Moriah’s Story

Moriah was born by C-section at Via Christi St. Francis Hospital in Wichita, Kansas, on November 6, 1996. The next morning during her checkup, the doctor heard an abnormal heart sound and called in a pediatric cardiologist to consult. Moriah was diagnosed with Tetrology of Fallot, a congenital heart defect and she was immediately transferred to the NICU for observation. We were told that Moriah would need either a heart catheter or surgery within 1-3 months. When Moriah was 4 days old, we were able to take her home. Ten days later at a cardiology checkup, we were informed that she needed surgery as soon as possible, and she was admitted the next day at Wesley Medical Center. Surgeons placed a Blaylock-Tussig shunt in her heart to re-direct blood flow to allow for better blood oxygenation. This procedure bought Moriah some time to gain weight and grow before a repair surgery.

On the same day of her first surgery, Moriah contracted Necrotizingenterocolitus (NEC). The doctors called it “dying of the colon” and explained that her tissue was being attacked by infection. The presenting symptom was the mottling of the skin. There were three possible outcomes of the infection: no damage, minimal damage requiring surgery to remove the dead part and reconnect healthy tissue, or death. Moriah was treated in the PICU with three powerful antibiotics for three weeks. She spent another week on a regular floor and transitioned to discharge. I stayed with her throughout the ordeal, learning as much as I could about her medical care. This included running a Kangaroo pump and inserting putting a nasal gastric tube (NG tube), which I was afraid to do because I didn’t want to hurt her. The nurse simply asked me “Do you want her to eat?” I replied, “yes”. She said, “Then put the tube down.” That was exactly the no-nonsense approach I needed to make me do it. Little did I know I would be learning how to change a tracheostomy tube in a few short years.

Moriah’s father, Travis, visited our baby daily before and after work. He whispered in her ear that she was doing a good job and encouraged her to keep fighting. He nicknamed her “mighty Mo” after the Mighty Missouri. Our family, friends, and church supplied nearly every meal for us during her month-long hospitilization. We are so thankful that there was no long-term damage done by the NEC. Moriah was fed through an NG tube for six months while we worked with therapists to maintain bottle feeding and eventually take in solid foods. Moriah also had physical and occupational therapy for a year to build up muscle tone lost from being immobile in the hospital, and to help her meet her developmental milestones.

During a cardiology checkup at about 3 months of age, chest x-rays showed a curve in Moriah’s spine. The cardiologist explained that congenital heart defects are often paired with another congential defect such as scoliosis. Moriah was referred to a local orthopedic doctor, who measured her thoracic curve at about 30 degrees. We were directed to wait and watch, with appointments every few months to see how the curve would progress. Travis inquired whether there were any type of cast or brace Moriah could wear to hinder the progression, and we were told there was not. The doctors were mainly focusing on resolving Moriah’s heart problems first, and did not want to restrict her chest cavity. We were not given any information on an RVAD measurement, or congential anomalies such as hemivertebrae.

At 7 months of age, Moriah had an MRI. Even then, we were not given any additional information or guidance beyond “wait and watch.” Years later, when we requested all of Moriah’s medical records, we found the report showed she did have at least two hemivertebrae at that time. By the time we had that knowledge, Moriah had undergone so much fusion that it was impossible to tell if the diagnosis had been correct. At the time of the initial diagnosis, my husband and I didn’t even know what questions to ask and we could find nothing on the internet to give us direction. We trusted the orthopedic doctor’s advice: we waited and watched Moriah’s scoliosis get progressively worse.

Moriah underwent her second open heart surgery to repair her Tetrology in April of 1998, at 17 months of age. This was our first surgery away from home and family. Thoracic surgeons at The Children’s Hospital (TCH) in Denver patched a hole to decrease the amount of mixing oxygenated and un-oxygenated blood, and they widened an artery. Moriah was sent home on a low dose of oxygen. We learned how to administer oxygen and monitor her oxygen saturation rate via a “sat” monitor.

By November of 1998, at two years of age, Moriah’s scoliosis measured 90 degrees with rotation and kyphosis. Our orthopedic doctor referred us to an orthopedic surgeon who measured Moriah’s curve at 115 degrees and recommended spinal fusion to slow the progression. We trusted this advice, and Moriah had her first fusion surgery on November 19th, at Via Christi St. Francis. She wore a TLSO brace 9 months following this surgery. We asked the surgeon whether the brace would correct the scoliosis at all before the fusion set. His response was no, the brace was only to protect the fusion as it set over the next 6 months to one year. In August of 1999, we again traveled to Denver for a heart catheter and to check on the results of the Tetrology repair surgery.

