Anna Grace Hulsebus was born on March 6, 2001 to Clint and Kary Hulsebus.

Shortly after birth, it was determined that Anna had problems with her oxygen saturations and they needed to intubate her immediately.  She was transported to Seattle Children’s Hospital within 7 hours of her birth.  She remained intubated for 48 hours and then they weaned her off of the respirator.  Within the first 48 hours of her life, they did an MRI, multiple echocardiograms of her heart and multiple blood tests but could not find anything significant that was wrong with her.  Her pulmonary pressures were 4 times the normal amount for a baby her age.  At the age of two weeks old, they decided to do another echocardiogram of her heart and found that she had a coarctation of the Aorta.  They transported her back to Children’s Hospital immediately and scheduled her for an operation to go in and cross clamp the aorta and remove the narrowed section of the lower aorta.

During the transport to Children’s, Anna suffered what they considered a seizure.  Because of her unknown neurologic state, we had to opt for the lesser invasive balloon dilatation of the aorta via cardiac catheterization.  We had this done hoping that this would normalize the pulmonary pressures.  This never did.  As our stay lingered on at Children’s, it was found that Anna wasn’t developing as quickly as a full-term infant should.  The EEG of her brain showed that she was at the developmental stage of a preemie.  Anna started off eating very slowly from a bottle, but was primarily fed via an NG tube down her nose, into her tummy.  We stayed at Children’s until April 9, 2001.  We had to learn how to administer the many medications she would be on as well as replace the NG tube down her nose. 

We picked up on Anna’s care pretty quickly.  We loved having her at home.  The doctors wanted her to grow some more and do some physical therapy in hopes that it would improve her ability to eventually sit, crawl, then walk.  Clint quit his job a week before Anna was born and stayed at home with her for the next 2 ½ years.  Many therapy sessions came and went.  Clint taught Anna how to eat by spoon, taught her how to sign for “more” and taught her how to clap. 

At about the age of 4 months, we went in for another cardiac cath to determine what the pressures in her lungs were.  To the doctor’s dismay, and ours, they hadn’t improved.  She didn’t respond to 100% oxygen at all.  Most patients do, our poor Anna didn’t.   More importantly, the blood vessels in the lungs were very thin and narrow.  She had two pretty large aneurysms in the left lower lobe of her lung.  At this point, they determined that whatever “unknown syndrome” Anna had, the formation of the blood vessels in the lungs was one of the results of that syndrome.  The cardiac team at Children’s continually saw Anna every month as well as the neurology, gastroenterology, genetics and opthalmology clinics.  She was growing well and responding ever so slightly to therapy.  She rolled around everywhere and would try to pull up her head when on her belly.

At the age of 6 months, we had a G tube permanently placed in her belly as her NG tube caused her to gag and vomit multiple times daily.  It is a miracle she didn’t choke through many of the nights that she gagged.  Poor little girl.

Also around the age of 6 months, Anna was diagnosed with Cortical Visual Impairment.  She was legally blind.  She responded somewhat to visual stimuli, but not to the extent of a “typical” child.  We asked about an MRI at this point and were told that the MRI performed at birth showed no visible brain damage (none resulting from lack of oxygen at birth).  They did not want to put her under for any unnecessary reason, so they determined that at the age of two years, they would perform another MRI as at this point in a child’s development, the brain has done most of its infantile development and they would have more of a chance seeing the brain for what was truly there. 

We continued with life this way and Anna underwent multiple blood tests, tissue tests, bone X-rays, etc.  Genetics could not find a disorder that linked all of Anna’s anomalies.  Anna was about 14 months old now.  Anna had been on Digoxin as well as two other blood pressure medications and none of them seemed to be helping.  The pressure in her lungs, as measured by echo every month, had not gone down as she grew.  The doctors at Children’s suggested we contact a specialist in Denver, Dr. Dunbar Ivy.  Dr. Ivy is one of two specialists in the field of Pediatric Pulmonary Hypertension.  He was testing out a pretty new drug called Tracleer that seemed to help a lot of patients who suffered with pulmonary hypertension. 

We got into contact with Dr. Ivy and his team.  They wanted us to fly Anna to Denver for a week so they could observe her and put her under to do a cardiac cath procedure to see for themselves.  We flew to Denver, with our little lovey in mid November of 2002.  Anna was 18 months old and the cutest ever.  We met with the team at Children’s Hospital in Denver.  They were an amazing group of people.   They got Anna into surgery the second day we were there.  They said as soon as they got in, they wanted to get out.  Anna’s pressures were up around 110 then.  Normal range is 30-50 for children her age.  They were very honest with us and told us that the only thing that could help Anna would be a heart/lung transplant and that because of her neurologic state, they would not do it for her.  They also were very blunt about the aneurysms in her lungs.  They said that Anna would most likely die in one of two ways:  a sudden heart attack  or one of the aneurysms could rupture and she would vomit blood and eventually bleed to death.  There is nothing you can do for a pulmonary aneurysm rupture.  I never thought I would ever sit in a hospital room holding my daughter’s beautiful little hand wishing that if she were to go, it would be from a sudden heart attack.  There are no words to describe this.  Since Anna was also close to the age of 2, they also did a second MRI.  The MRI did not show any brain damage.  Anna’s issues had to be contributed to by an unknown genetic disorder of some kind.  The MRI once and for all proved this.

Clint and I cried and cried and cried for a good part of that night.  We were basically told to go home and love her with all we had.  Our lives were transformed when Anna was born, but we didn’t know what to expect….until Denver.  Our lives were changed forever when we left Denver.  We were devastated and to this day, we still have not recovered.

We got home from Denver and loved Anna with all of our being.  We had so much fun with her.  None of the doctors could give us any kind of a guess as to how long Anna would live.  They just didn’t know.  They did start Anna on the Tracleer drug and it never helped with the pressures in her lungs.

