Leukoplakia (day +797)

Dyskeratosis Congenita was originally described as a triad of conditions: reticulated (net like) skin pigmentation, nail deformities, and oral Leukoplakia (white plaque). Even though this is an outdated description, those three physical aspects still hold true with Dyskeratosis. 

For these reasons, Clark sees his regular dermatologist every 6–12 months. She checks him head to toe for any skin abnormalities. She also monitors the deterioration of his fingernails and toenails, since he has Dysplastic nails. During Clark’s last visit, she noticed some Leukoplakia on the inside of his cheeks and tongue.

Leukoplakia is white plaque that forms on the tongue or the lining of the mouth. It can become painful, making it difficult to swallow or chew. Fortunately, Clark doesn’t have any pain.

There is the possibility that Leukoplakia can become cancerous. Because of this, Clark’s dermatologist referred him to see an oral specialist. I wasn’t sure what to expect; I was worried the specialist would need to do a biopsy. Thankfully, all he had to do was paint a blue dye in Clark’s mouth. If the Leukoplakia is cancerous, the dye visibly changes colour. It didn’t! Clark’s mouth is cancer free.

Due to the nature of Dyskeratosis, Clark will need to have his mouth monitored annually to be sure it remains cancer free.


Speech Therapy (Day +541)

Clark’s Speech Therapy is taught in a pre-school setting. His class has 5 other students, his teacher, and the teachers assistant. Clark was very shy at the beginning of the school year, but he has slowly warmed up to his classmates and is now making friends. We’ve already been on one play date after school, and will hopefully have more to follow.

Once a month his class has show-and-tell where the kids are able to tell the class about a special toy or book. They also have a “cooking” day where they make their own snacks. This usually consists of decorating a cookie, or making an animal face with a slice of bread, m&m’s and pretzels. Clark takes great pride in his projects, and loves to eat them afterwards. The kids work on enunciating words, sounds and full sentences while they cook, create art projects, sing and read stories.

Clark has come a long way since starting speech. He is now a little chatter box! I often need to ask Clark to repeat himself, but his language is becoming more clear each week. And because I understand what he is saying (most of the time), his temper tantrums have drastically reduced! We are all happier for it.

IEP (Day +505)

When Clark turned 3, not only was he a year older, but it was also time for him to switch over to another special education program. The Regional Center only helps children from birth until their 3rd birthday. With their help, they transitioned Clark to receive help through the school district.

Our school district set up meetings and evaluations. Clark was evaluated by a speech therapist, occupational therapist, psychologist and a physical therapist. He was evaluated twice with each group. I also had multiple meetings with them to explain the process and sign papers. It was a long, tedious process, but they made sure we were able to complete everything before Clark’s birthday. We are still waiting for an evaluation from the vision specialist though. Once Clark was approved for special education, the school set up an IEP for him.

An IEP  (Individualized Education Program) is a written statement of the educational program designed to meet a child’s individual needs. This is an important legal document that spells out Clark’s learning needs, the services the school will provide and how progress will be measured. Every child who receives special education services must have an IEP.

Clark is now taking Speech therapy 4 hours a week.  In 2 hour sessions, twice a week. He is also taking physical therapy for 30 minutes, once a week. In the short period of time Clark has been in therapy, I have already noticed improvement. We are extremely happy that Clark is able to get such great care in the areas where he needs help to thrive.


Eye Surgery (Day +499)

Ophthalmology insisted on taking a closer look at Clark’s eye before Christmas. We were scheduled for December 21st, but the boys caught a cold. As it turns out, an upper respiratory infection mixed with anesthesia can cause blocked airways. Something everyone would like to avoid.  We were able to push Clark’s procedure to December 30th.

So far, all of Clark’s surgeries have been relatively minor. Getting his central line (twice- once for a single lumen, and once for a double lumen), and having them removed, bone marrow biopsies, and now an eye exam under anesthesia. But even though these are all considered safe, they still scare us. There are studies showing a link between anesthesia before the age of 3, with ADHD, learning disabilities, and language delays. This will be Clark’s 7th surgery, and he only turned 3 this month.

Surgery went relatively well. Dr. Moore dilated Clark’s good (right) eye, before going under. Once he was asleep, they put orange dye through his veins to watch where the blood clots/leak was coming from. Then they took pictures, and lasered the leaking veins to prevent further leakage. The fluid coming out of the vessels in his eye can lead to a hormone release, which can cause blindness. This is why the laser treatment is so necessary.

Clark will come in for a follow up surgery to make sure the leak has stopped, and the blood clots are gone. If not, there will be more laser treatment. Lets hope this leak has disappeared, or at least shrunk!


Ophthalmology Check-up (Day +484)

Since Clark only has 1 working eye, we need to be very careful with it. Thankfully his Ophthalmologist is in 100% agreement. Because of this, Clark wears glasses for protection. Clark is so active, it would be very easy for him to get a stick in the eye, or damage it from a fall or hit. Another proactive measure we take, is by visiting ophthalmology every 3-6 months. Just so they can monitor his right (good) eye.

