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Tuesday, January 27, 2015

Medical Update

So it has been a while since we have given you a medical update on Ian. He is doing ok. We did just recently have a scare where we thought he had a trach infection. The culture came back growing lots of stuff, all of which were things they were expecting to come back with. Many thoughts and emotions were going through our minds, knowing he may have one and what that could or could not mean. 

He is becoming increasing harder to understand, especially at night when he is more fatigued. His eyes have been one of his greatest tools lately in communicating. 

Increased pain has been something else we have been struggling with. He has been getting his break through pain medicine more often, sometimes more then once a day. So when his Gilchrist team meets next they will discuss his and most likely increase his methadone. 

Eating has been something he has not been doing so much of. We have started a food journal to see what his intake really is knowing that he does not need what you and I would need. Laying in bed and not exerting yourself does not require much but does it require more then he is getting. Once we get the results from the food journal, if he is not getting enough, Brian and I will then have to decide if we are going to just let him continue to eat the way he is or going to start some tube feedings. Both have their pros and cons and this will be yet another difficult decision we will need to face.

Last night, Ian needed to get out some frustrations so he yelled, I HATE THIS DISEASE....we could not agree with him more.


Saturday, January 24, 2015

My Beautiful Boy

It hit me the other day, Ian is dying. As this hit me...I sat there wondering how it is possible that it is just hitting me...Ian is dying. Ian has been dying, slowly over time, slowly over time. I am not sure what brought me more to this realization yesterday, but it hit me right in the face, SMACK. 

I decided to look up the signs of death. According to caring.com, there are 10 signs death is near. These signs of approaching death are specific to the natural dying process (apart from the effects of particular illnesses the person may have). Not all symptoms show up in every person, but most people experience some combination of the following:

