Welcome to our new members, please introduce yourself!

[marc]

Hello. My grandson turned 3 two weeks ago but had to celebrate his birthday in the hospital. It was an extremely difficult time. He spent 10 days in the ICU intubated after a catheterization and echogram determined he had primary PH, cause unknown. He’s now home and returning to his new “normal.” He looks great, is having fun playing with his toys and his little sister, and not complaining yet about the pump. As a grandparent, I feel helpless and have been searching for answers to so many questions. We are just starting this journey and I hope to learn from all of you who have been there and done that. My grandson lives in Salt Lake City and is being looked after by Dr. Domingo and her team at Primary Childrens Hospital.

[jen-cueva]

Hi @marc, my heart aches as I think about how you must feel watching your grandson go through this. He is so tiny that the thought of a pump at 3 is challenging. But you’re doing an excellent job researching and reading about others’ experiences with PH.

I was diagnosed almost 19 years ago as a young adult, and the little ones with PH have always been my heroes and inspire me. They are so resilient and don’t make life much different. It’s incredible as I’ve watched several young PH kids grow into young adults and continue attending college, getting married, etc. If anyone was going to get PH, it’s not fun, but there are so many treatments available and more coming in the new year that sound much more manageable. For example, one is an injection every three weeks that many PH specialists are excited about.

Did the doctors mention if they plan to transition him off the pump later? Of course, we are all different, but we’ve all been in your shoes and can relate somewhat to what you are going through.

Please browse our topics and subforums, like caregivers and pediatric PH. If you have any questions for our members, please don’t hesitate. We look forward to learning how best to support you and your family on this new journey.

Thank you for sharing a bit about why you are here and your grandsoin. May I ask his name? His parents are more than welcome to join , too.

[marc]

Thank you very much, Jen. My grandson’s name is Gunnar. There’s been no mention of transitioning him off the pump except once he turns 18 and can rely on an alternative form of administering the Remodulin. I wish you all the best in your journey.

[jen-cueva]

Hi @marc, I will keep little Gunnar in my thoughts and prayers. Many doctors are transitioning patients off the pump with various treatments now available. I know for pediatrics, they have fewer selections, though, too. Did they add a second treatment with the Remodulin pump?

When I look on the PHA Find a Doc, I only see one pediatric PH specialist in the SLC area, which seems odd. Do you know if his doctor is a true PH specialist? That certainly makes a difference. You may want to schedule an appointment with this center since they specialize in pediatrics with PH. It could be even for a second opinion and see what they suggest for little Gunnar. 🙏
https://phassociation.org/business-directory/?You dosrch=1&q=&wpbdp_view=search&listingfields%5B1%5D=&listingfields%5B11%5D%5Bzip%5D=84130&listingfields%5B11%5D%5Bmode%5D=distance&listingfields%5B11%5D%5Bradius%5D=100&listingfields%5B11%5D%5Bcountry%5D=&listingfields%5B2%5D%5B%5D=131

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[Colleen]

@marc , it’s good to hear you describe Gunnar as happy, playful and compliant with his pump. Honestly, being started on Remodulin at a young age will probably help him deal with it as he gets older. It will be like another limb to him.

My son became symptomatic at 6 and was diagnosed at 8. He was placed on Flolan, similar to Remodulin but a little more complicated. The patient wears a pump 24/7 either way. It seems like many patients who have been on the pump can’t imagine dealing with the injections and vice versa for those who started with the injections.

If you are interested, I wrote a column about our choice to start Cullen on Flolan and if you browse thru my columns I have touched on it several other times as well.

https://pulmonaryhypertensionnews.com/columns/going-with-flo-my-sons-experience-flolan-pump-ph/

I know a former PH family from Utah. Their daughter was seen by the same PH specialist as my son, Dr. Jeffrey Feinstein at Lucile Packard Children’s Hospital, Stanford. Honestly one of the best pediatric PH specialists. If your family is willing to travel he is worth reaching out to.

https://www.stanfordchildrens.org/en/doctor/jeffrey-a-feinstein

[jen-cueva]

Hi @Colleen Steele; thanks so much for sharing your experience with @marc. I knew that you would probably have some words and encouragement and also share your experiences with Cullen with him. I know he, too , will greatly appreciate it.

