In My Heart Forever

A Completely Different Perspective

2010-11-06T15:08:00.000-07:00

Oh my dear Brennan,

I have come to terms with your death. Finally. Fifteen years after the fact.

I am grateful for the grace of God who's allowed me to realize while you are gone from my current life, you WILL be with me in the afterlife.

You never had to suffer the hardships that come with losing your innocence.

You never had to suffer humiliation and embarrassment. And me, being the over involved mother that I was, would have embarrassed you at some point. I delighted in activities like chaperoning junior high dances. While your brothers secretly enjoyed me being there, they acted like it was their worst nightmare. Haha. I kinda reveled in that.

So now instead of mourning you I embrace the joy you brought to me.

I embrace the lessons I have learned in the past 16 years. Having you in my life has made me a better woman. Losing you has made me a better Christian. Everything thing about you since conception had taught me so much about the heights of joy and the depths of despair.

I have been lifted out of the despair.

Only joy is left.

I will reunite with you on the plains of Heaven.

Woulda, coulda, shoulda

2009-06-09T23:19:00.000-07:00

My son you'd be 14 years old now. You'd be excited (and maybe a tiny bit scared) that middle school was almost over and that high school would begin for you in September.

If you took after your older brothers we'd be getting ready for All-Stars in Little League or perhaps you would have moved up to select baseball.

I cannot imagine you not being athletic like your mother. I loved swimming but competing in sports was not my way. I read all the books in the Children's part of the library by the time I was 11.

I began reading adult books. My mother did not censor me but I think she should have. Some of the books I read were complete trash. My mother read them first. That is the only reason I read them because they were easily available.

I remember discovering Steven King books with Salem's Lot. I was reading it at 3a.m. because it was summer in MI and too hot to sleep. It was a scary book about vampires and when a moth hit my window, I barely stifled the scream that was hidden in me. Then I laughed. How silly. It was fiction.

Maybe you would have liked tennis. Your dad was good enough to play the pro circuit, he just did not how to get sponsorship. He was not strong willed enough to defy his father at that point in his life. He was an incredible player.

You would have very limited use of a cell phone(no texting) and a computer. Sorry Bren but your brothers taught me the hard way. You'd whine but they get more time...........blah, blah, blah and I'd say sorry Charlie no go.

I can't imagine you being a shy child as shy doesn't exist in my family history. My mother told me she was a shy child but my brothers weren't shy and I am not shy and your brothers are anything but shy.

I still think of you and miss you just not 24/7 so much. Nursing school takes tons of time and helping people helps me to feel useful.

The Darkest Night

2008-12-18T22:24:00.000-08:00

My baby boy, how I miss holding you in my arms, against my chest. I miss hearing you breath, I miss feeling the weight of you as I rocked.

Some days are dark with loneliness and Christmas time is particularly difficult. I cannot get into the spirit even almost 13 years after you slipped away to heaven.

I have fallen into the darkness again. You'd think I would have a handle on this but it just doesn't work that way.

Maybe I am psychologically dysfuctional. They call it dysfuctional grieving in my nursing diagnosis list. Phatttttttttttttttttttttttttttttttttttttttttttt to them. No parent ever stops grieving for their lost children. No one.

Sad

2008-06-15T23:57:00.000-07:00

I am sad in a way most people could not understand. I wanted four kids. I loved being pregnant and having babies. With us carrying a genetic disease my hopes for another baby nearly dissipated. There was always the possibility of artificial inseimination by donor or invitro with healthy embryos. These ways have been beyong us financially.

I sad to give up hope. I'm sad to be aging out of the getting pregnant age. I'm sad to see my options wither away.

I am having such a difficult time accepting I'll never have a newborn of my own again. Being a grandparent is so NOT the same.

I don't kow what to do but go one with my life pushing through each day and hoping this sadness slowly dwindles away.

Alone

2008-05-31T23:38:00.000-07:00

I am alone again. Your brothers are out and your father is asleep and I sit here by myself. If you were here you'd most likely be asleep as well.

Sometimes being so alone hurts. It causes pain beyond belief. This really has nothing to do with you except after you died and your grandmother died I slipped into the abyss of depression.

No one's fault, it was the way my mind and body reacted to unthinkable agony. I have never been as much pain as I was in watching you suffer and die. You had no control over what happened and I wanted so badly to take the pain from you. I couldn't. I was helpless. I had no control over what you went through.

I am so sorry you had to suffer. No infant should have to go through what you went through.

Familes should not be changed, transfigured by a child's life, illness and death.
I would have gladly given my life to save yours.

I don't understand why terrible things happen. I will never comprehend why you had to suffer and die.

I still love you. I still miss you. I will always love and miss you. Part of me will always be alone without you.

Suffering Sweetly

2008-05-13T18:05:00.000-07:00

I have no answers. It makes no sense to me. Why did you, an innocent infant, have to suffer? Because You were my son, I felt your suffering acutely. I don't understand why any child has to suffer.

In Myanmar many thousands of children drowned or were blown away on high winds or hit by debris. Because of some stupid politically paranoid government, the children who survived may die of disease, or lack of water and food. Why don't people let others in the country to save the children?

In China a 7.9 earthquake killed thousands of children. They were crushed beneath buildings as the quake shook the roofs loose. Babies crushed in their cribs or with their parents in their homes.

Even thou you suffered my baby boy I was able to rock you and hold you until you took your very last breath. In some ways we were very lucky. We spent the vast majority of your ten months, 11 days together. You died in an embrace of love.
I was blessed to be holding you and you left this world and went on to the next. I'll be seeing you there some day.

Mother's Day Missing You

2008-05-12T21:49:00.000-07:00

On your first mother's day you were 6 weeks old. You had no idea what day it was, but that was fine. I knew what day it was. My first mother's day with three children. I had you, my sweet little baby, Riley who was a 5yo boy full of energy, and Keenan who was 7 and half and thought you were one cool baby.

It was also your last mother's day. I was blissfully unaware of that information.

Yesterday your father told me I wasn't his mother. He did not buy me a card or do anything except say Happy Mother's Day. Your 17 year brother Riley did not even bother to do that.

I still feel your dad deserves props for being a dad, even thought he isn't my dad. He is half the dna in all three of you. He was present whe you, your brothers and the the babies we lost to miscarriages were conceived.

He's been through the toddler years, the soccer years, the baseball years, the junior high years and the high school years. I respect him as your father.

I feel like your father diminished my role as a mother. Like now I'm irrelevent since Keenan and Riley are in their late teens. Maybe he doesn't feel I do any mothering any longer.

I miss you Brennan and wish you could have been with me yesterday.

Feeling A Little Down

2008-05-05T22:16:00.000-07:00

I have been pretty well acceptance and depression wise. In fact, I've been mostly even keeled which has been nice. When one has two teens and a husband secreting testosterone, it helps to be even keeled.

The Fantasy post has another side/The nightmare

If our first child had been born afflicted and began seizures at 5 months, his brain shrinking 25% on 3 weeks, losing his voluntary movement, etc we would have never had any biological children. A 1 in 4 risk is just too high for an always fatal disease. Because of the greater plan made by a greater power, I did get to feel the joy of creating children, carrying children, giving birth, nursing them, being head over heels on love with each of my sons.

