Corrigan had a well-visit at Hopkins yesterday. The trip was smooth, the baby well-behaved and his ammonia was 59. His branch chains came back on the high-end of normal which indicated that either his protein should be lowered or his medicine increased. The doctor increased his medicine (both) quite a bit and only lowered his protein about a half gram/day. Corrigan is already so protein restricted that they always hesitate to lower his protein unless really needed. For the first time in perhaps his entire little life he did NOT gain the expected amount of weight (or, normally, more) for the time period since last check-up. The expected weight-gain was 240 grams and Little C gained 130 grams. He did however manage to eek out some growing over the last weeks adding 1.2 cm to his length and 1.1 cm was expected. wooo hoo!
I have been gathering data and medical records recently and was given the Hopkins records of Corrigan’s initial admission on day 6 of age. Reading those records on the way home from Baltimore was eye-opening. There were so many things that I was either unaware of or forgot in the midst of the chaos. Reading the clinical information, so cold and unemotional, was almost too much to bear. I knew our baby was very sick. I knew that he was going to have to fight hard (and boy did he!) but I did not know some of the details.
I do not want to dwell on those bad memories any longer though. They are memories better left behind, perhaps pulled out in joyful comparison on special days like graduation (“Can you believe that you almost died but now you are going to WVU to play football?”) or on the day he is sworn in as President. Maybe I will read over these notes on birthdays, alone in the evening, just to keep myself grounded in how incredibly blessed we are to still have Corrigan. So that I never ever take for granted what a miracle he is…I will keep them but not dwell on them.
One of the main reason I wanted to do a blog was to connect with other families that are living with Urea Cycle Disorders and it has brought several amazing families into my life. Sadly, more will come in the months and years ahead and they will get online to try and grasp any handle of normalcy that they can find. The will find themselves overwhelmed with the factual/medical information. They will cry in their hearts at what the expected outcome is for this disorder. They will lay awake at night wondering what damage the ammonia has done to their child’s brain and whether their hopes and dreams for their child might changed. They will find the National Urea Cycles Disorder Foundation and will be able to learn more about the “life” side of things and then, maybe, they will find me. Maybe they will open the link to this blog and see a beautiful little miracle and be encouraged. Maybe our path will clear the way for someone else…just like The Kulina’s( http://taleof2cs.blogspot.com/ ) path cleared the way for us.
I want people to come here to be encouraged but I also want to be “real.” I do not want to candy-coat how bad it sucks to have a child that suffers the effects of a UCD and how hard it is for the entire family…but overall I think that “encouraging” is what I hope for. That being said, in reading the medical files from three hospitals and going through my own calendars and datebooks I have compiled some information regarding how hard the first year of life is on a family from a data standpoint. This might not be interesting to anyone not living with a UCD and this is ceratinly not promised as the same result a UCD family will have. I have found, through Facebook mainly, that many many UCD familes are sooo fortunate and their children do not seem to get sick nearly as often as Corrigan has and that is GOOD. What I want to put down on e-paper, both for my own records and to share with others beginning their journey is just the timeline of our first 14 months with this situation.
When a baby is born with a UCD the metabolic physicians want to see your child a lot. Weekly. I have heard that some are bi-weekly. TWICE. A. WEEK. We were fortunate that we did once a week for a long time and then moved to every 2 weeks. Now, thankfully Corrigan has stabilized a bit, prob. due to age, and we see them every 3-4 weeks. It is really important that the child be measured precisely and that accurate ammonia and plasma-aminos obtained. I hope that a family that receives this diagnosis lives close to a good hospital. We were told, very early on, that we should pack up and move closer to Johns Hopkins. We worked hard, putting in a lot of time on the road, to be able to stay here among friends and family. Support systems are so crucial. Without our family, and friends and church family we would have been emotionally sunk. Luckily, we live 143.71 miles from one of the best genetics programs in the country. However, thats a lot of driving.
Clinic appointments (well-visits):
We had 26 scheduled appointments at the PCRU. These I compiled from my own calendars. However, it does not include the several times that, after Hopkins discharge for admission, we were sent to my Dad’s house ( a bit North of Baltimore ) rather than back home to the mountains and we had to be back in the hospital early the next morning before we could be released to come home. We saw a LOT of the Pediatric Childrens Research Unit (PCRU) the first year of Corrigan’s life.
Our appointment dates were as follows:
July 1, July 8, July 15, July 25, Aug 4, Aug 13, Aug 27, Sept 9, Sept 23, Sept 30, Oct 1, Oct 7, Oct 20, Nov 18, Nov 24, Dec 18, Jan 12,
Jan13, Feb 9, Feb 25, Mar 11, Apr 8, May 14, May 27, June 26, Aug 4.
You will notice that there were some months that we did not have four appointments. That prob. means that there was a hospitalization in there instead. Let me share the dates of hospitalizations too.
Initial admission, day 6 of life, transported by helicopter…highest ammonia 622 Hopkins recorded, hemodialysis performed. In hastily written notes, at the WV university hospital, I have 751 written, but the highest Hopkins recorded, after he got an infusion of arginine alone, before life flight, was 622 upon arrival in Baltimore.
August 13, 2008- regular clinic visit, no problems expected, blood results showed ammonia 356. Admitted to Hopkins.
