Dear Mr. Sparks,
I am not much of a blogger, though I do write from my heart from time to time about my life with CDH. It has been quite a ride, and one that while difficult, scary, and heartbreaking. It has also been so filled with hope and joy and I would not trade it for anything in the world. Let me explain.
Just a little over 18 years ago our family was turned upside down. We had recently moved from the bustling Bay Area of California to a tiny town in Idaho of only 400. We had a special life planned, raising our own meat, dairy, having a horse for our 2 (going on 3) boys, real country style living. We fell in love with this little town... believe it or not the name of this town is Sweet, and our days there truly were delightful. Then came the whirlwind that changed our our life, tore down our secure little family, and turned me into the fighting CDH momma bear that I am today.
Sean was diagnosed when I was 33 weeks gestation. I had planned to deliver at a birthing center, about an hour from home, but was immediately told that my son needed to be born at a specialty center the opposite direction, at St Lukes in Boise. I was also told by this same OB that babies die from this. Ok then. Time to learn what I could, pray a LOT, and trust that God knew what He was doing when he allowed us to move here. Funny thing, I had read an article just a few months earlier about CDH, so it was not an unheard of diagnosis, I just never thought that I would be dealing with it myself.
Within a week I had my first appointment with the specialists in Boise who had to remove a great deal of amniotic fluid. That happened twice more over the next couple of weeks, or Sean would have drowned. On January 2nd we met our surgeon, the man who has become, after 18 years and numerous surgeries, very important to our family, and a life saver for our son. He gave us hope to hang on to, things to think about, and such care that we knew this was the right place, despite other, larger facilities having more experience. So St Luke's Children's Hospital was to become our new home away from home in a very short time.
My son was born on January 22, 1997. We had hoped to deliver without a c-section, but because he had difficulty with just the beginning part of labor an emergency section was performed. We knew from reading that we would likely not hear any cry from him. But he did. One weak little cry, but there it was... and it gave me such hope for him. He was whisked away to the NICU, my husband by his side while the OB took care of me. 2 hours later the neonatologist came to talk to my husband and I about what they had learned. Indeed Sean did have CDH, most of his abdominal organs were in his chest, including part of his liver. He was one very sick little boy, and had been placed on the oscillating ventilator. If you have never heard of this vent, it is also called a high frequency ventilator. It can push air in and out at a rate of 300 breaths per minute in order to keep the lungs from deflating. And it sounded awful... like a jack hammer next to the bed. It is a sound I will always hear whenever I go into a NICU, part of my PTSD from our experience. When we saw him, 3 hours after he was born, he had IV lines, wires, the vent, a little yellow ear covering to prevent him from hearing damage... and he was partially paralyzed so he wouldn't fight the treatments and life line supports. Dear sweet little baby boy.
Sean had surgery a week later. I had not been allowed to touch him the entire week since he was born, any noise, touching, anything could cause problems. It would be another week before I could touch him, a total of 22 days before I could hold him for the first time. Those where the longest 3 weeks of my life. The surgery was supposed to take 4-13 hours, with no guarantee he would survive. You see, when they put the organs back where they belong sometimes they don't fit, sometimes the ligaments and tendons don't reach where they are supposed to. Sometimes the anesthesia is too much. It took 5 hours, and our surgeon did such an amazing job. Sean's spleen was wrapped around his spine. His left lung looked like a little piece of chewing gum flapping in the breeze, with the ventilator doing those 300 breaths a minute. Everything was able to be put back, though nothing was exactly where it should have been. He had his appendix removed because it would have been behind his stomach, not someplace you typically look for an appendix!
Sean surprised everyone. We initially had been told that he would be on the vent at least 2 months. Day 22 and he was extubated, and stayed that way. We were told that usually these babies struggle with nursing, often never are able to and need a tube to eat. We indeed had him with a tube down into his stomach for a time, but he came home a nursing baby, with a little supplement through the tube. Sean came home with us just under 2 months from the day he was born true miracle, since he was still supposed to be on the vent! Later we were told that it was thought his case was so severe he would never even come home, and if he did it would be a minimum of 6 months. Glad they never told us or Sean that!
He had to have over 40% of the large and small bowel removed, and was a very sick little boy. We came so very close to losing him then, but still he fought hard and won! AND he again came home a nursing baby, after 7 more weeks in the hospital.