We were blessed to have had that. I am grateful for that.

I always wanted children and don't know how I would have reacted if the oldest had been the one with disease. I'm glad I did not have to find out.

God is good. Sometimes my vision is cloudy.

Fantasies

2008-05-03T23:08:00.000-07:00

I don't do this often but every now and again I think what my life would be like if Brennan had been a normal happy boy. He'd have a younger brother, number one. I wanted 4 children back in the day so I figure we'd have a 19yo, a 17yo, Bren would be 13years old and Kieran would be 10. Yes, Kieran was my next boy name.

Bren would be on the Meeker Middle school Baseball, jv, cuz he;d be in 7th grade. Kieran would be in 5th grade and on a Little League unless he decided to be very different from his bothers and decided to play spring soccer.

Bren and Kieran would be pals but mostly at home because Brennan is, after all, in middle school and not an elementary school baby anymore. Bren would tease Kieran and then one of the older boys would jump him for being mean. At 13 Brennan would only be 5 feet tall so both his older brothers would be much larger.

Brennan and Kieran would be my readers. They would love to read on their own exploring the imaginative world in books. They'd also love computer games but there would be a time limit on that.

Kieran would be struggling with long division as everyone in the house had, and Brennan would try(after I asked him nicely)to help.

Riley would have his driver's license because I'd need the help. There's be practices and games to get to and with 4 kids things get complicated.

I believe Brennan would be more thoughtful than his older brothers.(not hard) He'd also worship them. Kieran would still be his mama's boy snuggly and caring.

If only we got to plan our lives...............................

A Little Easier To Comprehend

2008-03-29T11:21:00.000-07:00

Hailey's Wish
A Ray of Hope

1. What are mitochondria?

Mitochondria (plural for mitochondrion) are sometimes described as cellular “power plants” because among other things, mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. In addition to making energy, mitochondria are also deeply involved in a variety of other activities, such as making steroid hormones and manufacturing the building blocks of DNA.

2. What is a mitochondrial disease?
Mitochondrial diseases result when your body’s mitochondria fail to function properly. Your body’s organ systems are severely affected when your mitochondria can’t provide the energy needed to keep them running. Some parts of the body that require the most energy to function are: the brain, heart, liver, skeletal muscles, kidneys, endocrine and respiratory systems.

3. What are some symptoms of a mitochondrial disease?
Given the fact that mitochondria are responsible for fueling nearly all of the body’s energy needs, there is a long list of symptoms depending on which organ or tissue is having the “energy crisis.” When symptoms arise from three or more organ systems a mitochondrial disease should definitely be considered.

poor growth (failure to thrive)
muscle weakness, poor coordination
sensory (vision, hearing) problems
reduced mental functions
disease of the organ (heart, liver)
dementia
respiratory problems
hypoglycemia
apnea
lactic acidosis
seizures
gastro-intestinal disorders and swallowing difficulties
developmental delays
movement disorders (dystonia, muscle spasms, tremors, chorea)
stroke
diabetes
brain atrophy
4. How common are mitochondrial diseases?
It is now said that mitochondrial diseases are nearly as common as childhood cancer! One in 4,000 children born in the United States every year will develop a mitochondrial disorder by age 10. In adults, many diseases of aging have been found to have defects of mitochondrial function. These include, but are not limited to, type 2 diabetes, Parkinson’s disease, atherosclerotic heart disease, stroke, Alzheimer’s disease, and cancer. In addition, many medicines can injure the mitochondria.

5. When do doctors suspect that a person may have a mitochondrial disorder?

Different symptoms indicative of mitochondrial diseases may present over time. As patients begin to present with multiple catastrophic symptoms it can be hard for physicians to pinpoint the main cause. It is crucial that they never remove or set aside a symptom to try and diagnose the problem. All symptoms must be given consideration.

Mitochondrial disease should be suspected when:

A “common disease” has atypical features that set it apart from the pack
Three or more organ systems are involved
Recurrent setbacks or flare ups in a chronic disease occur with infections

6. Is there a cure for mitochondrial diseases?
There is not a cure for mitochondrial diseases. Doctors must look at each patient on a case-by-case basis and try to develop a treatment plan. Treatment may involve special diets and/or a combination of vitamins, and reducing any stress on the body.

7. How is a mitochondrial disease inherited?
The types of mitochondrial disease inheritance include:

Nuclear DNA (DNA contained in the nucleus of the cell) inheritance. Also called autosomal inheritance.

— If this gene trait is recessive (one gene from each parent), often no other family members appear to be affected. There is a 25 percent chance of the trait occurring in other siblings.
— If this gene trait is dominant (a gene from either parent), the disease often occurs in other family members. There is a 50 percent chance of the trait occurring in other siblings.
MtDNA (DNA contained in the mitochondria) inheritance.

— There is a 100 percent chance of the trait occurring in other siblings, since all mitochondria are inherited from the mother, although symptoms might be either more or less severe.
Combination of mtDNA and nDNA defects:

— Relationship between nDNA and mtDNA and their correlation in mitochondrial formation is unknown
Random occurrences

— Diseases specifically from deletions of large parts of the mitochondrial DNA molecule are usually sporadic without affecting other family members disease

8. What is Alpers?
Alpers’ Syndrome is a disease of the brain and liver. There are 3 classical symptoms of Alpers’ Syndrome. These are: 1) seizures that are very difficult to treat and have a focal component, 2) episodic psychomotor regression or dementia (loss of developmental milestones, often associated with common childhood infections),
3) liver disease. The children are born and develop normally for a period of time in virtually every case. Symptoms begin between the first few weeks of life and about 25 years of age. Two-thirds of the cases begin to show symptoms of seizures, or episodic loss of developmental milestones, within the first 2 years of life. The liver disease is often subclinical in the early stages of disease, but can appear at any time as acute liver failure.

Alpers’ Syndrome is a recessive genetic disease with a frequency of about 1:250,000 live births. Many cases die before an accurate diagnosis is made, so the true frequency is still an estimate. Alpers is caused by inheriting two copies of the POLG gene that are dysfunctional. These are called mutant copies. The function of the POLG gene is to copy mitochondrial DNA. In Alpers’ Syndrome, POLG is defective, so after a period of time, the amount of mitochondrial DNA in the cell falls below a critical threshold of about 35% of normal. When this happens, the mitochondria become sick, and begin to misfire. This leads to the brain and liver disease of classical Alpers’ Syndrome.

References:
Alpers Syndrome—A Summary for Families
Robert K. Naviaux, MD, PhD
www.clevelandclinic.org
www.umdf.org
www.mdausa.org
www.mindbodyfocused.com

Information provided is intended for educational purposes only and should not be construed as advising or diagnosing or treatment of this or any other medical condition.

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What They Think Brennan Had

2008-03-29T11:11:00.000-07:00

Here's a lovely scientific description of the disease they assumed my baby had. They never did any genetic testing(esp back in 1995)because of the cost.

