A call for editors

As I mentioned Monday, I'm drafting a letter to B's new preschool explaining his hands. I think there is a lot of natural curiosity, and I'd rather answer parents' and childrens' questions in an upfront way, rather than one day walk into daycare and have a teacher ask me if Brody has Downs syndrome in front of everyone. (Yes, that really happened, and that was after Brody had been there for a few months. I was shocked that the assistant director would not be more observant, or you know, read Brody's file).

I found the letter on this website, which I was excited to learn about. The draft on the website, however, is a bit condescending in tone. I've tried to eliminate the condescension, and answer some basic questions about why his arms look the way they do.

I could really use your assistance in editing the letter and getting your gut reaction to it if you received it. In the letter, I do not mention Vacterl, because that would require sharing a lot of information about B's medical status that I'm just not willing to give out to parents of other children (the preschool has it, however). Really, please post your comments. I have permitted anonymous comments just for this reason. I want your honest responses. Here is the letter:

March 16, 2009

Dear Friends,

Our son Brody is a new classmate in the 2’s room at xxxx preschool.

We’re writing this letter because many of Brody’s classmates will probably be curious about his arms and hands, and we want to make sure that everyone is comfortable with his limb difference.

Brody was born with bilateral radial club hands: specifically, he was born without the radius bones in both forearms and without thumbs. Thanks to Shriners Hospital, he has had several operations to try to help straighten his arms (which do not like to grow straight), and in January he had the first thumb pollicization surgery, in which his right index finger was moved into thumb position. In April, he will have the same surgery on his left hand. In an effort to satisfy natural curiosity, I'd like to tell you a little bit about Brody.

First of all, this is the way Brody was born. The doctors do not know how or why, but the latest research shows that Brody’s arms were probably growing this way approximately 35 days into the pregnancy. We’ve had luck explaining this easily to kids as, "That's the way Brody was born. His arms didn't grow quite right when he was in his mommy's tummy." Some children hear the physical explanation and go about their business without second thoughts. Sometimes, though, kids might want more details.

We’ve learned that the easiest way to help a child understand something like this is to remind them how we are all different from each other. We have had success talking with kids (Brody’s cousins and other classmates) by pointing out the obvious differences: hair, eyes, glasses, height, skin color, size of feet, etc. The list is endless. It's also a good idea to explain that Brody’s arms are not broken, painful, shameful, sad or frightening. They are simply different.

While Brody’s arms and hands are unique, Brody doesn't consider them “special” and we do not refer to them as such. Sometimes children will also be concerned about how Brody will do certain things with his unique hands. I usually remind them that he does all the same things they do, just in different ways. Brody has never had radius bones and 10 fingers, so he learns to do everything with what he has (and then shows us how he does it).

Once you get to know Brody, it's easy to forget he has unique hands. He adapts easily. He feeds himself, likes drawing and playing catch, loves Play Dough, washes his own hands, and wants to build towers with legos on a daily basis. We are teaching him that he should be proud of his “new thumbs” (as we are) and he will probably show them off if you ask. There are tasks he gets frustrated with but most of the time he takes challenges in stride and manages creatively.

I just wanted to let you know that we welcome conversation about our son and we look forward to getting to know you and your children.

Sincerely,
x

I feel like I'm on candid camera but on the phone.

I am trying to schedule an appointment with the local orthopedic folks at the Denver Children's Hospital.

We don't have a local ortho surgeon, because we go to Shriner's in Salt Lake.

I go to the website. There is a Limb Deformaties Team. Not one of my favorite words, but the page on the website says to call and ask for an appointment for the Tuesday Limb Clinic. I call the number.

The woman who answers has never heard of the Tuesday Limb Clinic. Eventually she says "Oh, ortho is what you need. Please hold."

Then I arrive on the extension of another woman. I repeat I want the Tuesday Limb Clinic.

"What's your referral?" She asks.

"Ah. . . my son has bilateral radial club hands. We go to Shriner's in Salt Lake for the surgeries, but I need someone to splint his hand before the surgery in January."

"No, but who is the orthopedic surgeon at Children's?"

"We don't have one. I just need a splint."

"Ok, well you can't come to the Limb Clinic. You have to get a full workup by the PA before a surgeon will see you to see if (garbled)"

"To see if what? I didn't hear what you said."

"To see if it's a true limb discrepancy."

"My son does not have thumbs and he is missing radius bones in both forearms."

"Is this with his arms or his legs?"

"His. Arms."

"Oh, well then you need the hand clinic."

"Ok. Do you have that number?"

"No. That's me too. Ummm. . . .we have an opening on November 7, at 1:30pm."

This phone call is exactly why I avoided contacting them. Honestly, people who say that socialized medicine is frustrating due to bureaucracy have never actually been truly ill with the current scheme.

On a positive note, we are may be participating in this study by the National Institute of Health, although I just called the number that the intake person said to call, and they hung up on me.

Hmmm . . . . . . .

Then and Now

The first photos of Brody, in the NICU, after we were finally able to hold him. All 3 pounds of him.

