Drum Roll Please....

Sunday, December 12, 2010

(Bonomo Family - Christmas 2010)

Before I get to the good part - A little sneak peek at our 2010 Christmas Pictures. As most of you know we have the most amazing friend and photographer - Jennifer Bagwell. She has such instinct for photography and she is absolutely amazing with Kennedy and all of her special needs. She is always going out of her way to accommodate our family. Jen remains one of Kennedy's biggest advocates. She is always lending me an ear when I need to vent or just talk out my struggles and for that I am forever grateful. She is never judgmental and is always telling me how amazing and inspirational I am, which I won't lie is so nice to hear at times. She even named her daughter after Kennedy - Isabella Kennedy Bagwell. Anyways check out more of her work by clicking here!!!! Our pictures turned out amazing - I will post the rest of them in another post.

For the longest time Brian and I have battled with the decision of growing our family. Due to the fact that Kennedy's disease has a 25% reoccurrence rate we felt torn. We knew we wanted to expand upon our family, but also knew the inherent risks that would lie ahead. We did not want to bring another baby into this world that would be afflicted with same heinous disease as Kennedy! With a clear heart we could not consciously go ahead knowing the potientially negative outcome that may result.

We pondered and pondered for what felt like forever. Not only would the decision to have another baby affect both of us individually, but it would also affect Kennedy and our entire family dynamic. Could we handle it? Would it be feasible? Would I be asking to much of my husband? The questions continually flowed through my brain. It is times like these that I wish there was some sort of crystal ball I could ask. So I did the next best thing - I looked within and to God for guidance.

We finally decided to go for it! We knew that adding to our family was very important to us. The fact of the matter is - we had Kennedy because we wanted a family and just because Kennedy has some special needs it does not take away from our initial desires of wanting to grow our family.

We are so incredibly elated to announce that Kennedy will be a Big Sister!!! We are anticipating the arrival of Kennedy's Baby Brother around May 8, 2011. Words can not describe the emotions that I have been overcome with in the last 5 months since I found out I was pregnant. I am ecstatic that Kennedy will be able to take on the roll as big sister. We are anxiously awaiting the arrival of our little bundle of joy and are accepting of the impending challenges with open arms. Because we do not know with 100% certainty that Kennedy has Leigh's Syndrome (doctors have been unable to confirm diagnosis via blood) we will not know if this baby will be afflicted. At this time I put all my faith into the Lords hands and hope and pray for the best. I do not have any inclinations or instinctual feelings that anything is wrong at this point but I approach this as I try to approach everything else in my life, full of positivity and optimism.

(Our newest addition - Baby Boy Bonomo due May 8, 2011)

A New You

Sunday, November 28, 2010
Life is ever changing. It becomes very hard to get accustomed and develop a routine when what you thought was "normal" yesterday has become a thing of the past twenty-four hour later. We have experienced a rough couple of months. Back at the end of September Kennedy was becoming very irritable, crying non stop, and showing signs of respiratory distress. It appeared like we were in for the long haul. We immediately made an appointment with her Pediatrician and were told based off Kennedy's increased irritability, breath sounds (wheezing and crackling), and green secretions - Pneumonia was a brewing. We were prescribed antibiotics and some inhaled steroids with the hopes we could kick this before it got out of control.

It seemed to take about five days before Kennedy began to feel a little better. I was in shock how fast we were able to get this cleared up - or so I thought! Several days later she took a downward spiral. She was having severe chest retractions (which for a child with no muscle tone is almost impossible to do) and her saturations were holding steady in the low 67-71 range. To put this in perspective, Kennedy's normal resting saturation levels have always been 91-93. I began to panic a little. I made the call to increase her oxygen levels to 1LPM and have her on it around-the-clock.

In the meantime, I called her Pediatrician and we were able to get an appointment that afternoon. Kennedy's secretions had subsided, however, she was visibly struggling to breath, exhibiting constant grunting with every breath, extremely irritable, and looked completely wiped out. We were sent immediately to the lab for a chest x-ray which showed severe left lung atelectasis. Which just means that her alveoli had collapsed, thus allowing for inadequate gas exchange and decreased saturation levels. In addition, her left middle lung showed extreme haziness on the film, so much so that her heart was not visible through her left lung. This was a sign that the pneumonia was not cleared with the first round of antibiotics. We consulted with her doctor several hours later and were prescribed stronger antibiotics, more inhaled steroids, and a decongestant.

