Fast forward to November 2014. Willa's PH was being controlled and pressures lowered with the sildenafil. However, her November appointment with her cardiologist showed that her pressures had doubled from her previous appointment 6 months prior. Willa was upped to the highest dosage of her sildenafil and we were referred on to a pediatric pulmonary hypertension specialist at the Children's Hospital in Denver.
They scheduled Willa for a heart cath. A heart cath is the only way to get an accurate measurement of the pressures inside the heart. An echocardiogram gets a good estimate but it is just an estimate. Pediatric PH is very, very rare. Willa has what is called idiopathic PH, meaning, there is no reason for her having the PH. Some kids have other heart or lung problems that cause PH but Willa has no other issues thankfully. The doctors think that her PH was caused by prematurity and her lungs being underdeveloped. We all hope as she grows she will outgrow this. Until then, we will continue to see her cardiologist in Wichita every 3 months and see the doctors in Denver every 6-12 months. The good news we did get from her heart cath is that her pressures have gone down since her heart cath done in 2012.
I mentioned that PH in children is very rare. It is so rare that there are no FDA approved drugs to treat children with PH. Willa currently is on adult medication. I have to cut pills into 1/4 sized pieces to give her. Her medications are very high powered drugs. This new medication she is on is processed through the liver so she has to have a test every month before we refill the prescription to be sure her liver enzymes are not too high. I am so grateful to have good health insurance. My EOB statement shows this drug costs over $8,000 before insurance takes care of it! Yikes!!
The doctor Willa sees is one of the best in the country. He and a small group of other doctors specializing in PH are doing ongoing research to help find the right drugs to treat PH and hopefully find a cure. There is currently no cure for PH. There are only drugs that help treat the symptoms and help prevent further progress.
We are so fortunate that Willa is (for the most part) asymptomatic. Someone with PH will get winded easily, may have their lips turn blue, and have fainting spells. She does get pale if she gets going too fast or if she is really tired from a long day.
She did so great with all the appointments in Denver and her heart cath. She never cried, threw a fit, or got scared. She was so incredibly brave!
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| She found a purple cow - "Look its purple like K-State!" she said. |
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| We knew she was feeling better after the heart cath when she started singing along to Frozen. |
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| Such a trooper! |
To learn more about PH you can visit the PH Association website. PH is really hard to explain so this website has some good information on how the lungs and the heart work together. We really appreciated everyone's thoughts and prayers for Willa as she went though the testing. We are blessed to have such great supporters for Willa.
