Adjusting to our new normal

Here we are, half way through summer (or as we are calling it in north west Ohio, Monson season) and half way through 2015. Time has flown by and has shown no mercy for my desire for a slower pace. The last several months have been a challenge and have shown us that Batten disease does what it wants, when it wants and doesn’t care how much we plead with it to leave our daughter alone.

First let me apologize for the length of this update and for how long it has been since we shared with all of you how Kate is doing. It has been busy few months and over the last several months we have had to adjust to Kate’s new normal, which requires more of our time and attention and at times was hard to accept.

June 22 marked the five year anniversary of our daughter’s diagnosis. I hate June 22! I woke-up on June 22, 2010 without the knowledge of Batten disease and with the belief that our daughter would soon regain all of the skills she was losing and her seizures would get under control as soon as we found the right medication. I had no idea that in a few short hours Kate’s neurologist would call and turn our world upside down. Life today looks nothing like it did 5 years ago. The world is different. We are different. As with all changes, some things are better and some things are devastating. Over the last five years we have learned to see and focus on the good. How miserable would we be if we didn’t look for the silver lining in each cloud?!

Since late December Kate’s vomiting episodes and congestion have increased in frequency and severity. It has become a challenge to keep her meds and food in her system and keep her airways cleared. After meeting with a GI doctor and a pulmonologist it became clear that the congestion and vomiting were due to a common factor. Kate’s stomach was no longer working as it should. Kate was having a lot of reflux, meaning the contents of her stomach were leaving her stomach and moving up her esophagus. This caused her to vomit and could cause damage to her esophagus if left untreated. The pulmonologist was concerned that the fluid was entering her lungs, thus causing her congestion and was the reason she had pneumonia two times within the last six months. The GI doctor ordered a test to see how severe it was and the results were alarming. Based on their advice and the test results we decided to have a GJ tube placed in her stomach. She had a G tube already. The G tube allowed us to feed Kate via her stomach. The GJ tube would allow us to feed her via the stomach or small intestine. We have adapted to many things since Kate’s diagnosis. We have learned to use all types of medical equipment, learned about different medical conditions that affect her, etc. We have done so willingly and have adapted quickly to her new needs. When we first realized that Kate needed a G tube we were nervous and sad but moved forward quickly. We saw the benefits and were ready to do it for everyone’s benefit. I can tell you I didn’t accept the news of needing a GJ tube with the same mind set. The thought of the GJ tube to this day angers me. We had it placed a few weeks ago and I still don’t have peace with it. I know it was and is necessary. I see some of the benefits. I am thankful we had an option to keep her safe and a live. However, the thought of her being hungry because her stomach is empty bothers me and I hate that this disease has taken another piece of my daughter. The doctor assures me that Kate isn’t hungry. When someone is hungry they can feel weak, shaky and disoriented because they need nutrition. Since Kate is receiving nutrition via the small intestine she doesn’t feel those things. That brings some comfort. She has handled this a lot better than I have and once again proves that she is stronger than the disease.

Our hope was that the GJ tube would stop the vomiting and congestion. It hasn’t. This saddens me. Her congestion is about 80% better and while she doesn’t have anything to vomit we have seen a few times each week episodes of her wanting to vomit. Had she had food in her stomach she would have. Instead, she dry heaves. The GJ tube was not the miracle or solution we prayed for but it has increased her quality of life. For this I am thankful.

Kate became ill on Father’s Day, June 21. After a long day of treating her at home we went to the ER. It was determined during that visit that Kate had pneumonia; this was the third time since October. The doctor was willing to release her and allow us to treat her at home since we had everything the hospital would use at home. I was pleased with his decision and felt equipped to do everything Kate needed. I under estimated how sick she was and the difference between the IV antibiotic given at the hospital vs. the antibiotics we use at home. The following day was horrific!!! Kate was miserable and required constant care. She worked for every breath. Her oxygen levels would decrease to 70-80 without the use of oxygen and we didn’t have enough oxygen on hand to last an entire day. The oxygen company couldn’t deliver to our home for another day, so I had to leave Kate and travel 1.5 hours (one way) to get more oxygen. When I returned home she was showing signs of pain; her face communicated she was in pain, she had a few tears, and she looked like she wanted to scream. We havent seen her in pain since her brain surgery four years ago. Her heart rate was 150 (her norm is 70-120), which indicated she was in distress. Between her pain, O2 levels, and constant need for suctioning we were in over our heads. Thankfully we had a nurse with us during the day and at night. Brock and I agreed that if things weren’t better by the morning we would take her back and ask for her to be admitted. You can imagine how scared we were for her if we were asking to be admitted to the hospital. Thankfully she made a huge improvement over night and appeared to be 75% better. She still required a lot of attention and constant treatments – breathing treatments, vest treatments, suctioning, etc. We were relieved to see how much better she was doing. The following 10+ days were long and stressful but with each day she showed small improvement. It wasn’t until this weekend we felt she was back to herself.

Since Kate was so sick we were able to obtain new medical equipment that we requested several months ago; a cough assist and pulse ox machine. It is sad but a relief to have these machines. We know the equipment will help keep her healthy and safe.

Since Brock and I both work full time one of the grandmothers has been caring for Kate after school and during the summer. Due to change in the grandmother’s schedule we had to find someone to take care of Kate during the summer. Fortunately, we were approved for additional nursing hours and were able to find a few nurses to take care of Kate during the day. Kate seems to enjoy the nurses and the activities they do with her each day. We feel blessed to have found nurses that are compassionate and loving. I think Kate misses school but the nurses do all they can to keep Kate busy.

Over the last few weeks Batten disease has gained a lot of publicity due Charlotte and Gwyneth Gray, daughters of well-known Hollywood producer. Both of the girls have Batten disease and the family has set up a foundation to fund research. They have been interviewed, asked their famous friends to get involved in raising money and creating awareness, etc. The Batten community is torn on how to feel about this news. While we have prayed for awareness we never wanted another family to receive the devastating diagnosis. And we would never want a family to have both of their children diagnosed with Batten disease. We embrace this family and welcome them to the Batten community, pray for them as they watch their girls progress and pray that their efforts, fame and fortune can bring a treatment and cure!

This weekend we will travel to Chicago for the annual Batten conference. We are excited to see our Batten family, hug the kids and get updates from the doctors and scientist. It is always an emotional weekend but one that we look forward to each year.

We hope you are enjoying your summer and thank you for your support, prayers and kindness.

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