Treating Graves' Disease at CHOP's Pediatric Thyroid Center

Introduction

Chris Pierson (Bonnie’s dad): Our daughter Bonnie is the type of kid-- she's very outgoing. 

Michelle Pierson (Bonnie’s mom): She has academic honors, and she prides herself in being an intense athlete. 

Bonnie Pierson: My freshman year, I started getting really, really bad panic attacks and really high anxiety, and I had a lot of problems sleeping. 

Michelle Pierson: Different from the typical teenage-type angst and anxiety because it was the type of thing that wouldn't go away. 

Chris Pierson: She did have a history of her thyroid being of concern, but it was always in a monitoring stage, and our PCP was one that thought that at some point, you know, maybe this would become a problem. 

Michelle Pierson: It became an acute issue a year ago. She was starting to have issues with isolated incidents where she passed out, and they weren't sure what the problem was. And then she ended up having Graves' disease. 

Megan Lessig, MSN, CRNP: When a patient is initially told that they have Graves' disease, it can be very nerve-wracking for the patient and the family. 

Goli Mostoufi-Moab, MD: A lot of times the family is not expecting that there actually is an abnormality within the thyroid gland. And so they come to us with the element of surprise and clearly that surprise brings with it anxiety. 

Anne Marie Cahill, MD: They're aware that their thyroid gland or their child's thyroid gland is abnormal. And so the first thing they're thinking about is, is this a bad disease? 

Andrew J. Bauer, MD: Many centers, which provide excellent care for pediatric endocrinology, just don't have the patient population where they have all the resources that are combined in one center to afford the families a multidisciplinary approach to evaluation and treatment. 

N. Scott Adzick, MD: And that's why our multidisciplinary Pediatric Thyroid Center is so important for families such as that because from the word "go," once the diagnosis is made, or once the referral is made, we can sort of envelop that family and that child with all these layers of expertise in a multidisciplinary way to get the optimal care individualized for that child. 

A Unique Center for Graves' Disease

Andrew J. Bauer, MD: The thyroid gland is an endocrine organ, one of the largest endocrine organs. It's in the anterior portion of our neck. 

N. Scott Adzick, MD: It's shaped like a butterfly with two big wings, and then the middle part which is like the body. It rests right on the windpipe, just below the voice box. It's sort of the endocrinologic storehouse for thyroid hormone. 

Goli Mostoufi-Moab, MD: Depending on where you are in life, thyroid hormone plays important roles. So, for example, for a newborn baby up to 2 years of age, the thyroid hormone is actually very important for brain development and myelination or the completion of nerve development. And then subsequently in childhood, until you complete puberty, thyroid hormone is actually very important for growth. 

Andrew J. Bauer, MD: So when it's working wonderfully, like many things, it's something no one really considers. 

Andrew J. Bauer, MD: So when a family comes to the Thyroid Center at The Children's Hospital of Philadelphia, they come with various reasons and various diagnoses. It can be something like hypothyroidism. 

Goli Mostoufi-Moab, MD: --an underactive thyroid gland, so it's not capable of producing appropriate thyroid hormone. 

Andrew J. Bauer, MD: Could be Graves' disease or hyperthyroidism--

Goli Mostoufi-Moab, MD: --an overacting thyroid gland --

Andrew J. Bauer, MD: -- to something that involves no symptoms, something like a thyroid nodule. Oftentimes the families are told about this as an incidental finding, either on physical exam that someone noticed a lump or a bump in the front part of the neck where the thyroid is, or they had a radiology imaging procedure done, and all of a sudden someone is telling them that their otherwise well-appearing son or daughter has this nodule that needs further evaluation. 

N. Scott Adzick, MD: Thyroid disease in children is unusual. 

Andrew J. Bauer, MD: These diseases are more common in adults than they are in kids. And oftentimes what happens is the kids will be referred to an adult endocrinologist, and they get part of their evaluation in the adult endocrinology world, and they may get their surgery by an adult surgeon. But it's not in a pediatric environment, and so it's not attuned to the anxiety that parents have of their kids undergoing medical care. And it's not in a situation where, if something happens, that they have the pediatric support services to take care of it. 

N. Scott Adzick, MD: We have the multidisciplinary expertise that can be brought to bear for children with these rare forms of thyroid disease, and we want children to have the same access to that expertise that adult patients with thyroid disease do. 

Andrew J. Bauer, MD: So for those reasons and other reasons, the Thyroid Center at Children's Hospital was created. 

Pediatric Thyroid Center at CHOP: Evaluation of Patients With Graves' Disease

Megan Lessig, MSN, CRNP: When a patient is initially seen in the Thyroid Center, there are different disciplines within CHOP that they could potentially be referred to. 