Today we had our visit. It has been 5 months since the last appointment. And things don’t look good. It looks like Clark has a blood clot, or leak inside his eye. Dr. Moore needs to have a better look, which is only possible with anesthesia. No one likes to have their eyes pried open while a bright light is blinding you. Let alone a toddler! Considering the situation, Clark actually sat very still for his check up, but it was still too difficult for the doctor to understand what is going on.

These appointments usually take up half of our day, and then we are stuck in traffic on our way home. I guess that means we’re ordering in pizza tonight!

The Regional Center (Day +397 )

The Regional Center is a program through the government to help children, up to the age of 3. There are many programs they provide, such as speech, occupational therapy, physical therapy, help for the blind, deaf, and more. The child has to qualify to be accepted. Usually the kids need help because of medical issues, or an accident.  To apply, all they need is a referral from a doctor OR a parent! In Clark’s case, I referred him.

After filling out a LOT of paperwork describing Clark’s medical background and sending previous medical evaluations (speech, occupational and auditory), Clark was able to be evaluated first hand through the regional center. To be approved, Clark needed to be at least 33% behind what is normal for his age.

Clark was approved for Speech and Occupational Therapy, as well as approval to see a Vision Specialist (I didn’t even know there was such a thing!).  Occupational Therapy is once per week with Joanna, a lovely lady who comes to our house. She brings a huge bag of toys, and has Clark do activities with her, such as puzzles, drawing, play dough and more. Then we see Elizabeth once every 2 weeks. She is Clark’s Vision Specialist. Elizabeth takes Clark to different local playgrounds and helps to make sure he can safely play. Since he only has vision in 1 eye, Clark doesn’t have depth perception. So she works on key words to help him understand, such as “slope”, “Step”, “drop off”, etc… For Clark, this is definitely more play than work.

Clark was approved for speech therapy. However, there is no one available to help him! From what I understand, the Regional Center hires out local therapists. And it looks like everyone is full. This is very frustrating because speech is the main reason I had Clark evaluated in the first place.

On top of that, Clark’s evaluation for physical therapy showed that he doesn’t need help. He was slightly better than where he needed to be. I think Clark needs help. He can’t jump, has trouble walking up or down the stairs, and can’t run without tripping. Under normal circumstances, I would have him re-evaluated, but it wouldn’t help much. Clark will be 3 in December, and all of his therapy will be transferred to the school district, where they will re-evaluate him. Again.

I love the help Clark is getting. I just wish it was a little easier to navigate.


Speech Therapy (Day +309)

Clark finally had his 1st appointment with Speech Therapy since his BMT. This was more of an evaluation, instead of helping Clark out. They need to see how well he is doing, if he needs help, and what the best methods to help him might be.

We learned from this evaluation, that Clark’s cognitive skills are above average! He understands what we say to him, and can follow directions. We already knew this, but its always nice to hear it from a professional. :)

As for his speech… he is behind, but very interested in learning. He can say the first part of words, or has special sounds for words.  For example, when he says “sit down” it sounds exactly like “sit down”, but when he says “milk”, it sounds like “emm”.

As it turns out, UCSF’s Speech Therapy only helps temporarily. They do not do long term therapy. So now I need to find a local speech therapist that is covered by insurance. This is turning out to be a difficult task. I have already called a few local offices, and they either have a very long wait list, or don’t accept health insurance. And since each session will cost approximately $120, and Clark has been recommended to go twice per week…. that doesn’t sound like an option. I am not done looking though! I will be making more calls this week. :)

On a positive note, there is a Regional Center that is government funded. They do Speech, Occupational and Physical Therapy, as well as other programs. Clark is currently in the process of being evaluated. This may take a couple months before he is approved for help. If he is approved, Clark will have a speech therapist until he is 3, and I believe it is free of charge. This would be a huge help for him!

Pre-Vaccine: Thrill Seeker, Part 3 (Day +288)

14 hours after Clark’s pressure bandage was placed, I took it off. Clark had been itching to take it off all day because it covered some of his vision. As I took the bandage off,  I held my breath- hoping to see a big scab, with no bleeding. As the the pressure was released, there was a “pop” as blood squirted out onto the sidewalk. It wasn’t the actual sound of a “pop”, nor was it the scab “popping” off. The pop was more like popping a zit. Gross, I know., but the best description I could think of.

Blood continued to run down his face as I re-wrapped his cut. I knew that Clark needed to get his factor today, but I also knew that it wasn’t “urgent” since I was able to stop the bleeding with his pressure bandage. So before we headed to the Treatment Center at UCSF, we took an hour detour for Connor’s kindergarten evaluation. Which he did amazingly btw. :)

The treatment center did a great job. They distracted both my boys with toys, and played Planes on the giant tv. Before even attempting to poke Clark, they checked his veins, and put a numbing cream in the areas they thought would work best. Then we had to wait for 45 minutes for the cream to do its magic. It took 3 attempts to finally get Clark his factor. This was not fun for poor Clarkie. But I am so glad they were successful. Then they washed Clark’s cut, and put another pressure bandage on it.

