Teething

The process of teething has been difficult.

William has experienced additional secretions and has been diagnosed with three ear infections during the teething process.

Juliet also had ear infections while teething. She has not had an ear infection since getting her last tooth. Hopefully, William will follow the same pattern.

Looking for more help.

Spending time trying to find home care and equipment. Not sure where to begin. We received some advice and help from Tom S. We are appreciative for the help.

Random advice for parents

We are now nine months into this journey. The following is a list of essentials.
1. Get a good neuro-muscular specialist.
2. Get a pulmanologist.
3. Fire any doctor that is not aggressive.
4. Get a suction machine.
5. Get a sleep study.
6. Get a pulseoximeter.
7. If your doctors are not aggressive with colds, then get a new doctor.
8. Get in touch with MDA.
9. Get in touch with the Joshua Frase Foundation.

Twelve days in the PICU, an ER visit and a new pulmonologist.

Lots to talk about.

First, William was stricken with a respiratory infection. The infection can be life threatening due to his weakness and inability to cough up all of the mucous. His cold stayed in the bronchial tubes and did not enter the lungs. We were kept in the hospital for twelve days because the physicians wanted respiratory therapists to perform the deep suctioning.  We received training and are now equipped to perform deep suctioning at home.

As a result of the hospital stay we have a ton of new equipment in our house.  We have a pulseoximeter to continually measure Will's oxygen levels.  We have an oxygen machine that gives William a minimal amount of air throughout the night through a nasal cannula. Currently, William does not need the cannula during the day.  We also secured a cough assist machine that forces air into the lungs and then sucks out the air to mimic a cough.

After returning home from the hospital stay, William was improving until we noticed that one pupil was dilated and not as reactive as the other pupil.  We rushed to the ER where CT scan was performed and found to be normal.  We reached out to other families with MTM children and learned that silent seizures are common. (Special thanks to Scott F. for the emails).  An EEG was performed and was found to be normal.  After nearly 14 hours, Will's pupil returned to normal.  It appears that the cause may have been a new medication.

We were released from the Central DuPage Hospital on Monday afternoon and immediately drove to Comer Hospital to meet with a pulmonologist.  We were still searching for this important piece of our team as we had not yet found a pulmonologist that was willing / capable of handling a challenging patient like William.  After a long meeting and examination we were happy with Dr. Lester and her staff.  So it appears that we now have our team of physicians in place.  (A special thanks to Dr. Rimas Lukas for all his help in assembling the team).

Finally,  we had an extended conference with Paul and Alison Frase concerning the treatment research that is taking place in America and in Europe.  As of today there are no treatments at all for MTM.  The future looks promising as there are several pre-clinical trials (trials not in humans) taking place.  We remain hopeful that clinical trials (trials in humans) will begin in the next couple of years.

The next step for us will be raising funds to promote the research for treatments.  By all accounts, they are very close to developing a treatment.

William is home

Hooray! William is home from his 12 day hospital stay. We are so glad to have our little buddy home. He seems glad to be back too!

William is excited to see Papa

Papa came to visit and watch some basketball with William yesterday while mom and dad went to dinner. William loved hanging out with his Papa!!

This picture was taken when Papa first arrived at the hospital. Happy happy boy!!

William update

William is having a better day. We were able to resume g-tube feeding. He has more energy and is smiling again. He continues to struggle with deep secretions. It looks like he will be in the hospital until he no longer requires deep suctioning.

Another hospitalization

William came down with a cold that rapidly progressed into an upper respiratory infection. His oxygen levels were poor, so he was admitted to the hospital. He continued to decline and the worst possible thoughts crept into our minds. I am hoping he improves, but the doctors said he will likely get worse before he gets better.

We are fortunate to have my mom's help taking care of Juliet. Juliet struggles to stay entertained in the hospital room. Having Nema's help allows us to focus on Will.

Update: Will's condition has not significantly changed over the past 24 hours. He is on antibiotics. Deep suctioning is required every hour. One of the blood tests revealed elevated liver enzymes. A follow-up ultrasound of the liver was normal. The physicians decreased the flow of oxygen and are starting to give him food through the G-Tube. Mom has spent most of her time at the hospital while dad takes the early evening shift to give mom a small break. Mom returns late to allow dad to work and get some rest. She spends the night at Will's side. Mel is such a wonderful mom.

Nema and Will

Sitting upright.

Over the course of the past two weeks, William has increased his ability to sit upright (with vertical and lateral support).  He can sit for up to 30 minutes without choking on his own secretions.  This is a big increase from last month.  Previously we had to lay him down after 5-10 minutes.  He really likes sitting and looking around.  He also loves watching to see what Juliet will do next.  He finds her funny.  William has the same laugh as Grandpa Jim.  HA!

Nema and Papa have graciously volunteered to give us a night off in March.  We are excited to have a night out.