Breath of Fresh Air

Carly was discharged late yesterday afternoon and we are all back at home. It is amazing how much better she is after such a short time, she never ceases to amaze me. She is still congested and will take some time to get back to herself, but she is heading in the right direction.

Love, Brad

Midnight Run

We have another medical adventure to add to the record books. Christa, Carly and I headed up to Tahoe for the weekend as we typically do during the winter. With more big snow storms on the way we were ready for some powder therapy in the mountains. On Friday night Carly had a cough that kept her, and us, up all night. In the morning the cough subsided and she looked better so we headed to Alpine Meadows to meet our babysitter and hit the slopes. The snow was knee to thigh deep and we had a great morning, but when we came in at lunch Carly did not look good and seemed to be having an adrenal crisis (low cortisol.) We gave her a cortisol shot and she started to look better but her breathing was still very labored so we took her to the ER in Truckee for an exam. They didn't like how she looked either and they wanted her to be seen by pediatric specialists at the children's hospital in Reno. Christa and Carly were loaded into an ambulance and with lights and sirens blazing they were rushed down to Reno while I followed in our car. The team in Reno placed a central IV (which was tricky) and took blood labs. The early lab results did not show an obvious cause for the labored breathing, and they felt that Carly would be best cared for at UCSF where she is well-known.

By 10:30pm Christa and Carly were being loaded into another ambulance and taken to the Reno airport where a small, four seat airplane was waiting to transport them to San Francisco. The plane was tiny and Christa had to sit in the co-pilot seat while the nurses tended to Carly in the back. There was a massive storm system in the area and they had a harrowing one hour flight through 100 mph winds and snow to San Francisco airport where a final ambulance waited to take them to UCSF. During this time I made the 4.5 hour drive (through snow and heavy rain) from Reno back to San Francisco, finally arriving at the hospital around 3:30am. There was a lot of activity around Carly most of the early morning, and things finally settled down around 8am.

It looks like Carly has a lung infection that is most likely viral. After some respiratory therapy and coughing her breathing has improved considerably today and she is smiling and eating well. The virus will likely take several days to pass and she will need to stay in the hospital until the doctors are comfortable sending her home, but after the progress we have seen today that could happen pretty quickly. It was a crazy day and we are short on sleep, but seeing Carly smile makes us feel much better already.

Back Home Again

Our stay in intensive care was short and Carly was discharged around noon today.  She is doing well, is very hungry, and is already full of smiles again. She is a little wonder and lights up our home.

Love, Brad

Recovering

Carly's surgery went fine, with a few unexpected surprises. Dr. Auguste did not find a clog in this shunt. He still replaced the ventricular catheter to the material that may help if she is having an allergic reaction. The high protein could be the cause of the symptoms that looked like a clogged shunt, and hopefully the new shunt hardware helps in lowering this. At the end of the procedure they could hear a lot of congestion in Carly's airway so they left her on the breathing machine and sent her to the PICU for recovery. A chest X-ray did not show any signs of a major lung infection so she was extubated a few hours ago and is resting pretty comfortably now. We are hoping that she can get some much deserved rest.

Love, Brad

Fourteen

Over the last few weeks Carly has shown some signs of a malfunctioning shunt, which we were concerned about happening because of the elevated protein levels in her CSF. She is still having good moments full of smiles, but it is clear that she needs to get the shunt fixed. She will have surgery today at 3pm, and they will try a new shunt material to see if an allergy could be behind the elevated protein (and all of the clogged shunts.) Please keep her in your thoughts.

Love, Brad

Next Steps

We met with Dr. Haas-Kogan from radiation oncology at UCSF this week to discuss treatment options for Carly. Radiation is the preferred treatment from her perspective and it was the consensus from the UCSF tumor board meeting, as it has a good track record of success for craniopharyngiomas. Radiation is not without issues though, and there are some side effects that we need to consider. Treatment would cause some hair loss and will likely make Carly extra tired during treatment, both of which are pretty minor. The biggest risk is pituitary damage, but Carly has already lost her pituitary gland during surgery so this is not an issue for her. The radiation can also cause damage to her vision, which is already affected to some degree, but she felt that this risk is very low. The most concerning risk is neuro-cognitive damage, which is unavoidable to some extent. Because Carly’s tumor is very small at this point the overall radiation dose and area that requires treatment are both small which will minimize this risk. Dr. Haas-Kogan believes that because of this, the expected damage would be less than 10%.

The type of radiation treatment that Carly would receive at UCSF is called IMRT (image modulated radiation therapy) which is a method that allows them to control the intensity of the radiation to help preserve nearby tissue. She would get treatments for 30 minutes each day, five days a week for six weeks. Since she would have to hold perfectly still for the treatment, and she would need to have anesthesia each time so she would also need to have a central line placed. (Placing an IV each day would be impossible.) This treatment would most likely begin on March seventeenth, and Carly could stick with her school and weekend routine during the treatments if she felt up for it.

One other radiation option is proton treatment. This is a less common treatment method and only five centers in the United States offer it. The advantage of protons is that they are very targeted, slowing down very quickly after hitting the tumor. This helps preserve surrounding tissue and may reduce some of the potential side effects that are seen in other radiation methods. We are consulting with Harvard/Massachusetts General on this option now and will see if it is a suitable alternative for Carly.

Whichever option we go with, treatment will begin soon so the tumor does not have time to grow any larger. We all know that Carly is an incredibly strong little girl, and with the support of Team Carly she will get though this. Your support also helps Christa and I each day, and we are very grateful for it.

Love, Brad

Unexpected

Carly has been feeling better since her shunt surgery and is back at school again. She has also been smiling a lot more since the surgery and she loves playing with her stuffed animal friends. This we are very thankful for, but we also got some news today which is not as good. Carly’s most recent MRI that was taken last week shows what the team at UCSF believes to be recurrent tumor. They had noticed an area that had them concerned three months ago, but Dr. Gupta was hopeful that it was just scar tissue. The area grew in every dimension in the most recent scan which is indicative of tumor and not scar tissue.  The concerning area is pretty small now, about the size of a lima bean, but it has been growing slowly for several months.

There are a few options for treatment that range from radiation therapy, surgery or some type of chemotherapy.  Each has its own set of strengths and weaknesses which we will be reviewing with a range of specialists over the next few weeks. We will also get second opinions from some of the most respected centers around the US to ensure that all of the best minds are thinking of Carly.  We keep hoping that this area will just shrink away on its own, but we also have to be prepared for whatever Carly needs. I know she is in all of your thoughts and prayers, and she can use the extra strength and healing now as much as ever.

Love, Brad

Happy At Home

Carly was discharged late yesterday and we are back at home again. Her recovery from this surgery is probably her best yet, and she slept well through the night last night. She is a bit uncomfortable from her two incisions, but Tylenol seems to be helping and after a few days that should pass. She is one tough and amazing little girl.

During her surgery they took a sample of her CSF to check for infection and other cell counts.  The initial results for an infection do not show anything, and it is pretty unlikely that this is an issue.  The protein levels did come back very elevated again which was not expected. As you might remember, this was an issue for Carly last spring and many of her shunt failures last year were attributed to high protein. At one point last year her protein level was 30 times the normal level but it had dropped quite a bit over the last few months. It is now back up to about 20 times the normal level but the cause is a mystery. We will follow up with neurosurgery next week to see if they have any additional insight but for now we'll just wait and see, hoping that this shunt can last awhile. Thanks for the love and support, we can feel it each day.

Love, Brad