In the Life of a Child

from In the Life of a Child

September 2002

Our daughter was born with a rare birth defect called a lymphatic malformation (LM).

Here is our story…

After years of infertility and multiple miscarriages, my husband and I were just getting used to the idea that we were finally going to be parents. All was going along well until on a 20 week ultrasound we learned that our baby had a large mass on her neck. Opinions on what it was, and whether or not she would survive were mixed. We were absolutely terrified. And we were immediately pressed into the crushing onslaught of critical decisions that needed to be made for her… and the first option presented to us was abortion.

The first perinatologist involved in our pre-natal care suggested among other things, that we should carefully consider all of our options, including abortion. He was of the opinion that an abortion might be in our baby’s best interest, and in ours as well. We vehemently disagreed. I would challenge him to make the same suggestion today. A quick look through this site gives you a little glimpse of what the world would have lost had we taken his advice. He also stated that if we were to lose “it” (our baby) through miscarriage, that it may be for the best and that since we were young, we could always just “have another.” We made the decision to transfer the care of our baby to another perinatiologist even though there were no other perinatologists in our city. Our child is and has always been our child. From the moment of conception, she was ours. We have never considered her life to be expendable. We began making the three-hour drives to appointments with our new perinatologist because we trusted that God had a plan for our baby.

Our daughter was born in 2002 with a large mixed macrocystic/microcystic lymphatic malformation (LM) of the right side of her face and neck. She survived both the pregnancy and an emergency C-Section delivery, but we were just getting started on her journey. The ENT (Head and Neck Surgeon) who assisted in her delivery told us that her best option would be sclerotherapy with a drug in clinical trials known as OK-432 (picibanil). She would be eligible to enter the study after reaching 6 months of age.

At 2 1/2 weeks old, the LM swelled and she began having trouble breathing. We rushed her to the ER and she was transferred to the NICU where they began attempting to intubate her with a breathing tube so she could be put on a respirator. I immediately thought, “No! She doesn’t have enough oxygen reserves to tolerate a PICC line attempt… They need a secure airway first!” But I didn’t say anything. I argued with myself and told myself that they were the experts — that they knew what they were doing. Before they could intubate her, she flat-lined.

Here is what I remember about that day: I remember staring at the monitor in disbelief — at the zeros and horizontal lines while nurses and doctors swarmed over her tiny, blue-gray mottled body. I remember hearing a code blue paged for the NICU and it took a moment before it registered with me that it was for our little girl. For my child. I remember voices of people in the room sounding odd and strangely far away. I remember noticing that the outer edges of my vision were fading to black. I remember hearing my husband calling out our baby’s name, telling her to fight, not to give up. I remember standing there with my knees buckling, mentally shouting at myself that I had just killed our only child because I had failed to speak up. I remember pleading with God for a second chance — a second chance at everything.

I remember her suddenly coughing and taking a ragged shuddering gasp. I remember collapsing into tears as I watched her face flush violently from a surreal blue to a vivid, almost fluorescent pink which rapidly dissipated into an unnaturally stark shade of white.

She survived that awful day and it was years before I was able to forgive myself. I now think of it now as my wake-up call. That moment is what propels me to my feet in instant protest the second I sense a poor decision is being made in one of my children’s medical worlds. If I start to doubt my instincts, I remember that day and that awful shade of blue. Out of that nearly tragic moment of hesitation, I have found the courage within myself to insist on only the best for my children.

After recussitating her, the NICU staff successfully intubated her and transported her by a 2 1/2 hour ambulance ride back to the teaching hospital where she was born. Her ENT told us that an infection had triggered the swelling. The swelling caused a blood vessel to rupture and she bled into the LM. They started antibiotics hoping that the swelling would go down. After two weeks her ENT told us that he felt her best option would be for us to request early admission to the off-label branch of the OK-432 trial out of Iowa. We were warned that her results with OK-432 would not likely be as dramatic as they would have been if we could have waited to enter the study until after she was 6 months old. The reason for this was because OK-432 relies heavily on an individual’s immune system to do a lot of the work. At 1 1/2 months old, our little girl did not have an immune system to speak of, but it sounded like our only real option. Two weeks later she was accepted into the study and received her first treatment. The swelling began to recede after another 2 weeks and a tracheostomy was performed so that we would be able to take her off the ventilator and bring her home. A gastrostomy tube was placed at the same time for her to be fed through.

