Talking treatment options with patients with growth hormone deficiency

Courtney Reints, DNP, ARNP, CPNP-PC, a pediatric nurse practitioner at a Blank Children’s Hospital, a service of UnityPoint Health, in Des Moines, Iowa, talks about the flexibility afforded by weekly growth hormone deficiency (GHD) treatment options and how she counsels patients and families through their GHD journey.

Question:

Can you talk about the care and management of pediatric patients with growth hormone deficiency (GHD)? How do you talk with patients and their families about the condition and potential opportunities for treatment?

Courtney Reints, DNP, ARNP, CPNP-PC:

When patients are coming in, concerns of growth or short stature is definitely something that we see a large number of patients for. It could be that they’re falling off the growth curve. It could be they never established on a growth curve. It could be parents are really tall and my kiddo is really short. Kind of a starting point of why they’re coming in.

Then so kind of reviewing the growth chart and just seeing where do they compare toward their mid parental? What’s their predicted adult height? Does that build a picture for the patient? Then kind of exploring, do we need to get labs? Do we need to get a bone age? Do we need to discuss further? Ensuring that they have good nutrition so we’ve got adequate weight gain to promote good height.

Really just bringing the facts to the table and help setting expectations for families of… I always say, if both parents are 6’5″, I expect a very different child than if both parents are 5 foot. Kind of setting realistic expectations for their child, and then how they are kind of fitting in and growing on their curve.

Question:

There are daily and weekly growth hormone treatments available. Can you talk about both options? How do you counsel patients and their families when it comes to treatment?

Courtney Reints, DNP, ARNP, CPNP-PC:

There are lots of different options out there, which we love. Giving patients the choice to select or have as much selection as possible is ideal. Now, as a lot of us know, insurance dictates our world a lot, so I try and present all the options. Then help the family if we have a lot of needle phobia and injections are going to be a big struggle, then we may talk a little bit more about the weekly options or the daily. But I like to present all of the options because they may select 1 of the weekly formulations because that’s what we’ve decided is the best for that patient, but insurance may not approve that and so we may have to go to our second-best option.

The other thing is we’ve had a lot of shortages and supply concerns in the last couple of years, so a lot of families have had to bounce from 1 to another, and so kind of navigating that and saying they’re all good products. I would rather they have something, maybe it’s not our top choice, but I would rather they are able to continue on growth hormone and not have a major interruption in their therapy. Kind of having that discussion of laying it out all out on the table and we pick the best one for them and see if we can get it, but then also helping them understand and feel comfortable that if we need to utilize a different formulation of it, that it’s going to be okay for their child.

Question:

There are also different treatment administration options for GHD. Can you talk about that?

Courtney Reints, DNP, ARNP, CPNP-PC:

A lot of them are a pen device where you dial it up and you administer the medication. Some of them you must reconstitute as you are taking the cartridge of medication and putting it into the pen device. There is 1 on the market that does the reconstitution for you. It’s an automated system and so you just push buttons and it moves through the steps. Other ones you do as you are turning the device and putting it together. There is some that is used, I would say rare, but it is, that’s still a vile and syringe formulation.

Lots of different options out there. I would say not a huge point that families get stuck on. They kind of find their favorite, especially as we’ve had to switch between numerous different ones over the last couple of years. But I think they all have good options, and I’ve had some kids love certain brands and other kids hate it. I think having the ability to individualize it as much as possible to the patient and what they’re comfortable with or the caregiver who’s administering it, I think is huge. Anytime we can give a patient and the family a choice and they can help make that decision, I think is best.

Question:

What is some of the feedback you hear from patients related to the treatment options?

Courtney Reints, DNP, ARNP, CPNP-PC:

Some of the devices, families just felt like they didn’t like all the steps of having to reconstitute the medication. They wanted to just open it from the box and turn the dial and they were ready to go. Others who, I’ve got a couple kids that are kind of techy and they like all the different video games and things, and so the pen device that reconstitutes it on its own, it lights up and has sound. They found that very cool. Others were like, “I don’t need all that extra.”

Those are probably the most common things. Some families, they felt like this pen leaked more than another pen and they’re like, “That’s expensive medicine that we’re wasting.” Families, they figure out what they like and what they’re most comfortable with, and it seems like nothing can compare to what their favorite one is.

Question:

What feedback have patients and their families provided on treatment and lifestyle management for growth hormone deficiency?

Courtney Reints, DNP, ARNP, CPNP-PC:

Especially looking at it’s a nightly injection, that every night they’ve got to figure out how to do it. When the kids are younger and they’re at home and they’re not doing as many activities, it may be a little bit easier because the parents are more in control of their schedule. But thinking as they get older, if family wants to go on vacation or they go over to grandma and grandpa’s, or they’ve got a football game that night and they want to go hang out at a friend’s house, now when are they going to get their injection in? The fact that now we do have the weekly formulations is nice that they’ve got a little bit more flexibility in their dosing schedule. It doesn’t have to be done at night and it can be done first thing in the morning if that’s when it’s best for the family.

It also can be where you’ve got some swing time on, “My normal injection night is Sunday, but maybe we’re traveling so we’re going to do it a day earlier, a day late. Now I don’t have to pack it and figure out a hotel with a fridge and how I’m going to get it through security, or how I’m going to get a cooler to stay the whole time.” The way we’re moving from less frequent injections, not just from they have to give their child an injection and poke their kid every night, but just the flexibility of, “Now we don’t have to bring it on vacation” or “They can go to summer camp and not have to worry about taking it each night.” Or if you have split households, not forgetting, remembering to send it back and forth. I think moving to less injections, not only is that beneficial because what kid doesn’t want less injections? But also from a flexibility of they can just live their life and once a week they take an injection versus, “Every night we have to do this.”

Question:

How involved are you in patient education as it relates to GHD? What are your best practices and recommendations?

Courtney Reints, DNP, ARNP, CPNP-PC:

When I am bringing up, even from the very beginning of the first couple visits of seeing a patient with concerns of short stature, we’re already starting that discussion of what does it look like of, “How do we treat this? What of our treatment options?” Whether we find out it’s like a constitutional delay and we need to address the puberty aspect, or whether it’s growth hormone deficiency and we need to step in with growth hormone, starting that conversation there.

Then throughout, I just keep building on of, “Then we’re going to move into the stimulation testing, then we’re going to move into the MRI, then we’re going to move into… Now we’ve kind of met all the diagnostic criteria and now we’re a candidate for medication, and here’s all the different ones.” Helping them select the best we can, what’s the best fit for that patient and that family. Discussing they may need this treatment for many years, they may need it into adulthood. Then we touch on that and then bring it up more when they’re transitioning and we’re looking at coming off the medication, but just helping set the seen and set expectations for how this treatment is going to look.

Then our nursing staff supplements by we have a number of different handouts from different organizations that just talk about what are the basics of growth hormone deficiency? What are the different medications? What are the side effects? We’ve got a couple different books also that are more geared toward the child. How does the child now adjust to, “Am I different than everybody else? I have to get shots? Why don’t they have to get shots?” Continuing to provide education, not just to the parents, but also to the child of, “You’re ‘normal’ whatever normal is. You’re a normal kid and you can be happy and healthy and we’re just going to help you be the best kid that you are.”

I always tell them, “Everybody has something, whether it’s asthma or heart disease or diabetes or thyroid, whatever it is. Some of it we can see, some of it we can’t. We all have something, and this might just be your something, but we can do something about it and we can help you hopefully meet your potential with your growth.” Continuing that education of helping them understand what this journey is going to look like, but then also coaching the child, “You can go play soccer, you can be a ‘normal’ kid,” and that’s all we want.