Andrew J. Bauer, MD: What we try to do in the process when they come in is go over what's been done, see what questions that they have, and then try to educate them on what these things could mean and what the next step is in the evaluation process. When patients are referred to our center that have Graves' disease or hyperthyroidism, some of them have already been on therapy, and some of them may be in pretreatment. 

Megan Lessig, MSN, CRNP: Oftentimes the patients with Graves' disease that we see in the Thyroid Center have failed medical management. 

Andrew J. Bauer, MD: They're into therapy for a year or two, and they're still not able to come off the medicine. The evaluation process at that point really involves just looking back and seeing what's been done over that time frame. So if someone's been on an antithyroid medication for a long period of time, there are certain predictors that they're not going to be able to come off of that medication. That will then lend us to suggest to the family two different definitive treatment options: surgery or radioactive iodine ablation. 

Megan Lessig, MSN, CRNP: We provide them with information on both definitive treatment options, both the surgery and the radioactive iodine, in efforts to allow them to make an appropriate care that works for both the patient as well as their family. So when they leave that initial appointment, they have a good plan in place with appropriate referrals, as well as the care coordination to provide those referrals, whether it be nuclear medicine for radioactive iodine treatment or surgical resection. 

Thyroid Surgery in Children and Adolescents

Andrew J. Bauer, MD: The thyroid surgery in children doesn't occur very commonly, so you need to go to a place that has a thyroid surgeon that does this frequently. We did find that as a surgeon who is performing thyroid surgeries in children and adolescent at least 30 times a year. 

Chris Pierson (Bonnie’s dad): We decided it best to go with someone who is very specific in performing that type of surgery, and really, if we were going to do that, we had to come here at CHOP. 

Bonnie Pierson: I was really scared at first, but then when I met Dr. Adzick, and he sat me down and talked about everything, I felt more comfortable knowing that my life was going to be in his hands. And it was all basically the doctors themselves, meeting them helped a lot. 

N. Scott Adzick, MD: There are many different approaches to patients with thyroid disease who need surgery. For instance, there are those patients with Graves' disease, with large goiters who, for whatever reason, need an operation, usually because they can't be controlled well medically, and those children require a total thyroidectomy. 

Andrew J. Bauer, MD: So we remove 98 percent of the thyroid gland, 99 percent of the thyroid gland with the hopes that we make the patient hypothyroid. We want to remove as much of the thyroid gland as safely possible so that they need to be put on thyroid hormone replacement. 

Staff anesthesiologist: Hi, good morning I'm from anesthesia, how are you? 

Megan Lessig, MSN, CRNP: The anesthesiology team is very crucial before surgery, as well as during surgery. Calming the patient's fears, explaining everything so that they really understand what's going on, as well as providing the appropriate anesthesia during the surgical procedure. 

N. Scott Adzick, MD: Once the patient is anesthetized, the patient is positioned at an angle, as far as your torso goes, and your head is back, and your neck is extended. And the wound that's made for the operation is a sideways wound because that allows good exposure of the thyroid gland, and it also follows the skin lines in the neck to give the most cosmetically appealing healing process. And then there are strap muscles in front of the thyroid gland, and we push those muscles aside to expose the butterfly. In the course of doing the operation and removing either one side of the butterfly or the entire butterfly, it's important that I find and protect certain structures. First of all, two nerves, they're called the recurrent laryngeal nerves. There's one on each side, and they run right in the groove between the windpipe, which is called the trachea, and the esophagus, which is food tube behind. And those nerves come up from the chest and they run up to where the vocal cords are and the voice box, which is just above the thyroid gland. And those nerves are about the size, depending on the age of the patient, of an angel hair pasta strand, and they're very delicate. And, frequently, it will abut the thyroid gland, so we need to peel the thyroid gland off of those nerves, one or both sides. 

Goli Mostoufi-Moab, MD: Those nerves are nerves that actually control the voice box. And so, if you have damage on both sides, then a patient will have significant breathing problems to the point that they would need a tracheostomy. Or if the damage is not to both sides or a problem with the nerve, sometimes it can be permanent enough where it causes hoarseness. 

Andrew J. Bauer, MD: That occurs very infrequently, but when it does occur, it's a very debilitating process, and it's something, again, that should increase someone's concern of who they're sending the patient to as far as surgery because that really needs to be avoided in the best interest of long-term care for that patient. 

N. Scott Adzick, MD: The second important structures are parathyroid glands. And they are usually four in number. Two are on the top. Two are at the bottom. There can be anatomic variability as far as where they are. They frequently share blood supply with the thyroid gland. And they sometimes need to be peeled off the thyroid gland. For surgeons who operate on children with thyroid disease, it's important to have a sort of hand-in-glove relationship with the pediatric pathologist. 

Bruce R. Pawel, MD: Sometimes we will receive parathyroid glands or tissue that is suspicious for being parathyroid, and we can do an intraoperative consultation, a frozen section, to determine whether or not that's actually what they are, or whether they're thyroid or lymph nodes or connective tissue. 