All Databases PubMed Nucleotide Protein Genome Structure PMC OMIM

#203700 GeneTests, Links
ALPERS DIFFUSE DEGENERATION OF CEREBRAL GRAY MATTER WITH HEPATIC CIRRHOSIS

Alternative titles; symbols

ALPERS PROGRESSIVE INFANTILE POLIODYSTROPHY
ALPERS SYNDROME
ALPERS-HUTTENLOCHER SYNDROME
NEURONAL DEGENERATION OF CHILDHOOD WITH LIVER DISEASE, PROGRESSIVE; PNDC
Gene map locus 15q25

TEXT

A number sign (#) is used with this entry because Alpers syndrome can be caused by mutation in the nuclear gene encoding mitochondrial DNA polymerase gamma (POLG; 174763).

DESCRIPTION

Alpers syndrome is usually characterized by a clinical triad of psychomotor retardation, intractable epilepsy, and liver failure in infants and young children. Definitive diagnosis is shown by postmortem examination of the brain and liver (Harding et al., 1995).

CLINICAL FEATURES

The illness usually begins in early life with convulsions. A progressive neurologic disorder characterized by spasticity, myoclonus, and dementia ensues. Status epilepticus is often the terminating development.

Bernard Alpers (1931) described the neuropathology and clinical features in a 4-month-old girl with a one-month illness characterized by intractable generalized seizures. He termed the disorder 'diffuse progressive degeneration of the gray matter of the cerebrum.' Ford et al. (1951) described a brother and sister with a similar disorder. (See myoclonic epilepsy (254800) for reference to the same cases reported by Morse.) Familial cases were also reported by Palinsky et al. (1954) and Christensen and Hojgaard (1964).

Alberca-Serrano et al. (1965) reported a family in which 4 of 6 sibs were affected with spastic diplegia due to anoxic encephalopathy, which they termed 'Alpers' syndrome. The parents were unrelated. Several relatives of the father may have had the same disorder. All affected members had reacted to infections with violent convulsions. The authors suggested that this represented a familial susceptibility and that the cerebral damage was secondary to anoxia.

Blackwood et al. (1963) described 2 sibs in whom diffuse cerebral degeneration (Alpers disease) was associated with cirrhosis of the liver. Wefring and Lamvik (1967) described brother and sister who developed convulsions at ages 11 and 14 months, followed by progressive hypotonia, dementia and jaundice 4 and 2 weeks before death at the ages of 15 and 20 months. In addition to the typical findings of Alpers disease, the liver showed extensive atrophy with fibrosis, inflammation and bile duct proliferation. The diagnosis was made at autopsy.

Sandbank and Lerman (1972) reported 3 sibs with Alpers disease, characterized by progressive mental retardation, seizures, rigidity, and degeneration of the cerebral cortex. Neuropathologic examination showed disorganization of the cerebral cortex with neuronal loss and astroglial proliferation. There were abnormal mitochondria of variable sizes, some with electron dense inclusions. The authors suggested autosomal recessive inheritance.

Huttenlocher et al. (1976) reported 2 sibships with 2 affected children in each. Clinical features included early onset (average 2 years) of delayed motor development, vomiting, multifocal seizures, status epilepticus, stupor, hypotonia, paralysis, increased CSF protein, and later onset of hepatic disease. Intermittent, unexplained fever occurred frequently. None of the children survived beyond age 3 years. Pathologic examination showed degeneration of the cerebral gray matter with loss of neurons and reactive astrocytosis in the brain and fatty accumulation and cirrhosis in the liver. The authors rejected the idea of anoxic encephalopathy and suggested that the syndrome was a familial disorder with autosomal recessive inheritance. Huttenlocher et al. (1976) noted that hepatic involvement was absent in some cases reported earlier, including the case reported by Alpers (1931).

Harding (1990) reviewed the clinical, neurologic, electrophysiologic, and histopathologic features of Alpers syndrome in 32 patients. Birth was usually normal, with some developmental delay in infancy, often with hypotonia and bouts of vomiting. The seizure disorder usually had an abrupt onset and although clinical signs of liver disease often appeared later, biochemical evidence of liver disease was sometimes present before the onset of seizures. EEG and visual evoked potentials were abnormal. Most patients died before the age of 3 years. Less frequently, late presentation occurred, even up to 25 years of age. Some patients also had visual disturbances. Liver pathologic findings, including fatty changes, abnormal bile duct architecture, and fibrosis, were unrelated to anticonvulsant therapy. Neuropathology showed severe cortical neurodegeneration and astrocytosis. In 12 of the 26 families in their series, 2 or 3 sibs were affected, including one pair of twins.

Frydman et al. (1993) reported the cases of 8 patients from 2 families. Onset in the first family was prenatal; in the 4 patients who were examined, severe microcephaly, intrauterine growth retardation, and typical manifestations of fetal akinesia, including retrognathia, joint limitations, and chest deformity, were found. The second family presented with an early infantile form. All of the affected offspring had micrognathia and 1 had findings of fetal akinesia, comparable to those seen in the other family. Microcephaly was mild at birth and progressed with age. Refractory neonatal convulsions, swallowing difficulties, and pneumonia complicated the clinical course of patients in both families, and all of the infants died before age 20 months. Comprehensive biochemical and metabolic studies in both families yielded normal results, and the diagnosis was supported by demonstration of extensive progressive brain atrophy on computerized tomography and typical histologic findings; for example, the parietal cortex showed spongy state with focally accentuated severe loss of neurons. The cerebellar cortex showed severe loss of almost all granular cells and persistent Purkinje cells. Anomalies of dendritic arborization were also seen. Both families were of Israeli Arab ethnicity and the parents were first cousins in both cases.

Harding et al. (1995) reported the unusual cases of 2 unrelated girls, aged 17 and 18, with a progressive encephalopathy, visual signs and symptoms, multiple types of drug-resistant seizures, and liver failure. Brain imaging showed lesions in the occipital lobe, and EEG showed slow waves with polyspikes. Both patients had a rapid degenerative course and died within 8 months of onset.

OTHER FEATURES

Cases with a disturbance in pyruvate metabolism and NADH oxidation (10,9:Gabreels et al., 1981, 1984) have been described.

In a patient with mtDNA depletion and Alpers syndrome, Naviaux et al. (1999) found global reduction in respiratory chain complex I, II/III, and IV activity and deficiency of mitochondrial DNA polymerase gamma activity.

Gauthier-Villars et al. (2001) confirmed the mitochondrial respiratory chain abnormalities in the liver of 4 unrelated patients with Alpers syndrome. One patient had a complex I deficiency, another a complex IV deficiency, and 2 had a combined deficiency of complexes I and IV.

MOLECULAR GENETICS

Naviaux and Nguyen (2004) reported 3 patients with Alpers syndrome who were homozygous for a mutation (E873X; 174763.0008) in the POLG gene. They later published a correction (Naviaux and Nguyen, 2005) stating that 2 affected patients from 1 family with Alpers syndrome were compound heterozygous for 2 mutations in the POLG gene: E873X and A467T (174763.0002). Naviaux and Nguyen (2005) stated that the existence of a common 4-bp insertion in the POLG gene yielded the incorrect initial results. The clinical features of the family had been described by Naviaux et al. (1999).

In 4 patients with Alpers syndrome, Davidzon et al. (2005) identified compound heterozygosity for 2 mutations in the POLG gene (174763.0006 and 174763.0013). Liver biopsies from 3 patients showed mitochondrial DNA depletion ranging from 87 to 94%, and all 4 patients showed decreased activity of mtDNA-encoded respiratory chain complexes.