Just before Anna’s 2nd birthday, the last week of February, Clint was out in the garage working on the car and I was watching TV.  Clint had the baby monitor out in the garage with him.  At about 10:45pm that night, Clint heard a strange sound from Anna’s room.  He came in from the garage and went into her room.  I followed him just to check up on Anna and she sounded as if she were choking.  We were terrified.  I didn’t know what it was and then it dawned on me….a seizure.  We put her back in her crib and laid her on her side.  The choking sound stopped immediately as it was her drool she was choking on.  Her lips were not blue so I knew she was getting air.  I called 911 immediately.  The paramedics were at our house within 7 minutes and the seizure was still going.  They could not get an IV line, so they had to administer Diastat rectally which is a valium type drug.  The first dose didn’t do a thing, so they administered a second dose.  She was still seizing and it was 25 minutes into the seizure.  They were preparing to do an injection directly into her bone marrow when she finally came out of the seizure…30 minutes later.  She looked to be paralyzed on her left side.  Nobody knew whether it was a stroke or seizure that Anna had suffered. 

Anna was transported to Children’s Hospital that night.  They did an EEG of the brain and did not think that she had suffered from a stroke.  It was definitely a seizure.  The paralysis, which is called Todd’s paralysis, is very commonly seen in patients with seizures lasting 5 minutes or more.  The brain is so over stimulated that it partially shuts down for a time.

After that day, nothing was the same.  Anna had lost almost everything that her and Clint built together over the last two years.  She stopped eating completely, she didn’t clap her hands anymore, she didn’t try to sign at all.  She stopped rolling around.  We had to start from scratch.  The medicines they put her on for seizure control made her skin break out in a horrible rash and made her so unhappy.  All she did was cry and sleep for a good two months of her life.

Clint and I called Children’s and told them “No More Meds.”  We did not want to treat the seizures with medication so they started Anna on the ketogenic (high protein) diet for seizure control.  She seemed to do well on the diet as she never again had a long seizure like the first one, but the diet brought on kidney stones that she suffered with to the end of her life. 

In November of 2004, we admitted Anna to Children’s for an overnight EEG study to determine how her brain activity was in regards to the seizures.  Although Anna was not suffering from grand mal or clonic/tonic seizures, we did find out that she had 464 seizure-like events in only 24 hours.  Her poor little brain was at rest when she was in an awake state more than when she was asleep.

We ended up seeing another neurology specialist outside of Children’s to try and help Anna with these seizure events.  They suggested we start her on a medication called Sabril.  You could only get it out of Canada as the FDA in the USA hasn’t approved it for use yet.  We started her on this, but she ended up getting shaky and very out of it.  We wanted better for her and this medication just sedated her, it didn’t improve her quality of life.  We ended up putting her on a low dose of a sedative type drug and she was on that until she passed away.

Clint and I went through some hard times and I ended up moving with Anna back to Bellingham, WA.  I quit my job in the Seattle area and took six months off with Anna.  Clint stayed in the Seattle area, driving the 100 miles to Bellingham multiple times a week and every weekend to see Anna and me.  I found a job in Bellingham in August of 2004 and Clint moved up with us on January 1, 2005.  We were together again.

In mid January of 2005, Anna got a bad cold.  She went to the hospital and her heart rate was in the high 180’s to low 200’s.  They flew her to Children’s in Seattle.  In early November, Clint and I had met with Anna’s pediatrician to sign a DNR (Do Not Resuscitate) order for Anna.  Her cardiologists and pediatricians were trying to talk to us about this for a good part of a year.  We were never ready to sign it, until November 2004.  Signing that DNR was the single most hardest thing I have ever done in my life, aside from losing my little girl.  When that helicopter arrived in Bellingham to take Anna to Children’s, they had to talk to us about the DNR.  They had to clarify that if Anna’s heart stopped in flight for any reason, that they would not perform any life sustaining measures.  I was so torn apart.  One of Anna’s nurses, Kris, bent down and looked into my eyes and said “The most unselfish thing you could ever do for her was to sign that DNR.”  At that moment, I completely fell apart.  The gentleman from the helicopter medical team had told me that Clint nor I could fly with them as they didn’t have any extra room.  He was there with me when I broke down.  He got up for a minute, then came and sat next to Clint and I and said, “OK.  One of you can go with us.”  I was overjoyed.  Never, in Anna’s whole life, had we ever left her when she was sick.  Never.  I wanted one of us to be with her if she died.  Clint went with his daughter to Children’s that day and never left her side.  The day she was born, he never left her side.  He went to Children’s with her then too. 

We ended up making it out of Children’s after a two week stay.  There were a few days that were pretty touch and go, but Anna held on and recovered, as she always did.

We got back home to Bellingham and Anna had about a week and a half where she felt OK.  She got sick again.  We took her to St. Joseph hospital in Bellingham and decided to treat her there instead of flying her to Children’s.  She ended up getting pneumonia, and after a two week stay at St. Joes, she came home.

Anna was home for about two weeks.  On Tuesday morning, March 1st, I brought Anna to Kim’s house (the lady that watched her twice a week for us).  I left my little girl on her teddy bear blanket at 8:00am that morning.  At 9:00am, Kim called me frantic saying that Anna had died.  She had a heart attack at 9:00am on Tuesday, March 1st, 2005 and never came back.  The paramedics did try CPR as they did not have a copy of the DNR at their immediate disposal, but they never got a pulse.  Our little Anna Grace was gone.

Anna fought so hard and so long and had the heart of an 85 year old.  As much as I long to hold her in my arms every single moment of every single day, I would never, ever wish that suffering on her again.  I will see her in Heaven, I will…..