  • Loss of appetite - Energy needs decline. The person may begin to resist or refuse meals and liquids, or accept only small amounts of bland foods (such as hot cereals). Meat, which is hard to digest, may be refused first. Even favorite foods hold little appeal. How to respond - Don't force feed; follow the person's cues even though you may be distressed by a loss of interest in eating. Periodically offer ice chips, a popsicle, or sips or water.Use a moistened warm cloth around the mouth and apply balm to the lips to keep them moist and comfortable.
  • Excessive fatigue and sleep - The person may begin to sleep the majority of the day and night as metabolism slows and the decline in food and water contribute to dehydration. He or she becomes difficult to rouse from sleep. The fatigue is so pronounced that awareness of immediate surroundings begins to drift. How to respond - Permit sleep. Avoid jostling the person awake. Assume that everything you say can be heard, as the sense of hearing is thought to persist, even when the person is unconscious, in a coma, or otherwise not responsive. 
  • Increased physical weakness - A decline in food intake and lack of energy leads to less energy, even for activities like lifting one's head or shifting in bed. The person may even have difficulty sipping from a straw. How to respond - Focus on keeping the person comfortable. 
  • Mental confusion or disorientation - Organs begin to fail, including the brain. Higher-order consciousness tends to change. "Few conditions leave people hyperaware when they're dying," says palliative-care physician Ira Byock, author of Dying Well. The person may not be aware of where he or she is or who else is in the room, may speak or reply less often, may respond to people who can't be seen in the room by others, may seem to say nonsensical things, may be confused about time, or may act restless and pick at bed linens. How to respond - Remain calm and reassuring. Speak to the person softly, and identify yourself when you approach.
  • Labored breathing - Breath intakes and exhales become raggedy, irregular, and labored. A distinctive pattern called Cheyne-Stokes respiration might be heard: a loud, deep inhalation is followed by a pause of not breathing (apnea) for between five seconds to as long as a full minute, before a loud, deep breath resumes and again slowly peters out. Sometimes excessive secretions create loud, gurling inhalations and exhalations that some people call a "death rattle." How to respond - The stopped breathing or loud rattle can be alarming to listeners, but the dying person is unaware of this changed breathing; focus on overall comfort. Positions that may help: the head slightly elevated with a pillow, sitting up well-supported, or the head or lying body tilted to the side slightly. Moisten the mouth with a wet cloth and moisturize with lip balm or petroleum jelly. If there's a lot phlegm, allow it to drain naturally from the mouth, since auctioning it out can increase its quality. A vaporizer in the room might help. Some people are given oxygen for comfort. Br a calm, physical presence, stroking the arm or speaking softly. 
  • Social withdrawal - As the body shuts down, the dying person may gradually lose interest in those nearby. He or she may stop talking or mutter unintelligibly, stop responding to questions, or simply turn away. A few days before receding socially for the last time, the dying person sometimes surprises loved ones with an unexpected burst of alert, attentive behavior. This can last less than an hour or up to a full day. How to respond - Be aware that this is a natural part of the dying process and not a reflection of your relationship. Maintain a physical presence by touching the dying person and continuing to talk, if it feels appropriate, without demanding anything back. Treasure an alert interlude if and when it occurs, because it's almost always fleeting. 
  • Changes in urination - Little going in (as the person loses interest in food and drink) means little coming out. Dropping blood pressure, part of the dying process (and therefore not treated at this point, in tandem with other symptoms), also contributes to the kidneys shutting down. The concentrated urine is brownish, reddish, or tea-colored. Loss of bladder and bowel control may happen late in the dying process. How to respond - Hospice medical staff sometimes decides that a catheter is necessary,  although not in the final hours of life. Kidney failure can increase blood toxins and contribute to a peaceful coma before death. Add a bed pad when placing fresh sheets. 
  • Swelling in the feet and ankles - As the kidneys are less able to process bodily fluids, they can accumulate and get deposited in areas of the body away from the heart, in the feet and ankles especially. These places, and sometimes also the hands, face, or feet, take on a swollen, puffy appearance. How to respond - Usually no special treatment (such as diuretics) is given when the swelling seems directly related to the dying process. (The swelling is the result of the natural death process, not its cause).
  • Coolness in the tips of the fingers and toes - in the hours or minutes before death, blood circulation draws from the periphery of the body to help vital organs. As this happens, the extremities (hands, feet, fingers, toes) become notably cooler. Nail beds may also look more pale, or bluish. How to respond - A warm blanket can keep the person comfortable, or be or she may be oblivious. The person may complain about the weight of the coverings on the legs, so keep them loose.
  • Mottled veins - Skin that had been uniformly pale or ashen develops a distinctive pattern of purplish/reddish/bluish mottling as one of the later signs of death approaching. This is the result of reduced circulation. It may be seen first on the soles of the feet. How to respond - No special steps need to be taken.
NOTE - THESE GENERAL SIGNS OF IMPENDING DEATH CAN VARY IN SEQUENCE AND COMBINATION FROM PERSON TO PERSON. IF A PERSON IS OF LIFE SUPPORT (RESPIRATOR, FEEDING TUBE), THE PROCESS DYING FOLLOWS CAN BE DIFFERENT. THE SIGNS OF DEATH LISTED HERE DESCRIBE A NATURAL DYING PROCESS.

Thinking back to the last few days, I realized this hit me because of a conversation I had...a hard conversation but a good one to say the least. Through this conversation I realized a few things and wanted to find out more such what signs of the dying process. When someone begins the dying process it doesn't mean that it is today, it doesn't meant that it is tomorrow or it doesn't mean that it is in a couple of weeks or months, although it could mean that. Looking at the signs above, Ian has started some of them...he goes back and forth with his eating, some days he will eat one meal and that would not even be a meal for you and I, sometimes we have the excessive fatigue and sleep, and occasionally the social withdrawal, the smiles are not there as often and neither is the sparkle in his eyes. 

One of the other things I realized through this conversation is when someone dies their physical presence is no longer with us but their soul is. According to Chabad.org, the soul is the self, the "I" that inhabits the body and acts through it. Without the soul, the body is like a light bulb without electricity, a computer without the software, a space suit with no astronaut inside. With the introduction of the soul, the body acquires life, sight and hearing, thought and speech, intelligence and emotions, will and desire, personality and identity. Realizing this has helped me with my journey in acceptance and being "okay" with the process.

Remember treasure, dream and live.