We discussed the plan of action should Kennedy take a turn for the worse. It is well known by all of Kennedy's doctors that we are against going back to the hospital. Under no circumstance will we ever admit Kennedy back into the hospital. We have DNR's signed and a pallate of care set forth. My doctor is extremely supportive of our decisions. In addition, if need be he could have a doctor out to our house if the situation progressed further. Kennedy follows the saying, "slow and steady wins the race". It appeared that we did it. Kennedy fought off pneumonia again (oh her poor lungs must look like a 90 year-old chronic smoker with severe emphysema).

Even though Kennedy was able to successfully overcome this bout of Pneumonia it has left its mark. Well actually, I don't know if this last sickness has left her transformed or if her overall disease has progressed more so, and thus, has left her changed forever. It is sad.....Sad to look back and see the little things she had slip away. We have watched the events of yesterday become a thing of the past. The Bonomo family has learned to adjust, to become flexible, to always expect the unexpected, and to arrive at the realization that tomorrow is not a guarantee. We are in constant transformation and at times when we feel like we can not take it any more, we are able to step back and look at our daughter and know that if she is able to wake-up and fight everyday then so can we!!!!!

I am Back!!!

Friday, July 30, 2010

After taking a nearly 5 month blogging sabbatical - I am back!!!! There have been numerous reasons for my break (I will get to those in subsequent posts)! Kennedy is doing great! She is growing and has managed to stay sick free! We reached a huge milestone since I last posted....On June 3rd 2010 we celebrated one year hospital free! I can still remember the night we were discharged. Due to the persistence and little bit of convincing by Laura (Kennedy's Primary PICU nurse) and I we were able to get Kennedy discharged only 72 hours after extubation. Almost every doctor in that hospital expected us to be back within the month and others assumed we were taking our baby home to die. But true as form - Kennedy had other plans. So I would like to say again - Congratulations Kennedy you are nothing short of a miracle.

Now on to the topic I am sure you are all waiting for.....We were consulting with several doctors down @ UCSD Rady Children's Hospital back in April. We were referred to an amazing doctor - leading a world renowned Mitochondrial and Metabolic Research Center. After our initial consultation we were certain that this was going to be it. We were finally going to be given the answers we so rightfully deserved. It was two days before our appointment and I received one of the most upsetting phone calls - the hospital had lost all of Kennedy's blood and had mishandled her urine! To make matters worse the lady proceeded to ask how soon we could get Kennedy back down to USCD to redraw the labs. Lets recap here.....the amount of blood that was required in order to run all of these tests was bordering the maximum amount of blood they typically withdrawal from someone Kennedy's age. For about a month after the blood was drawn Kennedy was paying the price. She was not herself and you could tell something was wrong. She was very irritable and having a lot of breathing issues (her Pediatrician figured they probably overdid it and these were the repercussions we had to face). Lucky for us it took about a month for Kennedy's body to recoup and bounce back. In addition, the urine that was some how mishandled took me over two weeks to collect. Lets just say trying to have a two year old with no muscle tone pee in a bag is no easy feat.

We have had several (more then I care to admit) vials of blood go missing, but why did it have to be these vials??? I was absolutely devastated that this had happened to us. I am normally very good at staying grounded and not putting all my eggs in one basket but this kind of broke me. I kind of wrapped my future plans into finding out the results of these lab tests. So as it stands today Kennedy is still diagnosed with Leigh's Syndrome with Encephalomyopathy based off her MRI results, but nothing has been confirmed via DNA. We will not be re-performing the lab tests. We have accepted that we were not meant to find out.

There were so many reasons I wanted answers, but the biggest one of them all was because I really would love to have another baby. These tests results would have allowed us to know exactly what the odds were of this happening again. My husband has made it abundantly clear that under no circumstance does he want to potentially have another baby like Kennedy and I do not blame him for feeling that way. I actually agree with my husband. It would be completely irresponsible and selfish of us to bring another life into this world knowing the odds that he/she could be afflicted with Leigh's Syndrome. As it stands right now we have a 1 in 4 chance of having another baby with Leigh's Syndrome. Those are extremely high odds and if it were to happen that we got pregnant and had another baby like Kennedy it would not be fair to us, Kennedy, or the new baby. I could never knowingly bring a baby into this world and have them be afflicted with this disease Kennedy has - never! Having a child like Kennedy is probably one of the most challenging things I will ever have to deal with in my entire life. It affects you mentally, spiritually, emotionally and physically.

