Bless Madalinn's Heart

  It has been awhile since our last update.  Here area a few highlights we'd like to share. - Madalinn is 3 weeks into her 6 weeks of pulmonary cardiac rehab.  She goes on M/W for multiple kinds of therapies (Occupational, Recreational, Exercise, Nutrition, and massage). I have been SO impressed at her abilities to move her body and have endurance.  Her heart rate and oxygen are monitored while she works out and her stats have been very good.  She's done treadmill and stationary bike, weight training, circuit training, yoga, and other varieties of exercises and activities.  She is able to exercise for 2 hours (with short breaks throughout) like a champ!   To put this into perspective, before diagnosis she wasn't able to walk around the block without stopping because she felt like she was going to pass out.  It is obvious that the medications are making improvements. - She saw ENT Dr. Willett, whom Josh's family goes to for HHT nosebleed treatment....

 We hope you all had a very nice Christmas.  We traveled to Auburn, Indiana to visit Chloe's family Christmas weekend.  It was such a lovely time!  Josh and I performed Behold the Lamb of God (album) with my sister and her husband for their Christmas Eve service.  It was fun to share God's story through song with everyone and give them a peek into our yearly family Christmas tradition that we have done for the past 10 years in a living room.  As this year draws to an end, we are continually thankful for the vast amount of people who have prayed, given (food, money, childcare), and cared for us so well.  In the midst of such trial we were so thankful to be surrounded by so many people from different parts of our lives.  It was amazing to see the Church (capital C) work together so well.  This month I started monthly Spiritual Direction.  Basically, I meet with a Spiritual Director who helps me listen to what God wants to teach me.  S...

  Yesterday we had our first post hospital check up with the cardiologist.  It was a big day that started bright and early.  She had an echocardiogram, EKG, 6 minute walk test, lab work, and a 24 hr halter monitor. The great news is that her Echo showed signs of improvement in her heart which is a direct result of her medications working!  Thank you for praying for that.  Her heart is still seriously sick, but things are moving in the right directions!  Praise to our God who heals! She did great during her 6 minute walk test.  She walked more steps with fewer symptoms! She didn't have to stop at all and her heart rate only got up to 120.  When she was the in the hospital going from her bed to the bathroom made her heart rate soar to 145, so 120 after six minutes of walking is SUPER!   We knew she was going to rock this test because we have seen a difference in her endurance in normal daily living.  She can go to the grocery store or for ...

This week the boys went in for their Echocardiograms and EKGs.  They were excited to get to experience a little of what Madalinn has done.  It was an early 7:15 visit and they did amazing.  The great news is that neither of them have any signs of Pulmonary hypertension!   Soon we will be getting gene testing kits in the mail for the boys to be able to submit spit samples.  It will be about a month before we get those results back.  The test will be specifically looking for the gene that causes HHT. Speaking of genes, we had an appointment with the genetics person on Tuesday.  She mainly knew of this gene as it relates to HHT.  I'm looking forward to speaking with a cardiac genetics person about how it relates to PH.  What we do know is that severe forms of HHT can cause Pulmonary hypertension because of causing too much blood flow to the heart. This happens is about 13% of people with HHT.  That is not what is happening with Madalinn. ...

As I remember back to the first week in the hospital I realize how much we didn't know.  Doctors were doings CT scans, MRIs, heart catheterization, and so much more to discover what was going on inside her body and what was the root cause of her Pulmonary Hypertension.   At first we thought it was one thing causing it, but after more tests that was ruled out.  At that point they said it was idiopathic (no known cause) but we were still waiting for genetic results to come back.   This week we were informed that Madalinn has the ACVRL1 gene.  This gene causes HHT ( Hereditary Hemorrhagic Telangiectasia) and in some people it also causes  Pulmonary hypertension.  This is the answer of the Why.   Many people remember that I mentioned that some genes are not receptive to medications.  This is what our doctor said, "it is one of the genes that has been found to be not AS responsive to medications.  We will still be  aggressive with her me...

Being home has felt rather uneventful so I haven't felt the urgency to blog about it.  However, I know some of you are faithfully checking for updates.  We are thankful for you and your prayers!   Once home, we quickly got into a rhythm with Madalinn's medications.  Her pills are all very easy and can all be taken in the morning.  The Remodulin pump has to be changed daily (though someday the concentration will change and we will only have to change it every 3 days) so we are getting really comfortable with it.   For those curious for more information about her pump...the site in the back of her upper arm.  The site only changes when her body starts rejecting the site.  So far it's been in the same spot for over two weeks.  We hope it will last at least 4...hopefully more!  Maybe you're wondering, "What does change her pump daily mean?"  It means we fill a syringe cartridge with meds, place it into the pump (think old school pager s...

  Thursday started with Madalinn and us walking from her room to the Blue Jackets room (a hospitality place for families in the hospital) for a CPR class.  Madalinn was excited to take CPR because she hopes to be able to start babysitting in a few years.  She was also excited to be able to walk so far without a nurse escort! Then Lori from CVS specialty pharmacy came to teach us how to administer Madalinn's Remodulin.  This training was 4 hours long.  Our minds were swimming with so much new information. Thursday night Madalinn started going through some preliminary testing for a procedure that she might need in the future if medications don't end up working for her.   Friday was full of excitement and a long to do list so we could go home.  Madalinn went to three tests while Josh and I had a meeting with the pharmacist to go over all of her other medications.  Then Lori came back to finish our Remodulin training and watch us change over Madalinn ...