[marc]

Thank you all again for your words of encouragement and advice. My daughter and son-in-law will make the decisions; I’m just hoping to learn as much as I can in hopes of being able to share knowledge from your experiences.

[marc]

.

[Colleen]

Welcome to all our new members! I’m not happy that you or a loved one has PH, but I am thrilled that you are here. There was a time when PH patients traveled this rough road alone. You will discover much like the rest of us that having this support system helps in more ways than one!

[jen-cueva]

As a loving and supportive grandfather and father, they are fortunate to have you, @marc. It’s always lovely to hear family members wanting to learn more in hopes of helping and better understanding PH.

I hope you are also taking care of yourself. Is your family nearby to help support each other with Gunnar’s PH journey?

[marc]

Thank you. They live in SLC and we’re in FL so there’s quite a distance. However, we FaceTime often and my wife and I visit them every few months.

[jen-cueva]

Hi @marc, that’s awesome that you and your wife can travel from FL to SLC every few months to visit. Is this your only child? Or, do you also have family in FL?

Isn’t technology the best? It’s much better to have FaceTime and other options than just the good old-fashioned telephone service we had before. Geez, I remember when we didn’t have a home phone in our house. It was because my parents built our first home in the woods in rural MS. Geez, those days were tough.

Thanks for sharing about your family and how you and your wife support your Gunnar, grandson, and his parents. 🙏

[marc]

Hello jen-cueva. We have been blessed with two daughters. One lives in SLC (with 2 children and a 3rd on the way); and the other lives in FL (with 2 children), within 10 mins of our home. Nothing can replace face-to-face time together but technology – like FaceTime – certainly is very welcomed to fill the time gaps.

[jen-cueva]

Hi @marc, It’s wonderful that you are blessed with two daughters. That’s a plus that one lives so close by. At least you can have face-to-face interaction with her and those grandkids. Nothing beats that, but we are grateful for technology for those we can’t see as often as we wish.

Thanks for sharing, Marc. It sounds like you have a loving and supportive family.

[red]

I have actually followed this for years, I didn’t realize that I didn’t sign up. I have IPAH for 15 years, Im have been in many studies obver the years. Including what is now Opsumit, since diagnosis, I was on 10mg the whole study. Also was one of the first to get pulmonary arterial denervation in the USA. Also been on Revatio since the beginning and Uptravi for about 6 years.

Keep on fighting to cause grief and be a PIA :))

[jen-cueva]

Hi Red,

Wow, 15 years with PH is long-term, my PHriend. I will celebrate my 19th year in February. Kudos to you for managing your PH with a combination therapy plan.

I’ve also been on Revatio, well, sildenafil first, then brand name since my diagnosis in 2005. I’m also on Upttravi and have been on most of the other oral treatments for some time.

I don’t know anyone else with pulmonary arterial denervation (PADN). What year did you have yours? Like 2015? I may need to post a new topic to ask if anyone else has had one.

Thank you for checking us out through the years and conversing with our outstanding members. Your short bio is helpful, and we appreciate you sharing that with us all.

[red]

Hi Jen

I had my Denervation March 2018 at New York Presbyterian.

[jen-cueva]

Hey there! We’ve got some awesome new members in our forum. Let’s show them some love and make them feel right at home!

Welcome to the PH News forums, @Vicky , @Jojan , @Lizzy , @ViS , @Kate , @Dunta , @Micke , @Newkid68 , @Ande , and @LaceyLouise . Please share a little about yourself and your connection to PH if you haven’t. Here are a few questions to help you get started.

* Are you a patient or caregiver?
* How long ago were you diagnosed with PH?
* What PH medications are you currently on?
* Would you happen to have any questions for our members?

* What is one of your favorite hobbies?

We’re so glad to have you here and can’t wait to hear all about your PH journeys! If you have any questions or want to say hi, please don’t hesitate to contact @Colleen or me. We’re here to be your friendly guides and support you every step of the way!