Shortly before May 2000, we learned that Moriah’s fusion had not stopped her curve and that it was “crank shafting.” Moriah underwent a second spinal fusion surgery that month. She was now fused anterior and posterior. After this surgery, Moriah experienced difficulty breathing and needed C-pap and oxygen. She stayed in the hospital a little longer than normal, but went home without oxygen. Moriah again wore a TLSO brace for 6 months. Her little brother Declan was born October of 2000.

During the winter of 2001, Moriah’s cardiologist informed us that a leaky valve in her heart had worsened and needed to be replaced. Moriah had become pale, lost energy, and her saturation levels were lower. Because performing the necessary operation during the winter would risk pneumonia,  Moriah was scheduled for surgery in Denver in April. When we arrived at the hospital, her surgery was postponed due to poor lung function. While her valve did need to be replaced, it was her restricted lungs that were causing the low oxygen saturation numbers, according to pulmonologists and TCH. My husband and I had been told by both Moriah’s cardiologist and orthopedic surgeon that Moriah would have “restrictive lung disease.” They explained that this was due to her weakened heart function, and that she would become “winded” more easily. This was the first time we understood that there was a connection between Moriah’s scoliosis and her lung function. Previously, our focus had been on getting her through the numerous surgeries and having as normal a life as possible in between. While in Denver, Moriah had a heart catheter and a stent placed in one of her arteries. Doctors considered whether or not Moriah needed a tracheostomyat that time to help her breathe. At her heart surgeon’s request, we also consulted with a pediatricorthopedic surgeon. We discussed options to address Moriah’s scoliosis, which was threatening the repairs to her heart. Upon dismissal, we were advised to see a pulmonologist as soon as we got home. Ironically, the specialist we were referred to was in the same building, on the same floor, one hall down from Moriah’s cardiologist. Moriah began bi-pap therapy at night to help her exhale fully, expelling more carbon dioxide. Two sleep studies were done to check the bi-pap settings. Moriah also had carbon dioxide level checks periodically.

December 2001 Moriah had the leaky valve in her heart replaced at TCH. She was hospitalized only 8 days and went home on a low dose of oxygen. While we were in Denver this time, her heart surgeon again asked us to consult with the same pediatric orthopedic surgeon. X-rays showed that Moriah’s curve was again moving despite the fusions. The heart surgeon was concerned that Moriah’s progressing scoliosis would cause damage to the repairs done on her heart. He was also confident that, once recovered from the most recent surgery, Moriah’s heart would be strong enough to withstand a major spinal operation. We decided to have Moriah undergo halo traction in a wheelchair in an effort to reduce her curve and take pressure off her heart and lungs. We were told they expected up to a 50% curve reduction.

In May of 2002, surgeons in Denver broke apart some of Moriah’s previous fusions, removed some of her growth plates, and put in new fusion. A halo was applied to her head with 8 pins to distribute the traction weight. This proved to be her most painful surgery experience. Moriah required heavy pain medication to withstand the first two weeks of traction in the wheelchair. It took two weeks to wean Moriah from the ventilator this time. When, after 3 days, her CO2 levels went back to the 70s, we agreed to a tracheostomy surgery. Moriah was not doing well on bi-pap and passing out in her wheelchair traction. She did not have any energy to eat and the NG tube could not be removed.

All totaled, Moriah and I spent a month in Denver this visit. Travis flew home two days after the spinal surgery to resume working, so this was our first hospital stay apart. We had daily cell phone updates. Our son, Declan, was 18 months old at the time, and he stayed with us in Denver for 3 weeks at the Ronald McDonald House. My friend, my sister, and my aunt and young cousin each took week-long shifts, flying out to Denver to babysit Declan. When we knew Moriah was going to have the tracheostomy we decided to send Declan with my aunt and cousin. He was with them for 5 weeks, away from his parents and sister. Every trip to the Denver airport made me cry as others went home and we were still in the hospital. We saw Travis once more before transferring via medical flight to Wesley in Wichita, Kansas: he surprised us by arriving the day of the tracheostomy surgery. Moriah had told me before they took her in that she wished Daddy could be there because he helps her to be brave. When she woke up from an evening nap, there he was, smiling at her.