Ferrari et al. (2005) identified mutations in the POLG gene in 8 patients with Alpers syndrome.

GENOTYPE/PHENOTYPE CORRELATIONS

Nguyen et al. (2005) reported a child with Alpers syndrome who was homozygous for the A467T mutation. Unlike other children with the disorder, he showed late-onset at age 8.5 years and death by age 9 years.

HISTORY

As noted by Harding (1990) in a review of Alpers syndrome, there was much confusion in the past regarding the nosology, pathogenesis, and diagnosis of the disease. Some reported cases seemed to be caused by anoxia at birth or illness, whereas others were familial with normal births. Cerebral damage was also thought to be a result of intractable seizures or hepatic toxicity, and hepatic damage was believed in some cases to be caused by anticonvulsive drugs.

REFERENCES

1. Alberca-Serrano, R.; Fabiani, F.; Deneve, V.; Macken, J. :
Familial spastic diplegia due to anoxic encephalopathy (Alpers). A contribution to the study of vascular fragilities of the nervous system of genetic type. J. Neurol. Sci. 2: 419-433, 1965.
PubMed ID : 5878525

2. Alpers, B. J. :
Diffuse progressive degeneration of gray matter of cerebrum. Arch. Neurol. Psychiat. 25: 469-505, 1931.

3. Blackwood, W.; Buxton, P. H.; Cumings, J. N.; Robertson, D. J.; Tucker, S. M. :
Diffuse cerebral degeneration in infancy (Alpers' disease). Arch. Dis. Child. 38: 193-204, 1963.

4. Christensen, E.; Hojgaard, K. :
Poliodystrophia cerebri progressiva infantilis. Acta Neurol. Scand. 40: 21-40, 1964.
PubMed ID : 14108415

5. Davidzon, G.; Mancuso, M.; Ferraris, S.; Quinzii, C.; Hirano, M.; Peters, H. L.; Kirby, D.; Thorburn, D. R.; DiMauro, S. :
POLG mutations and Alpers syndrome. Ann. Neurol. 57: 921-924, 2005.
PubMed ID : 15929042

6. Ferrari, G.; Lamantea, E.; Donati, A.; Filosto, M.; Briem, E.; Carrara, F.; Parini, R.; Simonati, A.; Santer, R.; Zeviani, M. :
Infantile hepatocerebral syndromes associated with mutations in the mitochondrial DNA polymerase-gamma A. Brain 128: 723-731, 2005.
PubMed ID : 15689359

7. Ford, F. R.; Livingston, S.; Pryles, C. V. :
Familial degeneration of the cerebral gray matter in childhood with convulsions, myoclonus, spasticity, cerebral ataxia, choreoathetosis, dementia, and death in status epilepticus. Differentiation of infantile and juvenile types. J. Pediat. 39: 33-43, 1951.
PubMed ID : 14851183

8. Frydman, M.; Jager-Roman, E.; de Vries, L.; Stoltenburg-Didinger, G.; Nussinovitch, M.; Sirota, L. :
Alpers progressive infantile neuronal poliodystrophy: an acute neonatal form with findings of the fetal akinesia syndrome. Am. J. Med. Genet. 47: 31-36, 1993.
PubMed ID : 8368248

9. Gabreels, F. J.; Prick, M. J.; Trijbels, J. M.; Renier, W. O.; Jaspar, H. H.; Janssen, A. J.; Slooff, J. L. :
Defects in citric acid cycle and the electron transport chain in progressive poliodystrophy. Acta Neurol. Scand. 70: 145-154, 1984.
PubMed ID : 6439001

10. Gabreels, F. J. M.; Prick, M. J. J.; Renier, W. O.; Willems, J. L.; Trijbels, J. M. F.; Ter Laak, H. J.; Jaspar, H. H. J.; Slooff, J. L.; Van Haelst, U. J. G. M.; Sengers, R. C. A. :
Progressive infantile poliodystrophy (Alpers' disease) associated with disturbed NADH oxidation, lipid myopathy and abnormal muscle mitochondria.In: Busch, H. F. M.; Jennekens, F. G. I.; Scholte, H. R. : Mitochondria and Muscular Diseases. Beetsterzwaag, The Netherlands: Mefar (pub.) 1981. Pp. 165-171.

11. Gauthier-Villars, M.; Landrieu, P.; Cormier-Daire, V.; Jacquemin, E.; Chretien, D.; Rotig, A.; Rustin, P.; Munnich, A.; de Lonlay, P. :
Respiratory chain deficiency in Alpers syndrome. Neuropediatrics 32: 150-152, 2001.
PubMed ID : 11521212

12. Harding, B. N. :
Progressive neuronal degeneration of childhood with liver disease (Alpers-Huttenlocher syndrome): a personal review. J. Child Neurol. 5: 273-287, 1990.
PubMed ID : 2246481

13. Harding, B. N.; Alsanjari, N.; Smith, S. J. M.; Wiles, C. M.; Thrush, D.; Miller, D. H.; Scaravilli, F.; Harding, A. E. :
Progressive neuronal degeneration of childhood with liver disease (Alpers' disease) presenting in young adults. J. Neurol. Neurosurg. Psychiat. 58: 320-325, 1995.
PubMed ID : 7897414

14. Huttenlocher, P. R.; Solitare, G. B.; Adams, G. :
Infantile diffuse cerebral degeneration with hepatic cirrhosis. Arch. Neurol. 33: 186-192, 1976.
PubMed ID : 1252162

15. Naviaux, R. K.; Nguyen, K. V. :
POLG mutations associated with Alpers syndrome and mitochondrial DNA depletion. (Letter) Ann. Neurol. 58: 491 only, 2005.
PubMed ID : 16130100

16. Naviaux, R. K.; Nguyen, K. V. :
POLG mutations associated with Alpers' syndrome and mitochondrial DNA depletion. Ann. Neurol. 55: 706-712, 2004.
PubMed ID : 15122711

17. Naviaux, R. K.; Nyhan, W. L.; Barshop, B. A.; Poulton, J.; Markusic, D.; Karpinski, N. C.; Haas, R. H. :
Mitochondrial DNA polymerase gamma deficiency and mtDNA depletion in a child with Alpers' syndrome. Ann. Neurol. 45: 54-58, 1999.
PubMed ID : 9894877

18. Nguyen, K. V.; Ostergaard, E.; Ravn, S. H.; Balslev, T.; Danielsen, E. R.; Vardag, A.; McKiernan, P. J.; Gray, G.; Naviaux, R. K. :
POLG mutations in Alpers syndrome. Neurology 65: 1493-1495, 2005.
PubMed ID : 16177225

19. Palinsky, M.; Kozinn, P. J.; Zahtz, H. :
Acute familial infantile heredodegenerative disorder of the central nervous system. J. Pediat. 45: 538-545, 1954.
PubMed ID : 13212595

20. Sandbank, U.; Lerman, P. :
Progressive cerebral poliodystrophy--Alpers' disease: disorganized giant neuronal mitochondria on electron microscopy. J. Neurol. Neurosurg. Psychiat. 35: 749-755, 1972.
PubMed ID : 4647849