Tuesday, January 20, 2015

Court of Awards

Tonight Brian and I were both able to go to Becca's Court of Awards for Girl Scouts. Thanks Mark and Mary. Over the past few months, Becca along with her fellow Cadettes (6th-8th graders) have worked extremely hard to earn their It's Your Plant - Love It! Breathe Journey. In order to earn this they needed to do the following (from the Girl Scouts of Central Maryland site):

  • Cadettes engage all five senses as they clear the air - their own and Earth's.
  • Girls learn to assess air quality inside and out, getting an aerial view of everything from cigarette smoking to noise in the air to deforestation.
  • Along the way, they try some scientific experiments (that could even double as magic tricks to share with Brownies).
  • As they enjoy creating some "breathing room" in their lives, Cadettes may also find a new flair, think about "Hair," and perhaps even try making an eclair.
  • After becoming more Aware, the girls go on to Alert others to act for air, and then Affirm their impact on Earth, as they add these three uplifting leadership awards to their collection of Girl Scout honors.
Along this Journey, Cadettes have the opportunity to earn three leadership awards that engage them in improving the world's air quality while also supporting and nourishing their own abilities as leaders who are aware, alert, and able to affirm all they do.
Aware
  • Keep an Air Log throughout the Journey
  • Identify two experts who can guide you to greater air awareness.
  • Increase your AWAREness about the issues that impact Earth's air.
  • Decide the most important, personal reason you care about Earth's air.
  • With you Cadette team, choose an air issue to act on together.
Alert
  • Decide whom to educate and inspire - this is your Air Care Team (ACT)!
  • Decide what you will ask your Air Care Team to do.
  • Decide how to reach your Air Care Team to inspire them to act on your air issues
  • Educate and inspire! Give your ACT its call to action.
Affirm
  • Gather proof of progress or improvement through your efforts to educate and inspire.
  • Share the impact with your ACT and maybe even go further.
  • Get with your Cadette team and reflect on your efforts and their impact.
  • Affirm your commitment to strive to be an heir apparent of air and all of Plant Earth's elements.
The Journey Award which will go on her vest.
In order to meet the above requirements the girls made a book with information on environmental friendly cleaners as well as how to make them. 
In addition, the girls made a video showing all the important things they learned as well made a few of the environmental friendly cleaners. 

The girls all got their participation patches. ..and yes Becca is an overachiever and likes to do it all. 
All of Becca's participation patches.
The girls getting their Journey Award.
Overall the troop did very well with their cookie sales, selling over 3000 boxes. Becca did extremely well...thank you to all who supported her, she was top seller. 

Looking forward to the rest of this year for scouts. 


Wednesday, January 14, 2015

Not Contagious

Yesterday, Becca came home to share a story with me which I want to be able to share with everyone regarding the importance of education and knowledge. I don't know how the whole situation came about. Becca told me she was talking with a boy in her health class, something came up and they stated discussing it. He then turned around to her and said something to the affect of "what do you have your brother's disease?" I am not sure what this child intended by his comment but it was hurtful to Becca. Becca does know she is a carrier of the VRK1 mutation, however, I wanted to see what else she knew and understood. I realized that Becca does not completely understand what it means to be a carrier compared to having the disease, compared to being contagious. Ian's disease is not contagious, it is not spread from one person to another, others can not get it by being near him, touching him or kissing him. VRK1 is an autosomal recessive inheritance disease.  

So I figured if Becca did not really understand the whole thing and she lives with it as well as having had many different discussions regarding VRK1...others don't either. I am hoping a better understanding will be had by many after reading this post and perhaps parents can share with kids, so maybe, just maybe it will spare Becca from even one more child coming up to her with a comment such as "what do you have your brother's disease".

After doing some research on line, I went right to a reliable source...Dr. Julie Kaplan, a pediatric geneticists, and my wonderful cousin. I wanted a very simple or as simple a way as possible to be able to explain so here we go:

In order to discuss these types of inheritance, first some basic genetics. Genes are the blueprints that our bodies use to grow and function. Genes are carried on tiny structures called chromosomes that are found in every cell in our bodies. We have thousands of genes on 46 chromosomes, which come in pairs. The first 22 pairs are called autosomes. The 23rd pair is called the sex chromosomes. This pair determines if a child is a boy or a girl. Girls have two X chromosomes and boys have an X and a Y chromosome. For each pair of chromosomes, we receive one copy from our mother and one copy from our father.