November 16, 2008 (notice the AWESOME time between hospitalizations…3 MONTHS!…known as the “honeymoon period” with UCD babies) taken to local ER, ammonia 137, transported by helicopter to Hopkins
December 11, 2008- Taken to local ER after he vomits, ammonia 184, transported by ambulance to Hopkins
December 26, 2008 ( we asked God to let us get through Christmas, He gave us until 7am the next morning!) taken to ER locally, ammonia 131, transported to Hopkins
January 30, 2009 – vomited, taken to local ER, ammonia 99 then 133- then 90, transported by ambulance to Hopkins. (this was the time that he got gastroenteritis and also had his port surgery. This was an 8 day stay)
April 12, 2009- (Easter Sunday, sick on the way to church…threw up all over his Easter outfit) , taken to local ER, ammonia 149, transported by ambulance to Hopkins
April 30, 2009- vomiting, taken to local ER, ammonia 322, transported to Hopkins by ambulance.
May 19, 2009- vomiting, ammonia 84, given fluids and meds as precaution and kept locally at the Western maryland Health System(WMHS) (for the first time!) Discharged in 24 hours.
May 29, 2009- (the day after his first birthday) vomiting, taken to local ER, ammonia 160 and then 134, admitted locally at WMHS
July 3, 2009- vomiting, ammonia 90, admitted locally at WMHS, discharged after 2 days ( missed the 4th of July parties/fireworks..boo)
My greatest fear (if you have read the blog from the start) has always been that I would not know when he was sick. Blessedly, Corrigan’s body lets us know when he is toxic from ammonia by making him vomit. I know that this is not the case with all patients. When he was a little baby his body tolerated much higher numbers of ammonia (read above) before he would hurl and we would race him to the ER. However now, as evidenced by his last hospitalizations, he is getting sicker sooner. He now vomits around 80. We are so thankful for that because numbers over 100 are so dangerous and as it increased closer to 150-200 things can get bad. We always remind ourselves that Corrigan could be 6 hours from coma or death…it’s what spurs us on (now) the the ER every. single. time. he. pukes. No questions, we go. Honestly, that is not always true. Twice I thought we should wait, I thought he might have just gagged himself with his hand and Mark has demanded we go anyway and both times he was right. (thanks Mark)
There have been two false alarms though in the last year. Early that first November he seemed “off” and this was before I felt truly confident that I would “know” and he was fine. Slightly dehydrated but ammonia was 43. We were sent home. Also, in March of 2009 he seemed “off” again and did not vomit. We have come to rely on puke as our definitive proof and yet we didnt want to take a chance considering the late winter/early spring troubles he had already had, but when we got there he was good on ammonia but he DID have a double ear infection, that if left untreated would have caused him to get citru-sick ( hyperammonemic) within a few days anyway so it was a false-alarm but a good trip in.
Trust your gut.
okay so here are the numbers,
26 Baltimore appointments and 7 trips down chasing our baby in an ambulance or helicopter. 33 trips that I know of, for certain.
Mapquest tells me that it is 143.71 miles from my driveway to the front door of Johns Hopkins. That is 4742.43 miles on the road in the last 14 months. That does not include the many times that Mark and I raced down behind the ambulance and he would leave me to stay with the baby and he would have to drive back home, late that night, to stay with our oldest and go to work. The longest stay, in Feb. he drove three separate trips..the initial ride down and back to drop me off, then back down and back again mid-week when Corrigan had his port surgery and then later in the week to pick us up and bring us home. I did not count those. Nor does it count the times that we had to go north to my Dad’s and then back into Baltimore in the morning.
That is approx. 237.1 gallons of gas. The summer that Cor was born, 2008, gas was $4.00 a gallon but now it is around $2.55 so I don’t know how I should calculate that expense.
Just going by MapQuest and the 33 trips from above we have spent 5082 minutes traveling, or better shown, EIGHTY-FIVE HOURS, driving…most times with a screaming infant that hated the car and a husband that was, bless his heart, trying to work on his laptop, handling two phone lines while I drove. The time, in car, does not include the time we had to spend pulling over to change the baby, or let him eat, or get him out and walk him to sleep because he was losing his mind….nor the time we have spent stuck in traffic. Just the basics.
It was all worth it.
I mean, it’s overwhelming. I won’t lie. And the only time the trip has been good has when the rest of life has been stressful. Since Mark has been out of work, since 6/10 he has been able to come to the ER when the baby is unwell. Before, I had to do the long 8-10 hour stays in the local ER waiting on an ambulance and then sometimes drive alone to Baltimore…now? Now, Mark comes willingly and gladly with us. He spends it all with us even though he could find excuse not to. And those trips to Baltimore? Piece ‘o cake now that Corrigan is a better passenger and we don’t have to stress out that Mark’s customers might find him unprofessional working in a noisy car with screaming infant behind his head. Life is stressful in other ways now…but having Mark along for the Citrullinemia stuff is so awesome. I don’t know how I did it without him before…I dread the thought of doing it alone again but I know he wants a job, he wants to work and provide so I will just enjoy the company and in-person support while I have it.
So those are the numbers for our family. Boring to most but perhaps someone with a new diagnosis will be able to look at it and initially be overwhelmed but realize that it did not keep us from living a somewhat regular life. Modified, for sure…but we had joy. We had holidays..we had celebrations…and yeah, sometimes we had to celebrate a weekend later but we still celebrated. Citrullinemia just makes you realize that holidays are dates on calendars but the celebration counts no matter what day you do it.
and last night I celebrated Corrigan. His existence. On a Tuesday night. On no particular day of importance. Just because.