Moriah spent 5 weeks in the PICU at Wesley. Travis again visited every morning and after work. I slept at Moriah’s beside or in a nearby parent sleep room. Family and friends showered us with food and things to occupy our time. Teen volunteers decorated Moriah’s room like we were having a party, to cheer her up. Moriah recovered slowly as we increased the traction weight. Moriah only weighed 27 pounds, and had never been able to gain much weight on her own. We asked the pediatric intensivist for an appetite enhancing medication, and he recommended Megesterol (Megace), a medication often used for patients who experience appetite and weight loss due to AIDS and cancer. It is also used with individuals who have difficulty gaining weight due to multiple surgeries. Moriah was started on Megace, and resumed physical and occupational therapy to fight against muscle loss while in the wheelchair. She worked to be able to use the restroom out of traction with someone holding tension on the halo.

As a parent of a child with a trach, I learned to change and clean the trach tube. I also learned suctioning, manual chest percussion therapy (CPT), cleaning of the halo pin sites, and Moriah’s physical therapy routine. Before we left Denver, the physical therapists made a video of how to transfer Moriah from her bed to the wheelchair, how to hold tension on the halo while she walked out of traction, and how to hook up the traction line and weights, both in the bed and the wheelchair. I shared this video with the nurses and doctors at Wesley, as they had not worked with this type of traction before. Moriah was discharged from Wesley on a ventilator. Before we were allowed to take her home, in-home nursing staff was set up. My husband and I also had to demonstrate that we were capable of caring for Moriah on our own for a number of hours a day. This included understanding her vent settings, and what to do or who to call if complications arose. We turned one room of our house into a psuedo-hospital room. We isolated the wiring to its own breaker, had a medical bed delivered, set up organized space for supplies. We also had an oxygen concentrator, backup O2 for power outages, an external backup battery for the vent, a portable suction machine, and an O2 sat monitor. Moriah was also sent home with the NG tube, so we had the Kangaroo pump again. Our home nurses were used for pin-site care and trach-care, but were unfamiliar with Moriah’s traction. I had them watch the video and showed them what I had been taught.

The experience of having nurses in our home has been both comforting and stressful. We really have to be aware of having family time. The trach made it necessary for our home to be open to nurses, respiratory therapists, and their case managers. We also, for the first time, had to apply for Medicaid to cover Moriah’s increasing medical expenses. While it is great to have Medicaid pay for what insurance will not, the financial limits the system forced us to follow to retain coverage were unreasonable. Our family was used to being independent, and it was very hard to ask for help. No one, no matter their earnings, can afford the costs of the medical equipment rental and nursing. Yet, without those things in the home, Moriah would have spent her entire traction time in the hospital. Kid-Screen, a case management program offered in Kansas, fought several months to have Moriah placed on the TA (technology assisted) Waiver list. With this classification, Medicaid no longer scrutinized my husband’s wages, and we no longer feared losing our home while trying to earn under their limits.

Moriah was in halo wheelchair traction for a total of 7 months. X-rays were done monthly at Wichita Clinic with a local orthopedic doctor who emailed them to Moriah’s surgeon in Denver. During this time Moriah rode the wheelchair bus to kindergarten. Since the school did not have a full-time nurse to watch over Moriah, one of our home nurses attended school with her. The orthotist who made Moriah’s two previous TLSO braces furnished us with an “off the shelf” neck collar to wear on the bus. He enlarged an opening so her trach and ventilator tubing wouldn’t be disturbed. Moriah remained on the vent and eventually weaned to a Passy-Muir valve during the day. We put her T-bird vent, suction machine, O2 Sat machine, oxygen tank, and medical supply bag in a Radio Flyer wagon and pulled it behind the wheelchair everywhere Moriah went. By December, we were up to 25 lbs. of traction weight and Moriah’s scoliosis had reduced to approximately 60 degrees. She had gained 5 inches in height and 16 pounds in weight.

December 13, 2002, the halo was removed in the surgeon’s clinic in Denver. We had not been advised that this could turn into an overnight procedure involving anesthetic, and we had not planned for that in our trip. The surgeon said it would be alright to remove the halo in the clinic if Moriah could hold still enough. We had also not been told that traction weight should be removed as gradually as it was added, so Moriah went from 25 pounds of traction weight to nothing in a matter of minutes. She was so brave and still as they loosened each pin and pulled them out. When they told her it was safe to move, she lunged into my arms, crying. Although my husband and I asked, a brace was not recommended, nor was physical therapy prescribed. Moriah was in a great deal of pain without the traction support. We obtained orders for Lortab and physical therapy from our family doctor at home.