One possible type of inheritance is autosomal recessive. In autosomal recessive conditions, both copies of the gene that causes the condition must have a change or mutation that causes them to not work correctly. Therefore, a child with a recessive condition must have inherited a non-working copy of the gene from each parent. The parents are called carriers because they have one non-working copy of the gene and one copy of the gene that works correctly. Because carriers have one working copy of the gene, they DO NOT have the condition. With each, pregnancy, there is a 25% change that both parents will pass on their non-working copy and the child will have the condition. There is a 50% chance that only one parent will pass on their non-working copy of the gene while the other parent passes on their working copy. In this case, the child will be a healthy carrier like the parents (BECCA). And, there is a 25% chance that both parents will pass on their working copies in which case the child will be healthy and will also not be a carrier.
Autosomal recessive inheritance
Take from the MDA Website

I hope this is broken down enough for everyone to understand and those with children directly related to our children are willing to sit down and explain the process to their kids...Ian's disease is not contagious. You can touch him, visit our home, touch Becca, Brian or myself and you will NOT get it. 

Tuesday, January 13, 2015

Living with a Rare Disease - DAY-BY-DAY, HAND-IN-HAND

The theme of this years World Rare Disease Day is LIVING WITH A RARE DISEASE

Rare Diseases have enormous repercussions for the whole family. Living with a rare disease becomes a daily learning experience for patients and families. Though they have different names and different symptoms, rare diseases impact the daily lives of patients and families in similar ways. How to find a diagnosis, how to access treatments, how to find appropriate expertise, how to work with a team of caregivers, how to operate special equipment, how to administer treatments, how to identify and access social services, how to manage the economic burden of living with a rare disease and how to ensure the well-being of the entire family and balance priorities...just to name a few. 

Last year, I asked everyone to wear either a Blue Denim Genes Ribbon(TM) or their jeans and send me photos...I want to do the same this year...but I want to beat last year with more jeans (we had 44 least year) ...whose in....I hope you are. Need a ribbon, let me know I will get them to you. Need to know how to get me photos, just ask, we will figure it out. While wearing your jeans make sure to tell those around you why you are wearing them. Let's raise awareness for Ian , our family and others like us who are impacted by rare diseases.  

The Global Genes Blue Denim Genes Ribbon™Rare Disease Day email banner
So mark your calendars to wear jeans on February 28, 2015 for World Rare Disease Day to help raise awareness HAND-IN-HAND.

Saturday, January 10, 2015

Grief and Mourning

A month or two ago, Ian started talking more and more about Heaven. Asking questions about what was there, stating he was ok going there. This led to some interesting conversations with our Gilchrist Social Worker, helping us to prepare more for the inevitable. 

I have been doing some self-help reading regarding healing a grieving heart. Throughout this journey, I have been grieving on and off for the child I dreamed of having and the dreams he would have, which have led to different dreams. In addition, the book HEALING A PARENT'S GRIEVING HEART by ALAN D. WOLFELT, PH.D. was given to Brian and I. This led me to HEALING YOUR GRIEVING HEART FOR KIDS and HEALING A CHILD'S GRIEVING HEART. All have given me insight to some things which we have already dealt with as well as things which we will face in the future.

LEARN THAT GRIEF AND MOURNING ARE DIFFERENT
Grief is what you think and feel inside when someone you love dies. When you're in grief, you're grieving. It is the constellation of internal thoughts and feelings we experience when someone we love dies.  

Mourning means letting those thoughts and feelings come out somehow. It is the outward expression of our grief. Mourning is necessary for healing to take place. Wolfelt refers to children as "forgotten mourners." Because though all children grieve when someone loved dies, we (as a society, as families and often as individuals) rarely encourage them to mourn.

According to Wolfelt, there are 6 needs of mourning:

NEED 1: Accept the death; acknowledge the reality of the death. This is the most difficult thing to acknowledge and accept, do it bit by bit. And it will take time. 

NEED 2: Let yourself feel sad; feel the pain of the loss. You need to feel sad, but still have to remember that you have to laugh. Go for a walk, read a book, watch TV, talk to supportive friends and family about the normal things of everyday life. 

NEED 3: Remember the person who died. When someone loved dies, they live on in us through memory. It's good to talk about the person, look at photos, watch video. Remembering the past makes hoping for the future possible. 

NEED 4: Accept that your life is different now; develop a new self-identity. The way you defined yourself and the way society defines you is changed. You will need to re-anchor yourself, to reconstruct your self-identiy.  Part of who we are is formed by the person who passes away. No one can "fill in" for the person who dies. Supportive relationships - yes. Replacements - no.