God he was little. Everyone said so at the time, but I didn't think so then.

The photo we sent to every single person we knew.

Resting at home, at last, after spending the first 8 weeks of his life in the NICU.

The first time he was casted he was about 3 months old - November 2006

More then and now. . . .

Then, I didn't tell strangers about Vacterl association. When they would ask, I mean. I didn't want to think about it. I would get sick to my stomach when someone would ask me how he was doing, health wise. Especially about how his kidney was doing. I stayed on Paxil an extra few months because I thought he'd need kidney surgery in May 2007.

Now, I tell people about radial club hands, vacterl association, and Brody's upcoming 8th surgery all the time. I even have a carepage for him to talk about his medical issues.

Then, I worried how Brody would hit all his milestones, walking especially. And talking. I actually worried that he didn't have enough words.

Now, having been shown by Brody -- repeatedly -- that he can figure out every single thing he needs to do all by himself, I no longer worry that he will be hindered in any way. The truly astounding part of this journey is that he has taught himself every thing. Sitting up, scooting, eating, using his hands, adapting with casts, adapting with fixators, standing, cruising, walking, stairs . . . the kid literally one day just looked at the stairs and started crawling up them, the whole flight, without either Jeremy or me helping him.

With external fixators on.

Four pins in each arm.

I always remember when I was pregnant with Brody and in the hospital with preeclampsia, that I got the feeling whenever they'd put the heart rate monitor and movement monitor on my stomach, and I'd sit there anxiously watching his heart rate and feeling him kick, that he was saying "Look at what I can do, see? Look at me? I can do it! I'm going to be okay, mama! Look at what I can do!"

The thing is, when I felt he was telling me that, we didn't know anything about Vacterl association or that Brody had any of the medical issues he has. Hell, I didn't even know whether he was a boy or a girl. I called him Zoe Brody, because Zoe was his girl name.

My complaint against my former obgyn

My complaint against Dr. W is two-fold: first, he failed to properly treat my pre-eclampsia and second, he failed to diagnose that my son was suffering from a collection of congenital abnormalities, including the absence of thumbs, radius bones and a kidney, despite several ultrasounds during my pregnancy.

I was a 36 year old woman with a history of recurrent miscarriage before 8 weeks. I began treating with Dr. W in January 2005. At the time, I had had 2 miscarriages. After an additional miscarriage in May 2005, I became pregnant with our son, Brody, in January 2006. I delivered him at 33 weeks gestation, via c-section, on September 1, 2006 due to complications from pre-eclampsia.

I began seeing Dr. W after I transitioned from a reproductive endocrinologist at 12 weeks. Dr. W immediately informed me that I would be induced at around 38 weeks even though, at 12 weeks, there was no medical justification for doing so. In fact, Dr. W did not discuss with me his justification for induction at that time. I later learned from several other Dr. W patients (in the waiting room of his office and in our prenatal education classes) that he regularly schedules inductions regardless of medical necessity.

I began having high blood pressure early in my pregnancy. At around 20 weeks, Dr. W prescribed methyldopa. My pressures kept creeping up, however, even on the medication. Instead of prescribing bed rest, or additional or different medication, after getting a high reading during a visit, Dr. W would instruct me to lay on my left side. Then, while I was laying on my left side, he would take my blood pressure for a second time. The second reading would be within a normal range. However, at times, my blood pressure sitting up would be around 150/100.

Despite my high blood pressure, Dr. W still did not prescribe any additional medication, and he did not tell me to modify any behaviors such as work schedule. He suggested that I be on what he termed as “modified” bed rest which, he explained, would be 2 hours on my left side during the day, and 2 hours on my left side at night. Dr. W knew that I am an attorney and I was working full time in a fairly stressful job.

I had multiple ultrasounds during my pregnancy because of my history of miscarriage. In addition to the comprehensive ultrasound at 20 weeks, I had subsequent ultrasounds every couple of weeks in my second and third trimesters to check for growth and development. Although Dr. W did not attend any of my ultrasounds himself, he did review the results and photos with me during my visits. Dr. W routinely informed me that everything was fine. At no time did Dr. W express any concern regarding the bone or organ development of my baby nor did he recommend amniocentesis. I question now whether Dr. W even reviewed the photos of the ultrasounds.

In August 2006, at around 32 weeks of pregnancy, I went in for a scheduled appointment on a Monday. I had an ultrasound performed by Dr. W’s ultrasound technician. That same day, my blood pressure was 160/110. After the ultrasound, Dr. W indicated that my child had “fallen off the growth chart.” In particular, Dr. W said he was concerned that the baby’s head was measuring very small. He suggested I schedule an appointment with a perinatalogist. He casually mentioned that my fluid was low but did not prescribe any additional treatment or convey to me that there was any cause for concern. At no time prior to this appointment did Dr. W convey to me any cause to be concerned about the development of my baby.