21. Wefring, K. W.; Lamvik, J. O. :
Familial progressive poliodystrophy with cirrhosis of the liver. Acta Paediat. Scand. 56: 295-300, 1967.
PubMed ID : 6033104

CONTRIBUTORS

Cassandra L. Kniffin - updated : 2/15/2007
Cassandra L. Kniffin - updated : 10/13/2005
Cassandra L. Kniffin - updated : 8/31/2005
Cassandra L. Kniffin - reorganized : 8/15/2003
Victor A. McKusick - updated : 8/8/2003

CREATION DATE

Victor A. McKusick : 6/2/1986

EDIT HISTORY

wwang : 2/21/2007
ckniffin : 2/15/2007
carol : 11/15/2005
ckniffin : 10/13/2005
wwang : 9/6/2005
ckniffin : 8/31/2005
tkritzer : 8/13/2004
ckniffin : 8/4/2004
ckniffin : 7/12/2004
mgross : 3/17/2004
carol : 8/15/2003
ckniffin : 8/15/2003
carol : 8/8/2003
alopez : 6/10/1997
mimadm : 11/12/1995
terry : 4/21/1994
warfield : 3/7/1994
carol : 9/1/1993
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Dear Brennan Who Never Got the Opportunity to Grow up

2008-03-28T21:08:00.000-07:00

My dearest son,

I'm 46. It's been 12 years since you were ripped from our family by a bizarrely rare incredibly odd disease. You lived 10 months and 11 days. I have no idea how you comprehended your small time with us. In spite of pain and guilt and extreme frustration; I am delighted I got to love and nurture and care for you during your short life.

I was ecstatic when I learned we were pregnant with you. I had a miscarriage a few months before that was traumatic in a physical manner but not so devastating emotionally. This time I got to see the baby I miscarried, so there was a final closure to the event. My attitude was OK, God felt there was an inborn error that meant that baby could not survive.

Your father and I waited 6 weeks and tried again. We were thrilled to be trying to create you with the help of God. It never mattered if you were female or male, we just wanted a healthy infant to love, to show the product of our love, and to enjoy parenting. We relished raising your brothers at 6 and 4. They were more of a joy than work and they were loving, snuggly, loving children. I was fabulously happy being a full time mother and wife.

So when we got the news you were coming we were happy, slightly leery but happy. The pregnancy was different from my others. I chose a midwife. I didn't have a happy energized pregnancy with you as I did with Riley. I was more tired with you being 32 at the time. I had a child in 1st grade and one in preschool plus soccer for both.

My hair went flat ad lifeless for the first time ever-thanks. I had very little nausea or headaches as I had suffered through with your brothers. I was more tired.
Kinda klutzy as I fell through the stairs at Charmaine's. Nice bruises on my arms and knees

I fell another time in the middle of the night when Ry went pee in his bathroom but it was clear and I did not see it. WHAMMO went my 7 months pregnant body. I twisted so the brunt of the fall affected my back and upper side. I did need to call your dad to help me get up. I was fairly unwieldy by then (like a beached whale).

There was the exciting(yeah right) time Riley was sleeping bed with me and you inside me and he threw up all over my back and hair. Once again, I screamed for Daddy for help. You, I and Ry took a nice warm shower and Daddy did the laundry. He was such a good daddy/husband.

I would lie in bed and feel you move. You never matched the level of the frenzy of your brothers but you did move. You were breech from early on. Your hard head was under my ribs, your ankles tickled your ears and you little butt was wedged firmly down in the pelvis. You would get the hiccups and I would grin internally because I know this meant you alive and most likely normal.

Your time inside me was a gift. It's a gift to create a life. I can not describe the radiance and peace I felt when you were inside me. You were our child but again, a child is a gift, leaving the future to be unknown. We were okay with that risk. After 2 healthy boys and 2 miscarriages, we understood(or we thought) we understood the risk to love outweighs the fears.

Pop Up Feelings

2008-03-14T22:18:00.000-07:00

Because of our move a whole 11 blocks south, I've been sorting and packing in the last 10 days or so.

I came upon my breast pump accessories, totally unexpected, and I lost it. I wept for over 30 minutes.

I wept for a life cut short.

I wept for a future denied.

I wept for the sweet boy I never got to see turn into a toddler let alone a teen.

Brennan Reed would have been 13 on March 22.

I wept for the suffering my innocent infant had to bear.

I wept, selfishly for me. For that family picture of all three boys in baseball uniforms lined up tallest to smallest. The picture that only existed in my mind.

I wept for the sadness I carry with me everywhere, even if it invisible to others.

I did not feel better or worse after I stopped crying. I just felt very much alone.

I miss him~always.

Feeling It

2008-01-25T23:36:00.000-08:00

After our traumatic trip to the ER last week plus the traumatic drug reaction that brought me as close to psychotic as I ever want to be, I'm feeling the loss.

This time 12 years ago Bren was very very sick. He tried to die several times but my female Christian friends came to sit deathwatch with me. Six women who cared so much for Brennan and me. Brennan refused to die.

I wasn't ready to let him go quite yet. I literally willed that kid to live. He slept on my chest, sitting up on the couch, every night. I had a double receiving blanket under his head because he drooled incessantly. The poor dear was unable to swallow. He was also unable to keep his mouth closed. The part of his brain that controlled those things was damaged. He was not paralyzed but had very little voluntary movement.

He would lie his head on my chest, listening to my heart that kept beating despite my pain. He would whimper when not comfortable and hum when I sang to him or played his lullaby tape(by Micheal Card). I would sing and talk to him until I was sure he slept. Then I would sleep right there, sitting up, feeling his small heart still beating.

He was on an oximeter to measure how much oxygen he was getting. He was being fed through an NG tube 18 hours a day. He needed to be suctioned out because he could not rid himself of mucus. This became a routine part of the day. I also gave him his medication through a porta-cath on schedule. He was on a med that was every 4 hours for awhile and I set the alarm to get up to do it.

He slept on my chest every night for the last three weeks of his life. I would catch naps when the home pediatric nurse was there. I did not want to be away from him because I knew soon he would be gone forever.

My poor little boy who never had a chance at life. Who would never grow up to drive me nuts as a 7th grader(where he'd be now). He never got to even crawl. He did not know what he missed. He took comfort in Geo, my women friends who loved to hold him, the noises he could hear from his brothers and from me. We were one in a way that is impossible to describe.

I miss him. I miss him as a baby. I miss him as he would have been the last 12 years if he had been healthy. I just miss him. I pray we will meet again in heaven one day.

A New Viewpoint

2008-01-20T01:47:00.000-08:00

This is not meant to be a put myself down or blame myself for my reactions to what we went through. I just had some scales fall from my eyes so I could see how those around me were affected by Brennan's death and the way I reacted.

I have done my family a disservice. These events did not just happen to Brennan and me. They happened to the entire family.

This is where our fairly happy and functional family started on the long road to dysfunctionality and my long pity party.

I have always thought of this as my tragedy. My baby died. That little boy who grew in my body, was fed from my body, was a physical part of me~it was my loss more than anyone's. Wrong. It was our family's tragedy and my martyr type behavior caused the cracks of dysfunction and anger that we are now trying to deal with.

