Yeah, we got DENIED immediately for supplemental security income (SSI) for disability assistance (Gaucher’s Disease is considered a “disability” as it lasts longer than 12 months and requires continual treatment). Once she asked my husband’s income, well, that was pretty much the end of the interview. The thing that really bothers me is that we aren’t wealthy people, we are middle class. Since I got put on bedrest in early June last year, we have been supplementing his income with our savings. With Hannah’s situation, I haven’t been able to go back to work. Now knowing that she is going to have IV treatments every two weeks, developmental therapy every other week, occupational therapy every week, not to mention a whole multitude of doctors appointments, I don’t know how I can go back to a “day job” since all of this happens during the week, especially with 2 other kids. I thought about going back to medical transcriptioning (what I was doing before I got put on bedrest) — but work was so scarce even before I left, I’m not even sure that is an option now since my hours would have to be so sporatic.

They don’t care that a substantial portion of our income will now be going to go to medical treatments even after the insurance company pays what they said they will! Even if my husband earned $1000 less a month, we would only qualify for $84 a month. Basically, unless you are living just above the poverty level with our family of five, you don’t qualify — regardless of your needs!

I asked if we could appeal their decision. She said “of course,” but it would continue to be denied because of our income. If we HAD the income they think we have, then we wouldn’t need the assistance!

What pisses me off, and I am pissed with “the system,” is that why do I have to pay for something that I can’t benefit from when I truly need the help? Seriously, that is just not right.

I’m going to start working on a video to help increase awareness of Hannah’s condition, and I’m really stuck and have no idea what songs would be good for a montage of pictures and information. I want a song that is heartwarming but uplifting as well.

Any suggestions out there?

Hannah has redeveloped a bad habit she had as a baby. Scratching her face when she is tired. Most babies do that when they are newborns, but Hannah has just started doing it again this past week.

However now, even with her nails cut and filed very short, she is gashing up her forehead. I mean, really bad. We started putting mittens on her at night again while she sleeps. She only does it when she is tired, and she really just digs at her face. With her low platelets, it takes a bit longer than normal to clot as well.

We don’t think she is in pain, because she only does it when she is really tired.

Any parents out there have their kids do this past the newborn stage? Outside of mittens, does anyone have any ideas or suggestions?

We met with Hannah’s developmental therapist today from Early Intervention. It had been a month since she was evaluated and accepted into the program. She was surprised at how big Hannah had grown and was very pleased to see that there was definitely some progress in Hannah’s motor skills.

Hannah is now able to grab an item with two hands and is totally getting into the toys in her exersaucer (and can even turn herself around in there). We were explaining the freedom Hannah gets in there because she loves to stand up.

She still can’t turn over yet, and really isn’t close to doing it. Her abdomen is so huge because of her spleen and liver being so enlarged that she just can’t get herself over that hump so she doesn’t really try anymore. Also, Hannah LOVES to sit up, but because, again, her abdomen is so big, she finds it very uncomfortable to try and sit. However, she loves to sit on our laps with her knees and legs hanging down a bit. This is her absolute favorite position. She does try to sit up on her own when she is lying back on us or sitting on our laps. We only need to hold her waist, and she can balance herself.

So the developmental therapist told me that even though Hannah hates tummy time that we should really try to continue it as much as we can. We have to be careful because of her spleen and liver, but we are to try it at least a few times a day if not more. Also, she showed us some other tricks to work on to help her try and move over.

While she was here, Hannah was starving so I fed her. Because of her laryngomalacia (poor muscle tone), Hannah dribbles out a lot of formula to the point we joke she has a white beard. She isn’t choking, and even on the slowest nipple, she just has trouble getting it down. No worries about her not getting her nutrition though, she definitely is growing on track. I also explained to her that she thrusts her tongue instinctively while we try feeding her with a spoon. Kind of the same thing she does with her bottle.

So, she wants us to start with an occupational therapist to work on feeding issues with her. We should be starting that soon. We also will say J., our developmental therapist, again in two weeks.

When we met with the genetics doctor when we got the diagnosis, he mentioned she would receive the first few treatments of Cerezyme via IV and then they will probably want to put in a port so it is less stressful for Hannah. I didn’t think anything of it, as I think I was dealing with the overall picture and didn’t even think to ask any questions.

I have started researching into what all this “port” entails, and it is pretty scary. My baby girl is going to have to have surgery to implant this. Not only that, children apparently grow out of their ports and have to have another surgery to replace them. I don’t want my baby to have to have surgery. I know she may have to have with the alternative with someone trying to get an IV into her little body every two weeks, but surgery just scares me. Not only that, but she will probably need more surgeries to replace the port as she grows.