Just a few months later, in March 2003, we noticed that Moriah’s kyphosis looked worse. X-rays showed her curve had increased to 80 degrees. We contacted her surgeon, who explained that it was a “settling” effect from being out of the traction. We again asked for a brace to stabilize this “settling” and were told Moriah did not need one. By November 2003 Moriah’s scoliotic curve had reached 120 degrees with 120 degrees kyphosis and rotation.

About this time, we learned of the Vertical Expandable Titanium Rib Project (VEPTR) through the Infantile Scoliosis Outreach Program, and initiated contact with the University of Texas Health Sciences Center, in San Antonio, Texas, where the new procedure was being performed. In January of 2004, Moriah was rejected for the VEPTR implants because of the severity of her kyphosis.

February 2004 we took Moriah to Denver for a 3D CT scan. Both her scoliosis and kyphosis measured in excess of 120 degrees–almost immeasurable due to the rotation. The CT scan showed cracks in her fusion at T8 and T11. We later found documentation (referred to earlier) stating that Moriah had hemivertebrae at T9 and T10. After weighing options, we chose to repeat the halo traction process and to have as much of her previous fusion as possible removed. We were told that this should be her last halo in light of scar tissue, etc., and we wanted her spine to be as flexible as possible. The surgery could not be scheduled until May, and the surgeon did not feel her spine could get much worse in the meanwhile.

In May of 2004, Moriah underwent 9 hours of surgery. The surgeons were not able to release as much fusion as they did in the previous surgery. Nor did they have an optimistic prognosis for correction:  possible only to 90 degrees instead of the 60 degrees we were hoping for. Also during this operation, her thoracic surgeon, who was assisting in the approach, noticed two portions of diseased lung tissue, the size of fifty-cent pieces, on the lower lobe of the left lung. He removed the diseased tissue as it was “an infection waiting to happen.” Because Moriah’s spine was so close to her ribs, they cut the ribs apart to gain access to the fusion, and then wired them back together. Thankfully, Moriah experienced no nerve loss in that area. Some of the complications Moriah experienced from this surgery included: pneumonia, UTI, bladder retention, and clonis in her ankles. They put her on a cuffed trach with an inner bubble that blocked all air leaks, preventing her from speaking for over a week. This took a toll on Moriah’s usually cheery disposition.

While at TCH, I initiated discussions with the orthopedic department about constructing a traction walker from a photograph of one used at the Shriners Hospital in Utah. TCH orthopedics were willing to try if a walker could be found, and if the funding were approved for the extra traction tubing needed. The walker would cost $600 and was not in the hospital’s budget. A great friend of ours posted an email on Parent to Parent of Colorado explaining Moriah’s need for a specific type of walker. Within 8 hours of the post, a parent was at the hospital donating the exact walker needed.

Moriah was once again transferred to Wesley via air ambulance to complete her recovery. The walker was not yet complete, and the wheelchair wouldn’t fit on the plane, so we had to leave Denver without them. We were able to get a pediatric wheelchair from a medical supply company. While at Wesley, we waited for her home ventilator and backup vent to be shipped from California. The model we were to use was new to the Midwest. Moriah continued physical therapy and her clonis improved. Two weeks later, when Moriah was discharged from Wesley June 7th, the walker was still being built in Denver. Moriah began to experience weakened leg muscles and her knee locked uncontrollably when she was out of traction, walking to the restroom. Through the efforts of Lynx Collaborative Care Network, the walker was delivered to our home on the July 4th weekend. Lynx paid the travel expenses and hotel so the person who built the walker could deliver it and show us how to use it properly. The walker was the best thing for Moriah. She became stronger, and the clonis, weak leg muscles, and knee locking stopped. Mentally and emotionally, Moriah benefited from being able to walk around most anywhere. She attended second grade with the walker, feeling independent, and more like the other kids. She was also able to participate in more adapted activities in P.E. and at recess.

In addition to their assistance with the walker, Lynx executed the overwhelming task of compiling Moriah’s medical documentation from all the offices and hospitals where she had received care. They also sent an informative and concise update on Moriah’s progress to San Antonio for reassessment for the VEPTR implants: Moriah’s scoliosis had reduced from 120 degrees to 65 degrees and her kyphosis had reduced from 120 degrees to 72 degrees, making her a more viable candidate for VEPTR. The day before Thanksgiving in 2004, we learned that Moriah had been accepted to receive the implants.