I hugged my anger and my grief to myself for far too long. Anyone who tried to break that shell around me paid.

I think my biggest fear is that someone would know I was to blame for his death. Not rational but I was raised Catholic and our God was vengeful. Brennie was too young for God to be angry enough so in my self centered way, it must be my fault. Maybe Geo's fault. Someone had to be at fault. If no one was at fault, why did it happen?

I thought I was coping well, I lied to myself. I needed drugs to barely cope, I used the drugs to blunt my anger and grief, and that fact became obvious to me in the last few weeks.

Who helped Geo? Not me. I just chalked it up to men grieve differently and left him to twist in the wind.

I think I did okay with the kids except the after mother was not like the before mother. I still loved them, but my grief drowned them out many, many times.

I wallowed in my pool of martyrdom alone. I used people so that I could wallow without the kids around.

I had some great insights and I was completely blind.

I offered no comfort to my inlaws. I have no mercy for them. They were the enemy because I could feel what I thought to be my SIL's hostility. I thought she thought she could take better care of my kids.

Selfishly, I clung to my extreme anger not knowing who to be angry with.

Time to kick the martyr out of me.

Brennan was loved by many and I unfairly held him close to me.

Blessings

2007-10-27T00:23:00.000-07:00

In the midst of watching your infant die, the last thing one would think about is blessings. Well I did and I do. There were many mercies and blessings associated with Brennan's illness and death. I'm going to do a Janice list for this one.

Seeing the positive aspects of my youngest son's illness and death:

1. Brennan did not die the first time we ended up at Childrens Hospital. It was touch and go but he survived.

2. He wasn't very damaged by the first traumatic stay. He came home on phenobarb for his seizures but was still quite interactive when not wiped out from the barbiturates.

3. My mother came and visited, meeting Brennan for the first and last time, when he was still very cognizant.

4. He was home for over a week before we ended up in Childrens again.

5. The nurses and Dr. Deb who was in her last year of residency and was a friendly face from our first stay.

6. Brennan recognized me until the moment of his death.

7. Once we knew Brennie was going to die we got a private room which was soooooooo nice after being in rooms with 6-8 other babies.

8. The awesome glider rockers in which I rocked my infant for between 15-20 hours a day.

9. The 87 days we had with Brennan at home before he died. I cherish that time with him and loved him as much as if he lived to be 82 years old. I feel this time was a true gift from God.

10. The fact Brennan was never in a vegetative state and that he did not live for years that way. I think that would have been harder on our family.

11. The generosity and kindness of neighbors, friends, strangers and church members. It was very humbling to see how much others cared for our family, especially for Keenan and Riley.

12. My God given ability to rise to the occasion and be my very best Mom self when necessary.

13. I learned to be assertive without being obnoxious.

14. Brennan did not die on Riley's birthday but a week before. I am so grateful Riley's day was not turned into a sad event.

15. I got to hold my baby whenever I wanted and needed to. I needed to hold him often.

16. The circle of women friends who supported me and came to our house on death watch duty~just like in the days before modern technology. This was a wonderful example of what a community of women can do to help others. These women also truly loved Brennan.

This is what comes to mind right now. There were other blessings scattered around but these were the most important to me.

The Saga Continues

2007-09-30T23:13:00.000-07:00

That first night in NICU seemed to last 124 hours instead of 12 hours. The neonatalogist in charge that month, Dr. Straandjord took time to talk with me and explain any questions I might have. This doctor who was doing a quick Saturday round spent a half hour with me. He was so kind. I'll never forget how he made me feel. My baby was important, our family was important and we were people not case numbers. Dr. S made one of the best impressions as an attending of all the doctors we eventually went through. I will thank him profusely one day for taking 30 minutes away from his family to make me feel more comfortable and less scared.

Later, the attending neurologist, Dr.Snarl, came in with 6 of his residents. He wanted me to leave but I asked to stay. He grumbled I could say as long as I didn't interrupt his rounds (pompous ass). Bren was still drugged into an unconscious state and there were more tests ordered. This was the beginning of the collective minds of UW Medical School and Childrens Hospital scratching their heads and going, "HUH? This kid presents atypical and we be clueless." It would have been amusing if I still had a sense of humor, which I didn't at this point. The all followed their guru to see other patients.

If one has to go into a teaching hospital I have two pieces of adice: NEVER go in the month of July~all the interns and residents are brand new and clueless. Next try not to get admitted on the weekend. The special labs are slower, in fact all labs are slower but that's ok because most docs aren't in on the weekend.

I did use one of the small rooms on the third floor to store my stuff, try and sleep Saturday night(I fell asleep after 72 hours of being awake and there was fire drill at 2am.) I was sure God hated me now! They had strob lights in every room including my closet. Scared the hell out of me. I had gotten 45 minutes of sleep. That was all she wrote.

So much of this experience reminds me of how they torture prisoners; no sleep, lights on 24/7 in ICU, the monitors beeps constantly and it was live being trapped in a sesory deprovation tank but with light, noise and sound. There were no tvs in NICU so I was completely out of my usual newspaper, news shows, HNN world.

I'll be writing in here when I have the time and the stamina. The story will continue, just at random intervals. If the muse hits I'll be in here in a flash. Thanks to all who who read this. I heart you.

12 Years Ago at Exactly This Time

2007-09-15T22:57:00.000-07:00

I was sitting in a room in Infant(neonatal) ICU in Children's Regional and Medical Center of Seattle. I had spent my day watching my child come close to dying from intractable seizures. He was 5 months, 3 weeks old. Because of medical intervention Brennan Reed was on a ventilator and monitors in the Neonatal ICU. I was still wired from all the adrenaline that had been zooming around my body from 8am-midnight. I could not pull myself away from Brennie's side. They had a small room up a floor(a "call" room) I could use for 2 nights but I knew I wouldn't sleep. Who can sleep when your infant is in critical condition?

Not me. My mind kept going round and round like a merry go round who's off button was broken. It was making me slightly nauseous and dizzy but I could not stop my brain. I looked at my sweet little baby who was pale and covered with ivs and tubes and lead wires to the monitors. He looked immense next to the most common occupants in NICU which were preemies. My boy weighed around 15 lbs and had chubby little wrists and thighs. Preemies are known for their no fat look. The irony was my boy ended up being one of the sickest children in that NICU even if we did not know it. The vast majority of those preemies went home and lived. And are still living, hopefully going to school as 6th or 7th graders. I hope they are living the life Brennie never got to live.

Part of me could not comprehend the events of the day. It was as if I could force myself awake and this would all go away. No such luck.

The reason I had children was because I knew I could love them, take care of them, teach them, make them vital parts of our family and eventually send them out to make families of their own. In theory, I knew illness or an accident or SIDs could happen but I never considered those events for very long. It's too painful to even conjecture your child being ill or dead. As I spent the long night in the NICU, little unthinkable thoughts began to creep into my sub-conscious mind and bleed slowly into my conscious mind. This was shooting my whole super protective mommy self image all to hell. I wouldn't let that go yet. I shut those thought down. I clamped them with stainless steel and refused to visit until I was forced there weeks later.