I have found some helpful information on the CHOP website:
Information About Specific Surgical Procedures: Central Line/Subcutaneous Port

What is an implantable central line/subcutaneous port?
A subcutaneous port — also called an implantable central line — allows a child to receive drugs or other fluids directly into the bloodstream through a large vein. The port, which is actually placed under the skin — usually in the chest, forearm or upper arm — can’t be seen, although you will be able to feel a bump there.

How is it put in place?
A surgeon or interventional radiologist will place the port in your child while she’s in the operating room.

Will it hurt?
Your child will receive medicine so he doesn’t feel pain when the port is placed. For three to five days afterward, the area around the port may be tender and swollen. Your child’s doctor will prescribe pain medicine to help your child feel better.  The first few times a needle is used in the port, your child will feel some discomfort. A special cream, called EMLA, can numb the skin so the needle won’t hurt as much. Eventually, the skin will “toughen,” and your child won’t feel as much discomfort.

How is it used?
To inject medications or fluids, the doctor or nurse will put a special type of needle — called a  non-coring needle — through your child’s skin and into the port. If you’ll need to do this at home, you’ll learn how while your child is still in the hospital.   If your child needs medicine infused (injected) over long periods of time, the needle will stay in the port, with a dressing over it. If your child isn’t using the port, it only needs to be flushed once a month with heparin to prevent blood from clotting the catheter. You can do this at home, or a member of your child’s healthcare team can do it during an office visit.

How to care for it
For the first few days after the port is placed, the incision will have STERI-STRIP bandages or stitches.  If your child has STERI-STRIPS, don’t wash over them; they’ll fall off by themselves. Once they do fall off, you may gently clean the area with mild soap and water.  If the surgeon used stitches, she’ll take them out one to two weeks after surgery. Once the incision has healed, simply wash the area once a day with mild soap and water. Your child may take a shower or bath as usual. The only time the port will need special care is when it’s used.

How long will your child have it?
Your child will have the port as long as she needs a way to get medicine into her bloodstream. When she doesn’t need it anymore, the surgeon will remove it while your child is in the operating room.

How will it affect your child’s normal activities?
For the first five to seven days after the port is in place, have your child avoid rough activities. After the incision is healed, he can resume normal activities, but should avoid contact sports unless he has special permission from his doctor.

Words you need to know
If your child needs a subcutaneous port, you may hear her doctors and nurses using a variety of words you don’t understand. Here are some of the words you may hear — and what they mean:

Catheter: thin tubing attached to the port through which fluid travels to the blood
Continuous infusion: medication given for a period of time through a port
General anesthesia: medication that provides sleep and prevents one from feeling pain
Heparin flush: medication given to keep blood from clotting and blocking the inside of the port
Non-coring needle: a special needle with a flattened, long tip designed to prevent damage to the port. The needle is bent so that it’s flat against the skin when it’s inserted.
Port: the device placed under the skin to allow medication and fluid to be infused directly into the bloodstream
Septum: area on the top of port where needle enters
STERI-STRIPS: thin bandages covering the incision.
Subcutaneous: layer of tissue underneath the top layer of skin

My sister-in-law (my husband’s brother’s wife) is due to have my new niece any day now. We were communicating a couple of months ago about using my niece’s cord blood as a possible transplant for Hannah. Unfortunately, the cord blood company needed 45 days notice before her due date to get it set up, and I just couldn’t get an answer whether or not it could be a possible help. She is due to give birth any second now, literally, and is scheduled to be induced on Thursday.

I just found out that she has been communicating with Dr. W., the specialist back east who I have nicknamed “Hannah’s guardian angel” because he has gone above and beyond in trying to educate me and to help Hannah and hooked us up with Dr. S., the specialist we are seeing next month in Dallas. She never mentioned Hannah by name initially, but said she was asking about her 5-month-old niece. He put two and two together.

Once he realized they were both talking about Hannah, here was his response:

Carrie and I have been doing a lot of corresponding about Hannah. I am happy and honored to be able to play a role even if just by email. I (and everyone else) are hoping that Hannah will not have neuronopathic GD. Were that to be the case, I doubt that a cord blood infusion/transplantation would do much to alter the course. If Hannah has type 1 Gaucher, she should respond well to standard enzyme therapy. However, I really have no expertise in the use of cord blood in general, or as relates to Gaucher disease. When Carrie and Hannah see Dr. S., perhaps he can also address that question. I will however continue to research the matter as it is certainly an important consideration.

Thank you to my fantastic sister-in-law for continuing to fight for Hannah, even through her constant contractions!
Thank you to Dr. W. for continuing to be such a source of security and knowledge for Hannah. To say you would be “honored” to be involved is more than I could ever hope for!