N. Scott Adzick, MD: Once we're finished with the thyroid portion of the operation, those muscles in the front are put back together. And then it's very important to close the wound in layers using absorbable sutures that will dissolve once the child heals, so really the only thing that's on the wound are little Steri-Strips running sideways along the wound. And thyroid wounds, for the most part, heal beautifully. Initially, they may be a little red and a little raised and a little firm, but that's normal because that's the healing ridge that occurs with any wound. Long term, in six to nine months, it would be a very unusual circumstance in which there wasn't a very soft, flat, fine scar that might be barely noticeable.

The Days After Thyroid Surgery

Andrew J. Bauer, MD: When a patient undergoes thyroid surgery, the recovery part of that is actually pretty quick, maybe two or three days in the hospital. 

N. Scott Adzick, MD: They're monitored postoperatively to make certain that rare complications don't occur, such as bleeding or infection. We want to make certain that their pain management is good and that they can eat and drink. 

Andrew J. Bauer, MD: We're watching to ensure that the calcium does not decrease because the parathyroid glands are attached to the back of the thyroid. Those are the endocrine glands in our body that make parathyroid hormone, which is the hormone that controls calcium metabolism. And when the thyroid is removed, there can be a transient period of time when the parathyroid glands don't work as well, and we have to give calcium supplementation and vitamin D supplementation. Or it can be a permanent thing. So depending on the extent of the surgery depends on the risk for calcium issues, and that will also help determine how long the family and the patient will have to stay in the hospital. 

Radioactive Iodine Ablation to Treat Thyroid Disease

N. Scott Adzick, MD: For some conditions of the thyroid, there are really two approaches. You can surgically remove the entire thyroid gland, or you can treat it with radioactive iodine to basically ablate the thyroid tissue. The thyroid is unique in the body in that it takes up iodine or iodine-related compounds specifically. 

Andrew J. Bauer, MD: So radioactive iodine ablation, or radioactive iodine treatment, takes advantage of a normal process where the thyroid cells, whether they're thyroid cancer or normal thyroid cells or even Graves' cells, usually take iodine to make thyroid hormone, but this time we give the patient radioactive iodine to destroy the thyroid gland. 

Megan Lessig, MSN, CRNP: In a patient with Graves' disease, radioactive iodine treatment is more of a definitive therapy for that patient, and at a lower dose can be used in this manner, to destroy the thyroid cells from producing the excess thyroid hormone. 

Andrew J. Bauer, MD: We're trying to get rid of the entire thyroid gland. So if you use radioactive iodine, you're trying to give a big enough dose that you cause scarring down under the thyroid gland so you get rid of the Graves' disease. For radioactive iodine ablation, oftentimes we restrict it to children that are over age 5 and many times actually over age 10, if we're going to consider them for that type of therapy, compared to either staying on medical therapy or surgical intervention. 

Follow-up After Thyroid Surgery or Ablation

Andrew J. Bauer, MD: The follow-up that occurs after surgical removal of the thyroid gland for Graves' disease is actually a little less complicated than giving radioactive iodine. They can go on thyroid hormone replacement sooner. Thyroid hormone, compared to the medicines we use to treat Graves', is a much simpler prescribing schedule. It's at once-a-day medicine. It's a very stable medicine, and we may only need to follow thyroid functions every three to six months and eventually maybe even only one or two times a year, depending on the age of the patient. For a Graves' disease patient that's undergone ablation, usually it takes about four to six months after giving radioactive iodine to see that you've given enough of it to get rid of the thyroid gland. So the follow-up during that three to six months after giving radioactive iodine to a Graves' disease patient may be more frequent than every three months. It might be labs every couple weeks or at least labs every month to try to follow how effective that treatment dose was. 

It Takes a Team

N. Scott Adzick, MD: The expertise that's available here in the Pediatric Thyroid Center at CHOP is multiple, multiple layers, which I think lead to special care and special results. 

Megan Lessig, MSN, CRNP: We all work very closely together to make sure that we really come together as a team and give the best care that the patient really requires. 

Bruce R. Pawel, MD: The disciplines include surgery and the surgeons who actually remove the tissue. 

Anne Marie Cahill, MD: We have endocrinologists who are world famous in what they do in terms of the own endocrine glands. Coupled with that, we have a very good collaboration between endocrine and oncology. 

Bruce R. Pawel, MD: Radiology --

N. Scott Adzick, MD: Every single operation is done with the pediatric anesthesiologist. 

Bruce R. Pawel, MD: -- and the pathology as well. 

Megan Lessig, MSN, CRNP: Nursing is a very important component between disciplines. 

Andrew J. Bauer, MD: It's kind of a single point of contact. 