Why kept running through my brain? I went over every minute of my pregnancy, delivery, his newborn habits, his "slightly slower that his older brothers' developmental curve". What had I missed? What had I done wrong? Why this baby? This baby was sweet and slightly fussier than his brothers had been, he did not deserve the pain he had gone through in the past 24 hours! No baby deserved this. WHY? Okay Lord, I've been praying and praying and yet this still keeps happening. WHY? Then a little more existential...what had I done wrong in my life that God was punishing this baby? (Catholic upbringing, I was taught God punished sinners, always no matter how long it took. That is why there was limbo and purgatory. They had not taught me about hell on earth, I learned that one on my own....cuz hell is where I lived for the next 5 months of Brennan's life and his death and the aftermath...)

A Precious Gift to Our Family

2007-09-11T22:22:00.000-07:00

This is Brennan's Oma who was lying on the bed with her grandson. He was a very smiley baby and loved to coo face to face especially with his mama. 7/1995
Keenan who is 7 and a half here in holding his 6 week old baby brother, the Brenster!!!
On the left is Riley fast asleep at 4 and Brennan was close to 3/4 months. Their profiles were identical. June 1995
Brennie with the leads super glued to his head. This was for a 6 hour EEG study. He face was swollen from IV fluids. Doesn't he look so teeny? My sweet boy with the disintegrating brain(we weren't aware of that fact yet.)

Not My Smartest Move

2007-09-10T12:03:00.000-07:00

Last night after I wrote my previous post, I went and got out my copy of Brennan's hospital chart. I haven't been ready or able emotionally to really look through this. But some of my recall is fuzzy on the med schedule and the exact dates in October-November when we were in the hospital.

Oh my Good Lord, that baby boy suffered. I will never ever understand what good comes from a sweet, pure, innocent baby suffering in such a harsh way. I have stopped asking why maybe 10 years ago because there is NO answer to that question. There is no one to blame for what happened to my youngest son, my most planned child. It was one of those things. No one hurt him, we had no idea we carried anything harmful and there aren't any screening tests so I can't blame us. I can mourn what that sweet sweet baby went through during the course of this terrible, nasty, life sucking disease.

I was never told this~SURPRISE! NOT!but Brennan never had anything close to a normal EEG. An EEG is when they measure the electricity going on in a brain. This poor child never had normal sleep waves. There are four stage of sleep on the brain. A waves(normal awake brain activity), B waves, Theta waves, Delta waves(deep sleep) and REM or dreaming sleep. My poor kid never had normal sleep waves on both hemisphere of his brain. The right side of his brain was messed up electrically from our first visit to Childrens. I read all the EEG reports(12 of them) and he was never normal. Shouldn't I have been told this? I get very angry when doctors think it's better not to tell the parents the details. This tends to be because the doctor doesn't want to define all the parameters. It's too complex for us normal folk. I feel like punching all the doctors who never explained the EEG results. I would have had a clearer picture earlier. That means my son could have spent less time in the hospital.

Every time they had to do an EEG on my 5-7 month old infant they GLUED(like in superglue) leads to my infants head. These left welts when removed, sometimes open sores. How nice that must have felt. This was done at least 12 times and several times he had the leads on for 12-24 hours. His skin began to break down from the leads and their remover, acetone(nail polish remover).

I wonder if his brain ever worked normally. My instincts told me there was something wrong from the time he was 6 weeks old. Everyone told me he was just a bit slower in development than his big brothers. BPB rolled over at 2 months old. BBB walked by 10 months. Brennan could roll one way by 5 months. His gross motor skills seemed behind. His fine motor skills were fairly normal. Not only have I had 3 children but I had taken many developmental classes and knew(still do) the parameters. Still, I was treated by everyone who thought I was imagining problems that didn't exist. Thanks guys. Have we forgotten who we were placating? Miss 160 IQ(exceptionally high in verbal skills, comprehnesion, and I had a somewhat photographic memory for social studies).

Brennan was breech and delivered by c-setion. This adorable little boy could never hold his head straight up. It was always crooked to the side. I was told that was because his head was under my ribs and was mostly muscle/tendon immaturity from placement in the womb. He was near to normal by four months except if he was tired, then to the side his head went.

I'd like to make something clear, they really couldn't have diagnosed him much earlier. He was diagnosed from his second MRI where it was clear his brain had shrunk 25% in 3 weeks. The first MRI during our first admission showed some slight abnormalities but that is probably true of any infant or adult. Our brains don't look like they do in the Anatomy book.

Being dismissed is what upset me. Until Geo heard the dx from an actual neurologist he felt I was being pessimistic. Um no. From the minute we walked into Childrens I knew Brennan had something major wrong with him. I would have loved to be wrong but I wasn't. My pediatrician apologized to me about his lack of concern and trust of my Mommy instincts. Too late mate!

More 9/15-16/1995

2007-09-10T00:17:00.000-07:00

I was rocking in that uncomfortable wooden rocking chair watching any action going on that I could see. The nurse on duty for Bren and the other teeny baby in room found me an extra TALL chair that the nurses used to write in charts. I used the tall, padded chair to sit higher up and therefore close to Brennie. He had not moved since they drugged him to the gills with anti-seizures meds and whatever else they threw at him to get him to stop seizing. I know they gave him phenobarbital, dilantin, more ativan and paraldehyde which are all anti-seizure meds. He had an IV attached to his chest catheter. He wasn't quite as pale as he was earlier. He looked quite small in his bed. He was attached to a monitor that kept tract of his heart rate, oxygen in the blood(measured by an oximeter that was on his big toe), blood pressure and I think that's it.

I spent quite a bit of time watching the blinking on the monitor.

I tried to pump breast milk so when he woke up they could feed it to him. This is one of the biggest stresses I put on myself. Trying to get enough milk so they would not have to supplement. In the next seven weeks, it never happened. I did not feel let down anymore and I only had letdown when he was sucking. So trying to get milk out in their little breast pumping room was the most frustrating thing. I could get an ounce or two but that wasn't enough. I stressed myself out about so much when I really didn't need the extra stress. I'm a huge believer in breast feeding with baby led weaning. My first weened at 17 months old, my second was 2 and Brennie was still breast feeding only when he got sick. He'd had some trouble with nursing for about a week before we ended up in the ER and that affected my milk supply as well. I felt like a complete failure when I couldn't produce 4-6 ounces at a time. I know he had been eating enough as he'd more than doubled his birth weight by 4 months old.

I'm sure some people won't understand but I kind of fixated on my milk as the magic healing food. Breastfeeding and being a good mother went together in my mind(for me only. My standards were for myself.) When I could not do this successfully, it made the entire experience even worse and somewhat personal.

I was raised Catholic and guilt and I had been good friends for quite some time. My mind was struggling with the fact my son was suffering. I wondered, why him? Why an innocent perfect baby was being punished? Why us? (This thinking changed within a week or so to why not us? Were we in some magic circle where nothing bad would ever happen to my kids. NOOOOOO. It just took some attitude adjustment time for me to get there.) I was harboring a great anger, mostly at God. It would bubble and brew for weeks before I blew.

All my guilt and fear of failure to be a "good" mother all coiled together in my subconscious mind. None of the parenting expereince is simple, I have found. Dealing with a sick child just quadruples the complexity. I had pretty harsh standards for myself and this emergency woke them up and mixed them with vague guilt and terrible almost paralyzing fear.

