Tristan’s surgery is scheduled for early morning on Wednesday October 20.

This first initial surgery will take about five hours. Tristan will need to be in the NICU for two to three days after surgery, and he will then remain in the hospital for another three to five days, assuming there are no complications. Once the VEPTR is attached to Tristan’s rib and vertebra, he will have to return every six months to have adjustment surgeries. This means we are looking at approximately 20-24 surgeries over the next twelve years. Every two to three years, Dr. Flynn will need to install a new VEPTR. The goal is to get Tristan to age twelve or thirteen when his spine will be fully matured and then he will have a final corrective spinal surgery.

It has been a long challenging road, and we know it is just beginning. We are so very humbled by all of the prayers and support we have received already and thank God for placing each of you in our lives. We ask earnestly that you will add Tristan’s name to your daily calendar on the day of his surgery and the days that follow during his recovery. Thanks for joining the blog, and we will continue to update Tristan’s progress during this incredible process.


God Bless You All!
Adam and Rebecca West
Addison & Tristan