Goli Mostoufi-Moab, MD: In terms of getting all the information needed to have the patient evaluated in the Thyroid Center. 

Megan Lessig, MSN, CRNP: Providing the patient with the education and the care coordination to facilitate the more difficult medical system.

N. Scott Adzick, MD: We have patients who are referred locally, regionally, nationally and even internationally, who want that sort of expertise for their children, but coming from such a distance leads to special needs for lodging and advice and financial support in some cases. 

Megan Lessig, MSN, CRNP: We're fortunate in this center to have a social worker involved in the care of these patients which is very important so the patient is not only cared for in terms of what they need medically, but also what they need emotionally. 

N. Scott Adzick, MD: For a child going through this process, we have access to child life support. The child life specialists provide expertise in many of the psychological aspects of going through an operation that a child and a family really need. 

Andrew J. Bauer, MD: One of the advantages of where Children's Hospital of Philadelphia is located is that we're adjacent to the Hospital of the University of Pennsylvania. 

N. Scott Adzick, MD: We work in partnership with them and can cull all their expertise, whether it be in pathology of other aspects of care that we can now apply to children. 

Andrew J. Bauer, MD: And when the children are old enough and they're young adults, because we see kids up to age 23, which aren't really kids, they're young adults, but the transition of care then is easily set in place. 

Megan Lessig, MSN, CRNP: It's very important for the patient and their family to be very involved during the entire process. 

Bridget Ronca (patient): The team was all really helpful in explaining it, getting us through it because we never experienced any of this before. 

Phil Ronca (Bridget’s dad): It would have been a lot more difficult to get through this if we didn't have that Thyroid Center supporting us. 

Andrew J. Bauer, MD: They really need to understand the process. And we encourage them to write down questions, to bring them in, and we go through them. We've gone through lists sometimes as long as 15 to 20 questions long, and that's what we want. We want them to read. We want them to be informed. 

Bonnie Pierson: I did feel like I was part of the team effort. Because it started out off with Dr. Bauer diagnosing me, but then he gave me the options and all the doctors were willing to work with me no matter what route I took. 

Anne Marie Cahill, MD: The family and the patient provide a very important part of that team for us because they know their child best. The child knows themselves best. 

Michelle Pierson (Bonnie’s mom): From the beginning to the end, when you have your first appointment all the way through the surgery, they seem to be very much all in communication and willing to communicate with you and back with your concerns. 

Goli Mostoufi-Moab, MD: Even though there's a certain way to treat a disease, the patient is not a disease. The patient is a patient. 

Bruce R. Pawel, MD: When we look in the microscope, it's not just looking at the piece of tissue. It's really bearing in mind that this is something that comes from an individual. It comes from a child. It comes from a child with a family. 

Andrew J. Bauer, MD: The families inspire me and humble me and educate me and remind me that we don't have all the answers, but finding them is important. 

Thyroid Disease Research

Megan Lessig, MSN, CRNP: The future for these patients is very bright. They already have a very good prognostic read. 

Goli Mostoufi-Moab, MD: One of most important parts that the center can provide is a strong research component. 

Andrew J. Bauer, MD: We want to improve lifelong health of the child, not just cure disease in 10 or 15 years, but what's going to happen to them over their lifetime and research is really the key entry point to figuring that out. 

Bruce R. Pawel, MD: Research is really now focused upon the genetic underpinnings of disease. 

N. Scott Adzick, MD: That is a rigorous approach, but we're trying to understand the sort of secrets of thyroid disease is very important. 

Goli Mostoufi-Moab, MD: These types of endeavors can only happen in a big institution like The Children's Hospital of Philadelphia where it's recognized not only for the kind of clinical care we give, but also at the same time leaders in the field with respect to different areas of research and disciplines. 

Andrew J. Bauer, MD: It's one thing to identify that there's a need. Then it's another to try to do something about it. 

N. Scott Adzick, MD: And you have to have all the links with the research piece and education piece, all these smart folks focusing on this problem to help push the boulder up the hill to enhance our understanding of thyroid disease in children. 

Michelle Pierson (Bonnie’s mom): Bonnie's doing great. Her outlook is fantastic. It's the optimism that we always saw her to have before she got bogged down. 

Chris Pierson (Bonnie’s dad): There was a point where I thought she wants go to medical school. She wants to play college softball. I didn't think she was going to be able to do it because of just the way her life was unraveling at one point. And having seen her after the surgery, it's back to the old Bonnie, and I have no doubt that she can do those things and handle all of it. 

Bonnie Pierson: I'd love to be a pediatric endocrinologist. After working with Dr. Bauer and Dr. Gannon, I told them I want to do for kids what they did for me. They gave me my life back. They changed my life. And I really am interested in the endocrine system and the thyroid especially. So if I could do what they did for me for even one kid, that would just be the best.