All the while, Brennie lay unnaturally still except for the respirator or when the nurse did something necessary.

9/15/95 continued

2007-09-09T18:19:00.000-07:00

While I was thinking my infant looked dead, we were walking with the resident, a nurse, the social worker, Geo, my friend and I, to the CT Scanner. If you have not seen a CT Scanner up close they are pretty large. They are HUGE next to a five month old baby who weighs 15 lbs. They slid him in and scanned his head. They did not seen anything unusual in the scan but would let the radiologist look at it and let us know.Next stop~Infant ICU aka NICU aka Neonatal Intensive Care.

They set Brennan and his IVs and respirator up in a room with only 2 beds. There were 2 other rooms in the ICU area, a huge room with 20 babies and a small room which held only one baby. The room we were in also held the ECMO machine(A heart/lung machine for critically ill babies awaiting surgeries usually). Brennan had a nurse just for him and one other baby that first night.McHub had to go home and deal with the older boys, his father who happened to be visiting(OY VEY!), and call my parents and his mom and sister. My friend C, needed to get home but promised to come back later. She had to take care of her boys and husband and would bring me supplies back, as I was not leaving my Brennie's side.

I settled in a rocking chair right next to Brennie's metal crib bed. I kinda rocked and stared at him. I cannot remember a single coherent thought going through my head. The resident who had asked me about Brennie's medical history came up and kept me company for awhile. Dr. Deb was 25, a 3rd year pediatric resident who went through med school at University of Michigan(My favorite all time college on earth). Even though she had not slept in 2 days, Dr. Deb stayed for over an hour, which helped me to calm down.

I was calm on the outside the entire time but inside I think my stomach was full of heavy lead and somewhere in the very far back in my mind, I knew this was all a very bad scene. As if a teeny black cloud floated above me and Brennie, I could feel a terrible knowing this was not going to have a happy ending.

I kept getting asked if I was hungry and was offered a voucher for a meal in the cafeteria. I wasn't hungry. I don't think I was hungry for the next 6 months. They did kick me out as per their policy. It was for 30 minutes every so many hours I think. I can't remember now. I wandered down the hallway with the names of children who had died there on glass plaques on the walls extending for seemingly forever. I mentally flinched. I could not look at those names. I could look out of every window I passed but I could not make myself even glance at those plaques(perhaps knowing my child's name would be on one someday?).

I was 33 when this happened. I looked about 20. No makeup, no sleep, hair short, like a boys. When I was in the crash room rocking and praying they had to ask who I was, none of them thinking I was old enough or hysterical enough to be the mom. I'm pretty sure I was wearing an oversized t-shirt and an old pair of my hubby's sweats. That was my usual thing to sleep in. Yup, I was dressed for success. Like I cared.

I skipped the cafeteria and wandered into the gift shop. I bought a People magazine. It was their worst dressed celebrity issue and that was a stretch for my attention span. I went back into ICU and settled back in my very uncomfortable rocking chair. The sun was setting because I remember the slant of light in the room. The very quiet room except for respirators, monitors and IV pumps.

I rocked and stared at my still, silent beautiful baby and brushed his hair back or held onto his leg. It made me feel better to feel the warmth of his body. No matter how high tech weird all the stuff going into him looked, underneath, it was my baby boy.

I did not allow my mind to settle on anything. I would not let it wander into the dangerous territory of what was wrong with my son and what did it mean. Nope, I refused to go there. At this point, I just wanted my baby to get better so I could take him home. So my wandered, within the limits I set for it.

It's That Time of Year Again...

2007-09-09T18:16:00.001-07:00

WARNING: This story is graphic and heartbreaking to most parents. It wasn't easy to write and it's not easy to read. I'll understand if you move on. :)

Twelve years ago on 9/15, my 5 month old son was having intractable seizures. Major, whole-body-jerking seizures. One of scariest things I've ever seen. I called my friend, the NICU nurse and off to the local ER we went. Brennan and I had been at that ER the night before because he wasn't acting "right". I just did not know what I was seeing~small, barely detectable seizures coming at a constant rate. He looked like he was smiling except for the drool. The doctor who checked him out that night treated me like a first time paranoid parent. He watched him for an hour off and on and sent us home. Bad decision, Doctor. Bad all around.

I spent the night wondering what was wrong with my baby. I know he was "off". He wasn't eating. (Duh, how can one suck during successive seizures). He wasn't sleeping though he seemed to doze. I dozed off at about 5am and when I woke up at 8am, I realized Brennan was still very, very off. His diaper wasn't wet. He still wasn't making eye contact. I felt foreboding music fill my head(seriously). Called my friend, jumped in the van and off we went.

He began the major seizures on the way. The local ER was in a word, clueless. They could not find a vein until my friend offered her skill. This kid was seizing his brains out, almost literally. I was standing by, praying that my child would be OK and feeling ill with a headache beginning to inch up from the back of my neck.

They found a vein in his head(yes his head). Poor baby was so out of it, he wasn't feeling anything. They got some ativan(sedative used as an anti-seizure med) on board, stopping the seizures for the moment. Next, was the ambulance ride to Children's Hospital, which actually knew what to do with a 5 month old. I had to ride in the front and couldn't really see Brennie in the back. I was scared witless and babbled to the driver. Some moronic people did not pull over even though the siren was on LOUD and the lights were flashing. If I could have moved things with my mind, the Asian couple in the U District would STILL be orbiting the moon.(I make mention they were Asian not out of prejudice but out of detail. This is how it plays in my head. This is how it has played in my head a zillion times. They were an elderly Asian couple who were confused by the ambulance in the intersection and the traffic.)

This entire time I was almost outside of my own head. It's happening. I feel it happening. My eyes see it happening. My brain kept screaming, "This is NOT happening!!" Almost as if I was in 2 dimensions at the same time. Or as if part of my brain was floating above myself, watching it all from above. Surreal can't even begin to describe how I felt.

The ambulance backed into Children's trauma bay. Brennan had started seizing again and they rushed him into the crash room. I was asked again about how my son came to be seizing at Children's. I told them basically what I have described here. The social worker asked me if I had been okay when they had sent us home the night before from the local ER. I vehemently shook my head. I told them I felt something had been off about Brennan for months, mostly the past few weeks. I knew he was off the past 18 hours. I went over this story in more detail several times. George(kid's dad) and my friend were there. They watched as the 7 doctors worked on Brennan. I was out of the way in a rocking chair praying my heart out. I had a really bad headache by now, but I ignored it.

September 15, 1995~the day my world broke into pieces, I just didn't know it yet.

They did a cut-down on Brennan's chest and poured 9 medications into him(seriously 9). He had to be placed on a respirator because the meds caused him to be unable to breath on his own. He lay on the big gurney, with several iv's going in, hooked to a respirator, as unconscious as one can be and still be alive. He looked dead. To me, he looked dead. That is how I remember it. It is still a crystal clear image in my head. Permanently imprinted on my hippocampus for all time. May be continued another time... (This is only the beginning of 5 month journey to my angel's death. This is 10 hours of a 48 hour emergency and 7 days in the ICU....)