He didn't nurse for long after that, in fact he stopped eating anything within a few months of that time, but I am ever so thankful for the time I did have to nourish my "sweet" baby boy. He also came home with a central line, a permanently placed IV that led to his heart for nutrition. To this day he still receives some nutrition through a central line, having had numerous replacements over the years, several scares when his blood vessels clotted off because of it, and as recently as last year had a serious problem when a replacement went wrong and he ended up with a huge amount of blood in the lining around his heart.
For 6 months after that intestine loss as an infant he had to poop in a pouch. No problem... ok, lots of problems. Especially when it fell off and contaminated that oh so precious central line! Or it popped off because I didnt think about the gas that cucumbers might cause, one of the few times he ate solid food... 3 pop offs in 3 hours. Oh, and he had oxygen going too. So we had to carry him around with the IV bag, the poop pouch, and a tank of oxygen wherever we went. No problem, we had our son home! And at 9 months the poop pouch went away to be replaced with pooping 20-30 times a day for the next 2 years! But hey, we got rid of the oxygen for a few years!
Life went on, a line change here, a gastric tube placed there (a feeding tube directly into his stomach so we could give him nutrition that didnt have to go through his blood stream, since by this time his oral aversion was complete and he ate nothing at all). A surgery to keep him from vomiting at 15 months had to be redone at 2 years. That 2 year mark had us learning about the next hurdles to overcome. Sean was diagnosed with severe progressive scoliosis. Common in CDH kids. But not usually quite so severe as Sean's was. Over the next 3 years he had 5 surgeries to try to prevent it from growing too rapidly, culminating in a spinal fusion at the age of 5. Spinal fusion, complete with rods, screws, and killing the growth plate of the spine so his back could no longer grow beyond what it was... at the age of 5. His curve had progressed to 100 degrees with the hardware, close to 140 without the hardware. The fusion took it down to 70 degrees. Think what a 90 degree turn looks like when you drive your car and turn the corner. Then think what my son's back looks like, forever more.
Two years later the curve was growing again, only this time in a forward direction. Had we not
intervened he would have been paralyzed. This time we had no choice but to leave our wonderful hospital in Idaho and travel to Seattle Washington to find a fantastic orthopedist who could do the research needed (at 2 international conferences) to help our son. He ended up taking out the hardware and placing Sean in what is called Halo traction, where half of his body weight was strung up to hang off of a halo to straiten his spine for 2 months. At the end of that time he re-fused the spine again at 70 degrees, and this time it has held the same curvature now for 10+ years.
While the scoliosis was going on, Sean was also diagnosed with SVC Syndrome (Superior Vena Cava syndrome). That means that he clots easily in his blood vessels. He needed a cardiac cath, and fast when he was 2 1/2 yrs old because his upper body was blue, and the clot was large. It was the first of 16 cardiac caths that he has had over the years, and the first of 5 stents placed. Because of it he often got quite puffy as the blood collected in his upper body, sometimes scarily so. It was also the beginning of our lifelong journey with blood thinners, beginning with baby aspirin and progressing to coumadin and weekly blood tests to be sure his blood isn't so thin he bleeds out, or too thick so he clots. Essentially, a cardiac cath is like an angioplasty, that you think of older adults having when they have too much cholesterol in their blood and need to have vessels ballooned to open them back up. You don't typically think of a 2 yr old needing such invasive work... or a 4 yr old, 7 yr old, 16 yr old. We have had to travel for this problem to other medical centers as well. We have been to Boston Children's Hospital as well as to Denver Children's Hospital, though most of the work was able to be done at home in Idaho.
Sean had other issues along the way, he needed his gall bladder removed when he was 3 which led to a serious infection with critical complications called DIC. We nearly lost him yet again. He spent 2 weeks in the ICU, and another 3 weeks on the regular floor of pediatrics. Finally the surgeon came in and said you can manage him at home as well as we can here, please just go home and take care of him before anything else goes wrong. It meant going home with an anti fungal medication that is typically ONLY given in the hospital that is commonly referred to as "Ampho-terrible" because of the serious complications that can arise. But at least we were home. For a month. Then he had to go back for yet another back surgery.
Following a dental procedure under anesthesia at age 4 he had trouble coming off of the oxygen. It turned out his lungs needed more, and he went back on oxygen at night, and has been every since. At age 5 following his spinal fusion his lungs were not recovering the way we expected, so we had to head to Seattle to see a pulmonologist (one of the top physicians in the country) as well as a sleep specialist. It turned out after further testing that Sean had both central and obstructive apnea, and needed to go on bipap. That was when we were finally convinced that his lungs had not regained full function following his CDH repair, though X-rays often seemed to show that the teeny tiny chewing gum left lung was up to almost full size. When performing various tests, it was discovered that his left lung only provided a very small percentage of his oxygenation, with his right lung providing most of it. On top of that, his right lung had little air filled sacs (alveoli) that were not able to empty, and the bipap machine (a non-invasive ventilator) would help to empty the carbon dioxide that was accumulating and causing trouble in his blood stream.