At times you feel ostracized. Like you exist in this world all by yourself. The last 6 months have been extremely hard for me. Kennedy has digressed - she has a lot of issues managing her secretions. I have to suction her all the time and the night times are particularly a disaster. She is up every couple of hours and has increased seizure activity during the times she transitions from being awake to being asleep or vice versa. At times I surprise myself at how well I am able to adapt on such little sleep. I guess you do what you have to do - and I would do anything for my baby!

Closet of Dreams

Wednesday, March 24, 2010

We have received some exciting news in the last month. My sister is having her first baby and her boyfriend just got a job in California and will be moving down her in a couple weeks. While they are looking for a house they will be staying with us. In this exciting time in there lives I feel sadness. As I prepare my spare room for their arrival I come across the guest closet. It is never used and remains closed. Concealed behind the doors lies broken dreams, wishes, hopes, and a life of normalcy (something we do not often pay thanks for and is so often overlooked by others).

We were given no inclination that Kennedy was sick while I was pregnant. I went through my whole pregnancy being told by the "professionals" that everything was "normal". I had a typical baby shower - okay it was a big baby shower, but she was our first! I received tons of toys, clothes, baby essentials, jumper, walker, highchair, playpen, table/chairs etc....When it became apparent that Kennedy was far from "normal" we were forced to part with our dreams and aspirations we had created for our daughter. We painfully stored away all the gifts that were unusable to her (which was almost everything we received). The symbolism behind those doors is heartbreaking. The irrational side of me wanted to get rid of it all. I wanted no reminder of what I had lost and what I will never have with Kennedy. The logical commonsensical side thought towards the future and the what if's and someday' s . As usual the more rational side won out. It will always be hard to go through that stuff! But we continue to move forward and search-out the positives. I am determined that this disease will not beat us as a family. It has become an annoyance we live with daily but it will not come out on top.

We are still anxiously awaiting our follow-up appointment with Dr. Haas. Do I think we will get the results - the positive optimistic side says yes. I believe we will finally be rewarded with an answer after 20 long months of patiently wading through diagnosis after diagnosis. I know the results will tell me that Kennedy has a disease, either Leigh's Syndrome or Molybdenum Cofactor Deficiency and that it is NOT maternally inherited. On the other hand, the pessimistic cynical side (the feelings I think that most of us as a society would like to dismiss from our minds) feels there is a small possibility that the news bestowed upon our ears on April 20th will change our existence forever. Kennedy will have a maternally inherited disease and every child I ever have will be bequeathed with this heinous disorder. Only God knows but we will face April 20th with courage and strength. It is very comforting that no matter what we hear I will be supported by my husband, my mom, an amazing family, and awesome friends.

(Christmas 2009 - The Bonomo's)

The Strength of an Egg

Thursday, February 25, 2010

Parents of children with cancer, or really any serious condition, are often referred to or viewed as having strength "like a rock." Albiet flattering,it isn't quite true. It is more like the strength of an egg. An egg, you ask? Yes!

If you'll think about it, you'll see my point. An egg has a polished, smooth outer appearance, with no cracks or weak spots visible. It seems almost inconceivable that the inside might not be so smooth or solid.

Most children, at some point are shown the famous egg trick. An egg set at just the right angle can withstand enormous amounts of pressure and cannot be cracked or broken. Yet the same egg, tapped gently at an ever slightly different angle, will break. The contents, once so neatly concealed, will come spilling out. The no longer perfect shell will be crushed. It looks so fragile that it seems inconceivable that it ever held any strength.

A rock, on the other hand, is solid all the way through. To break it is almost impossible. If you succeed, you will find that there is nothing inside but more rock.

It takes a lot more than pure hardness to hold the hand of hope. Parents of [medically fragile] children are not solid all the way through. We hurt, we fear, we cry, we hope. It takes a very careful balancing act to keep the shell from being shattered. "Balancing an egg" while running a household, going for doctors' visits and hospital stays, keeping the family together, and holding on to the constantly unraveling ties of your sanity can be very tricky indeed! Occasionally, the angle will be off and the shell will break, shattering hope and all the neatly secured appearances of a truly fragile existence.

Unlike Humpty Dumpty, though, parents of [medically fragile] kids will pick themselves up and put themselves back together again.

~~ by Juliet Freitag ~~

Waiting

Thursday, February 25, 2010

The art of waiting......It has become a trait that I have learned to master. Waiting for test results, waiting for answers. At this time, waiting is a word that defines my life, my existence. While I lay in bed at night I eagerly wait for morning to come. Since Kennedy has been born I suffer with severe insomnia. It is so bad that I dread night time. I dread getting into bed and having to wait there for 6 hours. But why - is it the silence, the peace, the calmness? I do not know.

