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At home we struggled with how to provide the care Grace needed. The most frustrating part of this time was that we didn’t know what to do and no one else seemed to know how to help us. Our baby was suffering and we have never felt more helpless and hopeless. We thought about pursuing a bone marrow or stem cell transplant for Grace. Although the published research study did not suggest optimistic results, maybe Grace would be the one to beat this disease. Maybe Grace would be a miracle.

What a dilemma we faced. If we chose to transplant Grace, we risked losing her immediately to the chemotherapy treatment required to sustain a transplant. Having seen my mother go through chemo during her battle with cancer I was not enthused about subjecting my baby to that. The thought of having her thick dark hair, and her beautiful lashes fall out broke my heart. With a successful transplant as a symptomatic infant, Grace was unlikely to have a better quality of life, but it might be longer. She would spend months in the hospital with multiple machines, tests, scans, and blood draws. This is not what we wanted for Grace but we felt we needed to explore every option and called Duke.

After discussing Grace with a physician at Duke, she did not sound like a good candidate for transplant. However, we wouldn’t know for sure without an in person evaluation. We were unsure about the trip but decided it was important to go and not have any regrets. We were also eager to speak to someone who had seen Krabbe disease before and who could suggest therapies and medications to help keep Grace comfortable. During our visit to North Carolina, Grace was evaluated and deemed not a candidate for transplant. I was somewhat relieved that the decision was made for us but now all I could do, besides hope for a miracle, was wait.

Even when Grace was in Alysia’s belly I would whisper to her all of the things we were going to do. “We’re going to travel together, we’re going to read good books and talk about them. We’re going to go snorkeling in beautiful blue water. We’re going to have lots of adventures. You’re going to grow up and be a beautiful woman. You’re going to go to college; you’re going to help people. You’re going to fall in love with someone wonderful and you’re going to have beautiful babies.” Not long after she was diagnosed I stopped doing that. Later, when talking to another Krabbe parent he said that in addition to the death of your child you also suffer the death of a dream.

The first few weeks after diagnosis were a blur. We were trying to figure out how best to care for Grace. Fortunately, through a friend, we connected with a pediatric geneticist who was willing to work with us to manage Grace’s care. We experimented with medications and techniques. Looking back I feel bad. I feel that Grace suffered because we didn’t yet know how to care for her. The learning curve was steep and exacted a vicious toll on us in stress, frustration, and sleep. We did the best we could for her but we did not have the skills or knowledge we needed to care for a baby in her condition.

Grace was having muscle spasms. Her limbs would grow very stiff and she would cry. We were told that as the disease progressed Grace would become increasingly stiff and then would suddenly become flaccid. We were told that this usually occurred at the end. Very soon after we heard this Grace became flaccid and we were immediately alarmed because we thought her death was imminent. She remained limp for the next six months until her death.

The holidays were approaching and we didn’t think Grace would see Thanksgiving or Christmas or a New Year. We had planned to spend Thanksgiving in Florida but were no longer sure if this was a good idea. We consulted with Grace’s doctor, who said, “Go. Make as many memories as you can.” The night before we left for Florida, Grace had severe apnea and we worried she would not be alive to make the flight and trip. Of course, she continued to amaze us. The trip had ups and downs. Being someone who loves the water I was happy that we went to the beach and that I was able to carry her out into the surf and dip her in the ocean. It was also very cute to see her wearing her turkey bib at Thanksgiving dinner. Unfortunately, during the trip, her apnea increased, she developed seizures, and we had to start morphine. We worried Grace would not make it home.

Each of Grace’s symptoms required a different medication. Each medication had it’s own side effects, which sometimes required additional medications. For overall spasticity and irritability, Grace received Klonopin. Klonopin lead to increased secretions which is bad because children with Krabbe lose the ability to swallow and protect their airway. Grace received Rubinol to reduce the secretions but also needed frequent suctioning to reduce the risk of choking or aspirating. Grace received morphine for pain and difficulty breathing. Rubinol and morphine can cause constipation, which was also a major problem, and led to multiple attempts at remedies. Grace also received medicines for reflux and seizures.

It was also very sad when we started the Klonopin because even though it helped Grace to be more comfortable and cry less it made her very sleepy and she opened her eyes far less frequently than she had before. It was such a gut wrenching choice as a parent to make your child comfortable but not be able to look in their eyes and lose that interaction.

We tried to have a life but it became increasingly difficult. Our quality of life was being stripped away from us as a family as Grace’s disease progressed. We tried to go for walks but it became too stressful, as the bumps from just a simple walk would lead to reflux that would need to be suctioned. Fear of a sudden emergency and the sheer amount of necessary medical equipment and medications kept us from taking Grace out of the house. We felt so limited until we reframed our view of Grace’s quality of life. Now, the important thing to us is that Grace was held close every day until she went to bed between us at night, with Alysia holding one hand, and I the other. We can have no regrets about that.

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