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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

Pulmonary Function Testing


Today we headed to Mayo - Rochester for a pulmonary function test with Methacholine challenge.  Evelyn has had random wheezing in her lungs the past six months.  It is not connected to illness and is not directly connected to exercise.  Her PH specialist wanted some pulmonary function testing as he did not see it as connected to her PH.  She was scheduled for a test where she blows into equipment and it measures the speed and amount of air (spirometry breathing test).  Based on her readings they would give her methacholine, with causes airways to constrict, and/or albuterol, which opens up airways that are constricted.  She was nervous, but was a brave girl and completed the testing like a trooper.  Her initial readings were 62% so she was not given methacholine because it was below the percentile they would give it at.  They gave her albuterol and her percentage increased.  Long story short, she has some asthma going on.  Her symptoms are not frequent, fortunately.  So, she will have an albuterol inhaler to use when she feels short of breath or if her lungs are wheezing.  We have the inhaler now for at school or on the go. 

Another topic we discussed is the unknown of what her pulmonary pressures do when exercising.  She has complained about not keeping up with peers and feeling more shortness of breath this past year in phy ed.  There is no easy way to measure her pressures with exercise so at this point we will add the albuterol inhaler and keep on as is. 

The plan is to return for her next echocardiogram the beginning of Jan. 2016 in Rochester, and then we will head back to Denver in June after school is out.  That appointment will have some addition exercise testing and a heart cath. 

The summer has flown by!  Evie is enjoying her horse therapy once a week.  She just loves riding and caring for horses.  She starts the 3rd grade next week.  Her brother, August, will be starting pre-school the week after that. 

Hope you all have a safe and relaxing Labor Day weekend!


4 people hearted this



Sally Chennell
By Sally Chennell
3rd grade already! So glad shedid the horseback riding. My granddaughter loved that, it gave her so much confidence and she loved taking care of "her" horse!
1 person hearted this
Barb Rang
By Barrb Rang
Thanks for the update. Evie really is a brave girl--and smart.
Aunt Barb
1 person hearted this