Ask a Pediatrician with Dr. Erika Berman Rosenzweig

Erika Berman RosenzweigApril 22, 2009, Online Q&A Chat


Dr. Rosenzweig answered questions about being or raising a young PHer.

Chat Transcript

Moderator
PHA Medical Services Associate Rebekah Macfie

Dr_Erika_Berman_Rosenzweig(P):
I want to start by welcoming you all to this e-learning event. I am looking forward to an interactive Q&A session. Please feel free to ask all of your questions since chances are if they are important to you, they are also important to the PH community.

Rebekah_Macfie(P):
Hi everyone, my name is Rebekah Macfie, I am a Medical Services Associate here at PHA and will serve as the moderator for this chat. Thanks for logging on tonight!

marina(Q):
Hello, I heard that Tracleer can zap the iron in the body, is this true? I have a 7 yr old on Revatio and Tracleer,

Dr_Erika_Berman_Rosenzweig(A):
Tracleer can effect the hemoglobin level. This is usually mild and should be monitored along with the monthly liver function tests.

stephanievolino(Q):
Hello. My name is Stephanie. My daughter Anna, almost 6, was diagnosed last April. Hi, Dr. Berman. We were wondering what's new in the PH medical world? Any new medications being approved? Known person(s) cured?

Dr_Erika_Berman_Rosenzweig(A):
Hi Stephanie. Thankfully, there is a lot of research now searching for a cure for PH. We are close to having one or two new drugs possibly approved for the treatment of PH this year. These are all treatments to control the PH, and a definitive cure is not available yet, but we are getting closer. We do have some patients who have been able to transition off of Flolan to oral medications when appropriate.

AbbysMom(Q):
What are the differences in side effects between Flolan and Remodulin?

Dr_Erika_Berman_Rosenzweig(A):
That is a great question. It does vary from patient to patient, but some patients on Remodulin report less systemic (generalized) side effects, such as diarrhea, flushing, and foot pain. It is also longer acting, so may offer other benefits, for example if the pump is disrupted (rebound pulmonary hypertension).

MAllen(Q):
I need advice on ways to keep my 18-month-old from tearing off her central line bandage -- she's gotten quite good at it unfortunately!!

Dr_Erika_Berman_Rosenzweig(A):
Yes she has!! Do any of the other parents with young toddlers have advice for preventing pulling at the bandage? I typically have the little ones wear a onesy under the clothing. As they get older a tank top under the shirt may help.

Merle(Q):
With infants how would you know what meds they should be on -- I know many times Flolan is used

Dr_Erika_Berman_Rosenzweig(A):
It really depends on the individual patient. Flolan is often used with infants because of the lack of information/research on some of the other agents in young infants.

mitzgeof1(C):
MAllen, I have been there! Ava wore a onez 24/7. At night time, she slept with a zip up fleece or sometimes even mittens. She was always ripping off her dressing. She will grow out of that phase in time.

SUE_(Q):
Hi Erika. I've seen some posts on the PHA message boards about stem cells and PH...what are your thoughts about the promise of that path?

Dr_Erika_Berman_Rosenzweig(A):
Hi Sue. I believe an official statement on stem cell research is forthcoming from the PHA education committee. I am hopeful that current research will lead to more information about whether this may be a future treatment option. At present we do not know for certain.

camm(Q):
What are a few key ways pediatric patients differ from adult patients? Do these differences warrant pediatric-specific studies?

Dr_Erika_Berman_Rosenzweig(A):
Great question! We know that children are not just miniature adults. Metabolism, response to therapy and disease progression are often different than in adults. I firmly believe that more pediatric specific research is warranted to obtain proper dosing of some of the currently available drugs as well as searching for new/novel ways to treat PH in children.

mitzgeof1(Q):
Hi, I'm Mitzi and my daughter is Ava, 3, who has an ASD and PH. She has been on Flolan for two years and it swtiching to IV Remodulin in June. Is Remodulin "as good" as Flolan?

Dr_Erika_Berman_Rosenzweig(A):
I believe that IV Remodulin is an excellent agent, as is Flolan. As I said earlier, every patient responds differently and a transition is the only way to know for sure whether Ava will respond to the IV Remodulin as well.

Bonnie(Q):
My granddaughter Sarah has been on Flolan for almost 4 years. How is a decision made on whether you add another medication at some point?

Dr_Erika_Berman_Rosenzweig(A):
Serial reassessment is critical including follow up cardiac catheterizations to determine if an oral agent would be of benefit on top of Flolan. Goals of doing this are to try and improve the clinical symptoms and/or add therapy if there is any clinical worsening.

Merle(Q):
Is there a percentage of children out growing PH... or outgrowing the stronger meds used.