She came home with a truck-load of medical equipment and supplies. Our living room was a maze of boxes and people I didn’t know holding out clipboards for signatures and shouting instructions to me about how to run various pieces of equipment. We were fortunate enough to have home nursing care, but the first few nurses seemed even more lost and overwhelmed than I was. I parked one of them in front of her crib, told her not to touch anything and instructed her to yell into the monitor if anything was needed. I ran downstairs and succumbed to a mini-meltdown. I set a timer for 10 minutes and sobbed violently into a pillow. When the timer went off, I got up, splashed some cold water on my face, and went back upstairs.

Although things eventually became much easier after her trach was reversed when she was one year old, the trach was not her only other medical complication. She was still fed by G-tube and progress in encouraging her to eat by mouth was slow. When she was 3 months old she was diagnosed with severe reflux which later made it necessary for her to have a procedure called a nissen fundoplication in order to stop her constant vomiting. The following year she was diagnosed with cyclic vomiting syndrome (CVS) and classic migraine. It wasn’t until she reached the age of three that we were able to learn that her CVS had three main triggers: Wheat, dairy, and feeding volume. She improved dramatically after placing her on a gluten-free and casein-free diet and limiting her to three small tube feedings a day with a continuous feeding at night at a slow rate. We also learned that there was a possibility that the LM could be contributing to her CVS symptoms by compressing her vagus nerve, triggering migraines and interfering with normal nervous system stimulation of her digestive tract.

After bringing her home, we began a series of OK-432 treatments at 8 week intervals. After a total of five rounds with OK-432, we were told that her response to the drug had been moderate. The results were still significant. We believe OK-432 saved her life and enabled her to be taken off the ventilator. Although her LM was still very large, it was less than half the size that it was before treatment. We were told in March of that year that we had reached the end of what could be done through sclerotherapy and that the only remaining option for her was surgery. We began researching her surgery options and learned of Dr. Milton Waner.

November 2005 — For Surgery in NYC

Dr. Waner’s surgical experience with LMs was so compelling, that we decided to make the cross-country flight to New York for a consult with him. When we met Dr. Waner for the first time, we were impressed by a number of things. It was immediately evident to us that he cares about the children he has treated with intensity. He was kind and intelligent, with a quiet and patient voice. He was surrounded by a completely unexpected air of humility, while at the same time managing to exude confidence and authority. He treated us with respect — sitting down to speak to us, making eye contact, and never glancing at his watch. He willingly answered our questions and was able outline a treatment plan that included and respected our concerns. He was able to clearly articulate his surgical plan and was willing to answer our questions about potential complications in depth.

That November, we took our daughter back to New York for her LM resection surgery. It took just over 8 hours. She came out of surgery looking pale and perfect. My first glimpse of her face took my breath away. I have always thought her an incredibly beautiful little girl, but to finally see with my eyes the cherub-like face I have always seen with my heart — words don’t exist for that kind of experience. My husband and I started brushing away tears and Dr. Waner misunderstood them. His face wrinkled up with concern, “She’s fine — really — I know with the breathing tube and everything how it looks, but she’s doing quite well!” We laughingly continued to brush away happy tears and assured him that our tears were joyful ones.

However, our child being the child who she is, nothing is ever simple. There were some complications with the surgery. We knew there likely would be, so it really didn’t catch us too much by surprise. Her LM was even more extensive than originally thought and it made for some serious challenges for her surgeon. Dr. Waner told us that it was one of the most difficult surgeries that he had ever done and estimated that the mass they removed was about 2 pounds. It was so large that it had pressed all the structures in her neck far left of center — muscles, blood vessels, everything. That made it impossible to guess where everything was ultimately going to end up after things had a chance to settle down for a few months after the surgery. Because of this, they had to leave more skin than they had originally planned on to give things room to return to as close to normal as possible positions in her neck. This resulted in a baggy section of skin under her jaw-line and meant that a follow-up procedure would be necessary to do cosmetic touch-up work. So, just out of the gate we were already looking at a second surgery. Also, all of the fat tissue in her neck had been pushed up into her cheek or over under her chin by the LM with very little fat tissue in her neck or jaw line on the right side. This too would need additional surgery at a later time to position it correctly.

