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In Honor of Evelyn

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Evelyn’s Story

Evelyn Hazel Keltgen was born June 23, 2007.  She was diagnosed with primary pulmonary hypertension at the age of 6 weeks and started treatment by the age of 3 months.  Her PH specialist is Dr. Ivy in Denver, CO.

Evelyn is a wonderful girl - smart, active, and full of life! She captures the hearts of all who know her.

Evelyn was born June 23, 2007 and within minutes of birth started to turn grey and not be able to breath. She was diagnosised with bilateral tension pneumothorax and was air-lifted to Children's Hospital in Minneapolis. The flight team arrived a couple hours after her birth and put a chest tube in on the right side to relieve the pressure. The left was not severe enough to require a chest tube. She spent 6 days in the NICU. They gave her a clean bill of health with no long term effects. At the time of discharge her breathing was still rapid (about 80 breaths per minute) and they said it is common with pneumothorax and it should resolve in one to two weeks. Two weeks came and went and the breathing remained rapid. It wasn't until six weeks old that doctors started to become more concerned and she was referred to specialists (pulmonologist first, then cardiologist). She had echocardiograms that showed pulmonary hypertension and enlarged right atrium and ventricle due to the heart having to work hard to pump blood to the lungs. No heart defects were found. She had a CT scan of her chest and no abnormalities were reported. Therefore, she was given the diagnosis of primary pulmonary hypertension (no known cause for the pulmonary hypertension and therefore no way to fix a problem to correct it). In other words, there is no cure and will eventually lead to death. There are some drugs that are used to reduce the pressure and slow the progression of the disease, however, as the condition progresses the only other thing is a lung or lung-heart transplant. If left untreated the right side of the heart will be overworked and fail - cardiac arrest. None of the doctors can give us a time frame on how long she can sustain or her prognosis. None of the doctors have ever come across another case like Evelyn's before. Evelyn's care was initially provided in New York City by the late Dr. Robyn Barst, world-leading specialist of pediatric pulmonary hypertension. In April 2008 we started traveling to Denver, CO to her current PH specialist, Dr. Dunbar Ivy. Her follow-up care between heart catherizations is done in the Mayo Health System in Rochester, MN. We initially flew to NYC every three to four months where Evie had a heart catherization, EKG, CT, echocardiogram, and blood work. We currently travel to Denver once a year with a heart catherization annually.  Read our journal for on-going updates.

Evelyn has responded well to the medications and her pressures are remaining stable with a mild level of pulmonary hypertension.  She is growing well and developing normally.  She enjoys playing the piano, riding horse, riding bike, and learning about all types of animals. 

We have been humbled by people's generosity to us.  Your prayers and support are appreciated beyond words. 

Latest Journal Update

Denver Results

Hi,

Evie had good test results. Her echo looked good and they weren't able to measure the regurgitation so her pressures are probably still near normal. EKG still showed some mild thickening of the right ventricle, but on echo it was within normal limits. 6 minute walk was good with lowest oxygen levels of 91 at the end. She walked about the same distance as she did last year. The distance was about 80% less than the norm for kids her age. She had to have a blood draw so she cried and threw a fit, but stopped crying after the needle was in.


Due to her shortness of breath with activity and occasional wheezing in lungs without illness, she will have some exercise testing at Mayo Rochester to check for exercised induce bronchial spasms. Then next year in Denver they will do another test to look at how the heart and lungs are functioning together with exercise. She will most likely have a heart cath again next year.


They increased her Tadalafil to 30 mg (up from 25 mg) and left the rest of her medications the same. This is to account for growth.


She was very nervous for the appointment this time and has butterflies in her stomach. But she is doing okay. August has been a challenge. For a boy who thrives on running around, sitting in a car for multiple days on end is not good.


Kris and I joined one of the research studies on genetics and pulmonary hypertension. Our blood samples are now connected to hers and they will study them for possible connection to mutations on five or six different genes. We won't get results and the only benefit is that hopefully these research studies will help find a cure. They are also trying to see which medications respond best to the different genes mutations for improved treatment. This would help medication selection be less of a guessing game, like it currently is.


We are on the road heading home.


Thank you for your prayers!

Melissa




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Comments

3 Comments

Lois Fitzpatrick
By Lois
So happy to read this good report. Drive safely!
Nancy Clausen
By Nancy Clausen
Glad to know that you arrived safely and that Evie's tests are done and look as good as they do. I'm sure that Augie doesn't enjoy sitting still for so long, but I'm sure he has enough to do to make the time pass faster, even if he sleeps a bit! Drive safely.
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1 person hearted this
Holly Reichel
By Holly
Prayers will continue for your family! Safe travels home.
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1 person hearted this