Dr_Erika_Berman_Rosenzweig(A):
For the most part, unless PH is due to lung disease of prematurity, patients don't "outgrow" the disease. Sometimes patients do however require additional medications when previous treatment seems to be less effective.

Bonnie(Q):
Michelle (AKA Sarah Smiles) is having some writing issues on her computer, so this question is from her. At Sarah's eye appointment he thought he saw some cloudiness. Has there been any connection with Flolan and eye symptoms?

Dr_Erika_Berman_Rosenzweig(A):
Not specifically with Flolan.

MAllen(Q):
What is the longterm outlook for children with PH -- if there are no other major medical issues, do you think we should assume they have the sames chances of living a long life, as would any other child? Or does PH tend to shorten life span?

Dr_Erika_Berman_Rosenzweig(A):
I think in the current era the outcome for children in general is MUCH better than it was just 10 years ago. With new drugs becoming available, I believe this will continue to improve substantially. Be assured, we won't stop searching until there is a cure.

mitzgeof1(Q):
Do you have patients, or allow patients to draw (blood) off their IV Remodulin line? I know you can't on Flolan, but if Remodulin has a long half life, are you able to disrupt it and draw from the line?

Dr_Erika_Berman_Rosenzweig(A):
I would not recommend blood draws from the central line even with the longer half life. I still worry about bolus phenomenon.

camm(Q):
It is my understanding that breast cancer researchers now view "breast cancer" as a number of different types of tumors requiring different therapies. Are PH researchers similarly viewing PH as a number of related but separate conditions requiring different treatments? Thanks!

Dr_Erika_Berman_Rosenzweig(A):
I think many experts feel that there are different mechanisms that may be critical in different patients. There is research currently exploring pharmocogenetics, (Dr Ray Benza) and trying to determine at the genetic level why one patient responds to one therapy and not another. Ultimately, this may help us determine which treatment is best for an individual patient.

Kevin_J_88(Q):
What is your opinion on Tracleer?? The medicine states that their might cause liver disease.  Do you think that it will be still be a prescription drug for people with primary pulmonary hypertension??

Dr_Erika_Berman_Rosenzweig(A):
Tracleer is also an excellent drug and one of the few that we do have pediatric data on. It can cause abnormalities of the liver function tests which is why we monitor them (by blood draw) each month. In the rare case that there is a problem it is most often reversible and detected by the blood test.

Lois(Q):
My teen pher is very self conscious about skin flushing caused by Flolan. We've tried self tanners and steriod creams (prescibed by her dermatologist) but these have not worked. Do you have any suggestions on how to mask the flushing?

Dr_Erika_Berman_Rosenzweig(A):
It is very difficult to manage aside from adjusting the Flolan dose. I do not know of an agent that would specifically mask the flushing.

stephanievolino(Q):
Anna is complaining of a toothache. Anything specific I should know or tell the dentist? Does she need to take any special medication before she goes?

Dr_Erika_Berman_Rosenzweig(A):
In general, if your child is on coumadin, you should let the dentist know before they do anything other than a routine cleaning. It may need to be held before more invasive work. Also, you need to check with your PH doctor about antibiotics which may be needed before the dental work depending on the type of PH your child has. (for example if there is a heart defect)

Ashrafi(Q):
Dr. Berman: What is the future of Cicletanine, the new medicine for PH? When is it expected to be used in USA?

Dr_Erika_Berman_Rosenzweig(A):
Hi. Cicletanine is currently under clinical investigation in the US as an adult trial. Previously used extensively in Europe for high blood pressure. I anticipate that we are still a couple of years down the road. Just a guess though.

AbbysMom(Q):
Hi Dr. Berman. Abby is here and wants to know how often an exercise tolerance test is typically done (it wasn't her favorite test!).

Dr_Erika_Berman_Rosenzweig(A):
Hi Abby!! I hope it wasn't too bad. I think that as the children reach age 7-8 we do try to get them used to the exercise test as this is one of the best ways to follow their response to therapy as they get older. Typically for an older child, every 3-6 months. If on steady therapy, may be 6-12 months.

martalarta(Q):
Hi Dr. Berman, my son had a liver transplant due to Billiary Atresia and developed HP after TX, it is very mild and has been on Revation since almost 2 years ago. I have read a discussion taht was moderated by you, Dr. Abman, SDr. Ivy and Dr. Haworth were in it. I caought my attention taht in the end Dr. Haworth stated that she would rather prescribe Nifedipine because he had a comparison between one patient on Revatio and the other on Nifedipine and she said that the one on Revatio was a strange kid. What do you think that she meant by strange? and would Nifedipine be harmful for a patient that had a liver transplant?. His GI said that Daniel has a perfect liver no sign of rejection since the TX 2 1/2 years ago.