He fought it at first, but as time went on he told me that he was sleeping SO much better. Every year or two we have had to increase the pressures on the machine, until a few years ago he hit the top pressures allowed on it. Thankfully research has found a better machine, otherwise he would have had to be trached and placed on a more traditional ventilator. This new machine (AVAPS) is able to learn how he breathes, can alter its pressures throughout the night, and in addition to pressures it also can administer an appropriate volume of air into his lungs to be sure he is ventilating properly. He needs more time these days on the machine in order to clear out the CO2 from his his system so he can function well during the day, thankfully he is fine with that, as it gives him more time to read (a passion!).
As with all of our children we had decided to homeschool Sean. For a variety of reasons, to keep him medically healthy and away from the various viruses that tend to run rampant through the schools, to be able to alter schooling schedules as needed for medical appointments, and as we learned over time, to alter his educational style as needed so he could learn in HIS best fashion. We had many ups and downs with schooling, and when we hit a period of time when the struggles seemed to go on and on we had him tested. It turned out he was on the Autistic Spectrum. While we had always felt he had issues including developmental delay, we thought it was due to all of the medical interventions. Learning about the autism brought both sadness and a relief. At least now we had answers. The psychologist who diagnosed him told us that he was doing so much better because of our homeschooling and the ability to change as he needed change than he would have been had he been placed in the public schools.
Then began our journey learning about various interventional therapies for his disabilities, social therapies, occupational, developmental, keeping us busier than we ever had before! But they all proved to be so helpful to Sean. While some of his issues have increased in complexity, others have improved. We know better how to help him, and we know where to go to get help.
Now to the present. We moved to town, to be closer to medical care when Sean was 8. Sean is slowly weaning off of the IV nutrition. We are hopeful that within the year he will be finished with it. He is graduating from High School this year! Given that NO ONE thought he would live to even see the age of 10, it is truly a miracle to be here. His 18th birthday is this month, January, on the 22nd. How amazing is that... to see 18. So many of our CDH babies don't see one month of age, one year, let alone 18 years. For this we are ever so thankful. We have had to have some increases in his AVAPS machine due to worsening of his lung condition. He was recently diagnosed with Pulmonary Hypertension. PH is high blood pressure between the heart and lungs. It is one of the reasons that a high percentage of CDH babies die when they are young. And it is something that often returns as they get older. Sean has had it recur before, but we were always able to alter a medication (blood thinner or oxygen) or increase his ventilator settings which would send it into remission. This time it appears to be here to stay. We have had to add a new medication, and very likely will need more as the PH increases in intensity. There is little to no research on PH in children, though it is a devastating disease. Much of the time PH will be the cause of death within 3-5 years of diagnosis, thankfully we did discover it early and our physicians are staying on top of it so hopefully we will have more time. There are cases now where some (typically adults in their 40-50's) have survived 10 or more years.
To read this history you might think that Sean has had a really rough life. To some, yes, he has. But to Sean, he has had the most amazing life we could possibly give him. He converses at a high school at least, and even a college level on his subjects of interest (animals and medieval history, actually any history from the Civil War and before). He is a photographer, hopes someday to be a videographer and create video series of his favorite animals, spotted hyenas and maybe fossa's or leopard seals. He loves to travel, having had the opportunity to spend 4 months on the road with our family exploring the east coast and the tremendous history of our nation. He reads voraciously, and is even working on writing his own series of books geared to 10-14 year olds, fact based fiction about spiders! Emotionally he is only 6-8 years old due to his delays and the autism, but he has come so very far in his life, we couldn't be more proud.
Mr. Sparks, I hope in reading this story of our son that you have come to see that some of our CDH kids really do have difficult issues that last long beyond infancy. They also deserve whatever help we can give them to support them during their battle, for it truly is a battle they want to win. They have so very much to offer the world, no matter how devastating it may seem, being able to see our children grow up and thrive despite the problems through the years is SO worth it. CHERUBS has been a part of our lives since the beginning. If you can find some way to help our cause we would be forever grateful.
Thank you for reading, thank you for caring.
Heidi, Sean's mom.