Her facial nerve was drastically displaced by her LM, stretched down below her jawline when it should have extended straight out, parallel with her ear. This created a number of complications, ultimately requiring a nerve graft. During our stay in New York, she had another complication. Her drain, a device that was temporarily placed to route fluid away from her incision line, lost suction and she had to go back to surgery to have it repaired. The suture line became stressed from the extra procedures and some stitches prematurely broke loose requiring another surgery to put them back in place. All of this unfortunately made it impossible for the incision to heal in the nice thin line, as it would have done, had it been able to have been left undisturbed. Another surgery would be necessary to revise the scar.

Then there were breathing complications. She had to have a temporary trach put in which, incidentally, didn’t slow her down at all. In fact, she decided she was done with having a trach several months early at about 3 am on Christmas Eve morning. She pulled it out herself. She did such a very fine job of it that we were not able to put it back in place. So, after being scared out of our wits, followed by an early morning ambulance ride which she referred to as “allotta fun”, we had the unique joy of spending Christmas Eve day in the hospital. We waited while it was debated whether or not she needed to have her trach put back in place surgically. After several hours of her bouncing-off-the-wall antics, the hospital staff finally conceded that she didn’t appear to be in any eminent danger of respiratory distress. I think they all sighed in relief when the lobby doors closed behind us. My husband later asked her why she pulled her trach out. Her reply? “I had things to do.”

What a nut.

So, while there were complications, the surgery went very well over all. Her LM was successfully removed and we have had no incidence of recurrence, which is very good. Chances are very small now that it will re-generate. Her breathing is worlds better. We really have to listen to hear her now when she is sleeping and she no longer needs a heart-rate monitor for apnea. Her cyclic vomiting syndrome and migraine symptoms appear to have stabilized and she now has an episode once every three to five months instead of once every seven days. Most typically, her CVS episodes are now brought on by stress, illness, or a combination of the two… and once when she sneaked out and ate a whole pack of wheat-containing graham crackers while we were camping. In June of 2007 we were finally able to discontinue her tube feedings altogether because she had begun eating so well on her own. She has regained most of her nerve function in the right side of her face and continues to make improvements in this area.

Quite often, I am asked about whether we considered abortion since we learned of our baby’s condition early in our pregnancy. The answer is a simple and emphatic, “No.” My husband and I believe very firmly that life begins at conception and that from that instant, our baby became a very important member of our family. We would be no more likely to request of our child’s surgeon that he euthanize her today than we would have been to request an abortion then. In our opinion, both scenarios would result in the same outcome and are equally unthinkable.

I believe that God is God and that I am not. I believe that God is the author of life and that I have no right to attempt to define it in a way that the world might consider more convenient. I have been asked if I think that it is cruel to allow a child to be born when it is known that there will be suffering for that child. I believe that this is the wrong question. The question that should be asked is this: “Do you believe that you have the authority to decide what lives should be lived?” The answer to that question is, “No. I do not. I am not God.” Life is filled with different forms of suffering and no human being is exempt from suffering. It is my responsibility to be grateful for the children that God chooses to bless me with and to teach them to meet the inevitable challenges and sufferings of life with grace so that they may lead lives that honor him.

Our brave little girl had follow-up cosmetic surgery in New York in November of 2006. A fat graft from her abdomen was placed beneath her jawline to fill out and balance her face, the excess skin was tightened up and her scars were revised to smooth thin lines. The results were amazing and she is now living the life of a happy, relatively healthy little girl. At her annual check-up with her local ENT, we were told that she was doing great and that there was no need for any more surgery in her near or immediate future, that the recovery of her nerve function has been excellent, and best of all - absolutely no sign of her LM returning. She also just recently was taken completely off tube feedings and is eating all on her own now. We’re really looking forward to just letting her have fun being a kid for a while and are determined to just enjoy this reprieve, even if it ultimately becomes a short one.

April 2008 — Playing Happily in Her Own Backyard

We’re grateful for all of it. Every minute she’s here. When we start feeling like life’s not fair, when we feel like we can’t get through another day, we think of how fortunate we are that she’s here at all. Every breath she takes is a miracle and a blessing to us. Every moment is precious, priceless and to be cherished.

We hope that our story will encourage and inspire others facing similar or even worse challenges than we have. Our hearts go out to each parent who has lived through the pain and shattered hopes that go hand-in-hand with raising such a child. Our prayers are with you all.