Our new plan for Clark, is to have him wear the pressure bandage for 72 hours. I will also be taking Clark in to see Plastic Surgery on Monday. Hematology thinks that they may have more options to help close his cut, without making him scar too much.

Pre-Vaccine: Thrill Seeker, Part 2 (Day +287)

Last night was spent at the ER. Thankfully not ALL night, just a few hours.

Clark’s eyebrow had re-opened twice this week. Once on Saturday, and again on Sunday. Both times the bleeding lasted about 20 minutes (with constant pressure) before stopping. And then yesterday happened…

…Clark woke up from his afternoon nap, crying and covered in blood. To my surprise, I was not very surprised! I knew immediately that it was coming from his eyebrow. Plus, this is not the 1st time this has happened. Before BMT, Clark woke up covered in blood from nose bleeds. The difference this time is, I was calm enough to take a couple pictures. :)

I took Clark directly to the bathtub and held some paper towel on his cut. Then I asked Clark to hold the towel, while I took off all of his clothes and started the bath water. The mess doesn’t bother me (any more), but it does bother Clark. Every time he sees a drop of blood land at his feet, the floor, or on his clothes, he cries. So keeping the bath water running was calming, and helped wash away the blood. I also gave him a peanut butter rice crispy square, which helped a lot!


I realized that the cut re-opening every day for 3 days was not a sign of healing. So, once things started to calm down, I called the on-call hematologist. I needed to know if this meant Clark would need a dose of factor. After going back and forth, we agreed that the location of Clark’s cut was not dangerous (unless it wouldn’t stop bleeding on it’s own). If this had been inter-cranial, an organ, or a joint, we would need to take Clark in immediately. But his eyebrow is only a surface cut that wont leave long term damage. Our plan was to get the bleeding to stop, and go from there.

It did stop. But only for a short period of time. His eyebrow kept re-opening. I used bandages, gauze, and pressure. Nothing was working. Finally I decided to take Clark to ER. We got a private room right away, thanks to the blood covering Clark’s face. The room was actually really nice with a private bathroom and a TV! I was pleasantly surprised. Plus, the TV helped entertain Connor while Clark was getting his IV.

Over the last year and a half, we have had too many let downs in ER when attempting to get an IV in Clark. From those experiences we have learned a few things. 1) Have a Pediatric Anesthesiologist get the IV in, and 2) Don’t let them try as many times as they’d like. Because they will keep trying and trying and trying….  I didn’t have any luck getting the Pediatric Anesthesiologist… but I was able to stop the nurses after the 3rd attempt at an IV. Why hurt my baby 6 more times to continue to fail? I don’t blame the nurses. Clark has never been easy to get a vein (0 success in any ER).

Thankfully the ER visit wasn’t a complete waste of time (and money). The ER doctor was very helpful and sympathetic. He did not try to convince me to have the nurses try again. Instead, he came up with an alternate plan. He washed Clark’s wound to help prevent any infections, and he also wiped out the glue! Apparently glue (instead of stitches) can slow down the healing process. He suggested getting stitches next time, because this will help a cut heal faster, and help prevent bacteria and other germs from infecting the cut. After that, Doc made Clark a pressure bandage to hold him through the night.


Well, it worked. No more bleeding last night. Next step: wait until Clark has had 12+ hours with the bandage on. Hopefully with the newly washed cut, and the constant pressure, Clark will have a secure scab. If it does start bleeding, I will be skipping ER, and taking him to the Treatment Center at UCSF. They give children infusions, transfusions, and draw blood on a regular basis. I’m certain we will have more success there.

Pre-Vaccine: Dead Vaccines (Day + 253)

Clark has finally started getting his vaccines! Yay! This is very exciting for us since Clark needs the extra protection while his immune system is still weak.

Most children have their vaccines spread out over a few years, including booster shots. Clark will be on the fast-track to completing his vaccines. For the time being, Clark will only have “dead” vaccines (non-live) to prevent him from actually contracting the virus’. Clark has now had 2 rounds with about 5 shots each. In 8 weeks he goes in for his last round of booster shots.

If you can’t remember getting your last vaccine, they can be a bit painful.  Especially when you end up getting 10 shots in 4 weeks.  The crying was to be expected, but the little bumps (like a muscle knot) on each thigh where he was injected, was new to me. One of the injections was so painful that Clark refused to walk for almost 2 days! So we gave him a little Tylenol before bed, and held him throughout the day. :( Apparently this is common for some of the vaccines. Other than that, Clark has been great. He doesn’t fight me while he is getting his vaccines, and he forgets the pain quickly.

1 month after Clark’s last dose of “dead” vaccines, he will have labs drawn to make sure he is maintaining these new antibodies. If all goes well, we start talking about giving Clark his “Live” vaccines. These are the vaccines that I am impatiently awaiting. Even though Measles seems to be off the radar, Rotavirus is still common, and can be dangerous.