Dr_Erika_Berman_Rosenzweig(A):
Again, each child is different and surely she wasn't referring to your son. Nifedipine is a good drug for those who are responsive to it. We determine this by cardiac catheterization.

SUE_(Q):
Erika -- even with Dani doing so well on her meds, there are still times that she gets tired more easily than other children or seems to need more sleep or rest. Is there a reason for this ..or is it just one of those things that happens but we can't explain.

Dr_Erika_Berman_Rosenzweig(A):
Another good question. Most patients, even with a good response to therapy do report "good days and bad days". I suspect that that there could be a number of thing that factor into this, from lack of sleep, weather (extreme cold or heat), diet, etc. Most often, we can't pinpoint. I would comment though that if your child is having consistent bad days more than good, it's time to talk to your doctor.

Kevin_J_88(Q):
Why is PH at a higher level risk for women then it is for men?

Dr_Erika_Berman_Rosenzweig(A):
We know that it is more common in women than men but are not sure why. It may have to do with hormone levels, (either protective in men or harmful in women), but not known for sure.

Ashrafi(Q):
What is maximum dose of Flolan someone can tolerate?

Dr_Erika_Berman_Rosenzweig(A):
Flolan is also very patient dependent and what is high dose for one patient, may be low for another based on tolerating the side effects. In general, children require higher doses than adults with PH. An average dose for adults is around 40ng/kg/min and kids about 80ng/kg/min, but doses if tolerated can be as high as 100-200.

Rebekah_Macfie(P):
Hi everyone, so we have about ten more minutes left in the chat. I know some of you have sent in questions that Dr. Rosenzweig has not had the opportunity to answer yet. She is going to answer the questions she has now, but probably will not have time for additional questions. Thank you again for your participation and patience!

MAllen(Q):
Will those new drugs (possibly approved this year) be available for children soon?

Dr_Erika_Berman_Rosenzweig(A):
The trials aren't specifically for children, but once approved for adults, we can work toward toward developing pediatric dosing and use.

Sarah_Smiles(Q):
Hi my name is Michelle and my 6 year old daughter Sarah has been on Flolan for almost 4 yrs. She has accepted the fact that she can no longer swim. Is there any way it will ever become possible even on Flolan?

Dr_Erika_Berman_Rosenzweig(A):
Good question. I know this is a frustration shared by many of my patients on Flolan. Given the risk of infection and pump malfunction, I can't recommend swimming on the Flolan pump. I have heard of some using wetsuits, but this is not my recommendation.

Ashrafi(C):
Thanks Dr. Berman, Thanks all,

Kevin_J_88(Q):
Could Pulmonary Hypertension be passed down by genetics or is it a disease that has no known cause of how it developed?

Dr_Erika_Berman_Rosenzweig(A):
While Idiopathic pulmonary arterial hypertension has no known cause, there is a genetic link in some patients. Roughly 6-7% of patients have additional family members with the condition. In those cases, there is a BMPR2 mutation, that can be tested for. ( a blood test)

jromney(Q):

We're struggling with the decision to do another heart cath after the first (and last) one had some nearly fatal complications. I'm interested in your opinion about risk vs. benefit ratios with heart catheterizations. What are you looking for when you do follow-up heart caths after the initial diagnosis? How does what you learn from that change how you would treat a patient. In a situation where a child has had complications with a previous heart cath, how do the benefits of the procedure outweigh the potential risks?

Dr_Erika_Berman_Rosenzweig(A):
Another excellent question and I'm sure one that runs through all of your minds. At this time, the cardiac catheterization remains the "gold standard" for diagnosis of pulmonary hypertension. It is also critical to perform a follow up catheterization for most patients to determine the response to therapy. Probably more so in children than adults since children are often too young to exercise reliably (another way to monitor response to therapy). In the case of a child who has had complications with previous catheterizations, you absolutely have to weight the risks and benefits, keeping in mind that on the first catherization, the child was proabably not on treatment and at the follow up presumably they are and may respond better.

Rebekah_Macfie(P):
Ok, we're going to have to go ahead and wrap up. Thank you all again for participating. If you still have questions you would like answered check back for other E-Learning events (the next one will be next month on Simple Safety Tips for Summer). You can also send general questions to askadoc@phassociation.org; these questions MIGHT be chosen to be published in Pathlight. Thank you again to Dr. Rosenzweig, and thank you to our sponsor; PHA’s monthly e-Learning Event series is made possible through unrestricted educational grants from Eli Lilly & Company and Pfizer Inc. Transcripts for this event and information about upcoming events will be available at the e-Learning Events page at http://www.phassociation.org/Classroom/

Dr_Erika_Berman_Rosenzweig(P):
Thanks to all for the great questions!!

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