NEED 5: Think about why this happened; searching for meaning. When someone we love dies, it is natural to question the meaning and purpose of life, the why? Losing a child does not follow the natural course of life, these questions are particularly painful when this happens. Don't try to have all the answers to all the questions about the meaning of life. It is ok - even desirable - to admit that you struggle with the same issues. 

NEED 6: Let others help you, now and always; receive ongoing support from others. Per Alan Wolfelt, in Healing a Parent's Grieving Heart..,"As mourners, we need the love and understanding of others if we are to heal. Don't feel ashamed by your heightened dependence on others right now. If the death was recent, you may feel the need to be around people all the time. You may need to talk about the death often. You may need help getting meals together, doing laundry, completing paperwork. Don't feel bad about this. Instead, take comfort in the knowledge that others care about you. Unfortunately, our society places too much value on "carrying on" and "doing well" after a death. So, many mourners are abandoned by their friends and family soon after death. If the death of your child was long ago, you may have experienced this abandonment firsthand. Keep in mind the rule of thirds: one-third of your friends will be supportive of your need to mourn, one-third will make you feel worse, and one-third will neither help nor hinder. Grief is experienced in "doses" over years, not quickly and efficiently, and you will need the continued support of your friends and family for weeks, months and years. If you are not getting this support, ask for it. Usually people are more than willing to help-they just don't have any idea what to do (and what not to do)." In Healing a Child's Grieving Heart, he says "Grief is a process-not an event. Children, like adults, will grieve long after the person has died. The grieving child needs your compassionate support and presence not only in the days and weeks following the death, but in the months and years to come. As they grow and mature developmentally, children will naturally grieve the death on new and ever deeper levels. If you can help the grieving child mourn as the need arises (even years after the death), you will be helping her grow into a healthy, loving adult."

It is important to realize that the steps above will not happen in a particular order and may happen more then once. 

While reading this information, I have realized we have been grieving for a long time...even though Ian is still with us. We have been grieving for the dreams we had for him, we have been grieving for the younger Ian (the one who would hold our hand walking while trying to keep up with his friends, the one who would get himself out of bed and sit and slide into our room because his walking had started to regress, the one who would stand in the corner and yell at his speech therapist as loud as he could for her to hear him across the room, for the one who had his friends over playing the way boys play with their friends, the one who will graduate from high school, will have his first girlfriend, graduate college and walk down the aisle to his future wife and have his own children). While grieving the dreams we had, new dreams surfaced....the one for him to continue his schooling, the one for him to be able to go to sleep away camp, the one for him to have a wonderful quality of life with the time he has left, the one for him to be as pain free as his journey continues and the one for him to be at peace with his journey. Number 6 has been one which we have been doing on and off over the years with and with out the help of our village, I know some of you have been doing this as well....we know some don't realize we need these things now and have been going through all of this yet but we do and have. The support you have all given us has been wonderful and appreciated. I am asking for continued support over the years. I am asking for those who would like to come over and hang out to please do so...come play on the Wii with Ian, come watch a movie with us, come sit and chat about life in general...those are the things we need now. We rarely get out with Ian, someone is always home with him...the weekends tend to be the time when we could use the most help. We look forward to continuing our journey....

Wednesday, January 7, 2015

A Proud Becca Moment

I got a wonderful message from one of Becca's teachers today. Since starting a new school this year she has decided who and when she will share information about her family.  This is the message from her teacher...

"Becca really opened up in class today when participating.  My guess is she is feeling really comfortable and was just really speaking freely.  We were talking about the parts of culture for our new (very Social Studies-ish unit) and trying to come up with examples for how each element of culture is seen in America.  When we got to "technology" the topic of wifi came up and I explained how I helped my mom over break set up her new computer, learn how to email and connect to wifi.  The students thought it was funny my mom had trouble understanding what wifi was.  Becca shared that her grandparents are very on top of the new technology and use it when they travel and use it to communicate like skyping. Then when we discussed "Social Structure" and had to list who is valued and not valued we talked about how just because society places value on one thing that doesn't mean it is right. Like how pro athletes get paid millions but firefighters, police and teachers do not. Becca then told the class that she wanted to share with class her personal experience having a family member with a disability and how they are often not valued as much as they should be.  She was so relaxed and honested when she shared and the kids reacted so positively that it was just a great moment.  I wanted to share this with you just to let you know that she really seems to be coming to her own lately.  I was really proud of her!"

Way to go Becca....learning these things is great...we love you and are proud of you.