February 16, 2005, Moriah received two rib implants on her right side. The Family Medical Leave Act allowed Travis to take 3 weeks without pay from work, and we spent our tax refund to cover our bills so we could all be together in Texas. The implant surgery lasted 4 hours with no complications. Moriah’s recovery was quick due to great pain management, and no postoperative infections or complications. She was hospitalized only 2 weeks. All of the doctors, nurses, and respiratory and physical therapists were wonderful. We will return to San Antonio July 26th for Moriah’s second VEPTR implant surgery. She will have a single VEPTR placed on her left side. They will also move up the anchoring point of one or both of the VEPTRs on her right with the hope of better addressing her kyphosis.

We are so grateful to Doctors Campbell and Smith for inventing the VEPTR device. We would not be this far in Moriah’s treatment without the Infantile Scoliosis Outreach Program and Lynx Collaborative Care Network of Colorado. And we would not be sane without the unending support of our family, friends, church, home nurses, and community. Moriah is excited to be going on to third grade, and will have her same nurse there at her side until her trach can be taken out permanently.

There is too much as yet unknown about infantile scoliosis. It is unacceptable to “wait and watch” children like Moriah get worse. Research is a parent’s best tool in understanding scoliosis and working together with the doctors to chose the best treatment plan for their child. Moriah is a very strong girl and we have made a point of explaining to her, to the best of her understanding, every step taken to pursue her health. We teach her that while “medical stuff” involves a great part of her life, it is not her whole life. She can be and do anything she sets her mind to. It is our hope that, by reading Moriah’s story, you will learn from our mistakes, smile at Moriah’s progress, and be inspired to diligence in seeking the best care for your child. Make your list of regrets as short as possible.

Travis and Shellie Grant at shelliegrant@yahoo.com

Update on Moriah September 2006

Moriah is almost 10 now and enjoying 4th grade.  In July 2005 she had one VEPTR hybrid placed on her left side.  In February 2006 her surgeons re-seated the upper anchor on her right side to better address her kyphosis.  They also switched out the lower anchor at that time converting it into a hybrid.  Moriah now has one hybrid on each side and one cradle to cradle VEPTR on her outer right side.  July 2006 was Moriah’s first expansion only surgery.  It went better than we could have hoped.  She was ready to go home the very next day.  We have had zero complications with Moriah’s VEPTR implants.  She will continue to receive expansions of her implants every six months until she is between the ages of 14-16.

Moriah’s lung health has improved dramatically.  She is now off the ventilator completely.  Moriah has her trach capped 15hrs a day and uses oxygen at night.  Her pulmonologist is so pleased with the improvement that very soon Moriah will be capped 24 hrs a day.  If she has a good winter, we have hope that the trach will come out in the late Spring or early Summer 2007.  Routine bronchoscopies have documented the changes in Moriah’s airway.  It is now strong, not floppy, and her bronchi openings are nearly symmetrical, all due to a decrease in Moriah’s scoliotic curve.  She also recently had a complete heart exam during a catheterization that showed no signs of complications.  We are so excited to be on the other side of all the struggles these last several years.

Update on Moriah April 2007

Moriah had her second, expansion only, surgery Tuesday, March 27th.  Everything continues to go well with her VEPTR implants.  On March 13th, Moriah regained freedom she hasn’t had in 5 years! Her trach being no longer necessary, Moriah took it out herself, under doctor’s supervision at the hospital.  She was able to finish 4th grade without the supervision of a nurse going with her to school.

After 8 weeks the stoma had only partially closed.  An ENT closed it for her surgically May 29th. After 10 years of struggle, Moriah is finally at a maintenance point with her scoliosis, heart, and lung issues.   We will continue to vigilantly monitor her heart, lung and scoliosis as she grows.  We could not have come this far without our ISOP family.  This organization and the families involved are truly a blessing from God!

Update: September 2007

We were in Texas once again in August for expansions.  Everything went perfectly.  Moriah and I were invited to a Q & A session at The University of Texas Health Sciences Center at San Antonio.  Dr. Simmons spoke before the new medical students in their Gross Anatomy class.  Afterwards they asked Moriah and two other panelists questions.  She did a great job!  We put in a good word for ISOP!  Moriah is in the 5th grade this year!  She shocked us all by deciding to play the trumpet.  What a great thing to get her involved with her classmates.  Her doctors are happy about how it will improve her lungs!  It is truly wonderful to see her so healthy.  We’ve waited a long time for this and feel very blessed.