Lisa Santos’ Parent Diary: April 2009

matthew and familyUnfortunately Matthew is once again in the ICU at Children’s Hospital in Boston. The complications from Lafora disease are becoming more challenging. On Thursday and Friday of last week he was very agitated, drooling profusely so that we could not keep up with the suctioning. We all slept very little. His vital signs were within normal limits so we could not figure out what was going on. A cold virus was going through the house and we thought maybe he was fighting that. By Friday evening I noticed his respiration rate was up to 32 breaths per minute (normal is 12-20bpm). and knew that he was experiencing some form of respiratory distress. He had what appeared to be a canker sore on his tongue which we thought might be due to a new mouthcare regime of Hydrogen Peroxide mouthwash recommended by our dentist.

We brought him to the local ER for a chest x-ray. All was fine but the MD stated that he clearly was having respiratory difficulty as he required Oxygen to keep his Oxygen saturation levels at a normal level with the use of 10 liters of oxygen. My concern was that his jerking was affecting his throat musculature which would explain his excessive drooling, which was then going to lead to a case of aspiration pneumonia.

Well……………I should go to medical school! He was transferred to Children’s, by early morning he spiked a temperature of 102, chest x-ray showed a developing aspriation pneumonia and he has a UTI.

As of today, the pneumonia is resolving, he is on antibiotics which have caused gastric upset and diarrhea. I have told them that he should not get his feeding as usual but switch to pedialyte first, then gradually add the Nutren. They increased the Topamax after I asked repeatedly for the Hospitalist to consult with Neurology.

We are hoping for a discharge home on Sunday, but the Attending MD is not so certain about that. I am tired and in need of some respite. I am angry at the Healthcare system for introducing this Hospitalist system which I see as dangerous to the delivery of care to the complex medical patient and has the potential to increase medical costs and length of hospital stay for patients and families. It appears to me that the Hospitalist was trying to make decisions about Matthew’s increase in seizure activity and change in neurological status by reviewing his medical record and sending e mails to the Neurological team. My tip off to that came when he walked into Matthew’s room after a bout of 23 seizures that occured yesterday and asked me the question “they have used Dilantin and Phenobarbitol to contral Matthew’s seizures in the past, what do you think we should use now to control the seizures?.” I looked at him stunned and stated “I think that I can remember what happened when they used those meds in the past, but I don’t think that you or I should make that decision as we are not the Neurologist, they should make that call!” He left the room and about one hour later a Neurologist whom I have never met came and examined Matthew. Mind you, the Epilepsy team is 2 floors down form the ICU and every Neurologist on that team has met me and my son. They came up to his room upon my insistance later and explained that due to Dept. of Public Health ruling the Epilepsy team are not allowed to write orders in the ICU, only consult. I told them about the Hospitalist asking my opinion about the use of Dilantin or Phenobarbitol and that caught their attention and they stated that will not happen again. This is scary to me as I work in the Healthcare profession and understand how things work. I am astounded that despite my pleas to get Neurology consulted since Saturday, Yesterday was the first time they came in to visit. My supsicion is this is another cost cutting measure implemented by the hospitals and the insurance companies. Scott has taken over for me today and tomorrow. I hope that the two of us can stay at a hotel next to the hospital on Friday night and maybe go to dinner. I love my son dearly, but we need some respite.

I share this story with you for future reference. I hope you never experience this as I did.

I am not sure how much longer we have with him. This was another scare, that could have led to intubation and we don’t want to be alone when and if that decision has to be made. Yes, he could be intubated then it could be removed, but it gets harder for him to recover from these events each time.

Take Care,
Hugs and good thoughts to all of you.



Matthew did come home this past Monday night. He had been off of BiPap breathing support for several days, seizure free and appeared stable. I asked the doctor if we could go home and he agreed. I was tired of all the mistakes they were making in his care and felt we could manage him at home. We all slept very well Monday night. Tuesday morning he awoke having some difficulty managing his oral secretions. The nurse came, she was not worried at all. Tuesday night I had another nurse come and do an overnight. By Wednesday am she had some concerns as she thought he was struggling to breathe. By Wednesday night it became very apparent that we could not manage his breathing in the home setting.

We are now back in the ICU at Children’s. Yesterday was a very hard day. We met with the Attending MD in the ICU and once again had the life support question posed to us. I can’t imagine having Matthew live on ventilation support nor can I think of starting the use of Morphine to end his life. His vital signs are stable on BiPap and his spirit is stilling willing to fight. Scott and I had a very emotionally exhausting 48 hours. But last night he had a very good night here in the hospital and we are all rested and ready to go forward.

Thanks for your concern. I will try and keep you posted.
Love and good thoughts to you all,

We just had a meeting with Matthew’s neurologist. She feels that this may be a bump in the road for Matthew, but we should consider quality of life and understand that we should be prepared for what may be to come. We are not sure that the last increase in Topamax will do the trick for seizure control, but there is another drug on the market that we may be able to use. She would not want to add that to the mix, but replace one of his seizure meds with this drug. That may or not be in his future. We are having a meeting with the Pediatric Advance Care Team at 2:00 pm to discuss end of life issues. We may try and determine from Matthew what he would want.

We are trying to stay in the moment, listen to what his body is telling us to do. We don’t have to make any decisions right now.

I will keep you informed of how we doing as we travel this journey with Matthew.


**Read the update on Caring Bridges for Matthew DeSimas at the link below

To view more updates about Matthew go to

**CaringBridge is a free, nonprofit web service that connects family and friends to share information, love and support during a health care crisis, treatment and recovery.