Forty-eight hours later I was seen by a perinatalogist, Dr. S. Dr. S was not at all concerned about the size of my baby’s head, but was gravely concerned about my child’s well-being, my fluid level, and my blood pressure which remained at 160/110. She instructed her staff to bring in a wheelchair to the exam room, and one of her nurses immediately wheeled me into the adjacent Littleton Hospital for admission. Her staff indicated that my blood pressure was at the level at which people experience stroke and that my fluid level, which was presumably the same as it was 48 hours earlier, was dangerously low and was restricting the blood flow to my baby. She prescribed labetalol, which immediately lowered my blood pressure. At a minimum, I believe Dr. W should have more aggressively treated my pre-eclampsia and my high blood pressure or, if he was unable to do so, he should have referred me to someone else sooner. At no time prior to 32 weeks did Dr. W ever suggest I consult a perinatalogist for any reason. Moreover, clearly there are other medications used to treat high blood pressure during pregnancy, such as labetalol, that would have immediately lowered my blood pressure. Had Dr. W appropriately treated my high blood pressure, my fluid and my child’s growth would not have been so severely restricted.

During my hospitalization, Dr. W shifted my care to his partner, Dr. D due to Dr. W's vacation schedule. Although I was in the hospital for nine days before giving birth, Dr. W visited me only once on rounds.

On the 9th day of my hospital stay, in the evening of September 1, 2006, Dr. D delivered my son Brody via c-section. Although my fluid levels had been increasing since I was admitted, the c-section was necessary due to my pre-eclampsia. Brody was born at 3 lbs, 13 ozs and 17 inches long at 33 weeks.

Once Brody was delivered, however, it was immediately apparent that something was seriously wrong. His arms were short and his hands were bent 180 degrees backward. It looked like his palms were glued to the underside of his wrists. I heard the anesthesiologist murmur “Is that because of the low fluid?” to Dr. D, who did not answer. My husband Jeremy, who was holding my hand, began to shake uncontrollably in the OR. The NICU nurses wrapped Brody up, and took him to the NICU.

That night and the next few days, which should have been the happiest in our lives, were among the worst and most terrifying. Our family and friends at the hospital have told me that after Brody was born, my husband fell to the floor in the hallway, sobbing. The neonatalogist informed us that Brody did not have thumbs and was missing the radius bone in both arms. That night in the hospital room, my husband and I wept for hours, not knowing if our son would survive. I had never seen my husband cry before.

The next morning we were told that Brody had tracheo-esophageal fistula, meaning that his esophagus did not connect to his stomach. He had to be transferred to Presbyterian St Luke’s in Denver for emergency surgery. I remained at Littleton until September 4, when I could finally join my husband at my son’s side.

Over the coming days and weeks we learned that in addition to missing radius bones in both arms, missing thumbs, and TE fistula, Brody had only one kidney, and had a fused metopic suture in his skull. His lone kidney is misshapen, and he has a level 4-5 reflux, meaning that not all the urine drains from his bladder. To date, he has had 3 surgeries, and will require several more. Officially, Brody’s diagnosis is Vater, or Vacterl, association.

I’m filing this complaint not because I blame Dr. W for Brody’s congenital issues, but because he failed to diagnose any of them. In fact, Dr. W kept telling me I was measuring perfectly, and that the baby was growing. At no time prior to 32 weeks of pregnancy did Dr. W mention to me that any of the ultrasounds revealed concerns with the development of my baby. And yet, Brody has no thumbs, no radius bones, a missing kidney, a fused skull bone and only a partially grown esophagus. To this day, I cannot understand how Dr. W failed to notice these abnormalities.

Had we known of even some of Brody’s conditions, we would have immediately had an amniocentesis done to confirm that it was not genetic in nature. Instead, we waited agonizing week after agonizing week for the results of the genetic testing to tell us if our son had Fanconi’s anemia, a catastrophic diagnosis that would likely mean mental deficiencies, and increased likelihood of several forms of cancer and a greatly shortened lifespan. Every day I spent the majority of the day in absolute terror that our son had it, as it is also characterized by arm malformations.

Had we known of Brody’s condition, he would have been born at PS/L where they have a more advanced NICU, more experienced surgeons, and doctors who are familiar with Vater/Vacterl association. Instead, my son had to be transferred to PS/L while I was still in Littleton. Instead of being by my newborn son’s side, I was stuck in a hospital 45 minutes away, unable to see him before his surgery, unable to be with my husband and our family during that surgery, and unable to hold my son after his surgery.

I realize that the news of our son’s congenital issues would have been devastating to hear at any point during my pregnancy. But if we had known before the birth, we would have been prepared, emotionally and medically, to handle it and give the best care possible to Brody. We would have had a more positive birth experience, instead of living the nightmare of that weekend and the tortuous uncertainty of the weeks that followed. Even now, 7 months later, and even though I love my son more than anything in this world, the memory of my son’s birth is a heartrending and traumatic memory that has given me countless nightmares and sleepless nights.

Because of Dr. W's abject failure to properly treat my pre-eclampsia and utter failure to detect even the most basic of prenatal abnormalities, the absence of organs and digits, my life was put at risk and more importantly, my child’s life was put at risk. I write this complaint hoping to prevent this situation from occurring to anyone else.