Maybe it is because the night leaves me weak and vulnerable. Oh the things your mind can think of when it is idle - scary! And then it hits me, my daughter is dying and I don't know when, I don't know why, I don't even know what from (still waiting test confirmation). I wait. I wait for the day when just one moment, a split second will change my life forever. It will re-write my history and leave me a changed person. So, I wait.......

All in the Name of Love

Wednesday, February 10 , 2010

You may ask why we are still meeting with doctors and still trying to pinpoint what is plaguing Kennedy. I hear the comments and receive the stares more times then I would like to admit. But I stand behind our decision to fight on with our daughter. With the exception of a feeding tube, she breathes on her own and exists with this terminal disease she was inflicted with. Until she proves to me otherwise, my family and I will do everything in our means to keep Kennedy around. We have many limitations put in place for our daughter - a palette of care program setup and a D.N.R. established. To those who think I am playing god, I assure you I am not.

There must be something in the air, because it seems to be pregnancy season. As people share with me the news and excitement, there dreams, hopes, aspirations, wonder and curiosity of the new life they hold inside them I can't help, but to feel pain and sadness for myself and for my daughter. At this point in time I am unable to have another baby because of fear that I carry some maternally inherited defect that I could pass on to my offspring. This is the reason why I long to find out what is wrong with Kennedy. Her disease/syndrome holds so many answers. In essence, it is the key that will unlock my future.

Before I go into great detail about our appointment I would like to let everyone know how Kennedy is doing. She has been hospital free for over 8 months. This alone has been an amazing gift. Our family's number one goal is to keep Kennedy healthy. Brian and I could not have achieved such success with this goal if it was not for the help of my Mom! We have seen significant deterioration in the last 3 - 4 months. Kennedy is no longer able to manage her own secretions while sitting up. In addition, her seizures are back with a vengeance at night. Finally, her peripheral nervous system has become more damaged. She used to react to pain immediately, however, her reaction time as slowed immensely.

We were given this opportunity (by our Neurologist - who I think is amazing) to meet with a doctor down in San Diego. This doctor is head of UCSD's Mitochondrial and Metabolic Research Center. Dr. Haas seemed eager and willing to take on our case. He performed a very detailed examination (initial intake) on Kennedy, noting any and all abnormalities. We analyzed her current medication list and decided to add a couple more vitamins to Kennedy's Mito Cocktail. The following is Kennedy's new medication list:

• Lamictal - Used to control seizures
• Levocarnitine - helps to break fat from the food that we eat into energy
• Coenzyme Q10 - is a fat like substance present in every cell of your body
• Creatine Monohydrate - I guess he wants her to become a body builder (New Medication)
• B-100 - provides certain cofactors which are important to Mitochondrial reactions (New Medication)

Dr. Haas felt very confident that our Neurologists' diagnosis of Leigh's Syndrome with Encephalomyopathy was accurate, however, he could not rule out without any uncertainty a couple other disease/defects. The two other diagnosis we will be looking at are extremely rare. There is not much research and/or case studies written on either.

• Creatine Synthesis Defects
• Molybdenum Cofactor Deficiency

A plethora of lab work was ordered and will take two trips down to San Diego to complete. We will have our first set of labs drawn on Saturday and will again travel down to San Diego the following week or two. In addition, we have scheduled our fresh enzyme muscle biopsy. As long as the lab work yields positive result(s) we will not need to proceed with the muscle biopsy.

We continue to travel down this road with positivity and optimism in hopes to find an answer. Every day I get to spend with my daughter has been a gift and I have been lucky enough to have been given 18 months and counting. And when the time comes for Kennedy to go to a better place, I will continue to forever cherish the time I was able to spend in her presence. She is worth every up and down, happy and sad emotion I have experienced - all the pain, sorrow, sadness, happiness, joy, excitement, anticipation, anxiousness etc....Being able to call her "my daughter" was worth it!

Simply Kennedy

Monday, February 1, 2010

It has come to my attention that I have neglected my blog for sometime now. So I am composing a great post, but to hold everyone over until it is complete here are some pictures I had taken of my precious Kennedy. These were done by a wonderful friend and an amazing photographer - Jennifer Bagwell. You can see more of her work by going to: http://www.jenniferbagwellphotography.com/