[jen-cueva]

I am sharing this introduction from our new member, @JenJ. Jen, I’m sorry you had trouble posting. I’m happy to share this.

JenJ says, “Hello all, just joined this group. I live in Perth, Western Australia, Australia. I was diagnosed with Systemic Scleroderma 10 years ago but have had a pretty good run of things for the past decade, I do have Interstitial Lung Disease, PAH and Bronchiectasis. I have generally good exercise tolerance – can’t really do stairs or slopes or cardio but can walk pretty comfortably for a long way. A few months ago even that was becoming difficult. I still work full time as teacher but managed to get time off to go to lots of tests and appointments to figure out what was happening. I was on Opsumit and Sildenafil but have recently been added Selexipag. My dose is 800mcg at the moment. Still getting some headaches and sometimes migraines but hope these settle down when I go back to work in a few weeks. We are on our summer break. I have incredible specialists so I am in great hands. I think this might be my final year of full time work though. Take care every one.”

Please help me welcome JenJ to our close-knit PHamily. It sounds like she keeps busy with teaching while managing ILD, PH, and scleroderma. How do you do it, my PHriend? Do you have a strong support system? We are thrilled you found us and looking forward to learning more about you.

[jen-cueva]

Hi, y’all; I would like to share an introduction for one of our newer members, @LaceyLouise. Lacey is the caregiver to her fiance(Jason), and they have three young kids. It sounds like they have a ton to try to juggle.

Lacey shared some information in her welcome message. I thought of sharing here so others can read it and welcome her to the PHamily!

She shared, “his symptoms started in 2018 diagnosed in 2020. So we’ve definitely been living with this for a long while now. letairis yes since his bday 5/10 of this year. his Dr said there was a slight improvement but they were concerned because although there’s progress it isn’t nearly what they had hoped it would have helped. His symptoms are still there however he’s able to walk longer distances and bend over without an episode which is more than we can say for life prior to the letairis. yes kiddos. super hard. especially when they don’t understand why we can’t go skiing or snowboarding in the mountains on a cold winter day in UTAH! thr elevation is a constant issue.now the cold..or the hot.. it doesn’t matter much it’s all bad. but his legs just went purple/red and shiny (from the tightness) and I assume due to the temp.”

“he is on sildenifil, losartan hcl, and spironolactone.”

“we went to spokane washington last Christmas tp visit family (by plane), no oxygen or anything. he had just had an episode the day we left. literally fell off the toilet as we were about to head to the airport. I was soo sick with morning sickness I could barely get out of bed to pack but had no choice. he scrapped any.shred of evidence of skin off his knees from the fall and we should’ve gone to the hospital instead.of flying but we had non refundable tickets and this wad actually already a reschedule from the Christmas before due to his health. so he didn’t want to disappoint thr kids or my family in spokane. we went and left 45 mins before the plane was to take off but by the grace of God our plane was delayed 2 times allowing is to make it. we had to find parking, they had no wheel chairs for Jason or anyone to push him even if they had anyone to do it.

i had 2 sick and coughing kids as we sped walked through the airport. halfway through the slc airport (huge cluster Frick of an aiport) I saw an empty wheel chair and snagged it and pushed hi through the other half while I carried 3 luggage because the whole family was sick. Jason’s knees couldn’t even bend while sitting. I felt so horrible. anyways the trip was hard. because he had such an episode prior to leaving we can’t know how the elevation would’ve effected him one way or another bit he said he felt bad and weird when we were there (all he did was sleep, although so did I due to morning sickness) but yeah it sucked. I had hoped that the elevation would help him and that’d give us an option for him to get better. he said when we got home he felt an instant relief lime his lungs had adjusted better our elevation at this point. I dunno.”

Welcome aboard, Lacey! We are thrilled you found us and happy to help support you as you try and help your fiance while caring for those little ones, too. Do you have a close friend or family you can count on for support? You have to take care of your health, mental and physical, too. Thanks for sharing and I am adding this hgere so others will have see it, too.